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Primary CNS Lymphoma.pptx

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Primary CNS lymphoma
Primary CNS lymphoma
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Primary CNS Lymphoma.pptx

  1. 1. Medhat Moustafa, MD, Department of Neurosurgery Suez Canal University, Ismailia, Egypt
  2. 2. Primary CNS Lymphoma
  3. 3. Overview Primary central nervous system lymphoma (PCNSL) has been known by many other names, including reticulum cell sarcoma, diffuse histiocytic lymphoma, and microglioma. The proliferation of names reflects initial uncertainty about the cell of origin.
  4. 4. PCNSL is now known to be a form of extranodal, high-grade non-Hodgkin B-cell neoplasm, usually large cell or immunoblastic type.
  5. 5. It originates in the brain, cerebrospinal fluid, spinal cord, or eyes. It typically remains confined to the central nervous system (CNS), but 4%–7% of patients with newly diagnosed PCNSL and 10% of patients with relapsed PCNSL may have systemic disease.
  6. 6. Although the cells of origin are lymphocytes, PCNSL should be considered a brain tumor, because the therapeutic challenges resemble those of other brain tumors..
  7. 7. In particular, drug delivery is impaired by the blood- brain barrier, and cerebral toxicity limits the use of treatment modalities
  8. 8. Epidemiology Incidence Incidence of primary central nervous system lymphoma (PCNSL) in immunocompetent patients is approximately 51 cases per 10,000,000 per year. PCNSL has been reported in 6-20% of patients infected with HIV, and the incidence is expected to rise as patients with low CD4+ counts survive longer. Similar trends toward rising frequency of diagnosis of PCNSL are reported internationally.
  9. 9. Sex predilection Among immunocompetent patients with PCNSL, males have a higher incidence of PCNSL than females. Patients with HIV-associated PCNSL are more likely to be male. In one study, 74% or HIV patients with PCNSL were male
  10. 10. Age predilection The median age of immunocompetent patients with PCNSL is 55 years. There is an increased incidence with advancing age with the highest rate of PCSNL in patients aged 75 years or older. The median age of HIV-infected patients with PCNSL is 35 years.
  11. 11. Race predilection Black males aged younger than 50 years have greater than twice the incidence of white males, while white males aged 50+ years have twice the incidence of black males. A similar pattern to a lesser magnitude is present in females.
  12. 12. Clinical presentation Patients with primary central nervous system lymphoma (PCNSL) develop progressive neurologic deficits fairly rapidly, over weeks to months. These deficits are variable depending on the affected location within the CNS.
  13. 13. About 40%–50% of patients present with nonspecific neurocognitive symptoms, and about 50%–70% present with focal neurologic signs. Seizures may occur but are less common than in other mass lesions due to relative cortical sparing
  14. 14. Patients with HIV may be more likely to present with an encephalopathy than other patients with PCNSL. This correlates with the more often multifocal, diffuse enhancement pattern seen on magnetic resonance imaging (MRI) scans.
  15. 15. In contrast to systemic DLBCL, patients with PCNSL do not typically present with B symptoms of weight loss, fever, and/or night sweats.
  16. 16. As the presence of immune deficiency guides both the diagnosis and the treatment of PCNSL, much of the history taking should be devoted to establishing whether the patient may be immunocompromised. A careful sexual and drug abuse history is necessary. If the patient is a transplant recipient, the nature and duration of immune suppression must be clarified.
  17. 17. Although ocular involvement is not infrequent, it is often asymptomatic; if visual symptoms are present, patients may describe blurred vision, decreased acuity, or floaters. .
  18. 18. Relapsing, remitting lesions may disappear for periods of as long as several months to a year or more. Administration of corticosteroids may cause prolonged remission of clinical and radiographic signs and symptoms, but remission inevitably occurs
  19. 19. Diagnostic Overview The predilection of PCNSL for certain cerebral sites gives rise to its characteristic appearance on neuroimaging studies. Seventy-five percent of immunocompetent patients will present with solitary lesions. The dense cellularity of the tumor accounts for its isodense or hyperdense appearance on nonenhanced CT scan and hypointense appearance on long TR-weighted MRI imaging. Restricted diffusion . Homogenous enhancement.
