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Gupta V, Chopde A, Chaudhari V, Bal M, Shrikhande SV, Bhandare MS. Primary Pancreatic GIST - A-Single Centre Case Series and Systematic Review of Literature. J Gastrointest Cancer 2024; 55:572-583. [PMID: 38324136 DOI: 10.1007/s12029-024-01024-8] [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] [Accepted: 01/23/2024] [Indexed: 02/08/2024]
Abstract
PURPOSE GISTs arising from organs outside GI tract are defined as extragastrointestinal GISTs (EGIST). The majority of EGISTs arise from small intestinal mesentry, mesocolon, omentum, retroperitoneum, abdominal wall, liver and pancreas with pancreas comprising less than 5% of it. Due to limited data, it is unknown if the results of GIST can be generalised for EGISTs. We thereby present the largest single-centre case series of primary pancreatic GIST so far with review of existing literature. METHODS A total of 9 patients of primary pancreatic GIST were treated at our institute from September 2016 to February 2023. After literature search for all studies published before February 2023, 51 articles including 57 patients were identified. Their clinicopathological data and survival analysis were assessed. RESULTS The median age of patients treated at our centre was 53 years with a female predominance. The most common epicentre was pancreatic head with abdominal pain as the most common presenting symptom. All 57 patients documented in literature belonged to a similar age group with similar gender predilection. The factors impacting DFS were histologic type, mitotic index, NIH risk category and adjuvant therapy. The median DFS was 74 months with a 5-year DFS being 71.9%, while the 5-year OS was 90.4%. CONCLUSION Pancreatic GIST is a rare entity. Due to limited evidence and evolving literature, results cannot be generalised to a larger population. Larger case series with longer follow-up data are required to further understand the disease biology and long-term outcomes of pancreatic GIST.
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Affiliation(s)
- Vipul Gupta
- Gastrointestinal and Hepato-pancreato-biliary services, Department of Surgical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, 400012, India
| | - Amit Chopde
- Gastrointestinal and Hepato-pancreato-biliary services, Department of Surgical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, 400012, India
| | - Vikram Chaudhari
- Gastrointestinal and Hepato-pancreato-biliary services, Department of Surgical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, 400012, India
| | - Munita Bal
- Department of Pathology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, 400012, India
| | - Shailesh V Shrikhande
- Gastrointestinal and Hepato-pancreato-biliary services, Department of Surgical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, 400012, India
| | - Manish S Bhandare
- Gastrointestinal and Hepato-pancreato-biliary services, Department of Surgical Oncology, Tata Memorial Hospital, Homi Bhabha National Institute, Mumbai, Maharashtra, 400012, India.
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Bhatia A, Ahuja A, Goel H, Bhardwaj M. Primary Adrenal Gastrointestinal Stromal Tumor (GIST): a Rarest Incidentaloma and Diagnostic Challenge. Indian J Surg Oncol 2020. [DOI: 10.1007/s13193-020-01182-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
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Kwon HJ. Extra-gastrointestinal stromal tumor of the pancreas: report of a case. Ann Hepatobiliary Pancreat Surg 2017; 21:237-242. [PMID: 29264589 PMCID: PMC5736746 DOI: 10.14701/ahbps.2017.21.4.237] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/01/2017] [Accepted: 10/23/2017] [Indexed: 12/15/2022] Open
Abstract
Gastrointestinal tumors (GISTs) of the pancreas are extremely rare with limited individual case reports and small number of case series. Herein, we report a case of pancreatic extragastrointestinal stromal tumor (EGIST) along with literature review. A 64-year-old female patient was referred to us for treatment of an abdominal mass detected by ultrasonographic examination. The tumor was located in the periamullary region. Under a preoperative diagnosis of a duodenal GIST, we performed a pylorus preserving pancreatoduodenectomy for this lesion. Laboratory examination results were within normal ranges. On pathologic gross examination, the tumor measured at 7 cm in its greatest dimension almost entirely involved the pancreatic head. Its cut surface was rubbery and white. It was surrounded by a thin pseudocapsule and well demarcated. Histopathological examination of the specimen showed a cellular lesion with compressed pancreatic tissue at peripheral. Mitotic count was 5 per 50 high-power fields. Immunohistochemically, neoplastic cells were positive for antibodies against C-KIT (CD117), CD 34, and vimentin. However, smooth-muscle actin reactions with antibodies against S-100 or desmin were negative. Based on above findings, the tumor was finally diagnosed as GISTs originating from the pancreas. The patient has been followed up postoperatively for 72 months. There is no evidence of recurrence. Here we report this case of pancreatic EGIST presenting as a solid neoplasm along with literature review of cases previously described. Our review on pancreatic EGISTs is limited and insufficient to make a conclusion regarding its clinical features. Those manifested large masses tended to have an aggressive biological and clinical behavior. Thus, pancreatic EGISTs need to be carefully differentiated. Adequate surgical intervention is necessary for pancreatic EGISTs.
