(22) Currently, all OALs confined to the ocular adnexa are categorized and recorded by the Ann Arbor system as stage IE, whatever their exact location or size.
Noncontiguous Involvement of Tissues External to the Ocular Adnexa.--Commonly, OALs are associated with concurrent involvement of extranodal sites, such as the parotid and submandibular glands.
T1 categorizes conjunctival OALs according to whether they are bulbar (T1a; Figure 2), or nonbulbar (T1b; ie, confined to fornix and/or caruncle).
Stage T2 defines any OALs involving the orbit, whether or not the conjunctiva is affected.
Current staging systems for NHL are not useful for the clinical staging of OALs. A user-friendly staging system for OAL would improve communication about the stage of disease, selection of appropriate management, standardization of enrollment and response criteria in clinical trials, and collection/analysis of prospective survival data.
Lymph Node Involvement.--Recent studies indicate that conjunctival OAL with nodal involvement is associated with a worse prognosis.
Multicentricity and Bilaterality of OAL.--Ocular adnexal lymphomas can occur in separate noncontiguous sites in the ocular adnexa (eg, concurrent but noncontiguous conjunctival and orbital tumors).
Our OAL staging system (Table) aims to define disease extent more precisely and to facilitate future studies identifying clinical and histomorphologic features of prognostic significance.
Stage T3 indicates OAL infiltration of preseptal eyelid tissues (ie, tissues anterior to the orbital septum, such as dermis, orbicularis muscle, or eyelid skin) as defined previously (Figure 5).
Lymph node involvement of OAL at diagnosis is documented using the N stage.
The most common metastatic sites of OAL include salivary glands, gastrointestinal tract, lung, and liver.
This is the first TNM staging system for OAL. (26) It describes such tumors more precisely than the Ann Arbor system.