True about primary CNS lymphoma -a) Reticulin staining is doneb) Essentially B cell typec) Have an association with EBVd) Indolent disease with good prognosise) Chemotherapy is highly effective
High-Yield Explanation
Primary CNS lymphoma
It is most common CNS neoplasm in immunosuppressed patients & accounts for 1% of intracranial tumors.
Affects all ages. Its peak incidence is in sixth & seventh decades in immunocompetent but younger in immunosuppressed patients.
More than 98% of PCNSL are B-cell lymphomas
Often have multiple sites of tumor within brain parenchyma (c.f. NHL arising outside rarely involves brain parenchyma)
Most common locations are the frontal lobes, followed by deep nuclei and periventricular zone.
Reticulin stains demonstrate that the infiltrating cells are separated from one another by silver-staining material; this pattern is characteristic of primary brain lymphoma and is referred to as "hooping".
In immunocompromised patients, PCNS lymphoma are invariably associated with EBV infection° of tumor cells
The main stay of definitive therapy is chemotherapy by methotrexate, vincristine, procarbazine, cytarbine etc. Therapy also includes glucocorticoids and rituximab.
A unique features of PCNSL in comparison to other brain tumors is its exquisite sensitivity to corticosteroids.
Surgery is important to confirm the histological diagnosis but has no therapeutic role
Is an aggressive disease with relatively poor response to chemotherapy° compared to peripheral lymphoma
Survival with AIDS-related PCNSL is very poor, generally < 3 months°; the degree of immunosuppression and the extent of CNS dissemination at diagnosis all appear to influence outcome.