An adrenal gland tumor is a rare health condition that directly affects your body’s hormonal balance. These tumors are generally benign, but they can be malignant in about 10% of patients. Although they are most commonly seen in individuals aged 20 to 50, they can affect any age group. If left untreated, they can cause serious damage to your body. In this article, we will discuss everything you need to know about the symptoms, diagnosis, and treatment of pheochromocytoma in clear terms.
What is Pheochromocytoma?
Definition of Pheochromocytoma
Pheochromocytoma is a tumor that develops in the medulla region of the adrenal glands. This area, known as the adrenal medulla, produces hormones that your body needs during stressful situations. The tumor secretes stress hormones like adrenaline and noradrenaline in much larger quantities than normal.
This excessive hormone production directly affects your heart rate and blood pressure. Hormone secretion can occur in sudden attacks, leading to unexpected symptoms. Pheochromocytomas are rare tumors, but if not diagnosed, they can lead to serious health issues.
The vast majority of tumors are benign. However, 10% of cases exhibit malignant characteristics. Pheochromocytomas that develop outside the adrenal gland are usually malignant. Sporadic pheochromocytomas more frequently affect the right adrenal gland.
Function of the Adrenal Gland
The adrenal glands are small organs located just above your kidneys. Each gland consists of two different parts: the cortex on the outside and the medulla on the inside. These two parts perform vital functions in your body by producing different hormones.
The cortex produces cortisol, aldosterone, and androgen hormones. Cortisol regulates your stress levels and contributes to your immune system. Aldosterone maintains the balance of salt and water in your body. The medulla produces adrenaline and noradrenaline.
These hormones control your blood pressure, heart rate, metabolism, and stress responses. Adrenaline and noradrenaline particularly regulate your heart rate and prepare your body for emergencies. Therefore, any issues arising in these glands can lead to uncontrolled hormone production.
Types and Characteristics of Tumors
Different types of tumors can develop in the adrenal glands. These tumors are divided into two main groups based on whether they produce hormones: functional and non-functional masses.
Adenoma is the most common type of adrenal gland tumor. It is not cancerous and is classified as a benign mass. Adenomas can secrete cortisol or aldosterone.
Pheochromocytoma develops in the medulla of the adrenal glands and is a hormone-producing tumor. In most cases, it is not cancerous, but it secretes excessive amounts of catecholamines. At least 10% of pheochromocytomas are malignant. The diagnosis of malignant pheochromocytoma is made by detecting tumor accumulations in tissues that normally do not contain chromaffin cells.
Adrenal cortex cancer is a rare tumor that starts in the cortex of the gland and can lead to imbalances in hormone production. It constitutes the most common type of cancerous adrenal gland tumors.
Neuroblastoma is a type of cancer that most commonly starts in the adrenal gland in children. It can also develop in the neck, chest, or spinal cord. Pheochromocytomas can also be seen bilaterally in MEN Type II, familial pheochromocytoma syndrome, Von-Hippel Lindau disease, and rarely in neurofibromatosis.
What are the Symptoms of Adrenal Gland Tumors?
The excessive production of hormones is the main cause of the symptoms of adrenal gland tumors. These symptoms vary from person to person and can sometimes appear in sudden attacks. Stress, exercise, or the consumption of certain foods can trigger symptoms.
High Blood Pressure and Palpitations
High blood pressure is the most common finding in the diagnosis of adrenal gland tumors. Approximately 50% of patients experience persistent hypertension, while 40-50% experience intermittent high blood pressure attacks. The frequency and duration of hypertensive attacks vary among patients. Some patients may have an attack lasting one hour once a day, while others may experience it monthly for half an hour.
If blood pressure is not controlled, serious consequences such as stroke or heart attack can occur. High blood pressure can damage the cardiovascular system tissues, brain, and kidneys. This damage can lead to critical conditions such as heart disease, arrhythmia, heart failure, brain hemorrhage, kidney failure, and intraocular bleeding.
