Soft Tissue Sarcoma

2020 ◽  
Author(s):  
Aimee M. Crago ◽  
Samuel Singer

Soft tissue sarcoma (STS) refers to a rare group of cancers that develop from mesenchymal cells and their progenitors. Histologic subtype, in conjunction with tumor location and size, largely defines the biologic behavior of a given lesion and the associated clinical prognosis in these cancers. The diverse characteristics of these tumors means that their treatment is similarly complex. The etiology, tumor staging and prognosis, evaluation, and treatment of STS are discussed in this review, with an aim to present an algorithm for patient evaluation and treatment while highlighting common indications for diverging from this strategy as dictated by disease subtype and location. Figures show the histologic distribution of primary STS diagnosed in the extremity and retroperitoneum and intra-abdominal compartments; disease-specific survival for primary extremity and retroperitoneal and intra-abdominal tumors stratified by histologic subtype; local recurrence in primary extremity STS stratified by histologic subtype; disease-specific survival according to American Joint Committee on Cancer (AJCC) TNGM stage; a postoperative nomogram for prediction of sarcoma-specific death at 12 years postresection for patients with STS; representative cross-sectional images of an atypical lipomatous tumor, a myxofibrosarcoma, and a desmoid tumor; a treatment algorithm for STS of the extremity; a magnetic resonance image and intraoperative photographs showing a mixoid liposarcoma of the posterior thigh; and computed tomography showing a retroperitoneal dedifferentiated liposarcoma and a photograph of the surgical bed following resection.  This review contains 10 figures, 12 tables, and 49 references. Keywords:  Sarcoma, soft tissue, cancer, myxofibrosarcoma, leiomyosarcoma, liposarcoma, gastrointestinal stromal tumor, rhabdomyosarcoma,

2021 ◽  
pp. jnumed.121.262502
Author(s):  
Joseph Crompton ◽  
Wesley R. Armstrong ◽  
Mark A. Eckardt ◽  
Ameen Seyedroudbari ◽  
William D. Tap ◽  
...  

1996 ◽  
Vol 14 (3) ◽  
pp. 859-868 ◽  
Author(s):  
P W Pisters ◽  
L B Harrison ◽  
D H Leung ◽  
J M Woodruff ◽  
E S Casper ◽  
...  

PURPOSE This trial was performed to evaluate the impact of adjuvant brachytherapy on local and systemic recurrence rates in patients with soft tissue sarcoma. PATIENTS AND METHODS In a single-institution prospective randomized trial, 164 patients were randomized intraoperatively to receive either adjuvant brachytherapy (BRT) or no further therapy (no BRT) after complete resection of soft tissue sarcomas of the extremity or superficial trunk. The adjuvant radiation was administered by iridium-192 implant, which delivered 42 to 45 Gy over 4 to 6 days. The two study groups had comparable distributions of patient and tumor factors, including age, sex, tumor site, tumor size, and histologic type and grade. RESULTS With a median follow-up time of 76 months, the 5-year actuarial local control rates were 82% and 69% in the BRT and no BRT groups (P = .04), respectively. Patients with high-grade lesions had local control rates of 89% (BRT) and 66% (no BRT) (P = .0025). BRT had no impact on local control in patients with low-grade lesions (P = .49). The 5-year freedom-from-distant-recurrence rates were 83% and 76% in the BRT and no BRT groups (P = .60), respectively. Analysis by histologic grade did not demonstrate an impact of BRT on the development of distant metastasis, despite the improvement in local control noted in patients with high-grade lesions. The 5-year disease-specific survival rates for the BRT and no BRT groups were 84% and 81% (P = .65), respectively, with no impact of BRT regardless of tumor grade. CONCLUSION Adjuvant brachytherapy improves local control after complete resection of soft tissue sarcomas. This improvement in local control is limited to patients with high-grade histopathology. The reduction in local recurrence in patients with high-grade lesions is not associated with a significant reduction in distant metastasis or improvement in disease-specific survival.


