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Christodoulidis G, Kouliou MN, Ragias D, Chatziisaak D, Agko ES, Schizas D, Zacharoulis D. Last decade of advances in gastric neuroendocrine tumors: Innovations, challenges, and future directions. World J Clin Oncol 2025; 16:104577. [DOI: 10.5306/wjco.v16.i5.104577] [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] [Received: 12/24/2024] [Revised: 02/27/2025] [Accepted: 03/10/2025] [Indexed: 05/19/2025] Open
Abstract
BACKGROUND Gastric neuroendocrine tumors (G-NETs) are rare tumors originating from enterochromaffin-like cells, with an incidence of 0.4 per 100000 annually. There are three main types: (1) Type I, linked to chronic atrophic gastritis and hypergastrinemia, makes up 75%–80% of G-NETs; (2) Type II, associated with Zollinger-Ellison syndrome (ZES) and multiple endocrine neoplasia, comprises 5%; and (3) Type III, sporadic tumors with a higher metastatic potential, accounting for 15%–25%. Diagnosis involves endoscopy, biopsy, and histological examination. Additional methods include serum gastrin testing, immunohistochemistry, and imaging techniques such as computer tomography or magnetic resonance imaging for detecting metastasis. Type I treatment usually involves endoscopic resection (ER), with surgical resection for recurrence. Somatostatin analogs (SSAs) can reduce tumor size, and the prognosis is generally excellent. Type II treatment centers on surgical removal of the gastrinoma, with ER for smaller lesions and SSAs for symptom management. Type III requires surgical resection (partial or total gastrectomy) with lymph node dissection, and possibly chemotherapy. This type has a worse prognosis due to its aggressive nature. Emerging treatments like Peptide Receptor Radionuclide Therapy are promising for advanced cases, and ongoing research into immunotherapies is expanding future treatment options. Regular endoscopic follow-up is crucial to monitor for recurrence or metastasis across all types. Our literature review explores the current perspectives on G-NETs and highlights the importance of further research to improve diagnostic precision and treatment, particularly for those associated with less favorable cases.
AIM To improve diagnostic precision and treatment, particularly for those associated with less favorable cases.
METHODS A systematic search was conducted in PubMed, Scopus, and Web of Science until September 2024. Two independent reviewers screened titles, abstracts, and full texts for eligibility based on G-NET treatment in adults. Eligible studies included cohort studies, clinical trials, case series, and case reports, while in vitro, pediatric, and non-English studies were excluded. Relevant data were extracted independently, and disagreements were resolved through discussion. Study quality was assessed using appropriate tools.
RESULTS G-NETs are rare, classified into three types: (1) Type I; (2) Type II; and (3) Type III. Type I G-NETs, often associated with chronic atrophic gastritis, are typically slow-growing and low-grade, with favorable outcomes following surgical resection. Type II G-NETs arise in hypergastrinemia conditions like multiple endocrine neoplasia and ZES, showing moderate malignancy risk. Type III G-NETs, the most aggressive and least common, present with distant metastases and poor prognosis. Diagnosis relies on endoscopy, imaging, and biomarkers like chromogranin A. Treatment varies by type, ranging from ER to aggressive surgery and chemotherapy for advanced cases. Regular follow-up is essential to monitor recurrence, particularly for type III G-NETs.
CONCLUSION G-NETs require tailored diagnosis and treatment based on type and stage. Types I and II generally have better prognosis, while types III and IV are linked to poorer outcomes due to invasion and metastasis. Treatment strategies vary from ER for type I to extensive surgery for type III. Emerging therapies, like somatostatin analogs and peptide-receptor radionuclide therapies, show promise in advanced cases. Further research is essential to improve early diagnosis and treatment, particularly for high-risk lesions.
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Affiliation(s)
| | - Marina Nektaria Kouliou
- Department of Internal Medicine, General Hospital of Argolida-Hospital Unit of Nafplio, Nafplio 21100, Pelopónnisos, Greece
| | - Dimitrios Ragias
- Department of Oncology, 251 Air Force General Hospital, Athens 11525, Greece
| | - Dimitrios Chatziisaak
- Department of Surgery, Kantonsspital St.Gallen, St.Gallen 9000, Switzerland
- Department of Surgery, Centre Hospitalier Universitaire Vaudois, Lausanne 1005, Switzerland
| | - Eirini Sara Agko
- Department of Intensive Care Unit, Asklepios Paulinen Clinic Wiesbaden, Wiesbaden 65197, Germany
| | - Dimitrios Schizas
- Department of Surgery, National and Kapodistrian University of Athens, Athens 11527, Greece
| | - Dimitrios Zacharoulis
- Department of General Surgery, University of Thessaly, Larisa 41110, Thessalia, Greece
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Chen F, Gonzalez RS. Evaluation of enterochromaffin-like cell hyperplasia can help categorize patients with Helicobacter-negative atrophic gastritis. Am J Clin Pathol 2025; 163:601-609. [PMID: 39724194 DOI: 10.1093/ajcp/aqae159] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/11/2024] [Accepted: 12/19/2024] [Indexed: 12/28/2024] Open
Abstract
OBJECTIVES Atrophic gastritis (AG) is characterized by atrophy of gastric glands-in particular, oxyntic glands-in the setting of chronic inflammation; it is often autoimmune. The diagnosis is confirmed by immunohistochemistry (IHC) for gastrin (to confirm biopsy site), and pathologists often use IHC for neuroendocrine markers to evaluate for enterochromaffin-like cell hyperplasia (ECL-H). The utility of neuroendocrine staining is unclear, and we undertook this study to determine whether ECL pattern provided any additional information in cases of Helicobacter-negative AG. METHODS We reviewed clinicopathologic findings in 184 cases from 184 patients with histologic AG and no evidence of Helicobacter infection. Using neuroendocrine IHC markers, cases were divided into 3 groups: Group 1 showed complete ECL-H (both qualitative and quantitative criteria met), group 2 showed focal ECL-H (qualitative but not quantitative criteria met), and group 3 showed no ECL-H (neither criteria met). RESULTS Group 1 patients were more likely to have positive autoantibody serologies (73%, P = .0007 vs group 2) and higher mean gastrin levels (700 pg/mL, P = .017 vs group 3), and only these patients developed gastric neuroendocrine tumors. Group 2 patients were more likely to take proton pump inhibitors (64%, P = .0002 vs group 1). Group 3 patients were more likely to be male (70%, P = .008 vs group 1) and to have microcytic anemia (44%, P = .022 vs group 2) and less likely to have intestinal metaplasia (50%, P = .044 vs group 1). CONCLUSIONS Stratification based on degree of ECL-H is not necessary for diagnosis of AG but does lead to statistically significant clinical and pathologic differences among groups.
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Affiliation(s)
- Feidi Chen
- Department of Pathology, Beth Israel Deaconess Medical Center, Boston, MA, US
| | - Raul S Gonzalez
- Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, GA, US
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Zhou XP, Sun LB, Liu WH, Song XY, Gao Y, Xing JP, Gao SH. Development and validation of predictive models for distant metastasis and prognosis of gastroenteropancreatic neuroendocrine neoplasms. Sci Rep 2025; 15:9510. [PMID: 40108260 PMCID: PMC11923110 DOI: 10.1038/s41598-025-92974-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2024] [Accepted: 03/04/2025] [Indexed: 03/22/2025] Open
Abstract
Imaging examinations exhibit a certain rate of missed detection for distant metastases of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs). This study aims to develop and validate a risk prediction model for the distant metastases and prognosis of GEP-NENs. This study included patients diagnosed with gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) from the Surveillance, Epidemiology, and End Results (SEER) database between 2010 and 2015. External validation was performed with patients from the China-Japan Union Hospital of Jilin University. Univariate and multivariate logistic regression analyses were conducted on the selected data to identify independent risk factors for distant metastasis in GEP-NENs. A nomogram was subsequently developed using these variables to estimate the probability of distant metastasis in patients with GEP-NENs. Subsequently, patients with distant metastasis from GEP-NENs were selected for univariate and multivariate Cox regression analyses to identify prognostic risk factors. A nomogram was subsequently developed to predict overall survival (OS) in patients with GEP-NENs. Finally, the developed nomogram was validated using Receiver Operating Characteristic (ROC) curves, calibration curves, and Decision Curve Analysis (DCA). Kaplan-Meier analysis was employed to evaluate survival differences between high-risk and low-risk groups. A total of 11,207 patients with GEP-NENs were selected from the SEER database, and 152 patients from the China-Japan Union Hospital of Jilin University were utilized as an independent external validation cohort. Univariate and multivariate logistic regression analyses revealed that the primary tumor site, tumor grade, pathological type, tumor size, T stage, and N stage are independent predictors of distant metastasis in GEP-NENs. Additionally, among the 1732 patients with distant metastasis of GEP-NENs, univariate and multivariate Cox regression analyses identified N stage, tumor size, pathological type, primary site surgery, and tumor grade as independent prognostic factors. Based on the results of the regression analyses, a nomogram model was developed. Both internal and external validation results demonstrated that the nomogram models exhibited high predictive accuracy and significant clinical utility. In summary, we developed an effective predictive model to assess distant metastasis and prognosis in GEP-NENs. This model assists clinicians in evaluating the risk of distant metastasis and in assessing patient prognosis.
