Extra-gastrointestinal stromal tumor of the pancreas: case report and review of the literature

World Journal of Surgical Oncology

Cosimo Sperti¹  Stefano Merigliano¹  Sara Pizzi²  Davide Pastorelli³  Mario Gruppo¹  Valentina Beltrame¹ 

[1] Department of Surgery, Oncology and Gastroenterology, 3rd Surgical Clinic, University of Padua, Giustiniani 2, 35128, Padua, Italy;Department of Pathology, University of Padua, Padua, Italy;Department of Oncology, Rare Tumors Unit, Veneto Institute of Oncology, Padua, Italy

关键词: Pancreatic neoplasms;    Pancreatectomy;    Pancreas;    Gastrointestinal stromal tumor;    Differential diagnosis;   

Others  :  819848
DOI  :  10.1186/1477-7819-12-105

 received in 2013-09-09, accepted in 2014-04-07,  发布年份 2014

【 摘 要 】

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.

【 授权许可】

   
2014 Beltrame et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

Figure 3.

Figure 4.

【 参考文献 】

• [1]Beham AW, Scheefer IM, Schuler P, Cameron S, Ghadimi BM: Gastrointestinal stromal tumors. Int J Colorectal Dis 2012, 27:689-700.
• [2]Kindblom L-G, Remotti HE, Aldenborg F, Meis-Kindblom JM: Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cell of Cajal. Am J Pathol 1998, 152:1259-1269.
• [3]Sircar K, Hewlett BR, Huizinga JD, Chorneyko K, Berezin I, Ridell RH: Interstitial cell of Cajal as precursor of gastrointestinal stromal tumors. Am J Surg Pathol 1999, 23:377-389.
• [4]Robinson TL, Sircar K, Hewlett BR, Chorneyko K, Riddel RH, Huizinga JD: Gastrointestinal stromal tumors may originate from a subset of CD34-positiva interstitial cells of Cajal. Am J Pathol 2000, 156:1157-1163.
• [5]Beham A, Schaefer IM, Cameron S, von Hammerstein K, Fuzesi L, Ramadori G, Ghadimi MB: Duodenal GIST: a single center experience. Int J Colorectal Dis 2013, 28:581-590.
• [6]Buchs NC, Bucher P, Gervaz P, Ostermann S, Pugin F, Morel P: Segmental duodenectomy for gastrointestinal tumor stromal tumor of the duodenum. World J Gastroenterol 2010, 16:2788-2792.
• [7]Miettinen M, Kopczynski J, Makhlouf HR, Sarlomo-Rikala M, Gyorffy H, Burke A, Sobin LH, Lasota J: Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the duodenum: a clinicopathologic, immunohistochemical, and molecular genetic study of 167 cases. Am J Surg Pathol 2003, 27:625-631.
• [8]Reith JD, Goldblum GR, Lyles RH, Weiss SW: Extragastrintestinal (soft tissue) stromal tunor s: an analysis of 48 cases with emphasis on histologic predictors of outcome. Mod Pathol 2000, 13:577-585.
• [9]Miettinen M, Lasota J: Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol 2006, 23:70-83.
• [10]Miettinen M, Monihan JM, Sarlomo-Rikala M, Kovatich AJ, Carr NJ, Emory TS, Sobin LH: Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery: clinicopathologic and immunoistochemical study of 26 cases. Am J Surg Pathol 1999, 23:1109-1118.
• [11]Neto MR, Machuca TN, Pinho RV, Yuasa LD, Bleggi-Torres LF: Gastrointestinal stromal tumor: report of two unusual cases. Virchows Arch 2004, 444:594-596.
• [12]Yamura K, Kato K, Mijazawa M, Haba Y, Muramatsu A, Miyata K, Koide N: Stromal tumor of the pancreas with expression of c-kit protein: report of a case. J Gastrointestinal Hepatol 2004, 19:467-470.
• [13]Krska Z, Peskovà M, Povysil C, Horejs J, Sedlàckovà E, Kudrnovà Z: GIST of pancreas. Prague Med Rep 2005, 106:201-208.
• [14]Daum O, Klecka J, Ferda J, Treska V, Vanecek T, Sima R, Mukensnabi P, Michal M: Gastrointestinal stromal tumor of the pancreas: Case report with documentation of KIT gene mutation. Virchows Arch 2005, 446:470-472.
• [15]Showalter SL, Loyd JM, Glassman DT, Berger AC: Extra-intestinal stromal tumor of the pancreas: case report and a review of the literature. Arch Surg 2008, 143:305-308.
• [16]Yan BM, Pai RK, Dam JV: Diagnosis of pancreatic gastrointestinal stromal tumor by EUS guided FNA. JOP 2008, 9:192-196.
