Suzuki Ryosuke, Taki Yusuke, Arai Kazumori, Sato Shinsuke, Watanabe Masaya
Department of Gastroenterological Surgery, Shizuoka General Hospital, Shizuoka, JPN.
Department of Pathology, Shizuoka General Hospital, Shizuoka, JPN.
Cureus. 2023 Apr 11;15(4):e37431. doi: 10.7759/cureus.37431. eCollection 2023 Apr.
We report a case of a relatively large desmoid fibromatosis that responded completely to tamoxifen as a single drug therapy. A 47-year-old Japanese man underwent laparoscopy-assisted endoscopic submucosal dissection for a duodenal polyp. He developed postoperative generalized peritonitis and underwent an emergency laparotomy. Sixteen months after the surgery, a subcutaneous mass was found on the abdominal wall. Biopsy of the mass revealed estrogen receptor alpha-negative desmoid fibromatosis. The patient underwent total tumor resection. Two years after the initial surgery, he was found to have multiple intra-abdominal masses, with the largest mass measuring 8 cm in diameter. Biopsy revealed fibromatosis, as in the case of the subcutaneous mass. Complete resection was impossible due to the proximity of the duodenum and superior mesenteric artery. Tamoxifen was administered for three years, resulting in complete regression of the masses. No recurrence was observed for the following three years. This case indicates that relatively large desmoid fibromatosis can be successfully treated with a selective estrogen receptor modulator alone and that its effect is not dependent on the estrogen receptor alpha status of the tumor.
我们报告了一例相对较大的硬纤维瘤病，该病例对他莫昔芬单一药物治疗有完全反应。一名47岁的日本男性因十二指肠息肉接受了腹腔镜辅助内镜下黏膜下剥离术。他术后发生了全身性腹膜炎，并接受了急诊剖腹手术。手术后16个月，在腹壁发现一个皮下肿块。肿块活检显示雌激素受体α阴性的硬纤维瘤病。患者接受了肿瘤全切术。初次手术后两年，他被发现有多个腹腔内肿块，最大的肿块直径达8厘米。活检显示为纤维瘤病，与皮下肿块情况相同。由于十二指肠和肠系膜上动脉距离较近，无法进行完整切除。给予他莫昔芬治疗三年，肿块完全消退。在接下来的三年中未观察到复发。该病例表明，相对较大的硬纤维瘤病可以单独使用选择性雌激素受体调节剂成功治疗，并且其效果不依赖于肿瘤的雌激素受体α状态。
