Testicular Tumors in Children

  • Yaser El-Hout
  • Darius J. BägliEmail author


There is a growing body of evidence that shows testicular tumors in prepubertal children and infants to be distinct clinically from testicular tumors in adults, or even adolescents, where more benign pathologies are being encountered in younger patients. With testicular tumors in children being relatively rare, this observation culminated with scattered small case series, in addition to centralized tumor registries, for example, the Prepubertal Testis Tumor Registry by the Section of Urology of the American Academy of Pediatrics (1980) and national oncology trials namely, the Children’s Oncology Group in the USA, the German Society of Pediatric Oncology, and the Children’s Cancer Study Group in the UK. This has led to fine-tuning in the management of prepubertal testicular tumor by showing radical surgery to be unnecessarily aggressive. This has led in a shift in the paradigm of management in pediatric testicular tumors, where more testis-sparing surgery is being performed without compromising safety and prognosis, hence reducing morbidity of adjuvant treatment that is skipped.


Testicular tumors in children Pediatric testicular tumors Disorders of sex development Gonadal stromal tumors Radical orchiectomy Germ cell tumors 


  1. 1.
    Schneider DT, Calaminus G, Koch S, Teske C, Schmidt P, Haas RJ, Harms D, Göbel U. Epidemiologic analysis of 1442 children and adolescents registered in the German germ cell tumor protocols. Pediatr Blood Cancer. 2004;42:169–75.CrossRefPubMedGoogle Scholar
  2. 2.
    Coppes MJ, Rackley R, Kay R. Primary testicular and paratesticular tumors of childhood. Med Pediatr Oncol. 1994;22:329–40.CrossRefPubMedGoogle Scholar
  3. 3.
    Alanee S, Shukla A. Paediatric testicular cancer: an updated review of incidence and conditional survival from the surveillance, epidemiology and end results database. BJU Int. 2009;104:1280–3.CrossRefPubMedGoogle Scholar
  4. 4.
    Ross JH, Rybicki L, Kay R. Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the prepubertal testis tumor registry. J Urol. 2002;168:1675–9.CrossRefPubMedGoogle Scholar
  5. 5.
    Pohl HG, Shukla AR, Metcalfe PD, Cilento BG, Retik AB, Bagli DJ, Huff DS, Rushton HG. Prepubertal testis tumors: actual prevalence rate of histological types. J Urol. 2004;172(6 Pt 1):2370–2.CrossRefPubMedGoogle Scholar
  6. 6.
    Thorup J, McLachlan R, Cortes D, Nation TR, Balic A, Southwell BR, Hutson JM. What is new in cryptorchidism and hypospadias—a critical review on the testicular dysgenesis hypothesis. J Pediatr Surg. 2010;45(10):2074–86.CrossRefPubMedGoogle Scholar
  7. 7.
    Toppari J, Virtanen HE, Main KM, Skakkebaek NE. Cryptorchidism and hypospadias as a sign of testicular dysgenesis syndrome (TDS): environmental connection. Birth Defects Res A Clin Mol Teratol. 2010;88(10):910–9.CrossRefPubMedGoogle Scholar
  8. 8.
    Wood HM, Elder JS. Cryptorchidism and testicular cancer: separating fact from fiction. J Urol. 2009;181(2):452–61.CrossRefPubMedGoogle Scholar
  9. 9.
    Looijenga LH, Hersmus R, de Leeuw BH, Stoop H, Cools M, Oosterhuis JW, Drop SL, Wolffenbuttel KP. Gonadal tumours and DSD. Best Pract Res Clin Endocrinol Metab. 2010;24(2):291–310.CrossRefPubMedGoogle Scholar
  10. 10.
    Pleskacova J, Hersmus R, Oosterhuis JW, Setyawati BA, Faradz SM, Cools M, Wolffenbuttel KP, Lebl J, Drop SL, Looijenga LH. Tumor risk in disorders of sex development. Sex Dev. 2010;4(4–5):259–69.CrossRefPubMedGoogle Scholar
  11. 11.
    Li Y, Tabatabai ZL, Lee TL, Hatakeyama S, Ohyama C, Chan WY, Looijenga LH, Lau YF. The Y-encoded TSPY protein: a significant marker potentially plays a role in the pathogenesis of testicular germ cell tumors. Hum Pathol. 2007;38(10):1470–81.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Lau YF, Li Y, Kido T. Gonadoblastoma locus and the TSPY gene on the human Y chromosome. Birth Defects Res C Embryo Today. 2009;87(1):114–22.CrossRefPubMedGoogle Scholar
  13. 13.
