Medullary thyroid cancer (MTC): MTC originates in the C cells and is occasionally caused by a genetic condition known as multiple endocrine neoplasia type 2. (MEN2). This tumor bears little, if any, resemblance to normal thyroid tissue. MTC is frequently treatable if it is detected and treated early, before it spreads to other parts of the body. MTC accounts for around 3% of all thyroid malignancies. Familial MTC accounts for approximately 25% of all MTC. This suggests that the patient’s family members may receive a similar diagnosis. The RET proto-oncogene test can determine whether or not other family members have familial MTC (FMTC).
Anaplastic thyroid cancer: This kind of thyroid cancer is uncommon, accounting for only around 1% of all cases. It is a rapidly growing, poorly differentiated thyroid cancer that can develop from either a differentiated or benign thyroid tumor. Anaplastic thyroid carcinoma can be classified into giant cell subtypes. This type of thyroid cancer is more difficult to cure because it grows so quickly.
SYMPTOMS AND SIGNS OF THYROID CANCER
People with thyroid cancer may have few or no symptoms or indicators. A symptom, such as weariness, nausea, or discomfort, is something that only the person experiencing it can identify and explain. A symptom is something that others can recognize and quantify, such as a fever, rash, or an increased pulse. Signs and symptoms, when combined, can assist describe a medical situation. Thyroid cancer is frequently detected during a basic physical exam by examining the neck. They are also discovered accidently by x-rays or other imaging examinations performed for other causes. Thyroid cancer patients may have the following symptoms or indicators. Thyroid cancer patients may not exhibit any of the signs and symptoms listed below. Alternatively, the origin of a symptom or sign could be a medical disease other than cancer.
- A bump near the Adam’s apple in the front of the neck.
- Neck glands that are swollen.
- Throat or neck discomfort
- A persistent cough that is not caused by a cold.
- Other types of cancer may also begin in or around the thyroid gland.
Please with your doctor if you are concerned about any changes you are experiencing. In addition to other questions, your doctor will inquire as to how long and how frequently you have been experiencing the symptom(s). This is done to assist in determining the cause of the condition, which is referred to as a diagnosis.
Thyroid cancer, other thyroid disorders, such as a goiter, or an illness unrelated to the thyroid, such as an infection, can all cause these symptoms.
If cancer is discovered, symptom relief is an important element of cancer care and treatment. This is known as palliative care or supportive care. It is frequently initiated shortly after diagnosis and continues throughout treatment. Make an appointment with your health care provider to discuss your symptoms, especially any new or changing symptoms.
STAGES OF THYROID CANCER
Staging describes where the cancer is present, whether or not it has spread, and whether or not it is impacting other sections of the body.
Doctors utilize diagnostic tests to determine the stage of cancer, therefore staging may not be complete until all of the tests are completed. Knowing the stage allows the doctor to propose the best course of treatment and can help predict a patient’s prognosis. Distinct forms of cancer have different stage descriptions.
TNM staging system
The TNM system is one technique that clinicians use to describe the stage. Doctors use diagnostic test and scan results to address the following questions:
Tumor(T): What is the size of the main tumor? Where can I find it?
Node(N): Has the cancer spread to your lymph nodes? If so, where are they and how many?
Metastasis(M): Is the cancer in other parts of the body? If so, where and how much?
The results are aggregated to establish each person’s cancer stage. Thyroid cancer is classified into five stages: stage 0 (zero), stage I, stage II, and stage IV (1 through 4). The stage provides a common language for doctors to describe the cancer so that they can collaborate to determine the best treatments.
Papillary and follicular thyroid cancers are also staged based on the patient’s age, in addition to the TNM method.
Clinical and pathological staging are both possible. Clinical staging is determined by the results of pre-surgery diagnostics, which may include physical examinations and imaging tests. Pathological staging is determined by what is discovered during surgery, which may include a sample. Pathological staging, in general, provides the most information for determining a patient’s prognosis.
More information on each component of the TNM system for thyroid cancer can be found below:
Tumor (T)
The letter “T” plus a letter or number (0 to 4) is used in the TNM system to describe the size and location of the tumor. The size of a tumor is measured in millimeters (cm). A centimeter is approximately the width of a normal pen or pencil.
Stages can also be subdivided into smaller groups to assist describe the tumor in greater detail. Doctors may differentiate the broad categories of “T” in thyroid cancer by adding the letter “s” to indicate a solitary (single) tumor or “m” to signify multifocal (more than one) tumors. The information for each tumor stage is shown below.
TX: The primary cancer cannot be evaluated.
