Endoscopic endonasal surgery (EES) offers a direct ventral corridor to the skull base, potentially enabling maximal resection of extensive cholesteatomas (epidermoid cysts) in the posterior cranial fossa and parasellar region. However, outcomes and complications specific to this approach require detailed analysis. A retrospective review was conducted on 34 patients who underwent EES for cholesteatomas of the posterior fossa and parasellar region at a single center from 2011 to 2025 year. Extent of resection (near-total > 95
The endoscopic endonasal transsphenoidal approach (EETA) is recognized as an effective and safe method for the resection of pituitary and sellar/suprasellar tumors. Classified as a clean-contaminated procedure, EETA entails intraoperative exposure to the nasal cavity, thereby introducing a potential risk of contamination with Staphylococcus aureus. The procedure also involves the sphenoid sinus - an anatomical region whose microbiological profile is poorly characterized in the current literature. To determine microbial colonization patterns within the sphenoid sinus and their correlation with other anatomical sites for the preoperative identification of high-risk patients prior to EETA. This prospective study included patients who underwent EETA between December 2024 and February 2025. All patients underwent microbiological testing of nasopharyngeal swabs, sphenoid sinus mucosa biopsies, gastrointestinal tract specimens, and endotracheal aspirates obtained via the endotracheal tube. A cohort of forty-one patients with a mean age of 61 years, was enrolled in this study. Microbial colonization of the sphenoid sinus was highly prevalent, detected in 90
Background: Endoscopic endonasal surgery (EES) offers a direct ventral corridor to the skull base, potentially enabling maximal resection of extensive cholesteatomas (epidermoid cysts) in the posterior cranial fossa and parasellar region. However, outcomes and complications specific to this approach require detailed analysis. Methods: A retrospective review was conducted on 34 patients who underwent EES for cholesteatomas of the posterior fossa and parasellar region at a single high-volume center (2011-2025). Surgical approaches were tailored to the lesion's extent. Extent of resection (near-total >95%, subtotal 90-95%), complications, and neurological outcomes were analyzed. Results: Near-total resection was achieved in 65% of cases and subtotal in 35%. The most frequent complications were postoperative cerebrospinal fluid (CSF) leakage (23.5%) and meningitis (23.5%; 33.3% in patients with intradural extension). Rates were significantly higher than for other skull base tumors. Only visual symptoms showed consistent postoperative improvement. New or worsened cranial nerve deficits occurred in 12% of cases. The rates of CSF leakage and meningitis were 25% and 33%, respectively. Arrested hydrocephalus requiring shunting developed in 14.7% of patients. Conclusion: EES provides a valid alternative for the surgical management of posterior fossa and parasellar cholesteatomas, allowing for a high degree of resection. However, it is associated with a notably higher risk of CSF leakage and meningitis compared to EES for other pathologies, attributable to the lesion's adhesive and inflammatory characteristics. Furthermore, significant recovery of preoperative neurological deficits (except visual) is uncommon. These findings underscore that such complex procedures should be performed in specialized centers with extensive expertise in endoscopic skull base surgery and multidisciplinary perioperative care.
Combination of pituitary adenomas with meningiomas or vestibular schwannomas is a rare occurrence: in scientific literature, only individual cases or small case series are described. The article presents a rare clinical observation of a female patient who underwent surgery and radiotherapy due to hormonally inactive pituitary adenoma and neurofibromatosis type 2 (intracranial meningioma and neurinoma (schwannoma)) found during a follow-up exam. A combination of histologically different intracranial tumors (pituitary adenoma, meningioma, and neurinoma) is extremely rare and requires corresponding approaches to treatment.
