Workup/Staging

Question 1

With which CNS Sx do pts present?

Answer 1

Focal neurologic deficits (70%), neuropsychiatric/personality change (frontal lobe involvement [43%]), ↑ ICP ([33%] HA, n/v, CN VI deficit, blurred vision), seizures, leg weakness, urinary incontinence/retention, and ocular Sx (blurry vision)

Question 2

With which systemic Sx do pts present?

Answer 2

Fever, night sweats, and weight loss (80%)

Question 3

All PCNSLs are what stage? What type of NHL?

Answer 3

All PCNSLs are stage IE. PCNSL is considered an extranodal NHL.

Question 4

What brain location and specific structures are commonly involved?

Answer 4

The #1 location is the frontal lobe, often the deep white matter and frequently periventricular (↑ CSF spread).

Question 5

What are considered deep structures of the brain according to the International Extranodal Lymphoma Study Group (IELSG)?

Answer 5

Corpus callosum, basal ganglia, brainstem, and cerebellum

Question 6

How is the Dx of ocular lymphoma made?

Answer 6

The Dx of ocular lymphoma is made by slit lamp exam and vitrectomy.

Question 7

What infectious etiology is often confused with CNS lymphoma?

Answer 7

Toxoplasmosis is the infectious etiology often confused with CNS lymphoma.

Question 8

What is the DDx?

Answer 8

Secondary metastatic lymphoma, other primary brain tumors, mets, abscess, hemorrhage, multiple sclerosis, sarcoidosis, and toxoplasmosis in AIDS

Question 9

What is the 1st step if a pt has a brain MRI suggestive of lymphoma?

Answer 9

Bx of brain lesion, least invasive approach. Consider LP, if safe and would not delay Tx or diagnostic process, to obtain CSF (15–20 mL to increase diagnostic yield). If the MRI and CSF show unequivocal evidence of PCNSL, brain Bx may be deferred (per NCCN). Do not initiate steroids, if possible, prior to diagnostic procedure.

Question 10

What do you do if the Bx is nondiagnostic?

Answer 10

If the pt rcvd steroids prior to the Bx, then D/C the steroids and re-Bx when Dz progresses. If no steroids were given, can re-Bx or workup for other Dx.

Question 11

What is the workup of a pt with a new Dx of CNS lymphoma (per NCCN 2018)?

Answer 11

Ophthalmic slit lamp exam to r/o ocular involvement, LP if safe, MRI spine if symptomatic or +CSF, CBC, CMP, LDH, HIV status, PET/CT or contrast-enhanced body CT, consider BM Bx, consider testicular US (men >60 yo)

Question 12

What imaging studies should be performed?

Answer 12

MRI brain (MRI spine if Sx or CSF+), contrast-enhanced body CT or PET/CT

Question 13

How does CNS lymphoma appear on MRI?

Answer 13

Indistinct fluffy borders, periventricular location common, T1 enhancement with gadolinium, and ring enhancement (d/t central necrosis, often seen in AIDS), less edema than would be expected for similar-sized glioma or mets

Question 14

When is PCNSL more likely to be multifocal?

Answer 14

PCNSL is more likely to be multifocal when the pt is immunocompromised (50%–80% of such pts).

Question 15

What chemical abnormalities are seen in the CSF of pts with CNS lymphoma?

Answer 15

↑ Protein (85%), ↓ glucose (33%), ↑ LDH, ↑ β2-microglobulin

Question 16

What additional tests are necessary for AIDS pts with a possible Dx of CNS lymphoma?

Answer 16

Toxoplasmosis titer (r/o this and other opportunistic infections) and BM Bx

Question 17

How can the Dx of PCNSL be most definitively established?

Answer 17

Bx brain/globe or CSF sampling

Question 18

What are the 5 poor prognostic factors for PCNSL according to the IELSG?

Answer 18

Poor prognostic factors for PCNSL:

Age >60 yrs
ECOG PS >1
Elevated LDH
Elevated CSF protein
Deep brain involvement (periventricular, basal ganglia, brainstem, cerebellum)
(Fererri AJ et al., JCO 2003)

Question 19

What is the 2-yr OS for pts with 0–1, 2–3, and 4–5 factors?

Answer 19

2-yr OS for these pts is 80%, 50%, and 15%, respectively. (Fererri AJ et al., JCO 2003)

Question 20

What are other prognostic factors for PCNSL?

Answer 20

Poor response to chemo, AIDS, and multifocality

Question 21

Who are considered “good-risk” immunocompromised pts with PCNSL?

Answer 21

Non-HIV immunosuppression and HIV+ with CD4 >200