Primary Malignant Melanoma Of The Esophagus Research Articles

Current status of primary malignant melanoma of the esophagus: clinical features, pathology, management and prognosis

Primary malignant melanoma of the esophagus (PMME) is a rare disease with an extremely poor prognosis. Up to 2011, approximately 300 cases had been reported worldwide. The average age of onset is 60.5years old, with a prevalence of males (2:1). A typical finding of PMME is a lobular or polyploid, well-circumscribed and pigmented tumor, partly covered with normal mucosa. PMME represents various colors depending on its melanin quantity and commonly coexists with intramural metastases, melanocytosis or melanoma in situ. The tumor is located from the middle to lower thoracic esophagus. The accuracy of diagnosis from biopsy is approximately 80%, because many cases are misdiagnosed as a poorly differentiated carcinoma because of the absence of melanin granules. A definite diagnosis was made by immunohistochemical examination with positive results of S100 protein, HMB45 and neuron-specific enolase. PMME has a highly metastatic potential, and the incidence of distant metastasis at the initial diagnosis is around 40-80%. A metastatic tumor from cutaneous malignant melanoma is another pigmented esophageal tumor to be considered when making the differential diagnosis for PMME. Junctional activity with melanotic cells in the adjacent epithelium and the presence of in situ melanoma and/or a satellite tumor without a previous history of cutaneous melanoma are definitive. Most of the reported patients were treated with radical esophagectomy, which is believed to be an effective approach for localized PMME. Five-year survival rates have been achieved in 37% recently, while adjuvant therapy has not been proven to increase overall survival but plays a palliative role.

Primary Malignant Melanoma of the Esophagus

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: A rare entity diagnosed by endoscopic ultrasound guided fine‐needle aspiration

Diagnostic CytopathologyVolume 40, Issue 5 p. 462-465 Letter to the Editor Primary malignant melanoma of the esophagus: A rare entity diagnosed by endoscopic ultrasound guided fine-needle aspiration Katie L. Dennis M.D., Katie L. Dennis M.D. Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KSSearch for more papers by this authorPatricia A. Thomas M.D., M.A., Patricia A. Thomas M.D., M.A. Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KSSearch for more papers by this authorFang Fan M.D., PH.D., Corresponding Author Fang Fan M.D., PH.D. [email protected] Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KSDepartment of Pathology and Laboratory Medicine, University of Kansas Medical Center, 3901 Rainbow Blvd., Kansas City, KS 66160-7417Search for more papers by this authorMojtaba Olyaee M.D., Mojtaba Olyaee M.D. Department of Gastroenterology and Hepatology, University of Kansas Medical Center, Kansas City, KSSearch for more papers by this author Katie L. Dennis M.D., Katie L. Dennis M.D. Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KSSearch for more papers by this authorPatricia A. Thomas M.D., M.A., Patricia A. Thomas M.D., M.A. Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KSSearch for more papers by this authorFang Fan M.D., PH.D., Corresponding Author Fang Fan M.D., PH.D. [email protected] Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, KSDepartment of Pathology and Laboratory Medicine, University of Kansas Medical Center, 3901 Rainbow Blvd., Kansas City, KS 66160-7417Search for more papers by this authorMojtaba Olyaee M.D., Mojtaba Olyaee M.D. Department of Gastroenterology and Hepatology, University of Kansas Medical Center, Kansas City, KSSearch for more papers by this author First published: 17 March 2011 https://doi.org/10.1002/dc.21674Citations: 3Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume40, Issue5May 2012Pages 462-465 RelatedInformation

