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Hermann, A.;  L’herminé-Coulomb, A.;  Irtan, S.;  Audry, G.;  Cardoen, L.;  Brisse, H.J.;  Perre, S.V.;  Pointe, H.D.L. Epidemiology and Management  of Pediatric Testicular Tumors. Encyclopedia. Available online: https://encyclopedia.pub/entry/38209 (accessed on 27 July 2024).
Hermann A,  L’herminé-Coulomb A,  Irtan S,  Audry G,  Cardoen L,  Brisse HJ, et al. Epidemiology and Management  of Pediatric Testicular Tumors. Encyclopedia. Available at: https://encyclopedia.pub/entry/38209. Accessed July 27, 2024.
Hermann, Anne-Laure, Aurore L’herminé-Coulomb, Sabine Irtan, Georges Audry, Liesbeth Cardoen, Hervé J. Brisse, Saskia Vande Perre, Hubert Ducou Le Pointe. "Epidemiology and Management  of Pediatric Testicular Tumors" Encyclopedia, https://encyclopedia.pub/entry/38209 (accessed July 27, 2024).
Hermann, A.,  L’herminé-Coulomb, A.,  Irtan, S.,  Audry, G.,  Cardoen, L.,  Brisse, H.J.,  Perre, S.V., & Pointe, H.D.L. (2022, December 07). Epidemiology and Management  of Pediatric Testicular Tumors. In Encyclopedia. https://encyclopedia.pub/entry/38209
Hermann, Anne-Laure, et al. "Epidemiology and Management  of Pediatric Testicular Tumors." Encyclopedia. Web. 07 December, 2022.
Epidemiology and Management  of Pediatric Testicular Tumors
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This entry is adapted from the peer-reviewed paper 10.3390/cancers14133180

Pediatric testicular tumors are rare and represent 1% to 2% of all solid tumors in children. Germ-cell tumors are the most frequent etiology; most are benign in the pre-pubertal population, whereas post-pubertal testicular tumors are similar to those that occur in adults, with potential malignancy. 

pediatric testicular tumors tumor cell

1. Epidemiology of Pediatric Testicular Tumors

Pediatric testicular tumors are rare and represent 1% to 2% of all pediatric solid tumors. In patients < 14 years old, the incidence is estimated at 0.5 to 2 per 100,000 children [1][2]. Testicular tumors have a bimodal distribution: the first peak is seen in boys < 3 years of age, most being benign, and the second occurs after puberty, from age 15 to 19 years, with a higher proportion of malignant neoplasms. This observation highlights two distinct patterns of disease leading to different approaches to treatment.
Among all prepubertal intra-testicular tumors, 90% are germ-cell tumors (GCTs) and 10% to 15% are epidermoid cysts [3][4][5]. Reports suggest that benign GCTs are frequent, approximately 40% to 50% being teratomas. Most teratomas show mature elements, but immature teratomas in this age group have been reported [6]. Epidermoid cysts are always benign. Malignant GCTs are mainly represented by yolk-sac tumors, with a frequency ranging from 8% to 30% [4]. Among non-GCTs, Leydig cell tumors are more frequent, representing approximately 5% of the etiologies, followed by juvenile stromal granulosa cell tumors. The incidence of prepubertal testicular tumors peaks in the first three years of life [7]. As compared with yolk-sac tumors, teratomas usually develop in younger children, with a median age of approximately 8 to 13 months and 16 to 17 months, respectively [4][8]. Among prepubertal testicular malignancies, the 5-year relative survival is 97% for children with localized tumors and 73% for those with distant disease [9].
Between ages 4 to 11 years, the diagnosis of a testicular GCT is rare. In contrast, in post-pubertal adolescents 15 to 19 years old, the incidence increases and represents 12% of all cancers in adolescents [10]. In this population, tumors are more likely to be malignant and include immature teratomas, mixed non-seminomatous malignant GCTs and embryonal carcinomas [11]. In young adults, 95% of testicular cancers are malignant GCTs, and 50% of these are seminomas. The 2016 World Health Organization classification system redefines testicular GCTs into two main groups: those derived from germ-cell neoplasia in situ, which are representative of post-pubertal lesions, and those not derived from germ-cell neoplasia in situ, which characterize prepubertal neoplasms [12].
One specific tumor type is the gonadoblastoma, which includes both germ-cell and sex-cord stromal-cell tumor types and occurs almost exclusively in the setting of differences in sexual differentiation [13]. In patients presenting a scrotal mass, a para-testicular mass should also be accounted for in the differential diagnosis. Para-testicular rhabdomyosarcoma is the most frequent para-testicular malignant mass in children; it represents 5% of all testicular and para-testicular malignancies. The para-testicular tumor spectrum remains wide and also includes benign lesions such as hemangiomas, leiomyomas, fibromas, lipoblastomas [14], cystic lymphangiomas and the rare malignant melanotic neuroectodermal tumor of infancy [15]. A classification of testicular tumors in children is presented in Table 1.
Table 1. Classification of intratesticular tumors in children.
Tumoral Group Histotypes Prevalence (%)
Benign Germ-cell tumor Mature teratoma and dermoid cysts 40–50%
Non seminomatous malignant germ-cell tumor Immature teratoma rare
Yolk sac tumor 8–30%
(<2 years)
Choriocarcinoma rare
Embryonal carcinoma rare
Mixed non seminomatous malignant germ-cell tumor More frequent in post-pubertal patients
Seminomatous malignant germ-cell tumor Seminoma Exceptional in children
Sex-cord stromal tumors Leydig-cell tumor 5%
Sertoli-cell tumor <1%
Juvenile granulosa cell tumor <5%
Mixed sex-cord stromal tumor <5%
Pseudo-tumors Epidermoid cyst 10–15%
Testicular adrenal rest tumor 30–50% in the context of congenital adrenal hyperplasia
Secondary testicular involvement of lymphoma or leukemia   1–7%
Secondary involvement of primary solid tumor   <1%
Others   <1%
Testicular tumor markers are a major tool in evaluating pediatric testicular tumors; these include alpha-fetoprotein (AFP) and beta-human chorionic gonadotrophin (BHCG). Serum AFP levels are normally higher in children than in adults and are reduced to normal levels by age 1 (<10 ng/mL). Therefore, for children < 1 year old with testicular tumors, AFP level may be elevated in those with benign tumors, whereas for children > 1 year, a normal AFP level often indicates a benign tumor. Elevated serum AFP level is strongly associated with >90% of yolk-sac tumors, whereas immature teratomas may present a slightly elevated serum AFP level [16]. AFP should be measured before any therapeutic intervention and after surgery to assess an appropriate decrease in level.
BHCG is elevated in choriocarcinomas, embryonal carcinomas or seminomas, which are extremely rare in prepubertal boys but could be seen in adolescents. Thus, it is not useful in the diagnostic work-up of prepubertal boys with a testicular tumor but is useful in adolescents presenting a testicular mass [8]. Lactate dehydrogenase is also useful because high levels are associated with bulky disease and increasing levels after therapy may signify disease recurrence.

