Mashiyama S, Mori T, Seki H, Kanno M, Suzuki J
No Shinkei Geka. 1983 Jan;11(1):83-90.
Thirty-three cases of von Recklinghausen's disease, nine of which had multiple brain tumors, were experienced in our clinic. In this report, these nine cases were reported and discussed, especially representative two cases in detail. Nine cases had various combinations of multiple brain tumors: bilateral acoustic neurinomas in two cases, multiple neurinomas in two cases, multiple meningiomas in one case and multiple neurinomas and meningiomas in two cases. In two cases, multiple brain tumors were totally extirpated at one stage, but both died within ten months. In three cases, partial extirpation of multiple brain tumors were performed at one stage, one died thirteen years after the operation and two are alive but dependent on their families. In recent two cases, two stage operations were performed, by which tumors were successfully removed totally. These postoperative courses were good. The other two cases were treated conservatively. One is alive and working, but has severe auditory disturbance. The other died after six years from onset. Autopsy was performed in this case, which revealed multiple neurinomas in the left 3rd and bilateral 5-12th cranial nerves and numerous meningiomas. In the review of the cases with more than three brain tumors in von Recklinghausen's disease were discussed.
我院共收治33例冯雷克林霍增氏病患者，其中9例患有多发性脑肿瘤。本报告对这9例患者进行了报道和讨论，尤其详细介绍了具有代表性的2例。9例患者的多发性脑肿瘤有多种组合形式：2例为双侧听神经瘤，2例为多发性神经瘤，1例为多发性脑膜瘤，2例为多发性神经瘤和脑膜瘤。2例患者的多发性脑肿瘤在一期手术中全部切除，但均在10个月内死亡。3例患者在一期手术中对多发性脑肿瘤进行了部分切除，1例术后13年死亡，2例存活但需家人照料。最近2例患者接受了分期手术，肿瘤被成功全部切除。术后病程良好。另外2例患者接受了保守治疗。1例存活且能工作，但有严重听力障碍。另1例发病6年后死亡。对该例患者进行了尸检，发现左侧第3对脑神经以及双侧第5 - 12对脑神经有多发性神经瘤，还有大量脑膜瘤。文中还讨论了冯雷克林霍增氏病中脑肿瘤超过3个的病例回顾。
