scholarly journals NCCN Guidelines Insights: Soft Tissue Sarcoma, Version 1.2021

2020 ◽  
Vol 18 (12) ◽  
pp. 1604-1612
Author(s):  
Margaret von Mehren ◽  
John M. Kane ◽  
Marilyn M. Bui ◽  
Edwin Choy ◽  
Mary Connelly ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Body Wall ◽  
Panel Discussion ◽  
Soft Tissue Sarcomas ◽  
Nccn Guidelines ◽  
Stromal Tumors ◽  
Retroperitoneal Sarcomas ◽  
Head Neck

The NCCN Guidelines for Soft Tissue Sarcoma provide recommendations for the diagnosis, evaluation, treatment, and follow-up for patients with soft tissue sarcomas. These NCCN Guidelines Insights summarize the panel discussion behind recent important updates to the guidelines, including the development of a separate and distinct guideline for gastrointestinal stromal tumors (GISTs); reconception of the management of desmoid tumors; inclusion of further recommendations for the diagnosis and management of extremity/body wall, head/neck sarcomas, and retroperitoneal sarcomas; modification and addition of systemic therapy regimens for sarcoma subtypes; and revision of the principles of radiation therapy for soft tissue sarcomas.

scholarly journals Prognostic Factors in Extremity Soft Tissue Sarcoma Patients Treated with Preoperative Radiotherapy

2019 ◽  
Vol 4 (03) ◽  
Author(s):  
Berrin Inanc, MD ◽  
Kubilay Inanc, MD
Keyword(s):  
Prognostic Factors ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Preoperative Radiotherapy ◽  
Soft Tissue Sarcomas ◽  
Tumor Grade ◽  
Metastatic Recurrence ◽  
Extremity Soft Tissue Sarcoma ◽  
Metastatic Relapse

Purpose: The purpose of the study was to investigate the prognostic factors and survival after preoperative radiotherapy in Extremity Soft Tissue Sarcomas (ESTS). Materials and Methods: In this retrospective study, all patients treated for an extremity sarcoma with pre-operative radiotherapy followed by surgery. Results: The mean follow-up for all 24 ESTS patients was 15.5 (range: 10-39 months). At last follow-up, 9 patients (37%) were alive, 15 patients (62%) had died of distant disease progression. Among the patients died, there were 15 with metastatic relapse (13 lung and 2 cranial metastasis), 5 with both local and metastatic recurrence. The median OS was 16 month. The 2-years actuarial OS rate and 3-years OS rate were 39% and 26%, respectively. The median RFS was 14(12.5-15.4) month. The 2-years and 3-years RFS rate was 71%.The median MFS was 12 months. The 2-years and 3-years MFS rate were 33%, 17%, respectively. The effects of age, sex, histopathologic type, tumor size, tumor localization, tumor grade, tumor depth, radiation doses and recestion margin on OS, RFS, MFS were not observed. In univariate and multivariate model, it was observed that recurrence decreased OS time significantly (p<0.05). Conclusion: Recurrens and metastasis are strong and negative prognostic factor for survival in extremity soft tissue sarcoma patients.

scholarly journals Soft Tissue Sarcoma Follow-up Imaging: Strategies to Distinguish Post-treatment Changes from Recurrence

2020 ◽  
Vol 24 (06) ◽  
pp. 627-644
Author(s):  
Iris-M. Noebauer-Huhmann ◽  
Snehansh R. Chaudhary ◽  
Olympia Papakonstantinou ◽  
Joannis Panotopoulos ◽  
Marc-André Weber ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Surgical Techniques ◽  
Soft Tissue Sarcomas ◽  
Late Complications ◽  
Treatment Modalities ◽  
Recurrence Rates ◽  
Precise Knowledge ◽  
Inflammatory Pseudotumors

AbstractSoft tissue sarcomas encompass multiple entities with differing recurrence rates and follow-up intervals. The detection of recurrences and their differentiation from post-therapeutic changes is therefore complex, with a central role for the clinical radiologist. This article describes approved recommendations. Prerequisite is a precise knowledge of the current clinical management and surgical techniques. We review recurrence rates and treatment modalities. An adequate imaging technique is paramount, and comparison with previous imaging is highly recommended. We describe time-dependent therapy-related complications on magnetic resonance imaging compared with the spectrum of regular post-therapeutic changes. Early complications such as seromas, hematomas, and infections, late complications such as edema and fibrosis, and inflammatory pseudotumors are elucidated. The appearance of recurrences and radiation-associated sarcomas is contrasted with these changes. This systematic approach in follow-up imaging of soft tissue sarcoma patients will facilitate the differentiation of post-therapeutic changes from recurrences.

