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Carotid body paraganglioma(CBT1)

MedGen UID:
2853
Concept ID:
C0007279
Neoplastic Process
Synonyms: CAROTID BODY TUMORS; Chemodectoma; Paraganglioma, carotid body, somatic
SNOMED CT: Neoplasm of carotid body (127028003); Carotid body tumor (30699005); Carotid body paraganglioma (30699005)
 
HPO: HP:0030074
Monarch Initiative: MONDO:0021053
OMIM®: 168000

Definition

A usually benign neoplasm originating in the chemoreceptor tissue of the carotid body, glomus jugulare, glomus tympanicum, aortic bodies, or the female genital tract. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCarotid body paraganglioma

Conditions with this feature

Paragangliomas 3
MedGen UID:
340200
Concept ID:
C1854336
Disease or Syndrome
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Paragangliomas 4
MedGen UID:
349380
Concept ID:
C1861848
Neoplastic Process
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Paragangliomas 2
MedGen UID:
357076
Concept ID:
C1866552
Disease or Syndrome
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Chemodectoma, intraabdominal, with cutaneous angiolipomas
MedGen UID:
443934
Concept ID:
C2930928
Disease or Syndrome
Paragangliomas 1
MedGen UID:
488134
Concept ID:
C3494181
Neoplastic Process
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.

Professional guidelines

PubMed

Moore MG, Netterville JL, Mendenhall WM, Isaacson B, Nussenbaum B
Otolaryngol Head Neck Surg 2016 Apr;154(4):597-605. Epub 2016 Feb 9 doi: 10.1177/0194599815627667. PMID: 26861230

Recent clinical studies

Etiology

Tabb JN, Maas JA, Earla BP, Fallon KB, McDonald AM, Dobelbower MC
Diagn Pathol 2023 Feb 24;18(1):31. doi: 10.1186/s13000-023-01314-y. PMID: 36829189Free PMC Article
Lamblin E, Atallah I, Reyt E, Schmerber S, Magne JL, Righini CA
Eur Ann Otorhinolaryngol Head Neck Dis 2016 Nov;133(5):319-324. Epub 2016 Jun 11 doi: 10.1016/j.anorl.2016.05.006. PMID: 27297087
Moore MG, Netterville JL, Mendenhall WM, Isaacson B, Nussenbaum B
Otolaryngol Head Neck Surg 2016 Apr;154(4):597-605. Epub 2016 Feb 9 doi: 10.1177/0194599815627667. PMID: 26861230
Cvjetko I, Erdelez L, Podvez Z, Buhin M, Vidjak V, Borovecki A, Cvjetko T, Grsic K
J Craniofac Surg 2013 May;24(3):e242-5. doi: 10.1097/SCS.0b013e31828607ef. PMID: 23714977
Işik AC, Erem C, Imamoğlu M, Cinel A, Sari A, Maral G
Eur Arch Otorhinolaryngol 2006 Jan;263(1):23-31. Epub 2005 Nov 30 doi: 10.1007/s00405-004-0885-y. PMID: 16320027

Diagnosis

Abdelhady K, Durgam S, Orza D, Massad MG
Ann Thorac Surg 2017 Apr;103(4):e323-e325. doi: 10.1016/j.athoracsur.2016.09.062. PMID: 28359488
Dorobisz K, Dorobisz T, Temporale H, Zatoński T, Kubacka M, Chabowski M, Dorobisz A, Kręcicki T, Janczak D
Adv Clin Exp Med 2016 Nov-Dec;25(6):1173-1177. doi: 10.17219/acem/61612. PMID: 28028970
Moore MG, Netterville JL, Mendenhall WM, Isaacson B, Nussenbaum B
Otolaryngol Head Neck Surg 2016 Apr;154(4):597-605. Epub 2016 Feb 9 doi: 10.1177/0194599815627667. PMID: 26861230
Manzoor T, Ahmed B, Najam A, Ayub Z
J Coll Physicians Surg Pak 2009 Aug;19(8):523-5. PMID: 19651019
Işik AC, Erem C, Imamoğlu M, Cinel A, Sari A, Maral G
Eur Arch Otorhinolaryngol 2006 Jan;263(1):23-31. Epub 2005 Nov 30 doi: 10.1007/s00405-004-0885-y. PMID: 16320027

