glomus jugulare

頚静脈小体、頸静脈小体

WordNet

1. relating to or located in the region of the neck or throat; "jugular vein"
2. a vital part that is vulnerable to attack; "he always goes for the jugular"
3. seize upon or latch onto something; "The Republicans glommed onto Whitewater"

PrepTutorEJDIC

1. 頚(けい)部の;頚静脈の / =jugular vein

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/12/11 22:26:18」(JST)

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Paraganglioma
Micrograph of a carotid body tumor (a type of paraganglioma).
Classification and external resources
Specialty	oncology
ICD-10	C75.4, C75.5, D35.5, D35.6, D44.6, D44.7
ICD-9-CM	194.5, 194.6, 227.5, 227.6, 237.3
ICD-O	M8680/0 - M8693/9
DiseasesDB	33480
eMedicine	med/2994
MeSH	D010235

A paraganglioma is rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). About 97% are benign and cured by surgical removal; the remaining 3% are malignant because they are able to produce distant metastases. "Paraganglioma" is now the most-widely accepted term for these lesions, that have been also described as: glomus tumor, chemodectoma, perithelioma, fibroangioma, and sympathetic nevi.

Cellular origin and classification

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). 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).

Accordingly, paragangliomas are categorised as originating from a neural cell line in the World Health Organization classification of neuroendocrine tumors. In the categorization proposed by Wick, paragangliomas belong to group II. Given the fact that they originate from cells of the orthosympathetic system, paragangliomas are closely related to pheochromocytomas, which however are chromaffin-positive.

Clinical presentation

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).

Inheritance

About 75% of paragangliomas are sporadic; the remaining 25% are hereditary (and have an increased likelihood of being multiple and of developing at an earlier age). 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 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. Paragangliomas may also occur in MEN type 2A and 2B. They are seen in at a higher incidence in people living at high altitude. Other genes related to familiar paraganglioma are SDHAF2, VHL, NF1, TMEM127 and MAX.

Pathology

Mediastinal paraganglioma. The cut surface of a 3.9 x 3.5 x 2.5 cm tumor is triangular, with a bulging peripheral portion and a somewhat fibrotic center. It was surrounded by the heart, left lower lobe of the lung, aorta, esophagus, and diaphragm, and had been 1.8 cm in diameter 7 years before.

Micrograph of a carotid body tumor with the characteristic Zellballen. H&E stain.

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 sustentacular cells.

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

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. The sustentacular cells are S-100 positive and focally positive for glial fibrillary acidic protein. By histochemistry, the paraganglioma cells are argyrophilic, periodic acid Schiff negative, mucicarmine negative, and argentaffin negative.

Sites of origin

About 85% of paragangliomas develop in the abdomen; only 12% develop in the chest and 3% in the head and neck region (the latter are the most likely to be symptomatic). While most are single, rare multiple cases occur (usually in a hereditary syndrome). Paragangliomas are described by their site of origin and are often given special names:-

• Carotid paraganglioma (carotid body tumor): Is the most common of the head and neck paragangliomas. It usually presents as a painless neck mass, but larger tumors may cause cranial nerve palsies, usually of the vagus nerve and hypoglossal nerve.
• Glomus tympanicum and Glomus jugulare: Both commonly present as a middle ear mass resulting in tinnitus (in 80%) and hearing loss (in 60%). The cranial nerves of the jugular foramen may be compressed, resulting swallowing difficulty, or ipsilateral weakness of the upper trapezius and sternocleiodomastoid muscles (from compression of the spinal accessory nerve). These patients present with a reddish bulge behind an intact ear drum. This condition is also known as the "Red drum". On application of pressure to the external ear canal with the help of a pneumatic ear speculum the mass could be seen to blanch. This sign is known as "Brown's sign".
• Vagal paraganglioma: These are the least common of the head and neck paragangliomas. They usually present as a painless neck mass, but may result in dysphagia and hoarseness.
• Pulmonary paraganglioma: These occur in the lung and may be either single or multiple.

• Other sites: Rare sites of involvement are the larynx, nasal cavity, paranasal sinuses, thyroid gland, and the thoracic inlet, as well as the bladder in extremely rare cases.

Treatment

The main treatment modalities are surgery, embolization and radiotherapy.

Additional images

• Micrograph of a carotid body tumor.

• Glomus Jugulare Tumor.

• Ectopic functional paraganglioma (glomus jugulare) in a patient with VHL. T2 weighted MRI at the same location demonstrates a high signal mass consistent with a paraganglioma. Extra adrenal paragangliomas can be found in VHL (arrow).

• S100 immunostain highlighting the sustentacular cells in a paraganglioma.

