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Pediatric Thymoma: A Review and Update of the Literature
Cristiana Rossi1, Magda Zanelli2, Francesca Sanguedolce3
1Pathology Unit, Azienda Unità Sanitaria Locale ASL5, 19124 La Spezia, Italy.
Insights
Pediatric thymomas are rare tumors. Complete surgical resection is key for better outcomes in early-stage disease, while advanced stages and incomplete resection correlate with recurrence and death.
Area of Science:
- Oncology
- Pediatric Oncology
- Tumor Biology
Background:
- Pediatric thymomas are rare, slow-growing malignant tumors.
- Recent updates in staging and treatment guidelines necessitate a literature review.
- Understanding pediatric thymoma characteristics is crucial for improved patient management.
Purpose of the Study:
- To review the literature on pediatric thymomas.
- To analyze the characteristics, staging, and treatment of pediatric thymomas.
- To evaluate the impact of staging and resection completeness on outcomes.
Main Methods:
- Systematic literature search of PubMed, Cochrane, Web of Science, and Embase.
- Inclusion of 32 articles reporting on 82 pediatric thymomas.
- Analysis of patient demographics, histopathology, staging (Masaoka-Koga and UICC/AJCC TNM), treatment, and outcomes.
Main Results:
- Males comprised 60% of patients; 13% had myasthenia gravis (MG).
- Histotype B1 and Stage I were most frequent.
- Complete surgical resection (R0) was associated with better outcomes; advanced stage and incomplete resection correlated with recurrence and death.
Conclusions:
- Stage and completeness of resection are significant prognostic factors in pediatric thymomas.
- While histotype and staging show associations, mixed histologies may reduce the impact of subtyping.
- Further research is needed to fully understand the nuances of pediatric thymoma presentation and management.
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.
