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Bone and soft tissue sarcomas, tumors of the skin最新文献

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Paraneoplastic dermatosis in pediatric and adult patients 儿童和成人副肿瘤性皮肤病
Pub Date : 2024-04-08 DOI: 10.17650/2219-4614-2024-16-1-34-50
N. Murashkin, T. T. Valiev, T. Belysheva, R. A. Ivanov, D. Sofronov, Yu. E. Ryabukhina, M. I. Savelyeva, P. A. Zeynalova
Clinical, morpho-immunological and cytogenetic characteristics of malignancies are very polymorphic. And no less heterogeneous are paraneoplastic skin presentations arising before malignant tumour manifestation and/or proceeding in parallel. In the current literature review the most common paraneoplastic dermatosis are presented: pyoderma gangrenosa, dermatomyositis, paraneoplastic pemphigus, pityriasis rubra pilaris, Bazex syndrome, necrolytic migratory erythema, ptyriasis rotunda, Sweet syndrome. It is provided modern concepts in pathogenesis, clinical features and treatment approaches.
恶性肿瘤的临床、形态免疫学和细胞遗传学特征非常多变。在恶性肿瘤出现之前和/或同时出现的副肿瘤性皮肤病也同样具有异质性。在当前的文献综述中,介绍了最常见的副肿瘤性皮肤病:坏疽性脓疱病、皮肌炎、副肿瘤性天疱疮、红斑狼疮、巴泽克斯综合征、坏死性移行性红斑、斑丘疹、斯威特综合征。该书提供了有关发病机制、临床特征和治疗方法的现代概念。
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引用次数: 0
Rehabilitation of cancer patients: the use of pneumatic compression in secondary lymphedema treatment 癌症患者的康复:在继发性淋巴水肿治疗中使用气压疗法
Pub Date : 2024-04-08 DOI: 10.17650/2219-4614-2024-16-1-76-80
V. Ivashkov, A. V. Rovnaya, A. S. Denisenko, A. Kolsanov, A. Nikolaenko
Complex decongestive therapy is a cornerstone of conservative chronic lymphedema treatment. There are other conservative treatment options that include intermittent pneumatic compression. Its mechanism of action is based on pressure applying on the underlying tissues and promoting the lymph flow through the lymphatic structures. Pneumatic compression was invented in 1972; nevertheless it has not found widespread support of professional community as a self-sufficient method of conservative lymphedema treatment, though it’s used a lot in many rehab clinics worldwide.Aim. To determine the indications and effectiveness of pneumatic compression, as well as to identify the possibility of using this method in the practice of treating lymphatic edema.
复合减充血疗法是慢性淋巴水肿保守治疗的基石。其他保守治疗方法还包括间歇性气压疗法。其作用机理是对下层组织施加压力,促进淋巴液流经淋巴结构。气压疗法发明于 1972 年,尽管全球许多康复诊所都在大量使用,但作为一种自给自足的淋巴水肿保守治疗方法,气压疗法尚未得到专业界的广泛支持。确定气压疗法的适应症和有效性,以及在治疗淋巴水肿的实践中使用这种方法的可能性。
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引用次数: 0
Surgical treatment of a massive soft tissue neurofibroma in neurofibromatosis type 1 1 型神经纤维瘤病巨大软组织神经纤维瘤的手术治疗
Pub Date : 2024-04-08 DOI: 10.17650/2219-4614-2024-16-1-88-92
A. Valiev, A. Salkov, N. S. Petrochenko
Introduction. Neurofibromas developing in neurofibromatosis type 1 rarely grow to a large size. However, in cases of massive lesions, selection of optimal treatment tactics can be complicated. Clinicians usually resort to surgical treatment which currently serves as an effective instrument for improving patients’ quality of life and for achieving good cosmetic results.Aim. To describe a successful experience of surgical treatment in a patient with giant neurofibroma type 1 affecting a massive pool of soft tissues.Clinical case. Patient, 22 years, sought medical help at the N.N. Blokhin National Medical Research Center of Oncology with diagnosis of neurofibromatosis type 1 with massive lesions in the tissues of the lower third of the back, gluteal region, thighs. The disease has existed since birth, family medical history is fraught: father died of malignant manifestation of a neurogenic tumor. The younger sister is also diagnosed with neurofibromatosis affecting the brain and manifesting through cafe au lait spots on the body. Due to massive tumors significantly decreasing the patient’s quality  of life, cytoreductive surgical treatment was prescribed aimed at maximally radical resection of all available tumors to alleviate the patient’s condition.Conclusion. The presented clinical case demonstrates successful experience of surgical treatment of a patient with giant neurofibroma affecting a massive pool of soft tissues.