Palpitations and rapid heart rate occur in 51 to 73% of patients. The acceleration and irregularity of heartbeats are common symptoms. An irregular increase in adrenaline hormones speeds up heartbeats and affects breathing.
Sweating and Headaches
Excessive sweating occurs in 60-70% of patients. Sweating is usually more pronounced in the upper part of the body. The body releases a large amount of adrenaline, leading to palpitations and sweating. Due to the excessive secretion of adrenaline, narrowing of blood vessels can also cause paleness of the skin.
Headaches are seen in 72-93% of patients. They typically have a throbbing character and are most often felt in the back of the head or forehead. Headaches develop due to changes in blood flow to the brain and can be severe. Sudden and intense headaches may occur. Dizziness can also occur during attacks.
Weight Changes and Metabolic Symptoms
Weight loss occurs in 40-70% of patients. Unwanted and sudden weight loss develops due to excessive hormone production. However, some patients may also experience unexplained weight gain.
Nausea and vomiting occur in 26-43% of patients. Nausea can be persistent and may be accompanied by occasional vomiting. Abdominal or back pain, a feeling of fullness in the abdomen, and a sense of satiety after eating only a small amount are other symptoms.
Fatigue or tiredness is seen in 15-38% of patients. General fatigue and loss of energy occur. Muscle weakness and cramps may also arise. Shortness of breath and difficulty breathing are common symptoms.
Symptoms Related to Hormonal Imbalance
Anxiety and panic attacks occur in 35-40% of patients. Sudden anxiety and panic attacks can occur. Irritability is observed in a significant portion of patients.
In women, hormonal changes can cause facial hair growth, hair loss on the head, and irregular menstruation. In men, hormonal changes can lead to breast tissue growth and testicular shrinkage.
Red-pinkish-purple stretch marks on the skin, stretch marks on the abdomen, and fatty areas on the shoulders and back of the neck may be observed. Low potassium levels, high blood pressure, and the development of diabetes can occur. Fever, loss of appetite, and sexual problems are other symptoms.
Certain activities or conditions can worsen symptoms. Physical exertion, anxiety or stress, and changes in body position are among these situations. Foods high in tyramine can also trigger symptoms. Cheese, beer and wine, chocolate, dried or smoked meats are tyramine-containing foods.
How is Adrenal Gland Tumor Diagnosed?
Using the correct diagnostic methods increases the success of treatment for adrenal gland tumors. The diagnostic process requires a multidisciplinary approach and involves a comprehensive evaluation in collaboration with an endocrinologist, radiologist, and oncologist. In this process, your symptoms, physical examination findings, and family history are thoroughly examined to determine the most appropriate diagnostic methods.
Physical Examination and Anamnesis
The diagnostic process begins with a physical examination by a specialist physician. Your doctor reviews your medical history and listens to your patient history in detail. At this stage, information about whether family members have experienced hormonal imbalances is also important. Genetic predisposition is investigated.
During the physical examination, symptoms such as high blood pressure, excessive sweating, headaches, and palpitations are evaluated. Your doctor will ask when the symptoms started, their frequency, and severity. Your risk factors and family history are examined in detail. Based on this information, if there is suspicion of cancer, further tests will be decided.
Blood and Urine Tests
Catecholamine hormones secreted by the adrenal glands are measured through blood and urine tests. Levels of adrenaline, noradrenaline, and dopamine are determined through these tests. In patients with pheochromocytoma, hormone levels are high and irregular.
A 24-hour urine sample is taken to check the levels of metanephrine and normetanephrine. Metanephrines are breakdown products of catecholamines and are reliable markers since they are produced within the tumor. The determination of VMA and metanephrine in 24-hour urine has 97% sensitivity and 91% specificity. An abnormal increase in metanephrines in urine indicates pheochromocytoma.
Plasma free metanephrine or 24-hour fractionated urine metanephrines are measured. In some cases, an additional marker, 3-methoxytyramine, is also evaluated. Levels of hormones produced by the adrenal glands, such as cortisol, aldosterone, and androgens, are detected. In cases where plasma tests cannot be performed, metanephrines can be checked in 24-hour urine.
