Workup/Staging

Question 1

What are the common presenting Sx of ocular/orbital lymphomas?

Answer 1

Blurred vision, floaters, pain (uveitis/vitreitis), proptosis (if retro-orbital), and orbital lesion (e.g., salmon-colored conjunctival mass).

Question 2

What must the physical exam portion of the workup include?

Answer 2

The physical exam portion must include an ophthalmologic exam (fundoscopy, slit-lamp exam) as part of the workup.

Question 3

What lab/pathology tests are required during the workup?

Answer 3

CSF/vitrectomy, BM Bx, CBC, LFTs, ESR

Question 4

What imaging is recommended for ocular/orbital lesions?

Answer 4

MRI brain/orbits, ocular US, CT C/A/P (PET/CT if MALT: Perry C et al., Eur J Haematol 2007)

Question 5

What staging system is used for eye lymphomas?

Answer 5

Ann Arbor staging system:

Stage IE: localized eye lymphomas

Stage II: cancer located in 2 separate regions

Stage III: cancer on both sides of diaphragm, including 1 organ or area near LNs or spleen

Stage IV: diffuse or disseminated involvement of ≥1 extralymphatic organs (e.g., liver, BM, or nodular involvement of lungs)

Question 6

What is the most widely used classification system for lymphomas of the eye?

Answer 6

REAL (Revised European-American Classification of Lymphoid Neoplasms): 3 classes—indolent, aggressive, and highly aggressive