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Top Ten Facts about Pathology of Pulmonary Blastoma - Did You Know?

Dr Sampurna Roy MD   

 

 

                

                

Histological features of the lesion containing epithelial and mesenchymal component consistent with pulmonary blastoma.

                

Histological features of the lesion containing epithelial and mesenchymal component consistent with pulmonary blastoma.

 

Source: Alahwal MS, Maniyar IH, Saleem F, Alshiekh M. Pulmonary Blastoma: A Rare Primary Lung Malignancy. Case Reports in Medicine. 2012;2012:471613.

 

 
(1) Pulmonary blastoma is a rare aggressive neoplasm comprising 0.25-0.5% of all primary lung tumours.

 

 

(2) Morphologically this tumour mimics fetal lung tissue before 4 months gestation.

 

 

(3) Pulmonary blastoma was first described by Dr W G Barnard at St Thomas's Hospital- London in 1952.

He called this unusual tumour "Embryoma of Lung" based on the histological resemblance of the tumour to fetal lung tissue.

 

 

(4) The term blastoma was introduced in 1961 by Spencer who described the tumour origin from a pleuripotential pulmonary blastoma analogous to Wilm's tumor that appears to share the same histological features.

 

 

(5) Historically, the term pulmonary blastoma had included  three subgroups: classic biphasic pulmonary blastoma, well-differentiated fetal adenocarcinoma - also called monophasic pulmonary blastoma- and pleuropulmonary blastoma of childhood.

Well-differentiated fetal adenocarcinoma consists of epithelial malignant component only; the classic biphasic pulmonary blastoma is characterized by both epithelial and mesenchymal malignant components; pleuropulmonary blastoma, that is, a childhood tumour shows features of mesenchymal malignant components only.

 

 

(6) Recent World Health Orgnisation (WHO) reclassifications, separated well-differentiated fetal adenocarcinomas and pleuropulmonary blastomas from the classic biphasic tumours.

 

 

(7) Clinically, the patient typically presents with cough, hemoptysis, dyspnea or chest pain due to tumour impinging on the bronchi or pleura. Forty percent of cases may be asymptomatic. The average age at diagnosis is 40 years with an increased frequency in males.

 

 

(8) Pulmonary blastomas are biphasic tumours that are part of the sarcomatoid carcinoma subgroup, which also include carcinosarcomas (defined as a malignant tumour having a mixture of carcinoma- and sarcoma-containing heterologous elements such as malignant cartilage, bone, or skeletal muscle) and pleomorphic carcinomas (similar tumour without heterologous elements), both histologically resembling adult-type carcinomas and sarcomas.

 

                                                

    

  

 Histological analysis of the tumour.

a. Malignant glandular tissue with sub-nuclear vacuoles.

b. Pleomorphic stromal malignant blastematous component.

c. The malignant glandular component was diffusely positive for the epithelial marker Cam 5.2.

d. Positive for TTF-1 and negative for vimentin.

e. The stromal blastematous malignant component showed reverse expression.

f. The protein beta-cathenin was observed in both components.

Source: Smyth RJ, Fabre A, Dodd JD, Bartosik W, Gallagher CG, McKone EF. Pulmonary blastoma: a case report and review of the literature. BMC Research Notes. 2014;7:294.

 

(9) Classic biphasic blastoma has distinctive histopathological features.

The epithelial component of classic biphasic blastoma is composed of tubules of glycogen-rich, non-ciliated cells that resemble fetal lung of pseudo-glandular stage of lung development with sub-nuclear and supra-nuclear glycogen vacuoles.

The embryonic appearance of the stroma is due to the small size, oval and spindle shape of the cells, and myxoid matrix. Classically this blastematous stroma does not express cytokeratins or pulmonary markers.

 

 

(10) The tumour has a poor prognosis. 

Two thirds of patients die within 2 years and only 16% patients have 5-year survival. Prognosis is determined by size of the tumor at time of diagnosis, with tumors <5 cm doing better. Tumour metastasis and tumor recurrence despite resection both predict a poor prognosis.

 

 

Source

Smyth RJ, Fabre A, Dodd JD, Bartosik W, Gallagher CG, McKone EF. Pulmonary blastoma: a case report and review of the literature. BMC Research Notes. 2014;7:294.

 

Alahwal MS, Maniyar IH, Saleem F, Alshiekh M. Pulmonary Blastoma: A Rare Primary Lung Malignancy. Case Reports in Medicine. 2012;2012:471613.

 

 

 

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