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He YF, Wang HZ, Hu XD, Liu JQ, Li HM, Wang J, Lu SF. Pancreatic primitive neuroectodermal tumors: Clinical features, treatment, and influencing factors. World J Gastrointest Oncol 2025; 17:97298. [PMID: 39958532 PMCID: PMC11756011 DOI: 10.4251/wjgo.v17.i2.97298] [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: 05/27/2024] [Revised: 10/01/2024] [Accepted: 11/01/2024] [Indexed: 01/18/2025] Open
Abstract
BACKGROUND Data on clinical characteristics, treatment outcomes, and prognosis of pancreatic primitive neuroectodermal tumors (PNETs) are limited. AIM To analyze the clinical data of 30 patients with pancreatic PNETs to identify their clinical characteristics and factors associated with prognosis. METHODS We used the keywords "primary neuroectodermal tumor," "digestive tract," "pancreas," "pancreatic," and "gastrointestinal," individually or in combination, to collect data from a global database for all patients with pancreatic PNET to date. Univariate and Cox regression analyses were performed to identify prognostic factors for patient survival. RESULTS A total of 30 cases of pancreatic PNET were included in this study: 15 males and 15 females with a mean age of 24 years. The main symptom was abdominal pain (73.3%), and the median tumor size was 7.85 cm. Twenty-four patients (80.0%) underwent surgery and nineteen patients received adjuvant therapy. Local metastasis was observed in 13 patients (43.3%), lymph node metastasis in 10 patients (33.3%), and distant metastasis in 6 patients (20.0%). Local recurrence was observed in 13 patients (43.3%). The median survival time of all patients was 29.4 months, and the overall estimated 1-year and 3-year survival rates were approximately 66.0% and 36.4%, respectively. Univariate analysis showed that chemotherapy (P = 0.036), local metastasis (P = 0.041), lymph node metastasis (P = 0.003), distant metastasis (P = 0.049), and surgical margins (P = 0.048) were the prognostic factors affecting survival. Multivariate analysis revealed only lymph node metastasis (P = 0.012) as a prognostic factor. CONCLUSION Pancreatic PNET is extremely rare, occurs in young adults, has no apparent sex predisposition, has a high rate of metastasis and early recurrence, and has a very poor prognosis. The diagnosis of pancreatic PNET requires a combination of clinical symptoms, pathologic features, immunohistochemistry, and cytogenetic analysis. Univariate analysis suggested that chemotherapy, metastasis, and surgical margins were prognostic factors affecting survival, and multivariate analysis suggested that lymph node metastasis is an important prognostic factor. Therefore, early diagnosis, early and extensive resection, and adjuvant chemoradiotherapy may help improve prognosis.
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Affiliation(s)
- Yan-Fei He
- Health Management Center, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
| | - Huan-Zhi Wang
- College of Atmospheric Sciences, Lanzhou University, Lanzhou 730000, Gansu Province, China
| | - Xiao-Dong Hu
- Department of Endocrinology, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
| | - Jun-Qiang Liu
- Department of Thoracic Surgery, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
| | - Hu-Ming Li
- Department of Respiratory Medicine, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
| | - Jie Wang
- Health Management Center, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
| | - Shuang-Feng Lu
- Health Management Center, The Sixth Medical Center, Chinese PLA General Hospital, Beijing 100048, China
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2
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Liu Z, Bian J, Yang Y, Wei D, Qi S. Ewing sarcoma of the pancreas: a pediatric case report and narrative literature review. Front Oncol 2024; 14:1368564. [PMID: 38694785 PMCID: PMC11061524 DOI: 10.3389/fonc.2024.1368564] [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] [Received: 01/10/2024] [Accepted: 04/01/2024] [Indexed: 05/04/2024] Open
Abstract
Ewing's Sarcoma (ES) is an rare, small round-cell sarcoma that predominantly occurs in children and young adults, with both skeletal and extraskeletal manifestations. However, pancreatic ES, due to its rarity, is infrequently featured in scholarly literature, with only a scant 43 reported instances. Our study describes a case of pancreatic ES in an 8-year-old boy who was found to have an abdominal mass. Following an exhaustive examination, the boy was diagnosed with a neoplasm in the pancreatic head and underwent a complex surgical procedure encompassing pancreatoduodenectomy and partial transverse colectomy. Immunohistochemical assays confirmed the neoplastic cells' positivity for Cluster of Differentiation 99(CD99), Vimentin, and NK2 Homeobox 2(NKX2.2), while genomic testing identified an EWSR1-FLI1(Ewing Sarcoma Breakpoint Region 1-Friend Leukemia Integration 1) gene fusion. This led to a conclusive diagnosis of pancreatic Ewing's Sarcoma. The patient underwent seven cycles of adjuvant chemotherapy, alternating between VDC (Vincristine, Doxorubicin, Cyclophosphamide) and IE (Ifosfamide, Etoposide) tri-weekly, but did not undergo radiotherapy. At present, the patient remains neoplasm-free. Through our case analysis and comprehensive review of the existing literature, we aim to underscore th rarity of pancreatic Ewing's sarcoma and to highlight the efficacy of our individualized therapeutic approach.
