scholarly journals A sarcoma synoviale kezelési lehetőségei

2015 ◽  
Vol 156 (22) ◽  
pp. 875-880
Author(s):  
Dániel Deme ◽  
András Telekes
Keyword(s):  
Metastatic Disease ◽  
Tumour Size ◽  
Locally Advanced ◽  
Soft Tissue Sarcomas ◽  
Disease Recurrence ◽  
The Body ◽  
Malignant Neoplasms ◽  
Biological Behaviour ◽  
Synovial Sarcomas

Synovial sarcomas account for approximately 5 to 10% of soft tissue sarcomas and 0.05 to 0.1% of all malignant neoplasms. They predominantly affect the extremities but can occur in any part of the body. More than 50% of the patients are expected to develop metastatic disease within 3–5 years. In some patients disease recurrence may develop after 20 years. The 5-year overall survival rate is 10% for patients with metastatic disease and 76% for patients with localized one. Age, tumour size, histological subtype, and adjuvant radiotherapy influence prognosis. The role of adjuvant chemotherapy has not been proven yet. There are several ongoing clinical trials to determine the efficacy of active agents used for therapy of locally advanced, relapsed/refractory or metastatic disease. Better understanding of the biological behaviour of synovial sarcomas would provide the future way for the targeted therapy in combination with conventional treatments. Orv. Hetil., 2015, 156(22), 875–880.

Role of Surgery for Locally Advanced and Metastatic Renal Cell Carcinoma

2011 ◽  
Vol 9 (9) ◽  
pp. 985-993 ◽  
Author(s):  
Robert Torrey ◽  
Philippe E. Spiess ◽  
Sumanta K. Pal ◽  
David Josephson
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Metastatic Renal Cell Carcinoma ◽  
Metastatic Disease ◽  
Renal Cell ◽  
Locally Advanced ◽  
Disease Stage ◽  
Disease Characteristics ◽  
Multimodal Management

Both locally advanced and metastatic renal cell carcinoma (RCC) present a challenge in terms of their optimal management. This article reviews the literature and evaluates the role of surgery in the treatment of advanced RCC. Surgery is the optimal treatment for locally advanced RCC and minimal, resectable, metastatic disease. Patients with metastatic disease, and some forms of locally advanced disease, may also benefit from multimodal management with local surgical therapy and systemic treatment using either immunotherapy or targeted therapy. Regardless of the disease stage, patients with locally advanced or metastatic RCC represent heterogenous patient populations with different disease characteristics and risk factors. Individualization of care in the setting of a sound oncologic framework may optimize the risk/benefit ratio within individual patient cohorts.

Solitary fibrous tumor: A Brazilian cohort analysis.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 11068-11068
Author(s):  
Marcelle Goldner Cesca ◽  
Celso Lopes Mello ◽  
Tiago Felismino ◽  
Maria Nirvana Formiga ◽  
Ulisses Ribaldo Nicolau ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Systemic Treatment ◽  
Cohort Analysis ◽  
Locally Advanced ◽  
Soft Tissue Sarcomas ◽  
Primary Objective ◽  
The Body ◽  
Cancer Center ◽  
Wide Range ◽  
Fibrous Tumor

