Surgical Management and Minimally Invasive Approaches for the Treatment of Metastatic Sarcoma

2013 ◽  
pp. 457-464
Author(s):  
Peter Hohenberger ◽  
Bernd Kasper ◽  
Kamran Ahrar
Keyword(s):  
Soft Tissue ◽  
Minimally Invasive ◽  
Early Stage ◽  
Tumor Biology ◽  
Local Treatment ◽  
Soft Tissue Sarcomas ◽  
Metastatic Tumor ◽  
Soft Tissue Tumors ◽  
Tumor Spread ◽  
Systemic Treatments

Soft tissue sarcomas describe a very heterogeneous group of soft tissue tumors mainly arising in the lower extremities. If diagnosed at an early stage and a complete resection of the primary tumor is achieved, the patients' prognosis is excellent. However, metastatic tumor spread is common with only limited treatment possibilities. Despite an improved insight into tumor biology of sarcomas, no notable improvement has been gained in the last 20 years regarding prognosis of patients. Metastatic lung disease has long been the preserve of systemic treatments, local treatments being considered in a purely palliative intention. Several studies have objectified benefit to the local treatment of metastases, especially in an oligometastatic state. The development of techniques for stereotactic radiotherapy on the one hand and the refusal or contraindication for surgery on the other hand inaugurated studies in this direction. Besides surgery and radiotherapy, other local modalities have been investigated in the last few years such as thermal therapy (radiofrequency and laser ablation) or combined modalities (isolated limb perfusion and deep-wave hyperthermia plus chemotherapy) to help patients with metastatic soft tissue sarcoma. Minimally invasive, image-guided therapies such as thermal ablation should be considered particularly in patients who are not suitable surgical candidates or may have exhausted all other viable surgical options. Some of these techniques will be reviewed in this article, and their value for the patients will be evaluated in the light of indication from tumor biology and technical feasibility. These highly selected and specific procedures should only be performed after decision making in an interdisciplinary sarcoma-board.

scholarly journals Ultrasound Ablation: New Possibilities for Treatment of Bone and Soft-Tissue Tumors

2010 ◽  
Vol 17 (2) ◽  
pp. 41-49
Author(s):  
G I Nazarenko ◽  
V Sh Chen ◽  
A N Khitrova
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Tumor ◽  
Secondary Infection ◽  
Local Treatment ◽  
Soft Tissue Tumors ◽  
Treatment Technique ◽  
Tumor Type ◽  
Coagulative Necrosis ◽  
Ultrasound Ablation ◽  
Physical Principles

Focused high intensity ultrasound (HIFU) with sighting influence on tumor enabled to cause its complete coagulative necrosis without damage to intact environmental tissues. The possibilities of ultrasound ablation as the method of local treatment of bone and soft-tissue tumor were considered. Physical principles of method, criteria for patient selection, treatment technique as well as clinical outcomes and their evaluation were presented. Advantages of method are non-invasiveness, less traumatization, less technical complexity and dependence on surgeon skill compared with other sparing operations and methods of ablation. When necessary HIFU may be repeated several times. Effect of HIFU is independent on tumor type. Possible complications (secondary infection in coagulative necrosis zone, peripheral nerves damage, pathological fractures) and measures of their prevention are presented.

scholarly journals Improving Immunotherapy Efficacy in Soft-Tissue Sarcomas: A Biomarker Driven and Histotype Tailored Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Matthieu Roulleaux Dugage ◽  
Elise F. Nassif ◽  
Antoine Italiano ◽  
Rastislav Bahleda
Keyword(s):  
Soft Tissue ◽  
Immune Checkpoint ◽  
Response Rate ◽  
Checkpoint Inhibitors ◽  
Early Stage ◽  
Soft Tissue Sarcomas ◽  
Oncolytic Viruses ◽  
Cellular Therapies ◽  
M1 Macrophage ◽  
Synovial Sarcomas