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Affiliation(s)
| | - Jian Bian
- Department of General Surgery, Anhui Provincial Children’s Hospital, Hefei, Anhui, China
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3
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Patterson KN, Trout AT, Shenoy A, Abu-El-Haija M, Nathan JD. Solid pancreatic masses in children: A review of current evidence and clinical challenges. Front Pediatr 2022; 10:966943. [PMID: 36507125 PMCID: PMC9732489 DOI: 10.3389/fped.2022.966943] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/11/2022] [Accepted: 11/03/2022] [Indexed: 11/26/2022] Open
Abstract
Pancreatic tumors in children are infrequently encountered in clinical practice. Their non-specific clinical presentation and overlapping imaging characteristics often make an accurate preoperative diagnosis difficult. Tumors are categorized as epithelial or non-epithelial, with epithelial tumors further classified as tumors of the exocrine or endocrine pancreas. Although both are tumors of the exocrine pancreas, solid pseudopapillary neoplasm is the most prevalent solid pancreatic tumor in children, while pancreatoblastoma is the most common malignant tumor. Insulinoma is the most common pediatric pancreatic tumor of the endocrine pancreas. Malignant tumors require a complete, often radical, surgical resection. However, pancreatic parenchyma-sparing surgical procedures are utilized for benign tumors and low-grade malignancy to preserve gland function. This review will discuss the epidemiology, pathophysiology, clinical and diagnostic characteristics, and management options associated with both common and rare solid pancreatic masses in children. We will also discuss current challenges encountered in their evaluation and treatment.
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Affiliation(s)
- Kelli N Patterson
- Center for Surgical Outcomes Research, Abigail Wexner Research Institute, Nationwide Children's Hospital, Columbus, OH, United States
| | - Andrew T Trout
- Department of Radiology, Cincinnati Children's Hospital Medical Center, Departments of Radiology and Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States
| | - Archana Shenoy
- Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, Department of Pathology, The Ohio State University College of Medicine, Columbus, OH, United States
| | - Maisam Abu-El-Haija
- Division of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, United States
| | - Jaimie D Nathan
- Department of Abdominal Transplant and Hepatopancreatobiliary Surgery, Nationwide Children's Hospital, Department of Surgery, The Ohio State University College of Medicine, Columbus, OH, United States
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4
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Abstract
Primitive neuroectodermal tumors (PNET, previously referred to as peripheral neuroepithelioma) are rare malignant tumors with various degrees of differentiation belonging to the Ewing’s family of sarcomas. They are classified as round cell tumors arising from soft tissues. In rare instances, PNETs may arise from solid organs containing neuroendocrine cells of kidney, bladder, heart, lungs, parotid glands and pancreas. Most cases occur in the second decade of life with a slight preponderance in males. PNET of the pancreas is an aggressive tumor with multiple recurrences and a relatively poor prognosis. These tumors should be considered in the differential diagnosis, especially in a diagnosed pancreatic tumor in individuals less than 35 years of age. Due to the nature of the tumor, surgery with subsequent chemoradiation are widely accepted modalities despite the poor prognosis. In this article, we review 25 cases of extraosseous Ewing’s sarcoma (ES) of the pancreas which to the best of our knowledge, enlists most cases reported in the literature thus far.
