Introduction. Due to variety of morphological subtypes and poor clinical response, comprehensive molecular profiling for salivary gland cancers become a part of treatment strategy for chemotherapy-resistant cases. Tumors of the salivary glands include more than 20 histological types distinguished by their biological characteristics, response to therapy, and prognosis. The search for specific therapeutically significant markers and mutations for various histological types of salivary gland tumors may allow the development of a diagnostic algorithm and improve patient treatment results.

Aim. To search for therapeutically significant molecular and genetic targets and immunohistochemical markers in various histological types of malignant salivary gland tumors.

Materials and methods. We analyzed data on 280 patients with unresectable recurrent and metastatic salivary gland cancer to identify the main characteristic therapeutically significant immunohistochemical (androgen receptor (AR), epidermal growth factor receptor type 2 (HER2neu), epidermal growth factor receptor (EgfR), programmed cell death receptor 1 (pD-L1) (CpS), сD117, estrogen receptor (ER), progesterone receptor (pR), pan-TRk) and molecular genetic targets (ALK, BRAF, ERBB2, KIT, MET, RET, ROS1, SMO, AR, HRAS, PIK3CA, PDGFRA, NTRK1-3) depending on the histological subtype of the tumor.

Results. Based on the results obtained, in comparison with previous studies, a greater diversity of therapeutically significant mutations, fusions and immunohistochemical markers was revealed for various histological types of tumors. Interestingly, increased expression of AR was detected not only in salivary duct carcinoma, but also in carcinoma ex pleomorphic adenoma, adenocarcinoma not otherwise specified, myoepithelial, mucoepidermoid, adenoid cystic, acinic cell, polymorphous adenocarcinomas. ETV6-NTRK3 gene fusions in addition to secretory carcinoma were detected in mucoepidermoid and salivary duct carcinomas. mutations and fusions in the RET gene have been identified in ductal and mucoepidermoid carcinomas, as well as adenocarcinoma not otherwise specified.

Conclusion. The results of the study allow to expand the group of patients for molecular genetic and IHC testing in order to individualize therapy.

Introduction. Segmental mandibulectomy with exarticulation and single-step reconstruction is an important problem of craniomaxillofacial surgery. Revascularized autotransplants (iliac crest, fibula free flaps) are used to reconstruct defects of the mandible and restore joint function.

Aim. To evaluate functional, esthetic and oncological results of fragmental mandibular resection with exarticulation and single-step reconstruction using bone flaps.

Materials and methods. The study included 52 patients who underwent segmental mandibular resection with exarticulation and single-step reconstruction using bone flaps. For defects of the ramus and head of the mandible, reconstruction was performed using iliac crest free flap (5 cases); defects of the ramus and body of the mandible, fibula free flap was used (47 cases).

Results. Total necrosis of the flap was observed in 1 (4.7 %) case. Iliac crest free flap was modelled taking into account the size of mandibular ramus defect (usually to the angle of the mandible), mandibular head was formed from the distal part. At least one osteotomy was preformed on the autotrasplant near the angle, on the distal part the head was formed. Follow-up period varied between 1 and 5 years. Disease progression in the form of recurrence was observed in 15 (34.9 %) cases, in the form of distant metastases in 6 (13.9 %) cases.

Conclusion. For small defects of the ramus of the mandible, revascularized iliac crest free flap should be used, for large defects the method of choice is fibula free flap. Temporomandibular joint can be formed from the distal end of a bone autotransplant with its subsequent adaptation during functional load.

Introduction. Neoplasms of the orbit and periorbital region are usually represented by tumors of the eyelid skin  skin of the periorbital region  and paranasal mucosa. The most common histological types of this pathology are squamous cell and basal cell carcinomas. The main indications for orbital exenteration are tumor lesions in the extraocular muscles and orbital apex and irreversible vision loss in the affected eye at the time of surgery planning. In the absence of these pathologies  organ-sparing surgery is recommended. Resection of orbital and periorbital tumors (including orbital exenteration) leads to cosmetic and functional defects  therefore surgical treatment of this area should be performed only if other methods are impossible or ineffective.

