Pleuropulmonary blastoma: Long‐term survival and literature review

Abstract

Background and Procedure. Pleuropulmonary blastoma (PPB) identifies different types of pulmonary tumors in the pediatric age. It is extremely uncommon and is known to have a very poor prognosis, with only few cases surviving after 10 years follow-up. Our experience with such a patient and long-term follow-up (12 years) is therefore of interest. She was a 2-yearold girl admitted with a history of respiratory distress and underwent a left thoracotomy. The tumor was removed and after 1 month a polychemotherapy course was started using dactinomycin and vincristine for 6 months. The patient remains disease-free 12 years after the operation. Conclusions. The biologic behavior of the tumor is considered unpredictable, and for this reason much effort has been expended in trying to identify prognostic factors. The preoperative size of the mass (size <5 cm) in our patient, its complete excision, and the histologic aspects might be helpful in identifying favorable prognostic factors.

Treatment and prognosis of pleuropulmonary blastoma: A Single-Center Report of 31 Cases

“…Accordingly, Dehner et al classified PPB into three types as follows: Type I, tumor composed of cystic elements; Type II, tumor composed of both cystic and substantial elements; and Type III, tumor composed of substantial elements. 3 The three PPB pathologies form a dynamic continuum. While Type I PPB tends to be a low-grade malignancy, it can progress to the more malignant type II or III.…”

Hidden chromosomal abnormalities in pleuropulmonary blastomas identified by multiplex FISH

“…The outcome is particularly unfavourable because of recurrence and/or metastases. The central nervous system represents the main site of metastases [ 1 , 6 - 8 ].…”

Pleuropulmonary Blastoma: A Case Report

“…The rarity of PPB has allowed only slow elucidation of its clinical features according to prognosis and its response to therapy 20 . The treatment is primarily complete excision of the tumor 21,22 , followed by intense chemotherapy 23 .…”