See also

• Gangliocytic paraganglioma
• Pheochromocytoma

References

External links

• Clinical Trial information for patients with Paraganglioma
• (Description with pictures)
• GeneReviews/NCBI/NIH/UW entry on Hereditary Paraganglioma-Pheochromocytoma Syndromes
• (Bilateral Carotid Paraganglioma)

UpToDate Contents

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• 1. 傍神経節腫：疫学、臨床症状、診断、および組織学的検査 paragangliomas epidemiology clinical presentation diagnosis and histology
• 2. 傍神経節腫：限局性疾患の治療 paragangliomas treatment of locoregional disease
• 3. まれな脳腫瘍 uncommon brain tumors
• 4. 成人における難聴の病因 etiology of hearing loss in adults
• 5. 頚部腫瘤の鑑別診断 differential diagnosis of a neck mass

English Journal

• A Rare Case of Glomus Jugulare Tumor Presenting as Papilledema.

• Shah VM1, Prabhu V.
• Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society.J Neuroophthalmol.2015 Jul 28. [Epub ahead of print]
• PMID 26222371

• Multiple primary malignant paraganglioma of the head and neck with lymph node metastasis.

• Yetişer S1, Ramadan S.
• Kulak burun boğaz ihtisas dergisi : KBB = Journal of ear, nose, and throat.Kulak Burun Bogaz Ihtis Derg.2015 Jul-Aug;25(4):232-6. doi: 10.5606/kbbihtisas.2015.06432.
• Paragangliomas of the head and neck are usually benign tumors. Malignant form is quite rare and criteria for malignancy are not clear. Clinical, biochemical and histological features of malignant forms are not sufficient to reliably distinguish them from benign tumors and malignancy is established o
• PMID 26211865

• Lesions involving the jugular foramen: clinical characteristics and surgical management.

• Li W1, Dai C.
• Acta oto-laryngologica.Acta Otolaryngol.2015 Jun;135(6):565-71. doi: 10.3109/00016489.2014.1003094. Epub 2015 Feb 26.
• CONCLUSION: Lesions involving the jugular foramen (JF) present as various diagnoses. Pulsatile tinnitus is more common in glomus jugulare (GJ) tumors, whereas otalgia and facial nerve paresis are more prevalent in temporal bone malignancies (TBMs). Preoperative facial nerve electroneurography (ENoG)
• PMID 25719346

Japanese Journal

• 頸静脈孔傍神経節腫と鑑別困難であった頸静脈孔髄膜腫例

• 林 千江里,古川 正幸,春山 琢男 [他],奈良林 修,飯塚 崇,池田 勝久
• 耳鼻咽喉科臨床 104(8), 553-557, 2011-08-01
• … We report a case preoperatively diagnosed as glomus jugulare involving radical dissection, but determined postoperatively to be jugular foramen meningioma. …
• NAID 10029260321

• Glomus Tumors : When does Controversy become Convention?

• MICCO Alan G.
• Otology Japan 19(1), 33-39, 2009-03-25
• … Introduction<BR>Glomus tumors are rare benign neoplasms in the head and neck. … Glomus tympanicum and glomus jugulare lesions are associated with the temporal bone. … There is no controversy with the management of glomus tympanicums as they are mainly treated with surgical removal. …
• NAID 10025822303

• Middle Ear Carcinoid Tumor : Case Report and Literature Review

• Kanaya Hiroaki,Hirabayashi Hideki,Arakawa Keizoh,Fukami Satoru,Okubo Masaaki,Goto Kazutaka,Konno Wataru,Koizumi Saori,Baba Kohtaro
• Dokkyo journal of medical sciences 33(2), 133-137, 2006-07-25
• … The provisional clinical diagnosis was cholesteatoma or another type of tumor (e.g. glomus jugulare tumor or facial nerve neurilemmoma). …
• NAID 110006406447

Related Links

• Glomus jugulare tumor: MedlinePlus Medical Encyclopedia

A glomus jugulare tumor is a tumor of a part of the temporal bone in the skull. This tumor can affect the ear, upper neck, base of the skull, and the ... Causes A glomus jugulare tumor grows in the temporal bone of the ...

• Glomus jugulare by drtbalu

Synonyms: Paraganglioma, Chemodectoma, Ganglia tympanica, Vascular tumors of middle ear. Definition: Glomus jugulare is defined as a collection of ganglionic tissue within the temporal bone in close relationship with the jugular ...

Related Pictures

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★リンクテーブル★

リンク元	「頚静脈小体」「頸静脈小体」
拡張検索	「glomus jugulare tumor」
関連記事	「jugular」

「頚静脈小体」

　　[★]

ラ

glomus jugulare

関

頸静脈小体

「頸静脈小体」

　　[★]

ラ

glomus jugulare

関

頚静脈小体

「glomus jugulare tumor」

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同

頚静脈腫瘍, chemodectoma

「jugular」

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• adj.

• 頚静脈の、頸静脈の

関

cervical vein、jugular vein、vena jugularis