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Cazzato G, Cascardi E, Colagrande A, Lettini T, Resta L, Bizzoca C, Arezzo F, Loizzi V, Dellino M, Cormio G, Casatta N, Lupo C, Scillimati A, Scacco S, Parente P, Lospalluti L, Ingravallo G. The Thousand Faces of Malignant Melanoma: A Systematic Review of the Primary Malignant Melanoma of the Esophagus. Cancers (Basel) 2022; 14:3725. [PMID: 35954389 PMCID: PMC9367585 DOI: 10.3390/cancers14153725] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2022] [Revised: 07/27/2022] [Accepted: 07/28/2022] [Indexed: 02/01/2023] Open
Abstract
Primary Malignant Melanoma of the Esophagus (PMME) is an extremely rare cancer of the esophagus, accounting for 0.1−0.8% of all oro-esophageal cancers and <0.05% of all melanoma subtypes, with an estimated incidence of 0.0036 cases per million/year. We conduct a careful analysis of the literature starting from 1906 to the beginning of 2022, searching the PubMed, Science.gov, Scopus and Web of Science (WoS) databases. A total of 457 records were initially identified in the literature search, of which 17 were duplicates. After screening for eligibility and inclusion criteria, 303 publications were ultimately included, related to 347 patients with PMME. PMME represents a very rare entity whose very existence has been the subject of debate for a long time. Over time, an increasing number of cases have been reported in the literature, leading to an increase in knowledge and laying the foundations for a discussion on the treatment of this pathology, which still remains largely represented by surgery. In recent times, the possibility of discovering greater mutations in gene hotspots has made it possible to develop new therapeutic strategies of which nivolumab is an example. Future studies with large case series, with clinicopathological and molecular data, will be necessary to improve the outcome of patients with PMME.
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Affiliation(s)
- Gerardo Cazzato
- Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari “Aldo Moro”, 70124 Bari, Italy; (A.C.); (T.L.); (L.R.); (G.I.)
| | - Eliano Cascardi
- Department of Medical Sciences, University of Turin, 10124 Turin, Italy;
- Pathology Unit, FPO-IRCCS Candiolo Cancer Institute, Str. Provinciale 142 lm 3.95, 10060 Candiolo, Italy
| | - Anna Colagrande
- Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari “Aldo Moro”, 70124 Bari, Italy; (A.C.); (T.L.); (L.R.); (G.I.)
| | - Teresa Lettini
- Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari “Aldo Moro”, 70124 Bari, Italy; (A.C.); (T.L.); (L.R.); (G.I.)
| | - Leonardo Resta
- Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari “Aldo Moro”, 70124 Bari, Italy; (A.C.); (T.L.); (L.R.); (G.I.)
| | - Cinzia Bizzoca
- Section of General Surgery, Azienda Ospedaliero-Universitaria Policlinico di Bari, 70124 Bari, Italy;
| | - Francesca Arezzo
- Section of Gynecology and Obstetrics, Department of Biomedical Sciences and Human Oncology (DIMO), University of Bari “Aldo Moro”, 70124 Bari, Italy; (F.A.); (V.L.); (M.D.)
| | - Vera Loizzi
- Section of Gynecology and Obstetrics, Department of Biomedical Sciences and Human Oncology (DIMO), University of Bari “Aldo Moro”, 70124 Bari, Italy; (F.A.); (V.L.); (M.D.)
| | - Miriam Dellino
- Section of Gynecology and Obstetrics, Department of Biomedical Sciences and Human Oncology (DIMO), University of Bari “Aldo Moro”, 70124 Bari, Italy; (F.A.); (V.L.); (M.D.)
| | - Gennaro Cormio
- Oncology Unit IRCSS Istituto Tumori “Giovanni Paolo II”, Department of Interdisciplinary Medicine (DIM), University of Bari “Aldo Moro”, 70124 Bari, Italy;
| | - Nadia Casatta
- Innovation Department, Diapath S.p.A., Via Savoldini n.71, 24057 Martinengo, Italy; (N.C.); (C.L.)
| | - Carmelo Lupo
- Innovation Department, Diapath S.p.A., Via Savoldini n.71, 24057 Martinengo, Italy; (N.C.); (C.L.)
| | - Antonio Scillimati
- Department of Pharmacy-Pharmaceutical Sciences, University of Bari “Aldo Moro”, 70124 Bari, Italy;
| | - Salvatore Scacco
- Department of Basic Medical Sciences and Neurosciences, University of Bari “Aldo Moro”, 70124 Bari, Italy;
| | - Paola Parente
- Unit of Pathology, Fondazione IRCCS Ospedale Casa Sollievo della Sofferenza, 71013 San Giovanni Rotondo, Italy;
| | - Lucia Lospalluti
- Section of Dermatology and Venereology, Department of Biomedical Sciences and Human Oncology (DIMO), University of Bari “Aldo Moro”, 70124 Bari, Italy;
| | - Giuseppe Ingravallo
- Section of Pathology, Department of Emergency and Organ Transplantation (DETO), University of Bari “Aldo Moro”, 70124 Bari, Italy; (A.C.); (T.L.); (L.R.); (G.I.)
