Extranodal Lymphoma

Extranodal Lymphoma

Most of these cases are treated by combined chemotherapy (usually CHOP+/- Rituxan) followed by local radiation (combined modality therapy, see the NCCN flow chart below.) This generally results in the best outcome as the recent study of thyroid lymphoma demonstrates below. Also in the other study below, extranodal (e.g. Waldeyer's ring or GI) may do better than nodal.

Bone
Brain (CNS)
Gastric (Stomach)
NasoSinus (e.g. maxilla)
Oral / head and neck
Orbit (Eye)
Skin (cutaneous)
Testicle
Tonsil

Localized non-Hodgkin lymphoma involving the thyroid gland/ Relapse-Free Survival
Cancer 2001;91:629-35

Diffuse large B-cell lymphoma: clinical and biological characterization and outcome according to the nodal or extranodal primary origin.

Lopez-Guillermo A, J Clin Oncol. 2005 Apr 20;23(12):2797-804. Epub 2005 Feb 22.

Department of Hematology, Hospital Clinic, Villarroel 170, 08036 Barcelona, Spain.

PURPOSE: To study the main clinicobiologic features, response, and outcome of patients with diffuse large B-cell lymphoma (DLBCL) according to the primary site, lymph node, or different extranodal organs of the disease. PATIENTS AND METHODS: We included 382 patients consecutively diagnosed with DLBCL in a single institution during a 13-year period. Sites of the disease were: lymph node, 222 cases (58%); Waldeyer's ring (WR), 42 (11%); and extranodal sites, 118 (31%), including GI tract in 45 cases. Primary extranodal cases, particularly GI, showed a bcl-6 expression more frequently than nodal cases. Patients with primary WR or GI lymphomas presented with early-stage disease, no marrow infiltration, normal serum lactate dehydrogenase, and low- to low/intermediate-risk international prognostic index (IPI) more frequently than the remainder. Complete response (CR) rate was 63%, with WR and GI lymphomas having a higher CR rate (85% and 80%, respectively) than the other groups. In the whole series, 5-year overall survival (OS) was 52%. Patients with WR or GI lymphomas showed better OS (5-year OS: 77% and 68%, respectively) than patients with nodal or other extranodal sites. In the multivariate analysis, IPI, bulky disease, and beta2-microglobulin were the main variables to predict OS; no nodal or extranodal site maintained their prognostic value. CONCLUSION: In the present series, the primary site of disease was associated with particular clinicopathologic features and outcome, though the latter largely depended on other factors.