An Esophageal Gastrointestinal Stromal Tumor (GIST) in a Patient with MEN-1 Related Pancreatic Gastrinoma


Introduction: Both multiple endocrine neoplasia type 1 (MEN1)-related-gastrinomas and Gastrointestinal Stromal Tumors (GISTs) are rare neoplasms, and their association has been rarely reported.

Aim(s): We present a case of a man with an esophageal GIST and previously an MEN1-related pancreatic gastrinoma.

Materials and methods: The present report refers to a 44-year-old man who underwent surgical removal of a cephalo-pancreatic gastrinoma, peripancreatic lymph nodes and liver metastasis in April of 2004. The patient was then given long-term somatostatin analogs and diagnosed as having MEN-1 syndrome. The follow-up was uneventful until April 2009, when the patient reported a return of heartburn. The clinical assessment excluded a recurrence of gastrinoma, while upper gastrointestinal (GI) tract endoscopy findings were consistent with a narrowing of the distal third of the esophagus.

Conference: 9th Annual ENETSConcerence (2012)

Presenting Author:

Authors: Rossi R, Massironi S, Ferrero S, Spampatti M, Conte D,

Keywords: Gastrointestinal Stromal Tumors (GISTs), Multiple Endocrine Neoplasia 1 (MEN-1) syndrome, gastrinoma,

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