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Related Experiment Video

Updated: May 5, 2026

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Orbital indeterminate cell histiocytosis.

Angela J Oh1, Connie M Sears1, Akhila A Vadivelan2

  • 1Division of Orbital and Ophthalmic Plastic Surgery, Jules Stein Eye Institute, University of California, Los Angeles, California, USA.

Orbit (Amsterdam, Netherlands)
|February 20, 2024
PubMed
Summary

A rare case of indeterminate cell histiocytosis presented as an isolated orbital mass in a child. Treatment with hydroxyurea showed no progression of the lesion.

Keywords:
Langerhans cell histiocytosisOrbithistiocytosisindeterminate cell histiocytosis

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Area of Science:

  • Ophthalmology
  • Pediatric Oncology
  • Dermatology

Background:

  • Indeterminate cell histiocytosis (ICH) is a rare histiocytic disorder.
  • Orbital involvement in ICH is uncommon, especially as an isolated presentation.
  • Early diagnosis and management are crucial for favorable outcomes.

Purpose of the Study:

  • To report a rare case of isolated orbital indeterminate cell histiocytosis in a pediatric patient.
  • To highlight the clinical presentation, diagnostic workup, and treatment of this rare condition.
  • To emphasize the importance of considering ICH in the differential diagnosis of pediatric orbital masses.

Main Methods:

  • A case study of an 8-year-old female with a 3-month history of left upper eyelid fullness and edema.
  • Clinical examination, orbital imaging (MRI), and incisional biopsy with histopathology and immunohistochemistry.
  • Treatment with oral hydroxyurea and follow-up assessment of lesion progression.

Main Results:

  • Histopathology confirmed indeterminate cell histiocytosis with positive CD68 and CD163 staining.
  • The orbital mass showed no change in size after 3 months of hydroxyurea treatment.
  • No systemic involvement or associated dermatologic findings were observed.

Conclusions:

  • Isolated orbital indeterminate cell histiocytosis is a rare entity in children.
  • Orbital imaging and histopathology are key for diagnosis.
  • Hydroxyurea may be an effective treatment option for localized orbital ICH, warranting further investigation.