Primary malignant melanoma of the esophagus successfully treated with camrelizumab: A case report and literature review*

Abstract

Abstract An 83-year-old Chinese woman presented with a 3-month history of dysphagia. She also had a history of hypertension, type 2 diabetes, fundus hemorrhage, and cataract but no history of cutaneous, ocular, or other-site melanomas. Upper gastrointestinal tract angiography revealed gastritis and duodenal diverticulum; thus, an endoscopic review was recommended. Enhanced computed tomography of the chest and upper abdomen revealed the following: (1) Esophageal space-occupying lesions and mediastinal lymph node enlargement (considering the high possibility of esophageal cancer, further endoscopy was recommended) and (2) A small amount of right pleural effusion, with no significant lymph node infiltration or distant metastasis. Esophagoscopy identified a bulge mass blocking the esophagus from 23 to 30 cm from the incisors. The upper mass had a spherical clustering, while the lower mass significantly festered. Pathological biopsy samples were obtained from the esophagus 23 and 28 cm from the incisors. Tissue biopsy showed proliferation of large round tumor cells and melanocytes. Immunohistochemistry showed positive findings for HMB45 and MelanA; partially positive findings for S100, CK7, CK5/6, CAM5.2, LCA, P63, and TTF-1; and negative findings for Syn. The Ki-67 positivity index was approximately 60%. Based on these findings, the patient was diagnosed with malignant esophageal melanoma with enlarged mediastinal lymph nodes. She was then treated with five cycles of camrelizumab therapy combined with chemotherapy from October 18, 2019, to May 5, 2020. Gastroscopy review following two courses of combination therapy revealed that the esophagus was 23-25 cm away from the incisors, and there were two continuous uplifted and beaded masses that had a smooth and black surface, with each of them having a length and diameter of approximately 1 cm. Melanosis of the mucosa around the lumen was observed at 40 cm from the incisors to the cardia; the dentate margin was clear; and the cardia had no stenosis. The patient then received five courses of combination therapy and became consistently stable after partial remission. No severe adverse events related to the immunotherapy were recorded. Camrelizumab may be a viable treatment option for patients with PMME. Additional evidence from future clinical trials and research is necessary to fully validate our findings.