Lechner W, Ortner A, Thöni A, Schuler G, Mikuz G
Gynecol Oncol. 1983 Apr;15(2):253-60. doi: 10.1016/0090-8258(83)90081-1.
(1) Histiocytosis-X can manifest itself in virtually every organ, but in gynecology it is an absolute curiosity. (2) Differential diagnosis must exclude specific and nonspecific ulcerations and granulations such as syphilis, tuberculosis, Boeck's disease, and also neoplastic processes like lymphomas, sarcomas, carcinomas, and malignant diseases of the hemopoietic system. (3) The diagnosis by light microscopy alone, as in our case, may be insufficient; therefore, electron microscopy should be used. As soon as the diagnosis is confirmed histologically, an extensive examination of all organs is necessary in order to establish an exact prognosis and an optimal plan of therapy. (4) Because of the unknown etiology of histiocytosis-X, a causal treatment is not yet possible. In spite of this, with a symptomatic, individualized therapy by means of excision, low-dose irradiation and cytotoxic agents a 5-year survival of 90% was obtained for the patients. (5) Because of its rarity and multidisciplinary character, histiocytosis-X is a challenge to interdisciplinary and interregional cooperation. Though not being a malignoma in the strict sense, diagnosis, therapy, and in part prognosis are not essentially different from a malignant disease.
（1）组织细胞增多症-X几乎可在每个器官中表现出来，但在妇科领域却是一种绝对罕见的病症。（2）鉴别诊断必须排除特定和非特定的溃疡及肉芽组织，如梅毒、结核病、结节病，以及淋巴瘤、肉瘤、癌和造血系统恶性疾病等肿瘤性病变。（3）仅像我们病例中那样通过光学显微镜进行诊断可能并不充分；因此，应采用电子显微镜检查。一旦组织学诊断得到证实，为了确定准确的预后和最佳治疗方案，有必要对所有器官进行全面检查。（4）由于组织细胞增多症-X的病因不明，目前还无法进行病因治疗。尽管如此，通过手术切除、低剂量放疗和细胞毒性药物进行对症、个体化治疗，患者的5年生存率达到了90%。（5）由于其罕见性和多学科性质，组织细胞增多症-X对跨学科和跨地区合作构成了挑战。尽管严格意义上来说它并非恶性肿瘤，但其诊断、治疗以及部分预后情况与恶性疾病并无本质区别。
