Background: Gorham-Stout disease (GSD) is a rare, underreported and poorly investigated osteolytic condition which may affect bone of different regions of the human body. To our knowledge this is the first case of bone reparation during treatment that was traced carefully clinically and radiographically. Such complex craniomaxillofacial involvement [upper jaw with continuous extension to the base of the pterygoid Case Description: We present a case of a 40-year-old subject with massive resorption of the right upper jaw and the base of the ipsilateral PP which was diagnosed as GSD. He was admitted with a year-long history of uncontrollable midfacial pain. Radiographically osteolysis of the maxilla and the PP was evident. Microscopically the specimen lacked any specific signs and consisted of fibrous tissue mingled with blood vessels and lymphatics. Off-label sirolimus 1 mg once daily; zoledronic acid (ZA) 4 mg every 4 weeks and vitamin D 5,000 IU once daily treatment protocol was employed. The extraordinary prominent restoration of the maxillary anterior wall and the base of the pterygoid was first noted after 9 months of treatment (medication implemented for 3 years) and traced throughout the whole 4-year follow-up period. Conclusions: Medical treatment protocol consisting of the combination of the ZA, sirolimus, and vitamin D has showed its effectiveness in the treatment of GSD of the maxilla and the adjacent base of the PP. Further research of this treatment protocol is needed.

Background. Currently, the main method of treatment of locally advanced squamous cell carcinoma of the nasal cavity and paranasal sinuses is surgical treatment with subsequent radiation/chemoradiation therapy. However, the survival rate of patients has not changed significantly in the last 20 years. Head and neck squamous cell carcinoma is highly sensitive to chemotherapy and radiation which allows to perform treatment even in cases of locally advanced squamous cell carcinomas of the nasal cavity and paranasal sinuses. However, the question of prognostic factors of the effect of chemoradiation therapy remains open. Therefore, determination of these factors is an important problem. Aim. To evaluate the effectiveness of conservative treatment of locally advanced squamous cell carcinoma of the nasal cavity and paranasalsinuses. Materials and methods. The retrospective analysis included 55 patients with squamous cell carcinoma of the nasal cavity and paranasal sinuses (stages III–IVA) who were under observation and receiving treatment at the N.N. Blokhin National Medical Research Center of Oncology between 2000 and 2020. In 50 (90.9 %) patients, treatment began with induction chemotherapy with subsequent radiation therapy per the radical program. In this patient group, treatment effectiveness was evaluated per the RECIST 1.1 (Response Evaluation Criteria In Solid Tumors) criteria. Results. Five-year overall and progression free survival rates were 49.5 and 22.5 %, respectively. Objective response rate after treatment was achieved in 65.5 % of cases: complete response was observed in 38.2 % of cases (n = 21); partial response in 27.3 % (n = 15); stable disease in 18.2 % of cases (n = 10); disease progression in 16.4 % (n = 9). Conclusion. According to the results of the study, conservative treatment is a promising approach in locally advanced squamous cell carcinoma of the nasal cavity and paranasal sinuses. Patients with complete response to treatment have the highest odds of cure and improved long-term outcomes.

Arteriovenous malformation (AVM) of the head and neck is a rare disease developing during embryo- and vasculogenesis due to RASA1 mutation which is inherited in accordance with autosomal dominant pattern and is associated with CMC1 locus on the long arm of the 5th chromosome 5q13.3. Statistically, AVMs are rarer than other congenital vascular abnormalities such as lymphatic or venous malformations. While AVMs are non-malignant neoplasms, among all vascular abnormalities they are the most aggressive, destructive and complex in their structure. In international literature, a small number of articles describing cases of this pathology exists (for example, I.T. Jackson et al. analyzed 16 cases of this pathology, M.T. Chen et al. reported on 14 cases, M.P. Kohaut et al. analyzed 81 cases of extracranial AVMs).In Russin literature, the number of articles describing clinical cases of AVM is in the single digits, and this topic remains quite relevant. The article presents a rare clinical observ

