A 38 year-old male with advanced AIDS presents with severe headaches, fever and altered mental status, and dies several days later. An autopsy is performed, and a coronal section of the brain is shown here.
What is the most likely diagnosis?
A. Astrocytoma
B. Medulloblastoma
C. Metastatic carcinoma
D. Oligodendroglioma
E. Primary CNS lymphoma
(Scroll down for the answer)
The diagnosis in this case is primary CNS lymphoma. This is a malignancy that you almost never see in the general population. It’s way more common in immunosuppressed patients, especially patients with AIDS, who have a 1000x increased risk.
Presenting symptoms are often non-specific, and may include seizures, headaches, and constitutional symptoms. Primary CNS lymphomas are usually diffuse large B-cell lymphomas, and they generally occur as multiple, periventricular nodules which are more discrete than gliomas, but less well-defined than metastases. In the cross section of brain above, the tumor appears as a grayish, softly-demarcated area of discoloration around the ventricles.
Virtually all cases in immunosuppressed patients are associated with Epstein-Barr virus infection. Unfortunately, primary CNS lymphoma is an aggressive lymphoma which usually responds poorly to chemotherapy.
Here are some other cases for you to check out:
- Case 1: 20-year-old male who died suddenly
- Case 2: 72-year-old male with right calf mass
- Case 3: 67-year-old female with pancytopenia
- Case 4: 59-year-old male with severe headaches
- Case 5: 38-year-old female with deep venous thrombi
- Case 6: 13-year-old male with cerebellar mass
- Case 7: 45-year-old male with pulmonary emphysema
Dear Khristine
Your webblog is very intersting and exciting to read!
Thank you very much for helping us in our way throught the medical knowledge.
I have a question about this case: is there an explanation to why this neoplasia isn’t responsive to chemotherapy?
THX from Brazil
Thanks! I would imagine that the severely immunocompromised state of the patient would be the main reason for the ineffectiveness of chemotherapy. I don’t know that there is anything intrinsic to the tumor that makes it more difficult to treat – although some hematopoietic tumors that are strongly associated with viruses (like adult T-cell leukemia/lymphoma, which is associated with HTLV-1) seem to be more resistant to treatment.
and also, like all CNS tumours, the blood brain barrier makes delivering chemotherapy in tolerable doses very difficult. most chemo does not penetrate the cns.
yes we solved it.
Yay!
Brilliant. Seeing this for the first time.