[Fulminant hypercorticism due to ACTG producing pheochromocytoma].

Endogenous hypercorticism (EH) is a severe symptom complex caused by hypercortisolemia; according to the etiology, ACTH-dependent and ACTH-independent variants are distinguished, which, according to the literature, occur in 70-80% and 20-30% of cases, respectively. A rare cause of ACTH-dependent endogenous hypercorticism is ACTH-ectopic syndrome (ACTH-ES) (about 15-20% of cases). ACTH-ES is a syndrome of adrenocorticotropic hormone (ACTH) hyperproduction by neuroendocrine tumors of extrahypophyseal origin. Various tumors can secrete ACTH: bronchopulmonary carcinoid, small cell lung cancer, less frequently, thymus carcinoid, islet cell tumors and pancreatic carcinoid, medullary thyroid cancer, carcinoid tumors of the intestine, ovaries, as well as pheochromocytoma (PCC).This publication presents a clinical case of rarely detected paraneoplastic ACTH production by pheochromocytoma. The patient had clinical manifestations of hypercorticism, therefore, she applied to the Russian National Research Center of Endocrinology of the Ministry of Health of Russia. During the examination Cushing's syndrome (CS) was confirmed, multispiral computed tomography (MSCT) of the abdominal cavity revealed a voluminous formation of the left adrenal gland. Additional examination recorded a multiple increase in urinary catecholamine levels. Subsequently, the patient underwent left-sided adrenalectomy. The diagnosis of pheochromocytoma was confirmed morphologically, immunohistochemical study demonstrated intensive expression of chromogranin A and ACTH by tumor cells.

[Diagnosis and differential diagnosis of primary hyperaldosteronism].

This literature review concerns diagnosis and differential diagnosis of primary hyperaldosteronism, a frequent cause of arterial hypertension. It describes different phases of diagnosis of this condition including main medicamentous tests and instrumental methods as well as nosological entities from which primary hyperaldosteronism needs to be distinguished.

[Causes of recurrence of patients with chromaffin tumors]. / Prichiny retsidivov u bol'nykh opukholiami khromaffinnoi tkani.

From 1957 to 2001, 520 patients with pheochromocytomas were operated. Two hundred and sixty patients were followed up from 4 to 21 years (8.4 +/- 1.9 years on the average). Complaints of 83 (31.9%) patients were similar to those before removal of the primary tumor. There was no evidence of tumors in 34 patients. Chromaffin tumors were revealed in 49 (18.9%) patients with clinical symptoms of hypercatecholaminemia. In 27 (55.1%) cases contralateral tumors were detected, in 11 of 27--the disease was familial. Extraadrenal chromaffin tumors occurred in 6 (12.2%) patients. In 8 of 49 (16.3%) patients recurrent clinical symptoms resulted from metastasis, malignant tumor after the first operation was diagnosed morphologically only in 6 of them. True recurrence of tumor at the in site of primary operation was recorded in 8 (16.3%) patients, including in 2 patients with MEN-2 in multicentric but benign tumor. Thus, true recurrence was diagnosed in 6.15% of 260 patients. True recurrence is most probable in multicentric tumor, when the tumor involves adjacent organs and tissues, and in vascular emboli in great vessels.

[Potentialities of ultrasound examination in differential diagnosis of benign nodules and breast cancer]. / Vozmozhnosti ul'trazvukovogo issledovaniia v differentsial'noí diagnostike dobrokachestvennykh uzlovykh obrazovanií i raka molochnoí zhelezy.

The results of examination of 410 patients with nodular masses of the breast are analysed. Benign tumors were present in 314 (76.6%) of the patients, cancer of the mammary gland--in 96 (23.4%) of the patients. The tumors were classified into 3 types, depending on the intensity of the ultrasound wave behind the tumor. Based on the clinical and morphological analyses it was demonstrated that the presence of the acoustic wave behind the tumor is typical for the invasive cancer of the duct, scirrous carcinoma and lobular cancer. In medullar and mucosous cancer the intensity of the ultrasound wave does not change. In case of intracystic cancer the symptom of "back magnification" is present. The sensitivity of the ultrasound in detection of mammary gland cancer is 88.5%, specificity--96.5%. The analogous parameters of mammography are: 91.0% and 97.8%. In the use of ultrasound control in fine needle aspiration biopsy (FNAB) the sensitivity and specificity of the method increases from 88.5% to 92.7%. If the diagnosis of mammary gland cancer was established with the use of ultrasound and FNAB the additional use of mammography is not obligatory.