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Dai Y, Zhang Y, Chen Y, Fan X, Lin H, Pan J. Primary malignant melanoma in gastroesophageal junction with 36 months' recurrence-free: a case report. AME Case Rep 2022; 6:22. [PMID: 35928577 PMCID: PMC9343968 DOI: 10.21037/acr-21-83] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2021] [Accepted: 04/02/2022] [Indexed: 11/06/2022]
Abstract
BACKGROUND Melanoma is a sinister malignant tumor originates from melanocytes and is characterized by the presence of black pigmentation in the tissue. The vast majority of melanomas are cutaneous melanomas, and primary mucosal melanomas originating from the esophagus are extremely rare. Primary malignant melanoma of esophagus (PMME) accounts for 0.1% to 0.2% of all primary esophageal malignancies. PMME possess high invasiveness but are insensitive to various treatments, so the prognosis is disappointing. Most literature reported that patients are prone to death from complications of tumor metastasis soon, even they undergo radical surgery. CASE DESCRIPTION In this case report, we admitted a 67-year-old female patient with recurrent chest tightness for 2 years and chest pain for 15 days on October 4, 2017. Preoperative imaging examinations, including computerized tomography (CT) and upper gastrointestinal examination by barium revealed stenosis of the lower esophagus and the fundus of the stomach, with mucosa destruction and lymph node metastasis in the hepatic-gastric space. A laparoscope assisted total gastrectomy with D2 lymph node resection and Roux-en-Y anastomosis was performed without adjuvant immunotherapy or targeted therapies. Postoperative pathological examination and immunohistochemical staining indicated malignant melanoma. Meanwhile we did not find a cutaneous lesion, this patient was therefore diagnosed with a rare PMME. There was no sign of recurrence or metastasis during the latest follow-up of 36 months after the operation, which also exceeded the median recurrence-free survival time in the existing cases worldwide. CONCLUSIONS Therefore, we recommend early radical surgery, which may be beneficial to the PMME patient.
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Affiliation(s)
- Yili Dai
- Department of General Surgery, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Yiyin Zhang
- Department of General Surgery, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Yongle Chen
- Department of General Surgery, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Xiaoxiao Fan
- Department of General Surgery, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Hui Lin
- Department of General Surgery, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Junhai Pan
- Department of General Surgery, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- Zhejiang Engineering Research Center of Cognitive Healthcare, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China
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Hashimoto T, Makino T, Yamasaki M, Tanaka K, Miyazaki Y, Takahashi T, Kurokawa Y, Motoori M, Kimura Y, Nakajima K, Morii E, Mori M, Doki Y. Clinicopathological characteristics and survival of primary malignant melanoma of the esophagus. Oncol Lett 2019; 18:1872-1880. [PMID: 31423256 PMCID: PMC6614672 DOI: 10.3892/ol.2019.10519] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2019] [Accepted: 05/13/2019] [Indexed: 11/06/2022] Open
Abstract
Primary malignant melanoma of the esophagus (PMME) has been reported to be a rare and highly malignant disease, and to date a standard treatment strategy has not been established due to limited evidence. The aim of the present study was to investigate the clinicopathological characteristics of this extremely rare disease. A total of 6 out of 2,093 patients with PMME treated in our institution between 1995 and 2016 were retrospectively analyzed and their clinicopathological parameters including treatment course and long-term survival were investigated. The major clinicopathological characteristics of patients were that they were >70 years of age, male sex, dysphagia at first diagnosis, and macroscopic black protruding tumors located in the lower third of the thoracic esophagus. Four of the five patients receiving pretherapeutic endoscopic biopsy were correctly diagnosed with PMME, and two patients received preoperative treatment with ineffective histopathological responses. There were two unresectable cases, one was treated with an immune-checkpoint inhibitor and the other received palliative care. Three of the four patients receiving curative surgery developed hematogenous recurrence within two years of surgery and only one patient with pT1aN0M0 achieved long-term survival. The median overall survival of all six patients was 19.6 (6.4–40.5) months. Patients with stage I disease exhibited significantly more favorable prognoses than those with stage II–IV (P=0.025) and surgically-treated patients had significantly better prognoses than those who did not receive surgery (P=0.018). In conclusion, PMME was associated with highly malignant features and tended to develop hematogenous metastases even after radical resection. Early diagnosis appears to be important to cure this refractory disease.
