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Matti S. Aapro, MD, of the Genolier Cancer Center, reviews the state of the science in sarcoma treatment: specialized surgery, disease diversity, emerging therapies, and the diagnostic approach to soft-tissue disease.
Pembrolizumab monotherapy induced responses in patients with rare sarcomas that varied by histotype, according to findings presented by Jean-Yves Blay, MD, at the ESMO Virtual Congress 2020 (Abstract 1619O). Methods Dr. Blay presented first results from a cohort of patients with rare sarcomas in...
As reported in The Lancet Oncology by Bonvalot et al, the phase III EORTC-62092 (STRASS) trial showed that the addition of preoperative radiotherapy to surgery did not improve abdominal recurrence–free survival in patients with primary retroperitoneal sarcoma. Study Details In the open-label trial, ...
In a German Intergroup phase II study reported in the Journal of Clinical Oncology, Grünwald et al found that progression-free survival with first-line pazopanib was noninferior to doxorubicin in patients aged 60 years or older with inoperable metastatic soft-tissue sarcoma. Pazopanib was also...
Doxorubicin is a standard of care in patients with advanced inoperable soft-tissue sarcoma. In the EPAZ study, German researchers tested whether pazopanib showed comparable efficacy to doxorubicin in the first-line treatment of elderly patients with soft-tissue sarcoma. Grünwald et al reported in...
In an interim analysis of a joint Children’s Oncology Group and NRG Oncology phase II trial (ARST1321) reported in The Lancet Oncology, Aaron R. Weiss, DO, and colleagues found that the addition of pazopanib to preoperative chemoradiotherapy significantly increased the rate of pathologic...
Patricia Pautier, MD, of Institut Gustave Roussy, discusses final results of the phase II LMS-02 study, which showed the combination of doxorubicin and trabectedin to be an effective first-line therapy for patients with leiomyosarcoma, with an acceptable safety profile (Abstract 11506).
As reported in the Journal of Clinical Oncology by Alessandro Gronchi, MD, and colleagues, final results of a phase III trial conducted by the Italian, French, and Polish Sarcoma Groups showed no disease-free or overall survival benefit with use of histology-tailored vs standard...
On May 14, 2020, the U.S. Food and Drug Administration (FDA) expanded the indication of pomalidomide (Pomalyst) to include treating adult patients with acquired immunodeficiency syndrome (AIDS)-related Kaposi sarcoma after failure of highly active antiretroviral therapy and those with Kaposi...
Immunotherapy is showing promise for patients with rare cancers, offering new treatment opportunities and clinical trials to those with previously limited options. At the 2020 ASCO-SITC Clinical Immuno-Oncology Symposium, presenters discussed the use of immunotherapy in three low-incidence cancers: ...
In a study reported in JCO Oncology Practice, Villano et al found that increased hospital surgical volume was associated with overall improved survival in patients undergoing surgery for retroperitoneal soft-tissue sarcoma. However, increased volume was associated with poorer survival in patients...
In a phase I study reported in the Journal of Clinical Oncology, William D. Tap, MD, and colleagues found that the selective mutant-IDH1 inhibitor ivosidenib produced no objective responses but resulted in durable disease control in a cohort of patients with advanced chondrosarcoma. As noted by...
On January 23, 2020, tazemetostat was granted accelerated approval for the treatment of adults and pediatric patients aged 16 and older with metastatic or locally advanced epithelioid sarcoma not eligible for complete resection.1,2 Supporting Efficacy Data Approval was based on the finding of...
In the French phase II CABONE trial reported in The Lancet Oncology, Italiano et al found that the MET and VEGFR2 inhibitor cabozantinib was active in patients with advanced Ewing sarcoma or osteosarcoma. Study Details In the multicenter trial, 81 evaluable patients aged ≥ 12 years with progressive ...
Comprehensive profiling of tumor samples taken from patients with osteosarcoma showed that multiple factors contribute to the traditionally poor responses observed with immune checkpoint inhibitor treatment in patients with this malignancy, according to new research published by Wu et al in Nature...
In a single-institution phase II study reported in JAMA Oncology, Kelly et al found that the combination of talimogene laherparepvec (T-VEC) and pembrolizumab produced durable responses in patients with previously treated advanced sarcoma. As noted by the investigators, T-VEC has been found to...
On January 23, the U.S. Food and Drug Administration (FDA) granted accelerated approval to tazemetostat (Tazverik) for adults and pediatric patients aged 16 years and older with metastatic or locally advanced epithelioid sarcoma not eligible for complete resection. “Epithelioid sarcoma accounts...