T0: There is no indication of a tumor.
T1: The tumor is 2 centimeters (cm) or less in size and only affects the thyroid.
- T1a: The tumor is 1 cm or less in size.
- T1b: The tumor is more than 1 cm in diameter but less than 2 cm in diameter.
T2: The tumor is more than 2 cm but less than 4 cm, and it is restricted to the thyroid.
T3: The tumor is larger than 4 cm, yet it does not grow past the thyroid gland.
T4: The tumor might be of any size and has spread beyond the thyroid.
- T4a: Beyond the thyroid, the tumor has progressed to neighboring soft tissues, such as the larynx, trachea, esophagus, or recurrent laryngeal nerve.
- T4b: The tumor has progressed beyond the T4a regions.
Node (N)
The letter “N” in the TNM staging system denotes lymph nodes. Lymph node assessment is a crucial part in staging thyroid cancer. The head and neck region contains a large number of regional lymph nodes. Lymph nodes located in other sections of the body are referred to as distant lymph nodes.
NX: The lymph nodes in the region cannot be evaluated.
N0: In the regional lymph nodes, there is no sign of cancer.
N1: The cancer has progressed to the lymph nodes.
- N1a: The cancer has progressed to the lymph nodes surrounding the thyroid (called the central compartment; the pretracheal, paratracheal, and prelaryngeal lymph nodes).
- N1b: Cancer has migrated outside the central compartment, including unilateral cervical (lymph nodes on one side of the neck), bilateral cervical (lymph nodes on both sides of the neck), contralateral cervical (lymph nodes on the opposite side of the tumor), or mediastinal (chest) lymph nodes.
Metastasis (M)
The letter “M” in the TNM system indicates if cancer has migrated to other parts of the body, a process known as metastasis.
MX: It is impossible to assess distant metastases.
M0: The cancer has not spread to any other places of the body.
M1: Other regions of the body have been infected with cancer.
Cancer stage grouping
Doctors determine the cancer stage by integrating the T, N, and M classifications. This staging method for thyroid cancer vary depending on the type of tumor. The stage of papillary or follicular thyroid carcinoma is also determined by the patient’s age.
Papillary or follicular thyroid cancer in a person under the age of 55
Stage I: This stage denotes a tumor (any T) with or without lymph node spread (any N) and no distant metastases (M0).
Stage II: This stage denotes a tumor (any T) with any metastasis (M1), whether or not it has progressed to the lymph nodes (any N).
Papillary or follicular thyroid cancer in people aged 55 and over
Stage I: This stage denotes any tiny tumor (T1) with no lymph node spread (N0) and no metastases (M0).
Stage II: This stage denotes a bigger, noninvasive tumor (T2) with no lymph node spread (N0) and no metastases (M0).
Stage III: This stage denotes a tumor greater than 4 cm in size but still contained to the thyroid (T3), with no dissemination to lymph nodes (N0), and no metastasis (M0). Alternatively, any localized tumor (T1, T2, or T3) that has migrated to the core compartment of lymph nodes (N1a) but has not spread further (M0).
Stage IVA: This stage denotes a tumor that has spread to adjacent structures (T4a), regardless of whether it has spread to lymph nodes (any N), but not to distant locations (M0). Alternatively, this defines a tumor that is confined (T1, T2, or T3) with lymph node spread outside the core compartment (N1b) but no distant spread (M0).
Stage IVB: This stage denotes a tumor that has spread beyond adjacent structures (T4b), regardless of lymph node spread (any N), but there has been no distant metastasis (M0).
Stage IVC: When there is evidence of metastasis, this stage describes all tumors (any T, any N) (M1).
Medullary thyroid cancer
Stage I: This stage denotes a tiny tumor (T1) with no lymph node spread (N0) and no distant metastases (M0).
Stage II: This stage denotes a bigger localized tumor (T2 or T3) with no lymph node spread (N0) and no metastasis (M0).
Stage III: This stage refers to any localized tumor (T1, T2, or T3) that has spread to the core compartment of lymph nodes (N1a) but has not spread farther (M0).
Stage IVA: This stage denotes a tumor that has spread to adjacent structures (T4a), regardless of whether it has spread to lymph nodes (any N), but not to distant locations (M0). Alternatively, this defines a tumor that is confined (T1, T2, or T3) with lymph node spread outside the core compartment (N1b) but no distant spread (M0).
Stage IVB: This stage denotes a tumor that has spread beyond adjacent structures (T4b), regardless of lymph node spread (any N), but there has been no distant metastasis (M0).