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Affiliation(s)
- Tadayoshi Hashimoto
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan
| | - Tomoki Makino
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan
| | - Makoto Yamasaki
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan
| | - Koji Tanaka
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan
| | - Yasuhiro Miyazaki
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan
| | - Tsuyoshi Takahashi
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan
| | - Yukinori Kurokawa
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan
| | - Masaaki Motoori
- Department of Surgery, Osaka General Medical Center, Osaka 558-8558, Japan
| | - Yutaka Kimura
- Department of Surgery, Kindai University Faculty of Medicine, Osaka 589-8511, Japan
| | - Kiyokazu Nakajima
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan
| | - Eiichi Morii
- Department of Pathology, Graduate School of Medicine, Osaka University, Osaka 565-0871, Japan
| | - Masaki Mori
- Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan
| | - Yuichiro Doki
- Department of Gastroenterological Surgery, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan
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Ling C, Feng J, Li J, Liu Q. Primary malignant melanoma of the esophagus. TURKISH JOURNAL OF GASTROENTEROLOGY 2018; 29:711-713. [PMID: 30289400 DOI: 10.5152/tjg.2018.18065] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Affiliation(s)
- Chunxiang Ling
- Department of Radiology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China
| | - Jizhen Feng
- Department of Radiology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China
| | - Jiamei Li
- Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China
| | - Qingwei Liu
- Department of Radiology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China
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Izrailov RE, Titov KS, Kononets PV, Koshkin MA, Rotin DL, Drapun SV. [Primary esophageal melanoma]. Khirurgiia (Mosk) 2018:110-111. [PMID: 29799002 DOI: 10.17116/hirurgia20185110-111] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Affiliation(s)
- R E Izrailov
- Moscow Clinical Scientific and Practical Center of Moscow Healthcare Department, Moscow, Russia
| | - K S Titov
- Moscow Clinical Scientific and Practical Center of Moscow Healthcare Department, Moscow, Russia
| | - P V Kononets
- Moscow City Clinical Hospital No.62, Moscow Healthcare Department, Moscow, Russia
| | - M A Koshkin
- Moscow Clinical Scientific and Practical Center of Moscow Healthcare Department, Moscow, Russia
| | - D L Rotin
- Moscow Clinical Scientific and Practical Center of Moscow Healthcare Department, Moscow, Russia
| | - S V Drapun
- Moscow Clinical Scientific and Practical Center of Moscow Healthcare Department, Moscow, Russia
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Ahn JY, Hwang HS, Park YS, Kim HR, Jung HY, Kim JH, Lee SE, Kim MA. Endoscopic and pathologic findings associated with clinical outcomes of melanoma in the upper gastrointestinal tract. Ann Surg Oncol 2014; 21:2532-9. [PMID: 24633670 DOI: 10.1245/s10434-014-3637-2] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/24/2013] [Indexed: 11/18/2022]
Abstract
BACKGROUND Melanoma that involves the upper gastrointestinal (GI) tract is rare and studies relating to endoscopic and pathologic findings with clinical outcomes are lacking. We reviewed the gross and microscopic patterns of the upper GI tract in primary and metastatic melanoma, and examined their association with clinical outcomes. METHODS Twenty-nine cases of primary esophageal (n = 19) and metastatic gastric and/or duodenal melanoma (n = 10) that were detected during upper GI endoscopy between 1995 and 2011 were retrospectively analyzed. RESULTS Three types of gross patterns were recognized-nodular pattern in 7 cases, mass-forming pattern in 18 cases, and flat pigmented pattern in 4 cases. In primary esophageal melanoma, 13 patients (68.4 %) underwent surgery and 9 received palliative therapy. Of all cases, 22 patients (75.9 %) died of disease progression; the median overall survival period was 12 months (interquartile range [IQR] 4.5-24.5 months), and from recognition of upper GI tract melanoma the median overall survival period was 9 months (IQR 3.5-17.0 months). In primary esophageal cases, skin melanoma stage better discriminated the patients with good prognosis than the esophageal cancer stage. The flat pigmented gross pattern proved to be a good prognostic factor in primary and metastatic GI tract melanomas (p = 0.016 and p = 0.046, respectively). CONCLUSIONS Melanoma of the GI tract is a highly aggressive disease with a poor prognosis, both in primary and metastatic cases. However, in primary esophageal melanoma, careful inspection of the mucosa during endoscopic examination followed by surgical resection may result in extended survival.
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Affiliation(s)
- Ji Yong Ahn
- Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Li YH, Li X, Zou XP. Primary malignant melanoma of the esophagus: A case report. World J Gastroenterol 2014; 20:2731-2734. [PMID: 24627611 PMCID: PMC3949284 DOI: 10.3748/wjg.v20.i10.2731] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2013] [Revised: 11/12/2013] [Accepted: 01/06/2014] [Indexed: 02/06/2023] Open
Abstract
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.
