Adrenocortical Carcinoma

Published Date : Nov 2023
Category : Oncology
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"Unmasking the Threat: Adrenocortical Carcinoma Advances Lighten Path to Improved Diagnosis and Cure"

Primary adrenal cortical carcinoma, sometimes referred to as adrenocortical carcinoma (ACC), is an extremely rare and dangerous tumor. It exists as an active or dormant hormonal tumor.

As per the National Institutes of Health (NIH), benign non-functioning adrenocortical adenomas (ACAs), which are tiny tumors that afflict between 3% and 10% of the human population, usually cause no symptoms. By contrast, ACC is a very uncommon illness. An estimated 300 Americans are diagnosed with adrenocortical cancer per year in the United States.1. This type of cancer is frequently successfully treated if found early. However, advanced adrenocortical cancer is identified in about 70% of cases.

Based on the Surveillance, Epidemiology, and End Results (SEER) database, an estimate of the incidence is roughly 0.72 cases per million cases per year, or 0.2% of all cancer deaths in the US. Whereas the projected global incidence is between 0.2 and 0.3 per million children annually, in Southern Brazil the incidence throughout childhood is between 2.9 and 4.2 per million annually.

According to their morphological characteristics, the WHO has classified adrenal cortical carcinomas into four subtypes: Conventional, Oncocytic, Myxoid, and Sarcomatoid.

There are four stages of Adrenocortical Carcinoma staging.

Stage I: A tumour in stage I is limited to the adrenal gland and measures no more than five centimeters.

Stage II: Only the adrenal gland is affected and the tumour is more than five centimeters in size in this stage.

Stage III: A tumour of any size has progressed to adjacent lymph nodes, big blood arteries (such as the vena cava or renal vein), or adjacent tissues or organs (such as the liver, spleen, pancreas, kidney, or diaphragm).

Stage IV: A tumour of any size that has progressed to neighboring lymph nodes and other body regions like the lung, bone, or peritoneum is considered to be at stage IV.

It is uncertain what specifically causes malignant adrenal tumors. An increased risk of the disease exists in those who are born with specific genetic abnormalities.

Additionally, gene mutations (changes) happen on their own and are not inherited. The exact mechanism of the genetic alterations leading to adrenocortical cancer remains unclear to experts.


There are two ways that adrenal tumours induce symptoms, when an adrenal tumour becomes large enough, it impinges on surrounding organs and create symptoms associated with hormone excess. Pain or other symptoms result from this pressure.

Adrenocortical carcinoma symptoms vary depending on the underlying cause and include high blood pressure, high blood sugar, hypertension, weight gain in the face, neck, and trunk (but not the arms and legs), early puberty in boys and girls, breast enlargement in males or girls (gynecomastia), and excessive facial or body hair growth in women. Excessive production of one of Cortisol, Aldosterone, Testosterone, and Estrogen is a symptom of ACC. 

Diagnostic Analysis of ACC

The patient's indications and symptoms determine the tests and procedures performed to diagnose adrenocortical cancer. The following tests and techniques are employed:

Physical examination and medical history

 A review of the body to look for overall health indicators, such as lumps or anything else that seems out of the ordinary. The patient's medical history, including any previous illnesses and treatments, is also be recorded.

Blood Test

  • A Low-Dose Dexamethasone Suppression Test: It involves administering one or more modest doses of the drug. Urine or blood samples that are obtained over the course of three days are used to measure cortisol levels. To see if the adrenal gland is producing excessive amounts of cortisol, this test is performed.
  • A high-dose dexamethasone suppression test: It involves administering one or more high doses of the drug. Urine or blood samples that are obtained over the course of three days are used to measure cortisol levels. This test is performed to determine whether an excessive amount of cortisol is being produced by the adrenal glands or whether the pituitary gland is inducing an excessive amount of cortisol in the adrenal glands.

Imaging Tests

  • CT scan (CAT scan): An imaging technique that produces a number of finely detailed images of inside body parts from various viewpoints. The images are produced by connecting an x-ray machine to a computer. A dye is ingested or injected into a vein to improve the visibility of the tissues or organs. Computed tomography, computerised tomography, and computerised axial tomography are other names for this process.
  • Magnetic resonance imaging (MRI): A process that creates a number of finely detailed images of various body parts using radio waves, a magnet, and a computer. Nuclear magnetic resonance imaging is another name for this process (NMRI). Adrenocortical cancer is diagnosed via abdominal magnetic resonance imaging (MRI).
  • MIBG scan: The bloodstream is used to carry a very little quantity of radioactive material known as MIBG, which is injected into a vein. The radioactive substance is taken up by adrenal gland cells, which are then identified by a radiation detector. The purpose of this scan is to distinguish between pheochromocytoma and adrenocortical cancer.

