J Korean Med Sci.  2010 Jun;25(6):953-956. 10.3346/jkms.2010.25.6.953.

A Case of Multiple Endocrine Neoplasia Type 1 with Primary Liver Gastrinoma

• Lee SR
• Choi MC
• Ahn KJ

• KMID: 2150879
• DOI: http://doi.org/10.3346/jkms.2010.25.6.953

Abstract

Gastrinoma is the most frequent functional pancreaticoduodenal endocrine tumor in patients with multiple endocrine neoplasia type 1 (MEN 1). Primary hepatic gastrinomas in MEN 1 are very rare, with no previous reports published in the literature. We reported the case of a 39 yr old female patient with a history of repeated peptic ulcers and a hypoglycemia episode. Abdominal CT indicated a well-defined liver mass and a pancreatic head mass. Somatostatin-receptor scintigraphy with [111In] DTPA octeotride demonstrated a strong uptake of the radiotracer in the left lateral segment at the site of the hepatic mass. After operation, immunohistochemical staining was consistent with pancreatic insulinoma and primary hepatic gastrinoma. As the liver is a common site of metastases from gastrinoma, primary liver gastrinoma has not yet been reported with MEN 1. We diagnosed this patient using immunohistochemical studies and treated this patient by hepatic segmentectomy.

Keyword

Gastrinoma; Multiple Endocrine Neoplasia Type 1; Insulinoma

Figure

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  Fig. 1 CT scan of abdomen, (A) Arrowhead points to 1.5×1.2 cm size hypervascular mass in the pancreas head portion. (B) Arrow showed hypervascular mass at liver lateral segment.

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  Fig. 2 Somatostatin-receptor scintigraphy with [111In] DTPA octreotide showed strong uptake of the radiotracer in the left lateral segment at the site of the hepatic mass.

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  Fig. 3 Histopathological findings of the pancreas tumor. (A) Hematoxilin/eosin stained sections show a well differentiated pancreatic neuroendocrine tumor (original magnification, ×100). (B) Immunohistochemical stains of neuroendocrine tumor are positive for insulin (original magnification, ×100).

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  Fig. 4 Histopathological findings of the liver tumor. (A) Hematoxilin/eosin stained sections show a well differentiated liver neuroendocrine tumor (original magnification,×100). (B) Immunohistochemical stains of neuroendocrine tumor are positive for gastrin (original magnification, ×100).

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