Adult soft tissue myoepithelial carcinoma: treatment outcomes and efficacy of chemotherapy

• Published in: Medical oncology (Northwood, London, England) London, England), 2020-02, Vol.37 (2), p.13-13, Article 13
• Main Authors: Chamberlain, Florence, Cojocaru, Elena, Scaranti, Mariana, Noujaim, Jonathan, Constantinou, Anastasia, Thway, Khin, Fisher, Cyril, Messiou, Christina, Strauss, Dirk C., Miah, Aisha, Zaidi, Shane, Benson, Charlotte, Gennatas, Spyridon, Jones, Robin L.
• Format: Article
• Language: English
• Subjects: Adult; Aged; Chemotherapy; Doxorubicin - therapeutic use; Female; Hematology; Humans; Internal Medicine; Male; Medicine; Medicine & Public Health; Middle Aged; Myoepithelioma - drug therapy; Myoepithelioma - pathology; Neoplasm Staging; Oncology; Original Paper; Pathology; Patients; Prospective Studies; Retrospective Studies; Sarcoma; Soft Tissue Neoplasms - drug therapy; Soft Tissue Neoplasms - pathology; Survival Rate; Topoisomerase II Inhibitors - therapeutic use; Treatment Outcome; Tumors
• ISSN: 1357-0560; 1559-131X
• DOI: 10.1007/s12032-019-1335-4

Soft tissue myoepithelial carcinomas are a rare, malignant subgroup of myoepithelial tumours mostly arising in the extremities with equal predilection for women and men. The mainstay of management of localised disease is complete surgical resection. Despite optimal treatment, 40–45% of tumours recur. Data regarding the efficacy of systemic therapy for advanced and metastatic disease are lacking. The primary aim of this study was to evaluate the outcome of all patients with soft tissue myoepithelial carcinoma treated at a single referral centre. The secondary aim was to establish the efficacy of systemic therapies in patients with advanced disease. A retrospective review of the prospectively maintained Royal Marsden Sarcoma Unit database was performed to identify soft tissue myoepithelial carcinoma patients treated between 1996 and 2019. Patient baseline characteristics and treatment history were recorded. Response to systemic therapy was evaluated using RECIST 1.1. We identified 24 patients treated at our institution between 1996 and 2019,12 males and 12 females. Median age at presentation was 49.6 years [interquartile range (IQR) 40.5–63.3 years]. Twenty-two out of 24 patients (91.7%) underwent primary surgical resection. Nine patients (37.5%) received systemic treatment. A partial response was documented in one patient treated with doxorubicin. The median progression-free survival for first-line chemotherapy was 9.3 months. Myoepithelial carcinoma frequently recurs after complete surgical resection. Conventional chemotherapy demonstrated some activity in myoepithelial carcinoma, however, more effective systemic therapies are required and enrolment in clinical trial should be encouraged.