2003 ◽  
Vol 21 (14) ◽  
pp. 2719-2725 ◽  
Author(s):  
Jürgen Weitz ◽  
Christina R. Antonescu ◽  
Murray F. Brennan

Purpose: The objective of this study was to define whether survival of patients with extremity soft tissue sarcoma (STS), stratified for known risk factors, has improved over the last 20 years. Patients and Methods: From January 1982 to December 2001, 1,706 patients with primary and recurrent STS of the extremities were treated at our institution and were prospectively followed. From this cohort, we selected 1,261 patients who underwent complete macroscopic resection and had one of the following histopathologies: fibrosarcoma, liposarcoma, leiomyosarcoma, malignant fibrous histiocytoma, or synovial sarcoma. Median follow-up was 55 months. Patient, tumor, and treatment factors were analyzed as prognostic factors. Results: The 5-year disease-specific actuarial survival was 79% (78% for patients treated from 1982 to 1986, 79% for patients treated from 1986 to 1991, 79% for patients treated from 1992 to 1996, and 85% for patients treated from 1997 to 2001; P = not significant). For high-risk patients (high-grade, > 10 cm, deep tumors; n = 247), 5-year disease-specific survival was 51% (50% for patients treated from 1982 to 1986, 45% for patients treated from 1986 to 1991, 52% for patients treated from 1992 to 1996, and 61% for patients treated from 1997 to 2001; P = not significant). Tumor depth, size, grade, microscopic margin status, patient age, presentation status (primary tumor versus local recurrence), location (proximal versus distal), and certain histopathologic subtypes were significant prognostic factors for disease-specific survival on multivariate analysis; however, time period of treatment was not. Conclusion: Prognosis of patients with extremity STS, stratified for known risk factors, has not improved over the last 20 years, indicating that current therapy has reached the limits of efficacy.


2012 ◽  
Vol 106 (2) ◽  
pp. 155-161 ◽  
Author(s):  
Yi-Sheng Chou ◽  
Chun-Yu Liu ◽  
Wei-Ming Chen ◽  
Tain-Hsiung Chen ◽  
Paul Chih-Hsueh Chen ◽  
...  

1997 ◽  
Vol 15 (2) ◽  
pp. 646-652 ◽  
Author(s):  
J J Lewis ◽  
D Leung ◽  
M Heslin ◽  
J M Woodruff ◽  
M F Brennan

PURPOSE The aim of this study was to analyze local recurrence in a large cohort of prospectively followed patients with primary extremity soft tissue sarcoma. In particular, we analyzed the correlation of local recurrence with subsequent metastasis and disease-specific survival. PATIENTS AND METHODS Patients who underwent treatment for primary extremity soft tissue sarcoma from July 1982 through July 1995 at Memorial Sloan-Kettering Cancer Center were the subject of this study. Local recurrence, distant metastasis, and disease-specific survival were used as end points of the study. The influence of local recurrence on subsequent distant metastasis and disease-specific survival were examined using the Cox proportional hazards model. RESULTS We treated 911 patients, of whom 297 (33%) developed recurrent disease. Local recurrence occurred in 116 patients (13%), metastasis in 167 (18%), and synchronous local recurrence and metastasis in 13 (2%). Of 116 patients who developed local recurrence, 38 subsequently developed metastasis and 34 died of disease. Metastasis after local recurrence was predicted in patients with initial high-grade (P = .005; risk = 3.5) or deep (P = .02; risk = 2.9) tumors. Tumor mortality after local recurrence was predicted in patients with initial high-grade (P = .007; risk = 3.7) or large (> 5 cm; P = .01; risk = 3.2) primary tumors. DISCUSSION These findings suggest that there is a strong association of local recurrence with the development of subsequent metastasis and tumor mortality, and that local recurrence is a poor prognostic factor. It would seem prudent to consider patients who develop local recurrence and have high-grade tumors as being at high risk for systemic disease and therefore eligible for investigational adjuvant systemic therapy.


2020 ◽  
Vol 30 (8) ◽  
pp. 1118-1123
Author(s):  
Sarah Johnson ◽  
Malte Renz ◽  
Lindsay Wheeler ◽  
Elisabeth Diver ◽  
Oliver Dorigo ◽  
...  

