[Chordoid gliomas of the third ventricle]. / Opyt lecheniya khordoidnykh gliom III zheludochka.

BACKGROUND: Chordoid glioma is a rare slow-growing tumor of the central nervous system. Available world experience includes no more than 200 cases (lesion of the third ventricle in absolute majority of cases). Recognition and treatment of chordoid glioma are currently difficult problems due to small incidence of this disease. OBJECTIVE: To describe clinical manifestations and surgical treatment of chordoid glioma of the third ventricle considering literature data and own experience. MATERIAL AND METHODS: There were 12 patients (6 men and 6 women) with chordoid glioma between 2004 and 2023 (10 patients with lesion of the third ventricle, 1 - lateral ventricle, 1 - pineal region). Only patients with tumors of the third ventricle were analyzed. RESULTS: Total and subtotal resection was performed in 1 and 3 cases, respectively. Five patients underwent partial resection, 1 patient underwent biopsy. The follow-up data were available in 7 out of 10 patients (mean 25 months). Radiotherapy was performed in 4 patients (continued tumor growth in 2 cases). One patient died. CONCLUSION: Chordoid glioma is a benign tumor predominantly localized in the third ventricle. Preoperative MRI and CT in some cases make it possible to suspect chordoid glioma and differentiate this tumor from craniopharyngioma, meningioma and pituitary adenoma by such signs as isointense signal in T1WI, hyper- or isointense signal in T2WI, homogeneous contrast enhancement and edema of basal ganglia in T2 FLAIR images. The only effective treatment for chordoid glioma is surgery. Total resection is often impossible or extremely dangerous due to location of tumor, large size and invasion of the third ventricle. Postoperative mental disorders and diabetes insipidus, including severe hypernatremia, are common that requires mandatory monitoring of water and electrolyte balance.

[Reset osmostat syndrome - when hyponatremia become «a normal¼: diagnostics, case report].

Reset osmostat syndrome (ROS) is characterized by a change of normal plasma osmolality threshold (decrease or increase), which leads to chronic dysnatremia (hypo- or hypernatremia). We have described a clinical case of ROS and chronic hyponatremia in a patient with chordoid glioma of the III ventricle. It is known that the patient had previously been diagnosed with hyponatremia (131-134 mmol/l). She has not hypothyroidism and hypocorticism. There is normal filtration function of the kidneys was (CKD-EPI 91.7 ml/mi/1,73m2). Urine osmolality and sodium level were studied to exclude of concentration kidney function disorder. During first three days after removal of the tumor of the third ventricle (chordoid glioma, WHO Grade II), the sodium level decreased to 119 mmol/l. Repeated infusions of 200-300 ml hypertonic 3% sodium chloride solution, gluco- and mineralocorticoid therapy was ineffective, increasing plasma sodium levels by 2-3 mmol/l with the return to the initial level during 6-8 hours. Hypopituitary disorders did not develop after surgery. With further observation, the sodium level remained within 126-129 mmol/l for 6 months after surgery. The water load test make exclude the classic syndrome of inappropriate secretion of antidiuretic hormone, and confirmed the diagnosis of RSO. Because of absence of clinical symptoms associated with hyponatremia, no medical correction was required, patient was recommended to clinical follow-up.

[Adipsic diabetes insipidus after transsphenoidal surgery for suprasellar intraventricular].

Presented case demonstrates a rare diencephalic pathology - adipsic diabetes insipidus (ADI) with severe hypernatremia in a 58-year-old woman after ttranssphenoidal removal of stalk intraventricular craniopharyngioma. ADI was diagnosed because of hypernatremia (150-155 mmol/L), polyuria (up to 4 liters per day) and absence of thirst. Normalization of water-electrolyte balance occurred on the background of desmopressin therapy and sufficient hydration in postoperative period. After release from the hospital, the patient independently stopped desmopressin therapy and did not consume an adequate amount of fluid of the background of polyuria. This led to severe hypernatremia (155-160 mmol/L) and rough mental disorders.Patients with ADI need closely monitoring of medical condition and water-electrolyte parameters, appointment of fixed doses of desmopressin and adequate hydration.

[Reproductive disorders and preservation of fertility in males with benign and malignant brain tumors]. / Narusheniia reproduktivnoi funktsii i vozmozhnosti sokhraneniia fertil'nosti muzhchin s dobrokachestvennymi i zlokachestvennymi opukholiami golovnogo mozga.

