Dai Yili, Zhang Yiyin, Chen Yongle, Fan Xiaoxiao, Lin Hui, Pan Junhai
Department of General Surgery, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China.
Zhejiang Engineering Research Center of Cognitive Healthcare, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China.
AME Case Rep. 2022 Jul 25;6:22. doi: 10.21037/acr-21-83. eCollection 2022.
Melanoma is a sinister malignant tumor originates from melanocytes and is characterized by the presence of black pigmentation in the tissue. The vast majority of melanomas are cutaneous melanomas, and primary mucosal melanomas originating from the esophagus are extremely rare. Primary malignant melanoma of esophagus (PMME) accounts for 0.1% to 0.2% of all primary esophageal malignancies. PMME possess high invasiveness but are insensitive to various treatments, so the prognosis is disappointing. Most literature reported that patients are prone to death from complications of tumor metastasis soon, even they undergo radical surgery.
In this case report, we admitted a 67-year-old female patient with recurrent chest tightness for 2 years and chest pain for 15 days on October 4, 2017. Preoperative imaging examinations, including computerized tomography (CT) and upper gastrointestinal examination by barium revealed stenosis of the lower esophagus and the fundus of the stomach, with mucosa destruction and lymph node metastasis in the hepatic-gastric space. A laparoscope assisted total gastrectomy with D2 lymph node resection and Roux-en-Y anastomosis was performed without adjuvant immunotherapy or targeted therapies. Postoperative pathological examination and immunohistochemical staining indicated malignant melanoma. Meanwhile we did not find a cutaneous lesion, this patient was therefore diagnosed with a rare PMME. There was no sign of recurrence or metastasis during the latest follow-up of 36 months after the operation, which also exceeded the median recurrence-free survival time in the existing cases worldwide.
Therefore, we recommend early radical surgery, which may be beneficial to the PMME patient.
黑色素瘤是一种起源于黑素细胞的恶性肿瘤，其组织学特征为存在黑色色素沉着。绝大多数黑色素瘤为皮肤黑色素瘤，而起源于食管的原发性黏膜黑色素瘤极为罕见。原发性食管恶性黑色素瘤（PMME）占所有原发性食管恶性肿瘤的0.1%至0.2%。PMME具有高侵袭性，但对各种治疗不敏感，因此预后令人失望。大多数文献报道，即使患者接受了根治性手术，也容易很快死于肿瘤转移的并发症。
在本病例报告中，我们于2017年10月4日收治了一名67岁女性患者，该患者有2年反复胸闷病史及15天胸痛病史。术前影像学检查，包括计算机断层扫描（CT）和上消化道钡餐检查，显示食管下段和胃底狭窄，伴有黏膜破坏及肝胃间隙淋巴结转移。行腹腔镜辅助全胃切除术，D2淋巴结清扫及Roux-en-Y吻合术，未行辅助免疫治疗或靶向治疗。术后病理检查及免疫组化染色提示为恶性黑色素瘤。同时未发现皮肤病变，因此该患者被诊断为罕见的PMME。术后36个月的最新随访期间未发现复发或转移迹象，这也超过了全球现有病例的无复发生存时间中位数。
因此，我们建议早期行根治性手术，这可能对PMME患者有益。
