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

https://doi.org/10.4081/monaldi.2020.1462

Authors

  • Benhur Joel Shadrach Department of Pulmonary Medicine, All India Institute of Medical Sciences, Jodhpur, India. https://orcid.org/0000-0002-2314-2306
  • Deepak Vedant | dr.tomchi@gmail.com Department of Pathology, All India Institute of Medical Sciences, Jodhpur , India. https://orcid.org/0000-0003-0041-9314
  • Vikarn Vishwajeet Department of Pathology, All India Institute of Medical Sciences, Jodhpur , India. https://orcid.org/0000-0002-2927-2603
  • Priyank Jain Department of Pulmonary Medicine, All India Institute of Medical Sciences, Jodhpur, India. https://orcid.org/0000-0001-6898-0947
  • Naveen Dutt Department of Pulmonary Medicine, All India Institute of Medical Sciences, Jodhpur, India.
  • Binit Surekha Department of Radiodiagnosis, All India Institute of Medical Sciences, Jodhpur, India. https://orcid.org/0000-0001-7200-296X
  • Puneet Pareek Department of Radiation Oncology, All India Institute of Medical Sciences, Jodhpur, India. https://orcid.org/0000-0002-6055-9872
  • Poonam Abhay Elhence Department of Pathology, All India Institute of Medical Sciences, Jodhpur, India.

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.

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Published
2020-07-30
Info
Issue
Section
Pneumology - Case Reports
Keywords:
Pulmonary blastoma, endobronchial, histology, prognosis
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  • PDF: 138
How to Cite
Shadrach, Benhur Joel, Deepak Vedant, Vikarn Vishwajeet, Priyank Jain, Naveen Dutt, Binit Surekha, Puneet Pareek, and Poonam Abhay Elhence. 2020. “Endobronchial Pulmonary Blastoma – an Unusual Presentation of a Rare Lung Malignancy and Review of Literature”. Monaldi Archives for Chest Disease 90 (3). https://doi.org/10.4081/monaldi.2020.1462.

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