[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.

[Severe bone complications of primary hyperparathyroidism in a young patient with the rare verified mutation of MEN1].

Multiple endocrine neoplasia type 1 syndrome (MEN1) is a rare inherited disorder that can include combinations of more than 20 endocrine and non-endocrine tumors. Unfortunately, none of the described MEN1 mutations has been associated with a peculiar clinical phenotype, even within members of the same family, thus a genotype-to-phenotype correlation does not exist. MEN1 syndrome is the most common cause of hereditary primary hyperparathyroidism (PHPT), the disease penetrance of which exceeds 50% by the age of 20 and reaches 95% by the age of 40. At the same time, PHPT with hyperplasia or adenomas of the parathyroid glands (PTG) is the most distinctive manifestation of the MEN1 syndrome. One of the main symptoms of PHPT, both in sporadic and hereditary forms of the disease, is bone damage. At the time of diagnosis in PHPT/MEN1, the bone mineral density is generally lower in comparison with the sporadic form of PHPT. This may be due to excessive secretion of parathyroid hormone during the period of peak bone mass, concomitant components of the syndrome, extended surgical treatment, and the direct effect of a mutation in the menin gene on bone remodeling. This clinical case describes a young patient with severe bone complications of PHPT and uncertain rare MEN1 mutation. PHPT was diagnosed five years later from the first onset of bone complications and repeated orthopedic operations. There was the «hungry bones¼ syndrome after successful surgery of PHPT, which was managed with vitamin D and calcium carbonate supplementation and there is a positive dynamic in increased bone mineral density in the main skeleton after 6 months.

[The role of 3D-CT in surgery for pancreatic ductal adenocarcinoma: post-processing and tissue volume calculation]. / Trekhmernaia rekonstruktsiia komp'iuternykh tomogramm v vybore operativnogo vmeshatel'stva pri protokovoi adenokartsinome podzheludochnoi zhelezy.

AIM: To define the indications for pancreatoduodenectomy using 3D CT-imaging, to calculate the volume of damaged and intact tissues and to determine type of surgery depending on severity of disease in case of ductal pancreatic adenocarcinoma. MATERIAL AND METHODS: Retrospective analysis of CT-scans of 30 patients with ductal pancreatic adenocarcinoma was performed. In groups 1 and 2 by 15 patients total pancreatectomy and pancreatoduodenectomy were made respectively. All patients underwent contrast-enhanced CT examination (Brilliance iCT, Phillips) followed post-processing on Brilliance Workspace Portal platform. All data were assessed by two reviewers. RESULTS: In group 1 CT volume of the tumor was 24±19 cm3 (32% of total pancreas), in group 2 - 9.8±6 cm3 (16% of total pancreas). CT-volume of celiacomesenteric arteries and portal system was 25.8±10 mm/59.5±18.9 mm and 23.3±6/49.9±14.7 mm in groups 1 and 2 respectively. Greater volume of tumor was significantly associated with increased portal system (p<0.03). Sensitivity, specificity and accuracy of determining of tumor location were increased up to 94%, 50% and 80% respectively in group 1. There were no cases of wrong localization in group 2. Data of both reviewers were similar. CONCLUSION: Total pancreatectomy is advisable for pancreatic adenocarcinoma if its volume is over 31.8% of total pancreas according to 3D CT-scans. 3D-modeling improves preoperative assessment of resectability, accuracy of determining of tumor localization and identifying vascular invasion.