scholarly journals Oncoepidemiological situation of sarcomas in Kazakhstan

2020 ◽  
Vol 30 (Supplement_5) ◽  
Author(s):  
Z h U Pyssanova ◽  
G A Serikbaev ◽  
D R Kaydarova ◽  
D A Tuleuova ◽  
A S Shinbolatova ◽  
...  

Abstract Background Sarcoma is a malignant tumor of soft tissues and bones. They make up 1% of all malignant tumors in adults and 15% in children. More common in men 1.1: 1. By age: 20.7% - up to 40 years old, 27.6% - 40-60 years old and 51.7% over 60 years old. In 2018, in the Kazakhstan, malignant tumor of connective and soft tissues of 444 (1.4%) cases was registered and ranks 19th in the structure of cancer, bones and articular cartilage 188 (0.6%), takes 22nd place. Of these, with sore cases, bone sarcomas were recorded - 12.7%, soft tissues - 9.5%. The mortality rate from soft tissue sarcoma is -177 (1.2%) people, bone sarcoma - 85 (0.6%) people. Five-year survival of patients from soft tissue sarcomas is 53.8%, from bone sarcomas - 67.3%. Methods from 2015 to 2019, 1,068 patients with malignant tumor of bones and articular cartilage, 963 patients with malignant tumor of connective and soft tissues addmited to KazNIIOiR. Of these, 225 patients underwent organ-sparing surgeries - endoprosthesis replacement of limbs. Results In Kazakhstan over the past 5 years there has been an increase in the incidence of sarcomas of bones and soft tissues. Conclusions in comparison with previous years, there is an increase in the incidence of malignant tumor of bones and articular cartilage, connective and soft tissues, and an increase in mortality from tumor of bones and articular cartilage in men by 16.7%, in women by 50%. Late reversal of patients at the IV stage and a high proportion posthumously recorded in patients with maliganan tumors. Key messages Purpose: Assessment of the epidemiological situation and incidence of sarcomas of the bones and soft tissues in Kazakhstan. Out of 25 localizations of cancer, detection is worsened during professional examinations in 10 localizations, of which bones and articular cartilage, connective and other soft tissues.

Author(s):  
Jasminka Igrec ◽  
Michael H. Fuchsjäger

Background In the diagnosis of bone and soft-tissue sarcomas, the continuous advancement of various imaging modalities has improved the detection of small lesions, surgical planning, assessment of chemotherapeutic effects, and, importantly, guidance for surgery or biopsy. Method This review was composed based on a PubMed literature search for the terms “bone sarcoma,” “bone cancer” and “soft tissue sarcoma,” “imaging,” “magnetic resonance imaging”, “computed tomography”, “ultrasound”, “radiography”, and “radiomics” covering the publication period 2005–2020. Results and Conclusion As discussed in this review, radiography, ultrasound, CT, and MRI all play key roles in the imaging evaluation of bone and soft-tissue sarcomas. In daily practice, advanced MRI techniques complement standard MRI but remain underused, as they are considered time-consuming, technically challenging, and not reliable enough to replace biopsy and histology. PET/MRI and radiomics have shown promise regarding the imaging of sarcomas in the future. Key Points:  Citation Format


Bone sarcomas 296 Soft tissue sarcomas 300 Surgical treatments 302 Metastatic bone disease (MBD) 306 Bone sarcomas are extremely rare, with less than 550 new cases per annum in the UK. They account for only 0.2% of all new cancers, but 5% of childhood cancers. The main types of bone sarcoma/tumour are:...


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. 11550-11550
Author(s):  
Varun Monga ◽  
Rebecca Dodd ◽  
Amanda Scherer ◽  
Wade Robert Gutierrez ◽  
Munir Tanas ◽  
...  