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Affiliation(s)
- Hyung Jun Kwon
- Department of Surgery, Kyungpook National University Chilgok Hospital, Kyungpook National University School of Medicine, Daegu, Korea
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Abstract
GOALS The present study aimed to investigate the clinicopathologic features and prognosis of pancreatic gastrointestinal stromal tumor (GIST). BACKGROUND Reports on clinicopathologic features and prognosis of pancreatic GIST are limited due to the extremely rare incidence. STUDY One case of pancreatic GIST from our center and 44 cases reported in MEDLINE were enrolled in this study. Clinicopathologic features and prognosis of pancreatic GISTs were analyzed and compared with 297 gastric GISTs from our center. RESULTS The most common location was head of pancreas (38.5%). The majority of pancreatic GISTs exceeded 5 cm (74.4%), displayed cystic or mixed imaging features (56.4%), and were high risk (85.7%). The 5-year disease-free survival (DFS) and disease-specific survival rates were 66.1% and 95.8%, respectively. Mitotic index was the only risk factor for DFS of pancreatic GISTs. The distribution of tumor size, histologic type and National Institutes of Health risk category were significantly different between pancreatic and gastric GISTs. The 5-year DFS rate of pancreatic GISTs was significantly lower than that of gastric GISTs. Multivariate analysis showed that location was an independent prognostic factor for DFS between pancreatic and gastric GISTs. CONCLUSIONS The most common location was head of pancreas. The majority of pancreatic GISTs were large and highly malignant. Pancreatic GISTs differed significantly from gastric GISTs in respect to clinicopathologic features. The DFS of pancreatic GISTs was worse than that of gastric GISTs.
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GIST Manifesting as a Retroperitoneal Tumor: Clinicopathologic Immunohistochemical, and Molecular Genetic Study of 112 Cases. Am J Surg Pathol 2017; 41:577-585. [PMID: 28288036 DOI: 10.1097/pas.0000000000000807] [Citation(s) in RCA: 38] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
Most gastrointestinal stromal tumors (GISTs) occur in the tubular gastrointestinal (GI) tract, but some present apparently outside the GI tract. In this study, we analyzed 112 GISTs located in the retroperitoneum. These tumors occurred in 55 women and 57 men with a median age of 65 years (range: 21 to 89 y). On the basis of clinically or histologically detected connections to GI tract, 15 tumors were considered likely of gastric, 9 duodenal, and 13 of small intestinal origin. The remaining cases were categorized by location as peripancreatic (n=25), pelvic (n=11), mesenteric (n=4), and of unspecified/miscellaneous sites (n=35). The tumors varied in size 3 to 35 cm (median, 15 cm) and by mitotic rate per 5 mm, 0 to >100 (median, 10). Histologically the tumors apparently arising outside the GI tract had features of intestinal (n=41) and gastric GISTs (n=25); 9 cases had indeterminate histology. The histologic variants included spindled, epithelioid, vacuolated, nested, and myxoid potentially simulating other tumors such as liposarcoma and solitary fibrous tumor. Most GISTs were KIT-positive (106/112 cases), and the remaining 6 tumors were DOG1/Ano1-positive. Five cases showed focal nuclear positivity for MDM2. KIT mutations were detected in 42/59 cases, and PDGFRA mutations in 4/16 KIT wild-type and 3/5 of the KIT-negative tumors analyzed. One pelvic retroperitoneal GIST was succinate dehydrogenase deficient. All 79 patients were dead at last follow-up with a median survival of 14 months, with few survivals >5 years. Only operable versus inoperable tumor was a statistically favorable factor in univariate analysis (P<0.01). In multivariate analysis, mitotic rate >50/5 mm was significant for a shorter survival (hazard ratio, 5.25; 95% confidence interval, 1.65-16.8; P<0.01). Histologic and clinicopathologic similarity of extragastrointestinal retroperitoneal GISTs with GISTs of GI tract suggests their GI tract origin. Potentially overlapping features between GIST and other retroperitoneal tumors necessitate use of multiple diagnostic markers and molecular genetic studies.