Imaging Methods
Imaging methods such as CT and MRI help determine the location and size of the tumor. Computed tomography is the most commonly used imaging method for detecting and evaluating adrenal lesions. A non-contrast CT can diagnose cysts, myelolipomas, and bleeding. With modern imaging technologies, it can be determined with over 90% accuracy whether adrenal tumors are benign or malignant.
Magnetic resonance imaging offers superior imaging quality in terms of soft tissue contrast. MRI is often preferred in children and pregnant women to avoid radiation exposure. The chemical shift imaging technique plays an important role in identifying fat-rich adenomas.
MIBG scintigraphy has high accuracy in diagnosing pheochromocytoma. The sensitivity of I-123 MIBG scintigraphy in diagnosing pheochromocytoma is reported to be 94%, with a specificity of 92%. This test measures the uptake of tumor cells by injecting radioactive material.
Positron emission tomography plays a role in detecting metastatic disease by evaluating metabolic activity. The detection rate of Ga-68 DOTA-SST PET is 93%, which is higher than other methods. PET-CT is very useful in differentiating between benign and malignant lesions.
Biopsy and Advanced Tests
In necessary cases, a sample is taken from the tumor tissue for histopathological examination. The biopsy procedure is performed to determine whether the mass carries a risk of cancer. It provides information about the content of the tumor. Biopsy is usually preferred as a last resort.
Pathology includes the examination of the tumor under a microscope. Special tests such as immunohistochemistry are performed. In cases of suspected familial transmission, genetic analysis may be conducted. In selected cases, molecular or genetic tests guide long-term follow-up and family screening.
How is Adrenal Gland Tumor Treated?
Your treatment approach will depend on the type, size, and whether the tumor produces hormones. The primary goal in treating adrenal gland tumors is to remove the tumor from your body and restore hormonal balance to normal. In most cases, tumors are benign and can be successfully treated with surgical methods.
Surgical Treatment Methods
Surgical intervention is the most effective method for adrenal gland tumor surgery. In this operation, called adrenalectomy, the tumor-bearing gland is removed. The laparoscopic method is performed with small incisions, leading to a faster recovery time. Today, laparoscopic adrenalectomy has begun to replace open surgery as the standard treatment method for many adrenal masses.
The size of the tumor determines the surgical approach. Laparoscopic methods are preferred for small and localized tumors. Open surgery is planned for large or suspicious malignant cases. If no complications develop after surgery, patients’ blood pressures improve. Surgical treatment is the most effective method for hormone-producing masses.
Medication Treatment and Hormonal Regulation
It is vital to control your blood pressure before surgery. Alpha blockers dilate blood vessels and lower blood pressure and are used for at least 10-14 days. Phenoxybenzamine is the most commonly used long-acting alpha blocker. Doxazosin may be preferred due to its better side effect profile.
Beta blockers are added after alpha blockade is achieved and control your heart rate. They are never given alone because a crisis can be triggered. Calcium channel blockers can also be used in preoperative preparation. These medications significantly reduce the risk of a hormonal storm during surgery.
Chemotherapy and Radiotherapy
In malignant pheochromocytomas, a combination of chemotherapy and radiotherapy is applied after surgery. Radiotherapy is used to eliminate remaining cells after surgery or to reduce pain. Chemotherapy is applied as systemic drug treatment in advanced cases.
In metastatic or inoperable patients, radiotherapy is preferred for symptom control. MIBG-based treatments can be applied in selected cases. Targeted therapy is performed with drugs specifically developed for cancer cells.
Post-Treatment Follow-Up Process
In the first 24-48 hours after surgery, your blood pressure and heart rate are closely monitored. Fluid support and medication adjustments are made as needed. Urine catecholamine tests are performed to determine whether the entire tumor has been removed after surgery.
