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Soft Tissue Sarcomas
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Thomas F. DeLaney, David C. Harmon, Karol Sikora, Francis J. Hornicek
Although the foregoing generalizations apply to most STSs, different histologic subtypes of sarcomas display their own patterns of chemosensitivity. Myxoid/round cell liposarcomas appear sensitive to doxorubicin and to trabectedin. Synovial sarcoma has a strong dose response to ifosfamide. Non-uterine leiomyosarcomas appear to have lower response rates to doxorubicin and ifosfamide, but may respond to trabectedin. Angiosarcomas are almost unique in being sensitive to paclitaxel. Rhabdomyosarcoma, desmoplastic small round cell tumors, and peripheral neuroectodermal tumors respond to combinations that include ifosfamide, etoposide, vincristine, doxorubicin, dactinomycin cyclophosphamide, and topotecan/irinotecan. Sunitinib has proven useful against solitary fibrous tumor/hemangiopericytoma, alveolar soft part sarcoma, and clear cell sarcoma. Everolimus has activity against tumors with perivascular epithelioid cell differentiation. Imatinib can help in metastatic dermatofibroma protuberans. Cediranib is effective against alveolar soft part sarcoma. Crizotinib is active against ALK translocated inflammatory myofibroblastic tumor. Bevacizumab with temozolomide has been employed for solitary fibrous tumor.
Sarcomas
Published in E. George Elias, CRC Handbook of Surgical Oncology, 2020
Alveolar soft part sarcoma — is an extremely rare tumor that occurs in young patients usually on the lower extremity. It has a tendency to metastasize to the lungs with a long latent period of being disease free.
Immune RNA and Tumor Immunity*
Published in Edward P. Cohen, A. Arthur Gottlieb, Immune RNA, 2020
Two types of patients were studied: patients with grossly detectable and measureable metastatic disease and patients with “minimum residual disease.” Patients in the latter category had no clinically detectable disease following surgical resection of all gross tumor, but had a greater than 50% likelihood of developing recurrent and/or metastatic disease within 24 months. The histologic types of tumors treated included malignant melanoma, hypernephroma, sarcoma, breast carcinoma, cholangiocarcinoma, and carcinoma of the stomach. The number of patients within each disease category and of each tumor type is given in Table 2. Of the three sarcoma patients, one had an alveolar soft part sarcoma, one a liposarcoma, and one an osteogenic sarcoma. Patients with gross disease were accepted for treatment only if: They had failed all standard therapy (including standard chemotherapy).No standard therapy of proven efficacy existed.They had relatively stable disease and standard therapy could be interrupted or withheld for 8 weeks (the minimum duration of the study).
What’s the latest with investigational drugs for soft tissue sarcoma?
Published in Expert Opinion on Investigational Drugs, 2022
Elena Cojocaru, Andrea Napolitano, Cyril Fisher, Paul Huang, Robin L Jones, Khin Thway
The phase 3 trial APROMISS compared dacarbazine vs anlotinib in patients with synovial sarcoma, randomized in a 1:2 ratio [49]. Patients had the option of crossing over on the progression of their disease and the results were presented at the ASCO meeting in 2021. A total of 79 participants received treatment with either dacarbazine or anlotinib and the PFS was statistically significant in the anlotinib arm, at 2.89 months compared to 1.64 months in the control arm with dacarbazine. Although the duration of PFS was not remarkably longer, the number of patients with a prolonged PFS was significantly higher in the anlotinib arm at 4, 6 and 12 months compared to dacarbazine (48.1%, 42.3% and 26.0% compared to 14.85%, 11.1% and 3.7%). The study has completed accrual for synovial sarcoma and leiomyosarcoma and is currently recruiting patients with alveolar soft part sarcoma. More ongoing trials are currently studying the combination of anlotinib with other active drugs in the treatment of sarcomas such as nivolumab, (NCT04165330), pegylated liposomal doxorubicin (NCT 04765228) or toripalimab (NCT04172805).
A multidisciplinary approach to soft-tissue sarcoma of the extremities
Published in Expert Review of Anticancer Therapy, 2020
Robert Nakayama, Tomoaki Mori, Yusuke Okita, Yutaka Shiraishi, Makoto Endo
Because of the high distinctiveness of RMS, other tumors are often referred to as non-RMS STS. The most common non-RMS STSs in AYA are synovial sarcoma and myxoid liposarcoma. More than half of patients with synovial sarcoma are in the AYA category. This malignancy can arise in any part of the body, but approximately 70% of lesions arise in the deep extremities [18]. Myxoid liposarcoma, the most common liposarcoma subtype in children and adolescents, accounts for 20–30% of liposarcomas, and this malignancy is also predisposed to arise in the deep extremities [19–22]. Most synovial sarcomas bear the SS18-SSX fusion gene, and more than 90% of myxoid liposarcomas bear the FUS-DDIT3 fusion gene [23,24]. Similarly, other STSs in the AYA group including clear cell sarcoma and alveolar soft part sarcoma tend to be characterized by tumor-specific genetic alternations [25]. The common extremity STSs in the elderly are undifferentiated pleomorphic sarcoma, leiomyosarcoma, and myxofibrosarcoma [26–29], and these cancers are usually characterized by nonspecific genetic alterations and complex unbalanced karyotypes [30].
Specific immune landscapes and immune checkpoint expressions in histotypes and molecular subtypes of sarcoma
Published in OncoImmunology, 2020
A. Dufresne, T. Lesluyes, C. Ménétrier-Caux, M. Brahmi, E. Darbo, M. Toulmonde, A. Italiano, O. Mir, A. Le Cesne, S. Le Guellec, T. Valentin, C. Chevreau, S. Bonvalot, Y.M. Robin, J-M Coindre, C. Caux, J.Y. Blay, F. Chibon
A large body of clinical and biological observations supports the relevance of the use of immunotherapy in sarcoma but their heterogeneity for histological and molecular subtypes has limited generalized approaches of immunotherapy in these tumors. Single-agent PD-1/PD-L1 antibodies (Ab) have limited efficacy in unselected series of sarcomas included in phase II clinical trials.2–4 Response rates are consistently low, with a median progression-free survival (PFS) of 3 months or less, and virtually no long-term PFS. Combination of anti-CTLA-4 with PD-1/or PD-L1 Ab may provide higher response rates and PFS but at the cost of higher toxicity. Biomarkers are lacking in these clinical trials.5 Somewhat unexpectedly, immunotherapy provides higher tumor control rates in Alveolar Soft Part Sarcoma (ASPS) and chordoma than other sarcoma types.6,7