[Modern aspects of surgery for cushing's disease]. / Sovremennye aspekty khirurgicheskogo lecheniya bolezni Itsenko­Kushinga.

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.

[Primary sellar neuroblastoma (clinical case and literature review)]. / Pervichnaya sellyarnaya neiroblastoma. Klinicheskoe nablyudenie i obzor literatury.

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.

[Transcranial microsurgical decompression of the optic canal in surgical treatment of meningiomas of the sellar region]. / Transkranial'naya mikrokhirurgicheskaya dekompressiya kanala zritel'nogo nerva v khirurgii meningiom khiazmal'no-sellyarnoi oblasti.

RATIONALE: When removing the meningiomas of the sellar region, there is always a risk of visual impairment for various reasons, in particular, as a result of traction damage to the optic nerve. Decompression of the optic canal increases nerve mobility during tumor manipulation. In cases of meningioma growing into the canal, its decompression often seems necessary. AIM: Evaluation of the effectiveness and risks of performing decompression of the optic canal. MATERIALS AND METHODS: The study included patients with meningiomas of the parasellar location, who underwent surgical treatment at the Burdenko Neurosurgical Center for the period from 2001 to 2017. They were divided into two groups - main and control. The main group consisted of 129 patients who underwent decompression of the optic nerve canals when the tumor was removed. The tumor matrix in this group was most often located in the region of the tuberum sellae, supradiaphragmally, in the region of the anterior clinoid process and the optic canal. In 31 cases, decompression was bilateral - during one operation and using one access in 27 patients; in 4 cases, the decompression of the second canal was delayed for 1.5-3 months after the first operation. 160 decompressions were performed by the intradural and 7 - by extradural methods. During intradural decompression, the roof of the optic canal was resected, and during extradural decompression, the lateral wall of the canal was trephined. The control group consisted of 308 patients who did not undergo canal decompression when the tumor was removed. It included meningiomas with a predominant location of the matrix in the area of the tuberclum and diaphragm of the sella. Tumors in both groups were removed according to the same principles (matrix coagulation, mainly the gradual removal of the tumor, the use of ultrasonic aspirator, a situational decision on the radicality of the operation, etc.). The main difference between operations in these two groups was only canal related algorithms (with or without its trepanation), as well as the probable prevalence of significant lateral tumor growth in cases with canal trepanation. Visual functions in the «primary¼ group were evaluated before and after operations with trepanation of the canal depending on various factors - the initial state of vision and the radicality of the tumor excision, including removal from the canal. The differences in the postoperative dynamics of vision in the main and control groups were studied. The primary data processing was carried out using the program MSExcel. Secondary statistical processing was carried out using the program Statistica. To assess the statistical significance of differences in the results obtained in the compared patient groups, the Chi-square test was used, and in the case of small groups - the exact Fisher test was applied. RESULTS: In the main group postoperative vision improvement of varying degrees on the side of trepanation was registered in 36.9% (59 out of 160) cases, no vision changes were found in 36.9% (59 out of 160), and in 26,2% (42 out of 160) the eyesight deteriorated. If preserving vision is attributed to a satisfactory result, then in general the results of these operations should be considered good. A comparative study of the results of removal of meningiomas with trepanation of the canals (main group) or without it (control group) was carried out among patients with the most critical vision situation (visual acuity 0.1 and below, up to only light perception). These groups are comparable in the number of observations - 62 and 73 respectively. The predominance of cases with improved vision in the main group compared with the control group (50.0% versus 38.36%) and a lower incidence of vision impairment (22.58% versus 34.25%) were found. However, the revealed differences are statistically unreliable and make it possible for us to talk only about the trend. The complications associated with trepanation of the canal include mechanical damage to the nerve by the drill. In our series of observations, there was only 1 case of abrasion of the nerve surface with the burr, which did not lead to a significant visual impairment. With the intradural method of trepanation in the area of the medial wall of the canal, the sphenoid sinus may open (in our series, in 34 cases out of 160 trepanations). Immediately closure of these defects was performed by various auto- and allomaterials in various combinations (pericranium, fascia, muscle fragment, hemostatic materials, and fibrin-thrombin glue). A true complication - CSF rhinorrhea liquorrhea developed in only one case, which required transnasal plastic surgery of the CSF fistula using a mucoperiostal flap. CONCLUSIONS: 1. Trepanation of the optic canal in cases of meningiomas of parasellar localization is a relatively safe procedure in the hands of a trained neurosurgeon and does not worsen the results of operations compared with the excision of the same tumors without trepanation of the canal. 2. The literature data and the results of our study make it possible to consider the decompression of the optic canal as an optional, but in many cases, useful option that facilitates the transcranial removal of some meningiomas of the sellar region.

[The use of platelet gel for repair of a cerebrospinal fluid fistula of the skull base (a case report and literature review)]. / Primenenie trombotsitarnogo gelia dlia plastiki likvornoi fistuly osnovaniia cherepa (sluchai iz praktiki i obzor literatury).

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.

[Li-Fraumeni syndrome in a patient with multiple anaplastic oligodendrogliomas of the brain (a case report and literature review)]. / Sindrom Li-Fraumeni u patsienta so mnozhestvennymi anaplasticheskimi oligodendrogliomami golovnogo mozga (klinicheskoe nabliudenie i obzor literatury).

Li-Fraumeni syndrome (LFS) is a clinically and genetically heterogeneous hereditary syndrome with predominantly oncological manifestations, which is associated with mutations in the TP53, MDM2, and CHEK2 genes. The most common variant is a TP53 mutation. OBJECTIVE: To analyze the literature and present a clinical case of a patient with Li-Fraumeni syndrome and multiple anaplastic oligodendrogliomas of the brain. CLINICAL CASE: A 42-year-old male patient presented with complaints of headaches, word finding difficulty, memory loss, right hemianopsia, and generalized convulsive attacks. For 10 years, he underwent multiple interventions and chemotherapy courses for colon adenocarcinoma and recurrent B-cell lymphoma. MRI revealed multiple space-occupying lesions of the cerebraln hemispheres, which were located in the left temporo-occipital and right frontal regions. RESULTS: The patient underwent resection of multiple space-occupying lesions of the left temporo-occipital and right frontal regions. The postoperative period proceeded without complications. The histological diagnosis was WHO grade III anaplastic oligodendroglioma. The patient and one of his sons were detected with a R248W missense mutation in the TP53 gene. The patient underwent six courses of temozolomide chemotherapy. At a follow-up examination 20 months after surgery and chemotherapy, the patient's condition was satisfactory; he returned to work. Control MRI of the brain revealed no signs of continued tumor growth. CONCLUSION: An analysis of the literature and the clinical case indicate the success of multiple surgical interventions and chemotherapy courses performed for a long time in the patient with Li-Fraumeni syndrome manifested by colon adenocarcinoma, recurrent B-cell lymphoma, and multiple anaplastic oligodendroglioma of the brain. The patient had a good quality of life and returned to professional activity.