Background. The term “aggressive pituitary adenoma” arose as a result of transforming the term “atypical pituitary adenoma”, which was used in WHO classification for pituitary adenomas with specifications of higher labeling index Ki-67 (≥3 %) and increased number of mitoses with expression of the p53 proteins. However, not all tumors with these features were aggressive, and currently, according to the European Society of Endocrinology Clinical Practice Guideline for the management of aggressive pituitary tumors and carcinomas (2018), aggressive pituitary tumors are labeled as tumors that have invasive and unusually rapid or clinically significant growth which occurs despite the use of standard treatment protocols (pharmacological, surgical and radiation). Till date, there are numerous papers in literature describing various schemes and indications for the use of temozolomide. The efficiency of ongoing therapy, according to different authors, varies from 29 to 81 %, with an average effectiveness ranging between 40–45 %. Aim. To present our own experience of using temozolomide in the treatment of patients with aggressive pituitary tumors. Materials and methods. Three patients were included in the study, one male (45 years old) and 2 females (61 and 29 years old). All three patients included in the study first underwent surgical resection followed by radiation therapy if no response / hormonal activity didn’t resume. The decision to start temozolomide protocol was held by concerned medical commission under supervision of local ethical committee. Results. All patients responded well to temozolomide therapy with decrease in the size of tumor and decrease in levels of prolactin in cases of prolactinoma. Conclusion. The most important step in treating patients with aggressive pituitary adenomas is their early diagnosis, which could be possible only if the specific identifying markers of aggressiveness were found, which unfortunately are not found yet.
According to the modern WHO classification, pituitary carcinomas (or metastatic neuroendocrine pituitary tumors) are pituitary tumors with confirmed craniospinal and/or distant metastases. The main goal of histological analysis of pituitary carcinomas is to confirm pituitary origin of metastases. Treatment usually includes surgery and radiotherapy, dopamine agonists in maximum possible doses in case of prolactin-secreting pituitary carcinomas and chemotherapy with preferable temozolomide. OBJECTIVE:To present the results of diagnosis and treatment of two patients with prolactin-secreting pituitary carcinomas. MATERIAL AND METHODS:The authors describe 2 patients with prolactin-secreting pituitary carcinomas arising from drug-resistant aggressive prolactinomas with histologically confirmed metastases. In both cases, combined treatment included surgery, radio- and chemotherapy (cabergoline and temozolomide). RESULTS:A 47-year-old patient underwent surgery, radio- and dopamine agonist therapy with subsequent regression of tumor growth in the follow-up period. However, progressive increase in prolactin concentration necessitated PET/CT with detection of multiple metastases in bones and lymph nodes. Temozolomide therapy led to temporary shrinkage of metastatic foci with subsequent progression. The second case was characterized by multiple brain and spinal cord metastases in a 47-year-old woman. Resection of intracranial metastasis and temozolomide therapy stabilized the disease and normalized serum prolactin throughout 2-year follow-up with subsequent progression. CONCLUSION:Pituitary carcinoma is a rare tumor with unfavorable prognosis. Treatment is currently not standardized and determined by available world experience regarding various chemotherapeutic drugs. Temozolomide is the most effective drug. However, short-term remission is usually followed by subsequent disease progression in most cases.
Craniopharyngioma is a benign tumor in adults and children characterized by local invasion of surrounding brain structures. HARDI-CSD tractography is one of the modern MR methods for comprehensive visualization of functionally significant conduction pathways. OBJECTIVE:To determine the possibility of visualizing some structures of chiasmatic-sellar region and third ventricle (hypothalamic-pituitary tract (pituitary stalk), optic pathways, fornix, mammillothalamic tract) using HARDI-CSD tractography in patients with suprasellar craniopharyngiomas, especially after failed CT and standard MRI of these structures. MATERIAL AND METHODS:A pilot study included 4 patients (2 adults and 2 children) with craniopharyngiomas who underwent surgical treatment at the Burdenko Neurosurgery Center between January and July 2024. Patients underwent MR tractography before and after surgery. RESULTS:HARDI-CSD tractography reconstructed optic tracts in 3 patients before and after surgery. Simultaneous visualization of several structures (pituitary stalk, fornix, optic tracts) was performed before and after surgery in 1 case. These data were useful for planning surgery and evaluating postoperative outcomes. In one patient, imaging of mammillothalamic tract was obtained after resection of large cystic intra-extraventricular craniopharyngioma. CONCLUSION:HARDI-CSD tractography provides valuable information about basal cerebral pathways (optic pathways, pituitary stalk, fornix, mammillothalamic tract) when planning and evaluating the results of surgical treatment of craniopharyngiomas.
