PRIMARY EXTRAOSSEOUS EWING SARCOMA OF THE LUNG: CASE REPORT AND REVIEW

MOCAYAR MARÓN FJ, OLIVA J, PELLICER M, CAYADO GUTIÉRREZ N, D´ANGELO CR

Villa de Merlo - San Luis

Congreso; XXXV Reunión Científica Anual de la Sociedad de Biología de Cuyo.; 2017

Sociedad de Biología de Cuyo

Ewing sarcoma, a branch of neuroectodermal tumor (PNET), is the second most common primary osseous malignancy in children and young adults. Most cases of Ewing?s sarcoma are reported in the bone, and extraosseous Ewing?s sarcoma is an extremely rare disease. Approximately 17 cases of lung affection have been reported so far (Deokar K. et al 2015). In this case report, we describe such a case in a 8-year-old female. The patient was admitted with fever, dry cough, decay and respiratory distress. She was initially treated with antibiotics under suspicion of pneumonia. Radiological investigations were performed including ultrasonography, computed tomogra¬phy and PET-SCAN and they revealed solid expansive lesion within the right hemitorax and pleural efussion. A biopsy was taken and histopatological features were analized. Immunohistochemical stainings for CD99 and synaptophysin were positive. Translocation t (11, 22) (q24; q12); wich gives rise to the formation of the EWS-FLI 1 fusion gene, was positively tested with RT-PCR. Thus, the histological, immunohistochemical, and molecular findings were compatible with Ewings sarcoma PNET. She was started on chemotherapy but unfortunately the patient died. In addition to the case report we expose a brief resume of the new literature about the molecular cell biology of this neoplasm.