Introduction

Gonadal tumors are rare in children and raise two main questions. First, is the tumor benign or malignant? The risk of malignancy is around 50% in boys and about 10% to 25% in girls (if both solid and cystic lesions are included). For benign tumors, a conservative surgical procedure (tumorectomy with gonadal preservation) should be attempted when possible. Malignant or potentially malignant tumors have an excellent prognosis provided they are properly treated by a surgeon and oncologist – current protocols using platinum and bleomycin are very effective, even for advanced-stage tumors. Second, how is future gonadal function best preserved? Gonadal function can be almost normal after treatment of a unilateral gonadal tumor, but in synchronous or metachronous bilateral tumors, gonadal function may be entirely destroyed. This has two major consequences: a lack of gamete production inducing sterility and a failure of sex hormone production which prevents the progression of natural puberty and the development of secondary sexual characteristics. Hormonal failure can be managed by lifelong hormonal substitution. However, sterility may have major psychological effects. Thus, gonadal function should be preserved if at all possible.

Advances in detection, diagnosis, minimally invasive management, and chemotherapy have all required changes in clinical practice, which is aimed at reducing morbidity without compromising oncological treatment.

Excluded from this chapter are the rarer problems of leukemia or lymphoma located in the ovary or testis, rare malignant paratesticular tumors (rhabdomyosarcoma or mesenchymal tumor), and rare soft tissue tumors (fibroma, leiomyoma, hemangioma).