DOI: http://dx.doi.org/10.18203/issn.2454-5929.ijohns20194952

Out-of-the-box soft tissue tumor: pediatric synovial sarcoma of larynx

Albert Alhatem, Nicole Farber, Soly Baredes, Neena Mirani

Abstract


Synovial sarcoma (SS) is a very rare, aggressive, mesenchymal neoplasm that displays both spindle cell and epithelial histologic characteristics. To our knowledge, there are only 20 documented cases of primary laryngeal SS in the English language literature among all age groups and only three case reports among the pediatric population. Due to its rarity, recognition of this laryngeal neoplasm is often very difficult, and diagnosis relies heavily on immunohistochemistry and its unique translocation between chromosome X and 18 [t(X; 18) (p11.2; q11.2)]. The treatment of SS typically involves a multimodal approach with surgery, chemotherapy, and radiation but despite these options the prognosis remains poor.Here we report the fourth laryngeal SS pediatric patient along with a review of all SS published cases in children. The current report is not only presenting the youngest patient with laryngeal SS but also the pediatric patient with the longest survival.


Keywords


Synovial sarcoma, Larynx, Pediatric pathology, Translocation (X; 18)

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References


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