11068 Background: Solitary fibrous tumor (SFT) is a rare mesenchymal tumor that account for less than 2% of all soft tissue sarcomas. SFT has been identified in multiple anatomic locations and can arise anywhere in the body. Surgical management is the mainstay of treatment for localized disease. However, about 20% will develop locoregional recurrences or distant metastasis with a role for systemic treatment. Methods: A retrospective analysis was carried out in a large cancer center in Brazil. Our primary objective was to evaluate clinical and treatment aspects of metastatic/ locally advanced (Mtx/LA) SFT cohort and secondary to describe clinical characteristics of entire population diagnosed with SFT. Descriptive statistics was used for main results. Survival curves were estimated using Kaplan-Meier. Data were retrieved from electronic patient medical records. Results: From April, 1971 to October, 2017, 82 patients with SFT were treated. Median follow-up was 45.5 months. 67 (81.7%) were alive on the cut-off date. Median age at diagnosis was 51 (14-78). 40.2% men. Most common primary sites (PS) were pleura (19.8%), central nervous system (CNS - 11%) and pelvis (11%). 18 (21.9%) underwent chemotherapy for Mtx/LA disease. In this subgroup, 61.1% were men; PS retroperitoneal (22.2%), extremities (16.7%), CNS (16.7%). 66.7% had pulmonary, 44.4% hepatic, 27.8% bone metastasis and one (5.5%) local recurrence. All patients had at least one adverse prognostic factor (tumor size ≥ 10cm, positive margins, necrosis, ≥ 4/10 mitosis). One (5,5%) had Doege-Potter syndrome. 7 (38.9%) did one, 5 (27.8%) two and 6 (33.3%) ≥ 3 lines of treatment. First line was temozolomide/bevacizumab (TMZ/Bev) in 55.6%, followed by chemotherapy (Ch) in 27.8% and tyrosine kinase inhibitors (TKI) in 16.7%. Median progression-free survival was 3.5 months (95% IC: 0.0-7.4) and overall survival 27.3 months (95% IC: 18.7-36.0). Response rate using RECIST criteria was 12.5% for TMZ/Bev and 62.5% had stable disease. TKI and Ch had no response. Conclusions: SFT is rare and with heterogeneous clinical presentation. In our analysis, patients received a wide range of therapy, reflecting the lack of well-established systemic treatment option. TMZ/Bev showed consistent activity in Mtx/LA scenario.

scholarly journals Recurrent DMD Deletions Highlight Specific Role of Dp71 Isoform in Soft-Tissue Sarcomas

Cancers ◽  
2019 ◽  
Vol 11 (7) ◽  
pp. 922 ◽  
Author(s):  
Olivier Mauduit ◽  
Vanessa Delcroix ◽  
Tom Lesluyes ◽  
Gaëlle Pérot ◽  
Pauline Lagarde ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Cell Lines ◽  
Cell Cycle Progression ◽  
Soft Tissue Sarcomas ◽  
Metastatic Progression ◽  
Gene Encoding ◽  
M Phase ◽  
Synovial Sarcomas ◽  
Dmd Gene

Soft-tissue sarcomas (STS) are rare tumors whose oncogenesis remains unknown and for which no common therapeutic target has yet been identified. Analysis of 318 STS by CGH array evidenced a frequent deletion affecting the DMD gene (encoding dystrophin isoforms) in 16.5% of STS, including sarcomas with complex genomics, gastrointestinal tumors (GIST), and synovial sarcomas (SS). These deletions are significantly associated with metastatic progression, thus suggesting the role of DMD downregulation in the acquisition of aggressive phenotypes. We observed that targeted deletions of DMD were restricted to the 5’ region of the gene, which is responsible for the transcription of Dp427. Analysis of STS tumors and cell lines by RNA sequencing revealed that only the Dp71 isoform was widely expressed. Dp427 depletion had no effect on cell growth or migration. However, Dp71 inhibition by shRNA dramatically reduced the cell proliferation and clonogenicity of three STS cell lines, likely by altering the cell cycle progression through the G2/M-phase. Our work demonstrates that DMD deletions are not restricted to myogenic tumors and could be used as a biomarker for metastatic evolution in STS. Dp71 seems to play an essential role in tumor growth, thus providing a potential target for future STS treatments.

