Abstract
BackgroundMyoepithelial carcinoma of soft tissue is a rare, malignant neoplasm that is morphologically and immunophenotypically similar to its counterpart in salivary gland. It demonstrates myoepithelial differentiation, possessing both epithelial and myogenic characteristics. Thought to be chemotherapy insensitive, the optimal treatment regimen of this tumor has yet to be established and only a select few cases in the literature discuss treatment efficacy in detail.Case presentationHere we present a case of a young adult with metastatic myoepithelial carcinoma with an initial excellent response to systemic therapy utilizing carboplatin and paclitaxel with continued complete response after 3 years. The patient also underwent complete surgical excision and received adjuvant radiation to the primary site of disease. Exome sequencing revealed an inactivating mutation in RB1 which we believe to be the first such mutation to be reported in this cancer type.ConclusionsGiven increasing evidence suggesting RB1 loss is associated with responsiveness to conventional chemotherapies, particularly platinum-based regimens, we hypothesize that this genetic feature predisposed chemosensitivity in our patient’s tumor.
Highlights
BackgroundMyoepithelial tumors are rare salivary gland tumors classically found in the parotid gland
Myoepithelial carcinoma of soft tissue is a rare, malignant neoplasm that is morphologically and immunophenotypically similar to its counterpart in salivary gland
Given increasing evidence suggesting RB1 loss is associated with responsiveness to conventional chemotherapies, platinum-based regimens, we hypothesize that this genetic feature predisposed chemosensitivity in our patient’s tumor
Summary
Myoepithelial tumors are rare salivary gland tumors classically found in the parotid gland. The malignant counterpart, myoepithelial carcinoma, is even more rare and represents less than 2% of salivary gland carcinomas [1]. Most cases of myoepithelial carcinoma are de novo in origin but may occasionally arise in association with a preexisting myoepithelioma or benign mixed tumor (pleomorphic adenoma) [2]. These malignant tumors occur in non-salivary sites, such the nasopharynx, lung, breast, and skin [3,4,5,6]. About 50 cases of soft tissue locations of this tumor, both benign and malignant, have been described most often located in deep subcutaneous,
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