Operative management and outcomes of testicular tumors in children: a 10-year multicentric Egyptian experience

Testicular tumors in children are rare entities encompassing several pathological types that affect various pediatric age groups. Therefore, it is vitally important for treating surgeons to follow clear diagnostic and management strategies for these neoplasms. Pediatric testicular tumors exhibit two age peaks of incidence, indicating two distinct disease patterns [2, 3, 18]. Similarly, our study demonstrated a clear bimodal disease distribution, one peak in patients three years or younger and another after the onset of puberty. We observed that 81% of prepubertal patients (n = 21/26) had non-GCNIS derived tumors, specifically prepubertal-type mature teratoma and yolk sac tumors. Our findings were consistent with previous studies, which reported that non-GCNIS tumors represented 75–87.5% of patients in the prepubertal age group [14, 19, 20]. Seminomas and mixed malignant germ cell tumors constituted the majority of all pathological types among postpubertal patients in our study. This aligns with previous reports, which revealed these tumors were predominantly present in postpubertal patients [21, 22].

Children with testicular tumors typically present with a hard, painless scrotal swelling [9]. Some patients may also have a reactionary hydrocele, as noted in six patients from our study. Feminizing or virilizing syndrome may occur in some sex cord-stromal tumor cases [5, 18], but we did not observe these signs in any of our patients. Initial diagnosis generally relies on ultrasonography findings and serum tumor markers [23]. Testicular germ cell tumors secrete AFP, a specific marker for yolk sac tumors, with levels abnormally elevated in 90% of these cases [23]. In our study, we observed that 100% of patients with yolk sac tumors and 60% of those with mixed malignant germ cell tumors had abnormally elevated AFP levels at diagnosis. Additionally, no infant with a mature teratoma had an AFP level exceeding 100 ng/ml, which facilitated differentiation between teratomas and yolk sac tumors in infancy. Postpubertal tumors such as mixed malignant germ cell tumors produce hCG, which were rarely seen in prepubertal patients [22, 24]. Similarly, serum hCG was elevated in all patients with mixed malignant germ cell tumors in our study, while it was not elevated in any prepubertal patient. Tumor markers are valuable for initial tumor identification, treatment monitoring, and early relapse detection, and they are essential in selecting patients for TSS, as these markers should be negative for this procedure.

Surgical resection is the principal modality for treating pediatric testicular tumors, whether through radical orchiectomy or TSS. Surgery alone with close observation is strongly advocated for prepubertal patients, even those with yolk sac tumors, as over 85% of these tumors are stage I [16, 25]. However, Hass et al. and Schlatter et al. documented relapse rates of 15% and 20.6%, respectively, among stage I yolk sac tumors after orchiectomy, which were successfully treated with platinum-based chemotherapy [26, 27]. In our study, we treated all prepubertal patients with yolk sac tumors by surgery only followed by strict monitoring, and they remained disease-free without any oncological events. Similarly, O'Shea et al. reported that all their prepubertal patients with yolk sac tumors were exclusively managed with orchiectomy, demonstrating an excellent prognosis [14]. We confirm that a surgery-alone strategy is effective for stage I testicular tumors, regardless of pathological variant or patient age, and achieves excellent outcomes. However, strict follow-up is imperative to detect any recurrences early.

TSS is currently a preferred approach to prevent unnecessary orchiectomy in children. The oncological safety of TSS has been established, with several studies demonstrating favorable outcomes and very low recurrence rates in carefully selected prepubertal patients with benign tumors, specifically mature teratomas [11,12,13]. This technique preserves testicular tissue, providing notable cosmetic and psychological benefits, as well as crucial advantages for future fertility [28]. In our study, TSS was successfully performed for 25% of all patients (including teratomas, granulosa, and Leydig cell tumors), with no reported local recurrences. Interestingly, 57% of patients (n = 8/14) with mature teratomas required radical orchiectomy due to insufficient remaining testicular parenchyma, these patients could have benefited from TSS if they had been suitable candidates. We believe that identifying adequate healthy tissue is the key factor for performing TSS in any child with a benign tumor. Additionally, factors such as the surgeon's experience and the parents' preferences also influence the decision to perform TSS. Ultimately, we strongly encourage considering TSS for all prepubertal patients whenever possible and accurately determining appropriate candidates.

Retroperitoneal lymph node disease is rarely encountered in prepubertal patients, and adjuvant chemotherapy is very effective in children with stage II-IV tumors [29]. However, retroperitoneal nodal dissemination is more common in the postpubertal age group [16, 29]. In our study, all four patients with retroperitoneal nodal metastases were postpubertal. Adjuvant chemotherapy was administered after orchiectomy to these patients instead of primary RPLND, in order to minimize potential morbidity from sympathetic nerve injury and subsequent impotence. Nevertheless, RPLND was eventually performed for two of them to resect active tumor residuals. We observed that the outcomes in stage III-IV patients or the patient who had relapsed were very acceptable due to an excellent response to salvage chemotherapy, and there were no mortality among these patients. We confirm that precise monitoring of chemotherapy response through serial imaging studies and tumor markers is fundamental, as it may help save the child from undergoing extensive surgery like RPLND. Finally, this study has a few limitations, including a retrospective design and a small sample size. Despite these limitations, we believe that our findings will enhance knowledge and treatment of these rare tumors in Egypt. Additionally, future prospective studies with larger patient cohorts could provide a more comprehensive understanding and optimize outcomes for these neoplasms in our country.

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