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Paraganglioma-Online

Pathology of Carotid Body Tumour

 Dr Sampurna Roy MD                       

 

Paragangliomas are neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system.

Within the head and neck, they are generally defined and named according to their site of origin, and may be found frequently near vascular structures.

These tumors generally exhibit a slow rate of growth, most often presenting asymptomatically as a space occupying mass lesion noted clinically or radiographically.

The most common paraganglioma of the head and neck is the carotid body tumor followed by the jugulo-tympanic and vagal varieties.

Carotid body tumours (CBT) are a rare entity that should be considered in evaluating every lateral neck mass.

The tumour is classically located in the carotid bifurcation, between internal and external carotid arteries.

Diagram of Carotid Body Tumour between Internal and External Carotid Arteries.

The carotid body tumour was first described by von Haller in 1743.

The first two, unsuccessfully surgically treated carotid body tumours, were done by Reinger in 1880 (his patient died), and by Maydel in 1886 (his patient developed hemiplegia).

Scudder made the first successful surgical removal of the carotid body tumour in 1903.

The clinical course of carotid body paragangliomas is characterized by two modes of development: locoregional growth and distant metastatic dissemination.

Few cases of malignant carotid body paraganglioma have been reported in the literature.

Malignant forms of carotid paraganglioma are very uncommon and there is no cellular or histological appearance significantly contributing to the diagnosis of malignancy.

Clinical findings are the most reliable criteria for malignancy, with the development of distant metastases usually after about 9 years. Long follow-up is essential.

Histology is needed to establish the diagnosis of metastasis.

Some patients have von Hippel-Lindau syndrome, an autosomal dominant condition which includes cerebellar hemangioblastoma, renal cell carcinoma and paragangliomas amidst its features.

Zellballen pattern (balls of cells) - Images of Paraganglioma

 

Adrenal Pheochromocytoma ; Extraadrenal Paraganglioma ; Paraganglioma of the Urinary Bladder ; Duodenal Gangliocytic Paraganglioma ; Cardiac Paraganglioma ; Pulmonary Paraganglioma ;  Jugulotympanic Paraganglioma ;

 

Further reading

Synchronous bilateral carotid body tumor and vagal paraganglioma: a case report and review of literature.

Carotid body tumors: review of a 20-year experience.

Dignity of carotid body tumors. Review of the literature and clinical experiences

Carotid body tumor: review of the literature and report of a case with a rare sensorineural symptomatology.

Malignant carotid body tumors: report of three cases.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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