Primary Malignant Melanoma Of The Esophagus Research Articles

Endoscopic resection as a primary palliative therapy for advanced primary malignant melanoma of the esophagus

AbstractPrimary malignant melanoma of the esophagus is a rare aggressive malignant tumor with poor prognosis. It usually presents as dysphagia and retrosternal chest pain. Diagnosis is made by endoscopy and biopsy, and staging is done by computed tomography (CT) scan and fluorodeoxyglucose positron emission tomography scan. The mainstay of treatment is usually surgical with curative or palliative intent since radiotherapy and chemotherapy do not improve the outcome. Here, we report a case of 50-year-old female patient who presented with dysphagia. Esophagogastroduodenoscopy was done, which revealed a large black-colored polypoid lesion occluding the entire lumen of the esophagus. Histopathology confirmed it as malignant melanoma. CT of the chest was performed which showed a tumor mass extended into mediastinum abutting aorta and multiple mediastinal lymph nodes suggesting an advanced disease. As surgery could not be performed and radiotherapy and chemotherapy have no role, endoscopic resection of mass within the esophagus was done and the defect in the esophageal wall was closed with the clips. The patient was symptomatically improved following the resection. She expired after 3 months due to disseminated diseases.

Primary malignant melanoma of the esophagus treated by endoscopic submucosal dissection: A case report.

Primary malignant melanoma of the esophagus (PMME) is a rare malignant neoplasm of the esophagus. In the majority of cases, the disease originates in the mucosal layer of the esophagus, which is similar to other types of esophageal cancer. With the development of endoscopic submucosal dissection (ESD), endoscopic resection is possible for cases in which melanomas are limited to the mucosal and submucosal layer. However, few studies report the efficiency of ESD for PMME, and no studies perform long-term follow-up. The present study reported the case of a 71-year-old PMME patient who was successfully treated by ESD at The Third Affiliated Hospital of Soochow University (Changzhou, China) in Otober 2011, with a follow-up of >3 years conducted.

Characteristics and Surgical Outcomes for Primary Malignant Melanoma of the Esophagus.

Primary malignant melanoma of the esophagus (PMME) is an extremely rare disease with poor prognosis. We summarized and analyzed the characteristics of 17 PMME patients (with average age of 57.5 ± 10.3 years) who had received surgical resection in our center. The majority (13/17, 76.5%) of the patients were male. The percentage of patients with smoking and alcohol consumption was 41.2% and 23.5%, respectively. The preoperative diagnosis rate was 35.3%. Lymph node metastasis mainly involved the mid-lower mediastinal and upper abdominal area. Primary tumors that invaded beyond the submucosa layer (T2–T4) had much higher tendency of lymph node metastasis than those restricted to the submucosa layer (T1) (6/8, 75.0% vs. 3/9, 33.3%, p = 0.086). The 1-year and 5-year survival rate of the patients was 51% and 10%, respectively, with median survival time being 18.1 months. Survival analysis showed that TNM stage was a predictor for PMME prognosis (median survival time of 47.3 months vs. 8.0 months for stage I/II vs. stage III, respectively, p = 0.018), and multivariable Cox regression analysis revealed the independence of its prognostic value [HR (95% CI): 5.678 (1.125–28.658), p = 0.035].

Clinical significance of [18F]-fluoro-deoxy-glucose positron emission tomography/computed tomography in patients with primary malignant melanoma of the esophagus: report of three cases

Primary malignant melanoma of the esophagus (PMME) is a rare disease with poor prognosis. [18F]-Fluoro-deoxy-glucose positron emission tomography (FDG-PET) has been used extensively for initial tumor staging, therapeutic monitoring, and follow-up evaluation in various malignant tumors, but the significance of FDG-PET in PMME is currently unknown. Herein, we report 3 cases of PMME. In all 3 cases, histological and immunohistochemical examination of the biopsy specimens led to diagnoses of malignant melanoma. FDG-PET/computed tomography was performed in all cases, and was useful for the detection of distant metastases. However, the identification of lymph node metastases at initial diagnosis was difficult. Esophagectomy was performed in one case, while systemic chemotherapy was administered in 2 cases. All 3 patients had a poor prognosis; the mean survival time was 13.3 months. In conclusion, PET/computed tomography is useful for the detection of distant metastasis at initial diagnosis as well as the therapeutic monitoring of PMME.