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Affiliation(s)
- Dharti Patel
- Internal Medicine, Oak Hill Hospital, Brooksville, USA
| | - Nitish Singh Nandu
- Internal Medicine, Chicago Medical School, Rosalind Franklin University, North Chicago, USA
| | - Aravind Reddy
- Internal Medicine, Chicago Medical School, Rosalind Franklin University, North Chicago, USA
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5
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Metastasis of Ewing Sarcoma to the Pancreas: Case Report and Literature Review. Case Rep Oncol Med 2020; 2020:7075048. [PMID: 32257479 PMCID: PMC7115132 DOI: 10.1155/2020/7075048] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/21/2019] [Revised: 02/24/2020] [Accepted: 03/13/2020] [Indexed: 12/19/2022] Open
Abstract
Ewing sarcoma (ES) is a highly aggressive malignant bone cancer. ES is part of the Ewing sarcoma family of tumors (ESFT), which express characteristic t(11;22) translocation as well as higher levels of CD99. Given that metastasis and tumor burden are significant prognostic factors in patient's response to treatment, prompt diagnosis is needed to effectively treat ESFT patients. However, the challenges in classifying and characterizing ESFT complicate effective management and treatment of ES. In this report, we present a rare case of ES metastasis to the pancreas. Upon review of the literature, we found 39 cases of ESFT involving the pancreas, but only 3 were metastatic to the pancreas while the remaining cases of ESFT primarily originated from the pancreas. Given the rarity of such metastasis, the positive outcome in our patient's case may explain the importance of prompt diagnosis in order to initiate appropriate treatment.
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6
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Baião JM, Martins RM, Correia JG, Jordão D, Vieira Caroço T, Caetano Oliveira R, Agostinho PG, Ferrão H. Inflammatory Pseudotumor of the Pancreas Mimicking a Pancreatic Neoplasm. Case Rep Gastroenterol 2019; 13:245-252. [PMID: 31275087 PMCID: PMC6600032 DOI: 10.1159/000501064] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/02/2019] [Accepted: 05/18/2019] [Indexed: 12/24/2022] Open
Abstract
A 78-year-old woman was admitted to our hospital with a pancreatic tumor, incidentally discovered in an abdominal ultrasound exam. She was asymptomatic and without any previous personal pathological condition. The computed tomography (CT) and the magnetic resonance imaging (MRI) scan showed a mass lesion of 4 cm in diameter, located in the pancreatic body, conditioning the invasion of the splenic vein. The patient was admitted to surgery. During the laparotomy, we found a tumoral lesion highly suspicious of pancreatic neoplasia located in the transition of the head/body of the pancreas, with an invasion of the portal vein and several peri-regional lymph nodes. We performed biopsies of the pancreatic mass and lymphadenectomy of the peri-regional pancreatic lymph nodes. Histological analysis found an inflammatory pseudotumor of the head/body of the pancreas, without signals of malign epithelial neoplasm and also without criteria for immunoglobulin G4-related disease. During the follow-up, a PET/CT and MRI confirmed that the pancreatic lesion had disappeared without any treatment. Inflammatory pseudotumor of the pancreas is a rare entity not fully understood. Despite this, the administration of corticosteroids and immunosuppressive therapy could be taken into consideration as the disease carries a risk.
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Affiliation(s)
- José Miguel Baião
- Surgical Department, Instituto Português de Oncologia de Coimbra, Coimbra, Portugal
| | - Rui Miguel Martins
- Surgical Department, Instituto Português de Oncologia de Coimbra, Coimbra, Portugal
| | | | - Daniel Jordão
- Surgical Department, Instituto Português de Oncologia de Coimbra, Coimbra, Portugal
| | - Teresa Vieira Caroço
- Surgical Department, Instituto Português de Oncologia de Coimbra, Coimbra, Portugal
| | - Rui Caetano Oliveira
- Pathology Department, Centro de Diagnóstico Anatomo-Patológico (CEDAP), Coimbra, Portugal
| | - Paulo Gil Agostinho
- Radiology Department, Instituto Português de Oncologia de Coimbra, Coimbra, Portugal
| | - Henrique Ferrão
- Surgical Department, Instituto Português de Oncologia de Coimbra, Coimbra, Portugal
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7
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Komforti MK, Sokolovskaya E, D’Agostino CA, Benayed R, Thomas RM. Extra-osseous Ewing sarcoma of the pancreas: case report with radiologic, pathologic, and molecular correlation, and brief review of the literature. Virchows Arch 2018; 473:361-369. [DOI: 10.1007/s00428-018-2344-y] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2018] [Revised: 03/20/2018] [Accepted: 03/21/2018] [Indexed: 01/01/2023]
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8
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Nishizawa N, Kumamoto Y, Igarashi K, Nishiyama R, Tajima H, Kawamata H, Kaizu T, Watanabe M. A peripheral primitive neuroectodermal tumor originating from the pancreas: a case report and review of the literature. Surg Case Rep 2015; 1:80. [PMID: 26380804 PMCID: PMC4567589 DOI: 10.1186/s40792-015-0084-7] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2015] [Accepted: 09/07/2015] [Indexed: 12/12/2022] Open