Primary malignant melanoma of the esophagus: A case report

The authors present the clinical case of an 87-year-old Caucasian male admitted to the emergency room with hematemesis. He had a history of intermittent dysphagia during the previous month. Endoscopic evaluation revealed an eccentric, soft esophageal lesion located 25-35 cm from the incisors, which appeared as a protrusion of the esophagus wall, with active bleeding. Biopsies were acquired. Tissue evaluation was compatible with a melanoma. After excluding other sites of primary neoplasm, the definitive diagnosis of Primary Malignant Melanoma of the Esophagus (PMME) was made. The patient developed a hospital-acquired respiratory infection and died before tumor-directed treatment could begin. Primary malignant melanoma represents only 0.1% to 0.2% of all esophageal malignant tumors. Risk factors for PMME are not defined. A higher incidence of PMME has been described in Japan. Dysphagia, predominantly for solids, is the most frequent symptom at presentation. Retrosternal or epigastric discomfort or pain, melena or hematemesis have also been described. The characteristic endoscopic finding of PMME is as a polypoid lesion, with variable size, usually pigmented. The neoplasm occurs in the lower two-thirds of the esophagus in 86% of cases. PMME metastasizes via hematogenic and lymphatic pathways. At diagnosis, 50% of the patients present with distant metastases to the liver, the mediastinum, the lungs and the brain. When possible, surgery (curative or palliative), is the preferential method of treatment. There are some reports in the literature where chemotherapy, chemohormonotherapy, radiotherapy and immunotherapy, with or without surgery, were used with variable efficacy. The prognosis is poor; the mean survival after surgery is less than 15 mo.

Primary malignant melanoma of the esophagus: a study of clinical features, pathology, management and prognosis

Primary malignant melanoma of the esophagus (PMME) is a rare disease that is characterized by aggressive invasion, early metastasis, and poor prognosis. Treatment protocols are not well-established. To understand this condition more precisely, we performed a retrospective review of eight cases of PMME diagnosed at the Thoracic Department of the Cancer Center at Sun Yat-Sen University between 1985 and 2009. Eight PMME patients (five men and three women) with a mean age of 58 years (range: 48 to 72 years) were included. Dysphagia was the most common presenting symptom. All patients underwent an Ivor-Lewis esophagogastrectomy and lymph node dissection with postoperative adjuvant chemotherapy. One patient with stage III/pT4N0M0 underwent postoperative chemotherapy plus radiotherapy. Four patients died of distant metastases. The median survival time was 28 months (range: 11 months to 6 years). Our data confirm that PMME is a highly aggressive disease with a poor prognosis. If the diagnosis is suspected or confirmed as PMME, and the patients have no distal metastases or extensive lymph node enlargement, we suggest that surgery should be the first choice of treatment. With regard to adjuvant therapy, we recommend the addition of chemotherapy. The role of radiotherapy remains questionable and requires further investigation.

Radiotherapy combined with hyperthermia for primary malignant melanomas of the esophagus

Primary malignant melanoma of the esophagus (PMME) forms about 0.1% of all primary esophageal cancers. Treatment options are very limited for patients unfit for surgery. This is the first report describing the results of external radiotherapy combined with regional hyperthermia for two inoperable PMME patients. Two patients with a T2-3N0M0 PMME were considered unfit for surgery based on age and general condition. External radiotherapy of a total dose of 35 Gy was given in a scheme of seven times 5 Gy, two times per week, and once weekly combined with external and intraluminal hyperthermia (aim 43°C). Toxicity was mild and both patients completed treatment according to protocol. Adequate temperatures at the intraluminal border of the tumor were achieved. In both patients, a complete remission was achieved with complete relief of obstructive symptoms and without signs of locoregional tumor progression until the end of follow-up at 11 and 15 months. External radiation combined with regional hyperthermia could be a good alternative to resection in patients unfit for surgery with a malignant melanoma of the esophagus.

Primary Malignant Melanoma of the Esophagus, an Atypical Presentation of an Atypical Tumor

Primary Malignant Melanoma of the Esophagus, an Atypical Presentation of an Atypical Tumor

Primary malignant melanoma of the esophagus

Primary malignant melanoma of the esophagus is an extremely rare, but highly aggressive, tumor. The prognosis for primary malignant melanoma of the esophagus remains dismal in most literatures. We report here a case of primary malignant melanoma of the esophagus that was treated by surgical resection and, additionally, followed by chemotherapy. The clinical features, treatment, pathological findings, and prognosis are analyzed and the literature of primary malignant melanoma of the esophagus is reviewed.

