Primary melanoma of the duodenum is an extremely rare, aggressive and life-threatening malignant neoplasm. Published data regarding the effectiveness of current treatment strategies is limited, and our knowledge relies mostly on sporadic case reports. The diagnosis of primary duodenal melanoma is challenging and is based on the patient's medical history and findings from physical examination and radiological and endoscopic imaging as well as proper and careful pathological examinations of the tumor. Despite the many advances in cancer treatment, the prognosis for patients with this type of melanoma remains extremely poor. Delayed diagnosis at advanced disease stage, the general aggressive behavior of this neoplasm, the technical difficulty in achieving complete surgical resection, along with the rich vascular and lymphatic drainage of the intestinal mucosa, all have a negative impact on patients' outcome. In the present review, we aimed to collect and summarize the currently available data in the literature regarding the pathogenesis, clinical features, diagnosis, management and long-term outcomes of this rare, malignant tumor, in order to expand knowledge of its biological behavior and investigate optimal therapeutic options for these patients. Additionally, we present our experience of a case involving a 73-year-old female with primary duodenal melanoma, who was successfully treated with complete surgical resection.

2nd Department of Pathology ATTIKON General University Hospital Medical School National and Kapodistrian University of Athens Athens Greece

2nd Department of Pediatrics P and A Kyriakou Children's Hospital Medical School National and Kapodistrian University of Athens Athens Greece

3rd Department of Surgery ATTIKON General University Hospital Medical School National and Kapodistrian University of Athens Athens Greece

Department of Gastroenterology General Hospital Hippokration Athens Greece

Department of Paediatric Surgery ATTIKON General University Hospital Medical School National and Kapodistrian University of Athens Athens Greece

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