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Ahmed M. A clinician's perspective on the new organ mesentery and non-vascular mesenteropathies. Front Physiol 2024; 15:1336908. [PMID: 39296517 PMCID: PMC11408482 DOI: 10.3389/fphys.2024.1336908] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2023] [Accepted: 07/17/2024] [Indexed: 09/21/2024] Open
Abstract
Mesentery was discovered as a new organ in 2017. It is a continuous membranous tissue from the duodenojejunal flexure to the anorectal junction. It has distinct anatomy, physiology, and disease states. Primary mesenteropathies include vascular and non-vascular diseases. Some of them are common, and some of them are rarely seen in clinical practice. Secondary mesenteropathies occur when infection or malignancy in another organ spreads to the mesentery. Each entity has specific diagnostic and treatment protocols. Increased awareness of different mesenteropathies and an understanding of their various presentations at different stages of life can help in early diagnosis and improved clinical outcomes.
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Mita J, Tada K, Kuboyama Y, Hiroshige S, Nakamura S, Takahashi J, Sakata K, Mizuuchi H, Oba T, Yoshizumi F, Iwaki K, Takeuchi H, Kajiyama K, Fukuzawa K. Liver metastases 2 years after resection of a very-low-risk duodenal gastrointestinal stromal tumor: a case report. Surg Case Rep 2022; 8:195. [PMID: 36214924 PMCID: PMC9550939 DOI: 10.1186/s40792-022-01551-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2022] [Accepted: 10/03/2022] [Indexed: 11/06/2022] Open
Abstract
Background Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors, but are the most common mesenchymal tumors of the gastrointestinal tract. The risk classification of GISTs is based on the tumor size, mitotic index, tumor site, and presence of tumor rupture. Recurrence in the very-low-risk group is extremely rare. We herein report a case of liver metastases 2 years after resection of a very-low-risk duodenal GIST. Case presentation A 57-year-old woman presented to the hospital for evaluation of melena. Esophagogastroduodenoscopy showed bleeding from the exposed blood vessels at the top of a submucosal tumor approximately 20 mm in size located in the second (descending) part of the duodenum, and the bleeding was controlled with electrocoagulation. A GIST was suspected, and the patient underwent wedge resection of the duodenum. The resected specimen contained a 16- × 12-mm (< 20-mm) white submucosal tumor composed of spindle cells with a mitotic count of 4 per 50 high-power fields, and a histologically negative margin was achieved. Immunochemical analysis revealed positive tumor staining for c-kit protein and alpha-smooth muscle actin and negative staining for CD34, desmin, and S-100 protein. Therefore, the tumor was diagnosed as a very-low-risk duodenal GIST based on the Fletcher classification and modified Fletcher classification (Joensuu classification). The postoperative course was uneventful, and the patient was discharged on postoperative day 11. At the follow-up visit 2 years postoperatively, contrast-enhanced computed tomography revealed liver tumors in S8 and S6 measuring 26 × 24 and 10 × 10 mm, respectively. Both lesions showed peripheral dominant hyperenhancement with hypoenhancement inside, indicating tissue degeneration within the tumors. These imaging findings closely resembled those of the duodenal GIST. Hence, the patient was diagnosed with liver metastases of GIST 2 years postoperatively. She was subsequently started on treatment with 400 mg of imatinib. At the time of this writing (2 months after diagnosis), the patient was clinically well and asymptomatic and was continuing imatinib therapy. Conclusions Recurrence of very-low-risk GISTs is extremely rare. Even a small GIST with low mitotic activity can never be considered completely benign, and long-term follow-up is necessary.
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Affiliation(s)
- Junya Mita
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Kazuhiro Tada
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Yusuke Kuboyama
- grid.416795.80000 0004 0642 5894Department of Pathology, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Shoji Hiroshige
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Shun Nakamura
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Junichi Takahashi
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Kazuhito Sakata
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Hiroshi Mizuuchi
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Taro Oba
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Fumitaka Yoshizumi
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Kentaro Iwaki
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Hideya Takeuchi
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Kiyoshi Kajiyama
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
| | - Kengo Fukuzawa
- grid.416795.80000 0004 0642 5894Department of Surgery, Oita Red Cross Hospital, 3-2-37 Chiyomachi, Oita-Shi, Oita, Japan
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Kersting S, Janot-Matuschek MS, Schnitzler C, Chourio Barboza DE, Uhl W, Mittelkötter U. GIST: Correlation of risk classifications and outcome. J Med Life 2022; 15:932-943. [PMID: 36188659 PMCID: PMC9514809 DOI: 10.25122/jml-2021-0110] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2021] [Accepted: 06/22/2022] [Indexed: 11/05/2022] Open
Abstract
In clinical practice, there are often discrepancies between the oncological prognosis of gastrointestinal stromal tumors (GIST) and the actual clinical course. This study aimed to check with our collective how reliably the current classifications (Miettinen, Fletcher) predict the prognosis of GIST and to evaluate whether an extension of the classifications by the parameter proliferation activity could make sense. This prospective study enrolled 58 patients who underwent surgery on GIST from 01/2006 to 12/2016. The postoperative course (curation, recurrence, progress) was correlated with the identified risk classification and the proliferative activity. Coincidences with other tumors were strikingly common in patients with GIST (43%). Based on the risk group assignment of GIST, no assessment of the probability of the occurrence of second neoplasia could be derived. Individual patients were under- or over-graduated concerning the assessment of biological behavior based on the standard risk classifications. The inclusion of proliferative activity did not allow for a more precise predictive power - neither to the risk of recurrence and metastasis nor to the development of a second neoplasia. The study showed that there is currently no parameter or logarithm that reliably predicts the biological behavior of GIST. Due to the frequency of coincidence of second neoplasia and (rare) distant metastases, for everyday clinical practice, appropriate staging diagnostic and regular follow-up care should also be used for benign GIST.
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Affiliation(s)
- Sabine Kersting
- Department of General Surgery, Christliches Klinikum Unna Mitte, Unna, Germany,Corresponding Author: Sabine Kersting, Department of General Surgery, Christliches Klinikum Unna Mitte, Unna, Germany. E-mail:
| | | | - Carina Schnitzler
- Department of General Surgery, Christliches Klinikum Unna Mitte, Unna, Germany
| | | | - Waldemar Uhl
- Department of General Surgery, St. Josef-Hospital Bochum, Ruhr-University Bochum, Bochum, Germany
| | - Ulrich Mittelkötter
- Department of General Surgery, Christliches Klinikum Unna Mitte, Unna, Germany
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Qu H, Xu Z, Ren Y, Gong Z, Ju RH, Zhang F, Kang H, Xu Y, Chen X. Recent Advancements in the Treatment of Rectal Gastrointestinal Stromal Tumor: In Era of Imatinib. Cancer Manag Res 2022; 14:1141-1152. [PMID: 35321404 PMCID: PMC8934706 DOI: 10.2147/cmar.s352860] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/22/2021] [Accepted: 02/18/2022] [Indexed: 12/30/2022] Open
Abstract
Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumor of the gastrointestinal tract, with an annual incidence of 10–15 cases per million. However, rectal GIST has a low incidence, accounting for approximately 0.1% of all rectal tumors. The treatment of rectal GISTs is still controversial and the relative unified guidelines and consensus opinions are inadequate. Treatment is based primarily on the clinical experience of the physician. The widespread application of neoadjuvant imatinib therapy allows diversification of treatment, especially in the choice of surgical methods. Herein, we reviewed the most recent literature and summarized the new progress in rectal tumor treatment, with the aim of providing patients with more systematic and individualized therapeutic strategies.
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Affiliation(s)
- Hui Qu
- Department of Hernia and Colorectal Surgery, The Second Hospital of Dalian Medical University, Dalian, 116023, People’s Republic of China
- Dalian Medical University, Dalian, People’s Republic of China
| | - ZhaoHui Xu
- Department of Hernia and Colorectal Surgery, The Second Hospital of Dalian Medical University, Dalian, 116023, People’s Republic of China
- Dalian Medical University, Dalian, People’s Republic of China
| | - YanYing Ren
- Department of Hernia and Colorectal Surgery, The Second Hospital of Dalian Medical University, Dalian, 116023, People’s Republic of China
| | - ZeZhong Gong
- Department of Hernia and Colorectal Surgery, The Second Hospital of Dalian Medical University, Dalian, 116023, People’s Republic of China
- Dalian Medical University, Dalian, People’s Republic of China
| | - Ri Hyok Ju
- Department of Hernia and Colorectal Surgery, The Second Hospital of Dalian Medical University, Dalian, 116023, People’s Republic of China
- Dalian Medical University, Dalian, People’s Republic of China
| | - Fan Zhang
- Department of Hernia and Colorectal Surgery, The Second Hospital of Dalian Medical University, Dalian, 116023, People’s Republic of China
| | - HaoNan Kang
- Department of Hernia and Colorectal Surgery, The Second Hospital of Dalian Medical University, Dalian, 116023, People’s Republic of China
- Dalian Medical University, Dalian, People’s Republic of China
| | - Yang Xu
- Department of Hernia and Colorectal Surgery, The Second Hospital of Dalian Medical University, Dalian, 116023, People’s Republic of China
- Dalian Medical University, Dalian, People’s Republic of China
| | - Xin Chen
- Department of Hernia and Colorectal Surgery, The Second Hospital of Dalian Medical University, Dalian, 116023, People’s Republic of China
- Correspondence: Xin Chen, Tel +86 17709872266, Email
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Mohamed AA, Al Zahrani SM, Mohamed SA, Qureshi AS. Massive Gastrointestinal Haemorrhage Unusual Presentation of Gastrointestinal Stromal Tumors of the Jejunum: Case Report and Literature Review. Cureus 2021; 13:e14266. [PMID: 33959448 PMCID: PMC8093106 DOI: 10.7759/cureus.14266] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/02/2021] [Indexed: 11/05/2022] Open
Abstract
Although gastrointestinal stromal tumors (GISTs) are rare tumors, they are the most common tumors of mesenchymal origin of the gastrointestinal tract. GISTs present with nonspecific clinical manifestation and they are discovered incidentally during endoscopic or radiological investigations. Massive life-threatening bleeding that requires urgent surgery is rare. We present a case of small bowel GIST that presented with massive lower gastrointestinal bleeding that required urgent surgical intervention.