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Lin C, Wang L, Sheng J, Zhang D, Guan L, Zhao K, Zhang X. Transdifferentiation of pancreatic stromal tumor into leiomyosarcoma with metastases to liver and peritoneum: a case report. BMC Cancer 2016; 16:947. [PMID: 27955634 PMCID: PMC5154023 DOI: 10.1186/s12885-016-2976-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/12/2016] [Accepted: 11/28/2016] [Indexed: 12/19/2022] Open
Abstract
Background Primary pancreatic leiomyosarcoma is a rare pancreatic malignancy; the clinical presentation and treatment is not well-characterized. Further, the molecular mechanisms underlying its pathogenesis are not known. We report a patient with pancreatic stromal tumor that progressed to primary pancreatic leiomyosarcoma with hepatic and peritoneal metastases. Case presentation A 54-year-old woman was found to have pancreatic and hepatic tumor masses on routine health checkup. Owing to the difficulty in performing biopsy, this patient underwent open operation. Histopathological examination of pancreatic and liver biopsy specimen demonstrated spindle cells with nuclear mitoses. Immunohistochemical examination showed positive staining for Cluster of Differentiation117 (+) and negative staining for S-100 (-) and Smooth Muscle Actin (-). Thus, the patient was diagnosed as a case of advanced pancreatic stromal tumor with liver metastases. After surgery, treatment with oral imatinib mesylate combined with thymosin injection therapy was prescribed. Follow-up examination at 13-months revealed multiple nodular masses in liver and right peritoneum. The patient underwent a second surgery. Liver biopsy and the resected peritoneal specimen showed positive staining for Discovered On Gastrointestinal tumor-1(weak +), Actin (+), Smooth Muscle Actin (+) and negative staining for Cluster of Differentiation117 (-) Cluster of Differentiation34 (-) and S-100 (-). Histopathological examination showed spindle cells with nuclear mitoses. The final diagnosis was primary pancreatic leiomyosarcoma, transdifferentiating from pancreatic stromal tumor, with liver and peritoneal metastases. Conclusions Surgery is the first line treatment for primary pancreatic leiomyosarcoma and extra-gastrointestinal stromal tumors. In the present case, radical resection was not performed owing to hepatic metastases. Palliative treatment with radioactive 125I ion implantation and microwave coagulation therapy was administered. However, the long-term therapeutic effect needs to be assessed in future. Electronic supplementary material The online version of this article (doi:10.1186/s12885-016-2976-8) contains supplementary material, which is available to authorized users.
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Affiliation(s)
- Chao Lin
- Department of Hepatobiliary and Pancreas Surgery, China-Japan Union Hospital of Jilin University, Changchun, 130033, China
| | - Liping Wang
- Department of Pathology, China-Japan Union Hospital of Jilin University, Changchun, 130033, China
| | - Jiyao Sheng
- The Second Hospital of Jilin University, Ziqiang Street no. 218, Changchun, 130033, China
| | - Dan Zhang
- The Second Hospital of Jilin University, Ziqiang Street no. 218, Changchun, 130033, China
| | - Lianyue Guan
- Department of Hepatobiliary and Pancreas Surgery, China-Japan Union Hospital of Jilin University, Changchun, 130033, China
| | - Kai Zhao
- Department of Hepatobiliary and Pancreas Surgery, China-Japan Union Hospital of Jilin University, Changchun, 130033, China
| | - Xuewen Zhang
- The Second Hospital of Jilin University, Ziqiang Street no. 218, Changchun, 130033, China.
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Liu L, Zhu Y, Wang D, Yang C, Zhang QI, Li X, Bai Y. Coexisting and possible primary extra-gastrointestinal stromal tumors of the pancreas and liver: A single case report. Oncol Lett 2016; 11:3303-3307. [PMID: 27123107 PMCID: PMC4841060 DOI: 10.3892/ol.2016.4420] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/11/2014] [Accepted: 01/18/2016] [Indexed: 12/14/2022] Open
Abstract
Gastrointestinal stromal tumors (GIST) are mesenchymal neoplasms of the gastrointestinal tract (GI) that are defined, in part, by the expression of CD117, a c-Kit proto-oncogene protein. GISTs emerge outside of the GI at a very low frequency, typically in a single organ or location. GISTs that occasionally emerge outside of the GI are classified as extra-gastrointestinal stromal tumors (EGIST). The present study reports an extremely rare case of EGIST detected in the pancreas and the liver. The pancreatic and liver tumors were 4.5×2.5 cm and 2.0×1.5 cm in size, respectively. Both tumors consisted of CD117-positive spindle cells with a similar mitotic rate of 1–2 per 50 high power fields. The pancreatic and the hepatic EGISTs were at a low risk of malignancy, and both tumors were proposed to be primary stromal tumors. To the best of our knowledge, this is the first report of likely primary EGIST identified in the pancreas and liver of the same patient.
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Affiliation(s)
- Lei Liu
- Department of Ultrasound, First Hospital of Jilin University, Changchun, Jilin 130021, P.R. China
| | - Yingqiao Zhu
- Department of Ultrasound, First Hospital of Jilin University, Changchun, Jilin 130021, P.R. China
| | - Dongxuan Wang
- Department of Ultrasound, First Hospital of Jilin University, Changchun, Jilin 130021, P.R. China
| | - Changbin Yang
- Department of Radiation Oncology, The Tumor Hospital of Jilin Province, Changchun, Jilin 130012, P.R. China
| | - Q I Zhang
- Department of Ultrasound, College of Pharmacy, Jilin University, Changchun, Jilin 130012, P.R. China
| | - Xiukun Li
- Ultrasound Department, Jilin Provincial People's Hospital, Changchun, Jilin 130021, P.R. China
| | - Yang Bai
- Department of Ultrasound, First Hospital of Jilin University, Changchun, Jilin 130021, P.R. China
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Schöffel N, Groneberg DA, Kaul T, Laatsch D, Thielemann H. [Gastrointestinal stromal tumors (GIST)--literature review]. MMW Fortschr Med 2016; 158:60-62. [PMID: 27119704 DOI: 10.1007/s15006-016-7824-x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/05/2023]
Affiliation(s)
- Norman Schöffel
- Klinik für Allgemein- und Viszeralchirurgie Unfallkrankenhaus Berlin, Warener Str. 7, D-12683, Berlin, Deutschland.
