title: von Hippel-Lindau disease-associated solid microcystic serous adenomas masquerading as pancreatic neuroendocrine neoplasms.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Endocrine Oncology Section, Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda 20892, MD, USA.
authors.forename: Simon
authors.initials: S
authors.lastname: Turcotte
authors.forename: Baris
authors.initials: B
authors.lastname: Turkbey
authors.forename: Stephanie
authors.initials: S
authors.lastname: Barak
authors.forename: Steven K
authors.initials: SK
authors.lastname: Libutti
authors.forename: H Richard
authors.initials: HR
authors.lastname: Alexander
authors.forename: W Marston
authors.initials: WM
authors.lastname: Linehan
authors.forename: Marybeth S
authors.initials: MS
authors.lastname: Hughes
authors.forename: Naris
authors.initials: N
authors.lastname: Nilubol
authors.forename: Krisana
authors.initials: K
authors.lastname: Gesuwan
authors.forename: Corina
authors.initials: C
authors.lastname: Millo
authors.forename: Martha
authors.initials: M
authors.lastname: Quezado
authors.forename: Peter L
authors.initials: PL
authors.lastname: Choyke
authors.forename: Electron
authors.initials: E
authors.lastname: Kebebew
authors.forename: Giao Q
authors.initials: GQ
authors.lastname: Phan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018293:Cystadenoma, Serous / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D010180:Pancreatectomy; D010181:Pancreatic Cyst / Q000150:complications; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000175:diagnosis