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Childhood Extracranial Germ Cell Tumors Treatment (PDQ®)

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Last Modified: 06/06/2014

Treatment of Mature and Immature Teratomas in Children

Mature Teratomas (Nonsacrococcygeal Sites)
        Standard treatment options for mature teratomas (nonsacrococcygeal sites)
Immature Teratomas (Nonsacrococcygeal Sites)
        Standard treatment options for immature teratomas (nonsacrococcygeal sites)
Mature and Immature Teratomas (Sacrococcygeal Site)
        Standard treatment options for mature and immature teratomas (sacrococcygeal sites)
Current Clinical Trials

Mature and immature teratomas arise primarily in the sacrococcygeal region of neonates and young children and in the ovaries of pubescent girls. Less commonly, these tumors are found in the testicular region of boys younger than 4 years, the mediastinum of adolescents, and other sites.[1-3] The primary treatment for teratomas is surgery and depends on whether the tumor forms in a nonsacrococcygeal or sacrococcygeal site. Surgical options for sacrococcygeal teratomas are complex. The number of pediatric patients with postoperative residual mature or immature teratomas is very small.

Mature Teratomas (Nonsacrococcygeal Sites)

Standard treatment options for mature teratomas (nonsacrococcygeal sites)

Standard treatment options for mature teratomas in a nonsacrococcygeal site include the following:

  1. Surgery and observation.

Children with mature teratomas, including mature teratomas of the mediastinum, can be treated with surgery and observation, with an excellent prognosis.[1,4] In a review of 153 children with nontesticular mature teratoma, the 6-year relapse-free survival was 96% for completely resected disease and 55% for incompletely resected disease.[2]

Head and neck germ cell tumors (GCTs) in neonates should be cared for by a multidisciplinary team. Although most head and neck GCTs are benign, they present significant challenges to surgeons. Some tumors develop malignant elements, which may change the treatment strategy.[5]

Mature teratomas in the prepubertal testis are relatively common benign lesions and may be amenable to testis-sparing surgery.[6]

Immature Teratomas (Nonsacrococcygeal Sites)

Standard treatment options for immature teratomas (nonsacrococcygeal sites)

Standard treatment options for immature teratomas in a nonsacrococcygeal site include the following:

  1. Surgery and observation (stage I).
  2. Surgery and chemotherapy (stages II through IV).

The treatment options for immature teratomas at a nonsacrococcygeal site differ by stage of disease.

Stage I

Infants and young children with immature teratomas have an excellent prognosis if the tumor can be completely resected.[7-9] For these patients, the current standard of treatment is surgery and observation.

Evidence (surgery and observation for stage I disease):

  1. The benefit of adjuvant chemotherapy for children was investigated in a study by the Pediatric Oncology Group and Children's Cancer Group. Surgical resection followed by careful observation was used to treat patients with immature teratomas.[10]
    • Surgery alone was curative for most children and adolescents with resected ovarian immature teratomas of any grade, even when elevated levels of serum alpha-fetoprotein (AFP) or microscopic foci of yolk sac tumor were present.

    • The study demonstrated a 3-year event-free survival of 97.8% for patients with ovarian tumors, 100% for patients with testicular tumors, and 80% for patients with extragonadal tumors.

Stages II through IV

There is significant debate on the responsiveness of immature teratomas to chemotherapy. In adults, and perhaps adolescents, immature teratomas (primarily ovarian) reportedly have an aggressive clinical behavior [11] requiring surgery and chemotherapy. The decision to use chemotherapy is based on the tumor stage (II through IV) and grade. Further studies on the treatment of ovarian immature teratomas with chemotherapy are lacking. In a pediatric report from the United Kingdom, immature teratomas did not respond to chemotherapy.[12] This study did not include patients older than 15 years. (Refer to the PDQ summary on Ovarian Germ Cell Tumors Treatment for more information about the treatment of ovarian immature teratomas in postpubertal females.)

Mature and Immature Teratomas (Sacrococcygeal Site)

The sacrococcygeal region is the primary tumor site for most benign and malignant GCTs diagnosed in neonates, infants, and children younger than 4 years. These tumors occur more often in girls than in boys; ratios of 3:1 to 4:1 have been reported.[13]

Sacrococcygeal tumors present in the following two clinical patterns related to the child’s age, tumor location, and likelihood of tumor malignancy:[1]

  • Neonates: Neonatal tumors present at birth protruding from the sacral site and are usually mature or immature teratomas.

  • Infants and young children: Among infants and young children, the tumor presents as a palpable mass in the sacro-pelvic region compressing the bladder or rectum. These pelvic tumors are more likely to be malignant. An early survey found that the rate of tumor malignancy was 48% for girls and 67% for boys older than 2 months at the time of sacrococcygeal tumor diagnosis, compared with a malignant tumor incidence of 7% for girls and 10% for boys younger than 2 months at the time of diagnosis.[14] The pelvic site of the primary tumor has been reported to be an adverse prognostic factor, perhaps as a result of delayed diagnosis because it was unappreciated at birth or incomplete resection at the time of original surgery.[14-17]

Standard treatment options for mature and immature teratomas (sacrococcygeal sites)

Standard treatment options for mature and immature teratomas in a sacrococcygeal site include the following:

  1. Surgery and observation.

Surgery is an essential component of treatment. Complete resection of the coccyx is vital to minimize the likelihood of tumor recurrence;[2] however, one study reported that 11 of 12 patients with microscopic residual benign immature teratomas had no recurrence.[18] After successful resection, neonates diagnosed with benign mature and immature teratomas are closely observed with follow-up exams and serial serum AFP determinations for several years to ensure that the expected physiological normalization of AFP levels occurs and to facilitate early detection of tumor relapse.[19] A significant rate of recurrence among these benign tumors has been reported by several groups, ranging from 10% to 21%, with most relapses occurring within 3 years of resection.[9,13,19,20]

