Primary Esophageal Melanoma Research Articles

Primary Malignant Melanoma of Esophagus Treated with Endoscopic Mucosal Resection and Esophagectomy

A 74-year-old male with a long-standing history of chronic lymphocytic leukemia presented with dyspepsia. Upper endoscopy showed a subtle bluish 3–5-mm discoloration in the esophageal mucosa at approximately 20 cm from the incisors (Fig. 1). Biopsies demonstrated melanoma in situ and focal invasion into the superficial sub-mucosa, associated with melanosis, and consistent with primary esophageal melanoma (PMME, Fig. 2a, b). The cells stained positive for S-100 and MART-1a, which confirmed PMME. In an attempt to stage and treat the lesion, endoscopic mucosal resection (EMR) was done using the band and snare method. Pathology revealed an extensive in situ melanoma and a small cluster of invasive melanoma at one margin of excision within the sub-mucosa. A repeat EGD and biopsy demonstrated residual melanoma in situ at the margins of the resection. Repeat EMR was discussed; however, the patient wished to proceed with a transhiatal esophagectomy with cervical esophagogastric anastomosis. The surgical specimen revealed a 4.0×2.5-cm area of black–brown mucosal discoloration, located 1.5 cm form the proximal margin of resection. Microscopic sections showed extensive residual intramucosal melanoma involving the discolored area, the proximal cervical margin and random sections of mucosa away from the discolored area (Fig. 3a, b). Thirty weeks after the surgery, the patient is clinically stable except for cervical dysphagia for which he is treated with dilatations.

Multimodality management of a locally advanced primary oesophageal melanoma with radical surgery and adjuvant chemoimmunoradiotherapy

We present the case of a 46-year-old woman diagnosed with a primary oesophageal melanoma (PEM), who was treated with radical surgery followed by combined chemoimmunotherapy (interferon, carboplatin, dacarbazine and external radiotherapy) and who achieved a complete response after this treatment. PEMs are rare malignancies, with less than 300 cases described in the literature. The main differential diagnosis is with metastases of skin or ocular malignant melanomas. They are usually diagnosed at advanced stages and prognosis is typically poor. The main treatment modality should be radical surgery. The role of adjuvant treatment is uncertain, although some long responses have been seen with the use of chemotherapy or immunotherapy alongside surgery.

Comparison of Primary and Metastatic Malignant Melanoma of the Esophagus: Clinicopathologic Review of 10 Cases

Primary esophageal melanoma (PEM) is a rare disease and is difficult to distinguish from other esophageal malignancies and from metastatic melanoma. To develop diagnostic criteria for PEM, we compared the clinicopathologic features of 5 PEMs and 5 metastatic melanomas to esophagus. Ten cases of esophageal melanoma, including 4 surgically resected specimens, 2 autopsy cases, and 4 cases reported on mucosal biopsies, were reviewed. The histologic parameters used in this study were well-characterized features for cutaneous melanoma, including junctional component (in situ melanoma), radial growth phase, modified Breslow thickness, depth of invasion, lymphovascular invasion, satellitosis, predominant type of cytology, and regional lymph node metastasis. Clinical and follow-up information was obtained by reviewing patients' medical records. Previous history of cutaneous melanoma was present in all 5 cases of metastatic esophageal melanoma but was not present in the 5 patients with PEMs. In situ melanoma and/or radial growth phase were identified in all 5 PEMs but were not present in any of the metastatic cases. Among the 4 resected and 2 autopsy cases, melanocytosis and mixed epithelioid and spindle cell morphology was present in 2 (50%) of 4 PEMs but was not present in 2 (40%) of the metastatic melanomas. Melanin pigment was detectable in all cases. Patients with PEM had better survival than those who had metastatic melanoma to esophagus (P = .03). The presence of in situ melanoma, radial growth phase, melanocytosis, and mixed epithelioid and spindle cell morphology, in the context of no history of melanoma, distinguishes PEM from metastatic melanoma.

Comparison of primary and metastatic malignant melanoma of the esophagus: clinicopathologic review of 10 cases.

Abstract Context.—Primary esophageal melanoma (PEM) is a rare disease and is difficult to distinguish from other esophageal malignancies and from metastatic melanoma. Objective.—To develop diagnostic criteria for PEM, we compared the clinicopathologic features of 5 PEMs and 5 metastatic melanomas to esophagus. Design.—Ten cases of esophageal melanoma, including 4 surgically resected specimens, 2 autopsy cases, and 4 cases reported on mucosal biopsies, were reviewed. The histologic parameters used in this study were well-characterized features for cutaneous melanoma, including junctional component (in situ melanoma), radial growth phase, modified Breslow thickness, depth of invasion, lymphovascular invasion, satellitosis, predominant type of cytology, and regional lymph node metastasis. Clinical and follow-up information was obtained by reviewing patients' medical records. Results.—Previous history of cutaneous melanoma was present in all 5 cases of metastatic esophageal melanoma but was not present in the...

