Kato Kazuya, Nagase Atsushi, Onodera Kazuhiko, Matsuda Minoru, Iwasaki Yoshiaki, Kato Yurina, Kato Kimitaka, Kawakami Takako, Taniguchi Masahiko, Furukawa Hiroyuki
Department of Surgery, Pippu Clinic, 2-10, 1 Cyome Nakamachi, Pippu Town Kamikawa-gun, Hokkaido, 078-0343, Japan.
J Med Case Rep. 2011 Oct 23;5:521. doi: 10.1186/1752-1947-5-521.
Gastric tumors in patients affected by neurofibromatosis type 1 are usually carcinoids or stromal tumors, and rarely adenocarcinomas.
We report a case of an adenocarcinoma of the stomach in a 53-year-old Japanese man with neurofibromatosis type 1. An abdominal computed tomography scan and ultrasonography showed tumors in his liver. Gastric fibroscopy revealed a Borrmann type III tumor on his cardia that had spread to his esophagus and was highly suspicious for malignancy. Multiple biopsies showed an adenocarcinoma of the stomach, which was evaluated as gastric cancer, stage IV. Chemotherapy with TS-1 was performed. Our patient died four weeks after initial admission. Histological examination of a liver needle biopsy showed metastatic adenocarcinoma in his liver.
To the best of our knowledge, high serum levels of α-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 72-4, resulting from gastric adenocarcinoma, have not been reported previously in a patient with neurofibromatosis type 1. We report this rare case along with a review of the literature.
1型神经纤维瘤病患者的胃部肿瘤通常为类癌或间质瘤，腺癌罕见。
我们报告一例53岁日本男性1型神经纤维瘤病患者发生胃腺癌的病例。腹部计算机断层扫描和超声检查显示其肝脏有肿瘤。胃镜检查发现其贲门处有Borrmann III型肿瘤，已扩散至食管，高度怀疑为恶性肿瘤。多次活检显示为胃腺癌，评估为IV期胃癌。给予替吉奥化疗。患者初次入院四周后死亡。肝脏穿刺活检的组织学检查显示肝脏有转移性腺癌。
据我们所知，此前尚未有1型神经纤维瘤病患者因胃腺癌导致血清甲胎蛋白、癌胚抗原和糖类抗原72-4水平升高的报道。我们报告这一罕见病例并进行文献复习。
