Journal
INDIAN JOURNAL OF SURGICAL ONCOLOGY
Volume 12, Issue SUPPL 2, Pages 322-326Publisher
SPRINGER INDIA
DOI: 10.1007/s13193-021-01351-3
Keywords
Rhabdomyosarcoma; Embryonal rhabdomyosarcoma; Primary peritoneal rhabdomyosarcoma; Sarcomatosis; Cytoreductive surgery; HIPEC
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Pediatric primary peritoneal embryonal rhabdomyosarcoma is a rare entity with majority of cases being inadequately treated and having poor outcomes. However, in certain cases, aggressive treatment through cytoreductive surgery and hyperthermic intraperitoneal chemotherapy can lead to favorable outcomes.
Pediatric peritoneal sarcomatosis is an exceedingly rare entity with unknown incidence. Within these tumors, primary peritoneal rhabdomyosarcoma constitutes a small fraction. Majority of them are probably treated inadequately and have dismal outcomes. A favorable subset exists where aggressive treatment in the form of cytoreductive surgery supplemented by hyperthermic intraperitoneal chemotherapy in the multimodal setting can be attempted. We present a case of primary peritoneal embryonal rhabdomyosarcoma in a 2-year-old child who was treated with systemic chemotherapy, cytoreductive surgery, and hyperthermic intraperitoneal chemotherapy while avoiding radiation, with its evidence and rationale.
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