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Cushing’s disease is caused by a pituitary tumor which causes increased production of adrenocorticotropic hormone, leading to chronic hypersecretion of cortisol by the adrenal cortices. Endoscopic transnasal adenomectomy is the initial treatment of choice with the greatest efficiency for the treatment of the disease. However in the absence of remission or relapse of hypercortisolism after neurosurgical surgery, as well as in cases when surgical intervention cannot be carried due to medical contraindications to surgical intervention, radiation treatment is used as an alternative or adjoining therapy. In this literature review the efficiency of different radiation techniques (the conventional and the modern techniques), as well as possible complications of modern methods of radiosurgery and radiotherapy have been looked for.

The relevance of cachexia syndrome is determined by its high prevalence in clinical practice. It accompanies the course of not only oncological diseases, but also the majority of chronic somatic pathologies, such as chronic heart failure, renal failure, diabetes mellitus, chronic obstructive pulmonary disease, acquired immunodeficiency syndrome, rheumatoid arthritis, Alzheimers disease and others. It is known that even a slight weight loss in patients can determine an unfavorable prognosis of the underlying disease and reduce the effectiveness of therapy, and sometimes it becomes the direct cause of death of the patient. Cachexia is a complex metabolic syndrome, which is based on a violation of the central regulation of metabolism. The dangerous combination of decreased appetite (anorexia) and increased metabolism is the result of an imbalance in energy exchange. Treatment of cachexia syndrome is ineffective and limited in means. Given the progressive and irreversible nature of this syndrome, early diagnosis and prevention of its development are the primary task of the doctor. The article describes the main pathogenetic aspects of the development of cachexia syndrome. They can be common in different diseases. The article discusses the difficulties of diagnosing cachexia syndrome, the possibilities and prospects of treatment.

Introduction: Currently, cabergoline therapy is the main treatment for prolactinomas. The use of the drug in most cases leads to regression of the tumor, normalization of prolactin (PRL) levels, and restoration of gonadotropic function. The mechanism of its action in tumor cells “in vivo” tracked in dynamics in the same human tumor is of considerable interest. Materials and Methods: A 30-year-old male was admitted to N.N. Burdenko National Medical Research Center of Neurosurgery. An magnetic resonance imaging (MRI) revealed a giant pituitary adenoma. The level of PRL was more than 5000 mU/l (30-360) (serum dilution was not used to determine PRL). Transcranial microsurgical removal of the tumor was performed. He was treated by cabergoline after surgery. Endoscopic transsphenoidal approach was repeated with subtotal removal of the rest of the tumor. Morphological and immunohistochemical studies of the tumor were done. Results: A morphological study revealed PRL-positive tumor with a Ki-67 LI of 8% with a distinctive expression of D2R, CD31, and CD34 markers. Control MRI in 3 months after surgery revealed remnants of a tumor of endoinfrasellar localization, the tumor remainders were found in endoinfrasellar localization. The tumor retained pronounced immunopositivity to PRL and D2R and a decrease in the Ki-67 to 2% and in the expression of CD31 and CD34. Subsequent therapy with cabergoline resulted in persistent normoprolactinemia, restoration of androgenic function, and absence of tumor recurrence during the 10-year follow-up period. Conclusions: Cabergoline is an effective treatment for prolactinoma, which leads to tumor regression. One of its mechanisms is the reduction of the proliferative index and tumor angiogenesis.

The clinical picture of craniopharyngiomas (CPs) is diverse and polymorphic in its manifestations, including endocrine, neurological disorders, visual and mental impairment. Mental disorders in the clinical picture of CPs are detected from 6 to 100 %, according to the literature. But more often one can find a description of individual syndromes only — memory impairment, disorders of the emotional sphere and/or behavior. There is not enough information about mental disorders in different topographic and anatomical variants of craniopharyngioma, possible pathognomonic psychopathological symptoms and/or syndromes. Objective of the article is to describe mental disorders in the clinical picture of craniopharyngioma with different topographic and anatomical variants in adults. Materials and methods. The study included 120 adult patients (18–68 years old, mean age 39 ± 2; 59 women and 61 men) who were first admitted for treatment to the National Medical Research Center for Neurosurgery named after academician N. N. Burdenko with the diagnosis of craniopharyngioma (2007–2015). The clinical-psychopathological method was the main in the research. Results. Mental disorders in the clinical picture of CPs were detected in 103 (85.8 %) patients before surgery in the form of the main syndromes: asthenic syndrome was found in 83 (69 %) patients, emotional-volitional disorders in 79 (65,8 %), amnestic disorder in 71 (59,1 %), personality changes in 71 (59,1 %), paroxysmal conditions in 55 (45,8 %), sleep and dream disorders in 54 (45 %), consciousness disorders in 24 (20 %) patients. Mental disorders did not appear separately, but in combination with each other, prevailing in the clinical picture to one degree or another in various topographic and anatomical variants of craniopharyngioma, respectively. Conclusion. Mental disorders in the clinical picture of CPs were detected in 85,8 % of patients before surgery, they were determined by the location of the tumor lesion — topographic and anatomical variant of CP, the spread and influence of the tumor on the structures of the diencephalic region, limbic system, and third ventricle: in 65 % of cases with endosuprasellar craniopharyngioma, in 86 % of cases with stem craniopharyngioma, in 100 % of cases with extra- and intraventricular CPs.

Introduction Surgery is the primary treatment for Cushing's disease(CD). In cases with no biochemical remission after surgical resection or when recurrence occurs after a period of remission stereotactic radiosurgery (SRS) is used as alternative/adjuvant treatment. The aim of this study is to demonstrate the effectiveness of SRS and FSRS(Fractionated stereotactic radiosurgery) for the treatment of CD in a long term follow up. Methods This is a retrospective study in which 41 patient (36 females and 5 males) who underwent surgery for CD from 2009 to 2019 were included. Out of 41 cases, 34 cases had microadenomas while 7 had macroadenomas. These patients had recurrence or persistence of hypercortisolism post-operatively. After multidisciplinary evaluation, these patients were treated by CyberKnife (SRS & FSRS). Results Remission rate in our study was 60.97% with a median follow up period of 79.03 months. The median time to biochemical remission was 14 months. Tumour growth control was achieved in 95.12%. Hypopituitarism of different axes was seen in 34.14% patients. Secondary hypothyroidism was the most common pituitary insufficiency (34%) followed by secondary hypogonadism in 17%. Conclusion CyberKnife radiosurgery and hypofractionated radiosurgery can be used as an adjuvant treatment in patient with active disease and no biochemical remission after one or multiple surgical resections. Risk of radiation induced hypopituitarism and other complication is relatively low 34.14% and tumour growth control is significantly higher.

The article presents a clinical case from the practice of a patient with craniopharyngioma. The man of reproductive age with diencephalic obesity (BMI 35 kg/m2), recurrent craniopharyngioma, a long history of endocrine disorders (panhypopituitarism, including secondary hypogonadism, with the corresponding sexual function disorders and the lack of sexual activity), with visual disorders and psychiatric symptoms in the early postoperative period after shunting surgery and reducing the volume of the craniopharyngioma cyst, erection conditions arose against the background of episodes of disorders of consciousness within the framework of sleep dissociation. After the operation (installation of the Ommaya system), the patient had a state of «spontaneous» erection lasting up to 30 minutes against the background of dream-oneiric states of impaired consciousness with erotic experiences. These states were observed for 3 nights, the patient remembered the experiences and events that occurred to him «in a dream», and could tell about them to others on the next morning. The identity of the patient remained intact, he was active in the department, ordered in behavior; memory for current events and new information was intact. These disorders in the patient did not require specialized treatment, regressed independently on the 6th day of the postoperative period.

