Primary CNS lymphoma and primary intraocular lymphoma

Guideline recommendations

1. Stereotactic biopsy

§   Before steroid treatment

§   Preferred to surgical resection (has no role in the treatment)

§   Need biopsy even if MRI / LP suggestive

2. Staging

§   CT chest abdo pelvis

§   LP – CSF protein/glucose, cytology, flow cytometry, Ig gene rearrangement

§   Examination of eye (anterior chamber vitreous and fundus) – intraocular lesions should be biopsied but are difficult to interpret (little tissue – 30% failure rate)

§   HIV testing

3. Prognostic scoring

1.      Age >60

2.      Performance status >1

3.      Raised LDH

4.      Raised CSF protein

5.      Involvement of deep brain matter

4. Consent for neurocongitive deterioration with treatment

5. Dexamethasone for short term palliation

6. High dose MTX – first line in fit patients

§   3-5 doses >3g/m2 over 2-3h each dose being 2-3 weeks apart

§   Cytaribine may improve outcome (also penetrates CNS) – should be given in clinical trial

7. Whole brain radiotherapy

Consolidation following MTX in patients under 60 who achieve CR

Neurocognative side effects outweigh benefits if over 60

WBRT also useful for palliating patients who cannot tolerate HD-MTX

8. No evidence for CHOP or Intrathecal MTX

9. Experimental treatments only to be used as part of a trial include:

§   Autologous SCT as first line therapy

§   Rituximab (intrathecal or intraventricular)

§   Pharmacological disruption of the blood brain barrier

10. Relapse / refractory disease treatment

§   WBRT – if not previously done

§   Dexamethasone

§   Autologous SCT

§   Temozolomide

11. Concurrent intraocular and CNS lymphoma

§   High dose MTX with radiation to both globes and possibly whole brain.

§   Intravitreal MTX is effective in treating recurrent disease confined to the eyes