  20. 20. While lesions in immunocompetent patients tend to be solitary, periventricular, and homogenously enhancing, lesions in immunocompromised patients may be cortical or subcortical with a variable enhancement pattern, with ring enhancement most commonly seen
  21. 21. Since the clinical and neuroimaging presentation of PCNSL can be varied and the differential diagnostic possibilities are therefore large, no patient should be treated for PCNSL without definitive cytologic proof of diagnosis, either by vitrectomy, CSF sampling, or brain biopsy.
  22. 22. Corticosteroids should be avoided when possible Corticosteroids have a cytotoxic effect on lymphoma cells and can induce a radiographic response in up to half of patients, which limits the sensitivity of diagnostic tools like biopsy or lumbar puncture.
  23. 23. Additionally, a biopsy of lymphoma pre-treated with corticosteroids may reveal only gliosis or lymphocytic and histiocytic infiltrates without identifiable neoplastic cells. Responses to corticosteroids are not durable and thus only delay definitive diagnosis and treatment.
  24. 24. Procedures Bone marrow biopsy Bone marrow biopsy to evaluate for abnormal lymphomatous cells should be completed for staging purposes.
  25. 25. Lumbar puncture Lumbar puncture should be performed to evaluate CSF profile (glucose, protein, and cell count) and cylology and flow cytometry for detection of abnormal lymphomatous cells. Brain biopsy should not be delayed while awaiting this procedure.
  26. 26. Lumbar puncture is low-yield as the majority of patients with primary central nervous system lymphoma (PCNSL) will not have leptomeningeal or CSF involvement; however, if lumbar puncture identifies lymphoma cells, this may obviate the need for brain biopsy.
  27. 27. Brain biopsy Stereotactic brain biopsy is the most appropriate method for the diagnosis of PCNSL. If possible, the procedure should be performed before corticosteroids have been administered.
  28. 28. Treatment Chemotherapy Radiotherpay either focal or whole Surgery
  29. 29. Treatment The optimal treatment regimen has not been established. Standard systemic chemotherapy regimens such as CHOP (ie, cyclophosphamide, doxorubicin, vincristine, prednisone) are ineffective, which presumably reflects the difficulty of penetration of the blood-brain barrier by chemotherapeutic drugs.
  30. 30. Chemotherapy Methotrexate is the single most effective chemotherapeutic agent for PCNSL. For this reason, methotrexate based chemotherapy regimens are used as first line treatment. The optimal combination of chemotherapies that include methotrexate is not known, however, literature supports the use of ;mulit-agent chemotherapy over methotrexate monotherapy.
  31. 31. Chemotherapy Initial chemotherapy without radiation therapy results in excellent initial tumor response rates and avoids the toxicity associated with whole brain radiation.
  32. 32. Radiation Focal Whole brain
  33. 33. Radiation Focal radiation results in increased relapses outside of the radiation field, presumably because of microscopic diffuse infiltrative disease thought to be present at initial diagnosis
  34. 34. Radiation Whole brain radiation therapy PCNSL patients have been treated with whole brain radiation therapy alone. This has yielded high CR rates, but sustained responses are rare with a median overall survival of around a year..
  35. 35. Surgery The role of surgery in treatment of PCNSL is limited to biopsy for confirmation of diagnosis or for rapid reduction of intracranial pressure to prevent imminent herniation. Small retrospective studies have shown no benefit in outcomes when comparing surgical resection to supportive care
  36. 36. Treatment of Recurrence and Refractory Disease There is no standard approach to treatment of recurrent or refractory PCNSL. Survival rates after recurrence and chance of response to further treatment are much lower than at initial diagnosis.
  37. 37. Treatment complications Long-term sequelae of radiation therapy and chemotherapy in PCNSL are significant. Although median survival duration has been extended with combined chemotherapy and radiation therapy, the percentage of survivors with late cerebral white-matter toxicity resulting in cognitive dysfunction approaches 50%.
  38. 38. Serious leukoencephalopathy also is seen in patients receiving methotrexate chemotherapy alone, but the incidence appears to be lower than that of the cerebral white-matter toxicity seen with combination therapy.
  39. 39. A randomized trial investigated whether the addition of whole brain radiation therapy to methotrexate based chemotherapy regimens affected survival and found no survival benefit.