Mizuta Haruki, Takahashi Akira, Namikawa Kenjiro, Ogata Dai, Yamazaki Naoya
Department of Dermatologic Oncology, National Cancer Center Hospital, Tokyo, Japan.
Department of Plastic and Reconstructive Surgery, Graduate School of Medicine Osaka City University, Osaka, Japan.
Dermatol Ther. 2020 Jul;33(4):e13485. doi: 10.1111/dth.13485. Epub 2020 Jun 2.
Myoepithelial carcinoma is a rare tumor that occurs in the salivary glands. Therefore, primary cutaneous myoepithelial carcinoma is extremely rare. The malignancy of this tumor is due to its aggressive nature and high metastatic potential. Despite the general consideration that primary cutaneous myoepithelial carcinoma has a poor prognosis, there are few available prognostic data. Herein, we report two cases of primary cutaneous myoepithelial carcinoma to expand the literature on the clinical and pathological characteristics of this disease. In case 1, the tumor was completely resected and sentinel lymph node biopsy was performed, resulting in long-term survival. In contrast, case 2 progressed aggressively with multiple bone metastases despite treatment with docetaxel chemotherapy, and the patient ultimately died of the disease. Only 14 cases, including the present two cases, of primary cutaneous myoepithelial carcinoma have been reported to date. Overall, patients with clinical regional or distant metastases show a severe clinical course. Nevertheless, even in cases of inappropriate resection or local recurrence, if complete resection is ultimately achieved prior to clinical metastasis, the prognosis is good and survival is prolonged. Therefore, obtaining complete resection appears to be the most important treatment strategy for primary cutaneous myoepithelial carcinoma.
肌上皮癌是一种发生于唾液腺的罕见肿瘤。因此，原发性皮肤肌上皮癌极为罕见。该肿瘤的恶性程度源于其侵袭性和高转移潜能。尽管一般认为原发性皮肤肌上皮癌预后较差，但可用的预后数据很少。在此，我们报告两例原发性皮肤肌上皮癌病例，以扩充关于该疾病临床和病理特征的文献。病例1中，肿瘤被完全切除并进行了前哨淋巴结活检，患者长期存活。相比之下，病例2尽管接受了多西他赛化疗，仍发生多处骨转移且病情进展迅速，患者最终死于该疾病。迄今为止，包括本两例在内，仅报道了14例原发性皮肤肌上皮癌病例。总体而言，有临床区域或远处转移的患者临床病程严重。然而，即使在切除不当或局部复发的病例中，如果在临床转移之前最终实现完全切除，预后良好且生存期延长。因此，实现完全切除似乎是原发性皮肤肌上皮癌最重要的治疗策略。
