Castleman's disease in the orbit. A 20-year follow-up.
OBJECTIVE
To report a case of localized orbital Castleman's disease of mixed cell type with a follow-up of 20 years.


METHODS
A female patient presented at the age of 12 years with constitutional symptoms and left-sided proptosis. Laboratory tests revealed marked hypergammaglobulinaemia and high erythrocyte sedimentation rate (ESR), suggesting an immunological disturbance. A CT scan and MRI showed an infiltrating orbital mass lateral to and behind the eye.


RESULTS
Histological examination of orbital biopsies showed a lymphoid lesion consistent with Castleman's disease of the mixed cell type. The patient was treated with systemic steroids, immunosuppressives and irradiation. She is now 33 years old and has been without relapse for the last 7 years.


CONCLUSIONS
Orbital involvement in Castleman's disease is very rare. The clinical course, good prognosis and histological picture of the present case favour the diagnosis of localized Castleman's disease of mixed cell type. The successful medical treatment suggests that such a regime may substitute for surgery when the latter proves difficult.
Alyahya
Ghassan Ayish
GA
Eye Pathology Institute, University of Copenhagen, Denmark. gaja@eyepath.ku.dk
Prause
Jan Ulrik
JU
Heegaard
Steffen
S
D000328:Adult; D001379:Azathioprine / Q000627:therapeutic use; D005871:Castleman Disease / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D003131:Combined Modality Therapy; D005094:Exophthalmos / Q000473:pathology / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006942:Hypergammaglobulinemia / Q000473:pathology / Q000628:therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D009916:Orbital Diseases / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D011241:Prednisone; D018714:Radiotherapy, Adjuvant; D014057:Tomography, X-Ray Computed
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D001379:Azathioprine; D011241:Prednisone