Abstract
A peripheral primitive neuroectodermal tumor (pPNET) is a small round cell tumor occurring mostly in children or young adults and categorized into the Ewing sarcoma family of tumors. pPNETs originating from the pancreas are especially rare, and only 25 cases have been reported in the literature. We report a case of a 22-year-old man who had a giant expansive tumor located in the uncinate process of the pancreas, 80 mm in diameter resulting in obstruction in the duodenum. The patient underwent a pancreaticoduodenectomy. The histological examination showed that the pancreatic tumor was composed of atypical small round cells. Immunohistochemical findings were positive for CD99. An Ewing sarcoma breakpoint region 1 gene 22q12 rearrangement was proven by a two-color fluorescence in situ hybridization assay. We diagnosed the tumor as a pPNET of the pancreas, which, according to the literature, is highly aggressive with poor prognosis. A multidisciplinary approach to treat these neoplasms should improve the prognoses.
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Affiliation(s)
- Nobuyuki Nishizawa
- Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan
| | - Yusuke Kumamoto
- Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan
| | - Kazuharu Igarashi
- Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan
| | - Ryo Nishiyama
- Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan
| | - Hiroshi Tajima
- Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan
| | - Hiroshi Kawamata
- Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan
| | - Takashi Kaizu
- Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan
| | - Masahiko Watanabe
- Department of Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa 252-0374 Japan
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9
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Primitive Neuroectodermal Tumor of the Pancreas: A Case Report and Review of the Literature. Case Rep Surg 2015; 2015:276869. [PMID: 26101685 PMCID: PMC4460232 DOI: 10.1155/2015/276869] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2015] [Revised: 05/09/2015] [Accepted: 05/14/2015] [Indexed: 12/15/2022] Open
Abstract
Primitive neuroectodermal tumors (PNETs) are presented as rare malignant neoplasms. In unusual cases, those neoplasms may arise in solid organs containing neuroendocrine cells, such as the pancreas. Herein the case of a 28-year-old patient that underwent gastroduodenopancreatectomy after the diagnosis of a huge mass (PNET) located in both head and body of the pancreas is reported. This is the 19th case of pancreatic PNET reported in literature.
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10
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Mao Y, Sang X, Liang N, Yang H, Lu X, Yang Z, Du S, Xu Y, Zhao H, Zhong S, Huang J, Millis JM. Peripheral primitive neuroectodermal tumors arising in the pancreas: the first case report in Asia and a review of the 14 total reported cases in the world. Hepatobiliary Surg Nutr 2014; 2:51-60. [PMID: 24570916 DOI: 10.3978/j.issn.2304-3881.2012.08.01] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/05/2012] [Accepted: 08/01/2012] [Indexed: 11/14/2022]
Abstract
OBJECTIVE To discuss the diagnosis and treatment of peripheral primitive neuroectodermal tumors of the pancreas based on our case and all the cases in the world. METHODS The first case of peripheral primitive neuroectodermal tumors of the pancreas in Asia was preliminarily reported by our group in 2006. The patient underwent three operations for the primary tumor and recurrences over 41 months prior to the patient's death in November 2007. All 14 reported cases of pancreatic PNETs in the world were analyzed. The corresponding literatures on its diagnosis and treatment of were reviewed. RESULTS A 13 year-old female patient was diagnosed with pancreatic PNETs by the clinical, microscopic, immunohistochemical features, and cytogenetic analysis after the resection of the tumor located in the uncinate process of the pancreas at PUMC Hospital. During the follow-up course, radiotherapy and chemotherapy were given after the first operation. Two additional operations were performed 10 months and 25 months after the first one, respectively, because of tumor recurrence. The patient died 41 months after the initial diagnosis with the recurrence and metastasis that were not suitable for a further surgery. Primitive neuroectodermal tumors of the pancreas are extremely rare. A review of the world's literature on this tumor identified fourteen cases with a mean survival time of 12 months (ranging from 6 to 50 months). These patients often have no specific clinical symptoms, but most do present with abdominal pain and/or jaundice. The diagnosis is established by small round tumor cells seen on light microscopy, immunohistochemical features of positive P30/32(MIC2) with at least two positive neuronal markers., and cytogenetic analysis showing characteristic translocation of t[11;22][q24;q12]. Since pancreatic PNETs are highly aggressive, early diagnosis, immediate surgical resection and re-resection if possible, early radiotherapy and chemotherapy and close follow-up are required. CONCLUSIONS Peripheral primitive neuroectodermal tumors can arise in pancreas. The diagnosis and treatment should be made as early as possible, aggressive surgeries for the primary and recurrences may help to improve the prognosis.
