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

Abstract

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.

A series Cases study of Carcinomas derived from myoepithelial cells in head and neck regions

“…Despite this, our study demonstrated high recurrence rate, since 40% of the patients with CMCHN had recurrences after primary treatments, among which, 26.7% were local-regional recurrences, and 20% were distant metastases. These results were consistent with previous reports showing high incidences of local-regional recurrence and distant metastasis in patients with CMCHN [9][10][11]. In this study, the treatment was mainly complete surgical resection.…”

“…Carcinomas derived from myoepithelial cells in head and neck regions (CMCHN) can be mainly divided into three types: 1) myoepithelial carcinoma (MEC), which is purely composed of myoepithelial cells and accounted for < 2% of all salivary gland carcinomas [6]; 2) the epithelial myoepithelial carcinoma (EMC, also called adenomyoepithelioma), represents approximately 1% of salivary gland tumors [7], and is histologically consist of a biphasic arrangement of inner luminal ductal cells and outer myoepithelial cells; 3) adenoid cystic carcinoma (AdCC), a biphasic tumor comprised of ductal and myoepithelial cells which tends to be arranged in a more cribriform pattern. [8] The main therapy for CMCHN is surgical resection with clear margins, with or without pre-/post-operative radiation [9]. However, due to the rarity of this carcinoma, its clinical characteristics, proper treatment modalities, and their correlation with prognosis remain unclear [9][10][11].…”

“…[8] The main therapy for CMCHN is surgical resection with clear margins, with or without pre-/post-operative radiation [9]. However, due to the rarity of this carcinoma, its clinical characteristics, proper treatment modalities, and their correlation with prognosis remain unclear [9][10][11].…”

A first case report of clinical response to targeted therapy in a patient with primary myoepithelial carcinoma of the lung harboring EGFR exon 19 deletion

“…The most common practice to manage localized myoepithelial carcinoma is by performing surgical excision with or without adjuvant radiation therapy [7]. However, myoepithelial carcinoma has a high tendency of local recurrences and metastases that warrants close clinical follow-up.…”

Aggressive myoepithelial carcinoma with EWSR1‐POU5F1 fusion highly responsive to Ewing sarcoma combination chemotherapy

“…[1][2][3] It is considered to be relatively chemoresistant, with no standard therapy reported within the metastatic setting. [4][5][6] EWS RNA-binding protein 1 (EWSR1) gene rearrangements are present in approximately 82% of myoepithelial tumors arising from soft tissue, bone, and visceral locations, 7 as well as in approximately 39% of clear cell myoepithelial carcinomas arising from the salivary glands. 8 POU class 5 homeobox 1 (POU5F1) is the fusion partner of EWSR1 in approximately 28% of EWSR1 rearrangement-positive myoepithelial tumors arising from soft tissue, bone, and visceral locations, with a predilection toward a more malignant phenotype.…”