Soft tissue sarcomas are a rare and heterogeneous group of solid tumors originating from the mesenchyme and accounting for only 1 % of all malignant neoplasms in adults. The modern clinical and morphological World Health Organization of soft tissue sarcomas classification includes more than 100 forms of these tumors. Neurofibromatosis is a group of hereditary, autosomal dominant diseases characterized by the development of multiple tumor formations, often benign. Currently, there are 3 types: neutrophibromatosis type I, neurofibromatosis type II and schwannomatosis. The estimated risk of developing sarcoma in patients with neurofibromatosis type 1 at age 30 years is 25.1 %, and by age 50 years it is 38.8 %. Treatment of soft tissue sarcomas associated with neurofibromatosis fully complies with the standard recommendations of National Comprehensive Cancer Network, NCCN (NCCN), American Society of Clinical Oncology (ASCO), European Society for Medical Oncology (ESMO) and RUSSCO and does not have its own peculiarities. Over the past 15 years, at the A.F. Tsyb Medical Radiological Research Center – branch of the National Medical Research Radiological Center, more than 190 patients with locally advanced, high-grade soft tissue sarcomas have been treated, including five patients with sarcomas associated with neurofibromatosis. The article presents clinical cases of treatment of soft tissue sarcomas associated with neurofibromatosis.

Sarcomas are common among a rare and heterogeneous group of solid tumors of mesenchymal origin, accounting for only 1 % of all adult malignancies. Soft tissue sarcoma arises primarily from the embryonic mesoderm with some involvement of the neuroectoderm. The modern World Health Organization clinical and morphological classification includes more than 100 forms of these tumors. The diagnostic methods and treatment modalities of soft tissue sarcomas have evolved with the multidisciplinary approach. The standard treatment for localized phase sarcomas is local excision followed by radiotherapy, but since 2010 preoperative radiotherapy has been considered the preferred option (according to the recommendations of the National Comprehensive Cancer Network, NCCN). The use of vascular surgery and femoropopliteal autovenous grafting in surgical tactics allows performing radical operations with limb preservation. The article presents a clinical case of treatment of a patient with a diagnosis of soft tissue leiomyosarcoma of the right popliteal region. In the volume of combined treatment, the surgical intervention – removal of a soft tissue tumor low part of the right thigh and popliteal fossa with resection of the posterior thigh muscle group together with the involved popliteal artery, with simultaneous femoropopliteal autovenous prosthetics – was performed.

Soft tissue sarcoma is a rare malignant neoplasm. Despite the improvement of surgical techniques, the rate of relapses of the disease remains high. The search for alternative methods of treatment is relevant.Purpose. To evaluate safety and effectiveness of intraoperative photodynamic therapy of sarcoma in soft tissues.Material and methods. Forty-four patients with soft tissue sarcoma were included in the study. Patients were divided into three groups. Group 1 (n = 10): soft tissue sarcoma of stage T2-3N0M0G1, surgical treatment and intraoperative photodynamic therapy. Group 2 (n = 17): soft tissue sarcoma of stage T2-3N0M0G2-3, surgical treatment and intraoperative photodynamic therapy. Group 3 (n = 17): soft tissue sarcoma of stage T2-3N0M0-1G2-3, combined treatment – preoperative light therapy, surgical treatment, intraoperative photodynamic therapy, neoadjuvant and adjuvant chemotherapy. Fluorescence spectrometry was performed to determine the degree of photosensitizer accumulation in tumor tissue. Treatment methodology: photosensitizer was injected 2.5–3 hours before the surgery. The tumor was removed with resection of adjacent muscles and healthy tissues. Then, laser irradiation of the tumor bed was made using “Latus” device. Radiation power was up to 2,5 W, power density was up to 0,28 W/cm2, radiation dose was up to 100 J/cm2. Follow-up ranged from 14 to 44 months.Results. The spectrometry has revealed that photosensitizer was accumulated predominantly in tumor tissue. There were no reactions to photosensitizer injection, no changes in the tumor bed as well as no increase in wound healing period after intraoperative photodynamic therapy.Within the observation period, all patients stayed alive. Relapses of the disease were registered in 38.7 % of patients: in Group 1 – 30 %, in Group 2 – 35.3 %, in Group 3 – 53 %. Of the 20 patients with primary diagnosed neoplasms, relapses were seen in 30 %. Of the 24 patients with previously recurrent neoplasms, 45.8 % had repeated relapses.Conclusion. Intraoperative photodynamic therapy is a safe and effective technique for treating soft tissue sarcomas; it is also the promising direction for research. A small number of observations, a variety of histological forms and stages of the disease make it difficult to assess the effectiveness of the discussed approach. Further studies of intraoperative photodynamic therapy are required.