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Abou Al-Shaar H, Solimanie S, Azzam A, Amin T, Abu-Zaid A. Gastrointestinal stromal tumor of the adrenal gland:a case report and review of the literature. Endocr Pathol 2015; 26:27-32. [PMID: 25510634 DOI: 10.1007/s12022-014-9350-7] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
Gastrointestinal stromal tumors (GISTs) are rare neoplasms of the gastrointestinal (GI) tract. The occurrence of these neoplasms ectopically outside the GI tract is extremely uncommon. Only one case of primary adrenal GIST has been reported in the literature. In this account, we report a second case of primary adrenal GIST in a 34-year-old male who presented with a 5-week history of gradually progressive left flank pain and early satiety. Whole-body positron emission tomography (PET)/computed tomography (CT) scan showed a 14 × 11 cm hypermetabolic (18)fluorodeoxyglucose (FDG)-avid mass lesion involving the left adrenal gland and dorsal part of the left hemi-diaphragm. Biopsy of the lesion revealed tumor cells that are immunoreactive to CD-117 and CD-34 and negative to CD-31, S-100, cytokeratin, desmin, and vimentin, features characteristic of GIST. The patient was given imatinib, which drastically decreased his complaints with almost complete resolution of the tumor on his last follow-up radiographic images. Primary left adrenal GIST is an extremely rare neoplasm and can be confused with GISTs arising from the greater curvature of the stomach. Imatinib therapy is optimal in the management of these tumors.
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Affiliation(s)
- Hussam Abou Al-Shaar
- College of Medicine, Alfaisal University, P.O. Box 50927, Riyadh, 11533, Saudi Arabia
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Stromal tumor presents as a large extragastrointestinal mass in the abdominal cavity. ADVANCES IN DIGESTIVE MEDICINE 2014. [DOI: 10.1016/j.aidm.2014.07.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
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Akbulut S, Yavuz R, Otan E, Hatipoglu S. Pancreatic extragastrointestinal stromal tumor: A case report and comprehensive literature review. World J Gastrointest Surg 2014; 6:175-182. [PMID: 25276287 PMCID: PMC4176778 DOI: 10.4240/wjgs.v6.i9.175] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/26/2014] [Revised: 06/21/2014] [Accepted: 07/17/2014] [Indexed: 02/06/2023] Open
Abstract
AIM: To provide an overview of the literature on pancreatic extragastrointestinal stromal tumors (EGISTs).
METHODS: We report a case of pancreatic EGIST and review published studies on pancreatic EGIST accessed via the PubMed, MEDLINE, Google Scholar, and Google databases. The keywords used were “pancreas and GIST”, “pancreas and extra GIST”, “pancreas and gastrointestinal stromal tumor”, and “pancreas and extragastrointestinal stromal tumor”. Literature reviews and/or duplicate studies were excluded. The search included articles published in the English language between January 1, 2000 and May 15, 2014.
RESULTS: From our literature survey, 30 manuscripts on pancreatic EGISTs were considered, of which 27 met the search criteria and three were excluded. The studies involved 30 patients (15 men, 15 women) with a mean age of 55.3 ± 14.3 years (range 30-84 years). The mean age of the male patients was 50.8 ± 13.7 years (range 30-84 years); that of the female patients was 59.9 ± 13.3 years (range 38-81 years). Tumor dimensions were obtained for 28 cases (mean 114.4 ± 78.6 mm; range 20-350 mm). Tumors were diagnosed incidentally in 23.3% of patients; abdominal discomfort and weight loss were the major complaints in symptomatic patients. Risk of aggressive behavior according to Fletcher criteria was determined in 25 of 30 patients (68%: high risk, 28%: intermediate risk, 4%: low risk). Histopathological examination revealed the presence of spindle cells in 96.1% of cases; CD117 and CD34 were present immunohistochemically in 96.6% and 84% of patients, respectively. The most common surgical procedures were distal pancreatectomy with splenectomy (n = 9) and pancreaticoduodenectomy (n = 7). The total follow-up period for the 28 patients ranged from 3-66 mo, during which locoregional or distant metastases were diagnosed in six patients and two patients died.
CONCLUSION: Studies on EGISTs have only been published in the last decade. The lack of studies with large patient cohorts and long-term follow-up limits evidence-based commentary. In theory, each case should be assessed individually and further genetic and immunohistochemical studies are needed.
