Primary CNS lymphoma

Case contributed by Ammar Ashraf
Diagnosis certain

Presentation

Headache, gradual left upper limb weakness & unsteady gait for 4 weeks. History of 12 kg weight loss in the last two months. No fever, seizures, or sphincter disturbance.

Patient Data

Age: 65 years
Gender: Male
mri
  • a well-defined rounded soft tissue lesion measuring 1.6 x 1.8 cm is seen at the grey-white matter junction in the right frontal lobe. The lesion is isointense on T1 & T2-weighted images, is surrounded by significant vasogenic edema, shows restricted diffusion (high on DWI & low on ADC), and shows intense homogeneous contrast enhancement on the post-contrast study. Spectroscopy of the right frontal lobe lesion shows increased choline peak, reversed choline/creatinine ratio, low NAA, and high lactate levels
  • two other tiny suspicious nodules measuring 4 & 2 mm, showing restricted diffusion, are seen at the grey-white matter junction at the medial aspect of the right parietal lobe
  • no midline shift is seen
  • bilateral periventricular white matter ischemic changes and age-related brain involutional changes

Case Discussion

  • MRI features are suggestive of highly cellular cerebral lesions, likely metastases (considering patient's age, multiple lesions & marked vasogenic edema around the largest lesion), and imaging differential diagnosis includes lymphoma, leukemia, and small cell lung cancer
  • craniotomy was done and the right frontal lobe lesion was resected completely
  • pathological diagnosis: Diffuse large B-cell primary CNS lymphoma. The immunostain with the antibody anti-CD20 is diffusely positive in tumor cells.  CD79A, CD19, PAX5, and MUM-1 are positive. CD10 and BCL6 are focally positive.  Ki67 is very high (>90%).  CD3, CD5, CD30, CD38, CD138, EBV, and BCL2 are negative. The immunostain with the antibody anti-GFAP highlighted the brain tissue (the glial cells) invaded by the tumor
  • no gross abnormality (primary malignancy, metastases, or lymphadenopathy) was seen on the whole-body CT scan (done before the brain biopsy)

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