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Affiliation(s)
- Xuan-Peng Zhou
- China-Japan Union Hospital of Jilin University, Changchun, 130000, Jilin, People's Republic of China
| | - Luan-Biao Sun
- China-Japan Union Hospital of Jilin University, Changchun, 130000, Jilin, People's Republic of China
| | - Wen-Hao Liu
- China-Japan Union Hospital of Jilin University, Changchun, 130000, Jilin, People's Republic of China
| | - Xin-Yuan Song
- The Chinese University of Hong Kong, New Territories, 999077, Hong Kong Special Administrative Region, People's Republic of China
| | - Yang Gao
- Zhalute Banner People's Hospital, Tongliao, 029100, Inner Mongolia Autonomous Region, People's Republic of China
| | - Jian-Peng Xing
- China-Japan Union Hospital of Jilin University, Changchun, 130000, Jilin, People's Republic of China.
| | - Shuo-Hui Gao
- China-Japan Union Hospital of Jilin University, Changchun, 130000, Jilin, People's Republic of China.
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Gao Y, Ye L, Li X, He L, Yu B, Liu W, Cao Y, Chen L, Mou Y, Chen O, Xie J, Du J, Zhang Q, Hu B. Double Band Ligation-Assisted Endoscopic Submucosal Resection for Rectal Neuroendocrine Tumors: Comparison With Conventional Endoscopic Mucosal Resection With Ligation (With Video). Clin Transl Gastroenterol 2025:01720094-990000000-00372. [PMID: 39968991 DOI: 10.14309/ctg.0000000000000830] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/23/2024] [Accepted: 01/31/2025] [Indexed: 02/20/2025] Open
Abstract
INTRODUCTION Based on endoscopic mucosal resection with ligation (EMR-L), we developed double band ligation-assisted endoscopic submucosal resection (ESR) for complete resection of small submucosal rectal neuroendocrine tumors (NETs). Both procedures use a multiband device to perform resection, with the only difference being that ESR adds an additional band to obtain deeper resection margin. The aim of this retrospective study was to validate its feasibility, safety, and effectiveness compared with EMR-L. METHODS This retrospective study included consecutive patients with small (≤10 mm) suspected submucosal rectal NETs who underwent ESR (n = 45) or EMR-L (n = 26) between June 2018 and October 2023 at West China Hospital. En bloc resection rate, complete resection rate, procedure time, margin distance, and adverse events were compared between 2 groups. RESULTS En bloc resections were achieved in all patients. The complete resection rate of ESR was higher than EMR-L (100% vs 88.5%, P = 0.045). The vertical margin distance and lateral margin distance were significantly longer in ESR group than EMR-L group (vertical margin distance 782.31 ± 359.45 μm vs 363.84 ± 222.78 μm, P < 0.001; and lateral margin distance 4,205.75 ± 2,167.43 μm vs 3,162.94 ± 1,419.22 μm, P = 0.008, respectively). There were no significant differences in procedure time, adverse events, postprocedural hospital stay, or medical cost between 2 groups. In addition, there was no evidence of recurrence or metastasis during the follow-up. DISCUSSION ESR seems to be safe and effective for complete resection of small submucosal rectal NETs. Larger, multicenter, prospective studies are needed to further assess this technique.
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Affiliation(s)
- Yuan Gao
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
| | - Liansong Ye
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
| | - Xu Li
- Department of Clinical Research Management, Center of Biostatistics, Design, Measurement and Evaluation (CBDME), West China Hospital, Sichuan University, Chengdu, China
| | - Long He
- Department of Pancreatic Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Bin Yu
- Institute for Disaster Management and Reconstruction, Sichuan University, Chengdu, China
| | - Wei Liu
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
| | - Yuwan Cao
- School of Philosophical, Historical, and Interdisciplinary, Curating, University of Essex, Colchester, UK
| | - Liuxiang Chen
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
| | - Yi Mou
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
| | - Ou Chen
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
| | - Jia Xie
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
| | - Jiang Du
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
| | - Qiongying Zhang
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
| | - Bing Hu
- Department of Gastroenterology and Hepatology, Digestive Endoscopy Medical Engineering Research Laboratory, Med-X Center for Materials, West China Hospital, Sichuan University, Chengdu, China
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Zhang C, Dong HK, Gao JM, Zeng QQ, Qiu JT, Wang JJ. Advances in the diagnosis and treatment of MET-variant digestive tract tumors. World J Gastrointest Oncol 2024; 16:4338-4353. [PMID: 39554732 PMCID: PMC11551650 DOI: 10.4251/wjgo.v16.i11.4338] [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] [Received: 07/08/2024] [Revised: 08/24/2024] [Accepted: 09/10/2024] [Indexed: 10/25/2024] Open
Abstract
The receptor tyrosine kinase encoded by the MET gene plays an important role in various cellular processes such as growth, survival, migration and angiogenesis, and its abnormal activation is closely related to the occurrence and development of various tumors. This article reviews the recent advances in diagnosis and treatment of MET-variant digestive tract tumors. In terms of diagnosis, the application of next-generation sequencing technology and liquid biopsy technology makes the detection of MET variants more accurate and efficient, providing a reliable basis for individualized treatment. In terms of treatment, MET inhibitors such as crizotinib and cabotinib have shown good efficacy in clinical trials. In addition, the combination of immunotherapy and MET inhibitors also demonstrated potential synergies, further improving the therapeutic effect. However, the complexity and heterogeneity of drug resistance mechanisms are still one of the difficulties in current research. In the future, it is necessary to further deepen the understanding of the mechanism of MET variation and explore new combination treatment strategies to improve the overall survival rate and quality of life of patients. The diagnosis and treatment of MET-variant digestive tract tumors are moving towards precision and individualization, and have broad application prospects.
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Affiliation(s)
- Chen Zhang
- The First Department of Radiation Oncology, Lu’an Hospital of Traditional Chinese Medicine of Anhui Province, Lu’an 237000, Anhui Province, China
| | - Hu-Ke Dong
- The Fourth Department of Oncology, The First Affiliated Hospital of Anhui Medical University, Hefei 230000, Anhui Province, China
| | - Jian-Ming Gao
- The First Department of Oncology, The First Affiliated Hospital of Anhui University of Traditional Chinese Medicine, Hefei 230000, Anhui Province, China
| | - Qi-Qi Zeng
- Department of Gastroenterology, Nanjing University Affiliated Gulou Hospital, Nanjing 210008, Jiangsu Province, China
| | - Jiang-Tao Qiu
- Department of Gastrointestinal Surgery, Beijing Tsinghua Changgung Hospital, Beijing 100084, China
| | - Jia-Jia Wang
- Ultrasound of Medicine Department, The Second Affiliated Hospital of Anhui Medical University, Hefei 230601, Anhui Province, China
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Barona-Rommy D, Bravo JC, Varela MJ, Arango LG, Cañas CA. Erythema nodosum as first clinical manifestation of metastatic neuroendocrine tumor: A case report. Heliyon 2024; 10:e39405. [PMID: 39469682 PMCID: PMC11513511 DOI: 10.1016/j.heliyon.2024.e39405] [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: 05/13/2024] [Revised: 10/11/2024] [Accepted: 10/14/2024] [Indexed: 10/30/2024] Open
Abstract
Erythema nodosum (EN) is a reactive inflammatory panniculitis, which has been associated with medications, infections, autoimmune and autoinflammatory diseases. It has rarely been associated with neoplasms. We present the case of a 61-year old woman who was admitted because 3-week history of painful erythematous subcutaneous nodules on the lower limbs clinically consistent with EN, which was confirmed by skin biopsy. The patient denied use of medication. No general or systemic symptoms were present. As part of his study, an abdominal ultrasound and later magnetic resonance imaging (MRI) was done and lesions suggestive of liver metastases were reported. An ultrasound guided liver biopsy was then performed and pathology studies evidence a well differentiated grade II gastro-enteropancreatic neuroendocrine tumor (GEP-NET). A 9mTc-Octreotide scintigraphy evidenced a positive expression of somatostatin receptor in the liver and in a nodular mesenteric lesion in contact with an intestinal loop. The patient began treatment with lanreotide and was scheduled for cytoreductive surgery. During surgery, 50 cm of the small intestine, gallbladder, mesenteric fat and a 4.2x3.3 × 1 cm tumor located on the VII hepatic lobe were resected. Subsequently EN lesions of the lower extremities resolved. We present a rare case of GEP-NET-associated EN, that improved with surgical tumor cytoreduction and hormone therapy.
