This type of thyroid cancer arises from the parafollicular cells or C cells of the thyroid gland. Calcitonin is produced by medullary thyroid carcinoma, and a high calcitonin level is a crucial characteristic of this malignancy. It is derived from the neural crest-originating medullary C cells.
Recent developments in molecular aetiology and genetic testing have resulted in patient risk stratification and the identification of molecular therapeutic targets.
Patients having mutations that place them at a high risk should undergo preventative thyroidectomy. Multiple tyrosine kinase inhibitors are licensed for the treatment of progressive, metastatic medullary thyroid carcinoma.
Epidemiology
Between 4%-10% of all thyroid malignancies in the US are medullary carcinomas. Generally, the incidence of sporadic medullary thyroid carcinoma peaks when individuals are in their 40s or 50s, whereas when associated with MEN2B or MEN2A, the highest incidence is in the 2nd or 3rd decade.
Anatomy
Pathophysiology
In contrast to the typically unilateral medullary thyroid cancer related to sporadic MEN syndromes, the medullary thyroid cancer linked to various MEN syndromes is typically multicentric and bilateral.
Because C cells exist largely in the higher regions of the thyroid gland, these tumours typically affect the upper regions of both lobes. In addition to producing hormones including corticotropin, serotonin, prostaglandins, and melanin, paraneoplastic disorders such as Cushing syndrome and carcinoid syndrome can manifest as medullary thyroid cancer.
Etiology
Most medullary thyroid cancers are idiopathic, and only around 20%-25% are due to inherited disorders such as familial medullary thyroid cancer, MEN 2A and MEN 2B. RET mutations in thyroid neural crest tissue can result in the development of medullary thyroid cancer.
MEN2 and FMTC medullary thyroid tumours are linked to alterations in the germline. Approximately 40%-50% of medullary thyroid tumours of the sporadic origin have RET mutations.
Genetics
Prognostic Factors
The prognosis of medullary thyroid cancer is dependant on three main factors:
Age
Grade of tumor
Status of surgical resection
5-year survival rates for the first 3 grades of the tumor is extremely favourable at 93%, but stage 4 tumors only have a survival rate of 28%.
This type of thyroid cancer arises from the parafollicular cells or C cells of the thyroid gland. Calcitonin is produced by medullary thyroid carcinoma, and a high calcitonin level is a crucial characteristic of this malignancy. It is derived from the neural crest-originating medullary C cells.
Recent developments in molecular aetiology and genetic testing have resulted in patient risk stratification and the identification of molecular therapeutic targets.
Patients having mutations that place them at a high risk should undergo preventative thyroidectomy. Multiple tyrosine kinase inhibitors are licensed for the treatment of progressive, metastatic medullary thyroid carcinoma.
Between 4%-10% of all thyroid malignancies in the US are medullary carcinomas. Generally, the incidence of sporadic medullary thyroid carcinoma peaks when individuals are in their 40s or 50s, whereas when associated with MEN2B or MEN2A, the highest incidence is in the 2nd or 3rd decade.
In contrast to the typically unilateral medullary thyroid cancer related to sporadic MEN syndromes, the medullary thyroid cancer linked to various MEN syndromes is typically multicentric and bilateral.
Because C cells exist largely in the higher regions of the thyroid gland, these tumours typically affect the upper regions of both lobes. In addition to producing hormones including corticotropin, serotonin, prostaglandins, and melanin, paraneoplastic disorders such as Cushing syndrome and carcinoid syndrome can manifest as medullary thyroid cancer.
Most medullary thyroid cancers are idiopathic, and only around 20%-25% are due to inherited disorders such as familial medullary thyroid cancer, MEN 2A and MEN 2B. RET mutations in thyroid neural crest tissue can result in the development of medullary thyroid cancer.
MEN2 and FMTC medullary thyroid tumours are linked to alterations in the germline. Approximately 40%-50% of medullary thyroid tumours of the sporadic origin have RET mutations.
The prognosis of medullary thyroid cancer is dependant on three main factors:
Age
Grade of tumor
Status of surgical resection
5-year survival rates for the first 3 grades of the tumor is extremely favourable at 93%, but stage 4 tumors only have a survival rate of 28%.
Continue until the illness progresses or intolerable toxicity occurs
https://www.ncbi.nlm.nih.gov/books/NBK459354/
medtigo
Medullary thyroid carcinoma
Updated :
August 30, 2023
This type of thyroid cancer arises from the parafollicular cells or C cells of the thyroid gland. Calcitonin is produced by medullary thyroid carcinoma, and a high calcitonin level is a crucial characteristic of this malignancy. It is derived from the neural crest-originating medullary C cells.
Recent developments in molecular aetiology and genetic testing have resulted in patient risk stratification and the identification of molecular therapeutic targets.
Patients having mutations that place them at a high risk should undergo preventative thyroidectomy. Multiple tyrosine kinase inhibitors are licensed for the treatment of progressive, metastatic medullary thyroid carcinoma.
Between 4%-10% of all thyroid malignancies in the US are medullary carcinomas. Generally, the incidence of sporadic medullary thyroid carcinoma peaks when individuals are in their 40s or 50s, whereas when associated with MEN2B or MEN2A, the highest incidence is in the 2nd or 3rd decade.
In contrast to the typically unilateral medullary thyroid cancer related to sporadic MEN syndromes, the medullary thyroid cancer linked to various MEN syndromes is typically multicentric and bilateral.
Because C cells exist largely in the higher regions of the thyroid gland, these tumours typically affect the upper regions of both lobes. In addition to producing hormones including corticotropin, serotonin, prostaglandins, and melanin, paraneoplastic disorders such as Cushing syndrome and carcinoid syndrome can manifest as medullary thyroid cancer.
Most medullary thyroid cancers are idiopathic, and only around 20%-25% are due to inherited disorders such as familial medullary thyroid cancer, MEN 2A and MEN 2B. RET mutations in thyroid neural crest tissue can result in the development of medullary thyroid cancer.
MEN2 and FMTC medullary thyroid tumours are linked to alterations in the germline. Approximately 40%-50% of medullary thyroid tumours of the sporadic origin have RET mutations.
The prognosis of medullary thyroid cancer is dependant on three main factors:
Age
Grade of tumor
Status of surgical resection
5-year survival rates for the first 3 grades of the tumor is extremely favourable at 93%, but stage 4 tumors only have a survival rate of 28%.
https://www.ncbi.nlm.nih.gov/books/NBK459354/
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