Castleman's disease involving paranasal sinus and bilateral orbits: A case report and review of the literature

Abstract

Abstract Background Castleman's disease (CD) is a rare non-malignant lymphoproliferative disorder affecting lymph nodes of any region and rarely involving extranodal locations. We report the first case of paranasal sinus involvement in Castleman’s disease (CD), presenting along with bilateral orbital involvement. Additionally, we report the use of Fluorodeoxyglucose (FDG) positron emission tomography scan (PET/CT scan) in the initial and subsequent assessment of orbital CD and the use of chemotherapy and single-agent rituximab for its treatment.Case presentation: We report a 32-year-old female with a history of chronic progressive left periorbital swelling for seven years. Initial evaluation and subsequent computed tomography (CT) scans showed bilateral orbital masses with left optic nerve encasement and ethmoidal and frontal sinus involvement. Bilateral orbital mass biopsies showed CD of mixed cell type. FDG-PET/CT scan showed high uptake in the affected lesions and CT scan also confirmed multicentric involvement. She received rituximab + cyclophosphamide, vincristine and prednisone chemotherapy with a complete metabolic response (mCR). After initial mCR, she relapsed twice, three years apart but went into mCR after single-agent rituximab alone.Discussion A review of the literature of previously reported cases showed no cases of paranasal sinus involvement in CD. Only 24 cases of orbital CD were identified, 20 of which reported confirmed histological findings of CD from the orbital lesion. Ages ranged from 17–76 years, with nearly equal gender distribution. Hyaline vascular type was the most histology and eyelid ptosis and exophthalmos were the most common clinical presentations. Almost half of the reported cases were localized CD and were managed by surgical resection.Conclusion To our knowledge, this is the first reported case of paranasal sinus involvement and simultaneous bilateral orbital CD. Additionally, FDG-PET/CT has shown usefulness in assessing the patient’s disease status and response assessment.