Related Experiment Videos
Medullary carcinoma in children. Results of early detection and surgery
R L Telander1, D Zimmerman, G W Sizemore
1Department of Pediatrics, Mayo Clinic, Rochester, MN 55905.
Insights
Early screening and surgery for medullary thyroid carcinoma (MCT) in children, especially those with MEN-II syndromes, significantly improves outcomes and detects disease early, preventing metastasis.
Area of Science:
- Pediatric Endocrinology
- Surgical Oncology
- Genetics and Genomics
Background:
- Medullary thyroid carcinoma (MCT) in children necessitates early diagnosis and surgical intervention to prevent metastatic spread.
- Children with Multiple Endocrine Neoplasia (MEN) syndromes, specifically MEN-IIa and MEN-IIb, are at high risk for developing MCT.
- Optimal timing for screening and surgical treatment in pediatric MCT cases remains a critical consideration.
Purpose of the Study:
- To review surgical outcomes for children under 16 diagnosed with medullary thyroid carcinoma (MCT) or C-cell hyperplasia.
- To determine the effectiveness of screening protocols in early detection and management of pediatric MCT.
- To establish optimal screening and surgical timing for children with MEN-II syndromes.
Main Methods:
- Retrospective review of 33 children (6 months to 15.9 years) who underwent total thyroidectomy for MCT or C-cell hyperplasia between 1970 and 1988.
- Analysis of patient data including age at diagnosis, presence of metastasis, MEN syndrome type, and follow-up status.
- Correlation of basal immunoreactive calcitonin levels with disease presentation and stage.
Main Results:
- Screening detected MCT in 27 children, with 25 disease-free post-surgery, indicating high screening efficacy.
- MCT with metastasis was exclusively observed in children over 12 years old.
- Early-onset MCT was noted in a 6-month-old with MEN-IIb and a 3-year-old with MEN-IIa.
Conclusions:
- Screening is highly effective for early detection of MCT in children with MEN-II syndromes.
- Children with MEN-IIb should be screened shortly after birth; those with MEN-IIa should be screened by one year of age.
- Prompt surgical intervention based on screening results is crucial for improving outcomes in pediatric medullary thyroid carcinoma.
Abstract:
Early diagnosis and surgical treatment of medullary carcinoma of the thyroid (MCT) in children is essential to decrease the likelihood of metastatic spread. To determine the optimal timing of screening and surgery, we reviewed all children younger than 16 years old who were undergoing surgical treatment. From 1970 to 1988, 33 children 6 months old to 15.9 years old (mean, 9 years old) with MCT or C-cell hyperplasia or both underwent total thyroidectomy. Follow-up was 4 months to 15 years after surgery (mean, 9 years). Six of 31 patients presented with a neck mass. Basal immunoreactive calcitonin level was increased in 3 of 7 patients with C-cell hyperplasia alone, in 10 of 18 patients with nonmetastatic MCT, and in all 5 patients with metastatic MCT. At the time of surgery, 7 patients had C-cell hyperplasia, 19 patients had MCT without metastasis, and 7 patients had MCT with metastases. All children with metastasis were more than 12 years old. Medullary carcinoma of the thyroid was seen as early as age 6 months in a child with multiple endocrine neoplasia type IIb (MEN-IIb) and age 3 years in a child with MEN-IIa. At follow-up, 25 patients were free of disease and 8 had had recurrent disease. Two of the 8 patients with MEN-IIb had died. All 6 children with a neck mass at presentation had recurrent disease. Of 27 patients diagnosed by screening, 25 had no evident disease and 2 had persistent disease. Thus, in children with MEN-II syndromes, screening studies are highly effective in detecting MCT. All children with MEN-IIb should be studied shortly after birth, and those with MEN-IIa should be studied by 1 year old.