Fig. 2: Pleuropulmonary blastoma, type I presenting as a cystic lesion in the lung of a 1-year-old female.
From: DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma
A Septal markings and cysts are appreciated in this unbisected specimen whose multicystic structure is lost with bisection as the delicate septa collapse. B The peripheral multicystic lesion is composed of uniform septal structures abutting the pleural surface, a characteristic feature. C Uniformly expanded septa by primitive-appearing small, rounded and spindled-shaped cells, many showing desmin and myogenin positivity (not shown). D Cambium layer-like concentration of primitive small cells with adjacent fibrous stroma.
