A case of pulmonary blastoma lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung)

Background. Pulmonary blastoma is a rare lung tumor similar to fetal lung tissues. Surgical resection at early stage is more curative than other treatments, but there is no standard treatment in unresectable cases. We show a case treated with carboplatin and paclitaxel plus bevacizumab.Case. A 68-year-old man received surgical resection and was diagnosed with biphasic pulmonary blastoma (pT3N0M0 stage IIB). Metastasis to the spleen was detected six weeks after the surgery. Carboplatin, paclitaxel, and bevacizumab were administered and showed an effect on the metastasis. Four courses of the chemotherapy were completed, but a metastasis was found and the metastatic tumor in the spleen was enlarged. After that, chemotherapy was not effective afterward and he died of the progression of biphasic pulmonary blastoma on the 292nd day of illness.Conclusion. In this case, chemotherapy with carboplatin and paclitaxel plus bevacizumab was temporarily efficacious for biphasic pulmonary blastoma.

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Pulmonary Blastoma: A Rare Primary Lung Malignancy

Case Reports in Medicine ◽

10.1155/2012/471613 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 7

Author(s):

Mahmoud S. Alahwal ◽

Iqbal H. Maniyar ◽

Faiza Saleem ◽

Mariam Alshiekh

Keyword(s):

Objective Response ◽

Fetal Lung ◽

Surgical Excision ◽

Disease Recurrence ◽

Pulmonary Blastoma ◽

Complete Surgical Excision ◽

Lung Malignancy ◽

Platinum Based Chemotherapy ◽

Rare Malignancy ◽

Wall Mass

Pulmonary blastoma, a rare primary lung malignancy, is considered to be distinct from other lung tumors based on pathological features, clinical course, and prognosis. More than one hundred cases have been reported in literature highlighting an interesting fact about their distinctive biologic manner from histopathological features. Classic pulmonary blastoma is composed of a mixture of immature epithelial and mesenchymal tissue resembling fetal lung tissue. Surgery is the mainstay of treatment. The prognosis of this rare malignancy is poor and the overall 5-year survival is around 15%. Our patient presented with respiratory symptoms and was found to have right-sided chest wall mass. The patient underwent complete surgical excision followed by 6 cycles of platinum-based chemotherapy. The patient showed good subjective and objective response with no evidence of disease recurrence. We report this rare malignancy with a review of literature, and the potential to use adjuvant chemotherapy in the management of this condition.

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A Case of Biphasic Pulmonary Blastoma (High-grade Adenocarcinoma of Fetal Lung Type with a Sarcomatous Component).

Haigan ◽

10.2482/haigan.37.249 ◽

1997 ◽

Vol 37 (2) ◽

pp. 249-254 ◽

Cited By ~ 2

Author(s):

Kazuhiko Date ◽

Shinji Kato ◽

Kazuo Kondo ◽

Masashi Yoshihara ◽

Kazushige Beppu ◽

...

Keyword(s):

Fetal Lung ◽

Pulmonary Blastoma ◽

High Grade ◽

Sarcomatous Component

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Clinicopathologic features and genomic analysis of pulmonary blastomatoid carcinosarcoma

10.21203/rs.2.18869/v1 ◽

2019 ◽

Author(s):

Jikai Zhao ◽

Chan Xiang ◽

Ruiying Zhao ◽

Ping Guo ◽

Jingjing Zheng ◽

...

Keyword(s):

