Primary intracranial meningeal marginal zone B cell lymphoma of malt type (PMML) with osseous infiltration

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Dear Editor, We report the case of a 46-year-old immunocompetent woman with a 10-month-long history of progressive exophthalmia, right palpebral ptosis, and retro-orbital pain. The patient referred also a severe left ocular maculopathy since she was 17 years old. Physical examination revealed a right exophthalmia; blood cell count and other basic laboratory investigations were within normal ranges, with the exception of a not quantifiable monoclonal component at serum electrophoresis. Magnetic resonance imaging (MRI) and computed tomography (CT) scan showed a right cavernous sinus mass with implant on the meninges associated with intrasellar and intraorbital extension and erosion of the right superior orbital fissure without parenchymal infiltration. These findings supported a neuroradiological diagnosis of meningioma. Cerebrospinal fluid examination was normal. A partial resection of the intraorbital mass was performed. The gross specimen consisted of small fragments (i.e., 0.5 cm in maximum diameter) including bone spiculae. Histopathology revealed a mucosa-associated lymphoid tissue non-Hodgkin’s lymphoma (MALT-NHL) with an extensive plasma cell differentiation arising from the dura and infiltrating the underlying bone. Lymphoid cells were mainly represented by smallto intermediate-sized elements with somewhat regular nuclei and relatively abundant clear cytoplasm, forming loose aggregates in the intertrabecular areas of bone fragments (Fig. 1a). Lymphoid cells were immunoreactive for CD20 (Fig. 1c) and negative for CD3, CD21, CD138, IgM, IgD, cyclin D1, EMA, and myeloperoxidase. A significant proportion of tumor was represented by mature-appearing plasma cells, which, in some areas, were virtually the only recognizable elements (Fig. 1b) and showed immunoreactivity for CD138 (Fig. 1d) with lambda light chain restriction (Fig. 1e, f). The postoperative period was uneventful and a complete staging workup, including blood cell count, total-body CT scan, and bone marrow biopsy, ruled out any systemic lymphomatous involvement. Helicobacter pylori infection and gastric lymphoma were excluded by multiple biopsies. The patient’s well-known long history of left ocular maculopathy prevented the adoption of radiotherapy to avoid further damage to both eyes. Accordingly, treatment with the anti-CD20 monoclonal Ann Hematol (2009) 88:599–601 DOI 10.1007/s00277-008-0638-x