Primary Malignant Melanoma of the Esophagus

Abstract

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.

Primary Malignant Melanoma of the Esophagus With Unusual Endoscopic Findings

“…9 In a recently published series of 910 esophageal biopsies in Japan, only 2 were found to be melanoma (0.2%). 10 …”

Primary malignant melanoma of the oesophagus: two case reports

“…This malignancy tends to arise in the sixth and seventh decades with a 2:1 male-to-female preponderance [1]. In contrast to cutaneous melanoma, the oesophageal variant appears to have a higher racial predilection for the Japanese compared with Caucasians [2]. …”

“…Where the histological diagnosis is uncertain, definitive diagnosis can made through immunomarker staining for vimentin, S-100 protein, HMB-45, Melan-A and tyrosinase [2]. …”

“…Wide margins are advised due to the presence of satellite nodules which exist in 12% of cases [1]. Despite radical surgery, however, the prognosis is poor with a mean survival time post-operatively of 10–14 months and a 5-year survival rate of 4.5% [2]. Moreover, 77% of cases will ultimately succumb from recurrent disease within the first post-operative year [5].…”

“…In comparison with the cutaneous variant, mucosal melanoma has poorer outcomes due to late detection and high metastatic potential. The oesophagus is a rare site of origin for primary malignant melanoma with only 337 cases published in the literature [1, 2]. Here, we present two cases of metastatic primary oesophageal melanoma, which highlight the challenging nature in the management of rare but aggressive malignancy.…”