scholarly journals Lymph Node Metastasis after a Soft Tissue Sarcoma of the Leg: A Case Report and a Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
S. D. Nelen ◽  
F. J. Vogelaar ◽  
F. Gilissen ◽  
J. C. Van der Linden ◽  
K. Bosscha
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Lower Extremity ◽  
Distant Metastasis ◽  
Soft Tissue Sarcomas ◽  
Dermatofibrosarcoma Protuberans ◽  
Regional Lymph Nodes ◽  
Histological Types ◽  
Appropriate Treatment

Introduction. Soft tissue sarcomas (STSs) represent 1 percent of all adult malignancies and sarcomas only rarely spread to the regional lymph nodes.Case Presentation. We present a case of a woman with a dermatofibrosarcoma protuberans and a sarcoma not therwise specified of the lower extremity. The patient had no distant metastasis during follow-up, but did develop a regional lymph nodemetastasis (RLNM) in the groin. We reviewed the literature about RLNM in STSs.Discussion. Reviewing the literature we see that within specific histological types RLNM occurs as often as distant metastasis. Furthermore RLNM occurs in over 10% for specific histological types and in 24% of all patients with a soft tissue sarcoma of the lower extremity. Except for radical lymphadenectomy with a 5-year survival rate of 46% there is no appropriate treatment.Conclusion. The risk for a RLNM in certain histological types and anatomical locations might transcend the risk for a distant lung metastasis.

scholarly journals Retroperitoneal Sarcomas: An Update on the Diagnostic Pathology Approach

Diagnostics ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. 642
Author(s):  
Joon Hyuk Choi ◽  
Jae Y. Ro
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Soft Tissue Sarcomas ◽  
Accurate Diagnosis ◽  
Diagnostic Pathology ◽  
Heterogenous Group ◽  
Well Differentiated ◽  
Retroperitoneal Sarcomas ◽  
Histologic Types ◽  
Do So

Retroperitoneal sarcomas are a heterogenous group of rare tumors arising in the retroperitoneum. Retroperitoneal sarcomas comprise approximately 10% of all soft tissue sarcomas. Though any soft tissue sarcoma histologic types may arise in the retroperitoneal space, liposarcoma (especially well-differentiated and dedifferentiated types) and leiomyosarcoma do so most commonly. Retroperitoneal sarcomas are diagnostically challenging, owing to their diversity and morphological overlap with other tumors arising in the retroperitoneum. An accurate diagnosis is necessary for correct management and prognostication. Herein, we provide an update on the diagnostic approach to retroperitoneal sarcomas and review their key histologic findings and differential diagnoses.

Soft Tissue Sarcoma

2017 ◽  
Author(s):  
Marianne Berwick ◽  
Charles Wiggins
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Soft Tissue Sarcomas ◽  
Relative Survival ◽  
Major Complication ◽  
The United States ◽  
The Body ◽  
Molecular Characteristics ◽  
Relative Survival Rate ◽  
Head Neck

Soft tissue sarcoma (STS) is a rare tumor, occurring in approximately one to four of every 100,000 individuals worldwide. Soft tissue sarcomas can form anywhere in the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissues around joints. They are most common in the head, neck, arms, legs, trunk, and abdomen. Prognosis is generally poor, with a relative survival rate of approximately 65% at five years, with little difference by race. Approximately 11,930 cases and 4,870 deaths from STS occurred in the United States in 2015. The etiology of STS is still poorly understood, which makes prevention of this relatively rare cancer difficult. A major complication in studying STS is the histologic diversity —more than 100 subtypes. Newer investigations are evaluating molecular characteristics and prognostic factors but continue to be hampered by a lack of standardized histology.

scholarly journals Chemotherapy Combined With Recombinant Human Endostatin (Endostar) Significantly Improves the Progression-Free Survival of Stage IV Soft Tissue Sarcomas

2022 ◽  
Vol 11 ◽  
Author(s):  
Zhichao Liao ◽  
Chao Zhang ◽  
Tielong Yang ◽  
Haotian Liu ◽  
Songwei Yang ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Combined Therapy ◽  
Soft Tissue Sarcomas ◽  
Stage Iv ◽  
Significant Activity ◽  
Recombinant Human Endostatin ◽  
Chemotherapy Group ◽  
Significant Difference

