9例子宫脉管内平滑肌瘤病的临床与病理分析

INTRAVASCULAR LEIOMYOMATOSIS OF THE UTERUS CLINICOPATHOLOGIC ANALYSIS OF 9 CASES

本文子宫脉管内平滑肌瘤病除1例外,均伴有平滑肌瘤。平均年龄40.3岁,临床表现为月经过多(7/9)和盆腔肿块(8/9)。7例有分娩史,子宫从稍增大至似妊娠5个月(合并早孕),增大程度与合并平滑肌瘤相关,子宫切面可见扩大的脉管内有0.1～2.0cm的结节(3例),3例瘤灶有蒂与脉管壁相连,3例见瘤灶自肌层内向脉管内生长,5例累及子宫外静脉,瘤灶均为平滑肌组成,血管丰富。资料示瘤灶的组织发生来自子宫肌瘤或脉管平滑肌。生物行为具侵蚀性,因临床表现与子宫肌瘤相仿,手术时应仔细检查,彻底切除病灶,术后应长期随访。

Clinicopathologic study of 9 cases of intravascular leiomymatotis (IVL) was performed. The patient's ages ranged from 26 to 49 years (median 40.3 years). Seven cases presented menorrhagia, 8 presented pelvic mass, and one was complicated by early pregnancy. Gross examination disclosed uterine myoma in 8 cases: and wormlike plugs of tumor within myometrial vessel were noted in myometrial section in 4 cases. Extension of tumor into the veins of broad ligament was observed in 5 cases. In one case the tumor extended into the area behind bladder and perivagina. Microscopically, the intravascular tumors resembled the primary leiomyomas, hyaline degeneration necrosis and hemorrhage were found. All 4 cases with follow-up information are alive with no evidence of the disease at postoperative interval of 0.5 to 4 years. The clinicopathologic features, histogenesis, pathologic differential diagnosis and treatment are discussed.