Stage IVC: When there is evidence of metastasis, this stage is employed (any T, any N, M1).
Anaplastic thyroid cancer
Stage IV: Regardless of tumor size, location, or metastasis, all anaplastic thyroid cancers are classed as stage IV.
Stage IVA: This stage describes an anaplastic tumor that has spread to adjacent structures (T4a), regardless of whether it has progressed to lymph nodes (any N), but not to distant locations (M0).
Stage IVB: This stage denotes an anaplastic tumor that has spread beyond adjacent structures (T4b), regardless of lymph node dissemination (any N), but has not spread distantly (M0).
Stage IVC: When there is evidence of metastasis, this stage is employed (any T, any N, M1).
Positron emission tomography (PET) or PET-CT scan: A PET scan is frequently coupled with a CT scan (see above), resulting in a PET-CT scan. However, your doctor may refer to this technique simply as a PET scan. A PET scan is a technique for creating images of organs and tissues within the body. A radioactive sugar compound is put into the patient’s body in modest amounts. This sugar molecule is absorbed by the cells that consume the most energy. Cancer absorbs more radioactive stuff because it aggressively uses energy. The amount of radiation in the material, however, is too low to be dangerous. The material is then detected by a scanner, which produces images of the inside of the body.
TREATMENT OF THYROID CANCER
In many circumstances, a group of clinicians collaborates to develop a patient’s overall treatment plan, which integrates many sorts of therapy. This is referred to as a multidisciplinary team. This team may include a surgeon, medical oncologist, radiation oncologist, radiologist, nuclear medicine physician, and endocrinologist in the case of thyroid cancer. Other health care professionals on cancer care teams include physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, nutritionists, speech therapists, and others.
The kind and stage of thyroid cancer, potential side effects, and the patient’s preferences and overall health all influence treatment options and recommendations. Take the time to read about your treatment options, and don’t be afraid to ask clarifying questions. Discuss the goals of each treatment with your health care team, as well as what you can expect during treatment. These discussions are known as “shared decision-making.” When you and your doctors collaborate to identify therapies that meet the goals of your care, this is referred to as shared decision-making. Because there are various treatment choices for thyroid cancer, shared decision-making is very vital.
Cancer treatment is frequently chosen based on guidelines proposed by teams of professional physicians. Although most thyroid cancers are treatable, there may be differing views on how to treat thyroid cancer, notably on which combination of medicines to employ and when treatments should be administered. Patients are recommended to get a second opinion before beginning treatment because they should be confident in the treatment plan they select and should inquire about clinical trials.
Surgery
During a surgery, the tumor and some surrounding healthy tissue, known as a margin, are removed. A resection is another term for surgery. It is the primary treatment for the vast majority of thyroid cancer patients. A surgical oncologist is a specialist who specializes in the surgical treatment of cancer. Common surgical alternatives, depending on the size of the thyroid nodule, include:
- Lobectomy: This operation removes the lobe of the thyroid gland containing the malignant nodule.
- Near-total thyroidectomy: This procedure, also known as a subtotal thyroidectomy, removes everything but a little portion of the thyroid gland.
- Total thyroidectomy: The entire thyroid gland is removed during this procedure.
A thyroidectomy can be performed using a variety of surgical procedures, including:
Standard thyroidectomy: A minor incision (or cut) near the base or middle of the neck is made by the surgeon. This allows the surgeon immediate access to the patient’s thyroid gland during the procedure. Newer surgical procedures are being given at some cancer centers to lessen or avoid neck scars.
Endoscopic thyroidectomy: The physician makes a single tiny incision during this surgery. The procedure is similar to a regular thyroidectomy, except that a scope and video monitor are used to guide the procedure instead of surgical loupe magnification, which requires specialized eyewear.
Robotic thyroidectomy: The surgeon makes an incision somewhere else, such as an armpit, the hairline of the neck, the mouth, or the chest, and then performs the thyroidectomy with a robotic tool. Thyroid cancer should not be treated with a robotic thyroidectomy.
Not all surgical procedures are appropriate for all patients. Consult your doctor about the best way to treat you.
If there is evidence or a possibility of cancer spreading to the lymph nodes in the neck, the surgeon may conduct a neck dissection as well. This is a procedure used to remove the lymph nodes in the neck. Lymphadenectomy is another term for neck dissection.
Thyroid surgery risks can include injury to adjacent parathyroid glands, which assist regulate blood calcium levels, severe bleeding, or wound infections. If the laryngeal nerves are injured during surgery, this can result in temporary or permanent hoarseness or a “breathy” voice.