    Myrup C, Westergaard T, Schnack T, Oudin A, Ritz C, Wohlfahrt J, Melbye M. Testicular cancer risk in first- and second-generation immigrants to Denmark. J Natl Cancer Inst. 2008;100(1):41–7.CrossRefPubMedGoogle Scholar
  14. 14.
    Hardell L., van Bavel B., Lindström G., Carlberg M., Charlotte Dreifaldt A., Wijkström H., Starkhammar H., Eriksson M., Hallquist A., Kolmert T. Increased concentrations of polychlorinated biphenyls, hexachlorobenzene, and chlordanes in mothers of men with testicular cancer. Environ Health Perspect. 2003;111(7):930–4.Google Scholar
  15. 15.
    Nori F, Carbone P, Giordano F, Osborn J, Figà-Talamanca I. Endocrine-disrupting chemicals and testicular cancer: a case-control study. Arch Environ Occup Health. 2006;61(2):87–95.Google Scholar
  16. 16.
    Dieckmann KP, Hartmann JT, Classen J, Diederichs M, Pichlmeier U. Is increased body mass index associated with the incidence of testicular germ cell cancer? J Cancer Res Clin Oncol. 2009;135(5):731–8.CrossRefPubMedGoogle Scholar
  17. 17.
    Mongraw-Chaffin ML, Cohn BA, Anglemyer AT, Cohen RD, Christianson RE. Maternal smoking, alcohol, and coffee use during pregnancy and son’s risk of testicular cancer. Alcohol. 2009;43(3):241–5.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Daling JR, Doody DR, Sun X, Trabert BL, Weiss NS, Chen C, Biggs ML, Starr JR, Dey SK, Schwartz SM. Association of marijuana use and the incidence of testicular germ cell tumors. Cancer. 2009;115(6):1215–23.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Serter S, Gumus B, Unlu M, Tunçyürek O, Tarhan S, Ayyildiz V, Pabuscu Y. Prevalence of testicular microlithiasis in an asymptomatic population. Scand J Urol Nephrol. 2006;40:212–4.CrossRefPubMedGoogle Scholar
  20. 20.
    Peterson AC, Bauman JM, Light DE, McMann LP, Costabile RA. The prevalence of testicular microlithiasis in an asymptomatic population of men 18-35 years old. J Urol. 2001;166:2061–4.CrossRefPubMedGoogle Scholar
  21. 21.
    Husmann DA. Cryptorchidism and its relationship to testicular neoplasia and microlithiasis. Urology. 2005;66:424–6.CrossRefPubMedGoogle Scholar
  22. 22.
    Sakamoto H, Shichizyou T, Saito K, Okumura T, Ogawa Y, Yoshida H, Kushima M. Testicular microlithiasis identified ultrasonographically in Japanese adult patients: prevalence and associated conditions. Urology. 2006;68:636–41.CrossRefPubMedGoogle Scholar
  23. 23.
    Wasniewska M, De Luca F, Bertillonnai S, Matarazzo P, Weber G, Crisafulli G, Valenzise M, Lala R. Testicular microlithiasis: an unreported feature of McCune-Albright syndrome in males. J Pediatr. 2004;145:670–2.CrossRefPubMedGoogle Scholar
  24. 24.
    Furness PD III, Husmann DA, Brock JW III, Steinhardt GF, Bukowski TP, Freedman AL, Silver RI, Cheng EY. Multi-institutional study of testicular microlithiasis in childhood: a benign or premalignant condition? J Urol. 1998;160:1151–4.CrossRefPubMedGoogle Scholar
  25. 25.
    McEniff N, Doherty F, Katz J, Schrager CA, Klauber G. Yolk sac tumor of the testis discovered on a routine annual sonogram in a boy with testicular microlithiasis. AJR Am J Roentgenol. 1995;164:971–2.CrossRefPubMedGoogle Scholar
  26. 26.
    Arrigo T, Messina MF, Valenzise M, Rosano M, Alaggio R, Cecchetto G, Zirilli G, De Luca F. Testicular microlithiasis heralding mixed germ cell tumor of the testis in a boy. J Endocrinol Invest. 2006;29:82–5.CrossRefPubMedGoogle Scholar
  27. 27.
    Drut R. Yolk sac tumor and testicular microlithiasis. Pediatr Pathol Mol Med. 2003;22:343–7.CrossRefPubMedGoogle Scholar
  28. 28.