While there is no standard follow-up schedule, tumor markers are measured frequently for 3 years. Recurrent tumors will be malignant in 43% to 50% of cases, and yolk sac tumor is the most common histology. With early detection, recurrent malignant GCTs can be treated successfully with surgery and chemotherapy (overall survival, 92%).[21] Long-term survivors are monitored for complications of extensive surgery, which include constipation, fecal and urinary incontinence, and psychologically unacceptable cosmetic scars.[22]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood teratoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References
  1. Rescorla FJ: Pediatric germ cell tumors. Semin Surg Oncol 16 (2): 144-58, 1999.  [PUBMED Abstract]

  2. Göbel U, Calaminus G, Engert J, et al.: Teratomas in infancy and childhood. Med Pediatr Oncol 31 (1): 8-15, 1998.  [PUBMED Abstract]

  3. Pinkerton CR: Malignant germ cell tumours in childhood. Eur J Cancer 33 (6): 895-901; discussion 901-2, 1997.  [PUBMED Abstract]

  4. Schneider DT, Calaminus G, Reinhard H, et al.: Primary mediastinal germ cell tumors in children and adolescents: results of the German cooperative protocols MAKEI 83/86, 89, and 96. J Clin Oncol 18 (4): 832-9, 2000.  [PUBMED Abstract]

  5. Bernbeck B, Schneider DT, Bernbeck B, et al.: Germ cell tumors of the head and neck: report from the MAKEI Study Group. Pediatr Blood Cancer 52 (2): 223-6, 2009.  [PUBMED Abstract]

  6. Metcalfe PD, Farivar-Mohseni H, Farhat W, et al.: Pediatric testicular tumors: contemporary incidence and efficacy of testicular preserving surgery. J Urol 170 (6 Pt 1): 2412-5; discussion 2415-6, 2003.  [PUBMED Abstract]

  7. Valdiserri RO, Yunis EJ: Sacrococcygeal teratomas: a review of 68 cases. Cancer 48 (1): 217-21, 1981.  [PUBMED Abstract]

  8. Carter D, Bibro MC, Touloukian RJ: Benign clinical behavior of immature mediastinal teratoma in infancy and childhood: report of two cases and review of the literature. Cancer 49 (2): 398-402, 1982.  [PUBMED Abstract]

  9. Gonzalez-Crussi F, Winkler RF, Mirkin DL: Sacrococcygeal teratomas in infants and children: relationship of histology and prognosis in 40 cases. Arch Pathol Lab Med 102 (8): 420-5, 1978.  [PUBMED Abstract]

  10. Marina NM, Cushing B, Giller R, et al.: Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study. J Clin Oncol 17 (7): 2137-43, 1999.  [PUBMED Abstract]

  11. Norris HJ, Zirkin HJ, Benson WL: Immature (malignant) teratoma of the ovary: a clinical and pathologic study of 58 cases. Cancer 37 (5): 2359-72, 1976.  [PUBMED Abstract]

  12. Mann JR, Gray ES, Thornton C, et al.: Mature and immature extracranial teratomas in children: the UK Children's Cancer Study Group Experience. J Clin Oncol 26 (21): 3590-7, 2008.  [PUBMED Abstract]

  13. Rescorla FJ, Sawin RS, Coran AG, et al.: Long-term outcome for infants and children with sacrococcygeal teratoma: a report from the Childrens Cancer Group. J Pediatr Surg 33 (2): 171-6, 1998.  [PUBMED Abstract]

  14. Altman RP, Randolph JG, Lilly JR: Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 9 (3): 389-98, 1974.  [PUBMED Abstract]

  15. Ablin AR, Krailo MD, Ramsay NK, et al.: Results of treatment of malignant germ cell tumors in 93 children: a report from the Childrens Cancer Study Group. J Clin Oncol 9 (10): 1782-92, 1991.  [PUBMED Abstract]

  16. Marina N, Fontanesi J, Kun L, et al.: Treatment of childhood germ cell tumors. Review of the St. Jude experience from 1979 to 1988. Cancer 70 (10): 2568-75, 1992.  [PUBMED Abstract]

  17. Baranzelli MC, Kramar A, Bouffet E, et al.: Prognostic factors in children with localized malignant nonseminomatous germ cell tumors. J Clin Oncol 17 (4): 1212, 1999.  [PUBMED Abstract]

  18. De Backer A, Madern GC, Hakvoort-Cammel FG, et al.: Study of the factors associated with recurrence in children with sacrococcygeal teratoma. J Pediatr Surg 41 (1): 173-81; discussion 173-81, 2006.  [PUBMED Abstract]

  19. Huddart SN, Mann JR, Robinson K, et al.: Sacrococcygeal teratomas: the UK Children's Cancer Study Group's experience. I. Neonatal. Pediatr Surg Int 19 (1-2): 47-51, 2003.  [PUBMED Abstract]

  20. Gabra HO, Jesudason EC, McDowell HP, et al.: Sacrococcygeal teratoma--a 25-year experience in a UK regional center. J Pediatr Surg 41 (9): 1513-6, 2006.  [PUBMED Abstract]

  21. De Corti F, Sarnacki S, Patte C, et al.: Prognosis of malignant sacrococcygeal germ cell tumours according to their natural history and surgical management. Surg Oncol 21 (2): e31-7, 2012.  [PUBMED Abstract]

  22. Derikx JP, De Backer A, van de Schoot L, et al.: Long-term functional sequelae of sacrococcygeal teratoma: a national study in The Netherlands. J Pediatr Surg 42 (6): 1122-6, 2007.  [PUBMED Abstract]