Conservative management of rhabdomyosarcoma of uterine cervix: A case series

S. Mathews, P. Veena, Nithiyaanandan Natarajan
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Abstract

Background: Rhabdomyosarcoma (RMS) of the uterine cervix is a rare disease, usually affects pediatric and adolescent girls. Excellent treatment outcomes have been reported with combined modality treatment employing surgery, chemotherapy, and radiation therapy. Fertility-sparing treatment options can be considered in young patients without compromising outcome. Aim: This study aimed to analyze the outcome of patients with cervical RMS who were managed conservatively. Materials and Methods: We retrospectively reviewed the clinical and pathologic data of all patients with cervical RMS who were registered at our institution during 1995–2010. Long-term outcome data of patients who had conservative surgery were analyzed. Results: During this period, six patients with cervical RMS were registered. Five patients, managed conservatively, were eligible for analysis. The median age was 20 years (range: 14–21 years). Excessive vaginal discharge was the most common presenting symptom (n = 3). All patients initially had either a polypectomy (n = 4) or gross tumor resection (n = 1) followed by received chemotherapy with VAC regimen (Vincristine, Actinomycin-D and Cyclophosphamide). One patient received local radiation (HDR brachytherapy) for microscopic residual disease. All patients were disease-free at the completion of primary therapy. At a median follow-up of 10 years, four patients remain relapse-free; three, having retained fertility, had successful pregnancies and healthy children. One patient developed ovarian failure consequent to radiation treatment but remains disease free. One patient developed local recurrence, 8 years after primary treatment, and underwent salvage hysterectomy followed by second-line chemotherapy. This patient too remains disease-free, 3 years after salvage treatment. Conclusion: RMS of the uterine cervix is highly curable in early stages. The focus now is on improving the quality of life by reducing treatment-related morbidity and late effects. Fertility preservation approaches should be considered in young females with RMS of cervix.
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子宫颈横纹肌肉瘤的保守治疗:一个病例系列
背景:子宫颈横纹肌肉瘤(Rhabdomyosarcoma, RMS)是一种罕见的疾病,通常发生在儿童和少女。采用手术、化疗和放射治疗的联合治疗方法取得了良好的治疗效果。在不影响预后的情况下,年轻患者可以考虑保留生育能力的治疗方案。目的:本研究旨在分析保守治疗的宫颈RMS患者的预后。材料和方法:我们回顾性地回顾了1995-2010年间在我院登记的所有颈椎RMS患者的临床和病理资料。分析保守手术患者的长期预后数据。结果:在此期间,登记了6例颈椎RMS患者。5例保守治疗的患者符合分析条件。中位年龄为20岁(范围:14-21岁)。阴道分泌物过多是最常见的症状(n = 3)。所有患者最初均行息肉切除术(n = 4)或肿瘤切除术(n = 1),随后接受VAC方案(长春新碱、放线菌素- d和环磷酰胺)化疗。1例患者接受局部放射(HDR近距离放疗)治疗显微残留病变。所有患者在初始治疗结束时均无疾病。中位随访期为10年,4例患者无复发;其中三名妇女保持生育能力,怀孕成功,子女健康。一名患者因放射治疗而出现卵巢功能衰竭,但仍无疾病。1例患者在初次治疗后8年出现局部复发,行补救性子宫切除术并行二线化疗。这名患者在抢救治疗3年后仍然无病。结论:宫颈RMS早期治愈率高。现在的重点是通过减少与治疗有关的发病率和晚期影响来改善生活质量。年轻女性宫颈RMS患者应考虑保留生育能力的方法。
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