Summary about Disease
Adrenocortical carcinoma (ACC) is a rare cancer that begins in the adrenal cortex, the outer layer of the adrenal glands. These glands, located on top of the kidneys, produce essential hormones that regulate metabolism, immune system, blood pressure, and response to stress. ACC can be functional, meaning it produces excess hormones, leading to various symptoms, or non-functional, producing no excess hormones. It is an aggressive cancer that can spread to other parts of the body.
Symptoms
Symptoms vary depending on whether the tumor is functional (hormone-producing) or non-functional.
Functional Tumors (Hormone-Producing):
Excess Cortisol (Cushing's Syndrome): Weight gain, round face, high blood pressure, high blood sugar, skin changes (easy bruising, purple stretch marks), muscle weakness, mood changes.
Excess Androgens (Male Hormones): In women: increased facial/body hair, deepened voice, menstrual irregularities, acne. In men: enlarged breast tissue, shrunken testicles. In children: early puberty signs.
Excess Estrogen (Female Hormones): In men: breast enlargement, sexual dysfunction. In women: May cause no noticeable symptoms or irregular periods.
Excess Aldosterone: High blood pressure, low potassium levels, muscle weakness, fatigue.
Non-Functional Tumors:
Abdominal pain or pressure
A palpable mass in the abdomen
Feeling of fullness
Weight loss
Causes
The exact cause of adrenocortical carcinoma is usually unknown. Genetic factors and certain inherited conditions can increase the risk. These include:
Li-Fraumeni syndrome: Caused by mutations in the TP53 gene.
Multiple endocrine neoplasia type 1 (MEN1): Caused by mutations in the MEN1 gene.
Beckwith-Wiedemann syndrome: A growth disorder.
Familial adenomatous polyposis (FAP): Caused by mutations in the APC gene.
Lynch Syndrome: Caused by mutations in DNA mismatch repair genes.
Medicine Used
Mitotane (Lysodren): This is the most commonly used medication for ACC. It's an adrenolytic agent, meaning it damages the adrenal cortex and can reduce hormone production and tumor size.
Chemotherapy: Chemotherapy regimens, often including drugs like cisplatin, etoposide, doxorubicin, and streptozocin, are used, especially in advanced or metastatic ACC.
Hormone-Blocking Medications: Medications to manage symptoms caused by excess hormones (e.g., drugs to control blood pressure, blood sugar, or estrogen levels).
Targeted therapies and Immunotherapies: being investigated and used in some cases.
Is Communicable
No, adrenocortical carcinoma is not communicable or contagious. It cannot be spread from person to person.
Precautions
There are no specific precautions to prevent ACC, as the causes are largely unknown and often related to genetic predispositions. However, individuals with known genetic syndromes that increase the risk of ACC should undergo regular screenings and follow their doctor's recommendations. Maintaining a healthy lifestyle and avoiding known carcinogens may be beneficial in general cancer prevention.
How long does an outbreak last?
ACC is not an outbreak. It is a tumor that develops over time. It can spread quickly once it is developed, but the time it takes to develop and spread is different for everyone.
How is it diagnosed?
Physical Exam and Medical History: Review of symptoms and risk factors.
Blood and Urine Tests: To measure hormone levels (cortisol, androgens, estrogens, aldosterone) and assess kidney and liver function.
Imaging Tests:
CT Scan: To visualize the adrenal glands and surrounding structures.
MRI: Provides detailed images of the adrenal glands.
PET Scan: To detect cancer spread.
Adrenal Biopsy: A small sample of tissue is removed from the adrenal gland and examined under a microscope. Note: Biopsies are often avoided due to the risk of spreading cancer cells.
Adrenal Vein Sampling: Catheters are inserted into adrenal veins to measure hormone levels directly.
Timeline of Symptoms
The timeline of symptoms varies widely depending on whether the tumor is functional or non-functional, the aggressiveness of the tumor, and the individual's overall health.
Early Stages: May be asymptomatic or have vague, non-specific symptoms. Functional tumors may initially cause subtle hormonal imbalances.
Progression: As the tumor grows, symptoms become more pronounced. Functional tumors will cause significant hormonal imbalances, leading to more obvious signs of Cushing's syndrome, virilization, feminization, or hypertension/hypokalemia. Non-functional tumors may cause abdominal pain or a palpable mass.
Advanced Stages: Symptoms related to cancer spread (metastasis) to other organs (e.g., lung, liver, bone) may develop.
Important Considerations
Rarity: ACC is a rare cancer, making diagnosis and treatment challenging. It's essential to seek care at specialized cancer centers with experience in treating ACC.
Aggressiveness: ACC can be aggressive, with a high risk of recurrence even after surgery.
Hormone Production: The hormonal effects of ACC can significantly impact quality of life. Managing these effects is a key part of treatment.
Multidisciplinary Approach: Treatment typically involves a team of specialists, including endocrinologists, surgeons, oncologists, and radiation oncologists.
Clinical Trials: Due to the rarity of ACC, participating in clinical trials may offer access to the latest treatments and research.