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Affiliation(s)
| | - Juan C. Bravo
- Fundación Valle Del Lili, Pathology Department, Cali, 760031, Colombia
| | - María J. Varela
- Universidad Icesi, Facultad de Ciencias de La Salud, Cali, 760031, Colombia
| | - Luis G. Arango
- Fundación Valle Del Lili, Unit of Endocrinology, Cali, 760031, Colombia
| | - Carlos A. Cañas
- Fundación Valle Del Lili, Unit of Rheumatology, Cali, 760031, Colombia
- Universidad Icesi, CIRAT: Centro de Investigación en Reumatología, Autoinmunidad y Medicina Traslacional, Cali, 760031, Colombia
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Tan B, Zhang B, Chen H. Gastroenteropancreatic neuroendocrine neoplasms: epidemiology, genetics, and treatment. Front Endocrinol (Lausanne) 2024; 15:1424839. [PMID: 39411312 PMCID: PMC11474919 DOI: 10.3389/fendo.2024.1424839] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2024] [Accepted: 09/10/2024] [Indexed: 10/19/2024] Open
Abstract
The incidence of gastroenteropancreatic neuroendocrine neoplasms (GEP NEN) is increasing at a rapid pace and is becoming an increasingly important consideration in clinical care. Epidemiological data from multiple countries indicate that the incidence of gastroenteropancreatic neuroendocrine neoplasms (GEP NEN) exhibits regional, site-specific, and gender-based variations. While the genetics and pathogenesis of some GEP NEN, particularly pancreatic NENs, have been investigated, there are still many mechanisms that require further investigation. The management of GEP NEN is diverse, but surgery remains the primary option for most cases. Peptide receptor radionuclide therapy (PRRT) is an effective treatment, and several clinical trials are exploring the potential of immunotherapy and targeted therapy, as well as combination therapy.
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Affiliation(s)
- Baizhou Tan
- Department of Histology and Embryology, School of Basic Medical Sciences, Jiangxi Medical College, Nanchang University, Nanchang, China
- Queen Mary School, Jiangxi Medical College, Nanchang University, Nanchang, China
| | - Beiyu Zhang
- Department of Histology and Embryology, School of Basic Medical Sciences, Jiangxi Medical College, Nanchang University, Nanchang, China
- Queen Mary School, Jiangxi Medical College, Nanchang University, Nanchang, China
| | - Hongping Chen
- Department of Histology and Embryology, School of Basic Medical Sciences, Jiangxi Medical College, Nanchang University, Nanchang, China
- Jiangxi Key Laboratory of Experimental Animals, Nanchang University, Nanchang, China
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Chang L, Zhang X, Li J, Li Q. Clinicopathological Characteristics, Survival and Prognostic Factors in Gastrointestinal Large Cell Neuroendocrine Carcinoma: A Retrospective Cohort Study. Am J Clin Oncol 2024; 47:363-372. [PMID: 38629640 PMCID: PMC11265646 DOI: 10.1097/coc.0000000000001104] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/25/2024]
Abstract
OBJECTIVES Gastrointestinal large cell neuroendocrine carcinoma (GILCNEC) has a low incidence but high malignancy and poor prognosis. The main purpose of this study was to thoroughly investigate its clinicopathological features, survival and prognostic factors. METHODS Information on patients with GILCNEC was extracted from the Surveillance, Epidemiology, and End Result program, and prognostic factors were analyzed by analyzing clinicopathological data and survival functions. Finally, multivariate analysis was applied to identify independent risk factors associated with survival. RESULTS A total of 531 individuals were screened in our study from the Surveillance, Epidemiology, and End Result database. The primary sites are mainly from the following: esophagus in 39 (7.3%) patients, stomach in 72 (13.6%) patients, hepatobiliary in 51 (9.6%) patients, pancreas in 97 (18.3%) patients, small intestines in 27 (5.1%), and colorectum in 245 (46.1%) patients. Esophagus, stomach, pancreas, and colorectum large cell neuroendocrine carcinoma (LCNEC) were more common in males ( P = 0.001). Esophagus LCNEC had inferior overall survival (OS), whereas small intestine LCNEC was associated with better OS. The results of multivariate analysis showed that the American Joint Committee on Cancer Sixth Edition stage, surgery, and radiotherapy were independent prognostic indicators of OS in patients with GILCNEC ( P < 0.05). CONCLUSIONS The prognosis of patients with GILCNEC varies depending on the primary tumor site. American Joint Committee on Cancer Sixth Edition stage, surgery, and radiotherapy are independent prognostic factors of patients with GILCNEC. Although surgery and radiotherapy can prolong the survival of patients with GILCNEC, their prognosis remains poor, and further prospectively designed multicenter clinical studies are needed to indicate the decision for clinicians.
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Affiliation(s)
- Lele Chang
- Department of Gastrointestinal Medical Oncology, Harbin Medical University Cancer Hospital, Harbin
| | - Xuemei Zhang
- Department of Radiation Oncology, Quzhou People’s Hospital, Quzhou
| | - Jiaxin Li
- Laboratory Department, Mental Health Institute of Inner Mongolia Autonomous Region, The Third Hospital of Inner Mongolia Autonomous Region, Hohhot, China
| | - Qingwei Li
- Department of Gastrointestinal Medical Oncology, Harbin Medical University Cancer Hospital, Harbin
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Han L, Li J, Liang C, Chu Y, Wang Y, Lv L, Liu D, Tan Y. Risk factors for positive resection margins after endoscopic resection for gastrointestinal neuroendocrine tumors. Surg Endosc 2024; 38:2041-2049. [PMID: 38429572 DOI: 10.1007/s00464-024-10706-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2023] [Accepted: 01/18/2024] [Indexed: 03/03/2024]
Abstract
BACKGROUND In recent years, the incidence of gastrointestinal neuroendocrine tumors (GI-NETs) has remarkably increased due to the widespread use of screening gastrointestinal endoscopy. Currently, the most common treatments are surgery and endoscopic resection. Compared to surgery, endoscopic resection possesses a higher risk of resection margin residues for the treatment of GI-NETs. METHODS A total of 315 patients who underwent surgery or endoscopic resection for GI-NETs were included. We analyzed their resection modality (surgery, ESD, EMR), margin status, Preoperative marking and Prognosis. RESULTS Among 315 patients included, 175 cases underwent endoscopic resection and 140 cases underwent surgical treatment. A total of 43 (43/175, 24.57%) and 10 (10/140, 7.14%) patients exhibited positive resection margins after endoscopic resection and surgery, respectively. Multivariate regression analysis suggested that no preoperative marking and endoscopic treatment methods were risk factors for resection margin residues. Among the patients with positive margin residues after endoscopic resection, 5 patients underwent the radical surgical resection and 1 patient underwent additional ESD resection. The remaining 37 patients had no recurrence during a median follow-up of 36 months. CONCLUSIONS Compared with surgery, endoscopic therapy has a higher margin residual rate. During endoscopic resection, preoperative marking may reduce the rate of lateral margin residues, and endoscopic submucosal dissection may be preferred than endoscopic mucosal resection. Periodical follow-up may be an alternative method for patients with positive margin residues after endoscopic resection.
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Affiliation(s)
- Liu Han
- Department of Gastroenterology, The Second Xiangya Hospital of Central South University, Changsha, 410011, Hunan, China
- Research Center of Digestive Diseases, Central South University, Changsha, 410011, Hunan, China
- Clinical Research Center for Digestive Diseases in Hunan Province, Changsha, 410011, Hunan, China
| | - Jianglei Li
- Department of Gastroenterology, The Second Xiangya Hospital of Central South University, Changsha, 410011, Hunan, China
- Research Center of Digestive Diseases, Central South University, Changsha, 410011, Hunan, China
- Clinical Research Center for Digestive Diseases in Hunan Province, Changsha, 410011, Hunan, China
- Xiangya School of Pharmaceutical Sciences, Central South University, Changsha, 410000, Hunan, China
| | - Chengbai Liang
- Department of Gastroenterology, The Second Xiangya Hospital of Central South University, Changsha, 410011, Hunan, China
- Research Center of Digestive Diseases, Central South University, Changsha, 410011, Hunan, China
- Clinical Research Center for Digestive Diseases in Hunan Province, Changsha, 410011, Hunan, China
| | - Yi Chu
- Department of Gastroenterology, The Second Xiangya Hospital of Central South University, Changsha, 410011, Hunan, China
- Research Center of Digestive Diseases, Central South University, Changsha, 410011, Hunan, China
- Clinical Research Center for Digestive Diseases in Hunan Province, Changsha, 410011, Hunan, China
| | - Yongjun Wang
- Department of Gastroenterology, The Second Xiangya Hospital of Central South University, Changsha, 410011, Hunan, China
- Research Center of Digestive Diseases, Central South University, Changsha, 410011, Hunan, China
- Clinical Research Center for Digestive Diseases in Hunan Province, Changsha, 410011, Hunan, China
| | - Liang Lv
- Department of Gastroenterology, The Second Xiangya Hospital of Central South University, Changsha, 410011, Hunan, China
- Research Center of Digestive Diseases, Central South University, Changsha, 410011, Hunan, China
- Clinical Research Center for Digestive Diseases in Hunan Province, Changsha, 410011, Hunan, China
| | - Deliang Liu
- Department of Gastroenterology, The Second Xiangya Hospital of Central South University, Changsha, 410011, Hunan, China.
