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Tympanic paraganglioma

MedGen UID:
105375
Concept ID:
C0474820
Neoplastic Process
Synonyms: Glomus tympanicum paraganglioma; Glomus tympanicum tumor
SNOMED CT: Tympanic paraganglioma (253031000); Glomus tympanicum tumor (253031000)
 
HPO: HP:0006715
Monarch Initiative: MONDO:0023682

Definition

A middle ear paraganglioma arising from paraganglia around the tympanum. Signs and symptoms include a mass behind the tympanum, tinnitus, and conductive hearing loss. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTympanic paraganglioma

Conditions with this feature

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.
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

Shrivastava MK, Curran JF, Sheerin F, Mohammadi BJ, Halliday D, MacKeith SAC
Otol Neurotol 2022 Aug 1;43(7):840-844. Epub 2022 Jul 4 doi: 10.1097/MAO.0000000000003557. PMID: 35802032
Hackenberg S, Meyer TJ, Häfner J, Scheich M, Stöth M, Al-Tinawi F, Neun T, Mlynski R, Hagen R, Scherzad A
Eur Arch Otorhinolaryngol 2022 Dec;279(12):5623-5630. Epub 2022 May 5 doi: 10.1007/s00405-022-07416-5. PMID: 35511294Free PMC Article
Boedeker CC, Ridder GJ, Schipper J
Fam Cancer 2005;4(1):55-9. doi: 10.1007/s10689-004-2154-z. PMID: 15883711

Recent clinical studies

Etiology

Hackenberg S, Meyer TJ, Häfner J, Scheich M, Stöth M, Al-Tinawi F, Neun T, Mlynski R, Hagen R, Scherzad A
Eur Arch Otorhinolaryngol 2022 Dec;279(12):5623-5630. Epub 2022 May 5 doi: 10.1007/s00405-022-07416-5. PMID: 35511294Free PMC Article
Sharma PD, Johnson AP, Whitton AC
J Laryngol Otol 1984 Jun;98(6):621-9. doi: 10.1017/s0022215100147188. PMID: 6330253

Diagnosis

Ferjaoui M, Kolsi N, Boughzala W, Kharrat O, Bouatay R, Harrathi K, Elkorbi A, Koubaa J
Pan Afr Med J 2022;43:166. Epub 2022 Dec 2 doi: 10.11604/pamj.2022.43.166.29457. PMID: 36825128Free PMC Article
Shrivastava MK, Curran JF, Sheerin F, Mohammadi BJ, Halliday D, MacKeith SAC
Otol Neurotol 2022 Aug 1;43(7):840-844. Epub 2022 Jul 4 doi: 10.1097/MAO.0000000000003557. PMID: 35802032
Sweeney AD, Carlson ML, Wanna GB, Bennett ML
Otolaryngol Clin North Am 2015 Apr;48(2):293-304. Epub 2015 Feb 4 doi: 10.1016/j.otc.2014.12.004. PMID: 25659513
Blake DM, Tomovic S, Jyung RW
Ear Nose Throat J 2014 Apr-May;93(4-5):136, 138. PMID: 24817221
Boedeker CC, Ridder GJ, Schipper J
Fam Cancer 2005;4(1):55-9. doi: 10.1007/s10689-004-2154-z. PMID: 15883711

Prognosis

Ferjaoui M, Kolsi N, Boughzala W, Kharrat O, Bouatay R, Harrathi K, Elkorbi A, Koubaa J
Pan Afr Med J 2022;43:166. Epub 2022 Dec 2 doi: 10.11604/pamj.2022.43.166.29457. PMID: 36825128Free PMC Article
Shrivastava MK, Curran JF, Sheerin F, Mohammadi BJ, Halliday D, MacKeith SAC
Otol Neurotol 2022 Aug 1;43(7):840-844. Epub 2022 Jul 4 doi: 10.1097/MAO.0000000000003557. PMID: 35802032
Sweeney AD, Carlson ML, Wanna GB, Bennett ML
Otolaryngol Clin North Am 2015 Apr;48(2):293-304. Epub 2015 Feb 4 doi: 10.1016/j.otc.2014.12.004. PMID: 25659513
Sharma PD, Johnson AP, Whitton AC
J Laryngol Otol 1984 Jun;98(6):621-9. doi: 10.1017/s0022215100147188. PMID: 6330253

Clinical prediction guides

Araujo-Castro M, Redondo López S, Pascual-Corrales E, Polo López R, Alonso-Gordoa T, Molina-Cerrillo J, Moreno Mata N, Caballero Silva U, Barberá Durbán R
Ann Endocrinol (Paris) 2023 Aug;84(4):466-471. Epub 2022 Nov 2 doi: 10.1016/j.ando.2022.10.013. PMID: 36334803
Hackenberg S, Meyer TJ, Häfner J, Scheich M, Stöth M, Al-Tinawi F, Neun T, Mlynski R, Hagen R, Scherzad A
Eur Arch Otorhinolaryngol 2022 Dec;279(12):5623-5630. Epub 2022 May 5 doi: 10.1007/s00405-022-07416-5. PMID: 35511294Free PMC Article

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