A Pleuropulmonary Blastoma: A Rare Malignant Lung Disease in Children pleuropulmonary blastoma

Abstract

Pleuropulmonary blastoma (PPB) is Avery uncommon, mesenchymal malignant tumor in chest cavity that occurs mostly in children before preschool age (less than 6year old) . while it is the most popular primary pleural and lung tumor in children and adolescent malignancy. PPB  has three macroscopically subtypes on a tumor have poor progression pathway start from cystic PPB (type I) to mixed cystic and solid (type II) and pure solid (type III). In the majority of PPB cases A gremlin mutation `need multimodal aggressive therapies including surgery and chemotherapy and/or radiotherapy. the study perform in Basra oncology center during eight years between 2012 -2019 have six cases of PPB male to female 1:1 most of cases less five years of old. most of cases were poor prognosis type II / III (mixed / pure solid) three cases type II and other three cases are type III  and  inspite of forceful treated by multimodal of treatment involved surgical ,chemotherapy and radiotherapy have poor destiny and the death rate 33.3%.