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S3-8
(CP)
URACHAL RHABDOMYOSARCOMA: TWO CASE REPORTS
Mark, Hock Sheng LIM
1
, Chan Hon CHUI
2
and Kannan Laksmi NARASIMHAN
1
1) KK Women's and Children's Hospital, Paediatric Surgery, Singapore, SINGAPORE - 2) Mount Elizabeth Medical Centre,
Paediatric Surgery, Singapore, SINGAPORE
PURPOSE
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and is thought to arise from striated
muscle progenitor cells. The management of RMS involves surgical resection and subsequent multiagent chemotherapy
with or without radiotherapy, based on the Intergroup Rhabdomyosarcoma Study staging system group, and is well
studied. However, urachal RMS is rare and few case reports on urachal RMS have been described.Hence, we seek to
augment these numbers with two cases from our own experience as well as to review current literature.
MATERIAL AND METHODS
We describe the management of two cases of urachal rhabdomyosarcomas from 2004 to present, from the larger of two
tertiary paediatric surgical centres covering a population of 5.5 million. A review of surgical case logs was performed
covering the time period and patient case notes were retrieved for review. A definitive diagnosis of urachal
rhabdomyosarcomas was made based on (1) histological confirmation, (2) anatomical site arising from the urachus as
observed during surgical resection, and (3) absence of another primary lesion.
RESULTS
Our patients were diagnosed at 15 and 46 months and both underwent surgical resection and adjuvant chemotherapy
without radiotherapy. They have been recurrence free for 37 and 99 months for IRS group IV and IIb respectively. We
postulate a better prognosis for urachal rhabdomyosarcomas compared with other pelvic rhabdomyosarcomas, being
more ammenable to complete resection without sacrificing organ function.
CONCLUSIONS
Urachal rhabdomyosarcoma is rare and has unique characteristics that set it apart from other pelvic
rhabdomyosarcomas. As few cases are reported and with long term data lacking, a dedicated long term study of urachal
rhabdomyosarcomas woud be beneficial.