Sacrococcygeal Tumors in Children
        Standard treatment options
Nonsacrococcygeal Teratomas in Children
        Standard treatment options
Current Clinical Trials

Sacrococcygeal Tumors in Children

Standard treatment options

The sacrococcygeal region is the primary tumor site for the majority of benign and malignant germ cell tumors 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.[1] Sacrococcygeal tumors present in two clinical patterns related to the child’s age, tumor location, and likelihood of tumor malignancy. Neonatal tumors present at birth protruding from the sacral site and are usually mature or immature teratomas. Among infants and young children, the tumor presents as a palpable mass in the sacropelvic region compressing the bladder or rectum.[2] These pelvic tumors have a greater likelihood of being 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.[3] The pelvic site of the primary tumor has been reported to be an adverse prognostic factor, most likely caused by a higher rate of incomplete resection.[3-6]

After successful resection, neonates diagnosed with benign mature and immature teratomas are observed with close follow-up exams and serial serum alpha-fetoprotein (AFP) determinations for several years to ensure that the expected physiological normalization of AFP levels occurs and to facilitate early detection of tumor relapse.[7] 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.[1,7-9] While there is no standard follow-up schedule, follow-up should include scans and tumor markers for 3 years. Importantly, 43% to 50% of these recurrent tumors will be malignant and require adjuvant chemotherapy. Complete resection of the coccyx is vital to minimize the likelihood of recurrent tumor.[10] However, one study reported that 11/12 patients with microscopic residual benign immature teratoma had no recurrence.[11] Long-term survivors should be followed for complications of extensive surgery, which include, constipation, fecal and urinary incontinence, and psychologically unacceptable cosmetic scars.[12]

Mature and immature teratomas arise primarily in the sacrococcygeal region of neonates and young children, and in the ovaries of pubescent girls. These tumors are also less commonly found in the testicular region of boys younger than 4 years, the mediastinum of adolescents, and other sites.[2,10,13] Mature teratoma and epidermoid cyst in the prepubertal testis are relatively common benign lesions and may be amenable to testis-sparing surgery.[14] Children with mature teratomas, including mature teratomas of the mediastinum, can be treated with surgery and observation with an excellent prognosis.[2,15] In a review of 153 children with nontesticular mature teratoma, the 6-year relapse-free survival for completely resected disease was 96% versus 55% for incomplete disease resection.[10]

In infants and young children, immature teratomas have benign clinical behavior; [8,16,17] however, in adults immature teratomas (primarily ovarian) reportedly have an aggressive clinical behavior [18] requiring surgery and chemotherapy. The benefit of adjuvant chemotherapy for children was questioned in a study by the Pediatric Oncology Group and Children's Cancer Group that evaluated the use of surgical resection followed by careful observation for patients with immature teratomas. Surgery alone was curative for most children and adolescents with resected ovarian immature teratoma of any grade, even when elevated levels of serum AFP or microscopic foci of yolk sac tumor were present. The study demonstrated a 3-year event-free survival of 97.8%, 100%, and 80% for patients with ovarian, testicular, and extragonadal tumors, respectively.[19] It is important to emphasize that the number of pediatric patients with residual teratomas and immature teratomas is very small. There may be a role for surgical removal of residual benign lesions.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry 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, 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]
   
   
2. Rescorla FJ: Pediatric germ cell tumors. Semin Surg Oncol 16 (2): 144-58, 1999.  [PUBMED Abstract]
   
   
3. 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]
   
   
4. 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]
   
   
5. 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]
   
   
6. 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]
   
   
7. 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]
   
   
8. 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]
   
   
9. 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]
   
   
10. Göbel U, Calaminus G, Engert J, et al.: Teratomas in infancy and childhood. Med Pediatr Oncol 31 (1): 8-15, 1998.  [PUBMED Abstract]
    
    
11. 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]
    
    
12. 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]
    
    
13. Pinkerton CR: Malignant germ cell tumours in childhood. Eur J Cancer 33 (6): 895-901; discussion 901-2, 1997.  [PUBMED Abstract]
    
    
14. 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]
    
    
15. 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]
    
    
16. Valdiserri RO, Yunis EJ: Sacrococcygeal teratomas: a review of 68 cases. Cancer 48 (1): 217-21, 1981.  [PUBMED Abstract]
    
    
17. 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]
    
    
18. 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]
    
    
19. 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]
    
    

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