Myoepithelial carcinoma of the extremity: Response to isolated limb infusion

Abstract

We report our experience with two patients with myoepithelial carcinoma (MEC) of the extremity. An 83-year-old male and a 35-year-old female were treated with standard isolated limb infusion (ILI), using melphalan and dactinomycin. The first patient had a complete response (CR) that was sustained for two and a half years, until he developed a regional lymph node metastasis. The second patient had a 4.7 cm tumor located on her left hand with metastasis to the ipsilateral axillary lymph nodes. Initial treatment consisted of ILI and left axillary lymphadenectomy. The primary tumor regressed and showed signs of central necrosis, measuring 2.8 cm after 1 month. Though she was continuing to respond, a decision was made by her orthopedic surgeon to administer neoadjuvant radiation followed by surgical resection.

BRAF/MEK inhibitor-induced remission of primary cutaneous myoepithelial carcinoma after local recurrence

“… 2 Postsurgical local recurrence and metastases have been reported in up to 30% and 15% of cases, respectively; however, the true metastatic and recurrence potential is difficult to estimate given the rarity of this tumor. 1 Although surgical removal with clear margins is the treatment of choice, this can be impractical in some cases, such as for the patient described here.…”

“… 1 Cutaneous MEC is an even rarer entity that has been most commonly reported on the head and neck and the lower extremities. 1 , 2 , 3 , 4 Reported cases suggest that MEC has a slight female predominance with a bimodal age distribution (before adulthood or after the fifth to sixth decades). 2 , 3 , 4 …”

“… 2 In tumor form, myoepithelial cells show several morphologies, including spindled, hyaline, epithelioid, and clear cell, and their proliferation can lead to either benign myoepitheliomas or malignant MEC. 1 , 2 , 3…”

“…Cutaneous myoepithelial carcinoma (MEC), also referred to as malignant myoepithelial tumor of the skin, is an extraordinarily rare diagnosis, with fewer than 15 cases reported in the scientific literature. 1 , 2 , 3 , 4 These tumors are aggressive, and surgical excision with clear margins is the treatment of choice. 2 , 3 However, postsurgical local recurrence and metastases occur in up to 30% and 15% of cases, respectively.…”

“… 2 , 3 However, postsurgical local recurrence and metastases occur in up to 30% and 15% of cases, respectively. 1 Additional therapeutic options are limited; traditional chemotherapy and/or radiation have been described with variable and often disappointing results. 2 Novel treatment modalities are needed for patients who develop local or metastatic disease.…”