scholarly journals Systemic Treatment for Soft Tissue Sarcoma: What is Standard, What is New

2018 ◽  
Vol 1 (Supplement) ◽  
pp. 64
Author(s):  
D.C. Jinga ◽  
D. Chetroiu
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Adjuvant Treatment ◽  
Kinase Inhibitors ◽  
Systemic Treatment ◽  
Growth Factor Receptor ◽  
Soft Tissue Sarcomas ◽  
High Grade ◽  
History Of ◽  
New Treatment

Abstract Soft tissue sarcoma (STS) is a biologically heterogeneous malignancy with over 50 subtypes. This solid tumor is one of the most challenging diseases to treat for the medical oncologist. STS often forms in the body’s muscles, joints, fat, nerves, deep skin tissues, and blood vessels. The natural history of high-grade STS is characterized by a strong tendency toward local recurrence and metastatic spreading, despite optimal initial strategies. The lung is the most common site of metastases, with poor prognosis. We present the current international guidelines for the adjuvant treatment and systemic treatment for advanced STS and the new discoveries. Many new molecular targeting drugs have been tried in the last ten years, and some were approved for soft tissue sarcoma. The first approved was Imatinib, as a treatment for gastrointestinal stromal tumors (GISTs). Following Imatinib, other tyrosine kinase inhibitors (TKIs) received the approval for GISTs such as Sunitinib and Regorafenib, and Pazopanib for non-GIST soft tissue sarcomas. In 2016, FDA approved the first monoclonal antibody that targets platelet-derived growth factor receptor (PDGFR)-α, Olaratumab. The new treatment demonstrates an overall survival advantage. In this review, we aimed to summarize the results from the most recent studies on adjuvant treatment for high-grade STS and systemic strategies for advanced STS.

scholarly journals Smoking and Family Cancer History Are Associated With Sarcomas in A Japanese Population Study

2020 ◽  
Author(s):  
Yoshihiro Araki ◽  
Norio Yamamoto ◽  
Yoshikazu Tanzawa ◽  
Takahiro Higashi ◽  
Katsuhiro Hayashi ◽  
...  
Keyword(s):  
Family History ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Smoking Habit ◽  
Soft Tissue Sarcomas ◽  
High Grade ◽  
Cancer History ◽  
Family Cancer History ◽  
History Of ◽  
History Of Cancer

Abstract Background: Sarcoma is a rare cancer, and it is also the cause of the development of various kinds of sarcomas, such as gene abnormalities, which has recently becoming evident due to advances of genetic testing. The approach to solve the origin of diseases is essential to elucidate both the external environmental factors and the internal genetic factors. However, the lifestyle habits, lifestyle-related diseases, personal and family cancer history of sarcoma patients remain unclear.Methods: A total of 1320 sarcoma patients were enrolled in this study. A questionnaire on lifestyle habits, life-style diseases, and the patient’s personal and family cancer history was completed at presentation. A total of 1320 controls were selected by propensity score matching for age and gender. Smoking, drinking, obesity, hypertension, dyslipidemia and diabetes mellitus were compared. In addition, we investigated the incidence of a personal and family cancer history in sarcoma patients. Results: A smoking habit was the only independent risk factor for high-grade soft tissue sarcoma development in adults ≥20 years old (n=952), excluding low-grade and intermediate malignant soft tissue tumors (Odds ratio [OR], 2.45; 95% confidence interval [CI] 1.88-3.20, p<0.001). The ORs of high-grade liposarcoma and undifferentiated pleomorphic sarcoma (UPS) were 2.56 and 3.00, respectively. Eight percent of sarcoma patients had a personal history of another cancer. Thirty percent of soft tissue sarcoma patients had a family history of cancer in a first-degree relatives (malignant peripheral nerve sheath tumor, 52%; leiomyosarcoma, 46%). Conclusions: We confirmed that a smoking habit were associated with the development of high-grade soft tissue sarcomas. A family history of cancer might be associated with certain soft tissue sarcomas, but a further investigation will be necessary.