Multiple early-stage malignant melanoma of the esophagus with long follow-up period after endoscopic treatment: report of a case

Primary malignant melanoma of the esophagus (PMME) is a rare disease. Most patients are diagnosed at an advanced stage and few at an early stage. We report a patient with multiple early-stage PMME (tumor invasion was limited to the mucosa, and no lymph node metastasis was detected). Despite initial endoscopic treatment followed by systemic chemotherapy, frequent multiple metachronous lesions occurred. These lesions were all flat type, and disease control of the primary site was achieved only by repeated endoscopic treatment. Liver metastasis developed 7 years after the first diagnosis of this disease; however, the patient has been doing well with stable disease for 1 year after single transarterial chemoembolization.

Primary Malignant Melanoma of the Esophagus - Case Report and Review of the Literature

Purpose: Introduction: Primary malignant melanoma of the esophagus (PMME) is rare, with a reported incidence of 0.1% to 0.5% of esophageal malignancies and less than 260 reported cases. Case Report: A 59 year-old man presented with progressive dysphagia and weight loss for 2 months. Skin and eye exam were normal. Upper endoscopy (EGD) revealed an 8 cm fungating, darkly pigmented, ulcerated, partially obstructing mass in the lower esophagus. Biopsy showed subepithelial sheets of malignant, medium-sized, mononucleated cells with normal overlying squamous epithelium. Tumor cells were diffusely immunoreactive to HMB-45, Mart-1 and vimentin; weakly reactive to protein S100; and negative to cytokeratin and lymphoid markers. A diagnosis of small cell primary melanoma of the esophagus was made. Exploratory laparotomy confirmed celiac nodes involvement, and thus stage IV melanoma. A covered esophageal stent was placed endoscopically. Patient underwent palliative radiotherapy and chemotherapy. Three months later he presented with hematemesis. EGD revealed a recurrent mass above the stent with an adherent clot. Restenting was suggested, but the patient opted for comfort care and expired six months later. Discussion: Melanocytes occur in 4-8% of normal esophagi; however, PMME is exceedingly rare. The most common endoscopic finding is a polypoid submucosal mass covered by intact squamous mucosa. Pigmentation is present in 75-90% of cases. Satellite nodules are found in 12% of cases. Uncommonly, a fungating mass may be seen, as occurred presently. Endoscopic biopsy is diagnostic in only 50% of cases. EUS-FNA is 90% accurate and should be performed for a pathologic diagnosis when necessary. PMME tends to spread vertically in mucosa and submucosa, only rarely involving deeper layers. Overlying squamous mucosa is usually intact. Esophageal melanomas are amelanotic in 2% of cases. Standard histological diagnosis is difficult; PMME is missed in 50% cases, frequently being initially labeled as a poorly differentiated squamous tumor. On immunohistochemistry PMME stains positive for vimentin, protein S-100, HMB-45, and melan-A; and is negative for cytokeratin AE1/AE3, desmin, and CD117. Wide-margin surgical resection is the only curative option. Mean survival after radical resection is 14.8 months. Radiation therapy, chemotherapy, and immunotherapy can be used in advanced cases. Endoscopic palliation includes: dilation, tumor photodynamic ablation, and stenting. Prognosis is dismal. Mean survival time is 10-14 months. The 5 year survival rate is 4%. Conclusion: PMME is a rare tumor. Diagnosis can be very challenging, and requires multiple biopsies and detailed immunohistochemical analysis.

Atypical Cause of Dysphagia: A Case Report of Primary Malignant Melanoma of the Esophagus

