Pediatric thymoma with a difference: Report of a case and review of literature

Abstract

Thymoma represents <1% of all mediastinal tumors in children. Less than 50 cases of pediatric thymoma are reported in the literature. Thymomas are considered to be highly aggressive in pediatric patients, especially when age is <10 years. Paraneoplastic syndromes, of which around 70% are myasthenia gravis, correlate with poor prognosis. In this article, we report a case of a thymoma in an 8-year-old boy, who had favorable histopathology (Masaoka stage I, WHO type B2), despite the presence of young age and necrosis along with absence of myasthenia gravis. We have also reviewed the available literature on pediatric thymoma.

Cardiomegaly Masquerading as a Pediatric Thymoma: A Case Report

“…Paraneoplastic syndromes or autoimmune disease, most commonly myasthenia gravis, though often associated with adult mediastinal masses and thymomas, are not seen in the pediatric population [2,8]. In contrast to benign tumors, malignant tumors present with lymphadenopathy and B symptoms of fever, weight loss, and night sweats [5].…”

“…Due to disease rarity, discovery of pediatric thymoma is often an incidental finding on chest radiograph or CT [5]. A contrasted chest CT best evaluates anterior mediastinal masses in the ED; this particular imaging modality allows for differentiation of the mass, location in comparison to local anatomical structures, characterization of the mass, and aid in surgical planning [2,3,5,9]. Referral for biopsy after CT further classifies and stages mediastinal masses, determining the need for adjunctive chemotherapy and further evaluation for metastasis.…”

“…Thymoma in the pediatric population can present in a variety of ways, many of which are non-specific, overlapping with cardiopulmonary pathologies. Presenting symptoms have a wide range from asymptomatic and incidental finding to compressive symptoms of local anatomical structures, including SVC syndrome, respiratory distress, chest pain, cough, orthopnea, and dyspnea [ 2 - 4 , 6 ]. Patients with stage III-IV masses often present with SVC syndrome, though not present in the above case, contributing to the difficulty of diagnosis in ED [ 7 ].…”

“…In the ED, treatment should be focused on resuscitation and airway management as mass effect may cause airway compromise prompting advanced airway management. Long-term management is extrapolated from the adult population due to low pediatric prevalence [ 1 , 2 , 8 , 10 - 11 ].…”

“…As an exceptionally rare pediatric diagnosis, thymoma of the anterior mediastinum presents a challenging diagnosis in children, with only 23 pediatric cases identified by a pediatric tumor surveillance registry between 1973 and 2008 [1][2][3][4]. Though often incidentally found, children can present with non-specific upper respiratory symptoms or chest pain, dyspnea from chest pain, and dyspnea secondary to mass effect on mediastinal structures.…”