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Affiliation(s)
- Abbas A Mohamed
- Department of General Surgery, National Guard Hospital, Al Madinah, SAU
| | | | - Sarah A Mohamed
- Department of General Surgery, University Hospital of Wales, Cardiff, GBR
| | - Ahmad S Qureshi
- Department of Intensive Care Medicine, National Guard Hospital, Al Madinah, SAU
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Suresh Babu MC, Chaudhuri T, Babu KG, Lakshmaiah KC, Lokanatha D, Jacob LA, Rudresha AH, Lokesh KN, Rajeev LK. Metastatic gastrointestinal stromal tumor: A regional cancer center experience of 44 cases. South Asian J Cancer 2020; 6:118-121. [PMID: 28975120 PMCID: PMC5615881 DOI: 10.4103/sajc.sajc_290_16] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/08/2023] Open
Abstract
BACKGROUND Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. Historically, a poor prognosis for metastatic disease has been reported with systemic chemotherapy. Significant advances have been made in the last decade, since the introduction of different tyrosine kinase inhibitors (TKIs). Unfortunately, even though the TKIs have been used for a long time, there are very few published data of the experience of TKI therapy in metastatic GIST from India. MATERIALS AND METHODS Patients diagnosed with metastatic GIST from January 2005 to October 2016 at our center, who received first-line therapy with imatinib 400 mg/day, were reviewed retrospectively. Patients' profile, response to treatment, toxicity of TKI therapy, time to progression, and survival were evaluated. RESULTS Of the 44 metastatic GIST patients, 23 (52.2%) were males. Median age at diagnosis was 48 years. The most common presenting symptom was an abdominal pain (52%), followed by weight loss (23%). Most frequently affected metastatic site was liver (57%), followed by peritoneum (16%), and lungs (4.5%). Metastases to both liver and peritoneum were found in 10 patients (22.5%). All patients were initially treated with imatinib at a dose of 400 mg/day. Disease stabilization was documented in 21 cases (48%), and 13 patients (29%) achieved a partial response. TKI therapy was well-tolerated in most cases. Median progression-free survival (PFS) was 26 months, and estimated median survival was 48 months. Patients with lung metastases have a significantly inferior median PFS and overall survival, in comparison to patients with other metastatic sites (P < 0.05). CONCLUSIONS Imatinib therapy was well tolerated and induced a sustained clinical benefit in more than half of the patients with metastatic GIST. Lung metastases seemed to be a poor prognostic factor in this patient population.
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Affiliation(s)
- M C Suresh Babu
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Tamojit Chaudhuri
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - K Govind Babu
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - K C Lakshmaiah
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - D Lokanatha
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Linu Abraham Jacob
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - A H Rudresha
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - K N Lokesh
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - L K Rajeev
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
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Ahmed M. Recent advances in the management of gastrointestinal stromal tumor. World J Clin Cases 2020; 8:3142-3155. [PMID: 32874969 PMCID: PMC7441252 DOI: 10.12998/wjcc.v8.i15.3142] [Citation(s) in RCA: 28] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/25/2020] [Revised: 05/26/2020] [Accepted: 07/14/2020] [Indexed: 02/06/2023] Open
Abstract
Gastrointestinal stromal tumor (GIST) is a rare but an important clinical entity seen in our clinical practice. It is the most common mesenchymal tumor of the gastrointestinal tract and most common malignancy of the small intestine. Although the exact prevalence of GIST is not known, the incidence of GIST has been increasing. GISTs arise from interstitial cells of Cajal. Most of the GISTs occur due to mutation in c-kit gene or platelet derived growth factor receptor alpha gene. 15% of GISTs do not have these mutations and they are called wild-type GISTs. Almost all GISTs express KIT receptor tyrosine kinase. Histologically, GISTs look like spindle cell tumors most of the time but they can be epitheloid or mixed type. The median size of GISTs varies from 2.7 cm to 8.9 cm. Clinically, patients with small GISTs remain asymptomatic but as the GIST size increases, patients present with various symptoms depending on the location of the GIST. Most of GISTs are located in the stomach or small bowel. Diagnosis is suspected on imaging and endoscopic studies, and confirmed by tissue acquisition with immunohistochemical staining. The aggressiveness of GISTs depends on the size, mitotic index and location. Surgical resection is the treatment of choice. But various endoscopic modalities of resection are increasingly being tried. Tyrosine kinase inhibitors are extremely useful in the management of large GISTs, unresectable GISTs and metastatic GISTs. Treatment options for metastatic GISTs also include radiotherapy, chemotherapy, hepatic artery embolization, chemoembolization and radiofrequency ablation.
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Affiliation(s)
- Monjur Ahmed
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Thomas Jefferson University, Philadelphia, PA 19107, United States
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Jung H, Lee SM, Kim YC, Byun J, Kwon MJ. A pictorial review on clinicopathologic and radiologic features of duodenal gastrointestinal stromal tumors. Diagn Interv Radiol 2020; 26:277-283. [PMID: 32558653 PMCID: PMC7360074 DOI: 10.5152/dir.2019.19432] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2019] [Revised: 10/30/2019] [Accepted: 11/20/2019] [Indexed: 12/17/2022]
Abstract
Duodenal gastrointestinal stromal tumors (GISTs) are rare, and studies on their management are not sufficient. Owing to the complex anatomy of the duodenum and pancreatic head, GISTs can be misdiagnosed as pancreatic head tumors. Surgical resection is the first treatment for localized duodenal GISTs; thus, noninvasive imaging is important for the diagnosis and treatment of GISTs. Computed tomography, magnetic resonance imaging and endoscopic ultrasonography findings can be helpful for the diagnosis of duodenal GISTs and can help differentiate GISTs from other adjacent tumors.
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Affiliation(s)
- Haerang Jung
- From the Department of Radiology (H.J. , S.M.L.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (Y.C.K.), Hallym University Dongtan Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (J.B.) Hallym University Kangnam Sacred Heart Hospital, Seoul, Republic of Korea; and the Department of Pathology (M.J.K.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea
| | - Sang Min Lee
- From the Department of Radiology (H.J. , S.M.L.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (Y.C.K.), Hallym University Dongtan Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (J.B.) Hallym University Kangnam Sacred Heart Hospital, Seoul, Republic of Korea; and the Department of Pathology (M.J.K.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea
| | - Young Chul Kim
- From the Department of Radiology (H.J. , S.M.L.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (Y.C.K.), Hallym University Dongtan Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (J.B.) Hallym University Kangnam Sacred Heart Hospital, Seoul, Republic of Korea; and the Department of Pathology (M.J.K.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea
| | - Jieun Byun
- From the Department of Radiology (H.J. , S.M.L.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (Y.C.K.), Hallym University Dongtan Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (J.B.) Hallym University Kangnam Sacred Heart Hospital, Seoul, Republic of Korea; and the Department of Pathology (M.J.K.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea
| | - Mi Jung Kwon
- From the Department of Radiology (H.J. , S.M.L.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (Y.C.K.), Hallym University Dongtan Sacred Heart Hospital, Gyeonggi-do, Republic of Korea; Department of Radiology (J.B.) Hallym University Kangnam Sacred Heart Hospital, Seoul, Republic of Korea; and the Department of Pathology (M.J.K.) Hallym University Sacred Heart Hospital, Gyeonggi-do, Republic of Korea
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Feng X, Yang Z, Zhang P, Chen T, Qiu H, Zhou Z, Li G, Tao K, Wang H, Li Y. Which size is the best cutoff for primary small gastric gastrointestinal stromal tumor? J Gastrointest Oncol 2020; 11:402-410. [PMID: 32399280 DOI: 10.21037/jgo.2020.03.08] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
Background The biological behavior of primary small gastric gastrointestinal stromal tumor (gGIST) is indolent. The cutoff size categorizing small gGIST continues to be controversial. To date, there is no consensus regarding whether it should be 1 cm, 2 cm, or another size. We aimed to find a new cutoff size. Methods Retrospective clinicopathological and prognosis data of patients with small gGIST from January 1998 to January 2015 were collected among five medical centers in southern China. Tumor size was divided into two groups: <1 cm (Mirco group) and 1-2 cm (Small group). We compared the clinicopathological index and prognosis between these two groups and identified a new cutoff size to define small gGIST. Results During this 18-year period, there were 276 patients with primary small gGIST treated at these five medical centers. The range of tumor size was 0.2-2.0 cm. The median tumor size was 1.0 cm. The range of the mitotic count was 0-70/50 high power fields (HPFs) with counts ≤5/50 HPFs in 259 patients (93.8%), 5< counts ≤10/50 HPFs in 7 patients (2.5%), and counts >10/50 HPFs in 10 patients (3.6%). The median follow-up time was 38 months (3-156 months). The 5-year overall survival rate was 98.7% in the entire group. Using Pearson correlation analysis, there was a positive correlation between the mitotic count and tumor size as a continuous variable (r=0.164, P=0.006). There were 137 patients in the Micro group and 139 cases in the Small group. In the Micro group, mitotic counts were ≤5/50 HPFs in 134 patients, 5< counts ≤10/50 HPFs in 0 patients, and counts >10/50 HPFs in 3 patients; mitotic counts in the Small group were counts ≤5/50 HPFs in 125 patients, 5< counts ≤10/50 HPFs in 7 patients, >10/50 HPFs in 7 patients. There was a statistically significant difference between these two groups (P=0.002); the Small group had more intermediate/high-risk cases. Using the receiver operating characteristic curve (ROC curve), we observed that 1.15 cm was the new cutoff size to separate low-risk cases and intermediate/high-risk cases (AUC =0.707, P=0.004, sensitivity =0.824, 1-specificity =0.429). Conclusions Primary small gGIST has a good prognosis; gGIST <1 cm can be regarded as benign tumors that only requires endoscopic ultrasonography (EUS) follow-up. The proportion of potential intermediate/high-risk disease is high for patients with 1-2 cm gGIST. These patients should be treated with caution and the tumors should be resected if necessary. These results indicate that 1.15 cm may be the new cutoff size to separate small gGIST from large gGIST, but further studies are needed for verification.
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Affiliation(s)
- Xingyu Feng
- Department of General Surgery, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou 510000, China
| | - Zifeng Yang
- Department of Colorectal Surgery, the Sixth Affiliated Hospital of Sun Yat-sen University, Guangdong Provincial Key Laboratory of Colorectal and Pelvic Floor Diseases, the Sixth Affiliated Hospital of Sun Yat-sen University, Guangzhou 510000, China
| | - Peng Zhang
- Department of Gastrointestinal Surgery, Union Hospital Tongji Medical College Huazhong University of Science and Technology, Wuhan 430000, China
| | - Tao Chen
- Department of General Surgery, Southern Medical University Nanfang Hospital, Guangzhou 510000, China
| | - Haibo Qiu
- Department of Gastric and Pancreatic Surgery, Sun Yat-sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Guangzhou 510000, China
| | - Zhiwei Zhou
- Department of Gastric and Pancreatic Surgery, Sun Yat-sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center of Cancer Medicine, Guangzhou 510000, China
| | - Guoxin Li
- Department of General Surgery, Southern Medical University Nanfang Hospital, Guangzhou 510000, China
| | - Kaixiong Tao
- Department of Gastrointestinal Surgery, Union Hospital Tongji Medical College Huazhong University of Science and Technology, Wuhan 430000, China
| | - Hui Wang
- Department of Colorectal Surgery, the Sixth Affiliated Hospital of Sun Yat-sen University, Guangdong Provincial Key Laboratory of Colorectal and Pelvic Floor Diseases, the Sixth Affiliated Hospital of Sun Yat-sen University, Guangzhou 510000, China
| | - Yong Li
- Department of General Surgery, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou 510000, China
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10
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Xie F, Xiao W, Jiang Y, Xia X, Wang Y. Relationship between efficacy of sunitinib and KIT mutation of patients with advanced gastrointestinal stromal tumors after failure of imatinib: A systematic review. Medicine (Baltimore) 2019; 98:e15478. [PMID: 31083182 PMCID: PMC6531104 DOI: 10.1097/md.0000000000015478] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/28/2023] Open
Abstract
BACKGROUND A large number of studies have shown that KIT mutations are closely related to the prognosis of gastrointestinal stromal tumors (GISTs). At the same time, sunitinib (SU) has become the second-line recommended drug for GISTs because of its efficacy. We initiated a systematic review to compare the efficacy of SU after failure of Imatinib (IM) in different KIT mutations. METHODS We searched for SU-treated patients with advanced GISTs after failed IM treatment by using databases such as PubMed, EMBASE, and the Cochrane Library, up to March 2018. We conducted statistical analyses to calculate the odds ratio (OR), hazard ratio (HR), and 95% confidence interval (CI) using fixed-effects and random-effects models by Review Manager 5.3 software. RESULTS We included a total of 474 patients from 3 retrospective studies and 2 cohort studies. Patients with exon 9 mutations had higher clinical benefit (OR = 2.61, 95% CIs = 1.32-5.18, P = .006) rates and longer progression-free survival (progressive disease, HR = 0.51, 95% CIs = 0.36-0.72, P = .0001) compared with exon 11, but there was no statistically significant difference in overall survival (OS, HR = 0.93, 95% CIs = 0.34-2.55, P = .89) and there was greater heterogeneity (Tau = 0.72, Chi = 21.45, df = 3, P < .001, I = 86%). Subgroup analysis suggests that race may be one of the sources of heterogeneity. CONCLUSION The results show that efficacy of SU is closely associated with KIT genotypes in GISTs. Moreover, racial factor also directly affects the prognosis of different KIT mutational status, so GISTs patients of different genotypes might also consider the use of targeted drugs in consideration of ethnic differences.
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Affiliation(s)
- Fuming Xie
- Department of Gastroenterology, The Second Affiliated Hospital of Chongqing Medical University
| | - Weidong Xiao
- Department of General Surgery, Xinqiao Hospital, Army Medical University, Chongqing, P. R. China
| | - Yahui Jiang
- Department of Gastroenterology, The Second Affiliated Hospital of Chongqing Medical University
| | - Xiao Xia
- Department of Gastroenterology, The Second Affiliated Hospital of Chongqing Medical University
| | - Yaxu Wang
- Department of Gastroenterology, The Second Affiliated Hospital of Chongqing Medical University
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11
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Jilg S, Rassner M, Maier J, Waldeck S, Kehl V, Follo M, Philipp U, Sauter A, Specht K, Mitschke J, Lange T, Bauer S, Jost PJ, Peschel C, Duyster J, Gaiser T, Hohenberger P, Bubnoff N. Circulating
cKIT
and
PDGFRA
DNA indicates disease activity in Gastrointestinal Stromal Tumor (GIST). Int J Cancer 2019; 145:2292-2303. [DOI: 10.1002/ijc.32282] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/09/2018] [Revised: 01/10/2019] [Accepted: 03/05/2019] [Indexed: 12/14/2022]
Affiliation(s)
- Stefanie Jilg
- III Medical Department for Hematology and Oncology, Klinikum Rechts der IsarTechnische Universität München Munich Germany
| | - Michael Rassner
- Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of MedicineUniversity of Freiburg Freiburg Germany
| | - Jacqueline Maier
- Center for Internal Medicine, Department of Hematology/Oncology and HemostaseologyUniversity of Leipzig Leipzig Germany
| | - Silvia Waldeck
- Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of MedicineUniversity of Freiburg Freiburg Germany
- German Cancer Consortium (DKTK) partner site Freiburg and German Cancer Research Center (DKFZ) Heidelberg Germany
- Faculty of BiologyUniversity of Freiburg Freiburg Germany
| | - Victoria Kehl
- Institute for Medical Informatics, Statistics, and Epidemiology, Klinikum Rechts der IsarTechnische Universität München Munich Germany
| | - Marie Follo
- Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of MedicineUniversity of Freiburg Freiburg Germany
| | - Ulrike Philipp
- Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of MedicineUniversity of Freiburg Freiburg Germany
| | - Andreas Sauter
- Department of Diagnostic and Interventional Radiology, Klinikum Rechts der IsarTechnische Universität München Munich Germany
| | - Katja Specht
- Institute of Pathology, Klinikum Rechts der IsarTechnische Universität München Munich Germany
| | - Jan Mitschke
- Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of MedicineUniversity of Freiburg Freiburg Germany
- German Cancer Consortium (DKTK) partner site Freiburg and German Cancer Research Center (DKFZ) Heidelberg Germany
| | | | - Sebastian Bauer
- Sarcoma Center, West German Cancer CenterUniversity Hospital Essen, University Duisburg‐Essen Essen Germany
| | - Philipp J. Jost
- III Medical Department for Hematology and Oncology, Klinikum Rechts der IsarTechnische Universität München Munich Germany
| | - Christian Peschel
- III Medical Department for Hematology and Oncology, Klinikum Rechts der IsarTechnische Universität München Munich Germany
| | - Justus Duyster
- Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of MedicineUniversity of Freiburg Freiburg Germany
- German Cancer Consortium (DKTK) partner site Freiburg and German Cancer Research Center (DKFZ) Heidelberg Germany
| | - Timo Gaiser
- Institute of Pathology, University Medical Center MannheimRuprecht‐Karl University of Heidelberg Mannheim Germany
| | - Peter Hohenberger
- Division of Surgical Oncology and Thoracic SurgeryUniversity Medical Center Mannheim, Ruprecht‐Karl University of Heidelberg Mannheim Germany
| | - Nikolas Bubnoff
- Department of Hematology, Oncology and Stem Cell Transplantation, Medical Center, Faculty of MedicineUniversity of Freiburg Freiburg Germany
- German Cancer Consortium (DKTK) partner site Freiburg and German Cancer Research Center (DKFZ) Heidelberg Germany
- Department of Hematology and Oncology, Medical CenterUniversity of Schleswig Holstein Lübeck Germany
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12
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Kaneko M, Emoto S, Murono K, Sonoda H, Hiyoshi M, Sasaki K, Shuno Y, Nishikawa T, Tanaka T, Hata K, Kawai K, Nozawa H. Neoadjuvant imatinib therapy in rectal gastrointestinal stromal tumors. Surg Today 2018; 49:460-466. [PMID: 30443673 DOI: 10.1007/s00595-018-1737-5] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/26/2018] [Accepted: 10/20/2018] [Indexed: 02/09/2023]
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13
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The use of endoscopic ultrasonography in the detection and differentiation of pathology in the wall of the upper gastrointestinal tract. GASTROENTEROLOGY REVIEW 2018; 13:30-34. [PMID: 29657608 PMCID: PMC5894450 DOI: 10.5114/pg.2018.74560] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 11/12/2016] [Accepted: 04/18/2017] [Indexed: 11/19/2022]
Abstract
Introduction The growing incidence of gastrointestinal diseases forces to improve imaging techniques. Identification of lesions located inside the wall of intestinal tract or in close proximity often was not possible using endoscopy or computed tomography. Aim To assess the usefulness of endosonography (EUS) in the differentiation between compression from the outside and intramural lesions of the upper gastrointestinal tract. Material and methods For 4 years 20,012 patients with performed gastroscopies were enrolled in the study. One hundred and ninety-nine patients (96 females, 103 males; age 62.2 ±14.1 years) with pathology of the wall of the upper gastrointestinal tract qualified for further diagnosis. Endosonography and computed tomography (CT) were performed in each patient. A chest CT was performed in patients with a lesion in the oesophagus. An abdomen CT was performed in patients with pathology in the stomach or duodenum. Based on the results of EUS, histopathology, and imaging, each patient qualified for treatment, endoscopic observation, surgery, or cancer treatment. Results In EUS 129 (64.8%) intramural lesions were identified. Five (2.5%) diagnoses were false negative. In 62 (31.2%) patients no intramural changes were recognised and three (1.5%) results were false positive. The sensitivity and specificity of EUS was 96.3% and 95.4%, respectively, with positive predictive value 90.7%, negative predictive value 97.8%, and overall accuracy 95% (p < 0.05). Endoscopic therapy was performed in 31 (15.6%) patients, and 99 (49.8%) were classified for endoscopic observation. Surgery was performed in 50 (25.1%) patients, and 19 (9.5%) patients required oncologic treatment. Conclusions Endosonography exceeds computed tomography in differentiating compression from the outside and intramural lesions of the upper gastrointestinal tract.
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14
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Cirocchi R, Farinella E, La Mura F, Cavaliere D, Avenia N, Verdecchia GM, Giustozzi G, Noya G, Sciannameo F. Efficacy of Surgery and Imatinib Mesylate in the Treatment of Advanced Gastrointestinal Stromal Tumor: A Systematic Review. TUMORI JOURNAL 2018; 96:392-9. [DOI: 10.1177/030089161009600303] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
Aims and background In patients with localized gastrointestinal stromal tumors, surgery remains the elective treatment. Nowadays, imatinib therapy has been standardized in advanced gastrointestinal stromal tumors, showing continuous improvements in progression-free and overall survival. A combination of imatinib therapy and surgery may also be effective in a subset of patients with metastatic or unresectable gastrointestinal stromal tumors. In this review, the authors analyzed the role of imatinib mesylate associated to surgery in unresectable and/or metastatic gastrointestinal stromal tumors. Methods and study design We searched for all published and unpublished randomized controlled clinical trials and controlled clinical trials. We conducted the review according to the recommendations of The Cochrane Collaboration. We used Review Manager 5 software for the statistical analysis. Results There are currently no randomized controlled clinical trials or controlled clinical trials on this issue. We performed a subgroup analysis in the patients pre-operatively treated with imatinib mesylate. This subgroup revealed a minor incidence of recurrent or metastatic gastrointestinal stromal tumors and a greater incidence of locally unresectable gastrointestinal stromal tumors in the responsive disease group (P = 0.001). In this patient group, more complete resections were observed (P = 0.00001). Furthermore, in the same patient group we observed a more significant 12 and 24-month disease-free survival after imatinib treatment and complete resection (respectively P = 0.06 and P = 0.003) and also a better 24-month overall survival (P = 0.004). Conclusions There is actually only one ongoing European randomized study evaluating surgery of residual disease in patients with metastatic gastrointestinal stromal tumors responding to imatinib mesylate. Imatinib mesylate represents the standard treatment as preoperative supplement for locally unresectable and/or metastatic gastrointestinal stromal tumors, and a trial to compare the approach versus surgery alone is not necessary. For patients responding to imatinib or patients with prolonged stable disease, resection of residual disease should be considered. A phase III randomized study evaluating surgery of residual disease in patients with metastatic gastrointestinal stromal tumor responding to imatinib mesylate, EORTC 62063, has been opened. Moreover, surgery should be considered for patients at higher risk of complications during pharmacological debulking. In advanced gastrointestinal stromal tumors, the advantages of the integrated treatment are significant in the complete or partial response disease group in terms of more complete resections and better disease-free and overall survival.
