Superior vena cava syndrome in childhood and adolescence: a review of the literature and report of three cases

Abstract

Obstruction of the superior vena cava is rare in childhood and adolescence. Its etiology is now mainly iatrogenic. Mediastinal tumors are the main primary causes of the superior vena cava syndrome (SVCS) in childhood and adolescence. The most common mediastinal tumors presenting with the SVCS are the lymph node tumors and especially the lymphosarcomas. About 50% of the mediastinal tumors are primary in the mediastinum. Early treatment is designed to cause a rapid shrinking of the tumor and should not be delayed by an overly diligent pursuit of the diagnosis. Treatment should consist of a combination of radiation therapy, chemotherapy, and steroids. Surgery should be limited to obtaining tissue for diagnosis, except when the tumor is localized to the mediastinum and is completely resectable. One hundred and seventy two children and adolescents with the SVCS are reviewed and three patients are added.