Adrenal Angiography:

 A technique used to examine the blood flow and arteries around the adrenal glands. The adrenal arteries are given an injection of contrast dye. A series of x-rays are taken to see whether any arteries are obstructed as the dye passes through the arteries.

Adrenal venography:

An examination of the adrenal veins and the blood flow around the adrenal glands is known as adrenal venography. An adrenal vein is injected with a contrast dye injection. A series of X-rays are taken to see whether any veins are obstructed while the contrast dye passes through the veins. A very tiny tube called a catheter is placed into the vein to draw blood, which is then examined for anomalous hormone levels.


The process of removing cells or tissues so a pathologist examines them under a microscope and look for indications of cancer. A narrow needle known as a fine-needle aspiration (FNA) biopsy or a bigger needle known as a core biopsy are used to obtain the sample.

The names of the industry leaders who are revolutionising healthcare with their cutting-edge diagnostic advancements in the diagnosis of ACC are listed in the following table:

                                                                   Diagnostic Market Players

Imaging Test

Tissue Sampling

Annon Piezo Technology Co. LIMITED

APC International, Ltd. (U.S.)

Canon Medical Systems Corporation

Becton, Dickinson, and Company (BD)

Hitachi, Ltd.

Foundation Medicine, Inc.

Toshiba Corporation

Danaher Corporation

Siemens Healthineers

Genomic Health, Inc.

GSK plc (U.K.),

F. Hoffmann-La Roche Ltd

Philips Healthcare

Biocept, Inc.

Shimadzu Corporation

Guardant Health, Inc.

Thermo Fisher Scientific

Biodesix, Inc

Bristol-Myers Squibb Company

Hologic, Inc.

Esaote S.p.A.

Roche Diagnostics

Neusoft Medical Systems Co., Ltd.

Abbott Laboratories

Mindray Medical International Limited


Bio-Rad Laboratories, Inc.

Teledyne Defense Electronics

Genomic Health, Inc.


Agilent Technologies, Inc.


TRS Technologies, Inc.


General Electric Company (GE Healthcare)



                                                                 Diagnosis Products

Imaging Tests

Tissue Sampling

SIGNA™ Architect


Optima™ CT






Discovery™ CT




SIGNA™ Pioneer


Revolution™ CT


Discovery™ PET/CT




VUE Point™ FX


Discovery™ MI

BD SurePath™


BD Vacutainer®


BD Vacutainer®




Treatment Analysis of ACC

"Promoting Lives, overcoming Adrenal Cancer: Exploring Hope through Specialised Treatment Solutions."

There are various treatment options for those suffering from ACC. Certain therapies are being analysed in clinical trials, while others are conventional (the treatment that is currently being used).

The following categories of treatment are applied:

Adrenocortical cancer is frequently treated with surgery known as an adrenalectomy. Occasionally, the cancerous tissue that has spread to neighbouring lymph nodes and other tissues is removed surgically.

Radiation Therapy

High-energy X-rays and other radiation therapies are used in radiation therapy to either kill or stop the growth of cancer cells. Two varieties of radiation therapy exist:

  • External radiation therapy directs radiation toward the cancerous part of the body using equipment that is external to the patient.
  • A radioactive material enclosed in needles, seeds, wires, or catheters is used in internal radiation therapy, and these devices are inserted into or close to the cancer.


 Chemotherapy is a cancer treatment that employs medications to either kill or prevent cancer cells from proliferating. Chemotherapy that is administered orally, intravenously, or intramuscularly enters the bloodstream and travels throughout the body to target cancer cells (systemic chemotherapy). Chemotherapy concentrates its effects on cancer cells in the locations it is applied directly to, such as the abdomen, an organ, or the cerebrospinal fluid (regional chemotherapy). Combination chemotherapy involves the use of many anticancer medications. The kind and stage of the disease being treated determine how the chemotherapy is administered.


The goal of cancer immunotherapy is to locate and eliminate malignant cells by stimulating the immune system. Immunotherapy comes in many forms, but all of them function by enhancing the immune system's capacity to combat cancer. Certain cancer patients have longer lived thanks to immunotherapy.

Targeted Therapy

Drugs or other substances are used in targeted therapy to target and destroy particular cancer cells. Generally speaking, targeted therapies are less harmful to normal cells than radiation or chemotherapy.

One medication that has been around for a while and has been used to treat advanced adrenal cancer has shown potential in postponing the disease's return following surgery. For patients with a high risk of cancer recurrence, Mitotane (Lysodren) is advised after surgery.


Adrenocortical cancer is frequently treated with surgery known as an adrenalectomy. Sometimes, the cancerous tissue that has spread to neighbouring lymph nodes and other tissues is removed surgically. It entails using a small camera and specialised tools to make three or four small incisions and remove the adrenal gland.