Anti-PD-(L)1 therapies yield a disappointing response rate of 15% across soft-tissue sarcomas, even if some subtypes benefit more than others. The proportions of TAMs and TILs in their tumor microenvironment are variable, and this heterogeneity correlates to histotype. Tumors with a richer CD8+ T cell, M1 macrophage, and CD20+ cells infiltrate have a better prognosis than those infiltrated by M0/M2 macrophages and a high immune checkpoint protein expression. PD-L1 and CD8+ infiltrate seem correlated to response to immune checkpoint inhibitors (ICI), but tertiary lymphoid structures have the best predictive value and have been validated prospectively. Trials for combination therapies are ongoing and focus on the association of ICI with chemotherapy, achieving encouraging results especially with pembrolizumab and doxorubicin at an early stage, or ICI with antiangiogenics. A synergy with oncolytic viruses is seen and intratumoral talimogene laherpavec yields an impressive 35% ORR when associated to pembrolizumab. Adoptive cellular therapies are also of great interest in tumors with a high expression of cancer-testis antigens (CTA), such as synovial sarcomas or myxoid round cell liposarcomas with an ORR ranging from 20 to 50%. It seems crucial to adapt the design of clinical trials to histology. Leiomyosarcomas are characterized by complex genomics but are poorly infiltrated by immune cells and do not benefit from ICI. They should be tested with PIK3CA/AKT inhibition, IDO blockade, or treatments aiming at increasing antigenicity (radiotherapy, PARP inhibitors). DDLPS are more infiltrated and have higher PD-L1 expression, but responses to ICI remain variable across clinical studies. Combinations with MDM2 antagonists or CDK4/6 inhibitors may improve responses for DDLPS. UPS harbor the highest copy number alterations (CNA) and mutation rates, with a rich immune infiltrate containing TLS. They have a promising 15-40% ORR to ICI. Trials for ICB should focus on immune-high UPS. Association of ICI with FGFR inhibitors warrants further exploration in the immune-low group of UPS. Finally translocation-related sarcomas are heterogeneous, and although synovial sarcomas a poorly infiltrated and have a poor response rate to ICI, ASPS largely benefit from ICB monotherapy or its association with antiangiogenics agents. Targeting specific neoantigens through vaccine or adoptive cellular therapies is probably the most promising approach in synovial sarcomas.

Diagnosis and Management of Genitourinary Rhabdomyosarcoma

2019 ◽  
Author(s):  
Ahmed Abdelhalim ◽  
Zhan Tao (Peter) Wang ◽  
Ali Nael ◽  
Antoine E Khoury
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Risk Stratification ◽  
Collaborative Study ◽  
Tumor Biology ◽  
Study Groups ◽  
Soft Tissue Tumors ◽  
Tumor Surveillance ◽  
Future Developments ◽  
International Collaborative Study

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Genitourinary RMS accounts for 15 to 25% of all RMSs and is a heterogeneous group of soft tissue tumors that vary in presentation, distribution, and prognosis. This article reviews the pathophysiology and tumor biology of RMS. It will also describe the initial approach to its diagnosis and current tumor surveillance protocols. Furthermore, this article presents the evidence behind a number of different staging and risk stratification systems currently used to guide treatment. Lastly, this article reviews future developments of investigational studies and risk stratification under investigation by a number of large international collaborative study groups. This review contains 17 figures, 7 tables, and 68 references. Keywords:  Rhabdomyosarcoma, genitourinary, staging, diagnosis, paratesticular, bladder, prostate, RMS

A randomized trial for the treatment of high-grade soft-tissue sarcomas of the extremities: preliminary observations.

1986 ◽  
Vol 4 (4) ◽  
pp. 552-558 ◽  
Author(s):  
F Gherlinzoni ◽  
G Bacci ◽  
P Picci ◽  
R Capanna ◽  
P Calderoni ◽  
...  
Keyword(s):  
Adjuvant Chemotherapy ◽  
Soft Tissue ◽  
Local Treatment ◽  
Soft Tissue Sarcomas ◽  
Rank Test ◽  
High Grade ◽  
Chemotherapy Group ◽  
The Difference ◽  
To Receive ◽  
Observation Period

A new trial for evaluating the effectiveness of adjuvant chemotherapy in high-grade soft-tissue sarcomas of the extremities in adult patients is presented. All patients after local treatment were randomized into two arms, one without further therapy and the other to receive adjuvant chemotherapy (Adriamycin [Farmitalia-Carlo Erba, Milan, Italy], 450 mg/m2). The preliminary results of the study are reported at a median observation period of 27.6 months. Of the 59 patients who entered the study, 79.1% in the chemotherapy group are without sign of disease, whereas the corresponding figure in the nonadjuvant chemotherapy group is 54.3%. The difference between the two groups is statistically significant (P less than .005, log rank test). These preliminary observations encourage continuation of the study.

