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Childhood Extracranial Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Pediatric GCT Biology

The following paragraphs describe the biologically distinct subtypes of GCTs found in children and adolescents. It should be emphasized that very few pediatric GCT specimens have been analyzed to date. Biologic distinctions between GCTs in children versus adults may not be absolute.[1,2,3]

Testicular GCTs

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  • Children: These GCTs typically present during early childhood. The tumors are commonly composed of pure yolk sac tumor (also known as endodermal sinus tumor), are generally diploid or tetraploid, and usually lack the isochromosome of the short arm of chromosome 12 that characterizes testicular cancer in young adults.[1,4,5,6,7] Deletions of chromosomes 1p, 4q, and 6q and gains of chromosomes 1q, 3, and 20q are reported as recurring chromosomal abnormalities for this group of tumors.[6,7,8]
  • Adolescents and young adults: These tumors typically possess an isochromosome of the short arm of chromosome 12 [9,10,11,12] and are aneuploid.[4,12] Although adolescent testicular germ cell patients may be best treated at pediatric oncology centers, the treatment regimens for adolescents older than 14 years follow regimens used in adults. (Refer to the PDQ summary on Testicular Cancer Treatment for more information.)

Ovarian GCTs

Ovarian GCTs occur primarily in adolescent and young adult females. While the majority of ovarian GCTs are benign mature teratomas, a heterogeneous group of malignant GCTs occur in females, including immature teratomas, dysgerminomas, yolk sac tumors, and mixed GCTs. Patients with pediatric ovarian GCTs have an excellent prognosis. One series of 66 patients followed over 44 years reported recurrence and mortality rates of 4.5% and 3%, respectively.[13] The malignant ovarian GCT commonly shows increased copies of the short arm of chromosome 12.[14] (Refer to the PDQ summary on Ovarian Germ Cell Tumors Treatment for more information.)

Extragonadal Extracranial GCTs

  • Children: These tumors typically present at birth or during early childhood. The majority of these tumors are benign teratomas occurring in the sacrococcygeal region, and hence SEER data do not include them.[15,16] Malignant yolk sac tumor histology occurs in a minority of these tumors, however, with cytogenetic abnormalities similar to those observed for tumors occurring in the testes of young males.[5,6,7,8]
  • Older children, adolescents, and young adults: The mediastinum is the most common primary site for extragonadal GCTs in older children and adolescents.[17] Mediastinal GCTs in children younger than 8 years share the same genetic gains and losses as sacrococcygeal and testicular tumors in young children.[18,19,20] The gain in chromosome 12p has been reported in mediastinal tumors in children aged 8 years and older.[20,21]
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