Metastatic malignant soft tissue myoepithelioma: a case report showing complete response after locoregional and systemic therapy

Abstract

We report on the case of a 61-year-old man with a soft tissue malignant myoepithelioma of the second toe of the right foot. After removal of the primary tumor, the patient developed in-transit metastases of the limb that we later treated with limb perfusion, using extracorporeal circulation with complete response. Following the appearance of lymph node metastases, the patient underwent inguinal, iliac and obturator lymphadenectomy. Subsequent pelvis metastases were treated with chemotherapy and radiotherapy, with complete response. Currently, after 3 years, the patient is alive and no evidence of any residual disease is apparent.

Myoepithelial carcinoma of the extremity: Response to isolated limb infusion

“…Previously, the use of HILP in treating MEC was reported in a 61‐year‐old male patient . The patient presented with a well‐differentiated MEC on the plantar surface of the second toe of the right foot which metastasized to the third toe of the right foot and back of right foot 3 months after presentation.…”

“…A subsequent pelvic recurrence was treated with systemic chemotherapy and radiation and he had a complete response. The patient has remained well for 3 years [3].…”

“…Studies on age and sex distribution of MEC report inconsistent results. The treatment of choice is complete surgical excision with negative margins, but a local recurrence rate of ∼30% suggests additional means of treatment need to be explored .…”

“…The tumors can show a wide range of morphologic and cytologic diversity with four major types of cells: spindle, hyaline, epithelioid, and clear cell . Local recurrence, lymph node metastasis, and distant metastasis are common, occurring in ∼30, ∼12, and ∼15%, respectively . Treatment recommendations and prognostic information are difficult to find in the literature, most reports contain few cases or incomplete information.…”

“…Treatment recommendations and prognostic information are difficult to find in the literature, most reports contain few cases or incomplete information. Treatment usually consists of surgical excision with negative margins, when feasible . Additional strategies are required, however, for patients who present with locally advanced extremity disease.…”