Primary Malignant Melanoma of the Esophagus
An 89-year-old female was admitted to hospital with progressively worsening dysphagia for solids during the previous month. Physical examination revealed cachexia and paleness. Apart from a mild normocytic anemia, blood tests were within the normal range. Her medical history included arrhythmia and coronary artery stent placement within the past 7 years. Upper gastrointestinal endoscopy revealed a polypoid ulcerated tumor with purple pigmentation and vascular appearance protruding into the esophageal lumen, located 27–35 cm from the incisors (fig. ). Due to its vascular appearance, endoscopic biopsy was not initially performed; however, endoscopic ultrasonography (EUS) demonstrated an iso-hypoechoic, heterogeneous solid tumor mass, including cystic-anechoic areas 5 cm in diameter, in the esophageal wall. Doppler ultrasonography showed that the lesion was not a vascular mass but contained a few intratumoral vascular flows towards the periphery of the lesion. EUS demonstrated that the tumor had not penetrated the adventitia of the esophageal wall. Although the tumor was adjacent to large vessels and the left atrium, tumor features were not found in the vessel, and the interface echo was preserved between the tumor and the vessels (fig. ). At the time of EUS, multiple endoscopic forceps biopsies were obtained. Diagnosis was based on histologic and immunohistochemical examinations. Histologically, there was infiltration of atypical, pleomorphic fusiform cell in the submucosa. Some of the samples had an ulcerated surface, while in others hyperplasia in the squamous mucosa was noted but intraepithelial spread was not seen. Upon immunohistochemical examination, positive diffuse S-100, melan-A and human melanoma black (HMB-45) reactions were detected as melanoma determinants. The samples were also positive for CD117, a prognostic marker of malignant melanoma (fig. ). Thoracic and abdominal computed tomography showed no metastatic disease. Because of her debilitated condition and comorbidities, radical surgery was not considered. A metallic esophageal stent was placed to facilitate feeding.\nDuring the first month, dysphagia was partially relieved. However, after 1.5 months of progressively worsening dysphagia and repetitive hematemesis and blood–tinged vomitus, upper gastrointestinal endoscopy was performed, revealing stent occlusion as a result of tumor overgrowth. The tumor was fragile and bled easily. There was no hemodynamic instability or severe anemia related to repetitive bleeding, and barium contrast did not pass beyond the lesion. A percutaneous endoscopic gastrostomy tube was placed surgically for nutritional support. The patient remains under follow-up care in our department 6 months after the primary diagnosis.