TY - JOUR
T1 - Castleman's disease in the orbit. A 20-year follow-up
AU - Alyahya, Ghassan Ayish
AU - Prause, Jan Ulrik
AU - Heegaard, Steffen
PY - 2002/10
Y1 - 2002/10
N2 - PURPOSE: 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.CONCLUSION: 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.
AB - PURPOSE: 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.CONCLUSION: 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.
KW - Adult
KW - Azathioprine/therapeutic use
KW - Castleman Disease/diagnostic imaging
KW - Combined Modality Therapy
KW - Exophthalmos/pathology
KW - Female
KW - Follow-Up Studies
KW - Glucocorticoids/therapeutic use
KW - Humans
KW - Hypergammaglobulinemia/pathology
KW - Immunosuppressive Agents/therapeutic use
KW - Magnetic Resonance Imaging
KW - Orbital Diseases/diagnostic imaging
KW - Prednisone
KW - Radiotherapy, Adjuvant
KW - Tomography, X-Ray Computed
UR - https://www.scopus.com/pages/publications/0036772863
U2 - 10.1034/j.1600-0420.2002.800515.x
DO - 10.1034/j.1600-0420.2002.800515.x
M3 - Journal article
C2 - 12390168
SN - 1395-3907
VL - 80
SP - 540
EP - 542
JO - Acta Ophthalmologica Scandinavica
JF - Acta Ophthalmologica Scandinavica
IS - 5
ER -