- Research Center of Digestive Diseases, Central South University, Changsha, 410011, Hunan, China.
- Clinical Research Center for Digestive Diseases in Hunan Province, Changsha, 410011, Hunan, China.
| | - Yuyong Tan
- Department of Gastroenterology, The Second Xiangya Hospital of Central South University, Changsha, 410011, Hunan, China.
- Research Center of Digestive Diseases, Central South University, Changsha, 410011, Hunan, China.
- Clinical Research Center for Digestive Diseases in Hunan Province, Changsha, 410011, Hunan, China.
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McKnight CA, Diehl LJ, Bergin IL. Digestive Tract and Salivary Glands. HASCHEK AND ROUSSEAUX' S HANDBOOK OF TOXICOLOGIC PATHOLOGY 2024:1-148. [DOI: 10.1016/b978-0-12-821046-8.00001-3] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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11
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Shah SC, Boeder S, Piazuelo MB, Li D. The Stomach Looks Suspicious, But Is It Pernicious? Gastroenterology 2023; 165:1342-1351. [PMID: 37640254 PMCID: PMC11058005 DOI: 10.1053/j.gastro.2023.08.032] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/25/2023] [Revised: 08/18/2023] [Accepted: 08/18/2023] [Indexed: 08/31/2023]
Affiliation(s)
- Shailja C Shah
- Gastroenterology Section, Jennifer Moreno Department of Veterans Affairs Medical Center, La Jolla, California; Division of Gastroenterology, University of California, San Diego, La Jolla, California.
| | - Schafer Boeder
- Division of Endocrinology and Metabolism, University of California, San Diego, La Jolla, California
| | - M Blanca Piazuelo
- Division of Gastroenterology, Hepatology, and Nutrition, Vanderbilt University Medical Center, Nashville, Tennessee; Center for Mucosal Inflammation and Cancer, Vanderbilt University Medical Center, Nashville, Tennessee
| | - Dan Li
- Department of Gastroenterology, Kaiser Permanente Northern California, Santa Clara, California; Division of Research, Kaiser Permanente Northern California, Oakland, California
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12
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Wang N, Shu L, Liu S, Yang L, Bai T, Shi Z, Liu X. Comparing endoscopic mucosal resection with endoscopic submucosal dissection in colorectal adenoma and tumors: Meta-analysis and system review. PLoS One 2023; 18:e0291916. [PMID: 37768914 PMCID: PMC10538725 DOI: 10.1371/journal.pone.0291916] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2023] [Accepted: 08/07/2023] [Indexed: 09/30/2023] Open
Abstract
AIMS This study aimed to evaluate the safety, efficacy, and long-term outcomes of endoscopic mucosal resection (EMR) and endoscopic submucosal dissection (ESD) for treating colorectal adenomas and tumors. METHODS A systematic literature review was conducted using databases including PubMed, Web of Science, and Embase. Parameters such as number of patients or lesions, histological diagnosis, lesion size, surgery time, en-bloc resection, R0 resection, severe postoperative complications, and local recurrence were extracted and pooled for analysis. RESULTS A total of 12 retrospective studies involving 1289 patients and 1850 lesions were included in the analysis. EMR was found to have a shorter operation time by 53.6 minutes (95% CI: 51.3, 55.9, P<0.001) and fewer incidences of severe postoperative complications such as perforation and delayed bleeding (OR = 0.40, 95%CI: 0.23, 0.71, P<0.001). On the other hand, ESD had higher rates of en-bloc resection (OR = 0.15, 95%CI: 0.07, 0.30, P<0.001) and R0 resection (OR = 0.32, 95%CI: 0.16, 0.65, P<0.001). Recurrence after EMR was found to be significantly higher than that after ESD surgery (OR = 5.88, 95%CI: 2.15, 16.07, P = 0.037). CONCLUSIONS The study suggests that the choice of surgical method may have a greater impact on recurrence compared to the pathological type, and that ESD may be more suitable for the treatment of malignant lesions despite its higher rates of severe postoperative complications and longer operation time.
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Affiliation(s)
- Nian Wang
- Division of Gastroenterology, Wuhan No. 1 Hospital, Wuhan, China
| | - Lei Shu
- Division of Gastroenterology, Wuhan No. 1 Hospital, Wuhan, China
| | - Song Liu
- Division of Gastroenterology, Wuhan No. 1 Hospital, Wuhan, China
| | - Lin Yang
- Division of Gastroenterology, Wuhan No. 1 Hospital, Wuhan, China
| | - Tao Bai
- Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Zhaohong Shi
- Division of Gastroenterology, Wuhan No. 1 Hospital, Wuhan, China
| | - Xinghuang Liu
- Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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13
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Ortega Larrode A, Farrais Villalba S, Guerrero Muñoz C, Blas Jhon L, Martin Relloso MJ, Sanchez-Fayos Calabuig P, Calero Baron D, Varela Silva A, Porres Cubero JC. Detection of Neuroendocrine Tumours by Enteroscopy: A Case Report. MEDICINA (KAUNAS, LITHUANIA) 2023; 59:1469. [PMID: 37629759 PMCID: PMC10456339 DOI: 10.3390/medicina59081469] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/27/2023] [Revised: 08/09/2023] [Accepted: 08/14/2023] [Indexed: 08/27/2023]
Abstract
We present the case of a 62-year-old patient who developed melenas and in whom conventional endoscopic tests could not detect any bleeding lesion. In our case, capsule endoscopy and enteroscopy were the pivotal elements in establishing the diagnosis of a neuroendocrine tumour with an atypical location. As a result, it was possible to surgically remove the lesions at an early stage of the malignancy without metastatic disease and without the need for adjuvant therapy. Our case demonstrates the need for these new techniques in tumours of atypical location and aggressive course. Otherwise, this malignancy may be underdiagnosed until an advanced stage.
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Affiliation(s)
- Adriana Ortega Larrode
- Department of Gastroenterology, Fundación Jiménez Díaz University Hospital, 28040 Madrid, Spain; (S.F.V.); (C.G.M.); (L.B.J.); (M.J.M.R.); (P.S.-F.C.); (D.C.B.); (A.V.S.); (J.C.P.C.)
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14
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Banerjee J, Ranjan RP, Alam MT, Deshmukh S, Tripathi PP, Gandhi S, Banerjee S. Virus-associated neuroendocrine cancers: Pathogenesis and current therapeutics. Pathol Res Pract 2023; 248:154720. [PMID: 37542862 DOI: 10.1016/j.prp.2023.154720] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2023] [Revised: 07/22/2023] [Accepted: 07/26/2023] [Indexed: 08/07/2023]
Abstract
Neuroendocrine neoplasms (NENs) comprise malignancies involving neuroendocrine cells that often lead to fatal pathological conditions. Despite escalating global incidences, NENs still have poor prognoses. Interestingly, research indicates an intricate association of tumor viruses with NENs. However, there is a dearth of comprehension of the complete scenario of NEN pathophysiology and its precise connections with the tumor viruses. Interestingly, several cutting-edge experiments became helpful for further screening of NET for the presence of polyomavirus, Human papillomavirus (HPV), Kaposi sarcoma-associated herpesvirus (KSHV), Epstein Barr virus (EBV), etc. Current research on the neuroendocrine tumor (NET) pathogenesis provides new information concerning their molecular mechanisms and therapeutic interventions. Of note, scientists observed that metastatic neuroendocrine tumors still have a poor prognosis with a palliative situation. Different oncolytic vector has already demonstrated excellent efficacies in clinical studies. Therefore, oncolytic virotherapy or virus-based immunotherapy could be an emerging and novel therapeutic intervention. In-depth understanding of all such various aspects will aid in managing, developing early detection assays, and establishing targeted therapeutic interventions for NENs concerning tumor viruses. Hence, this review takes a novel approach to discuss the dual role of tumor viruses in association with NENs' pathophysiology as well as its potential therapeutic interventions.