    Slaughenhoupt B, Kadlec A, Schrepferman C. Testicular microlithiasis preceding metastatic mixed germ cell tumor–first pediatric report and recommended management of testicular microlithiasis in the pediatric population. Urology. 2009;73(5):1029–31.CrossRefPubMedGoogle Scholar
  29. 29.
    Dagash H, Mackinnon EA. Testicular microlithiasis: what does it mean clinically? BJU Int. 2007;99:157–60.CrossRefPubMedGoogle Scholar
  30. 30.
    Benson CB, Doubilet PM, Richie JP. Sonography of the male genital tract. AJR Am J Roentgenol. 1989;153:705–13.CrossRefPubMedGoogle Scholar
  31. 31.
    Shapeero LG, Vordermark JS. Epidermoid cysts of testes and role of sonography. Urology. 1993;41:75–9.CrossRefPubMedGoogle Scholar
  32. 32.
    Wu JT, Book L, Sudar K. Serum alpha fetoprotein (AFP) levels in normal infants. Pediatr Res. 1981;15(1):50–2.CrossRefPubMedGoogle Scholar
  33. 33.
    Palmer JS, Morris K, Steinberg GD, Kaplan WE. Testicular, sacrococcygeal, and other tumors. In: Vogelzang NJ, Scardino PS, Shipley WU, Coffey DS, editors. Comprehensive textbook of genitourinary oncology. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2000.Google Scholar
  34. 34.
    Hinman F Jr, Baskin LS. Radical orchiectomy. In: Hinman F Jr, Baskin LS, editors. Hinman’s atlas of pediatric urologic surgery. 2nd ed. Philadelphia: Saunders Elsevier; 2009. p. 612–8.Google Scholar
  35. 35.
    Steiner H, Höltl L, Maneschg C, Berger AP, Rogatsch H, Bartsch G, Hobisch A. Frozen section analysis-guided organ-sparing approach in testicular tumors: technique, feasibility, and long-term results. Urology. 2003;62(3):508–13.Google Scholar
  36. 36.
    Shukla AR, Woodard C, Carr MC, Huff DS, Canning DA, Zderic SA, Kolon TF, Snyder HM 3rd. Experience with testis sparing surgery for testicular teratoma. J Urol. 2004;171(1):161–3.CrossRefPubMedGoogle Scholar
  37. 37.
    Metcalfe PD, Farivar-Mohseni H, Farhat W, McLorie G, Khoury A, Bägli DJ. Pediatric testicular tumors: contemporary incidence and efficacy of testicular preserving surgery. J Urol. 2003;170(6 Pt 1):2412–5; discussion 2415–6.Google Scholar
  38. 38.
    Wu H, Snyder HM 3rd. Pediatric urologic oncology: bladder, prostate, testis. Urol Clin North Am. 2004;31:619–27.CrossRefPubMedGoogle Scholar
  39. 39.
    Kato K, Ijiri R, Tanaka Y, Toyoda Y, Chiba K, Kitami K. Testicular immature teratoma with primitive neuroectodermal tumor in early childhood. J Urol. 2000;164:2068–9.CrossRefPubMedGoogle Scholar
  40. 40.
    Carver B, Al-Ahmadie H, Sheinfeld J. Adult and pediatric testicular teratoma. Urol Clin North Am. 2007;34:245–51.CrossRefPubMedGoogle Scholar
  41. 41.
    Bahrami A, Ro J, Ayala A. An overview of testicular germ cell tumors. Arch Pathol Lab Med. 2007;131:1267–80.PubMedGoogle Scholar
  42. 42.
    Manivel J, Reinberg Y, Nehans G, et al. Intratubular germ cell neoplasia in testicular teratomas and epidermoid cysts—correlation with prognosis and possible biologic significance. Cancer. 1989;64:715–20.CrossRefPubMedGoogle Scholar
  43. 43.
    Harms D, Zahn S, Gobel U, et al. Pathology and molecular biology of teratomas in childhood and adolescence. Klin Padiatr. 2006;218:296–302.CrossRefPubMedGoogle Scholar
  44. 44.
    Hasegawa T, Maeda K, Kamata N, et al. A case of immature teratoma originating in intra-abdominal undescended testis in a 3-month-old infant. Pediatr Surg Int. 2006;22:570–2.CrossRefPubMedGoogle Scholar
  45. 45.
    Walsh C, Rushton HG. Diagnosis and management of teratomas and epidermoid cysts. Urol Clin North Am. 2000;27(3):509–18.CrossRefPubMedGoogle Scholar
  46. 46.
