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静脉内平滑肌瘤病的子宫:18 例临床病理研究,强调早期诊断和适当的治疗策略。

Intravenous leiomyomatosis of the uterus: a clinicopathologic study of 18 cases, with emphasis on early diagnosis and appropriate treatment strategies.

机构信息

Department of Pathology, Tianjin Cancer Hospital, Tianjin Medical, University, Tianjin 300070, PR China.

出版信息

Hum Pathol. 2011 Sep;42(9):1240-6. doi: 10.1016/j.humpath.2010.10.015. Epub 2011 Jul 20.

DOI:10.1016/j.humpath.2010.10.015
PMID:21777942
Abstract

Intravenous leiomyomatosis is a rare variant of leiomyoma that could result in death. Early and accurate diagnosis and appropriate treatment strategies play a dominant role in good prognosis. Eighteen cases of Intravenous leiomyomatosis , along with clinicopathologic data, were retrieved from our database. Most of the patients who ranged in age from 33 to 54 years (median, 44 years) presented with a pelvic mass or abnormal uterine bleeding. The diagnosis was confirmed by a immunohistochemical staining for smooth muscle actin, CD34, and Ki67. Surgical exploration confirmed the presence of a uterine mass (mean size, 5.08 cm). Wormlike plugs were identified within the broad ligament in 5 cases. The tumor penetrated to the inferior vena cava in 1 case. Histologic variants were noted in 33.33% (6/18) of our cases, which were classified as cellular intravenous leiomyomatosis (3 cases) and intravenous leiomyomatosis with papillary-like contour (1 case) and with fat metaplasia (2 cases). The 18 cases are made up 0.097% of all genital smooth muscle tumor cases of the hospital. The ratios of intravenous leiomyomatosis with uterine leiomyoma, with adenomyosis, with uterine leiomyoma and adenomyosis were 38.89% (7/18), 11.11% (2/18), and 27.78% (5/18), respectively. Follow-up information was available for 16 patients, with a follow-up duration of 26 to 104 months (mean, 55 months). Three cases (16.67%) recurred in patients younger than 40 years (33, 34, and 37 years). We propose that young patients undertake hysterectomy and unilateral salpingo-oophorectomy if they do not have any birthing requests. The cases of intravenous leiomyomatosis were underestimated because early diagnosis was easily missed. It is important to adequately sample all uterine leiomyomas and carefully examine the soft tissue on either side of the lower uterine segment below the peritoneal reflection to identify early-stage intravenous leiomyomatosis.

摘要

静脉内平滑肌瘤病是一种罕见的平滑肌瘤变体,可能导致死亡。早期、准确的诊断和适当的治疗策略对良好的预后起着主导作用。从我们的数据库中检索到 18 例静脉内平滑肌瘤病病例,并结合临床病理数据进行分析。大多数患者年龄在 33 至 54 岁之间(中位数 44 岁),表现为盆腔肿块或异常子宫出血。通过平滑肌肌动蛋白、CD34 和 Ki67 的免疫组织化学染色来确认诊断。手术探查证实存在子宫肿块(平均大小 5.08cm)。在 5 例中发现了宽韧带内的虫样栓子。1 例肿瘤穿透下腔静脉。我们的病例中有 33.33%(6/18)存在组织学变体,分为细胞性静脉内平滑肌瘤病(3 例)、乳头状轮廓的静脉内平滑肌瘤病(1 例)和脂肪化生的静脉内平滑肌瘤病(2 例)。18 例占医院所有生殖系统平滑肌肿瘤病例的 0.097%。静脉内平滑肌瘤病与子宫平滑肌瘤、腺肌病、子宫平滑肌瘤和腺肌病的比例分别为 38.89%(7/18)、11.11%(2/18)和 27.78%(5/18)。16 例患者可获得随访信息,随访时间为 26 至 104 个月(平均 55 个月)。3 例(16.67%)年龄小于 40 岁的患者复发(33、34 和 37 岁)。我们建议没有生育要求的年轻患者行子宫切除术和单侧输卵管卵巢切除术。由于早期诊断容易被忽视,静脉内平滑肌瘤病的病例被低估了。充分采样所有子宫平滑肌瘤并仔细检查腹膜反射下方子宫下段两侧的软组织,以识别早期静脉内平滑肌瘤病,这一点非常重要。

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