Table 4: Etiological/Molecular Classification of PGs of the Head and Neck*.
1. Sporadic forms (the most frequent ones; there is a possibility of concealed paraganglioma) 2. Familial forms (relatively frequent) related to PGL syndromes: - PGL1 (mechanism detected in SDHD gene). Frequency in multicentric PGs. - PGL2 (genetic mechanism in SDHAF2 gene) - PGL3 (mechanism detected in SDHC gene) - PGL4 (mechanism detected in SDHB gene). Frequency in malignant forms. 3. Forms involving multiple systems (exceptionally associated with head and neck PGs): 3.1. Associated with syndromes: - Von Hippel-Lindau (VHL) disease (mechanism detected in VHL gene) - Multiple endocrine neoplasia type II (mechanism detected in RET gene) - Neurofibromatosis type I (mechanism detected in NF1 gene) - Carney’s triad (unknown genetic mechanism, it may be familial) 3.2. Not associated with syndromes (unknown genetic mechanisms): - Parathyroid adenoma - Astrocytoma - Thyroid carcinoma - Pituitary tumour 4. Unknown associations: Thymoma |
PGs, paragangliomas.
*Modified of Baysal, 2001 [9] and Offergeld, et al. 2012 [12].