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Affiliation(s)
- Roberto Cirocchi
- Department of General and Emergency Surgery, St. Maria Hospital, Terni, University of Perugia
| | - Eriberto Farinella
- Department of General and Emergency Surgery, St. Maria Hospital, Terni, University of Perugia
| | - Francesco La Mura
- Department of General and Emergency Surgery, St. Maria Hospital, Terni, University of Perugia
| | - Davide Cavaliere
- Department of Surgical Oncology, Hospital of Forlì, Forlì, Italy
| | - Nicola Avenia
- Department of General and Emergency Surgery, St. Maria Hospital, Terni, University of Perugia
| | | | - Gianmario Giustozzi
- Department of General and Emergency Surgery, St. Maria Hospital, Terni, University of Perugia
| | - Giuseppe Noya
- Department of General and Emergency Surgery, St. Maria Hospital, Terni, University of Perugia
| | - Francesco Sciannameo
- Department of General and Emergency Surgery, St. Maria Hospital, Terni, University of Perugia
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15
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The incidence, mutational status, risk classification and referral pattern of gastro-intestinal stromal tumours in the Netherlands: a nationwide pathology registry (PALGA) study. Virchows Arch 2018; 472:221-229. [PMID: 29308530 PMCID: PMC5856869 DOI: 10.1007/s00428-017-2285-x] [Citation(s) in RCA: 40] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2017] [Revised: 10/31/2017] [Accepted: 12/18/2017] [Indexed: 01/17/2023]
Abstract
Symptomatic gastrointestinal stromal tumours (GIST) are infrequent with an incidence of 12.7 per million inhabitants in the western population. We studied whether the incidence of GIST has further increased between 2003 and 2012 and assessed the frequency of mutations, risk groups, histological subtypes and immunohistochemistry results. From PALGA, the nationwide Dutch Pathology Registry, pathology excerpts from all patients with a GIST or GIST-like tumour between 2003 and 2012 were retrieved to calculate incidence rates. Full pathology reports were retrieved of resections in 2011 and 2012 to study the frequency of mutations, risk groups, histological subtypes and immunohistochemistry results. The incidence of GIST increased to 17.7 per million inhabitants in 2012 with a median age of 67 years. Mutational analysis was performed in 33.9% of patients with a resection between 2011 and 2012 (KIT mutation 67.5%, PDGFRA 16.3%, wild-type 11.4%). The percentage of high risk patients in the different risk classifications varied from 19.9% to 38.0% depending on the used classification. Only 35.9% of patients had diagnosis or revision of pathology diagnosis within three months in a designated GIST referral centre. No increase in proportion of central pathology reviews was found. Proportion of patients with mutational analysis increased over the years. The registered incidence of GIST, 17.7 per million inhabitants in 2012 in the Netherlands, is still rising. Despite incorporation in the ESMO GIST guidelines since 2008 for mutational testing and since 2010 for central review of pathology, both are performed in a minority of patients.
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16
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Recurrent Gastrointestinal Stromal Tumors in the Imatinib Mesylate Era: Treatment Strategies for an Incurable Disease. Case Rep Oncol Med 2017; 2017:8349090. [PMID: 29333308 PMCID: PMC5733166 DOI: 10.1155/2017/8349090] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2017] [Accepted: 10/09/2017] [Indexed: 12/20/2022] Open
Abstract
Introduction Recurrence of gastrointestinal stromal tumors (GISTs) after surgical resection and imatinib mesylate (IM) adjuvant therapy poses a significant treatment challenge. We present the case of a patient who underwent surgical resection after recurrence and review the current literature regarding treatment. Case Presentation A 58-year-old man with a large intra-abdominal jejunal GIST was treated with complete surgical resection followed by IM. The patient experienced disease recurrence 3.5 years later and underwent IM dose escalation and reresection. Conclusion Current strategies to treat recurrent GIST include dose escalation, modifying adjuvant tyrosine kinase inhibitor therapy, and surgery. High-level evidence will be required to better define the combinatory roles of tyrosine kinase inhibitor therapy, guided by molecular profiling, and surgery in the management of recurrent GIST.
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17
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Molinaro E, Romei C, Biagini A, Sabini E, Agate L, Mazzeo S, Materazzi G, Sellari-Franceschini S, Ribechini A, Torregrossa L, Basolo F, Vitti P, Elisei R. Anaplastic thyroid carcinoma: from clinicopathology to genetics and advanced therapies. Nat Rev Endocrinol 2017; 13:644-660. [PMID: 28707679 DOI: 10.1038/nrendo.2017.76] [Citation(s) in RCA: 318] [Impact Index Per Article: 39.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
Anaplastic thyroid carcinoma (ATC) is a rare malignancy, accounting for 1-2% of all thyroid cancers. Although rare, ATC accounts for the majority of deaths from thyroid carcinoma. ATC often originates in a pre-existing thyroid cancer lesion, as suggested by the simultaneous presence of areas of differentiated or poorly differentiated thyroid carcinoma. ATC is characterized by the accumulation of several oncogenic alterations, and studies have shown that an increased number of oncogenic alterations equates to an increased level of dedifferentiation and aggressiveness. The clinical management of ATC requires a multidisciplinary approach; according to recent American Thyroid Association guidelines, surgery, radiotherapy and/or chemotherapy should be considered. In addition to conventional therapies, novel molecular targeted therapies are the most promising emerging treatment modalities. These drugs are often multiple receptor tyrosine kinase inhibitors, several of which have been tested in clinical trials with encouraging results so far. Accordingly, clinical trials are ongoing to evaluate the safety, efficacy and effectiveness of these new agents. This Review describes the updated clinical and pathological features of ATC and provides insight into the molecular biology of this disease. The most recent literature regarding conventional, newly available and future therapies for ATC is also discussed.
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Affiliation(s)
- Eleonora Molinaro
- Endocrine Unit, Department of Clinical and Experimental Medicine, University Hospital of Pisa
| | - Cristina Romei
- Endocrine Unit, Department of Clinical and Experimental Medicine, University Hospital of Pisa
| | - Agnese Biagini
- Endocrine Unit, Department of Clinical and Experimental Medicine, University Hospital of Pisa
| | - Elena Sabini
- Endocrine Unit, Department of Clinical and Experimental Medicine, University Hospital of Pisa
| | - Laura Agate
- Endocrine Unit, Department of Clinical and Experimental Medicine, University Hospital of Pisa
| | - Salvatore Mazzeo
- Diagnostic and Interventional Radiology Department of Translational Research and New Technologies in Medicine and Surgery, University Hospital of Pisa
| | - Gabriele Materazzi
- Division of Endocrine Surgery, Department of Surgical Pathology, University Hospital of Pisa
| | | | | | - Liborio Torregrossa
- Department of Surgical, Medical and Molecular Pathology, University Hospital of Pisa, Via Paradisa 2, 56124 Pisa, Italy
| | - Fulvio Basolo
- Department of Surgical, Medical and Molecular Pathology, University Hospital of Pisa, Via Paradisa 2, 56124 Pisa, Italy
| | - Paolo Vitti
- Endocrine Unit, Department of Clinical and Experimental Medicine, University Hospital of Pisa
| | - Rossella Elisei
- Endocrine Unit, Department of Clinical and Experimental Medicine, University Hospital of Pisa
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18
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Wang H, Shi L, Chen P. Application of laparoscopy, endoscopy and laparoscopic and endoscopic cooperative surgery in gastrointestinal stromal tumors. Shijie Huaren Xiaohua Zazhi 2016; 24:4133-4143. [DOI: 10.11569/wcjd.v24.i30.4133] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Gastrointestinal stromal tumors (GISTs) constitute the most common mesenchymal neoplasms of the gastrointestinal tract, among which 60%-70% are located in the stomach. All GISTs have malignant potential, varying from small lesions to aggressive sarcomas. Surgical resection with negative margins remains the best treatment for GISTs. Lymphadenectomy is not necessary because GISTs rarely metastasize to lymph nodes. Because of these biological behavior characteristics, the advantages of minimally invasive surgeries such as laparoscopic surgery and endoscopic surgery can be fully reflected in the surgical management of GISTs. The advent of laparoscopic and endoscopic cooperative surgery has broadened the scope and enhanced the safety of minimally invasive surgical treatment of GISTs. In this paper we will discuss the application of laparoscopy, endoscopy and laparoscopic and endoscopic cooperative surgery in the treatment of GISTs.
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19
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Mueller CL, Braun J, Leimanis ML, Mouhanna J, Feldman LS, Ferri LE. Application of an individualized operative strategy for wedge resection of gastric gastrointestinal stromal tumors: Effectiveness for tumors in difficult locations. Surgery 2016; 160:1038-1048. [PMID: 27486000 DOI: 10.1016/j.surg.2016.06.004] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/23/2016] [Revised: 06/07/2016] [Accepted: 06/07/2016] [Indexed: 12/19/2022]
Abstract
BACKGROUND There is some concern that wedge resection of gastric gastrointestinal stromal tumors is not feasible in certain anatomic locations, such as the cardia or antrum. We sought to review our experience with treatment of gastric gastrointestinal stromal tumors with a particular focus on nonanatomic wedge resections in these challenging locations. METHODS Patients undergoing resection of gastrointestinal stromal tumors from 2000-2014 at the Montreal General Hospital were identified from a prospectively collected database, and outcomes were tabulated. An individualized operative strategy was used to guide resection based on tumor location, size, and characteristics. Disease-free survival and overall survival analyzed using the Kaplan-Meier method. Data are presented as median (range). RESULTS We identified 59 patients who underwent operative resection for gastric gastrointestinal stromal tumors. Tumor location was fundus/body/greater curvature in 35 (59%) patients, lesser curvature in 8 (14%) patients, antrum in 8 (14%) patients, and cardia in 8 (14%) patients. Median tumor size was 4.5 cm (1.4-25 cm). The majority of cardia and antral lesions were removed with wedge resections (14/16, 87%). For cardial and antral tumors, on-table gastroscopy was used to guide the operative approach and prevent narrowing of the Gastroesophageal junction or pylorus in all patients undergoing wedge resection. Negative pathologic margins were achieved in all patients. The 5-year disease-free survival was 91% and 5-year overall survival was 95%. CONCLUSION When selected appropriately, and under the guidance of on-table gastroscopy, laparoscopic nonanatomic wedge resection can be performed successfully in the majority of cases, even for gastrointestinal stromal tumors near the GEJ or pylorus, with excellent oncologic outcomes.
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Affiliation(s)
- Carmen L Mueller
- Department of Surgery, Montreal General Hospital, McGill University Health Center, Montreal, Canada.
| | - Josef Braun
- Department of Surgery, Montreal General Hospital, McGill University Health Center, Montreal, Canada
| | - Mara L Leimanis
- Department of Surgery, Montreal General Hospital, McGill University Health Center, Montreal, Canada
| | - Jack Mouhanna
- Department of Surgery, Montreal General Hospital, McGill University Health Center, Montreal, Canada
| | - Liane S Feldman
- Department of Surgery, Montreal General Hospital, McGill University Health Center, Montreal, Canada
| | - Lorenzo E Ferri
- Department of Surgery, Montreal General Hospital, McGill University Health Center, Montreal, Canada
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20
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Schöffel N, Groneberg DA, Kaul T, Laatsch D, Thielemann H. [Gastrointestinal stromal tumors (GIST)--literature review]. MMW Fortschr Med 2016; 158:60-62. [PMID: 27119704 DOI: 10.1007/s15006-016-7824-x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/05/2023]
Affiliation(s)
- Norman Schöffel
- Klinik für Allgemein- und Viszeralchirurgie Unfallkrankenhaus Berlin, Warener Str. 7, D-12683, Berlin, Deutschland.