导言。神经纤维瘤病 1 型的神经纤维瘤很少会长得很大。然而,在出现大面积病变的情况下,选择最佳治疗策略可能会很复杂。临床医生通常会采用手术治疗,这是目前改善患者生活质量和达到良好美容效果的有效手段。描述一名巨大神经纤维瘤 1 型患者的成功手术治疗经验。患者现年 22 岁,在 N.N. Blokhin 国立肿瘤医学研究中心就医,诊断为 1 型神经纤维瘤病,背部下三分之一、臀部和大腿组织出现大面积病变。患者自出生起就患有此病,家族病史也很复杂:父亲死于神经源性肿瘤的恶性表现。妹妹也被诊断出患有神经纤维瘤病,脑部受到影响,身体上出现咖啡斑。由于巨大的肿瘤大大降低了患者的生活质量,为了缓解患者的病情,医生为其开具了细胞切除手术治疗处方,旨在最大限度地根治切除所有肿瘤。本临床病例展示了手术治疗巨大神经纤维瘤患者的成功经验。
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引用次数: 0
Intraosseous resection of bone tumors: our experience 骨肿瘤的骨内切除术:我们的经验
Pub Date : 2024-04-08 DOI: 10.17650/2219-4614-2024-16-1-51-63
V. V. Teplyakov, S. V. Dobrosotsky, A. Shaposhnikov, A. V. Lazukin, A. P. Ukhvarkin, E. A. Saprykina, V. Solodky
Introduction. The effectiveness of efficient surgical interventions in patients with benign, borderline, and malignant bone tumors remains an important topic because such operations imply lower injury rate and volume of surgical intervention while being more physiological. In terms of radicality and consequently recurrence rates they are comparable to more traumatic surgeries such as segmental resections with various types of reconstructions (arthrodesis, endoprosthesis, auto- and allotransplantation using bone fragments, et al) and amputation.Aim. To analyze the results of intraosseous resection for benign, malignant, giant cell, and metastatic bone tumors.Materials and methods. Between 2015 and 2023, 45 interventions of intraosseous resection were performed in 44 patients: 19 (43 %) had enchondroma, 6 (14 %) – giant cell tumor, 4 (9 %) – fibrous dysplasia, 3 (7 %) – solitary bone cyst, 3 (7 %) – aneurysmal bone cyst, 2 (5 %) – chondromyxoid fibroma, 4 (9 %) – metastatic bone lesions, 1 (2 %) – osteoid osteoma, 1 (2 %) – benign fibrous histiocytoma, 1 (2 %) – chondrosarcoma G1 . Among them, 27 interventions were performed in combination with adjuvants (liquid nitrogen cryoablation, argon plasma coagulation, hydrogen peroxide, ethanol). In 24 patients, reconstruction was performed using lyophilized corpse bones, in 5 using autotransplant (fragments of the iliac crest), in 10 using plastic material (in 6 – polymethacrylate, in 4 – osseointegrative material “Rekost”), in 6 using xenoplastic material “Silorif.” Additionally, 16 surgeries were augmented with osteosynthesis (14 extramedullary and 3 intramedullary osteosyntheses).Results. Complications in the early postoperative period were observed in 3 (7 %) patients, in the late postoperative period in 2 (5 %) patients. At follow ups (between 2 and 97 months), 42 (95 %) patients did not have signs of recurrence. Musculoskeletal Tumor Society Score (MSTS) at these follow ups in recurrence-free patients was excellent (mean score – 93.6 %) and good (mean score – 77 %). Eastern Cooperative Oncology Group (ECOG) performance status also showed high functional potential of intraosseous resection: its mean value was 0.3.Conclusion. Use of intraosseous resection including augmentation with adjuvant measures, allowed to achieve excellent functional results in 42 (95 %) of patients with mean follow-up duration of 39.8 months. Fractures in 2 patients demonstrate the necessity of extramedullary osteosynthesis in surgeries on long bones experiencing high load irrespective of the volume of intervention. The obtained results show applicability and high effectiveness of intraosseous resection. For certain indications, it can be the treatment of choice.
