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Orbital Cellular Epithelioid Hemangioma.

Hillary C Stiefel1, John D Ng1, David J Wilson1

  • 1Department of Ophthalmology, Casey Eye Institute, Oregon Health and Science University, Portland, Oregon, USA.

Ocular Oncology and Pathology
|November 27, 2019
PubMed
Summary

Immunohistochemical stains for FOSB and CAMTA1 can help differentiate benign cellular epithelioid hemangioma (EH) from malignant epithelioid hemangioendothelioma (EHE). This aids in diagnosing rare orbital tumors.

Keywords:
Angiolymphoid hyperplasia with eosinophiliaCytogeneticsEpithelioid hemangioma

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Area of Science:

  • Ophthalmology
  • Pathology
  • Oncology

Background:

  • Orbital tumors are rare, with epithelioid hemangioma (EH) being an uncommon benign vascular neoplasm.
  • Distinguishing between benign EH and malignant epithelioid hemangioendothelioma (EHE) is crucial for appropriate patient management.
  • Recurrent proptosis can indicate orbital mass recurrence or progression.

Purpose of the Study:

  • To present a case of orbital cellular epithelioid hemangioma (EH).
  • To evaluate the utility of FOSB and CAMTA1 immunostains in diagnosing orbital EH.
  • To explore the role of cytogenetic rearrangements in differentiating benign EH from malignant EHE.

Main Methods:

  • A case report detailing a patient with recurrent proptosis and a subsequent orbital mass.
  • Histopathological examination of the orbital mass.
  • Immunohistochemical staining for FOSB and CAMTA1 to detect genetic rearrangements.

Main Results:

  • The orbital mass was characterized as a circumscribed neoplasm of epithelioid endothelial cells.
  • FOSB immunostaining showed multifocal nuclear positivity, indicating a FOSB genetic rearrangement.
  • CAMTA1 immunostaining was negative. These findings supported a diagnosis of benign cellular EH over malignant EHE.

Conclusions:

  • Immunohistochemical detection of cytogenetic rearrangements can assist in differentiating benign EH from malignant EHE.
  • Recognizing the clinical and histopathologic features of orbital EH is important due to its rarity.
  • Clarifying the nomenclature of epithelioid hemangioma is essential for consistent diagnosis and reporting.