Very rare, cancer of the adrenal glands is potentially aggressive if discovered at an advanced stage. Among these cancers, adrenocortical and pheochromocytomas. Causes, symptoms, location and management with Pr Jérôme Bertherat, head of the endocrinology department at Cochin Hospital.
THE adrenal gland cancer is localized in the kidneys. Each of the two kidneys is effectively “capped” by an adrenal gland. These glands participate in the secretion of hormones. Cancer can affect one or both adrenals.
Various types of tumors can develop in the adrenal gland, some being benign (the most frequent), others malignant. Among the tmalignant tumorsso cancers, we distinguish:
- THE adrenocortical tumors that develop in the cortical area of the adrenal gland,
- and the malignant pheochromocytomas, in the medullary area. Nevertheless, pheochromocytomas are benign in 9 out of 10 cases.
The adrenal gland consists of two distinct areas:
→ The first is the adrenocortical area which secretes different hormones: glucocorticoids, hormones including cortisol which essentially affects glycemia (blood sugar level); THE mineralocorticoids represented by aldosterone which intervenes on the kidney to balance the amount of sodium and potassium in the body; and part of androgenshormones of the development of male sexual characteristics.
→ The other zone is the adrenal medulla which secretes the‘adrenaline and the norepinephrinewhich affect the rhythm of the heart, the contraction or dilation of blood vessels and increase blood pressure.
“Among the well-identified causes, there are: genetic causes that are common in pheochromocytomas where they represent 30 to 40% of cases. In adrenocortical tumors, genetic causes (in the hereditary sense of the term) are found mainly in children, much more rarely in adults (less than 5%). Of the molecular abnormalities limited to tumor cells can also be identified in adults”, says Professor Jérôme Bertherat.
Symptoms of adrenal cancer will therefore depend on the area affected. In case of adrenocortical tumorthe signs will be represented by:
- THE Cushing’s syndrome (which can have multiple causes but rarely cancer of the adrenal gland) related to an increase in the secretion of cortisol, with weight gain or even obesity android predominant on the abdomen, arterial hypertension, appearance of stretch marks on the abdomen, loss of muscle mass…
- A diabetes.
- I’hyperandrogenismby excess of androgens resulting in an increase in hair growth, and masculinization in women.
- I’hypokalemia (low potassium) which may cause the patient to drink a lot of water or to have muscle or heart problems.
In case of malignant pheochromocytomathe symptoms will be in the typical form: sudden discomfort which frequently associate headaches, headaches, palpitations, sweats and above all very severe sudden onsets of arterial hypertension. “5% of people have a lump in or near the adrenal gland. These cancers can be discovered by exploring symptoms but also simply by chance, on a medical imaging examination for another disease: this is what the it is called an incidentaloma”, comments the endocrinologist.
- Stage 1 and 2: the cancer is localized to the adrenal gland.
- Stage 3: regional invasion.
- Stage 4: metastases.
“There are two classification systems between adrenocortical tumors and pheochromocytomas. The initial stage is localized purely in the adrenal. If the disease is diagnosed at this stage and completely operated on, a cure can be achieved. At stage 3, the invasion is either regional or there are distant metastases, depending on the tumours. For adrenocortical tumors, it is above all the liver and the lungs that are affected, while in pheochromocytomas, it is more often the bone and possibly subsequently the liver and the lung. details the specialist. “For pheochromocytoma, the diagnosis of malignancy is sometimes difficult to make as long as there are no metastases”, observes Professor Jérôme Bertherat.
Faced with signs suggestive of an adrenal tumour, a blood test with dosage of the different hormones is necessary to highlight the excess secretion. Subsequently, imaging of the adrenals, usually a to scan, often associated with an MRI, allows visualization of the tumour. A scintigraphy is also conceivable. If a cancerous tumor is suspected, a extension assessment will be performed to look for the migration of cancer cells to other organswhich, in the presence of metastases, will signify the cancerous character of the tumour.
As for all cancerous tumours, the treatment will be adapted according to the evolution of the tumour, its size, the general condition of the patient and the results of the extension assessment. “THE first-line treatment when the tumor is not too metastatic is the surgeryby a team specializing in tumors of the adrenal gland”, says the endocrinologist. It consists of removing the entire tumour, the surrounding tissues likely to be carrying cancerous cells and the nearby lymph nodes if they are affected by cancer. “If the tumor recurs or there are distant metastases that we cannot operate on, Medical treatments can be discussed on a case-by-case basis: mitotane for adrenal cortex and targeted therapies (temodal, kinase inhibitors) for malignant pheochromocytoma. You should know that we have very little use of cytotoxic chemotherapy in these tumours. It is reserved for failures or very aggressive forms because it works less well than in other cancers”, he continues. Radiation therapy is less common in this setting. The control of hormonal hypersecretion and its symptoms is also part of the treatment.
Life expectancy varies depending on whether it is an adrenocortical tumor or a malignant pheochromocytoma.
→ Adrenocortical tumor : “If we obtain a complete excision, the life expectancy at 5 years is more than 80%. On the other hand, in the metastatic stages, the life expectancy does not exceed 30% at 5 years“, explains Professor Jérôme Bertherat.
→ Pheochromocytoma : the cancer of this endocrine gland has the particularity of developing very slowly. The life expectancy is therefore greater than 80% at 5 years. “However, this does not mean that the cure is proven, patients can be metastatic and sometimes progress over more than 15 to 20 years”nuance the endocrinologist.
Thank you to Pr Jérôme Bertherat, head of the endocrinology department at Cochin Hospital and coordinator of the national reference network for rare adrenal cancers ENDOCAN-COMETE.