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Bone and soft tissue sarcomas, tumors of the skin

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Vol 12, No 4 (2020)
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EDITORIAL

11-21 497
Abstract

The article represents a literature review of the giant cell tumor management based on the RANKL overexpression and according this use of the denosumab.Weconsidered the pathogenesis of giant cell tumor based on the process of osteoclastogenesis (RANK/RANKL) discovered in the 1990s and a new H3F3A driver mutation. The history, experience and results of the denosumab treatment, as well as the issues of evaluation and interpretation of the treatment response are represented.

TUMORS OF THE SKIN

22-31 137
Abstract

Melanoma  of  the  skin  (MS)  is  a  tumor  with  a  predictable  course,  when  diagnosed  at  the  pT1a  stage, the patient has an almost 100 % chance of curing the disease. Diagnosis of MS is carried out mainly by doctors of primary contact, the accuracy of the diagnosis is low. In a large industrial region with more than three million people, a study was conducted that showed a significant increase in the number of cases. In this regard, a multi-agent technology for  melanoma  diagnostics  was  developed  in  the  Samara  region.  From  2010  to  2019,  an  experiment  was  carried  out to introduce a multi-agent system for early diagnosis of MS, the results were analyzed for the period of its use (2010– 2019) and in comparison with another period – 2000–2009.

The aim of the study is to assess the effectiveness of early diagnosis of MS in the period from 1995 to 2019 in the Samara region in connection with the introduction of multi-agent technology.

Materials and methods.A retrospective analysis of the quality of diagnostics was carried out using a multi-agent system, consisting of the author’s expert system based on artificial intelligence (patent for invention No 2018620399, 2018613016), and without its implementation for the periods from 2000–2009 and 2010–2019. The primary documentation was analyzed: form 7, 35, 025/u. The coefficients of reliability (t, p) of the proportion of stages I–II and “thin” skin melanoma, linear and polynomial trend approximations were calculated. The result of histological examination was used as a reference method.

Results.It was shown that the proportion of diagnosed MS at stages I–II significantly increased as a result of the introduction of a multi-agent system (t= 0.04113219, p<0.05), the proportion of “immature” MS did not give statistically significant changes (t= 0.07303817, p>0.05).

Conclusion.It was concluded that for effective diagnosis of MS at stages I–II, the use of multi-agent technology is sufficient, but for diagnosis at the pre-invasive stage it is ineffective and requires an increase in the number of trained expert doctors.

32-39 214
Abstract

Aim of the study– to analyze the molecular genetic profile of rare melanocytic tumors in children, including skin melanoma.

Materials and methods.The study of melanocytic neoplasms tissue samples of 11 pediatric patients with melanocytic tumors was carried out by the method of targeted genomic DNA sequencing. The coding regions of genes involved in the processes of melanogenesis, melanomogenesis, proliferation, cell adhesion were investigated.

Results.It was found that in two cases in giant congenital melanocytic nevi there were mutations in the NRASgene, the same mutations were found in samples of melanomas that developed against the background of these nevi. In both cases, the developed melanomas were highly malignant. In one patient, a mutation in the CTNNB1 gene was detected in a melanoma sample, which is absent in the nevi tissue cells. In two cases of Spitz nevus, in the lentignous melanocytic nevus and in the Reed nevus, mutations in the promoter region of the TERTgene (c.-269G> A and c.-348G> C) were detected. The V600E mutation in the BRAFgene was found in dysplastic nevus and Reed nevus; in the latter case, its combination with mutations in the TERT gene promoter was revealed.In a sample of a blue nevus, the p.Q209L mutation in the GNAQ gene was detected, which is a pathognomonic genetic aberration, associated with this type of melanocytic nevus.

Conclusion.The study of the spectrum of mutations in samples of children with rare melanocytic tumors, including melanoma, indicates that the type of mutation identified was largely associated with the clinical characteristics of the nevus.

SOFT TISSUE SARCOMAS

40-47 385
Abstract

It performed the analysis of literature data on development of isolated limb perfusion (ILP) in the treatment of soft tissue sarcomas (STS), its clinical efficacy and safety. The historical aspects of the method introduction into clinical practice are considered. It is noted that for more than two decades the method has been used for locally advanced limb sarcomas, unifocal tumors localized in the neurovascular bundle with invasion into the bundle or bone, tumor recurrence in previously irradiated areas without the possibility of radical resection. The technique of performing the method, drugs (melphalan and tumor necrosis factor-α) and their modes of application are described.

It was noted that systemic toxicity during ILP is directly associated with the volume of the drug entering the systemic circulation, as a rule, the value of this indicator is less than 3–4 %. Discussion issues of the criteria for the effectiveness of STS treatment are considered, it is indicated that the main result of ILP is used by most authors the criterion of the extremity rescue frequency, its value ranges from 72 to 96 %. Survival without signs of local recurrence within 5 years after ILP with melphalan/tumor necrosis factor-αand tumor resection is from 73 to 87 %. The tasks of improving ILP are described, including the need to search for combined treatment regimens for local and systemic therapy in patients who undergo this treatment. Promising directions are the improvement of forecasting tools and the creation of innovative drugs that can be used in ILP, which will contribute to a higher clinical efficacy and safety of the method in the treatment of STS.

BONE TUMORS

48-58 300
Abstract

The article is devoted to description of a new method of reconstruction and plastic surgery of the acetabulum after partial resection  of  the  bones  (pubic,  sciatic,  iliac)  forming  it,  with  tumor  lesions. A  new  method  characterized  in that  due to the use of biosynthetic tissue and anchor screws with threads and needles, as well as muscle flaps moved for plastic surgery – allows you to comply with the principles of cancer radicalism, to avoid endoprosthetics of the acetabulum and hip joint, to prevent dislocation of the femoral head, with an uncomplicated postoperative period and the ability to achieve good and excellent functional results in a short time.

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ISSN 2219-4614 (Print)
ISSN 2782-3687 (Online)