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Pediatric Rhabdomyosarcoma in India: A Single-center Experience.

Indian Pediatrics 2017 September 16
OBJECTIVE: Analyze the profile and outcome of children with rhabdomyosarcoma from a pediatric-oncology unit.

DESIGN: Retrospective analysis of case records over 23 years (1990-2012).

Setting: Government-run, tertiary-care, university hospital in Northern India.

PARTICIPANTS: 159 children (<12-years) with a diagnosis of rhabdomyosarcoma were enrolled. The median age was 4 years; 13% were infants.

MAIN OUTCOME MEASURE: Five-year event free survival.

RESULTS: The median symptom interval was 2-months. Head and neck region was the most frequent site (44%), followed by tumors in the extremity (15.7%). The majority (67%) of the tumors were located at 'unfavorable' sites; 68% were >5 cm in size. The most frequent (58%) pathological subtype was embryonal. Treatment was based on the 'Intergroup Rhabdomyosarcoma Study (IRS) Group' risk-stratification. 33% were 'low-risk' children, 11% were 'high-risk'. Treatment-refusal (18%) and abandonment (33%) were major impediments. The median ± SE five-year event free survival of those taking treatment was 43.6 ± 6%.

Conclusion: Large sized tumors, tumors at unfavorable locations, and treatment refusal/abandonment contributed to inferior outcome in children with rhabdomyosarcoma.

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