Advanced primary amelanotic malignant melanoma of the esophagus: A case report

Abstract

BACKGROUNDPrimary malignant melanoma of the esophagus accounts for 0.1%-0.2% of all esophageal malignancies, including melanotic and amelanotic melanomas. Primary amelanotic malignant melanoma of the esophagus is extremely rare, and only about 20 cases have been published in the literature to date. Most primary malignant melanomas of the esophagus are diagnosed following development of metastatic lesions and thus have a very poor prognosis. The median survival duration of patients with metastatic melanoma has been reported to be 6.2 mo.CASE SUMMARYA 49-year-old woman was referred to our hospital with a diagnosis of esophageal cancer. Endoscopy, biopsy, imaging evaluation, and physical examination at our hospital indicated a diagnosis of advanced primary amelanotic malignant melanoma of the esophagus. Immunohistochemical staining confirmed melanoma. Nuclear medicine examination revealed a left iliac bone metastatic lesion. After discharge, the patient self-administered apatinib for 3 mo, followed by oral treatment with Chinese medicines (also self-administered) for 2 mo. No treatments had been taken since then. The patient has survived with no growth out to the most recent follow-up (24 mo post diagnosis), and she always presented with a positive attitude about her condition during this period.CONCLUSIONSurvival following metastatic melanoma might be related to the pharmaceutical and Chinese medicine treatment and the patient's positive attitude.

A Rare Case of Primary Malignant Melanoma of the Esophagus Presenting as Dysphagia

“…9 While a black pigmented lesion on EGD for our patient was consistent with a diagnostic criterion outlined by Allen and Spitz (1953) for suspected melanoma, evidence shows that up to 10%-25% of PMME cases are amelanotic and colorless. 10,11 Therefore, immunohistochemical staining techniques and genetic analysis have become critical to the accurate diagnosis and prognostication of PMME.…”

“…While demonstration of melanin in tumor and adjacent tissue would be helpful, this would miss out on the significant number of malignant melanomas that are amelanocytic. 11 It is therefore imperative for providers to conduct a thorough physical examination to ensure that there are no other melanoma lesions on skin and other mucosal surfaces. Thus, a diagnosis of PMME in our patient was made due to a lack of physical evidence of other potential primary lesions, a positive MART-1 antigen and negative cytokeratin antigen on immunohistochemical testing.…”

“…The treating physician elected combination immunotherapy (nivolumab/relatlimab), based on available evidence of progression-free survival of 15.6 months with immunotherapy compared with 3 months for chemotherapy only. 11 …”

Primary malignant melanoma of the esophagus combined with squamous cell carcinoma: A case report

“…Primary malignant melanoma of the esophagus (PMME) is a rare disease that accounts for 0.5% of all nonskin melanomas[ 2 ]. Primary melanin-free melanoma is also extremely rare, with slightly more than 20 cases reported thus far[ 3 ], and combined esophageal squamous cell carcinoma is even rarer.…”

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

“…It is important to underline that among the PMMEs, a rate of between 15 and 25% is present as amelanotic lesions [ 83 , 106 , 171 , 172 , 174 , 178 , 194 , 266 , 267 , 268 , 269 , 270 , 271 , 272 , 273 , 274 , 275 ]. Kobayashi et al, among other authors, reported a case of amelanotic malignant melanoma of the esophagus in a 68-year-old man previously operated on for the detection of gastric carcinoma.…”