Mosel A
Geburtshilfe Frauenheilkd. 1979 Mar;39(3):217-21.
Endometrial carcino-sarcoma is a rare rapidly growing mixed muellerian tumor. The pathogenesis of this tumor is not definitely known. However, the current explanation favors the theory of the growth of this tumor from pluripotential sub-epithelial cells. The incidence of the tumor is increased in post-menopausal women. Many of these women have a history of radiotherapy of the genital organs for benign gynaecological disease. A causal relationship between occurence of carcino-sarcoma and estrogen treatment as in carcinoma of the endometrium is not mentioned in the literature. The clinical signs and symptoms are non-specific as in many other malignant tumors of the uterine body. The prognosis is very bad. The average survival from the onset of the first symptoms is only a few months. The treatment of choice appears to be total abdominal hysterectomy and bilateral salpingo-oophorectomy. Radical operations and ancillary radiotherapy and chemotherapy do not appear to improve the survival rate. The extremely rare coincidence of a carcino-sarcoma in a young woman with Turner Syndrome gonadal dysgenesis after five years of treatment with estrogen led to the present case report.
子宫内膜癌肉瘤是一种罕见的、生长迅速的混合性米勒管肿瘤。该肿瘤的发病机制尚不完全清楚。然而,目前的解释倾向于认为这种肿瘤起源于多能性上皮下细胞。绝经后女性中该肿瘤的发病率有所增加。这些女性中的许多人有因良性妇科疾病接受生殖器官放疗的病史。文献中未提及癌肉瘤的发生与子宫内膜癌中雌激素治疗之间的因果关系。其临床体征和症状与许多其他子宫体恶性肿瘤一样不具有特异性。预后非常差。从首次出现症状开始计算,平均生存期仅几个月。首选的治疗方法似乎是全腹子宫切除术及双侧输卵管卵巢切除术。根治性手术以及辅助放疗和化疗似乎并不能提高生存率。一名患有特纳综合征性腺发育不全的年轻女性在接受雌激素治疗五年后极其罕见地并发了癌肉瘤,由此才有了本病例报告。