Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature

Abstract

Biphasic pulmonary blastoma (BPB) is an extremely rare highly aggressive malignant tumor that arises from fetal lung tissue and has the classical biphasic histology of epithelial and mesenchymal components. It is usually seen in adults with a slight male predominance and smokers. Previously grouped along with well-differentiated fetal adenocarcinoma (WDFA), and pleuropulmonary blastoma (PPB), now it is considered a separate variant and grouped under sarcomatoid neoplasms. Symptoms include chest pain, cough, hemoptysis and it is asymptomatic in at least one-third of the cases. A biopsy is essential for diagnosis and surgical excision is the treatment of choice. Prognosis is poor with 5-year survival less than 20% and recurrence occurring within 12 months of surgery. An aggressive multimodality approach is required for its management and active follow up surveillance is needed to look for recurrence.

Pulmonary Blastoma: A Comprehensive Overview of A Rare Entity

“…PB has both a local growth pattern invading adjacent structures and a hematogenous metastatic spread. The most common symptoms www.journals.viamedica.pl that occur are cough, hemoptysis, shortness of breath, recurrent pneumonia, fever, and weight loss, but asymptomatic tumors, accounting for 40% of cases, may also be detected incidentally [22][23][24][25]. There is a similarity in the anatomical presentation of these tumors.…”

Classic biphasic pulmonary blastoma: A case report and review of the literature from 2000 to 2022

“…Originally delineated by Barnett et al, CBPB exhibits distinctive biphasic histological characteristics, combining aspects of low‐grade fetal adenocarcinoma and primitive mesenchymal components. Unfortunately, this malignancy is notorious for its aggressive behavior and its associated poor patient survival rates 2 . Currently, early surgical intervention remains to be the primary treatment strategy; however, a review of case reports over recent years suggests that improved prognosis has been achieved with new treatment modalities.…”

“…Unfortunately, this malignancy is notorious for its aggressive behavior and its associated poor patient survival rates. 2 Currently, early surgical intervention remains to be the primary treatment strategy; however, a review of case reports over recent years suggests that improved prognosis has been achieved with new treatment modalities. This literature review aims to synthesize 22 years of developments, advancements, and alternative treatment methodologies relating to CBPB.…”