Primary malignant melanoma of the esophagus with multiple lymph node metastases

Abstract

Rationale: Primary malignant melanoma of the esophagus (PMME) is a very rare malignancy accounting for only 0.1% to 0.2% of all malignant esophageal lesions. Presently, there are no standard strategies or clear guidelines for PMME treatment. Patient concerns: Herein, we report a patient who had PMME with multiple lymph node metastases (LNMs) who was treated successfully by esophagectomy. In March 2018, a 74-year-old man with symptoms of continuous dysphagia was referred to our hospital. Diagnosis: Upper gastrointestinal endoscopic examination revealed melanin pigmentation in the middle thoracic esophagus and a pigmented polypoid mass in the lower esophagus. Histopathological examination of the endoscopic biopsy specimen revealed malignant melanoma. Contrast-enhanced computed tomography showed a 3 cm tumor lesion with several enlarged lymph nodes without distant metastasis. The preoperative diagnosis based on the TNM classification was cT2N2M0 stage III. Interventions: The patient underwent esophagectomy with lymph node dissection. Outcomes: Histopathological examination showed that the tumor extended to the submucosal layer of the esophageal wall, with multiple LNMs. Although multiple LNMs were detected, computed tomography scan 15 months after surgery showed no recurrence. Additionally, we analyzed the relationship between the overall survival and the clinicopathological factors including LNMs in 48 previously reported cases of PMME that were surgically treated. Lessons: To our knowledge, this is the first report on the effect of LNMs on the prognosis of PMME patients. The analysis revealed the prognostic value of the TNM stage. Early tumor detection and esophagectomy with lymph node dissection may play as key factors for achieving a better overall survival of PMME patients.

Primary malignant melanoma oesophagus: A rare cause of dysphagia

“…Esophageal melanoma typically affects older people in the 6 th or 7 th decade of life with a maleto-female ratio of 2-3:1 [1]. About 90% of cases involve the middle or lower third of the oesophagus and typically present as dysphagia.…”

“…Primary malignant melanoma is an exceedingly rare neoplasm accounting for about 0.1% to 0.2% of all malignant esophageal neoplasms [1]. It is mostly reported in elderly males, typically present as dysphagia.…”

“…Surgical resection is considered the most accepted modality of treatment in operable cases. Despite the advancements in surgery and chemotherapy, the overall prognosis remains dismal, even in operable cases [1][2][3].…”

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

“…Therefore, these authors renewed the need to employ techniques such as massive gene sequencing to be able to search for biomarkers capable of predicting the response of PMME to anti-PD1 immunocheckpoint inhibitors. After another 38 cases of PMME [ 274 , 277 , 278 , 279 , 280 , 281 , 282 , 283 , 284 , 285 , 286 , 287 , 288 , 289 ], Endo F. et al [ 290 ] reported a case of a 70-year-old man who complained of dysphagia due to the presence of a polypoid lesion in the lower thoracic esophagus. Although the histopathological examination had provided the diagnosis of esophageal squamous carcinoma at first, with subsequent pT3N1M0 staging and clinical stage III, a histological re-evaluation after radical esophagectomy revealed the presence of atypical melanocyte cells positive for S-100 protein, Melan-A and HMB-45, and, therefore, PMME was diagnosed.…”

Longitudinal genomic alternations and clonal dynamics analysis of primary malignant melanoma of the esophagus

“…About 18.4% of PMME patients present with metastatic disease at the time of diagnosis and 89.7% develop recurrence or metastasis within a few months from diagnosis [1] . As a result, PMME tends to have a poor 5-year overall survival (OS) ranging between 4% to 37.5% [1] , [2] , [3] , [4] .…”