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Affiliation(s)
- Yilei Mao
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Xinting Sang
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Naixin Liang
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Huayu Yang
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Xin Lu
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Zhiying Yang
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Shunda Du
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Yiyao Xu
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Haitao Zhao
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Shouxian Zhong
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
| | - Jiefu Huang
- Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and PUMC, Beijing 100730, China
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Reilly C, Zenoni S, Hasan MK, Varadarajulu S, Tran TA, de la Fuente SG, Arnoletti JP. Primary pancreatic Ewing's sarcoma with portal vein tumor thrombosis. J Gastrointest Surg 2013. [PMID: 23192427 DOI: 10.1007/s11605-012-2098-9] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
BACKGROUND Extraosseous Ewing's sarcoma (EES) is a mesenchyme-derived small blue cell tumor, which is distinguished by its rarity, aggressiveness, dismal prognosis, and distinct pathogenesis. Occurring almost exclusively among children and young adults, EES can arise from a variety of organs and portends a rapid clinical deterioration and high likelihood of recurrence. DISCUSSION We present the first reported case of a primary pancreatic Ewing's sarcoma in a patient with concomitant portal vein thrombosis. The atypical presentation of this extraordinarily rare tumor underscores the imperative to maintain EES in the differential diagnosis of suspicious, indistinct pancreatic lesions in young patients. In addition, we review the available literature describing additional cases of primary pancreatic Ewing's sarcoma.
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Affiliation(s)
- Chris Reilly
- University of Central Florida College of Medicine, Oviedo, FL 32765, USA.
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12
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Adult pancreatic neuroblastoma, an unusual site and fatal outcome. Pathol Oncol Res 2011; 18:239-43. [PMID: 21837482 DOI: 10.1007/s12253-011-9434-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2011] [Accepted: 07/05/2011] [Indexed: 10/17/2022]
Abstract
In this report, we describe a classic case of stroma rich neuroblastoma, nodular type in a 22 year old female presented with a pancreatic mass. This rare and unusual presentation elicits several differential diagnostic categories including solid pseudopapillary tumor, pancreatic endocrine tumor, pancreatoblastoma and PNET. In this report, we tried to differentiate between them depending on the histopathological features and using panel of epithelial and neuroendocrine markers. Although of the rarity of pancreatic neuroblastoma as a primary site of origin, however it should be considered in the differential diagnosis of pancreatic masses in children and young adult. Neuropil and ganglionic differentiation are helpful features to recognize neuroblastoma and differentiate them from other small blue cell tumors. The fatal outcome of adult neuroblastoma confirming the independence of age as a prognostic factor in this neoplasm regardless of stage and histology.
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13
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Localized intrapancreatic malignant mesothelioma: a rare entity that may be confused with other pancreatic neoplasms. Virchows Arch 2010; 456:455-61. [PMID: 20148337 DOI: 10.1007/s00428-010-0885-9] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2009] [Revised: 12/22/2009] [Accepted: 01/11/2010] [Indexed: 12/19/2022]
Abstract
Malignant mesothelioma usually presents with diffuse involvement of the pleura or peritoneum. Circumscribed or localized malignant mesothelioma has been described in these locations, as well as the viscera, in which case it may cause diagnostic confusion with other, more common entities. Herein, we describe the first well-documented case of primary intrapancreatic malignant mesothelioma in the English literature. The patient was an otherwise healthy 39-year-old woman who presented with a symptomatic mass in the head of the pancreas that was completely resected via pancreaticoduodenectomy. The tumor was composed of cysts, papillae, and tubules lined by cells with abundant eosinophilic cytoplasm and immunohistochemically expressed CA-125, calretinin, and D2-40. Follow-up revealed no evidence of residual or recurrent disease 32 months after surgery. This report also describes the clinical and pathologic characteristics of an intrapancreatic mesothelioma and provides a review of the literature regarding entities that may be considered in the differential diagnosis of this tumor.
