Massive thymic hyperplasia: Characterization of a rare mediastinal mass

Abstract

Thymic hyperplasia is a rare cause of an anterior mediastinal mass in children. True thymic hyperplasia is characterized by massive thymic hypertrophy with retention of normal thymic architecture, and must be distinguished from more commonly seen tumors of the anterior mediastinum. Previous reports of thymic hyperplasia primarily have been descriptive, with minimal analysis of the cellular characteristics of the tumor. To better describe the cellular characteristics of thymic hyperplasia, the authors report on a 10-year-old boy found to have an asymptomatic left paracardiac mass during cardiac evaluation. The mass enlarged rapidly during the following 2 weeks and filled the entire left side of the chest. An open biopsy specimen showed normal thymic architecture. Because of the size and rapid growth of the mass, the authors decided to resect it. Cellular analysis of the mass did not show differences from normal thymic elements. There was conserved thymic architecture. The expression of cell surface markers and the proliferative response of thymocytes to cytokines appeared to be normal. Similarly, a long-term cell culture of the thymocytes did not result in clonal proliferation. Immunohistologic staining of stromal cells showed no striking differences from normal thymic elements. Moreover, immunologic analysis of the patient by standard hematological parameters, lymphocyte subsets, quantitative immunoglobins, and immunoelectrophoresis showed no abnormalities except for lymphocytosis, which resolved after tumor resection. The authors conclude that thymic hyperplasia occurs in immunologically normal children and may exhibit (1) rapid growth with minimal associated symptoms, (2) normal thymic architecture, (3) normal thymocyte and stromal growth characteristics in vitro, and (4) a normal thymocyte response to cytokine stimulation. These data confirm, at a cellular level, the clinical impression that thymic hyperplasia represents benign hypertrophy of the thymus rather than a thymic neoplasm.