A Difficult Diagnosis to Swallow

Purpose: Primary melanoma of the esophagus is an extremely rare diagnosis. Bulky esophageal melanomas are even rarer. We report a case of Malignant Melanoma involving the esophagus in a patient with no prior history of the disease who presented with complaints of dysphagia. Case Report: 79 yo male with a history of prostate cancer, CAD with CABG, and CVA comes to the hospital with complaints of progressive solid food dysphagia and weight loss for 2 months. The patient had no history of Melanoma. A heterogeneously hyperpigmented bulky lesion was seen on EGD from 25 cm to 35 cm in the mid esophagus. The mass was friable and involved 2/3 of the lumen. A small, 3 mm, dark, hyperpigmented lesion was also seen in the gastric body. Biopsies confirmed Melanoma on s100 staining at both sites. Pt was note to have metastatic disease to the liver, and multiple lung nodules on staging CT. The primary Internal Medicine team did not note any evidence of Melanoma on skin exam. Eye exam done by Ophthalmology also did not find any evidence of Melanoma. In view of advanced disease, only palliation was sought. A gastric feeding tube was placed by interventional radiology. Pt was started on Temodar by Oncology, and sent with Hospice follow up. Discussion: Primary Esophageal Melanoma of the esophagus is rare diagnosis with less then 250 cases reported in the world literature to date. Esophageal Melanoma is a very aggressive tumor which carries a poor overall prognosis. There is limited data, primarily in the form of case reports, about potential surgical cures for very early stage disease. Treatment is evolving. Once metastatic, the prognosis for the disease is poor and palliative care should be considered.FigureFigure

Primary Malignant Melanoma of the Esophagus Arising from a Melanotic Lesion: Report of a Case

We herein report a case of primary esophageal malignant melanoma in which the development from a preceding benign melanotic lesion and the growing process of the tumor were chronologically observed by serial endoscopic examinations. Biopsy specimens repeatedly taken from the tumor failed to identify the presence of malignant melanoma. A positron emission tomography scan and gross changes of the tumor endoscopically observed were useful for detecting the presence of malignant transformation. The patient eventually died of generalized metastatic disease soon after undergoing an esophagectomy. An early diagnosis may therefore be crucial for improving the treatment outcome of esophageal malignant melanoma. Therefore, esophageal melanotic lesions should be carefully followed up even if biopsy specimens repeatedly show no malignancy.

Primary malignant melanoma of the esophagus with multiple esophageal lesions

A 48-year-old Chinese woman presented with a 2-month history of progressive dysphagia for solids, and (less frequently) liquids. She had minimal weight loss and no family history of cancer. Physical examination, conventional esophagogastroduodenoscopy, CT, fluorodeoxyglucose-PET, endoscopic ultrasonography, and histopathologic examination of excised tumor and biopsy specimens. Primary malignant esophageal melanoma with submucosal infiltration but no distant metastasis. Endoscopic polypectomy followed by near-total esophagectomy with esophagogastric anastomosis.

Primary Malignant Melanoma of the Esophagus: A Case Report

Primary malignant melanoma of the esophagus is extremely rare, with less than 200 cases reported in the literature. However, primary malignant melanoma is an aggressive tumor. We report a case of primary esophageal malignant melanoma that presented with a large polypoid mass in the lower esophagus, and subsequently showed multiple metastatic nodules in the subcutaneous layer, skin and retroperitoneum. Primary malignant melanoma should be included in the differential diagnosis of a polypoid esophageal mass, especially in cases where metastases to unexpected areas occur or in cases of unexpected metastatic patterns.

Solitary pulmonary metastasis from primary melanoma of the oesophagus 5 years after resection of the primary tumor

BackgroundPrimary malignant melanoma of the oesophagus (PMME) is an uncommon tumor. PMME has an aggressive biological behavior, similar to melanomas developed elsewhere in the body. Most patients die from distant metastases, and the overall 5 year survival rate is approximately 4%.Case presentationWe report a rare case of a solitary pulmonary metastasis found 5 years after curative resection of primary esophageal melanoma. No other sites of metastatic disease were identified. Video-assisted lung wedge resection of the lung nodule was carried out successfully.ConclusionThis supports the concept that patients with primary melanoma of the oesophagus treated should be carefully followed up.

