Pediatric Teratomas and Other Germ Cell Tumors Treatment & Management

Updated: Jul 31, 2019
  • Author: E Stanton Adkins, III, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Treatment

Approach Considerations

For children with extracranial germ cell tumors, surgery is an essential component of treatment. Depending on the clinical factors present, appropriate treatment may involve one of the following [18] :

  • Surgical resection followed by careful monitoring for disease recurrence
  • Initial surgical resection followed by platinum-based chemotherapy
  • Diagnostic tumor biopsy and preoperative platinum-based chemotherapy followed by definitive tumor resection
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Medical Care

In the United States, the standard chemotherapy regimen for both adults and children with malignant nonseminomatous germ cell tumors includes cisplatin, etoposide, and bleomycin (PEB). An every-21-days regimen that has been used is as follows [18]

  • Bleomycin - 15 units/m2 on day 1 (maximum, 30 units)
  • Etoposide - 100 mg/m2 on days 1-5
  • Cisplatin - 20 mg/m2 on days 1-5

The combination of carboplatin, etoposide, and bleomycin has been studied in the United Kingdom but has not been compared with PEB in a trial focusing on pediatric germ cell tumors. [18]

Radiation therapy is rarely recommended. [18]

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Surgical Care

In general, gross total resection of tumor is the goal. The tumor and involved adjacent structures should be resected en bloc, if this is possible and does not lead to disfigurement.

Sacrococcygeal tumor

Typically, the surgeon approaches this tumor through a posterior transsacral route. The coccyx must be resected en bloc with the tumor to minimize the risk of recurrence. Control and division of the middle sacral artery early in the procedure is advisable. If the sacrum or rectum is invaded by the tumor, complete resection may not be advisable at the initial operation. Treating these tumors with chemotherapy is reasonable, with resection after the maximum response is obtained.

When the tumor extends high into the pelvis and abdomen, laparotomy or laparoscopy is required in addition to the posterior incision. Ascitic fluid should be collected or peritoneal washings obtained. The tumor may then be mobilized for removal from below or above, depending on the anatomy. Samples should be obtained in lymph nodes from the retroperitoneum. In tumors with a moderate pelvic component, laparoscopy may allow clip placement in the middle sacral artery and mobilization of the pelvic portion of the tumor.

Cowles et al reported preoperative embolization of the major vessels that supply a large teratoma followed by radiofrequency ablation (RFA) of the zone between normal tissue and tumor. [38] Damage to the nerves supplying the leg has been reported with antenatal RFA.

Ovarian tumor

Open resection is the preferred approach to these tumors. (See the image below.) Typically, laparoscopy requires morcellation of the tumor in a bag. The consequent destruction of the tumor capsule prevents pathologic staging; thus, patients must be treated as stage II. Appropriate measures should be taken to minimize tissue dispersion and the potential for tumor dissemination. [39]

Ovarian yolk sac tumor at surgery. Ovarian yolk sac tumor at surgery.

Ascitic fluid or peritoneal washings should undergo cytologic analysis. The entire peritoneal cavity should be inspected. Any suspicious implants should be sampled or resected. Gliomatosis peritonei does not worsen the stage of a tumor, but all implants must have mature glial tissue. Immature tissue suggests metastatic disease and requires more intensive therapy. The omentum must be inspected. If disease is possible (eg, adherence, nodules, implants), the affected area should be resected at this time.

Ipsilateral oophorectomy or salpingo-oophorectomy should be performed. Uninvolved fallopian tubes should be preserved if possible. In cases of mature teratoma, the contralateral ovary should be inspected. If it appears normal, it should be left alone. Bilateral malignant tumors require bilateral oophorectomy, but hysterectomy is unnecessary for germ cell tumors. Some authors advocate ovary-sparing resection of mature teratomas. This is not always possible.

Samples of suspicious and involved lymph nodes should be obtained. Random bilateral sampling is no longer required because it did not have an impact on survival in the last Intergroup study. [30]

Testicular tumor

Testicular teratomas may be treated with local resection in prepubertal patients. The tumor should be removed with a small rim of normal testicle. If the testicular tissue shows signs of pubertal change, radical inguinal orchiectomy should be performed.

In all malignant cases, radical inguinal orchiectomy should be performed with high ligation of the spermatic cord. For very large tumors, the incision may be enlarged by extending the medial portion of the incision downward into the upper scrotum.

Transscrotal resection with intact capsule is now treated as a stage I tumor, provided that the cord structures are completely removed and are uninvolved. If transscrotal biopsy was performed prior to resection, the stage is at least stage II. Because most of these preadolescent tumors are responsive to chemotherapy, hemiscrotectomy is rarely necessary.

