Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-77c89778f8-5wvtr Total loading time: 0 Render date: 2024-07-16T23:48:50.187Z Has data issue: false hasContentIssue false

62 - Extragonadal germ cell tumors

from Part VI - Oncology

Published online by Cambridge University Press:  08 January 2010

By
Christian J. Streck  and
Andrew M. Davidoff
Edited by
Mark D. Stringer ,
Keith T. Oldham  and
Pierre D. E. Mouriquand
Christian J. Streck
Affiliation:
St. Jude Children's Research Hospital, Memphis, TN, USA
Andrew M. Davidoff
Affiliation:
St. Jude Children's Research Hospital, Memphis, TN, USA
Mark D. Stringer
Affiliation:
University of Otago, New Zealand
Keith T. Oldham
Affiliation:
Children's Hospital of Wisconsin
Pierre D. E. Mouriquand
Affiliation:
Debrousse Hospital, Lyon
Get access

Summary

Germ cell tumors (GCTs) are relatively uncommon in childhood, accounting for about 2% to 3% of all neoplasms in patients younger than 15 years. With an annual incidence of approximately 4 per million in this age group, there are about 250 new cases in the United States each year. Germ cell tumors can arise in the gonads or in extragonadal sites, doing so with nearly equal frequency overall. However, extragonadal and testicular tumors predominate in children younger than 3 years, and gonadal tumors predominate in older children. Approximately 30% to 40% of all GCTs are malignant, though the percentage is somewhat higher when considering only extragonadal tumors. Overall, GCTs are more likely to develop in girls than in boys.

Embryogenesis

Germ cell tumors arise from primordial germ cells derived from the embryonic yolk sac endoderm. These primordial germ cells are first recognized in the caudal portion of the yolk sac, near the allantoic stalk, at about the 24th day (2–3 mm embryos). During the 5th and 6th weeks of embryogenesis, these cells migrate along the mesentery of the hindgut toward the genital ridge, which is forming from mesenchyme and mesodermal epithelium. The path of the migrating cells appears to be directed by the interaction of the c-kit ligand, stem cell factor (expressed with an increasing gradient from the yolk sac to the genital ridges), and the c-kit receptor (expressed on the primordial germ cells).

Type
Chapter
Information
Pediatric Surgery and Urology
Long-Term Outcomes
 , pp. 815 - 825
Publisher: Cambridge University Press
Print publication year: 2006

