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Chemodectoma(CBT1)

MedGen UID:
2853
Concept ID:
C0007279
Neoplastic Process
Synonyms: Carotid body paraganglioma; CAROTID BODY TUMORS; 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

Paragangliomas, also referred to as 'glomus body tumors,' are tumors derived from paraganglia located throughout the body. Nonchromaffin types primarily serve as chemoreceptors (hence, the tumor name 'chemodectomas') and are located in the head and neck region (i.e., carotid body, jugular, vagal, and tympanic regions), whereas chromaffin types have endocrine activity, conventionally referred to as 'pheochromocytomas,' and are usually located below the head and neck (i.e., adrenal medulla and pre- and paravertebral thoracoabdominal regions). PGL can manifest as nonchromaffin head and neck tumors only, adrenal and/or extraadrenal pheochromocytomas only, or a combination of the 2 types of tumors (Baysal, 2002; Neumann et al., 2004). The triad of gastric leiomyosarcoma, pulmonary chondroma, and extraadrenal paraganglioma constitutes a syndrome that occurs mainly in young women and is known as the Carney triad (604287). This triad is not to be confused with the other Carney syndrome of myxoma, spotty pigmentation, and endocrinopathy (160980). Baysal (2008) provided a review of the molecular pathogenesis of hereditary paraganglioma. Genetic Heterogeneity of Paragangliomas See also PGL4 (115310), caused by mutation in the SDHB gene (185470) on chromosome 1p36; PGL3 (605373), caused by mutation in the SDHC gene (602413) on chromosome 1q21; PGL2 (601650), caused by mutation in the SDHAF2 gene (613019) on chromosome 11q13; PGL5 (614165), caused by mutation in the SDHA gene (600857) on chromosome 5p15; PGL6 (618464), caused by mutation in the SLC25A11 gene (604165) on chromosome 17p13; and PGL7 (618475), caused by mutation in the DLST gene (126063) on chromosome 14q24. [from OMIM]

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

Powell S, Peters N, Harmer C
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Recent clinical studies

Etiology

Butt N, Baek WK, Lachkar S, Iwanaga J, Mian A, Blaak C, Shah S, Griessenauer C, Tubbs RS, Loukas M
Br J Neurosurg 2019 Oct;33(5):500-503. Epub 2019 May 27 doi: 10.1080/02688697.2019.1617404. PMID: 31130023
Spina A, Boari N, Gagliardi F, Bailo M, Del Vecchio A, Bolognesi A, Mortini P
Head Neck 2018 Dec;40(12):2677-2684. Epub 2018 Nov 20 doi: 10.1002/hed.25517. PMID: 30456888
Motus IY, Bazhenov AV, Massard G
Asian Cardiovasc Thorac Ann 2015 Sep;23(7):846-50. Epub 2015 Jun 12 doi: 10.1177/0218492315592072. PMID: 26071604
Ribet ME, Cardot GR
Ann Thorac Surg 1994 Oct;58(4):1091-5. doi: 10.1016/0003-4975(94)90464-2. PMID: 7944757
Powell S, Peters N, Harmer C
Int J Radiat Oncol Biol Phys 1992;22(5):919-24. doi: 10.1016/0360-3016(92)90788-j. PMID: 1313406

Diagnosis

Jadhav SS, Dhok AP, Mitra KR
Pan Afr Med J 2023;44:182. Epub 2023 Apr 19 doi: 10.11604/pamj.2023.44.182.38636. PMID: 37484598Free PMC Article
Bryant JP, Wang S, Niazi T
Adv Exp Med Biol 2020;1296:151-162. doi: 10.1007/978-3-030-59038-3_9. PMID: 34185291
Butt N, Baek WK, Lachkar S, Iwanaga J, Mian A, Blaak C, Shah S, Griessenauer C, Tubbs RS, Loukas M
Br J Neurosurg 2019 Oct;33(5):500-503. Epub 2019 May 27 doi: 10.1080/02688697.2019.1617404. PMID: 31130023
Casarim AL, Tincani AJ, Del Negro A, Aguiar CG, Fanni RV, Martins AS
Sao Paulo Med J 2014;132(3):133-9. Epub 2014 Apr 14 doi: 10.1590/1516-3180.2014.1323452. PMID: 24760216
Schild SE, Foote RL, Buskirk SJ, Robinow JS, Bock FF, Cupps RE, Earle JD
Mayo Clin Proc 1992 Jun;67(6):537-40. doi: 10.1016/s0025-6196(12)60460-1. PMID: 1331629

Therapy

Chen W, Qi J
J Tongji Med Univ 1998;18(4):262-4. doi: 10.1007/BF02886488. PMID: 10806861
Muros MA, Llamas-Elvira JM, Rodríguez A, Ramírez A, Gómez M, Arráez MA, Valéncia E, Vílchez R
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Shapiro B
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Schild SE, Foote RL, Buskirk SJ, Robinow JS, Bock FF, Cupps RE, Earle JD
Mayo Clin Proc 1992 Jun;67(6):537-40. doi: 10.1016/s0025-6196(12)60460-1. PMID: 1331629
Massey V, Wallner K
Cancer 1992 Feb 1;69(3):790-2. doi: 10.1002/1097-0142(19920201)69:3<790::aid-cncr2820690329>3.0.co;2-u. PMID: 1309681

Prognosis

Spina A, Boari N, Gagliardi F, Bailo M, Del Vecchio A, Bolognesi A, Mortini P
Head Neck 2018 Dec;40(12):2677-2684. Epub 2018 Nov 20 doi: 10.1002/hed.25517. PMID: 30456888
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
Motus IY, Bazhenov AV, Massard G
Asian Cardiovasc Thorac Ann 2015 Sep;23(7):846-50. Epub 2015 Jun 12 doi: 10.1177/0218492315592072. PMID: 26071604
Spratt JS, Meyer JS, Spratt JA
J Surg Oncol 1996 Jan;61(1):68-83. doi: 10.1002/1096-9098(199601)61:1<68::aid-jso2930610102>3.0.co;2-e. PMID: 8544465
Ribet ME, Cardot GR
Ann Thorac Surg 1994 Oct;58(4):1091-5. doi: 10.1016/0003-4975(94)90464-2. PMID: 7944757

Clinical prediction guides

Aubertine CL, Flieder DB
Ann Diagn Pathol 2004 Aug;8(4):237-41. doi: 10.1053/j.anndiagpath.2004.04.008. PMID: 15290677
Spratt JS, Meyer JS, Spratt JA
J Surg Oncol 1996 Jan;61(1):68-83. doi: 10.1002/1096-9098(199601)61:1<68::aid-jso2930610102>3.0.co;2-e. PMID: 8544465
Ribet ME, Cardot GR
Ann Thorac Surg 1994 Oct;58(4):1091-5. doi: 10.1016/0003-4975(94)90464-2. PMID: 7944757
Handel SF, Miller MH, Wallace S, Goepfert H, Jesse RH
AJR Am J Roentgenol 1977 Sep;129(3):477-80. doi: 10.2214/ajr.129.3.477. PMID: 197843
Lacquet LK, Moulijn AC, Jongerius CM, Limburg M, Rensing JB
Thorax 1977 Apr;32(2):203-9. doi: 10.1136/thx.32.2.203. PMID: 194350Free PMC Article

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