Paraganglioma

glomus jugulareparagangliomasChemodectomacarotid body tumorGlomusglomus jugulare tumour
A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen).wikipedia
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Pheochromocytoma

phaeochromocytomapheochromocytomasadrenal cancers
Most paragangliomas are either asymptomatic or present as a painless mass. While all contain neurosecretory granules, only in 1–3% of cases is secretion of hormones such as catecholamines abundant enough to be clinically significant; in that case manifestations often resemble those of pheochromocytomas (intra-medullary paraganglioma).
Extra-adrenal paragangliomas (often described as extra-adrenal pheochromocytomas) are closely related, though less common, tumors that originate in the ganglia of the sympathetic nervous system and are named based upon the primary anatomical site of origin.

SDHAF2

succinate dehydrogenase assembly factor 2
Other genes related to familial paraganglioma are SDHAF2, VHL, NF1, TMEM127, MAX and SLC25A11.
Mutations in this gene are associated with paraganglioma.

Succinate dehydrogenase complex subunit C

SDHCC560_HUMANSDHC (gene)
Mutations of the genes for the succinate dehydrogenase, SDHD (previously known as PGL1), SDHA, SDHC (previously PGL3) and SDHB have been identified as causing familial head and neck paragangliomas.
Mutations in this gene have been associated with paragangliomas.

Succinate dehydrogenase

Complex IISDHsuccinate - coenzyme Q reductase
Mutations of the genes for the succinate dehydrogenase, SDHD (previously known as PGL1), SDHA, SDHC (previously PGL3) and SDHB have been identified as causing familial head and neck paragangliomas.
Loss-of-function mutations result in paraganglioma, a neuroendocrine tumour.

SDHB

succinate dehydrogenase deficient
Mutations of the genes for the succinate dehydrogenase, SDHD (previously known as PGL1), SDHA, SDHC (previously PGL3) and SDHB have been identified as causing familial head and neck paragangliomas.
Germline mutations in the gene can cause familial paraganglioma (in old nomenclature, Paraganglioma Type PGL4).

SDHD

DHSD_HUMAN
Mutations of the genes for the succinate dehydrogenase, SDHD (previously known as PGL1), SDHA, SDHC (previously PGL3) and SDHB have been identified as causing familial head and neck paragangliomas.
Mutations in the SDHD gene can cause familial paraganglioma.

Mitochondrial 2-oxoglutarate/malate carrier protein

SLC25A11
Other genes related to familial paraganglioma are SDHAF2, VHL, NF1, TMEM127, MAX and SLC25A11.
Inactivating mutations in this gene predispose to metastasic paraganglioma.

SDHA

SDH1
Mutations of the genes for the succinate dehydrogenase, SDHD (previously known as PGL1), SDHA, SDHC (previously PGL3) and SDHB have been identified as causing familial head and neck paragangliomas.
SDHA is a tumour suppressor gene, and heterozygous carriers have an increased risk of paragangliomas as well as pheochromocytomas and renal cancer.

Zellballen

Individual tumor cells are polygonal to oval and are arranged in distinctive cell balls, called Zellballen.
A zellballen pattern is diagnostic for paraganglioma or pheochromocytoma.

TMEM127

Other genes related to familial paraganglioma are SDHAF2, VHL, NF1, TMEM127, MAX and SLC25A11.
TMEM127 is a tumor suppressor gene, inactivating germline mutations in which causes hereditary pheochromocytoma and paraganglioma.

Neural cell adhesion molecule

CD56NCAMNCAM1
With immunohistochemistry, the chief cells located in the cell balls are positive for chromogranin, synaptophysin, neuron specific enolase, serotonin, neurofilament and Neural cell adhesion molecule; they are S-100 protein negative.

Gangliocytic paraganglioma

A gangliocytic paraganglioma is a rare tumour that is typically found in the duodenum and consists of three components: (1) ganglion cells, (2) epithelioid cells (paraganglioma-like) and, (3) spindle cells (schwannoma-like).

Neuroendocrine tumor

neuroendocrine tumorsneuroendocrine tumourneuroendocrine cancer
A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). By light microscopy, the differential diagnosis includes related neuroendocrine tumors, such as carcinoid tumor, neuroendocrine carcinoma, and medullary carcinoma of the thyroid.
The NCI has guidelines for several categories of NET: islet cell tumors of the pancreas, gastrointestinal carcinoids, Merkel cell tumors and pheochromocytoma/paraganglioma.

Carotid body

carotid bodiescarotidcarotid body cell
These cells normally act as special chemoreceptors located along blood vessels, particularly in the carotid bodies (at the bifurcation of the common carotid artery in the neck) and in aortic bodies (near the aortic arch).
A paraganglioma is a tumor that may involve the carotid body and is usually benign.

Aortic body

aortic bodiesaorticaortic baroreflex
These cells normally act as special chemoreceptors located along blood vessels, particularly in the carotid bodies (at the bifurcation of the common carotid artery in the neck) and in aortic bodies (near the aortic arch).
A paraganglioma is a tumor that may involve the aortic body.

Glomus cell

glomus (type I) cellsglomus cellsglomus type I chemoreceptor cells
Paragangliomas originate from paraganglia in chromaffin-negative glomus cells derived from the embryonic neural crest, functioning as part of the sympathetic nervous system (a branch of the autonomic nervous system).
Neoplasms of glomus cells are known as paraganglioma, among other names, they are generally non-malignant.

Neoplasm

tumortumorstumour
A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen).

Cancer

cancersmalignanciescancerous
Unlike other types of cancer, there is no test that determines benign from malignant tumors; long-term followup is therefore recommended for all individuals with paraganglioma.

Catecholamine

catecholaminescatecholamine synthesiscatecholamine systems
Most paragangliomas are either asymptomatic or present as a painless mass. While all contain neurosecretory granules, only in 1–3% of cases is secretion of hormones such as catecholamines abundant enough to be clinically significant; in that case manifestations often resemble those of pheochromocytomas (intra-medullary paraganglioma).

Multiple endocrine neoplasia

multiple endocrine neoplasia syndromeMultiple endocrine adenomatosisMEN
Paragangliomas may also occur in MEN type 2A and 2B.

Von Hippel–Lindau tumor suppressor

von Hippel-Lindau tumor suppressorVHLpVHL
Other genes related to familial paraganglioma are SDHAF2, VHL, NF1, TMEM127, MAX and SLC25A11.

Neurofibromin 1

NF1neurofibromin
Other genes related to familial paraganglioma are SDHAF2, VHL, NF1, TMEM127, MAX and SLC25A11.

MAX (gene)

MAX
Other genes related to familial paraganglioma are SDHAF2, VHL, NF1, TMEM127, MAX and SLC25A11.

Sustentacular cell

sustentacularsustentacular (type II) cellssustentacular cells
These cell balls are separated by fibrovascular stroma and surrounded by sustentacular cells.

Carcinoid

carcinoid tumorcarcinoid tumorscarcinoid tumour
By light microscopy, the differential diagnosis includes related neuroendocrine tumors, such as carcinoid tumor, neuroendocrine carcinoma, and medullary carcinoma of the thyroid.