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.