导言。对良性、边缘性和恶性骨肿瘤患者进行高效手术干预的有效性仍然是一个重要课题,因为此类手术意味着更低的损伤率和手术干预量,同时更符合生理。就根治性和复发率而言,它们可与创伤更大的手术相媲美,如节段切除和各种类型的重建(关节置换术、内假体、使用骨碎片的自体和异体移植等)以及截肢手术。分析良性、恶性、巨细胞和转移性骨肿瘤的骨内切除术效果。2015 年至 2023 年间,对 44 名患者进行了 45 次骨内切除术:19例(43%)为软骨瘤,6例(14%)为巨细胞瘤,4例(9%)为纤维发育不良,3例(7%)为单发骨囊肿,3例(7%)为动脉瘤性骨囊肿、2(5 %)-软骨样纤维瘤,4(9 %)-转移性骨病变,1(2 %)-类骨瘤,1(2 %)-良性纤维组织细胞瘤,1(2 %)-软骨肉瘤 G1。其中,27 例患者的手术结合了辅助治疗(液氮冷冻消融、氩等离子凝固、过氧化氢、乙醇)。24名患者使用冻干尸骨进行了重建,5名患者使用自体移植(髂嵴碎片),10名患者使用塑料材料(6名患者使用聚甲基丙烯酸酯,4名患者使用骨结合材料 "Rekost"),6名患者使用异种塑料材料 "Silorif"。此外,16 例手术采用了骨合成技术(14 例髓外骨合成,3 例髓内骨合成)。术后早期出现并发症的患者有 3 例(7%),术后晚期有 2 例(5%)。在随访期间(2至97个月),42名患者(95%)没有出现复发迹象。在这些随访中,无复发患者的肌肉骨骼肿瘤协会评分(MSTS)为优(平均分-93.6%)和良(平均分-77%)。东部合作肿瘤学组(ECOG)的表现状态也显示了骨内切除术的高功能潜力:其平均值为 0.3。42例(95%)患者采用骨内切除术,包括辅助措施,取得了极佳的功能效果,平均随访时间为39.8个月。2 名患者的骨折表明,无论干预量有多大,在对承受高负荷的长骨进行手术时,都有必要进行髓外骨合成。获得的结果表明,骨内切除术的适用性和有效性很高。对于某些适应症,它可以作为首选治疗方法。
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引用次数: 0
Mechanisms of sexual dimorphism in the pathogenesis of sarcomas 肉瘤发病机制中的性双态性机制
Pub Date : 2024-04-08 DOI: 10.17650/2219-4614-2024-16-1-11-24
G. A. Belitsky, T. Fetisov, P. A. Shtompel, E. Lesovaya, B. Bokhyan, M. Yakubovskaya, K. Kirsanov
Sarcomas of non-genital organs affect women 1.5–3 times less often than men. The mechanism of such dimorphism is studied at various levels, mainly in the field of influence on the pathogenesis of sex hormones and their receptors, the effect of which is significantly related to the histogenesis of the tumor, its localization, the mechanism of malignant transformation and the stage of progression. Sex hormone receptors are often found in the early stages of tumor development and are lost during progression. At the same time, the expression of the same receptors in tumors of different histogenesis sometimes correlates with the opposite prognosis of the disease and sensitivity to hormonal therapy. For example, in uterine leiomyosarcomas, the expression of estrogen and androgen receptors correlates with a better prognosis and greater effectiveness of therapy, and in osteo- and fibrosarcomas, vice versa. Estrogens stimulate proliferation of osteosarcoma cells, and androgens that of rhabdomyosarcoma and small round cell tumor, which growth is inhibited by antiandrogens used in the treatment of prostate cancer. In this regard, when trying to include a hormonal component in a therapeutic complex, an individual study of the hormonal sensitivity of the tumor is necessary. One of the methodological approaches to this could be testing a culture of tumor cells from surgical material for sensitivity to agonists/antagonists of hormonal receptors separately and in combination with chemotherapy drugs.
