Embryonal Rhabdomyosarcoma: A Tale of Two Cases with Unusual Presentation

Abstract

Rhabdomyosarcoma (RMS) is a rare soft tissue tumour, accounting for 3% of all childhood cancers. RMS can arise virtually anywhere in the body as it originates in embryonal mesenchyme. Embryonal Rhabdomyosarcoma (ERMS) and Alveolar Rhabdomyosarcoma (ARMS) are the most prevalent subtypes of RMS but arise through diverse biological mechanisms. RMS generally presents as an expanding mass that tends to be very painful and causes symptoms related to the compression of structures present nearby. Metastases of such tumours are common and can occur in bone, lungs and other organs leading to pain, difficulty with respiration, pleural effusion, anaemia, thrombocytopenia, and neutropenia. In this article, two unusual presentations of ERMS have been reported. First case is of a 28-year-old adult male who presented in surgical Out Patient Department (OPD) with recurrent episodes of painful acute urinary retention since one year. Another case was of a six-year-old girl presenting with aural fullness and serosanginous discharge from right ear, ear pain and decreased hearing since two years. Since five year survival rate of such tumour is less than 30%, therefore, an awareness of the typical signs and symptoms, radiological features, histomorphological features in a case of paediatric and adult ERMS can help a pathologist to consider this tumour in the differential diagnoses, even at unusual sites.