carotid body tumor

WordNet

1. invest with or as with a body; give body to (同)personify
2. the external structure of a vehicle; "the body of the car was badly rusted"
3. the main mass of a thing
4. a natural object consisting of a dead animal or person; "they found the body in the lake" (同)dead body
5. the entire structure of an organism (an animal, plant, or human being); "he felt as if his whole body were on fire" (同)organic structure, physical structure
6. a collection of particulars considered as a system; "a body of law"; "a body of doctrine"; "a body of precedents"
7. a group of persons associated by some common tie or occupation and regarded as an entity; "the whole body filed out of the auditorium"; "the student body"; "administrative body"
8. an individual 3-dimensional object that has mass and that is distinguishable from other objects; "heavenly body"
9. the central message of a communication; "the body of the message was short"
10. of or relating to either of the two major arteries supplying blood to the head and neck
11. an abnormal new mass of tissue that serves no purpose (同)tumour, neoplasm

PrepTutorEJDIC

1. 〈C〉『身体』,肉体 / 〈C〉(人・動物の)『胴体』 / 〈C〉)物の)『主要部』,本体《+『of』+『名』》 / 〈C〉(…の)『団体』,群れ:(…のたくさんの)集まり《+『of』+『名』》 / 〈C〉物体,…体 / 〈U〉実質;(酒・味などの)こく / 〈C〉《話》人
2. 頚(けい)動脈 / 頚動脈の
3. 《英話》人;(特に)男

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/03/29 20:16:37」(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

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 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. 頚部腫瘤の鑑別診断 differential diagnosis of a neck mass
• 4. 頚部腫瘤の評価 evaluation of a neck mass
• 5. 傍神経節腫および褐色細胞腫：悪性疾患のマネージメント paraganglioma and pheochromocytoma management of malignant disease

English Journal

• Instability of Succinate Dehydrogenase in SDHD Polymorphism Connects Reactive Oxygen Species Production to Nuclear and Mitochondrial Genomic Mutations in Yeast.

• Chang YL1, Hsieh MH, Chang WW, Wang HY, Lin MC, Wang CP, Lou PJ, Teng SC.
• Antioxidants & redox signaling.Antioxid Redox Signal.2014 Oct 20. [Epub ahead of print]
• Aims: Mitochondrial succinate dehydrogenase (SDH) is an essential complex of the electron transport chain and tricarboxylic acid cycle. Mutations in the human SDH subunit D frequently lead to paraganglioma (PGL), but the mechanistic consequences of the majority of SDHD polymorphisms have yet to be u
• PMID 25328978

• Sleep-Mediated Heart Rate Variability After Bilateral Carotid Body Tumor Resection.

• Niemeijer ND, Corssmit EP, Reijntjes RH, Lammers GJ, van Dijk JG, Thijs RD.
• Sleep.Sleep.2014 Oct 17. pii: sp-00286-14. [Epub ahead of print]
• Study Objectives: The carotid bodies are thought to play an important role in sleep-dependent autonomic changes. Patients who underwent resection of bilateral carotid body tumors have chronically attenuated baroreflex sensitivity. These subjects provide a unique opportunity to investigate the role o
• PMID 25325476

Japanese Journal

• A case report of dislocation of the common carotid artery

• 松本 吉史,横井 秀格,甲能 直幸
• 頭頸部外科 22(2), 169-172, 2012
• … We report on a case of aberrant common carotid artery. … The patient was an 88-year-old-woman with a foreign-body sensation, which got worse when she swallowed. … Pharyngeal tumor was pointed out by a doctor of internal medicine after gastrofiberscopy was performed, and the patient then visited our department. … A CT scan demonstrated an aberrant course of the common carotid artery. …
• NAID 130002136990

• Dopamine-secreting Carotid Body Paragangliomas-Biochemical Control with Radiotherapy

• Soh Abel Wah Ek,Kek Peng Chin
• Internal Medicine 51(6), 613-618, 2012
• … We report a 62-year-old Chinese man with bilateral carotid body tumors which were exclusively dopamine secreting. … The left-sided tumor invaded the skull base and encased the left carotid artery. … Progressive decline and eventual normalization of urinary dopamine excretion was seen together with a slight reduction in tumor size. …
• NAID 130002061918

Related Links

• Carotid Body Tumor

What is a carotid body tumor (also called chemodectomas, paraganglioma)? Two carotid arteries (one on each side of the neck) supply blood to the large, front part of the brain, where thinking, speech, personality and sensory and ...

• carotid body tumor - definition of carotid body tumor by ...

1. A neoplasm, benign or malignant. See Benign tumor, Blue cell tumor, Bone tumor, Borderline tumor, Brain tumor, Carcinoid tumor, Carcinoma ex-mixed tumor, Carotid body tumor, Desmoid tumor, Desmoplastic round cell tumor ...

Related Pictures

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★リンクテーブル★

リンク元	「頚動脈球腫瘍」「頸動脈球腫瘍」
関連記事	「carotid」「body」「tumor」

「頚動脈球腫瘍」

　　[★]

英

carotid body tumor

ラ

tumor glomus caroticum

関

頸動脈球腫瘍、非クロム親和性傍神経節腫

「頸動脈球腫瘍」

　　[★]

英

carotid body tumor

関

頚動脈球腫瘍

「carotid」

　　[★]

• adj.

• 頚動脈の、頸動脈の

関

carotid artery

　 　

「body」

　　[★]

• n.

• 体、本体、身体、ボディー

「tumor」

　　[★]

• n.

• 腫瘍、腫瘤、腫脹