Isolated pediatric endobronchial primary anaplastic large cell lymphoma

Anaplastic large cell lymphoma (ALCL) is rare, accounting for 10–15% of all childhood non-Hodgkin's lymphomas. We present a case of primary isolated ALCL in the lung of a 5-year-old boy. An asymptomatic 5-year-old boy had absent breath sounds over his right hemithorax on routine physical exam. Chest X-ray showed complete white-out of the right hemithorax. Chest CT scan demonstrated a mass occluding the right mainstem bronchus. The first bronchoscopic biopsy was reported as an endobronchial neoplasm with an immunophenotype consistent with Ewing's sarcoma/PNET. One week later, a repeat second bronchoscopy with re-biopsy confirmed the correct diagnosis of ALCL, null phenotype. Clinical and radiological staging revealed no evidence of extrathoracic disease in the past, present, or for three-months after presentation, confirming isolated primary endobronchial ALCL. Complete remission at 6-months with polychemotherapy was achieved. Although a rare tumor of the lung, ALCL should be considered in the differential diagnosis of ‘unusual lung neoplasms’ in children.