Despite slow growth of most pituitary tumors and high rates of total resection and/or effective therapy, pituitary neoplasms are characterized by aggressive behavior with high growth rate, frequent relapses and resistance to standard treatments in 10% of cases. In modern WHO classifications of tumors of the central nervous system, endocrine and neuroendocrine tumors, the authors propose the definition «pituitary neuroendocrine tumor» instead of previous «pituitary adenoma» and «metastasizing pituitary neuroendocrine tumor» instead of «pituitary carcinoma». Currently, there are no effective prognostic markers of aggressive tumors. This complicates early diagnosis. It is proposed to apply a five-stage prognostic classification based on proliferation rate (including mitotic count, Ki-67 index and p53 immunoexpression) and morphometric markers of invasiveness for all resected pituitary neoplasms. This approach would be valuable for earlier detection of aggressive tumors and pituitary carcinomas. Compression of visual pathways, third ventricle and brain stem due to rapid growth of aggressive tumors usually requires redo surgeries with subsequent radiotherapy. Hormonally active tumors require therapy with somatostatin analogues and dopamine agonists in maximum possible doses. Chemotherapy with temozolomide as first-line option is recommended if standard treatment is ineffective. Alternative treatment includes peptide receptor radionuclide therapy (PRRT), molecular targeted therapy (bevacizumab, tyrosine kinase inhibitors, everolimus and cyclin-dependent kinase inhibitors) and immunotherapy (checkpoint inhibitors). Considering the need for combined treatment, these cases should always be discussed by a multidisciplinary team (neurosurgeon, endocrinologist, radiotherapist, oncologist, pathologist) with necessary qualifications and experience in treating these patients. Treatment of aggressive tumors and pituitary carcinomas is becoming an active and rapidly developing direction in neurosurgery, endocrinology and oncology.
Pituitary carcinoma (metastatic neuroendocrine tumor of the pituitary gland) is the pituitary tumor with confirmed craniospinal and/or systemic metastases. These tumors are extremely rare accounting for only 0.1% to 0.5% of all pituitary tumours and are characterized by high mortality. In the presented case, pituitary carcinoma with intracranial metastasis was diagnosed in a young patient 25 years after manifestation of an aggressive recurrent prolactin-secreting pituitary tumor. Standard therapy (removal of metastasis, radiation therapy, cabergoline therapy) resulted in a long-term remission of the disease.
Background. Cavernoma of cavernous sinus is a fairly rare benign neoplasm, accounting for less than 3 % of all neoplasms in this area. Due to the rarity of this pathology, a standardized protocol for diagnosis or treatment has not been developed. Surgical and radiosurgical methods are used for treatment.Aim. Analysis of the results of treatment of patients with cavernous sinus cavernomas operated at the N. N. Burdenko National Medical Research Center of Neurosurgery from 2000 to 2022 using endoscopic transnasal access.Materials and methods. We present our own experience in the treatment of 9 patients who underwent transnasal endoscopic removal of cavernous sinus cavernoma.Results. A total of about 300 cases of treatment of patients with cavernous sinus cavernomas have been described in the literature, and endoscopic removal has been described in only 12 cases.As a result, subtotal removal was achieved in 7 cases. In 2 cases, the removal was partial. The postoperative period proceeded in all patients without peculiarities and without complications.Conclusion. Endoscopic transnasal access allows partial or subtotal removal of the formation without risk of injury to cranial nerves and with minimal soft tissue injury, which has a positive effect on the postoperative period of patients. In combination with radiosurgical methods, it is possible to achieve satisfactory treatment results.
Craniopharyngioma (CP) is a benign epithelial tumor predominantly localized in chiasmatic-sellar region and third ventricle. There are two histological variants of CP: adamantinoma-like (ACP, 85%) and papillomatous (PCP). These types differ significantly in origin and histological structure. PCP predominantly occurs in adults. Treatment of CP, including effectiveness of radiotherapy, is described in multiple studies and mainly devoted to pediatric patients. OBJECTIVE:To clarify disease-free survival in adults with newly diagnosed CP depending on resection quality; to evaluate the effectiveness of stereotactic irradiation and treatment depending on histological characteristics of tumor. MATERIAL AND METHODS:We analyzed treatment outcomes in 398 adults over 10-year follow-up. Stereotactic irradiation was performed in 11.6% of patients. The follow-up data were obtained in 68.1% of patients. We compared 5-year disease-free survival rates after different resections with and without subsequent irradiation. RESULTS:Total resection is effective and provides significantly lower risk of CP recurrence. Effectiveness of stereotactic irradiation after incomplete resection was also confirmed. CONCLUSION:Total resection of ACP and PCP provides high disease-free survival rates. Stereotactic irradiation after incomplete resection is followed by similar outcomes. PCPs are characterized by less aggressive growth and do not recur after total resection.
