Endocrine Tumors & Cancer (Stevie JoEllie's Cancer Care Fund )

Endocrine Tumors & Cancer

Contents
1 Summary Review
2 Familial thyroid cancer: Abstract
3 Pediatric Thyroid Cancer
3.1 Overview
3.2 More About Differentiated Pediatric Thyroid Cancer: Papillary and Follicular
3.3 Statistics on Differentiated Pediatric Thyroid Cancer
3.4 Causes
3.5 Pediatric Differentiated Thyroid Cancer
3.6 Pediatric Medullary Thyroid Cancer

Summary Review

Thyroid nodules, thyroid lumps, and the thyroid enlargement known as a goiter By Wilma Ariza, Thyroid Cancer Advocate

If your doctor has diagnosed you as having a thyroid nodule, a thyroid lump or an enlargement of the thyroid known as goiter, you'll want to know more about the diagnosis and treatment process, the relationship between nodules to thyroid cancer, and follow up. Alternatively, you may experience a swelling of the thyroid gland, also known as a goiter.

Thyroid nodules are very common. A nodule is a swelling or lump, which can be a solid or liquid filled cyst or mass. Most are benign, but a small percentage can be cancerous. So you should always have a nodule evaluated by your physician as soon as you notice it. An estimated one in 12 to 15 women and one in 50 men has a thyroid nodule. More than 90 percent of all thyroid nodules are not cancerous.

Some symptoms of thyroid nodules include palpitations, insomnia, weight loss, anxiety, and tremors, common in hyperthyroidism.

Common hypothyroidism symptoms might include weight gain, fatigue, depression. Some people will cycle back and forth between hyperthyroid and hypothyroid symptoms. Others may have difficulty swallowing, a feeling of fullness, pain or pressure in the neck, a hoarse voice, or neck tenderness. And finally, many people have nodules wiht no obvious symptoms related to thyroid dysfunction at all.

A goiter is an enlargement of the thyroid, and is sometimes used as a term to refer to an enlarged thyroid. The thyroid becomes large enough so that it can be seen as enlarged on ultrasounds or x- rays, and may be e nlarged enough to

enlarge the neck area visibly.

Some symptoms of a goiter include tenderness to the touch, pressing on your windpipe or your esophagus, coughing, hoarseness, shortness of breath, fullness in your neck, choking or shortness of breath at night, or a feeling that food is getting stuck in your throat.

There are four types of Thyroid Cancer:

1. Papillary cancer, is the most common type of cancer, perhaps because papillaries are quite common in the thyroid gland. Papillary cancer mostly involves one side of the thyroid and sometimes spreads into the lymph nodes. The cure rate is very high.

2. Follicular cancer, the second most common type of thyroid cancer, is somewhat more malignant than papillary. The thyroid gland is comprised of follicles which produce thyroid hormones that are essential for growth and development of all body tissues. This cancer doesn't usually spread to the lymph nodes, but it may spread to arteries and veins of thyroid gland and more distantly (lung, bone, skin, etc), though that is uncommon. Follicular cancer is more common in older people.

3. Medullary thyroid cancer, is the third most common type of thyroid cancer, and usually originates in the upper central lobe of the thyroid. It spreads to the lymph nodes earlier than papillary or follicular cancers. It differs from papillary and follicular cancer, however, in that it does not arise from cells that produce thyroid hormone, but instead from C cells. These C cells make the hormone calcitonin. This type of cancer can run in families.

4. Anaplastic thyroid cancer, is the rarest and most serious thyroid cancer. It can spread early to lymph nodes, thus usually the cause for a visit to the doctor is a mass in the neck. It also is the form of thyroid cancer most likely to spread to other organs beyond the thyroid or lymph nodes. This type of thyroid cancer is more common in those over 65 and in men. Long-term survival rates are far less than for the other three types of cancer.

Familial thyroid cancer: Abstract

Opinion Article in Oncology. 13(1):44-51, January 2001.
Alsanea, Osamah MD *; Clark, Orlo H. MD +

Familial thyroid cancer can arise from parafollicular cells (familial medullary thyroid cancer) or from follicular cells (familial nonmedullary thyroid cancer). Familial medullary thyroid cancer may occur in isolation or as part of multiple endocrine neoplasia (MEN) type II syndromes. Genetic testing for a RET mutation on chromosome 10 is used to identify new family members who are gene carriers.

Total thyroidectomy should be used in gene carriers without clinical disease before age 6 in medullary thyroid cancer and MEN type IIA, and as soon as the diagnosis is made in MEN type IIB after the first year of life. Those with clinical disease should have at least a bilateral central neck dissection. Modified radical neck dissection is recommended for patients when the primary tumor is 1.5 cm.

A normal postoperative serum calcitonin level suggests that the operation has been curative. Physicians need to be aware of ethical and lifestyle issues related to patients with familial disease and their family members.

Familial nonmedullary thyroid cancer occurs as a discrete entity or as part of other family cancer syndromes such as Gardner syndrome, Cowden disease, and other rare syndromes.

Familial nonmedullary thyroid cancer almost exclusively includes patients with papillary or Hurthle cell cancers. These families appear to have more benign thyroid conditions. The gene (or genes) for familial papillary thyroid cancer is yet to be identified, whereas that for some Hurthle cells (TCO) has been mapped to chromosome 19p13.2.

Familial nonmedullary thyroid cancer is somewhat more aggressive than its sporadic counterpart, but is less aggressive than medullary thyroid cancer. Total thyroidectomy and central neck dissection followed by radioactive iodine ablation and thyroid hormone suppression appear to be the most effective therapy.