Esophageal melanocytosis morphologic features and review of the literature.

Endoscopic or macroscopic esophageal melanocytosis is a benign clinicopathologic entity characterized by melanocytic proliferation in esophageal squamous epithelium and melanin deposition in the mucosa. Little is known about the etiology and natural course of this condition, although it has been suggested to be a precursor of primary esophageal melanoma by some authors. Following a search of the bibliographic databases (PubMed and Medline) regarding esophageal melanocytosis and melanosis, thirty-four cases of isolated esophageal melanocytosis (including one unpublished case from us) were found. The histopathologic features of esophageal melanocytosis are reviewed and its differential diagnosis with other pigmented esophageal lesions is discussed.

Esophageal Melanocytosis Morphologic Features and Review of the Literature

Endoscopic or macroscopic esophageal melanocytosis is a benign clinicopathologic entity characterized by melanocytic proliferation in esophageal squamous epithelium and melanin deposition in the mucosa. Little is known about the etiology and natural course of this condition, although it has been suggested to be a precursor of primary esophageal melanoma by some authors. Following a search of the bibliographic databases (PubMed and Medline) regarding esophageal melanocytosis and melanosis, thirty-four cases of isolated esophageal melanocytosis (including one unpublished case from us) were found. The histopathologic features of esophageal melanocytosis are reviewed and its differential diagnosis with other pigmented esophageal lesions is discussed.

Amelanotic Malignant Melanoma of the Esophagus: Case Report

We present the case of a primary malignant esophageal melanoma arising in a 75-year-old male, initially diagnosed as anaplastic squamous cell carcinoma. After resection of the tumor, histological work-up was indicative of a marked morphological heterogeneity, resembling a focally amelanotic primary malignant melanoma. Primary malignant melanomas of the esophagus are exceptionally rare. An exact preoperative diagnosis is critical with respect to the appropriate therapeutic strategy. Clinicopathological features of this entity with a brief review of the literature are presented.

Primary Esophageal Melanoma in a Patient With Limited Scleroderma

Primary Esophageal Melanoma in a Patient With Limited Scleroderma

Primary melanoma of the oesophagus well palliated by radiotherapy

A case of a 16 cm primary melanoma of the mid oesophagus in a Caucasian male is reported. Radiological investigations at presentation revealed asymptomatic mediastinal and lower oesophageal metastases. The patient was treated with hypofractionated radiotherapy and achieved durable local disease control and excellent palliation of his dysphagia and chest pain until his death from widespread metastatic disease 5 months after treatment. The role of external beam radiotherapy in the treatment of primary oesophageal melanoma is reviewed.

Endosonographic features of primary esophageal melanoma

Malignant melanoma most commonly arises in the skin; however, primary melanomas can also arise from the mucosal epithelial lining of the gastrointestinal tract. Primary esophageal melanoma is rare and constitutes only 0.1% of primary esophageal tumors. There are only a few reports on the use of endosonography in this condition. We report here a case of a 45‐year‐old male with a history of painful deglutition and describe the endosonographic features of primary esophageal melanoma.

Primary malignant melanoma of the oesophagus

Primary malignant melanomas of the oesophageal squamous mucosa are exceedingly rare. We present here the clinical and pathological findings of 10 patients (mean age 64 years) with primary oesophageal melanoma, with emphasis on the immunophenotype of the tumours. The majority of melanomas were located in the mid to distal oesophagus and were large (mean tumour size at the time of diagnosis 6.2 cm; mean depth of invasion 1.86 cm). All but two of the melanomas were associated with an extensive in situ component. Half of the tumours were amelanotic. The histological spectrum was wide, including appearances mimicking lymphoma, poorly differentiated adenocarcinoma or sarcoma. Immunohistochemical studies were performed on six tumours using monoclonal antibodies (MAb) to S100 protein, tyrosinase (MAb T311), Melan-A (MAb A103), and gp100 (MAb HMB-45), as well as antibodies to five cancer/testis (CT) antigens (MAb CT7-33 to CT7/MAGE-C1, MAb ESO121 to NY-ESO-1, MAb 57B to MAGE-A4, MAb MA454 to MAGE-A1, and MAb M3H67 to MAGE-A3). Seven patients had metastatic disease at the time of presentation. All but one patient underwent resection of the tumour with negative surgical margins. Survival was poor, with a mean survival of 19.8 months. One patient, however, whose tumour was limited to the submucosa, is still alive 108 months post-oesophagectomy. All six melanomas examined by immunohistochemistry were positive for all the melanocyte differentiation markers tested. In addition, they were all positive for CT antigens, with MA454 being the most commonly found, suggesting that CT antigens may be a promising immunotherapeutic target for oesophageal melanomas.