We recommend acromegaly to be ruled in all patients with characteristic changes in appearance (A3). In all patients without characteristic changes in appearance, we recommend to rule out acromegaly, if several clinical signs suspicious for acromegaly are present (B3). We recommend acromegaly to be ruled out in all patients with pituitary adenoma (B3). We recommend to rule out acromegaly in all patients with prolactin-secreting adenomas (C4). For laboratory confirmation of acromegaly, we recommend to measure serum insulin-like growth factor-1 (IGF-1, somatomedin C) level (A3). In patients without obvious clinical signs of acromegaly and/or with a moderate increase in IGF-1 levels (IGF-1 index less than 1.3), we recommend to assess the response of somatotropic hormone (GH) to hyperglycemia (GH during an oral glucose tolerance test) (B3). In all patients with biochemically confirmed acromegaly, magnetic resonance imaging of the pituitary gland with intravenous contrast is recommended to assess pituitary adenoma size, location and growth pattern (A3). We recommend to measure blood glucose and glycated hemoglobin in all patients with confirmed acromegaly (B3). We recommend to measure serum prolactin levels in all patients with acromegaly (B3).In all patients with acromegaly and pituitary adenoma, transnasal transsphenoidal adenomectomy is recommended as first-line treatment if the patient’s consent is given and there are no contraindications (A1). For all patients with acromegaly for whom neurosurgical treatment is indicated, surgical intervention is recommended in medical centers, specializing in pituitary diseases, by neurosurgeons who perform at least 50 such operations per year (A3). We recommend that morphological and immunohistochemical examination of the removed pituitary adenoma is performed in all patients with acromegaly (A3). We do not recommend routine medical therapy for acromegaly before surgery as a mean to reduce tumor size (B2).For patients with acromegaly in whom neurosurgical treatment is not indicated or ineffective or while patient awaiting for the effect of radiation therapy, we recommend medical therapy (A1). Long-acting first-generation somatostatin analogues are recommended as first-line drug therapy for acromegaly. In case of partial/complete resistance to or intolerance to somatostatin analogs, pegvisomant is recommended as a second-line medical therapy for acromegaly (A1). We recommend starting pegvisomant from a single dose of 40–80 mg per day subcutaneously, then continue injections at a starting dose of 10 or 15 mg per day with IGF-1 monitoring after 4–6 weeks and dose adjustment if necessary (B2). In patients with acromegaly and a moderate increase in IGF-1 levels, we recommend to initiate cabergoline as medical monotherapy or, if somatostatin analogs are ineffective, in combination with them (B2).In patients with acromegaly, if the disease remains active after surgical treatment, if drug treatment is unavailable, intolerable or ineffective, and repeated neurosurgical intervention is inappropriate, it is we recommend radiation therapy (A1). A multidisciplinary approach is recommended in the management of acromegaly complications (C3).
The article presents an original method for personalized predicting surgical outcomes in patients with tumors of diencephalic and suprasellar localization based on a clinical and epidemiological approach and the use of intelligent analysis (Data Mining) of clinical and electroencephalo-graphic data. Use of the methods allowed us to programmatically select nosologically specific test for making surgical outcome prognosis, including sensitivity and specificity calculations.
Dysembryoplastic neuroepithelial tumor (DNET) is a benign mixed neuronal-glial neoplasm (WHO grade 1). DNET is most often localized in temporal lobes and found in children and young people with epilepsy. There a few cases of DNET in ventricular system with dissemination along the ependyma in the world literature. MATERIAL AND METHODS:We present a rare case of T1- and T2-negative ventricular system tumor. Only FIESTA imaging revealed dissemination with multiple focal lesions of the third ventricle, its bottom and lateral walls, anterior horns of lateral ventricles, cerebellar vermis, cervical and lumbar spinal cord. RESULTS:The patient underwent transcortical endoscopic biopsy of the third ventricle tumor with simultaneous ventriculoperitoneal shunting. DNET was diagnosed, and radiotherapy was subsequently performed. Literature data on this issue were analyzed. CONCLUSION:To date, disseminated forms of DNET are extremely rare. X-ray features and morphological results allow us to establish the correct diagnosis and determine further treatment strategy.
