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Endolymphatic sac tumor

MedGen UID:
389657
•Concept ID:
C2348239
•
Neoplastic Process
Synonyms: Aggressive papillary middle ear tumor; Aggressive papillary middle ear tumour; aggressive papillary tumor of the temporal bone; aggressive papillary tumour of the temporal bone; ELST; endolymphatic sac neoplasm; endolymphatic sac neoplasm (disease); Endolymphatic Sac Tumor; endolymphatic sac tumor; endolymphatic sac tumor (disease); Endolymphatic sac tumour; endolymphatic sac tumour (disease); Heffner Tumor; Heffner tumor; Heffner tumour; Low Grade Papillary Adenocarcinoma of Endolymphatic Sac Origin; Low-grade adenocarcinoma of endolymphatic sac origin; Low-Grade Papillary Adenocarcinoma of Endolymphatic Sac Origin; Neoplasm of endolymphatic sac; neoplasm of endolymphatic sac; tumor of endolymphatic sac; tumour of endolymphatic sac
SNOMED CT: Neoplasm of endolymphatic sac (699817008); Endolymphatic sac tumor (699817008)
 
HPO: HP:0030393
Monarch Initiative: MONDO:0006190

Definition

A low-grade papillary epithelial neoplasm (adenocarcinoma) with a slow growth pattern. The endolymphatic duct emerges from the posterior wall of the saccule (of the inner ear) and ends in a blind pouch, the endolymphatic sac. Endolymphatic sac tumors (ELSTs) are known under different names in the literature (Heffner tumor, aggressive papillary middle ear tumor, and low-grade adenocarcinoma of endolymphatic sac origin). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEndolymphatic sac tumor

Professional guidelines

PubMed

Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes.
Tang JD, Grady AJ, Nickel CJ, Ryan LE, Malone A, Canvasser L, Boyev KP
Otolaryngol Head Neck Surg 2023 Mar;168(3):282-290. Epub 2023 Jan 27 doi: 10.1177/01945998221108313. PMID: 35763364
Clinical features and treatment of endolymphatic sac tumor.
Bae SH, Kim SS, Kwak SH, Jung JS, Choi JY, Moon IS
Acta Otolaryngol 2020 Jun;140(6):433-437. Epub 2020 Feb 18 doi: 10.1080/00016489.2020.1722855. PMID: 32068496
Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone.
Mendenhall WM, Suárez C, Skálová A, Strojan P, Triantafyllou A, Devaney KO, Williams MD, Rinaldo A, Ferlito A
Adv Ther 2018 Jul;35(7):887-898. Epub 2018 Jun 19 doi: 10.1007/s12325-018-0730-0. PMID: 29923043Free PMC Article

Recent clinical studies

Etiology

Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes.
Tang JD, Grady AJ, Nickel CJ, Ryan LE, Malone A, Canvasser L, Boyev KP
Otolaryngol Head Neck Surg 2023 Mar;168(3):282-290. Epub 2023 Jan 27 doi: 10.1177/01945998221108313. PMID: 35763364
Surgical management of endolymphatic sac tumor: classification, outcomes and strategy. A single institution's experience.
Wu N, Ma X, Shen W, Hou Z, Han W, Dai P, Zhao H, Huang D, Han D, Yang S
Eur Arch Otorhinolaryngol 2023 Jan;280(1):69-76. Epub 2022 Jun 23 doi: 10.1007/s00405-022-07447-y. PMID: 35739425
The endolymphatic sac tumor: challenges in the eradication of a localized disease.
Sykopetrites V, Piras G, Giannuzzi A, Caruso A, Taibah A, Sanna M
Eur Arch Otorhinolaryngol 2021 Jul;278(7):2297-2304. Epub 2020 Sep 5 doi: 10.1007/s00405-020-06323-x. PMID: 32889625
Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone.
Mendenhall WM, Suárez C, Skálová A, Strojan P, Triantafyllou A, Devaney KO, Williams MD, Rinaldo A, Ferlito A
Adv Ther 2018 Jul;35(7):887-898. Epub 2018 Jun 19 doi: 10.1007/s12325-018-0730-0. PMID: 29923043Free PMC Article
A blue middle ear mass: Cholesterol granuloma mimicking a glomus tumor and endolymphatic sac tumor.
Olcott C, Strasnick B
Am J Otolaryngol 2017 Jan-Feb;38(1):100-102. Epub 2016 Sep 28 doi: 10.1016/j.amjoto.2016.09.013. PMID: 27931523

Diagnosis

Endolymphatic sac tumor mimicking an aneurysmal bone cyst.
Dier C, Abath Neto OL, Policeni B, Freitas LF
Arq Neuropsiquiatr 2024 Oct;82(10):1-3. Epub 2024 Aug 31 doi: 10.1055/s-0044-1789202. PMID: 39216488Free PMC Article
Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes.
Tang JD, Grady AJ, Nickel CJ, Ryan LE, Malone A, Canvasser L, Boyev KP
Otolaryngol Head Neck Surg 2023 Mar;168(3):282-290. Epub 2023 Jan 27 doi: 10.1177/01945998221108313. PMID: 35763364
The endolymphatic sac tumor: challenges in the eradication of a localized disease.
Sykopetrites V, Piras G, Giannuzzi A, Caruso A, Taibah A, Sanna M
Eur Arch Otorhinolaryngol 2021 Jul;278(7):2297-2304. Epub 2020 Sep 5 doi: 10.1007/s00405-020-06323-x. PMID: 32889625
Von Hippel-Lindau disease.
Chittiboina P, Lonser RR
Handb Clin Neurol 2015;132:139-56. doi: 10.1016/B978-0-444-62702-5.00010-X. PMID: 26564077Free PMC Article
Endolymphatic sac tumor.
Banerjee A, Whyte A, O'Sullivan P, Atlas MD
Otol Neurotol 2005 Jul;26(4):819-20. doi: 10.1097/01.mao.0000178143.06387.44. PMID: 16015191