Introduction. Routine endoprosthesis production does not have ready solutions for such rare locations as forearm bones, scapula, and breastbone. In the last decade, 3D printing of personalized implants has been actively developing. Its benefits are accurate anatomical compliance with the resected segment, quick manufacturing time, and low cost. Oncoortopedics finds use of more additional technologies.Aim. To show through clinical observations the advantages of qualitatively new biologically and mechanically compatible implants used to replace bone defects in anatomically complex locations and to promote the use of such endoprosthesis in clinical practice.Materials and methods. Organ-saving treatment using personalized implants manufactured using 3D printing technology was performed in 7 patients with bone tumors: 3 with primary bone tumors (sarcomas) and 4 with metastases. Tumor lesions were localized in the humerus in 2 cases, in the scapula in 2 cases, in the breastbone in 2 cases, and in the distal part of the radius in 1 case. The implants were designed based in the spiral computed tomography data. For implant manufacturing, direct metal laser sintering (DMLS) was used. Titanium alloy Ti6Al4V certified for production of medical implants was used as the material. Design and manufacturing took 3 weeks.Results. Morphological examination showed negative tumor resection margin in all patients. All 7 patients are alive. No intraoperative complications were reported. Follow up varied between 1 and 8 months. During follow up, disease progression was not observed. Mean functional score per the Musculoskeletal Tumour Society score (MSTS) was 80 % (between 72 and 94 %).Conclusion. Development of qualitatively new Russian oncological implants using 3D-printing technology is one of the most important areas in bone pathology.

Soft tissue sarcomas (STS) are rare malignant tumors of mesenchymal origin. They account for about 1% of all malignant neoplasms. Haematogenous spread is the most common route of me-tastasis for STS and bone metastases occur in 9.4% of cases. When creating treatment plan for STS, it is necessary to consider a multimodal approach. Combination treatment can include pre-operative or postoperative radiation therapy (RT), chemotherapy (CT), and local hyperthermia (LHT). Surgery for STS should be radical. This paper presents 9 STS clinical cases and treatment outcomes in patients with secondary bone disease. Four patients underwent chemotherapy and surgery. Two patients received chemoradiotherapy (CRT) and surgery. Three patients received thermo-chemo-radiotherapy (TCHRT) followed by surgery. Accelerated fractionation radiotherapy was given using a single tumor dose of 3 Gy, twice a day with interval of 4 hours between ses-sions, 3 times a week to a total tumor dose (TTD) of 30 Gy (isoeffective TTD – 42 Gy, TDF – 69 Gy). Local hyperthermia for soft tissue tumor treatment was performed over 6 sessions: 2 ses-sions were combined with CHT courses before and after RT, and 4 sessions were combined with RT. The follow up period for 6 patients varied from 12 to 1.5 months, for 3 patients it varied from 6 to 8 months, for 1 patient it lasted 3 months and for 1 patient – 2 years. According to the RECIST criteria, more than half of the patients had tumor stabilization and 22% of patients had a partial response. Grade III-IV the rapeutic pathomorphosis was observed in 70% of patients after pre-operative combination treatment. The use of CT, CRT or TCHRT in combination treatment of STS with secondary bone disease enabled us to achieve a pronounced therapeutic pathomorphosis of tumors and to perform organ preservation surgery with endoprosthetic replacement.

