scholarly journals Extraskeletal Osteosarcoma: Analysis of Outcome of a Rare Neoplasm

Sarcoma ◽  
2000 ◽  
Vol 4 (3) ◽  
pp. 119-123 ◽  
Author(s):  
Martin D. McCarter ◽  
Jonathan J. Lewis ◽  
Cristina R. Antonescu ◽  
Murray F. Brennan

Purpose.Extraskeletal osteosarcoma represents an unusual soft-tissue sarcoma that historically is reported to carry an exceptionally poor prognosis.The objectives of this study were to use a prospectively gathered sarcoma database to test the prevailing clinical bias and more accurately describe the natural history, characterize the prognostic features, estimate survival and evaluate treatment strategies for this unusual sarcoma.Patients and methods.From a large database of nearly 4000 sarcomas at a single institution, 15 patients with pathologically confirmed extraskeletal osteosarcoma were analysed.Results and discussion.Extraskeletal osteosarcoma usually occurs as a large, deep, high-grade lesion in the lower extremity of older patients. Overall and disease-specific survival at 5 years was 50%, with a median follow-up of 35 months (range 3– 200 months). Use of adjuvant chemotherapy or radiation therapy did not appear to influence survival, but an effect may have been missed by the relatively low numbers in each group.When matched to a comparable group of patients with stage III extremity sarcomas, there was no significant difference in overall or disease-specific survival between groups. Treatment for extraskeletal osteosarcoma should follow established guidelines for treatment of soft-tissue sarcomas, with the decision regarding adjuvant therapy to be based on individual risk factors.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e23571-e23571
Author(s):  
Edward De Leo ◽  
Wissam Hanayneh ◽  
Chintan Shah ◽  
Yu Wang ◽  
Ji-Hyun Lee ◽  
...  

e23571 Background: Soft tissue sarcomas (STS) are a rare, heterogenous group of cancers that tend to have a poor prognosis and few effective treatment strategies. Beta blockers (BB), in particular non-selective BB such as propranolol, have been shown to have an antitumor effect in different malignancies, including breast, colon, and angiosarcomas. The underlying mechanism is unclear but it is thought to be related to the inhibition of beta receptors on malignant cells, which leads to decrease in tumor vascularity and hematogenous spread. In this retrospective analysis, we examined the effect of BB on the outcomes of STS at a tertiary referral center. Methods: Adult patients from 2000 through 2019 with a diagnosis of STS with adequate follow up were included, and were divided into 2 groups: patients on BB at any point during treatment and/or up to 6 months prior to diagnosis, and patients without BB. The patient and tumor data were extracted and analyzed. The primary outcome was overall survival (OS). Statistical methods included descriptive analysis, univariable and multivariable Cox proportional hazard regression analyses and Kaplan-Meier survival plots. Results: 448 patient charts were analyzed. Median age at diagnosis was 55 (range 18-89). 54% were male. 4.9% of patients were diagnosed with stage 4 disease. Histologies included: undifferentiated pleomorphic sarcoma 26.1%, leiomyosarcoma 10%, synovial sarcoma 9.2%, liposarcoma 7.8%, osteosarcoma 2.7%, Ewing sarcoma 2% and others 42.2%. 18.1% of patients were on BB during treatment, with metoprolol the most commonly used (59%). 96% of patients on BB had HTN and 44% had coronary artery disease. Patients on BB were less likely to receive adjuvant therapy (18% vs 26%). Patients on BB were more likely to have stage 4 disease at presentation (9.9% vs 3.8%). Multivariate analysis showed there was no significant difference in overall survival between patients on BB and those not on BB, HR 1.3 (CI 0.8-2.1, p = 0.24). Conclusions: There is no difference in overall survival in patients on beta blockers during treatment for STS after controlling for stage, comorbidities, smoking, and age. Further studies are needed to evaluate the effect of specific beta blocker drugs on soft tissue sarcoma outcomes.


Sarcoma ◽  
2010 ◽  
Vol 2010 ◽  
pp. 1-10 ◽  
Author(s):  
Thomas Karsten Kilvaer ◽  
Andrej Valkov ◽  
Sveinung W. Sorbye ◽  
Tom Donnem ◽  
Eivind Smeland ◽  
...  

