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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-gradeSmall lymphocytic
Follicular small cleaved cell
Follicular mixed, small cleaved and large cell
Intermediate-gradeFollicular large cell
Diffuse small cleaved cell
Diffuse mixed, small and large cell
Diffuse large cell
High-gradeLarge cell, immunoblastic
Lymphoblastic
Small non-cleaved cell
Ann Arbor Staging [49]Stage IInvolvement of a single lymph node region or extralymphatic site (IE)
Stage IIInvolvement of 2 or more lymph nodes, lymphatic structures, or extralymphatic regions alone on the same side of the diaphragm (IIE)
Stage IIIInvolvement of lymph nodes on both sides of the diaphragm with localized extralymphatic (IIIE) or splenic (IIIS) involvement, or both (IIIES)
Stage IVInvolvement 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]T0No evidence of lymphoma
T1Lymphoma involving the conjunctiva alone without orbital involvement
T2Lymphoma with orbital involvement ± any conjunctival involvement
T3Lymphoma with preseptal eyelid involvement ± orbital involvement ± any conjunctival involvement
T4Orbital adnexal lymphoma extending beyond orbit to adjacent structures, such as bone and brain
N0No evidence of lymph node involvement
N1Involvement of ipsilateral regional lymph nodes
N2Involvement of contralateral or bilateral regional lymph nodes
N3Involvement of peripheral lymph nodes not draining ocular adnexal region
N4Involvement of central lymph nodes
M0No evidence of involvement of other extranodal sites
M1Lymphomatous involvement in other organs recorded either at first diagnosis or subsequently
  1. TNM= Tumor, Node, Metastasis