    Ulbright TM, Roth LM. Recent developments in the pathology of germ cell tumors. Semin Diagn Pathol. 1987;4:304–19.PubMedGoogle Scholar
  47. 47.
    Grady RW. Current management of prepubertal yolk sac tumors of the testis. Urol Clin North Am. 2000;27(3):503–9.CrossRefPubMedGoogle Scholar
  48. 48.
    Grady RW, Ross JH, Kay R. Patterns of metastatic spread in prepubertal yolk sac tumor of the testis. J Urol. 1995;153(4):1259–61.CrossRefPubMedGoogle Scholar
  49. 49.
    Gómez García I, Romero Molina M, López-García Moreno A, et al. Sertoli cell tumor, a rare testicular tumor, our experience and review of the literature. Arch Esp Urol. 2010;63(5):392–5.PubMedGoogle Scholar
  50. 50.
    Thomas JC, Ross JH, Kay R. Stromal testis tumors in children: a report from the prepubertal testis tumor registry. J Urol. 2001;166(6):2338–40.CrossRefPubMedGoogle Scholar
  51. 51.
    Diamond FB Jr, Root AW, Hoover DL, Monteforte H. Hetero- and isosexual pseudoprecocity associated with testicular sex-cord tumors in an 8 year-old male. J Pediatr Endocrinol Metab. 1996;9(3):407–14.Google Scholar
  52. 52.
    Gabrilove JL, Freiberg EK, Leiter E, Nicolis GL. Feminizing and non-feminizing Sertoli cell tumors. J Urol. 1980;124(6):757–67.CrossRefPubMedGoogle Scholar
  53. 53.
    McGarrity TJ, Amos C. Peutz-Jeghers syndrome: clinicopathology and molecular alterations. Cell Mol Life Sci. 2006;63(18):2135–44.Google Scholar
  54. 54.
    Washecka R, Dresner MI, Honda SA. Testicular tumors in Carney’s complex. J Urol. 2002;167(3):1299–302.CrossRefPubMedGoogle Scholar
  55. 55.
    Young R, Koelliker D, Scully R. Sertoli cell tumors of the testis, not otherwise specified: a clinicopathologic analysis of 60 cases. Am J Surg Pathol. 1998;22:709–21.CrossRefPubMedGoogle Scholar
  56. 56.
    Al-Agha OM, Axiotis CA. An in-depth look at Leydig cell tumor of the testis. Arch Pathol Lab Med. 2007;131(2):311–7.PubMedGoogle Scholar
  57. 57.
    Shukla AR, Huff DS, Canning DA, et al. Juvenile granulose cell tumor of the testis: contemporary clinical management and pathological diagnosis. J Urol. 2004;171(5):1900–2.CrossRefPubMedGoogle Scholar
  58. 58.
    Cortez J, Kaplan G. Gonadal stromal tumors, gonadoblastomas, epidermoid cysts, and secondary tumors of the testis in children. Urol Clin North Am. 1993;20:15–26.PubMedGoogle Scholar
  59. 59.
    Young R, Lawrence W, Scully R. Juvenile granulosa cell tumor—another neoplasm associated with abnormal chromosomes and ambiguous genitalia, a report of 3 cases. Am J Surg Pathol. 1985;9:737–43.CrossRefPubMedGoogle Scholar
  60. 60.
    Cools M, Stoop H, Kersemaekers AM, et al. Gonadoblastoma arising in undifferentiated gonadal tissue within dysgenetic gonads. J Clin Endocrinol Metab. 2006;91(6):2404–13.CrossRefPubMedGoogle Scholar
  61. 61.
    Cooper C, Cooper M, Carter J, Russell P. Gonadoblastoma progressing to dysgerminoma in a 55-year-old woman with normal karyotype. Pathology. 2007;39(2):284–5.CrossRefPubMedGoogle Scholar
  62. 62.
    Bianco B, Lipay MV, Melaragno MI, Guedes AD, Verreschi IT. Detection of hidden Y mosaicism in Turner’s syndrome: importance in the prevention of gonadoblastoma. J Pediatr Endocrinol Metab. 2006;19(9):1113–7.CrossRefPubMedGoogle Scholar
  63. 63.
    Gajjar A, Ribeiro RC, Mahmoud HH, Sandlund JT, Liu Q, Furman WL, Santana VM, Crist WM, Rivera GK, Pui CH. Overt testicular disease at diagnosis is associated with high risk features and a poor prognosis in patients with childhood acute lymphoblastic leukemia. Cancer. 1996;78(11):2437–42.CrossRefPubMedGoogle Scholar
  64. 64.
