Embryonal Rhabdomyosarcoma Of The Lung In A Child: Case Report

Introduction Rhabdomyosarcoma (RMS), tumor of skeletal muscle origin, is the second most common soft tissue sarcoma encountered in childhood after osteosarcoma. The common sites of occurrence are the head & neck region, genitourinary tract and retroperitoneum. The histogenesis of RMS is still unclear, but the most widely accepted hypothesis is that RMS arises due to the proliferation of embryonic mesenchymal tissue. RMS is a highly malignant tumor with extensive local invasions and early hemorrhagic and lymphatic dissemination. There is a slight predilection for disease in males . Despite aggressive approaches incorporating surgery, chemotherapy, and radiation therapy, the outcome for patients with metastatic disease remains poor, survival rate ranged from 20% to 35% in reported series. We report a case of lung rhabdomyosarcoma in a 3 year old. Case report A 3 yr old initially presented with history of increased work of breathing with reduced air entry on the left mid and lower zones, blood investigations showed raised inflammatory markers, Strep pneumoniae positive, Chest X-ray suggestive of extensive consolidation.The child was treated with a 5-day course of intravenous antibiotics with clinical improvement however the X-ray showed persistent consolidation. Re-presented a month later with worsening cough. Repeat chest X-ray showed large consolidation, collapse/effusion with pockets of fluid, case discussed with tertiary centre as per advice given a further course of antibiotics. The child represented 2 weeks later with similar complaints ,ultrasound suggested massive semisolid lesion/collection in the left hemithorax. Had a CT chest which suggested massive pleural collection on the left side. Surgical opinion -advised to insert video assisted thoracic drain, intraoperatively copious amount of bloody pleural fluid drained, the child became unstable needing volume resuscitation and subsequent transfer to PICU.The pleural fluid analysis was suggestive of neoplasm, had further imaging which suggested growth in the left lung, underwent resection of mass. Histology of mass suggested rhabdomyosarcoma of embryonal type. The child was commenced on chemotherapy. Conclusion We conclude that the presence of rare conditions, such as tumors of the lung, should be kept in mind in the differential diagnosis of a child who presents with an abnormal shadow on a radiograph, so that early surgical intervention can be undertaken, thus preventing long-term morbidity and mortality from the advanced stage of the disease. Our report is in agreement with the view that patients with pulmonary rhabdomyosarcoma without associated cystic lesion present late in the course of disease, which normally results in poor prognosis.