This study was aimed to analyze the most common diagnostic and therapeutic errors in orthopaedic oncological diseases. Bone tumors usually do not have characteristic symptoms, especially in the early stages of disease development. Therefore, they can often mimic benign tumors and non-tumor diseases. Careful history taking, detailed clinical and X-ray examinations in a timely manner are essential diagnostic tools for patients with bone sarcomas. Moreover, a correct analysis of clinical and radiological findings with regard to the dynamic relationship between them can help make more accurate diagnosis at the first doctor’s visit. Subsequent X-ray examinations performed in oncological centers using special methods such as spiral CТ, MRI, PET/CT with mandatory morphological verification of the diagnosis enable us to make a more precise diagnosis and to provide an objective assessment of pathological processes. The most common diagnostic and therapeutic errors that occur in orthopaedic oncological diseases were analyzed. In 35 % of cases, diagnostic or tactical errors were made by general practitioners. In 15 % of cases, the errors were associated with a latent course of disease and 12 % of cases were wrongly diagnosed as a benign neoplasm.

INTRODUCTION. Chest wall tumors represent a variety of morphological forms and variants of lesions. According to different authors, primary malignant tumors of the chest wall account for 0.2–2 % of all malignant neoplasms. Of them, soft tissue sarcomas constitute about 45 %. Metastatic tumors of the chest wall occur much more frequently and most commonly develop from malignant tumors of the mammary, prostate and thyroid glands, lungs, kidneys and ovaries.MATERIALS AND METHODS. The standard of the treatment of primary and metastatic tumors of the chest wall is combination or comprehensive therapy. In some cases, preoperative care allows to create a more favorable environment for performing surgical treatment being considered the best option for chest wall tumors. The choice of a technique for the replacement of the post-resection chest wall defect is of special importance to preserve the physiological chest volume, to restore chest rigidity, to prevent paradoxical respiration and to seal the pleural cavity.RESULTS. There are different surgical techniques for skeleton reconstruction. A wide range of materials used for a skeleton reconstruction include bone tissues obtained from patient’s own body (bone autoplasty, autografts), polymeric mesh (polypropylene, polytetrafluoroethylene (Gore-Tex), bone cement (polymethyl methacrylate), stainless steel and titanium constructions as well as titanium bars and rib clips (STRATOS). In spite of a large number of techniques for sternal reconstruction described in the literature, searching for new materials and ways of their usage appears relevant.CONCLUSION. Our clinical case studies demonstrate that modern reconstructive techniques combined with careful surgical planning allow to perform radical surgery with a successful outcome preventing serious postoperative complications.

Objective. To determine an optimal method for preventing regional relapses in patients with locally advanced cutaneous melanoma (CM).Material and methods. The investigation included 755 patients with locally advanced CM who had been treated at the Medical Radiology Research Centre. Among them, 213 patients had wide excision only for the primary tumor; 242 patients were additionally treated with preventive lymphadenectomy (pLAE), and 300 patients underwent sentinel lymph node (SLN) biopsy. The groups were matched for the local extent of a tumor in the TNM staging (p = 0.178), which allowed a proper comparative analysis of treatment results. The Russian radiopharmaceutical 99mTc-Technefit having necessary lymphotropic properties was used to identify SLN. Intraoperative navigation was performed using a Radical handheld gamma probe counter (Amplitude Research and Technology Center, Russia).Results. Regional relapses were observed in 20.2% of cases in the primary CM excision group. In the pLAE group, latent regional lymph node metastases were detected in 10.7% of the patients; and 12.0% more of the patients developed subsequently regional relapses in the intact lymph nodes. The total number of latent regional metastases in this group was 22.7%; these were diagnosed as a result of pLAE in less than half of the cases (47.3%). SLN biopsy revealed subclinical regional metastases in 20.7% of the patients and regional relapses were seen only in 3.3%. Overall, SLN biopsy could detect latent lymph node metastases in 86.1% of all cases; these were demonstrated with clinical relapses only in 13.9%. The efficiency of SLN biopsy in preventing regional relapses was significantly higher than was achievable during pLAE (p =0.001). The five and seven-year survival rates were 61.5 ± 4.3 and 54.3 ± 4.6% in the CM excision group, respectively; 68.8 ± 0.35 and 61.3±3.9% in the pLAE group; and 84.8 ± 6.5 and 84.8 ± 6.5% in the SLN biopsy group (p = 0.001).Conclusion. The investigation of SLN is the most effective, safe, and reproducible technique for the early detection of subclinical regional metastases and for the prevention of regional relapses in patients with locally advanced CM. Reliable regional control in turn makes it possible to hope for the best long-term treatment results.