• [17]Yang F, Jin C, Fu D, Ni Q: Extra-gastrointestinal stromal tumor of the pancreas: Clinical characteristics, diagnosis, treatment, and outcome. J Surg Oncol 2011, 103:739-740.
• [18]Harindhanavdhi T, Tanawuttiwat T, Pyle J, Sillva R: Extra-gastrointestinal stromal tumor presenting as hemorrhagic pancreatic cyst diagnosed by EUS-FNA. JOP 2009, 10:189-191.
• [19]Trabelsi A, Yacoub-Abid LB, Mtimed A, Ben Abdelkrim S, Hammedi F, Ben Ali A, Mokni M: Gastrointestinal stromal tumor of the pancreas. A case report and review of the literature. North Am J Med Sci 2009, 1:234-236.
• [20]Goh BK, Kesavan SM, Wonk WK: An unusual cause of pancreatic head tumor. Gastroenterology 2009, 137:e5-e6.
• [21]Padhi S, Kongara R, Uppin MS, Prayaga AK, Challa S, Nagari B, Regulagadda SA: Extra-gastrointestinal stromal tumors arising in the pancreas: a case report with a review of the literature. JOP 2010, 11:244-248.
• [22]Saif MW, Hotchikiss S, Kaley K: Gastrointestinal stromal tumors of the pancreas. JOP 2010, 11:405-406.
• [23]Crisan A, Nicoara E, Cucui V, Cornea G, Laza R: Prolonged fever associated with gastrointestinal stromal tumor-case report. J Exp Med Surg Res 2010, 17:219-224.
• [24]Joshi J, Rustagi T: Pancreatic extra-gastrointestinal stromal tumor: an unusual presentation of a rare diagnosis. Gastrointest Cancer Res 2010, Suppl 1:S29-S30.
• [25]Rao RW, Vij M, Singla N, Kumar A: Malignant pancreatic extra-gastrointestinal stromal tumor diagnosed by ultrasound fine needle aspiration cytology. A case report with a review of literature. JOP 2011, 12:283-286.
• [26]Cecka F, John B, Ferko A, Subrt Z, Nioliv DH, Tycova V: Long term survival of a patient after resection of gastrointestinal stromal tumor arising from the pancreas. Hepatobiliry Pancreat Dis Int 2011, 10:330-332.
• [27]Vij M, Agrawal V, Pandey R: Malignant extra-gastrointestinal stromal tumor of the pancreas. A case report and review of literature. JOP 2011, 12:200-204.
• [28]Soufi M, Bouziane M, Massrouri R, Chad B: Pancreatic GIST with pancreas divisum: a new entity. Int J SurgCase Rep 2013, 4:68-71.
• [29]Kim HH, Koh YS, Park EK, Seoung JS, Hur YH, Kim JC, Cho CK: Primary extragastrointestinal stromal tumor arising in the pancreas: report of a case. Surg Today 2012, 42:386-390.
• [30]Babu SR, Kumari S, Zhang Y, Su A, Wang W, Tian B: Extragastrointestinal stromal tumor arising in the pancreas: a case report and literature review. J Gastroenterol Hepatol Res 2012, 1:80-83.
• [31]Yamamoto H, Oda Y, Kawaguchi K, Nakamura N, Takahira T, Tamiya S, Saito T, Oshiro Y, Ohta M, Yao T, Tsuneyoshi M: C-kit and PDGFRA mutations in extragastrointestinal stromal tumor (gastrointestinal stromal tumor of the soft tissue). Am J Surg Pathol 2004, 28:479-488.
• [32]Sobin LH, Gospodarovicz MK, Wittekind C: UICC TNM Clasification of malignant tumours. 7th edition. Hoboken, NJ: Wiley-Blackwell; 2009.
• [33]Agaimy A: Gastrointestinal stromal tumors (GIST) from risk stratification system to the new TNM proposal: more question and answers? A review emphasizing the need for a standardization GIST reporting. Int J Clin Exp Pathol 2010, 3:461-471.
• [34]Visser BC, Yeh BM, Qayyum A, Way LW, McCulloch CE, Coakley FV: Characterization of cystic pancreatic masses: relative accuracy of CT and MRI AJR. Am J Roentgenol 2007, 189:648-656.
• [35]Lee HJ, Kim MJ, Choi JY, Hong HS, Kim KA: Relative accuracy of CT and MRI in the differentiation of benign from malignant pancreatic cystic lesions. Clin Radiol 2011, 66:315-321.
• [36]Akahoshi K, Sumida Y, Matsui N, Oya M, Akinaga R, Kubokawa M, Motomura Y, Honda K, Watanabe M, Nagaie T: Preoperative diagnosis of gastrointestinal stromal tumour by endoscopic ultrasound-guided fine needle aspiration. World J Gastroenterol 2007, 13:2077-2082.