The study objective is evaluate the effectiveness and risks of using intradural and extradural decompression of the optic nerve channels during standard transcranial removal of meningiomas of the chiasmal-cellar region region. Materials and methods. The results of 161 decompression of channel of optic nerve in 129 patients are analyzed. In most patients (n = 122), an intradural decompression (resection) of the canal roof was performed. In 7 patients, an extradural decompression of the upper and lateral walls of the canal was performed. Results. In assessing the risks of visual impairment as a result of channel decompression, we found that decompression itself, without attempts to separate the tumor from the nerve, causes vision impairment in 8.6 % of cases. Attempts to separate the tumor from the optic nerves increase the risk of vision impairment from 16.7 to 26.9 %, depending on the radicality. Conclusion. A small number of complications (iatrogenic amaurosis and nasal liquorrhea) make it possible to recommend the described technique as an effective and safe addition to standard transcranial operations to remove the meningiomas of the chiasmal-cellar region.

The combination of intracranial tumors and asymptomatic brain aneurysms is an urgent problem, since it can significantly affect surgical intervention. Aneurysms are common in patients with meningioma, glioma and pituitary adenoma. According to certain authors, combination of aneurysms with pituitary adenomas is 7 times more common than with other tumors. In these cases, a comprehensive examination of the patient and decision-making on surgical strategy are required. This review is devoted to epidemiology, diagnosis and treatment of patients with a combination of pituitary adenomas and intracranial aneurysms detected intraoperatively or at the preoperative stage. The manuscript is illustrated by cases observed at the Burdenko Neurosurgery Center.Сочетание внутричерепных опухолей с бессимптомными аневризмами сосудов головного мозга является достаточно актуальной проблемой, так как может существенно влиять на ход хирургического вмешательства. Наиболее часто аневризмы обнаруживаются у больных с менингиомами, глиомами и аденомами гипофиза, причем с последними подобное сочетание встречается в несколько раз чаще, чем с другими опухолями. В таких случаях необходимо всестороннее обследование пациента и принятие решения о тактике хирургического лечения и ведения больного. Представлен обзор современной литературы, посвященный эпидемиологии, диагностике и лечению пациентов с сочетанием аденом гипофиза и интракраниальных аневризм, выявленных на дооперационном этапе или интраоперационно. Обзор иллюстрирован клиническими наблюдениями из опыта работы ФГАУ «НМИЦ нейрохирургии им. акад. Н.Н. Бурденко».

Neuroblastoma is a malignancy developing from the embryonic neuroblasts of sympathetic nervous system. Primary sellar neuroblastomas are extremely rare (there are currently only 11 case reports in the literature). Possible development of neuroblastoma in sellar region expands differential diagnosis of local processes due to inclusion of neuroblastoma into the spectrum of suspected tumors. We report a literature review and description of a patient with primary sellar neuroblastoma.Нейробластома является злокачественной опухолью, развивающейся из эмбриональных нейробластов симпатической нервной системы. Первичные нейробластомы селлярной локализации встречаются крайне редко (в настоящее время в литературе представлено лишь 11 случаев). Возможность их развития в хиазмально-селлярной области расширяет объем дифференциальной диагностики локализующихся здесь патологических процессов из-за необходимости включения нейробластом в спектр предполагаемых опухолей. В нашей работе представлены анализ литературы и описание клинического наблюдения пациента с первичной селлярной нейробластомой.

Currently, cabergoline therapy is the main method of treatment with prolactin. The use of the drug in most cases leads to tumor regression, normalization of prolactin levels and restoration of gonadotropic function. The mechanism of its impact on tumor cells in vivo, which is dynamically traced in the same human tumor, is the case of considerable interest. We observed a 30-year-old patient who was operated on twice for a giant prolactinoma before and on treatment by cabergoline. The morphological study after the first surgery (before introducing of cabergoline therapy) revealed a prolactin-positive pituitary tumor with a Ki-67 labeling index of 8% and with strong expression of dopamine type 2 receptors (D2R), CD31 and CD34. After 4 months, during which the patient received cabergoline at a dose starting from 0.5 mg to 1.5 mg per week, a second transsphenoidal surgery was performed with subtotal removal of residual tumor tissue. During the morphological study of the second biopsy sample, the tumor retained a pronounced immunopositivity to prolactin and D2R, with a decrease in the labeling index Ki-67 to 2%, as well as a decrease in the expression of CD31 and CD34. Subsequent cabergoline therapyresulted in persistent normoprolactinemia, restoration of androgen (and reproductive) status, and no tumor recurrence over a 10-year period on cabergoline treatment. Thus, one of the mechanisms of effect of cabergoline that leads to tumor regression is a decrease in the proliferative index and angiogenesis of the tumor.

Brain metastases of various types of cancer are diagnosed in 8-10% of all cancer patients. In the world literature, only 30 cases of cancer metastasis to the pituitary adenoma are described. This article presents yet another observation of a patient with breast cancer metastasis into the hormone-inactive pituitary adenoma at the Burdenko neurosurgical center, Russia The patient underwent endoscopic endonasal transsphenoid removal of the neoplasm. During microscopy and immunohistochemical studies of the biopsy, two types of tissue (pituitary adenoma and cancer metastasis) with different Ki-67 treated surgically (1% and over 40%) were found.Метастазы различных видов рака в головной мозг выявляются у 8-10% от всех онкологических пациентов. В мировой литературе описано всего 30 случаев метастазирования рака в аденому гипофиза. В нашей публикации представлено еще одно наблюдение хирургического лечения пациентки с метастазом рака молочной железы в гормонально-неактивную аденому гипофиза, которое проведено в ФГАУ 'Национальный медицинский исследовательский центр нейрохирургии им. акад. Н.Н. Бурденко' Минздрава России. Пациентка подверглась эндоскопическому эндоназальному транссфеноидальному удалению новообразования. При проведении микроскопии и иммуногистохимического исследования биоптата выявлено два вида ткани (аденома гипофиза и метастаз рака) с разными индексами Ki-67 (1% и свыше 40%).

Introduction: Germinogenic central nervous system (CNS) tumors represent a heterogeneous group of tumors, constituting approximately 0.4% of all primary brain tumors. Removal of the tumor has no prognostic value. In “pure” primary germinomas of the CNS, the alpha-fetoprotein and human chorionic gonadotropin levels are within normal limits, and no specific biochemical tumor markers currently exist for this tumor type, making histological verification crucial for the choice of treatment tactics. When the tumor is located in the chiasmosellar region, one of the possible verification methods is endoscopic endonasal transsphenoidal biopsy. Objective: the main objective of the study is to demonstrate the feasibility and safety of endoscopic transsphenoidal approach for histological verification of primary germinomas of the CNS with chiasmosellar localization. Materials and Methods: The current study includes 13 patients with “pure” germinomas of the chiasmosellar region who underwent endoscopic endonasal surgical interventions with subsequent treatment according to the “Germinoma 2008” protocol. Results: The extent of surgical intervention ranged from biopsy (4) to partial (5) and total (4) removal of the tumor. In all cases, histological verification of the diagnosis was achieved and none of the patients presented with cerebrospinal fluid leaks and/or meningitis in the postoperative period, allowing to evaluate endoscopic intervention in our patient series as safe and effective. Two out of 13 patients were lost to follow-up. Conclusion: The endoscopic endonasal approach for histological verification and removal of chiasmosellar region germinomas is safe, and in some cases, less traumatic for the patient than transcranial and transventricular approaches.