P2-1-54 - Primary malignant melanoma of the esophagus successfully treated with nivolumab: A case report

P2-1-54 - Primary malignant melanoma of the esophagus successfully treated with nivolumab: A case report

Genetic mutations in primary malignant melanoma of the esophagus: case report and literature review

The most frequent genetic aberrations in mucosal melanoma are activating mutations of c-KIT. Primary malignant melanomas of esophagus (PMME) are uncommon entities, with aggressive biological behavior and poor prognosis. The better definition of their genotype could improve therapeutic options. We report a case of a 66 years old man with a PMME in the lower third of the esophagus. Analysis of c-kit, KRAS, NRAS and BRAF genes resulted negative for mutations. On the basis of a computerized (PuMed/Medline) bibliography search we retrieved a total of other 35 cases of PMME analyzed for genetic alterations in RAS, BRAF, and KIT. When we compared mutations frequency of PMME with those of other mucosal melanomas, it appeared that PMME are characterized by a relative higher percentage of NRAS mutations. PMME seem to show a specific pattern of genetic alterations suggesting that they could represent a distinct entity among mucosal melanomas.

A Case of Primary Malignant Melanoma of the Esophagus

Primary malignant melanoma of the esophagus is a rare disease and aggressive tumor of the esophagus. Primary malignant melanoma represents 0.1% to 0.2% of all primary esophageal cancers. Hematogenous and lymphatic metastasis are common. The prognosis is quiet poor, so early dectection and accurate diagnosis should be made. We encountered a patient with a black esophageal lesion, which was histopathologically confirmed to be primary malignant melanoma of the esophagus. A 65-year-old men was admitted for evaluation of a black pigmentation at lower esophagus which was found at regular general physical exam. He had no symptoms, no history of any illness and no family history of cancer. Physical examination found no abnormal pigmentations of the skin or mucosa. Endoscopy revealed multiple scattered 0.4cm sized black pigmented ulcerative lesion at 37cm from the incisors (Figure 1). Pathological findings from endoscopy show melanin pigment deposition and melanophage infiltration (Figure 2). Immunohistochemical staining was positive for HMB45, Ki-67, S100 and melan-A. Computed tomography of chest and abdomen showed no evidence of discernible lesion in the stomach, lung and regional lymphadenopathy. Positron emission tomography demonstrated no regional or distant metastasis. He received esophagectomy and gastroesphagostomy. Muscularis mucosa was involved, but the surgical margin of the esophagus was free from tumors. And all regional lymph nodes were found free of tumor.Figure 1Figure 2

Long-term outcome of esophagectomy for primary malignant melanoma of the esophagus: a single-institute retrospective analysis.

Primary malignant melanoma of the esophagus (PMME) is a highly malignant tumor with a poor prognosis. Because PMME is an extremely rare disease, therapeutic strategies against the tumor have yet to be established, and the efficacy of esophagectomy remains unclear. The objective of this study was to evaluate the post-esophagectomy survival of PMME patients. Ten patients who underwent esophagectomy for PMME between March 2005 and April 2013 at the Department of Gastroenterological Surgery, Cancer Institute Hospital, Tokyo, Japan, were identified from the institutional database. We retrospectively retrieved clinical information and data on the long-term outcomes from the patients' records. Survival rates after esophagectomy were calculated by the Kaplan-Meier method, and the hazard ratios of mortality were determined using the Cox's model. A follow-up study of the 10 patients revealed 7 cancer recurrences and 5 deaths. Median survival time was 34.5 months, and 5 of 10 patients survived longer than 2 years. The 1-year disease-free survival rate was 40%, and the 1- and 3-year overall survival rates were 70% and 60%, respectively. Importantly, all three of the non-relapsing patients were histologically confirmed as free of lymph node involvement. The four patients with lymph node metastasis relapsed within 1 year. The disease-free survival was significantly shorter in patients with lymph node involvement than in those without lymph node involvement (univariate hazard ratio = 13.3, 95% confidence interval 1.85-266.4; P = 0.009). In conclusion, esophagectomy might benefit PMME patients with no lymph node metastasis. Further large-scale cohort studies are needed to establish the treatment strategy for PMME.

Primary malignant melanoma of the esophagus: A case report and review of the literature

Primary malignant melanoma of the esophagus (PMME) is a rare malignancy and the prognosis is typically poor. There is currently a lack of appropriate treatment strategies and clear guidance. The current study presents the case of a 65-year-old female with a two-month history of progressive dysphagia, the investigations of which resulted in a diagnosis of PMME. The patient was treated with a radical transhiatal esophagectomy with subcarinal lymphadenectomy followed by combined chemoimmunotherapy. The patient remains alive with no evidence of tumor progression at the 12-month follow-up. The purpose of the present study was to report a new case and to review the recent relevant literature regarding the treatment of PMME.

