A RARE MALIGNANCY

Abstract

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Ewing's sarcoma is a malignant neoplasm which arises from the skeletal bones. It most commonly affects children and young adults with a female preponderance. Ewing’s sarcoma of the rib is a rare enity as long bones are usually affected . CASE PRESENTATION: A 24 year-old male presented with complaints of shortness of breath , cough and left chest pain for 1 month. He also had episodes of seizures since last 3 years. He had multiple soft cutaneous nodules and macular patches over chest , back and upper limbs. There was bulging of left anterior chest wall with shifting of trachea as well as apex beat to right. There was increased vocal fremitus as well dull percussion note on left. There were reduced breath sounds with increased vocal resonance on left. Routine blood investigations were within normal limits. Chest X -ray revealed a homogeneously white left hemithorax with shifting of mediastinum to the right. Pleural fluid cytology had features suggestive of small round cell tumor. CECT thorax revealed a large heterogeneous mass in the left hemithorax with shifting of mediastinum to right and lytic destruction of left 6 th rib. There was minimal left pleural effusion with nodular pleural deposits. USG guided trucut biopsy from the left lung mass revealed small cell neuroendocrine carcinoma. The tumour was CD 99, Synaptophysin, CD 56 , FLI-1 - positive and negative for CK, CK7, TTF-1 and chromogranin. Dermatology opinion favoured the skin nodules to be neurofibromatosis. So a final diagnosis of Ewing’s sarcoma left sixth rib with Neurofibromatosis and seizure disorder was made. The patient received Vincristine , Adriamycine and Cyclophosphamide (VAC),alternating with Ifosfamide and Etoposide as well as was planned for radiotherapy DISCUSSION: Ewing sarcoma is an uncommon disease, which usually arises from the skeletal bones. It constitutes approximately 10 % of all primary malignant bone tumours. Only 40 % of such cases are seen in flat bones like innominate, ribs, scapula and sterum #1.A characteristic chromosomal translocation, (11; 22) (q24; q12) can be detected in 90% of cases .Patients with Ewing's Sarcoma of the ribs present with breathlessness, weight loss and painful chest wall mass. #2 pleural effusion as in our case, is an unusual finding #3 . The tumour has propensity to spread inwards towards the thoracic cavity and thus,may manifest as an extra pleural mass.our case unique in a wayas it had several other associated finding like neurofibromatosis,seizure disorder as well as skin lesion although such association are not commonly reported CONCLUSIONS: This case showcases the significance of IHC an important tool in diagnosis of lung malignancies and lays stress on excluding extrathoracic involvement in all such cases. Reference #1: MirraJM. Bone tumors: clinical, radiologic, and pathologic correlations. Philadelphia: Lea&Febiger, 1989; 1087-1117. Reference #2: Gladish GW, Sabloff BM, Munden RF, Truong MT, Erasmus JJ, Chasen MH. Primary thoracic sarcomas. Radiographics 2002 May-Jun;22(3):621-637 Reference #3: Wolf G, Aigner RM, Schwarz T. Massive pleural effusion with dyspnea in a 17-year-old boy as the first sign of ewing sarcoma. J Pediatr Hematol Oncol 2002 Jun-Jul;24(5):420 DISCLOSURES: No relevant relationships by UMER AHMED SYED, source=Web Response