Pleuropulmonary blastoma in an adult patient: Report of a case

Abstract

Pleuropulmonary blastoma (PPB) typically presents in young children, most younger than 5 years, as dysontogenetic neoplasm. It has poor prognosis with three different subtypes: cystic (type I), combined cystic and solid (type II) and solid (type III). Our patient was admitted with dyspnea and was diagnosed at 25 years of age. He had upper right lobectomy and diagnosed as Type II PPB followed by alternating IE/VAC (Ifosfamide, etoposide/vincristine, dactinomycin, cyclophosphamide) polychemotherapy. Immunohistochemical studies showed vimentin positive while cytogenetic studies showed absence of trisomies 8 and 2 abnormalities. Our patient is presently alive and disease-free thirty months after diagnosis. It is not possible to obtain definite knowledge about the prognostic factors and survival rates in adult type PPB. Complete resection is proposed to be one of the important prognostic factors in literature. Longer follow up results in larger patient groups are required for obtaining definitive data.