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.

An esophageal gastrointestinal stromal tumor in a patient with MEN1-related pancreatic gastrinoma : an unusual association and review of the literature / S. Massironi, R. Rossi, S. Ferrero, F. Cavalcoli, M. Spampatti, D. Conte, S. Corbetta, M. Peracchi. - In: JOURNAL OF CANCER RESEARCH AND THERAPEUTICS. - ISSN 0973-1482. - 10:2(2014), pp. 443-445. [10.4103/0973-1482.136685]

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

S. Massironi
;
R. Rossi
Secondo
;
S. Ferrero;F. Cavalcoli;M. Spampatti;D. Conte;S. Corbetta
Penultimo
;
M. Peracchi
Ultimo
2014

Abstract

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.
Gastrinoma; gastrointestinal stromal tumors; multiple endocrine neoplasia type 1 syndrome; neuroendocrine tumor; zollinger ellison syndrome; Adult; Esophageal Neoplasms; Gastrinoma; Gastrointestinal Stromal Tumors; Humans; Liver Neoplasms; Male; Multiple Endocrine Neoplasia Type 1; Neoplasms, Second Primary; Pancreatic Neoplasms; Proto-Oncogene Proteins; Oncology; Radiology, Nuclear Medicine and Imaging; Medicine (all)
Settore MED/13 - Endocrinologia
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/328760
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