Adrenocortical carcinoma (ACC) is one of the most aggressive solid tumors in the human body. It is characterized by high mortality and is often diagnosed at an advanced stage. It is a very rare tumor, the annual incidence of which is currently estimated at 0.5-2 cases per million inhabitants.
Adrenocortical carcinoma (ACC) is one of the most aggressive solid tumors in the human body. It is characterized by high mortality and is often diagnosed at an advanced stage. It is a very rare tumor, the annual incidence of which is currently estimated at 0.5-2 cases per million inhabitants.
The prognosis of the disease is unfavorable — five-year survival is only around 5-10%, with the clinical stage of diagnosis playing a crucial role. In patients with localized disease (without spreading beyond the adrenal gland) and the possibility of radical surgical resection, the prognosis may be slightly better. Conversely, with disseminated disease, the chance of long-term survival is very low.
The incidence of ACC typically has a bimodal distribution — it occurs more frequently in early childhood (up to 5 years of age) and then between the ages of 40—60.
The tumor can be:
It is the hormonal activity of the tumor that in many patients tends to be the first guide to the diagnosis.
In a non-functional tumor that does not produce any hormones, diagnosis is often made only at an advanced stage — the tumor is detected accidentally on imaging or as a result of symptoms caused by local growth (e.g. pain in the shoulders, abdominal pressure) or the presence of metastases (lungs, liver, lymph nodes).
Every case is different. If you are not sure how to proceed further in a patient with a suspected adrenal tumor, please contact us. Together, we will assess the situation and recommend the next professional procedure — quickly, factually and in partnership.
Adrenocortical carcinoma is usually a very bulky tumor (larger than 5—8 cm), with irregular, infiltrative margins, signs of necrosis, bleeding, and rapid growth. It usually occurs unilaterally — the left and right sides are affected approximately equally often.
Imaging methods often reveal local invasion of surrounding structures — for example, the kidney, diaphragm, regional lymph nodes, or large vessels such as the inferior vena cava or renal vein.
At the time of diagnosis, metastases (most often in the lungs, liver, bones or lymph nodes) are present in most patients, which significantly affects further treatment and prognosis.
An important role in differential diagnosis is played by:
Tumors suspected of ACC should always be investigated within a specialized multidisciplinary team.
Adrenocortical carcinoma is a metabolically very active tumor, meaning that it shows a high accumulation of FDG and is thus intensely PET-positive.
Note Tumor biopsy is not recommended for adrenocortical carcinoma because it can lead to tumor cell proliferation and results are not usually conclusive in terms of histopathological evaluation.
Treatment of ACC depends on the extent of the disease, the presence of metastases and the general condition of the patient.
In selected patients, surgery can be curative, or at least significantly prolong survival and improve quality of life.
In most cases, especially with advanced or metastatic disease, the main treatment approach is:
Adrenocortical carcinomas are rare and highly aggressive tumors. Diagnosis and treatment of this disease should always be guided in specialized centers, in the hands of an experienced team of specialists who focus on adrenal tumors.
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