11550 Background: Sarcomas are a heterogeneous group of tumors and are associated with high rates of metastases leading to poor prognosis. Numerous epigenetic changes including hypermethylation have been identified in several sarcoma subtypes. Restoration of normal methylation patterns using DNA hypomethylating agent when combined with chemotherapy has shown to slow tumor growth in preclinical studies. Low continuous dosing of hypomethylating agent has epigenetic modulating effect with less toxicity. We conducted a Phase 1b study evaluating safety & tolerability and identifying the recommended phase 2 dose (RP2D) of subcutaneous (SQ) decitabine (DEC) given with fixed dose infusion gemcitabine (GEM) in patients with advanced soft tissue sarcomas (STS) and bone sarcomas. Methods: Eligible patients at least age 18 yrs with metastatic histologically confirmed STS or bone sarcoma after progression on one line or if refused adriamycin for STS were included. Prior GEM use was permitted. A modified 3+3 dose escalation design was used exploring two dose cohorts of DEC, 0.1 and 0.2 mg/kg SQ administered on a twice weekly schedule for three weeks and GEM given as 900 mg/m2, IV over 90 min on days 1, 8 & 15 of a 28-day cycle. Treatment was continued until disease progression or unacceptable toxicity. Dose limiting toxicity (DLT) was defined as any drug related non-hematological grade 3 or 4 toxicity per CTCAE v4.0. Disease assessment was performed every 8 weeks using RECIST v1.1. Results: 31 patients (25 STS & 6 bone sarcomas) were enrolled of which 7 were non-evaluable. There were 12 evaluable patients in each dosing cohort. Of the total 744 adverse events (AE) 17.2% were grade 3/4 and most were neutropenia without neutropenic fever. 45.7% AEs were possibly (44.6%) or probably (1.1%) attributed to DEC use. The toxicities were not significantly different between DEC doses. No DLTs were observed. One patient died due to progressive disease. Conclusions: Combination of fixed dose infusion GEM with low dose subcutaneous DEC is moderately toxic. Most toxicities were hematological. While there were few responses, the RP2D of DEC selected was 0.1 mg/kg as it showed prolonged disease stabilization. Clinical trial information: NCT02959164 . [Table: see text]


Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 512
Author(s):  
Celine Jacobs ◽  
Lore Lapeire

Soft tissue sarcomas are a group of rare mesenchymal tumors with more than 70 subtypes described. Treatment of these subtypes in an advanced setting is mainly according to a one-size-fits-all strategy indicating a high unmet need of new and more targeted therapeutic options in order to optimize survival. The introduction of advanced molecular techniques in cancer has led to better diagnostics and identification of new therapeutic targets, leading to more personalized treatment and improved prognosis for several cancer types. In sarcoma, a likewise evolution is seen, albeit at a slower pace. This manuscript describes how in the past years advanced molecular profiling in soft tissue sarcomas was able to identify specific and often pathognomonic aberrations, deferring standard sarcoma treatment in favor of more targeted treatment from an oncologist’s point of view.


Cancers ◽  
2021 ◽  
Vol 13 (15) ◽  
pp. 3865
Author(s):  
Tânia Fortes-Andrade ◽  
Jani Sofia Almeida ◽  
Luana Madalena Sousa ◽  
Manuel Santos-Rosa ◽  
Paulo Freitas-Tavares ◽  
...  

Soft-tissue sarcomas (STS) represent about 80% of sarcomas, and are a heterogeneous group of rare and malignant tumors. STS arise from mesenchymal tissues and can grow into structures such as adipose tissue, muscles, nervous tissue and blood vessels. Morphological evaluation has been the standard model for the diagnosis of sarcomas, and even in samples with similar characteristics, they present a diversity in cytogenetic and genetic sequence alterations, which further increases the diversity of sarcomas. This variety is one of the main challenges for the classification and understanding of STS patterns, as well as for their respective treatments, which further decreases patient survival (<5 years). Despite some studies, little is known about the immunological profile of STS. As for the immunological profile of STS in relation to NK cells, there is also a shortage of studies. Observations made in solid tumors show that the infiltration of NK cells in tumors is associated with a good prognosis of the disease. Notwithstanding the scarcity of studies to characterize NK cells, their receptors, and ligands in STS, it is noteworthy that the progression of these malignancies is associated with altered NK phenotypes. Despite the scarcity of information on the function of NK cells, their phenotypes and their regulatory pathways in STS, the findings of this study support the additional need to explore NK cell-based immunotherapy in STS further. Some clinical trials, very tentatively, are already underway. STS clinical trials are still the basis for adoptive NK-cell and cytokine-based therapy.


2021 ◽  
Vol 22 (14) ◽  
pp. 7518
Author(s):  
Marcella Tazzari ◽  
Laura Bergamaschi ◽  
Alessandro De Vita ◽  
Paola Collini ◽  
Marta Barisella ◽  
...  

