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Table 2 Clinical staging of ocular adnexal lymphoma (OAL)

From: Classification, diagnosis, and management of conjunctival lymphoma

National Cancer Institute Working Formulation [48]

Low-grade

Small lymphocytic

Follicular small cleaved cell

Follicular mixed, small cleaved and large cell

Intermediate-grade

Follicular large cell

Diffuse small cleaved cell

Diffuse mixed, small and large cell

Diffuse large cell

High-grade

Large cell, immunoblastic

Lymphoblastic

Small non-cleaved cell

Ann Arbor Staging [49]

Stage I

Involvement of a single lymph node region or extralymphatic site (IE)

Stage II

Involvement of 2 or more lymph nodes, lymphatic structures, or extralymphatic regions alone on the same side of the diaphragm (IIE)

Stage III

Involvement of lymph nodes on both sides of the diaphragm with localized extralymphatic (IIIE) or splenic (IIIS) involvement, or both (IIIES)

Stage IV

Involvement of one or more organs or tissues outside the lymphatic system

 

A: Without B symptoms

B: Fever, night sweats, weight loss of > 10% body weight over the last 6 months

TNM Staging System [50]

T0

No evidence of lymphoma

T1

Lymphoma involving the conjunctiva alone without orbital involvement

T2

Lymphoma with orbital involvement ± any conjunctival involvement

T3

Lymphoma with preseptal eyelid involvement ± orbital involvement ± any conjunctival involvement

T4

Orbital adnexal lymphoma extending beyond orbit to adjacent structures, such as bone and brain

N0

No evidence of lymph node involvement

N1

Involvement of ipsilateral regional lymph nodes

N2

Involvement of contralateral or bilateral regional lymph nodes

N3

Involvement of peripheral lymph nodes not draining ocular adnexal region

N4

Involvement of central lymph nodes

M0

No evidence of involvement of other extranodal sites

M1

Lymphomatous involvement in other organs recorded either at first diagnosis or subsequently

  1. TNM= Tumor, Node, Metastasis