ObjectiveVulvar cancers account for 5% of all gynecologic malignancies; only 1%–3% of those vulvar cancers are primary vulvar sarcomas. Given the rarity of vulvar sarcomas, outcome data specific to histopathologic subtypes are sparse. The aim of this study was to identify clinical and pathologic factors of primary vulvar sarcomas that are associated with survival and may inform treatment decisions.MethodsThe Surveillance, Epidemiology, and End Results (SEER) database was searched for women diagnosed with vulvar sarcoma between 1973 and 2018. We identified 315 patients and reviewed their demographic, clinicopathologic, surgical, and survival information. Statistical analyses included χ2 and t-tests, Kaplan–Meier survival, and Cox regression analyses.ResultsThe most common histopathologies of vulvar sarcomas were dermatofibrosarcomas (85/315, 27%) and leiomyosarcomas (72/315, 22.9%). Rhabdomyosarcomas (18/315, 5.7%), liposarcomas (16/315, 5.1%), and malignant fibrous histiocytomas (16/315, 5.1%) were less frequent. The majority of patients underwent surgery (292/315, 92.7%), which included lymph node dissections in 21.6% (63/292). Survival and lymph node involvement varied significantly with histologic subtype. The 5-year disease-specific survival for dermatofibrosarcomas, liposarcomas, and fibrosarcomas was 100% and only 60.3% and 62.5% for malignant fibrous histiocytomas and rhabdomyosarcomas, respectively. None of the patients with (dermato)fibrosarcomas, liposarcomas, or leiomyosarcomas had positive lymph nodes, in contrast to rhabdomyosarcomas and malignant fibrous histiocytomas with 77.8% and 40% positive lymph nodes, respectively. The 5-year disease-specific survival for women with positive lymph nodes was 0%.ConclusionsVulvar sarcomas are heterogeneous with survival highly dependent on the histopathologic subtype. While surgical excision is the mainstay of treatment for all vulvar sarcomas, staging lymphadenectomy should be deferred for (dermato)fibrosarcomas, liposarcomas, and leiomyosarcomas as there were no cases of lymph nodes metastases.


2019 ◽  
Vol 157 (06) ◽  
pp. 644-653 ◽  
Author(s):  
Sebastian Scheidt ◽  
Cornelius Jacobs ◽  
Sebastian Koob ◽  
Kristian Welle ◽  
Sebastian Walter ◽  
...  

AbstractSoft tissue sarcomas are a heterogeneous group of neoplasias that due to their often clinically silent appearance often remain undetected or experience delayed treatment. Especially soft tissue swelling is often misinterpreted by patients and doctors and trivialized or verified with an incorrect biopsy technique. The hereby evoked complications for the patients are serious and may be reduced by simply following the available guidelines. The treatment of soft tissue sarcomas requires a close interdisciplinary coordination between specialists in tumor orthopedics, oncology, radiology, pathology and radiotherapy. On the basis of a selective literature review, the following article points out the current evidence on the treatment and illustrates a treatment algorithm.


2005 ◽  
Vol 23 (6) ◽  
pp. 1218-1227 ◽  
Author(s):  
Christiane Voit ◽  
Martina Kron ◽  
Juergen Rademaker ◽  
Markus Schwürzer-Voit ◽  
Wolfram Sterry ◽  
...  

Purpose To assess the prognostic value of serial reverse transcriptase polymerase chain reaction (RT-PCR) -based measurements of tyrosinase mRNA in peripheral blood of stage II and III melanoma patients. Patients and Methods During routine follow-up of American Joint Committee on Cancer stage II and III melanoma patients, serial testing for tyrosinase transcripts in peripheral blood was performed by RT-PCR. The PCR results were compared with the clinical data collected during the follow-up. Results Over a period of 3 years, 111 patients (78 stage II and 33 stage III patients) were enrolled, and tyrosinase determinations were carried out. The 6-year disease-specific survival probability was 97% for patients always showing negative RT-PCR results and 67% for patients who tested positive at least once. In a Cox proportional hazards model, the prognostic value of sex, age, site of primary tumor, histologic subtype, stage, Breslow's tumor thickness, Clark level, and the time-dependent variable PCR result was assessed. Patients with a positive RT-PCR test had a distinctly higher risk of dying from melanoma, with a hazard ratio of 12.6 (95% CI, 3.4 to 46.3; P < .001). Conclusion Our study shows a strong association between PCR and disease-specific survival time. Detection of tyrosinase mRNA in peripheral blood may be of similar importance for the clinical course of melanoma as the detection of micrometastatic disease in the sentinel lymph node. Whether a combination of these two factors leads to a better definition of the prognosis of melanoma patients is under investigation in current studies.


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