Primary malignant melanoma originating in the digestive tract is particularly rare, mainly affecting the anorectum and oral cavity. Primary malignant melanoma of the esophagus has been the subject mostly of case reports. This tumor has a dismal prognosis with a frequency estimated to be approximately 0.1% to 0.2% of all esophageal malignancies. According to the review by Volpin et al of November 2002, 238 cases of primary malignant melanoma of the esophagus have been published up to early 2001. We present an additional case of primary malignant melanoma of the esophagus of the amelanotic variant in a 69-year-old man. The patient was preoperatively investigated by esophageal endoscopy and endoscopic ultrasound. The surgically resected tumor specimen was examined histologically and supplemented by immunohistochemical and ultrastructural analysis. Intra-abdominal relapse occurred after 8 months at the site of surgery, necessitating repeat resection. The patient died of advanced intra-abdominal disease 14 months after the primary diagnosis. A comprehensive computerized (PubMed/Medline) review of the world literature was also carried out and 99 additional cases (after the review by Volpin et al) were found, 9 of them from the 1990s which escaped previous tabulations, and 90 from the years 2000 to 2010, amounting to a grand total of 337 ever published.
Departments of *Pathology, Division of Anatomic Pathology
†Medical Sciences, Division of Gastroenterology
§Surgical Sciences, Division of Abdominal Surgery, IRCCS “Casa Sollievo della Sofferenza” Hospital, San Giovanni Rotondo
‡Etromapmax Pole, Biomedical Sciences School, Lesina (FG)
∥Department of Specialistic Medicine, Clinical Dermatology, Ospedali Riuniti di Foggia, Foggia
‡‡Unit of Anatomic Pathology, IRCCS-CROB, Referral Cancer Center of Basilicata, Rionero in Vulture (PZ), Italy
¶Department of Hematology, Oncology, and Clinical Pathology, Division of Surgical Pathology, Policlinico “S. Orsola” and University of Bologna, Bologna, Italy
Reprints: Michele Bisceglia, MD, Department of Pathology, Division of Anatomic Pathology, IRCSS “Casa Sollievo della Sofferenza” Hospital, V.le Cappuccini, 71013 San Giovanni Rotondo), Italy (e-mail: firstname.lastname@example.org).
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