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子宫多形性横纹肌肉瘤——病例报告及文献系统综述

Pleomorphic Rhabdomyosarcoma of the Uterus - Case Report and a Systematic Review of the Literature.

作者信息

Alavi Sara, Eckes Lena, Kratschell Robert, Taube Eliane Tabea, Rosseck Friederike, Wieners Gero, Sehouli Jalid

机构信息

Department of Gynecology with Center for Oncological Surgery, European Competence Center for Ovarian Cancer, Charité - Campus Virchow-Klinikum, University Medicine of Berlin, Berlin, Germany.

Department of Pathology, Charité - Campus Virchow-Klinikum, University Medicine of Berlin, Berlin, Germany.

出版信息

Anticancer Res. 2017 May;37(5):2509-2514. doi: 10.21873/anticanres.11592.

Abstract

BACKGROUND

Pleomorphic rhabdomyosarcomas of the uterus, mainly occurring in postmenopausal women with leading symptoms of vaginal bleeding and abdominal pain, are very rare malignant tumors of the female genital tract. Due to the inefficiency of the adjuvant therapy, the outcome remains poor in the majority of the reported cases.

PATIENT AND METHODS

We present a case of a 73-year-old patient diagnosed with pleomorphic rhabdomyosarcoma of the uterus. Together with the case report, a systematic review of the literature is presented focusing on different treatment strategies and their outcome. The 95% confidence interval (CI) of the overall mean survival and the respective mean survival of each different treatment strategy was calculated using SAS Studio.

RESULTS

In the presented case, the patient showed no symptoms and was admitted into hospital due to abnormal uterine findings during a routine gynecological examination. Vaginal ultrasound scans showed a severely enlarged and intracavitaryly filled uterus. The patient underwent hysterectomy, as well as bilateral salpingo-oophorectomy. Regarding the systematic review of the literature, patients with adjuvant chemotherapy show the best outcome with a mean survival rate of 15.8±7.3 months (one patient excluded), whereas with a mean survival rate of 4.1±5.2 months, patients with sole surgical treatment show the shortest survival after diagnosis.

CONCLUSION

Although there is no standardized approach in the treatment of this rare disease, we present a differentiated overview.

摘要

背景

子宫多形性横纹肌肉瘤主要发生于绝经后女性,主要症状为阴道出血和腹痛,是女性生殖道非常罕见的恶性肿瘤。由于辅助治疗效果不佳,在大多数报道的病例中,预后仍然很差。

患者与方法

我们报告一例73岁诊断为子宫多形性横纹肌肉瘤的患者。结合病例报告,对文献进行系统综述,重点关注不同的治疗策略及其结果。使用SAS Studio计算总体平均生存的95%置信区间(CI)以及每种不同治疗策略的各自平均生存时间。

结果

在本病例中,患者无症状,因常规妇科检查时子宫异常而入院。阴道超声扫描显示子宫严重增大且宫腔内充满物质。患者接受了子宫切除术及双侧输卵管卵巢切除术。关于文献的系统综述,接受辅助化疗的患者预后最佳,平均生存率为15.8±7.3个月(排除1例患者),而仅接受手术治疗的患者诊断后生存时间最短,平均生存率为4.1±5.2个月。

结论

尽管在这种罕见疾病的治疗中没有标准化方法,但我们给出了一个有区别的概述。

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