If images do not reveal lymph node enlargement, sampling of ipsilateral retroperitoneal lymph nodes is not required. When images show positive findings of nodal enlargement of 2-4 cm, perform a biopsy of the enlarged nodes. Nodes with a diameter exceeding 4 cm are treated as stage III metastatic disease and do not require biopsy. Tumor debulking is no longer recommended.

Mediastinal tumor

The approach to the resection may be via median sternotomy or lateral thoracotomy. Small lesions have been resected by using video-assisted thoracoscopic surgery (VATS). Large lesions may cause airway compromise and require intubation and care in the intensive care unit (ICU). Many of these large tumors are best managed with initial biopsy, neoadjuvant chemotherapy, and delayed complete resection. [40]

Adherent nonvital structures (eg, pericardium and thymus) should be removed en bloc with the tumor. Lymph nodes should be sampled.

A study of children and adolescents with primary mediastinal and retroperitoneal germ cell tumors, conducted by the French Society of Pediatric Oncology, found that a strategy of delayed aggressive surgery after cisplatin-based chemotherapy yielded a favorable prognosis in children. [41] ​ With aggressive surgery, a microscopic complete resection was achieved in 12 of the 15 patients in the primary mediastinal group and in four of the five in the retroperitoneal group. Overall survival as 88% (14/16) for the former group and 80% (4/5) for the latter. The prognosis was better in younger patients; three adolescents died of tumor progression.

Neck tumor

These lesions present special surgical challenges. In large congenital lesions, the airway may be compromised, and intubation may be difficult. The ex-utero intrapartum treatment (EXIT) procedure, in which a cesarean delivery is performed and the neonate remains attached to the placenta, may allow enough time for bronchoscopic airway placement.

Resection should be total but not at the expense of vital structures. A staged procedure is acceptable in this circumstance. Complete resection may then be possible after chemotherapy.

Recurrent disease

Recurrent disease must be surgically staged. The extent of disease is an important prognostic factor. Surgically resectable recurrent disease has a far more favorable prognosis than unresectable disease. The best prognosis exists when complete surgical resection is accompanied by high-intensity chemotherapy with autologous stem cell rescue.

Additionally, recurrent disease may have a different tissue type than that of the original tumor. PNET, for example, is a frequent component of germ cell tumors that may not respond to bleomycin-etoposide-cisplatin (BEP) therapy.

Metastatic disease

When these tumors are metastatic, initial chemotherapy may lead to resolution of metastatic disease. If resolution is not achieved, residual disease may be necrotic tumor, mature teratoma, persistent malignant disease, or combinations of the above. No current radiologic test reliably distinguishes between these possibilities. Surgical biopsy may help guide therapy. Resection is recommended when possible. [42]

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Complications

Complications of chemotherapy include, but are not limited to, the following:

  • Myelosuppression
  • Nephrotoxicity
  • Ototoxicity
  • Pulmonary failure
  • Neurotoxicity
  • Infertility
  • Secondary malignancy

Fertility is of particular importance for these patients. Adult males treated with chemotherapy and surgery similar to current pediatric protocols face a 20-30% reduction in fertility with standard-dose BEP. [43]  Among adult females, the results may be worse. In one study, only three of 26 patients were able to conceive, and none of these conceptions led to live births. [44]

Park et al analyzed oncologic and reproductive outcomes of 42 pediatric and young adolescents with malignant ovarian germ cell tumors treated with fertility-sparing surgery, of whom 31 received adjuvant BEP therapy. [45]  The 5-year disease-free survival (DFS) and overall survival (OS) rates were 85% and 97%, respectively. Seven of the surviving 41 patients were premenarchal, 30 had regular menstruation, and three had irregular menstruation. No patient had premature ovarian failure.

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Diet

Maintaining adequate nutrition is often difficult during chemotherapy. Additionally, intestinal obstruction may be a consequence of an abdominal tumor. Nutritional supplements or parenteral nutrition may be necessary. In cases other than those involving frank obstruction, enteral tube feeding has proven useful.

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Consultations

Psychological support is important for both the patient and the family after any diagnosis of cancer. For older patients, fertility issues, as well as issues of sexual identity, may also be important.

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Long-Term Monitoring

All patients with sacrococcygeal teratomas should be monitored with serial rectal examinations and serum markers every 3 months for the first 3 years to detect signs of recurrence.

Computed tomography (CT) or magnetic resonance imaging (MRI) may also be helpful, especially in cases of questionable masses found during rectal examination, increased serum markers, or inadequate resection margins.

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