RESUMO
Rosai-Dorfman disease (RDD) is characterized by histiocytic proliferation and massive cervical lymphadenopathy, although some patients have extra-nodal involvement. We report a case of extranodal RDD in a five-year-old child, initially misdiagnosed as orbital inflammatory disease and treated with oral steroids. A subsequent orbital biopsy three years later confirmed the diagnosis of Rosai Dorfman disease.
RESUMO
A 41-year-old man presented with chronic eyelid swelling, conjunctival injection, and decreased ocular motility in all gaze directions. MRI showed bilateral enlarged extraocular muscles, including the tendons. Laboratory tests revealed elevated levels of angiotensin-converting enzyme. An orbital biopsy showed collections of monotonous small lymphocytes, and granulomatous inflammation that included multinucleated giant cells, predominantly Langhans type. Flow cytometric analysis of tissue demonstrated a light chain-restricted clonal population of B cells, a finding that confirmed the morphologic impression of lymphoma. This case demonstrates that elevated angiotensin-converting enzyme and granulomatous inflammation can occur in lymphoma. Careful histopathologic examination and flow cytometric analysis are essential to avoid an erroneous diagnosis that could lead to inappropriate management.
Assuntos
Granuloma de Células Gigantes/diagnóstico , Leucemia Linfocítica Crônica de Células B/diagnóstico , Neoplasias Orbitárias/diagnóstico , Sarcoidose/diagnóstico , Adulto , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Murinos , Antineoplásicos/uso terapêutico , Biópsia , Diagnóstico Diferencial , Seguimentos , Granuloma de Células Gigantes/complicações , Granuloma de Células Gigantes/tratamento farmacológico , Granuloma de Células Gigantes/radioterapia , Humanos , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Leucemia Linfocítica Crônica de Células B/radioterapia , Imageamento por Ressonância Magnética , Masculino , Neoplasias Orbitárias/complicações , Neoplasias Orbitárias/tratamento farmacológico , Neoplasias Orbitárias/radioterapia , Tomografia por Emissão de Pósitrons , Radioterapia Adjuvante , Rituximab , Sarcoidose/complicações , Sarcoidose/tratamento farmacológico , Sarcoidose/radioterapiaRESUMO
PURPOSE: To report a case of isolated optic nerve lymphoma diagnosed by optic nerve biopsy. DESIGN: Case report. METHODS: A 66-year-old woman was referred to the Neuro-Ophthalmology Service because of a decrease in visual acuity and right optic disk edema. RESULTS: A magnetic resonance image of the brain showed only enhancement of the optic nerve. An examination that included ANA, c-ANCA, p-ANCA, Lyme titers, FTA-ABS, ACE level, chest x-ray, and lumbar puncture was negative. Because of rapid progression on clinical examination and serial imaging, an optic nerve biopsy was performed, which showed B-cell lymphoma. CONCLUSION: Optic nerve lymphoma can be confused with a variety of inflammatory and neoplastic infiltrations of the optic nerve on clinical and radiographic examinations. Optic nerve biopsy can be valuable in diagnosing isolated optic nerve lymphoma if other diagnostic tests are unrevealing, but the procedure carries considerable risk of loss of visual acuity and should be recommended judiciously.