Without the thyroid gland, the body ceases to produce thyroid hormone, which is required for proper body function. The best approach is hormone replacement therapy, which is usually administered as a daily pill. If the parathyroid gland’s function is impaired following surgery, the patient may need to take vitamin D and calcium supplements.
Unresectable or inoperable tumors are those that cannot be removed surgically. Other treatment alternatives will subsequently be suggested by the doctor.
Before surgery, consult with your health care team about the potential side effects of the specific surgery you will undergo, as well as what to expect throughout your recovery.
Hormone treatment
Thyroid hormone therapy is generally required for patients who have surgery. In addition to providing the hormone that the body requires, thyroid hormone treatment may decrease the growth of any differentiated cancer cells that remain.
Levothyroxine is a thyroid hormone replacement (Levothroid, Levoxyl, Synthroid, Tirosint, Unithroid, and other brand names.) Levothyroxine is normally available as a pill that should be taken on a daily basis, at the same time every day, to ensure that the body receives a steady amount. Thyroid hormone replacement is typically administered by an endocrinologist, a specialist who specializes in the treatment of hormone, gland, and endocrine system issues.
Also, discuss with your doctor any other drugs you are taking, including dietary supplements such as iron or calcium, to avoid interactions with your thyroid hormone replacement.
Thyroid medication may cause a few negative effects. During the first few months of treatment, some individuals may develop a rash or lose some hair.
Hyperthyroidism is a disorder characterized by an excess of thyroid hormone. Weight loss, chest pain, high heart rate, irregular heartbeat, cramps, diarrhea, a feeling of being overheated, sweating, and bone loss or osteoporosis are all possible side effects.
Hypothyroidism is a condition in which the thyroid hormone is insufficient. It can induce weariness, weight gain, dry skin and hair, and a chilly sensation.
The required amount of thyroid hormone, known as a dose, varies depending on the patient and tumor type, and it can fluctuate as a person matures or their weight changes. Your thyroid hormone levels will be monitored by the doctor through regular blood testing. Consult your doctor about the warning signs that indicate it is time to change your hormone supplement dose.
Radioactive iodine therapy
Almost all iodine that enters the body is absorbed by the thyroid. As a result, a type of radiation therapy known as radioactive iodine (commonly known as I-131 or RAI) can locate and eliminate thyroid cells that have not been removed by surgery as well as those that have spread beyond the thyroid. Endocrinologists or nuclear medicine experts are the most common doctors that prescribe radioactive iodine therapy.
Some persons with papillary, follicular, or Hurthle cell thyroid cancer may benefit from radioactive iodine treatment. People with differentiated thyroid carcinoma that has progressed to lymph nodes or distant places are treated with radioactive iodine. A tiny test dose may be administered before to the complete course of treatment to ensure that the I-131 is absorbed by the tumor cells. I-131 should not be used to treat patients with MTC or anaplastic thyroid carcinoma.
I-131 treatment is administered in the form of a liquid or a tablet. Patients who get I-131 to eliminate cancer cells may be hospitalized for 2 to 3 days, depending on a variety of conditions, including the amount administered. Patients are recommended to drink water to help the I-131 flow through their bodies as fast as possible. The majority of the radiation is gone within a few days. Discuss with your doctor how to prevent radiation exposure to other people, especially children, who may be in your vicinity during this treatment and the days following it.
Patients are frequently asked to follow a low-iodine diet for 2 to 3 weeks prior to I-131 therapy after surgery. In addition to the low-iodine diet, patients will be advised to either temporarily discontinue thyroid hormone replacement pills or to undergo injections of recombinant TSH (Thyrogen) while on the hormone replacement. If the hormone therapy is interrupted during the preparation phase, the patient may most likely encounter hypothyroidism adverse effects.
It is critical to discuss with your doctor the potential short- and long-term effects of I-131 therapy. Patients may experience nausea and vomiting on the first day of treatment. In some cases, discomfort and edema may develop in the sites where radioactive iodine is collected. Because iodine is abundant in salivary gland tissue, patients may have salivary gland edema. This can lead to xerostomia, often known as dry mouth.
Large or cumulative doses of radioactive iodine may result in infertility, or the inability to bear a child, particularly in men. Pregnancy should be avoided for at least a year following I-131 treatment. With the use of I-131, there is a danger of secondary cancer. Patients may occasionally require multiple treatments over time. However, there is a limit cumulative dose of radioactive iodine allowed over time, and once achieved, future use of this treatment may be prohibited.
