Miki Masami, Kawabe Ken, Igarashi Hisato, Abe Tatsuro, Ohishi Yoshihiro, Hashimoto Risa, Karashima Takashi, Yamasaki Ichiro, Inoue Keiji, Ito Tetsuhide, Ogawa Yoshihiro
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Japan.
Department of Anatomic Pathology Science, Graduate School of Medical Sciences, Kyushu University, Japan.
Intern Med. 2018 Jul 15;57(14):2007-2011. doi: 10.2169/internalmedicine.0416-17. Epub 2018 Feb 28.
A 45-year old woman who underwent several surgeries for tumors associated with von Hippel-Lindau disease (VHL) was referred to our hospital due to a pancreatic tumor and liver tumors. She was diagnosed with pancreatic neuroendocrine tumor (NET) with a Ki67 index of 40% based on the examination of a biopsy specimen of the liver tumors. She was treated with everolimus for 6 months and sunitinib for 6 weeks as first- and second-line therapies. She survived for 13 months. At autopsy the diagnosis of pancreatic neuroendocrine tumor (NET)-G3 was confirmed. We herein report an aggressive clinical course of VHL-related NET G3. The further accumulation of cases is required to reach a consensus on treatment for this disease.
一名45岁女性因与冯·希佩尔-林道病（VHL）相关的肿瘤接受了多次手术，因胰腺肿瘤和肝脏肿瘤转诊至我院。根据肝脏肿瘤活检标本检查，她被诊断为胰腺神经内分泌肿瘤（NET），Ki67指数为40%。她接受了依维莫司6个月和舒尼替尼6周的一线和二线治疗。她存活了13个月。尸检确诊为胰腺神经内分泌肿瘤（NET）-G3。我们在此报告一例VHL相关NET G3的侵袭性临床病程。需要进一步积累病例以就该疾病的治疗达成共识。
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