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Young, J. L. Jr., Ries, L. G., Silverberg, E., Horm, J. W., & Miller, R. W.Cancer incidence, survival, and mortality for children younger than age 15 years. Cancer 1986; 58(2)Suppl.:598–602.3.0.CO;2-C>CrossRefGoogle Scholar
Witschi, E.Migration of the germ cells of human embryos from the yolk sac to the primitive gonadal fold. Contrib. Embryol. 1948; 32(69).Google Scholar
Strohmeyer, T., Reese, D., Press, M., Ackermann, R., Hartmann, M., & Slamon, D.Expression of the c-kit proto-oncogene and its ligand stem cell factor (SCF) in normal and malignant human testicular tissue. J. Urol. 1995; 153(2):511–515.CrossRefGoogle ScholarPubMed
Altman, R. P., Randolph, J. G., & Lilly, J. R.Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J. Pediatr. Surg. 1974; 9(3):389–398.CrossRefGoogle Scholar
Wu, J. T., Book, L., & Sudar, K.Serum alpha fetoprotein (AFP) levels in normal infants. Pediatr. Res. 1981; 15(1):50–52.CrossRefGoogle ScholarPubMed
Flake, A. W., Harrison, M. R., Adzick, N. S., Laberge, J. M., & Warsof, S. L.Fetal sacrococcygeal teratoma. J. Pediatr. Surg. 1986; 21(7):563–566.CrossRefGoogle ScholarPubMed
Rescorla, F. J., Sawin, R. S., Coran, A. G., Dillon, P. W., & Azizkhan, R. G.Long-term outcome for infants and children with sacrococcygeal teratoma: a report from the Childrens Cancer Group. J. Pediatr. Surg. 1998; 33 (2):171–176.CrossRefGoogle ScholarPubMed
Schropp, K. P., Lobe, T. E., Rao, B.et al.Sacrococcygeal teratoma: the experience of four decades. J. Pediatr. Surg. 1992; 27(8):1075–1078.CrossRefGoogle ScholarPubMed
Kamata, S., Imura, K., Kubota, A.et al. Operative management for sacrococcygeal teratoma diagnosed in utero. J. Pediatr. Surg. 2001; 36(4):545–548.CrossRefGoogle ScholarPubMed
Billmire, D., Vinocur, C., Rescorla, F.et al.Malignant mediastinal germ cell tumors: an intergroup study. J. Pediatr. Surg. 2001; 36(1):18–24.CrossRefGoogle ScholarPubMed
Cho, B. K., Wang, K. C., Nam, D. H.et al.Pineal tumors: experience with 48 cases over 10 years. Childs Nerv. Syst. 1998; 14(1–2):53–58.CrossRefGoogle ScholarPubMed
Schild, S. E., Scheithauer, B. W., Haddock, M. G.et al.Histologically confirmed pineal tumors and other germ cell tumors of the brain. Cancer 1996; 78(12):2564–2571.3.0.CO;2-U>CrossRefGoogle Scholar
Ogawa, K.Toita, T., Nakamura, K.et al. Treatment and prognosis of patients with intracranial nongerminomatous malignant germ cell tumors: a multiinstitutional retrospective analysis of 41 patients. Cancer 2003; 98(2):369–376.CrossRefGoogle ScholarPubMed
Billmire, D., Vinocur, C., Rescorla, F.et al.Malignant retroperitoneal and abdominal germ cell tumors: an intergroup study. J. Pediatr. Surg. 2003; 38(3):315–318.CrossRefGoogle ScholarPubMed
Rescorla, F., Billmire, D., Vinocur, C.et al. The effect of neoadjuvant chemotherapy and surgery in children with malignant germ cell tumors of the genital region: a pediatric intergroup trial. J. Pediatr. Surg. 2003; 38(6):910–912.CrossRefGoogle ScholarPubMed
Einhorn, L. H. & Donohue, J.Cis-diamminedichloroplatinum, vinblastine, and bleomycin combination chemotherapy in disseminated testicular cancer. Ann. Intern. Med. 1977; 87(3):293–298.CrossRefGoogle ScholarPubMed
Davidoff, A. M., Hebra, A., Bunin, N., Shochat, S. J., & Schnaufer, L.Endodermal sinus tumor in children. J. Pediatr. Surg. 1996; 31(8):1075–1078.CrossRefGoogle ScholarPubMed
Calaminus, G., Schneider, D. T., Bokkerink, J. P.et al.Prognostic value of tumor size, metastases, extension into bone, and increased tumor marker in children with malignant sacrococcygeal germ cell tumors: a prospective evaluation of 71 patients treated in the German cooperative protocols Maligne Keimzelltumoren (MAKEI) 83/86 and MAKEI 89. J. Clin. Oncol. 2003; 21(5):781–786.CrossRefGoogle ScholarPubMed
Gobel, U., Schneider, D. T., Calaminus, G., Haas, R. J., Schmidt, P., & Harms, D.Germ-cell tumors in childhood and adolescence. GPOH MAKEI and the MAHO study groups. Ann. Oncol. 2000; 11(3):263–271.CrossRefGoogle ScholarPubMed
Gobel, U., Schneider, D. T., Calaminus, G.et al.Multimodal treatment of malignant sacrococcygeal germ cell tumors: a prospective analysis of 66 patients of the German cooperative protocols MAKEI 83/86 and 89. J. Clin. Oncol. 2001; 19(7):1943–1950.CrossRefGoogle ScholarPubMed
Schneider, D. T., Calaminus, G., Koch, S.et al.Epidemiologic analysis of 1,442 children and adolescents registered in the German germ cell tumor protocols. Pediatr. Blood Cancer 2004; 42 (2):169–175.CrossRefGoogle ScholarPubMed
