Epithelial thymic tumours in paediatric age: a report from the TREP project

• Main Authors: Migliorati Roberta, Di Cataldo Andrea, Conte Massimo, Ferrari Andrea, Inserra Alessandro, Carretto Elena, Cecchetto Giovanni, Bisogno Gianni
• Format: Article
• Language: English
• Published: BMC 2011-05-01
• Series: Orphanet Journal of Rare Diseases
• Online Access: http://www.ojrd.com/content/6/1/28

<p>Abstract</p> <p>Background</p> <p>Thymic epithelial tumours (thymoma and carcinoma) are exceptionally rare in children. We describe a national multicentre series with a view to illustrating their clinical behaviour and the results of treatment.</p> <p>Methods</p> <p>From January 2000 all patients under 18 years of age diagnosed with "<it>rare paediatric tumours</it>" were centrally registered by the Italian centres participating in the TREP project (<b>T</b>umori <b>R</b>ari in <b>E</b>tà <b>P</b>ediatrica [Rare Tumours in Paediatric Age]). The clinical data of children with a thymic epithelial tumour registered as at December 2009 were analyzed for the purposes of the present study.</p> <p>Results</p> <p>Our series comprised 4 patients with thymoma and 5 with carcinoma (4 males, 5 females; median age 12.4 years). The tumour masses were mainly large, exceeding 5 cm in largest diameter. Based on the Masaoka staging system, 3 patients were stage I, 1 was stage III, 1 was stage IVa and 4 were stage IVb.</p> <p>All 3 patients with stage I thymoma underwent complete tumour resection at diagnosis and were alive 22, 35 and 93 months after surgery. One patient with a thymoma metastasizing to the kidneys died rapidly due to respiratory failure.</p> <p>Thymic carcinomas were much more aggressive, infiltrating nearby organs (in 4 cases) and regional nodes (in 5), and spreading to the bone (in 3) and liver (in 1). All patients received multidrug chemotherapy (platinum derivatives + etoposide or other drugs) with evidence of tumour reduction in 3 cases. Two patients underwent partial tumour resection (after chemo-radiotherapy in one case) and 4 patients were given radiotherapy (45-54 Gy). All patients died of their disease.</p> <p>Conclusions</p> <p>Children with thymomas completely resected at diagnosis have an excellent prognosis while thymic carcinomas behave aggressively and carry a poor prognosis despite multimodal treatment.</p>