In a study reported in the Journal of Clinical Oncology, Kelley et al found that pathologic fracture was associated with poorer overall survival among adult—but not pediatric—patients with primary central high-grade osteosarcoma of the extremities. Study Details The study involved retrospective...
The likelihood of a patient responding to immune checkpoint blockade may depend on B cells in the tumor, located within specialized immune-cell clusters known as tertiary lymphoid structures, according to three studies all recently published in Nature. The studies showed that enrichment of B cells...
Edward A. Stadtmauer, MD, of the University of Pennsylvania Abramson Cancer Center, discusses phase I results of immune cells, modified with CRISPR/Cas9 technology, and infused in three patients (two with multiple myeloma and one with sarcoma). Researchers observed the cells expand and bind to their tumor targets with no serious side effects (Abstract 49).
In the phase III RMS 2005 trial reported by Gianni Bisogno, MD, and colleagues in The Lancet Oncology, maintenance vinorelbine and low-dose continuous cyclophosphamide was associated with nonsignificant improvement in disease-free survival and significantly improved overall survival vs no further...
As reported in the Journal of Clinical Oncology by Dirksen et al, the R2Pulm trial showed no significant event-free or overall survival benefit with busulfan/melphalan high-dose chemotherapy with autologous stem cell rescue (BuMel) vs standard chemotherapy with whole-lung irradiation in patients...
Recently, the U.S. Food and Drug Administration (FDA) granted Breakthrough Therapy designation to tepotinib for metastatic non–small cell lung cancer harboring MET exon 14 skipping alterations, and Orphan Drug designations to novel chimeric antigen receptor T-cell therapies in both multiple myeloma ...
The 2019 ASCO Annual Meeting featured a wealth of presentations on important topics. In addition to our regular news coverage of the meeting, we present below some highlights of other studies that add to our knowledge base for treatment of various cancers. Olaratumab in Soft-Tissue Sarcoma...
Findings from a cohort of a phase II study reported by Stacchiotti et al in The Lancet Oncology indicated activity of the antiangiogenic agent pazopanib in advanced extraskeletal myxoid chondrosarcoma. As noted by the investigators, this rare sarcoma has low sensitivity to cytotoxic chemotherapy,...
Recently, the U.S. Food and Drug Administration (FDA) approved the inclusion of overall survival from the PACIFIC trial in the U.S. prescribing information for durvalumab and accepted applications for a new drug in the treatment of epithelioid sarcoma and two orphan drugs in the treatment of acute...
In the phase II/III Act.In.Sarc trial reported in The Lancet Oncology, Bonvalot et al found that preoperative addition of the radioenhancer hafnium oxide nanoparticle NBTXR3 to radiotherapy may improve outcomes in patients with locally advanced soft-tissue sarcoma. The agent acts to increase...
In the phase II CASPS trial reported in The Lancet Oncology, Judson et al found evidence of activity of cediranib in alveolar soft-part sarcoma. In the double-blind trial, 48 patients from 12 sites in the United Kingdom, Spain, and Australia were randomly assigned 2:1 between July 2011 and July...
In a phase II trial reported in The Lancet Oncology, Breelyn Wilky, MD, and colleagues found evidence of activity of axitinib plus pembrolizumab in advanced sarcomas, including alveolar soft-part sarcoma. Study Details The study involved 33 patients with advanced or metastatic sarcomas, including...
In the phase II SARC024 trial in sarcoma subtypes reported in the Journal of Clinical Oncology, Davis and colleagues found that regorafenib improved progression-free survival vs placebo in the cohort of patients with metastatic osteosarcoma. The double-blind study included 42 patients from 12 U.S. ...
William D. Tap, MD, of Memorial Sloan Kettering Cancer Center, discusses negative study findings on doxorubicin plus olaratumab vs doxorubicin plus placebo, which showed no difference in overall survival between the two treatments in patients with advanced soft-tissue sarcomas. The manufacturer is currently withdrawing olaratumab from the global market (Abstract LBA3).
In the European phase II VIT-091 trial, researchers examined the efficacy of the combination of vincristine and irinotecan with or without the addition of temozolomide in children and adults with relapsed or refractory rhabdomyosarcoma. Their results were presented by Defachelles et al at the 2019...
In 1996, the National Comprehensive Cancer Network® (NCCN®) published its first set of Clinical Practice Guidelines in Oncology (NCCN Guidelines®), covering eight tumor types. NCCN Guidelines are now published for more than 70 tumor types and topics. Some of the key updates for 2019 were presented...
