Trabectedin in the treatment of soft tissue sarcomas: up to date data

Soft tissue sarcomas are a rare heterogeneous group of malignant tumors with mesenchymal nature. Soft tissue sarcomas accounts for less than 1 % of all cancers. Low efficiency of chemotherapy for soft tissue sarcomas, especially in advanced disease, is a burning issue. Trabectedin is one of the active agents approved for the treatment of advanced soft tissue sarcomas after the failure of standard chemotherapy. The article provides a literature review of the latest world-wide data of the effectiveness and role of trabectedin in the modern approach of soft tissue sarcomas treatment.

Prognostic value of the Tokuhashi scale when choosing a method of surgical treatment of metastatic lesions of the spine

Introduction. Metastatic spinal lesion remains one of the most difficult problems of modern medicine. The purpose of treatment of patients with metastatic spinal lesion is to reduce pain, improve the quality of life, restore stability in the affected segment, prevent or reduce neurological deficits. In order to build the right treatment tactics, it is necessary to evaluate the prognosis of the life of a patient with spinal metastases before treatment.The study objective is to evaluate the prognostic significance of the Tokuhashi scale in patients with metastatic spinal lesion.Materials and methods. The study included 124 patients with metastatic spinal lesion operated in the spinal department in the period from March 2013 to July 2019 (64 (52 %) men and 60 (48%) women). The average age was 59 years and ranged from 18 to 78 years. The catamnesis ranged from 1 month to 6 years. 6 (5 %) spinal tumors were radically removed, palliative removal was performed in 77 (62 %) cases. In the remaining cases, 41 (33 %) underwent vertebroplasty. The mandatory diagnostic protocol of the preoperative examination included oncological status, clinical and neurological examination. The functional status of the patient was assessed on the Karnofski scale, life expectancy – on the Tokuhashi scale. In terms of preoperative examination, patients underwent computed tomography examination with intravenous contrast of three zones: the thoracic, abdominal cavities and pelvic region to determine the degree of dissemination of the process and, in some cases, to identify the primary focus. Currently, positron emission tomography is the optimal method of investigation at the preoperative stage. However, this method is not yet available in all clinics, so performing computed tomography scans of three zones is currently the optimal “gold standard” for examining this category of patients..Results. The life expectancy estimate was calculated for each patient according to the Tokuhashi scale (2005). Survival analysis was performed using Kaplan-Meier curves within the Tokuhashi prognostic groups. The median survival within the Tokuhashi groups was more than 3 years for the group of 12–15 points (life expectancy over a year), 14 months for the group of 9–11 points (life expectancy 6 to 12 months) and 7 months for the group of 1–8 points (life expectancy less than 6 months). The Cox model was used to identify factors related to survival.Conclusion. Determining the tactics of surgical treatment of a patient with a metastatic lesion of the spine is a complex and multicomponent question to which there is no unambiguous answer. A multidisciplinary consultation is currently the most adequate and reliable way to determine the treatment strategy for this category of patients. The use of the Tokuhashi prognostic scale is an additional effective tool for determining the life expectancy of patients with spinal metastases, and thus, in some cases, serve as the tool with which it is possible to determine the tactics of surgical treatment of patients.

The frequency of demand and options for surgical treatment of breast cancer metastases in long and flat bones, depending on the histological subtype of the tumor

Introduction. Bone metastases increase the risk of developing a pathological fracture, spinal cord compression, pain syndrome and require palliative, radiation therapy and oncoortopedic operations. The appearance of these metastases and the above complications is associated with an unfavorable prognosis and negatively affects the quality of life of patients.Objective – to analyze the outcomes of surgical treatment in patients with breast cancer metastases to long and flat bones and to identify an optimal surgical tactics depending on the morphological subtype of the tumor.Materials and methods. Between April 2015 and April 2021, a total of 731 patients with breast cancer and bone metastases were consulted in the departments of the Russian Research Center of Radiology, Ministry of Health of Russia. Two hundred and thirty patients (31.5 %) had indications for orthopedic surgery. This study included 78 patients with breast cancer metastases to long or flat bones. Of them, 50 individuals had metastases to bones only, whereas 28 participants additionally had metastases to other organs. Luminal A and B breast cancer was diagnosed in 27 patients (34.6 %) and 40 patients (51.2 %), respectively. Ten participants (12.8 %) were found to have HER2-positive tumors, whereas one patient (1.3 %) had triplenegative cancer.Results. Eighteen patients with metastatic lesions in long bones have undergone bone grafting; 17 patients had intramedullary internal fixation with osteoplasty; and 1 patient had extramedullary internal fixation with osteoplasty. Forty two patients with metastatic lesions in the flat bones have undergone 39 osteoplasty surgeries of the iliac bone, while 3 patients had osteoplasty surgeries of the sternum. The survival rates of patients with luminal A, luminal B, HER2- positive, and triple-negative breast cancer were as follows: 1-year survival – 95, 84, 87 and 100 %, respectively; 3-year survival – 83, 64, 23 and 0 %, respectively; 5-year survival – 66, 32, 23 and 0 %, respectively. Thirty-one patients were lost to follow-up between month 0 and month 46. Eighteen patients died between month 1 and month 58.Conclusion. Patients with luminal A and B breast cancer and threatened pathologic fracture should undergo intramedullary/extramedullary internal fixation, while for patients who already developed pathologic fracture and their estimated life expectancy does not exceed 1 months, it is recommended to perform bone grafting regardless of the tumor immunohistochemical type. Patients with threatened pathologic fractures demonstrated a more favorable prognosis, better functional status, and higher survival rates than patients with pathologic fractures: 1-year survival was 90 and 87 %, respectively; 3-year survival was 67 and 58 %, respectively, and 5-year survival was 54 and 0 %, respectively. Radiation therapy increases the risk of pathological fractures, but is not a contraindication for orthopedic surgery.

