Clinical characteristics and outcome of rhabdomyosarcoma in South African children

Abstract

AIM To review a series of 33 children, treated for rhabdomyosarcoma at Tygerberg Children's Hospital between 1983-2005, with emphasis on age, gender, clinical presentation, site, histological subtype, TNM stage, treatment modalities and survival.

METHOD A retrospective study based on data obtained from the Tygerberg tumour registry.

RESULTS The age at diagnosis ranged from 7 days to 13 years; males 57% and females 42%. More than half of cases presented with a mass at varying sites. Almost a quarter had pressure symptoms due to bladder tumours. The predominant complaint in 9% was pain. The primary site of disease was the head and neck in 45% and the pelvis in 42%. The most common histological subtype was in 45% embryonal rhabdomyosarcoma. At the time of diagnosis, 63% of patients were TNM stage 3.  Most patients received multimodality therapy (radiotherapy, chemotherapy and surgery). The survival rate from diagnosis to subsequent demise was poor - between 13 days and 3.5 years. The overall 5-year survival rate was 45%, which is partially explained by delay in diagnosis and advanced stage of disease

CONCLUSION  The clinical characteristics of rhabdomyosarcoma at Tygerberg Children's  Hospital are similar to those of first world countries. The majority of presentations were in the advanced stages of disease but sensitive to multimodal therapy. Recognition of early warning signs and public awareness could lead to earlier presentations and improve outcome. Further multicentre studies are needed in Africa to better report our experiences with rhabdomyosarcoma.