title: Masses in the membranes.
abstract: A 24-year-old woman with systemic lupus erythematosus presented with a 1-year history of painless vision loss in the right eye. Examination was notable for a bitemporal hemianopia. Brain imaging revealed multiple contrast enhancing dural masses, including one along the planum sphenoidale. She underwent excisional biopsy for a presumed diagnosis of multiple meningiomas. Five years later, she developed worsening vision in the left eye, hypesthesia in the V1 distribution, and oculomotor nerve palsy. Repeat imaging showed an enhancing mass in the cavernous sinus and orbital apex. Biopsy demonstrated a lymphoplasmacyte rich infiltrate in dense extracellular material. She was diagnosed with lupus-induced hypertrophic pachymeningitis and started on immunosuppressive therapy. On further worsening of symptoms, her initial biopsy was reexamined and revealed a kappa light chain restricted B-cell and plasmacyte population. This led to the final diagnosis of central nervous system extranodal marginal zone lymphoma.
authors.affiliation: Department of Ophthalmology, University of Minnesota, Minneapolis, Minnesota, USA.
authors.forename: Sandip
authors.initials: S
authors.lastname: Suresh
authors.affiliation: Department of Ophthalmology, University of Minnesota, Minneapolis, Minnesota, USA.
authors.forename: Anne S
authors.initials: AS
authors.lastname: Abel
authors.affiliation: Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Brian R
authors.initials: BR
authors.lastname: Younge
authors.affiliation: Skull Base Division, Neuro-Ophthalmology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, USA.
authors.forename: Jurij R
authors.initials: JR
authors.lastname: Bilyk
authors.affiliation: Department of Ophthalmology, University of Minnesota, Minneapolis, Minnesota, USA. Electronic address: mikelee@umn.edu.
authors.forename: Michael S
authors.initials: MS
authors.lastname: Lee
mesh_terms: D014408:Biomarkers, Tumor / Q000378:metabolism; D016543:Central Nervous System Neoplasms / Q000175:diagnosis* / Q000378:metabolism; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D020945:Lupus Vasculitis, Central Nervous System / Q000175:diagnosis; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008577:Meningeal Neoplasms / Q000175:diagnosis; D008579:Meningioma / Q000175:diagnosis; D008581:Meningitis / Q000175:diagnosis; D014786:Vision Disorders / Q000175:diagnosis; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor
keywords: CNS MALT lymphoma; EMZL; dural lymphoma; hypertrophic pachymeningitis; multiple meningioma; systemic lupus erythematosus