Von Hippel-Lindau disease-associated solid microcystic serous adenomas masquerading as pancreatic neuroendocrine neoplasms

Simon Turcotte, Baris Turkbey, Stephanie Barak, Steven K. Libutti, H. Richard Alexander, W. Marston Linehan, Marybeth S. Hughes, Naris Nilubol, Krisana Gesuwan, Corina Millo, Martha Quezado, Peter L. Choyke, Electron Kebebew, Giao Q. Phan

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Background: Patients 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. Methods: Clinical, 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. Results: For 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. Conclusion: SMSAs 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.

Original languageEnglish (US)
Pages (from-to)1106-1117
Number of pages12
JournalSurgery (United States)
Issue number6
StatePublished - Dec 1 2012
Externally publishedYes


All Science Journal Classification (ASJC) codes

  • Surgery

Cite this

Turcotte, S., Turkbey, B., Barak, S., Libutti, S. K., Alexander, H. R., Linehan, W. M., Hughes, M. S., Nilubol, N., Gesuwan, K., Millo, C., Quezado, M., Choyke, P. L., Kebebew, E., & Phan, G. Q. (2012). Von Hippel-Lindau disease-associated solid microcystic serous adenomas masquerading as pancreatic neuroendocrine neoplasms. Surgery (United States), 152(6), 1106-1117. https://doi.org/10.1016/j.surg.2012.08.010