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Barrios CH, Blackstein ME, Blay JY, Casali PG, Chacon M, Gu J, Kang YK, Nishida T, Purkayastha D, Woodman RC, Reichardt P. The GOLD ReGISTry: a Global, Prospective, Observational Registry Collecting Longitudinal Data on Patients with Advanced and Localised Gastrointestinal Stromal Tumours. Eur J Cancer 2015; 51:2423-33. [PMID: 26248685 DOI: 10.1016/j.ejca.2015.07.010] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2015] [Accepted: 07/13/2015] [Indexed: 12/18/2022]
Abstract
BACKGROUND Gastrointestinal stromal tumours (GISTs) are the most common gastrointestinal sarcomas. This global, prospective registry followed patients with advanced or localised GIST (2007-2011). METHODS Current and evolving diagnostics, treatments and outcome measures in patients with GIST were assessed. Eligible patients were diagnosed with advanced or localised GIST within 15months of registry entry. No treatment plan was prescribed, and no visit schedule was mandated. Treating physicians recorded patient information, including tumour response, diagnostic methods, medications, surgeries performed, mutation status and adverse events leading to dose/medication changes. Survival outcomes were estimated using the Kaplan-Meier method. Other data were analysed using descriptive statistics. RESULTS The registry included 1663 patients (advanced GIST, n=1095; localised GIST, n=537). Medications (e.g. tyrosine kinase inhibitor use and dosing), disease progression or recurrence and physician assessment of response to treatment in registry patients were consistent with controlled trials and prevailing clinical recommendations. In advanced GIST, estimated 30-month progression-free survival (PFS) (59.8%) and overall survival (OS) (82.7%) were higher than results from previously reported trials (≈40% and ≈70%, respectively). Consistent with treatment guidelines, the most common initial treatments were imatinib for advanced GIST, and complete surgical resection for localised GIST. Computed tomography scans were the most common imaging technique used at diagnosis and follow-up. Mutation analysis was performed at diagnosis in only 15.3% and 14.5% of patients with advanced and localised GIST, respectively. CONCLUSIONS In this real-world GIST registry, patients with advanced GIST were treated with imatinib and patients with localised GIST received surgical resection, in accordance with prevailing clinical recommendations.
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Affiliation(s)
| | | | | | | | - Matias Chacon
- Alexander Fleming Institute, Department of Medical Oncology, Buenos Aires, Argentina.
| | - Jin Gu
- Peking University Cancer Hospital, Beijing, China.
| | - Yoon-Koo Kang
- University of Ulsan College of Medicine, Seoul, South Korea.
| | - Toshirou Nishida
- National Cancer Center Hospital East, 6-5-1 Kashiwanoha, Kashiwa, Chiba 277-8577, Japan.
| | - Das Purkayastha
- Novartis Pharmaceuticals Corporation, East Hanover, NJ, USA.
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22
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A 59-Year-Old Woman with Upper Gastrointestinal Hemorrhage: Understanding the Gist of GISTs. Dig Dis Sci 2015; 60:1928-30. [PMID: 26022705 DOI: 10.1007/s10620-015-3718-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/10/2015] [Accepted: 05/11/2015] [Indexed: 12/09/2022]
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23
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Govindaraj S, Dias BH, Gautham SL. A Sporadic Small Jejunal GIST Presenting with Acute Lower Gastrointestinal Hemorrhage: A Review of the Literature and Management Guidelines. Indian J Surg 2015; 77:143-6. [PMID: 25972676 DOI: 10.1007/s12262-015-1208-9] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/28/2014] [Accepted: 01/05/2015] [Indexed: 12/17/2022] Open
Abstract
Gastrointestinal stromal tumors (GISTs) represent the majority of primary nonepithelial neoplasms of the digestive tract, most frequently expressing the KIT protein detected by immunohistochemical staining for the CD117 antigen. Jejunal GISTs account for approximately 10 % of GISTs. Patients usually present with abdominal discomfort. Jejunal GISTs may cause symptoms secondary to obstruction or hemorrhage. Pressure necrosis and ulceration of the overlying mucosa may cause gastrointestinal bleeding, and patients who experience significant blood loss may suffer from malaise and fatigue. Literature has classified small-bowel GISTs on the basis of size, and various established guidelines have advised conservative management of small jejunal GISTs (<2 cm). We here report the clinical, macroscopic, and immunohistological features of a small jejunal GIST presenting with acute lower gastrointestinal hemorrhage in a 50-year-old postmenopausal woman necessitating an emergency laparotomy to control the bleed. The management of very small (<2 cm) small-bowel GISTs is controversial. While guidelines are primarily based on the risk of malignancy in GISTs, no guideline predicting the risk of complications in small-bowel GISTs exists. Hence, these tumors should be removed even if incidentally detected.
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Affiliation(s)
- Sridar Govindaraj
- Department of General Surgery, St. John's Medical College Hospital, Bangalore, 560034 Karnataka India
| | - Brendan Hermenigildo Dias
- Department of General Surgery, St. John's Medical College Hospital, Bangalore, 560034 Karnataka India
| | - S L Gautham
- Department of General Surgery, St. John's Medical College Hospital, Bangalore, 560034 Karnataka India
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24
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Al-Jiffry BO, Allam HM, Hatem M. Single-center experience of surgically resected gastrointestinal stromal tumors: A report of six cases, including a rare case involving the lower esophagus. Oncol Lett 2014; 9:745-748. [PMID: 25624901 PMCID: PMC4301538 DOI: 10.3892/ol.2014.2792] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/11/2014] [Accepted: 11/21/2014] [Indexed: 11/17/2022] Open
Abstract
Gastrointestinal stromal tumors (GISTs) are rare, but remain the most common GI mesenchymal neoplasms. In the present study, six cases of GIST are reported, and one of these cases, a patient with esophageal GIST, is reported in-depth. Certain recent developments in the clinical therapy of GISTs are also discussed. The records of all surgically-resected GI stromal tumors treated at the Al-Hada Military Hospital between January 2007 and December 2012 were reviewed. There were six cases of surgically resected GISTs during this time period, three males and three females, with a mean age of 69.3±16.4 years. The stomach was involved in 66.7% of cases, the small intestine in 16.7% and the esophagus, which is an extremely rare site, in 16.7% of cases. The most common symptom at presentation was abdominal pain, followed by GI bleeding. The mean tumor size was 8.7±6.3 cm. Surgery was indicated by the presence of the aforementioned symptoms or a tumor size >5 cm. All tumors were completely resected with histologically negative margins. The diagnoses were established by immunohistochemistry. Four patients were classified as possessing a high-grade variant, and were administered with tyrosine kinase inhibitors (TKIs). Following a mean follow up of 31 months, no recurrence or mortality was detected. Complete surgical resection with tumor-free margins is the standard treatment for GISTs, and TKIs should be used as adjuvant therapy if the risk of progressive disease is high.
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Affiliation(s)
- Bilal O Al-Jiffry
- Department of Surgery, Taif University, Taif 21944, Saudi Arabia ; Department of Surgery, Al-Hada Military Hospital, Taif 21944, Saudi Arabia
| | - Hisham M Allam
- Department of Surgery, Al-Hada Military Hospital, Taif 21944, Saudi Arabia
| | - Mohammed Hatem
- Department of Surgery, Taif University, Taif 21944, Saudi Arabia
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Roshanravan R, Heidari Esfahani M, Moslemi S, Hosseini SV, Muzhir Gabash K. Gastric Gastrointestinal Stromal Tumor (GIST) Incidentally Found After Laparoscopic Sleeve Gastrectomy: A Case Report. ACTA ACUST UNITED AC 2014. [DOI: 10.17795/acr-24855] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/07/2023]
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Wiraszka GR, Głuszek S, Kozieł D. Characteristics of gastrointestinal stromal tumours, diagnostic procedure and therapeutic management and main directions of nursing practice in gastrointestinal stromal tumours. Contemp Oncol (Pozn) 2014; 18:384-90. [PMID: 25784835 PMCID: PMC4355651 DOI: 10.5114/wo.2014.40557] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2013] [Revised: 09/13/2013] [Accepted: 02/03/2014] [Indexed: 01/22/2023] Open
Abstract
Gastrointestinal stromal tumours (GIST) constitute a separate group of mesenchymal neoplasms of the gastrointestinal tract. They have been commonly recognized for a few years, they have created a new problem in medical practice. GIST are more often centred in the stomach. They equally affect female and male patients and occur mainly in patients older than 50 years of age. The clinical picture of the tumour is non-specific. Radical surgical treatment and molecularly targeted therapy with tyrosine kinase inhibitors are used in GIST treatment. Nursing practice with reference to GIST danger is connected with biopsychosocial interventions of perioperative, oncological and palliative procedures and involves the area of health education mainly oriented towards shaping preventive procedures which favour early disease detection and support therapy and recovery.
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Affiliation(s)
- Grażyna R. Wiraszka
- Institute of Nursing and Obstetrics, Faculty of Health Sciences, Jan Kochanowski University in Kielce, Poland
| | - Stanisław Głuszek
- Institute of Nursing and Obstetrics, Faculty of Health Sciences, Jan Kochanowski University in Kielce, Poland
- Clinical Department of General, Oncological and Endocrinological Surgery, Voivodship, Hospital of Kielce, Poland
| | - Dorota Kozieł
- Institute of Nursing and Obstetrics, Faculty of Health Sciences, Jan Kochanowski University in Kielce, Poland
- Clinical Department of General, Oncological and Endocrinological Surgery, Voivodship, Hospital of Kielce, Poland
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Atay S, Godwin AK. Tumor-derived exosomes: A message delivery system for tumor progression. Commun Integr Biol 2014; 7:e28231. [PMID: 24778765 PMCID: PMC3995727 DOI: 10.4161/cib.28231] [Citation(s) in RCA: 88] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2014] [Revised: 02/13/2014] [Accepted: 02/14/2014] [Indexed: 12/23/2022] Open
Abstract
Intercellular communication is a key process in the development and progression of cancer. The dynamic and reciprocal interplays between the tumor and its microenvironment orchestrate events critical to the establishment of primary and metastatic niches and maintenance of a permissive environment at the tumor−stroma interface. Atay and colleagues found that gastrointestinal stromal tumor cells secrete vesicles known as exosomes. These exosomes contain oncogenic KIT and their transfer and uptake by surrounding smooth muscle cells lead to enhanced AKT and MAPK signaling and phenotypic modulation of several cellular processes, including morphological changes, expression of tumor-associated markers, secretion of matrix metalloproteinases, and enhanced tumor cell invasion. This provocative study emphasizes that exosome-mediated signaling within the tumor microenvironment acts as a positive feedback loop that contributes to invasiveness and that interfering with this message delivery system may represent promising therapeutic approaches, not only for GIST, but for other types of cancer.
