Adrenocortical cancer

There is currently no exact data on the cause of the neoplasm. It is believed that AKP develops from a hyperplastic node, which subsequently transforms into an adenoma, and then into a carcinoma. This hypothesis is based on some observations in which patients noted increased hormonal activity for 10 years. In cytogenetic studies, the pathogenesis of the disease began to be associated with the loss of heterozygosity of chromosomes 11p, 13q and 17v ^[3] .

Patients complain of pain in the back or abdomen, which is associated with the presence of education in the abdominal cavity. Excess hormone production is also noted, which causes the following symptoms:

• Hypersecretion of cortisol: general and muscle weakness, weight gain, redistribution of subcutaneous fat according to dysplastic type, bruising rashes, skin impurities, fractures, bone pain, infertility / hypogonadism, increased blood pressure;
• Hypersecretion of aldosterone: increased blood pressure, muscle weakness due to hypokalemia;
• Hypersecretion of testosterone: the growth of unwanted hair on the body, a decrease in voice, hypertrophy of the clitoris in women, an increase in oily and unclean skin
• Hypersecretion of estrogens: gynecomastia in men, metrogagia in postmenopausal women ^[3] .

T - primary tumor

• T1 - tumor ≤5 cm, absence of invasion;
• T2 - tumor> 5 cm, absence of invasion;
• T3 - local invasion of the surrounding fatty tissue;
• T4 - the tumor grows into neighboring organs.

N - regional lymph nodes

• N0 - no metastases in the regional lymph nodes;
• N1 - there are metastases in the regional lymph nodes.

M - distant metastases

• M0 - no distant metastases;
• M1 - there are distant metastases.

Stage Grouping

The classification according to ENSAT (European Network for the Study of Adrenal Tumors) seems to be clinically more adapted and modern:

• Stage I - T1 N0 M0
• Stage II - T2 N0 M0
• Stage III - T1-2 N1 M0, T3-4 N0-1 M0
• Stage IV - T1-4 N0-1 M1 ^[4] .

Laboratory Diagnostics:

• Determination of cortisol in the early morning hours on the background of an overwhelming test with 1 mg of dexamethasone;
• In the absence of physiological suppression of cortisol levels - the determination of adrenocorticotropic hormone in the morning.
• Determination of (nor) methanephrine in daily urine or plasma.

Instrumental Diagnostics:

• Ultrasound
• MRI
• CT (Gold Standard);
• Osteoscintigraphy with suspected metastatic damage to the skeleton bones;
• For tumor sizes up to 4 cm, it is recommended that positron emission tomography with 18 fluorodeoxyglucose (PET18FDG) be performed.

It should be noted that the puncture biopsy of adrenal tumors did not demonstrate an improvement in the results of differential diagnosis, on the contrary, it led to an increase in complications, false-positive and false-negative conclusions. At the moment, puncture is advisable only if there is a suspicion of metastatic damage to the adrenal glands, where the sensitivity of the cytological examination is 80–86% ^[3] .

The main form of AKP treatment is surgery . The operation should be carried out in the usual way (abdominal surgery). Laparoscopic surgery is prohibited, every patient should know this, otherwise there is a risk of seeding the tumor.

Adjuvant (prophylactic) therapy after surgery is carried out with the drug mitotan ( chloditan , lysodren ) is included in most protocols for AKP chemotherapy.

Currently, the largest experience in the use and monitoring of patients taking mitotan in Russia are the Endocrinological Research Center of the Ministry of Health of the Russian Federation and the Russian Cancer Research Center named after Nikolai Nikolayevich Blokhin. The greatest experience in treating AKP in Russia has been accumulated in the RSC them. Blokhin , there were treated at least 400 people.

Mitotan is currently registered in the Russian Federation (Mitotan tablets 500 mg, LP-004684 from 02/05/2018). Previously, Mitotan was also produced under the trade name Hloditan in what is now Ukraine , where the drug ceased to be produced due to the decline of the pharmaceutical industry in the 1990s throughout the post-Soviet space.

As for the effectiveness of the use of mitotan, disputes around it are ongoing. It is believed that mitotan is effective in 30% of cases of its use. Self-administration of this drug is unacceptable and can be done only under the supervision of doctors, since mitonan suppresses the functioning of a healthy adrenal gland and the use of mitotan without replacement therapy (one of the replacement therapy drugs is cortef) can lead to death of the patient.

Scientific studies of adrenocortical cancer in Russia are not conducted, the most famous centers for the study of AKP at the moment are the University of Michigan , as well as the National Institute of Health in the USA and the University of Würzburg in Germany .

The vast majority of cancer hospitals around the world sometimes experience this disease only once in a decade, so the Internet community is trying to create forums or groups that bring together patients diagnosed with adrenocortical cancer to share treatment experiences and maintain morale in the fight against this disease. A group called “Adrenocortical cancer - adrenal cortex cancer” was created on Facebook, where patients from Russia, Ukraine, and other countries of the near abroad, their relatives and friends become participants and do not feel lonely, and also receive important information about where to get the necessary treatment. Now in this group are registered more than 100 participants.