Brain tumors rank third in the incidence rate among cancer nosologies. However, improvement of neurosurgical treatment methods and the use of modern regimens of chemotherapy and radiotherapy have increased survival of patients with benign and malignant brain tumors. A significant proportion of patients with brain tumors are young people in the reproductive age who are interested in maintaining their fertility. Surgical removal of tumors, mainly in the hypothalamo-pituitary area as well as the use of chemotherapy and radiotherapy for malignant brain tumors of any localization may be complicated by hypogonadism and infertility. At present, a simple and reliable method of preserving male fertility is sperm cryopreservation. Neurosurgeons as well as oncologists and radiologists should inform patients with brain tumors about a potential risk of hypogonadism and infertility after treatment and about opportunities of sperm cryopreservation, which increases the chances of having future genetic progeny.

[Preoperative and postoperative endocrine disorders associated with pituitary stalk injuries caused by suprasellar growing tumors]. / Éndokrinnye rasstroistva do i posle operatsii pri razlichnykh porazheniiakh steblia gipofiza supraselliarno rastushchimi opukholiami.

The pituitary stalk (PS) is a relatively thin bundle connecting the hypophyseal stalk to the pituitary gland; it consists of both axons of the hypothalamic nuclei (terminating in the neurohypophysis) and the system of portal vessels. Compression of the PS by a space-occupying lesion or its transection (forced or intended) during surgery may lead to the development of endocrine disorders: hypopituitarism, diabetes insipidus, and hyperprolactinemia. The modern literature lacks studies evaluating the severity of endocrine disorders depending on the PS condition before and after surgery. PURPOSE: The study purpose was to investigate endocrine disorders in patients with sellar region (SR) tumors and the PS that was compressed before surgery and preserved or transected during a neurosurgical intervention. MATERIAL AND METHODS: The study included 139 patients with various SR tumors. In 82 patients, a preoperatively compressed PS was preserved (41 patients with hormonal inactive adenoma (HIA) and 41 patients with suprasellar meningioma); in 57 patients, the PS was transected during surgery (46 patients with pituitary stalk craniopharyngioma and 11 patients with hormonally inactive endosuprasellar pituitary adenoma). The hormonal status (PRL, TSH, LH, FSH, fT4, cortisol, testosterone, or estradiol) was examined in all patients both before and after surgery. RESULTS: Hyperprolactinemia was preoperatively detected in 37% of patients with tumors compressing the PS. Elimination of PS compression (tumor resection) led to normalization of the PRL level in most patients and was not accompanied by aggravation of hypopituitarism symptoms. Transection of the PS caused panhypopituitarism in 100% of patients and diabetes insipidus in 93% of cases. After transection of the PS, hyperprolactinemia did not develop in 59% of patients with craniopharyngiomas (CPs) and 82% of patients with HIA. CONCLUSIONS: Given the difference in symptoms associated with compression and surgical transection of the PS, we believe that these two concepts should be clearly distinguished. The PS compression syndrome includes primarily hyperprolactinemia (37% of cases); elimination of PS compression leads to normalization of the PRL level in most patients and is not accompanied by aggravation of hypopituitarism symptoms. The PS transection syndrome in patients with CP and HIA led to the development of panhypopituitarism in all patients and permanent diabetes insipidus in most of them. The causes of the absence of hyperprolactinemia in many patients with PS transection require further research. The surgeon planning intraoperative PS transection to increase the radicality of surgery should be well informed about the consequences of this procedure for the patient's endocrine status.

[A rare case of diencephalic cachexia in an adult female with cranio-pharyngioma]. / Redkii sluchai razvitiia diéntsefal'noi kakheksii u vzrosloi zhenshchiny s kraniofaringiomoi.

Diencephalic cachexia (DС) is progressive weight loss despite a normal caloric intake and a satisfactory state of health, which is caused by hypothalamic lesions. This is a rare (about 100 cases were reported) and potentially fatal disorder of unknown pathogenesis. At present, there is no effective pharmacological therapy for the disorder. Cachexia may regress only if the tumor reduces in size, therefore the timely diagnosis and treatment are of vital importance for the patient. DС is typical of early childhood, and only a few cases have been reported in adults. We present a rare case of DС in a 24-year-old female with papillary craniopharyngioma.

[The rate of hyponatremia in neurosurgical patients (comparison between the data from the Burdenko Neurosurgical Instutite and the literature) and recommendations for the diagnosis and treatment].