The study objective is to describe currently available and optimal methods of central skull base reconstruction.Material and methods. This article describes a 10-year experience of endoscopic surgery in reconstructing the defects after the removal of chiasmosellar tumors.Results. We developed a strategy for assessing the risks for postoperative liquorrhea and choosing an optimal surgical technique. This strategy allows maintaining the incidence of postoperative liquorrhea of 4 %.Conclusion. Autografts are the most widely used material for reconstructing skull base defects after the removal of chiasmosellar tumors. The multilayer reconstruction is more preferable due to the high risk of postoperative liquorrhea.

McCune-Albright Syndrome is a rare genetic disease characterized by the formation of fibrous osteodysplasia foci of various localization, including the bones of skull base. Having a gross lesion of the main bone body and the simultaneous formation of the pituitary adenoma, its transnasal removal becomes very difficult.Two clinical observations are presented where at patients with the classic manifestation of McCune-Albright syndrome we were able to successfully remove somatotropinomas with endoextrasellar growth. In both cases the presence of visual disorder was the indication for surgery.Despite pronounced deviations in the anatomy of the skull base bones in both cases we managed to access the Turkish saddle. Removal of the pituitary tumor did not differ from standard surgery. Improved vision after surgery was observed in two patients. Tumors were removed subtotally and clinical and biochemical remission of acromegaly was not achieved. In both cases, the tumors had morphological signs of atypia. Patients continued to receive therapy with somatostatin analogues and radiation.The possibility of performing transnasal surgery in this category of patients using specific instruments and intraoperative navigation is shown.Синдром МакКьюна-Олбрайта - редкое генетическое заболевание, характеризующееся формированием очагов фиброзной остеодисплазии различной локализации, в том числе в костях основания черепа. При грубом поражении тела основной кости и одновременном формировании аденомы гипофиза ее трансназальное удаление представляется очень сложным. Материал и методы. Приведено два клинических наблюдения, в которых у пациентов с классическим проявлением синдрома МакКьюна-Олбрайта нам удалось успешно удалить соматотропиномы с эндоэкстраселлярным ростом. В обоих случаях показанием к операции было наличие зрительных нарушений. Результаты. Несмотря на выраженные нарушения анатомии костей основания черепа, нам удалось получить доступ к турецкому седлу у обоих пациентов. Удаление опухоли гипофиза ничем не отличалось от стандартной операции. Улучшение зрения после операции отмечено у обоих больных. Опухоли удалены субтотально, клинико-биохимическая ремиссия акромегалии не достигнута. В обоих случаях опухоли имели морфологические признаки атипии. Дальнейшее лечение включало аналоги соматостатина и лучевую терапию. Выводы. Показана возможность выполнения трансназальной операции у данной категории больных с использованием специальных инструментов и интраоперационной навигации.

Introduction. Pituicytomas and spindle cell oncocytomas (SCO) are very rare benign tumors of sellar and suprasellar region (grade I). In case of these tumors, the clinical symptoms and the results of neuroimaging are not specific, so it is almost impossible to diagnose the pituicytoma or SCO preoperatively. The feature of these tumors is ample blood supply which results in intense intraoperative bleeding. Such events on background of high density and infiltrative growth of the tumors make a radical resection difficult. Objective is to present our own experience in surgical treatment and perioperative management of 8 SCO and 5 pituicytomas and compare it with the data of the scientific literature. Materials and methods. The retrospective study included 8 patients with SCO and 5 patients with pituicytomas that underwent endoscopic endonasal transsphenoidal removal in 2012–2017. Also, the literature data on pituicytoma and SCO were analyzed. Results. Total resection of SCO was successful in 4 cases, subtotal resection was done in 2 cases, and partial resection was done in 2 cases. All pituicytomas were resected extensively. Relapse of SCO was found in 4 patients. Postoperative complications (visual disorders and hemorrhagic complications) occurred in 2 SCO patients and in 1 pituicytoma patient. Postoperative improvement was seen in 3 patients. Conclusion. Optimal treatment method for pituicytoma and SCO of sellar and suprasellar region is a total resection using endoscopic endonasal access. The prognosis is favorable if the resection was total, however, if the resection was not extensive a relapse of the tumor can occur, and repeated operation or radiation therapy can be required. In case of slow asymptomatic progression of the tumor, an observation can be continued.

Background. The pituitary stalk (PS) is an anatomical structure consisting of the portal vessel system and axons of the hypothalamic nuclei terminating in the posterior pituitary lobe. Surgical injury or compression (by a tumor or another space-occupying process) of the PS can lead to hypopituitarism, diabetes insipidus, and hyperprolactinemia. However, the literature lacks studies on the extent of these disorders depending on PS injury or compression in clinical practice. Aim. The study aim was to investigate pre- and postoperative endocrine disorders in patients with chiasmo-sellar region (CSR) tumors and the PS compressed and preserved or involuntarily transected during neurosurgery. Material and methods. The PS compressed before surgery was preserved in 82 patients (41 patients with non-functioning endosuprasellar adenoma and 41 — with suprasellar meningioma). The PS was transected during transcranial surgery in 57 patients (46 patients with pituitary stalk craniopharyngioma and 11 patients with non-functioning endosupresellar pituitary adenoma). All patients underwent blood tests for prolactin (PRL), TSH, LH, FSH, free T4, cortisol, testosterone, or estradiol levels before and 6 months after surgery. Results. Hyperprolactinemia was detected in 37.4% of patients with CSR tumors compressing the PS. Elimination of PS compression led to normalization of the PRL level in most patients and was not accompanied by worsening of hypopituitarism symptoms. Transection of the PS resulted in panhypopituitarism in 100% of patients and diabetes insipidus in 93% of cases. There was no evidence of hyperprolactinemia in 58.7% of patients with craniopharyngiomas and 81.9% of patients with non-functioning pituitary adenomas. Conclusion. Given the difference in symptoms, we distinguished two syndromes: PS compression syndrome and PS transection syndrome. Syndrome of PS compression by a CSR tumor was characterized mainly by hyperprolactinemia (37.4% of cases); elimination of PS compression due to tumor resection led to normalization of the PRL level in most patients and was not accompanied by worsening of hypopituitarism symptoms. Syndrome of surgical PS transection in patients with craniopharyngioma (CP) and non-functioning pituitary adenoma (NFPA) manifested as panhypopituitarism in all patients and as permanent diabetes insipidus in most of them. The causes for the absence of hyperprolactinemia in many patients with the transected PS require further research. We can not exclude both adenohypophysis ischemia (due to its impaired blood supply) with partial or complete atrophy of lactotrophic cells and pituitary revascularization with restoration of dopamine transport.

The key structure in the regulation of energy homeostasis is the hypothalamus. The damage of the hypothalamic region can lead to imbalance of energy exchange with the development of obesity or cachexia. The most common metabolic disorders occur in case of craniopharyngiomas. The article presents two clinical observations of papillary craniopharyngioma in young women. Cases were accompanied by different disturbances of the energy balance, in one - with the development of obesity, in the other - cachexia.