scholarly journals Progress in the Systemic Treatment of Advanced Soft-Tissue Sarcomas

1998 ◽  
Vol 5 (1) ◽  
pp. 1-5 ◽  
Author(s):  
Scott H. Okuno ◽  
John H. Edmonson
Keyword(s):  
Clinical Trials ◽  
Soft Tissue ◽  
Systemic Treatment ◽  
Soft Tissue Sarcomas ◽  
Multimodality Treatment ◽  
Clinical Situation ◽  
Controlled Clinical Trials ◽  
Systemic Treatments ◽  
Review Current ◽  
Systemic Therapies

Background: Despite the plethora of chemotherapeutic remedies for advanced soft-tissue sarcomas, little evidence has developed to indicate that these efforts have been curative. No controlled comparison has yet proven that patients receiving multidrug regimens survive longer than those receiving doxorubicin alone. Methods: The authors review current systemic treatments and then discuss some investigational efforts now in progress. Also, they seek to demonstrate how the therapies currently available can be integrated with surgery and radiation therapy to accomplish more than might be anticipated from chemotherapy alone. Results: While working to develop better systemic therapies for advanced soft-tissue sarcomas, the integrated use of our best chemotherapy regimens in combination with selected surgical and radiotherapy efforts may provide patients with the best available therapy. Some recent observations involving the use of molgramostim plus chemotherapy have been intriguing. Conclusions: Progress in the systemic treatment of advanced soft-tissue sarcomas may be gradual, but it is real. Our daily challenge is to be certain that we offer each patient the best available multimodality treatment applicable to his or her clinical situation. Molgramostim should be made available for further study with chemotherapy in controlled clinical trials.

IMMUNOSUPPRESSIVE POTENTIAL OF PERIPHERAL REGULATORY T-LYMPHOCYTES IN THE PROCESS OF TUMOR PROGRESSION IN PATIENTS WITH METASTATIC SOFT TISSUE SARCOMAS

2018 ◽  
Vol 64 (3) ◽  
pp. 400-407
Author(s):  
Nino Pipia ◽  
Irina Baldueva ◽  
Anna Danilova ◽  
Natalya Avdonkina ◽  
Aleksey Novik ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Tumor Microenvironment ◽  
Peripheral Blood ◽  
Soft Tissue Sarcomas ◽  
High Strength ◽  
Metastatic Tumor ◽  
Treg Cells ◽  
National Medical ◽  
Regulatory T Lymphocytes ◽  
Quantitative Content

In tumor microenvironment the self-maintenance condition of T-regulatory lymphocytes (Treg) are created by tumor cells due to the production of vascular endothelial growth factor VEGF and chemokine CCL2. In the present work there was evaluated the quantitative content of these proteins in cultured cell supernatants of metastatic soft tissue sarcomas (STS) as well as characterized the immunophenotype of peripheral blood Treg by flow cytometry. The study included 35 patients who received treatment at the N.N. Petrov National Medical Research Center of Oncology. For the study- samples of metastatic tumor were taken to obtain sarcoma cell culture and samples of peripheral blood of patients in the absence of tumor growth (stable disease-SD) or disease progression (PD). The statistically significant differences were found in the quantitative content of CCR10+Treg (9.1 % and 4.5 %, respectively, p=0.001), CCR4+Treg (10 % and 3.3 %, respectively, p=0.001), neuropilin-1 (Nrp1+) Treg (6 % and 4.5 %, respectively, p=0.021) in patients with PD and SD. A direct correlation of high strength was found between production of VEGF, CCL2 by metastatic STS cells and expression of Nrp1 (r=0.93, p=0.001), VEGFR-2(r=0.88, p=0.007), CCR4(r=0.81, p=0.024) by Treg cells. Statistically significant differences in the CCR10+Treg (9.5 % and 2.93 %, respectively, p=0.012) and CCR4+Treg (68, 3 % and 3.95 %, respectively, p=0.007) were detected in the group of patients with liposarcoma and synovial sarcoma. Thus in patients with metastatic STS - there was directional chemotaxis of Treg into tumor microenvironment providing the creation of tumor-induced tolerance, which could be associated with DP. The revealed regularities could be used to plan adjuvant and palliative treatment of STS patients.

