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Coexistent orbital dermatofibrosarcoma protuberans and bilateral lymphoid hyperplasia
1Department of Ophthalmology, University of Wisconsin Medical School, Madison.
Ophthalmic Plastic and Reconstructive Surgery
|January 1, 1987
Summary
Dermatofibrosarcoma protuberans can rarely affect the orbit, presenting as secondary involvement. Multiple orbital masses may indicate concurrent, distinct conditions like reactive lymphoid hyperplasia.
Area of Science:
- Oncology
- Ophthalmology
- Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor with fibrohistiocytic origins.
- Orbital involvement by DFSP is exceptionally uncommon, particularly as a secondary occurrence.
- Reactive lymphoid hyperplasia (RLH) can manifest as orbital masses, often associated with immune system activity.
Observation:
- A 72-year-old male presented with secondary orbital involvement by DFSP, 36 years post-initial forehead tumor removal.
- Multiple bilateral inferior orbital masses were identified in the same patient.
- Serum immunoelectrophoresis showed polyclonal elevations in IgG and IgA.
Findings:
- Pathologic examination confirmed DFSP in the orbit and RLH in the multiple bilateral masses.
- Immunohistochemical analysis supported a fibroblast origin for the DFSP.
- The patient exhibited polyclonal hypergammaglobulinemia (elevated IgG and IgA).
Implications:
- This case highlights the potential for DFSP to cause secondary orbital disease years after initial presentation.
- The simultaneous presence of DFSP and RLH in the orbit is a unique finding.
- Multiple orbital masses necessitate a comprehensive diagnostic approach to rule out distinct underlying pathologies.