From the Guidelines
Germ cell tumors in pediatric patients are malignant tumors that mainly occur in girls, adolescents, and younger women.
Characteristics of Germ Cell Tumors
- They include dysgerminomas, immature teratomas, embryonal tumors, and endodermal sinus (yolk sac) tumors 1
- The median age at diagnosis is 16 to 20 years 1
- Germ cell tumors are the predominant ovarian tumor in this age group 1
- Gonadal dysgenesis is a risk factor for germ cell tumors 1
Treatment and Prognosis
- Women with malignant germ cell tumors have an excellent prognosis 1
- The 5-year survival rate is more than 85% after appropriate treatment 1
- Fertility-sparing surgery should be considered for those desiring fertility preservation, regardless of stage 1
- Postoperative chemotherapy with bleomycin, etoposide, and platinum is recommended for most malignant ovarian germ cell tumors 1
- Close monitoring and follow-up are essential due to the potential for late effects from treatment 1
From the Research
Characteristics of Germ Cell Tumors in Pediatrics
- Pediatric germ cell tumors represent a diverse group of tumors that present from in utero through adolescence at many nongonadal locations, from the neck to the sacrococcygeal region 2
- Germ cell tumors (GCTs) arising in infants, children, and adolescents present a set of special challenges, making up about 3% of malignancies in children aged 0-18 and nearly 15% of cancers in adolescents 3
- Pediatric germ cell tumors are a diverse group of neoplasms with variable clinical behaviors, depending upon the age and site of presentation 4
Treatment and Survival
- Platinum-based chemotherapy has dramatically improved the event-free and overall survival outcomes of pediatric patients with malignant germ cell tumors over the past two decades 4
- Patients with gonadal germ cell tumors have at least a 95% 5-year survival for early stage disease and at least a 85% 5-year survival for advanced stages 4
- Introduction of cisplatin has improved the outcome of children with malignant germ cell tumors to nearly 90% 5 year survival 5
- Carboplatin can successfully substitute for cisplatin during the treatment of pediatric germ cell tumors without sacrificing response or survival, with limited long-term effects such as nephrotoxicity and ototoxicity 6
Prognosis and Risk Factors
- Prognosis is dependent on tumor stage and location, with extragonadal germ cell tumors carrying a poorer prognosis and mediastinal location having the worst outcomes 4
- Cytogenetic research studies have found chromosomal aberrations specific to some of these tumors that may serve as prognosticators and even direct therapy 4
- Epidemiologic and molecular evidence suggests that GCTs in young children likely represent a distinct biologic group as compared to GCTs of older adolescents and adults, motivating the search for molecular targets for novel therapies 3