Introduction. Transnasal endoscopic resection of sinonasal tumors is currently used in many clinics around the world. Short- and long-term oncological outcomes primarily depend on tumor histology. However, most currently existing studies are based on a small number of observations due to the rarity and heterogeneity of sinonasal malignancy.Aim. To evaluate short- and medium-term oncological outcomes of treatment of malignant sinonasal tumors. Materials and methods. The study is based on retrospective analysis of 95 patients who underwent endoscopic surgery for sinonasal malignancy between 2014 and 2023 at the N.N. Blokhin National medical Research Center of Oncology. Squamous cell carcinoma was diagnosed in 27 (28.4 %) patients, olfactory neuroblastoma in 13 (13.7 %), mucosal melanoma in 30 (31.6 %), adenocarcinoma in (6.3 %), adenoid cystic carcinoma in 7 (7.3 %), sarcomas in 12 (12.7 %). Results. In the study group, 2-years overall survival and recurrence-free survival were 81.3 and 61.4 % respectively, survival without local recurrence was 87.5 %. The outcomes significantly depended on tumor histology.Conclusion. Oncological outcomes of endoscopic resections of sinonasal tumors significantly depend on tumor histology, but in general they allow to achieve good local control. Small number of observations, variety of histological forms, and short follow-up periods do not allow to accurately evaluate the effectiveness of this approach for each tumor type. further research in large populations is needed to establish risk factors.

Introduction. Surgical intervention with subsequent chemoradiation therapy is one of the treatment standards in squamous cell carcinoma of the nasal cavity and paranasal sinuses. However, the question of the optimal therapy for this pathology remains open. Aim. To compare treatment results in patients with locally advanced squamous cell carcinoma of the nasal cavity and paranasal sinuses after conservative and surgical treatment. Materials and methods. The study included 86 patients with squamous cell carcinoma of the nasal cavity and paranasal sinuses, stages III–Iva, who between 2000 and 2020 received treatment at the N.N. Blokhin National medical Research Oncology Center. Surgical treatment with subsequent radiation or chemoradiation therapy was performed in 31 patients, conservative treatment (radiation or chemoradiation therapy) was performed in 55 patients. Results. Five-year overall survival in the conservative treatment group was 46.2 %, in the surgical treatment group – 59.7 % (р = 0.081); 5-year progression-free survival was 17 and 50.5 %, respectively (p = 0.002). In patients with Т3N0 tumor who received surgical treatment, 5-year progression-free survival was significantly higher than in patients who received conservative treatment (p <0,05). Comparison of overall survival and progression-free survival of patients who achieved objective response (complete response + partial response) after conservative treatment (n =36) and after surgical treatment (n = 31) did not show statistically significant differences (р = 0.469 and р = 0.183, respectively). The overall 5-year survival rate in the salvage surgery treatment group (24/55) was 63.1 % compared with 59.7 % in the surgical treatment group (n = 31), there were also no significant differences (p = 0.95). In patients with T3 tumor who received conservative treatment in this study, higher risk of progression was observed compared to patients who underwent surgical treatment. Conclusion. Surgical intervention remains an important treatment method for patients with locally advanced squamous cell carcinoma of the nasal cavity and paranasal sinuses, especially in cases of smaller tumor extent. Conservative therapy seems promising but large-scale prospective controlled randomized clinical trials are necessary to confirm its effectiveness and safety in various patient groups.

Hypoparathyroidism is an endocrine disease characterized by reduced production of parathyroid hormone by the parathyroid glands or tissue resistance to its action. This is accompanied by disturbances in phosphorus-calcium metabolism. The main cause of hypoparathyroidism is damage or removal of the parathyroid glands during surgery on the neck organs. Autoimmune hypoparathyroidism is the second most common form of the disease, which occurs, as a rule, within the autoimmune polyglandular syndrome type 1. The development of chronic hypoparathyroidism of any etiology requires lifelong appointment of multicomponent therapy, as well as careful monitoring and an individual approach. In the absence of adequate dynamic monitoring, multiple complications develop from vital organs, in particular calcification of the urinary system, soft tissues and the brain; cardiovascular pathology; visual disturbances; muscle and bone diseases leading to decreased quality of life of patients. Thus, timely diagnosis, rationally selected drug therapy and competent patient management will reduce the risks of complications, improve the prognosis, and reduce the frequency of hospitalizations and disability of patients with chronic hypoparathyroidism. The article presents the main recommendations of the new project of clinical guidelines for patients with hypoparathyroidism, approved by the Ministry of Health of the Russian Federation in 2021. They include the algorithms of diagnosis, treatment and dynamic monitoring of hypoparathyroidism, as well as management of postsurgical hypocalcemia and medical care of and the disease during pregnancy.

