Pediatric Thymoma: A Review and Update of the Literature

Abstract

Pediatric thymomas are extremely rare and slow-growing malignant tumors. The recent publication of the first Union for International Cancer Control (UICC)/American Joint Committee on Cancer (AJCC) Tumor-Node-Metastasis (TNM) stage classification and updated treatment guidelines for thymomas has prompted us to perform a review of the literature on pediatric thymomas. A search of English-language articles in the PubMed, Cochrane, Web of Science, and Embase databases was conducted. Additional articles were identified through reference lists of retrieved publications. Thirty-two articles involving 82 pediatric thymomas were included. Males comprised 60% of patients, and 13% manifested myasthenia gravis (MG). Histotype B1 (45%) and stage I (52% Masaoka-Koga and 71% UICC/AJCC TNM) were the most frequent. Of note is the possibility that the lack of cases with mixed histologies in the reviewed publications might be related to a sampling issue, as it is well known that the more sections are available for review, the more likely it is that the majority of these neoplasms will show mixed histologies. Both staging systems showed a gradual increase in the percentage of cases, with more advanced stages of disease moving from type A to B3 thymomas. Complete surgical resection (R0) was the main therapeutic approach in Masaoka-Koga stage I (89%) and UICC/AJCC TNM stage I (70%) thymomas. Advanced stages of disease and incomplete surgical resection were most often associated with recurrence and death. An association between stage and outcome, and completeness of resection and outcome, was found. Interestingly, though an association between histotype and staging was found, this does not take into account the possibility of mixed histologies which would reduce the clinical impact of histologic subtyping over staging.

Keywords: mediastinal tumor; pediatric thymoma; thymoma.

Figure 1

Stage distribution in thymoma histotypes according to the Masaoka–Koga staging system. WHO, World Health Organization.

Figure 2

Stage distribution in thymoma histotypes according to the UICC/AJCC TNM stage classification. UICC/AJCC TNM, Union for International Cancer Control/American Joint Committee on Cancer Tumor–Node–Metastasis; WHO, World Health Organization.

Figure 3

Treatment according to Masaoka–Koga stage. CTX, chemotherapy; RT, radiotherapy.

Figure 4

Treatment according to UICC/AJCC TNM stage. CTX, chemotherapy; RT, radiotherapy; UICC/AJCC TNM, Union for International Cancer Control/American Joint Committee on Cancer Tumor–Node–Metastasis.