What is the management approach for suspected testicular tumors in children?

Management of Testicular Tumors in Children

Initial Diagnostic Evaluation

Any solid testicular mass in a child must be managed as malignant until proven otherwise, requiring immediate scrotal ultrasound with Doppler and serum tumor markers. 1

Essential Diagnostic Steps

• Scrotal ultrasound with Doppler is the mandatory first-line imaging modality, with nearly 100% sensitivity for detecting intrascrotal masses and 98-100% accuracy for distinguishing intratesticular from extratesticular processes 2, 3

• Serum tumor markers (AFP, β-HCG, LDH) must be drawn and measured before any treatment, including orchiectomy 1, 2

  • AFP is particularly important in children as yolk sac tumors (the most common pediatric testicular malignancy) produce elevated AFP 4, 3, 5
  • Pure seminoma does not secrete AFP; elevated AFP indicates non-seminomatous histology even if pathology suggests seminoma 1
  • Normal AFP with testicular mass requires repeat measurement, as 87% of pediatric cases have elevated preoperative markers 6

• Baseline blood work including complete blood count, creatinine, electrolytes, and liver enzymes should be obtained if an intratesticular mass is identified 2

Ultrasound Interpretation in Children

• Benign features: mainly cystic component, well-defined borders, echogenic rim, or normal to increased echogenicity compared to healthy parenchyma 3

• Malignant features: inhomogeneous, hypoechoic, poorly circumscribed, or diffuse infiltration pattern 3

• Important caveat: These ultrasonographic findings may overlap significantly in pediatric patients, making intraoperative frozen section critical 6, 3

Age-Specific Considerations

Prepubertal boys (<12 years) have predominantly benign tumors (71% non-germinal), while pubertal/adolescent boys have higher rates of malignancy similar to adults. 4, 3

Prepubertal Children (Most Common: Yolk Sac Tumor and Teratoma)

• Yolk sac tumors represent 49% of pediatric testicular tumors 4
• Mature teratoma (prepubertal-type) is the most common benign histologic type 3
• Metastases occur less frequently (9%) compared to adults (61%) 4
• Dissemination is predominantly hematogenous rather than lymphatic 4

Adolescents/Post-Pubertal

• Tumor behavior and treatment should follow adult protocols, not pediatric protocols, as inferior outcomes occur when post-pubertal males <18 years are treated according to pediatric guidelines 1

Surgical Management Algorithm

Radical Inguinal Orchiectomy (Standard Approach)

Radical inguinal orchiectomy through an inguinal incision is the standard surgical approach for suspected malignant testicular tumors in children. 1

• Never use a scrotal approach for suspected malignancy, as scrotal violation is associated with higher local recurrence rates 1, 2
• The tumor-bearing testis is resected with the spermatic cord at the level of the internal inguinal ring 1
• Surgery should be performed before any further treatment unless life-threatening metastatic disease requires immediate chemotherapy 1

Testis-Sparing Surgery (Selective Use)

Testis-sparing surgery with intraoperative frozen section is appropriate for highly selected pediatric cases, particularly when ultrasound suggests benign pathology. 6, 3

Indications for Testis-Sparing Approach:

• Masses <2 cm with equivocal ultrasound/physical exam findings and negative tumor markers 1
• Preoperative ultrasound findings suggestive of benign tumor (97% accuracy in one pediatric series) 6
• Bilateral testicular tumors or solitary testis 1

Technical Requirements:

• Intraoperative frozen section biopsy is mandatory before proceeding with tumorectomy 1, 6
• 98% concordance between intraoperative biopsy and final pathology in pediatric series 6
• Tumorectomy with safety margins preserving healthy testicular tissue 6
• Only 6% of patients required subsequent orchiectomy after initial tumorectomy in multicenter pediatric study 6

Fertility Preservation

Sperm banking must be discussed and offered before any therapeutic intervention in post-pubertal boys, particularly those without a normal contralateral testis or with known subfertility. 1, 2

