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巨大原发性食管恶性黑色素瘤:一例报告及文献复习

Huge primary malignant melanoma of the esophagus: A case report and literature review.

作者信息

Zhang Lin, Ma Wei, Li Yun

机构信息

Department of Thoracic Surgery, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China.

出版信息

Thorac Cancer. 2013 Nov;4(4):479-483. doi: 10.1111/1759-7714.12063.

Abstract

Primary malignant melanoma of the esophagus (PMME) is a relatively rare esophageal malignancy accounting for 0.1-0.2% of all esophageal tumors. Because the tumor cells occur mainly in the esophagus mucosa and grow along the longitudinal axis in the lumen, it causes relatively late obstruction symptoms, even though the lesion is a much larger mass than found in most cases of esophagus carcinoma, protruding into the esophageal lumen. Invasive growth results in stenosis, followed by difficulty eating. A patient may not be aware of their illness until transesophageal endoscopic biopsy or postoperative pathology is confirmed. Positive expression of immunohistochemical markers human melanoma black (HMB) 45 and S-100, are the most important factors in confirming diagnosis. Because of the high degree of malignancy and the poor prognosis of PMME, upon diagnosis total or subtotal esophagectomy plus three-field systemic lymph node dissection with incisions in the left cervical, right chest, and superior belly, is critically required. In summary, early diagnosis and expanding esophageal resection may be the best approach to prevent local recurrence and distant metastasis.

摘要

原发性食管恶性黑色素瘤(PMME)是一种相对罕见的食管恶性肿瘤,占所有食管肿瘤的0.1 - 0.2%。由于肿瘤细胞主要发生在食管黏膜,并沿管腔内的纵轴生长,尽管病变肿块比大多数食管癌病例中发现的要大得多,突出到食管腔内,但它引起梗阻症状相对较晚。浸润性生长导致狭窄,进而引起进食困难。患者可能直到经食管内镜活检或术后病理确诊才意识到自己患病。免疫组化标志物人黑色素瘤黑色(HMB)45和S - 100的阳性表达是确诊的最重要因素。由于PMME恶性程度高且预后差,一旦确诊,急需进行全食管或次全食管切除术加三野系统性淋巴结清扫术,切口位于左颈部、右胸部和上腹部。总之,早期诊断和扩大食管切除术可能是预防局部复发和远处转移的最佳方法。

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