Aim. To analyze the experience of resection of orbital and periorbital tumors and to evaluate short- and long-term results of surgical treatment of this pathology.

Materials and methods. Retrospective analysis of patients with tumors of the orbit and periorbital region who underwent organ-sparing and exenteration surgeries between 2003 and 2023 was performed.

Results. Overall 5-year survival was 55 %. It varied depending on a number of factors. The best 5-year survival rates were observed for basal cell carcinoma of the eyelid skin and periorbital region (83 %)  the worst for squamous cell carcinoma (34 %) and melanoma (41 %). For localized tumors (Т1)  overall survival was 76 %  for locally advanced (Т4) – 43 %.

Conclusion. Orbital and periorbital neoplasms are characterized by a variety of tumor morphological types and locations which requires multidisciplinary approach to treatment and rehabilitation of patients with this pathology.

The article provides the latest data of our foreign colleagues on the potential use and dosing regimen of lenvatinib in real clinical practice. Since adverse events during lenvatinib treatment often lead to dose reduction or its discontinuation and, as a consequence, to a decrease in progression-free survival and overall survival benefit, optimization of lenvatinib dosing regimen is of great interest to experts from different countries. Today, so-called drug holidays are being actively studied as a possible option to reduce lenvatinib toxicity, without loss of efficacy in patients with advanced radioiodine-refractory differentiated thyroid cancer. In addition, given the small number of therapeutic options for this patient group, their low efficacy and poor availability of tumor mutation profiling and effect on target genes, a question arises: “Is it possible to benefit in progression-free survival and overall survival with lenvatinib rechallenge after disease progression in the 2^nd or 3^rd treatment lines?”

Nasopharyngeal carcinoma is a rare pathology. It develops in the nasopharyngeal mucosa and is characterized by aggressive (recurrent and metastatic) progression. Due to complicated location, this disease is often diagnosed at late stages and requires systemic therapy. Due to the rareness of nasopharyngeal carcinoma, until recently there were no studies on modern drug therapy of this pathology with high evidence level.

Aim. To describe the capabilities and mechanism of action of camrelizumab in nasopharyngeal carcinoma. The results of studies investigating the mechanism of action of camrelizumab were analyzed. The effectiveness of this drug in progression of nasopharyngeal cancer with previous chemotherapy and in combination with chemotherapy as the 1^st line treatment of recurrent and / or metastatic process was evaluated. Based on the phase II trial results, the use of camrelizumab in patients with disease progression during standard chemotherapy allows to achieve progression-free survival of 8.5 months. Data obtained in the phase III trial demonstrated clinical benefit of adding this drug to standard chemotherapy in the 1st line therapy of recurrent and / or metastatic nasopharyngeal carcinoma: median progressionfree survival significantly increased from 6.9 to 9.7 months.

Therefore, camrelizumab is a modern highly effective immunotherapeutic drug designed to treat patients with nasopharyngeal carcinoma.

Despite a significant amount of information on approaches to managing patients with craniofacial tumors, the increasing number of modern high-tech technologies, there remain a number of controversial issues regarding the choice of treatment strategy that fully satisfies the requirements of objective assessment of effectiveness and the improvement of quality of life for patients suffering from oncological disease. Craniofacial approaches sufficiently adhere to oncological principles and allow for the removal of even extensive tumors which greatly improve immediate and long-term outcomes, reliably enhancing quality of life, which is the most important task in the surgery of malignant skull base tumors. As evident from the aforementioned modifications and classical approaches to reconstruction of defects following craniofacial resections, in order to summarize the vast amount of information on the advantages and disadvantages of corresponding methods, a multicenter independent prospective study is needed, during which data from different clinics would undergo critical analysis using modern evidence-based medicine methods. On the path to methodological comprehension of existing concepts and actions towards creating one’s own algorithms lies a multifactorial analysis of personal experience in management and undoubtedly the care of patients from a group with such an unfavorable prognosis for survival. An immediate observation that follows the realization of the practical significance of a surgical treatment algorithm common to all physicians and mid-level medical staff is that the most effective achievement of the above task lies in learning from one’s own mistakes during patient care. Very encouraging research results, in which attempts are made at mastering new materials, herald a future scientific breakthrough in craniofacial reconstruction. The use of bioprosthetics and synthetic analogs is particularly relevant for those patients for whom reconstruction using their own tissues is contraindicated for various reasons. This article demonstrates the most outstanding achievements in the treatment of patients with sinonasal tumors.