Primary Malignant Melanoma of the Esophagus Treated by Early Diagnosis and Surgical Resection

Primary Malignant Melanoma of the Esophagus Treated by Early Diagnosis and Surgical Resection

Primary malignant melanoma of the esophagus: Clinical features, signaling pathway, management, and survival.

e20015 Background: Primary malignant melanoma of the esophagus (PMME) is a rare disease that has a poor prognosis and develops multiple metastases in the early stage. Treatment protocols are not well-established. Methods: Clinical data of 28 cases of PMME were retrospectively analyzed including clinical features, signaling pathway, management and survival in Peking University Cancer Hospital between 2006 and 2013. Somatic mutations in genes in MAP kinase (c-Kit, BRAF, NRAS) were detected by DNA sequencing. PI3K-AKT-mTOR (pmTOR, pS6RP, p4E-BP1 and pAKT) were examined by immunohistochemistry (IHC) assays. Results: 1) Clinical features:The average age was 56.4 years, and 75.0% were male. Overall, most tumors were located in the lower thoracic esophagus (53.6%) and majority were in early stages (I to III-85.7% according to 7th UICC TNM) when diagnosed. 24/28 patients accepted subtotal esophagectomy with lymph node dissection. 16/24 patients underwent adjuvant chemotherapy consisted of four courses of dacarbaz...

Primary malignant melanoma of the esophagus: A case report

Primary malignant melanoma of the esophagus (PMME) is a malignant tumor which occurs in the melanin cells of esophageal mucosal epithelial basal layer. PMME is a rare disease with an extremely poor prognosis. PMME represents only 0.1% to 0.2% of all esophageal malignant tumors. Dysphagia, retrosternal or epigastric discomfort or pain is the most frequent symptom at presentation. Retrosternal, epigastric discomfort, melena or hematemesis are the major clinical manifestations. The tumor is often located from the middle to lower thoracic esophagus. The characteristic endoscopic finding of PMME is a polypoid lesion that is usually pigmented. Immunohistochemical examination with positive results of S100 protein, HMB45 and neuron-specific enolase allow a definitive diagnosis. PMME metastasizes via hematogenic and lymphatic pathways. Esophagectomy is believed to be an effective approach for localized PMME. Five-year survival rates of 37% or higher have been achieved recently. Herein, we report a case of an 65-year-old female admitted for progressive difficulty in swallowing for more than 4 mo. After upper gastrointestinal endoscopy and biopsy, upper gastrointestinal series and computed tomography examination, the patient accepted radical esophagectomy, and the postoperative pathologic and immunohistochemical examination showed PMME.

Primary malignant melanoma of the esophagus: misdiagnosis and review of literature

Primary malignant melanoma of the esophagus: misdiagnosis and review of literature

Primary malignant melanoma of the esophagus: a rare and aggressive disease

Primary malignant melanoma of the esophagus is an uncommon tumor, with approximately 300 cases having been reported thus far. The purpose of this study was to describe a case of a 60 year-old man with a 10 month history of progressive dysphagia and thoracic pain, the investigations of which led to a diagnosis of primary malignant melanoma of the esophagus. The patient underwent a transhiatal esophagectomy with subcarinal lymphadenectomy, and isoperistaltic gastric tube replacement of the esophagus. Nine months after surgery, he developed ischemic colitis, and metastasis in the mesentery was diagnosed. His disease progressed and he died one year after the esophagectomy. A review of the literature was performed.

Diagnosis and Surgical Outcomes for Primary Malignant Melanoma of the Esophagus: A Single-Center Experience

We summarize the experience of diagnosis and surgical therapy for primary malignant melanoma of the esophagus (PMME). Clinical data of 13 patients diagnosed as having PMME treated by surgery as their primary therapy from 2000 to 2012 were retrospectively analyzed, and survival information was collected through follow-up. The average age (±standard deviation) of participants in this study was 66.4±7.6 years, and 84.6% were male. Overall, 61.5% of tumors were located in the lower thoracic esophagus. The accuracies of clinical T stage, N stage, and TNM stage were 53.9%, 46.2%, and 38.5%, respectively, compared with pathological staging (kappa=0.252, p=0.023). Surgical mortality and morbidity were 7.7% and 53.9%, respectively. The incidence of lymph node metastasis for patients with tumor invading within the mucosa was 0, but increased to 42.9% (3 of 7) with tumor invading to the submucosal layer. Primary malignant melanoma of the esophagus in the mid third of the thoracic esophagus had a greater chance to metastasize to perigastric lymph nodes (2 of 5) than to middle mediastinal lymph nodes (1 of 5). For PMME located at the lower third of the thoracic esophagus, upper mediastinal lymph node metastasis was more likely to occur (2 of 4) with tumor invasion penetrating the proper muscle layer. Recurrence occurred within 1 year in all patients with tumor later than Stage Ib. The most common recurrent organ was the liver. The overall 1-year and 5-year postoperative survival rates were 54.0% and 35.9%, respectively, and lymph node metastasis was the independent predictive factor for postoperative survival (p=0.013; odds ratio, 15.05). Despite the similarity in lymph node metastatic patterns to squamous cell carcinoma, PMME is more inclined to distant metastasis. Clinical staging was inconsistent with pathological staging for PMME based on endoscopy and computed tomography. Surgical therapy was the optimal treatment for PMME at an earlier stage. Early diagnosis and aggressive lymph node dissection were beneficial for accurate staging, potentially reducing recurrence and thus improving survival.

