“…Microscopically, they grow in a diffuse pattern or form trabeculae with a rich vascular network. In young patients, the limits of the tumors may not be well-demarcated, since no capsule is present, and the tumor may engulf and infiltrate peripheral seminiferous tubules (see Nistal and colleagues [74]).…”
Testicular tumors in the prepubertal age are relatively rare, representing only 9.4% of the total testicular and paratesticular specimens from a 20-year review performed at a large pediatric hospital [ 1 ]. They account for 1% to 2% of all solid tumors in the pediatric age group, with an annual incidence between 0.5/100 000 and 2/100 000 boys according to Coppes et al [ 2 ] and data from the Prepubertal Testicular Tumor Registry [ 3 ]. Similar to other neoplasms afflicting children, a bimodal age distribution is observed. The first peak is between birth and 3 years of age, and a second one occurs at the onset of puberty, extending to the fourth decade. Reports on their frequency vary because some investigators include the adolescent period, while others do not [ 4 ]. The vast majority of testicular tumors are germ cell neoplasms, accounting for 95% across all ages [ 5 ]. In children, germ cell tumors also predominate, representing 71% of all testicular neoplasms. These include yolk sac tumors (49%), teratomas (13%), seminomas and mixed germ cell tumors (9%), and sex-cord stromal tumors (29%). Malignant potential is significantly lower (less than 70%) in the pediatric age group compared to adults (90%) [ 6 ]. According to Pohl et al, 74% of prepubertal testicular tumors are benign [ 7 ].
“…Microscopically, they grow in a diffuse pattern or form trabeculae with a rich vascular network. In young patients, the limits of the tumors may not be well-demarcated, since no capsule is present, and the tumor may engulf and infiltrate peripheral seminiferous tubules (see Nistal and colleagues [74]).…”
Testicular tumors in the prepubertal age are relatively rare, representing only 9.4% of the total testicular and paratesticular specimens from a 20-year review performed at a large pediatric hospital [ 1 ]. They account for 1% to 2% of all solid tumors in the pediatric age group, with an annual incidence between 0.5/100 000 and 2/100 000 boys according to Coppes et al [ 2 ] and data from the Prepubertal Testicular Tumor Registry [ 3 ]. Similar to other neoplasms afflicting children, a bimodal age distribution is observed. The first peak is between birth and 3 years of age, and a second one occurs at the onset of puberty, extending to the fourth decade. Reports on their frequency vary because some investigators include the adolescent period, while others do not [ 4 ]. The vast majority of testicular tumors are germ cell neoplasms, accounting for 95% across all ages [ 5 ]. In children, germ cell tumors also predominate, representing 71% of all testicular neoplasms. These include yolk sac tumors (49%), teratomas (13%), seminomas and mixed germ cell tumors (9%), and sex-cord stromal tumors (29%). Malignant potential is significantly lower (less than 70%) in the pediatric age group compared to adults (90%) [ 6 ]. According to Pohl et al, 74% of prepubertal testicular tumors are benign [ 7 ].
“…LH/hCG receptor gene mutations Both gonadotropins share the same receptor, and activating mutations are responsible for familial male precocious puberty [124,125] (see Nistal and colleagues [126]). Inactivating mutations of the LH/HCG receptor lead to male undermasculinization of external genitalia due to the poor differentiation and activity of Leydig cells, which are either absent or hypoplastic (see Nistal and colleagues [127]).…”
Section: Mutations In Gonadotropin Receptor Genesmentioning
Normal testicular physiology requires appropriate function of endocrine glands and other tissues. Testicular lesions have been described in disorders involving the hypothalamus-hypophysis, thyroid glands, adrenal glands, pancreas, liver, kidney, and gastrointestinal tract. Testicular abnormalities can also associate with chronic anemia, obesity, and neoplasia. Although many of the disorders that affect the above-mentioned glands and tissues are congenital, acquired lesions may result in hypogonadism in children and adolescents.
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