Testicular and Paratesticular Tumors of Children and Adolescents

Abstract

Testicular and paratesticular tumors of children and adolescents are rare entities with similar surgical management. Testicular tumors represent 1–2 % of all pediatric tumors, with annual incidence of 0.5–2.0 per 100,000 children [1–3]. A bimodal age distribution is observed with first peak in the first 3 years of life, and then again after puberty [4]. Primary testicular tumors are divided into germ cell tumors (seminoma, yolk sac, embryonal carcinoma, choriocarcinoma, and teratoma) and stromal tumors (leydig cell, sertoli cell, and granulosa cell). Germ cell tumors usually present with painless testicular mass and have malignant behavior. Stromal tumors typically have a benign course, although some can become metastatic, and can be associated with hormonal production leading to precocious puberty. Treatment of testicular tumors is directed by the histologic type and age at presentation. The bimodal age distribution, histologic subtype, and stage encountered at different ages exemplifies the different molecular and behavior of these tumors. Of prepubertal tumors, 68–74 % carry a benign pathology, with mature teratoma making up the largest proportion (48 %) [5–7]. Furthermore, over 85 % of prepubertal patients present with stage I (localized) disease [8]. Regardless of the age at presentations, patients require a formal evaluation with scrotal ultrasound, tumor markers (AFP, βHCG, and LDH), and if indicated hormonal studies to evaluate for a stromal tumor. Scrotal ultrasound helps localize the tumor, determine if it has characteristics that will permit testicular sparing surgery, and it assess the status of the contralateral testicle. If metastatic disease is highly suspected based on tumor markers, physical exam, or symptoms, preoperative staging imaging of the chest, abdomen, and pelvis can be considered. Pre-operative staging imaging can also enable simultaneous central venous access placement at that time of radical inguinal orchiectomy if subsequent chemotherapy is warranted. The initial treatment of a testicular tumor is to establish a diagnosis. Prepubertal tumors are generally less aggressive, as is their treatment. For prepubertal stage I tumors, surveillance is recommended and for metastatic disease chemotherapy is the first line. Postpubertal tumors tend to have similar tumor characteristics and behaviors as adult tumors, as such they are treated more aggressively following established adult protocols.