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14
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Doi H, Ichikawa S, Hiraoka A, Ichiryu M, Nakahara H, Ochi H, Tanabe A, Kodama A, Hasebe A, Miyamoto Y, Ninomiya T, Horiike N, Takamura K, Kawasaki H, Kameoka C, Kan M, Doi S, Soga Y, Tamura H, Maeda T, Asaki A, Seno S, Iguchi H, Hasegawa T. Primitive neuroectodermal tumor of the pancreas. Intern Med 2009; 48:329-33. [PMID: 19252356 DOI: 10.2169/internalmedicine.48.1484] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
The primitive neuroectodermal tumor (PNET) of the pancreas, a member of Ewing's sarcoma family of tumors, is extremely rare. We treated a 37-year-old Japanese man who had a solitary pancreatic tumor 40 mm in diameter and multiple hepatic tumors with surgical resection. The PNET was positive for CD99 on immunohistochemical staining. Fluorescence in situ hybridization (FISH) was also performed, which revealed a Ewing sarcoma breakpoint region 1 (EWSR1) 22q12 rearrangement. According to the Japan-Ewing protocol, chemotherapy with Ifomide (ifosfamide), etoposide, vincristine, and cyclophosphamide was given after surgery. To the best of our knowledge, to date 13 PNET cases have been reported with a mean age for all patients of 19.3 years old. Surgical resection was performed in most cases and some patients received postoperative chemotherapy. The clinicopathologic characteristics and management of this extremely rare disease are also discussed.
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Affiliation(s)
- Hirokazu Doi
- Department of Gastroenterology, Ehime Prefectural Central Hospital, Matsuyama
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15
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Welsch T, Mechtersheimer G, Aulmann S, Mueller SA, Buechler MW, Schmidt J, Kienle P. Huge primitive neuroectodermal tumor of the pancreas: Report of a case and review of the literature. World J Gastroenterol 2006; 12:6070-3. [PMID: 17009412 PMCID: PMC4124421 DOI: 10.3748/wjg.v12.i37.6070] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Primitive neuroectodermal tumor (PNET) of the pancreas is an extremely rare tumor that usually occurs in children or young adults. We report a case of a 33-year-old male patient with an 18 cm × 18 cm × 16 cm mass arising from the pancreatic body and tail with a one-day history of abdominal pain. Initial CT scan showed no signs of metastatic tumor spread. The tumor caused intrabdominal bleeding and the patient underwent primary tumor resection including partial gastrectomy, left pancreatic resection and splenectomy. Diagnosis of PNET was confirmed by histology, immunohistochemistry and FISH analysis. All neoplastic cells were stained positive for MIC2-protein (CD99). Approximately one month after surgery, several liver metastases were observed and the patient underwent chemotherapy according to the Euro-Ewing protocol. Subsequent relaparotomy excluded any residual hepatic or extrahepatic abdominal metastases. Although PNET in the pancreas is an extremely rare entity, it should be considered in the diffential diagnosis of pancreatic masses, especially in young patients. This alarming case particularly illustrates that PNET in the pancreas although in an advanced stage can present with only a short history of mild symptoms.
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Affiliation(s)
- Thilo Welsch
- Deptartment of Surgery, University of Heidelberg, D-69120 Heidelberg, Germany.
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Mizukami H, Yajima N, Wada R, Matsumoto K, Kojima M, Klöppel G, Yagihashi S. Pancreatic malignant fibrous histiocytoma, inflammatory myofibroblastic tumor, and inflammatory pseudotumor related to autoimmune pancreatitis: characterization and differential diagnosis. Virchows Arch 2006; 448:552-60. [PMID: 16538442 DOI: 10.1007/s00428-006-0157-x] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/24/2005] [Accepted: 01/10/2006] [Indexed: 12/24/2022]
Abstract
Malignant fibrous histiocytoma (MFH) and inflammatory myofibroblastic tumor (IMT) are uncommon primary non-epithelial cell tumors of the pancreas. In addition, there are inflammatory pseudotumors (IPT) that may arise in the course of autoimmune pancreatitis (AIP). In the English language literature, only 24 cases of IMT and nine cases of MFH in the pancreas have been reported to date. We investigated three individual spindle cell tumors of the pancreas that were identified as MFH, IMT, and IPT, respectively, using immunohistochemical and molecular analysis. Both the MFH and the IMT, but not the IPT, showed nuclear p53 expression and mutations of the p53 gene. The MFH and the IMT also had higher mitotic and Ki-67 (MIB-1) indexes than the IPT. The IPT was found to be a tumor-like case of AIP. Many IgG4-positive plasma cells, which are considered to be a feature of AIP, were found in all three tumors. It is concluded that in this series of spindle cell tumors of the pancreas, apart from immunohistochemical features, the demonstration of p53 mutations may be helpful in distinguishing true neoplastic tumors from pseudotumors such as IPTs arising in the context of AIP.