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Affiliation(s)
- Safinur Atay
- Department of Pathology and Laboratory Medicine; University of Kansas Medical Center; Kansas City, KS USA
| | - Andrew K Godwin
- Department of Pathology and Laboratory Medicine; University of Kansas Medical Center; Kansas City, KS USA ; University of Kansas Cancer Center; Kansas City, KS USA
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Surgical management of gastrointestinal stromal tumors: a single center experience. Wideochir Inne Tech Maloinwazyjne 2014; 9:71-82. [PMID: 24729813 PMCID: PMC3983553 DOI: 10.5114/wiitm.2014.40987] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/24/2013] [Revised: 09/15/2013] [Accepted: 11/05/2013] [Indexed: 12/13/2022] Open
Abstract
INTRODUCTION Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal (GI) tract. Although the therapy targeted at inhibiting tyrosine kinases has shown dramatic results in metastatic and inoperable GISTs, the mainstay of treatment in primary localized forms remains surgical resection. AIM To provide an overview of our experience of GIST diagnosis and management, with emphasis on comparison of minimally invasive and open surgical resection for primary GISTs. MATERIAL AND METHODS We retrospectively reviewed the medical records of all patients who underwent surgical removal of GISTs from 2008 to 2012. Patient demographics, clinical data, surgery, complications, histopathological data and clinical course were analyzed. RESULTS Forty-four patients were identified. Average age at diagnosis was 63 years. Minimally invasive (MIS) and open surgery (OS) were each attempted in 22 (50.0%) patients. Laparoscopic removal was performed in 20, laparoendoscopic in 1, and laparoscopy-assisted endoscopic removal in 1. Conversion to an open procedure was performed in 4 (18.2%). We found significant differences in postoperative length of stay (8.5 days vs. 10.1 days, p < 0.001) and tumor size (2.93 cm vs. 5.78 cm, p = 0.018) between MIS and OS groups, respectively. CONCLUSIONS Laparoscopic removal is safe and effective for GISTs not exceeding 6 cm. Gastroesophageal junction and cardia GISTs require careful preoperative evaluation and planning to remove safely. We recommend avoiding laparoscopic removal of these tumors due to the high rate of conversion (100.0%) to an open procedure. Laparoendoscopic surgical approach is an appropriate technique for removal of small-sized intraluminal benign GISTs not involving the muscularis propria layer.
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Reichardt P, Morosi C, Wardelmann E, Gronchi A. Gastrointestinal stromal tumors: evolving role of the multidisciplinary team approach in management. Expert Rev Anticancer Ther 2014; 12:1053-68. [DOI: 10.1586/era.12.48] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2023]
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Rotert JV, Leupold J, Hohenberger P, Nowak K, Allgayer H. Src activity is increased in gastrointestinal stromal tumors--analysis of associations with clinical and other molecular tumor characteristics. J Surg Oncol 2014; 109:597-605. [PMID: 24391050 DOI: 10.1002/jso.23544] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/14/2013] [Accepted: 11/30/2013] [Indexed: 12/16/2022]
Abstract
BACKGROUND Increased activity of Src has been found in several human cancers, and often is associated with poor clinical outcome. The present study aimed to determine whether Src activity is increased in gastrointestinal stromal tumors (GIST), and whether it correlates with established tumor or patient characteristics and prognosis. METHODS Tumor/normal tissues of 29 patients were analyzed for Src activity/protein with kinase assays, and for VEGF/VEGFR with immunohistochemical staining. RESULTS Src activity was higher in tumor than in normal tissues (P = 0.093). However, when imatinib responders were excluded from the analyses, it was significantly higher in the tumor tissue (P = 0.017). Additionally, it was higher in primary compared to recurrent tumors or metastasis (P = 0.04). Univariate survival analysis showed a longer overall survival for patients with high Src activity (P = 0.038). In multivariate analysis, the response to imatinib treatment was the only survival-influencing factor (P = 0.072). CONCLUSIONS Src activity is increased in GIST. In contrast to most other tumor entities, it does not correlate with poor clinical outcome, but decreases during the progression from primary tumor to recurrence and metastasis, especially under therapy with imatinib. Additionally, our results show that higher Src activity is associated with longer overall survival.
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Affiliation(s)
- Julia Valerie Rotert
- Department of Surgery, Mannheim Medical Faculty, Heidelberg University, Mannheim, Germany
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Han NY, Park BJ, Park SS, Sung DJ, Kim MJ, Cho SB, Lee KS. Modified fusion imaging combining CT gastrography and CT angiography: an initial experience of preoperative mapping prior to laparoscopic exogastric wedge resection of small (<3 cm) gastric submucosal lesions. ABDOMINAL IMAGING 2014; 39:242-50. [PMID: 24375020 DOI: 10.1007/s00261-013-0055-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
PURPOSE To evaluate the feasibility of modified fusion imaging (MFI) combining CT gastrography (CTG) and CT angiography (CTA) in the preoperative mapping and intraoperative localization of small (<3 cm) submucosal lesions (SMLs) during laparoscopic exogastric wedge resection. METHODS Thirty consecutive patients scheduled for laparoscopic wedge resection of small SMLs (<3 cm) were enrolled. MFI was reconstructed using a volume rendering of the arterial phase CT data acquired after gastric distension. With MFI, the possibility of preoperative mapping and feasibility for successful intraoperative localization was evaluated using intraoperative findings as the reference standard. RESULTS In 21 of 30 patients (70%), preoperative mapping was possible. Preoperative mapping was feasible for successful intraoperative localization in 13 of 14 patients (93%) who underwent exogastric resection. CONCLUSIONS MFI combining CTG and CTA is a feasible method for developing preoperative and intraoperative "road maps" for performing laparoscopic exogastric wedge resection of small SMLs.
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Affiliation(s)
- Na Yeon Han
- Department of Radiology College of Medicine, Korea University, Anam Hospital, 126-1 5-Ka, Anam-Dong, Sungbuk-ku, Seoul, 136-705, Korea
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Fujimoto Y, Akiyoshi T, Konishi T, Nagayama S, Fukunaga Y, Ueno M. Laparoscopic sphincter-preserving surgery (intersphincteric resection) after neoadjuvant imatinib treatment for gastrointestinal stromal tumor (GIST) of the rectum. Int J Colorectal Dis 2014; 29:111-6. [PMID: 24018650 DOI: 10.1007/s00384-013-1769-7] [Citation(s) in RCA: 45] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 08/29/2013] [Indexed: 02/04/2023]
Abstract
BACKGROUND Gastrointestinal stromal tumors (GISTs) of the rectum are rarely found, and radical surgery such as abdominoperineal resection would be necessary for large rectal GIST. On the other hand, therapy for GIST has changed significantly with the use of imatinib. Neoadjuvant imatinib therapy may reduce tumor size and may potentially prevent extended surgery. Moreover, when sphincter-preserving surgery is carried out laparoscopically, it can be performed as minimally invasive surgery with preservation of the anus. METHODS From 2008 to 2011, five patients with rectal GIST were treated in our hospital. All patients received preoperative imatinib treatment (400 mg/day) and underwent laparoscopic sphincter-preserving surgery after 4-12 months of this treatment. RESULTS Initial median tumor size was 31 mm (range, 24-88). At the time of operation, the median tumor size was 24 mm (range, 11-52). Sphincter-preserving surgery was performed in all patients. Three patients underwent laparoscopic intersphincteric resection (ISR), and two patients underwent transanal full-thickness local resection and recto-anal anastomosis following laparoscopic ISR. Macroscopically complete resection was achieved, and microscopically, the resection margin was not involved of residual tumors. The median duration of postoperative hospital stay was 16 days (range, 13-30). No recurrence occurred in all patients during 1 to 4 years. CONCLUSIONS The present study suggests that neoadjuvant imatinib therapy might be effective to prevent extended surgery for rectal GIST, and laparoscopic sphincter-preserving surgery is safe and technically feasible. We recommend a combination of neoadjuvant imatinib therapy and laparoscopic ISR for locally advanced rectal GIST.
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Affiliation(s)
- Yoshiya Fujimoto
- Department of Gastroenterological Surgery, Cancer Institute Hospital, 3-8-31, Ariake, Koto-ku, Tokyo, 135-8550, Japan,
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Ou Z, Cao Z, He Y, Tang D. Diagnosis and multimodal therapy for extragastrointestinal stromal tumor of the prostate: A case report. Exp Ther Med 2013; 6:378-380. [PMID: 24137192 PMCID: PMC3786811 DOI: 10.3892/etm.2013.1156] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/05/2013] [Accepted: 05/30/2013] [Indexed: 01/13/2023] Open
Abstract
Extragastrointestinal stromal tumors (EGISTs), which are neoplasms outside the digestive tract, are predominantly observed in the greater omentum and retroperitoneum. The clinicopathological and molecular characteristics of EGISTs are similar to those of gastrointestinal stromal tumors (GISTs). EGISTs originating from the prostate are extremely rare. In this study, we report a case of a prostatic EGIST in a 39-year-old male, who presented with frequency, urgency, dysuria and a prostatic mass. A 10-core transrectal ultrasound-guided prostate biopsy was performed, and the histological and immunohistochemical results confirmed the diagnosis of EGIST. The patient received a radical prostatectomy, followed by targeted therapy with imatinib (400 mg, daily) for 1 year. Neither recurrence nor metastasis was detected at a 24-month follow-up.
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Affiliation(s)
- Zhenyu Ou
- Department of Urology, Xiangya Hospital, Central South University, Changsha, Hunan 410008
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Bachet JB, Tabone-Eglinger S, Dessaux S, Besse A, Brahimi-Adouane S, Emile JF, Blay JY, Alberti L. Gene expression patterns of hemizygous and heterozygous KIT mutations suggest distinct oncogenic pathways: a study in NIH3T3 cell lines and GIST samples. PLoS One 2013; 8:e61103. [PMID: 23593401 PMCID: PMC3625162 DOI: 10.1371/journal.pone.0061103] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2012] [Accepted: 03/05/2013] [Indexed: 12/18/2022] Open
Abstract
Objective Most gain of function mutations of tyrosine kinase receptors in human tumours are hemizygous. Gastrointestinal stromal tumours (GIST) with homozygous mutations have a worse prognosis. We aimed to identify genes differentially regulated by hemizygous and heterozygous KIT mutations. Materials and Methods Expression of 94 genes and 384 miRNA was analysed with low density arrays in five NIH3T3 cell lines expressing the full-length human KIT cDNA wild-type (WT), hemizygous KIT mutation with del557-558 (D6) or del564-581 (D54) and heterozygous WT/D6 or WT/D54. Expression of 5 of these genes and 384 miRNA was then analysed in GISTs samples. Results Unsupervised and supervised hierarchical clustering of the mRNA and miRNA profiles showed that heterozygous mutants clustered with KIT WT expressing cells while hemizygous mutants were distinct. Among hemizygous cells, D6 and D54 expressing cells clustered separately. Most deregulated genes have been reported as potentially implicated in cancer and severals, as ANXA8 and FBN1, are highlighted by both, mRNA and miRNA analyses. MiRNA and mRNA analyses in GISTs samples confirmed that their expressions varied according to the mutation of the alleles. Interestingly, RGS16, a membrane protein of the regulator of G protein family, correlate with the subcellular localization of KIT mutants and might be responsible for regulation of the PI3K/AKT signalling pathway. Conclusion Patterns of mRNA and miRNA expression in cells and tumours depend on heterozygous/hemizygous status of KIT mutations, and deletion/presence of TYR568 & TYR570 residues. Thus each mutation of KIT may drive specific oncogenic pathways.
