title: Primary localized amyloidosis presenting as diffuse amorphous calcified mass in both orbits: case report.
abstract: Primary localized amyloidosis is rare in the orbit. We report the case of a 63-year-old woman that presented with bilateral proptosis and ophthalmoplegia. A computed tomography scan revealed an infiltrative amorphous and markedly calcified mass in both orbits while a magnetic resonance scan showed a heterogeneous hypointense signal on T2-weighted images. A biopsy was performed through an anterior orbitotomy. Microscopy revealed extracellular amorphous and eosinophilic hyaline material which stained pink with Congo red and displayed green birefringence on polarized microscopy, leading to a diagnosis of amyloidosis. The results of the systemic workup were completely normal. A two-year follow-up period without any treatment disclosed no worsening of the condition. While calcification of nonvascular orbital lesions has often been regarded as suggestive of malignant disease, our case is a reminder that it can also be a characteristic presenting sign of orbital amyloidosis.
authors.affiliation: Department of Ophthalmology, Universidade de São Paulo, Brazil.
authors.forename: Allan Christian Pieroni
authors.initials: AC
authors.lastname: Gonçalves
authors.forename: Rodrigo Bernal da Costa
authors.initials: RB
authors.lastname: Moritz
authors.forename: Mário Luiz Ribeiro
authors.initials: ML
authors.lastname: Monteiro
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis*; D002114:Calcinosis / Q000175:diagnosis* / Q000209:etiology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009916:Orbital Diseases / Q000175:diagnosis* / Q000209:etiology; D014057:Tomography, X-Ray Computed