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Paraganglioma

A paraganglioma is a rare neoplasm that can be found in the head and neck region and other less common areas. They are usually considered benign and complete surgical removal results in cure. However, in about 3% of cases they are malignant and have the ability to metastasize. Paragangliomas are still sometimes called glomus tumors (not to be confused with glomus tumors of the skin) and chemodectomas, but paraganglioma is the currently accepted and preferred term. more...

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Medicines

Paragangliomas arise from the glomus cells, which are special chemoreceptors located along blood vessels that have a role in regulating blood pressure and blood flow. The main concentration of glomus cells are found are in the carotid body, located in the upper neck at the branching of the common carotid artery, and the aortic bodies, located near the aortic arch. The glomus cells are a part of the paraganglion system composed of the extra-adrenal paraganglia of the autonomic nervous system, derived from the embryonic neural crest. Thus, paragangliomas are a type of neuroendocrine tumor, and are closely related to pheochromocytomas. Although all paragangliomas contain neurosecretory granules, only about 1-3% have clinical evidence of oversecretion.

Paragangliomas are found predominantly in the abdomen (85%) and the thorax (12%), and only 3% are found in the head and neck region. Most occur as single tumors. When they occur in multiple sites they are usually found as a part of a heritable syndrome such as multiple endocrine neoplasia types II-A and II-B and Carney's complex.

According to the World Health Organization classification of neuroendocrine tumors, paragangliomas are classified as having a neural cell line of origin. In the categorization proposed by Wick, the paragangliomas belong to Group II.

Inheritance

Familial paragangliomas account for approx. 25% of cases, are often multiple and bilateral, and occur at an earlier age. Mutations of the genes SDHD (previously known as PGL1), PGL2, and SDHC (previously PGL3) have been identified as causing familial head and neck paragangliomas. Mutations of SDHB play an important role in familial adrenal pheochromocytoma and extra-adrenal paraganglioma (of abdomen and thorax), although there is considerable overlap in the types of tumors associated with SDHB and SDHD gene mutations.

Pathology

The paragangliomas appear grossly as sharply circumscribed polypoid masses and they have a firm to rubbery consistency. They are highly vascular tumors and may have a deep red color.

On microscopic inspection, the tumor cells are readily recognized. Individual tumor cells are polygonal to oval and are arranged in distinctive cell balls, called Zellballen. These cell balls are separated by fibrovascular stroma and surrounded by sustenacular cells.

By light microscopy, the differential diagnosis includes related neuroendocrine tumors, such as carcinoid tumor, neuroendocrine carcinoma, and medullary carcinoma of the thyroid; middle ear adenoma; and meningioma.

With immunohistochemistry, the chief cells located in the cell balls are positive for chromogranin, synaptophysin, neuron specific enolase, serotonin and neurofilament; they are S-100 protein negative. The sustenacular cells are S-100 positive and focally positive for glial fibrillary acid protein. By histochemistry, the paraganglioma cells are argyrophilic, periodic acid Schiff negative, mucicarmine negative, and argentaffin negative.

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Pathology of Adrenal & Extra-Adrenal Paraganglia $39.88 Clinical impact of somatostatin receptor scintigraphy in the management of paragangliomas of the head and neck : An article from: The Journal of Nuclear Medicine $20.00
Imaging case study of the month: FDG PET imaging of malignant paraganglioma of the neck : An article from: The Annals of Otology, Rhinology & Laryngology $10.00 Cauda equina paraganglioma presenting with intracranial hypertension: case report and review of the literature.(Case Report) : An article from: Neurology India $5.95
Paraganglioma of the cervical sympathetic trunk : An article from: The Annals of Otology, Rhinology & Laryngology $10.00 Pitfalls in imaging: differentiating intravagal and carotid body paragangliomas. : An article from: Ear, Nose and Throat Journal $5.95
Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): Natural history, adrenocortical component, and possible famiilial ... : An article from: Mayo Clinic Proceedings $20.00 Glomus jugulare tumors interpretation of clinical and laboratory findings selection of therapy: A self-instructional package from the Committee on Continuing ... self-instructional packages)
The Paraganglionic Chemoreceptor System: Physiology, Pathology and Clinical Medicine $337.00 Head and neck paragangliomas: A clinical genetic and pathological study of glomus tumors

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Gangliocytic paraganglioma (GP) is a rare neoplasm described almost exclusively in the gastrointestinal tract, especially the periampullary region. However, ...
Pigmented cardiac paraganglioma
A pigmented left atrial paraganglioma was found at autopsy in a 40-year-old black man who died unexpectedly. The cause of death was ascribed to coronary ...
Paraganglioma of the tongue
Paragangliomas are neuroendocrine tumors arising from extra-adrenal autonomic ganglia. We present what is to our knowledge the first immunohistochemically ...
Intrasellar paraganglioma presenting as nonfunctioning pituitary adenoma
Paragangliomas arising in the sellar region are rare. We report a case of intrasellar paraganglioma of a 54-year-old man who presented with gradually ...

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