Pediatric laryngeal sarcoma: Systematic review and pooled analysis

BACKGROUND:Sarcomas are a rare, diverse tumor class of mesenchymal origin affecting all age groups. Survival after diagnosis is influenced by disease site. To date, there are no analyses evaluating treatment of pediatric sarcoma within the larynx specifically. METHODS:A structured literature review following PRISMA guidelines was preformed to identify case reports of pediatric (age 17 and younger) laryngeal sarcoma. RESULTS:Twenty-nine case reports documenting 37 pediatric patients diagnosed with sarcoma in the larynx were identified since 1980. The majority of patients were male (79.4%). The most common histological subtypes were rhabdomyosarcoma (69.4%) and synovial sarcoma (19.4%). The supraglottis was the most common site of disease (62.1%) among laryngeal subsites. Only two patients were known to have succumbed to their disease. Overall survival was not statistically impacted by primary site of tumor, treatment strategy, histology or gender. CONCLUSIONS:Soft tissue sarcoma is rarely found in the pediatric larynx. Patient and tumor characteristics studied were not shown to affect outcomes. Increased documenting of high-quality case reports is needed to advance understanding of this disease.