NASOPHARYNGEAL RHABDOMYOSARCOMA: A CASE REPORT

Dr. Sunita Singh (Senior Professor), Dr. Bharti Sharma (Junior Resident), Dr. Ritesh Kumar Sheorain* (Junior
Resident) and Dr. Rajeev Sen (Senior Professor and Head of the Department)

ABSTRACT

Introduction: Rhabdomyosarcoma is a soft tissue sarcoma arising from cells of myogenic lineage. Most common sites being extremities, perirectal and perineal region. It is a malignant tumor of soft tissue in children under 15 years of age, accounting 5% to 10% of all childhood malignancies. Head and neck region is frequently involved in childhood. For pediatric rhabdomyosarcoma cases, most of them have orbital primaries in the head and neck region, nasopharynx and nasal cavity cases are rarely seen. Case Report: An 11 years old boy presented with history of nasal mass, nasal obstruction and epistaxis. These symptoms started 4 to 5 months back and gradually progressed. The histological diagnosis of rhabdomyosarcoma was confirmed by immunohistochemistry method. We report this case because of its unique location in nasopharynx. Discussion: Rhabdomyosarcoma originates from immature mesenchymal cells that are committed to skeletal muscle differentiation. It is the most frequent soft tissue tumor in children. Histologically, it is distinguished in 3 types: embryonic, alveolar and pleomorphic. Embryonic type is mainly affect children less than 5 years of age. Pediatric rhabdomyosarcoma cases in head and neck region mainly have orbital primaries whereas nasopharynx and nasal cavity rhabdomyosarcoma cases are rarely seen. Only histology with immunohistochemistry provides specific diagnosis, in the form of diffuse desmin and myogenin staining. Conclusion: Rhabdomyosarcoma is a most common type of soft tissue sarcoma in children. It is localized in the head and neck region in 40% cases. Treatment of rhabdomyosarcoma is complex, including multi-drug chemotherapy, radiotherapy and surgery.

Keywords: Rhabdomyosarcoma, Nasopharynx, Myogenin.