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Pathology of Sclerosing Pneumocytoma of the Lung (Pulmonary Sclerosing Hemangioma) - Top Ten Facts

Dr Sampurna Roy MD   

 

 

    

Patrini D, Shukla R, Lawrence D, Borg E, Hayward M, Panagiotopoulos N. Sclerosing hemangioma of the lung showing strong FDG avidity on PET scan: Case report and review of the current literature.  Respiratory Medicine Case Reports. 2016;17:20-23. 

        

Schiergens TS, Khalil PN, Mayr D, et al. Pulmonary sclerosing hemangioma in a 21-year-old male with metastatic hereditary non-polyposis colorectal cancer: Report of a case. World Journal of Surgical Oncology. 2011;9:62. doi:10.1186/1477-7819-9-62.

 

 

(1) Sclerosing pneumocytoma of the lung, was first described by Liebow and Hubbel in 1956 and represents a rare and, in the majority of cases, benign neoplasm of the lung.

 

(2) This tumour predominantly affects Asian women in the fifth decade of life.

 

(3) Sclerosing pneumocytoma has been traditionally called sclerosing hemangioma, because it was thought to be endothelial and vascular in origin.

 

(4) Recent immunohistochemical studies have revealed that sclerosing pneumocytoma originates from undifferentiated respiratory epithelium, most likely type II alveolar pneumocytes.

 

(5) These tumors are mostly found incidentally on chest radiographs, but some patients may present with chest pain, cough, and hemoptysis.

 

(6) Lymph node metastases have been reported in a few cases but did not seem to affect patient prognosis.

 

(7) Histologically, this tumour is essentially characterized by two epithelial cell types: cuboidal surface cells, which resemble type II pneumocytes, and polygonal stromal cells (round cells) with bland nuclei and pale cytoplasm, which are thought to stem from primitive respiratory epithelium.

 

(8) The two cell types form four histological patterns; papillary, which often appears to be the predominant type, but epitheloid, sclerotic and hemorrhagic configurations are also found in some cases. 

 

(9) Predominantly papillary growth patterns might make it challenging to differentiate sclerosing pneumocytoma from a carcinoma that also exhibits a papillary pattern and tumours such as metastatic papillary thyroid carcinoma, mesothelioma and bronchioloalveolar carcinoma should be carefully excluded.

 

(10) The uniform positivity for EMA (Epithelial Membrane Antigen) is consistent with the notion that the tumour cells of sclerosing pneumocytoma are epithelial, and the strong thyroid transcription factor-1 positivity suggests differentiation toward pulmonary epithelium. 

 

 

        

      

 

 

Source:

 

Patrini D, Shukla R, Lawrence D, Borg E, Hayward M, Panagiotopoulos N. Sclerosing hemangioma of the lung showing strong FDG avidity on PET scan: Case report and review of the current literature. Respiratory Medicine Case Reports. 2016;17:20-23. 

 

Schiergens TS, Khalil PN, Mayr D, et al. Pulmonary sclerosing hemangioma in a 21-year-old male with metastatic hereditary non-polyposis colorectal cancer: Report of a case. World Journal of Surgical Oncology. 2011;9:62. doi:10.1186/1477-7819-9-62.

 

Kim YP, Lee S, Park HS, Park CH, Kim TH. Sclerosing Pneumocytoma with a Wax-and-Wane Pattern of Growth: A Case Report on Computed Tomography and Magnetic Resonance Imaging Findings and a Literature Review. Korean Journal of Radiology. 2015;16(4):947-950.

 

 


 

 

 

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