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Proptosis Caused by Rhabdomyomatous Mesenchymal Hamartomata Occurring in the Orbit

Isabella T Phan1, R Jackson Courtney, Douglas P Marx

  • 1*Department of Ophthalmology, University of California, San Francisco, San Francisco, California; †Department of Ophthalmology, Casey Eye Institute; and ‡Department of Pathology, Oregon Health and Science University, Portland, Oregon, U.S.A.

Ophthalmic Plastic and Reconstructive Surgery
|August 9, 2014
PubMed

Insights

Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare orbital tumor in infants. While typically benign, orbital RMH can exhibit rapid growth, necessitating careful monitoring.

Area of Science:

  • Ophthalmology
  • Pediatric Pathology
  • Pediatric Radiology

Background:

  • Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare benign tumor.
  • Orbital tumors in infants can present with proptosis.
  • Understanding the behavior of rare pediatric orbital masses is crucial.

Observation:

  • Two infants presented with progressive orbital proptosis.
  • MRI revealed homogenous orbital masses isointense with rectus muscles.
  • Histopathology showed spindle cell proliferation with entrapped skeletal muscle.

Findings:

  • Immunochemistry confirmed skeletal muscle origin of the abnormal tissue.
  • The diagnosis was rhabdomyomatous mesenchymal hamartoma (RMH).
  • RMH of the orbit may exhibit rapid growth, unlike cutaneous forms.

Implications:

  • Observation was chosen due to the generally benign nature of RMH.
  • The potential for rapid growth in orbital RMH requires vigilance.
  • This highlights the importance of distinguishing orbital RMH from other pediatric orbital masses.

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