Background. Osteosarcoma is a high-grade malignant bone tumor that accounts for up to 6 % of all bone neoplasms. There are only a few published cases of primary sternal osteosarcomas, reflecting the rarity of these tumors. Recently, there has been a growing interest in performing radical excisions of chest wall tumors followed by combined single-stage reconstruction of the rib cage with implants made of biologically compatible materials. Material and methods. We present the case of a 49-year-old woman diagnosed with osteosarcoma of the sternum. The patient received a combined modality treatment including chemotherapy, radiotherapy and surgery. Titanium nickelid implants were used for the reconstruction of chest wall defects. Results. The multimodality treatment with reconstruction of postoperative chest wall defects allowed the achievement of the optimal quality of life for 21 months in the patient with very poor prognosis. Conclusion. The chest wall reconstruction using biocompatible materials from titanium nickelid appeared to be an effective and safe technique for the treatment of chest wall tumors.

Background. Osteosarcoma is a high-grade malignant bone tumor that accounts for up to 6 % of all bone neoplasms. There are only a few published cases of primary sternal osteosarcomas, reflecting the rarity of these tumors. Recently, there has been a growing interest in performing radical excisions of chest wall tumors followed by combined single-stage reconstruction of the rib cage with implants made of biologically compatible materials. Material and methods. We present the case of a 49-year-old woman diagnosed with osteosarcoma of the sternum. The patient received a combined modality treatment including chemotherapy, radiotherapy and surgery. Titanium nickelid implants were used for the reconstruction of chest wall defects. Results. The multimodality treatment with reconstruction of postoperative chest wall defects allowed the achievement of the optimal quality of life for 21 months in the patient with very poor prognosis. Conclusion. The chest wall reconstruction using biocompatible materials from titanium nickelid appeared to be an effective and safe technique for the treatment of chest wall tumors.

Introduction. The aim of this study was to analyze the surgical treatment for multiple intrapulmonary metastases sarcomas of bone and soft tissue. Methods. This analysis includes retrospective survival data from 24 patients with soft tissue sarcomas and bone sarcomas who were treated from January 2004 to December 2014 in MRRC and meet the following criteria: the presence of more than four lesions in the lungs, no progression of the primary tumor, no distant metastases and overall satisfactory status (ECOG 0–1). Survival of patients assessed by Kaplan-Meier method. results. In total 49 pulmonary metastasectomy were performed. There were no postoperative deaths. In 4 patients underwent repeated operations. From this cohort 1-year survival has survived 10 patients (41.6 %), 5-year – 3 (12.5 %). 3 patients are alive 28, 62 and 139 months after surgery with no evidence of progression of tumors. conclusion. The detection of lesion in the pulmonary parenchyma is most often regarded as the poor prognosis of the disease. Individually tailored treatment strategy allows you to increase the overall life expectancy and improve quality of life. The surgical method can be considered as a stage of complex treatment, as in the progression of the disease in the lungs, and in synchronous lesions. Surgical repeated resection may be an appropriate treatment for patients with progression and the justified possibility of an operation.

183 patients with locally advanced STS treated in MRRC were analyzed. In group 1 were included 107 patients who received preoperative thermo-chemo-radiotherapy (ТCRТ) and surgery (S). The group 2 consisted of 76 patients treated with preoperative chemo-radiotherapy (CRТ) followed by S too. The locoregional-recurrencefree-survival (LRFS), metastasis-free survival and overall survival rates (93 %, 69 %, 84 %) at 5 years were higher in groups with ТCRТ compared to the CRT (78 %, 56 %, 68 %). Local hyperthermia didn’t increase an incidence of postoperative complications.