Risk assessment in high grade sarcoma patients during neoadjuvant chemotherapy using multiple tracer PET

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 20006-20006
Author(s):  
J. F. Eary ◽  
E. Conrad ◽  
J. Link ◽  
A. Cizik ◽  
D. Mankoff ◽  
...  
Keyword(s):  
Drug Resistance ◽  
Soft Tissue ◽  
Neoadjuvant Chemotherapy ◽  
Soft Tissue Sarcoma ◽  
Pet Imaging ◽  
Cellular Proliferation ◽  
Soft Tissue Sarcomas ◽  
Response To Treatment ◽  
High Grade ◽  
Hypoxic Volume

20006 Background: Patients with high grade soft tissue sarcomas are treated with neoadjuvant chemotherapy. Sarcomas have biological features that may predict for poor outcome. Some of these features are tumor proliferation rate, level of tumor hypoxia, and upregulation of tumor drug resistance mechanisms. Methods: We have a group of specific PET imaging agents to quantify the level of activity of these tumor processes. Patients with soft tissue sarcomas receive [C-11]Thymidine (TdR) to assess cellular proliferation, [O-15] Water to quantify tumor blood flow and to serve as the input function for quantification of the other tracers, [C-11]Verapamil to assess drug resistance mechanism activity, and [F-18]Fluoromisonidazole) FMISO to quantify changes in tumor hypoxic volume in response to treatment. These studies are performed in a single PET imaging session prior to neoadjuvant chemotherapy, after the second of four cycles of therapy and in the week prior to resection. Results: An example of this complex study result, is demonstrated by a recent patient with a high grade soft tissue sarcoma. The tumor showed increased TdR uptake, a moderate hypoxic volume, and [C-11] verapamil uptake prior to initiation of neoadjuvant adriamycin based chemotherapy. After 2 cycles of therapy, there was a significant decrease in the maximum level and volume of TdR uptake and a large reduction in tumor hypoxic volume. Conclusions: These data would imply a high risk soft tissue sarcoma due the presence of increased cellular proliferation, a significant hypoxic volume and the absence of p-glycoprotein activity determined by the presence of [C-11]Verapamil uptake. However, early response is also suggested by the findings above. Patient outcome will be assessed and correlated with these tumor parameters to further understand what tumor biological risk factors can be quantified non-invasively and repeated throughout the clinical course in soft tissue sarcoma patients. Supported by NIH NCI PO1 42045–18 and S10 RR017229–01 [Table: see text] No significant financial relationships to disclose.

scholarly journals Don’t cancel the surgery just yet! A case report of positive preoperative pregnancy test due to a soft tissue sarcoma production of ectopic beta human chorionic gonadotropin

Rare Tumors ◽  
2018 ◽  
Vol 10 ◽  
pp. 203636131878972
Author(s):  
Alan Todd Blank ◽  
Mazdak Khalighi ◽  
R Lor Randall ◽  
Kevin B Jones
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Chorionic Gonadotropin ◽  
Human Chorionic Gonadotropin ◽  
Immunohistochemical Staining ◽  
Elevated Serum ◽  
Soft Tissue Sarcomas ◽  
High Grade ◽  
Beta Human Chorionic Gonadotropin ◽  
Rare Group

Soft tissue sarcomas are a rare group of mesenchymal malignancies which can range from low to high grade. These tumors have different clinical, radiographic, and histopathological characteristics. Beta human chorionic gonadotropin is a naturally secreted hormone by placental syncytiotrophoblast cells during pregnancy. On very rare occasions, sarcomas can develop the ability to ectopically produce human chorionic gonadotropin. Very few cases exist in the literature of soft tissue sarcomas expressing this hormone. We report the case of a 55-year-old female who presented with a posterior thigh soft tissue sarcoma who on the day of surgical resection was found to have an unusually elevated serum human chorionic gonadotropin. Positive immunohistochemical staining of the resected mass confirmed the sarcoma as the source of the beta human chorionic gonadotropin.

Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma.

1996 ◽  
Vol 14 (3) ◽  
pp. 859-868 ◽  
Author(s):  
P W Pisters ◽  
L B Harrison ◽  
D H Leung ◽  
J M Woodruff ◽  
E S Casper ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Local Control ◽  
Distant Metastasis ◽  
Soft Tissue Sarcomas ◽  
Complete Resection ◽  
High Grade ◽  
Disease Specific Survival ◽  
Recurrence Rates ◽  
Disease Specific

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.

scholarly journals Chemotherapy in Pediatric-Type Soft-Tissue Sarcomas

2020 ◽  
Vol 26 ◽  
Author(s):  
K. M. Ingley ◽  
S. Cohen-Gogo ◽  
A. A. Gupta
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Alternative Treatment ◽  
Systemic Treatment ◽  
Treatment Options ◽  
Soft Tissue Sarcomas ◽  
Targeted Agents ◽  
Soft Tissue Tumours ◽  
Cooperative Approach ◽  
Multi Agent

Soft-tissue sarcoma (STS) is rare and represents approximately 7% of cancers in children and in adolescents less than 20 years of age. Rhabdomyosarcoma (RMS) is most prevalent in children less than 10 years of age and peaks again during adolescence (16–19 years of age). Multi-agent chemotherapy constitutes the mainstay of treatment for RMS. In other non-rhabdomyosarcoma soft-tissue tumours, such as synovial sarcoma, evidence for routine use of chemo-therapy is less robust, and alternative treatment options, including targeted agents and immunotherapy, are being explored. In this review, we focus on chemotherapy for pediatric-type RMS and discuss the advances and challenges in systemic treatment for select non-rhabdomyosarcoma soft-tissue tumours in children and adolescents. We  support an increasingly cooperative approach for treating pediatric and adult STS.