Cerebrospinal fluid (CSF) leak is a rare complication in of the prolactinomas treatment with dopamine agonists. In most cases CSF leak develops within the first three months of treatment starting. The article presents a rare clinical case – later development of CSF leak after pharmacological treatment of giant prolactinoma. Women 29 years with giant endo-supra-infra-laterasellar pituitary adenoma (8,7 cm in diameter), visual impairment, cachexia, secondary amenorrhea and prolactin level more than 2 million 200 thousand mU/l treated with cabergoline. On this background, complete regression of the tumor, recovery of visual function, body mass and normalization of prolactin level was marked. Nevertheless, after 6.5 years after beginning of cabergoline therapy CSF leak occured, which required endoscopic endonasal surgery with the plastic of a CSF fistula. All patients with large and giant prolactinomas which invade into skull base structures, receiving cabergoline even for a long time, should be aware of the possibility of such complications as CSF leak and, if necessary, should urgent appeals to the otolaryngologist and the neurosurgeon. In this case closure of skull base defect is a main tactics of treatment.

Background: Central nervous system (CNS) aspergillosis is more often met in patients with expressed immune suppression. Still, in 50% of cases of meningitis caused by Aspergillus spp., it is observed in patients without expressed immune suppression. The prognosis of CNS aspergillosis is unfavorable with the general rate of lethality around 70%. Case Description: Clinical case of a 58-year-old man who developed an Aspergillus abscess in the chiasmosellar region and an associated mycotic aneurysm of the right middle cerebral artery (MCA) and intracerebral hemorrhage. Microsurgical clipping of the fusiform-ectatic aneurysm of the right MCA in the conditions of rupture was performed. An extra-intracranial micro anastomosis was formed on the right. An open biopsy of the neoplasm in the chiasmosellar region was made. The neoplasm was yellow and destroyed the bone plate of the skull base. Biopsy results: Mycotic lesion (aspergillosis). The analysis of surgical treatment for mycotic aneurysms in the acute period of hemorrhage in patients with aspergillosis revealed a high rate of lethality. The issue of the feasibility and effectiveness of complicated revascularization interventions in the patients with hemorrhage and aspergillosis remains unsolved. Conclusion: The lack of generally accepted tactics of the treatment of this pathology requires further studies and systemic analysis. A high risk of the lethal outcome in patients with invasive mycotic infection and rupture of mycotic aneurysm highlight the importance of timely diagnostics and the beginning of antimycotic therapy. WThe issue of the evaluation of the revascularization methods effectiveness in patients after surgical treatment of a mycotic aneurysm associated with cerebral aspergillosis remains poor.

Background The lateral extended transsphenoidal endoscopic approach (LETEA) is used to remove tumors located lateral to the cavernous segment of the internal carotid artery under direct visual control and provides access to Meckel cave, pterygopalatine fossa, medial part of the middle cranial fossa, and orbit. We describe an extended transsphenoidal approach to the amygdalohippocampectomy through the pterygopalatine fossa. Methods The LETEA to the middle cranial fossa through the pterygopalatine fossa was studied on 3 injected human cadavers at the Burdenko Neurosurgery Institute in Moscow, Russia. Results LETEA and trepanation of the greater wing of the sphenoid bone allow access to the medial part of the middle cranial fossa. Medial segments of the temporal lobe (hippocampus and amygdala) and the temporal pole were removed under guidance of the 45° angled endoscope. Conclusions LETEA through the pterygopalatine fossa is minimally invasive and provides direct access to the temporal pole and medial part of the temporal lobe. This approach may reduce risk of neurologic deficit and help to avoid cosmetic defects in the frontotemporal region associated with injury to temporal muscle and facial nerve injury as can occur during transcranial approaches. Disadvantages that limit application of LETEA include risk of cerebrospinal fluid leak and skills needed for manipulation in a narrow and deep surgical field with angled 30° and 45° endoscopes.

In the article the authors presented a modified score for the evaluation of the management of adult patients with craniopharyngioma. Its application allows to estimate dynamics of patients before and after surgery, and in following-up observation. The main advantage of the score is the possibility of its application in patients with impaired self-esteem, which is very important in cases of craniopharyngioma. The application of the proposed scale is possible in scientific research on groups of patients, during the medical-social examination and evaluation of the effectiveness of rehabilitation

Introduction. Pituitary adenomas comprise 10–15 % of all intracranial tumors. Despite being benign tumors, 25–55 % of them exhibit invasive growth spreading into surrounding structures (sphenoid sinus, cavernous sinus, etc.). In literature, there isn’t a generally accepted definition of aggressive pituitary adenomas. As a result, there aren’t any studies reporting on the therapy of choice for this disease, except for several reports on using temozolomide as a “salvage” therapy in patients who didn’t respond to standard treatment methods.We present a clinical observation of a patient with “refractory” pituitary adenoma who successfully underwent 4 surgical interventions and 2 courses of high-dose stereotactic radiotherapy.Results. Application of stereotactic radiotherapy at high (59.4 Gy) dose, which is usually used for treatment of patients with malignant tumors, allowed to achieve long recurrence-free period of 5.5 years (65 months). Observation has shown that repeated courses of stereotactic irradiation can be sufficiently effective and safe in patients with large and gigantic tumors including “refractory” pituitary adenoma.Conclusion. Further studies are necessary for specification of parameters of “refractory” adenomas and their detection at the early stages of diagnosis. Confirmed diagnosis requires more aggressive surgical, radiological, and drug treatment.

Background Despite the rarity of epidermoid cysts in the chiasmatic region, their surgical treatment is particularly complicated because of the tendency toward massive dissemination of the epidermoid masses along cerebrospinal fluid pathways and significant deviation of the tumor from the midline. Objective The purpose of the present work is evaluation of the role of extended transsphenoidal endoscopic endonasal approaches in the surgery of epidermoid cysts. Methods The study included 6 patients with epidermoid cysts in the chiasmatic region who were operated on at the Burdenko Neurosurgery Institute in the last 5 years using an anterior extended transsphenoidal endoscopic endonasal approach. Results The epidermoid masses were totally removed in 5 patients, but in no patient was it possible to completely remove the epidermoid cyst capsule. Visual deterioration was not noted in any patient, nor did new focal neurologic symptoms appear. One of the patients developed hypopituitary disorders in the postoperative period. No recurrence of the epidermoid cysts was observed during the follow-up period. Conclusions Removal of epidermoid cysts in the chiasmatic region using an anterior extended transsphenoidal endoscopic approach may be an alternative to transcranial microsurgery operations. This technique is widely accepted as an approach to this area.