2. Management of Pediatric Testicular Tumors

For decades, TSS has been reported to control pediatric testicular benign tumors, with potential psychologic, cosmetic and functional advantages. The size of the tumor is an important factor in choosing the therapeutic option, with significantly smaller lesions in children after TSS versus radical orchidectomy [1]. Actually, a cut-off tumor size of <2.5 cm is generally considered a good candidate for TSS [1]. TSS has shown reliability and feasibility with no atrophy and rare cases of recurrence [17][18]. A recent systematic review [19] approved this strategy in selected cases of prepubertal testicular tumors.
However, tumor size only does not seem a sufficient criterion to differentiate benign from malignant neoplasms. Imaging, especially US, when combined with clinical data and tumor marker levels, is a reliable protocol for the differential diagnosis of benign and malignant lesions. However, intra-operative frozen section analysis seems to more accurately predict the pathological diagnosis [20], which suggests that it should be systematically performed during TSS to confirm the benign diagnosis and that microscopically margin-negative resection has been achieved. In the case of microscopic positive margins in a malignant or potentially malignant tumor, orchidectomy should be performed [8]. Orchidectomy with the inguinal approach should be considered if normal testicular parenchyma is no longer detectable on preoperatively high-resolution US and/or with AFP level > 100 ng/mL in a child > 12 months, considering high suspicion of yolk-sac tumor [8], or with AFP level > 23 ng/mL in a child < 8 months with a tumor > 2.5 cm because of increased frequency of immature teratomas [21].