Pediatric Thyroid Cancer

Overview

Thyroid cancer occurs in all age groups, from children and adolescents through seniors.

Thyroid cancer remains a rare disease in children less than age 10, with an annual incidence of less than one per million. It is more common in older children and adolescents, with 15.4 cases per million per year in 15-19 year olds. It has a peak incidence at age 50 and beyond.
The number of female adolescents with thyroid cancer has slightly increased in the United States in recent years. Males and young children under age 15 have had no increase in incidence over the last several decades.
The two main types of pediatric thyroid cancer are:
- Differentiated Thyroid Cancer: This includes papillary and follicular thyroid cancer and their variants.Papillary thyroid cancer is the most common type in both children and adults.
- Medullary Thyroid Cancer. About 30% to 35% are familial tumors transmitted by a gene from either the mother or father.
Children and Adolescents: Thyroid Nodules and Well-Differentiated Thyroid Cancer: http://www.thyca.org/refbk_kids.htm
Are There Differences in the Presentation of Thyroid Nodules and Thyroid Cancer in Adults and Children? http://www.thyca.org/refbk_presentation.htm

More About Differentiated Pediatric Thyroid Cancer: Papillary and Follicular

Because of the lack of prospective research, differentiated thyroid cancer in children and adolescents generally is managed similarly to that in adults. Papillary thyroid cancer is the type of tumor that affects children in the vast majority of cases and which, compared with other types of thyroid cancer, has the best prognosis.
Each case is approached individually, to fit the needs of each patient.
In children and adolescents, papillary thyroid cancer tends to be more advanced at the time of diagnosis than it is in adults with the same disease.
The majority of children with papillary thyroid cancer have local spread to the lymph nodes of the neck at the time the thyroid cancer is diagnosed. About 10% to 20% of the children have distant metastases, most commonly to the lung, compared with only 5% of the adults with this disease.
Recurrence of papillary thyroid cancer is more common in children than it is in adults.
However, despite higher rates of recurrence and more widespread disease at presentation, the prognosis is excellent with appropriate treatment.
Follicular thyroid cancer, which is rare in children, has been reported to demonstrate more aggressive characteristics and poorer prognosis due to vascular invasion.
Still, the prognosis is better for children than for their adult counterparts who present with a similar extent of disease.
Follicular Thyroid Cancer. Follicular thyroid cancer is not very common in childhood. The primary tumor is usually a single lesion within the thyroid gland. Lymph node metastases can occur with less differentiated variants of follicular thyroid cancer but are not generally expected. It is associated with a worse prognosis (as compared to papillary thyroid carcinoma) due to its propensity for vascular invasion, which increases the risk of metastases to distant sites of the body, such as the lungs and bones.
Very few children die from this disease. Even those children with distant metastases at diagnosis can anticipate survival for years to decades.
The goals of treatment are to eliminate the disease and to reduce the chance of recurrence.
Sometimes the disease cannot be entirely eradicated, and therefore, another therapeutic goal is to achieve stable disease and no symptoms of disease.

Statistics on Differentiated Pediatric Thyroid Cancer

Papillary and follicular thyroid cancer accounts for only approximately 1% of all pediatric cancers in the 5-9 year old age group and up to 7% of cancers in the 15-19 year old age group.
Only 1 in a million children younger than age 10 years will get thyroid cancer.
In children under age 10, thyroid cancer tends to affect boys and girls with about equal frequency, but thereafter it generally becomes more common in girls. The ratio of girls to boys with differentiated thyroid cancer reaches a peak of over 5 to 1 in the 15-to-20 year-old age group.
Medullary thyroid carcinoma (MTC) accounts for 5% to 10% of all thyroid cancers. In children and adolescents, it is a very rare disease, affecting less than one child per million per year.

Causes

As the National Cancer Institute notes, no one knows the exact causes of thyroid cancer. Doctors can seldom explain why one person gets this disease and another does not.

Research has shown that people with certain risk factors are more likely than others to develop thyroid cancer. The following risk factors are associated with an increased chance of developing pediatric thyroid cancer:

Pediatric Differentiated Thyroid Cancer

For differentiated thyroid cancer, a risk factor is exposure at a young age to ionizing radiation, whether from ingestion of radioactive iodines (iodine is the building block of thyroid hormones) or external radiation, as used to treat some childhood cancers. The main example of this was the increased rate of thyroid cancer identified in children following the 1986 Chernobyl nuclear power plant accident.
In general, the differentiated thyroid carcinomas are not diseases that are passed from one generation to the next. However, there are some hereditary forms of the disease, although the exact cause of the disease in these families is not yet known. Some genetic syndromes such as Gardner syndrome and the Carney complex can have papillary thyroid carcinoma as part of the spectrum of disease. Some follicular thyroid carcinomas can be associated with a mutation in the PTEN gene, which causes Cowden syndrome. Gene mutations (RET/PTC, BRAF, among others) that cause differentiated thyroid cancer have also been identified within tumor cells. These mutations are not in all cells of the body and therefore are not expected to be hereditary.

Pediatric Medullary Thyroid Cancer

In children and adolescents, medullary thyroid cancer is almost always the familial form, meaning that it is due to a specific mutation (defect) in the DNA of the cells of the body. This mutation occurs in a gene called the RET proto-oncogene.

Subpages (1): Thyroglobulin Measuring Tests

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