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Pathology of Pulmonary Paraganglioma

Dr Sampurna Roy MD




Primary paraganglioma of the lung are rare tumours.

Few cases of primary pulmonary paraganglioma have been reported since the first report by Heppleston in 1958. 

Primitive lung paragangliomas are often discovered incidentally with examination of routine chest radiographs, and patients are free of symptoms and hypertension.

Most cases are benign and are treated by surgical excision.

Since normal paraganglia have not been demonstrated in the lung, some authors regard these tumours as unusual carcinoid.

Morphological features:  Proliferation of round to oval cells with large hyperchromatic nuclei and abundant cytoplasm. Nested (Zellballen) growth pattern of the tumour cells are often present.

Immunohistochemistry:  Tumour cells react with neuroendocrine markers (chromogranin, synaptophysin and NSE). Most cases are negative for keratin. 

Sustentacular cells surrounding the nests of tumour cells are S-100 protein positive.

Differential diagnosis:  Pulmonary paragangliom needs to be differentiated from the following diseases :

Lung cancer; lung tuberculoma ; inflammatory pseudotumor ; hamartoma, lung carcinoid tumour and lung metastatic tumour, etc.

Bronchial carcinoid (cells are usually arranged in rosettes, ribbons and trabeculae).In paraganglioma large atypical hyperchromatic and often multilobated nuclei are present.


Further reading:

Pathology of Carotid Body Tumour

Pathology of Cardiac Paraganglioma

Pathology of Extra-adrenal Paraganglioma

Pathology of Duodenal Gangliocytic Paraganglioma

Pathology of Adrenal Pheochromocytoma

Pathology of Paraganglioma of the Urinary Bladder

Pathology of Jugulotympanic Paraganglioma

Primary paraganglioma of the lung: a case report and literature review.

Primary pulmonary paraganglioma with Hodgkin's lymphoma.

Primary pulmonary paraganglioma: case report and  literature review.

Spindle cell carcinoids of the lung with paraganglioid features: a reappraisal of their histogenetic origin from paraganglia using immunohistochemical and electronmicroscopic techniques. 

Ectopic adrenocorticotropic hormone hypersecretion due to a primary pulmonary paraganglioma. 

Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms.

Fine needle aspiration cytology of primary pulmonary paraganglioma. A case report.

Primary pulmonary paraganglioma: report of a functioning case with immunohistochemical and ultrastructural study.

Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms.



Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)







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