Vol 12, No 2 (2018)

Clinical practice
Factors affecting the probability of recurrence of the Cushing’s disease within 3 years after effective neurosurgical treatment
Nadezhdina E.Y., Rebrova O.Y., Ivashenko O.V., Azizyan V.N., Arapova S.D., Grigoriev A.Y.

Background. Cushing’s disease (СD) is а severe neuroendocrine disease that can rapidly progress with the development of severe complications of hypercorticism requiring immediate treatment. The main method of treatment is a neurosurgical operation, the effectiveness of which at the present time can reach 80% or more, however, about a quarter of patients after successful neurosurgical treatment experience reccurence.

Aim. The analysis of prognostic factors potentially affecting the occurrence of recurrence of CD after successful primary transnasal adenomectomy.

Material and methods. A retrospective monocenter comparative study of treatment outcomes in 219 patients (32 men, 187 women) with confirmed diagnosis of Cushing's disease who underwent endoscopic transsphenoidal adenomectomy between 2007 and 2014 was performed. The inclusion criteria were: the absence of previous pathogenetic treatment for this disease and the development of remission of the disease in the early postoperative period. The duration of follow-up period was three years and more. We used methods of statistical comparison of groups, including survival analysis and ROC-analysis.

Results. Within 3 years the remission was preserved in 172 patients, the recurrence of Cushing's disease developed in 47 patients (21.5% [16%; 28%]). The probability of CD recurrence was associated with morning levels of ACTH and cortisol and evening ACTH in the early postoperative period. As a result of the ROC-analysis for morning ACTH and cortisol, the optimal cutting points were 7 pg/ml and 123 nmol/l respectively. In patients with ACTH level less than 7 pg/ml, three years recurrence appeared to be 7%, 95% CI [3%, 14%], while at the level of ≥7 pg/ml recurrence was observed in 31% [23%, 40%] cases, RR 0.22 [0.09; 0.51], ОR – 0.16 [0.06; 0.43]. In patients with cortisol level below 123 nmol/l the recurrence developed in 13% [9%, 20%] of cases, while in patients with cortisol level ≥123 nmol/l, recurrence was equal to 45% [32%, 59%], RR 0.29 [0.18; 0.50], ОR – 0.19 [0.09; 0.39].

Conclusion. The development of adrenal insufficiency (a decrease in ACTH level less than 7 pg/ml and cortisol level less than 123 nmol/l) statistically significantly reduces the probability of the recurrence of CD within three years after surgery.

Endocrine Surgery. 2018;12(2):70-80
Clinical Case
Polyakov A.P., Mordovskiy A.V., Nikiforovich P.A., Ratushnyy M.V., Rebrikova I.V., Boyko A.V., Bolotina L.V., Gevorcov A.R.

Well differentiated thyroid cancer (WDTC) is referred to non-aggressive tumors with a relatively favorable course. However, in 10% of cases, distant metastases are recorded in this pathology, of them 5–15% of cases develop refractoriness to I131 therapy. Resistance to radioactive iodine therapy in patients with WDTC significantly worsens the overall and disease-free survival. In 2014, the targeted drug Sorafenib was registered in our country, as the first drug of choice for the treatment of patients with metastatic radio-refractory WDTC. The article presents his own experience in the therapy of radiorefractory thyroid cancer with a multikinase inhibitor – Sorafenib. This drug is effective for the treatment of this cohort of patients and also helps to maintain a satisfactory quality of life. Therapy with a multikinase inhibitor statistically significantly increases the time to progression and the median time without progression. In this way, patients have been monitored for a process that would not have been possible to achieve without the use of a targeted drug with multikinase inhibition.

Endocrine Surgery. 2018;12(2):81-88
Inferior petrosal sinus sampling in differential diagnosis of ACTH-dependent hypercortisolism
Sitkin I.I., Malygina A.A., Belaya Z.E., Rozhinskaya L.Y., Buryakina S.A.

Survival rate of patients with endogenous hypercortisolism, as well as complications reversibility and treatment effectiveness depends on on-time and precise diagnosis. In case of apparent clinical picture with all laboratory tests confirmation, the most challenging appears the differential diagnosis of ACTH-dependent hypercortisolism as none laboratory test can reveal the genesis of ACTH hypersecretion. When there is no visualization of the pituitary adenoma, the only method to confirm the diagnosis is bilateral inferior petrosal sinus sampling (BIPSS). Nevertheless, around 16% of patients have inactive pituitary incidentalomas in combination with carcinoid tumor of other localization. Epidemiologic researches show that the size of such incidentalomas tend to be less than 6 mm. According to clinical guidelines in case of such small neoplasms it’s recommended to perform bilateral inferior petrosal sinus sampling. This clinical case represent a rare exception to the rules when there is a pituitary adenoma more than 6 mm and mild hypercortisolism, possibly as the result of lung carcinoid tumor.

Endocrine Surgery. 2018;12(2):89-95
Surgical treatment for the patient with the neuroendocrine lung tumor associated with ectopic ACTH-secretion syndrome: case report
Pikunov M.Y., Pechetov A.A., Esakov Y.S., Lednev A.N.

Among primary neoplasms of the lungs, the proportion of neuroendocrine tumors is up to 20–25%. According to the Surveillance Epidemiology and Results (SEER) database, since 1970 there has been a significant increase in primary carcinoid tumors of various organs with the highest incidence of morbidity in the broncho-pulmonary localization group. Carcinoid tumors of the lung level are approximately 2% of all primary lung tumors, but only 5% of these formations are associated with ACTH production.

Despite the severity of clinical manifestations and the acceleration of hypercorticoid processes, the topical diagnosis of ACTH-ectopic syndrome often causes certain difficulties. The difficulty in diagnosing ectopic tumors is due to the fact that most of these formations are so small that they can be missed by standard methods of examination (X-ray, ultrasound, MSCT or MRI). After the diagnosis of ACTH-ectopic syndrome is completely optimal method of pathogenetic therapy is a radical removal of the tumor, which leads to the normalization of the functions of the adrenal cortex and the elimination of hypercorticoidism. However, the achievement of this goal in many cases is difficult due to late topical diagnosis, the prevalence of the tumor process and extensive metastasis.

There is a clinical observation of a patient with a diagnosis of a neuroendocrine lung tumor with ACTH-ectopic syndrome. The importance of the multidisciplinary approach, the methods of differential diagnosis, the features of the perioperative period and the long-term results of treatment are described.

Endocrine Surgery. 2018;12(2):96-101

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