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Surgery in MEN 2A Patients Older Than 5 Years with Micro-MTC: Outcome at Long-term Follow-up
Francesco Tonelli1, Francesco Giudici2, Tommaso Marcucci2
1Department of Surgery and Translational Medicine, Surgical Unit, Medical School, University of Florence, Florence, Italy fmed@libero.it.
Abstract:
In multiple endocrine neoplasia syndrome type 2A (MEN 2A), early total thyroidectomy (TT; performed before the age of 5 years) is the best option to prevent medullary thyroid carcinoma (MTC) development, but the management of MEN 2A patients diagnosed after childhood is still under debate. Seventeen consecutive patients diagnosed with MEN 2A after the age of 5 years (mean age, 23.3 years) with a pathologic diagnosis of micro-MTC without nodal involvement were enrolled. All patients underwent TT with thymectomy and central compartment lymph node dissection. During surgery, parathyroid tissue removal occurred in 14 patients. No major postoperative complications nor persistent hypoparathyroidism was observed. After a mean follow-up of 16.6 years, no patient developed primary hyperparathyroidism or disease recurrence. Even if TT is recommended before the age of 5, when MEN 2A diagnosis is performed after this age in micro-MTC without nodal involvement, TT with thymectomy and central compartment lymphadenectomy can provide good oncologic and functional results.
Insights
For patients diagnosed with Multiple Endocrine Neoplasia syndrome type 2A (MEN 2A) after age five, total thyroidectomy with thymectomy and lymph node dissection offers good outcomes for micro-MTC without nodal involvement.
Area of Science:
- Endocrinology
- Surgical Oncology
- Genetics
Background:
- Multiple Endocrine Neoplasia syndrome type 2A (MEN 2A) management after early childhood is debated.
- Early total thyroidectomy (TT) before age five is standard for preventing medullary thyroid carcinoma (MTC).
Purpose of the Study:
- To evaluate the oncologic and functional outcomes of TT in MEN 2A patients diagnosed after age five with micro-MTC.
Main Methods:
- Seventeen consecutive MEN 2A patients diagnosed after age five underwent TT, thymectomy, and central compartment lymph node dissection.
- Parathyroid tissue was removed in 14 patients; follow-up averaged 16.6 years.
Main Results:
- No major postoperative complications or persistent hypoparathyroidism were observed.
- No patient developed primary hyperparathyroidism or disease recurrence during follow-up.
Conclusions:
- TT with thymectomy and lymphadenectomy is a safe and effective option for MEN 2A patients with micro-MTC diagnosed after age five.
- This approach yields favorable oncologic and functional results, even when diagnosis is delayed.