Adjuvant carboplatin for clinical stage I testicular seminoma in New Zealand centers: Retrospective analysis of efficacy and long-term adverse effects.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e16041-e16041 ◽  
Author(s):  
Elias Aris Chandran ◽  
Aaron Farai Chindewere ◽  
Richard T. North ◽  
Michael B. Jameson
Keyword(s):  
Adverse Effects ◽  
Retrospective Analysis ◽  
Tumour Size ◽  
Clinical Stage ◽  
The Body ◽  
Stage I ◽  
Malignant Neoplasms ◽  
Testicular Seminoma ◽  
Long Term Adverse Effects

e16041 Background: Adjuvant carboplatin reduces relapse risk in clinical stage I (CS1) testicular seminoma, though there is a paucity of long-term safety data. While some studies report that two cycles is more effective than one, there is no randomised trial evidence or consensus on the optimal number of cycles. European guidelines recommend a risk-based approach to consider adjuvant carboplatin in those with either rete testis involvement (RTI) or tumour size > 4cm, and surveillance for other patients. We report long-term outcomes of 2 cycles of adjuvant carboplatin dosed at area under the curve (AUC) of 7 as standard management of CS1 seminoma since 2000. Methods: We performed a retrospective analysis on treatment and outcomes of patients with CS1 seminoma who received adjuvant carboplatin from 2000 to 2016 at the Waikato, Lakes and Bay of Plenty District Health Boards. We also collected information on mortality, causes of death and subsequent malignant neoplasms (SMN). Results: Of 160 patients, median age 39 (range 20-73) years, 154 received 2 cycles of carboplatin: 148 dosed at AUC7 and six at AUC6; another six patients had one cycle of carboplatin AUC7, curtailed due to toxicity in 3 patients. Two relapses occurred: one at 10 months (in hindsight stage 2a at diagnosis) and one at 22 months (died of pulmonary embolism 2 months after achieving complete response with BEP chemotherapy). Neither RTI (present in 21.3%) nor tumour size > 4cm (in 43.1%) were predictive of relapse. No patients died of seminoma. At median follow up of 109 (range 17-209) months, relapse-free survival was 98.7%, overall survival was 97.5% and disease-specific survival was 100%. A SMN occurred in 11 patients (6.9%) at median 96 months and caused 4 deaths (melanoma, myeloma, small cell lung cancer and glioblastoma); 4 patients (2.6%) had a contralateral testicular germ cell tumour (at median 69 months). One patient had persistent grade 1 thrombocytopenia at 46 months. Conclusions: This data adds to the body of evidence that two cycles of carboplatin AUC7 is safe, effective adjuvant treatment for CS1 seminoma. It did not have significant long-term adverse effects in our population.

scholarly journals Immunotherapy Strategies for Gastrointestinal Stromal Tumor

Cancers ◽  
2021 ◽  
Vol 13 (14) ◽  
pp. 3525
Author(s):  
Junaid Arshad ◽  
Philippos A. Costa ◽  
Priscila Barreto-Coelho ◽  
Brianna Nicole Valdes ◽  
Jonathan C. Trent
Keyword(s):  
Monoclonal Antibodies ◽  
Soft Tissue ◽  
Kinase Inhibitors ◽  
Checkpoint Inhibitors ◽  
Locally Advanced ◽  
Soft Tissue Sarcomas ◽  
Standard Of Care ◽  
Current Standard ◽  
Molecular Alterations

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal soft tissue sarcoma of the gastrointestinal tract. The management of locally advanced or metastatic unresectable GIST involves detecting KIT, PDGFR, or other molecular alterations targeted by imatinib and other tyrosine kinase inhibitors. The role of immunotherapy in soft tissue sarcomas is growing fast due to multiple clinical and pre-clinical studies with no current standard of care. The potential therapies include cytokine-based therapy, immune checkpoint inhibitors, anti-KIT monoclonal antibodies, bi-specific monoclonal antibodies, and cell-based therapies. Here we provide a comprehensive review of the immunotherapeutic strategies for GIST.

scholarly journals Human papillomavirus and endometrial cancer (review of literature and meta-analysis)

