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复发性阴道上皮样平滑肌肉瘤;来自博茨瓦纳的一例病例报告及文献复习。

Recurrent vaginal epithelioid leiomyosarcoma; a case report from Botswana and review of the literature.

机构信息

University of Botswana, Faculty of Medicine, Department of Obstetrics and Gynecology.

University of Botswana Faculty of Medicine, Department of Pathology.

出版信息

Afr J Reprod Health. 2021 Feb;25(1):161-168. doi: 10.29063/ajrh2021/v25i1.17.

Abstract

Primary vaginal leiomyosarcoma is a rare gynecological malignancy. The clinical presentation is a benign looking well circumscribed mobile mass which might however occasionally present with distant metastases. Post treatment recurrence is common, and the clinical course is often unpredictable. Primary surgical management plus radiotherapy is the commonly practiced treatment of choice. We report a case of primary vaginal epithelioid leiomyosarcoma that recurred twice after local surgical resection. The patient subsequently underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy for a recurrent vaginal leiomyosarcoma. Histopathology of the vaginal mass revealed an epithelioid leiomyosarcoma of the vagina. She also received a course of adjuvant radiotherapy. The patient was free of recurrence at 3 year follow up. Vaginal mass must be evaluated with a high index of suspicion for malignancy. Local surgical resection alone is insufficient for primary vaginal leiomyosarcoma. Surgical resection with adjuvant radiotherapy offers better treatment outcomes with decreased risk of recurrence. Empirical oophorectomy in patients with completed family size might confer additional benefit in preventing disease recurrence in resource limited settings where testing for estrogen and progesterone receptor status of the tumour is unavailable. Due to the unpredictable course of the disease, lifelong patient follow-up is critical for better outcomes.

摘要

原发性阴道平滑肌肉瘤是一种罕见的妇科恶性肿瘤。临床表现为良性、边界清楚、活动良好的肿块,但偶尔也会出现远处转移。治疗后复发很常见,临床病程往往不可预测。原发性手术治疗加放疗是目前常用的治疗选择。我们报告了一例原发性阴道上皮样平滑肌肉瘤,局部手术切除后复发两次。患者随后因阴道平滑肌肉瘤复发而行全子宫切除术加双侧输卵管卵巢切除术。阴道肿块的组织病理学显示为阴道上皮样平滑肌肉瘤。她还接受了辅助放疗。患者在 3 年随访时无复发。阴道肿块必须高度怀疑恶性肿瘤。单纯局部手术切除不足以治疗原发性阴道平滑肌肉瘤。手术切除加辅助放疗可降低复发风险,获得更好的治疗效果。对于已完成生育的患者,行经验性卵巢切除术可能在资源有限的情况下(无法检测肿瘤的雌激素和孕激素受体状态)额外有助于预防疾病复发。由于疾病的不可预测病程,对患者进行终身随访对于获得更好的治疗效果至关重要。

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