PRIMARY MALIGNANT ESOPHAGEAL MELANOMA: CASE REPORT

Introduction: Primary malignant esophageal melanoma (MMPE) is a rare neoplasm and by 2011, less than 340 cases had been reported in the literature. This melanoma is characterized by aggressiveness, with multiple and early metastases. Radical esophagectomy with lymphadenectomy is the treatment of choice. Objectives: To discuss the clinical presentation, diagnosis and therapeutic management of primary esophageal melanoma. Methodology: Case report of a patient diagnosed with MMPE. Case Report: M.D.B., 79 years old. Complaint of dysphagia. Physical examination: no changes. EDA showing vegetative lesion, multilobulated, 32cm from the ADS, reaching the luminal space, measuring 2.0cm. Anatomopathological evidence of malignant neoplasia of epithelioid cells. Staging showed perigastric lymph node enlargement, and total esophagectomy plus lymphadenectomy was indicated. At biopsy, multinodular growth melanoma with focally junctional activity was detected, maximum invasion thickness of 9 mm, mitotic index equal to 5 mitoses / mm³ without angiolymphatic and perineural invasion and IHC: revealed negative AE1 / AE3 and CD45 RO cytokines and Melan -A, HMB-45, SOX10 and Protein S100 positive. In addition, metastases were identified in one periesophageal and one celiac lymph node. Discussion and results: In 1906, Baur described the first case of MMPE. The clinical picture in more than 70% of patients denotes complaints of dysphagia and late onset epigastric pain. The diagnostic criteria for MMPE are: 1) typical histological pattern of melanoma, with melanin granules within the tumor cells and 2) origin in an area of junctional activity in the squamous epithelium. The association of these criteria with the presence of melanoma in situ without previous history of cutaneous melanoma leads to the absolute diagnosis of MMPE. Treatment should be individualized and, when possible, surgery is the preferred method, with radical resection with wide margins being recommended. As for prognosis, the average survival after surgical treatment is less than 15 months and about 85% of patients with MMPE die from disseminated disease. Conclusion: Therefore, MMPE is a rare neoplasm derived from esophageal melanocytes and usually has a late diagnosis and poor prognosis. We report the case of a 79-year-old female patient diagnosed with MMPE who underwent total esophagectomy with lymphadenectomy, with metastases in perigastric and celiac lymph nodes. Patient in clinical follow-up.

Publication History

Article published online:
23 October 2019