2021 ◽  
Vol 16 (4) ◽  
pp. 91-99
Author(s):  
M. К. Ibragimova ◽  
E. V. Kokorina ◽  
М. M. Tsyganov ◽  
О. N. Churuksaeva ◽  
N. V. Litviakov
Keyword(s):  
Cervical Cancer ◽  
Endometrial Cancer ◽  
Human Papillomavirus ◽  
Meta Analysis ◽  
Hpv Infection ◽  
Carcinogenic Risk ◽  
The Body ◽  
Malignant Neoplasms ◽  
Cancer Review

The important role of human papillomavirus (HPV) of high carcinogenic risk in the emergence and development of cervical cancer is undeniable. Approximately 90 % of cases of verified cervical cancer are HPV positive. The level of infection with this virus exceeds that of gonococci, chlamydia and yeast infections. Currently, one of the most discussed issues is the possibility of the association of HPV with the risk of developing malignant neoplasms when localized in organs that are anatomically close to the cervix. One of these localizations is the body of the uterus. It is known that endometrial cancer is based on its pronounced hormone dependence. Nevertheless, many factors are involved in the carcinogenesis of endometrial neoplasms, including genetic and epigenetic disorders, as well as risk factors, which include alimentary, hormonal, hereditary causes. At the same time, a controversial issue is the involvement of HPV in the development of this type of cancer. The data on the presence of HPV in endometrial cancer are extremely contradictory: the researchers claim both the complete absence of the effect of the virus on the development of this type of cancer, and the detection of HPV in 60–80 % of cases of tumors. In this regard, it becomes necessary to systematize the currently available research results on this issue and to conduct a meta-analysis of the association of HPV infection with the risk of endometrial cancer.

scholarly journals Imaging features of primary sites and metastatic patterns of angiosarcoma

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Basrull N. Bhaludin ◽  
Khin Thway ◽  
Margaret Adejolu ◽  
Alexandra Renn ◽  
Christian Kelly-Morland ◽  
...  
Keyword(s):  
Metastatic Disease ◽  
Wide Spectrum ◽  
Imaging Techniques ◽  
Soft Tissue Sarcomas ◽  
Final Diagnosis ◽  
The Body ◽  
Imaging Features ◽  
Imaging Findings ◽  
Wide Range ◽  
Metastatic Patterns

AbstractAngiosarcomas are rare, aggressive soft tissue sarcomas originating from endothelial cells of lymphatic or vascular origin and associated with a poor prognosis. The clinical and imaging features of angiosarcomas are heterogeneous with a wide spectrum of findings involving any site of the body, but these most commonly present as cutaneous disease in the head and neck of elderly men. MRI and CT are complementary imaging techniques in assessing the extent of disease, focality and involvement of adjacent anatomical structures at the primary site of disease. CT plays an important role in the evaluation of metastatic disease. Given the wide range of imaging findings, correlation with clinical findings, specific risk factors and patterns of metastatic disease can help narrow the differential diagnosis. The final diagnosis should be confirmed with histopathology and immunohistochemistry in combination with clinical and imaging findings in a multidisciplinary setting with specialist sarcoma expertise. The purpose of this review is to describe the clinical and imaging features of primary sites and metastatic patterns of angiosarcomas utilising CT and MRI.

scholarly journals Tumor growth - a modern view of pathogenesis and pharmacotherapy (lecture)

2021 ◽  
Author(s):  
Alexander I. Tyukavin ◽  
Sergei V. Suchkov
Keyword(s):  
Tumor Markers ◽  
Molecular Mechanisms ◽  
Malignant Tumors ◽  
Malignant Cell ◽  
The Body ◽  
Molecular Diagnostic ◽  
Malignant Neoplasms ◽  
Diagnostic Approaches ◽  
Oncological Centers

