Systemic malignant lymphoma 17 years after bilateral orbital pseudotumor.
OBJECTIVE
To report a patient who developed systemic malignant lymphoma 17 years after bilateral orbital pseudotumor. CASE. A 24-year-old man developed bilateral orbital pseudotumor. He was stable until 17 years later, when he was afflicted with systemic malignant lymphoma, a diffuse large B-cell lymphoma, and he died of the disease. He showed no new orbital lesion at the presentation of systemic lymphoma. Pathological diagnosis at autopsy was systemic malignant lymphoma with bilateral orbital pseudotumor.


METHODS
Immunohistochemical reexamination of the paraffin-embedded tissue and polymerase chain reaction of paraffin sections.


RESULTS
Histopathologically, the orbital masses resected on different occasions consisted of small lymphocytes and plasma cells. Immunohistochemical staining revealed an immunoglobulin light chain kappa and lambda bitype of B-cell lymphocytes and plasma cells. In contrast, the cervical lymph node showed large lymphoma cells that were positive only for lambda light chain, indicative of a lambda monotype. Amplification by polymerase chain reaction of the immunoglobulin heavy chain gene from paraffin sections demonstrated clonal rearrangement in the cervical lymph node but showed no clonality in the orbital tumor, supporting altogether the diagnosis of orbital pseudotumor.


CONCLUSIONS
Orbital pseudotumor should be carefully followed for possible development of systemic malignant lymphoma.
Department of Ophthalmology, Okayama University Graduate School of Medicine and Dentistry, Okayama 700-8558, Japan. matsuot@cc.okayama-u.ac.jp.
Toshihiko
T
Matsuo
Yumiko
Y
Sato
Ryu
R
Kuroda
Nobuhiko
N
Matsuo
Tadashi
T
Yoshino
D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology; D017809:Fatal Outcome; D015322:Gene Rearrangement, B-Lymphocyte / Q000235:genetics; D005803:Genes, Immunoglobulin / Q000235:genetics; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000276:immunology; D007146:Immunoglobulin lambda-Chains / Q000276:immunology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D016727:Orbital Pseudotumor / Q000150:complications* / Q000276:immunology / Q000473:pathology; D016133:Polymerase Chain Reaction; D013997:Time Factors
D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains