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Medullary thyroid cancer (MTC), also known as medullary thyroid carcinoma, is a type of thyroid cancer.
The thyroid gland is located just below the throat in the front of the neck. The thyroid makes hormones that control body functions. MTC starts in parafollicular C cells of the thyroid gland. These cells make a hormone called calcitonin.
Medullary thyroid cancer is rare in children. Most often, childhood MTC is linked to an inherited change in the RET gene. This causes a condition called familial MTC or multiple endocrine neoplasia type 2 (MEN2 syndromes).
Surgery to remove the thyroid gland is the main treatment for medullary thyroid cancer. For children with a genetic predisposition, doctors may remove the thyroid before the cancer develops.
Patients need ongoing care from a team of specialists to manage hormones and watch for the return of cancer.
Signs and symptoms of MTC include:
Your child may not have any symptoms of MTC. The doctor may find it during your child's regular physical exam.
MTC is rare in children, but certain gene changes can increase risk. Parents may sometimes pass down gene changes to their children.
A genetic disorder called hereditary or familial MTC can run in families. In this disorder, there is a change (mutation) in the RET gene so it does not work properly. A child may inherit this mutation from either parent. Or your child may be the first person in your family with this gene change.
RET mutations can occur in a condition known as multiple endocrine neoplasia type 2 (MEN2). MEN2 increases risk of tumors and hormone problems including medullary thyroid cancer, pheochromocytoma, and hyperparathyroidism.
If your child has a RET mutation, your family should get genetic counseling and genetic testing.
Doctors use several types of tests to diagnose medullary thyroid cancer. Tests may include:
The stage of cancer depends on the tumor size and if the cancer has spread to lymph nodes or other parts of the body.
|Stage||Extent of disease|
|Stage 1||Tumor is 2 centimeters (cm) or less across. There is no spread of disease.|
|Stage 2||Tumor is larger than 2 cm across. It is only in the thyroid. There is no spread to lymph nodes or other parts of the body.|
|Stage 3||The tumor may have grown slightly outside the thryoid gland, and the disease has spread to nearby lymph nodes in the neck.|
|Stage 4||The disease is morderate or advanced. The tumor has spread outside of the thyroid into other tissue. The tumor has spread to nearby lymph nodes or distant body parts.|
It is important that a team of specialists treat children with MTC. The treatment depends on gene mutations and other health conditions. The care team will focus on:
All patients with MTC need ongoing care and long-term follow-up.
MTC is a rare cancer, and many factors affect long-term outcomes. These factors include:
After treatment, patients need lifelong monitoring and follow-up care from a team of specialists. This is important because:
Your child's long-term monitoring may include:
The type and frequency of tests and medical visits depend on your child's needs, thyroid cancer type, and the stage. Specialists will monitor your child and watch for signs of other cancers related to any known genetic mutations.
Care team members from psychology, child life, social work, and other areas can help your child cope with their illness. Genetic counselors can help you understand inherited cancers and available support services.
Concerns that patients and families may have include:
To stay well, all cancer survivors should take steps to live a healthy lifestyle. Get regular physical checkups and screenings by a health care provider who understands cancer survivorship issues.
Your care team may prepare a survivorship care plan. This plan is a complete record of your child's treatment, medical concerns, and health screenings they need.
Reviewed: December 2022