    Finklestein JZ, Miller DR, Feusner J, Stram DO, Baum E, Shina DC, Johnson DG, Gyepes MT, Hammond GD. Treatment of overt isolated testicular relapse in children on therapy for acute lymphoblastic leukemia. A report from the Children’s Cancer Group. Cancer. 1994;73(1):219–23.CrossRefPubMedGoogle Scholar
  65. 65.
    Jacobs JE, Hastings C. Isolated extramedullary relapse in childhood acute lymphocytic leukemia. Curr Hematol Malig Rep. 2010;5(4):185–91.CrossRefPubMedGoogle Scholar
  66. 66.
    Arima K, Hasegawa D, Ogawa C, Kato I, Imamura T, Takusagawa A, Takahashi H, Kitagawa Y, Hori T, Tsurusawa M, Manabe A, Hosoya R. Detection of submicroscopic disease in the bone marrow and unaffected testis of a child with T-cell acute lymphoblastic leukemia who experienced “isolated” testicular relapse. Int J Hematol. 2009;90(3):370–3.CrossRefPubMedGoogle Scholar
  67. 67.
    Srikanth MS, West BR, Ishitani M, Isaacs H Jr, Applebaum H, Costin G. Benign testicular tumors in children with congenital adrenal hyperplasia. J Pediatr Surg. 1992;27(5):639–41.CrossRefPubMedGoogle Scholar
  68. 68.
    Ghazi AA, Hadayegh F, Khakpour G, Azizi F, Melby JC. Bilateral testicular enlargement due to adrenal remnant in a patient with C11 hydroxylase deficiency congenital adrenal hyperplasia. J Endocrinol Invest. 2003;26(1):84–7.CrossRefPubMedGoogle Scholar
  69. 69.
    Erdogan S, Ergin M, Cevlik F, Yuksel B, Tuncer R, Tunali N, Polat S. Testicular adrenal rest hyperplasia due to 21-hydroxylase deficiency: a case report. Endocr Pathol. 2006;17(1):83–7.CrossRefPubMedGoogle Scholar
  70. 70.
    Connolly JA, Gearhart JP. Management of yolk sac tumors in children. Urol Clin North Am. 1993;20:7.PubMedGoogle Scholar
  71. 71.
    Haas RJ, Schmidt P, Göbel U, Harms D. Testicular germ cell tumors an update—results of the German Cooperative Studies 1982–1997. Klin Paediatr. 1999;211:300–4.CrossRefGoogle Scholar
  72. 72.
    Lo Curto M, Lumia F, Alaggio R, et al. Malignant germ cell tumors in childhood: results of the first Italian cooperative study “TCG 91”. Med Pediatr Oncol. 2003;41:417–25.CrossRefPubMedGoogle Scholar
  73. 73.
    Mann JR, Raafat F, Robinson K, et al. The United Kingdom Children’s Cancer Study Group’s second germ cell tumor study: carboplatin, etoposide, and bleomycin are effective treatment for children with malignant extracranial germ cell tumors, with acceptable toxicity. J Clin Oncol. 2000;18:3809–18.CrossRefPubMedGoogle Scholar
  74. 74.
    Rogers PC, Olson TA, Cullen JW, et al. Treatment of children and adolescents with stage II testicular and stages I and II ovarian malignant germ cell tumors: a Pediatric Inter Group study—Pediatric Oncology Group 9048 and Children’s Cancer Group 8891. J Clin Oncol. 2004;22:3563–9.CrossRefPubMedGoogle Scholar
  75. 75.
    Schlatter M, Rescorla F, Giller R, et al. Excellent outcome in patients with stage I germ cell tumors of the testes: a study of the Children’s Cancer Group/Pediatric Oncology Group. J Pediatr Surg. 2003;38:319–24.CrossRefPubMedGoogle Scholar
  76. 76.
    Cushing B, Giller R, Cullen JW, et al. Randomized comparison of combination chemotherapy with etoposide, bleomycin, and either high-dose or standard-dose cisplatin in children and adolescents with high-risk malignant germ cell tumors: a Pediatric Inter group study—Pediatric Oncology Group 9049 and Children’s Cancer Group 8882. J Clin Oncol. 2004;22:2691–700.CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany 2018

Authors and Affiliations

  1. 1.American University of Beirut Medical CenterRiad El Solh, BeirutLebanon
  2. 2.Department of Surgery, Division of UrologyThe Hospital for Sick Children, University of TorontoTorontoCanada

Personalised recommendations