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Timmers TK, Schadd EM, Monkelbaan JF, Meij V. Survival after resection of a primary malignant melanoma of the small intestine in a young patient: report of a case. Case Rep Gastroenterol 2013; 7:251-60. [PMID: 23874263 PMCID: PMC3712819 DOI: 10.1159/000352017] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
The occurrence of primary melanoma of the small intestine is rare. We describe the case of a 25-year-old man found to have a primary melanoma of the ileum. The patient presented with gradual onset of abdominal pain, fever, diarrhea, weight loss and fatigue. A preoperative diagnosis of a small intestinal tumor was based on the findings of computed tomography scanning. This diagnosis was confirmed at laparoto-my and a partial small bowel resection was performed. Histopathological examination of the resected specimen clarified the exact nature of the lesion, confirming the diagnosis of melanoma. Thorough postoperative investigation did not reveal a primary lesion in the skin, gastrointestinal tract, oculus or brain. Thus, we diagnosed this tumor as a primary lesion. One year after his operation, the patient remains well without any evidence of recurrence. Thus, we diagnosed this small bowel tumor as a primary melanoma of the small intestine.
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Affiliation(s)
- T K Timmers
- Department of Surgery, University Medical Center Utrecht, Utrecht, The Netherlands
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Ishizaki M, Aibara Y, Furuya K. Primary malignant melanoma of the esophagogastric junction: Report of a case. Int J Surg Case Rep 2013; 4:700-3. [PMID: 23810918 DOI: 10.1016/j.ijscr.2013.04.037] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2013] [Revised: 04/15/2013] [Accepted: 04/17/2013] [Indexed: 11/18/2022] Open
Abstract
INTRODUCTION Primary malignant melanoma of the gastrointestinal tract is very rare, especially in the stomach. We report an extremely rare case of primary malignant melanoma of the esophagogastric junction mainly situated in the stomach. PRESENTATION OF CASE The patient was a 72-year-old woman who complained of shortness of breath due to severe anemia. Upper endoscopy revealed a soft easy-bleeding polypoid tumor just adjacent to the esophagogastric junction in the stomach. Biopsy of the tumor did not indicate a definite result, except malignant tumor. We performed total gastrectomy with splenectomy, and histological and immunohistological examination revealed malignant melanoma of the esophagogastric junction. She had no remote metastasis or lymphnodal metastasis at the point of surgery; however, she died of multiple metastases 11 months after the operation. DISCUSSION A definite preoperative diagnosis of primary malignant melanoma was very difficult to make from the preoperative biopsy specimen. This present case was first misinterpreted as undifferentiated carcinoma, or malignant lymphoma. Following the diagnosis of malignant melanoma, the question arose as to whether this was primary or metastatic (as malignant melanoma from other sites is known to metastasize to the stomach). Finally this tumor was diagnosed as a primary one due to the pathologic characteristics such as the existence of junctional activities. CONCLUSION We report an extremely rare case of primary malignant melanoma of the esophagogastric junction present in the stomach.
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Affiliation(s)
- Masahiro Ishizaki
- Department of Surgery, Okayama Rosai Hospital, 1-10-25 Chikkou-midorimachi, Minami Ward, Okayama 702-8055, Japan.
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Arora A, Javed A, Agarwal AK. Primary Malignant Melanoma of the Esophagus. Oman Med J 2013; 28:e040. [PMID: 31435466 PMCID: PMC6667805 DOI: 10.5001/omj.2013.20] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2012] [Accepted: 12/02/2012] [Indexed: 11/03/2022] Open
Abstract
Primary malignant melanoma of the esophagus is an extremely rare neoplasm arising from the esophageal mucosal melanocytes. We herein describe a patient of primary malignant melanoma of the esophagus, who was managed by thoracolaparoscopic esophagectomy.
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Affiliation(s)
- Asit Arora
- Department of GI Surgery, GB Pant Hospital & MAM College, Delhi University, New Delhi, India
| | - Amit Javed
- Department of GI Surgery, GB Pant Hospital & MAM College, Delhi University, New Delhi, India
| | - Anil K. Agarwal
- Department of GI Surgery, GB Pant Hospital & MAM College, Delhi University, New Delhi, India
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Grilliot MA, Goldblum JR, Liu X. Signet-ring cell melanoma of the gastroesophageal junction: a case report and literature review. Arch Pathol Lab Med 2012; 136:324-8. [PMID: 22372909 DOI: 10.5858/arpa.2011-0042-cr] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
We report the first case, to our knowledge, of a possible primary, signet-ring cell melanoma of the gastroesophageal junction. The mass was initially diagnosed as an invasive, poorly differentiated carcinoma; however, on further review and immunohistochemical workup, the diagnosis of signet-ring cell melanoma was made. The lesion consisted of oval to round epithelioid cells undermining the gastric mucosa and infiltrating the muscularis mucosae. Tumor cells demonstrated abundant cytoplasm and eccentrically located nuclei, many with signet-ring cell morphology. The tumor cells were negative for mucin and pancytokeratin, strongly positive for S100 protein and Melan-A, and focally but strongly positive for human melanoma black-45. Diagnostic imaging failed to prove another site of melanoma, and no history of melanoma or cutaneous lesion was reported by the patient. Therefore, it was determined this was likely a primary lesion. We review the literature and previously reported cases of this rare histologic variant of melanoma.
