Overview

The adrenal gland cortex forms the outer section of the adrenal gland, which Adrenocortical Carcinoma (ACC) affects as a rare but aggressive form of cancer. The Adrenal glands are small, triangular-shaped glands situated on top of each kidney. These glands produce vital hormones that regulate blood pressure, metabolic functions, immune defense, stress response, and sexual maturation.

Doctors need to diagnose and treat Adrenocortical Carcinoma at its early stages because the disease progresses quickly, and it can spread throughout the body. The condition/disease can affect both adults and children, with two peak age groups: early childhood and middle adulthood (ages 40-50). The hormonal disruption that occurs in ACC leads to symptoms, developing from both tumor expansion and excessive hormone production.

What is Adrenocortical Carcinoma?

Adrenocortical Carcinoma is a cancer that begins in the adrenal cortex — the outer part/portion of the adrenal gland responsible for producing:

  • The primary stress hormone, cortisol
  • Aldosterone, which controls blood pressure and salt level maintenance
  • Androgens, which are male hormones
  • Estrogens, which are female hormones produced in limited quantities

Tumors that develop in this region exhibit two types of behavior:

  • A functioning tumor that produces excessive hormones
  • A non-functioning tumor that does not create any hormones

The symptoms of functioning tumors develop sooner because the body sees hormone imbalance as a visible condition. ACC demonstrates different characteristics from benign adrenal tumors known as adenomas, which occur more frequently and typically remain non-cancerous.

Symptoms & Causes

What Causes Adrenocortical Carcinoma?

The exact cause of ACC is not fully understood. Cancer develops when genetic mutations cause adrenal cells to grow uncontrollably. Certain inherited genetic conditions increase the risk of ACC:

  • Li-Fraumeni syndrome
  • Beckwith-Wiedemann syndrome
  • Lynch syndrome
  • Multiple endocrine neoplasia (MEN1)

Most cases, however, occur sporadically without a known inherited cause.

What are the Symptoms of ACC?

The symptoms show two different patterns based on which hormone the tumor produces in excess. The symptoms show two different patterns based on which hormone the tumor produces in excess.

Symptoms Due to Excess Cortisol (Cushing Syndrome)

  • The body rapidly gains weight, especially in the face and abdomen.
  • The body develops a round, moon-shaped facial appearance.
  • The body experiences elevated blood pressure.
  • The body experiences elevated blood sugar.
  • The person experiences decreased muscle strength.
  • The person experiences skin injuries with minimal force.
  • The body develops purple marks that appear like stretch marks.

Symptoms Due to Excess Androgens (More Common in Women)

  • The person develops excessive facial or body hair.
  • The woman experiences unpredictable menstrual cycles.
  • The woman develops a deeper voice.
  • The person develops acne.
  • The person experiences hair thinning.

Symptom: Excess Estrogen (More Common in Men)

  • The body develops breast tissue.
  • The man experiences diminished sexual desire.
  • The man experiences erectile dysfunction.
  • The body experiences elevated blood pressure.
  • The body experiences a potassium deficiency.
  • The person experiences muscle cramps.
  • The person experiences fatigue.

Non-functioning tumors cause symptoms that include abdominal pain, back pain, a feeling of fullness, unexplained weight loss, and a noticeable abdominal mass. Early medical evaluation is essential because symptoms may resemble those of other hormonal disorders.

Diagnosis & Tests

Doctors use laboratory tests and imaging studies to establish an ACC diagnosis. Medical History and Physical Examination: The doctor evaluates hormone-related symptoms, blood pressure changes, patterns of weight gain, and a family history of genetic syndromes.

Hormone Testing

Blood and urine tests measure hormone levels, including:

  • Cortisol
  • ACTH
  • Aldosterone
  • Renin
  • Androgens
  • Estrogen

These tests determine whether the tumor is functioning (hormone-producing). The tests identify the patient's health condition.

Imaging Tests

Imaging studies enable health professionals to track tumor location and assess its dimensions and distribution.

  • CT scan of the abdomen
  • MRI scan
  • PET scan (if metastasis is suspected)

Tumor size larger than 4–6 cm raises suspicion for malignancy.

Biopsy

Surgeons do not perform biopsy procedures to establish a diagnosis because they lead to potential tumor dispersal. The surgical removal of the tumor allows doctors to confirm a diagnosis.