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Affiliation(s)
- Jean-Baptiste Bachet
- EA4340 'Epidémiologie et Oncogénèse des tumeurs digestives', Faculté de médecine PIFO, UVSQ, Guyancourt, France
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Kapoor R, Khosla D, Kumar P, Kumar N, Bera A. Five-year follow up of patients with gastrointestinal stromal tumor: recurrence-free survival by risk group. Asia Pac J Clin Oncol 2013; 9:40-46. [PMID: 22897235 DOI: 10.1111/j.1743-7563.2011.01494.x] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
AIM Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract. There is limited published data on GIST from the Indian subcontinent. This 5-year retrospective analysis of 49 patients treated for GIST reports clinical and pathological features and survival outcome by risk stratification. METHODS We reviewed 49 cases of GIST from January 2004 to December 2008. Imatinib was administered after surgery in patients with either high-risk, residual or metastatic disease and at onset of recurrence or metastatic disease in patients with intermediate risk. RESULTS The mean age was 50 years (range, 17-80 years). Patients with localized tumor were classified as low (n = 2), intermediate (n = 4) and high risk (n = 32), based on the primary tumor and mitotic index. At a median follow up of 21 months, 2-year and 3-year recurrence or progression-free survival rates were 61 and 39%, respectively, for all patients. The median recurrence-free survival rate in the intermediate-risk and high-risk groups were 7 and 49 months, respectively. The median progression-free survival in the residual (n = 4) and metastatic disease group (n = 7) was 10 and 29 months, respectively. CONCLUSION This study demonstrates the role of imatinib in adjuvant and therapeutic settings. Responses have been durable and most patients tolerate the drug well at clinically effective doses. In view of high recurrence rates in the intermediate-risk group in our study, it would be better to keep these patients on strict follow up to detect recurrence at the earliest opportunity.
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Affiliation(s)
- Rakesh Kapoor
- Department of Radiotherapy and Oncology, Post-graduate Institute of Medical Education and Research, Regional Cancer Centre, Chandigarh, India
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Sanon M, Taylor DCA, Parthan A, Coombs J, Paolantonio M, Sasane M. Cost-effectiveness of 3-years of adjuvant imatinib in gastrointestinal stromal tumors (GIST) in the United States. J Med Econ 2013; 16:150-9. [PMID: 22762291 DOI: 10.3111/13696998.2012.709204] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
BACKGROUND Recent clinical trial data have demonstrated that 3 years vs 1 year of adjuvant imatinib therapy for patients with surgically resected Kit+ Gastrointestinal Stromal Tumors (GIST) leads to a significant improvement in recurrence-free survival and overall survival. This study assesses the cost-effectiveness of treating patients with 3 years vs 1 year of imatinib from a US payer's perspective. METHODS A Markov model was developed to predict GIST recurrence and treatment costs. Patients enter the model after surgery and transition among three health states: free of recurrence, recurrence, and death. Recurrence, mortality, costs, and utilities were derived from clinical trial and published literature. Expected costs and quality-adjusted life years (QALYs) were estimated and discounted at 3%/year. Deterministic and probabilistic sensitivity analyses were conducted. RESULTS Patients receiving 3 years of imatinib had higher QALYs (8.53 vs 7.18) than those receiving 1 year of imatinib. Total lifetime per-patient cost was $302,100 for 3 years vs $217,800 for 1 year of imatinib. Incremental cost effectiveness ratio of 3 years vs 1 year of imatinib was $62,600/QALY. Model results were sensitive to long-term rate of GIST recurrence (beyond 5 years) and cost of imatinib. At a threshold of $100,000/QALY, 3 years vs 1 year of imatinib was cost-effective in 100% of simulations. LIMITATIONS The model is a simplified representation of disease natural history and may not account for all possible health states and complications associated with disease. Resource utilization on treatment was estimated using the resource use data from previous trials, therefore calculated medical costs might be over-estimated compared to the real-world setting. CONCLUSIONS Model results suggest that treatment with 3 years vs 1 year of imatinib is cost-effective at a $100,000/QALY threshold. Clinical and economic results suggest treating surgically resected Kit+ GIST patients with 3 years of imatinib would result in improved quality-adjusted survival.
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Liu SL, Chen G, Zhao YP, Wu WM, Zhang TP. Optimized dose of imatinib for treatment of gastrointestinal stromal tumors: a meta-analysis. J Dig Dis 2013; 14:16-21. [PMID: 23121684 DOI: 10.1111/1751-2980.12010] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECTIVE To investigate the efficacy and safety of standard-dose versus high-dose imatinib. METHODS After a systematic review of English language articles, five studies including 2008 patients were eligible for the meta-analysis. Data extracted from each study were synthesized into overall odds ratios (OR). RESULTS The overall OR for the high dose vs standard dose was 1.19 (95% CI 1.00-1.42) and the Z-score for the overall effect was 1.93 (P = 0.054), suggesting that high-dose imatinib added no survival benefits. The dose-related toxicity was also assessed in the same way. The rates of rash, hemorrhage, nausea, vomiting and taste disturbance increased as dose increased (P < 0.05), whereas the incidence of headache, abdominal pain, edema, fatigue, anemia, infection, muscle cramp and constipation was nearly identical and showed no significant difference. CONCLUSIONS Imatinib at a standard dose produces a similar effect to that at a high dose. The severity of the toxicity is associated with the dose of imatinib. However, larger and randomized studies are needed to draw definitive conclusions.
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Affiliation(s)
- Shang Long Liu
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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Prolonged Therapy with Imatinib Mesylate before Surgery for Advanced Gastrointestinal Stromal Tumor Results of a Phase II Trial. Int J Surg Oncol 2012; 2012:761576. [PMID: 23316352 PMCID: PMC3534224 DOI: 10.1155/2012/761576] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2012] [Revised: 11/15/2012] [Accepted: 11/15/2012] [Indexed: 01/13/2023] Open
Abstract
Purpose. Proven efficacy of imatinib mesylate in gastrointestinal stromal tumour (GIST) has led to its use in advanced disease and, more recently, in adjuvant and neoadjuvant settings. The purpose of this study was to evaluate the optimal neoadjuvant imatinib duration to reduce the morbidity of surgery and increase the possibility of resection completeness in advanced tumours. Patients and Method. Patients with advanced GIST were enrolled into a registered open-label multicenter trial and received imatinib daily for a maximum of 12 months, followed by en bloc resection. Data were prospectively collected regarding tumour assessment, response rate, surgical characteristics, recurrence, and survival. Results. Fourteen patients with advanced GIST were enrolled. According to RECIST criteria, 6 patients had partial response and 8 had stable disease. The overall tumour size reduction was 25% (0–62.5%), and there was no tumour progression. Eleven patients underwent tumour resection, and all had R0 resection. After a median followup of 48 months, 4-year OS and DFS were 100% and 64%, respectively. Conclusion. This prospective trial showed that one year of neoadjuvant imatinib in advanced GIST is safe and associated with high rate of complete microscopic resection. It is not associated with increased resistance, progression, or complication rates.
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Pavlidis N, Stahel R, Pentheroudakis G, Cervantes A. ESMO Consensus Conferences: another source of ESMO Clinical Practice Guidelines. Ann Oncol 2012; 23 Suppl 7:vii7-10. [PMID: 22997457 DOI: 10.1093/annonc/mds222] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022] Open
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Xue D, Chen H, Chen Y. Giant extragastrointestinal stromal tumor in the transverse mesocolon concomitant with gastric cancer in an elderly patient: Case report. Oncol Lett 2012; 5:627-630. [PMID: 23420829 PMCID: PMC3573037 DOI: 10.3892/ol.2012.1030] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2012] [Accepted: 11/06/2012] [Indexed: 12/13/2022] Open
Abstract
Extragastrointestinal stromal tumors (EGISTs) are neoplasms located outside the gastrointestinal tract in sites including the omentum, mesentery and retroperitoneum. EGISTs of the transverse mesocolon are rarely noted in the literature. Herein, we describe a rare case of giant EGIST concomitant with gastric cancer in a 78-year-old male who presented with upper abdominal pain and a palpable mass. The patient underwent en bloc resection of the tumor with a distal gastrectomy, with a D2 lymphadenectomy for the gastric cancer, accompanied with resection of a segment of the transverse colon. The patient received targeted therapy (imatinib 400 mg, daily) and adjuvant chemotherapy with FOLFOX (six cycles). Neither recurrence nor metastasis was observed after 24 months of follow-up.
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Affiliation(s)
- Dong Xue
- Department of General Surgery, Qilu Hospital, Shandong University, Jinan 250012, P.R. China
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Poveda A, Rivera F, Martín J. SEOM guidelines for gastrointestinal stromal sarcomas (GIST). Clin Transl Oncol 2012; 14:536-40. [PMID: 22721799 DOI: 10.1007/s12094-012-0837-8] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Gastrointestinal stromal sarcomas (GISTs) are the most common mesenchymal tumours originating in the digestive tract. These tumours have become a model of multidisciplinary work in oncology: the participation of several specialities (oncologists, pathologists, surgeons, molecular biologists, radiologists and others) has allowed advances in the understanding of this tumour and the consolidation of a targeted therapy, imatinib, as the first molecular treatment that is efficacious in solid tumours. Following the introduction of this drug, median survival of patients with advanced stage GIST has gone from 18 to more than 60 months. Therapy planning of GIST must be considered within a multidisciplinary context, and it is advisable that it takes place in reference centres for the care of sarcomas and GIST participating in clinical trials.
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Affiliation(s)
- Andrés Poveda
- Servicio de Oncología Médica, Fundación Instituto Valenciano de Oncología, Valencia, Spain.
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Koh Y, Lee HE, Oh DY, Kim JH, Lee SH, Kim SH, Kim DW, Im SA, Kim TY, Heo DS, Kim WH, Bang YJ. The lack of CD34 expression in gastrointestinal stromal tumors is related to cystic degeneration following imatinib use. Jpn J Clin Oncol 2012; 42:1020-1027. [PMID: 22952296 DOI: 10.1093/jjco/hys138] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
Abstract
OBJECTIVE We evaluated the characteristics of the gastrointestinal stromal tumors that showed discrepancies between their assessment using the Response Evaluation Criteria in Solid Tumor (RECIST) and Choi's criteria. We also investigated the clinical applicability of Choi's criteria to Korean gastrointestinal stromal tumor patients undergoing imatinib therapy. METHODS Patients with advanced gastrointestinal stromal tumors treated with frontline imatinib were analyzed. Computed tomography images of these patients were reviewed and genotyping for the KIT and PDGFRA genes was performed. Immunohistochemical staining of c-KIT, CD34, platelet derived growth factor receptor-alpha, platelet derived growth factor receptor-beta, AKT, P-ERK and vascular endothelial growth factor was followed. RESULTS Ninety-five patients were enrolled. When using Choi's criteria to evaluate the 61 patients who achieved at least partial response by Choi's criteria, 27 patients showed discrepancies in their response to treatment between these two sets of criteria. A lack of CD34 expression in tumors was found to be related to cystic degeneration after imatinib treatment (P=0.001). Patients who showed partial response by Choi's criteria but stable disease by RECIST criteria had a similar progression-free survival to cases who showed a partial response under both systems (P=0.951). CONCLUSIONS Gastrointestinal stromal tumors showing cystic degeneration after imatinib treatment lack CD34 expression. Choi's criteria have a clinical value in terms of the progression-free survival in Korean patients treated with imatinib.
