Adrenocortical carcinoma

What it is

From: Orphanet

A rare malignant adrenal disease characterized by a tumor that arises from the adrenal cortex. The clinical presentation depends on the hormones secreted; in children virilization is a predominant feature; other manifestations can include symptoms of Cushing syndrome or symptoms related to tumor volume or metastases.

Reported clinical features and what the terms mean

The following findings are associated with this condition in Orphanet. They are not a checklist for diagnosing yourself, and they do not all occur in every affected person. Some are examination, imaging or laboratory findings that cannot be recognised at home.

The frequency labels describe how often a finding was reported among people with the condition in the source. They do not give the chance that a person with that symptom has the condition. Definitions below reproduce HPO terminology; they explain the term, not the likely severity in an individual.

Adrenocortical carcinoma · Obligate (100%)

A malignant neoplasm of the adrenal cortex that may produce hormones such as cortisol, aldosterone, estrogen, or testosterone.

Abdominal pain · Frequent (79-30%)

An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) and perceived to originate in the abdomen.

Abnormal serum dehydroepiandrosterone level · Frequent (79-30%)

A deviation from the normal concentration of dehydroepiandrosterone in the circulation.

Abnormality of reproductive system physiology · Frequent (79-30%)

An abnormal functionality of the genital system.

Adrenocorticotropic hormone deficiency · Frequent (79-30%)

A reduced ability to secrete adrenocorticotropic hormone (ACTH), a hormone that stimulates the adrenal cortex to secrete of glucocorticoids such as cortisol.

Anxiety · Frequent (79-30%)

Intense feelings of nervousness, tension, or panic often arise in response to interpersonal stresses. There is worry about the negative effects of past unpleasant experiences and future negative possibilities. Individuals may feel fearful, apprehensive, or threatened by uncertainty, and they may also have fears of falling apart or losing control.

Diabetes mellitus · Frequent (79-30%)

A group of abnormalities characterized by hyperglycemia and glucose intolerance.

Elevated serum 11-deoxycortisol · Frequent (79-30%)

Increased concentration of 11-deoxycortisol in the circulation. 11-deoxycorticosterone, which is also known as simply deoxycorticosterone and 21-hydroxyprogesterone, is a steroid hormore that is produces in the adrenals and is a precursor to aldosterone.

Hyperhidrosis · Frequent (79-30%)

Abnormal excessive perspiration (sweating) despite the lack of appropriate stimuli like hot and humid weather.

Hypertension · Frequent (79-30%)

The presence of chronic increased pressure in the systemic arterial system.

Hypertrichosis · Frequent (79-30%)

Hypertrichosis is increased hair growth that is abnormal in quantity or location.

Hypokalemia · Frequent (79-30%)

The concentration of potassium(1+) in the blood circulation is below the lower limit of normal.

Increased body weight · Frequent (79-30%)

Abnormally increased body weight.

Increased circulating aldosterone concentration · Frequent (79-30%)

Overproduction of the mineralocorticoid aldosterone by the adrenal cortex.

Other findings in the same source

From: Orphanet

Additional reported features include Increased circulating androgen concentration (Frequent (79-30%)); Increased circulating androstenedione concentration (Frequent (79-30%)); Increased circulating cortisol level (Frequent (79-30%)); Increased serum estradiol (Frequent (79-30%)); Increased urinary cortisol level (Frequent (79-30%)); Irritability (Frequent (79-30%)); Muscle weakness (Frequent (79-30%)); Palpitations (Frequent (79-30%)); Panic attack (Frequent (79-30%)); Striae distensae (Frequent (79-30%)). This is a selected summary, not a complete description of the condition.

When it may begin

From: Orphanet

Childhood

Inheritance in the source

From: Orphanet

Not applicable

Frequency and the population described

From: Orphanet

Point prevalence: 1-9 / 1 000 000; Europe; Value and class. Annual incidence: <1 / 1 000 000; Portugal; Value and class. Annual incidence: 1-9 / 1 000 000; Austria; Value and class. Annual incidence: 1-9 / 1 000 000; Belgium; Value and class.

Which doctor should you see?

The suggested department for discussing Adrenocortical carcinoma is Oncology, with a oncologist and relevant organ specialist as the relevant type of clinician. General physician / Family Medicine; paediatrician for children. Referral depends on symptoms.

Additional services that may be relevant, depending on the findings, include: Clinical Genetics; Relevant organ specialist / Surgical Oncology as indicated.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• Has the exact tumour type been confirmed, and is staging relevant?
• What is the goal of each proposed treatment option?
• How will side effects, daily function and supportive care be addressed?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for Adrenocortical carcinoma. That gap does not mean that treatment is unavailable. A clinician needs to establish the diagnosis and review current guidance before recommending medicines, procedures, rehabilitation or other support.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

The collected references do not establish a complete prevention or long-term outlook section for this entry. Missing information should not be interpreted as proof that prevention is impossible or that a particular outcome is inevitable. Ask what is known for the exact subtype, stage and personal circumstances, and which uncertainties remain.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.