Dramatic response of metastatic peripheral nerve sheath sarcoma to cetuximab combined with doxorubicin-based chemotherapy: Report of a case

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 19503-19503
Author(s):  
J. P. Holmes ◽  
T. J. Reid
Keyword(s):  
Abdominal Pain ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Growth Factor Receptor ◽  
Soft Tissue Sarcomas ◽  
Poor Response ◽  
Tumor Burden ◽  
Promote Tumor Growth ◽  
Routine Surveillance ◽  
Aggressive Tumors

19503 Background: Soft tissue sarcomas are aggressive tumors that often show poor response to conventional chemotherapy agents. Some of these tumors over express epidermal growth factor receptor (EGFR) which is thought to promote tumor growth. Cetuximab, a monoclonal antibody to EGFR, is FDA approved for use in colon cancer. We report a case here of use in a patient with an aggressive soft tissue sarcoma. Case Report: A 32 yr male presented in April 2005 for routine follow up three months after completing adjuvant radiotherapy for retroperitoneal soft tissue sarcoma. At this time the patient was found to have recurrence of disease on routine surveillance scans. During workup of the recurrent masses, he developed acute bowel obstruction and went emergently to the OR. Multiple masses were removed and he was rendered grossly disease free. Adjuvant adriamycin and ifosfamide were planned; however, bulky masses were again noted on a pre-chemo staging study. After a single cycle of chemotherapy, he presented with abdominal pain, and an urgent CT scan ordered showed progressive disease. Immunohistochemical stains demonstrated strong expression of EGFR on his tumor. Cetuximab was added to his chemotherapy regimen. Repeat studies showed dramatic (>30%) decrease in his tumor burden after only two doses. As cycle three began, the patient developed acute abdominal pain and fever; emergent scans showed free air within the abdomen. The majority of his tumor burden had continued to decrease. The perforation could not be surgically managed and the patient was discharged to hospice. He expired ten days after discovery of the perforation. Conclusions: Addition of cetuximab to therapy for an aggressive soft tissue sarcoma which overexpressed EGFR resulted in dramatic decrease in tumor burden. Unfortunately, the patient suffered perforation of abdominal viscera and subsequently expired. Rapid tumor shrinkage likely contributed to the perforation. Use of cetuximab in patients with aggressive tumors expressing EGFR should be explored in the setting of clinical trials. No significant financial relationships to disclose.

scholarly journals Predicting the Efficacy and Safety of TACTICs (Tumor Angiogenesis-Specific CAR-T Cells Impacting Cancers) Therapy for Soft Tissue Sarcoma Patients

Cancers ◽  
2020 ◽  
Vol 12 (10) ◽  
pp. 2735
Author(s):  
Kento Fujiwara ◽  
Shigemi Sasawatari ◽  
Sho Nakai ◽  
Keisuke Imaeda ◽  
Seina Nagai ◽  
...  
Keyword(s):  
T Cells ◽  
Soft Tissue ◽  
Tumor Angiogenesis ◽  
Growth Factor Receptor ◽  
Soft Tissue Sarcomas ◽  
Efficacy And Safety ◽  
Normal Tissues ◽  
Car T Cells ◽  
Car T ◽  
New Treatment

Soft tissue sarcomas (STSs) are heterogeneous and aggressive malignancies with few effective therapies available. We have developed T cells expressing a vascular endothelial growth factor receptor 2 (VEGFR2)-specific chimeric antigen receptor (CAR) to establish a tumor angiogenesis-specific CAR-T cells impacting cancers (TACTICs) therapy. In this study, we optimized the manufacturing and transportation of mRNA-transfected anti-VEGFR2 CAR-T cells and collected information that allowed the extrapolation of the efficacy and safety potential of TACTICs therapy for STS patients. Although 5-methoxyuridines versus uridines did not improve CAR-mRNA stability in T cells, the utilization of CleanCap as a 5′ cap-structure extended the CAR expression level, increasing VEGFR2-specific cytotoxicity. Furthermore, 4 °C preservation conditions did not affect the viability/cytotoxicity of CAR-T cells, contrarily to a freeze-thaw approach. Importantly, immunohistochemistry showed that most of the STS patients’ specimens expressed VEGFR2, suggesting a great potential of our TACTICs approach. However, VEGFR2 expression was also detected in normal tissues, stressing the importance of the application of a strict monitoring schedule to detect (and respond to) the occurrence of adverse effects in clinics. Overall, our results support the development of a “first in humans” study to evaluate the potential of our TACTICs therapy as a new treatment option for STSs.