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Beltrame V, Gruppo M, Pastorelli D, Pizzi S, Merigliano S, Sperti C. Extra-gastrointestinal stromal tumor of the pancreas: case report and review of the literature. World J Surg Oncol 2014; 12:105. [PMID: 24755359 PMCID: PMC4000617 DOI: 10.1186/1477-7819-12-105] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/09/2013] [Accepted: 04/07/2014] [Indexed: 02/08/2023] Open
Abstract
Primary extra-gastrointestinal stromal tumor (EGISTs) arising in the pancreas is extremely rare: only 20 cases have previously been reported in the English literature from 2000 to 2013. We reported a case of EGIST of the pancreas in a 69-year-old woman who presented with abdominal pain and with a solid, heterogeneously enhancing neoplasm in the uncinate process of the pancreas, revealed preoperatively by an abdominal computed tomography scan. A diagnosis of neuroendocrine tumor was suggested. Positron emission tomography with 68Ga-DOTATOC did not show pathological accumulation of the tracer in the pancreas. The patient underwent enucleation, under ultrasonic guidance, of the pancreatic tumor that emerged to the surface of the pancreas. Histopathology and immunohistochemical examination confirmed the final diagnosis of EGIST of the pancreas (CD117+), with one mitosis per 50 high-power fields. Although rarely, GIST can involve the pancreas as a primary site, and this tumor should be considered in the differential diagnosis of pancreatic neoplasms.
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Affiliation(s)
- Valentina Beltrame
- Department of Surgery, Oncology and Gastroenterology, 3rd Surgical Clinic, University of Padua, Giustiniani 2, 35128, Padua, Italy
| | - Mario Gruppo
- Department of Surgery, Oncology and Gastroenterology, 3rd Surgical Clinic, University of Padua, Giustiniani 2, 35128, Padua, Italy
| | - Davide Pastorelli
- Department of Oncology, Rare Tumors Unit, Veneto Institute of Oncology, Padua, Italy
| | - Sara Pizzi
- Department of Pathology, University of Padua, Padua, Italy
| | - Stefano Merigliano
- Department of Surgery, Oncology and Gastroenterology, 3rd Surgical Clinic, University of Padua, Giustiniani 2, 35128, Padua, Italy
| | - Cosimo Sperti
- Department of Surgery, Oncology and Gastroenterology, 3rd Surgical Clinic, University of Padua, Giustiniani 2, 35128, Padua, Italy
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Tian YT, Liu H, Shi SS, Xie YB, Xu Q, Zhang JW, Zhao DB, Wang CF, Chen YT. Malignant extra-gastrointestinal stromal tumor of the pancreas: report of two cases and review of the literature. World J Gastroenterol 2014; 20:863-868. [PMID: 24574760 PMCID: PMC3921496 DOI: 10.3748/wjg.v20.i3.863] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/28/2013] [Revised: 11/25/2013] [Accepted: 01/02/2014] [Indexed: 02/06/2023] Open
Abstract
Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that arise from the gastrointestinal tract. In rare cases, these tumors are found in intra-abdominal sites unrelated to the gastrointestinal tract, such as the mesentery, omentum and retroperitoneum. However, pancreatic extra-gastrointestinal stromal tumors are extremely rare, with only 14 previous cases reported. A 61-year-old man with no clinical symptoms had a routine check-up, during which an abdominal mass located in the pancreas tail was detected. Abdominal surgery was performed with resection of the pancreas tail and the spleen, and he was diagnosed with low-risk GISTs. Another 60-year-old man with no clinical symptoms underwent Computed tomography which revealed a well-demarcated tumor, 6 cm in diameter, in the head of the pancreas. He was diagnosed with pancreatic GISTs. Here, we describe two rare cases of pancreatic GISTs and review the cases previously reported in the literature.
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Wegge J, Bartholomew DM, Burke LH, Miller LA. Pancreatic extra-gastrointestinal stromal tumour masquerading as a bleeding duodenal mass. BMJ Case Rep 2012; 2012:bcr-2012-007040. [PMID: 23087281 DOI: 10.1136/bcr-2012-007040] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/10/2023] Open
Abstract
We describe a 55-year-old man presenting to our institution with a gastrointestinal bleed. He was found to have a 5 cm pancreatic extra-gastrointestinal stromal tumours (EGISTs) eroding into the duodenum and ampulla of Vater. Pancreaticoduodenectomy was performed and the tumour was noted to be positive for CD117 and CD34 with six mitotic figures per 50/high-powered field. At 5 months postoperatively he is receiving treatment with imatinib and doing well. To the best of our knowledge, our patient is only the 18th case reported in the literature to date.
