Outcome of paediatric rhabdomyosarcoma attended in a tertiary care hospital, Dhaka, Bangladesh

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 no statist...

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Veröffentlicht in:Bangladesh Medical Research Council bulletin 2020-06, Vol.46 (1), p.17-21
Hauptverfasser: Rahman, ATM Atikur, Begum, Momena, Kibria, CSH, Akter, Mehnaz, Azad Siddiqua, Khurshida, Siddiqua, Farzana, Jamal, Choudhury Yakub, Shah, Salahuddin
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Sprache:eng
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Zusammenfassung: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 no statistical data on RMS in Bangladesh because no study has done in Bangladesh. Objective: To evaluate the clinical characteristics and treatment outcome of children with rhabdomyosarcoma among tertiary care. 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 was ratio 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 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 is 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 is may be due to advanced stage of 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 developed countries. Further multicentre studies are needed to report our experiences with rhabdomyosarcoma. Bangladesh Med Res Counc Bull 2020; 46(1): 17-21
ISSN:0377-9238
2224-7238
DOI:10.3329/bmrcb.v46i1.47464