成人罕见会阴部胚胎性横纹肌肉瘤：一例报告。
An uncommon perineal embryonal rhabdomyosarcoma in adult: A case report.
机构信息
Medical Integration and Practice Center, Cheeloo College of Medicine, Shandong University, Jinan, China.
Department of Urology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China.
出版信息
Medicine (Baltimore). 2022 Dec 30;101(52):e32529. doi: 10.1097/MD.0000000000032529.
RATIONALE
Embryonal rhabdomyosarcoma (ERMS) is a major subtype of rhabdomyosarcoma, mainly affect children. There is seldom report for perineal ERMS in adults, since its rare location and the age.
PATIENT CONCERNS
A 20-year old male adult was admitted due to the perineal mass.
DIAGNOSES
Diagnosis by histopathological examination of the biopsy sample was ERMS. Magnetic resonance imaging showed the tumor was found in the perineal region, with metastasis to pelvic cavity, right testis, lymph nodes and bone.
INTERVENTIONS
The patient received Isophosphamide and Epirubicin for 4 cycles, followed by Irinotecan and Vindesine Sulfate for 2 cycles, then cisplatin, Dacarbazine and Apatinib for 3 cycles.
OUTCOME
The patient showed no response to chemotherapy.
LESSONS
Perineal ERMS in adults is very rare. There is still no standard therapy for adult ERMS. Personalized therapy might be promising treatment for each individual.
背景
胚胎性横纹肌肉瘤（ERMS）是横纹肌肉瘤的主要亚型，主要影响儿童。由于其罕见的位置和年龄，成人会阴部 ERMS 很少见有报道。
病例报告
一名 20 岁男性因会阴部肿块入院。
诊断
活检样本的组织病理学检查诊断为 ERMS。磁共振成像显示肿瘤位于会阴部，已转移至盆腔、右侧睾丸、淋巴结和骨骼。
干预措施
患者接受了异环磷酰胺和表柔比星 4 个周期治疗，随后伊立替康和硫酸长春地辛 2 个周期治疗，接着顺铂、达卡巴嗪和阿帕替尼 3 个周期治疗。
结果
患者对化疗无反应。
教训
成人会阴部 ERMS 非常罕见。目前尚无成人 ERMS 的标准治疗方法。个体化治疗可能是每个患者有希望的治疗方法。
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