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Affiliation(s)
- Jacqueline Wegge
- Department of Surgery, Western Michigan University School of Medicine, Kalamazoo, Michigan, USA
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Soufi M, Bouziane M, Massrouri R, Chad B. Pancreatic GIST with pancreas divisum: A new entity. Int J Surg Case Rep 2012; 4:68-71. [PMID: 23123418 DOI: 10.1016/j.ijscr.2012.09.007] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2012] [Revised: 09/10/2012] [Accepted: 09/25/2012] [Indexed: 12/15/2022] Open
Abstract
INTRODUCTION Gastrointestinal stromal tumours (GISTs) are uncommon intra-abdominal tumours. These tumours tend to arise with a higher frequency in the stomach and the small bowel. In fewer than 5% of cases, they originate primarily from extra-gastrointestinal tumours (EGISTs). Gastrointestinal stromal tumour of the pancreas is very rare. Only few cases have been published. We report the first case of stromal tumour of the pancreas with concomitant pancreas divisum. PRESENTATION OF CASE A 39-year-old male who presented with constipation and abdominal pain. A computerized tomography demonstrated a 9cm pancreatic mass, without liver lesions. A Whipple procedure with segmental colectomy was performed with success. After 24months follow up, the patient is doing well and disease-free. DISCUSSION Mesenchymal tumours of the pancreas are extremely rare, accounting for less than 1% of all pancreatic tumours. The endoscopic ultrasound is helpful for diagnosis. Surgical resection with negative pathologic margins remains the treatment of choice. CONCLUSION in our knowledge, this is the first case of pancreatic GIST with pancreas divisum. Although pancreatic GISTs are uncommon tumours, they must be considered in the differential diagnosis of solid pancreatic lesions. Even though the tumour can be evaluated as high risk, treatment must be aggressive in order to improve survival rate.
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Affiliation(s)
- Mehdi Soufi
- Division of Digestive Surgery, Oujda CHU, University Mohammed First, Oujda, Morocco.
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Abstract
OBJECTIVES Pancreatic interstitial cells are located among acini, ducts, nerves, and blood vessels. They are essential for pancreas development, physiology, and for oncogenic microenvironment. We identified cells with characteristic ultrastructural features of telocytes in pancreatic interstitium. Telocytes were initially described as interstitial Cajal-like cells, but it gradually became clear that they were a distinct novel cell type not directly related to canonical interstitial Cajal cells. METHODS Serial ultrathin sections of human pancreatic tissue were studied by transmission electron microscopy. Computer analysis software was used to obtain 2-dimensional compositions from serial micrographs and to perform morphometry. RESULTS Pancreatic telocytes appear as small-body cells with prolongations called telopodes. The ultrastructural features of telopodes are the following: (a) number: 1 to 3; (b) length: tens of micrometers; (c) moniliform aspect: with podoms (thicker portions) and podomers (thin segments, with a mean width of 60 nm, undetectable by light microscopy); (d) dichotomous branching forming a network; (e) establish homocellular and heterocellular junctions; (f) release of microvesicles/multivesicular bodies. Telopodes pass close to blood vessels, nerves, and pancreatic acinar cells and ducts. CONCLUSIONS Telocytes are present as distinct interstitial cells in the exocrine pancreatic stroma. They act as important players in intercellular signaling via stromal synapses and shed vesicle transfer.
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Li ZY, Liang QL, Chen GQ, Zhou Y, Liu QL. Extra-gastrointestinal stromal tumor of the liver diagnosed by ultrasound-guided fine needle aspiration cytology: a case report and review of the literature. Arch Med Sci 2012; 8:392-7. [PMID: 22662017 PMCID: PMC3361056 DOI: 10.5114/aoms.2012.28572] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2011] [Revised: 10/28/2011] [Accepted: 11/21/2011] [Indexed: 12/27/2022] Open
Affiliation(s)
- Zhou-Yu Li
- Department of Oncology, Affiliated Hospital of Guangdong Medical College, Zhanjiang, China
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18
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Primary extragastrointestinal stromal tumor arising in the pancreas: report of a case. Surg Today 2012; 42:386-90. [PMID: 22258729 DOI: 10.1007/s00595-011-0080-x] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2010] [Accepted: 03/27/2011] [Indexed: 01/12/2023]
Abstract
Primary extragastrointestinal stromal tumors (EGISTs) arising in the pancreas are extremely rare, with only ten cases documented to our knowledge. We report a further case of EGIST of the pancreas. The patient was a 55-year-old man who presented with postprandial abdominal discomfort. Abdominal computed tomography and magnetic resonance imaging showed a lobulated heterogenous enhancing mass, 11 cm in diameter, in the abdominal cavity. No regional lymphadenopathy, ascites, or metastasis was seen radiologically. There was no obvious lesion in the stomach or small intestine. The initial diagnosis was a solid pseudopapillary tumor or serous cystic neoplasm. The patient underwent distal pancreatectomy with splenectomy. Microscopically, the tumor consisted of spindle cells arranged in short fascicles. Mitotic figures were seen in 7/50 high-power fields. Immunohistochemical examination revealed strongly positive staining for CD117. Based on these findings, the final pathologic diagnosis was a primary EGIST of the pancreas. This case consolidates the possibility that this rare tumor can involve the pancreas as a primary site and should be included in the differential diagnosis of cystic lesions in this site.
