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[Adrenocortical carcinoma in children: retrospective study of 54 cases]
C Teinturier1, L Brugières, J Lemerle
1Service d'endocrinologie pédiatrique, hôpital Saint-Vincent-de-Paul, Paris, France.
Insights
Pediatric adrenal tumors have a poor prognosis, with complete surgical resection being the only effective treatment. Chemotherapy offers limited benefit, and further studies are needed to improve outcomes for these rare childhood cancers.
Area of Science:
- Pediatric oncology
- Endocrinology
- Surgical oncology
Context:
- Adrenal tumors are rare in children.
- Criteria for malignancy and chemotherapy efficacy are poorly defined.
- This study analyzes outcomes for 45 children diagnosed between 1973 and 1993.
Purpose:
- To evaluate the clinical characteristics, treatment outcomes, and prognosis of childhood adrenal tumors.
- To assess the effectiveness of surgical resection and medical treatments, including chemotherapy.
Summary:
- Seventy-six percent of children presented with virilization; 57% had palpable tumors.
- Most patients had localized disease (80%).
- Complete surgical resection was performed in all 45 children, with a 40% recurrence rate.
- Medical treatment (o,p'-DDD or chemotherapy) yielded a tumoral response in one-third of patients.
- The overall 5-year survival rate was 49%.
Impact:
- Childhood adrenocortical neoplasms have a poor prognosis.
- Complete surgical resection is the primary curative treatment.
- Current chemotherapy lacks proven efficacy as an adjuvant therapy.
- Multicentric studies are ongoing to explore effective therapeutic strategies.
Background:
Adrenal tumors rarely occur in childhood. Their criteria for malignancy, as well as the effects of chemotherapy remain poorly defined.
Population:
Fourty-five children (median age: 4 years) with an adrenal tumor diagnosed between 1973 and 1993 were included in this study.
Results:
Seventy-six percent of the children showed various degrees of virilization. Tumor was palpable in 57%. Most patients (80%) had local disease, 7% loco-regional disease and 13% distant metastases. Forty-five children underwent an apparently complete surgical resection. Recurrence occurred 2 to 17 months after surgery in 18 of them (40%). Twenty-four children received medical treatment (o.p'-DDD or chemotherapy) and one-third had a tumoral response. The overall 5 year survival rate was 49%.
Conclusions:
Adrenocortical neoplasms have a poor prognosis in childhood. Complete resection is the only effective and potentially curative treatment. Currently no effective chemotherapy exists, and the value of adjuvant therapy remains unproven. Multicentric studies are underway to evaluate the efficacy of therapeutic approaches.