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宫颈肺泡状横纹肌肉瘤:一例报告。

Alveolar rhabdomyosarcoma of cervix: A case report.

作者信息

Zhang Xiuli, Xu Junfen

机构信息

Department of Gynecologic Oncology, Women's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.

出版信息

Medicine (Baltimore). 2025 Aug 1;104(31):e43675. doi: 10.1097/MD.0000000000043675.

Abstract

RATIONALE

Alveolar rhabdomyosarcoma (ARMS) is a rare and highly aggressive malignant soft tissue tumor. ARMS is associated with a poor prognosis, especially in adults, occurring in the uterine cervix infrequently. To date, only 6 cases have been reported in the literature. We discuss the challenges in diagnosing and managing cervical ARMS and highlights the need for ongoing research into optimal treatment strategies for this malignancy.

PATIENT CONCERNS

A 51-year-old female was diagnosed with advanced-stage ARMS. Positron emission tomography and computed tomography scans indicated a high metabolism mass in the uterus region, along with multiple lymph nodes enlargement in the pelvic, para-aorta and mediastinal regions. Cervical biopsy and segmental curettage revealed a small cell malignant tumor with poor differentiation. Histological and immunohistochemical examination confirmed ARMS.

DIAGNOSES

The final diagnosis was cervical ARMS, staged IV according to the Intergroup Rhabdomyosarcoma Studies Group based on the imaging and histology results.

INTERVENTIONS

The patient underwent radical hysterectomy, bilateral salpingo-oophorectomy, pelvic lymphadenectomy, and para-aortic lymphadenectomy. Postoperatively, the patient received a series of chemotherapy regimens including VAC† (vincristine, epirubicin, cyclophosphamide), EP (etoposide and cisplatin), VIP (etoposide, ifosfamide, cisplatin), gemcitabine and bevacizumab, gemcitabine and docetaxel, pertuzumab and lenvatinib and radiotherapy.

CONCLUSION

ARMS has low incidence with unique clinical pathological characteristics. The biological behavior is more aggressive and the prognosis is worse in ARMS. Further research is necessary to refine treatment protocols and improve survival rates for this aggressive tumor.

摘要

理论依据

肺泡状横纹肌肉瘤(ARMS)是一种罕见且具有高度侵袭性的恶性软组织肿瘤。ARMS预后较差,尤其是在成人中,在子宫颈发生的情况较少见。迄今为止,文献中仅报道了6例。我们讨论了诊断和管理宫颈ARMS的挑战,并强调了对这种恶性肿瘤最佳治疗策略进行持续研究的必要性。

患者情况

一名51岁女性被诊断为晚期ARMS。正电子发射断层扫描和计算机断层扫描显示子宫区域有一个高代谢肿块,同时盆腔、腹主动脉旁和纵隔区域有多个淋巴结肿大。宫颈活检和分段刮宫显示为低分化小细胞恶性肿瘤。组织学和免疫组织化学检查确诊为ARMS。

诊断

根据横纹肌肉瘤研究组的标准,结合影像学和组织学结果,最终诊断为宫颈ARMS,分期为IV期。

干预措施

患者接受了根治性子宫切除术、双侧输卵管卵巢切除术、盆腔淋巴结清扫术和腹主动脉旁淋巴结清扫术。术后,患者接受了一系列化疗方案,包括VAC†(长春新碱、表柔比星、环磷酰胺)、EP(依托泊苷和顺铂)、VIP(依托泊苷、异环磷酰胺、顺铂)、吉西他滨和贝伐单抗、吉西他滨和多西他赛、帕妥珠单抗和乐伐替尼以及放疗。

结论

ARMS发病率低,具有独特的临床病理特征。ARMS的生物学行为更具侵袭性,预后更差。有必要进一步研究以完善治疗方案并提高这种侵袭性肿瘤的生存率。

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