Kanazawa A, Higashino M, Shiokawa C, Osugi H, Kinoshita H, Yamamoto T, Sakurai M
Second Department of Surgery, Osaka City University Medical School, Japan.
Nihon Geka Gakkai Zasshi. 1994 Nov;95(11):843-6.
We treated a patient with von Recklinghausen's disease and angiosarcoma. This combination has not been reported in Japan to the best of our knowledge. An emergency operation for massive gastrointestinal bleeding was performed on a 68-year-old woman with von Recklinghausen's disease, who had been known to have a retroperitoneal tumor; The bleeding was controlled and the tumor was also resected. Postoperative course was uneventful also but one month after operation intra-abdominal and intrathoracic bleeding developed, and the patient died of respiratory failure. Autopsy showed hemorrhagic lesions, which were diagnosed pathologically metastatic angiosarcoma. The possibility of hemorrhagic lesions in the patients with von Recklinghausen's disease should be kept in mind.
我们治疗了一名患有冯·雷克林霍增氏病(神经纤维瘤病)和血管肉瘤的患者。据我们所知,这种组合在日本尚未有过报道。一名68岁患有冯·雷克林霍增氏病且已知患有腹膜后肿瘤的女性因大量胃肠道出血接受了急诊手术;出血得到控制,肿瘤也被切除。术后病程也平稳,但术后一个月出现腹腔内和胸腔内出血,患者死于呼吸衰竭。尸检显示有出血性病变,经病理诊断为转移性血管肉瘤。应牢记冯·雷克林霍增氏病患者出现出血性病变的可能性。