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

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• 作者：Simon Turcotte ; MD^a ; Baris Turkbey ; MD^b ; Stephanie Barak ; MD^c ; Steven K. Libutti ; MD^d ; H. Richard Alexander ; MD^e ; W. Marston Linehan ; MD^f ; Marybeth S. Hughes ; MD^a ; Naris Nilubol ; MD^a ; Krisana Gesuwan ; CRNP^a ; Corina Millo ; MD^g ; Martha Quezado ; MD^c ; Peter L. Choyke ; MD^b ; Electron Kebebew ; MD^a ; Giao Q. Phan ; MD^a ; ^{Giao.Phan@nih.gov}
• 刊名：Surgery
• 出版年：2012
• 出版时间：December, 2012
• 年：2012
• 卷：152
• 期：6
• 页码：1106-1117
• 全文大小：2813 K

文摘

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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 ¡À 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 ¡À 1.2) compared with 6 SMSAs (4.2 ¡À 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.