Ewing Sarcoma of Larynx: A Rare Case in a 5-Year-Old Boy

Larynx cancer diagnosed as Ewing Sarcoma in a 41-year-old man: A case report

“…So far, 5 cases of EES in the larynx were reported at advanced ages (between 33 and 74 years) [ [5] , [6] , [7] , [8] , [9] ] similar to our case, in contrast to classic Ewing sarcoma which is more common in younger ages [ 12 ]. Furthermore, 3 cases were reported at far younger ages (newborn, 9 months, 5 years) [ 4 , 10 , 11 ].…”

“…Ewing sarcoma and Primitive Neuroectodermal Tumor (PNET) are regarded as a single category called “PNET/Ewing Sarcoma” from a current perspective since they share similar chromosomal translocations especially t(11:22) (q24:q12), practical diagnosis, and immunohistochemical characteristics of CD99 expression, as well as therapeutic strategies [ 18 ]. This perspective was adopted in several reports that made the diagnosis of PNET/Ewing Sarcoma based on histological findings and CD99 marker besides other markers to exclude other differential diagnoses, and ours was one of them [ 4 , 5 , 8 ]. The opposing perspective tries to separate them into different entities depending on neural differentiation and markers exhibited by PNET (S-100 protein, vimentin ..) [ 6 , 18 ].…”

“…A few reports used microbiology of these tumors that is characterized by a reciprocal translocation leading to the formation of hybrid genes with the EWSR1-FTI1 being the most common one and the gold standard for diagnosis using Fluorescence in situ hybridization (FISH) [ 4 , 7 , 8 , 18 ].…”

“…Although a solid conclusion on the treatment of Ewing Sarcoma in the larynx remains to be established due to the small number of cases in medical literature, some reports mentioned applying surgical treatment with/without adjuvant chemo-radiotherapy [ 4 , 9 ] while others applied chemo-radiotherapy without surgery depending on the case [ [6] , [7] , [8] ]. Larger studies investigated the favorable treatment of Ewing sarcoma outside the bone and in the head and neck region [ 19 , 20 ].…”

“…Ewing sarcoma, which is a bony sarcoma, is even rarer as a subtyped laryngeal sarcoma and to the best of our knowledge, only a small number of cases of this rare presentation were previously reported [ [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] ]. Hence, since laryngeal cancer rarely manifests as Ewing sarcoma which itself rarely manifests outside the skeleton, we present an extremely rare case of Ewing sarcoma of the larynx.…”