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Management and results of thyroidectomies in pediatric patients with MEN 2 syndrome
- Source :
- Journal of pediatric surgery. 56(11)
- Publication Year :
- 2020
-
Abstract
- Aim of the study To evaluate the outcome of prophylactic thyroidectomies (PT) in patients with MEN 2 syndrome in a tertiary center. Methods A retrospective study was designed, including all patients with MEN 2 syndrome who underwent PT between 2000 and 2019. Demographics, gene mutation, postoperative complications and histopathological findings were registered. Main results 30 patients were included (29 MEN 2A and 1 MEN 2B) with a median age at surgery time of 7.0 ± 3.2 years. Familiar history was present in all but 3 patients. A therapeutic thyroidectomy was performed in 2 patients due to evidence of medullary thyroid carcinoma (MTC, both were late diagnosis), and in the other 28 cases, a PT was performed. 8 patients had a RET mutation ranked as Moderate Risk (American Thyroid Association): median age at surgery was 7.2 ± 4.2 years, and histological findings were C-cell hyperplasia (n = 6) and no alterations (n = 2). 16 patients had a high risk mutation; median age at surgery time was 6.9 ± 2.8 years and histological findings were normal thyroid gland (n = 1), C Cell Hyperplasia (n = 8), microcarcinoma (n = 6), and MTC (n = 1). The mean hospital stay was 1.4 ± 0.68 days. No intraoperative complications or recurrent laryngeal nerve injuries were registered. 7 patients presented a transient hypoparathyroidism and 1 patient had permanent hypoparathyroidism. Conclusions Early PT in patients with MEN 2 syndrome is a safe procedure when performed by an experienced team of Pediatric Surgeons and with a multidisciplinary approach. Early genetic analysis and familial counselling is essential to prevent the development of a MTC.
- Subjects :
- medicine.medical_specialty
Pediatrics
medicine.medical_treatment
Multiple Endocrine Neoplasia Type 2a
Gene mutation
Thyroid carcinoma
03 medical and health sciences
0302 clinical medicine
030225 pediatrics
Pediatric surgery
medicine
Humans
Thyroid Neoplasms
Child
Retrospective Studies
business.industry
Thyroid
Thyroidectomy
Retrospective cohort study
General Medicine
medicine.disease
medicine.anatomical_structure
C-Cell Hyperplasia
Hypoparathyroidism
030220 oncology & carcinogenesis
Carcinoma, Medullary
Pediatrics, Perinatology and Child Health
Surgery
business
Subjects
Details
- ISSN :
- 15315037
- Volume :
- 56
- Issue :
- 11
- Database :
- OpenAIRE
- Journal :
- Journal of pediatric surgery
- Accession number :
- edsair.doi.dedup.....e81d54ec030a3647549ddcb8a39ad317