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AI InsightPerformance of an adequate surgical procedure during the initial intervention and detection of patients with MTC at earlier ages by screening improve the survival rates.
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Özet
Medullary thyroid carcinoma (MTC) may present in various clinical forms, either sporadic or a counterpart of hereditary endocrine disorders. The appropriate surgical procedure for MTC is total thyroidectomy combined with central neck clearance. The aim of this study was to document the initial clinical presentation, surgical treatment and clinical course of MTC in 27 patients. Thirteen (48.1%) cases were diagnosed as hereditary, either MEN 2a(7/27, 25.9%) or 2b syndrome (1/27, 3.7%) or familial non-MEN MTC (5/27, 18.5%), and fourteen cases were diagnosed as sporadic. Eighteen primary thyroid operations and twelve reoperative surgical procedures were performed on 24 patients. Eleven of the primary thyroid operations were total thyroidectomy combined with central neck clearance and bilateral modified radical neck dissection. Approximately half of the patients (45.5%) who underwent primary and total thyroidectomy had regional lymph node metastasis. There was no recurrent nerve palsy or hypoparathyroidisim. One patient died of acute respiratory insufficiency. External radiation therapy was performed in 9 of 24 patients Postoperative follow up periods ranged from 3 to 97 months, the mean being 38 months. Local recurrence occurred in three (13.6%) patients, two of whom developed distants metastasis as well. The overall five-year survival rate was found to be 85%. MTC is often diagnosed in advanced stages, especially in sporadic cases; however, total thyroidectomy combined with central neck clearance and modified radical neck dissection followed by external radiation provide long-term survival. Performance of an adequate surgical procedure during the initial intervention and detection of patients with MTC at earlier ages by screening improve the survival rates.
Abstract
Medullary thyroid carcinoma (MTC) may present in various clinical forms, either sporadic or a counterpart of hereditary endocrine disorders. The appropriate surgical procedure for MTC is total thyroidectomy combined with central neck clearance. The aim of this study was to document the initial clinical presentation, surgical treatment and clinical course of MTC in 27 patients. Thirteen (48.1%) cases were diagnosed as hereditary, either MEN 2a(7/27, 25.9%) or 2b syndrome (1/27, 3.7%) or familial non-MEN MTC (5/27, 18.5%), and fourteen cases were diagnosed as sporadic. Eighteen primary thyroid operations and twelve reoperative surgical procedures were performed on 24 patients. Eleven of the primary thyroid operations were total thyroidectomy combined with central neck clearance and bilateral modified radical neck dissection. Approximately half of the patients (45.5%) who underwent primary and total thyroidectomy had regional lymph node metastasis. There was no recurrent nerve palsy or hypoparathyroidisim. One patient died of acute respiratory insufficiency. External radiation therapy was performed in 9 of 24 patients Postoperative follow up periods ranged from 3 to 97 months, the mean being 38 months. Local recurrence occurred in three (13.6%) patients, two of whom developed distants metastasis as well. The overall five-year survival rate was found to be 85%. MTC is often diagnosed in advanced stages, especially in sporadic cases; however, total thyroidectomy combined with central neck clearance and modified radical neck dissection followed by external radiation provide long-term survival. Performance of an adequate surgical procedure during the initial intervention and detection of patients with MTC at earlier ages by screening improve the survival rates.