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Childhood Extracranial Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Histologic Classification of Childhood Extracranial GCTs

Childhood extracranial germ cell tumors (GCTs) comprise a variety of histologic diagnoses and can be broadly classified into mature or immature teratomas and malignant GCTs.

Mature Teratomas

Mature teratomas usually occur in the ovary or at extragonadal locations and are the most common histological subtype of childhood GCT.[1,2,3] These teratomas usually contain well-differentiated tissues from the ectodermal, mesodermal, and endodermal germ cell layers, and any tissue type may be found within the tumor. Mature teratomas are benign, though some mature and immature teratomas may secrete enzymes or hormones, including insulin, growth hormone, androgens, prolactin, and vasopressin.[4,5,6]

Immature Teratomas

Immature teratomas contain tissues from all three germ cell layers, but immature tissues, primarily neuroepithelial, are also present. Immature teratomas are graded from 0 to 3 based on the amount of immature neural tissue found in the tumor specimen.[7] Tumors of higher grade are more likely to have foci of yolk sac tumor.[8] Immature teratomas occur primarily in young children at extragonadal sites and in the ovaries of girls near the age of puberty, but there is no correlation between tumor grade and patient age.[8,9]

Malignant GCTs

GCTs contain frankly malignant tissues of germ cell origin, and rarely, tissues of somatic origin. Isolated malignant elements may constitute a small fraction of a predominantly mature or immature teratoma.[9,10] Malignant germ cell elements of children, adolescents, and young adults can broadly be classified by location (see Tables 2 and 3).

Table 2. Histology of Malignant Germ Cell Tumors in Young Childrena

Malignant Germ Cell ElementsLocation
E = extragonadal; O = ovarian; T = testicular.
a Modified from Perlman et al.[11]
Yolk sac tumorE, O, T
Dysgerminoma (rare in young children)O

Table 3. Histology of Malignant Germ Cell Tumors in Adolescents and Young Adultsa

Malignant Germ Cell ElementsLocation
E = extragonadal; O = ovarian; T = testicular.
a Modified from Perlman et al.[11]
Yolk sac tumorE, O, T
ChoriocarcinomaE, O, T
Embryonal carcinomaT
Mixed germ cell tumorsE, O

Yolk sac tumors produce alpha-fetoprotein (AFP), while germinomas (seminomas and dysgerminomas), and especially choriocarcinomas, produce beta-human chorionic gonadotropin, resulting in elevated serum levels of these substances. Most children with malignant GCTs will have a component of yolk sac tumor and have elevations of AFP,[12,13] which is serially monitored during treatment to help assess response to therapy.[9,10,12]

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