Primary thyroid lymphoma: a review of recent developments in diagnosis and histology-driven treatment

Abstract

Purpose of review: Primary thyroid lymphoma (PTL) is a rare but clinically important malignancy of the thyroid. This article reviews the diagnosis, histologic subtypes, pathogenesis, and treatment of PTL, with the objective of optimizing diagnosis and management of the disease.

Recent findings: Recent studies have shed light on the clinicopathologic features of the histologic subtypes of PTL. Analysis of the pathogenesis of PTL indicates that both antigenic stimulation in the setting of Hashimoto's thyroiditis and aberrant somatic hypermutation may play a role. The first large, population-based study of PTL indicated that age, stage, histologic subtype, and treatment modality have prognostic implications. The U.S. Food and Drug Administration approval of rituximab for non-Hodgkin's lymphoma has improved standard chemotherapeutic options, as data on efficacy in nodal and other extranodal lymphomas have been extrapolated to PTL.

Summary: Advances in the understanding of the histologic subtypes of PTL have led to more accurate diagnosis and tailored treatments. The introduction of a new chemotherapeutic agent has expanded the treatment paradigm for PTL. Important prognostic indicators of survival for patients with PTL have been confirmed in the first population-based study. This article emphasizes current diagnostic and treatment approaches and discusses the potential for future developments.