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    Childhood Extracranial Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Mature and Immature Teratomas in Children

    Mature and immature teratomas arise primarily in the sacrococcygeal region of neonates and young children and in the ovaries of pubescent girls. Less commonly, these tumors are found in the testicular region of boys younger than 4 years, the mediastinum of adolescents, and other sites.[1,2,3] The primary treatment for teratomas is surgery and depends on whether the tumor forms in a nonsacrococcygeal or sacrococcygeal site. Surgical options for sacrococcygeal teratomas are complex. The number of pediatric patients with postoperative residual mature or immature teratomas is very small.

    Mature Teratomas (Nonsacrococcygeal Sites)

    Standard treatment options for mature teratomas (nonsacrococcygeal sites)

    Standard treatment options for mature teratomas in a nonsacrococcygeal site include the following:

    1. Surgery and observation.

    Children with mature teratomas, including mature teratomas of the mediastinum, can be treated with surgery and observation, with an excellent prognosis.[1,4] In a review of 153 children with nontesticular mature teratoma, the 6-year relapse-free survival was 96% for completely resected disease and 55% for incompletely resected disease.[2]

    Head and neck germ cell tumors (GCTs) in neonates should be cared for by a multidisciplinary team. Although most head and neck GCTs are benign, they present significant challenges to surgeons. Some tumors develop malignant elements, which may change the treatment strategy.[5]

    Mature teratomas in the prepubertal testis are relatively common benign lesions and may be amenable to testis-sparing surgery.[6]

    Immature Teratomas (Nonsacrococcygeal Sites)

    Standard treatment options for immature teratomas (nonsacrococcygeal sites)

    Standard treatment options for immature teratomas in a nonsacrococcygeal site include the following:

    1. Surgery and observation (stage I).
    2. Surgery and chemotherapy (stages II through IV).

    The treatment options for immature teratomas at a nonsacrococcygeal site differ by stage of disease.

    Stage I

    Infants and young children with immature teratomas have an excellent prognosis if the tumor can be completely resected.[7,8,9] For these patients, the current standard of treatment is surgery and observation.

    Evidence (surgery and observation for stage I disease):

    1. The benefit of adjuvant chemotherapy for children was investigated in a study by the Pediatric Oncology Group and Children's Cancer Group. Surgical resection followed by careful observation was used to treat patients with immature teratomas.[10]
      • Surgery alone was curative for most children and adolescents with resected ovarian immature teratomas of any grade, even when elevated levels of serum alpha-fetoprotein (AFP) or microscopic foci of yolk sac tumor were present.
      • The study demonstrated a 3-year event-free survival of 97.8% for patients with ovarian tumors, 100% for patients with testicular tumors, and 80% for patients with extragonadal tumors.
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