Carotid Body, Paraganglioma [LMP52195]

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Diagnostic Modality: 
Whole Slide Image
Disease Category: 
Neoplasia
Diagnosis: 
Paraganglioma
Species: 
Human
Organ System: 
Head and Neck
Gender: 
Male
Age: 
71 years
Diagnosis Code: 
ICD-10: D44.6 | ICD-O: 8680/1
Slide Contributor Information
Mrkonjic, M., Lu, F.

Keywords

extra-adrenal, paraganglia, phaeochromocytoma, pheochromocytoma, chemodectoma, parasympathetic

Clinical History

Neck mass.

Clinical Discussion

Tumours arising from the parasympathetic paraganglia are rare, and are named according to anatomical site of origin. 30% of all paragangliomas occur in the head and neck with carotid body being the most common location, followed by jugular, tympanic, vagal and larynx sites. It occurs more frequently in women than men in a ratio of 4-8:1. It usually presents as a painless and slow growing mass.

Microscopically, it has a nested (zellballen), alveolar or whorled architecture composed of small epithelioid chief cells with granular basophilic cytoplasm and sustentacular supporting cells. Mitotic figures are rare. Chief cells are positive for neuroendocrine markers and sustentacular cells for S100 and GFAP.

Treatments include surgery and radiation therapy with excellent prognosis. Recurrence is rare and is more common in SDH-mutated tumours.