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Endocrinological Cancers

Summary

The endocrine system produces hormones that communicate information about the body from cell to cell. It affects many different aspects of the body such as growth, development, and metabolism. Organs included in the endocrine system that produce these hormones are the pituitary and pineal glands in the brain, thyroid gland, parathyroid gland, adrenal glands, kidneys, and pancreas. Cancers of the endocrine system are rare and account for only 1.5% of new cancers.

Forms of Endocrine Cancer

Thyroid cancer is the most common cancer of the endocrine system and accounts for the majority of deaths. There are many different types of thyroid tumors with different aspects to each. Thyroid cancer is usually seen more in females than males an as a patient increases in age. Almost 26,000 cases are seen a year. A patient may notice swelling in the area of the neck where the thyroid is located. This may cause discomfort or hoarseness. Few thyroid cancers are diagnosed with metastasis, but those that are can be found in the lymph nodes, bone, or lung.

Papillary thyroid carcinoma is the most common type of thyroid cancer. Can be caused by an altered, inherited gene or genetic mutations occurring over a lifetime found on the RAS, BRAF, TRK, and RET genes or . A mutation of the p53 tumor suppressor gene has the ability to cause this cancer to advance in stage. Though it is considered to be the least aggressive of thyroid cancer it may become aggressive. Metastasis of the carcinoma is usually seen first in the lung.

Follicular thyroid carcinoma is seen in patients with Cowden’s disease. In thyroid cancer, follicular thyroid carcinoma accounts for 14% of cases. It is thought to be more aggressive than papillary thyroid carcinoma. Metastasis of this carcinoma can be seen in lymph nodes of the neck, bones, and lungs. This carcinoma may results from genetic mutations in genes such as MEN1, RAS oncogene, and p53 tumor suppressor gene.

Medullary thyroid carcinoma accounts for 3% of thyroid cancers. Cases have been found to be inherited genetically, caused by genetic mutations, or occur spontaneously. Genetic analysis is important to diagnosis of this carcinoma. It is important that if a gene defect is discovered that all family member be tested for the defect detected. Early metastases can be seen in the trachea, nearby lymph nodes; while late metastases can be seen in bones, lungs, adrenal glands and liver. Genetic mutations that may result in this carcinoma are seen in the ret oncogene and MEN 2 gene.

Anaplastic thyroid carcinoma is seen in 2% of thyroid cancers. It is characterized by early metastases, highly aggressive, and seen more often in older patients. Genetic mutations of the p53 tumor suppressor gene may be a factor in causing this carcinoma.
Lymphoma of the thyroid is usually seen in older women, frequently seen as a B Cell Lymphoma, and the most common sign is a fast growing throat mass.

Pancreatic cancer has greater than a 98% fatal outcome. Pancreatic cancer has been seen to be more prevalent in males and when looking at race, blacks. Risk factors for pancreatic cancer include obesity, cigarette smoking, having chronic pancreatitis, and diabetes mellitus (Type II). Though the causes of this cancer are not clear, studies have found genetic mutations in the K-ras, p16INK4, p53, DPC4, and BRCA2 genes in patients. Because of the lack of understanding in cause, making it difficult to treat; pancreatic cancer is the fifth leading cause of cancer death in the United States.

Ductal adenocarcinoma is seen in 90% of pancreatic cancers. It occurs more frequently in the head than in the body than the tail of the pancreas. This is important due to… Symptoms may include jaundice (with dark urine and clay like stool), intense abdominal pain with discomfort, weight loss, enlarged gallbladder, glucose intolerance, venous thrombosis, Trousseau’s syndrome, stomach/intestinal hemorrhaging.

Islet cell tumors are seen in 10% of pancreatic cancer. Syndromes of islet cell tumors are Insulinoma, Gastrinoma, VIPoma, Glucagonoma, Somatostatinoma, and PPoma. Insulinomas are the most common syndrome of islet cell tumor in patients. They are 90-100% benign but can have lasting neurologic symptoms.