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Čečka F, Jon B, Ferko A, Šubrt Z, Nikolov DH, Tyčová V. Long-term survival of a patient after resection of a gastrointestinal stromal tumor arising from the pancreas. Hepatobiliary Pancreat Dis Int 2011; 10:330-2. [PMID: 21669581 DOI: 10.1016/s1499-3872(11)60056-8] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
BACKGROUND Gastrointestinal stromal tumors (GISTs) may arise in any part of the gastrointestinal tract; extra-gastrointestinal locations are extremely rare. Only a few cases of extragastrointestinal stromal tumor arising from the pancreas were reported. None of the reports described a long-term follow-up of the patients. METHOD This report describes an interesting and unusual case of GIST arising from the pancreas. RESULTS A 74-year-old female presented with a palpable abdominal mass. CT scan showed a large mass 11 x 8 x 4 cm originating from the tail of the pancreas. Percutaneous biopsy revealed a GIST predominantly with spindle cells, but some parts also contained epitheloid cells. The patient was treated by distal pancreatic resection with splenectomy. Immunohistochemistry of the tumor showed a staining pattern characteristic of GIST. The patient has achieved a long-term survival of five years and six months without any sign of recurrence of the disease. CONCLUSION This is the first reported case of an extra-gastrointestinal stromal tumor arising from the pancreas treated surgically, with a long-term survival.
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Affiliation(s)
- Filip Čečka
- Department of Surgery, Faculty of Medicine and University Hospital Hradec Králové, 500 05 Hradec Králové, Czech Republic.
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20
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Meng L, Fang SH, Jin M. An unusual case of pancreatic and gastric neoplasms (2010: 12b). Malignant GISTs originating from the pancreas and stomach. Eur Radiol 2011; 21:663-5. [PMID: 21287351 DOI: 10.1007/s00330-010-1893-5] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/29/2010] [Revised: 06/12/2010] [Accepted: 06/21/2010] [Indexed: 12/12/2022]
Abstract
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the digestive tract and are believed to originate from the neoplastic transformation of the interstitial cells of Cajal from their precursors. GIST can occur anywhere but mainly affects the stomach (60%), jejunum and ileum (30%), duodenum (4-5%), rectum (4%), colon, appendix and esophagus. Primary GISTs of the pancreas are very rare, with only five previous cases reported. This is an unusual case of multiple GISTs in the pancreas and stomach.
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Affiliation(s)
- Lei Meng
- Department of Radiology, Sir Run Run Shaw Hospital, Sir Run Run Shaw Institute of Clinical Medicine of Zhejiang University, Hangzhou, 310016, Zhejiang Province, China
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21
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Sereg M, Buzogány I, Gonda G, Sápi Z, Csöregh E, Jakab Z, Rácz K, Tóth M. Gastrointestinal stromal tumor presenting as a hormonally inactive adrenal mass. Endocrine 2011; 39:1-5. [PMID: 21061093 DOI: 10.1007/s12020-010-9406-5] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/03/2010] [Accepted: 09/07/2010] [Indexed: 02/07/2023]
Abstract
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Rarely, tumors with the same histological and immunohistochemical features arise in organs having no connection to the tubular gastrointestinal tract designated as extra-gastrointestinal stromal tumors (EGISTs). In this article, we report the first case of an EGIST presenting as a hormonally inactive adrenal mass. A 68-year-old woman with a 3-month history of right abdominal pain was clinically diagnosed as having a hormonally inactive right adrenal tumor sizing 15 cm in diameter. This mass and the tightly fixed right adrenal gland were resected en bloc.Histologically, the tumor was composed primarily of monomorphic spindle cells. Mitotic figure was 2–3 per 50 high power fields. Immunohistochemical analysis revealed strong positivity for CD117 (c-KIT) and smooth muscle actin (a-SMA), but negativity for beta-catenin, CD34, pan-keratin,S-100, desmin, and H-caldesmon. Genetic analysis showed no mutations in KIT gene exons 9, 11, 13, and 17,and in exon 18 of the platelet-derived growth factor-2 receptor gene (PDGFR). The patient proved to be tumor-free at the 18-month follow-up. This case under study demonstrates that EGIST should be included in the differential diagnosis of hormonally inactive adrenal tumors.CD117 (c-KIT) immunohistochemistry should be applied in the pathological workup of soft tissue adrenal tumors.This case is an additional example suggesting that the prognosis of even a very large EGIST is not definitely grave.