Summary A 32-year-old woman presented with primary amenorrhoea, prolactin (PRL) level of 154 150 mIU/L and was diagnosed with a giant pituitary adenoma measuring maximum 6.2 cm. Cabergoline (CAB) treatment at a dose of 0.5 mg/week was prescribed to the patient. The treatment decreased the tumour size after 3 months (MRI scans of the brain) and brought back to normal the level of the PRL (345 mIU/L) after 6 months of CAB treatment. After 7 months of CAB treatment, menarche was achieved, and after 12 months, the patient became pregnant. She discontinued taking CAB at 4-week gestation. The pregnancy resulted in a missed miscarriage at 6–7 weeks; an abortion was conducted by the vacuum aspiration method. The MRI scans of the brain did not show any tumour enlargement. After 18 months from the start of the treatment the patient got pregnant for the second time. At 25-week gestation an MRI scan of the brain was conducted which did not show any increase in the tumour size. At 38 weeks the patient delivered a healthy full-term girl via C-section. The patient chose not to breastfeed and resumed CAB therapy after the delivery. During the treatment, the PRL level returned to the normal range and the menstrual cycle was restored. After 3 years the patient got pregnant for the third time. The patient did not receive CAB during the pregnancies; the examination did not show any tumour enlargement. Further MRI scans did not show any tumour growth. CAB therapy was effective in normalization of the PRL level, tumour shrinkage, menarche and pregnancy-induction which led to the birth of healthy children in a woman with primary amenorrhoea and a giant prolactinoma invading the skull base bones. Learning points: Giant prolactinomas are very rarely found in women. Cabergoline therapy can be effective in the normalization of the PRL level, tumour shrinkage, menarche induction in a woman with primary amenorrhoea, and giant prolactinoma. Cabergoline therapy can be effective in pregnancy induction which leads to the birth of children in a woman with giant prolactinoma. Cabergoline discontinuation did not trigger tumour enlargement during pregnancy.

Oculomotor nerve neurinoma not associated with neurofibromatosis type II is an extremely rare pathology. According to the topography, cisternal, cisternocavernous, cavernous, orbitocavernous, and orbital tumor groups are distinguished. The clinical picture of the disease is characterized mainly by either oculomotor disorders or pyramidal symptoms, depending on the tumor localization. Neurinomas of the oculomotor nerve rarely occur without oculomotor disorders. However, in some patients with these tumors, the third nerve function remains intact. In this paper, we present clinical cases of two patients with oculomotor nerve neurinomas and analyze the relevant literature.Невринома глазодвигательного нерва при отсутствии нейрофиброматоза II типа является крайне редкой патологией. По топографии выделяют цистернальные, цистернально-кавернозные, кавернозные, орбитально-кавернозные и орбитальные опухоли. В клинической картине заболевания преобладают глазодвигательные нарушения, реже они дополняются другой симптоматикой. Невриномы глазодвигательного нерва редко протекают без глазодвигательных нарушений. Однако в некоторых наблюдениях функция III черепного нерва может оставаться сохранной. В нашей работе представлены клинические наблюдения двух пациентов (один из которых оперирован) с невриномами глазодвигательного нерва и проведен анализ литературы по данной проблеме.

Cases of thyrotoxicosis associated with a previous case of secondary hypothyroidism are extremely rare. This article presents a rare clinical case of Graves' disease manifestation in a patient with secondary hypothyroidism after radiosurgical treatment of acromegaly. A 38-year old woman presented with acromegaly and endo-supra-laterosellar pituitary adenoma. After non-radical removal of the pituitary adenoma, radiosurgical treatment of the of the residual tissue of the pituitary tumor in the cavernous sinus area was performed. After 14 months of radiation therapy, the acromegaly was in remission; after 24 months of radiation therapy, panhypopituitarism developed (secondary hypothyroidism, adrenal insufficiency, hypogonadism, and growth hormone deficiency). Furthermore, 1.5 years after the panhypopituitarism was diagnosed, the manifestation of Graves' disease was also noted, requiring thyrostatic and radioactive iodine treatments. Diagnostic criteria for secondary hypothyroidism are low levels of the thyroid hormones free T4 and free T3, with a reduced, normal or slightly elevated level of thyroid stimulating hormone (TSH). The criterion for the development of thyrotoxicosis in the context of the secondary hypothyroidism was the persistent increase in the level of free T4 despite adequate drug therapy with levothyroxine. In the case report, the patient's diagnosis of Graves' disease was confirmed by the presence of a high level of antibodies to the TSH receptor.Случаи манифестации тиреотоксикоза на фоне предшествующего вторичного гипотиреоза крайне редки. В статье представлено клиническое наблюдение манифестации диффузного токсического зоба у пациентки с вторичным гипотиреозом, развившимся после радиохирургического лечения акромегалии. Пациентке 38 лет с акромегалией и эндо-супра-латероселлярной аденомой гипофиза после нерадикального удаления было проведено радиохирургическое лечение остаточной ткани опухоли гипофиза в области кавернозного синуса. Через 14 мес достигнута ремиссия акромегалии, через 24 мес после лучевого лечения развился пангипопитуитаризм (вторичный гипотиреоз, гипокортицизм, гипогонадизм, СТГ-дефицит). Однако через 1,5 года после диагностики пангипопитуитаризма была отмечена манифестация диффузного токсического зоба, что потребовало тиреостатической терапии и радиойодтерапии. Диагностическими критериями вторичного гипотиреоза является низкое содержание тиреоидных гормонов (св. Т4 и св. Т3) при сниженной, нормальной или слегка повышенной концентрации ТТГ. Критерием развития тиреотоксикоза на фоне вторичного гипотиреоза было стойкое повышение концентрации св. Т4 на фоне адекватной терапии левотироксином. Выявление высокой концентрации антител к рецептору ТТГ в описанном случае подтвердило манифестацию диффузного токсического зоба.

Surgery of suprasellar meningiomas is a challenge and associated with a high risk of injury to the vascular-neural structures lying along the approach and surrounding the tumor. Currently, many foreign clinics and our Center have introduced a technique for resection of suprasellar meningiomas through the anterior extended transsphenoidal endoscopic endonasal approach.The study objective was to evaluate the role of extended transsphenoidal endoscopic endonasal approaches in surgery of suprasellar meningiomas.The present study is a retrospective analysis of surgical treatment outcomes in 45 patients (11 males and 34 females aged 23 to 70 years (median, 50 years) with suprasellar meningiomas who underwent surgery for skull base tumors using the anterior extended transsphenoidal endoscopic endonasal approach in the Surgery Department of the Burdenko Neurosurgical Institute in the period from 2009 to 2017. In all cases, surgery was the primary method of treatment.Total tumor resection (the tumor was resected completely together with an infiltrated DM - Simpson 1) was achieved in 77.8% (35/45) of cases; subtotal resection (more than 80% of the tumor was removed) was in 17.8% (8/45) of cases; in 4.4% (2/45) of cases, resection was partial (less than 80% of the tumor was resected). Worsening or development of visual impairments immediately after surgery occurred in 13 (28%) of 45 patients. In 3 of them, vision was completely recovered on conservative treatment by the time of hospital discharge. In 4 patients, vision partially improved by the time of discharge. In 6 patients, vision was not recovered by discharge (in 2 of them, vision partially improved during follow-up). Therefore, by the time of hospital discharge, deterioration in vision occurred in 10 (22%) of 45 patients. In 7 (21.2%) out of 33 patients who had visual impairments before surgery, there was an improvement in vision in the postoperative period.Analyzing the findings and generalizing our experience, we may say that, in surgery of suprasellar meningiomas, the anterior extended transsphenoidal endoscopic endonasal approach should be used for relatively small (up to 3 cm), medially located symmetrical tumors that do not involve large vessels. The efficacy of this technique for tumors extending into the optic nerve canals requires additional analysis in a larger series of cases.Хирургия супраселлярных менингиом представляет особую сложность и сопряжена с высоким риском повреждения сосудисто-нервных структур, лежащих по ходу доступа и окружающих опухоль. В настоящее время во многих зарубежных клиниках и нашем Центре внедрена методика удаления супраселлярных менингиом с использованием переднего расширенного транссфеноидального эндоскопического эндоназального доступа. Цель исследования - оценка роли расширенных транссфеноидальных эндоскопических эндоназальных доступов в хирургии супраселлярных менингиом. Материал и методы. Настоящее исследование представляет собой ретроспективный анализ результатов хирургического лечения 45 пациентов (11 мужчин и 34 женщины, возраст пациентов составил от 23 до 70 лет, медиана 50 лет) с супраселлярными менингиомами, оперированных в отделении хирургии опухолей основания черепа НМИЦ нейрохирургии им. акад. Н.Н. Бурденко с использованием переднего расширенного транссфеноидального эндоскопического эндоназального доступа за период с 2009 по 2017 г. Во всех случаях оперативное вмешательство было первичным методом лечения. Результаты. Тотальное удаление опухолей (опухоль удалена полностью вместе с инфильтрированной ТМО - Simpson 1) было достигнуто в 77,8% (35/45) случаев, субтотальное (удалено более 80% опухоли) - в 17,8% (8/45), и в 4,4% (2/45) удаление было расценено как частичное (удалено менее 80% опухоли). У 13 (28%) из 45 пациентов сразу после операции отмечено нарастание или появление зрительных нарушений. У 3 из них зрение полностью восстановилось к выписке из стационара на фоне проводимого консервативного лечения. У 4 пациентов к моменту выписки зрение улучшилось частично. У 6 пациентов зрение не восстановилось к выписке (в катамнезе у 2 из них частично улучшилось). Таким образом, к моменту выписки из стационара ухудшение зрения отмечалось у 10 (22%) из 45 пациентов. У 7 (21,2%) пациентов из 33, имевших зрительные нарушения до операции, в послеоперационном периоде отмечалось улучшение зрения. Заключение. Анализируя полученные результаты и обобщая наш опыт, можем сказать, что передний расширенный транссфеноидальный эндоскопический эндоназальный доступ в хирургии супраселлярных менингиом целесообразно применять при относительно небольших (до 3 см), срединно расположенных симметричных опухолях без обрастания крупных сосудов. Эффективность данной методики при распространении опухоли в каналы зрительных нервов требует дополнительного анализа на большей серии наблюдений.

