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Metachronous Type I pleuropulmonary blastoma and atypical choroid plexus papilloma in a young child
Author(s) -
Liu David J.,
Perrier Renee,
Wei XingChang,
Joseph Jeffrey T,
Strother Douglas
Publication year - 2016
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26160
Subject(s) - medicine , headaches , pathology , choroid plexus papilloma , choroid plexus , lesion , papilloma , vomiting , pulmonary blastoma , germline , central nervous system , lung , surgery , biochemistry , chemistry , gene
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.