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Affiliation(s)
- Juni Banerjee
- Institute of Advanced Research, Koba Institutional Area, Gandhinagar, Gujarat 382426, India.
| | - Ramya P Ranjan
- National Institute of Animal Biotechnology (NIAB), Gachibowli, Hyderabad, Telangana 500032, India
| | - Md Tanjim Alam
- CSIR-Indian Institute of Chemical Biology (IICB), 4, Raja S. C. Mullick Road, Kolkata 700032, India; IICB-Translational Research Unit of Excellence(IICB-TRUE), Kolkata 700091, India
| | - Sanika Deshmukh
- Institute of Advanced Research, Koba Institutional Area, Gandhinagar, Gujarat 382426, India
| | - Prem Prakash Tripathi
- CSIR-Indian Institute of Chemical Biology (IICB), 4, Raja S. C. Mullick Road, Kolkata 700032, India; IICB-Translational Research Unit of Excellence(IICB-TRUE), Kolkata 700091, India.
| | - Sonu Gandhi
- National Institute of Animal Biotechnology (NIAB), Gachibowli, Hyderabad, Telangana 500032, India.
| | - Shuvomoy Banerjee
- Institute of Advanced Research, Koba Institutional Area, Gandhinagar, Gujarat 382426, India.
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15
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Zhang Y, Liu X, Liang H, Liu W, Wang H, Li T. Late-stage esophageal neuroendocrine carcinoma in a patient treated with tislelizumab combined with anlotinib: a case report. J Int Med Res 2023; 51:3000605231187942. [PMID: 37498227 PMCID: PMC10387792 DOI: 10.1177/03000605231187942] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/28/2023] Open
Abstract
Esophageal neuroendocrine carcinoma (ENEC) is an extremely rare tumor with highly malignant potential, rapid growth, and a poor prognosis. Advanced extrapulmonary neuroendocrine carcinoma should be treated with chemotherapeutic regimens suitable for small cell lung cancer. However, ENEC has no clear second-line treatment options. The clinical application of immunotherapy and targeted therapy in small cell lung cancer has produced good therapeutic effects. We describe the case of an elderly woman with multiple metastatic advanced ENEC treated with tislelizumab combined with anlotinib as second-line therapy, achieving complete remission in a short period and long-term survival. In total, 21 cycles of tislelizumab combined with anlotinib were given to this patient. After two cycles, the patient's neuron-specific enolase level decreased from 181.8 to 22.9 µg/L and remained at normal levels throughout treatment. Progression-free survival and overall survival were 16 and 21 months, respectively, in this patient. No obvious side effects were observed. Thus, tislelizumab and anlotinib could represent a novel therapeutic option for advanced ENEC.
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Affiliation(s)
- Yanqi Zhang
- Department of Oncology, Dezhou People's Hospital (Qilu Hospital of Shandong University Dezhou Hospital), 1166 Dongfanghong West Road, Dezhou, P.R. China
| | - Xiaoyu Liu
- Department of Oncology, Dezhou People's Hospital (Qilu Hospital of Shandong University Dezhou Hospital), 1166 Dongfanghong West Road, Dezhou, P.R. China
| | - Honglu Liang
- Department of Radiotherapy, Dezhou People's Hospital (Qilu Hospital of Shandong University Dezhou Hospital), 1166 Dongfanghong West Road, Dezhou, P.R. China
| | - Weihua Liu
- Department of Gastroenterology, Dezhou People's Hospital (Qilu Hospital of Shandong University Dezhou Hospital), 1166 Dongfanghong West Road, Dezhou, P.R. China
| | - Haiyan Wang
- Department of Infectious Diseases, Dezhou People's Hospital (Qilu Hospital of Shandong University Dezhou Hospital), 1166 Dongfanghong West Road, Dezhou, P.R. China
| | - Tao Li
- Department of Oncology, Dezhou People's Hospital (Qilu Hospital of Shandong University Dezhou Hospital), 1166 Dongfanghong West Road, Dezhou, P.R. China
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Abstract
PURPOSE OF REVIEW Gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN) represent a heterogenous group of rare tumors emanating from neuroendocrine cells that are clinically silent for prolonged periods of time without detection. Traditional biomarkers lack sufficiently high enough specificity and sensitivity for these tumors and their secreted products. New molecules are sought to improve accuracy of detection and monitoring of GEP-NENs. The purpose of this review is to highlight recent advances in the discovery of novel biomarkers and their potential characteristics and utility as markers of GEP-NENs. RECENT FINDINGS Several recent GEP-NEN investigations regarding NETest demonstrate superior sensitivity and specificity in diagnosis and disease monitoring as compared with chromogranin A. Among several tissue-based emergent candidate molecules as biomarkers for GEP-NEN INSM1 has demonstrated consistently excellent characteristics when compared with traditional markers including chromogranin A, synaptophysin, and CD56. SUMMARY For the diagnosis and clinical monitoring of NEN, there still exists a considerable need for better biomarkers. Novel technology has resulted in a promising liquid biopsy for the detection and monitoring of GEP-NENs. The search for improved tissue biomarkers has resulted in identification of one potential candidate whereas several others remain in the investigatory phase.
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Affiliation(s)
- Isa Mulingbayan Jacoba
- Boston Medical Center/Boston University Chobanian & Avedisian School of Medicine, Department of Pathology & Laboratory Medicine
| | - H Christian Weber
- Boston Medical Center/Boston University Chobanian & Avedisian School of Medicine, Department of Pathology & Laboratory Medicine
- Department of Medicine
- VA Boston Healthcare System, Section of Gastroenterology and Hepatology, Boston, Massachusetts, USA
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17
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Zhang HW, Jiang Y, Huang ZY, Zhou XC. Analysis of surgical treatment of appendix neuroendocrine neoplasms-17 years of single-center experience. World J Surg Oncol 2023; 21:150. [PMID: 37194067 PMCID: PMC10190068 DOI: 10.1186/s12957-023-03025-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/08/2023] [Accepted: 04/27/2023] [Indexed: 05/18/2023] Open
Abstract
BACKGROUND/AIM This study investigated the clinicopathological characteristics and treatment of appendix neuroendocrine neoplasms in appendectomy specimens of our center. MATERIALS AND METHODS The clinicopathological data, including age, sex, preoperative clinical manifestation, surgical method, and histopathological examination results of 11 patients with appendix neuroendocrine neoplasms confirmed by surgery and pathology between November 2005 and January 2023, were retrospectively analyzed. RESULTS In the histopathological examination of 7277 appendectomy specimens, 11 cases (0.2%) had appendix neuroendocrine neoplasms. Among the 11 patients, 8(72.7%) were males, and 3(27.3%) were females, with an average age of 48.1 years. All patients underwent emergency surgery. A total of 9 patients underwent open appendectomy, including 1 patient who underwent second-stage simple right hemicolectomy after an appendectomy, and two who underwent laparoscopic appendectomy. All 11 patients were followed up for a period of 1 to 17 years. All patients survived without any indication of tumor recurrence. CONCLUSION Appendiceal neuroendocrine neoplasms are low-grade malignant tumors originating from neuroendocrine cells. They are rarely seen in clinical practice and are often treated based on acute and chronic appendicitis symptoms. These tumors are challenging to diagnose before surgery due to the lack of specificity in clinical manifestations and auxiliary examinations. The diagnosis generally depends on postoperative pathology and immunohistochemistry. Despite the diagnostic challenges, these tumors have a favorable prognosis.
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Affiliation(s)
- He-wei Zhang
- Departments of Hepatobiliary and Pancreatic Surgery, The Dingli Clinical Institute of Wenzhou Medical University (Wenzhou Central Hospital), Wenzhou, Zhejiang Province People’s Republic of China
| | - Yi Jiang
- Departments of Pathology, The Dingli Clinical Institute of Wenzhou Medical University (Wenzhou Central Hospital), Wenzhou, Zhejiang Province People’s Republic of China
| | - Zhi-yang Huang
- Departments of Gastroenterology, The Dingli Clinical Institute of Wenzhou Medical University (Wenzhou Central Hospital), Wenzhou, Zhejiang Province People’s Republic of China
| | - Xiao-cong Zhou
- Departments of Colorectal Surgery, The Dingli Clinical Institute of Wenzhou Medical University (Wenzhou Central Hospital), Wenzhou, Zhejiang Province People’s Republic of China
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18
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Lee S, Jyala A, Ghazanfar H, Shin D, Patel H. Diagnostic Challenge of Small Bowel Neuroendocrine Tumor in a Young Female Patient. Cureus 2023; 15:e37925. [PMID: 37220442 PMCID: PMC10200070 DOI: 10.7759/cureus.37925] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/21/2023] [Indexed: 05/25/2023] Open
Abstract
Neuroendocrine tumors (NETs) are rare cancers arising from neuroendocrine cells and are characterized by their ability to secrete functional hormones causing distinctive hormonal syndromes. The incidence of NET has increased over the years, and small bowel neuroendocrine tumor (SBNET) is one of the most challenging to detect due to its varied presentation and poor accessibility with traditional endoscopic methods. Patients with SBNET present with variable hormonal symptoms, such as diarrhea, flushing, and nonspecific abdominal pain, which often delay the diagnosis. We present the case of a young patient who underwent multidisciplinary workups leading to a successful diagnosis of SBNET promptly. The patient was a 31-year-old female who presented to the emergency department with complaints of nausea, vomiting, and sudden-onset, severe, sharp abdominal pain. CT scan of her abdomen showed an area of irregular intraluminal soft tissue density suspicious for a mass in the mid-small bowel. The patient's initial enteroscopy was normal. A video capsule endoscopy showed a small bowel mass, which was consistent with SBNET confirmed by pathology later. This case emphasizes the importance of considering SBNET as a differential diagnosis in young patients with nonspecific symptoms of abdominal pain and highlights the role of multidisciplinary approaches in achieving prompt diagnosis and treatment.
