Various clinical scenarios of primary melanoma of the esophagus: A retrospective 20-year analysis from two university thoracic surgery centers

Abstract

Background. Primary melanoma of the esophagus (PME) represents a rare type of gastrointestinal malignancy with an exceptionally poor diagnosis. So far, only few descriptions of PME which satisfactorily summarize their clinical characteristics and prognosis have been published.Objectives. The aim of our study was to summarize our experience with PME patients. Materials and methods.In a group of 1387 patients who underwent esophagectomy due to neoplastic process in the years 2000-2020 in 2 high-volume university thoracic surgery centers, we identified those with confirmed PME diagnosis. Subsequently, their clinical characteristics, imaging and histopathological results were compared. The data regarding the long-term survival were obtained from the Polish National Death Registry.Results. The PME was identified in 4 (0.29%) patients. Three of them (75%) were males. The mean age on admission was 64.3 ±17.5 years. The main symptom in all patients was dysphagia. In 1 patient with the most advanced PME, the clinically relevant weight loss was noted. In 3 patients, Ivor Lewis esophagectomy was performed, and 1 patient underwent McKeown resection. Histopathologic examination revealed a metastasis of lymph nodes only in 1 patient. The average maximum size of tumor was 6.9 ±4.7 cm and all tumors were located in distal part of the esophagus. Two out of those 4 patients are still alive and the longest survival time is 17 years. One patient died due to postoperative massive gastrointestinal bleeding complicated with cardiac arrest and the other one due to progression of PME systemic dissemination 6 months after surgical treatment. Conclusions.The PME is an extremely rare diagnosis. A long-term survival can be achieved with the complete resection. Clinical scenarios of surgically treated PME patients may significantly differ.

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

“…Although Nivolumab has been shown to be less effective in mucosal melanomas than in cutaneous melanomas, the standard treatment and efficacy of ICI in PMME remain unclear due to its rarity [ 275 , 288 , 289 , 290 , 291 , 292 , 293 , 296 , 297 , 298 , 299 , 300 , 301 , 302 , 303 , 304 ]. As we have analyzed, some studies have revealed promising results, but there is a need for randomized controlled trials with larger series of PMME patients [ 186 , 218 , 275 , 305 ].…”

“…In recent years, the greater diffusion of molecular biology techniques such as next generation sequence (NGS) and parallel massive sequencing have made it possible to deepen the knowledge related to PMME by analyzing the different mutations responsible for this entity [ 275 , 289 , 291 , 292 , 293 , 296 , 297 , 298 , 299 , 300 , 301 , 302 , 303 ]. More specifically, in a very recent work, Tsuyama et al studied 13 cases of PMME and 10 cases of malignant skin melanoma (SKMM) with NGS and immunohistochemistry.…”