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子宫静脉内平滑肌瘤病。16例具有不寻常组织学特征病例的临床病理分析。

Intravenous leiomyomatosis of the uterus. A clinicopathological analysis of 16 cases with unusual histologic features.

作者信息

Clement P B, Young R H, Scully R E

机构信息

Department of Pathology, Vancouver General Hospital, Canada.

出版信息

Am J Surg Pathol. 1988 Dec;12(12):932-45.

PMID:3202247
Abstract

We describe 16 cases of intravenous leiomyomatosis of the uterus with unusual histologic features. Most of the patients, who ranged in age from 28 to 76 years (median, 42 years), presented with abnormal uterine bleeding or pelvic pain. On pelvic examination, they had an enlarged uterus. Extension of tumor into the broad ligament was noted at operation in six patients; it was documented on pathologic examination in an additional three cases. Gross examination disclosed enlargement of the uterus by solitary or multiple masses; wormlike plugs of tumor within myometrial veins were noted on initial examination in only five cases. Histologic examination in each case revealed proliferations of benign-appearing smooth muscle within myometrial vessels, at least some of which were large veins. The tumors differed from typical intravenous leiomyomatosis (IVL). They were classified as cellular IVL (six cases), epithelioid IVL (four cases), IVL with bizarre nuclei (two cases), intravenous lipoleiomyomatosis (two cases), myxoid IVL (one case), and IVL with an endometrial component (one case). Of 13 patients with follow-up information, 11 are alive with no evidence of disease or have died of unrelated causes at postoperative intervals of 0.5 to 19 years. One patient underwent resection of pelvic recurrent tumor 2 years postoperatively and was alive with no evidence of disease 2 years later. Another patient had pulmonary metastases 9 years postoperatively, but was alive with stable disease after an additional 6 years. This analysis indicates that most of the histologic variants of benign uterine leiomyoma can exhibit the intravascular growth pattern of IVL. These variants of IVL, except for their cellular composition, resemble typical IVL in both their clinical and pathological features. They should be distinguished from other tumors, particularly endometrial stromal sarcoma and leiomyosarcoma, which differ in their behavior and management.

摘要

我们描述了16例具有不寻常组织学特征的子宫静脉内平滑肌瘤病病例。大多数患者年龄在28至76岁之间(中位数为42岁),表现为异常子宫出血或盆腔疼痛。盆腔检查时,她们的子宫均增大。手术中发现6例肿瘤延伸至阔韧带;另有3例在病理检查中得到证实。大体检查显示子宫因单个或多个肿块而增大;最初检查时仅在5例中发现肌层静脉内有蠕虫状肿瘤栓子。每例组织学检查均显示肌层血管内出现良性外观的平滑肌增生,其中至少一些是大静脉。这些肿瘤与典型的静脉内平滑肌瘤病(IVL)不同。它们被分类为细胞性IVL(6例)、上皮样IVL(4例)、具有奇异核的IVL(2例)、静脉内脂肪平滑肌瘤病(2例)、黏液样IVL(1例)和具有子宫内膜成分的IVL(1例)。在有随访信息的13例患者中,11例存活且无疾病证据,或在术后0.5至19年因无关原因死亡。1例患者术后2年接受盆腔复发性肿瘤切除术,2年后存活且无疾病证据。另1例患者术后9年出现肺转移,但又过了6年后仍存活且病情稳定。该分析表明,大多数良性子宫平滑肌瘤的组织学变异型均可表现出IVL的血管内生长模式。这些IVL变异型,除细胞组成外,在临床和病理特征上均类似于典型IVL。它们应与其他肿瘤相鉴别,尤其是子宫内膜间质肉瘤和平滑肌肉瘤,它们在行为和治疗上有所不同。

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