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Sarcomas
Published in E. George Elias, CRC Handbook of Surgical Oncology, 2020
Epithelioid sarcoma — is a rare soft tissue sarcoma that is more common in young males. It has a benign clinical appearance as it often presents as subdermal hard nodules that may later ulcerate. It is commonly detected in the distal portion of the extremities such as the fingers, hands, wrists, forearms, feet, ankles, and legs; 10 to 30% may metastasize to the regional lymph nodes. Local recurrence is common if the primary is not widely excised. This excision may entail amputation.
Malignant tumors
Published in Archana Singal, Shekhar Neema, Piyush Kumar, Nail Disorders, 2019
Epithelioid sarcoma is a rare, clinically non-specific lesion often diagnosed only very late. Although a slow-growing neoplasm, the 5-year survival rate is barely 50%. At the distal digit, it appears as a tender swelling or firm myxoid pseudocyst. Most patients are young persons and the predominant localization is near a joint. Treatment of choice is amputation, but even then, the recurrence rate is high.95,96
Skin and soft tissue
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
Predictor of lymph node recurrence Histopathological tumour type – epithelioid sarcoma, RMS and clear cell sarcoma
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
Epithelioid sarcoma is a rare subtype of sarcoma, with very high morbidity and mortality rate. Tazemetostat is an inhibitor of enhancer of zeste homologue 2 (EZH2), a methyltransferase that plays a role in epithelioid sarcoma pathogenesis [32]. Integrase interactor 1 (INI1) loss is present in >90% of patients with epithelioid sarcoma [33]. Preclinical in vitro and in vivo models show that loss or dysfunction of INI1 can lead to aberrant EZH2 activity, resulting in oncogenic dependence on EZH2 [34]. Targeting EZH2 in relapsed or refractory follicular lymphoma, resulted in 69% objective and prolonged responses [35]. Tazemetostat has further been investigated in patients with epithelioid sarcoma with INI1 loss, and the results of the phase 2 trial demonstrated an objective response rate (ORR) at the data cut-off (September 2018) of 15%, corresponding to nine of 62 evaluable patients which had a reduction of their tumor. Some responders had a long clinical benefit [36]. Tazemetostat proved to be a well-tolerated drug, with the most common side effects being fatigue, nausea, and vomiting. The grade 3–4 side effects included anemia and weight loss [35,36].
Risk factors for the development of local recurrence in extremity soft-tissue sarcoma
Published in Expert Review of Anticancer Therapy, 2022
Fabio Tirotta, Raza Sayyed, Robin L Jones, Andrew J Hayes
Despite these concerns, it remains the case that surgery is the primary treatment for localized epithelioid sarcoma. The role of isolated limb perfusion (ILP) is currently unclear, with insufficient data to support its use other than in in-transit metastases, multifocal or refractory disease [31,32]. As over 90% of epithelioid sarcomas display INI1 loss leading to oncogenic dependence on the transcriptional repressor EZH2, early data with tazemetostat, a selective inhibitor of EZH2, has demonstrated some encouraging results for patients with advanced disease [32]. Retrospective studies have shown that conventional chemotherapy has similar response rates to tazemetostat in the advanced setting [33,34]. However, the favorable side effect profile of tazemetostat makes it an attractive option to consider in the management of this particular subtype.
Different approaches to advanced soft tissue sarcomas depending on treatment line, goal of therapy and histological subtype
Published in Expert Review of Anticancer Therapy, 2020
Javier Martín-Broto, Peter Reichardt, Robin L Jones, Silvia Stacchiotti
Clinical experience has indicated that doxorubicin + ifosfamide and gemcitabine-based regimens can be effective in epithelioid sarcoma, although duration of disease control is limited. In a retrospective collaborative effort involving 16 institutions in the EU, US, and Japan, retrospective data were collected on 115 patients with epithelioid sarcoma [100]. Anthracycline-based chemotherapy was associated with a RECIST response rate of 22% and a median PFS of 6 months, values comparable to those attained in other STS subtypes. Gemcitabine-based chemotherapy produced a response rate of 27% and a median PFS of 4 months; corresponding values with pazopanib were 0% and 3 months. Response rates were higher with anthracycline-based regimens in proximal versus distal primary tumors (26 vs. 18%), and with gemcitabine-based regimens in distal versus proximal primary tumors (40 vs. 14%).