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[Prophylactic thyroidectomy in multiple endocrine neoplasia syndrome]
1Hospital Materno-Infantil La Fe. Valencia. aliena1981@hotmail.com
Early prophylactic thyroidectomy is recommended for children with Multiple Endocrine Neoplasia type 2a (MEN 2a) between ages 3-4. This intervention is crucial due to the risk of medullary thyroid microcarcinoma, even with normal diagnostic tests.
Area of Science:
- Endocrinology
- Genetics
- Pediatric Surgery
Background:
- Multiple Endocrine Neoplasia type 2a (MEN 2a) is a genetic disorder characterized by medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism.
- The identification of the RET proto-oncogene has significantly improved the understanding and prognosis of MEN 2a.
Purpose of the Study:
- To retrospectively analyze patients diagnosed with MEN 2a over seven years.
- To determine the optimal age for surgical intervention in pediatric patients with MEN 2a.
Main Methods:
- Retrospective study of ten pediatric patients diagnosed with MEN 2a (ages 1.5-11 years).
- Pre-operative assessments included ultrasound, calcitonin, catecholamines, and urinary metanephrine levels.
- Surgical intervention involved total thyroidectomy, with selective lymph node resection in some cases.
Main Results:
- Mean age at operation was 6.4 years.
- Medullary thyroid microcarcinoma (MTMC) was found in 3 out of 10 patients, with elevated preoperative calcitonin in one.
- No post-operative complications were observed, with discharge on days 2-4.
- Nine out of ten patients maintained normal levels of key biomarkers post-surgery.
Conclusions:
- Prophylactic thyroidectomy is strongly recommended at early ages (3-4 years) for children with MEN 2a.
- This recommendation is based on the occurrence of MTMC in young children and the limitations of diagnostic tests.
- Early surgical intervention can prevent disease progression and improve patient outcomes.
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