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    Childhood Extracranial Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information About Childhood Extracranial Germ Cell Tumors (GCTs)

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    GCTs arise from primordial germ cells, which migrate during embryogenesis from the yolk sac through the mesentery to the gonads.[3,4] Childhood extracranial GCTs can be divided into the following two types:

    • Gonadal.
    • Extragonadal.

    Most childhood extragonadal GCTs arise in midline sites (i.e., sacrococcygeal, mediastinal, and retroperitoneal); the midline location may represent aberrant embryonic migration of the primordial germ cells.

    Childhood extracranial GCTs are broadly classified as the following:

    • Mature teratomas.
    • Immature teratomas.
    • Malignant GCTs.

    GCTs comprise a variety of histologic diagnoses and can also be divided into the following histologic types:

    • Germinoma.
      • Germinoma.
      • Dysgerminoma (ovary).
      • Seminoma (testis).
    • Nongerminoma.
      • Teratomas (mature and immature).
      • Yolk sac tumor (endodermal sinus tumor).
      • Choriocarcinoma.
      • Embryonal carcinoma.
      • Gonadoblastoma.
      • Mixed GCT (contains more than one of the histologies above).

    (Refer to the PDQ summary on Childhood Central Nervous System Germ Cell Tumors Treatment for information about the treatment of intracranial germ cell tumors.)

    Incidence

    Childhood GCTs are rare in children younger than 15 years, accounting for approximately 3% of cancers in this age group.[5,6,7] In the fetal/neonatal age group, most extracranial GCTs are benign teratomas occurring at midline locations, including sacrococcygeal, retroperitoneal, mediastinal, and cervical regions. Despite the small percentage of malignant teratomas that occur in this age group, perinatal tumors have a high morbidity rate caused by hydrops fetalis and premature delivery.[8,9]

    Extracranial GCTs (particularly testicular GCTs) are much more common among adolescents aged 15 to 19 years, representing approximately 14% of cancers in this age group.

    The incidence of extracranial GCTs by 5-year age group and gender is shown in Table 1.

    Table 1. Incidence of Extracranial Germ Cell Tumors by Age Group and Gender (per 106 population)a

    0-4 years 5-9 years 10-14 years 15-19 years
    a Rates are per 1 million children from 1986 to 1995 for the nine Surveillance, Epidemiology, and End Results regions plus Los Angeles.
    Males 7 0.3 1.4 31
    Females 5.8 2.4 7.8 25.3

    Histologic Classification of Childhood Extracranial GCTs

    Childhood extracranial GCTs comprise a variety of histologic diagnoses and can be broadly classified as the following:

    • Mature teratomas.
    • Immature teratomas.
    • Malignant GCTs.
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