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CC BY 4.0 · Arq Neuropsiquiatr 2024; 82(10): s00441789202
DOI: 10.1055/s-0044-1789202
Images in Neurology

Endolymphatic sac tumor mimicking an aneurysmal bone cyst

Tumor do saco endolinfático mimetizando um cisto ósseo aneurismático
Carlos Dier 
1   University of Iowa Hospitals and Clinics, Department of Neurology, Iowa City IA, United States.
,
Osorio Lopes Abath Neto 
2   University of Iowa Hospitals and Clinics, Department of Pathology, Iowa City IA, United States.
,
Bruno Policeni 
3   University of Iowa Hospitals and Clinics, Department of Radiology, Division of Neuroradiology, Iowa City IA, United States.
,
Leonardo Furtado Freitas 
3   University of Iowa Hospitals and Clinics, Department of Radiology, Division of Neuroradiology, Iowa City IA, United States.
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A 61-year-old female patient presented with a large, right temporal mass causing hearing loss and vertigo. Imaging studies revealed multiple cysts, spiculated lesions, and heterogenous enhancement ([Figures 1],[2],[3]). Soft-tissue components and epithelial cysts ([Figure 4]) were recognized after surgery.

Zoom Image
Figure 1 Skull x-ray anteroposterior (A) and lateral (B) views showing a large predominantly cystic lesion in the right temporo-occipital region, with soft tissue component and extensive erosion of the mastoid trabeculae (dashed white circles). Superficial soft-tissue grayscale (C) and doppler (D) ultrasonography with anechoic content, multiple septations and mild vascularization.
Zoom Image
Figure 2 High-resolution computed tomography images of the temporal bones (A-F). Giant destructive osseous lesion centered in the right mastoid and occipital bones. Multiple spiculated appearance and multicystic soft-tissue component (white asterisks) can be observed.
Zoom Image
Figure 3 Brain magnetic resonance imaging dedicated to posterior fossa evaluation. Coronal T1-weighted non-contrast (A), axial fluid-attenuated inversion recovery (B-C), axial T2-weighted (D-E), diffusion (F), apparent diffusion coefficient map (G), susceptibility-weighted imaging (H), and fat saturation T1 non-contrast (I), as well as fat saturation T1-weighted postgadolinium (J) images. Greater conspicuity of the heterogeneous cystic component, with variable signal due to hyper proteinaceous and hemorrhagic contents. There was facilitated diffusion and no significant enhancement.
Zoom Image
Figure 4 Hematoxylin and eosin-stained slides (A and C, 200X; B, 400X, D, 40X) show multiple cysts lined by a single layer of epithelium (A, arrows), focally forming pseudopapillary arrangements (B). Cysts were associated with extensive hemorrhage and hemosiderin deposition, in many areas undermining the epithelium (C, arrows). Cholesterol clefts (D, arrows), morphologic indicators of longstanding tissue reaction, were present in cystic areas and within reactive soft tissue infiltrated by the tumor. Immunohistochemical stains show that the neoplastic cells are positive for pan-keratin (C, 100X) and PAX8 (D, 200X).

Endolymphatic sac tumors (ELSTs) are rare adenomatous neoplasms from the vestibular aqueduct's endolymphatic tissue.[1] These tumors are usually associated with Von Hippel Lindau disease.[2] Paragangliomas and hemangiomas should be included in the differential.[3] Endolymphatic sac tumors can mimic the imaging features of aneurysmal bone cysts.[4] Nevertheless, bone spicules and posterior petrous rim expansion on computed tomography and spontaneous hyperintense regions on magnetic resonance imaging suggest ELST.[5]


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Conflict of Interest

The authors have no conflict of interest to declare.

Editor-in-Chief

Ayrton Roberto Massaro.


Associate Editor

Antonio José da Rocha.


Authors' Contributions

CD, OLAN, BP: conceptualization and validation; LFF: conceptualization, supervision, and writing – review & editing.


