Only a small number of children and adolescents with extracranial germ cell tumors (GCTs) have a recurrence.[1,2] However, the approach to the treatment of recurrent disease and its success depend on the initial treatment regimen and on the response of the tumor to treatment.
Treatment Options for Recurrent Malignant GCTs in Children
The age-adjusted incidence of carcinoid tumors worldwide is approximately 2 per 100,000 persons.[1,2] The average age at diagnosis is 61.4 years. Carcinoid tumors represent about 0.5% of all newly diagnosed malignancies.[2,3]
Carcinoid tumors are rare, slow-growing tumors that originate in cells of the diffuse neuroendocrine system. They occur most frequently in tissues derived from the embryonic gut. Foregut tumors, which account for up to 25% of cases, arise...
There are no standard treatment options for recurrent pediatric GCTs. Information about ongoing clinical trials is available from the NCI Web site.
Treatment options for recurrent childhood malignant GCTs include the following:
Surgery followed by chemotherapy.
Chemotherapy followed by surgery and possibly radiation therapy.
High-dose (HD) chemotherapy and hematopoietic stem cell rescue.
Radiation therapy followed by surgery (for brain metastases).
The role for surgery in selected patients who have recurrent GCTs has not been established but should be considered.
Despite overall cure rates greater than 80%, children with extracranial GCTs who have disease recurrence after surgery and three-agent, platinum-based combination chemotherapy (cisplatin, etoposide, and bleomycin [PEB] or carboplatin, etoposide, and bleomycin [JEB]) have an unfavorable prognosis. Reports regarding the treatment and outcome of these children are based on small patient samples.
Reports of salvage treatment strategies used in adult recurrent GCTs include larger numbers of patients, but the differences between children and adults regarding the location of the primary GCT site, pattern of relapse, and the biology of childhood GCTs may limit the applicability of adult salvage approaches to children. Treatments that have been explored in adults include the following:
In adults with recurrent GCTs, several chemotherapy combinations have achieved relatively good disease-free status.[4,5,6,7,8,9] A combination of paclitaxel and gemcitabine has demonstrated activity in adults with testicular GCTs who relapsed after HD chemotherapy and hematopoietic stem cell transplant (HSCT).
Surgery followed by chemotherapy
If a tumor recurs, boys with stage I testicular disease originally treated with surgical resection and observation can usually undergo salvage therapy with further surgical excision and standard PEB or JEB chemotherapy.[11,12]
For stage I ovarian GCT patients originally treated with surgery and observation, several European studies have reported encouraging salvage rates with further surgical excision and chemotherapy.[13,14]