Problems in lymphoma management: special sites of presentation

The staging and treatment of the common presentations of malignant lymphoma are readily familiar to experienced medical oncologists and hematologists. However, because of their rarity and variable and unusual behavior, certain special sites of lymphoma present a major challenge even to experienced clinicians. This article focuses on five such special sites of presentation: the eye, paranasal sinuses, central nervous system (CNS), testicle, and stomach. In each case, an appreciation of the usual histologic types, unique mode of metastatic spread, special patterns of resistance to systemic treatment, and, in the stomach, the remarkable association with a causative organism, Helicobacter pylori, is needed to optimize management. Only if these special characteristics are understood can the clinician plan a multiphase treatment course that offers each patient the best chance of disease control or cure.