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 1st 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.

The most common cause of total temporomandibular joint (TMJ) prosthesis is the terminal stage of degenerative dystrophic diseases. Patients with oncologic pathology usually undergo extended surgical interventions with TMJ exarticulation which in contrast to non-tumor pathology are characterized by resection of the whole ligamentous apparatus of the joint, the ramus of the mandible, and surrounding structures. Due to various causes, the use of traditional systems of open-type joint prosthesis (without fixation of the joint head in the glenoid fossa) frequently leads to loosening, luxation, and eventual dislocation of the joint head from the glenoid fossa. Currently, multiple different TMJ prosthetic systems have been proposed but only 2 are routinely used: TMJ Concepts (USA) and TMJ Biomet (USA). It should be noted that these systems are also open. We have studied the used constructions of total TMJ endoprostheses and haven’t found any that solve the problem of the condyloid component dislocation from the glenoid fossa after extended resections due to tumors of the TMJ. Moreover, analysis of the clinical outcomes after the use of such systems showed that even in cases of relatively limited resections of TMJ structures (11–30 %), joint prosthesis elements dislocation still occurs. Therefore, a promising solution for the problem of the joint head dislocation can be development of a closed total endoprosthesis with joint head fixation and preservation of joint mobility in all planes.

This article presents the experience of successful use of immunotherapy in treatment of locally advanced inoperable squamous cell carcinoma of the hairy part of the head.

Purpose Metastatic involvement of the lingual lymph nodes (LLNs) in oral cavity squamous cell cancer (SCC) has recently been proven to significantly reduce locoregional control and survival. Despite recent refinements in the detection of these lesions, the understanding of the LLN topographic anatomy among clinicians is limited. A proposition of a topographic division on LLN based on a comprehensive literature search and synthesis may be helpful in this condition. Methods A literature search and election based on contemporary PRISMA guidelines was performed for sources on LLN anatomy with special attention on their subdivision. Results Four topographic LLN subgroups were defined: median-between genioglossal and geniohyoid muscles; intermediate parahyoid-medial to the hyoglossal muscle, at the greater cornu of the hyoid bone; lateral sublingual (paraglandular) LLNs-at the sublingual salivary gland; lateral submandibular (paraglandular) LLNs -lateral to the hyoglossal muscle, at the deep surface of the submandibular salivary gland. Conclusion The development and implementation of a unified anatomical topographic classification of LLN subgroups may be among the important conditions for improving the detection and treatment of LLN lesions.

The article presents a clinical case of a patient with locally advanced anaplastic thyroid cancer and concomitant cardiovascular pathology. Anaplastic thyroid cancer is a very rare and highly aggressive disease requiring long-term multistage treatment, often using cardiotoxic drugs. Combination of this pathology with ischemic heart disease affecting coronary arteries is a complex clinical problem requiring timely evaluation of cardiac risk at every stage. Treatment of this patient cohort is possible only by a multidisciplinary team of specialists.

The article presents a draft of clinical recommendations for the diagnosis and treatment of differentiated thyroid cancer in adult patients, which provides a modern examination algorithm, discusses the basic principles of laboratory, instrumental diagnostics and treatment approaches.

The article is the consensus opinion of specialists involved in ultrasound examination, computed and magnetic resonance imaging of peripheral, abdominal and retroperitoneal lymph nodes, oncologists, authors of clinical recommendations of the Russian Society of Head and Neck Tumor Specialists based on an analysis of modern literary sources devoted to the problem of assessing the condition lymph nodes. The purpose of the publication is to bring ultrasound terms to uniform standards when describing lymph nodes, to introduce the Node-RADS system into the practice of ultrasound examination, to improve mutual understanding between diagnosticians and clinicians in the interpretation of ultrasound results in assessing the condition of lymph nodes, optimize the work of primary care ultrasound diagnostic doctors.