                                                                     Treatment Market Players

Radiation Therapy


Drug Therapy

Varian Medical Systems

Siemens Healthineers

Pfizer Inc.


Thermo Fisher Scientific Inc.

Amgen Inc.


GE Healthcare

Novartis AG

Siemens Healthineers

Halyard Health, Inc.

Eli Lilly and Company


Mevion Medical Systems

Merck & Co

Mitsubishi Electric Corporation

Becton, Dickinson and Company

Bayer AG

Hitachi, Ltd.

Stryker Corporation

Bristol-Myers Squibb Company,

Brainlab AG

Varian Medical Systems

Takeda Pharmaceutical

Best Theratronics Ltd


F. Hoffmann-La Roche Ltd

EnGeneIC Ltd.


AstraZeneca PLC

Exelixis, Inc.


Gilead Sciences, Inc.

Merck & Co.



Millendo Therapeutics



Orphagen Pharmaceuticals, Inc.





                                                                         Treatment Products

Radiation Therapy


Drug Therapy


BD Plastipak™






SIGMA Spectrum® Infusion System





MRIdian Cobalt

BD Alaris™



Alaris™ PC unit



BD Alaris™



BD Alaris™



BD Nexiva™



BD Angiocath™



BD Insyte™



BD Introsyte™



BD Introsyte™



BD CareFusion™



ARROW® Catheters



Recent Developments

The clinical-stage radiopharmaceutical company Radionetics Oncology, Inc. is dedicated to finding and developing new agents for treating a variety of oncology indications. The company announced its Phase 1 Radiopharmaceutical Programme Against Novel Target for Adrenocortical Carcinoma in October 2023, with support from Frazier Healthcare, 5 AM Ventures, Crinetics Pharmaceuticals, and GordonMD Global Investments.

Regulatory Framework

Growing businesses greatly benefit from the revised regulatory requirements provided by DiseaseLandscape Insights (DLI). With these updates, businesses stay on top of changing industry standards, stay out of trouble with regulations, and improve their marketing and development processes.

The FDA approved tivozanib on March 10, 2021, for the treatment of patients with advanced renal cell carcinoma (RCC) who had received two or more prior systemic treatments but were now relapsed or refractory (R/R). Based on a randomized trial comparing tivozanib to sorafenib in patients with R/R advanced RCC, the TIVO-3 study provided the basis for approval.

Clinical Trial Assessment

The DiseaseLandscape Insights consultancy firm provides valuable support in future market trends on the development of new pharmaceutical products. This support helps to streamline the planning and execution of clinical trials of novel medications and treatments, implement effective patient recruitment strategies, ensure regulatory compliance, and increase the likelihood of successful trial outcomes.

The below table gives information about some currently ongoing clinical trials, including their study titles and respective stages:

Phase 1

Phase 2

Phase 3

Phase 1 Study of 68Ga-R8760

A Phase II Study to Evaluate the Efficacy and Safety of Pembrolizumab in Combination with Mitotane in Patients with Advanced Adrenocortical Carcinoma


Mitotane With or Without Cisplatin and Etoposide After Surgery in Treating Patients with Stage I-III Adrenocortical Cancer with High Risk of Recurrence

A Novel Therapeutic Vaccine (EO2401) in Metastatic Adrenocortical Carcinoma, or Malignant Pheochromocytoma/Paraganglioma

Evaluation of the Efficacy of Addition of Progesterone to Standard Chemotherapy in Adrenocortical Carcinoma (ACC)


A Phase 1/2 Study of [225Ac]-FPI-1434 Injection

Cabozantinib in Advanced Adrenocortical Carcinoma


B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients with Solid Tumors (3CAR)

Phase II Study for Combination of Camrelizumab and Apatinib in the Second-line Treatment of Recurrent or Metastatic Adrenocortical Carcinoma


A Study of E7386 in Participants with Advanced Solid Tumor Including Colorectal Cancer (CRC)

Study of Radiotherapy and Pembrolizumab in People with Adrenocortical Carcinoma




DiseaseLandscape Insights (DLI) helps companies build and run effective strategies to prevent and control ACC epidemics. Furthermore, as awareness and anticipated epidemics grow, there is a growing demand for diagnostic tools, clinical evaluations, and novel therapeutics.

Major players involved in the production of medicinal items might benefit from the information and experience provided by DiseaseLandscape Insights. The assistance provided by DLI facilitates patient recruitment strategies, regulatory compliance, and the planning and execution of clinical trials for novel medications and pharmaceuticals.

This ultimately motivates the leaders to conduct qualitative research, investigate manufacturing companies, and find out about raw material sources. All industry participants gain a stronger foothold in Adrenocortical Carcinoma and keep one step ahead with the help of DiseaseLandscape Insights.

Vishal SawantBusiness Development

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