非生殖器官肉瘤对女性的影响是男性的 1.5-3 倍。人们从不同层面研究了这种二态性的机理,主要是性激素及其受体对发病机理的影响,性激素及其受体的作用与肿瘤的组织发生、定位、恶性转化机制和进展阶段有很大关系。性激素受体通常出现在肿瘤发生的早期阶段,并在肿瘤发展过程中消失。同时,不同组织发生机制的肿瘤中相同受体的表达有时与疾病的预后和对激素治疗的敏感性相反。例如,在子宫白肌瘤中,雌激素和雄激素受体的表达与较好的预后和较高的治疗效果相关,而在骨肉瘤和纤维肉瘤中则相反。雌激素刺激骨肉瘤细胞增殖,雄激素刺激横纹肌肉瘤和小圆细胞瘤细胞增殖,而治疗前列腺癌的抗雄激素可抑制这些细胞的生长。因此,当试图在治疗复合物中加入激素成分时,有必要对肿瘤的激素敏感性进行单独研究。方法之一是从手术材料中培养肿瘤细胞,分别测试其对激素受体激动剂/拮抗剂的敏感性,以及与化疗药物联合使用的敏感性。
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引用次数: 0
Components of the microenvironment of soft tissue sarcomas. Part I 软组织肉瘤微环境的组成部分。第一部分
Pub Date : 2024-04-08 DOI: 10.17650/2219-4614-2024-16-1-25-33
A. Lokhonina, E. Jumaniyazova, D. Dzhalilova, A. Kosyreva, G. G. Kazaryan, T. Fatkhudinov
Soft tissue sarcomas (STS) are a rare heterogeneous group of malignant neoplasms characterized by aggressive course and poor response to treatment. This determines the relevance of research aimed at studying the pathogenesis of STS. The tumor microenvironment consists of stromal, immune cells, blood, lymphatic vessels, and extracellular matrix. By now, it is known that STS is characterized by complex relationships between tumor cells and components of the microenvironment. Dynamic interactions between tumor cells and components of the microenvironment enhance adaptation to changing environmental conditions, which provides high aggressive potential of STS and resistance to antitumor therapy. Basic research aimed at investigating the role of microenvironmental components in STS carcinogenesis may serve as a key to the discovery of both new prognostic predictor biomarkers and targets for new antitumor drugs. This review article is devoted to the role and interaction of STS cells with components of the microenvironment.
软组织肉瘤(STS)是一种罕见的异质性恶性肿瘤,其特点是病程凶险、治疗反应差。这就决定了研究软组织肉瘤发病机制的重要性。肿瘤微环境由基质、免疫细胞、血液、淋巴管和细胞外基质组成。目前已知,STS 的特点是肿瘤细胞与微环境成分之间关系复杂。肿瘤细胞与微环境成分之间的动态相互作用增强了对不断变化的环境条件的适应性,从而使 STS 具有很高的侵袭潜力,并对抗肿瘤治疗产生抗药性。旨在研究微环境成分在 STS 癌变中的作用的基础研究可能是发现新的预后预测生物标志物和新的抗肿瘤药物靶点的关键。这篇综述文章将专门讨论 STS 细胞与微环境成分的作用和相互作用。
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引用次数: 0
Validation of the Russian version of Spine Oncology Study Group Outcomes Questionnaire 脊柱肿瘤研究小组成果问卷俄文版的验证
Pub Date : 2024-04-08 DOI: 10.17650/2219-4614-2024-16-1-64-75
E. B. Tsyrenova, O. Y. Shchelkova, D. Sofronov, K. A. Borzov, A. Valiev, A. V. Kulaga, V. L. Matanova
Introduction. Surgical intervention is the only or main component of combination treatment of the majority of malignant tumors of the spine. Pronounced pain syndrome and neurological deficit caused by spinal tumors significantly worsen disease progression, decrease patients’ quality of life.Aim. To develop a Russian-language version of a specialized questionnaire to study the quality of life of patients suffering from tumor lesions of the spine Spine Oncology Study Group Outcomes Questionnaire (SOSG-OQ).Materials and methods. In 82 patients with tumors of various parts of the spine who are treated at the N.N. Blokhin National Medical Research Center of Oncology, psychometric evaluation of the SOSG-OQ questionnaire was performed confirming its convergent validity and reliability–consistency (Cronbach’s α = 0.899).Results. Exploratory factor analysis confirmed adequacy of the questionnaire’s structure and its conformity to the basic factor model. Instruments of the validated Russian version of the SOSG-OQ questionnaire (text and data analysis instructions) for its use in clinical practice are presented. Results obtained using the new SOSG-OQ questionnaire show a significant decrease in quality of life of patients with spinal tumors. Additionally, after surgery patients described their health-related quality of life significantly higher than prior to treatment.Conclusion. Validated Russian version of the SOSG-OQ questionnaire can be recommended for application in oncological clinic for more precise and differentiated evaluation of performance status, emotional condition, experience of patients, evaluation of life circumstances associated with the disease to promote more targeted and personalized psychological care.