  • References

  • 1 Talukdar R, Epari S, Sahay A. et al. Endolymphatic sac tumor: single-institution series of seven cases with updated review of literature. Eur Arch Otorhinolaryngol 2022; 279 (05) 2591-2598
    CrossrefPubMedSuche in Google Scholar
  • 2 Tang JD, Grady AJ, Nickel CJ. et al. Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes. Otolaryngol Head Neck Surg 2023; 168 (03) 282-290
    CrossrefPubMedSuche in Google Scholar
  • 3 Wick CC, Manzoor NF, Semaan MT, Megerian CA. Endolymphatic sac tumors. Otolaryngol Clin North Am 2015; 48 (02) 317-330
    CrossrefPubMedSuche in Google Scholar
  • 4 Alkonyi B, Günthner-Lengsfeld T, Rak K, Nowak J, Solymosi L, Hagen R. An endolymphatic sac tumor with imaging features of aneurysmal bone cysts: differential diagnostic considerations. Childs Nerv Syst 2014; 30 (09) 1583-1588
    CrossrefPubMedSuche in Google Scholar
  • 5 Le H, Zhang H, Tao W. et al. Clinicoradiologic characteristics of endolymphatic sac tumors. Eur Arch Otorhinolaryngol 2019; 276 (10) 2705-2714
    CrossrefPubMedSuche in Google Scholar

Address for correspondence

Leonardo Furtado Freitas

Publikationsverlauf

Eingereicht: 22. April 2024

Angenommen: 09. Juni 2024

Artikel online veröffentlicht:
31. August 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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Bibliographical Record
Carlos Dier, Osorio Lopes Abath Neto, Bruno Policeni, Leonardo Furtado Freitas. Endolymphatic sac tumor mimicking an aneurysmal bone cyst. Arq Neuropsiquiatr 2024; 82: s00441789202.
DOI: 10.1055/s-0044-1789202

  • References

  • 1 Talukdar R, Epari S, Sahay A. et al. Endolymphatic sac tumor: single-institution series of seven cases with updated review of literature. Eur Arch Otorhinolaryngol 2022; 279 (05) 2591-2598
    CrossrefPubMedSuche in Google Scholar
  • 2 Tang JD, Grady AJ, Nickel CJ. et al. Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes. Otolaryngol Head Neck Surg 2023; 168 (03) 282-290
    CrossrefPubMedSuche in Google Scholar
  • 3 Wick CC, Manzoor NF, Semaan MT, Megerian CA. Endolymphatic sac tumors. Otolaryngol Clin North Am 2015; 48 (02) 317-330
    CrossrefPubMedSuche in Google Scholar
  • 4 Alkonyi B, Günthner-Lengsfeld T, Rak K, Nowak J, Solymosi L, Hagen R. An endolymphatic sac tumor with imaging features of aneurysmal bone cysts: differential diagnostic considerations. Childs Nerv Syst 2014; 30 (09) 1583-1588
    CrossrefPubMedSuche in Google Scholar
  • 5 Le H, Zhang H, Tao W. et al. Clinicoradiologic characteristics of endolymphatic sac tumors. Eur Arch Otorhinolaryngol 2019; 276 (10) 2705-2714
    CrossrefPubMedSuche in Google Scholar

   Lizenzen und Reprints
Zoom Image
Figure 1 Skull x-ray anteroposterior (A) and lateral (B) views showing a large predominantly cystic lesion in the right temporo-occipital region, with soft tissue component and extensive erosion of the mastoid trabeculae (dashed white circles). Superficial soft-tissue grayscale (C) and doppler (D) ultrasonography with anechoic content, multiple septations and mild vascularization.
Zoom Image
Figure 2 High-resolution computed tomography images of the temporal bones (A-F). Giant destructive osseous lesion centered in the right mastoid and occipital bones. Multiple spiculated appearance and multicystic soft-tissue component (white asterisks) can be observed.
Zoom Image
Figure 3 Brain magnetic resonance imaging dedicated to posterior fossa evaluation. Coronal T1-weighted non-contrast (A), axial fluid-attenuated inversion recovery (B-C), axial T2-weighted (D-E), diffusion (F), apparent diffusion coefficient map (G), susceptibility-weighted imaging (H), and fat saturation T1 non-contrast (I), as well as fat saturation T1-weighted postgadolinium (J) images. Greater conspicuity of the heterogeneous cystic component, with variable signal due to hyper proteinaceous and hemorrhagic contents. There was facilitated diffusion and no significant enhancement.
Zoom Image
Figure 4 Hematoxylin and eosin-stained slides (A and C, 200X; B, 400X, D, 40X) show multiple cysts lined by a single layer of epithelium (A, arrows), focally forming pseudopapillary arrangements (B). Cysts were associated with extensive hemorrhage and hemosiderin deposition, in many areas undermining the epithelium (C, arrows). Cholesterol clefts (D, arrows), morphologic indicators of longstanding tissue reaction, were present in cystic areas and within reactive soft tissue infiltrated by the tumor. Immunohistochemical stains show that the neoplastic cells are positive for pan-keratin (C, 100X) and PAX8 (D, 200X).