title: Thymoma in children: report of 2 cases and review of the literature.
abstract: Thymoma is an uncommon and slow-growing neoplasm. It is derived from thymic epithelial cells and comprises about 20% to 30% of mediastinal masses in adults, but only about 1% in pediatric patients. Patients usually present with mass-associated respiratory symptoms, superior vena cava syndrome, or paraneoplastic syndrome including myasthenia gravis, pure red cell aplasia, or acquired hypogammaglobulinemia, and connective tissue disorders. Due to the limited number of cases, knowledge, and experience with thymoma in pediatric patients, the diagnosis and treatment are very challenging for this age group. In this article, we report 2 cases of thymoma in childhood and provide a comprehensive review and analysis of the reported pediatric cases in the past 30 years (total of 32 cases). We found that patients younger than age 10 years were predominantly male (M:F = 6:1) and had advanced tumor stage more frequent than patients older than age 10 (P = .03). There were also significant associations of male sex with more advanced tumor stage and less favorable outcome (P = .03). These findings suggest that age and sex may be additional potential prognostic contributors in pediatric patients with thymoma. The clinicopathologic features, differential diagnosis, and current therapeutic recommendations of this uncommon tumor in pediatric patients are also addressed.
authors.affiliation: Department of Pathology, The Children's Hospital, Aurora, CO, USA. liang.xiayuan@tchden.org
authors.forename: Xiayuan
authors.initials: X
authors.lastname: Liang
authors.forename: Mark A
authors.initials: MA
authors.lastname: Lovell
authors.forename: Kelley E
authors.initials: KE
authors.lastname: Capocelli
authors.forename: Edythe A
authors.initials: EA
authors.lastname: Albano
authors.forename: Sarah
authors.initials: S
authors.lastname: Birch
authors.forename: Amy K
authors.initials: AK
authors.lastname: Keating
authors.forename: Douglas K
authors.initials: DK
authors.lastname: Graham
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000628:therapy; D011859:Radiography; D012074:Remission Induction; D012737:Sex Factors; D013934:Thymectomy; D013945:Thymoma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D013953:Thymus Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy