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Rapid progressive extramedullary plasmacytoma in the orbit
P Knecht1, R Schuler, K Chaloupka
1Institute of Ophthalmology, University Hospital Zurich, Switzerland. pascalknecht@gmx.ch
Klinische Monatsblatter Fur Augenheilkunde
|May 6, 2008
Summary
Primary extramedullary plasmacytoma (EMP) of the orbit is rare. This case highlights rapid tumor growth despite standard treatments, emphasizing the need for accurate diagnosis and tailored therapeutic strategies for orbital plasmacytoma.
Area of Science:
- Ophthalmology
- Oncology
- Pathology
Background:
- Orbital plasma cell tumors are rare, often indicating multiple myeloma (MM).
- Primary extramedullary plasmacytoma (EMP) of the orbit is exceptionally rare, with few reported cases.
- Differentiating EMP from MM is crucial due to significant survival differences (9.5 years for EMP vs. 3-4 years for MM).
Observation:
- A 68-year-old man presented with rapidly progressing right orbital extramedullary plasmacytoma.
- MRI revealed a large intraorbital mass (5.2 x 4.7 x 3 cm).
- Biopsy confirmed lambda light chain type plasmacytoma; systemic work-up showed no further tumor spread.
Findings:
- Standard treatments including high-dose irradiation (80 Gy) and chemotherapy were ineffective.
- Surgical exenteration with skin grafting was performed.
- Despite maximal irradiation, the Thiersch graft healed successfully.
Implications:
- Rapid tumor growth, unusual for EMP, was observed, potentially due to poorly differentiated, blastic morphology.
- Specific molecular features like t(4;14) were not identified as a cause.
- This case underscores the aggressive nature some orbital plasmacytomas can exhibit and the challenges in treatment.