8693/1 Chemodectoma



Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72

A benign or malignant paraganglioma arising from sympathetic or parasympathetic paraganglia outside the adrenal gland.



Thyroid gland
ICD-O-3 topography code: C73

A benign or malignant paraganglioma arising from sympathetic or parasympathetic paraganglia outside the adrenal gland.

Thyroid paragangliomas are rare tumours 1
Click to access Pubmed
LaGuette J, Matias-Guiu X, Rosai J (1997)
Thyroid paraganglioma: a clinicopathologic and immunohistochemical study of three cases.
Am J Surg Pathol 21: 748-53



. All cases have occurred in females.
In all reported cases, thyroid paragangliomas have behaved as benign neoplasms
2
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



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Adrenal gland
ICD-O-3 topography code: C74

Extra-adrenal paraganglioma: Carotid body, jugulotympanic, vagal, laryngeal, aortico-pulmonary tumours arising from the paraganglia distributed along the parasympathetic nerves in the head, neck and mediastinum. These tumours are histologically identical and are named by anatomical site of origin
3
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



.

These neoplasms are rare. Jugulotympanic and carotid body tumours are the two most frequent. A significant increase in the incidence of carotid body paraganglioma has been reported in humans dwelling at high altitude, where a marked female predilection has been reported
4
Click to access Pubmed
Rodríguez-Cuevas S, López-Garza J, Labastida-Almendaro S (1998)
Carotid body tumors in inhabitants of altitudes higher than 2000 meters above sea level.
Head Neck 20: 374-8



.
It is generally accepted that a paraganglioma is determined to be malignant only when metastases are demonstrated.



Ovary
ICD-O-3 topography code: C56

A benign or malignant paraganglioma arising from sympathetic or parasympathetic paraganglia outside the adrenal gland.



Prostate gland
ICD-O-3 topography code: C61

A benign or malignant paraganglioma arising from sympathetic or parasympathetic paraganglia outside the adrenal gland.



Gallbladder and extrahepatic biliary tract
ICD-O-3 topography code: C23-C24.0

A benign or malignant paraganglioma arising from sympathetic or parasympathetic paraganglia outside the adrenal gland.

Paraganglioma of the gallbladder and extrahepatic bile ducts
This generally benign neural tumour is composed of nests of neural cells (chief cells) often lined by Schwann cell-related sustentacular cells. The former are immunohistochemically positive for chromogranin A and synaptophysin and negative for keratins and S100 protein, whereas the latter are positive for S100 protein and often for glial fibrillary acidic protein (GFAP). The tumour is usually located in the outer wall of the gallbladder, but can also involve the extrahepatic bile ducts. It is usually an incidental microscopic finding in cholecystectomy specimens. Some cases occur in association with multiple endocrine neoplasia (MEN) syndromes
5
Click to access Pubmed
Mehra S, Chung-Park M (2005)
Gallbladder paraganglioma: a case report with review of the literature.
Arch Pathol Lab Med 129: 523-6



. It can be difficult to distinguish between normal paraganglionic structures and a very small paraganglioma 6
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Urinary system: Renal pelvis, ureter, bladder, urethra
ICD-O-3 topography code: C65-C68

A benign or malignant paraganglioma arising from sympathetic or parasympathetic paraganglia outside the adrenal gland.



Testis/Male genital organs
ICD-O-3 topography code: C62-C63

A benign or malignant paraganglioma arising from sympathetic or parasympathetic paraganglia outside the adrenal gland.