State Key Laboratory of Oncology in South China, Department of Thoracic Surgery, Cancer Center, Sun Yat-Sen University, Guangzhou, China.
Dis Esophagus. 2011 Feb;24(2):109-13. doi: 10.1111/j.1442-2050.2010.01111.x. Epub 2010 Oct 11.
Primary malignant melanoma of the esophagus (PMME) is a rare disease that is characterized by aggressive invasion, early metastasis, and poor prognosis. Treatment protocols are not well-established. To understand this condition more precisely, we performed a retrospective review of eight cases of PMME diagnosed at the Thoracic Department of the Cancer Center at Sun Yat-Sen University between 1985 and 2009. Eight PMME patients (five men and three women) with a mean age of 58 years (range: 48 to 72 years) were included. Dysphagia was the most common presenting symptom. All patients underwent an Ivor-Lewis esophagogastrectomy and lymph node dissection with postoperative adjuvant chemotherapy. One patient with stage III/pT4N0M0 underwent postoperative chemotherapy plus radiotherapy. Four patients died of distant metastases. The median survival time was 28 months (range: 11 months to 6 years). Our data confirm that PMME is a highly aggressive disease with a poor prognosis. If the diagnosis is suspected or confirmed as PMME, and the patients have no distal metastases or extensive lymph node enlargement, we suggest that surgery should be the first choice of treatment. With regard to adjuvant therapy, we recommend the addition of chemotherapy. The role of radiotherapy remains questionable and requires further investigation.
食管原发性恶性黑色素瘤（PMME）是一种罕见疾病，其特征为侵袭性强、早期转移和预后差。目前尚未建立完善的治疗方案。为了更准确地了解这种疾病，我们对中山大学肿瘤防治中心胸科 1985 年至 2009 年期间诊断的 8 例 PMME 患者进行了回顾性研究。8 例 PMME 患者（男 5 例，女 3 例），平均年龄 58 岁（范围：48~72 岁）。最常见的临床表现为吞咽困难。所有患者均接受了 Ivor-Lewis 食管胃切除术和淋巴结清扫术，术后辅助化疗。1 例 III 期/pT4N0M0 患者接受了术后化疗加放疗。4 例患者死于远处转移。中位生存时间为 28 个月（范围：11 个月至 6 年）。我们的数据证实 PMME 是一种侵袭性强、预后差的疾病。如果怀疑或确诊为 PMME，且患者无远处转移或广泛淋巴结肿大，我们建议手术应作为首选治疗方法。关于辅助治疗，我们建议加用化疗。放疗的作用仍存在疑问，需要进一步研究。
