Orbital pseudotumor as an initial manifestation of multicentric Castleman's disease.

BACKGROUND

To report on a patient with multicentric Castleman's disease (MCD) who showed an orbital pseudotumor as an initial manifestation.

CASE: A Japanese male patient, 65 years old, initially exhibited swelling of the left eyelid. Computed tomography and magnetic resonance imaging showed an infiltrating orbital mass around the left eye.

OBSERVATIONS: The patient was treated several times with short courses of oral corticosteroids without significant response. Eight years after the first examination, he was referred to our neuroophthalmology clinic owing to persistent eyelid swelling. He exhibited an accelerated erythrosedimentation rate, polyclonal hypergammaglobulinemia, and multiple cervical and thoracic mediastinal lymphadenopathies. Histopathological examination revealed mixed-type MCD from a cervical lymph node and chronic inflammatory cell infiltration from eyelid swelling. Four months after his first visit, the patient's condition was complicated by fever, general fatigue, and hypoxemia. A cell marker study of the swollen lymph node showed monoclonality, indicating the development of non-Hodgkin's lymphoma.

CONCLUSIONS

The clinical course in this case suggests that an inflammatory reaction in the orbital tissue was initially present, before MCD, and that non-Hodgkin's lymphoma subsequently developed.