PurposeOur previously study showed that recombinant human endostatin (Endostar) combined with chemotherapy had significant activity to increase the mPFS in patients with advanced sarcomas with tolerable side effects. However, the small cohort size and short follow-up time made it difficult to screen sensitive sarcoma subtypes and determine whether there is an overall survival benefit. With the largest sarcoma cohort to our knowledge, we try to confirm the efficacy and safety of chemotherapy combined with Endostar in stage IV sarcomas, with the specific purpose of finding out the sensitive sarcoma types for this combined treatment.MethodsAfter the exclusion of ineligible patients, 156 patients with stage IV bone and soft tissue sarcomas were included in this study according to the inclusion criteria.ResultsBy the end of follow-up, the ORR was 10.7% (9/84) vs 1.4% (1/72) (p=0.041), the DCR was 26.2% (22/84) vs 5.6% (4/72) (p=0.001) in the combined group and chemotherapy group, respectively. The mPFS of combined group was significantly longer than the chemotherapy group (10.42 vs 6.87 months, p=0.003). The mOS were 26.84 months and 23.56 months, without significant difference (p= 0.481). In osteogenic sarcoma, there was no statistically significant difference in the mPFS between the two groups (p=0.59), while in the soft tissue sarcoma, the mPFS in the combined group was significantly higher than that of the chemotherapy group (11.27 vs 8.05 months, p=0.004). Specifically, undifferentiated polymorphic sarcoma (UPS) was the possible sarcoma subtypes that benefited from the combined therapy. For the 38 UPS patients (28 patients in the combined group and 10 patients in the chemotherapy group), the mPFS in the combined group was up to 14.88 months, while it was only 7.1 months in the chemotherapy group, with a significant difference (p=0.006). The most common adverse events in the combined group were myelosuppression, gastrointestinal reactions and abnormal liver function, without significant difference in two groups.ConclusionChemotherapy plus Endostar could prolong mPFS and improve ORR and DCR in patients with stage IV soft tissue sarcoma, suggesting that the combined therapy could improve the patient prognosis in soft tissue sarcomas, especially the UPS patients.

scholarly journals The problem of local recurrence and metastasis in soft-tissue sarcoma

2003 ◽  
Vol 11 (1) ◽  
pp. 9-11
Author(s):  
Aleksandar Kiralj ◽  
Zlata Janjic ◽  
Mladen Jovanovic ◽  
Nada Vuckovic
Keyword(s):  
Prognostic Factor ◽  
Soft Tissue ◽  
Local Recurrence ◽  
Soft Tissue Sarcoma ◽  
Distant Metastasis ◽  
Soft Tissue Sarcomas ◽  
Radical Resection ◽  
Surgical Margins

BACKGROUND: The purpose of this study was to evaluate local recurrence of soft-tissue sarcomas as a prognostic factor reflecting adequate or inadequate excision. METHODS: We reviewed the cases of 53 patients who had soft-tissue sarcomas and were treated between 1991 and 2001. All patients were treated operatively, but 11 of them (20.75%), before being sent to us were operated elsewhere with inadequate surgical margins. The oncology status, including local recurrence and metastasis was determined at the follow-up evaluation. RESULTS: All of 11 patients treated with inadequate excision had palpably or histologically determined local recurrence. The most common histological diagnosis of local recurrence was dermatofibroma protuberans (7 patients, 63.63%). In patients who were treated with planned and adequate excision there were 4 (9.52%) recurrences. Five patients (45.45%) had metastases in the group of inadequate and only one patient (1.88%) in the group of adequate surgical margins. CONCLUSION: Our study demonstrated that excellent rates of survival and low rates of local recurrence and distant metastasis of soft-tissue sarcomas could be obtained with the use of carefully planned radical resection. The quality of operation is the most important factor.

scholarly journals Surgery for retroperitoneal soft tissue sarcomas: aggressive re-resection of recurrent disease is possible

2011 ◽  
Vol 93 (1) ◽  
pp. 39-43 ◽  
Author(s):  
R Lochan ◽  
JJ French ◽  
DM Manas
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Median Survival ◽  
Recurrent Disease ◽  
Soft Tissue Sarcomas ◽  
Disease Free Survival ◽  
Patient Demographics ◽  
Included Patient ◽  
Therapeutic Problem