The lecture presents up-to-date information on the prevalence of cancer in the world and in the Russian Federation. The main risk factors and causes of malignant tumors are considered. Particular attention is paid to the mechanisms of transformation of normal cells into tumor cells, and the role of oncogenes and anti-oncogenes in the initiation of malignant growth is shown. Based on modern information about carcinogenesis, the pathogenetic significance of the molecular mechanisms of malignant cell growth at various stages (initiation, promotion, progression) of the tumor process is shown. The mechanisms of evasion of tumors from the influence of immune and other mechanisms that restrain their emergence and development in the body are described, and it is also shown how the spread (metastasis) of malignant cells occurs. The modern tumor markers are presented, on the basis of which the earlier detection of malignant diseases is performed. Particular attention is paid to molecular diagnostic approaches to assessing the risk of occurrence and early diagnosis of malignant neoplasms. Genomic, epigenetic and interactomic tumor markers, which are used in leading domestic and foreign oncological centers, are considered. The most promising approaches to the creation of effective anticancer drugs obtained on the basis of the achievements of molecular biology and bioinformatics are highlighted.

scholarly journals Adult Head and Neck Soft Tissue Sarcomas: Treatment and Outcome

Sarcoma ◽  
2008 ◽  
Vol 2008 ◽  
pp. 1-5 ◽  
Author(s):  
Rabindra P. Singh ◽  
Robert J. Grimer ◽  
Nabina Bhujel ◽  
Simon R. Carter ◽  
Roger M. Tillman ◽  
...  
Keyword(s):  
Overall Survival ◽  
Soft Tissue ◽  
Local Recurrence ◽  
Head And Neck ◽  
Metastatic Disease ◽  
Tumour Size ◽  
Soft Tissue Sarcomas ◽  
Histological Subtypes ◽  
Median Size ◽  
Adult Head

We have retrospectively analysed the experience of a musculoskeletal oncological unit in the management of adult head and neck soft tissue sarcomas from 1990 to 2005. Thirty-six patients were seen, of whom 24 were treated at this unit, the remainder only receiving advice. The median age of the patients was 46 years. Most of the sarcomas were deep and of high or intermediate grade with a median size of 5.5 cm. Eleven different histological subtypes were identified. Wide excision was possible only in 21% of the cases. 42% of the patients developed local recurrence and 42% developed metastatic disease usually in the lungs. Overall survival was 49% at 5 years. Tumour size was the most important prognostic factor. Adult head and neck soft tissue sarcomas have a high mortality rate with a high risk of local recurrence and metastatic disease. The rarity of the disease would suggest that centralisation of care could lead to increased expertise and better outcomes.

scholarly journals Synovial Sarcoma: A Clinical Review

2021 ◽  
Vol 28 (3) ◽  
pp. 1909-1920
Author(s):  
Aaron M. Gazendam ◽  
Snezana Popovic ◽  
Sohaib Munir ◽  
Naveen Parasu ◽  
David Wilson ◽  
...  
Keyword(s):  
Synovial Sarcoma ◽  
Soft Tissue Sarcomas ◽  
Chromosomal Translocation ◽  
Surgical Excision ◽  
Tumor Grade ◽  
Disease Recurrence ◽  
Definitive Treatment ◽  
Adjuvant Radiation ◽  
Subsequent Formation ◽  
Synovial Sarcomas

Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5–10% of all STS. Synovial sarcoma differs from other STS by the relatively young age at diagnosis and clinical presentation. Synovial sarcomas have unique genomic characteristics and are driven by a pathognomonic t(X;18) chromosomal translocation and subsequent formation of the SS18:SSX fusion oncogenes. Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. Increasing size, age and tumor grade have been demonstrated to be negative predictive factors for both local disease recurrence and metastasis. Wide surgical excision remains the standard of care for definitive treatment with adjuvant radiation utilized for larger and deeper lesions. There remains controversy surrounding the role of chemotherapy in the treatment of SS and there appears to be survival benefit in certain populations. As the understanding of the molecular and immunologic characteristics of SS evolve, several potential systematic therapies have been proposed.

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