• David J Liu, Renee Perrier, Xing-Chang Wei, Jeffrey T Joseph, and Douglas Strother.
• MD Program, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada.
• Pediatr Blood Cancer. 2016 Dec 1; 63 (12): 2240-2242.

AbstractPleuropulmonary blastoma (PPB) is a rare childhood tumor, often associated with germline DICER1 mutations and a risk for development of other benign and malignant tumors, a constellation termed DICER1 syndrome. A 1-year-old male was diagnosed with Type I PPB and screened regularly thereafter for detection of intrathoracic and intraabdominal disease. Ten months after diagnosis of PPB, he presented with headaches and vomiting. He was diagnosed with atypical choroid plexus papilloma, a lesion not previously reported with PPB. The presence of central nervous system symptoms in patients with PPB or a phenotype suggestive of DICER1 syndrome should prompt early intracranial imaging.© 2016 Wiley Periodicals, Inc.

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