• Simon Turcotte, Baris Turkbey, Stephanie Barak, Steven K Libutti, Alexander H Richard HR, W Marston Linehan, Marybeth S Hughes, Naris Nilubol, Krisana Gesuwan, Corina Millo, Martha Quezado, Peter L Choyke, Electron Kebebew, and Giao Q Phan.
• Endocrine Oncology Section, Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda 20892, MD, USA.
• Surgery. 2012 Dec 1; 152 (6): 1106-17.

BackgroundPatients with von Hippel-Lindau disease (VHL) commonly develop pancreatic cysts and neuroendocrine neoplasms (PNENs or PNETs). Solid microcystic serous adenoma (SMSA), a rare neoplasm described in VHL patients, can be mistaken for PNEN on imaging.MethodsClinical, pathologic, and radiologic data were reviewed on VHL patients who underwent surgery for a preoperative diagnosis of PNEN since 1994 at 1 institution. Blinded to the pathologic diagnoses, radiologists reassessed available imaging.ResultsFor 55 patients, 79 pancreatectomies were performed for presumed PNENs. Ten (18%) patients underwent 12 (15%) resections for neoplasms diagnosed as SMSA on final pathology. The average size of a SMSA leading to operation was 3.6 ± 0.4 cm. Four out of 11 SMSAs were still mistaken for PNENs when imaging was reassessed. The mean FDG-positron emission tomography (PET) standardized uptake value was greater for 17 PNENs (12.1 ± 1.2) compared with 6 SMSAs (4.2 ± 0.5; P = .002). The mean doubling time of SMSAs and PNENs was similar. Seven (15%) patients with pathologically proven PNENs had malignant disease.ConclusionSMSAs can mimic PNENs on nonfunctional imaging; FDG-PET may help to differentiate them. A high index of suspicion is needed to minimize operations performed for SMSA and to counsel VHL patients of their risks of undergoing operation for a lesion with no known malignant potential.Published by Mosby, Inc.

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