Abstract
A 64-year-old man was admitted to our hospital with hematemesis. Emergency upper gastrointestinal endoscopy revealed bleeding from a submucosal tumor (SMT) in the antrum of the stomach, with two other SMTs at different sites. Based on his family history, we diagnosed familial multiple gastric gastrointestinal stromal tumors (GISTs) and performed laparoscopic total gastrectomy. Three distinct tumors were found: one in the fornix, one in the lesser curvature of the angle, and one in the antrum of the stomach. Microscopic examination of the resected specimens revealed different cytomorphologies, of the spindle and epithelioid type, as well as immunophenotypes in the tumors. Mutation analysis revealed different sites of mutation in c-kit and PDGFRA. No mutation was detected in the normal tissue of the stomach. These findings confirmed a diagnosis of multiple sporadic gastric GISTs. Thus, investigating germline mutation might assist in the preoperative diagnosis of multiple gastric GISTs.
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Liegl-Atzwanger B, Fletcher JA, Fletcher CD. Gastrointestinal stromal tumors. Virchows Arch. 2010;456:111–27.
Kang DY, Park CK, Choi JS, Jin SY, Kim HJ, Joo M, et al. Multiple gastrointestinal stromal tumors: clinicopathologic and genetic analysis of 12 patients. Am J Surg Pathol. 2007;31:224–32.
Gasparotto D, Rossi S, Bearzi I, Doglioni C, Marzotto A, Hornick JL, et al. Multiple primary sporadic gastrointestinal stromal tumors in the adult: an underestimated entity. Clin Cancer Res. 2008;14:5715–21.
Agarwal R, Robson M. Inherited predisposition to gastrointestinal stromal tumor. Hematol Oncol Clin North Am. 2009;23:1–13.
Haller F, Schulten HJ, Armbrust T, Langer C, Gunawan B, Füzesi L. Multicentric sporadic gastrointestinal stromal tumors (GISTs) of the stomach with distinct clonal origin: differential diagnosis to familial and syndromal GIST variants and peritoneal metastasis. Am J Surg Pathol. 2007;31:933–7.
Okabe H, Obama K, Tanaka E, Nomura A, Kawamura J, Nagayama S, et al. Intracorporeal esophagojejunal anastomosis after laparoscopic total gastrectomy for patients with gastric cancer. Surg Endosc. 2009;23:2167–71.
Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol. 2002;33:459–65.
Hirashima K, Takamori H, Hirota M, Tanaka H, Ichihara A, Sakamoto Y, et al. Multiple gastrointestinal stromal tumors in neurofibromatosis type 1: report of a case. Surg Today. 2009;39:979–83.
Agaimy A, Dirnhofer S, Wünsch PH, Terracciano LM, Tornillo L, Bihl MP. Multiple sporadic gastrointestinal stromal tumors (GISTs) of the proximal stomach are caused by different somatic KIT mutations suggesting a field effect. Am J Surg Pathol. 2008;32:1553–9.
Agaimy A, Märkl B, Arnholdt H, Wünsch PH, Terracciano LM, Dirnhofer S, et al. Multiple sporadic gastrointestinal stromal tumours arising at different gastrointestinal sites: pattern of involvement of the muscularis propria as a clue to independent primary GISTs. Virchows Arch. 2009;455:101–8.
Miselli F, Conca E, Casieri P, Grosso F, Schiavo M, Tamborini E, et al. A sporadic multiple GIST with unusual pathologic, molecular, and genetic features. Am J Surg Pathol. 2008;32:340–1.
Kawanowa K, Sakuma Y, Sakurai S, Hishima T, Iwasaki Y, Saito K, et al. High incidence of microscopic gastrointestinal stromal tumors in the stomach. Hum Pathol. 2006;37:1527–35.
Corless CL, McGreevey L, Haley A, Town A, Heinrich MC. KIT mutations are common in incidental gastrointestinal stromal tumors one centimeter or less in size. Am J Pathol. 2002;160:1567–72.
Nishida T, Hirota S, Yanagisawa A, Sugino Y, Minami M, Yamamura Y, et al. Clinical practice guidelines for gastrointestinal stromal tumors (GIST) in Japan: English version. Int J Clin Oncol. 2008;13:416–30.
Agarwal R, Robson M. Inherited predisposition to gastrointestinal stromal tumor. Hematol Oncol Clin N Am. 2009;23:1–13.
DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg. 2000;231:51–8.
Novitsky YW, Kercher KW, Sing RF, Heniford BT. Long-term outcomes of laparoscopic resection of gastric gastrointestinal stromal tumors. Ann Surg. 2006;243:738–47.
Goh BK, Chow PK, Chok AY, Chan WH, Chung YF, Ong HS, et al. Impact of the introduction of laparoscopic wedge resection as a surgical option for suspected small/medium-sized gastrointestinal stromal tumors of the stomach on perioperative and oncologic outcomes. World J Surg. 2010;34:1847–52.
Nishimura J, Nakajima K, Omori T, Takahashi T, Nishitani A, Ito T, et al. Surgical strategy for gastric gastrointestinal stromal tumors: laparoscopic vs. open resection. Surg Endosc. 2007;21:875–8.
Sasaki A, Koeda K, Obuchi T, Nakajima J, Nishizuka S, Terashima M, et al. Tailored laparoscopic resection for suspected gastric gastrointestinal stromal tumors. Surgery. 2010;147:516–20.
Dell’Avanzato R, Carboni F, Palmieri MB, Palmirotta R, Guadagni F, Pippa G, et al. Laparoscopic resection of sporadic synchronous gastric and jejunal gastrointestinal stromal tumors: report of a case. Surg Today. 2009;39:335–9.
Demetri GD, Mehren M, Antonescu CR, DeMatteo RP, Ganjoo KN, Maki RG, et al. NCCN task force report: update on the management of patients with gastrointestinal stromal tumors. J Natl Comp Cancer Network. 2010;8(suppl):1–43.
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We thank Chihiro Watanabe for his critical review of the manuscript, Koichi Araki for excellent technical assistance, and Hideo Mishima for providing the sections from the sister of the patient.
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Fujimoto, A., Kobayashi, T., Uchida, S. et al. Laparoscopic total gastrectomy for multiple sporadic gastric gastrointestinal stromal tumors: report of a case. Surg Today 42, 84–88 (2012). https://doi.org/10.1007/s00595-011-0011-x
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DOI: https://doi.org/10.1007/s00595-011-0011-x