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12
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Kim SG. [Primary malignant melanoma of the esophagus]. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2011; 57:262-4. [PMID: 21519182 DOI: 10.4166/kjg.2011.57.4.262] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/03/2022]
Affiliation(s)
- Sang Gyun Kim
- Department of Internal Medicine and Liver Research Institute, Seoul National University College of Medicine, Seoul, Korea
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Yu H, Huang XY, Li Y, Xie X, Zhou JL, Zhang LJ, Fu JH, Wang X. Primary malignant melanoma of the esophagus: a study of clinical features, pathology, management and prognosis. Dis Esophagus 2011; 24:109-13. [PMID: 21040150 DOI: 10.1111/j.1442-2050.2010.01111.x] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
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.
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Affiliation(s)
- H Yu
- State Key Laboratory of Oncology in South China, Department of Thoracic Surgery, Cancer Center, Sun Yat-Sen University, Guangzhou, China
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Eyles J, Puaux AL, Wang X, Toh B, Prakash C, Hong M, Tan TG, Zheng L, Ong LC, Jin Y, Kato M, Prévost-Blondel A, Chow P, Yang H, Abastado JP. Tumor cells disseminate early, but immunosurveillance limits metastatic outgrowth, in a mouse model of melanoma. J Clin Invest 2010; 120:2030-9. [PMID: 20501944 DOI: 10.1172/jci42002] [Citation(s) in RCA: 383] [Impact Index Per Article: 25.5] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2009] [Accepted: 04/21/2010] [Indexed: 12/31/2022] Open
Abstract
Although metastasis is the leading cause of cancer-related death, it is not clear why some patients with localized cancer develop metastatic disease after complete resection of their primary tumor. Such relapses have been attributed to tumor cells that disseminate early and remain dormant for prolonged periods of time; however, little is known about the control of these disseminated tumor cells. Here, we have used a spontaneous mouse model of melanoma to investigate tumor cell dissemination and immune control of metastatic outgrowth. Tumor cells were found to disseminate throughout the body early in development of the primary tumor, even before it became clinically detectable. The disseminated tumor cells remained dormant for varying periods of time depending on the tissue, resulting in staggered metastatic outgrowth. Dormancy in the lung was associated with reduced proliferation of the disseminated tumor cells relative to the primary tumor. This was mediated, at least in part, by cytostatic CD8+ T cells, since depletion of these cells resulted in faster outgrowth of visceral metastases. Our findings predict that immune responses favoring dormancy of disseminated tumor cells, which we propose to be the seed of subsequent macroscopic metastases, are essential for prolonging the survival of early stage cancer patients and suggest that therapeutic strategies designed to reinforce such immune responses may produce marked benefits in these patients.
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Affiliation(s)
- Jo Eyles
- Singapore Immunology Network, BMSI, A-STAR, Singapore
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Ellis ZM, Jassim AD, Wick MR. Anorectal melanoma in childhood and adolescence. Ann Diagn Pathol 2010; 14:69-73. [DOI: 10.1016/j.anndiagpath.2009.07.003] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2009] [Revised: 07/13/2009] [Accepted: 07/16/2009] [Indexed: 12/28/2022]
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Terada T. Amelanotic malignant melanoma of the esophagus: report of two cases with immunohistochemical and molecular genetic study of KIT and PDGFRA. World J Gastroenterol 2009. [PMID: 19496203 DOI: pmid/19496203] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/10/2023] Open
Abstract
The author reports herein two cases of amelanotic malignant melanoma of the esophagus. Case 1 is an 87-year-old woman who was admitted to our hospital because of nausea and vomiting. Endoscopic examination revealed an ulcerated tumor of the distal esophagus, and a biopsy was taken. The biopsy showed malignant polygonal and spindle cells. No melanin pigment was recognized. Immunohistochemically, the tumor cells were positive for melanosome (HMB45), S100 protein, KIT and Platelet derived growth factor receptor-alpha (PDGFRA). The patient was treated by chemotherapy and radiation, but died of systemic metastasis 12 mo after the presentation. Case 2 is a 56-year-old man presenting with dysphagia. Endoscopic examination revealed a polypoid tumor in the middle esophagus, and a biopsy was obtained. The biopsy showed malignant spindle cells without melanin pigment. Immunohistochemically, the tumor cells were positively labeled for melanosome, S100 protein, KIT and PDGFRA. The patient refused operation, and was treated by palliative chemotherapy and radiation. He died of metastasis 7 mo after the admission. In both cases, molecular genetic analyses of KIT gene (exons 9, 11, 13 and 17) and PDGFRA gene (exons 12 and 18) were performed by the PCR direct sequencing method, which showed no mutations of KIT and PDGFRA genes. This is the first report of esophageal malignant melanoma with an examination of the expression of KIT and PDGFRA and the mutational status of KIT and PDGFRA genes.
