Pediatric Teratomas and Other Germ Cell Tumors Treatment & Management

  • Author: E Stanton Adkins, III, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Apr 28, 2015
 

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.[18] Damage to the nerves supplying the leg has been reported with prenatal 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.

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.[19]

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 trans-scrotal 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. Many of these large tumors are best managed with initial biopsy, neoadjuvant chemotherapy, and delayed complete resection.[20]

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

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, and cisplatin (BEP)-type 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.

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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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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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Contributor Information and Disclosures
Author

E Stanton Adkins, III, MD Clinical Associate Professor, Departments of Pediatrics and Surgery, University of South Carolina School of Medicine

E Stanton Adkins, III, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Deborah F Billmire, MD Associate Professor, Department of Surgery, Indiana University Medical Center

Deborah F Billmire, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Phi Beta Kappa, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Rebeccah Brown, MD Associate Director of Trauma Services, Associate Professor, Department of Clinical Surgery and Pediatrics, Cincinnati Children's Hospital Medical Center and University of Cincinnati Hospital

Rebeccah Brown, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Medical Womens Association

Disclosure: Nothing to disclose.

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Sacrococcygeal teratoma in a female neonate. This particular tumor is largely external with no intrapelvic extension.
This is an ovarian mixed germ cell tumor in a 13-year-old girl. This tumor caused right lower quadrant pain. It is largely cystic in composition. No calcifications are observed within the mass.
Ovarian yolk sac tumor at surgery.
Yolk sac tumor of the testis. The tumor is metastatic to the retroperitoneum. It encases the aorta and renal arteries. The vena cava and renal veins are displaced anteriorly by the mass.
Esophagram in an infant with massive thoracic germ cell tumor. Note how the esophagus is displaced posteriorly and laterally by the left mediastinal tumor.
Chest radiograph of the patient in Media file 5 after treatment with chemotherapy. The size of the tumor has not decreased.
CT scan of the chest in the patient in Media file 6. The carina is displaced posteriorly and to the right. The vena cava is displaced anteriorly, and the aorta is compressed between the mass and the spine.
 
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