• Yu-Ming Chu, Chih-Sheng Hung, and Ching-Shui Huang.
• Division of Digestive Medicine, Department of Internal Medicine, Cathay General Hospital.
• Medicine (Baltimore). 2021 Jun 25; 100 (25): e26467e26467.

RationaleMost gastrointestinal melanomas are metastatic from an oculocutaneous primary lesion; however, primary gastrointestinal melanomas have been found in all levels of the gastrointestinal tract. We present the case of Primary malignant melanoma of the esophagus and discuss the diagnostic methods, differentiation from metastatic lesions and treatment options.Patient ConcernsA 78-year-old male patient presented with fresh blood vomiting and tarry stools for 1 day.DiagnosesEsophagogastroduodenoscopy of this patient revealed a tumor ∼4 cm in size at the cardia side of the esophagogastric junction with dark-red and gray pigmentation. Immunohistochemical stains of the biopsy specimens were positive for S-100 and HMB-45, which are specific markers of melanoma.InterventionsLaparotomy with proximal gastrectomy was performed by the surgeon. Histological examination of the surgical specimen revealed the tumor arose from the distal esophagus with invasion of the proximal stomach. Primary malignant melanoma of the esophagus was diagnosed after a full skin and ophthalmic examination and positron emission tomography, which revealed no lesions elsewhere in the body.OutcomesNo tumor recurrence was noted at the 1-year follow-up.LessonsPrimary malignant melanoma of the esophagus is an extremely rare but highly aggressive tumor. The special pattern of pigmentation should be recognized while performing endoscopy. Early detection and radical resection of the tumor are critical to ensure favorable outcomes.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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