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Pancreatic Somatostatinoma With von Recklinghausen's Disease

Published:September 04, 2008DOI:https://doi.org/10.1016/j.cgh.2008.08.036
      A 48-year-old man with von Recklinghausen's disease (VRD) was hospitalized because of appetite loss and body weight loss. Laboratory studies showed an alkaline phosphatase level of 1333 IU/L, γ-glutamyl transpeptidase level of 1202 IU/L, and total bilirubin level of 1.0 mg/dL. Contrast-enhanced computed tomography images showed a small tumor in the head of the pancreas (Figure A, arrows) and multiple tumors occupying nearly the entire liver, which were enhanced in the early phase (Figure A). Esophagogastroduodenoscopy showed submucosal bulging in the descending part of the duodenum, although no epithelial change was found, including the papilla of Vater. Endoscopic ultrasonography showed a heterogeneous hypoechoic tumor, 2 cm in size, in the head of the pancreas. Integrated [18F]fluorodeoxyglucose–positron emission tomography (PET) detected accumulation of a glucose analog in the pancreatic tumor (Figure B, arrowheads) and the hepatic tumors (Figure C, a PET scan image; Figure C, a PET computed tomography fusion image). The standardized uptake values of the pancreatic tumor and hepatic tumors were 4.1 and 11.5, respectively. Percutaneous liver biopsy was performed and the specimen showed typical features of an endocrine tumor, composed of uniform cells with glandular cavity formation (Figure C). Hormonal immunohistochemical staining of the tumor was strongly positive for somatostatin (Figure D). Additional laboratory studies showed that the plasma somatostatin level was increased markedly (16,000 pg/mL; normal range, 1.0–12 pg/mL), whereas insulin, gastrin, and glucagon levels were within normal limits.
      VRD is a common hereditary disorder with a wide range of clinical features, including abnormalities of the skin, soft tissues, and the nervous system. It is known that patients with VRD frequently develop neoplasms, for example, neurofibrosarcoma, pheochromocytoma, and juvenile chronic myelogenous leukemia. Only 3 cases of pancreatic somatostatinoma associated with VRD have been reported until now.
      • Saurenmann P.
      • Binswanger R.
      • Maurer R.
      • et al.
      Somatostatin-producing endocrine pancreatic tumor in von Recklinghausen's neurofibromatosis.
      • Mao C.
      • Shah A.
      • Hanson D.J.
      • et al.
      Von Recklinghausen's disease associated with duodenal somatostatinoma: contrast of duodenal versus pancreatic somatostatinomas.
      • Fujisawa T.
      • Osuga T.
      • Maeda M.
      • et al.
      Malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease.
      Somatostatinomas are difficult to diagnose before surgery or biopsy because not all patients with somatostatinoma show the classic somatostatin syndrome (diarrhea, diabetes, and cholelithiasis). Meanwhile, the plasma somatostatin level can be a useful diagnostic marker in that it is increased markedly in many patients, including in our patient. Definitive therapy for pancreatic somatostatinoma is surgery. However, because metastatic tumors in the liver were thought to be a prognostic factor in this patient, transcatheter arterial chemoembolization was performed. As a result, the hepatic tumors were reduced and the plasma somatostatin level decreased (3800 pg/mL). The possibility of a malignant endocrine tumor, including somatostatinoma, should be considered when a pancreatic tumor is found in a patient with VRD.

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