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Affiliation(s)
- Márta Sereg
- 2nd Department of Medicine, Semmelweis University, Szentkirályi u. 46, Budapest, Hungary
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Abstract
Gastrointestinal stromal tumors (GISTs) have emerged from being a poorly understood and therapeutically refractory sarcoma to a tumor whose biology has not only provided insight into a mechanism of oncogenesis but has also led to a rational basis for therapy. Most GISTs are characterized by KIT protein (CD117) expression and constitutive activating mutations in either the c-kit or platelet-derived growth factor receptor α genes. This information can now be obtained from routine formalin-fixed and paraffin-embedded tissue. Because the correct diagnosis is the key to successful treatment of this tumor, it is incumbent on the pathologist to be familiar with the various gross and histologic patterns shown by these tumors. GISTs range from small incidental stromal nodules to large cystic and solid tumor masses. GISTs show a variety of microscopic patterns and therefore several other tumors enter the differential diagnosis. Fortunately, with an understanding of GIST histology, and with the proper use of immunohistochemistry and molecular analysis, a correct diagnosis can usually be made. In addition to the correct diagnosis, several key attributes of the tumor need to be determined because they provide the basis for proper clinical management. This article summarizes the gross, microscopic, and molecular findings of GISTs, and discusses the differential diagnosis and key attributes of this interesting group of neoplasms.
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Hirabayashi K, Fujihira T, Oyamada H, Serizawa A, Yamashita T, Tobita K, Imaizumi T, Kajiwara H, Nakamura N, Osamura RY. First case of primary phyllodes tumor of the pancreas: case report and findings of immunohistochemical and ultrastructural studies. Virchows Arch 2010; 456:587-93. [PMID: 20182743 DOI: 10.1007/s00428-010-0893-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2010] [Accepted: 02/05/2010] [Indexed: 01/05/2023]
Abstract
A 37-year-old Japanese man with a solid and cystic pancreatic mass was referred to our hospital. Computed tomography revealed a well-demarcated solid and cystic mass measuring approximately 3.0 cm in diameter in the pancreatic body. The patient underwent middle segment pancreatectomy, and the retrieved tumor specimen was found to be a well-demarcated solid and cystic lesion measuring 3.0 x 3.0 cm. On histological examination, the cyst walls were found to be lined with a monolayer of non-atypical tall columnar epithelial cells. The solid areas surrounded the cystic ones and showed storiform proliferation of spindle cells that contained round, oval, or elongated nuclei and were present among abundant collagen fibers. The solid areas sent phylloid projections into the cystic spaces and the main pancreatic duct. The spindle cells were found to be diffusely positive for alpha-smooth muscle actin, desmin, and h-caldesmon on immunohistochemical analysis. Electron microscopy revealed that these cells possessed well-developed myofilaments with dense bodies, pinocytic vesicles, and basal lumina. Neither metastasis nor local invasion was detected. After the operation (4 years), tumor recurrence has not occurred. The main differential diagnoses of spindle cell tumors are leiomyomas, leiomyosarcomas, inflammatory myofibroblastic tumors, solitary fibrous tumors, extra-gastrointestinal stromal tumors, and schwannomas. However, the histological findings in the present case differed from those of these tumors. The present lesion is the first reported case of a primary pancreatic phyllodes tumor.
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Affiliation(s)
- Kenichi Hirabayashi
- Department of Pathology, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, Japan.
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Yamamoto H, Miyamoto Y, Nishihara Y, Kojima A, Imamura M, Kishikawa K, Takase Y, Ario K, Oda Y, Tsuneyoshi M. Primary gastrointestinal stromal tumor of the liver with PDGFRA gene mutation. Hum Pathol 2010; 41:605-9. [PMID: 20096441 DOI: 10.1016/j.humpath.2009.09.016] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/22/2009] [Revised: 09/14/2009] [Accepted: 09/15/2009] [Indexed: 12/28/2022]
Abstract
Gastrointestinal stromal tumor is a mesenchymal tumor with KIT or PDGFRA gene mutation, occurring primarily in the stomach and intestine and rarely outside the digestive tract. KIT-negative tumors with epithelioid cell morphology and PDGFRA mutation represent a minor subset of gastrointestinal stromal tumor. Here, we describe a case of gastrointestinal stromal tumor in the liver of a 70-year-old man. The tumor was shown to be completely limited within the liver by radiologic, intraoperative, and pathologic examinations. Histopathologically, the tumor showed epithelioid cell-type morphology and immunohistochemical expression of CD34 and protein kinase C theta but was negative for cytokeratin, EMA, S-100, and HMB-45. KIT protein expression was very faint, and we judged it as negative. Mutation analysis revealed the presence of PDGFRA gene mutation (V561D) at exon 12. These findings are essentially the same as those typically seen in ordinary KIT-negative epithelioid cell-type gastrointestinal stromal tumor of the digestive tract. Although KIT-negative gastrointestinal stromal tumor occurring outside the gastrointestinal tract is very rare, this entity should be considered as a potential primary hepatic neoplasm.
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Affiliation(s)
- Hidetaka Yamamoto
- Department of Anatomic Pathology, Pathological Sciences, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.
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Goh BKP, Kesavan SM, Wong WK. An unusual cause of a pancreatic head tumor. Gastroenterology 2009; 137:e5-6. [PMID: 19567238 DOI: 10.1053/j.gastro.2009.01.060] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2009] [Accepted: 01/22/2009] [Indexed: 12/02/2022]
Affiliation(s)
- Brian K P Goh
- Department of Surgery, Singapore General Hospital, Singapore PR
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