Doi Hirokazu, Ichikawa Soichi, Hiraoka Atsushi, Ichiryu Misa, Nakahara Hiromasa, Ochi Hironori, Tanabe Atsushi, Kodama Akihiro, Hasebe Aki, Miyamoto Yasunao, Ninomiya Tomoyuki, Horiike Norio, Takamura Kazuhito, Kawasaki Hideki, Kameoka Chieko, Kan Miki, Doi Shigekazu, Soga Yoshiko, Tamura Hiromi, Maeda Toshiharu, Asaki Akinori, Seno Seijin, Iguchi Haruo, Hasegawa Tadashi
Department of Gastroenterology, Ehime Prefectural Central Hospital, Matsuyama.
Intern Med. 2009;48(5):329-33. doi: 10.2169/internalmedicine.48.1484. Epub 2009 Mar 2.
The primitive neuroectodermal tumor (PNET) of the pancreas, a member of Ewing's sarcoma family of tumors, is extremely rare. We treated a 37-year-old Japanese man who had a solitary pancreatic tumor 40 mm in diameter and multiple hepatic tumors with surgical resection. The PNET was positive for CD99 on immunohistochemical staining. Fluorescence in situ hybridization (FISH) was also performed, which revealed a Ewing sarcoma breakpoint region 1 (EWSR1) 22q12 rearrangement. According to the Japan-Ewing protocol, chemotherapy with Ifomide (ifosfamide), etoposide, vincristine, and cyclophosphamide was given after surgery. To the best of our knowledge, to date 13 PNET cases have been reported with a mean age for all patients of 19.3 years old. Surgical resection was performed in most cases and some patients received postoperative chemotherapy. The clinicopathologic characteristics and management of this extremely rare disease are also discussed.
胰腺原始神经外胚层肿瘤（PNET）是尤因肉瘤肿瘤家族的一员，极为罕见。我们治疗了一名37岁的日本男性，他有一个直径40毫米的孤立性胰腺肿瘤和多个肝脏肿瘤，接受了手术切除。免疫组织化学染色显示PNET对CD99呈阳性。还进行了荧光原位杂交（FISH），结果显示尤因肉瘤断点区域1（EWSR1）22q12重排。根据日本尤因方案，术后给予异环磷酰胺、依托泊苷、长春新碱和环磷酰胺化疗。据我们所知，迄今为止已报告了13例PNET病例，所有患者的平均年龄为19.3岁。大多数病例都进行了手术切除，一些患者接受了术后化疗。本文还讨论了这种极其罕见疾病的临床病理特征及治疗方法。