Craniopharyngiomas (CP) are benign chiasmatic-sellar tumors in children and adults. Surgical resection is currently the main method for CP and largely depends on topographic and anatomical location of tumor. OBJECTIVE:To analyze topographic and anatomical classifications of CP available in the world literature. MATERIAL AND METHODS:Searching for literature data on classifications of CPs was performed in the PubMed, eLibrary and CyberLeninka databases between 1960 and 2023. We used the following keywords: «craniopharyngioma», «third ventricle», «endoscopic transnasal surgery for craniopharyngiomas». RESULTS:We found 21 eligible manuscripts. The vast majority of studies are devoted to topographic and anatomical location of CPs in their own series: baseline growth along hypothalamic-pituitary axis (4 classifications), relation to the third ventricle (5 classifications), pituitary stalk (2 classifications), optic nerves (2 classifications) and meninges (3 classifications), classification of papillomatous CPs (1 report) and adamantinomatous CPs (1 report), functional classifications of hypothalamic lesions (3 reports). CONCLUSION:Topographic classifications of CPs characterize their relation to the most important basal brain structures: pituitary gland, hypothalamus, optic pathways and circle of Willis. There are also single topographic and anatomical classifications with emphasis on morphological component of CP. Exact anatomical and topographic variant of CP is often of decisive importance for choosing the optimal surgical approach, possibility and expediency of total resection. Further clarification of relationship of CP to diencephalic structures is associated with improvement of diagnostic methods, in particular, high-resolution MRI (7.0 T) and MR tractography.
Нейрохирургические операции при опухолях гипоталамо-гипофизарной области являются наиболее частой причиной центрального несахарного диабета и гипонатриемии вследствие синдрома неадекватной секреции антидиуретического гормона (СНСАДГ). Распространенность послеоперационного несахарного диабета составляет 13—30%, гипонатриемии у 3-25 %. Одно из редких и потенциально опасных для жизни форм несахарного диабета является его адипсический вариант. Он характеризуется центральным неса- харным диабетом и отсутствием нормальной реакции на жажду при гиперосмолярности. Потеря чувства жажды затрудняет диагностику и способствует развитию обезвоживания и тяжелой гипернатриемии. Это может проявляться выраженной цефалгией, гипертермией, тошнотой, рвотой, судорогами, психомоторным возбуждением, нарушением сознания. Другое редкое изменение водно-электролитного баланса – это синдром переустановки осмостата. В ли- тературе его часто описывают как один из типов аномального высвобождения вазопрессина при СНСАДГ. Секреция вазопрессина происходит при более низкой осмоляльности плазмы, чем обычно, вследствие чего происходит изменение нормального порога осмоляльности плазмы и развитие хронической дисна- триемии (гипо- или гипернатриемии). Чаще всего гипонатриемия имеет легкое течение без клинических проявлений. Мы наблюдали развитие адипсического несахарного диабета после эндоскопического трансназального транссфеноидального удаления стебельно-интравентрикулярной краниофарингиомы. На фоне терапии десмопрессином и адекватного восполнения жидкости в послеоперационном периоде была отмечена нормализация водно-электролитного баланса. Однако самостоятельное прекращение пациенткой терапии десмопрессином после выписки из стационара и отсутствие адекватного потребления жидкости на фоне полиурии привело к выраженной гипернатриемии (155-160 ммоль/л) и грубым психическим нарушениям. Синдром переустановки осмостата мы диагностировали у пациентки с хордоидной глиомой III желудоч- ка на основании хронической гипонатриемии до и после оперативного лечения, отсутствия гипотиреоза и гипокортицизма, сохранной фильтрационной и концентрационной функции почек, а также сохраняющей- ся гипонатриемией при попытках коррекции уровня натрия гипертоническим раствором и положительно- го теста с водной нагрузкой. Учитывая отсутствие клинических симптомов, связанных с гипонатриемией, медикаментозной коррекции не потребовалось. ВЫВОДЫ: водно-электролитные нарушения могут существенно ухудшать состояние пациентов, в част- ности, после удаления опухолей гипоталамо–гипофизарной области и требуют незамедлительного лече- ния. Однако не всегда требуется сведение лабораторных показателей в общепопуляционный интервал. Персонализированный подход к диагностике водно-электролитных нарушений позволит определить наиболее оптимальную тактику ведения пациентов.