Background. Optimal treatment of nongastrointestinal stromal tumor soft-tissue sarcomas (non-GIST STSs) is resection with wide margins. This study investigates the prognostic impact of the angiogenesis-associated platelet-derived growth factors (PDGFs) and their receptors (PDGFRs) in non-GIST STS patients with wide and nonwide resection margins.Method. Tumor samples and clinical data from 249 patients with non-GIST STS were obtained, and tissue microarrays were constructed for each specimen. Immunohistochemistry was used to evaluate the expression of PDGF-A, -B, -C, and -D and PDGFR-αand -β.Results. In the multivariate analysis of patients with wide resection margins, high expression of PDGF-B (, HR = 2.954, and 95% CI = 1.255–6.956) and the coexpression of PDGF-B and PDGFR-α(overall; , high-low/low-high; , HR = 2.678, 95% CI = 0.996–7.200, high/high; , HR = 3.930, 95% CI = 1.542–10.015) were independent negative prognostic markers for disease-specific survival.Conclusion.PDGF-B and the coexpression of PDGF-B and PDGFR-αare strong and independent prognostic factors in non-GIST STSs with wide resection margins.


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e23529-e23529
Author(s):  
Ari Rosenberg ◽  
Sara Kochanny ◽  
James Dolezal ◽  
Cindy Wang ◽  
Heather Chen ◽  
...  

e23529 Background: Soft tissue sarcomas (STS) are histologically and molecularly heterogenous with variable biologic behavior and differential therapeutic responses. Molecular analysis of STS is commonly utilized to identify targetable mutations or identify STS subtypes that have predictive and prognostic implications. To address the challenge of rapid, accurate classification of STS subtypes, we sought to develop an approach using a form of artificial intelligence called deep learning to identify meaningful hidden information directly from digital H+E slide images. We trained a deep learning model using genotype and phenotype data from The Cancer Genome Atlas (TCGA) and companion diagnostic digital H+E slide images, in order to identify therapeutic biomarkers and prognostic molecular subtypes. Methods: Digital whole slide images (WSIs) from TCGA were downloaded and regions of tumor were annotated by pathologists. WSIs were carved into 604 um by 604 um square tiles within the tumor region and saved at an image size of 598 px by 598 px for a resolution of ~1um/px. These tiles were used as input to a deep convolution neural network (Xception model pretrained on imagenet). Performance results are reported at the as the average of three-fold cross-validation, averaged at the patient level. Results: 206 sarcomas available through the TCGA were used as training set. Sarcoma histologies include 80 leiomyosarcoma (LMS) (27 uterine LMS and 53 non-uterine LMS), 50 dedifferentiated liposarcoma (DDLPS), 44 undifferentiated pleomorphic sarcoma (UPS), 72 myxofibrosarcoma (MFS), and 10 synovial sarcoma (SS). FNCLCC grade 1 (n = 14), grade 2 (n = 112), and grade 3 (n = 80). Histology could be predicted automatically with very high accuracy for LMS (Area under Receiver-operator curve (AUROC) 0.93 and 0.95 for uterine and non-uterine LMS respectively), DDLPS (AUROC 0.70), UPS (AUROC 0.76), and MFS (AUROC 0.76). FNCLCC grade could be predicted for grade 3, 2, 1 with AUROC of 0.79, 0.73, and 0.81 respectively. High vs. low mitotic rate (threshold 15) could be predicted with AUROC of 0.70. Within non-uterine LMS, prognostic molecular cluster could be predicted with AUC of 0.62 for cluster with worse recurrence-free and disease specific survival. Within DDLPS, DNA methylation cluster 2 associated with worse disease specific survival was predicted with AUC of 0.80. Conclusions: Deep learning algorithm analysis of histologic slides was able to predict clinically meaningful characteristics and molecular clusters with reasonable accuracy. Retrospective and prospective validation is needed.