Primary malignant melanoma of the oesophagus: a clinical and pathological study with emphasis on the immunophenotype of the tumours for melanocyte differentiation markers and cancer/testis antigens.

Primary malignant melanomas of the oesophageal squamous mucosa are exceedingly rare. We present here the clinical and pathological findings of 10 patients (mean age 64 years) with primary oesophageal melanoma, with emphasis on the immunophenotype of the tumours. The majority of melanomas were located in the mid to distal oesophagus and were large (mean tumour size at the time of diagnosis 6.2 cm; mean depth of invasion 1.86 cm). All but two of the melanomas were associated with an extensive in situ component. Half of the tumours were amelanotic. The histological spectrum was wide, including appearances mimicking lymphoma, poorly differentiated adenocarcinoma or sarcoma. Immunohistochemical studies were performed on six tumours using monoclonal antibodies (MAb) to S100 protein, tyrosinase (MAb T311), Melan-A (MAb A103), and gp100 (MAb HMB-45), as well as antibodies to five cancer/testis (CT) antigens (MAb CT7-33 to CT7/MAGE-C1, MAb ESO121 to NY-ESO-1, MAb 57B to MAGE-A4, MAb MA454 to MAGE-A1, and MAb M3H67 to MAGE-A3). Seven patients had metastatic disease at the time of presentation. All but one patient underwent resection of the tumour with negative surgical margins. Survival was poor, with a mean survival of 19.8 months. One patient, however, whose tumour was limited to the submucosa, is still alive 108 months post-oesophagectomy. All six melanomas examined by immunohistochemistry were positive for all the melanocyte differentiation markers tested. In addition, they were all positive for CT antigens, with MA454 being the most commonly found, suggesting that CT antigens may be a promising immunotherapeutic target for oesophageal melanomas.

Primary malignant melanoma of the esophagus: radiographic findings in seven patients.

To evaluate the radiographic findings in a series of patients with primary malignant melanoma of the esophagus. Seven cases of primary esophageal melanoma were collected from the Hospital of the University of Pennsylvania and the radiologic archives of the Armed Forces Institute of Pathology. All patients underwent esophagography (double-contrast esophagograms in four patients, single-contrast esophagograms in three patients). Medical, endoscopic, and pathology reports were also reviewed. Six patients presented with dysphagia (average duration, 5 months). The remaining patient had recent onset of melena. In all patients, barium studies revealed bulky, polypoid intraluminal masses that focally expanded the esophagus without causing obstruction. The tumors were located in the distal third of the thoracic esophagus in four patients, the middle third in two, and the proximal third in one. Tumor pigmentation was noted at endoscopy in only one patient. All patients underwent extensive esophageal resection. Of four patients with clinical follow-up findings, two were alive and well 2 and 6 months after surgery; the other two had metastases to the lung and liver within 2 months of surgery. Primary esophageal melanomas have strikingly similar barium study findings, appearing as bulky, polypoid intraluminal masses that focally expand the esophagus without causing obstruction. Despite its rarity, primary esophageal melanoma should be considered when characteristic findings are present on barium studies.

Primary malignant melanoma of the esophagus

A 48-year-old woman presented with a 6-month history of dysphagia, often associated with retrosternal chest pain. Upper endoscopy revealed an unusual pigmented lesion within the middle portion of the esophagus, and multiple biopsies were obtained. The histopathology and immunohistochemical profile of the tissue specimens were diagnostic of malignant melanoma. A thorough clinical and radiographic evaluation was performed, providing no additional findings or alternative primary source. Of approximately 11,500 melanoma patients entered in the Duke University melanoma database since 1970, this represents the only case of primary esophageal melanoma. The case is described and a review of the literature is presented.

Primary malignant melanoma of the oesophagus with a left atrial metastasis.

A 75 year old man with an eight month history of dysphagia and weight loss underwent pericardiocentesis for a massive pericardial effusion. The echocardiogram showed a mass in the left atrium, and computed tomography also showed distal oesophageal narrowing, which was found to be due to a malignant melanoma. The left atrial mass, investigated by immunoscintigraphy with technetium-99m labelled monoclonal antibody, was diagnosed as metastatic melanoma. This represents a rare case of primary oesophageal melanoma with left atrial metastasis.