INTRODUCTION Retroperitoneal soft tissue sarcomas represent a relatively rare and complex therapeutic problem where surgery forms the mainstay of treatment and is technically demanding. In this study, we review a single UK centre’s experience with the surgical management of retro-peritoneal soft tissue sarcoma. PATIENTS AND METHODS We present analysis of data on patients treated between 1997 and 2006, our first 75 patients. Data collected from the Access database, included patient demographics, staging modalities, peri-operative details, treatment, outcome, pathological diagnosis and subsequent complications. RESULTS A total of 75 patients (M:F, 44:31) underwent 115 resectional procedures as part of the management of retroperitoneal soft-tissue sarcoma. There were 12 major complications for the 115 procedures (morbidity of 8.69%). The 30-day operative mortality was zero and the 90-day mortality rate was 1.33% (1/75). Follow-up ranged from 16–131 months. The median disease-free survival was 69 months (range, 59-78 months). Recurrences developed in 46 patients; median time to overall recurrence was 13 months (range, 3-71 months). Of these 46, 22 developed localised recurrence, which was amenable to further resection. In the cohort of patients with recurrent disease, median survival in those who underwent surgery was 53 months (range, 30–76 months) and median survival in those who did not undergo surgery was 30 months (range, 18–41 months) and this difference was statistically significant (log rank, P = 0.01). CONCLUSIONS Extensive resectional surgery with minimal morbidity, devoid of mortality is feasible in the treatment of retroperitoneal sarcoma. Development of recurrent disease is a significant factor influencing survival; however, localised recurrences are amenable to surgery and this can lead to improved survival.

scholarly journals Primary Soft Tissue Sarcoma of the Heart: An Emerging Chapter in Cardio-Oncology

Biomedicines ◽  
2021 ◽  
Vol 9 (7) ◽  
pp. 774
Author(s):  
Pietro Scicchitano ◽  
Maria Chiara Sergi ◽  
Matteo Cameli ◽  
Marcelo H. Miglioranza ◽  
Marco Matteo Ciccone ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Soft Tissue Sarcomas ◽  
Cardiac Tumors ◽  
Malignant Cells ◽  
Future Perspectives ◽  
Histological Differentiation ◽  
Biological Features ◽  
Literature Overview ◽  
Primary Localization

Primary malignant cardiac tumors are rare, with a prevalence of about 0.01% among all cancer histotypes. At least 60% of them are primary soft tissue sarcomas of the heart (pSTS-h) that represent almost 1% of all STSs. The cardiac site of origin is the best way to classify pSTS-h as it is directly linked to the surgical approach for cancer removal. Indeed, histological differentiation should integrate the classification to provide insights into prognosis and survival expectancy of the patients. The prognosis of pSTS-h is severe and mostly influenced by the primary localization of the tumor, the difficulty in achieving complete surgical and pharmacological eradication, and the aggressive biological features of malignant cells. This review aims to provide a detailed literature overview of the most relevant issues on primary soft tissue sarcoma of the heart and highlight potential diagnostic and therapeutic future perspectives.

scholarly journals Outcome and Toxicity of an Ifosfamide-Based Soft Tissue Sarcoma Treatment Protocol in Children. The Importance of Local Therapy

Sarcoma ◽  
1998 ◽  
Vol 2 (3-4) ◽  
pp. 171-177
Author(s):  
S. Murray Yule ◽  
Roderick Skinner ◽  
Martin W. English ◽  
Mike Cole ◽  
Andrew D. J. Pearson ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Primary Tumour ◽  
Treatment Protocol ◽  
Disease Free Survival ◽  
Local Therapy ◽  
Complete Response ◽  
One Year ◽  
Sarcoma Treatment

Background.Although the survival of children with soft tissue sarcoma (STS) has improved considerably, the outcome of patients with metastatic disease, and those with primary tumours of the extremities or parameningeal sites remains disappointing. We describe the clinical outcome of an ifosfamide-based regimen with local therapy directed only to children who failed to achieve a complete response to initial chemotherapy.Patients and Methods.Twenty-one children with STS (16 rhabdomyosarcoma) who presented with unresectable tumours were treated with five courses of ifosfamide (9 g/m2) and etoposide (600 mg/m2). Patients who did not achieve a complete response then received local therapy. Chemotherapy with ifosfamide combined with etoposide, vincristine (1.5 mg/m2and doxorubicin (60 mg/m2) or vincristine and actinomycin D (1.5 mg/m2) was continued for one year.Results and Discussion.Objective responses to five courses of ifosfamide and etoposide were seen in all patients. Disease free survival (DFS) at a median follow up of 59 months was 57% (95% CI 29–75%). The DFS of children who received local therapy was 89% compared with 33% in those who received chemotherapy alone (p=0.027). Locoregional recurrences did not occur in children who received radiotherapy to the site of the primary tumour. Ifosfamide-based chemotherapy does not reduce the incidence of loco-regional recurrence in children who do not receive local therapy.

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