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Affiliation(s)
- Somin Lee
- Internal Medicine, BronxCare Health System, Bronx, USA
| | | | | | - Dongmin Shin
- Internal Medicine, BronxCare Health System, Bronx, USA
| | - Harish Patel
- Gastroenterology, BronxCare Health System, Bronx, USA
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19
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Karamchandani DM, Cox B, La Rosa S, Bellizzi AM, Shi C, Gonzalez RS. Practice patterns for reporting digestive system neuroendocrine neoplasms: results from a large, comprehensive international survey. Histopathology 2023; 82:541-554. [PMID: 36507623 DOI: 10.1111/his.14851] [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: 09/25/2022] [Revised: 12/05/2022] [Accepted: 12/08/2022] [Indexed: 12/14/2022]
Abstract
AIMS Criteria for the interpretation of digestive system neuroendocrine neoplasms (NENs) continue to evolve. Although there are some literature recommendations regarding workup and diagnosis of these lesions, different practice patterns exist among pathologists when signing out these specimens. The aim of this study was to assess practice trends among pathologists worldwide when reporting these neoplasms. METHODS AND RESULTS We created an online survey with multiple questions pertaining to digestive NENs. The results were analysed based on type of practice setting, years of sign-out experience, and practice location. Respondents included 384 practicing pathologists: 70% academic, 30% private practice; 63% gastrointestinal (GI) pathology-subspecialised, 37% not; 39% North American, 42% European, 19% others; 45% with ≤10 years in practice; 55% with >10 years. Some question responses were chosen by the majority (e.g. 85% use both mitotic count and Ki67 index for grading NENs, 82% complete a synoptic, and Ki67 stain even for small incidental appendiceal neuroendocrine tumours [NETs], and 96% utilize the diagnosis of grade 3 NET). However, some questions showed varying responses, including counting mitotic figures, Ki67 stain interpretation, and pancreatic grade 3 NEN workup. Pathologists also had some variability in interpreting regional metastatic foci of small bowel NETs and in choosing blocks for Ki67 staining in multifocal lesions. CONCLUSION There existed scenarios wherein practice patterns varied despite recommendations in the literature, and there were also scenarios lacking clear guidelines wherein pathologists used varying judgement. This survey highlights current key grey areas in digestive system NEN evaluation, leading to variation in practice patterns.
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Affiliation(s)
| | - Brian Cox
- Department of Pathology, Cedars Sinai Health System, Los Angeles, CA, USA
| | - Stefano La Rosa
- Unit of Pathology, Department of Medicine and Surgery, University of Insubria, Varese, Italy
| | | | - Chanjuan Shi
- Department of Pathology, Duke University, Durham, NC, USA
| | - Raul S Gonzalez
- Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, GA, USA
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20
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Ma XX, Wang LS, Wang LL, Long T, Xu ZL. Endoscopic treatment and management of rectal neuroendocrine tumors less than 10 mm in diameter. World J Gastrointest Endosc 2023; 15:19-31. [PMID: 36925647 PMCID: PMC10011890 DOI: 10.4253/wjge.v15.i2.19] [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] [Received: 09/13/2022] [Revised: 11/19/2022] [Accepted: 02/01/2023] [Indexed: 02/13/2023] Open
Abstract
Rectal neuroendocrine tumors (rNETs) measuring less than 10 mm in diameter are defined as small rNETs. Due to the low risk of distant invasion and metastasis, endoscopic treatments, including modified endoscopic mucosal resection, endoscopic submucosal dissection, and other transanal surgical procedures, are effective. This review article proposes a follow-up plan according to the size and histopathology of the tumor after operation.
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Affiliation(s)
- Xiao-Xin Ma
- Department of Gastroenterology, Shenzhen People’s Hospital, the 2nd Clinical Medical College of Jinan University, Shenzhen 518000, Guangdong Province, China
| | - Li-Sheng Wang
- Department of Gastroenterology, Shenzhen People’s Hospital, the 2nd Clinical Medical College of Jinan University, Shenzhen 518000, Guangdong Province, China
| | - Luo-Lin Wang
- Department of Gastroenterology, Shenzhen People’s Hospital, the 2nd Clinical Medical College of Jinan University, Shenzhen 518000, Guangdong Province, China
| | - Ting Long
- Department of Pathology, Shenzhen People’s Hospital, the 2nd Clinical Medical College of Jinan University, Shenzhen 518000, Guangdong Province, China
| | - Zheng-Lei Xu
- Department of Gastroenterology, Shenzhen People’s Hospital, the 2nd Clinical Medical College of Jinan University, Shenzhen 518000, Guangdong Province, China
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21
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Poston LM, Gupta S, Alvarado CE, Sinopoli J, Vargas LT, Linden PA, Towe CW. Contemporary outcomes of esophageal and gastroesophageal junction neuroendocrine tumors. Dis Esophagus 2023:6995427. [PMID: 36688874 DOI: 10.1093/dote/doad001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/05/2022] [Revised: 11/28/2022] [Accepted: 12/31/2022] [Indexed: 01/24/2023]
Abstract
Adenocarcinoma and squamous cell esophageal cancers have been extensively studied in the literature. Esophageal neuroendocrine (NET)/carcinoid tumors are less commonly studied and have only been described in small series. The purpose of this study was to describe the demographics and natural history of esophageal NETs, as well as optimal treatments. We hypothesized that surgical resection would be the best treatment of esophageal NETs. The National Cancer Database was used to identify adult patients with esophageal or gastroesophageal junction (GEJ) cancer from 2004 to 2018. Patients were characterized as carcinoid/NET, adenocarcinoma, or squamous cell cancer. Clinical and demographic characteristics were compared between the histology groups. The primary outcome was overall survival, which was assessed by multivariable Cox analysis. Multivariable Cox analysis was also used to analyze factors associated with survival among NET patients who underwent surgery. Among 206,321 patients with esophageal cancer, 1,563 were NETs (<0.01%). Relative to the other two histologies, NETs were associated with younger age, female sex, and advanced clinical stage at diagnosis. Multivariate analysis suggested that NETs were less likely to be treated with surgical resection (OR 0.51, P < 0.001). Nonetheless, surgical resection was associated with improved survival (HR 0.64, P = 0.003). Among patients with NETs who received surgery, neoadjuvant therapy was associated with improved overall survival (HR 0.38, P = 0.013). NET of the esophagus presents with more advanced disease than other common histologies. Among patients with nonmetastatic cancer, surgical resection appears to be the best treatment. Neoadjuvant systemic therapy may offer survival benefit, but future studies are necessary.
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Affiliation(s)
- Lauren M Poston
- Case Western Reserve University School of Medicine, Cleveland, OH, USA
| | - Shreya Gupta
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Christine E Alvarado
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Jillian Sinopoli
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Leonidas T Vargas
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Philip A Linden
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
| | - Christopher W Towe
- Division of Thoracic and Esophageal Surgery, Department of Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA
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22
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Muacevic A, Adler JR, Qureshi K, Farooq MU. Anti-Hu-Associated Encephalomyelitis as a Presentation of Primary Extrapulmonary Small Cell Carcinoma of the Small Bowel: A Case Report. Cureus 2023; 15:e33605. [PMID: 36779130 PMCID: PMC9910764 DOI: 10.7759/cureus.33605] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/10/2023] [Indexed: 01/12/2023] Open
Abstract
Small cell carcinoma (SCC) is a neuroendocrine tumor (NET) commonly found in the lung, known for rapid proliferation and early metastasis. Extrapulmonary small cell carcinomas (ESCC) are rare, with GI tract carcinomas exceedingly so. Due to the lack of clinical data on the treatment of ESCC, the standard regimen is the same as the SCC of the lung. Documented accounts of paraneoplastic encephalomyelitis associated with NETs are also uncommon. We present a patient who suffered from neurologic deficits before being diagnosed with paraneoplastic encephalomyelitis from a duodenal ESCC. The patient presented with ear pain and hematemesis. New symptoms arose after the resolution of initial symptoms, including shortness of breath and numbness. Autoimmune workup was positive for anti-Hu antibodies. A position emission tomography (PET) scan showed increased uptake in the duodenal region. Biopsy results from a duodenal ulcer revealed poorly differentiated neuroendocrine carcinoma with positive synaptophysin and strong positivity of Ki-67, consistent with ESCC. Numerous treatments, including platinum-based chemotherapy, yielded no neurologic improvement for the patient. This case details an atypical presentation of ESCC, which should be considered in patients suspected of paraneoplastic encephalomyelitis.