Adrenal Glands, located above the kidneys, use several produced hormones to control body responses to stress, maintain the correct water/salt concentration, and regulation of blood pressure. They are composed of the medulla at the center and the cortex surrounding the medulla on the outside. The cells in the medulla are responsible for producing adrenaline and noradrenaline which are the key hormone in the fight or flight response. The cells in the cortex are dedicated to producing hormones from cholesterol.

Adrenocortical Cancer is a rare cancer seen in children and adults that is classified as functioning or nonfunctioning. Functioning tumors release hormones such as cortisol, androgens, estrogens, and aldosterone; while nonfunctioning tumors do not. Genetic mutations on chromosomes 2, 9, and 11 and genes p53 and MENIN have been found to contribute to the development of this cancer. It has been seen that the removal of the tumor increases the rate of survival.

Pheochromocytomas are rare tumors seen more frequently in adults in their 40s and 50s, but often in children. These tumors can be found on one or both adrenal glands, malignant or benign, and on the inside or outside of the adrenal gland. It occurs frequently in patients that have inherited mutations of MEN2A and MEN2B on the RET gene. Signs and Symptoms of this cancer can be high blood pressure, headaches, anxiety, chest and abdominal pain, vomiting, and shortness of breath. A simple 24 urine samples can be used to diagnose this cancer.

Thymus Gland is composed of two structures, the medulla and cortex. It is in these structures where hormones are produced, that are important for the immune response to foreign bacteria and viruses, and white blood cells travel to mature and then circulate in the blood.

Thymoma is the most common type of thymus cancer and is seen frequently in adults and rarely in children. Surgical removal of the tumor improves survival and remission of the cancer. Signs and symptoms of this cancer may include chest pains, coughing, and myasthenia gravis. These tumors are usually contained and present with benign features. Malignant thymomas are called Thymic carcinoma. This malignant form is usually not contained, prone to metastases, and has poor outcomes for patients. Survival of a patient is determined by the stage and histology of the tumor. Those with early stages have a higher suggested survival rate than those with advanced stages.

Thymolipoma are contained, rare, benign tumors that usually do not metastases. These tumors can go unnoticed for long periods of time, allowing them to grow into large masses.

Pineal Gland is found in center of the left and right sides of the brain. The pineal gland is stimulated by the sensation of darkness to start the production of melatonin, which is thought to regulate sleep. Once the sensation of darkness is replaced by the sensation of light the production stops. Tumors in this gland may cause symptoms of increased pressure in the brain cavity, visual difficulties and problems with walking. Treatment usually involves surgery and radiation at the tumor site.

Germinomas are tumors of the pineal gland that have the ability to compress parts of the brain, block ducts, and hinder the flow of blood in blood vessels. Germinomas are the most common form of pineal gland tumors.

Pinealoma is composed of pineocytomas and pineoblastomas. These tumors have similar growth patterns as Germinomas, are considered to be invasive, and their mass enlarges the pineal gland.

Gliomas have the same structural characteristics of Astrocytomas and are classified into stages by the degree of malignancy. Increased pressure of the brain cavity, visual abnormalities, and weakness are some of the signs and symptoms of this cancer. At one point doctor thought these tumors to be inoperable but today through the use of new technologies these tumor can be removed. It has been seen that some patients have survived 5 or more year after surgery to remove the tumor.

Pituitary is found at the base of the brain protected by bone and tissue. The main purpose of the pituitary gland is to regulate and maintain a stable environment of the body. Some of the processes that the pituitary helps to regulate are growth and development, blood pressure, organ function, metabolism, temperature and water balance.

Pituitary Adenomas are tumors found on the anterior portion of the pituitary gland. As a patient gets older these tumors become more numerous. These tumors are most commonly composed of chromphobe cells which secret hormones and by the detection of these hormones a diagnosis are made. Visual problems and headaches are some of the signs and symptoms that a patient may have with this condition.