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Affiliation(s)
- Tadashi Terada
- Department of Pathology, Shizuoka City Shimizu Hospital, Miyakami 1231 Shimizu-Ku, Shizuoka 424-8636, Japan.
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Terada T. Amelanotic malignant melanoma of the esophagus: Report of two cases with immunohistochemical and molecular genetic study of KIT and PDGFRA. World J Gastroenterol 2009; 15:2679-83. [PMID: 19496203 PMCID: PMC2691504 DOI: 10.3748/wjg.15.2679] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
The author reports herein two cases of amelanotic malignant melanoma of the esophagus. Case 1 is an 87-year-old woman who was admitted to our hospital because of nausea and vomiting. Endoscopic examination revealed an ulcerated tumor of the distal esophagus, and a biopsy was taken. The biopsy showed malignant polygonal and spindle cells. No melanin pigment was recognized. Immunohistochemically, the tumor cells were positive for melanosome (HMB45), S100 protein, KIT and Platelet derived growth factor receptor-α (PDGFRA). The patient was treated by chemotherapy and radiation, but died of systemic metastasis 12 mo after the presentation. Case 2 is a 56-year-old man presenting with dysphagia. Endoscopic examination revealed a polypoid tumor in the middle esophagus, and a biopsy was obtained. The biopsy showed malignant spindle cells without melanin pigment. Immunohistochemically, the tumor cells were positively labeled for melanosome, S100 protein, KIT and PDGFRA. The patient refused operation, and was treated by palliative chemotherapy and radiation. He died of metastasis 7 mo after the admission. In both cases, molecular genetic analyses of KIT gene (exons 9, 11, 13 and 17) and PDGFRA gene (exons 12 and 18) were performed by the PCR direct sequencing method, which showed no mutations of KIT and PDGFRA genes. This is the first report of esophageal malignant melanoma with an examination of the expression of KIT and PDGFRA and the mutational status of KIT and PDGFRA genes.
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18
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Cheung MC, Perez EA, Molina MA, Jin X, Gutierrez JC, Franceschi D, Livingstone AS, Koniaris LG. Defining the role of surgery for primary gastrointestinal tract melanoma. J Gastrointest Surg 2008; 12:731-8. [PMID: 18058185 DOI: 10.1007/s11605-007-0417-3] [Citation(s) in RCA: 93] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/22/2007] [Accepted: 10/31/2007] [Indexed: 01/31/2023]
Abstract
OBJECTIVE The objective of the study was to determine the outcomes for primary gastrointestinal melanomas (PGIM). MATERIAL AND METHODS The Surveillance, Epidemiology, and End Results database (1973-2004) was queried. RESULTS Overall, 659 cases of PGIM were identified. The annual incidence of PGIM was approximately 0.47 cases per million in 2000. Overall median survival time was 17 months. Tumors were identified in the oral-nasopharynx (32.8%), anal canal (31.4%), rectum (22.2%), esophagus (5.9%), stomach (2.7%), small bowel (2.3%), gallbladder (1.4%), and large bowel (0.9%). Univariate analysis demonstrated age, tumor location, stage, surgery, and lymph node status were significant predictors of improved survival. MST has not been reached for tumors located in the large bowel, while tumors located in the stomach demonstrated the shortest median survival (5 months). Improvement in MST was observed for those patients undergoing surgical resection. The presence of lymph node involvement conferred a poorer prognosis. Multivariate analysis of the cohort identified that location, advanced tumor stage, failure to undertake surgical resection, positive lymph node status, and age were all independent predictors of poorer outcome. CONCLUSION PGIM occurs most often in the oral-nasopharynx and anal canal. Surgical extirpation is the only identifiable treatment modality that significantly improves survival.