Purpose: Primary malignant melanoma of the esophagus (PMME) is an extremely rare entity that is thought to be caused by malignant degeneration of the esophageal melanocytes. It accounts for an estimated 0.1% of esophageal tumors and it carries a poor prognosis with a median survival rate at 5 years of less than 5%. This patient, an 80-year-old female, was referred to our hospital for further workup of an esophageal mass found during esophagogastroduodenoscopy (EGD). Her initial chief complaint was progressive dysphagia to solids and then liquids accompanied by weight loss, decreased appetite, night sweats and fatigue. Her co-morbidities included hypertension, hypercholesterolemia, and diabetes mellitus type II with no smoking or alcohol abuse history. She was cachectic on physical exam. An EGD done at the outside hospital showed an esophageal mass measuring 3.4 × 2.7 × 4.3 cm just above the esophagogastric (EG) junction. The pathology from the EGD biopsy revealed partially necrotic malignant neoplasm with spindle and epithelioid malignant cells containing punctuate and coarse intracytoplasmic dark brown pigment, confirming the diagnosis. After receipt of biopsy results confirming the diagnosis of PMME, an EGD with endoscopic ultrasound (EUS) was done which led to the staging diagnosis of T2 N1 metastatic malignant melanoma. The EUS further characterized the mass as pedunculated, not extending beyond the muscularis propria. CT scan revealed no metastasis. The surgical team was consulted, but the patient opted out of surgical intervention. An Ultraflex covered 7 cm esophageal stent was placed for symptomatic relief. The patient declined any chemo or radiation treatments. Primary malignant melanoma of the esophagus (PMME) is a non-epithelial neoplasm that is extremely rare accounting for less than 0.1% of all primary esophageal tumors. Recent research has concluded that abnormal migration of melanocytes during embryogenesis likely leads to esophageal melanocytosis predisposing to development of PMME. In the United States, the incidence of PMME has been estimated to be approximately 8 cases annually. Clinical manifestations include primary complaints of dysphagia, weight loss and non-specific retrosternal pain. Endoscopically, PMME is characterized by white to black-brown large, polypoidal and often focally ulcerated lesions that are difficult to distinguish from poorly differentiated carcinoma. It mainly affects the lower two-thirds of the esophagus. Our case highlights the typical presentation of PMME and adds to the small number of reported cases. It further re-emphasizes the non-surgical treatment options available for patient comfort.

SLOW GROWING FLAT‐TYPE PRIMARY MALIGNANT MELANOMA OF THE ESOPHAGUS TREATED WITH CAP‐ASSISTED EMR

We report a rare case of flat-type primary malignant melanoma of the esophagus treated with endoscopic mucosal resection (EMR). A 64-year-old woman was referred for examination of a small pigmented lesion located in the mid esophagus. On endoscopy, the lesion exhibited almost no change in size over the year. Cap-assisted EMR was performed en bloc. The histopathological findings showed atypical melanocyte proliferation in the basal layer, spindle cells, and epithelioid cell proliferation with nuclear enlargement and a few mitotic figures. Histopathological examination confirmed the diagnosis of primary malignant melanoma. Immunostaining for S-100 protein and HMB-45 antibody were positive, and the Ki-67 index was low. The patient was discharged without additional surgical resection and/or chemotherapy. The patient had no symptoms and no signs of recurrence 20 months after EMR. There has been no report on a slow growing esophageal melanoma. It is difficult to estimate the malignant behavior of this case.

Melanoma primario del esófago

Resumen en: Primary malignant melanoma of the esophagus is an extremely rare tumor. Less than 300 cases have been published worldwide. Although surgical excision is ...

Effective palliative radiotherapy in primary malignant melanoma of the esophagus: a case report

IntroductionPrimary malignant melanoma of the esophagus is a rare but highly aggressive tumor with poor prognosis. Surgical resection is the treatment of choice. However, some cases may be diagnosed with advanced inoperable disease. Palliative radiotherapy may be used to relieve symptoms caused by the esophageal tumor.Case presentationWe report on a case of advanced inoperable primary malignant melanoma of the esophagus treated with palliative radiotherapy. The patient’s dysphagia resolved with radiotherapy.ConclusionMalignant melanoma of the esophagus is rare. Patients with advanced inoperable malignant melanomas of the esophagus benefit from radiation therapy. Radiation therapy is effective for palliation.

Nevus-Like Appearance of Primary Malignant Melanoma of the Esophagus

The primary malignant melanoma of the esophagus (PMME) is a rare malignant disease, accounting for only 0.1–0.2% of all esophageal neoplasms, and the majority of the patients are diagnosed at advanced stages with poor prognosis. We present here a case of 56-year-old woman with epigastric pain and her endoscopic finding revealed several flat and black pigmented mucosal lesions within the distal portion of the esophagus which looked like flat nevus. The histopathology and immunohistochemical profile of the tissue specimens were diagnostic of malignant melanoma.

