Tamura K, Nishimori I, Ito T, Yamasaki I, Igarashi H, Shuin T. Diagnosis and management of pancreatic neuroendocrine tumor in von Hippel-Lindau disease. World J Gastroenterol 2010; 16(36): 4515-4518 [PMID: 20857520 DOI: 10.3748/wjg.v16.i36.4515]
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Kenji Tamura, Ichiro Yamasaki, Taro Shuin, Department of Urology, Kochi Medical School, Nankoku, Kochi 783-8505, Japan
Isao Nishimori, Nishimori’s Clinic, Sakawa, Kochi 789-1233, Japan
Tetsuhide Ito, Hisato Igarashi, Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Higashi-ku, Fukuoka 812-8582, Japan
Author contributions: Nishimori I and Ito T contributed equally to this work; Shuin T designed the research; Yamasaki I and Igarashi H analyzed the data; Tamura K and Nishimori I wrote the paper.
Supported by The Health and Labor Sciences Research Grant for a nationwide clinical survey and establishment of guidelines in the diagnosis and treatment for von Hippel-Lindau disease in Japan
Received: February 10, 2010 Revised: April 25, 2010 Accepted: May 2, 2010 Published online: September 28, 2010
Abstract
The pancreatic manifestations seen in patients with von Hippel-Lindau (VHL) disease are subdivided into 2 categories: pancreatic neuroendocrine tumors (NET), and cystic lesions, including simple cyst and serous cystadenoma. The VHL-associated cystic lesions are generally asymptomatic and do not require any treatment, unless they are indistinguishable from other cystic tumor types with malignant potential. Because pancreatic NET in VHL disease are non-functioning and have malignant potential, it is of clinical importance to find and diagnose these as early as possible. It will be recommended that comprehensive surveillance using dynamic computed tomography for abdominal manifestations, including pancreatic NET, should start from the age of 15 years in VHL patients. Unlike sporadic non-functioning NET without VHL disease, in which surgical resection is generally recommended, VHL patients at lower metastatic risk of pancreatic NET should be spared the risks of operative resection.