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23
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Gastric neuroendocrine neoplasms: a primer for radiologists. ABDOMINAL RADIOLOGY (NEW YORK) 2022; 47:3993-4004. [PMID: 35411433 DOI: 10.1007/s00261-022-03509-1] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/28/2022] [Revised: 03/22/2022] [Accepted: 03/23/2022] [Indexed: 01/18/2023]
Abstract
Gastric neuroendocrine neoplasms are uncommon tumors with variable differentiation and malignant potential. Three main subtypes are recognized: type 1, related to autoimmune atrophic gastritis; type 2, associated with Zollinger-Ellison and MEN1 syndrome; and type 3, sporadic. Although endoscopy alone is often sufficient for diagnosis and management of small, indolent, multifocal type 1 tumors, imaging is essential for evaluation of larger, high-grade, and type 2 and 3 neoplasms. Hypervascular intraluminal gastric masses are typically seen on CT/MRI, with associated perigastric lymphadenopathy and liver metastases in advanced cases. Somatostatin receptor nuclear imaging (such as Ga-68-DOTATATE PET/CT) may also be used for staging and assessing candidacy for peptide receptor radionuclide therapy. Radiotracer uptake is more likely in well-differentiated, lower-grade tumors, and less likely in poorly differentiated tumors, for which F-18-FDG-PET/CT may have additional value. Understanding disease pathophysiology and evolving histologic classifications is particularly useful for radiologists, as these influence tumor behavior, preferred imaging, therapy options, and patient prognosis.
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Small bowel neuroendocrine neoplasm: what surgeons want to know. ABDOMINAL RADIOLOGY (NEW YORK) 2022; 47:4005-4015. [PMID: 35312820 DOI: 10.1007/s00261-022-03485-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/28/2022] [Revised: 03/01/2022] [Accepted: 03/02/2022] [Indexed: 01/18/2023]
Abstract
Neuroendocrine neoplasms of the small bowel are a diverse group of tumors with a broad spectrum of imaging findings and clinical implications. Most tumors originate in close proximity to the ileocecal valve and most commonly metastasize to the mesentery and liver. This review will highlight the imaging findings of primary and metastatic small bowel neuroendocrine neoplasm that are most relevant to the surgical team.
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Lucandri G, Fiori G, Lucchese S, Pende V, Farina M, Giordano M, Santoro E. Extended surgical resection for nonfunctioning duodenal neuroendocrine tumor. J Surg Case Rep 2022; 2022:rjac391. [PMID: 36081781 PMCID: PMC9448358 DOI: 10.1093/jscr/rjac391] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/06/2022] [Revised: 07/18/2022] [Accepted: 08/09/2022] [Indexed: 11/13/2022] Open
Abstract
Duodenal neuroendocrine tumors (NETs) account for <3% of all gastrointestinal NET. Most lesions are small-sized and are located in the first or second duodenal part. Tumoral grading, evaluated by Ki67 index, strongly influences patient’s outcome. Endoscopic resection is recommended for lesions measuring <2 cm, while pancreaticoduodenectomy should be the treatment of choice for large duodenal NET; Whipple procedure should be preferred in case of duodenal origin and contiguity with gastric antrum. Involvement of surrounding structures, as well as the presence of resectable liver metastases, does not contraindicate surgical resection. Herein we report a case of a 68-year-old male, presenting with an extensive mass of the descending pre-ampullary duodenal part, with involvement of the right colon and the presence of a pericholecystic single liver metastasis. In spite of such advanced disease, surgery on the patient was successful, with an uneventful postoperative outcome.
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Affiliation(s)
- Giorgio Lucandri
- 1st Department of Surgery, San Giovanni-Addolorata Hospital , Rome , Italy
| | - Giulia Fiori
- 1st Department of Surgery, San Giovanni-Addolorata Hospital , Rome , Italy
| | - Sara Lucchese
- 1st Department of Surgery, San Giovanni-Addolorata Hospital , Rome , Italy
| | - Vito Pende
- 1st Department of Surgery, San Giovanni-Addolorata Hospital , Rome , Italy
| | - Massimo Farina
- 1st Department of Surgery, San Giovanni-Addolorata Hospital , Rome , Italy
| | - Marco Giordano
- Department of Pathology, San Giovanni-Addolorata Hospital , Rome , Italy
| | - Emanuele Santoro
- 1st Department of Surgery, San Giovanni-Addolorata Hospital , Rome , Italy
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Fernandes CJ, Leung G, Eads JR, Katona BW. Gastroenteropancreatic Neuroendocrine Tumors. Gastroenterol Clin North Am 2022; 51:625-647. [PMID: 36153114 DOI: 10.1016/j.gtc.2022.06.002] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/21/2023]
Abstract
Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are the most common type of neuroendocrine tumors and are being increasingly identified in clinical practice. The diagnosis, staging, management, and surveillance of GEP-NETs rely heavily on endoscopy, and consequently, it is important for gastroenterologists to have a solid understanding of these tumors. This article reviews the presentation, diagnosis, and management of both localized and advanced GEP-NETs, with increased emphasis on the role of endoscopy, to enable gastroenterologists and other practitioners to have the necessary tools for the care of patients with these tumors.
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Affiliation(s)
- Conrad J Fernandes
- Department of Medicine, Hospital of the University of Pennsylvania, 3400 Civic Center Boulevard, 751 South Pavilion, Philadelphia, PA 19104, USA
| | - Galen Leung
- Division of Gastroenterology and Hepatology, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, 3400 Civic Center Boulevard, 751 South Pavilion, Philadelphia, PA 19104, USA
| | - Jennifer R Eads
- Division of Hematology/Oncology, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, 3400 Civic Center Boulevard, 751 South Pavilion, Philadelphia, PA 19104, USA
| | - Bryson W Katona
- Division of Gastroenterology and Hepatology, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, 3400 Civic Center Boulevard, 751 South Pavilion, Philadelphia, PA 19104, USA.
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Bancheno WM, Adidam SR, Melaku MA. Metastatic rectal neuroendocrine carcinoma presenting with treatment-refractory immune thrombocytopenia: A case report and literature review. Medicine (Baltimore) 2022; 101:e29616. [PMID: 35866784 PMCID: PMC9302362 DOI: 10.1097/md.0000000000029616] [Citation(s) in RCA: 1] [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: 01/04/2023] Open
Abstract
RATIONALE Colorectal neuroendocrine cancer (CRNEC) accounts for < 1% of all colorectal cancers. It presents in advanced stages and carries a risk of poor survival. CRNEC may be diagnosed incidentally on imaging or routine endoscopy. When symptomatic, it may mimic colorectal adenocarcinoma. Syncope and immune thrombocytopenic purpura (ITP) have not previously been reported as clinical presentations. PATIENT CONCERNS A 57-year-old man with human immunodeficiency virus infection on treatment, seizure and stroke without residue presented for the evaluation of fall and syncope. DIAGNOSIS Physical examination revealed tachycardia, swelling, and ecchymosis of his proximal left lower extremity. Laboratory tests showed a new isolated thrombocytopenia of 26,000/mm3. Computed tomography for a trauma survey showed an incidental left posterior rectal wall mass. After hospital admission, his platelet count dropped to 14,000/mm3. A peripheral blood smear revealed low platelet count, no schistocytes or immature cells. ITP at a high risk for bleeding was diagnosed and treated with standard medical therapy but remained refractory. Bone marrow biopsy showed metastatic neuroendocrine carcinoma, likely from the rectum. INTERVENTIONS Patient received courses of high-dose dexamethasone and intravenous immunoglobulin. He also receive eleven units of platelet transfusion. A course of rituximab was administered. The platelet count response was suboptimal or short lived with drop to a nadir of 4000/mm3. However, after initiation of Eltrombopag, the thrombocytopenia resolved. Patient was started on etoposide, carboplatin, Atezolizumab. His hospital stay was complicated by neutropenia and sepsis, which was successfully treated. OUTCOMES He was discharged to subacute rehab in stable condition. About 4 months later, he was readmitted for severe thrombocytopenia, septic shock, and acute respiratory failure. Despite appropriate treatment, the patient deteriorated and expired. CONCLUSION CRNEC is a rare aggressive disease with dismal outcome that lacks standardized treatment. Metastasis to the bone marrow is uncommon and concomitant ITP has not been reported. We report a rare case of rectal neuroendocrine carcinoma metastatic to bone marrow associated with refractory ITP and review the relevant literature.
