Open AccessEndobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature
Author(s) -
Benhur Joel Shadrach,
Deepak Vedant,
Vikarn Vishwajeet,
Priyank Jain,
Naveen Dutt,
Binit Surekha,
Puneet Pareek,
Poonam Elhence
Publication year - 2020
Publication title -
monaldi archives for chest disease. pulmonary series/monaldi archives for chest disease/monaldi archives for chest disease. cardiac series
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.196
H-Index - 46
eISSN - 2465-101X
pISSN - 1122-0643
DOI - 10.4081/monaldi.2020.1462
Subject(s) - pulmonary blastoma , medicine , malignancy , asymptomatic , lung , biopsy , adenocarcinoma , presentation (obstetrics) , pathology , radiology , surgery , cancer
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.