Okay, so are you experiencing sudden hair growth? Is your voice getting deeper? Is your face starting to look like the moon? If you are having these symptoms and you are not hitting puberty, then you should give this blog a read.
Around two people per million suffer from this rare disease called Adrenocortical Carcinoma. It is because this tiny gland can get afflicted by adrenocortical carcinoma - which roughly translates into cancer of the adrenal glands.
But before we talk about adrenocortical carcinoma, let’s first talk about the adrenal glands. As humans, we are blessed with or cursed with, depending on how you see it, two adrenal glands. These glands aren’t too big and roughly triangular-shaped, with one adrenal gland atop each kidney.
This tiny little gland has two parts. The outer layer of the adrenal gland is known as the adrenal cortex, and the centre of the adrenal gland is known as the adrenal medulla.
When a tumour forms in the adrenal cortex, you might classify it as functioning (makes more hormones than usual) or nonfunctional (does not make more hormones than usual).
The majority of adrenocortical tumours are functioning, which means they continue to make hormones despite being afflicted with cancer. That’s just like us; they keep going even when life seems bleak and worthless.
The adrenal gland has so many responsibilities you’d think it was your average Indian male.
It balances out the water and salt in the body.
Make sure that blood pressure is normal.
Assisting how our body uses protein, fat and carbohydrates.
It is in charge of making sure whether we have masculine or feminine characteristics.
Adrenocortical carcinoma happens to both adults and children. Treatment for children is different from treatment for adults.
Adrenocortical carcinoma is quite rare, unlike cervical cancer, and we don’t see too many instances of this disease in India. In this condition, your adrenal glands develop tumours that release a number of hormones.
There are two types of adrenaline carcinoma: functioning and non-functioning. These causes varied symptoms.
Functioning tumours -
The majority of adrenocortical carcinomas are functioning. The symptoms of releasing a litany of hormones like cortisol, aldosterone, testosterone, and estrogen vary depending on the type of hormone.
Non-functioning tumours -
Such tumours don’t impact hormone protection. However, they can become so big that they strain nearby organs and tissues.
Symptoms are often late in appearing. According to studies, around 20% to 30% of adrenocortical carcinoma diagnoses occur because healthcare providers carry out imaging tests for unrelated health issues. When symptoms do develop, however, they include the following -
Abdominal or belly pain -
This is quite common in patients suffering from adrenocortical carcinoma (ACC). It happens because the growing tumour pressures the surrounding organs like the liver, intestines and kidney. This might be a persistent, sharp, dull pain in the upper abdomen accompanied by bloating or fullness.
Hirsutism (excess body or facial hair in women) -
Hirsutism is characterised by excess hair growth in areas where men typically grow hair, like the back, face and chest. Women having ACC can have a tumour that produces too much androgen, resulting in dark, coarse facial or body hair.
Gynecomastia -
This is when the breast tissue in men gets enlarged, caused by hormonal imbalances when ACC occurs. Adrenal gland tumours might produce excessive amounts of estrogen or alter the balance of sex hormones, leading to a noticeable enlargement of the breast in men.
High blood pressure -
This is another symptom of adrenocortical carcinoma, occurring because of the overproduction of cortisol or aldosterone hormones, which spoil the body’s ability to regulate blood pressure, resulting in constant hypertension.
High blood pressure can strain the heart and blood vessels, bringing a fair share of risks.
High blood sugar -
Adrenocortical carcinoma can result in hyperglycemia because of excess cortisol production, which raises blood sugar levels by stimulating glucose prediction in the liver. Over time, this might lead to diabetic symptoms. Unchecked, it can cause nerve or kidney damage.
Weight gain in the face, neck and trunk -
This is a common symptom of adrenocortical carcinoma, which happens because of the overproduction of cortisol, resulting in Cushing’s syndrome. This can cause your face to look like the moon - not particularly great for your self-esteem.
What causes adrenocortical carcinoma? Your guess is as good as ours. Researchers, too, don’t know the exact cause. Some people develop this disease because they have inherited conditions that enhance their risk of developing it. In other cases, particular genetic mutations increase risk.
For instance, research shows that tumour suppressor genes TP53 and IGF2 drive adrenocortical carcinoma. Tumour suppressor genes take care of cell growth. When such genes change, cells might multiply uncontrollably and become cancerous tumours.
What Inherited Conditions Increase the Risk of Developing Adrenocortical Carcinoma?
Having any of the below conditions boosts your chances of developing adrenocortical carcinoma -
Beckwith-Wiedemann syndrome
Beckwith-Wiedemann Syndrome, or BWS, refers to a genetic disorder that impacts the growth of children, increasing their risk of developing certain childhood cancers. This is a relatively rare disorder. People who have BWS have no noticeable physical differences.
Carney complex
This condition creates changes in your skin’s colour. It also results in noncancerous (benign) tumours in the body’s connective tissue and endocrine glands. A change in a gene that parents pass to their offspring is the impetus behind this condition.