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Affiliation(s)
- Michael C Cheung
- Division of Surgical Oncology, DeWitt Daughtry Family Department of Surgery, University of Miami Miller School of Medicine, Miami, FL 33136, USA
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19
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Abstract
Primary malignant melanoma of the esophagus (PMME) is an extremely rare tumor with only scattered cases reported. Although surgical resection has been considered as the best possible option, the prognosis has been nonetheless poor. We report a case of PMME which was treated by surgical resection and additionally followed by chemotherapy. A 60-yr-old man underwent an esophagoscopy due to a 3-month history of dysphagia and upper abdominal discomfort. A pigmented polypoid mass in the lower third of the esophagus was discovered, and a biopsy identified the mass as a malignant melanoma. Consequently, a subtotal esophagectomy and intrathoracic esophagogastrostomy was carried out. At follow-up four months after discharge, lymph node enlargements in the cervical area and celiac axis area were found. As a result, the patients was started on systemic chemotherapy treatment, which included Dacarbazine. The patient has been doing well and is now 35 months post-operative.
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Affiliation(s)
- Song Am Lee
- Department of Thoracic and Cardiovascular Surgery, Konkuk University Hospital, Seoul, Korea
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20
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Tarantino L, Nocera V, Perrotta M, Balsamo G, Schiano A, Orabona P, Sordelli IFM, Ripa C, Parmeggiani D, Sperlongano P. Primary small-bowel melanoma: color Doppler ultrasonographic, computed tomographic, and radiologic findings with pathologic correlations. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2007; 26:121-7. [PMID: 17182718 DOI: 10.7863/jum.2007.26.1.121] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/13/2023]
Affiliation(s)
- Luciano Tarantino
- Interventional Ultrasound Unit, Department of Internal Medicine, San Giovanni di Dio Hospital, Frattamaggiore, Italy.
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21
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Abstract
Primary malignant melanoma originating in the digestive tract is extremely rare. A case of primary malignant melanoma in the descending colon is described. The tumor was an elevated mass with surface necrosis. Histologically, tumor cells were arranged with compact nests surrounded by fibrous stroma. The tumor cells had pleomorphic nuclei and rich cytoplasm. In some areas, cells of signet ring-like appearance were found. An immunohistochemical examination showed that most of the tumor cells were positive for S-100 protein, HMB-45, melan-A, vimentin and CD38. Ultrastructural examination confirmed some premelanosomes. EWS-ATF-1 fusion transcript, which is usually detected in clear cell sarcoma, was not demonstrated on reverse transcriptase-polymerase chain reaction. Because there was no evidence of either cutaneous or ocular primary melanoma, the tumor was thus diagnosed as primary colonic malignant melanoma. The patient has remained free of recurrent disease for 3 years after a surgical resection. Colonic malignant melanoma must be differentiated from other intestinal tumor, and the possibility of metastasis from another more common primary site must be ruled out.
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Affiliation(s)
- Daisuke Mori
- Department of Pathology and Biodefense, Faculty of Medicine, Saga University, Saga, Japan.
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22
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Liang KV, Sanderson SO, Nowakowski GS, Arora AS. Metastatic malignant melanoma of the gastrointestinal tract. Mayo Clin Proc 2006; 81:511-6. [PMID: 16610571 DOI: 10.4065/81.4.511] [Citation(s) in RCA: 92] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
Malignant melanoma is one of the most common malignancies to metastasize to the gastrointestinal (GI) tract. Metastases to the GI tract can present at the time of primary diagnosis or decades later as the first sign of recurrence. Symptoms may include abdominal pain, dysphagia, small bowel obstruction, hematemesis, and melena. We report 2 cases of malignant melanoma metastatic to the GI tract, followed by a review of the literature. The first case is a 72-year-old man who underwent resection of superficial spreading melanoma on his back 13 years previously who presented with dysphagia. A biopsy specimen of a mucosal fold in a gastric fundus noted during endoscopy was taken and revealed metastatic malignant melanoma, which was resected 1 month later. Three weeks later, the patient was found to have an ulcerated jejunal metastatic melanoma mass, which was also resected. The second case is a 63-year-old man with an ocular melanoma involving the chorold of the left eye that had been diagnosed 4 years previously, which had been excised several times, who presented with anorexia, dizziness, and fatigue. He was found to have cerebellar and stomach metastases. He underwent adjuvant radiation therapy, chemotherapy, and surgical resection of the gastric melanoma metastasis. In patients with a history of melanoma, a high index of suspicion for metastasis must be maintained if they present with seemingly unrelated symptoms. Diagnosis requires careful inspection of the mucosa for metastatic lesions and biopsy with special immunohistochemical stains. Management may include surgical resection, chemotherapy, immunotherapy, observation, or enrollment in clinical trials. Prognosis is poor, with a median survival of 4 to 6 months.
