Adrenal Cortical Carcinoma

There are different types of adrenocortical tumors: benign or malignant and functioning (hormon secreting) or non-functioning. Adrenal adenomas are relatively common benign tumors, as illustrated in incidental findings on imaging done for other medical reasons, such as prenatal ultrasounds or CT (computed tomography) scans to evaluate kidney or gallstone disease. Most adenomas are termed incidentalomas, meaning they are found acidentally in the body and are generally not life threatening. However, there is a subset of adenomas that secrete hormones that can cause endocrine abnormalities in the body such as Cushing's syndrome, Conn's syndrome, virilization and feminization. Adrenal cortical carcinomas (ACCs) are very rare. The average incidence is about 0,0001% (one in one million of the population per year) but may vary depending on the geographical region. As same as adrenal adenomas, ACCs may be functioning (they secrete hormones) or non-functioning (they do not secrete hormones). Functioning cancers, besides of their malignancy, can cause serious abnormalities in the body due to abnormal secretion of hormones such as cortisol, aldosterone and sex hormones. The cause of this cancer is unknown.
SYMPTOMS are non-specific in case of non-functioning tumours and may include abdominal pain or a back pain or they are found incidentally during a routine medical examination. Functioning ACCs cause the same abnormalities as described above with functioning adenomas, but they occur at a higher frequency relative to non-functioning ACCs and, when advanced, may become rapidly fatal, as opposed to adenomas. Distant spreading ACCs generally metastasize to the lungs, liver, bone, and lymph nodes. It remains controversial, however, whether functioning and non-functioning tumors are more aggressive.
DIAGNOSIS: There are number of methods that can be used to detect adrenocortical tumors. These are a physical examination, hormone tests, imaging studies  such as CT (computerized tomography), MRI (magnetic resonance imaging) and PET (positron emission tomography) scans, ultrasound examination and most importantly a pathohistological examination of a specimen from a needle biopsy or surgical specimen from the tissue in question, which, once reviewed is classified as noncancerous or cancerous. If cancerous, the tumor is staged as Stage I (early stage) - Stage IV (most advanced).
TREATMENT: Early stage disease is highly curable through surgical resection of the tumor by specially trained surgical oncologists, such as endocrine surgeons who specialize in treating this disease.  While there is no curative treatment available for metastatic and recurrent ACC, patients may still benefit from various modalities of therapy. It includes pharmacotherapy with mitotane and/or other cytotoxic drugs such as etoposide, doxorubicin, streptozocin, vinca alkaloids etc. For advanced disease, other local modalities of therapies may be pursued in terms of palliating (improving) symptoms. These modalities, including radiation therapy, radiofrequency ablation (RFA), cryotherapy, and nerve ablation techniques, may be important to manage tumor complications or growth.


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