Polymorphic reticulosis: a reappraisal

We studied 18 patients (15 men and three women) evaluated for a destructive sinonasal lesion that had been diagnosed as "polymorphic reticulosis." The histologic features of each lesion were those of angiocentric immunoproliferative lesions," characterized by atypical lymphoid infiltrates with polymorphous, angiocentric, and necrotic features; 13 were grade 2 and five were grade 3. The neoplastic cells in each patient had a T-cell phenotype. Epstein-Barr virus RNA was detected in the neoplastic cells of 17 of the 18 T-cell lesions. Initial treatment consisted of local radiation therapy in each patient, chemotherapy in two patients, and prednisone in another patient. Two patients were lost to follow-up and the other 16 had a median follow-up of 14 years, 2 months (range, 4 months to 32 years, 5 months). Four patients are alive and disease free, four patients died not of disease or complication of therapy, and eight patients died of disease. The Kaplan-Meier estimate of survival was 63% at 5 years and 50% at 15 years. Histologic progression of angiocentric immunoproliferative lesions from grade 2 to grade 3 was observed in two patients, and a correlation between angiocentric immunoproliferative grade and survival could not be detected. These data suggest that polymorphic reticulosis is an Epstein-Barr virus-related angiocentric T-cell lymphoma.