简介手术治疗是大多数脊柱恶性肿瘤综合治疗的唯一或主要手段。脊柱肿瘤引起的明显疼痛综合征和神经功能缺损会显著恶化疾病的进展,降低患者的生活质量。编制俄语版专门调查问卷,研究脊柱肿瘤病变患者的生活质量 脊柱肿瘤研究小组结果问卷(SOSG-OQ)。对82名在N.N. Blokhin国立肿瘤医学研究中心接受治疗的脊柱不同部位肿瘤患者进行了SOSG-OQ问卷的心理测量学评估,确认了其收敛效度和信度一致性(Cronbach's α = 0.899)。探索性因素分析证实了问卷结构的适当性及其与基本因素模型的一致性。本文介绍了经过验证的俄文版 SOSG-OQ 问卷的工具(文本和数据分析说明),以便在临床实践中使用。使用新版 SOSG-OQ 问卷得出的结果显示,脊柱肿瘤患者的生活质量明显下降。此外,手术后患者对健康相关生活质量的描述明显高于治疗前。经过验证的俄文版 SOSG-OQ 问卷可推荐用于肿瘤临床,以更准确、更有区别地评估患者的表现状态、情绪状况、经历,评估与疾病相关的生活环境,从而促进更有针对性的个性化心理护理。
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引用次数: 0
A rare clinical case of diagnosis and treatment of metastases of Merkel carcinoma in the right inguinal lymph nodes without an identified primary focus 诊断和治疗右腹股沟淋巴结梅克尔癌转移的罕见临床病例,且未发现原发病灶
Pub Date : 2024-04-08 DOI: 10.17650/2219-4614-2024-16-1-81-87
K. Titov, I. Lebedinsky, M. M. Magomedova, S. R. Dzhamilov, D. Grekov, M. V. Sorokina, Yu. V. Karabach
Introduction. Merkel’s carcinoma is a neuroendocrine malignant epithelial skin tumor, rapidly progressive, prone to local recurrence and metastasis to regional lymph nodes and internal organs. The etiology and pathogenesis of Merkel’s carcinoma are still an insufficiently studied issue. Because of its rare occurrence, only single observations of Merkel’s carcinoma metastasis without an identified primary focus, most often with regional lymph node involvement, have been described in the world literature.Clinical case. This article presents a clinical case of metastatic lesion of inguinal lymph nodes with microinvasion into femoral artery in Merkel’s carcinoma without identified primary focus. After a comprehensive examination and onco-consilium the patient underwent surgical treatment in the scope of cytoreductive excision of the inguinal lymph node conglomerate on the right side and artery prosthesis with an autovenous graft. According to the routine postoperative pathomorphologic and immunohistochemical studies, the diagnosis of Merkel’s carcinoma metastasis was finally confirmed.Conclusion. Identification and description of such clinical cases are practically significant and can serve for formation of certain algorithms for treatment of Merkel’s carcinoma both with local manifestations and in progressive forms. Patients with Merkel’s carcinoma should be discussed in oncological consiliums, as treatment may include surgical stage, including biopsy of sentinel lymph nodes, adjuvant radiotherapy, chemotherapy and immunotherapy in case of tumor dissemination.
简介梅克尔癌是一种神经内分泌恶性上皮性皮肤肿瘤,进展迅速,易局部复发和向区域淋巴结及内脏转移。梅克尔癌的病因和发病机制仍是一个研究不足的问题。由于梅克尔癌的罕见性,世界文献中仅报道了单例梅克尔癌转移病例,且未发现原发灶,多伴有区域淋巴结受累。本文介绍了一例腹股沟淋巴结转移病变并伴有股动脉微侵犯的梅克尔癌临床病例。在进行了全面检查和脐带穿刺后,患者接受了右侧腹股沟淋巴结清扫性切除和自体静脉移植动脉假体的手术治疗。根据术后常规病理形态学和免疫组化检查,最终确诊为梅克尔癌转移。对此类临床病例的识别和描述具有重要的实际意义,有助于形成治疗梅克尔癌的特定算法,无论是局部表现还是进展期形式。梅克尔癌患者应在肿瘤联盟中进行讨论,因为治疗可能包括手术分期,包括前哨淋巴结活检、辅助放疗、化疗以及肿瘤扩散时的免疫治疗。
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引用次数: 0
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Bone and soft tissue sarcomas, tumors of the skin
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