Therapy

Gupta SK, Singla S, Karunanithi S, Damle N, Bal C
Clin Nucl Med 2014 May;39(5):440-1. doi: 10.1097/RLU.0000000000000273. PMID: 24217545
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Ear Nose Throat J 2002 Aug;81(8):536-8, 540, 542 passim. PMID: 12199171
Higo R, Asai M, Sugasawa M, Takeuchi N, Nemoto S
Auris Nasus Larynx 1994;21(2):122-5. doi: 10.1016/s0385-8146(12)80031-9. PMID: 7993226
Khafagi F, Egerton-Vernon J, van Doorn T, Foster W, McPhee IB, Allison RW
J Nucl Med 1987 Apr;28(4):528-31. PMID: 2437268
Gruber H, Metson R
Ann Intern Med 1980 Jun;92(6):800-2. doi: 10.7326/0003-4819-92-6-800. PMID: 7387023

Prognosis

Tabb JN, Maas JA, Earla BP, Fallon KB, McDonald AM, Dobelbower MC
Diagn Pathol 2023 Feb 24;18(1):31. doi: 10.1186/s13000-023-01314-y. PMID: 36829189Free PMC Article
Lang F, Jha A, Meuter L, Pacak K, Yang C
Front Endocrinol (Lausanne) 2021;12:731096. Epub 2021 Sep 20 doi: 10.3389/fendo.2021.731096. PMID: 34616365Free PMC Article
Dorobisz K, Dorobisz T, Temporale H, Zatoński T, Kubacka M, Chabowski M, Dorobisz A, Kręcicki T, Janczak D
Adv Clin Exp Med 2016 Nov-Dec;25(6):1173-1177. doi: 10.17219/acem/61612. PMID: 28028970
Lamblin E, Atallah I, Reyt E, Schmerber S, Magne JL, Righini CA
Eur Ann Otorhinolaryngol Head Neck Dis 2016 Nov;133(5):319-324. Epub 2016 Jun 11 doi: 10.1016/j.anorl.2016.05.006. PMID: 27297087
Cvjetko I, Erdelez L, Podvez Z, Buhin M, Vidjak V, Borovecki A, Cvjetko T, Grsic K
J Craniofac Surg 2013 May;24(3):e242-5. doi: 10.1097/SCS.0b013e31828607ef. PMID: 23714977

Clinical prediction guides

Greenberg SE, Holman R, Kohlmann W, Buchmann L, Naumer A
Clin Endocrinol (Oxf) 2021 Sep;95(3):447-452. Epub 2021 Jul 22 doi: 10.1111/cen.14559. PMID: 34255389
Lamblin E, Atallah I, Reyt E, Schmerber S, Magne JL, Righini CA
Eur Ann Otorhinolaryngol Head Neck Dis 2016 Nov;133(5):319-324. Epub 2016 Jun 11 doi: 10.1016/j.anorl.2016.05.006. PMID: 27297087
Moore MG, Netterville JL, Mendenhall WM, Isaacson B, Nussenbaum B
Otolaryngol Head Neck Surg 2016 Apr;154(4):597-605. Epub 2016 Feb 9 doi: 10.1177/0194599815627667. PMID: 26861230
Plukker JT, Brongers EP, Vermey A, Krikke A, van den Dungen JJ
Br J Surg 2001 Oct;88(10):1382-6. doi: 10.1046/j.0007-1323.2001.01878.x. PMID: 11578296
Lord RS, Chambers AJ
Cardiovasc Surg 1999 Jan;7(1):134-8. doi: 10.1016/s0967-2109(98)00005-2. PMID: 10073773

Recent systematic reviews

Jackson RS, Myhill JA, Padhya TA, McCaffrey JC, McCaffrey TV, Mhaskar RS
Otolaryngol Head Neck Surg 2015 Dec;153(6):943-50. Epub 2015 Sep 16 doi: 10.1177/0194599815605323. PMID: 26378186

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