Lung Adenocarcinoma ◽

Genomic Analysis ◽

Fetal Lung ◽

Molecular Profile ◽

Pulmonary Blastoma ◽

Low Grade ◽

High Grade ◽

Clinicopathologic Features ◽

Epithelial Component ◽

Additional Mutation

Abstract Background: This study was designed to investigate the clinicopathologic features of pulmonary blastomatoid carcinosarcoma and explore the genomic profiles of epithelial and mesenchymal components in this tumor. Methods: Three cases of pulmonary blastomatoid carcinosarcoma were enrolled in this study. Clinic-pathologic information and prognostic data were retrospectively reviewed. Diagnostic immunohistochemistry was performed. The epithelial and mesenchymal components were microdissected to investigate the genomic profiles by performing capture-based targeted next generation sequencing. Results: The epithelial component in patient one was consistent of low-grade and high-grade fetal lung adenocarcinoma and displayed aberrant nuclear expression of β-catenin and missense mutation of CTNNB1 in its low-grade epithelial. The epithelial component in another two patients were consistent of high-grade fetal lung adenocarcinoma/enteric adenocarcinoma and harbored no mutation of CTNNB1. The mesenchymal components in all three tumors were primitive round/spindle cells in morphology without definite differentiation and showed cytoplasmic dot positive of β-catenin and no corresponding mutation. Within a tumor, both components exhibited relatively comparable molecular profile. In patient one, 4 mutations: RB1, FAT3, PTCH1 and LRP1B were shared by both epithelial and mesenchymal components. Epithelial had additional mutations in BCOR, CTNNB1, CTCF, FAT1 and DICER1. In patient two, 12 mutations were shared. The epithelial had BRCA2 mutations and the mesenchymal had mutations in CREBBP, ALK, DNMT3A, ASXL2, MYCN andRICTOR. Patient three had 6 shared mutations. The epithelial had an additional mutation in KAT6A and the mesenchymal had an additional mutation in APC. Collectively, we observed heterogeneity between epithelial and mesenchymal components of the same tumor. Conclusions: Parallel detection of genetic abnormalities in epithelial and mesenchymal components of blastomatoid carcinosarcoma could provide evidence for tumor differentiation, molecular targeting and further distinguish them from conventional pulmonary blastoma and carcinosarcoma.

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

Monaldi Archives for Chest Disease ◽

10.4081/monaldi.2020.1462 ◽

2020 ◽

Vol 90 (3) ◽

Author(s):

Benhur Joel Shadrach ◽

Deepak Vedant ◽

Vikarn Vishwajeet ◽

Priyank Jain ◽

Naveen Dutt ◽

...

Keyword(s):

Fetal Lung ◽

Surgical Excision ◽

Pleuropulmonary Blastoma ◽

Pulmonary Blastoma ◽

Review Of Literature ◽

Male Predominance ◽

Lung Malignancy ◽

Well Differentiated ◽

Multimodality Approach

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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Pulmonary blastoma with argyrophil cells and lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung)

The American Journal of Surgical Pathology ◽

10.1097/00000478-198203000-00009 ◽

1982 ◽

Vol 6 (2) ◽

pp. 165-172 ◽

Cited By ~ 79

Author(s):

Richard L. Kradin ◽

Robert H. Young ◽

G. Richard Dickersin ◽

Sara E. Kirkham ◽

Eugene J. Mark

Keyword(s):

Fetal Lung ◽

Pulmonary Blastoma ◽

Argyrophil Cells

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Well-Differentiated Fetal Adenocarcinoma: Rare Tumor in the Pediatric Population

Pediatric and Developmental Pathology ◽

10.1007/s10024-003-4046-x ◽

2003 ◽

Vol 6 (6) ◽

pp. 564-567 ◽

Cited By ~ 12

Author(s):

Megan J. DiFurio ◽

Aaron Auerbach ◽

Keith J. Kaplan

Keyword(s):

Pediatric Population ◽

Neuroendocrine Differentiation ◽

Fetal Lung ◽

World Health ◽

Pulmonary Blastoma ◽

Rare Tumor ◽

Argyrophil Cells ◽

Well Differentiated ◽

Health Organization ◽

Age Range

Well-differentiated fetal adenocarcinoma (WDFA) is a rare tumor of the lung, which has gone by many names over the years. The lesion was first described by Kradin et al., in 1982, who called it “pulmonary blastoma with argyrophil cells and lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung).” Since then, there have been at least 65 cases reported in the literature. Although there has been no consensus in the literature as to the best pathological term for this entity, the most recent World Health Organization classification of lung and pleural tumors uses the term well-differentiated fetal adenocarcinoma. Characteristically, this lesion consists of an epithelium, which recapitulates fetal lung at 3–5 months of gestation and demonstrates neuroendocrine differentiation. Although the classic age range is 30–40 years, there have been seven reports of WDFA in the pediatric age. We report an additional pediatric case of this tumor and review the pediatric cases in the existing literature.

