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Hyperostosis in orbital rhabdomyosarcoma

Paolo Nico Aseron Rogelio1, Felice Katrina Trio Ranche2, Mary Rose Pe-Yan2

  • 1Department of Ophthalmology and Visual Sciences, Philippine General Hospital, Manila, Philippines pnarogeliomd@gmail.com.

BMJ Case Reports
|June 20, 2022
PubMed

Insights

Rhabdomyosarcoma, a common pediatric soft-tissue sarcoma, can present as an orbital tumor. This case report details a rare instance of orbital rhabdomyosarcoma causing hyperostosis, a thickening of the skull bone.

Area of Science:

  • Oncology
  • Pediatric Oncology
  • Ophthalmology

Background:

  • Rhabdomyosarcoma is the most frequent soft-tissue sarcoma in children.
  • Orbital rhabdomyosarcoma typically presents as a unilateral lesion, potentially causing bony erosion.
  • Imaging is crucial for diagnosing orbital masses and associated bony changes.

Observation:

  • A preschool girl presented with proptosis and temporal bossing, indicative of an orbital mass.
  • Imaging revealed a well-defined, extraconal orbital mass with associated hyperostosis of the sphenoid bone.
  • This represents the first reported case of hyperostosis linked to orbital rhabdomyosarcoma.

Findings:

  • Histopathology confirmed embryonal rhabdomyosarcoma.
  • The tumor was excised, and the hyperostotic sphenoid bone was debulked.
  • No malignant infiltration into the adjacent bone was detected.

Implications:

  • This case highlights an unusual presentation of orbital rhabdomyosarcoma.
  • Periosteal reaction is suggested as the mechanism behind the observed hyperostosis.
  • Further research may elucidate the relationship between rhabdomyosarcoma and bony changes like hyperostosis.

Keywords:
OphthalmologyPaediatric oncologyPaediatricsPathologyRadiology

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