Introduction
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an aggressive lymphoma with occasional multifocality.
Methods, Objectives
We retrospectively investigated the clinicopathologic characteristics of MEITL patients, with a focus on multifocality and prognosis. Among the 18 patients with MEITL, 17 patients (94.4%) experienced relapse or progression after treatment, and 14 patients (77.8%) died (median follow-up, 9.8 months; range, 0.3~74 months). Multifocality was defined by distinctive wall-thickening or ulceroinfiltrative focal lesions (>2) grossly recognizable in surgical specimens and/or by endoscopy/positron-emission tomography/computed tomography in gastrointestinal and/or extragastrointestinal regions at presentation.
Results
The mean age was 64.7 y (range, 37-84 y), with a slight male predilection (61.1%, 11/18). Twelve patients (66.7%) underwent resection, and fifteen patients (83.3%) received chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone with or without etoposide. Immunostaining revealed predominant expression of CD56 (100%, 18/18) and CD8 (88.9%, 16/18) and rare CD5 expression (6.3%, 1/16). Multifocality was observed in 44.4% (8/18) of the patients and was associated with stage III-IV disease (p=0.004), extranodal lesions (≥2) (p=0.013) and the international prognostic index (IPI; score 3-5; p=0.031, Fisher's exact test). Survival analysis (Kaplan-Meier) for progression-free survival (PFS) revealed that Eastern Cooperative Oncology Group performance status (ECOG PS; ≥ 2) (p=0.001), stage (p=0.038), extranodal lesions (≥ 2) (p=0.034) and multifocality (p=0.001) were prognostic factors, and a multivariate model (Cox) incorporating these variables revealed that multifocality was an independent factor (p=0.006, hazard ratio (HR)=9.6). Overall survival (Kaplan‒Meier) was associated with ECOG PS (p<0.001), IPI (p=0.009) and multifocality (p=0.005). A multivariate model incorporating the ECOG PS and multifocality revealed ECOG PS as an independent factor (p=0.003, HR=27.0).
Conclusions
Multifocality may add prognostic value to MEITL, which requires further validation.
