Sarcomas of soft tissues and bone and gastrointestinal stromal tumour

2016 ◽  
pp. 844-866
Author(s):  
Alessandro Gronchi ◽  
Angelo P. Dei Tos ◽  
Paolo G. Casali
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Soft Tissues ◽  
Lung Metastases ◽  
Gastrointestinal Stromal Tumour ◽  
Histologic Diagnosis ◽  
Course Quality ◽  
First Line Therapy ◽  
Multidisciplinary Consultation ◽  
Localized Disease

Sarcomas are rare tumours that can arise from bone or a variety of soft tissues in a variety of body sites. Surgery is the treatment mainstay for localized disease. Margins of resection are contingent on accurate histologic diagnosis. Adjuvant chemotherapy is routinely employed in osteosarcoma and Ewing Sarcomas, while it is used only on an individualized basis in high-risk soft tissue sarcoma. Isolated lung metastases from either bone or soft tissue sarcoma are primarily treated by surgery, especially if the disease-free interval is long and the number limited. Otherwise chemotherapy is the first-line therapy and subsequent treatments are decided according to response and disease evolution. Treatment planning should include multidisciplinary consultation to determine optimal therapy, taking into consideration tumour histology, site, and extent of the disease, its natural history and sensitivity to available treatments, surgical challenges and, of course, quality of life.

Sarcomas of soft tissues and bone

2016 ◽  
pp. 844-866 ◽  
Author(s):  
Alessandro Gronchi ◽  
Angelo P. Dei Tos ◽  
Paolo G. Casali
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Soft Tissues ◽  
Lung Metastases ◽  
Histologic Diagnosis ◽  
Course Quality ◽  
First Line Therapy ◽  
Multidisciplinary Consultation ◽  
Localized Disease ◽  
Tumour Histology

Sarcomas are rare tumours that can arise from bone or a variety of soft tissues in a variety of body sites. Surgery is the treatment mainstay for localized disease. Margins of resection are contingent on accurate histologic diagnosis. Adjuvant chemotherapy is routinely employed in osteosarcoma and Ewing Sarcomas, while it is used only on an individualized basis in high-risk soft tissue sarcoma. Isolated lung metastases from either bone or soft tissue sarcoma are primarily treated by surgery, especially if the disease-free interval is long and the number limited. Otherwise chemotherapy is the first-line therapy and subsequent treatments are decided according to response and disease evolution. Treatment planning should include multidisciplinary consultation to determine optimal therapy, taking into consideration tumour histology, site, and extent of the disease, its natural history and sensitivity to available treatments, surgical challenges and, of course, quality of life.

Non-surgical Treatments for Lung Metastases in Patients with soft Tissue Sarcoma: Stereotactic Body Radiation Therapy (SBRT) and Radiofrequency Ablation (RFA)

2020 ◽  
Vol 16 ◽  
Author(s):  
Cecilia Tetta ◽  
Maria Carpenzano ◽  
Areej Tawfiq J Algargoush ◽  
Marwah Algargoosh ◽  
Francesco Londero ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Stereotactic Body Radiation Therapy ◽  
Lung Metastases ◽  
Disease Free Interval ◽  
Stereotactic Body Radiation ◽  
Free Interval ◽  
Disease Free

Background: Radio-frequency ablation (RFA) and Stereotactic Body Radiation Therapy (SBRT) are two emerging therapies for lung metastases. Introduction: We performed a literature review to evaluate outcomes and complications of these procedures in patients with lung metastases from soft tissue sarcoma (STS). Method: After selection, seven studies were included for each treatment encompassing a total of 424 patients: 218 in the SBRT group and 206 in the RFA group. Results: The mean age ranged from 47.9 to 64 years in the SBRT group and from 48 to 62.7 years in the RFA group. The most common histologic subtype was, in both groups, leiomyosarcoma. : In the SBRT group, median overall survival ranged from 25.2 to 69 months and median disease-free interval from 8.4 to 45 months. Two out of seven studies reported G3 and one G3 toxicity, respectively. In RFA patients, overall survival ranged from 15 to 50 months. The most frequent complication was pneumothorax. : Local control showed high percentage for both procedures. Conclusion: SBRT is recommended in patients unsuitable to surgery, in synchronous bilateral pulmonary metastases, in case of deep lesions and in patients receiving high-risk systemic therapies. RFA is indicated in case of a long disease-free interval, in oligometastatic disease, when only the lung is involved, in small size lesions far from large vessels. : Further large randomized studies are necessary to establish whether these treatments may also represent a reliable alternative to surgery.

scholarly journals Extraskeletal Mesenchymal Chondrosarcoma

2018 ◽  
Vol 142 (11) ◽  
pp. 1421-1424 ◽  
Author(s):  
Komal Arora ◽  
Nicole D. Riddle
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Soft Tissues ◽  
Hyaline Cartilage ◽  
Mesenchymal Chondrosarcoma ◽  
Small Cells ◽  
Diagnostic Challenge ◽  
Biphasic Pattern ◽  
Biopsy Specimens ◽  
Extraskeletal Mesenchymal Chondrosarcoma

Extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the lower extremities, meninges, and orbits. It usually presents during the second to third decades of life, and has a slight predominance in females. Histologically, it has a typical biphasic pattern comprising small cells and islands of hyaline cartilage. It can pose a diagnostic challenge in small biopsy specimens where 1 of the 2 components can be absent. The prognosis is extremely variable; survival varies depending on the location of the tumor.

scholarly journals Which Soft Tissue Sarcoma Patients with Lung Metastases Should not Undergo Pulmonary Resection?