Combination of oncological and cardiological pathologies is a complex clinical problem requiring a multidisciplinary team of doctors for treatment of a patient. This article presents a case of successful treatment of a patient with serious concomitant pathologies.

Intrоduction . Sinonasal papilloma is a benign tumor. The standard treatment for sinonasal inverted papilloma (SIP) is surgical intervention. The tumor is located primarily in the maxillary sinus (50.9 %). Sinonasal inverted papilloma originated from the maxillary sinus has aggressive characteristics and can dislodge local anatomical structures, infraorbital canal in particular. Aim . To present a descriptive characteristic of structural changes in the infraorbital canal during SIP growth and determine the optimal volume of canal dissection. Materials and methods . A retrospective study of patients with SIP was performed. From the total cohort (n = 37), 15 patients with primary localization of sinonasal inverted papilloma in the maxillary sinus were selected. In all cases, the same diagnostic algorithm was used. Separately, evaluation of infraorbital canal anatomy based on preoperative analysis of computed tomography of the paranasal sinuses and intraoperative endoscopic exam was performed. Surgical strategy used in all cases consisted of endoscopic SIP tissue removal, total resection of the mucoperiosteum and subperiosteal dissection of all walls of the maxillary sinus using a bur. During surgery material was collected for control histological examination. Results . In 20 % of cases, changes in the walls of the infraorbital canal in the form of hyperostosis, erosion and dehiscence were observed. In 13.3 % of cases, growth of sinonasal inverted papilloma caused distortions in the structure of infraorbital canal walls. In all cases, computed tomography data showed the same results as intraoperative endoscopic visualization. For removal of the lesion and pathologically changed walls of the infraorbital canal, transnasal endoscopic partial maxillectomies (type 3 or 4) were performed. Follow-up duration varied between 1 and 5 years, mean follow-up duration was 3 years. SIP resection was effective in all patients, no recurrences were observed. Conclusion . For SIPs growing in the maxillary sinus, condition of the infraorbital canal must be of special interest. The study showed the effectiveness and flexibility of the surgical strategy consisting of the combination of total mucoperiosteum resection with subperiosteal dissection of all walls of the maxillary sinus.

The article describes clinical observation of locally advanced metastatic anaplastic thyroid cancer. Difficulties in instrumental and laboratory diagnosis of this pathology and the role of molecular diagnosis in search for highly effective treatment are discussed.

Esophageal cancer is one of the hardest to treat malignant cancers. Patient prognosis remains unsatisfactory despite considerable progress in surgical, radiation, and drug treatments. Surgery remains the main form of treatment for this pathology as it is the only radical treatment method. The objective is to evaluate short-term treatment results for cancer of the cervical esophagus and laryngopharynx after large-scale resection with reconstruction and plastic surgery stage. A clinical case of locally advanced squamous cell carcinoma of the mucosa of the cervical esophagus is presented. Use of free radial fasciocutaneous flap for reconstruction of the cervical esophagus with preservation of the pharynx is described. Problems of diagnosis and surgical treatment of this pathology from the point of view of our clinic’s experience are considered. We show that radial forearm flap can be used for reconstruction of the pharynx and cervical esophagus during circular resections.