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Affiliation(s)
- Youngil Koh
- Department of Internal Medicine, Seoul National University Hospital, Cancer Research Institute, Seoul National University College of Medicine, 101 Daehangro, Chongno-gu, Seoul 110-744, Republic of Korea
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Zhou L, Liu C, Bai JG, Wei JC, Qu K, Tian F, Tai MH, Wang RT, Meng FD. A rare giant gastrointestinal stromal tumor of the stomach traversing the upper abdomen: a case report and literature review. World J Surg Oncol 2012; 10:66. [PMID: 22540369 PMCID: PMC3488525 DOI: 10.1186/1477-7819-10-66] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2011] [Accepted: 04/27/2012] [Indexed: 01/01/2023] Open
Abstract
We present the case of a 66-year-old woman with a huge gastrointestinal stromal tumor of the stomach that traversed her upper abdomen. The predominant abdominal sign was a huge, palpable mass, but there were no other distinctive findings in her physical examination or her routine blood workup, including biochemical markers. It was difficult to judge the origin of the mass upon imaging. Furthermore, radiological findings revealed that the mass had a complex relationship with many major blood vessels. An exploratory laparotomy revealed a huge tumor protruding from the anterior wall of the stomach fundus, on the lesser curvature of the stomach, measuring approximately 21 × 34 × 11 cm in diameter and weighing 5.5 kg. A complete resection was performed and the tumor was characterized on immunohistochemistry as a gastrointestinal stromal tumor of the stomach. Preoperative diagnosis of gastrointestinal stromal tumors can be difficult, and we hope that the presentation of this rare case and literature review will benefit other diagnosing clinicians having similar problems.
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Affiliation(s)
- Lei Zhou
- Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
| | - Chang Liu
- Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
| | - Ji-Gang Bai
- Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
| | - Ji-Chao Wei
- Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
| | - Kai Qu
- Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
| | - Feng Tian
- Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
| | - Ming-Hui Tai
- Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
| | - Rui-Tao Wang
- Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
| | - Fan-Di Meng
- Department of Hepatobiliary Surgery, the First Affiliated Hospital, School of Medicine, Xi’an Jiao tong University, Xi’an, 710061, China
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Chandrasekhara V, Ginsberg GG. Endoscopic management of gastrointestinal stromal tumors. Curr Gastroenterol Rep 2012; 13:532-9. [PMID: 21931997 DOI: 10.1007/s11894-011-0224-6] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Subepithelial lesions are increasingly being identified with improved endoscopic imaging technologies. Many of these lesions are now recognized as gastrointestinal stromal tumors (GISTs). Recent advances in immunohistochemistry have allowed for reliable differentiation of GISTs from other subepithelial tumors, thereby significantly improving our understanding of these lesions. The wealth of recent information and continual evolution in our understanding of GISTs has exposed some knowledge gaps pertaining to the optimal management of these lesions. In this article, we review the endoscopic management of GISTs as it relates to the identification, diagnosis and management of these lesions based on the best available literature and our own clinical experience to date.
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Affiliation(s)
- Vinay Chandrasekhara
- Hospital of the University of Pennsylvania, GI Division, 3 Ravdin, 3400 Spruce Street, Philadelphia, PA 19104, USA.
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Barnes T, Reinke D. Practical management of imatinib in gastrointestinal stromal tumors. Clin J Oncol Nurs 2012; 15:533-45. [PMID: 21951740 DOI: 10.1188/11.cjon.533-545] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Gastrointestinal stromal tumors (GISTs) have an incidence of 7-15 occurrences per million people. Tyrosine kinase inhibitors (TKIs) have significantly improved clinical outcomes as part of multidisciplinary disease management. The authors will review developments in the management of GISTs, including diagnosis, risk stratification, prognosis, and treatment with imatinib. Imatinib is recommended for postsurgical adjuvant therapy and, where appropriate, neoadjuvant therapy. Clinical practice guidelines recommend first-line imatinib for metastatic and unresectable GISTs based on trials showing efficacy at the standard dose (400 mg per day) and at higher doses of 600-800 mg per day. Oncology nurses play a key role in patient management through (a) patient education about GISTs and their treatment including the use of imatinib, (b) timely scheduling of radiologic follow-up to assess treatment response, (c) monitoring treatment adherence, (d) helping to sustain imatinib dose intensity by monitoring toxicities and drug interactions and by counseling patients to prevent treatment interruptions, and (e) collaborating with the multidisciplinary medical team to pursue imatinib dose escalation or other treatment options if patients have primary or acquired mutation-based resistance to imatinib.
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Affiliation(s)
- Tamara Barnes
- Investigational Cancer Therapeutics, University of Texas MD Anderson Cancer Center in Houston, USA.
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Pisters PWT, Colombo C. Adjuvant imatinib therapy for gastrointestinal stromal tumors. J Surg Oncol 2011; 104:896-900. [PMID: 22069174 DOI: 10.1002/jso.22002] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/18/2023]
Abstract
Surgery is the standard of care for primary resectable gastrointestinal stromal tumors (GISTs), but half of surgically treated patients relapse. Imatinib (IM) has been shown to prolong recurrence-free survival after complete surgery and is now approved as adjuvant therapy (400 mg/day) for high-risk GIST patients. IM is well tolerated, with mild to moderate side effects observed. Whether adjuvant IM prolongs overall survival is under evaluation in two ongoing clinical trials.
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Affiliation(s)
- Peter W T Pisters
- Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas 77030-4009, USA.
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Linhares E, Gonçalves R, Valadão M, Vilhena B, Herchenhorn D, Romano S, Ferreira MA, Ferreira CG, Ramos CDA, Jesus JPD. Tumor estromal gastrointestinal: análise de 146 casos do centro de referência do Instituto Nacional do Câncer - INCA. Rev Col Bras Cir 2011; 38:398-406. [DOI: 10.1590/s0100-69912011000600006] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/21/2010] [Accepted: 02/25/2011] [Indexed: 01/14/2023] Open
Abstract
OBJETIVO: Avaliar os resultados do tratamento de GIST no INCA. MÉTODOS: Análise retrospectiva de todos os casos de GIST tratados no INCA no período de 1997 a 2009. RESULTADOS: Analisamos 146 pacientes, com média de idade de 44,5 anos e predomínio do sexo feminino. O principal sintoma foi dor abdominal. Tivemos ocorrência de segundo primário em 22% dos casos e na imuno-histoquímica, 92% foram positivos para CD117. A localização mais frequente foi estômago e predominou o grupo de alto risco. A cirurgia foi R0 (extenso) em 70% e os principais sítios de metástases foram fígado e peritônio. A sobrevida global foi, respectivamente, em dois e cinco anos de 86% e 59%. Houve significante diferença entre a sobrevida global (p=0,29) do grupo de alto risco versus os demais. CONCLUSÃO: Os nossos pacientes apresentam-se principalmente sob forma de doença de alto risco com repercussão óbvia na sobrevida. O uso de Imatinib melhorou a sobrevida dos pacientes com doença metastática e recidivada. Devemos estudar seu uso no cenário de adjuvância e neoadjuvancia visando melhorar os índices do grupo de alto risco. A criação de centros referenciais é uma necessidade para o estudo de doenças pouco frequentes.
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Outcomes of single-centre experience of hepatic resection and cryoablation of sarcoma liver metastases. Am J Clin Oncol 2011; 34:317-20. [PMID: 20622642 DOI: 10.1097/coc.0b013e3181e1d078] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Abstract
BACKGROUND Liver metastasis from sarcoma in metastatic disease is common and its effect on prognosis remains ill defined. Surgery remains paramount in sarcoma management; however, there is inadequate literature reporting the role of hepatic surgery as treatment for sarcoma liver metastases. METHODS Fifteen patients who underwent hepatic resection for metastatic sarcoma between January 1, 1995 and January 1, 2009 were identified from a prospective hepatobiliary database. Clinicopathologic, operative, recurrence, and long-term survival data are presented. RESULTS Three patients had synchronous liver metastases. The median time to developing liver metastasis was 26 (range, 0-206) months. The overall median survival from hepatic resection of liver metastasis was 103 (95% confidence interval, 6-200) months with a 5- and 10-year survival of 51% and 37%, respectively. The median disease-free survival from hepatic resection was 14 (95% confidence interval, 11-18) months. CD117 positive tumors was associated with an improved survival with a 3-year survival of 80% compared with 33% in CD117 negative tumors (P = 0.02). CONCLUSIONS Hepatic resection for sarcoma liver metastases with or without extrahepatic disease is a reasonable management strategy if a complete resection may be achieved. Concomitant treatment of CD117 positive tumors with imatinib leads to long-term survival.
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Iannicelli E, Sapori A, Panzuto F, Pilozzi E, Delle Fave G, David V. Oesophageal GIST: MDCT Findings of Two Cases and Review of the Literature. J Gastrointest Cancer 2011; 43:481-5. [DOI: 10.1007/s12029-011-9295-8] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Otto C, Agaimy A, Braun A, Rädecke J, Hoeppner J, Illerhaus G, Werner M, Kontny U, Haller F. Multifocal gastric gastrointestinal stromal tumors (GISTs) with lymph node metastases in children and young adults: a comparative clinical and histomorphological study of three cases including a new case of Carney triad. Diagn Pathol 2011; 6:52. [PMID: 21663639 PMCID: PMC3130635 DOI: 10.1186/1746-1596-6-52] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/28/2011] [Accepted: 06/10/2011] [Indexed: 12/30/2022] Open
Abstract
Background Gastrointestinal stromal tumors (GISTs) are the most frequent mesenchymal tumors of the gastrointestinal tract usually occurring in the 6th to 7th decade of life, while their occurrence in children is rare (1-2%). Carney triad (CT), a non-hereditary association of gastric GIST with pulmonary chondroma and/or extraadrenal paraganglioma, is an even much rarer disease (to date ~120 cases reported worldwide) usually affecting young adult females. Pediatric GISTs differ from CT-associated GISTs solely by the absence of other components of the triad and are completely different from sporadic GISTs of the adult. Both, pediatric and CT-GISTs, metastasize frequently to regional lymph nodes (29%) and are usually wild type (WT) for common KIT-/PDGFRA mutations. Case presentation and results We compare one new CT GIST with two pediatric/young adult multifocal gastric GISTs presenting with lymph node metastases. We put special focus on histomorphological growth pattern in the primary tumors and in the metastases. The two cases of pediatric/young adult GIST without the other components of CT showed all the features of the triad: female gender, young age, multifocal antral-based gastric GIST with biphasic histological growth pattern, lymph node metastases, hypercellularity and WT status for common KIT-, PDGFRA- and B-RAF mutations. Discussion and conclusion Pediatric/CT-associated GISTs and sporadic GISTs of the adults differ significantly from each other with regard to patients' age, gender, tumor localisation, histomorphological growth pattern, mutational status and risk for metastasis. Our cases of pediatric/young adult GISTs show all characteristics of CT except for the absence of other components of the triad. Therefore these GISTs are probably not sporadic, but may represent either early manifestation or forme fruste of the CT. Thus, these patients need to be regularly examined for the development of extraadrenal paraganglioma or pulmonary chondroma.
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Affiliation(s)
- Claudia Otto
- Institute of Pathology, University Hospital Freiburg, Germany.
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