Metachronous Type I pleuropulmonary blastoma and atypical choroid plexus papilloma in a young child. Issue 12 (21st July 2016)

• Record Type: Journal Article
• Authors: Liu, David J.; Perrier, Renee; Wei, Xing‐Chang; Joseph, Jeffrey T; Strother, Douglas
• Is Part Of: Pediatric blood & cancer. Volume 63:Issue 12(2016)
• Page Start: 2240
• Page End: 2242
• Publication Date: 2016-07-21
• Subjects: atypical choroid plexus papilloma -- brain tumor -- pleuropulmonary blastoma -- DICER1; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.26160
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: Pleuropulmonary blastoma (PPB) is a rare childhood tumor, often associated with germline DICER 1 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.