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Pathology of Jugulotympanic Paraganglioma

Dr Sampurna Roy MD    

 

                                                                                                                      

 

 
Jugulo-tympanic paragangliomas are the most common primary neoplasm of the middle ear.

It is also known as "glomus" tumour or chemodectoma.

Jugulotympanic paragangliomas were first described approximately 75 years ago.

Since that time, there has been considerable evolution in knowledge of tumor biology, methods of classification, and appropriate management strategies.

The tumour is usually located in the middle ear. 

The middle ear paraganglioma arise from the paraganglia situated in the wall of the jugular bulb.

Clinical presentation: Usually noted in the 4th to 7th decades of life. Solitary lesions are common in women.

The patient usually presents with conductive hearing loss. There may be pain in the ear, facial palsy, hemorrhage and tinnitus.

Otoscopic examination reveals red vascular mass either behind an intact eardrum or invading it to extend to the external auditory canal.

Macroscopic features:  It is a soft reddish tumour.

In the external ear it may extend through eardrum. 

In jugular type the petrous temporal bone is infiltrated by the tumour. 

In the middle ear space soft tumour extends as far as the tympanic membrane.

Microscopic feature:

The tumour cells form "small clusters" with numerous intervening blood vessels.

Atypical and sometimes multinucleate cells are noted.

Alveolar pattern like in middle ear adenoma has been described.

Immunohistochemistry:  

Neuron-specific enolase, and chromogranin are usually positive.

Sometimes S100 protein positive cells are present. Cytokeratin is negative. 

Formalin-induced fluorescence indicate presence of cathecholamine.

Zellballen pattern (balls of cells) - Images of Paraganglioma

 

 

Adrenal Pheochromocytoma ;

Extraadrenal Paraganglioma ;

Paraganglioma of the Urinary Bladder ;

Duodenal Gangliocytic Paraganglioma ;

Cardiac Paraganglioma ;

Pulmonary Paraganglioma ;

Pathology of Carotid Body Tumour.

 

Further reading:

Regression and local control rates after radiotherapy for jugulotympanic paragangliomas: systematic review and meta-analysis.

Jugulotympanic paragangliomas: 75 years of evolution in understanding.

Immunohistochemical profile of neurotrophins and MIB-1 in jugulotympanic paragangliomas: prognostic value and review of the literature.

Function-preserving therapy for jugulotympanic paragangliomas: a retrospective analysis from 2000 to 2010.

Immunohistochemical profile of neurotrophins and MIB-1 in jugulotympanic paragangliomas: prognostic value and review of the literature.

Paragangliomas of the head and neck.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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