References

  1. Ye, Y.-L.; He, Q.-M.; Zheng, F.-F.; Guo, S.-J.; Zhou, F.-J.; Qin, Z.-K. Trends of testis-sparing surgery for pediatric testicular tumors in South China. BMC Surg. 2017, 17, 31.
  2. Jiménez Isabel, M.A.; Gómez Fraile, A.; Aransay Brantot, A.; López Vázquez, F.; Delgado Muños, M.D.; Encinas Goenechea, A.; Matute de Cárdenas, M.D.; Berchi García, F.J. Testicular tumors in childhood. Review of cases in the course of 13 years. Cir. Pediatr. 1996, 9, 13–16.
  3. Metcalfe, P.D.; Farivar-Mohseni, H.; Farhat, W.; McLorie, G.; Khoury, A.; Bägli, D.J. Pediatric Testicular Tumors: Contemporary Incidence and Efficacy of Testicular Preserving Surgery. J. Urol. 2003, 170, 2412–2416.
  4. Wang, X.; Xu, S.; Tang, D.; Li, M.; Wu, D.; Huang, Y. Prepubertal testicular and paratesticular tumors in China: A single-center experience over a 10-year period. J. Pediatr. Surg. 2012, 47, 1576–1580.
  5. Friend, J.; Barker, A.; Khosa, J.; Samnakay, N. Benign scrotal masses in children—Some new lessons learned. J. Pediatr. Surg. 2016, 51, 1737–1742.
  6. Li, Z.; Zhang, W.; Song, H.; Sun, N. Testis-Preserving Tumor Enucleation Is Applicable in Children with Immature Testicular Teratoma. Urol. Int. 2021, 105, 27–30.
  7. Bujons, A.; Sfulcini, J.C.; Pascual, M.; Feu, O.A.; Garat, J.M.; Villavicencio, H. Prepubertal testicular tumours and efficacy of testicular preserving surgery. Br. J. Urol. 2011, 107, 1812–1816.
  8. Stein, R.; Quaedackers, J.; Bhat, N.R.; Dogan, H.S.; Nijman, R.J.; Rawashdeh, Y.F.; Silay, M.S.; Hoen, L.A.; Tekgul, S.; Radmayr, C.; et al. EAU-ESPU pediatric urology guidelines on testicular tumors in prepubertal boys. J. Pediatr. Urol. 2021, 17, 529–533.
  9. Alanee, S.; Shukla, A. Paediatric testicular cancer: An updated review of incidence and conditional survival from the Surveillance, Epidemiology and End Results database. Br. J. Urol. 2009, 104, 1280–1283.
  10. Kusler, K.A.; Poynter, J.N. International testicular cancer incidence rates in children, adolescents and young adults. Cancer Epidemiol. 2018, 56, 106–111.
  11. O’Shea, K.; Tong, A.; Farrelly, P.; Craigie, R.; Cheesman, E.; Shukla, R.; Losty, P. Management and outcome of paediatric testicular tumours—A 20 year experience. J. Pediatr. Surg. 2021, 56, 2032–2036.
  12. Williamson, S.R.; Delahunt, B.; Magi-Galluzzi, C.; Algaba, F.; Egevad, L.; Ulbright, T.M.; Tickoo, S.K.; Srigley, J.R.; Epstein, J.; Berney, D.; et al. The World Health Organization 2016 classification of testicular germ cell tumours: A review and update from the International Society of Urological Pathology Testis Consultation Panel. Histopathology 2017, 70, 335–346.
  13. Roth, L.M.; Cheng, L. Gonadoblastoma: Origin and outcome. Hum. Pathol. 2020, 100, 47–53.
  14. Yada, K.; Ishibashi, H.; Mori, H.; Shimada, M. Intrascrotal lipoblastoma: Report of a case and the review of literature. Surg. Case Rep. 2016, 2, 34.
  15. Soles, B.S.; Wilson, A.; Lucas, D.R.; Heider, A. Melanotic Neuroectodermal Tumor of Infancy. Arch. Pathol. Lab. Med. 2018, 142, 1358–1363.
  16. Schneider, D.T.; Calaminus, G.; Göbel, U. Diagnostic value of alpha1-fetoprotein and beta-human chorionic gonadotropin in infancy and childhood. Pediatr. Hematol. Oncol. 2001, 18, 11–26.
  17. Ross, J.H. Prepubertal Testicular Tumors. Urology 2009, 74, 94–99.
  18. Radford, A.; Peycelon, M.; Haid, B.; Powis, M.; Lakshminarayanan, B. Testicular-sparing surgery in the pediatric population: Multicenter review of practice with review of the literature. Curr. Opin. Urol. 2019, 29, 481–486.
  19. Kooij, C.D.; Hulsker, C.C.; Kranendonk, M.E.; Zsiros, J.; Littooij, A.S.; Looijenga, L.H.; Klijn, A.J.; Mavinkurve-Groothuis, A.M. Testis Sparing Surgery in Pediatric Testicular Tumors. Cancers 2020, 12, 2867.
  20. Caldwell, B.T.; Saltzman, A.F.; Maccini, M.A.; Cost, N.G. Appropriateness for testis-sparing surgery based on the testicular tumor size in a pediatric and adolescent population. J. Pediatr. Urol. 2019, 15, 70.e1–70.e6.
  21. Chang, M.-Y.; Shin, H.J.; Kim, H.G.; Kim, M.-J.; Lee, M.-J. Prepubertal Testicular Teratomas and Epidermoid Cysts: Comparison of Clinical and Sonographic Features. J. Ultrasound Med. 2015, 34, 1745–1751.
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Subjects: Medicine, Research & Experimental
Contributors MDPI registered users' name will be linked to their SciProfiles pages. To register with us, please refer to https://encyclopedia.pub/register :
Anne-Laure Hermann
,
Aurore L’Herminé-Coulomb
,
Sabine Irtan
,
Georges Audry
,
Liesbeth Cardoen
,
Hervé J. Brisse
,
Saskia Vande Perre
,
Hubert Ducou Le Pointe
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Update Date: 08 Dec 2022
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