Sarcoma ◽  
2002 ◽  
Vol 6 (2) ◽  
pp. 57-60 ◽  
Author(s):  
Albertus N. van Geel ◽  
Joost Rm van Der Sijp ◽  
Paul Im Schmitz
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Pulmonary Resection ◽  
Lung Metastases ◽  
Meta Analysis ◽  
Lung Surgery ◽  
Second Best

Using the second best method of meta-analysis it is significantly shown that patients with an interval of less than 7 months between diagnosis of soft tissue sarcoma and lung surgery for metastases do not benefit.

scholarly journals Update on Systemic Therapy for Advanced Soft-Tissue Sarcoma

2020 ◽  
Vol 27 (11) ◽  
pp. 25-33 ◽  
Author(s):  
A. Smrke ◽  
Y. Wang ◽  
C. Simmons
Keyword(s):  
Overall Survival ◽  
Clinical Trials ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Systemic Therapy ◽  
English Language ◽  
Standard Of Care ◽  
Current Evidence ◽  
First Line ◽  
First Line Therapy

Background: Soft-tissue sarcoma (sts) represents a rare group of mesenchymal neoplasms comprising more than 50 heterogeneous subtypes. Great efforts have been made to increase the understanding of the treatment of advanced sts (unresectable or metastatic disease). We set out to determine whether outcomes for patients with advanced sts have improved over time and to assess the current evidence for systemic therapy. Methods: In a scoping review, we evaluated the contemporary evidence for systemic treatment of advanced sts in adults (>18 years of age). Phase i, ii, and iii studies of systemic therapy for advanced sts published in the English language were included. After abstract and full-text review of seventy-seven studies, sixty-two trials met the inclusion criteria. Results: The number of clinical trials conducted and published in advanced sts has increased over the last 30 years. Although median overall survival has increased, attempts at improving first-line therapy through dose intensification, doublet chemotherapy, or alternative backbones have not been successful. The optimal therapy beyond anthracyclines remains a challenge, especially given the heterogeneity that grouping multiple sts subtypes within clinical trials creates. However, increasing numbers of agents are being studied, and several studies had shown isolated benefit in progression-free or overall survival. Summary: First-line systemic therapy with an anthracycline remains the standard of care for advanced sts. However, choice of subsequent therapy beyond anthracyclines remains challenging. Novel systemic therapies, use of molecular diagnostics to direct therapy, subtype-specific trials, and learnings from real-world retrospective data are all important for improving outcomes in patients with advanced sts.

Local Ablative Therapies to Metastatic Soft Tissue Sarcoma

2016 ◽  
pp. e566-e575 ◽  
Author(s):  
Alessandro Gronchi ◽  
B. Ashleigh Guadagnolo ◽  
Joseph Patrick Erinjeri
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Lung Metastases ◽  
Cord Compression ◽  
Histologic Subtype ◽  
Ablative Therapies ◽  
Patient Status ◽  
Isolated Lung ◽  
Metastatic Soft Tissue Sarcoma

The approach to metastatic soft tissue sarcoma is complex and depends upon several factors, such as the extent of the disease, the histologic subtype of the primary tumor, the disease-free interval, patient status and comorbidities, and previous treatments. The effect of systemic chemotherapy is suboptimal, therefore local ablative therapies are often considered when the disease is limited, especially if confined to a single site/organ. Historically, surgery has been considered the treatment of choice for isolated lung metastases. This approach also has been extended to metastases in the liver, although a formal demonstration of its benefit has never been provided. Radiation therapy instead has been mainly used to obtain pain control and to reduce the risk of bone fracture and cord compression. Advances in techniques, such as the development of more precise conformational modalities and the employment of particles, may change the role of this modality in the strategic approach to metastatic soft tissue sarcoma. Recently, the use of interventional radiology in this scenario has expanded. Ablative approaches, such as radiofrequency ablation and cryoablation, have shown durable eradication of tumors. Catheter-directed therapies, such as hepatic artery embolization, are potential techniques for treating the patient who has multiple unresectable liver metastases. Understanding the timing and role of these three different modalities in the multidisciplinary approach to metastatic soft tissue sarcoma is critical to provide better care and to personalize the approach to the single patient.

scholarly journals PML expression in soft tissue sarcoma: Prognostic and predictive value in alkylating agents/antracycline-based first line therapy

2012 ◽  
Vol 227 (4) ◽  
pp. 1657-1662 ◽  
Author(s):  
Bruno Vincenzi ◽  
Daniele Santini ◽  
Gaia Schiavon ◽  
Anna Maria Frezza ◽  
Marianna Silletta ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Predictive Value ◽  
Alkylating Agents ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy
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