Primary Melanosarcoma of the Esophagus

Primary Malignant Melanoma of Esophagus

“…Some pathologists still consider all melanomas of the esophagus as metastatic but several facts support the finding that malignant melanoma can arise primarily in the esophagus. This is confirmed as Burnett and St. John19 reviewed 22,000 autopsies at Johns Hopkins, Baltimore, MD, USA and found 25 malignant melanomas at various sites, but they did not find a single metastasis or a primary in the esophagus. Mofatt et al14 found that the literature contained only two cases of melanoma metastasizing to the esophagus and De La Pava et al3 demonstrated the presence of melanoblasts and granules in 4% of the population, establishing the presence of cells from which a primary tumor might develop.…”

The Thousand Faces of Malignant Melanoma: A Systematic Review of the Primary Malignant Melanoma of the Esophagus

“…Although a rare entity and considered anecdotal for a long time, PMME has been studied and described over the decades, with different levels of description ranging from single case reports of “pigmented” lesions in correspondence with the esophageal mucosa, up to actual studies of clinicopathological and molecular characterization of this primary esophageal neoplasm [ 4 , 5 , 6 ]. In 1952, Garfinkle J. M. et al reported the first case with histological correlation described in the literature.…”

“…Although PMME is a rare subtype of esophageal cancer, it has been relatively well reviewed. PMME is a highly aggressive tumor that is associated with a poor prognosis, with a 5-year overall survival (OS) rate of <5% [ 5 , 6 ]. The debate on its genesis starting from the esophagus has been quite heated since it was not believed at the time of the first descriptions that there could be melanocyte cells at the esophageal mucosal level [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 ], and only with the first autoptic series did it begin to prove in a clear way that melanoma could also originate from this part of the body [ 20 ].…”

“…PMME is a highly aggressive tumor that is associated with a poor prognosis, with a 5-year overall survival (OS) rate of <5% [ 5 , 6 ]. The debate on its genesis starting from the esophagus has been quite heated since it was not believed at the time of the first descriptions that there could be melanocyte cells at the esophageal mucosal level [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 ], and only with the first autoptic series did it begin to prove in a clear way that melanoma could also originate from this part of the body [ 20 ]. From a topographical point of view, 90% of PMME cases involve the middle/distal third of the esophagus, which is usually solitary, but in about 13% of cases it presents with multiple lesions.…”

Primary Malignant Melanoma of the Esophagus

“…Those 9 cases reported between 1906 and 1951 do not fulfill the criteria of Allen and Spitz, and thus cast doubt on the primary nature of the tumor [2,5,6,14,22,24,[32][33][34]. Eight other cases published between 1952 and 1962 have some unclear nosographie interpretation because of scar city of documentation in favor of a primary tumor [4,6,11,13,16,26,29,33], Therefore a positive diagnosis is only possible in 104 cases and in particular in the report of Di Costanzo and Urmacher [10] and in our report, where immunohistochemical techniques added addi tional confirmational data (S-100 protein).…”