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Gerardo Graue, Gary J. Lelli, Jr., Paul T. Finger, Elizabeth Maher, David Della Rocca, Robert Della Rocca; Ocular Adnexal Lymphoma Staging and Treatment: American Joint Committee on Cancer Versus Ann Arbor. Invest. Ophthalmol. Vis. Sci. 2012;53(14):1263.
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© ARVO (1962-2015); The Authors (2016-present)
To evaluate the prognostic utility of the American Joint Committee on Cancer (AJCC) staging system for Ocular Adnexal Lymphoma (OAL) and provide treatment recommendations.
A retrospective chart review of a consecutive case series of patients with biopsy proven unilateral or bilateral conjunctival, orbit, eyelid or lacrimal gland/sac lymphoma was performed. Each patient was staged according to the Ann Arbor and AJCC systems. Local or systemic progression was considered only if observation as treatment was employed and growth or extension was demonstrated (clinically or radiographically). Systemic recurrence was considered when a disease free period of at least 6 months was achieved prior to evidence of growth. Statistical analysis included demographic evaluations and the Kaplan-Meier survival probability method.
Between the years 2000 and 2010, of 113 patients that underwent surgery at our participating institutions, 83 were included in the final statistical analysis. Females (n=51/83,62%) were more commonly affected. Patients were a mean 64 years old (range 21 to 90). The overall mean follow up was 43.3 months (range 6 to 274).Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) was the most common histopathological diagnosis (n=55/83, 66%). The most common Ann Arbor (AA) clinical stages were IE (76%) followed by II (17 %) and III (7%). Pathology staging identified 13 cases (15%) that where restaged as group IV (p=0.017). Corresponding AJCC clinical stages were T1 (28%), T2 (64%), T3 (6%) and T4 (2%). Treatment approaches were multiple and complex. They ranged from EBRT (n=35/83,42%), single agents (most frequently rituximab) and multi-drug systemic chemotherapy (n=24/83,29%), a combination of oral antibiotics, prednisone and/or topical interferon (n=9/83,11%), a combination of EBRT and systemic chemotherapy (n=11/83,13%) and observation (n=4/83,5%). Local control was achieved in 75% (n=59/79) treated patients. There were 19 local recurrences (mean follow up 53, range 6-133 months) from which 14 (74%) belonged to the non-radiation treatment groups and only 1 systemic progression. Lower stage groups (T1 and T2 without lymph node involvement or metastatic disease of AJCC and IE of AA) had longer disease free survival (mean 123.5 vs. 88.4 months) than higher-risk groups (T3, T4 and T1, T2 with nodal involvement or metastatic disease plus Ann Arbor II, III, IV). Tumor location was significantly more precise using the "site specific" AJCC TNM system (p=0.001).
Regardless of stage, recurrence and disease free survival was more closely related to histopathology and treatment type rather than tumor size or site-specific location (p = 0.02).
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