Clin Pediatr Hematol Oncol.  2015 Apr;22(1):30-37. 10.15264/cpho.2015.22.1.30.

Overview of Pediatric Extracranial Germ Cell Tumors

Affiliations
  • 1Department of Pediatrics, College of Medicine, Chungnam National University, Daejeon, Korea. pedonco@cnuh.co.kr

Abstract

Germ cell tumors the designation given to neoplasm arising from the cells of the germline, the cells that are destined to become either the egg or the sperm. These tumors have a number of unique features that includes bimodal and wide age distribution, remarkable phenotypic diversity, and varying biologic behavior. During infancy, sacrococcygeal locations predominate with either teratomas in neonates or endodermal sinus tumors in infants above three months. After puberty, non-germinomatous germ cell tumors predominate with gonadal, mediastinal or intracranial tumor. Specific subtypes of germ cell tumors secrete proteins as tumor markers. Surgical resection of the tumor is necessary to establish the diagnosis and for staging of the extent of tumor spread. Except for teratoma, germ cell tumors are highly sensitive to chemotherapy in particular cisplatin. The most commonly used chemotherapy regimen for malignant germ cell tumors is PEB (cisplatin, etoposide and bleomycin). Prognosis is good even in metastatic diseases. Patients with relapsed or recurrent disease may be candidates for high dose chemotherapy and autologous hematopoietic stem cell transplantation.

Keyword

Pediatrics; Germinoma; Teratoma; Endodermal sinus tumor; Choriocarcinoma

MeSH Terms

Adolescent
Age Distribution
Choriocarcinoma
Cisplatin
Diagnosis
Drug Therapy
Endodermal Sinus Tumor
Etoposide
Female
Germinoma
Gonads
Hematopoietic Stem Cell Transplantation
Humans
Infant
Infant, Newborn
Neoplasms, Germ Cell and Embryonal*
Ovum
Pediatrics
Pregnancy
Prognosis
Puberty
Spermatozoa
Teratoma
Biomarkers, Tumor
Cisplatin
Etoposide
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