UNLABELLED: Hyponatremia is a relatively frequent and serious complication in patients with various neurosurgical pathologies. OBJECTIVE: This study is aimed at assessing the incidence of hyponatremia in neurosurgical patients depending on the pathology. MATERIAL AND METHODS: This paper presents a retrospective analysis 39 479 cases of patients operated on at the Burdenko Neurosurgical Institute from 2008 to 2014. RESULTS: A total of 785 hyponatremic patients with Na level lower than 130 mmol/l (2% of all operated patients) were identified. Mortality in patients with hyponatremia was 14.3%, which is tenfold higher compared to the rest of population of patients without hyponatremia who were operated on during the same period. In adults, hyponatremia most frequently occurred after resection of craniopharyngiomas (11%) and as a result of acute cerebrovascular accident (22%). In children, it occurred after resection of craniopharyngiomas (10%), astrocytomas (7%), ependymomas (24%), and germ cell tumors (10.5%). CONCLUSION: This study, which was mainly statistical one, was not aimed at detailed investigation of hyponatremia in different groups of neurosurgical patients. We only tried to draw the attention of various experts to those categories of patients, where focused and in-depth developments are more than important. Obviously, already gained international experience should be taken into account for this PURPOSE: Therefore, this article presents the literature data on the etiology and pathogenesis of hyponatremia. We describe the details of the various classifications of hyponatremia, its clinical symptoms, diagnosis, and treatments, primarily based on the recommendations of the last European consensus of various specialists (2014).

[Clinical and morphological characteristics, diagnostic criteria, and outcomes of surgical treatment of TSH-secreting pituitary adenomas]. / Kliniko-morfologicheskie kharakteristiki, diagnosticheskie kriterii i rezul'taty khirurgicheskogo lecheniya TTG-sekretiruyushchikh adenom gipofiza.

Thyrotropinomas (TSH-secreting tumors) are a rare type of pituitary adenomas, which account for about 0.5-2.0% of all pituitary tumors. The criterion of thyrotropinoma is visualization of the tumor in the presence of a normal or elevated level of the thyroid-stimulating hormone (TSH) in the blood and elevated concentrations of free T4 (fT4) and free T3 (fT3). OBJECTIVE: To study the clinical, diagnostic, and morphological characteristics and treatment outcomes of TSH-secreting pituitary tumors. MATERIAL AND METHODS: The study included 21 patients aged from 15 to 67 years with pituitary adenoma and a normal or elevated blood TSH level combined with elevated fT4 and fT3 levels who were operated on at the Neurosurgical Institute in the period between 2002 and 2015. Before surgery, in the early postoperative period, and 6 months after surgery, the patients were tested for levels of TSH, fT4, fT3, prolactin, cortisol, the luteinizing hormone (LH), the follicle-stimulating hormone (FSH), estradiol/testosterone, and the insulin-like growth factor (IGF-1). The thyroid status was evaluated using the following reference values: TSH, 0.4-4.0 mIU/L; fT4, 11.5-22.7 pmol/L; fT3, 3.5-6.5 pmol/L. An immunohistochemical study of material was performed with antibodies to TSH, PRL, GH, ACTH, LH, FSH, and Ki-67 (MiB-1 clone); in 13 cases, we used tests with antibodies to somatostatin receptors type 2 and 5 and to D2 subtype dopamine receptors. RESULTS: Thyrotropinomas were detected in patients aged from 15 to 67 years (median, 39 years), with an equal rate in males (48%) and females (52%). Before admission to the Neurosurgical Institute, 11 (52%) patients were erroneously diagnosed with primary hyperthyroidism; based on the diagnosis, 7 of these patients underwent surgery on the thyroid gland and/or received thyrostatics (4 cases). Hyperthyroidism symptoms were observed in 16 (76%) patients. The blood level of TSH was 2.47-38.4 mIU/L (median, 6.56); fT4, 22.8-54.8 nmol/L (median, 36); fT3, 4.24-12.9 pmol/L (median, 9.66). Tumors had the endosellar localization in 4 (19%) cases and the endo-extrasellar localization in 17 (91%) cases. Total tumor resection was performed in 7 (33%) patients. All these tumors had the endosellar and endo-suprasellar localization. No total resection was performed in patients with infiltrative growth of adenoma (invading the skull base structures). An immunohistochemical study of tumor resection specimens detected only TSH expression in 3 (14%) cases; 18 (86%) tumors were plurihormonal and secreted TSH and GH and/or PRL. Of 13 tumors, expression of the type 2 dopamine receptor was detected in 9 (69%) cases; expression of somatostatin receptors type 5 and type 2 was found in 6 (46%) and 2 (15%) cases, respectively. CONCLUSION: The criterion for total tumor resection was a postoperative decrease in the TSH level to 0.1 mIU/L or less. Total resection was performed in 33% of patients with tumors of only the endosellar and endo-suprasellar localization. In most cases, tumors were plurihormonal and secreted TSH and GH and/or PRL.