Testicular Tumors

© Springer International Publishing Switzerland 2014
Ahmed H. Al-SalemAn Illustrated Guide to Pediatric Surgery10.1007/978-3-319-06665-3_64

64. Testicular Tumors

Ahmed H. Al-Salem1  
(1)
Pediatric Surgery Department, Maternity and Children Hospital, Dammam, Saudi Arabia
 
 
Ahmed H. Al-Salem
Email: ahsalsalem@hotmail.com
Introduction
Sertoli Cell Tumors
Leydig Cell Tumors
Juvenile Granulosa Cell Tumors
Gonadoblastoma
Paratesticular Tumors
Clinical Features and Investigations
Classification
Staging
Treatment
Recommended Reading
Keywords
Testicular cancerGerm cell tumorsLeydig cellsSertoli cellsYolk-sac tumor

Introduction

  • Testicular cancer is the most common cancer in males aged 20–39 years, and is rarely seen before the age of 15 years.
  • Testicular tumors account for 1– 2 % of all pediatric tumors, with an incidence of 0.05–2 per 100,000 children.
  • A bimodal age distribution is observed:
    • One peak occurs in the first 2 years of life.
    • The second occurs in young adulthood.
  • Although testicular cancer can be derived from any cell type found in the testicles, more than 95 % of testicular cancers are germ cell tumors .
  • Most of the remaining 5 % are sex cord-gonadal stromal tumors derived from Leydig cells or Sertoli cells .
  • Of testicular tumors, 2–3 % are bilateral.
  • The two main categories of testicular tumors are:
    • Germ cell tumors (95 %)
    • Sex cord-stromal tumors (5 %)
  • Germ cell tumors account for 60–77 % of testicular tumors in children but account for 95 % of testicular tumors in adults.
  • Adult germ cell tumors with malignant potential, such as seminoma and embryonal carcinoma, are not seen in prepubertal patients.
  • Teratomas, which are uniformly benign in children, are often malignant in adults.
  • The most common germ cell tumors are:
    • Teratomas (62 %).
    • Yolk-sac tumors (26 %).
    • Prepubertal teratomas in children are uniformly benign.
  • Gonadal stromal tumors are significantly less common than germ cell tumors and primarily include:
    • Juvenile granulosa-cell tumors
    • Leydig cell tumors
    • Sertoli cell tumors
  • The vast majority (85 %) of yolk-sac tumors in children present as clinical stage I disease, compared with 35 % in adults.
  • Alpha-fetoprotein (AFP) can be used as a reliable tumor marker because levels are increased in more than 90 % of yolk-sac tumors.
  • Epidermoid cysts are benign tumors of epithelial origin and account for 10–15 % of cases.
  • Seminomas and mixed germ cell tumors are extremely rare in prepubertal children.

Sertoli Cell Tumors

  • Are the most common gonadal stromal tumors in prepubertal children.
  • They tend to appear as painless masses in boys younger than 6 months.
  • They produce no endocrinologic effects; however, 14 % of patients present with gynecomastia.
  • All reported cases in children younger than 5 years have been benign.
  • Large-cell calcifying Sertoli cell tumor is a variant with large amounts of cytoplasm and calcification.
  • One third of patients with this tumor have associated genetic abnormalities; however, these tumors are universally benign.

Leydig Cell Tumors

  • These are the second most common gonadal stromal tumors in children.
  • They are benign tumors.
  • These tumors most often occur in boys aged 5–10 years.
  • The synthesis of testosterone by these tumors may produce:
    • Precocious puberty
    • Gynecomastia
    • Elevated levels of 17-ketosteroids
  • Leydig cell tumors must be differentiated from hyperplastic nodules that develop in boys with poorly controlled congenital adrenal hyperplasia (CAH).

Juvenile Granulosa Cell Tumors

  • Account for approximately 3 % of all neonatal testicular tumors.
  • Commonly appear as cystic, painless testicular masses.
  • They almost always appear in the first year of life, and most appear by age 6 months.
  • They can be associated with:
    • Anomalies of the Y chromosome
    • Mosaicism
    • Ambiguous genitalia
  • These tumors are hormonally inactive and benign.

Gonadoblastoma

  • Occurs in association with disorders of sexual development (intersex).
  • About 80 % of cases involve phenotypic females with intra-abdominal testes or streak gonads.
  • The gonadoblastoma gene is on the Y chromosome, and the tumor almost always develops in a child with a Y chromosome.
  • The streak gonads in patients with mixed gonadal dysgenesis often develop gonadoblastomas.
  • The incidence peaks at puberty, and early gonadectomy is recommended in patients at risk for gonadoblastoma.
  • Metastatic spread of a gonadoblastoma occurs in 10 % of patients.
  • These tumors may have elevated serum levels of beta-human chorionic gonadotropin (beta-HCG).
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Mar 8, 2017 | Posted by in PEDIATRICS | Comments Off on Testicular Tumors

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