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Year : 2014  |  Volume : 10  |  Issue : 2  |  Page : 443-445

An esophageal gastrointestinal stromal tumor in a patient with MEN1-related pancreatic gastrinoma: An unusual association and review of the literature

1 Gastroenterology and Endoscopy Unit, Department of Pathophysiology and Transplant, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Italy
2 Gastroenterology and Endoscopy Unit, Department of Pathophysiology and Transplant, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico; Universitą degli Studi di Milano, Milan, Italy
3 Universitą degli Studi di Milano, Milan; Division of Pathology, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Italy
4 Universitą degli Studi di Milano; Endocrinology and Diabetology Unit, IRCCS Policlinico S. Donato, Milan, Italy

Correspondence Address:
Sara Massironi
Gastroenterology Unit II, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Via F. Sforza 35, Milano
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.136685

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Both multiple endocrine neoplasia type 1 (MEN1)-related gastrinomas and gastrointestinal stromal tumors (GISTs) are rare neoplasms, and their association has been rarely reported. We describe an unusual association between a GIST and a MEN1-related gastrinoma. A 44-year-old man had undergone surgical removal of a pancreatic gastrinoma in 2004 and was then administered long-term somatostatin analogs, and diagnosed as having MEN1 syndrome. Following an uneventful follow-up, in April 2009, an upper gastrointestinal tract endoscopy showed esophageal narrowing, with evidence of a 2-cm solid mass on endoscopic ultrasonography. Histology revealed a tumor composed of elongated cells with plump cytoplasm arranged in a storiform pattern. The immunophenotype of the lesion was CD117 and Platelet Derived Growth Factor (PDGF) positive, whereas alpha-1 muscle actin and S-100 protein were negative. Due to morphological and immunohistochemical results, a final diagnosis of esophageal GIST was made. The association between GISTs and MEN1 could be casual, although a single case of the coexistence of a GIST and a MEN1-related gastrinoma has already been reported. A role of the MEN1 gene in the pathogenesis of GISTs could be hypothesized.

Abstract in Chinese

一个食管胃肠间质瘤病人并发MEN1相关的胰腺胃泌素瘤:不寻常的联系及文献回顾 摘要 多发性内分泌瘤病1型(MEN1)相关的胃泌素瘤和胃肠道间质瘤(GIST)都是罕见的肿瘤,而它们两者相伴发生很少有报道。我们报道一例少见的GIST 和MEN1相关的胃泌素瘤。一个44岁的病人在2004年接受了胰腺胃泌素瘤的手术切除,然后给予长效生长抑素类似物,并被诊断为MEN1综合征。后来的随访无特别事件,到2009年4月,上消化道内镜检查发现食管狭窄,内镜超声检查证实为2厘米的固体团块。组织学显示为细长的胞质和饱满的细胞排列成席纹状图案的肿瘤。病变的免疫表型为CD117和血小板源性生长因子(PDGF)阳性,而α1肌动蛋白和S 100蛋白均为阴性。由于形态学和免疫组化结果,确诊为食管瘤。GIST和MEN1之间的关联可能是偶然的,尽管已有一个两病共存的报告。MEN1基因在胃肠道间质瘤的发病机制起作用可成为一种假设。 关键词:胃泌素瘤,胃肠道间质瘤,多发性内分泌肿瘤1型综合征,神经内分泌肿瘤,Zollinger-Ellison综合征

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