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Affiliation(s)
- Wouhabe Marai Bancheno
- Internal Medicine, Howard University Hospital, Washington, DC
- * Correspondence: Wouhabe Marai Bancheno, MD, MSc, Internal Medicine, Howard University Hospital, 2041 GA Ave NW, Washington, DC 20060 (e-mail: )
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Shi H, Wang C, Wu J, Qin B, Jiang J, Liu N, Song Y, Qin Y, Ma S. Underwater endoscopic mucosal resection for rectal neuroendocrine tumors (with videos): a single center retrospective study. BMC Gastroenterol 2022; 22:276. [PMID: 35655173 PMCID: PMC9161598 DOI: 10.1186/s12876-022-02350-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2021] [Accepted: 05/11/2022] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Underwater endoscopic mucosal resection (UMER) is a new method of endoscopic resection to completely remove the lesion without submucosal injection. But few attempts have been carried out for rectal neuroendocrine tumors (rectal NETs). METHODS We retrospectively investigated data on the tumor characteristics and outcomes of patients with ≤ 10 mm rectal NETs who underwent UEMR or endoscopic submucosal dissection (ESD) from January 2019 to June 2021 in our institute. RESULTS The endoscopic resection rate was 100% in both UEMR and ESD groups. The histological complete resection rate of the UEMR group (95.5%) was lower than that of the ESD group (96.4%) with no significant difference. The average operation time, hospitalization time and operation cost of UEMR group were less than those of ESD group (P < 0.05). The incidence of postoperative abdominal pain and abdominal distention in the UEMR group was lower than that in the ESD group (P < 0.05). There was no significant difference in the incidence of delayed bleeding and perforation between the two groups. There was no local recurrence or distant metastasis in the two groups during the follow-up period. CONCLUSIONS Both the UEMR and ESD can effectively treat ≤ 10 mm rectal NETs with invasion depth confined to the mucosa and submucosa. UEMR is superior to ESD in operation time, hospitalization time, operation cost, postoperative abdominal pain and abdominal distention.
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Affiliation(s)
- Haitao Shi
- Department of Gastroenterology, The Second Affiliated Hospital of Xi'an Jiaotong University, 157 Xiwu Street, Xi'an, 710004, Shaanxi, China
| | - Chuying Wang
- Department of Gastroenterology, The Second Affiliated Hospital of Xi'an Jiaotong University, 157 Xiwu Street, Xi'an, 710004, Shaanxi, China
| | - Jie Wu
- Department of Pathology, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, 710004, Shaanxi, China
| | - Bin Qin
- Department of Gastroenterology, The Second Affiliated Hospital of Xi'an Jiaotong University, 157 Xiwu Street, Xi'an, 710004, Shaanxi, China
| | - Jiong Jiang
- Department of Gastroenterology, The Second Affiliated Hospital of Xi'an Jiaotong University, 157 Xiwu Street, Xi'an, 710004, Shaanxi, China
| | - Na Liu
- Department of Gastroenterology, The Second Affiliated Hospital of Xi'an Jiaotong University, 157 Xiwu Street, Xi'an, 710004, Shaanxi, China
| | - Yahua Song
- Department of Gastroenterology, The Second Affiliated Hospital of Xi'an Jiaotong University, 157 Xiwu Street, Xi'an, 710004, Shaanxi, China
| | - Yun Qin
- Department of Gastroenterology, The Second Affiliated Hospital of Xi'an Jiaotong University, 157 Xiwu Street, Xi'an, 710004, Shaanxi, China
| | - Shiyang Ma
- Department of Gastroenterology, The Second Affiliated Hospital of Xi'an Jiaotong University, 157 Xiwu Street, Xi'an, 710004, Shaanxi, China.
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Yin F, Wu ZH, Lai JP. New insights in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms. World J Gastroenterol 2022; 28:1751-1767. [PMID: 35633912 PMCID: PMC9099195 DOI: 10.3748/wjg.v28.i17.1751] [Citation(s) in RCA: 10] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/21/2021] [Revised: 01/06/2022] [Accepted: 04/04/2022] [Indexed: 02/06/2023] Open
Abstract
Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are rare epithelial neoplasms derived from pluripotent endocrine cells along the gastrointestinal tract and pancreas. GEP-NENs are classified into well-differentiated neuroendocrine tumors and poorly differentiated neuroendocrine carcinomas. Despite overlapping morphological features, GEP-NENs vary in molecular biology, epigenetic, clinical behavior, treatment response, and prognosis features and remain an unmet clinical challenge. In this review, we introduce recent updates on the histopathologic classification, including the tumor grading and staging system, molecular genetics, and systemic evaluation of the diagnosis and treatment of GEP-NENs at different anatomic sites, together with some insights into the diagnosis of challenging and unusual cases. We also discuss the application of novel therapeutic approaches for GEP-NENs, including peptide receptor radionuclide therapy, targeted therapy, and immunotherapy with immune checkpoint inhibitors. These findings will help improve patient care with precise diagnosis and individualized treatment of patients with GEP-NENs.
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Affiliation(s)
- Feng Yin
- Department of Pathology and Anatomical Sciences, University of Missouri, Columbia, MO 65212, United States
| | - Zi-Hao Wu
- Department of Surgery, University of Missouri, Columbia, MO 65212, United States
| | - Jin-Ping Lai
- Department of Pathology, Kaiser Permanente Sacramento Medical Center, Sacramento, CA 95825, United States
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Kohno S. Diagnosis and Surgical Treatment of Gastroenteropancreatic Neuroendocrine Neoplasms: A Literature Review. CANCER DIAGNOSIS & PROGNOSIS 2022; 2:115-125. [PMID: 35399177 PMCID: PMC8962810 DOI: 10.21873/cdp.10085] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/27/2021] [Accepted: 01/31/2022] [Indexed: 06/14/2023]
Abstract
This review aimed to highlight the characteristics and surgical treatments of tumours, and answer questions regarding the assessment of gastrointestinal neuroendocrine neoplasms (NENs) and optimal therapy. NENs comprise tumours that can produce hormones and cause a secretory syndrome. The diagnostic method and accuracy differ depending on the site of occurrence; hence, the relevant scientific society has created NEN treatment guidelines for each organ. Gastroenteric pancreatic (GEP) NENs have been unified and classified together according to the 2019 World Health Organization classification. Treatment is based on complete tumour resection, and when metastatic or primary lesions cannot be completely resected, lesions and symptoms are treated. Except for surgery for NENs, chemotherapy, molecularly targeted drugs, transarterial chemoembolization, etc., have also been confirmed as treatments. GEP NEN treatment methods will continue to advance and change because of surgery and other advances in treatment and diagnostic methods.
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Affiliation(s)
- Shuzo Kohno
- Department of Surgery, The Jikei University Katsushika Medical Center, Tokyo, Japan
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Risk Factors Associated with the Development of Metastases in Patients with Gastroenteropancreatic Neuroendocrine Tumors: A Retrospective Analysis. J Clin Med 2021; 11:jcm11010060. [PMID: 35011798 PMCID: PMC8745312 DOI: 10.3390/jcm11010060] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2021] [Revised: 12/21/2021] [Accepted: 12/21/2021] [Indexed: 11/30/2022] Open
Abstract
Neuroendocrine tumors develop from systemic endocrine and nerve cells, and their occurrence has increased recently. Since these tumors are heterogeneous, pathological classification has been based on the affected organ. In 2019, the World Health Organization introduced a change expected to influence neuroendocrine tumor research, as gastroenteropancreatic neuroendocrine tumors are now included within a unified classification. This retrospective study aimed to investigate the characteristics (e.g., lymph node metastases and all other metastases) of gastroenteropancreatic neuroendocrine tumors using this new classification in 50 cases. Tumor size, depth, MIB-1 index, lymphatic invasion, venous invasion, and neuroendocrine tumor grade were significantly correlated with lymph node metastasis and other metastases. The venous invasion was more strongly correlated with lymph node metastasis and all other types of metastases than with lymphatic invasion. Identification rates for lymphatic invasion were considered lower because of structural problems such as lymphatic vessels being much thinner than veins. However, venous invasion was considered effective in compensating for the low identification rate in cases of lymphatic invasion. In future research, a unified classification and standardized framework for assessment will be important when analyzing the characteristics of neuroendocrine tumors, and large-scale studies are required.
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Redondo De Oro K, Redondo Bermúdez C, Mendoza Durán LD, Marrugo Padilla V, Otero Urda MS. Tumor neuroendocrino en recto simulando una neoplasia benigna: reporte de caso y revisión de la literatura. REVISTA COLOMBIANA DE CIRUGÍA 2021. [DOI: 10.30944/20117582.813] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Los tumores neuroendocrinos se definen como un grupo heterogéneo de neoplasias de origen epitelial, provenientes de células enterocromafines diseminadas por todo el organismo, y representan alrededor del 1 al 4 % de todas las neoplasias. Su mayor distribución se encuentra en el tracto gastrointestinal, donde se localiza el 75 % de los tumores neuroendocrinos, siendo los ubicados en el recto, el 27 % de todos los que afectan el tracto gastrointestinal. A propósito de esta revisión de tema, presentamos el caso de un paciente de 71 años de edad, que consultó por sangrado rectal rojo rutilante, sin otra sintomatología asociada, y se le diagnosticó un tumor neuroendocrino grado 1, que se comportaba como una lesión benigna del recto.
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