True about primary CNS lymphoma ?
High-Yield Explanation
Ans. is 'a' i.e., Reticulin staining is done; 'b' i.e. Essentially B cell type; 'c' i.e., Have an association with EBV Primary CNS lymphoma o It is most common CNS neoplasm in immunosuppressed2 patients & accounts for 1% of intracranial tumors. o Affects all ages. Its peak incidence is in sixth & seventh decades in immunocompetent but younger in immunosuppressed patients. o More than 98% of PCNSL are B-cell lymphomas o Often have multiple sites of tumor within brain parenchyma (c.f. NHL arising outside rarely involves brain parenchyma) o Most common locations are the frontal lobes, followed by deep nuclei and periventricular zone. o 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". o In immunocompromised patients, PCNS lymphoma are invariably associated with EBV infectiondeg of tumor cells o The main stay of definitive therapy is chemotherapy by methotrexate, vincristine, procarbazine, cytarbine etc. Therapy also includes glucocoicoids and rituximab. o A unique features of PCNSL in comparison to other brain tumors is its exquisite sensitivity to coicosteroids. o Surgery is impoant to confirm the histological diagnosis but has no therapeutic role o Is an aggressive disease with relatively poor response to chemotherapydeg compared to peripheral lymphoma o Survival with AIDS-related PCNSL is very poor, generally < 3 monthsdeg; the degree of immunosuppression and the extent of CNS dissemination at diagnosis all appear to influence outcome.