Pleuropulmonary Blastoma: More Than a Lung Neoplasm of Childhood

Louis P. Dehner, Kris Ann Schultz, D. Ashley Hill

Research output: Contribution to journalArticlepeer-review

26 Scopus citations

Abstract

Pleuropulmonary blastoma (PPB), the most common primary malignant neoplasm of the lung in childhood, occurs in the same early age group (0-6 years) as the other more common solid tumors such as neuroblastoma and Wilms tumor. The tumor begins as a cystic lung lesion with the potential over a period of 3-5 years to progress to a high grade multipatterned primitive sarcoma in the absence of a malignant epithelial component. Several years after its initial description as a unique clinicopathologic entity, this and other tumors appeared to have a familial predilection which was later confirmed with the discovery of a heterozygous germline mutation in DICER1 whose protein is a member of ribonuclease III family of enzymes. It is estimated that 75%-80% of children with a PPB have the germline mutation. The other notable finding from our studies is the identification of a family of extrapulmonary neoplasms, including cystic nephroma and Sertoli-Leydig cell tumor of the ovary as two examples, also with DICER1 mutations.

Original languageEnglish
Pages (from-to)206-210
Number of pages5
JournalMissouri Medicine
Volume116
Issue number3
StatePublished - May 1 2019

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