External beam radiation therapy
Another type of radiation therapy is external-beam radiation, in which high-energy x-rays are delivered from a machine outside the body to eliminate cancer cells. A radiation oncologist is a doctor who specializes in external-beam radiation therapy. An external-beam radiation therapy regimen (schedule) typically consists of a predetermined number of treatments administered over a predetermined time period. Radiation therapy is often administered as outpatient therapy, either in a hospital or clinic, 5 days a week for roughly 5 to 6 weeks to treat thyroid cancer.
External-beam radiation therapy is only utilized in specific cases of thyroid cancer, generally when the cancer has advanced to key parts of the neck such as the trachea, voice box, or esophagus. Radiation therapy is typically administered following surgery, and the treatment is focused on a specific spot, harming just cancer cells at that location. In most cases, radiation therapy is not utilized to treat thyroid cancer in young people.
Side effects may include skin redness, odynophagia (difficult swallowing), cough, occasional hoarseness, nausea, and weariness, depending on the treatment dosage and location. The majority of negative effects fade quickly after treatment is completed.
Chemotherapy
Chemotherapy is the use of medications to eradicate cancer cells, typically by preventing the cancer cells from growing, dividing, and proliferating. A chemotherapy regimen, or schedule, typically consists of a predetermined number of cycles administered over a predetermined time period. A patient may be administered one medicine at a time or a mixture of drugs at the same time.
Chemotherapy side effects vary depending on the individual and the dose used, but they can include exhaustion, infection risk, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These adverse effects normally fade away once the treatment is completed.
Targeted therapy
Targeted therapy is a type of cancer treatment that targets specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This sort of treatment inhibits cancer cell growth and spread while causing minimal damage to normal cells.
The targets of all cancers are not the same. Your doctor may order tests to determine the genes, proteins, and other variables in your tumor in order to find the most effective treatment. This enables clinicians to provide the most effective treatment to each patient whenever possible. Furthermore, research investigations are continuing to learn more about specific molecular targets in the many forms of thyroid cancer, as well as new medicines aimed at them.
The U.S. Food and Drug Administration (FDA) has approved various targeted therapy for papillary and follicular thyroid cancers:
- In 2013, the FDA approved sorafenib (Nexavar), a targeted therapy, for later-stage or recurrent differentiated thyroid cancer when I-131 therapy (see above) had failed. Sorafenib commonly causes hand-foot skin responses or other skin problems, diarrhea, weariness, weight loss, and elevated blood pressure.
- In 2015, the FDA authorized lenvatinib (Lenvima, E7080) as a targeted therapy for later-stage differentiated thyroid cancer when surgery, I-131 treatment, or both had failed. High blood pressure, diarrhea, decreased appetite, weight loss, and nausea are some of the side effects of lenvatinib.
- The FDA approved larotrectinib (Vitrakvi) in 2018 for the treatment of uncommon cases of papillary and follicular thyroid tumors with an NTRK gene fusion mutation. Larotrectinib side effects include fatigue, nausea, dizziness, vomiting, constipation, and diarrhea.
- Entrectinib (Rozlytrek) was also approved by the FDA in 2019 for thyroid cancers with an NTRK gene fusion mutation. Fatigue, constipation, swelling, disorientation, taste difficulties, diarrhea, nausea, nerve pain or discomfort, shortness of breath, muscular pain, thinking and memory problems, weight gain, cough, vomiting, fever, joint pain, and visual abnormalities are common adverse effects of entrectinib.
- The FDA approved pralsetinib (Gavrecto) and selpercantinib (Retevmo) in 2020 for the treatment of rare patients of advanced metastatic RET fusion-positive thyroid cancer that require systemic therapy. Constipation, hypertension, weariness, musculoskeletal discomfort (pain in the muscles, bones, and/or nerves), and diarrhea are some of the side effects of pralsetinib. Dry mouth, diarrhea, hypertension, weariness, edema, elevated cholesterol, rash, and constipation are some of the side effects of selpercantinib. Selpercantinib can potentially impact platelet levels, as well as the amounts of certain enzymes and proteins in the body. These levels must be checked every two weeks for the first three months of treatment, and then once a month thereafter.
The FDA-approved targeted treatment options for MTC include:
- Vandetanib tablets (Caprelsa, zd6474) were approved by the FDA in 2011 as a type of targeted medicine known as a tyrosine kinase inhibitor. Vandetanib, in particular, is now considered a standard treatment for adults when MTC cannot be removed surgically, when the disease is progressing, or when MTC has migrated to other parts of the body.