Familial adenomatous polyposis (FAP)
This is a genetic disorder that makes you develop precancerous colon polyps named adenomas. Colon polyps refer to abnormal growths in the lining of the colon or rectum. They aren’t cancer, but certain types can develop into colorectal cancer.
Li-Fraumeni syndrome
This syndrome is a rare genetic disorder that increases the risk of you and your family members developing cancer. Everyone who has this condition has a 90% chance of developing one or more types of cancer by the time they turn 60.
Lynch syndrome
This genetic condition increases your risk of developing cancer. People who have this syndrome are more likely to get cancer before they turn 50. It can affect anyone because it is the result of genetic mutation.
Multiple endocrine neoplasia (MEN1)
Multiple endocrine neoplasia (MEN) is a rare condition caused by a genetic mutation that impacts different glands in your endocrine system. There are two types of MEN, with symptoms varying from person to person.
Neurofibromatosis Type 1 (NF1)
Neurofibromatosis type 1 (NF1) is the most common type of neurofibromatosis. It impacts the skin and nervous system. Symptoms include café au lait spots and tumours in the skin and nerves.
Von Hippel-Lindau (VHL) syndrome
People who have this disease have an increased risk of developing cancerous and noncancerous tumours, which can grow throughout their bodies. It is a rare genetic disorder that occurs if you inherit a particular genetic mutation.
First, tests for other issues are carried out. If these tests reveal a tumour on the adrenal gland or you exhibit specific symptoms, your healthcare provider will carry out the following -
Imaging tests like MRI, CT scan or PET scan can be used to locate any tumours if present.
Blood tests and urinalysis to gauge hormone levels.
Your doctor might perform a biopsy to confirm whether a tumour is cancerous or to obtain specific tissue samples.
So, You’ve Been Diagnosed With Adrenocortical Cancer. What Is the Way Forward?
The most well-known treatment for this cancer is adrenalectomy, to take out one or both of the adrenal glands. Additional medication, too, may be prescribed. Such medications include:-
Mitotane (Lysodren)
This drug curtails adrenal gland activity, which might bring down the risk of a remission. Doctors use this medication post-surgery to remove one adrenal gland or if surgery is not an option. This medication shuts down hormone production, so people who take it also take hormone replacement medication.
Metyrapone (Metopirone)
Your doctor might prescribe similar drugs to reduce symptoms when tumours produce excess hormones.
In some instances, however, adrenocortical cancer is not diagnosed till after the tumours have become too big to be safely taken out with surgery. In such cases, doctors might resort to chemotherapy. This won’t get rid of the cancerous tumours. However, it can ease symptoms and bring down tumour growth.
Tough luck - you can’t prevent this disease from happening. According to researchers, half of all cases of this cancer occur when particular genes mutate and create cancerous cells that multiply and become tumours. However, they don’t know what triggers such mutations, so they can’t tell you ways to prevent this from happening.
That being said, certain inherited disorders increase the risk of developing this condition. If you have a family history of such disorders, speak to a healthcare provider regarding genetic tests to detect mutations that cause this cancer.
In conclusion, if you have been diagnosed with adrenocortical cancer, there’s nothing to fear. Speak to your healthcare provider for a proper diagnosis and treatment plan. Adrenocortical cancer, or cancer of the adrenal glands, is a rare and challenging condition.
Understanding the symptoms, causes and treatment options is crucial for managing this disease. Early diagnosis through imaging and blood tests is vital in improving outcomes. Treatment options like surgery, medication and chemotherapy can help curtail this disease.
While there is no way you can prevent this cancer, staying informed and working in conjunction with your healthcare provider can make a massive difference to your treatment journey. If you get wind of any unusual symptoms, don’t hesitate to ask for medical advice. This cancer can be managed, and you can go on to lead a fulfilling life!
What is Adrenocortical Carcinoma?
Adrenocortical carcinoma (ACC) is a rare cancer that develops in the adrenal glands, which are located on top of your kidneys.
What are the symptoms of Adrenocortical Carcinoma?
Symptoms of ACC can include abdominal pain, excess body or facial hair in women (hirsutism), gynecomastia (enlarged breast tissue in men), high blood pressure, high blood sugar, and weight gain, especially in the face, neck, and trunk.
How is Adrenocortical Carcinoma diagnosed?
Diagnosis typically involves imaging tests like MRI, CT, or PET scans to identify tumours.
What causes Adrenocortical Carcinoma?
The exact cause is poorly understood, but certain genetic mutations and inherited conditions, such as Beckwith-Wiedemann syndrome or Li-Fraumeni syndrome, can increase the risk of developing ACC.
What are the treatment options for Adrenocortical Carcinoma?
Treatment often involves surgery to remove the adrenal gland(s) affected by the tumour. Medications like mitotane or metyrapone may be prescribed to reduce hormone production or manage symptoms.
