ACC or Adrenocortical Carcinoma

Adrenocortical Carcinoma (ACC) is a rare and aggressive cancer of the adrenal glands. It forms in the adrenal cortex and is classified as functional ACC and non-functional ACC. The former is characterized by increased hormone production, while the latter does not affect hormone production. A rapid surge in cortisol, aldosterone, testosterone or estrogen levels is usually seen in functional ACC. This carcinoma highly metastasizes and can occur in both children and adults. Although it is more common in females, the presence of a combination of risk factors predisposes everyone to develop ACC.

Causes

While the exact cause of ACC is yet to be identified, several factors contributing to its development have been discovered. Genetic mutations in the DNA of the adrenal gland cell may lead to ACC. In such cases, the cells are signaled to multiply uncontrollably, accumulate, and form a tumor.

The risk factors for getting ACC are:

• · Inherited syndromes like Beckwith-Wiedemann syndrome, Carney complex, Li-Fraumeni syndrome, Lynch syndrome, Multiple endocrine neoplasias (MEN-1), and
• · Family history of ACC.

ACC Symptoms

ACC presents different symptoms in adults and children. Functional ACC is mainly seen in children. Virilization, or the development of adult male characteristics in young males and females, is a prominent sign. The other signs and symptoms of ACC are:

Non-functional adrenocortical tumor doesn’t cause any signs in the early stages. Abdominal or flank pain, varicocele, and renal vein thrombosis are distinctive signs of this type of tumor.

Screening tests

The healthcare provider will first perform a physical exam and question the symptoms experienced by the patients. Following this, other screening tests for the confirmation of ACC are performed. These include:

Ø Blood chemistry tests: The amounts of certain substances like potassium or sodium released into the blood by the organs are measured by analyzing the patient’s blood sample. An abnormally high level indicates the possibility of an adrenal tumor.

Ø Twenty-four-hour urine test: In this test, the urine is collected for 24 hours to measure the amounts of cortisol or17-ketosteroids. An abnormally high amount of this hormone in the urine ascertains the malfunctioning of the adrenal gland.

Ø Imaging tests: Radiological studies of the abdomen, like CT scans and MRI scans, pinpoint the site of the tumor and determine the extent of tumor metastasis.

Ø Biopsy: In a biopsy, a section of the tumor cells or tissues is removed by a fine needle and observed under a microscope by a pathologist. This is done to confirm the presence of ACC and distinguish it from other small cancers.

ACC Treatment

Complete surgical excision or tumor removal is the only curative treatment for adrenocortical carcinoma. Although the 5-year survival rate of

this procedure is about 50-60%, it is not feasible for all patients owing to age and other health conditions. In such cases, other treatment options used for palliation are:

· Chemotherapy – Drugs are administered to the patients to facilitate the death of the tumor cells or to stop them from dividing. The two most common chemotherapeutic regimens are –

• Cisplatin, doxorubicin, etoposide + mitotane
• Streptozocin + mitotane

· Radiation therapy – High-powered beams such as x-rays or protons kill the cancer cells or prevent their metastasis. The two types of radiation therapy are internal radiation therapy and external radiation therapy. A radioactive substance is injected near the cancer site in internal radiation therapy. At the same time, in the latter case, a machine sends radiation toward the tumor area in the body.