Approach to diagnosis and treatment of pediatric primary tumors of the diaphragm

Abstract

Background/Purpose Tumors of the diaphragm in the pediatric population are extremely rare. We present 5 cases diagnosed at the Hospital for Sick Children, Toronto, and together with a review of the world literature, provide an approach to the diagnosis and management of these tumors. Methods A clinical retrospective review of patients diagnosed as having primary diaphragmatic tumor (PDT) at the Hospital for Sick Children as well as a review of the world literature. Results Forty-one cases of PDT in the pediatric population have been described from 1868 to 2005 inclusive. There is an equal incidence in boys and girls, they are found with the same frequency on the left as on the right, and 78% are malignant. Rhabdomyosarcoma is the most commonly occurring malignant tumor. The mean age at diagnosis is 10 years. “Chest-associated” symptoms are more common than “abdomen-associated” symptoms. Imaging often fails to identify the site of origin as the diaphragm. Surgery is the cornerstone of therapy for PDT. Conclusion A multidisciplinary team approach is needed for successful treatment and management of PDT.