Paediatric urachal rhabdomyosarcoma: the role of radiotherapy about a case report and review.

Abstract

Introduction: This report discusses the case of a 10-year-old girl diagnosed with rhabdomyosarcoma (RMS) of the urachus, a rare form of soft tissue cancer in paediatric oncology. RMS, representing 3%-4% of paediatric cancers, arises from primitive muscle cells and requires a multidisciplinary treatment approach. The goal of this case is to enhance understanding of radiotherapy's role in treating RMS in children, particularly in rare sites like the urachus.

Case: The patient, with no significant medical history, presented with right lower abdominal pain and was found to have a large abdominal mass. Imaging revealed a massive necrotic tumour and pulmonary metastases. The tumour was initially deemed unresectable, and a biopsy confirmed RMS. Chemotherapy was initiated using the RMS 2005 protocol, which resulted in a 70% tumour reduction. Surgical resection was then performed, and the patient received radiotherapy targeting both the primary tumour and metastases. The treatment showed no significant side effects and follow-up for a year showed no signs of recurrence.

Discussion: RMS is a paediatric malignancy with poor survival rates in high-risk and recurrent cases. The urachal origin of RMS is extremely rare, with few cases reported in the literature. Management includes chemotherapy, surgery and radiotherapy. While no known tumour markers exist, associations with genetic conditions like neurofibromatosis and Li-Fraumeni syndrome have been observed. Treatment aims to cure the disease while minimizing morbidity, with surgery typically preceded by chemotherapy to reduce tumour size.

Conclusion: While RMS is the most common soft tissue tumour in children, urachal RMS remains rare. Treatment involves surgery and radiotherapy, but further research is needed to establish standardized treatment protocols for such tumours.