Paratesticular rhabdomyosarcoma in young adults: A tertiary care institute experience

Abstract

Introduction: Paratesticular rhabdomyosarcoma (RMS) is a rare tumor arising from the mesenchymal tissues of the spermatic cord, epididymis, testis and testicular tunics. It represents only 7% of all patients entered in the Intergroup Rhabdomyosarcoma Study (IRS) and 17% of all malignant intrascrotal tumors in children less than 15 years old. We present our experience in combined modality management of 10 successive patients of paratesticular RMS. Material and Methods: We retrospectively reviewed 10 patients of paratesticular RMS treated in our institute from July 2004 to December 2010. Clinical characteristics and treatment modality in form of surgery and chemotherapy (CCT) were noted. Statistical analysis was done with regards to progression-free survival (PFS) and overall survival (OS) using Kaplan-Meier survival analysis. Results: The median age of the patients was 16.5 years. The median duration of symptoms was 5 months. Five patients had retroperitoneal lymphadenopathy (RPLAP) while three had lung metastases and one had orbital metastases. All patients underwent high inguinal orchidectomy followed by systemic chemotherapy (CCT). Retroperitoneal node dissection was not a required staging procedure. Four patients had partial response to treatment while six had complete response. Mean duration of PFS was 48 months and mean OS was 56 months. Conclusions: Paratesticular RMS are rare neoplasms with aggressive growth patterns. Cure rates have dramatically improved and 60% of patients in our series had complete response. This success is due to development of multimodality and risk adapted treatment approaches.