Primary Central Nervous System Lymphoma (PCNSL)

1. PRIMARY
CENTRAL NERVOUS SYSTEM
LYMPHOMA
(PCNSL)
Ade Wijaya, MD – June 2020

2. OUTLINE:
 Definition
 Epidemiology
 Risk Factors
 Pathobiology
 Clinical Presentations
 Diagnosis / Staging
 Treatment
 Prognosis
 Summary

3. DEFINITION
Extranodal malignant lymphomas arising inside the central nervous system
(including eyes) in the absence of systemic diffusion at the time of diagnosis
(Villano et al., 2011)

4. EPIDEMIOLOGY
1% of non-Hodgkin lymphomas (NHL)
3% of all primary brain tumours
(Villano et al., 2011)

5. RISK FACTORS
 Acquired immune deficiency syndrome (AIDS)
 Recipients of transplants receiving immunosuppressive therapy
 Congenital immunodeficiency disorders (Wiskott-Aldrich syndrome, X linked
immunodeficiency, ataxia teleangectasia)
 Secondary malignancy due to the immunosup-pressive therapy or an inherent
immune impairment
 Family history of malignancies
 Age > 60
(Krogh-Jensenet al., 1995) (DeAngelis„ 1991) O’Neill et al.(1995a)

6. PATHOBIOLOGY
 >95% are diffuse large B-cell lym-phomas (DLBCL)
 Very high proliferative activitywith Ki67 indexes of 70–90%
 The pathogenesis of PCNSL is still largely unclear and quite complex
Citterio G, Reni M, Gatta G, Ferreri AJ. Primary central nervous system lymphoma. Critical reviews in oncology/hematology. 2017 May 1;113:97-110.

7. CLINICAL PRESENTATIONS
 Subacute onset
 Cognitive decline and/or personality changes
 Focal neurological deficits
 Increased intracranial pressure
 Seizures are less frequent (<10%)
 Floaters and/or blurred vision
(Bataille et al., 2000)(Chan et al., 2011)

8. NEUROIMAGING
 MRI with contrast enhancement typically shows intense and homogeneously
enhancing single lesions (70%) or multiple lesions (30%) with modest
surrounding edema, usually located in periventricular areas and/or deep grey
matter (Bühringet al., 2001; Küker et al., 2005)
 FDG-PET (Kawaiet al., 2010; Yamashita et al., 2013),
 Diffusion tensor imaging (Tohet al., 2008)
 Dynamic susceptibility contrast MRI (DSC-MRI) (Tohet al., 2013; Kickingereder
et al., 2014)
 Proton MR spectroscopy

9. OTHER DIAGNOSTIC TESTS
CSF analysis
Vitreous analysis
Brain biopsy
Citterio G, Reni M, Gatta G, Ferreri AJ. Primary central nervous system lymphoma. Critical reviews in
oncology/hematology. 2017 May 1;113:97-110.

10. STAGING
 Aim: To specify the extent of the lymphoma within the CNS and to exclude the
presence of the diseaseelsewhere
 CT scan of the chest, abdomen and pelvis
 Bone marrow biopsy
 Testicular ultrasound in elderly males
 FDG body PET
 HIV blood test
 Ophthalmologic evaluation with a fundoscopy and a slit lamp examination
Citterio G, Reni M, Gatta G, Ferreri AJ. Primary central nervous system lymphoma. Critical reviews in oncology/hematology. 2017 May 1;113:97-110.

11. TREATMENT (BASED ON NCCN GUIDELINES 2019)

12. TREATMENT (BASED ON NCCN GUIDELINES 2019)

13. TREATMENT (BASED ON NCCN GUIDELINES 2019)

14. PROGNOSIS
 Poor prognosis
 Prognosis scoring:
IELSG (Ferreri et al., 2003)
MSKCC (Abrey et al.,2006)
Nottingham-Barcelona score (Bessell et al., 2001)

15. SUMMARY
AIDS-related
DLBCL
Methotrexate
Poor prognosis

16. THANK YOU