8693/1 Nonchromaffin paraganglioma, NOS
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
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Thyroid paraganglioma: a clinicopathologic and immunohistochemical study of three cases.
Am J Surg Pathol 21: 748-53
In all reported cases, thyroid paragangliomas have behaved as benign neoplasms 2
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004
| 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 origin3
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
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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
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Gallbladder paraganglioma: a case report with review of the literature.
Arch Pathol Lab Med 129: 523-6
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010
| 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.