One of the most serious/potentially fatal complications of transsphenoidal surgery (TSS) is internal carotid artery (ICA) injury. Of 6230 patients who underwent TSS, ICA injury occurred in 8 (0.12%). The etiology, possible treatment options, and avoidance of ICA injury were analyzed. ICA injury occurred at two different stages: (1) during the exposure of the sella floor and dural incision over the sella and cavernous sinus and (2) during the resection of the cavernous sinus extension of the tumor. The angiographic collateral blood supply was categorized as good, sufficient, and nonsufficient to help with the decision making for repairing the injury. ICA occlusion with a balloon was performed at the injury site in two cases, microcoils in two patients, microcoils plus a single barrel extra-intracranial high-flow bypass in one case, stent grafting in one case, and no intervention in two cases. The risk of ICA injury diminishes with better preoperative preparation, intraoperative navigation, and ultrasound dopplerography. Reconstructive surgery for closing the defect and restoring the blood flow to the artery should be assessed depending on the site of the injury and the anatomical features of the ICA.

Cushing's disease is a severe neuroendocrine disorder caused by chronic hypersecretion of ACTH by pituitary adenoma (corticotropinoma). Surgical resection is a gold standard for this disease resulting stable remission in about 65-95% of cases. Despite benign nature of corticotropinoma, recurrence rate is still higher (25-35%) even in specialized neurosurgical centers. Modifications in surgical strategy can prolong recurrence-free period.Болезнь Иценко—Кушинга — тяжелое нейроэндокринное заболевание, вызванное хронической гиперпродукцией адренокортикотропного гормона кортикотрофной опухолью гипофиза (кортикотропиномой). Ее хирургическая резекция остается «золотым стандартом» в лечении болезни Иценко—Кушинга и приводит к ремиссии в 65—95% случаев. Несмотря на доброкачественную в основном природу заболевания, даже в специализированных нейрохирургических центрах частота рецидива составляет 25—35% случаев. Усовершенствование способов хирургического удаления кортикотропином может позволить повысить вероятность длительной ремиссии заболевания.

Objective The endoscopic extended transsphenoidal approach for suprasellar craniopharyngiomas may be a really alternative to the transcranial approach in many cases. The authors present their experience with this technique in 136 patients with craniopharyngiomas. Methods From the past 7 years 204 patients with different purely supradiaphragmatic tumors underwent removal by extended endoscopic transsphenoidal transtuberculum transplanum approach. Most of the patients (136) had craniopharyngiomas (suprasellar, intra-extraventricular). The patients were analyzed according to age, sex, tumor size, growth and tumor structure, and clinical symptoms. Twenty-five patients had undergone a previous surgery. The mean follow-up was 42 months (range, 4–120 months). The operation is always performed with the bilateral endoscopic endonasal anterior extended transsphenoidal approach. Results A gross-total removal was completed in 72%. Improvement of vision or absence of visual deterioration after operation was observed in 89% of patients; 11% had worsening vision after surgery. Endocrine dysfunction did not improve after surgery, new hypotalamopituitary dysfunction (anterior pituitary dysfunction or diabetes insipidus) or worsening of it was observed in 42.6%. Other main complications included transient new mental disorder in 11%, temporary neurological postoperative deficits in 3.7%, bacterial meningitis in 16%, cerebrospinal fluid leaks in 8.8%. The recurrence rate was 20% and the lethality was 5.8%. Conclusions Resection of suprasellar craniopharyngiomas using the extended endoscopic approach is a more effective and less traumatic technology, able to provide resection of the tumor along with high quality of life after surgery, and relatively rare postoperative complications and mortality.

Sinonasal malignant tumors are characterized by high histological variability and complexity of the differential diagnosis. Currently, there are classifications of these tumors, which are based on their localization and involvement of various anatomical structures. However, generally accepted algorithms for treatment of this pathology have not yet been developed. This review describes the most important algorithms for treatment of the most common histological variants of sinonasal malignant tumors: squamous cell carcinoma, adenocarcinoma, sinonasal undifferentiated carcinoma, esthesioneuroblastoma, adenoid cystic cancer, and sinonasal adenocarcinoma. The main problems in choosing the approach for treating these tumors are the lack of generally accepted resectability criteria and contradictions between oncological and neurosurgical indications for surgical treatment. Further research is needed to study the role of radiosensitizers and radioprotectors in comprehensive treatment of sinonasal malignant tumors.Синоназальные злокачественные опухоли отличаются большой гистологической вариабельностью и сложностью дифференциальной диагностики. На сегодняшний день предложены их классификации, учитывающие локализацию и вовлечение различных анатомических структур. Однако общепринятые алгоритмы лечения этой патологии пока не разработаны. В данном обзоре рассмотрены наиболее актуальные алгоритмы лечения самых распространенных гистологических вариантов синоназальных злокачественных опухолей: плоскоклеточного рака, аденокарциномы, синоназальной недифференцированной карциномы, эстезионейробластомы, аденокистозного рака, синоназальной аденокарциномы. Основными проблемами при выборе тактики лечения таких опухолей являются отсутствие общепринятых критериев резектабельности и противоречия, возникающие между онкологическими и нейрохирургическими показаниями к хирургическому лечению. Необходимы дальнейшие исследования для изучения роли радиосенсибилизаторов и радиопротекторов в комплексной терапии синоназальных злокачественных опухолей.

