April Case of the Month Clinical History: A 1 year old female presented with increasing respiratory distress and hemoptysis. The baby was born full term in an uncomplicated vaginal delivery. A CT scan demonstrated a 6.8 x 5.5 x 4.4 cm cystic lesion in right lower lobe. A right lower lobe lobectomy was completed, and grossly multiple cysts, measuring up to 7x5x5 cm (Fig 1) were identified. No nodules, masses or pleural thickening are seen. Histologic sections show multiple irregularly shaped cysts with branching papillae and lined by pseudostratified columnar epithelium. (Fig 2). The wall of the cyst shows bundles of smooth muscle fibers scattered mononuclear cells (lymphocytes and few plasma cells) and few lymphoid aggregates. (Fig 3, 4) The adjacent lung parenchyma shows focal areas of collapse (Fig 6) and emphysematous changes. (Fig 5). No mucinous cell proliferations or clusters are identified. There is no evidence of blastemal component or dysplasia or malignancy. No abnormal systemic arterial supply was identified.

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