Esophageal Melanocytosis.

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Abstract
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Esophageal melanocytosis is a rare entity defined by the proliferation of a melanocytic basal layer of the esophageal squamous lining and deposition of melanin in the esophageal mucosa. Esophageal melanocytosis is considered a benign entity of unknown etiology; however, it has been reported as a melanoma precursor. We report a case of esophageal melanocytosis in a diabetic and hypertensive 67-year-old male with recurrent dizziness and syncope for the past 6 months. Given his complaint of dyspepsia, he underwent an upper gastrointestinal endoscopy, in which an esophageal biopsy revealed the diagnosis of esophageal melanocytosis. The definitive diagnosis of esophageal melanocytosis can only be made by histological analysis. The histologic differential diagnoses include melanocytic nevi and malignant melanoma. Therefore, they need to be ruled out.

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Esophageal Melanocytosis in African American Male
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Esophageal melanocytosis is usually regarded as a benign finding with potential progression to malignant melanoma, Endoscopic surveillance has been suggested to prevent disease progression. We describe a case report with incidental finding of esophageal melanocytosis during an upper endoscopy. A 64-year-old African American male with a past medical history of GERD, erosive esophagitis and chronic hepatitis C was admitted for workup of anemia. He was noted to have guiac positive stool without any overt symptoms of bleeding. On admission, vital signs were stable and physical examination was unremarkable. Prior endoscopic workup included negative colonoscopy, multiple upper endoscopies for gastric ulcers, erosive esophagitis and portal hypertensive gastropathy. After negative routine blood workup to diagnose anemia, an upper endoscopy was performed. He was noted to have a discolored patch of mucosa in the posterior pharynx extending into epiglottis and proximal esophagus. Gastric and duodenal biopsies showed chronic inflammation. Biopsy from esophagus showed esophageal squamous mucosa with benign melanin staining cells in the basal epithelium. Histological pattern was consistent with benign melanocytosis. Esophageal melanocytosis is a rare, benign condition, with unclear etiology and pathogenesis. There seems to be an association with chronic inflammatory stimuli like acid reflux and heavy alcohol consumption. Some studies report association with conditions like esophageal squamous cell carcinoma, Laugier-Hunziker syndrome, Addison's disease, oral melanoma. 25 to 30% of surgical specimens of primary malignant melanoma showed melanocytosis indicating that it could be a precursor of melanoma. Histologically, esophageal melanocytosis is characterized by proliferation of melanocytes in the basal layer of squamous epithelium with melanin deposition in mucosa. The lesions should be differentiated histologically from blue nevus or malignant melanoma. It commonly found in mid to lower esophagus, but our patient had involvement of proximal esophagus. Esophageal melanocytosis is assumed to be a benign condition without need for surveillance or follow-up with further endoscopies and biopsies. Without definitive evidence for ongoing surveillance, our patient was treated for gastritis/esophagitis with Proton pump inhibitors and discharged home. To date, there is no clear and definitive evidence of progression of Esophageal melanocytosis to melanoma.Figure 1Figure 2Figure 3

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Esophageal Melanocytosis Morphologic Features and Review of the Literature
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Endoscopic or macroscopic esophageal melanocytosis is a benign clinicopathologic entity characterized by melanocytic proliferation in esophageal squamous epithelium and melanin deposition in the mucosa. Little is known about the etiology and natural course of this condition, although it has been suggested to be a precursor of primary esophageal melanoma by some authors. Following a search of the bibliographic databases (PubMed and Medline) regarding esophageal melanocytosis and melanosis, thirty-four cases of isolated esophageal melanocytosis (including one unpublished case from us) were found. The histopathologic features of esophageal melanocytosis are reviewed and its differential diagnosis with other pigmented esophageal lesions is discussed.

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Esophageal melanocytosis (EM) is a rare entity, which is characterized by a non-atypical melanocytic proliferation and melanin deposits in the esophageal mucosa. The confusion between the terms of melanosis and melanocytosis in the literature, the rarity of this lesion (less than 50 cases reported in the literature), its uncertain pathobiological course and the lack of experience of pathologists and gastroenterologists prompt us to draw the attention to this particular entity by reporting two cases and reviewing the literature. Magnifying endoscopy to observe intensive melanin accumulation followed by a biopsy are key for the diagnosis.

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Esophageal melanocytosis is a rare clinical and pathological condition characterized by non-atypical melanocytic proliferation and increased melanin in the esophageal mucosa, which is normally histologically non-melanocytic. Intensive melanin accumulation and hyperpigmentation are necessary for endoscopic recognition. Due to the fact that it is a rare gastrointestinal system pathology, experience and knowledge about its diagnosis, treatment and course are also limited. Although it is argued that chronic stimulating factors have an influence, there is no clear information about its etiology and pathogenesis. Malignant melanomas and melanocytic nevus in particular come to the fore in the differential diagnosis. Opinions and findings indicating that melanocytosis may be a precursor for malignant melanoma make the recognition and follow-up of this clinical and pathological entity more important. In this article, a patient with esophageal melanocytosis diagnosed by endoscopic evaluation is presented, with the aim of increasing the awareness of clinicians, especially endoscopists and pathologists, on this subject.

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A 66-year-old Japanese man was admitted to our hospital, presenting with massive hematemesis. Emergency endoscopy revealed a bleeding tumor at the esophagogastric junction. The endoscopic appearance of the tumor was that of a Borrmann 2-like tumor, with a brownish-black discoloration. Bioptic histology confirmed the diagnosis of malignant melanoma. Atypical melanocytes with junctional changes were also found at a small pigmented patch in the lower esophagus, separate from the gross tumor. Melanocytosis was noted in the adjacent esophageal epithelium in the resection specimen following surgery. No primary lesion was found elsewhere, even in the patient's skin. These pathologic findings support the possibility of multicentric occurrence of malignant melanoma in esophageal melanocytosis. The patient is alive 11 months later, with multiple liver metastases. Massive hematemesis is an unusual presentation of primary malignant melanoma of the esophagus.

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Esophageal Melanocytosis in Oral Opium Consumption
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Esophageal melanocytosis is a rare and benign condition, characterized by melanocytic proliferation of the esophageal squamous epithelium with heavy melanin deposition. The etiology and pathogenesis has not been exactly known but it seems to be a chronic stimulus such as gastroesophageal reflux. This condition is very rare and about 35 cases have been reported so far, most of which have been from India and Japan. Herein, we present a case of esophageal melanocytosis in a patient with long history of oral opium consumption. To the best of our knowledge, such a history has not been reported.

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