Staging of Adrenocortical Carcinoma (ACC)

The system used to find ACC has three levels of classification. For finding ACC, the staging system has three levels of classification. There are three levels of classification in the staging system used to find ACC.

  • The complete adrenal gland contains all stages I to II tumors.
  • Stage III: Local spread to nearby tissues or lymph nodes
  • Stage IV: Distant metastasis (liver, lungs, & bones)

Management & Treatment

The treatment approach relies on the tumor's stage and hormonal activity, as well as the patient's overall health. The treatment approach relies on the tumor's stage and hormonal activity, as well as the patient's overall health.

Surgery

Surgery or operation is the primary treatment for patients with localized ACC.

  • The surgical procedure, adrenalectomy, involves the complete removal of the adrenal gland from the body.
  • The surgical procedure involves removing lymph nodes that show signs of cancerous involvement.
  • The complete removal of all tumors from the body presents the highest probability of achieving complete healing.

Adjuvant Therapy

Physicians will recommend extra treatment procedures that aim to decrease the chances of cancer returning after the surgical procedure.

Mitotane Therapy

Mitotane is a specialized medication that prevents adrenal cells from producing hormones. Physicians use this medication mainly after surgery.

Chemotherapy

Doctors use chemotherapy to treat patients with advanced-stage or metastatic ACC. The standard drug combinations which doctors use include:

  • Etoposide
  • Doxorubicin
  • Cisplatin

Doctors use chemotherapy together with the medication mitotane.

Radiation Therapy

Doctors use radiation therapy to treat patients who require:

  • Patients need radiation therapy after their surgical procedure to prevent the development of cancer at the surgical site.
  • Patients use radiation therapy to manage their symptoms, which occur during their disease progression.

Targeted Therapy & Clinical Trials

Researchers study ACC because it is an uncommon disease, enabling the discovery of new treatment approaches through targeted-therapy research programs. The medical team will recommend clinical trials to patients whose cancer has advanced.

Management of Hormone-Related Symptoms

Doctors must maintain control over hormone excess during treatment.

  • The medications decrease cortisol production.
  • Meds help maintain blood pressure within normal limits.
  • Potassium supplements help people who need extra potassium.
  • Diabetes patients need to control their blood sugar levels.

Endocrinologists specialize in the treatment of hormonal imbalances.

Side Effects of Treatment

The treatment process for ACC induces the following side effects:

  • Fatigue
  • Nausea and vomiting
  • Hormonal deficiencies

The treatment increases the likelihood of developing adrenal insufficiency.

Chemotherapy causes patients to lose their hair.

The treatment raises the possibility of patients developing infections.

Patients who undergo complete bilateral adrenal gland removal require hormone replacement for their entire lives.

Outlook / Prognosis

The medical team bases patient prognosis on these factors:

  • Stage at diagnosis
  • Complete surgical removal
  • Tumor size
  • Spread to other organs.

The medical team can achieve better patient outcomes in early-stage ACC by achieving complete tumor elimination. Patients with advanced-stage disease face a greater chance that their cancer will return.

Patients who survive cancer treatment need regular imaging tests and hormone assessments because their condition can come back even after several years.

Living With Adrenocortical Carcinoma

The oncology and endocrinology staff need to monitor patients with cancer long-term. The medical team provides follow-up care through:

  • Imaging tests
  • Hormone testing
  • Monitoring for recurrence

Patients who need hormone replacement will receive long-term management services.

Psychological support, along with rehabilitation services, provides patients with tools to help them deal with their post-surgery and cancer therapy recovery process.

Why choose Tender Palm Hospital for Adrenocortical Carcinoma (ACC) Treatment in Lucknow, India?

Tender Palm Super-Speciality Hospital offers advanced Adrenocortical Carcinoma (ACC) treatment in Lucknow, India, at an affordable cost. We have a team of experienced surgical oncologists and endocrinology specialists who provide accurate diagnosis and both non-surgical and surgical treatment options including adrenalectomy, chemotherapy, and targeted therapy procedures. Our Surgical Oncology and Endocrinology team has decades of experience in successfully treating Adrenocortical Carcinoma (ACC) in Lucknow, India.

To seek an Expert consultation for Adrenocortical Carcinoma (ACC) Treatment in Lucknow, India:

Call us at +91-9076972161
Email at care@tenderpalm.com

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