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MESH Headings
- Adult
- Autoimmune Diseases/complications
- Base Sequence
- Diagnosis, Differential
- Female
- Gene Expression
- Gene Expression Profiling
- Granuloma, Plasma Cell/etiology
- Granuloma, Plasma Cell/genetics
- Granuloma, Plasma Cell/pathology
- Histiocytoma, Malignant Fibrous/genetics
- Histiocytoma, Malignant Fibrous/metabolism
- Histiocytoma, Malignant Fibrous/pathology
- Humans
- Immunohistochemistry
- Ki-67 Antigen/metabolism
- Male
- Middle Aged
- Mutation
- Neoplasms, Muscle Tissue/genetics
- Neoplasms, Muscle Tissue/metabolism
- Neoplasms, Muscle Tissue/pathology
- Pancreatectomy
- Pancreatic Neoplasms/genetics
- Pancreatic Neoplasms/metabolism
- Pancreatic Neoplasms/pathology
- Pancreatitis/complications
- Pancreatitis/pathology
- Polymerase Chain Reaction
- Tumor Suppressor Protein p53/genetics
- Tumor Suppressor Protein p53/metabolism
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Affiliation(s)
- Hiroki Mizukami
- Department of Pathology, Hirosaki University School of Medicine, and Division of Surgical Pathology, National Hirosaki Hospital, 036-8562, Japan
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Abstract
Mesenchymal tumors of the pancreas are rare. They are resected because a solid or cystic pancreatic tumor is suspected. Benign mesenchymal tumors comprise lymphangiomas, hemangiomas, schwannomas, solitary fibrous tumors, adenomatoid tumors, clear cell tumors, and hamartomas. Inflammatory pseudotumors are a special case. Malignant mesenchymal tumors include leiomyosarcomas, malignant peripheral nerve sheath tumors (MPNST), liposarcomas, malignant fibrous histiocytomas, Ewing's sarcomas, and primitive neuroectodermal tumors (PNET). It is important to differentiate these tumors from anaplastic carcinomas and retroperitoneal tumors that infiltrate pancreatic tissue.
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Affiliation(s)
- U Pauser
- Institut für Allgemeine Pathologie, Universitätsklinikum Schleswig-Holstein--Campus Kiel, Michaelisstrasse 11, 24105 Kiel, Germany.
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18
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Kosmahl M, Pauser U, Peters K, Sipos B, Lüttges J, Kremer B, Klöppel G. Cystic neoplasms of the pancreas and tumor-like lesions with cystic features: a review of 418 cases and a classification proposal. Virchows Arch 2004; 445:168-78. [PMID: 15185076 DOI: 10.1007/s00428-004-1043-z] [Citation(s) in RCA: 253] [Impact Index Per Article: 12.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/19/2004] [Accepted: 04/08/2004] [Indexed: 12/15/2022]
Abstract
Although cystic neoplasms and lesions of the pancreas are rare, they have attracted a great deal of attention because of their potential curability. Since, in recent years, several new entities have been identified, the relative frequency of the tumors and their classification need to be reevaluated. In a series of 1454 tumorous lesions of the pancreas collected between 1971 and 2003 in our surgical pathology files and consultation files, all cystic pancreatic neoplasms and tumor-like lesions were identified and typed both histologically and immunohistochemically. There were 418 cases (29%) showing cysts with a diameter ranging between 0.5 cm and 27 cm. Most common were solid pseudopapillary neoplasms (21%) and intraductal papillary-mucinous neoplasms (18%). When only the cystic neoplasms and lesions that had been resected in a single institution were considered, intraductal papillary mucinous neoplasms were the most frequent cystic neoplasms, while solid pseudopapillary neoplasms took fifth place behind ductal adenocarcinomas with cystic features, serous cystic neoplasms and mucinous cystic neoplasms. The most frequent cystic tumor-like lesions were pancreatitis-associated pseudocysts. New and rare entities that have recently been identified are mucinous nonneoplastic cysts, acinar cell cystadenomas and cystic hamartomas. Bearing in mind that figures from referral centers such as ours may be biased regarding the relative frequency of lesions, we concluded from our data that intraductal papillary-mucinous neoplasms are the most frequently occurring pancreatic cystic neoplasms, rather than solid pseudopapillary neoplasms. It was possible to classify all cystic lesions encountered in our files or described in the literature in a new system that distinguishes between neoplastic and nonneoplastic lesions, with further subdivisions into epithelial (adenomas, borderline neoplasms and carcinomas) and nonepithelial tumors. This classification is easy to handle and enables a distinction on the basis of clinical behavior and prognosis.