According to previously accepted criteria, pituitary microadenoma is characterized by a diameter of less than 10 mm. Improvement and widespread use of MRI are accompanied by increased incidence of diagnosis of these neoplasms. Pituitary microadenomas is an extremely heterogeneous group of tumors with different biological behavior, endocrine secretion and clinical symptoms despite the common MR characteristics. Treatment is mainly determined by endocrine secretion. Endocrine-active microadenoma requires medication (in case of microprolactinoma) and surgical treatment (in case of microsomatotropinoma and ACTH-releasing tumor). Follow-up is advisable for endocrine-inactive microadenoma (microincidentaloma). Modern data on the incidence, clinical and endocrine features, diagnosis and treatment of various pituitary microadenomas are discussed in the article.Микроаденомы гипофиза — это аденомы, диаметр которых по ранее и давно принятым критериям не превышает 10 мм. Вследствие совершенствования и более широкого распространения аппаратов магнитно-резонансной томографии (МРТ) в настоящее время возрастает частота выявления этих опухолей. Несмотря на общность характеристик микроаденом, по данным МРТ, это крайне неоднородная группа опухолей, различающаяся биологическим поведением, гормональной продукцией и соответствующей клинической симптоматикой. Тактика лечения определяется, преимущественно, гормональной продукцией. При выявлении гормонально-активной микроаденомы показано проведение медикаментозного (в случае микропролактином) и хирургического (в случае микросомато- и кортикотропином) лечения. При гормонально-неактивной микроаденоме (микроинциденталоме) признано целесообразным динамическое наблюдение. В статье приведены современные данные о частоте выявления, клинических и гормональных особенностях, диагностике и тактике лечения различных микроаденом гипофиза.

To clarify the concept of 'aggressive pituitary adenoma' using analysis of the current concepts, as well as to determine the optimal treatment algorithm for this disease and the place of chemotherapy in this treatment. Pituitary adenomas comprise from 10 to 15% of intracranial neoplasms. Despite the fact that pituitary adenomas are benign neoplasms, in 25-55% of cases they demonstrate invasive growth, growing into the surrounding structures (sphenoid sinus, cavernous sinus, etc.). Due to the lack of a standard definition of aggressive pituitary adenomas (due to the lack of clear criteria for this disease), there are no studies in the literature reporting optimal treatment for this group of patients, except for several publications describing the use of Temozolomide as palliative therapy.Аденомы гипофиза составляют от 10 до 15% внутричерепных новообразований. Несмотря на то что аденомы гипофиза являются доброкачественными новообразованиями, в 25-55% случаев они характеризуются инвазивным ростом, прорастая в окружающие структуры (пазуха основной кости, кавернозный синус и др.). Отсутствие стандартного определения агрессивных аденом гипофиза связано с тем, что нет четких критериев этого заболевания. Вероятно поэтому в литературе нет исследований, сообщающих об оптимальной терапии этой группы пациентов, кроме нескольких публикаций, описывающих использование препарата темозоломид в качестве паллиативной терапии. Цель обзора - уточнить понятие 'агрессивная аденома гипофиза' на основании анализа существующих на сегодняшний день представлений, а также определить оптимальную тактику лечения этого заболевания и место химиотерапии в этом лечении.

Nasal liquorrhea is a serious problem in surgery of skull base tumors, which is associated with a high risk of purulent-septic complications. This paper presents a case of successful repair of a cerebrospinal fluid fistula with an autologous platelet gel in the postoperative period after removal of meningioma of the anterior cranial fossa base, which was accompanied by a purulent-inflammatory complication in the surgical wound area.Назальная ликворея в хирургии опухолей основания черепа является серьезной проблемой, которая сопряжена с высоким риском развития гнойно-септических осложнений. В данной работе представлен пример успешной пластики ликворной фистулы аутологичным тромбоцитарным гелем в послеоперационном периоде после удаления менингиомы основания передней черепной ямки при гнойно-воспалительном осложнении в области операционной раны.

At present, pharmacological therapy of prolactinomas with dopamine agonists (DAs) is considered the treatment of choice. In most cases, giant prolactinomas respond to treatment with dopamine agonists and decrease in size during the first months of the treatment. One of the rare but dangerous complications of conservative treatment of prolactinomas with invasive growth is cerebrospinal fluid rhinorrhea.We present a retrospective analysis of 15 patients with macropropactinomas who underwent surgery for cerebrospinal fluid rhinorrhea developed due to primary therapy with dopamine agonists at the Burdenko Neurosurgical Institute (BNI) in the period between 2005 and 2015. All patients had large and giant tumors (according to the classification adopted at the BNI). When cerebrospinal fluid rhinorrhea was detected, patients were hospitalized to the BNI for examination, detection of a CSF fistula, reconstruction of a defect, and resection (if possible) of the tumor.In the period between 2005 and 2015, 15 patients (8 males and 7 females) with prolactinomas of a large and giant size at the onset of conservative therapy underwent surgery for cerebrospinal fluid rhinorrhea at the BNI. All patients underwent transnasal reconstruction of a skull base defect, with 13 out of 15 patients undergoing simultaneous resection of the tumor. After tumor resection, reconstruction was performed using auto-fat, fascia, and glue (in 8 cases). In the remaining cases, apart from auto-fat, fascia, and glue, a mucoperiosteal flap and auto-bone were used. Fourteen patients were followe-up. In 13 cases, there was no relapse of cerebrospinal fluid rhinorrhea after skull base reconstruction. In 1 case, there was a relapse of cerebrospinal fluid rhinorrhea.Conservative treatment of patients with giant prolactinomas should be performed under regular control of ENT doctors and neurosurgeons for timely detection and surgical treatment of cerebrospinal fluid rhinorrhea.В настоящее время фармакологическая терапия пролактином агонистами дофамина (АДs) считается методом выбора. Крупные пролактиномы в большинстве случаев реагируют на лечение АДs и уменьшаются в размерах в течение первых месяцев лечения. Одним из редких, но опасных осложнений консервативного лечения пролактином с инвазивным ростом является назальная ликворея. Материал и методы. Мы представляем ретроспективный анализ 15 пациентов с макропролактиномами, оперированных в НМИЦ нейрохирургии им. акад. Н.Н. Бурденко с 2005 по 2015 г. по поводу назальной ликвореи, возникшей на фоне первичной терапии АДs. Исходно все опухоли были больших (36-59 мм) и гигантских (≥60 мм) размеров (согласно классификации, принятой в ННПЦН). При выявлении назальной ликвореи пациенты госпитализировались в ННПЦН для обследования, выявления ликворной фистулы, пластики дефекта и удаления (по возможности) опухоли. Результаты. С 2005 по 2015 г. в ННПЦН было проведено хирургическое лечение по поводу назальной ликвореи 15 пациентам (8 мужчин, 7 женщин) с пролактиномами, которые имели большой и гигантский размер на момент начала консервативной терапии. Всем пациентам была выполнена трансназальная пластика дефекта основания черепа, из них в 13 из 15 случаев с одномоментным удалением опухоли. После удаления опухоли выполнялась пластика с использованием аутожира, фасции, клея (в 8 случаях). В остальных случаях помимо аутожира, фасции и клея были использованы мукопериостальный лоскут и аутокость. Катамнез прослежен у 14 пациентов. В 13 случаях рецидива назальной ликвореи после выполненной пластики основания черепа не наблюдалось. В 1 случае наблюдался рецидив назальной ликвореи. Выводы. Консервативное лечение пациентов с гигантскими пролактиномами должно проводиться под регулярным контролем ЛОР-врачей и нейрохирургов с целью своевременного выявления и хирургического лечения назальной ликвореи.