Introduction. Oral squamous cell carcinoma is associated with a high risk of regional metastasis even in early stages (T1–2N0M0). Morphological examination reveals concealed metastases in 20 % of removed clinically unaffected lymph nodes.Objective – to evaluate the effectiveness of flow cytometry for detection of concealed metastases in sentinel lymph nodes as an indication for cervical lymph node dissection in patients with stage сT1–2N0M0 oral squamous cell carcinoma.Materials and methods. This study included 27 patients, including 13 patients (48.1 %) with stage T1N0M0 cancer and 14 patients with stage T2N0M0 cancer (51.9 %). At the first stage, all participants underwent primary tumor removal and cervical lymph node dissection with sentinel lymph node examination. After mobilization, half of these lymph nodes was used for routine morphological examination followed by immunohistochemical examination, while the second half of them was analyzed using flow cytometry. Then we compared the results obtained.Results. We examined 46 removed sentinel lymph nodes from 27 patients. Conventional histological examination revealed 4 concealed metastases (8.7 %). Polymerase chain reaction allowed us to detect another 16 metastases (37.8 %). Levels I, II, and III lymph nodes were most frequently affected. Flow cytometry demonstrated that 20 % of patients with a <4 mm invasion had concealed metastases, whereas patients with a 4 to 8 mm invasion had concealed metastases in 60 % of cases. Median follow-up time was 20.4 ± 11.7 months (range: 2.2 to 42.5 months; median 19.5 months). All participants were alive during this time. Seventeen out of 27 patients with stage T1–2N0M0 disease (63 %) were found to have concealed metastases, while the remaining ten patients (37 %) had no metastasis. One patient with stage T1N0M0 oral floor squamous cell carcinoma underwent the removal of the primary tumor and sentinel lymph node. Morphological examination and flow cytometry showed no metastatic lesions. After 14.8 months, the patient developed ipsilateral regional CN3 metastasis. The three-year progression-free survival rate was 94.7 ± 5.1 %.Conclusion. Highly sensible and rapid flow cytometry can become the method of choice in the diagnosis of metastases and deciding on cervical lymph node dissection in patients with cN0 disease. The study is still ongoing.

The article presents a clinical observation of treatment of locally advanced squamous cell oropharyngeal carcinoma. The problems of nutritional support of patients during primary chemoradiation treatment are considered. The presented clinical case shows that use of sip feeds can significantly improve nutritional condition in patients with head and neck tumors and improve tolerability and results of antitumor treatment.

Aim. To evaluate advantages and disadvantages of transoral ultrasonography (US) in tongue cancer.Materials and methods. US was performed in 165 patients between the ages of 15 and 85 years with malignant tumors of the mobile tongue (74 (44.9 %) women, 91 (55.1 %) men).Among 165 patients, 144 had primary tongue tumor. The study also included 21 (12.7 %) patients with recurrent tumor which clinically could be represented by a true recurrence (tumor development 6 months after the end of treatment) or by continued growth (tumor development less than 6 months after the end of treatment). The study included patients with tongue tumors Т1 - 50 (30.3 %) patients, Т2 - 78 (47.3 %) patients, Т3 - 16.4 % of patients, Т4 - 6.1 % of patients.Three types of approach to tongue tumor visualization were used: submandibular, transoral, and transbuccal with a standard linear transducer (4-9 MHz) and intraoperative linear transducer (5-14 MHz). In total in 165 patients with tongue tumors, 147 (89.1 %) transoral US, 86 (52.1 %) submandibular US and 25 (15.2 %) transbuccal US examinations were performed.Results. Among 165 patients, agreement between the sizes measured using US and histological examination was observed in 142 (86.1 %) patients taking into account 15 % error.With increasing tumor thickness and, correspondingly, T criterion, increased frequency of agreement between US data and histological data was observed. Thus, for T1 stage frequency of agreement with US data was observed in 61.8 % of cases, for Т2 stage in 81.1 % of cases, for Т3 stage in 93.8 %, and for Т4 stage agreement was observed in 100 % of cases.Frequency of agreement with histological data in evaluation of tongue tumor thickness for transoral approach was significantly higher than for submandibular approach (р = 0.014). Transoral technique was more accurate for measurement of thickness of primary tumors - 80.3 % of results agreed with histological examination, and for recurrences frequency of agreement was only 33.3 %. Submandibular approach for primary tumors showed accurate measurements only in 67.6 % of cases, in recurrent tumors in 58.3 % of cases. Transbuccal approach also showed higher measurement accuracy for tumor thickness in primary patients (70.0 %) compared to recurrent tumors (40.0 %).Conclusion. Use of transoral ultrasonography significantly improves clinical staging of tongue tumors at the preoperative stage.