The ocular adnexal lymphomas (OAL) arise in the conjunctiva, orbit, lacrimal gland, and eyelids. To date, they have been clinically staged using the Ann Arbor staging system, first designed for Hodgkin and later for nodal, non-Hodgkin lymphoma. The Ann Arbor system has several shortcomings, particularly when staging extranodal non- Hodgkin lymphomas, such as OAL, which show different dissemination patterns from nodal lymphomas.

To describe the first TNM-based clinical staging system for OAL.

Retrospective literature review.

We have developed, to our knowledge, the first American Joint Committee on Cancer-International Union Against Cancer TNM-based staging system for OAL to overcome the limitations of the Ann Arbor system. Our staging system defines disease extent more precisely within the various anatomic compartments of the ocular adnexa and allows for analysis of site-specific factors not addressed previously. It aims to facilitate future studies by identifying clinical and histomorphologic features of prognostic significance. This system is for primary OAL only and is not intended for intraocular lymphomas.

Our TNM-based staging system for OAL is a user-friendly, anatomic documentation of disease extent, which creates a common language for multicenter and international collaboration. Data points will be collected with the aim of identifying biomarkers to be incorporated into the staging system.