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Bone and soft tissue sarcomas, tumors of the skin

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Vol 12, No 2-3 (2020)
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EDITORIAL

5-15 239
Abstract

Introdaction. In the general structure of oncological endoprosthetics, primary lesions of the distal tibia are rarely affected. Endoprosthetics of the ankle joint region are associated with characteristic difficulties consisting in soft tissue deficiency and restoration of the biomechanics of the lost joint. The existing scientific volume of the literature data on primary ankle replacement is insignificant. At the moment, there is no experience and clear strategy in revision endoprosthetics of this area, complications remain insufficiently explored.

Objective. The aim of the study was a discrete analysis of medium- and long-term results, the structure of complications, and functional results in a group of patients after primary and repeated oncological ankle replacement.

Materials and methods. The study included 20 patients with benign and malignant bone tumors, who from July 2008 to November 2019 underwent 33 primary and revision replacements in case of distal tibia tumor lesion. In the study group of patients, 70 % were diagnosed with a primary malignant tumor and 30 % had a benign lesion of the tibia. The mean follow-up period was 58,6 months.

Results. The leading complication after primary and revision endoprosthetics was early aseptic instability (type IIA) – 20,0 and 23,1 %, respectively. Primary and revision endoprosthetics survival after 5 years was 40,1 ± 12 %.

Conclusion. The average functional result after primary and revision ankle replacement was evaluated according to the MSTS scale and after 6 months was 70,5 % and varied from 40 to 87 %. After 12 months, this figure was 76 % and ranged from 46,7 to 96,7 %.

The choice of an endoprosthesis, taking into account the optimal biomechanics design of the endoprosthesis unit, methods of fixation, the introduction of innovative technological solutions in the design, materials of the endoprosthesis, adherence to the principle of radicalism will become a means of reducing the frequency of complications.

TUMORS OF THE SKIN

16-25 231
Abstract

Introduction. Skin melanoma in children is a very rare disease that differs in many aspects from skin melanoma in adults. The conventional criteria for clinical diagnostics of melanoma observed in adults are often absent in children and adolescents, making it difficult to make a diagnosis on time.

Objective. The aim of the study is to assess the clinical significance of various diagnostic criteria in the analysis of a rare case of congenital pigment skin neoplasm in a child.

Materials and methods. The clinical case of a congenital melanocytic lesion in a wrist joint area in a girl 5 months old.

During the primary diagnosis, an ultrasound examination was carried out. The tumor excision was performed with replacement of defect using moved skin flap. Histological, genetic and cytogenetic (fluorescent hybridization in situ, FISH) examination of a tumor sample was carried out.

Results. The histological examination revealed areas of cellular atypia with high mitotic activity and the presence of pathological mitoses. According to FISH results, an increase in the copy of the RREB1 gene was observed in the sample, which may indicate amplification of this chromosome region. Genetic testing revealed a Q61K mutation in the NRAS gene.

Conclusion. Despite the presence of histological features of neoplasm malignancy and ambiguous FISH results, the described case should be considered as congenital nevus with atypical proliferative nodes based on combination of clinical data (early age at the time of surgery, expansive growth pattern, multiplicity of nodes, lack of necrosis, long relapse-free period).

SOFT TISSUE SARCOMAS

26-35 203
Abstract

Objective. The  aim  of  this  study  is  to  improve  the  results  of  surgical  treatment  of  lung  metastases  of  soft  tissue sarcomas, to analyze the factors that may affect the effectiveness of surgical treatment.

Materials and methods.The study included 80 patients with metastases of bone sarcomas in the lungs, who received treatment in the period from 2000 to 2013. Among the patients there were 42 (48,5 %) men and 38 (51,5 %) women.

The median age at diagnosis was 33 years (range 17 to 75). The majority of soft tissue sarcomas were represented by malignant  fibrous  histiocytoma  (27,5 %)  and  synovial  sarcoma  (28,5 %).  Almost  all  patients  (96 %)  underwent chemotherapy before surgery for lung metastases. The indication for surgery in most cases (52,5 %) was metastases in the lungs as the only manifestation of the disease. Most often, operations were performed with unilateral lung lesions (67,5 %).

