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

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...

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Main Authors:	Benhur Joel Shadrach, Deepak Vedant, Vikarn Vishwajeet, Priyank Jain, Naveen Dutt, Binit Surekha, Puneet Pareek, Poonam Abhay Elhence
Format:	Article
Language:	English
Published:	PAGEPress Publications 2020-07-01
Series:	Monaldi Archives for Chest Disease
Subjects:	Pulmonary blastoma endobronchial histology prognosis
Online Access:	https://monaldi-archives.org/index.php/macd/article/view/1462

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spelling	doaj-31b5e67b1d724f4ca4d246abc54cc8fc2020-11-25T03:28:18ZengPAGEPress PublicationsMonaldi Archives for Chest Disease1122-06432532-52642020-07-0190310.4081/monaldi.2020.1462Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literatureBenhur Joel Shadrach0Deepak Vedant1Vikarn Vishwajeet2Priyank Jain3Naveen Dutt4Binit Surekha5Puneet Pareek6Poonam Abhay Elhence7Department of Pulmonary Medicine, All India Institute of Medical Sciences, JodhpurDepartment of Pathology, All India Institute of Medical Sciences, Jodhpur Department of Pathology, All India Institute of Medical Sciences, Jodhpur Department of Pulmonary Medicine, All India Institute of Medical Sciences, JodhpurDepartment of Pulmonary Medicine, All India Institute of Medical Sciences, JodhpurDepartment of Radiodiagnosis, All India Institute of Medical Sciences, JodhpurDepartment of Radiation Oncology, All India Institute of Medical Sciences, JodhpurDepartment of Pathology, All India Institute of Medical Sciences, Jodhpur 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. https://monaldi-archives.org/index.php/macd/article/view/1462Pulmonary blastomaendobronchialhistologyprognosis
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	Benhur Joel Shadrach Deepak Vedant Vikarn Vishwajeet Priyank Jain Naveen Dutt Binit Surekha Puneet Pareek Poonam Abhay Elhence
spellingShingle	Benhur Joel Shadrach Deepak Vedant Vikarn Vishwajeet Priyank Jain Naveen Dutt Binit Surekha Puneet Pareek Poonam Abhay Elhence Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature Monaldi Archives for Chest Disease Pulmonary blastoma endobronchial histology prognosis
author_facet	Benhur Joel Shadrach Deepak Vedant Vikarn Vishwajeet Priyank Jain Naveen Dutt Binit Surekha Puneet Pareek Poonam Abhay Elhence
author_sort	Benhur Joel Shadrach
title	Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature
title_short	Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature
title_full	Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature
title_fullStr	Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature
title_full_unstemmed	Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature
title_sort	endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature
publisher	PAGEPress Publications
series	Monaldi Archives for Chest Disease
issn	1122-0643 2532-5264
publishDate	2020-07-01
description	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.
topic	Pulmonary blastoma endobronchial histology prognosis
url	https://monaldi-archives.org/index.php/macd/article/view/1462
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Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature

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