Table 1: Carotid paragangliomas (CPG) related to syndromes and diseases. Literature review.
Clinical situation |
Authors,year [reference] |
# |
Age/sex |
Side-CPG features |
Other tumours (side) |
Genetic test |
Surgery (+) |
CPG & Syndromes vonHippel-Lindau disease
MEN-II
Neurofibromatosis-type 1
Carney triad
Carney-Stratakis syndrome |
Hull, et al. 1982 [32] Zanelli & van der Walt, 1996 [45] Gros, et al. 1996 [30] Schimke, et al. 1998 [41] Majchrzak, et al. 2011 [35]
Erickson, et al. 2001 [29] Petr & Else, 2018 [39] Carney, et al. 1977 [28] Carney, et al. 1999 [27] Wintermark, et al. 2001 [43] Nicholas, et al. 2015 [36] |
1 1 1 1 1 1 1 1 7 1 1 |
18/M 28/M NR NR 49/F NR NR 25/F NR 18/F 39/M |
Right CPG malignant (metastasis) Left CPG CPG Right CPG functional Right CPG Neck PG (possible CPG) Neck PG (possible CPG) Left CPG One case bilateral/Six unilateral CPG Left CPG Bilateral CPGs (R, large; L, small) |
PHEO (R) None PHEO PHEOs, retinal angiomas, renal and pancreatic cysts Hemangioblastoma intramedullary MEN type IIB NR Functional PG (thoracic sympathetic chain) NR GISTs (metastatic), PCH, PHEO (R), PGs abdominal GIST, PHEO (L) |
NR NR NR Positive Positive NR NR NR NR NR Positive |
CPG, PHEO CPG NR CPG, PHEOs Hemang; “I refuse CPG” NR NR All (CPG, GISH, PCH, PG) NR GIST, PHEO, CPG PHEO, CPG (R), GIST |
CPG & Diseases Parathyroid adenoma
Astrocytoma
Thyroid carcinoma
Pituitary tumours
Thymoma |
Steely, et al. 1987 [42] Ali, et al. 1994 [23] Ophir, 1991 [37]
Albores & Espino, 1968 [22] Albores, et al. 1969 [21] Zbuk, et al. 2007 [46] Huguet, et al. 2013 [31] Papathomas, et al. 2013 [38] Bugalho, et al. 2015 [26] Lin et al. 2019 [34] Berg, et al. 1976 [25] Larraza-Hernandez, et al. 1982 [33] Xekouki, et al. 2012 [44] Papathomas, et al. 2013 [38] Refior & Mees, 2000 [40] Bano, et al. 2015 [24] |
1 1 1 1 1 2 1 1 1 1 1 1 1 1 1 1 1 1 1 |
21/M 56/F 12/F* 55/F 52/F NR 43/M** 43/F*** 25/F 58/F 51/M 46/F 70/F 56/F 32/F 41/M 60/M 59/M 49/F |
Right CPG Left CPG Bilateral CPGs Left CPG Right CPG Unilateral Bilateral CPGs Right CPG Bilateral CPGs Right CPG Right CPG Right CPG Bilateral CPGs Bilateral CPGs Bilateral CPGs Bilateral CPGs Multiples neck PGs (possible CPG) Bilateral CPGs Right CPG |
L-Parathyroid adenoma L-Parathyroid adenoma, pancreatic pseudocysts JPG More thyroid follicular adenoma More thyroid follicular adenoma Follicular adenoma, Mixed parotid tumour (one case) Uterine leiomyoma, fibrocystic breast disease Abdominal PG, JPG (L) JPG, PHEOs, extra-adrenal PG None None Bronchial carcinoid, HPT, uterus myoma Thyroid carcinoma, HPT, gastric leiomyoma None None PHEOs, seven PGs (3 neck, 3 thorax, 1 abdomen) PHEO Thyroid adenoma None |
NR NR NR NR NR NR Positive Positive Positive Positive Negative NR Mother (F) Daughter (F) Granddaughter (F) Positive Positive Positive Negative |
CPG, parathyroid Parathyroid, CPG CPGs, JPG CPG + thyroid Thyroid, CPG NR CPGs, thyroid Thyroid, PG (in progress) PCG (L), thyroid NR CPG + thyroid All, post-CPG (died) Died after admission (A) NR NR Pituitary, PHEOs PGs, pituitary, PHEO CPG CPG, thymoma |
M: Male; F: Female; R: Right; L: Left; NR: Not Reported; PG: Paraganglioma (PGs, multiple); CPG: Carotid Paraganglioma; JPG: Jugularparaganglioma; PHEO: Pheochromocytoma (PHEOs, bilateral); MEN: Multiple Endocrine Neoplasia; GIST, Gastrointestinal Stromal Tumour (GISTs, multiple); PCH: Pulmonary Chondroma; HPT: Hyperplasia parathyroid. (+) Simultaneous Surgery, Rest Sequential Surgery; A: Autopsy; F: Familiar Study.
*Her father died of a cerebral astrocytoma and her motherunderwent surgical removal of a large mediastinal PG; **Cowden syndrome; ***Dandy-Walker malformation.