Surgical treatment of patients with metastatic bone lesions during the COVID-19 pandemic

Introduction. In March 2020, the World Health Organization announced the COVID-19 as a pandemic. The emergence and spread of the new infection hit most healthcare systems globally. The pandemic caused temporary restrictions for elective medical care, which affected many patients at risk, including cancer patients, since some hospitals were closed for quarantine. Deferred pharmacotherapy, radiotherapy, and surgery in these individuals might have resulted in disease progression and even death. In this situation, healthcare professionals had to quickly adjust the manage ment strategy for cancer patients and identify an optimal approach to therapy with the lack of accurate data.Objective – to assess the risk of COVID-19 in patients with metastatic bone lesions during hospitalization for elective surgery and 14 days after their discharge from hospital.Materials and methods. In this article, we describe treatment of 182 patients with metastatic bone lesions treated in the Department of Surgical Treatment for Bone and Soft Tissue Tumors, National Medical Radiology Research Center, Ministry of Health of Russia between March 30, 2020 and August 1, 2021.Results. Eleven patients developed symptoms of coronavirus infection during their stay in the hospital. The incidence of nosocomial COVID-19 was 6 % according to the results of testing. Timely diagnosis and initiation of special treatment ensured the survival rate of 100 %.Conclusion. Adherence to epidemiological measures at all stages of hospitalization minimizes the risk of COVID-19 in patients with secondary bone lesions, allowing timely elective surgery. However, a tailored approach should be used when deciding on surgery during the pandemic.

Results of neoadjuvant denosumab in giant cell tumor of the bone depending of the tumor location and the surgical grade

Background. The standard treatment for giant-cell tumors of the bone includes radical surgery. However, specific anatomical location of the tumor and/or its spread may hinder its complete excision or result in poor functional outcomes. Currently, combination treatment that includes preoperative denosumab and surgery is preferable. It saves patients’ lives and improves their quality of life. Reduction of local recurrence rate by combination therapy for giant-cell tumors of the bone is being actively studied now.Objective – to analyze treatment outcomes of patients with giant-cell tumors of the bone, including those who received combination treatment that included preoperative therapy with denosumab followed by surgery.Materials and methods. This study included 277 patients with giant-cell tumors treated in N.N. Blokhin National Cancer Research Center between 2005 and 2020. The mean duration of follow-up was 56 months. Study participants were divided into two groups. Group 1 included patients who received surgical treatment alone (n = 212), whereas Group 2 comprised patients who received combination treatment (n = 65). Neoadjuvant therapy included subcutaneous denosumab 120 mg on days 1, 8, 15, and 28, then every 4 weeks until stable effect. There were two variants of surgical treatment: radical (removal by a single block or segmental resection with defect replacement, with or without fixation) and non-radical (excochleation or marginal resection with defect replacement, with or without fixation).Results. During treatment, patients in Group 2 had a significantly milder pain syndrome (assessed both using the visual analog scale for pain and Watkins scale) compared to Group 1. In case of radical surgery, the incidence of local recurrence was 12 % and 0 % in Groups 1 and 2, respectively; the difference was significant (р <0.05). Tumor location and volume of surgery played an important role in disease recurrence (р <0.05). The incidence of complications after radical surgery was 36.9 % and 12.5 % in Groups 1 and 2, respectively; the difference was significant (р <0.05). In addition to that, neoadjuvant therapy with denosumab substantially reduced the duration of surgery and blood loss in patients with challenging anatomical location of the tumor (р <0.05).Conclusion. Combination treatment for giant-cell tumors that includes neoadjuvant therapy with denosumab reduces the risk of recurrence, duration of surgery, blood loss, and the risk of postoperative complications. However, it is important to consider tumor location and the volume of surgery. Since the disease is quite rare, further study of long-term efficacy and safety of combination treatment for giant-cell tumors, including rare ones and those with challenging anatomical location, is necessary.

