Metastatic Extra-Testicular Embryonal Rhabdomyosarcoma in a 19year old boy- 6 years after; A Case Report.

Abstract

Introduction: Embryonal Rrhabdomyosarcoma is a rare and aggressive form of malignancy arising from connective tissues histologically resembling the developing skeletal muscles of an embryo. It is commoner in children less than 18years and embryonal Rhabdomyosarcoma primarily from the testes are rare. Adult embryonal Rhabdomyosarcoma are not thoroughly studied with no particular prognostic indices or proper guideline of management as oppose those in children, our study shows a 19year old with metastatic embryonal Rhabdomyosarcoma of the testes, intergroup Rhabdomyosarcoma study group (IRS) grouping system group IV who had radical inguinal orchiectomy and adjuvant chemotherapy with complete remission 6years post-operative. Case report: A 19year old boy who presents with 6month history of slowly progressively growing, painless right scrotal mass. Ultrasound scan shows a right lone liver deposit, chest x-ray shows no evidence of metastatic deposit. The alpha fetoprotein was markedly elevated 230ng/ml but free beta human chorionic gonadotrophin (HCG) was within normal range at 1.6Miu/L. Patient had radical inguinal orchiectomy (histology shows para-testicular tumor with haphazardly arranged primitive cells, strap cells and bizarre 'tadpole' cells, some with intensely eosinophilic cytoplasm dispersed in myxoid stroma, the cells display angiocentrism) and adjuvant chemotherapy with complete disappearance of the liver deposit; he has been on follow up for 6years now on yearly alpha fetoprotein level check, doing well with no any evidence of reoccurrence of disease. Conclusion: Radical inguinal orchiectomy and adjuvant chemotherapy is a hopeful terrain to explore in the management of, even, metastatic embryonal Rhabdomyosarcoma of the testes.