Therapy

Iatrogenic inner ear dehiscence associated with lateral skull base surgery: a systematic analysis of drilling injuries and their causal factors.
Ben-Shlomo N, Jayender J, Guenette JP, Corrales CE
Acta Neurochir (Wien) 2023 Oct;165(10):2969-2977. Epub 2023 Jul 11 doi: 10.1007/s00701-023-05695-3. PMID: 37430067Free PMC Article
Clinical response to pazopanib in a patient with endolymphatic sac tumor not associated with von Hippel-Lindau syndrome.
Nelson T, Hu J, Bannykh S, Fan X, Rudnick J, Vail E
CNS Oncol 2020 Mar 1;9(1):CNS50. Epub 2020 Mar 6 doi: 10.2217/cns-2019-0019. PMID: 32141326Free PMC Article

Prognosis

Surgical management of endolymphatic sac tumor: classification, outcomes and strategy. A single institution's experience.
Wu N, Ma X, Shen W, Hou Z, Han W, Dai P, Zhao H, Huang D, Han D, Yang S
Eur Arch Otorhinolaryngol 2023 Jan;280(1):69-76. Epub 2022 Jun 23 doi: 10.1007/s00405-022-07447-y. PMID: 35739425
The endolymphatic sac tumor: challenges in the eradication of a localized disease.
Sykopetrites V, Piras G, Giannuzzi A, Caruso A, Taibah A, Sanna M
Eur Arch Otorhinolaryngol 2021 Jul;278(7):2297-2304. Epub 2020 Sep 5 doi: 10.1007/s00405-020-06323-x. PMID: 32889625
A blue middle ear mass: Cholesterol granuloma mimicking a glomus tumor and endolymphatic sac tumor.
Olcott C, Strasnick B
Am J Otolaryngol 2017 Jan-Feb;38(1):100-102. Epub 2016 Sep 28 doi: 10.1016/j.amjoto.2016.09.013. PMID: 27931523
Nonparaganglioma jugular foramen tumors.
Thomas AJ, Wiggins RH 3rd, Gurgel RK
Otolaryngol Clin North Am 2015 Apr;48(2):343-59. Epub 2015 Feb 4 doi: 10.1016/j.otc.2014.12.008. PMID: 25659512
Papillary endolymphatic sac tumor: Catastrophic presentation in a child.
Tuñón-Pitalúa MC, Domínguez-de la Ossa L, Alcalá-Cerra G, Baena-del Valle J
Acta Otorrinolaringol Esp 2015 Jan-Feb;66(1):52-5. Epub 2013 Sep 21 doi: 10.1016/j.otorri.2013.06.002. PMID: 24060358

Clinical prediction guides

Impact of an Endolymphatic Sac Tumor on Subjective and Objective Audiovestibular Function.
Hansen JO, West NC, Cayé-Thomasen P
Otol Neurotol 2022 Sep 1;43(8):956-961. Epub 2022 Aug 4 doi: 10.1097/MAO.0000000000003601. PMID: 35960881
Symptoms and clinical features in patients affected by endolymphatic sac tumor: a systematic review and meta-analysis.
Gioacchini FM, Kaleci S, Chiarella G, Viola P, Pisani D, Scarpa A, Tulli M, Pace A, Iannella G, Re M
Eur Arch Otorhinolaryngol 2022 Nov;279(11):5081-5088. Epub 2022 Jun 15 doi: 10.1007/s00405-022-07469-6. PMID: 35704074
The endolymphatic sac tumor: challenges in the eradication of a localized disease.
Sykopetrites V, Piras G, Giannuzzi A, Caruso A, Taibah A, Sanna M
Eur Arch Otorhinolaryngol 2021 Jul;278(7):2297-2304. Epub 2020 Sep 5 doi: 10.1007/s00405-020-06323-x. PMID: 32889625
Nonparaganglioma jugular foramen tumors.
Thomas AJ, Wiggins RH 3rd, Gurgel RK
Otolaryngol Clin North Am 2015 Apr;48(2):343-59. Epub 2015 Feb 4 doi: 10.1016/j.otc.2014.12.008. PMID: 25659512
von Hippel-Lindau disease.
Singh AD, Shields CL, Shields JA
Surv Ophthalmol 2001 Sep-Oct;46(2):117-42. doi: 10.1016/s0039-6257(01)00245-4. PMID: 11578646

Recent systematic reviews

Surgical Management of Endolymphatic Sac Tumor: A Systematic Review and Meta-Analysis.
Gioacchini FM, Chiarella G, Kaleci S, Iannella G, Viola P, Pisani D, Scarpa A, Tulli M, Re M
J Int Adv Otol 2023 Jun;19(3):248-254. doi: 10.5152/iao.2023.22957. PMID: 37272644Free PMC Article
Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes.
Tang JD, Grady AJ, Nickel CJ, Ryan LE, Malone A, Canvasser L, Boyev KP
Otolaryngol Head Neck Surg 2023 Mar;168(3):282-290. Epub 2023 Jan 27 doi: 10.1177/01945998221108313. PMID: 35763364
Symptoms and clinical features in patients affected by endolymphatic sac tumor: a systematic review and meta-analysis.
Gioacchini FM, Kaleci S, Chiarella G, Viola P, Pisani D, Scarpa A, Tulli M, Pace A, Iannella G, Re M
Eur Arch Otorhinolaryngol 2022 Nov;279(11):5081-5088. Epub 2022 Jun 15 doi: 10.1007/s00405-022-07469-6. PMID: 35704074

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