Regular check-ups improve your quality of life and help you return to your previous state. Blood pressure and hormonal balance are monitored regularly. Biochemical tests and imaging continue to assess for recurrence. With timely diagnosis and appropriate surgery, most symptoms improve.
Frequently Asked Questions
Is adrenal gland tumor cancer?
Pheochromocytoma is generally a benign tumor, but 10% of cases exhibit malignant characteristics. Most cases are not cancerous and can be completely cured with appropriate treatment. However, in some cases, the tumor may exhibit cancerous behavior or spread to other organs. The diagnosis of malignancy is usually made when metastasis is detected in tissues that do not normally contain chromaffin cells. Approximately 80-85% of masses detected in the adrenal gland are benign.
What is the recovery process like after surgery?
Most patients feel well within a few days after surgery. Patients are monitored in a normal ward after laparoscopic adrenalectomy and can be discharged within a day or two. However, it takes some time for your body to fully recover and heal. Patients with hormone-producing tumors may need to take cortisol supplements after surgery. Extra treatment should continue until the remaining adrenal tissue shows normal function and produces adequate amounts of hormone. You can perform daily activities at home and take a shower the day after discharge. After a week, you can drive and work on light tasks.
Is there a risk of tumor recurrence?
Tumor recurrence can be observed in the same area after treatment. Therefore, follow-up and monitoring should continue to assess the possibility of tumor recurrence after treatment. There is a risk of recurrence, and regular biochemical monitoring and imaging at appropriate intervals are recommended. Even in cases where postoperative biochemical cure is observed, close follow-up is required due to the risk of malignancy. It should be noted that hypertension may continue for 4-8 weeks in some patients after successful surgery.
In which age group is it most commonly seen?
Adrenal gland tumors can occur at any age, but patients are usually diagnosed between the ages of 30 and 50. Pheochromocytomas are most commonly discovered in people aged 20 to 50. They are most frequently seen between the ages of 30 and 50, particularly affecting children under 5 and individuals in their 40s and 50s. The disease can occur at different ages. Tumors occur equally in both sexes in adults.
Does it show genetic transmission?
Pheochromocytoma can occur due to high genetic predisposition. Genetic testing is recommended for every patient diagnosed with pheochromocytoma. Familial cases are seen in approximately 30% of cases. Individuals with certain genetic syndromes have an increased risk of pheochromocytoma. It is also associated with MEN Type 2, Von Hippel-Lindau syndrome, Neurofibromatosis Type 1, and SDHB-SDHD gene mutations. If a family member has an adrenal gland tumor, it is recommended that other family members also undergo genetic testing. If a mutation is detected, first-degree relatives should also undergo germline mutation screening.
What happens if left untreated?
Untreated adrenal gland tumors can continue to grow and exert pressure on other organs. This pressure affects the functioning of the organs and can lead to other symptoms. If left untreated, serious complications such as hypertensive crisis, heart attack, brain hemorrhage, cardiac arrhythmias, kidney damage, and acute pulmonary edema can occur. High blood pressure can damage the cardiovascular system tissues, brain, and kidneys. The disease can turn into life-threatening situations and lead to organ failure. If hormone secretion increases during surgery or childbirth, sudden spikes in blood pressure can be fatal.
In Summary: Adrenal Gland (Pheochromocytoma) Tumor
Adrenal gland tumors may seem complex at first glance, but successful results can be achieved with the right diagnosis and treatment. As you have seen in this article, recognizing the symptoms allows you to consult a doctor in a timely manner. The surgical method offers a permanent solution for most patients and restores your quality of life.
If you are experiencing suspicious symptoms, it is important to consult a specialist immediately. Early diagnosis reduces the risk of complications and increases your treatment options. If you have regular check-ups after treatment, you can lead a healthy life. Remember that if there is a family history, undergoing genetic screening provides long-term protection.
What is an adrenal gland tumor, what are its symptoms, and how is it treated? Learn everything you need to know about pheochromocytoma in this comprehensive guide.