[Diagnosis and treatment of breast cysts]. / Diagnostika i lechenie kist molochnoi zhelezy.

The results of examination and treatment of 62 patients with breast cysts are presented. Basing on ultrasound and cytological examination evidence, indications and contraindications for puncture treatment of breast cysts with simultaneous 96% ethanol sclerosing with have been worked out. This treatment was carried out in 45 patients, the rate of recurrences being 11.1% compared to 21.9% in the control group. The puncture treatment is indicated in the mass of regular shape of homogenous unechogenous structure with symmetric "lateral shades" revealed by US examination, absence of atypical intracystic epithelium according to cytologic examination data. Contraindications for this method are the following: presence of intracystic growth, irregular shape of the cyst, asymmetric "lateral shades", polycystic disease of the breasts, cellular atypia revealed by cytologic examination, recurrent cyst, previuns mastectomy for breast cancer, atypical ductal or lobular hyperplasia revealed in sectoral resection.

[Target thin-needle puncture biopsy in diagnosis of thyroid cancer]. / Pritsel'naia tonkoigol'naia punktsionnaia biopsiia v diagnostike raka shchitovdnoi zhelezy.

One hundred medical histories of patients with thyroid cancer (TC) were analyzed. Patients were divided into two groups depending on the variant of thin-needle biopsy technique (TNB). In group 1TNB was performed with "blind" method (with palpation control)--43 cases, in group 2 target TNB (ultrasound-guided) was carried out--57 cases. Patients of each group were divided depending on cytological presurgical diagnosis. Statistical analysis was carried out with two-sided Fisher's criterion. It is demonstrated that detection of TC in group 2 (84.2%) was higher than in group 1 (62.8%) where "blind" method of puncture was used (p = 0.020). It is recommended to perform target TNB in all cases of nodular goiter that improves quality of TC diagnosis.

[Diagnosis and treatment of syndrome of multiple endocrine neoplasia type 2]. / Diagnostika i lechenie sindroma mnozhestvennykh éndokrinnykh neoplazii 2-go tipa.

Since 1969 to 2000 twenty one patients from 16 families with syndrome of multiple endocrine neoplasia (MEN) type 2 were examined. Medullary cancer of the thyroid gland (MCTG) was diagnosed in 18 patients, pheochromocytoma--in 15 (in 13 of them--two-sided), primary hyperparathyroidism--in 2. In 9 patients from 5 families syndrome MEN 2 was confirmed genetically (mutation in codon 634 of 11th exon RET in 7 patients with MEN 2a and in codon 918 in 2 patients with MEN 2b). None of the patients had extraadrenal pheochromocytoma, in 9 (60%) patients multicentric tumors within one adrenal gland were diagnosed. All the 18 patients with MCTG underwent extrafascial thyroidectomy with removal of fat and lymph nodes of paratracheal zone, 9 patients--one-sided (6) or two-sided (3) removal of fat and lymph nodes of lateral triangle of neck. Prophylactic thyreoidectomy was performed in 11-year old patient with genetically verified MEN 2a and without topical data of MCTG, 2 patients of 3 and 19 years of age with genetically verified MEN 2 are to undergo prophylactic thyroidectomy. Prophylactic thyroidectomy is necessary in the presence of genetic disorders in members of families with MEN 2 despite absence of structural changes in thyroid gland. Level of basal and stimulated calcitonin may be used as marker of recurrence or metastatic growth only. In MEN 2 after organ-saving operation rate of true recurrence of tumor is high because of genetic damage of medullary layer of adrenal gland.