Results. Atypical  lung  resections  58  (72 %)  were  the  most  frequently  performed  volume  of  surgical  intervention, pneumonectomy, bilobectomy and lobectomy were used in 17 (22 %) cases. Combined resections were performed in six patients. Postoperative complications were observed in 6 (7,5 %) patients. The radicality of the operations performed in the study group was 93 % in the R0 volume, in the R1 volume, 4 % of the operations were performed, in the R2 volume, 1 patient was operated on. The most frequent postoperative complication was pneumonia, one patient died in the early postoperative period from complications of surgery. The median follow-up in the study group was 49,2 months (from 3,5 to 239 months). The overall 5-year survival rate in the study group was 41,5 %. Five-year overall survival was more than 30 % higher in the group of patients who had less than 3 metastatic lesions at baseline. Only the following factors have a direct impact on OS indicators: the number and size of metastatic foci, radical removal of metastatic foci, the effect of previous chemotherapy, and the timing of the development of metastatic lesions. In our study, there were also no differences in OS and PFS depending on the surgical approach used.

Conclusion.Surgical treatment is currently fully justified and the only really effective treatment method that can lead to longer-term survival in patients with metastases of sarcomas in the lungs, despite the fact that some metastases have  a  clear  resistance  to  all  types  of  conservative  therapy,  while  The  best  treatment  results  are  achieved  in  the presence of a long disease-free interval (DFI), a small number of metastases and their removal in the R0 volume.

BONE TUMORS

36-44 349
Abstract

The objective: аnalysis of complications after endoprosthetic reconstruction in children and adolescents according to the Henderson classification of failure of limb salvage surgery modified by the International Society of Limb Salvage committee (ISOLS).

Materials and methods.This study evaluates complications rates and mode of failures after endoprosthetic reconstruction for the tumor around the knee in 97 patients treated from 2012 to 2018 in D. Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology.

Results. Overall  19,6 %  of  failures  were  identified.  The  most  common  postoperative  complications  were  structural failu res, infection and soft-tissue failure (6,19; 5,15 and 3,1 % respectively). None of the developed failures affected the timing of treatment protocol.

Conclusion. Literature  review  and  the  study  results  demonstrate  that  higher  risk  of  failure  in  the  paediatric  group of patients is attributed to deficiency of adjacent soft-tissue and bone, skeletal immaturity, using of expandable endoprostheses and increased patient activity at this age. Multicenter studies are needed to optimize prevention and management of failures after endoprosthetic reconstruction.

45-53 207
Abstract

Introdaction.Ewing’s sarcoma of the pelvic bones in children and adolescents is a complex, poorly studied problem. This is explained by the rare occurrence of the tumor and its biological characteristics: aggressive course, a tendency to develop early hematogenous metastases and frequent relapses.

The objective: analysis of the results of treatment of 72 children and adolescents with localized and disseminated Ewing sarcoma with primary lesions of the pelvic bones was carried out.

Materials and methods.During the study, a retrospective and prospective analysis of the results of treatment of 72 children and adolescents with localized and disseminated Ewing sarcoma with primary lesions of the pelvic bones was carried out. The patients were divided into 3 clinical groups (Induction, High-dose chemotherapy, Polychemotherapy) depending on the treatment performed. The patients received treatment under the MMES-99 protocol.

Results.The overall 1-year survival rate of all 72 patients included in the study was 86,1 %, 3-year – 50,0 %, 5-year – 42,5 % and 10-year – 39,3 %; disease-free survival rate – 66,6; 43,9; 41,9 % and 38,3 % respectively. Our analysis of overall survival did not reveal statistically significant differences between the High-dose chemotherapy and Polychemotherapy groups (p= 0,83). There were also no statistically significant differences in relapse-free survival (p= 0,68).

Conclusion.Considering that the indicators of overall and relapse-free survival did not significantly differ when performing one or another type of consolidation (High-dose chemotherapy versus Polychemotherapy groups) in patients with a good antitumor effect achieved on induction, when choosing the type of consolidation, one should take into account the capabilities of the medical institution, the presence of experience and equipment for carrying out autologous transplantation, the desired total duration of therapy (with High-dose chemotherapy, it is significantly shorter), as well as the total cost of the program.

REHABILITATION

54-59 267
Abstract

The article is devoted to description of psychological assistance program in oncoorthopedic clinic, based on the results of studying psychological characteristics and dynamics of the main parameters of quality of life in patients with oncoorthopedic pathology.The main stages of complex psychological assistance are presented, approaches to psychodiagnostics in oncoorthopedic clinic are proposed. The main directions of psychological assistance for patients with bone tumors are identified and substantiated.

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