Epithelioid Hyalinizing Sarcoma With MGA-NUTM1 Fusion

2020 ◽  
Vol 154 (6) ◽  
pp. 859-866
Author(s):  
Caroline I M Underwood ◽  
Diana M Cardona ◽  
Rex C Bentley ◽  
Guomiao Shen ◽  
Xiaojun Feng ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Immunohistochemical Study ◽  
Soft Tissue Sarcomas ◽  
Soft Tissue Tumors ◽  
Molecular Testing ◽  
Molecular Alterations ◽  
Poorly Differentiated ◽  
Next Generation Sequencing Ngs ◽  
Generation Sequencing

Abstract Objectives Soft tissue sarcomas are a group of tumors derived from the mesenchymal origin. Historically, they have been classified according to morphologic and immunohistochemical characteristics. The advent of multiplexed next-generation sequencing (NGS), specifically RNA sequencing, has modified the classification of such tumors and others by determining categorization based on molecular alterations. The NUTM1 rearrangement, previously thought to be present only in carcinomas, has recently been reported in poorly differentiated high-grade sarcomas of the soft tissue. We present the first reported case of an epithelioid hyalinizing sarcoma harboring the MGA-NUTM1 fusion in an acral site. Methods Histopathologic, immunohistochemical, and molecular testing were performed on resection tissue. Results Histologically, the tumor showed an epithelioid morphology with prominent background hyalinization. Immunohistochemically, the tumor expressed CD99 and nuclear NUT-1. By NGS the tumor harbors MGA-NUTM1 fusion. Conclusions Our findings support more extensive use of NGS for accurate sarcoma classification and identification of potential therapeutic targets. Furthermore, they corroborate the fact that NUTM1-rearranged soft tissue tumors represent a spectrum of heterogeneous morphologic entities. This case also highlights the utility of NUT-1 immunohistochemical study as a possible screening tool for NUTM1-fused sarcomas.

Aberrant Immunohistochemical Expression in Nonrhabdomyosarcoma Soft Tissue Sarcomas of Infancy: Retrospective Review of Clinical Material

2002 ◽  
Vol 5 (6) ◽  
pp. 579-586 ◽  
Author(s):  
Neil James Sebire ◽  
Alan Drummond Ramsay ◽  
Gillian Levitt ◽  
Marian Malone ◽  
Rupert Anthony Risdon
Keyword(s):  
Soft Tissue ◽  
Smooth Muscle Actin ◽  
Diagnostic Category ◽  
Young Patients ◽  
Soft Tissue Sarcomas ◽  
Mitotic Rate ◽  
Soft Tissue Tumors ◽  
Undifferentiated Sarcoma ◽  
Infantile Fibrosarcoma ◽  
Infantile Hemangiopericytoma

Malignant soft tissue tumors other than rhabdomyosarcoma (RMS) are uncommon in infancy, representing approximately 5% of pediatric sarcomas. The pathological categorization of non-RMS soft tissue malignancies from these young patients is complicated by variation in both morphologic and immunohistochemical features. A search covering an 11-year period identified 19 patients presenting at birth or in infancy with a clinical or referral diagnosis of soft tissue sarcoma. After histologic and immunohistochemical review, nine of these tumors were classified as primitive neuroectodermal tumor (PNET), three as infantile hemangiopericytoma (HPC), two as infantile fibrosarcoma (FS), and five as undifferentiated sarcoma. Those identified as undifferentiated sarcomas showed an atypical spindle and ovoid cell morphology, with cellular pleomorphism and high mitotic rate, but lacking the fascicular growth pattern of classic infantile fibrosarcoma. Immunohistochemical staining in this group showed variable weak positivity for a range of markers (desmin, smooth muscle actin, Myo-D1, PGP, NSE, S100, CO56, cytokeratin, and CD99), and did not fit readily into any distinct diagnostic category. In this series, tumors classified as soft tissue PNETs had a poor prognosis despite aggressive treatment. However, once RMS, PNET, and other rare specific lesions are excluded, the remaining undifferentiated sarcomas, despite their unusual morphology and immunohistochemistry, appear to behave in a similar favorable manner to infantile fibrosarcoma.

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