Parathyroid is located behind the thyroid. It usually consists of four glands but has been seen to have as many as eight. The parathyroid’s main purpose is to regulate calcium levels in the blood so that the muscular and nervous system can function properly. To increase the level of blood calcium, parathyroid hormones are produced and secreted into the blood stream from the parathyroid gland. In reverse of this the thyroid produces and secretes calcitonin to decrease the amount of calcium in the blood. Between the parathyroid and thyroid glands a delicate balance of calcium blood levels is achieved.

Parathyroid Adenoma is usually surgically removed and diagnosis is made by determining the amount of parathyroid hormone present in the tissue.

Carotid body, Paraganglia is small organ close to where the common carotid artery splits in two to form the right and left carotid arteries. It is composed of chemoreceptor cells that are sensitive to changes in oxygen, carbon dioxide, temperature and pH of the blood. The carotid body detects these changes and sends the information to the central nervous system so that adjustments can be made.

Carotid Body Tumor (Paraganglioma) are usually benign but can be malignant or become malignant. These tumors are found where the carotid artery splits into two arteries running parallel up and down the neck. They are commonly painless growths with symptoms of sleep apnea, reduced respiratory rates, danger of fluctuation of blood pressure, and stroke. Some patients have been found to have a family history of this cancer leading scientist to believe that inherited genetic mutations are present.

 

Preventive measures

 

 

Diagnostic Process

 

Tests

 

Treatments

 

Advanced Science & Research

 

Clinical Trials

We are actively participating in clinical trials and research for our cancer patients. Newly developed treatments or investigational drugs may reduce tumor size and eliminate symptoms better than treatments currently available.

Patients are asked if they would like to participate in our clinical trials to evaluate new cancer prevention and treatment choices. Participation is completely voluntary. Ask the doctor about clinical trials at your visit.

See the patient information on Clinical Trials at the Feist-Weiller Cancer Center. Additional information is available for medical professionals.  For more information about ongoing clinical trials at the Feist-Weiller Cancer Center, please call us toll-free at 1-866-LSU-FWCC (578-3922) or (318) 813-1410.

Cancer Support Group

The Feist Weiller Cancer Center offers two patient support groups, meeting once a week, for all types of cancer. For more information about these two support groups, please call:

Jo Ann Stewart, RN at (318) 813-1409
Susie Wiggins, RN at (318) 813-1417
Ron Nierman at (318) 470-6180

Genetic Testing and Counseling 

Almost all of us have known someone with cancer, be it a colleague, friend, or family member. For many, these acquaintances are few, and family occurrences sporadic, but for some families, cancer appears to have a much higher prevalence and may be passed throughout many generations. Our expanding knowledge regarding the hereditary aspect of cancer has enabled genetic counselors, nurses, and physicians to provide risk counseling to patients; and advances in genetics have allowed us to develop tests that help to pinpoint this hereditary risk. 

At the Feist-Weiller Cancer Center, the Hereditary Cancer Risk Assesment Program performs an initial cancer risk assessment for all patients by recording a complete family history and creating a pedigree in which all affected relatives are shown. If the patient has a positive family history of cancer, he or she is referred for genetic counseling in order to determine if genetic testing is the next appropriate step. Once genetic testing is completed, patients receive an additional counseling session in which results are disclosed and discussed. If a patient tests positively for a given mutation, specific surveillance and preventive options, as well as additional genetic testing for other family members, are discussed.

If a genetic mutation is detected in at-risk individual, which predisposes him to development of a hereditary cancer syndrome, this information is crucial in guiding the future medical and surgical management of this individual. In addition, certain hereditary cancers have unique behaviors at both a clinical and molecular level, and detection of specific mutations may help to guide therapy for these types of cancer. Discovery of a mutation specific for a hereditary cancer syndrome is not only helpful in guiding future management of that individual, but it also provides useful information about disease risk in other family members, and also the risk of the affected individual passing the mutation to his or her unborn offspring.