Soft tissue sarcomas (STSs) are a family of rare malignant tumors encompassing more than 80 histologies. Current therapies for metastatic STS, a condition that affects roughly half of patients, have limited efficacy, making innovative therapeutic strategies urgently needed. From a molecular point of view, STSs can be classified as translocation-related and those with a heavily rearranged genotype. Although only the latter display an increased mutational burden, molecular profiles suggestive of an “immune hot” tumor microenvironment are observed across STS histologies, and response to immunotherapy has been reported in both translocation-related and genetic complex STSs. These data reinforce the notion that immunity in STSs is multifaceted and influenced by both genetic and epigenetic determinants. Cumulative evidence indicates that a fine characterization of STSs at different levels is required to identify biomarkers predictive of immunotherapy response and to discover targetable pathways to switch on the immune sensitivity of “immune cold” tumors. In this review, we will summarize recent findings on the interplay between genetic landscape, molecular profiling and immunity in STSs. Immunological and molecular features will be discussed for their prognostic value in selected STS histologies. Finally, the local and systemic immunomodulatory effects of the targeted drugs imatinib and sunitinib will be discussed.


Author(s):  
Kunhi Mohammed K. P. ◽  
Snehasis Pradhan ◽  
Supratim Bhattacharyya ◽  
Prafulla Kumar Das ◽  
Muhammed Navas N. K.

Background: Soft tissue sarcomas are a rare and heterogeneous group of malignant tumors of mesenchymal origin that comprise less than 1 percent of all adult malignancies. Although they occur anywhere in the body, they involve most commonly in extremities, trunk, retroperitoneum and head and neck. The aim of the study was to analyze clinical and histopathological features of various soft tissue sarcomas.Methods: This was a retrospective study, conducted in tertiary cancer centre in Odisha during the period 2015 to 2018. We collected clinical parameters like age, sex, site of swelling, any associated pain and biopsy reports and these variables were correlated with final histopathology reports.Results: A total of 107 patients were included in the study, with male to female ratio of 2:1(71 and 36) and average age of 43.45 years. All of them presented with a swelling. The lower extremities were the most common sites i.e. 44.62%. Pleomorphic sarcoma was the most frequent histologic variety comprising 43% and less frequent variety were angiosarcoma, and myxoid sarcoma.Conclusions: Soft tissue sarcoma are predominant in males and middle aged population are frequently affected. Most common affected site is lower extremity and pleomorphic sarcoma is the prominent histologic type.


2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Asmae Mazti ◽  
Mohamed El Idrissi ◽  
Abdelhalim El Ibrahimi ◽  
Mustapha El Maaroufi ◽  
Ghizlane El Koubaiti ◽  
...  

Soft-tissue sarcomas are malignant tumors that require good management within specialized centers. Our study aims to assess the benefit of handling these kinds of tumors using the Multidisciplinary Meeting (MDM) approach. The current paper details this approach through a prospective study that has lasted for 42 months in the HASSAN II University Hospital Center, Fez, Morocco. During this research work, 116 cases were selected with an average age of 53 years. In 95.7% of the cases, it was found that the lower limb was the most frequent tumor type (78.4%). Also, ninety-two (92) patients (79.3%) have had a prior biopsy. Ninety-nine (99) patients (85.3%) have received a magnetic resonance imaging scan (MRI) before surgery. Sixty-three (63) patients were operated on, including R0 resection used for 37 patients, R1 used for 21 patients, and R2 used for five patients. As a result, liposarcomas were the most frequent type (30.1%), followed by synovial sarcomas (14.6%), leiomyosarcomas (9.5%), ewing sarcoma (8.6), and undifferentiated pleomorphic sarcomas (7.7%). In addition, neoadjuvant chemotherapy was used for 36 patients. The other 22 patients received adjuvant chemotherapy and/or radiotherapy. The overall survival rate was 60.56 months, which proves a significant improvement, thanks to the multidisciplinary meeting approach. Conclusion. The conducted investigation has shown that using MDM for managing soft-tissue sarcomas of extremities improves the patients’ survival rate. Moreover, results have proven MDM might allow optimal treatment regarding less local recurrence and metastasis.


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