Malignant tumors of the sinonasal region are rare and highly diverse in their histological origin neoplasms, accounting for an average of 0.2–1.1 % of all newly diagnosed tumors. Risk factors, to varying degrees pathogenetically linked to disease progression, include a mutation of the TP53 gene in 80 % of cases, prolonged exposure to industrial air pollutants, smoking, male gender, and age over 55 years in 85 % of cases, infection with human papillomavirus and Epstein–Barr virus. Despite a substantial amount of information on approaches to managing this patient population, the increasing number of modern technology-intensive methods has led to ongoing debates regarding the selection of a treatment strategy that fully meets the requirements for objectively assessing efficacy and improving the quality of life of patients suffering from oncological disease.

Aim. To investigate modern approaches to treatment of patients with tumors of the craniocephalic location, identify factors of surgical treatment success and favorable long-term prognosis, develop a management algorithm for patients with sinonasal neoplasms, provide rationale for using various reconstruction techniques for postresection defects. Systemic approach to the analysis of effectiveness and expediency of modern approaches to treatment of patients with tumors of the craniocephalic region requires integration of several key concepts. Taking into account individual characteristics of the patient by an experienced surgical team can lead to good treatment results including increased survival rates.

Development of algorithms for management of patients of this category and individualization of selection of reconstructive techniques are important problems in practical healthcare. Minimization of complications is one of the main goals of postoperative patient observation. The optimal approach to treatment even in this prospectless in the context of survival and social rehabilitation group of patients assumes greatest possible preservation of facial esthetic features and increased quality of life which can be achieved only with well-coordinated work of highly professional interdisciplinary team.

Introduction. Multikinase inhibitors are used to treat nonresectable locally advanced and/or metastatic medullary thyroid cancer (MTC). However  they are characterized by high toxicity associated with kinase inhibition. Selective RET inhibitor selpercatinib demonstrates high selectivity and tolerance which makes it a promising agent for MTC treatment.

Aim. To evaluate selpercatinib effectiveness and tolerance in patients with metastatic MTC associated with a mutation in the RET gene.

Materials and methods. The study included 9 patients with metastatic MTC and mutation in the RET gene who received treatment with selpercatinib 160 mg 2 times a day. The drug effectiveness was evaluated every 2–3 months based on the results of multispiral computed tomography of the whole body and tumor marker (calcitonin and carcinoembryonic antigen) levels.

Results. Median duration of therapy was 29 months  overall response rate was 78 %; complete response was observed in 56 % of patients. After 12 months of therapy  progression-free survival was 100 %; after 24 months  it was 89 %. Persistent decrease in calcitonin level (by more than 90 %) was achieved in all patients. The most common adverse events were arterial hypertension and insignificant creatinine increase.

Conclusion. The results of therapy show significant improvement in the rate of objective response and progression[1]free survival which makes selpercatinib a preferential treatment choice in this category of patients.

Laryngeal chondrosarcoma is a rare malignant tumor that originates from cartilage tissue. The incidence of this pathology varies from 1 to 12 %. Only isolated clinical cases of laryngeal chondrosarcoma and observations of small groups of patients with this disease are described  since statistical data on such rare types of tumors are often unavailable. Survival in this pathology may vary depending on various factors  such as the stage of the disease  the size of the tumor  the presence of metastases  the general condition of the patient and the effectiveness of treatment. Since laryngeal chondrosarcoma is a rare disease  there is relatively little scientific data on prognosis and survival. The article presents a clinical case of diagnosis of treatment and primary chondrosarcoma of the larynx.