Prepubertal Testicular and Paratesticular Tumors Treatment & Management

Updated: Mar 03, 2020
  • Author: Christopher S Cooper, MD, FACS, FAAP; Chief Editor: Marc Cendron, MD  more...
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Treatment

Medical Care

Current chemotherapeutic regimens for yolk-sac tumors are platinum-based protocols. Common agents include etoposide, bleomycin, and cisplatin. Most often, a pediatric oncologist enrolls patients who require chemotherapy into a multigroup research protocol. The protocols vary, depending on the particular trial.

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Surgical Care

The treatment for yolk-sac tumors is inguinal orchiectomy and close surveillance. [25] The tumor usually spreads to the lungs.

Follow-up should include monthly tests of serum alpha fetoprotein (AFP) levels, chest radiography every 2 months for 2 years, and computed tomography (CT) or magnetic resonance imaging (MRI) of the retroperitoneum every 3 months for the first year and then biannually.

Because spread to the retroperitoneal lymph nodes is uncommon, routine prophylactic dissection of the nodes is not performed.

Chemotherapy is administered in patients with radiographic evidence of metastatic disease or persistently elevated serum AFP levels. The use of combination chemotherapy with cisplatin, etoposide, and bleomycin has been an effective treatment for metastatic disease, with a survival rate approaching 90%.

More than 99% of all patients with yolk-sac tumors are expected to survive.

Chemotherapy is recommended in all patients with yolk-sac tumors and stage II disease. In boys with persistently elevated levels of tumor markers after chemotherapy, dissection of the retroperitoneal lymph node may be required. Boys with stage III or IV germ-cell tumors are treated with chemotherapy. If elevated marker levels or retroperitoneal disease persists, biopsy or resection of residual tumor is performed.

Prepubertal testicular teratomas, Leydig-cell tumors, and Sertoli-cell tumors are benign, and orchiectomy or testis-sparing surgery [26] with complete excision is curative.

Stage II or higher teratocarcinomas require treatment with cisplatin, bleomycin, and vinblastine.

Treatment of gonadoblastoma involves removal of the gonad. Streak gonads are routinely removed because of the risk of malignant degeneration.

Seminoma is rare before puberty and is managed in a manner similar to that in the adult population.

Juvenile granulosa-cell tumor is managed with inguinal orchiectomy and follow-up chest radiography for 1 year. Juvenile granulosa-cell tumors rarely metastasize. Testis-sparing surgery has not been routinely recommended, though there is growing evidence that this approach is reliable and safe in selected patients. [27, 17, 28, 29, 18]

Perform radical inguinal orchiectomy to treat paratesticular rhabdomyosarcoma. If the scrotum is involved, hemiscrotectomy should be performed, with adjuvant chemotherapy.

All children with rhabdomyosarcoma require vincristine, doxorubicin (Adriamycin), and dactinomycin (VAC) chemotherapy or treatment with a combination of these drugs. Patients with evidence of metastatic disease may require radiation therapy.

Routine dissection of the retroperitoneal lymph nodes is recommended in all patients aged 10 years or older (regardless of the imaging results) and in patients younger than 10 years whose images show retroperitoneal disease.

Children younger than 10 years have a survival rate of nearly 95%. Children older than 10 years have a worse prognosis and an increased risk for involvement of the retroperitoneal lymph nodes; therefore, an aggressive approach with dissection of the retroperitoneal lymph nodes is recommended.

Routine dissection of the retroperitoneal lymph nodes is not performed to manage prepubertal testicular tumors. The incidence of metastases to the retroperitoneal lymph nodes is lower for these tumors than for postpubertal testicular tumors. However, the prognosis of children older than 10 years who have rhabdomyosarcoma is poor; modified retroperitoneal lymph node dissection is now recommended for these patients.

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Complications

Treatment-related complications include known short- and long-term effects of surgery, chemotherapy, and external-beam irradiation. The effects include risk of infertility, hemorrhagic cystitis, and subsequent development of secondary malignancy.

Children should be counseled regarding these risks before treatment.

Long-term follow-up care is required.

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Consultations

Consult a pediatric oncologist if chemotherapy is to be administered; consult radiation oncologists for external-beam irradiation, as indicated.

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