• This discussion should occur before orchiectomy, not after 1
• Determination of total testosterone, LH, and FSH should be performed before operation 1
• Semen analysis and sperm banking should be completed before operation or chemotherapy 1

Staging and Post-Operative Management

Tumor Marker Kinetics

Tumor markers must be repeated after orchiectomy until normalization, with adequate time allowed for marker half-lives (HCG: 24-36 hours; AFP: 5-7 days). 1

• Markers should be drawn within 10 days before making management decisions 1
• Persistently elevated or rising markers indicate metastatic disease requiring systemic therapy 1

Staging Imaging

• Chest imaging is mandatory in all newly diagnosed germ cell tumors 1
• Abdominopelvic imaging (CT or MRI) to evaluate retroperitoneal lymph nodes 1, 3
• Chest CT specifically indicated if elevated/rising post-orchiectomy markers or evidence of metastases 1
• PET scanning should NOT be obtained for initial staging 1

Risk Stratification

Patients must be assigned TNM-s category and IGCCCG risk group (good, intermediate, or poor-risk) based on histology, presence/absence of non-pulmonary visceral metastasis, and post-orchiectomy tumor markers. 1

Stage-Specific Treatment

Clinical Stage I Disease (Confined to Testis)

Prepubertal Yolk Sac Tumor (<2 years old):

• Orchiectomy alone is sufficient for infants <2 years with stage I disease 4, 5
• No adjuvant chemotherapy required if no vascular invasion and markers normalize 5
• Retroperitoneal lymph node dissection (RPLND) is not necessary in pediatric stage I disease 5

Prepubertal Yolk Sac Tumor (≥2 years old):

• Orchiectomy followed by adjuvant chemotherapy is recommended 4
• Chemotherapy indicated in approximately 15% of pediatric testicular malignancies overall 4

High-Risk Features Requiring Chemotherapy:

• Vascular invasion on pathology 5
• Bilateral testicular tumors 5
• Age ≥2 years with yolk sac tumor 4

Mature Teratoma (Prepubertal-Type):

• Orchiectomy alone is curative 4
• No adjuvant therapy required 4

Stage I Seminoma (Adolescents/Post-Pubertal):

• Surveillance is the preferred approach after orchiectomy, as >80% are cured with surgery alone 1
• Adjuvant carboplatin or radiotherapy are less preferred alternatives 1

Metastatic Disease:

• Good-risk disease: BEP (bleomycin, etoposide, cisplatin) x3 cycles or EP (etoposide, cisplatin) x4 cycles 1, 7
• Intermediate or poor-risk disease: BEP x4 cycles 1

Multidisciplinary Management

Management decisions must be made in a multidisciplinary setting involving experienced clinicians in pediatric urology, medical oncology, pathology, and radiology. 1

• Expert pathology review should be considered, as testicular cancers are heterogeneous with multiple histologic elements 1
• Management decisions should be based on imaging within 4 weeks and tumor markers within 10 days 1

Follow-Up and Surveillance

• Mean follow-up in pediatric series: 39 months with no cases of testicular atrophy after tumorectomy 6
• Prognosis is excellent in pediatric testicular tumors overall 3
• Stage I yolk sac tumor treated with orchiectomy alone: no recurrence in 9/10 patients at 3-year mean follow-up 5
• One patient with bilateral disease developed metastases at 23 months but achieved complete remission with salvage chemotherapy 5

Critical Pitfalls to Avoid

• Never perform scrotal biopsy or scrotal incision for suspected malignancy—only inguinal approach is appropriate 1, 2
• Never delay obtaining tumor markers before orchiectomy, as this information is essential for staging 1
• Never treat post-pubertal adolescents with pediatric protocols—they require adult treatment algorithms 1
• Never perform RPLND routinely in pediatric stage I disease—it is not necessary 5
• Never dismiss the need for fertility counseling in post-pubertal boys before any intervention 1
• Never assume benign pathology without intraoperative frozen section when attempting testis-sparing surgery 6