Outcome of paediatric rhabdomyosarcoma attended in a Tertiary Care Hospital in Dhaka

Abstract

Background: Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin, is the most common form of paediatric soft tissue sarcoma. It is the third most common solid tumor comprising 4.5% of all childhood cancer with an annual incidence of 4.5 cases per million children. There is so far no data on RMS in Bangladesh. Objective: To evaluate the clinical characteristics and treatment outcome of children with rhabdomyosarcoma among patients in a tertiary care hospital. Methods: A retrospective study carried out analysing the medical records of 39 patients with rhabdomyosarcoma from the BSMMU tumor registry. Medical records of children with rhabdomyosarcoma were reviewed over a period of 6 years from June 2011 to May 2017. Most patients received multimodality therapy (chemotherapy, surgery, radiotherapy). Results: The male female ratio was 1.2:1. More than half of cases presented with a mass at varying sites. The predominant complaint- 12.0% was pain. The primary site of disease was the head and neck in 46.0% followed by pelvis 40.0%. Pathologically, the most frequent histological subtype was in 46.0% embryonal rhabdomyosarcoma followed by alveolar rhabdomyosarcoma 23.0%, botryoid type 5.0%, spindle cell type 5.0% and unspecified 21.0%. At the time of diagnosis, 64.0% of patients were TNM stage 3, next frequency was stage IV 23.0%. The survival rate from diagnosis to subsequent demise was poor between 13 days and 3.5 years. The overall 5 year survival rate was 41.0%, which might be due to advanced stage of the disease. Conclusion: The majority of presentations were in the advanced stages of disease but sensitive to multimodality therapy. Poor survival rate is correlated with the advanced stage of the disease. The clinical characteristics of rhabdomyosarcoma at BSMMU Hospital are similar to worldwide countries, but not like the developed countries. Further multicentre studies are needed to compare the current experiences with rhabdomyosarcoma.