This week, the U.S. Food and Drug Administration granted designations in relapsed or refractory multiple myeloma, adenoid cystic carcinoma, and Kaposi sarcoma; and the FDA’s Oncologic Drugs Advisory Committee (ODAC) held votes on treatments for tenosynovial giant cell tumor and acute...
In a phase II trial reported in The Lancet Oncology, Wilky et al found evidence of activity of axitinib plus pembrolizumab in advanced sarcomas, including alveolar soft-part sarcoma. Study Details The study involved 33 patients with advanced or metastatic sarcomas, including alveolar soft-part...
THE 2019 American Association for Cancer Research (AACR) Annual Meeting was held March 29 to April 3 in Atlanta. In addition to our regular coverage of news stories from the meeting, here are some brief highlights of additional noteworthy studies. Stage IV HER2-Positive Breast Cancer: Surgery or No ...
As reported in the Journal of Clinical Oncology by Davis et al, outcomes in the osteosarcoma cohort of the phase II SARC024 trial indicated a progression-free survival benefit with regorafenib vs placebo in previously treated metastatic osteosarcoma. Study Details The randomized...
On April 25, Eli Lilly and Company announced that the company has been working to facilitate the withdrawal of olaratumab from the market for the treatment of advanced soft-tissue sarcoma. Lilly's actions to withdraw olaratumab from the market follow completion of the international phase III...
A small phase I study by Navai et al investigated a combination of lymphodepletion chemotherapy and HER2-specific chimeric antigen receptor (CAR) T-cell therapy for pediatric and adult patients with advanced HER2-positive sarcoma. The combination showed promising antitumor activity and was found to ...
Those dealing with sarcoma know that resources and support can be difficult to find. Members of the newly formed Sarcoma Coalition are working together to change that. The Sarcoma Coalition is a nonprofit collaboration of sarcoma advocacy organizations from around the country working toward a...
It is estimated that 20% to 33% of patients undergoing cancer treatment are concomitantly using a gastric acid suppressant, most commonly a proton pump inhibitor (including omeprazole and esomeprazole magnesium) or a histamine H2-receptor blocker (such as ranitidine). A study by Mir et al...
In a study reported in the Journal of Clinical Oncology, Vaarwerk et al found that presence of indeterminate pulmonary nodules at diagnosis of pediatric rhabdomyosarcoma did not adversely affect treatment outcome in otherwise localized disease. The study involved 316 patients from the European...
The recently reported results of ANNOUNCE, the phase III study of olaratumab in combination with doxorubicin in patients with advanced or metastatic soft-tissue sarcoma, did not confirm the clinical benefit of olaratumab in combination with doxorubicin as compared to doxorubicin, a...
In a study reported in the Journal of Oncology Practice, Villano et al found that regionalization of retroperitoneal sarcoma surgery—recognized as a complex cancer surgery—to high-volume hospitals has been slower than that observed for similarly complex pancreatic cancer surgery over...
In a phase II trial reported in The Lancet Oncology, Duffaud et al found evidence of activity of regorafenib (Stivarga) in a cohort of adult patients with previously treated metastatic osteosarcoma. This noncomparative, double-blind trial involves 4 cohorts of patients, consisting of those with...
The U.S. Food and Drug Administration (FDA) recently approved levoleucovorin (Khapzory) injection, a folate analog, for three indications: rescue after high-dose methotrexate therapy in patients with osteosarcoma; diminishing the toxicity associated with overdosing on folic acid antagonists or...
In a study reported in the Journal of Clinical Oncology, Walterhouse et al found that age ≥ 10 years at diagnosis and tumor size > 5 cm are adverse prognostic features in localized paratesticular rhabdomyosarcoma. Study Details The study was a pooled analysis of data from North American and ...
The U.S. Food and Drug Administration (FDA) recently approved levoleucovorin (Khapzory) injection, a folate analog, for three indications: rescue after high-dose methotrexate therapy in patients with osteosarcoma; diminishing the toxicity associated with overdosage of folic acid...
Angiosarcoma of the head, face, neck, or scalp is a rare cancer associated with high rates of local recurrence, distant metastasis, and poor prognosis. Because angiosarcoma is so rare, it is difficult to conduct large-scale research in the cancer. To address this issue, the Broad Institute of MIT...
As reported in the Journal of Clinical Oncology by Whelan et al, combined trials (EURO-EWING99 and Ewing-2008) have shown evidence of improved outcomes with high-dose therapy with busulfan and melphalan (BuMel) and autologous stem cell transplant (SCT) vs standard chemotherapy with vincristine,...