Prophylactic thyroidectomy in multiple endocrine neoplasia type 2A in children: a single centre experience
| Autor: | Pilar Guillén Redondo, Rocío Espinosa Góngora, Clara Rico Espiñeira, Ana Luis Huertas, Juan Carlos Ollero Fresno, Henar Souto Romero, Jose Lorenzo Alonso Calderón, Francisco José Bautista, Manuel Espinoza Vega, Cristina Garcés Visier |
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| Rok vydání: | 2019 |
| Předmět: |
Parathyroidectomy
Male medicine.medical_specialty Medullary cavity Endocrinology Diabetes and Metabolism medicine.medical_treatment 030209 endocrinology & metabolism Multiple Endocrine Neoplasia Type 2a Pheochromocytoma 03 medical and health sciences 0302 clinical medicine Endocrinology Medicine Humans Child Retrospective Studies Hyperparathyroidism business.industry Thyroid Thyroidectomy Retrospective cohort study medicine.disease Prognosis Surgery medicine.anatomical_structure Calcitonin 030220 oncology & carcinogenesis Child Preschool Pediatrics Perinatology and Child Health Female business Follow-Up Studies |
| Zdroj: | Journal of pediatric endocrinologymetabolism : JPEM. 32(8) |
| ISSN: | 2191-0251 |
| Popis: | Background To describe the complications and long-term results in patients with multiple endocrine neoplasia type 2A (MEN 2A) syndrome in whom a prophylactic thyroidectomy was performed, in relation to the recommendations of the American Thyroid Association (ATA). Methods A retrospective study of 14 patients with MEN 2A thyroidectomized between 2000 and 2017. We reviewed demographic, clinical, analytical and radiological data. Postoperative complications and long-term follow-up were analyzed. Results We treated eight boys and six girls with a median age of 5 years old (range 2–10). The predominant genetic mutation belonged to codon 634 (8/14, 57.14%). Total thyroidectomy (TT) without cervical lymphadenectomy was performed in all patients. A right upper parathyroidectomy was performed in one patient due to intraoperative suspicion of increased volume. Histological study revealed no alterations. Two patients presented transient hypocalcemia postoperatively and no patient had permanent hypocalcemia or nerve damage. Pathological anatomy confirmed medullary thyroid microcarcinoma in 5/14 patients: all carrying codon 634 mutation and three of them with preoperative basal calcitonin levels Conclusions Prophylactic thyroidectomy without cervical lymphadenectomy is an effective and safe preventive treatment in patients with MEN 2A syndrome when it is performed by experienced surgeons in reference centers. |
| Databáze: | OpenAIRE |
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