Primary epldidymul rhabdomyosarcoma; one ease report
- VernacularTitle:原发性附睾横纹肌肉瘤一例报告
- Author:
Shunliang YANG
;
Jinquan CAI
;
Zhixian WU
;
Jianming TAN
- Publication Type:Journal Article
- Keywords:
Rhabdomyosarcoma;
Epididymis;
Diagnosis;
Treatment
- From:
Chinese Journal of Urology
2008;(11):782-785
- CountryChina
- Language:Chinese
-
Abstract:
Objective To discuss the clinical features of primary epididymal rhabdomyosarcoma and review the diagnostic and therapeutic advances. Methods One 16-yesr-old male presented with right scro-turn swelling mass for 4 months. Physical examination revealed an ovoid and solid mass of 4.5×3.5×3.0 cn in right scrotum. The mass was hard and its surface was smooth without tenderness. Transillumination test was negative. There was no palpable inguinal lymph node. Pre-operative diagnosis was inflammatory nodule of right epididymis. Results Right epididymectomy was performed under caudal anesthesia une-ventfully. The pathological study showed that the neoplasm was composed of small round shape ceils with small and anachromasis nucleus. The immunohistochemical study showed that these cells were positive for myoglobin, aetin and desmin, but negative for smooth muscle actin. Electron microscopy showed that paral-leling thin filaments were found with no asrcomere-like structure in the intracytoplasm. The pathologic diag-nosis was right epydidymal rhabdomyosarcoma. The patient refused further therapy. A mass was found with rapid growth accompanied with pain in the right scrotum 2 months after the first operation and was clinically diagnosed as tumor recurrence. This patient had then undergone right radical orchiectomy. Frozen section analysis revealed negative surgical margin. The pathologic diagnosis was embryonal rhabdomyosarcoma re-currence. Patient underwent subsequent chemotherapy with ifosfamide, etoposide and vincristine. After one year follow-up, he was generally well without evidence of recurrence. Conculsions Primary embryonal rhabdomyosarcoma is a rare tumor with aggressive growth patterns. The diagnosis depends on the pathologi-cal examination because the clinical presentation is not specific. Combination therapy with radical orchiecto-my, chemotherapy and radiotherapy provides patients an excellent long-term prognosis.