1996 ◽  
Vol 14 (5) ◽  
pp. 1679-1689 ◽  
Author(s):  
P W Pisters ◽  
D H Leung ◽  
J Woodruff ◽  
W Shi ◽  
M F Brennan

PURPOSE To identify specific independent adverse clinicopathologic factors for event-free survival in a cohort of consecutively treated patients with extremity soft tissue sarcomas. PATIENTS AND METHODS Prospectively collected data from a population of 1,041 adult patients with localized (American Joint Committee on Cancer [AJCC] stage IA to IIIB) extremity soft tissue sarcomas were analyzed. Patients were treated at a single institution between 1982 and 1994. Patient, tumor, and pathologic factors were analyzed by univariate and multivariate techniques to identify independent prognostic factors for the end points of local recurrence, distant recurrence, disease-specific survival, and post-metastasis survival. RESULTS The 5-year survival rate for this cohort of patients was 76%, with a median follow-up time of 3.95 years. Significant independent adverse prognostic factors for local recurrence were age greater than 50 years, recurrent disease at presentation, microscopically positive surgical margins, and the histologic subtypes fibrosarcoma and malignant peripheral-nerve tumor. For distant recurrence, intermediate tumor size, high histologic grade, deep location, recurrent disease at presentation, leiomyosarcoma, and nonliposarcoma histology were independent adverse prognostic factors. For disease-specific survival, large tumor size, high grade, deep location, recurrent disease at presentation, the histologic subtypes leiomyosarcoma and malignant peripheral-nerve tumor, microscopically positive surgical margins, and lower extremity site were adverse factors. For post-metastasis survival, only large tumor size ( > 10 cm) was an adverse prognostic factor. CONCLUSION The independent adverse prognostic factors for distant recurrence and disease specific survival differ from those identified for subsequent local recurrence. Patients with microscopically positive surgical margins or patients who present with locally recurrent disease are at increased risk for subsequent local recurrence and tumor-related mortality. Specific histopathologic subtypes are associated with increased risks for local failure and tumor-related mortality.


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.


Cancer ◽  
2001 ◽  
Vol 92 (4) ◽  
pp. 869-874 ◽  
Author(s):  
Axel Hoos ◽  
Alexander Stojadinovic ◽  
Stephen Mastorides ◽  
Marshall J. Urist B.S. ◽  
David Polsky ◽  
...  

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

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.


1987 ◽  
Vol 5 (6) ◽  
pp. 851-861 ◽  
Author(s):  
L H Baker ◽  
J Frank ◽  
G Fine ◽  
S P Balcerzak ◽  
R L Stephens ◽  
...  

The term soft tissue sarcoma refers to a large variety of malignant tumors arising in extraskeletal connective tissues that connect, support, and surround discrete anatomic structures. All visceral organs also contain a connective stroma that can undergo malignant transformation. Because of the histological similarities of this group of tumors and their relative rarity, treatment prescriptions for patients that have disseminated disease are most often uniform. In this study, we asked the question whether adding a third drug (cyclophosphamide or actinomycin D) to Adriamycin (Adr [Adria Laboratories, Columbus, OH])-(3,3-dimethyl-1-triazeno)- imidazole-4-carboxamide (DTIC) would improve the response rate and/or survival. A unique feature of this cooperative group clinical trial was the mandatory pathology review of the histological material. All patients of the Southwest Oncology Group between June 1, 1976, and November 17, 1979, who had a biopsy-confirmed diagnosis of a soft tissue sarcoma with convincing clinical or biopsy-documented evidence of metastatic disease were eligible for the study. Patients were randomized to receive (1) Adr, 60 mg/m2 intravenously, day 1, and DTIC, 250 mg/m2 every 3 weeks (104 patients); (2) Adr and DTIC as in (1) and cyclophosphamide, 500 mg/m2, day 1 (112 patients); or (3) Adr and DTIC as in (1) and actinomycin D, 1.2 mg/m2, day 1, (119 patients). There was no statistically significant difference in response rates (33%, 34%, and 24%) (P = .25). Median durations of response were 31 weeks in the Adr-DTIC arm, 26 weeks in the cyclophosphamide-DTIC-Adr arm, and 23 weeks in the Adr-DTIC-Actinomycin D arm (P = .78). Median durations of survival were 37, 42, and 50 weeks, respectively. Again, no statistically significant differences were observed (P = .59). Toxicities from each of these treatment arms were formidable and were equivalent. Prognostic factor analysis showed a prognosis based on bone marrow reserve, sex, and pathology subtype favorable to patients.


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