Dynamics of quality of life main parameters in patients with spine tumors underwent surgical treatment

In this study we analyzed the quality of life in 62 patients (35.5 % of men and 64.5 % of women; mean age 55 years) who had tumor lesions in different parts of the spine and were treated in the Department of Vertebral Surgery, N.N. Blokhin Russian Cancer Research Center, Ministry of Health of Russia. We assessed the association between the quality of life and psychological characteristics of patients – coping strategies and personal resources. We used a general questionnaire for assessing patients’ quality of life (The Medical Outcomes Study Short Form 36 Items Health Survey) and special questionnaires for cancer patients (Quality of Life Questionnaire – Core 30, QLQ-C30), patients with tumor lesions to the spine (Spine Oncology Study Group Outcomes Questionnaire, SOSG OQ), and psychodiagnostic methods, such as Ways of Coping, Big Five, and Life-meaning Orientations. We have identified an association between the quality of life and all parameters of psychodiagnostic questionnaires, better quality of life correlated with more pronounced psychological characteristics reflecting the maturity of the individual (internality, activity, presence of meaningful goals, ability to seek social support, etc.). Lower personal maturity and poorer constructive coping strategies were associated with worse somatic symptoms and their impact on the quality of life.

Multilevel radical resection in treatment of patients with spine tumors

Introduction. Radical surgical resections in the treatment of malignant tumors of the spine have proven effective in improving both overall and disease-free survival of patients. Indications for such interventions are primary malignant tumors of the spine, primary benign tumors with aggressive growth, and solitary metastatic lesions in patients with a favorable oncological prognosis. Due to the anatomical features of the vertebral column, it is very difficult to perform radical resections. An even more difficult task is to perform such interventions for multilevel spinal lesions.The study objective is to evaluate the results of radical surgical treatment for multilevel tumor lesions of the spine.Materials and methods. From 2004 to 2019, in the department of spine surgery of the N.N. Blokhin National Medical Research Center of Oncology treated 36 patients with spinal tumors, who underwent multilevel radical resections. The analysis of this surgical treatment of patients with multilevel neoplastic lesions of the spine was carried out.Results. Performing radical surgical interventions on the spine makes it possible to reduce the frequency of local relapses, improve functional results and overall survival of patients. After performing multilevel resections, complications often occur.

Primary Ewing sarcoma of the sphenoid bone with infrasellar spread in a 17-year-old patient: case report and literature review

We performed retrospective analysis of publications describing cases of Ewing sarcoma of the skull base with exceedingly rare locations (including the area of the sphenoid bone, its wings, sinus, and adjacent structures). Ewing sarcoma is usually diagnosed in children. The peak incidence is registered in patients aged 10–15 years, whereas adults and children under 5 years of age are very unlikely to develop this disease. In this article, we also report a case of Ewing sarcoma of the skull bones affecting the sphenoid bone and sphenoid sinus. The patient has undergone surgery followed by chemoradiotherapy. High invasion potential (tumor spread in several directions), high malignancy, and compression of the adjacent structures of the brain make the analysis of Ewing sarcoma located in areas that cannot be easily approached particularly interesting for neurosurgeons, since surgery is the main treatment option for such patients.

Experience in the treatment of primary malignant tumors (retinoblastoma and osteosarcoma): analysis of the clinical case

Background. Retinoblastoma is a malignant intraocular tumor developing from the retinal neuroectoderm and diagnosed primarily in young children. This type of cancer is associated with a high risk of multiple primary tumors emerging after treatment completion. Multiple primary tumors are two or more independent tumors developing in one patient. Treatment of this disease is challenging.Objective – to evaluate the impact of risk factors on the efficacy of therapy for multiple primary tumors and to analyze treatment outcomes.Materials and methods. A 2-year-old boy was diagnosed with bilateral retinoblastoma (OD – stage T3bN0M0 and OS – stage T3cN0M0). He received special treatment from September 2005 to November 2006. In 2012, the patient underwent cataract surgery: the lens was removed, then an intraocular lens was installed, and laser dissection of the posterior capsule of the lens was performed. Six years later, in August 2018, the patient was diagnosed with osteosarcoma. The boy received combination organ-sparing therapy according to the EURAMOS-1 treatment protocol for osteosarcoma. During therapy, he developed a secondary tumor, namely osteoblastic osteosarcoma. Both the boy and his father were found to have a mutation in the RB1 gene.Results. Currently, patient’s condition is satisfactory; he has no complains. The boy is in remission for 2 years.Conclusion. The development of secondary tumors depends on the genetic factors, type of treatment for primary tumor, and environmental factors. Therefore, it is extremely important to assess risk factors for multiple primary tumors at the moment of primary retinoblastoma detection. The results of such assessment will help to choose an optimal treatment strategy.

Modern approach to the treatment of sacrococcygeal chordomas

Chordomas of the sacrococcygeal region account for more than 50 % of all sacral tumors. These malignant neoplasms grow slowly and are asymptomatic for a long time. As a result, chordomas often reach large sizes and affect the neurovascular structures of the sacrum and pelvic organs. The use ofen-bloc resection allows to increase survival rates and reduce the risk of progression. However, this method of chord treatment is difficult for surgeons and in most cases, after surgery, the quality of life of patients decreases. The improvement of imaging methods, the success of oncological orthopedics and radiation therapy allow performing radical organ-preserving operations. In this article, we will consider the modern concept of treatment with a sacrococcygeal chord.