• [37]Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, Miettinen M, O’Leary TJ, Remotti H, Rubin BP, Shmookler B, Sobin LH, Weiss SW: Diagnosis of gastrointestinal stromal tumors; a consensus approach. Hum Pathol 2002, 33:459-465.
• [38]Gervaz P, Huber O, Morel P: Surgical management of gastrointestinal stromal tumors. Br J Surg 2009, 96:567-578.
• [39]Golpadas RR, Toedter LJ, Dorman SA, Rohatgi C: Gastrointestinal stromal tumor of the pancreatoduodenal complex: a detailed review and development of new prognostic scoring system. Cancer Ther 2008, 6:577-596.
• [40]Zhou B, Zhang M, Wu J, Yan S, Zhou J, Zheng S: Pancreaticoduodenectomy versus local resection in the treatment of gastrointestinal stromal tumors of the duodenum. World J Gastroenterol 2013, 11:196-201.
• [41]Demetri GD, VonMehren M, Antonescu CR, DeMatteo RP, Ganjoo KN, Maki RG, Pisters PW, Raut CP, Riedel RF, Schuetze S, Sundar HM, Trent JC, Wayne JD: NCCN task force report: Update on the management of patients with gastrointestinal stromal tumors. J Natl Compr Canc Net 2010, 8:S1-S41.
• [42]Demetri GD, Mehren von M, Blanke CD, Van den Abbeele AD, Eisemberg B, Roberts PJ, Heinrich MC, Tuveson DA, Singer S, Janicek M, Fletcher JA, Silverman SG, Silberman SL, Capdeville R, Kiese B, Peng B, Dimitrijevic S, Druker BJ, Corless C, Fletcher CD, Joensuu H: Efficacy and safety of Imatinib mesylate in advanced gastrointestinal stromal tumor. N Engl J Med 2002, 347:472-480.
• [43]Demetri GD, Oosterom Van AT, Garrett CR, Blackstein ME, Shah MH, Verweij J, McArthur G, Judson IR, Heinrich MC, Morgan JA, Desai J, Fletcher CD, George S, Bello CL, Huang X, Baum CM, Casali PG: Efficacy and safety of Sunitinib in patients with advanced gastrointestinal stromal tumour after failure of Imatinib: a randomized controlled trial. Lancet 2006, 368:1329-1338.
• [44]Blanke CD, Rankin C, Demetri GD, Ryan CW, von Mehren M, Benjamin RS, Raymond AK, Bramwell VH, Baker LH, Maki RG, Tanaka M, Hecht JR, Heinrich MC, Fletcher CD, Crowley JJ, Borden EC: Phase III randomized, intergroup trial assessing imatinib mesylate at two dose levels in patients with unresectable or metastatic gastrointestinal stromal tumors expressing the kit receptor tyrosine kinase. S0033. J Clin Oncol 2008, 26:626-632.
• [45]Dematteo RP, Ballman KW, Antonescu CR, Maki RG, Pisters PW, Demetri GD, Blackstein ME, Blanke CD, von Mehren M, Brennan MF, Patel S, McCarter MD, Polikoff JA, Tan BR, Owzar K, American School of Surgeons Oncology Group (ACOSOG) Intergroup Adjuvant GIST Study Team: Adjuvant Imatinib mesylate after resection of localized, primary gastrointestinal stromal tumour: a randomized, dowble-blind, placebo-controlled trial. Lancet 2009, 373:1097-104.
• [46]Dubreuil P, Letard S, Ciufolini M, Gros L, Humbert M, Castéran N, Borge L, Hajem B, Lermet A, Sippl W, Voisset E, Arock M, Auclair C, Leventhal PS, Mansfield CD, Moussy A, Hermine O: Masitinib (AB 1010) A potent and selective tyrosine Khinase inhibitor targeting Kit. PLoS One 2009, 4:e7258.
• [47]Sawaki A, Nishida T, Doi T, Yamada Y, Komatsu Y, Kanda T, Kakeji Y, Onozawa Y, Yamasaki M, Ohtsu A: Phase 2 study of Nilotinib as third-line therapy for patients with gastrointestinal stromal tumor. Cancer 2011, 117:4633-4641.
• [48]Park SH, Ryu MH, Ryoo BY, Im SA, Kwon HC, Lee SS, Park SR, Kang BY, Kang YK: Sorafenib in patients with metastatic gastrointestinal stromal tumors who failed two or more prior tyrosine kinase inhibitors: a phase II study of Korean gastrointestinal stromal tumors study group. Invest New Drugs 2012, 30:2377-2383.
• [49]Joensuu H, Eriksson M, Sundby Hall K, Hartmann JT, Pink D, Schutte J, Ramadori G, Hohenberger P, Duyster J, Al-Batran SE, Schlemmer M, Bauer S, Wardelmann E, Sarlomo-Rikala M, Nilsson B, Sihto H, Monge OR, Bono P, Kallio R, Vehtari A, Leinonen M, Alvegard T, Reichardt P: One vs Three years of adjuvant Imatinib for operable gastrointestinal stromal tumor: a randomized trial. JAMA 2012, 307:1265-1272.