Introduction. The aim of this study was to analyze the surgical treatment for multiple intrapulmonary metastases sarcomas of bone and soft tissue. Methods. This analysis includes retrospective survival data from 24 patients with soft tissue sarcomas and bone sarcomas who were treated from January 2004 to December 2014 in MRRC and meet the following criteria: the presence of more than four lesions in the lungs, no progression of the primary tumor, no distant metastases and overall satisfactory status (ECOG 0–1). Survival of patients assessed by Kaplan-Meier method. results. In total 49 pulmonary metastasectomy were performed. There were no postoperative deaths. In 4 patients underwent repeated operations. From this cohort 1-year survival has survived 10 patients (41.6 %), 5-year – 3 (12.5 %). 3 patients are alive 28, 62 and 139 months after surgery with no evidence of progression of tumors. conclusion. The detection of lesion in the pulmonary parenchyma is most often regarded as the poor prognosis of the disease. Individually tailored treatment strategy allows you to increase the overall life expectancy and improve quality of life. The surgical method can be considered as a stage of complex treatment, as in the progression of the disease in the lungs, and in synchronous lesions. Surgical repeated resection may be an appropriate treatment for patients with progression and the justified possibility of an operation.

In the recent years, the effectiveness of the treatment of even advanced cases of metastatic renal cell carcinoma is relatively high due to the possibility of targeted therapy, removal of metastatic lesions. Therefore, the issue of the quality of life of such patients often comes to the fore. This paper presents a clinical case of radical surgical treatment of metastasis located in the humerus, resulting in partial recovery of the limb function which eventually led to the improvement of the patient’s quality of life.

From 2004 to 2014 forty-six patients with sarcomas of the musculoskeletal system with metastatic lesions in the lungs were operated in MRRC. In 22 patients with a primary bone sarcoma metastases were detected in 27% cases, and in 29% of 24 patients with soft tissue sarcomas metastases were identified at the time of diagnosis. Twenty women and twenty-six men aged 16 to 72 years (mean age was 39 years) were operated. In most cases metastases were located subpleurally. Solitary metastases were observed in 8 patients (17%), single in 11 (24%) and multiple in 27 (68%) and 18 patients of them had bilateral disease. In all cases, chemotherapy was conducted at the first stage of treatment, according to histology of the tumor. In cases of large metastases or close location to the root of the lung (27 patients) preoperative distal radiotherapy was conducted in hyperfraction regimen with a daily dividing of the dose (2 times a day with an interval of 4 h every other day with single dose of 3 Gy and the total dose of 30 Gy that was isoefficiency to 42 Gy), after that the removal of pulmonary metastases was performed. A total of 73 surgical interventions performed in 46 patients. Most often precision resection of the lung using a neodymium YAG surgical laser MY 40 (wavelength 1318 nm) was performed. One patient underwent pneumonectomy. Videothoracoscopy was used in 6 cases. From 1 to 103 metastases were excised during one intervention. Pneumonia with abscesses were diagnosed in 3 patients, persistent pneumothorax in 5 patients, all complications were treated conservatively. There was no postoperative mortality. Fourteen patients underwent 2 procedures, two patients – 3 procedures, two patients – 4 procedures and one patient had 6 procedures. Follow-up ranged from 3 to 126 months. Twenty-six patients died due to progression of the disease. 3-year and 5-year survival was 46% and 41% respectively. Median survival was 32 months. Thus, the combined treatment of generalized forms of sarcomas of the musculoskeletal system can achieve long-term remission and significantly prolong life. Surgical resection should be applied in patients with solitary and with multiple metastases in the lungs and should be combined with systemic treatment (PCT) and, if indicated, with additional local treatment (distant radiotherapy) of metastases. Precision resection is adequate and well-tolerated method of surgical treatment of metastases in the lungs and does not correlate with severe postoperative complications.