Synonyms and subtypes:
a.k.a. carotid body tumor, glomus jugulare tumor, glomus tympanicum
Diagnosis:
Suggested by MRI and confirmed by pathology (brain biopsy/resection)
Findings on Investigations:
MRI:
- Neck mass (carotid body tumor, glomus jugulare tumor), middle ear canal mass (glomus tympanicum)
- Filum terminale
- Enhancing lesion
MRA: blood supply can be assessed.
Catheter Angiogram:
- To assess vascular supply & embolisation prior to surgery
Pathology:
Histology:
- Nests of chief cells (Zellballen), surrounded by sustentacular cells, separated by vessels,
Immunohistochemistry: positive for Chromogranin (chief cells), neurofilament (chief cells), synaptophysin (chief cells), NSE (chief cells), S100 (sustentacular cells),
Urine testing: Free catecholamines, metanephrines MTA & vanillylmandelic acid VMA (3-mehoxy-4-hydroxymandelic acid). Metanephrine:creatinine ratio
Treatment:
Consider Presurgical embolisation
Surgical Resection