Stereotactic radiotherapy/radiosurgery (RT/ES) is an effective technique for treating craniopharyngiomas (CPs). However, enlargement of the cystic part of the tumor occurs in some cases after irradiation. The enlargement may be transient and not require treatment or be a true relapse requiring treatment.In this study, we performed a retrospective analysis of 79 pediatric patients who underwent stereotactic RT or RS after resection of craniopharyngioma.Five-year relapse-free survival after complex treatment of CP was 86%. In the early period after irradiation, 3.5 months (2.7-9.4) on average, enlargement of the cystic component of the tumor was detected in 10 (12.7%) patients; in 9 (11.4%) of them, the enlargement was transient and did not require treatment; in one case, the patient underwent surgery due to reduced visual acuity. In 8 (10.1%) patients, an increase in the residual tumor (a solid component of the tumor in 2 cases and a cystic component of the tumor in 6 cases) occurred in the long-term period after irradiation - after 26.3 months (16.6-48.9) and did not decrease during follow-up in none of the cases, i.e. continued growth of the tumor was diagnosed. A statistical analysis revealed that differences in the terms of transient enlargement and true continued growth were statistically significant (p0.01).Enlargement of a craniopharyngioma cyst in the early period (up to 1 year) after RT/RS is usually transient and does not require surgical treatment (except cases where worsening of neurological symptoms occurs, or occlusive hydrocephalus develops).Стереотаксическая лучевая терапия (ЛТ) и радиохирургия (РХ) являются эффективными методами лечения краниофарингиом (КФ). Однако в ряде случаев после облучения отмечается увеличение объема кистозной части опухоли, которое может иметь транзиторный характер и не требовать лечения, либо являться истинным рецидивом, требующим вмешательства. Материал и методы. В настоящем исследовании проведен ретроспективный анализ 79 пациентов детского возраста, получивших стереотаксическую ЛТ или РХ после удаления краниофарингиомы. Результаты. Пятилетняя безрецидивная выживаемость (БРВ) после комплексного лечения КФ составила 86%. У 10 (12,7%) пациентов в ранние сроки - в среднем через 3,5 мес (2,7-9,4 мес) - после облучения было выявлено увеличение кистозного компонента опухоли, у 9 (11,4%) из них оно было транзиторным и не требовало лечения, а в одном случае пациент был оперирован в связи со снижением зрения. У 8 (10,1%) пациентов увеличение остатка опухоли (у 2 - солидного и у 6 - кистозного компонента опухоли) появилось в поздние сроки после облучения - через 26,3 мес (16,6-48,9) - и ни в одном случае не уменьшалось в ходе наблюдения, т.е. имел место продолженный рост опухоли. При статистическом анализе выявлено, что различия в сроках транзиторного увеличения и истинного продолженного роста были статистически значимы (p0,01). Выводы. Увеличение кисты краниофарингиомы в ранние сроки (до 1 года) после ЛТ или РХ, как правило, является транзиторным и не требует хирургического лечения (за исключением случаев, в которых отмечается нарастание неврологической симптоматики или развитие окклюзионной гидроцефалии).

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.Под диэнцефальной кахексией (ДК) понимают прогрессирующее снижение массы тела, обусловленное опухолевым поражением гипоталамуса. Это редкое (в литературе описано около 100 случаев), но потенциально смертельное расстройство с неизвестным до сих пор патогенезом. В настоящее время не существует эффективных фармакологических методов его лечения. Регресс кахексии может отмечаться только на фоне уменьшения объема опухоли, поэтому своевременная диагностика и лечение становятся жизненно важными для пациента. Развитие ДК характерно для раннего детского возраста и лишь единичные случаи зарегистрированы у взрослых. Мы представляем редкое наблюдение - развитие ДК у женщины 24 лет с папиллярной краниофарингиомой.

Surgical treatment for epidermoid cysts of the chiasmatic region is a challenge because of the tendency to a massive spread of epidermoid masses through the cerebrospinal fluid pathways and a significant lesion deviation from the midline.To analyze capabilities of the extended endoscopic endonasal transsphenoidal approach in surgery for epidermoid cysts.The study included 6 patients with epidermoid cysts of the chiasmatic region who were operated on using the extended anterior endoscopic endonasal transsphenoidal approach at the Burdenko Neurosurgical Institute in the past 5 years.Epidermoid masses were completely removed in 5 patients; in none of the cases, complete removal of the epidermoid cyst capsule was achieved. There were no cases of vision deterioration and the development of new focal neurological symptoms. One female patient developed hypopituitary disorders in the postoperative period. There was no recurrence of epidermoid cysts during follow-up.Removal of epidermoid cysts of the chiasmatic region using the extended anterior endoscopic transsphenoidal approach may be an alternative to transcranial microsurgery.Проблема хирургического лечения эпидермоидных кист хиазмального расположения особенно сложна в связи с тенденцией к массивному распространению эпидермоидных масс по ликворным путям и значительному отклонению новообразования от средней линии. Цель исследования - изучить возможности применения расширенного транссфеноидального эндоскопического эндоназального доступа в хирургии эпидермоидных кист. Материал и методы. В исследование включены 6 пациентов c эпидермоидными кистами хиазмальной области, которые были оперированы в ННПЦН им. акад. Н.Н. Бурденко за последние 5 лет с использованием переднего расширенного транссфеноидального эндоскопического эндоназального доступа. Результаты. Полностью эпидермоидные массы удалены у 5 пациентов, ни в одном случае не удалось полностью удалить капсулу эпидермоидной кисты. Ни в одном случае не отмечено ухудшения зрения и появления новой очаговой неврологической симптоматики. У одной пациентки в послеоперационном периоде развились гипопитуитарные нарушения. Рецидивов эпидермоидных кист за время катамнестического наблюдения не наблюдалось. Заключение. Удаление эпидермоидных кист хиазмальной области с использованием переднего расширенного транссфеноидального эндоскопического доступа может использоваться как реальная альтернатива транскраниальным микрохирургическим операциям.

An increased blood level of the thyroid stimulating hormone (TSH) is usually associated with primary hypothyroidism (PHT) but can also be observed in such rare cases as TSH-secreting pituitary tumor. The article describes four clinical cases of elevated TSH blood levels: 1) TSH-secreting pituitary adenoma with hyperthyroidism; 2) TSH-secreting adenoma with hypothyroidism; 3) hormonally inactive pituitary adenoma combined with primary hypothyroidism; 4) reversible thyrotropic hyperplasia. These clinical situations substantiate the importance of considering different diagnoses in a patient with a pituitary gland tumor associated with an increased TSH blood level.Повышение уровня тиреотропного гормона (ТТГ) в крови, как правило, вызвано первичным гипотиреозом, но может наблюдаться и в таких редких случаях, как ТТГ-секретирующая опухоль гипофиза. В статье рассматриваются четыре клинических случая повышения уровня ТТГ в крови у пациентов: 1) ТТГ-секретирующая аденома гипофиза с гипертиреозом; 2) ТТГ-секретирующая аденома с гипотиреозом; 3) гормонально-неактивная аденома гипофиза в сочетании с первичным гипотиреозом; 4) случай обратимой тиреотрофной гиперплазии. Эти клинические ситуации обосновывают важность рассмотрения различных диагнозов у пациента с опухолью гипофиза, сопровождающейся повышением уровня ТТГ в крови.