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Angiosarcoma metastatic to the orbit

M A Burnstine1, B R Frueh, V M Elner

  • 1Department of Ophthalmology, W.K. Kellogg Eye Center, Ann Arbor, USA.

Archives of Ophthalmology (Chicago, Ill. : 1960)
|January 1, 1996
PubMed
Summary
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Orbital angiosarcoma, a rare endothelial cell tumor, can present as exophthalmos. This case highlights the challenges in diagnosing and treating this aggressive cancer, even with extensive intervention.

Area of Science:

  • Ophthalmology
  • Oncology
  • Pathology

Background:

  • Angiosarcoma is a rare malignant endothelial cell tumor.
  • Primary orbital angiosarcoma is exceptionally uncommon, with limited case reports available.
  • Orbital tumors can present with varied symptoms, including proptosis and diplopia, necessitating thorough diagnostic evaluation.

Observation:

  • A 46-year-old woman presented with progressive diplopia and right exophthalmos.
  • Initial imaging suggested a cavernous hemangioma, while ultrasound indicated a metastatic lesion.
  • Biopsy confirmed angiosarcoma, histologically similar to a prior breast angiosarcoma.

Findings:

  • The orbital mass was diagnosed as angiosarcoma via histopathology and immunohistochemistry.
  • Despite surgical intervention (subtotal excision and orbital exenteration), the tumor recurred.

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  • The patient succumbed to the disease despite aggressive multidisciplinary treatment.

    • Implications:

    • This case underscores the diagnostic challenges of orbital angiosarcoma, often mimicking benign or metastatic lesions.
    • Aggressive management, including surgery and multidisciplinary care, may not prevent recurrence in advanced orbital angiosarcoma.
    • Further research into the pathogenesis and treatment of orbital angiosarcoma is warranted to improve patient outcomes.