Olaratumab: A Novel Platelet-Derived Growth Factor Receptor α-Inhibitor for Advanced Soft Tissue Sarcoma

2017 ◽  
Vol 51 (12) ◽  
pp. 1090-1098 ◽  
Author(s):  
Benjamin J. Andrick ◽  
Arpita Gandhi
Keyword(s):  
Growth Factor ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Literature Search ◽  
Data Extraction ◽  
Treatment Options ◽  
Growth Factor Receptor ◽  
Soft Tissue Sarcomas ◽  
Progression Free Survival ◽  
Advanced Soft Tissue Sarcoma

Objective: To review and summarize data on olaratumab, which was approved by the US Food and Drug Administration (FDA) in October 2016, in combination with doxorubicin, for the treatment of advanced soft tissue sarcoma. Data Sources: A literature search using PubMed was conducted using the search terms olaratumab, IMC-3G3, and advanced soft tissue sarcoma from January 2005 to June 2017. Study Selection and Data Extraction: The literature search was confined to human studies published in English. Trials of olaratumab for advanced soft tissue sarcomas were prioritized. Data Synthesis: Olaratumab is a human antiplatelet-derived growth factor receptor α monoclonal antibody. Its accelerated FDA approval was based on a phase II randomized trial of olaratumab plus doxorubicin (n = 66) versus doxorubicin monotherapy (n = 67) in patients with advanced soft tissue sarcoma. Olaratumab 15 mg/kg was administered intravenously (IV) on days 1 and 8 in combination with doxorubicin 75 mg/m2 IV on day 1 every 21 days for a total of 8 cycles compared to doxorubicin 75 mg/m2 IV monotherapy. The response rate was 18.2% with combination therapy versus 11.9% with monotherapy and median progression-free survival of 6.6 and 4.1 months, respectively. Additionally, overall survival was increased by 11.8 months in the olaratumab arm (26.5 months vs 14.7 months). Clinically relevant adverse effects in the olaratumab + doxorubicin arm included neutropenia (58%), mucositis (53%), nausea (73%), vomiting (45%), and diarrhea (34%). Conclusion: Olaratumab, in combination with doxorubicin, represents a novel treatment strategy for advanced soft tissue sarcoma and provides a significant survival advantage for this rare disease state with limited treatment options.

A High-Grade Primary Leiomyosarcoma of the Bladder in a Survivor of Retinoblastoma

2001 ◽  
Vol 125 (9) ◽  
pp. 1231-1234
Author(s):  
Sharon X. Liang ◽  
Yegappan Lakshmanan ◽  
Bruce A. Woda ◽  
Zhong Jiang
Keyword(s):  
Soft Tissue ◽  
Urinary Bladder ◽  
Soft Tissue Sarcomas ◽  
Common Type ◽  
Prior History ◽  
Adjuvant Radiation ◽  
High Grade ◽  
Cyclophosphamide Therapy ◽  
The Past ◽  
History Of

Abstract Second nonocular malignancies develop with increased incidence in patients with hereditary retinoblastoma. Osteosarcoma is by far the most common type with an incidence of up to 50%, followed by soft tissue sarcomas. Visceral leiomyosarcoma is extremely rare and only 2 cases have been reported in the past 2 decades, one in the liver and another one in the urinary bladder, both of which developed after cyclophosphamide therapy. Here we report a case of vesical leiomyosarcoma that was diagnosed in a 49-year-old woman 47 years after the diagnosis of a hereditary retinoblastoma. The patient's retinoblastoma was treated with unilateral enucleation without adjuvant radiation or chemotherapy. We believe that this is the first report of vesical leiomyosarcoma occurring in a patient with retinoblastoma without a prior history of radiation or chemotherapy. This report is significant not only because of the rarity of vesical leiomyosarcoma as a second nonocular tumor in retinoblastoma patients, but also because of the infrequency of vesical leiomyosarcoma in general. We also investigated the potential molecular pathogenesis of the leiomyosarcoma.

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