However, magnetic resonance imaging (MRI) of her brain showed multifocal lesions scattered across the left occipital and parietal lobes; the largest one measured 16 mm in its transverse dimension. lymphoma, radiation therapy Introduction Lymphoplasmacytic lymphoma is usually a low-grade, B-cell neoplasm composed of small lymphocytes, plasmacytoid lymphocytes, and plasma cells that typically involve the bone marrow and it is associated with an immunoglobulin M (IgM) gammopathy.1 The diagnosis of LPL is mainly Cyclo(RGDyK) based on the histological evaluation of involved tissue, usually bone marrow or lymph nodes. Although the vast majority of LPL cases are Waldenstr?m macroglobulinemia (WM), there are some exceptions not satisfied with the diagnosis of WM.1 They include tumors producing other immunoglobulins, combined immunoglobulins, mixed cryoglobulins, gamma heavy chains, or non-gammopathy disease.2 Primary CNS lymphoma is an uncommon variant of non-Hodgkin lymphoma that involves the brain, leptomeninges, spinal cord, or eyes without systemic involvement. In literature, only a few cases diagnosed with LPL in the setting of primary CNS Cyclo(RGDyK) lymphoma have been reported. The current treatment strategy is not consistent due to the rarity and limited knowledge of this entity. Case Presentation A forty-six-year-old female was presented to the emergency department of our hospital with a two-month-headache and progressive numbness of her right leg. She also complained of nausea and dizziness 2 weeks before admission. The patients medical and family history revealed no significance. On admission, the patient seemed excitable with a Glasgow Coma Scale (GCS) of 14 (E:4; V:4; M:6); other vital signs were in their normal ranges. In a general examination, no lymphadenopathy or organomegaly was found. A neurological examination revealed numbness in her right lower limb with no motor dysfunction, cranial neuropathy, or signs of increased intracranial pressure. However, magnetic resonance imaging (MRI) of her brain showed multifocal lesions scattered across the left occipital and parietal lobes; the largest one measured 16 mm in its transverse dimension. These lesions exhibited a hypointense signal around the T1W sequence, a hyperintense signal on FLAIR, and no abnormal diffusion restriction on DWI images (Physique 1). They also revealed rim enhancement following gadolinium contrast injection. Open in a separate window Physique 1 Contrasted brain MRI revealed enhanced masses in the occipital and parietal lobes (arrow). Concern for brain metastases prompted further diagnostic procedures, including thoracic and abdominal contrast CT, lumbar and thoracic spine MRI, endoscopy of the gastrointestinal tract and ENT, but they identified no abnormalities. The patient then KIAA0288 underwent a surgical biopsy Cyclo(RGDyK) with two samples resected from the two largest masses in the left occipital lobe. Microscopically, the lesions were composed of small lymphocytes and neoplasm cells with plasma cell morphological features. The immunohistochemical (IMH) test showed tumor cells are positive for CD79a, CD138, CD38, and MUM-1, but unfavorable for CD3 and CD20 (Physique 2). These microscopic and IMH findings were consistent with lymphoplasmacytic lymphoma. Quantitative determination of the serum globulin test showed an increase in IgE at 740 IU/mL; other globulin levels were in their normal ranges (IgG 1206 mg/dL, IgM 155 mg/dL, IgA 220 mg/dL, Free Kappa 12.2 mg/L, Free Lambda 12.5 mg/L). Her serum protein electrophoresis and serum immunoelectrophoresis assessments identified no monoclonal gammopathy. Bone marrow biopsy and cerebrospinal fluid (CSF) analysis detected no cancerous involvement. Pieces of the evidence above confirmed the diagnosis of stage I primary CNS LPL, according to the Arbor-Cotswold staging system for lymphomas. Open in a separate window Physique 2 Immunohistochemistry results: (A) CD79a (+); (B) CD138 (+); (C) CD3 (-); (D) GFAP (-). Since the disease was confined to her.