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Affiliation(s)
- M Kosmahl
- Department of Pathology, University of Kiel, Germany.
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19
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Bergmann F, Hackert T, Mechtersheimer G, Penzel R, Bläker H, Berger I, Esposito I, Büchler MW, Otto HF. Differential diagnosis of non-epithelial tumors of the pancreas: malignant non-epithelial pancreatic tumor with focal pigmentation. Virchows Arch 2004; 444:190-3. [PMID: 15046038 DOI: 10.1007/s00428-003-0940-x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
Abstract
Non-epithelial tumors only rarely affect the pancreas. In this report, we describe a malignant non-epithelial tumor with combined characteristics of malignant peripheral nerve sheath tumor (MPNST) and malignant melanoma. To more closely define the differential diagnosis of MPNST with focal pigmentation versus metastatic melanoma resembling MPNST, the tumor was investigated using histomorphology, immunohistochemistry, electron microscopy, and comparative genomic hybridization. As a result, from these analyses and from clinical findings, the diagnosis of a pancreatic MPNST with focal pigmentation was favored. However, the diagnosis of a malignant melanoma or a composite tumor could not be definitely ruled out, due to the considerable morphological and genotypical overlap between both entities, which can be explained by the close histogenetic relationship between both tumor entities.
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Affiliation(s)
- Frank Bergmann
- Institute of Pathology, University of Heidelberg, Germany.
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20
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Movahedi-Lankarani S, Hruban RH, Westra WH, Klimstra DS. Primitive neuroectodermal tumors of the pancreas: a report of seven cases of a rare neoplasm. Am J Surg Pathol 2002; 26:1040-7. [PMID: 12170091 DOI: 10.1097/00000478-200208000-00009] [Citation(s) in RCA: 67] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Primitive neuroectodermal tumors (PNETs) have rarely been described in solid organs. We report a series of seven PNETs of the pancreas. The clinical, gross, microscopic, and immunohistochemical features of these seven PNETs of the pancreas are described, as are the genetic analyses in five cases. The patients ranged in age from 6 to 25 years (mean 18 years). Four of the patients were male. All of the patients presented with jaundice and/or abdominal pain. All of the tumors were located in the head of the pancreas, and they ranged in size from 3.5 to 9.0 cm. Light microscopy revealed the typical morphologic features of PNETs. By immunohistochemistry the neoplastic cells in all seven cases expressed O13 (CD99, p30/32MIC2). In five of six tested cases, the neoplastic cells also expressed cytokeratin. All of the tumors expressed neural-neuroendocrine markers. Two of the three cases examined ultrastructurally showed prominent epithelial features. There was cytogenetic or molecular genetic evidence of the t(11;22)(q24;q12) in four of five cases examined. Clinical follow-up was available in five cases. Two of the patients were alive with no evidence of disease at 33 and 43 months. One patient was alive with disease at 27 months. One patient died of postoperative complications. Another patient died of disease 4 years after diagnosis. PNET can sometimes arise as a primary neoplasm of the pancreas. Like PNETs arising in more conventional sites, pancreatic PNETs occur in the pediatric and adolescent population, show the characteristic staining with O13, and typically harbor the t(11;22)(q24;q12) chromosomal translocation. PNETs should be included in the differential diagnosis of poorly differentiated small round cell tumors of the pancreas. Moreover, they should not be confused with pancreatic endocrine tumors, which also demonstrate dual epithelial and neuroendocrine differentiation by immunohistochemistry and express O13 in 30% of cases.
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