Primary Malignant Melanoma of the Esophagus With Separate Foci of Melanoma In Situ and Atypical Melanocytic Hyperplasia in a Patient Positive for Human Immunodeficiency Virus: A Case Report and Review of the Literature

Abstract Primary malignant melanoma of the esophagus (PMME) is a rare neoplasm. The primary nature of esophageal melanoma has been questioned in the past because most reported cases have not been able to demonstrate local tumorigenesis or evolution of the malignancy from a preceding benign lesion or cell. In addition, the occurrence of metastatic melanoma to the esophagus is much more common than PMME. We report a case of PMME with separate foci of melanoma in situ and atypical melanocytic hyperplasia in a patient with human immunodeficiency virus infection. To our knowledge, this is the first reported case of PMME in a patient with a previous diagnosis of human immunodeficiency virus infection. In addition to adding data to the limited literature on this subject, we also provide histologic evidence that demonstrates the primary nature of some esophageal melanomas. This case also documents the occurrence of PMME in a patient positive for human immunodeficiency virus. We also provide a review of the releva...

Primary Malignant Melanoma of the Esophagus With Separate Foci of Melanoma In Situ and Atypical Melanocytic Hyperplasia in a Patient Positive for Human Immunodeficiency Virus: A Case Report and Review of the Literature

Primary malignant melanoma of the esophagus (PMME) is a rare neoplasm. The primary nature of esophageal melanoma has been questioned in the past because most reported cases have not been able to demonstrate local tumorigenesis or evolution of the malignancy from a preceding benign lesion or cell. In addition, the occurrence of metastatic melanoma to the esophagus is much more common than PMME. We report a case of PMME with separate foci of melanoma in situ and atypical melanocytic hyperplasia in a patient with human immunodeficiency virus infection. To our knowledge, this is the first reported case of PMME in a patient with a previous diagnosis of human immunodeficiency virus infection. In addition to adding data to the limited literature on this subject, we also provide histologic evidence that demonstrates the primary nature of some esophageal melanomas. This case also documents the occurrence of PMME in a patient positive for human immunodeficiency virus. We also provide a review of the relevant literature.

Primary malignant melanoma of the esophagus

Primary malignant melanoma of the esophagus (PMME) is an extremely rare and aggressive tumor, comprising less than 0.2% of all primary esophageal neoplasms. There are fewer than 270 reported cases in worldwide literature, most of which are scattered cases reported. This tumor has usually been reported as a pedunculated, polypoid lesion in the middle and lower third of the esophagus. Hematogenic and lymphogenic metastases are commonly reported for PMME cases. Although surgical resection has been considered as the best possible option, the prognosis has been nonetheless poor, even combined with adjuvant chemotherapy postoperatively, according to the literatures. Here we reported such a case on its clinical presentations and treatment.

Primary malignant melanoma of the esophagus

Primary malignant melanoma of the esophagus is a rare disease, with only 200 cases being reported since this condition became an established clinical entity in 1963. This tumor, which accounts for only 0.1–0.2% of all esophageal neoplasms, is typically aggressive and disseminates early via the bloodstream and lymphatics, with only some 30% of patients surviving > 1 year after diagnosis. Management of patients with esophageal melanomata is unsatisfactory, as most tumors are advanced at diagnosis, and therapeutic options are limited by inaccessibility and early dissemination of the neoplasms. Poor survival rates reflect the inoperability of many tumors and the ineffectiveness of radiation and chemotherapy in eradicating advanced tumors and metastases. We present two patients with primary melanoma of the esophagus and discuss the treatment options currently available.

Two cases of early-stage esophageal malignant melanoma with long-term survival

Herein is described two patients with early-stage primary malignant melanoma of the esophagus with long-term survival who were treated with esophagectomy. Both tumors had similar pathological findings, and were mainly at the stage of radial growth phase. Widespread melanoses were present in the mucosa surrounding the tumors in both cases. The two patients recovered uneventfully after surgery and were in remission at follow up of 33 months and 53 months.