Schneider, D. T., Wessalowski, R., Calaminus, G.et al.Treatment of recurrent malignant sacrococcygeal germ cell tumors: analysis of 22 patients registered in the German protocols MAKEI 83/86, 89, and 96. J. Clin. Oncol. 2001; 19(7):1951–1960.CrossRefGoogle ScholarPubMed
Huddart, S. N., Mann, J. R., Robinson, K.et al.Sacrococcygeal teratomas: the UK Children's Cancer Study Group's experience. I. Neonatal. Pediatr. Surg. Int. 2003; 19(1–2):47–51.Google ScholarPubMed
Mann, J. R., Raafat, F., Robinson, K.et al.UKCCSG's germ cell tumour (GCT) studies: improving outcome for children with malignant extracranial non-gonadal tumours – carboplatin, etoposide, and bleomycin are effective and less toxic than previous regimens. United Kingdom Children's Cancer Study Group. Med. Pediatr. Oncol. 1998; 30(4):217–227.3.0.CO;2-J>CrossRefGoogle ScholarPubMed
Mann, J. R., Raafat, F., Robinson, K.et al.The United Kingdom Children's Cancer Study Group's second germ cell tumor study: carboplatin, etoposide, and bleomycin are effective treatment for children with malignant extracranial germ cell tumors, with acceptable toxicity. J. Clin. Oncol. 2000; 18(22):3809–3818.CrossRefGoogle ScholarPubMed
Baranzelli, M. C., Kramar, A., Bouffet, E.et al.Prognostic factors in children with localized malignant nonseminomatous germ cell tumors. J. Clin. Oncol. 1999; 17(4):1212.CrossRefGoogle ScholarPubMed
Marina, N. M., 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. 1999; 17(7):2137–2143.CrossRefGoogle ScholarPubMed
Rescorla, F., Billmire, D., Stolar, C.et al. The effect of cisplatin dose and surgical resection in children with malignant germ cell tumors at the sacrococcygeal region: a pediatric intergroup trial (POG 9049/CCG 8882). J. Pediatr. Surg. 2001; 36(1):12–17.CrossRefGoogle Scholar
Gross, R. W., Clatworthy, H. W. Jr., & Meeker, I. A. Jr.Sacrococcygeal teratomas in infants and children; a report of 40 cases. Surg. Gynecol. Obstet. 1951; 92(3):341–354.Google ScholarPubMed
Gobel, U., Calaminus, G., Engert, J.et al. Teratomas in infancy and childhood. Med. Pediatr. Oncol. 1998; 31(1):8–15.3.0.CO;2-H>CrossRefGoogle ScholarPubMed
Bilik, R., Shandling, B., Pope, M., Thorner, P., Weitzman, S., & Ein, S. H.Malignant benign neonatal sacrococcygeal teratoma. J. Pediatr. Surg. 1993; 28(9):1158–1160.CrossRefGoogle ScholarPubMed
Hawkins, E., Issacs, H., Cushing, B., & Rogers, P.Occult malignancy in neonatal sacrococcygeal teratomas. A report from a Combined Pediatric Oncology Group and Children's Cancer Group study. Am. J. Pediatr. Hematol. Oncol 1993; 15(4):406–409.Google ScholarPubMed
Wessalowski, R., Kruck, H., Pape, H., Kahn, T., Willers, R., & Gobel, U.Hyperthermia for the treatment of patients with malignant germ cell tumors: a phase I/II study in ten children and adolescents with recurrent or refractory tumors. Cancer 1998; 82(4):793–800.3.0.CO;2-S>CrossRefGoogle ScholarPubMed
Malone, P. S., Spitz, L., Kiely, E. M., Brereton, R. J., Duffy, P. G., & Ransley, P. G.The functional sequelae of sacrococcygeal teratoma. J. Pediatr. Surg. 1990; 25(6):679–680.CrossRefGoogle ScholarPubMed
Havranek, P., Hedlund, H., Rubenson, A.et al. Sacrococcygeal teratoma in Sweden between 1978 and 1989: long-term functional results. J. Pediatr. Surg. 1992; 27(7):916–918.CrossRefGoogle ScholarPubMed
Boemers, T. M., Gool, J. D., Jong, T. P., & Bax, K. M.Lower urinary tract dysfunction in children with benign sacrococcygeal teratoma. J. Urol. 1994; 151(1):174–176.CrossRefGoogle ScholarPubMed
Reinberg, Y., Long, R., Manivel, J. C., Resnick, J., Simonton, S., & Gonzalez, R.Urological aspects of sacrococcygeal teratoma in children. J. Urol. 1993; 150(3):948–949.CrossRefGoogle ScholarPubMed
Rintala, R., Lahdenne, P., Lindahl, H., , M.iimes, & Heikinheimo, M.Anorectal function in adults operated for a benign sacrococcygeal teratoma. J. Pediatr. Surg. 1993; 28(9):1165–1167.CrossRefGoogle ScholarPubMed
Lakhoo, K., Boyle, M., & Drake, D. P.Mediastinal teratomas: review of 15 pediatric cases. J. Pediatr. Surg. 1993; 28(9):1161–1164.CrossRefGoogle ScholarPubMed
Dulmet, E. M., Macchiarini, P., Suc, B., & Verley, J. M.Germ cell tumors of the mediastinum. A 30-year experience. Cancer 1993; 72(6):1894–1901.3.0.CO;2-6>CrossRefGoogle ScholarPubMed
Matsutani, M., Sano, K., Takakura, K.et al.Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J. Neurosurg. 1997; 86(3):446–455.CrossRefGoogle ScholarPubMed

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×