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Expression and mutation analysis of EGFR, c-KIT, and β-catenin in pulmonary blastoma

Journal of Clinical Pathology ◽

10.1136/jcp.2010.085696 ◽

2011 ◽

Vol 64 (4) ◽

pp. 349-353 ◽

Cited By ~ 17

Author(s):

Stephan Macher-Goeppinger ◽

Roland Penzel ◽

Wilfried Roth ◽

Hendrik Dienemann ◽

Michael Thomas ◽

...

Keyword(s):

Mutation Analysis ◽

Nuclear Translocation ◽

Therapeutic Option ◽

Fetal Lung ◽

Diagnostic Value ◽

Lung Tumour ◽

Pulmonary Blastoma ◽

Egfr Inhibition ◽

Egfr Expression

IntroductionPulmonary blastoma (PB) is a rare malignant lung tumour with an immature mesenchymal and epithelial component resembling fetal lung. In order to define potential therapeutic targets in PB, the authors analysed the status and possible role of EGFR, HER2 and c-KIT in the pathogenesis of this tumour type, and the diagnostic value of β-catenin mutation analysis in PB.Methods5 PBs were analysed for EGFR, HER2, c-KIT, and β-catenin expression, as well as for mutations in EGFR, c-KIT, k-ras and the β-catenin gene (CTNNB1).ResultsEGFR expression was observed in all PBs. An EGFR mutation was found in one of the tumours. No overexpression of c-KIT or HER2 was seen. No mutations were found in k-ras or c-KIT. 3 of 5 PBs displayed CTNNB1 mutations. Nuclear translocation of β-catenin was seen in 2 of these tumours.ConclusionsDetection of EGFR expression and mutation in PB suggest EGFR inhibition as a potential therapeutic option in the treatment of advanced PB. Moreover, the data confirm a crucial role of CTNNB1 mutations in the pathogenesis of PB, and indicate that CTNNB1 gene sequencing may be a useful in distinguishing PB from other types of lung cancer.

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Classic biphasic pulmonary blastoma (CBPB): a rare primary pulmonary malignancy

BMJ Case Reports ◽

10.1136/bcr-2021-244151 ◽

2021 ◽

Vol 14 (8) ◽

pp. e244151

Author(s):

Shashank Pooniya ◽

Angela McKinnie ◽

Thomas Taylor ◽

Malcolm Will ◽

William Wallace

Keyword(s):

Treatment Guidelines ◽

Fetal Lung ◽

Surgical Excision ◽

Pulmonary Blastoma ◽

Pathological Features ◽

Ct Guided ◽

Team Meetings ◽

Complete Surgical Excision ◽

Ct Guided Biopsy ◽

Rare Malignancy

Classic biphasic pulmonary blastoma (CBPB) is a very rare primary pulmonary malignancy with distinctive clinical and pathological features. Usually CBPB presents with either non specific symptoms or is diagnosed incidentally. Histologically CBPB is composed of a mixture of malignant epithelial and stromal cells resembling fetal lung tissue. Surgical resection is the mainstay of treatment with further chemotherapy or radiotherapy on a case-by-case basis. However, due to its rarity, no definite treatment guidelines are available. CBPB overall has a very poor prognosis with a 5-year survival rate of only 15%. Our patient presented with cough and haemoptysis. Her chest radiograph demonstrated a large right-sided lung mass. Further investigations included CT, CT-guided biopsy and PET CT which were discussed at multidisciplinary team meetings. The patient then underwent complete surgical excision. We report this rare malignancy with radiological and pathological features, comparing them with previously reported cases.

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