Primary Multifocal Malignant Melanoma of the Esophagus

Melanomas usually arise from the skin. Lesions arising from the mucosal epithelium are rare accounting for only 1.3 % of all cutaneous and non-cutaneous melanomas in an US American national cancer database including 84,836 patients [1]. A vast majority of gastrointestinal tract melanomas are metastatic and are mainly located in the small intestine [2]. Except for anorectal lesions, the primary location in the gastrointestinal tract is rare [3], and this holds especially true for a primary malignant melanoma of the esophagus with aggressive behavior and poor prognosis [4].

Multiple hematogenous metastasis after curative surgery in a patient with pT1a-LPM pN0 primary malignant melanoma of the esophagus

Primary malignant melanoma of the esophagus (PMME) is a rare disease. Here, we describe a case of PMME that was identified at a very early stage. A 65-year-old man underwent upper gastrointestinal endoscopy and was found to have a pigmented small nodule in the lower esophagus. A biopsy was taken and diagnosed as malignant melanoma, so the patient then received radical esophagectomy. Histopathological examination showed atypical cell infiltrate in the lamina propria mucosae without lymph-node metastasis. The patient was followed up without adjuvant chemotherapy. Multiple liver and lung metastases were found at 19 months after surgery, and the patient received DAV-feron (DTIC/ACNU/VCR/IFN-β) therapy. However, the tumors progressed and the patient died of the disease at 27 months after surgery. More detailed surveys after surgery with diagnostic imaging and tumor markers are recommended. Adjuvant chemotherapy is worth considering, even in patients with early-stage PMME.

Amelanotic primary malignant melanoma of the esophagus: report of a case treated with minimally invasive esophagectomy and adjuvant chemotherapy

Amelanotic primary malignant melanoma of the esophagus (PMME) is extremely rare. We report a case of amelanotic PMME that was accurately diagnosed and subsequently treated by radical resection with adjuvant chemotherapy. A 69-year-old woman was admitted to our hospital because of dysphagia. Endoscopic examination revealed a tumor without pigmentation in the upper thoracic esophagus, and melanosis in the lower thoracic esophagus. Tumor biopsy showed proliferation of malignant spindle cells with conspicuous nucleoli. Immunohistochemically, the tumor cells were positive for melanosomes (HMB45). The tumor was diagnosed as PMME. Positron emission tomography and computed tomography showed accumulation of the tracer only in the primary lesion. The preoperative diagnosis according to the extent of the tumor, whether cancer cells have spread to lymph nodes, and whether metastasis has occurred (TNM) classification was malignant melanoma in the upper third of the esophagus, T2N0M0 stage IIA. The patient underwent minimally invasive video assisted esophagectomy in the prone position with three-field lymph node dissection. On the resected specimen, the malignant cells were positive for HMB45, KIT, and melan-A. Masson-Fontana staining demonstrated no melanin pigmentation. The patient was diagnosed with amelanotic PMME and received adjuvant chemotherapy, consisting of dacarbazine, nimustine, cisplatin, and tamoxifen. The patient showed no systemic metastasis, and is alive 18 months after the operation with no evidence of recurrence.

Primary Malignant Melanoma of the Esophagus With Extensive Intraepithelial Extension

Primary malignant melanoma of the esophagus (PMME) is an exceedingly rare tumor. The prognosis of PMME is very poor, and the 5-year survival rate is only 2.2% due to its aggressive biologic character. We present patient with PMME with wide extension into almost the entire esophagus, without endoscopic abnormalities. This patient's presentation is extremely valuable because it has enabled us to observe the long-term natural progression of PMME, including recurrent disease with a wide range of atypical melanocytic cells in the entire esophagus.

Early-stage primary malignant melanoma of the esophagus detected simultaneously with esophageal squamous cell carcinoma

Primary malignant melanoma of the esophagus is a rare disease. The majority of patients are diagnosed at an advanced stage, and only a few are detected at an early stage. We herein describe a case of early-stage primary malignant melanoma of the esophagus that was detected simultaneously with early-stage primary esophageal squamous cell carcinoma. Both tumors were detected during esophagogastroduodenoscopy for heartburn. The malignant melanoma tumor was a nevus-like flat-type lesion in the upper thoracic esophagus, and the squamous cell carcinoma was a slightly depressed lesion in the abdominal esophagus. The tumor was resected by thoracoscopic esophagectomy. Histologically, the invasion of both tumors was limited to the mucosal layer, and no lymph node metastasis was detected. Immunohistochemically, the malignant melanoma cells were strongly positive for HMB-45, melan-A, and S-100 protein. The patient has survived without recurrence for 17 months after the operation.