Wollina Uwe, Hansel Gesina, Zimmermann Felicitas, Schönlebe Jacqueline, Nowak Andreas
Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Friedrichstrasse 41, 01067, Dresden, Germany,
Wien Klin Wochenschr. 2015 May;127(9-10):402-5. doi: 10.1007/s00508-014-0660-z. Epub 2015 May 6.
Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin. Although its association with other malignancies is well known, an association with gastrointestinal stromal tumor (GIST) has yet not been described.We report about a 65-year-old female patient who presented with a hypervascularized subcutaneous tumor mass of her left calf. Resection of the primary tumor and histopathological investigations confirmed the diagnosis of MCC. The patient was treated by delayed Mohs surgery, and tumor-free margins were obtained. Sentinel lymph node biopsy was negative for metastatic spread. Primary tumor and lymph node basin were treated by adjuvant radiotherapy. During staging of the patient, a second malignancy-a GIST-was detected. Neoadjuvant treatment with multikinase inhibitor imatinib induced a partial response of GIST that was eventually removed by surgery. However, 8 months later, the patient developed subcutaneous regional metastases of MCC, which were surgically removed. Adjuvant therapy was planned by oncologists.To the best of our knowledge, the occurrence of MCC and GIST in the same patient has yet not been reported. In contrast to GIST, MCC did not respond to imatinib, although c-kit mutations are common in MCC.
默克尔细胞癌（MCC）是一种罕见的皮肤神经内分泌肿瘤。尽管其与其他恶性肿瘤的关联已为人熟知，但与胃肠道间质瘤（GIST）的关联尚未见报道。我们报告了一名65岁女性患者，其左小腿出现一个血管丰富的皮下肿瘤肿块。原发性肿瘤切除及组织病理学检查确诊为MCC。患者接受了延迟的莫氏手术，切缘无肿瘤。前哨淋巴结活检未见转移扩散。原发性肿瘤和淋巴结区域接受了辅助放疗。在患者分期过程中，发现了第二种恶性肿瘤——GIST。用多激酶抑制剂伊马替尼进行新辅助治疗使GIST出现部分缓解，最终通过手术切除。然而，8个月后，患者出现了MCC的皮下区域转移，予以手术切除。肿瘤学家计划进行辅助治疗。据我们所知，同一患者同时发生MCC和GIST的情况尚未见报道。与GIST不同，尽管c-kit突变在MCC中很常见，但MCC对伊马替尼无反应。