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Affiliation(s)
- Kelly V Liang
- Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
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23
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Huang W, Zhang X, Li D, Chen J, Meng K, Wang Y, Lu Z, Zhou X. Osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcoma of soft parts. Virchows Arch 2005; 448:200-3. [PMID: 16220298 DOI: 10.1007/s00428-005-0051-y] [Citation(s) in RCA: 34] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2005] [Accepted: 07/22/2005] [Indexed: 12/12/2022]
Abstract
Clear cell sarcoma is a high-grade sarcoma with morphological features resembling those of malignant melanoma. An osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts is very rare. Herein, we report an unusual stomach tumor with microscopic and immunohistochemical characteristics of an osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts. The tumor cells were predominantly oval, admixed with some round and spindle elements arranged in nests and fascicles, and admixed with scattered osteoclast-like multinucleated giant cells. Neoplastic cells were positive for S-100 protein, and osteoclast-like multinucleated giant cells were immunoreactive to CD68. The unusual morphology of the tumor caused significant diagnostic difficulties. The differential diagnosis included gastrointestinal stromal tumor, primary or metastatic melanoma, and epithelioid malignant peripheral nerve sheath tumor. To the best of our knowledge, this is possibly the second description of an osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts.
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Affiliation(s)
- Wenbin Huang
- Department of Pathology, Jinling Hospital, Clinical School of Medical College, Nanjing University, 305 Zhongshan East Road, 210002 Nanjing Jiangsu, PR China
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24
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Jelincic Z, Jakic-Razumovic J, Petrovic I, Cavcic AM, Unusic J, Trotic R. Primary malignant melanoma of the stomach. TUMORI JOURNAL 2005; 91:201-3. [PMID: 15948553 DOI: 10.1177/030089160509100219] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
We report a 54-year-old patient with a complaint of weakness, abdominal pain and weight loss. During the clinical examination a palpable tumor resistance in the abdomen was found as well as iron deficiency anemia. Gastroscopy showed an exulcerated, dark brown, fungiform tumor about 4 cm in diameter at the great curve of stomach. Endoscopic biopsy revealed the diagnosis of malignant melanoma by demonstrating the presence of melanin containing tumor cells in gastric mucosa. The patient underwent subtotal gastrectomy, appendectomy and splenectomy. The diagnosis of gastric melanoma with regional lymph node metastases, as well as metastases in appendix adjacent tissue was confirmed by histology and immunohistochemistry. In three years follow up period patient developed cerebral and retroauricular subcutaneous metastases that were treated by surgery, adjuvant chemotherapy and radiotherapy. Finally, an explorative laparatomy was revealed advanced intraabdominal tumor dissemination with dark pigmented ascites. Concerning that all available diagnostic procedures failed to prove other site of melanoma, presented case was considered as primary gastric melanoma as a possible rare site of tumor.
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Affiliation(s)
- Zeljko Jelincic
- Department of Surgery, Clinical Hospital Centre Zagreb, Kispatićeva 12, Zagreb, Croatia
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25
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Uetsuka H, Naomoto Y, Fujiwara T, Shirakawa Y, Noguchi H, Yamatsuji T, Haisa M, Matsuoka J, Gunduz M, Takubo K, Tanaka N. Primary malignant melanoma of the esophagus: long-term survival following pre- and postoperative adjuvant hormone/chemotherapy. Dig Dis Sci 2004; 49:1646-51. [PMID: 15573920 DOI: 10.1023/b:ddas.0000043379.60295.cb] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/09/2022]
Affiliation(s)
- Hirokazu Uetsuka
- Department of Gastroenterological Surgery, Transplant, and Surgical Oncology, Graduate School of Medicine and Dentistry, Okayama University, 2-5-1 Shikatacho, Okayama 700-8558, Japan
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26
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Sudhamshu K C, Kouzu T, Matsutani S, Hishikawa E, Nikaido T, Taro A, Hiromitsu S. Primary malignant melanoma of the esophagus treated with heavy-ion radiotherapy. J Clin Gastroenterol 2003; 37:151-154. [PMID: 12869887 DOI: 10.1097/00004836-200308000-00012] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/09/2022]
Abstract
Primary malignant melanoma of the esophagus (PMME) is an uncommon but aggressive tumor with very poor prognosis. There is no established treatment plan for the disease, which may be attributed to its rarity and aggressiveness. Surgery is the choice of treatment in early cases. Radiotherapy follows surgery, and chemotherapy has an insignificant role in its treatment. Radiation with heavy ion beams is showing promising results in cancer therapy. Compared to conventional radiation, it permits selective irradiation with minimal injury to the surrounding normal tissue, and treatment with a low dose within a short interval of time is possible. We herein report a case of PMME treated with heavy ion radiation, the first case to be reported so far, and review the relevant literature.
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Affiliation(s)
- Sudhamshu K C
- Department of Medicine and Clinical Oncology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba Japan.
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27
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Alazmi WM, Nehme OS, Regalado JJ, Rogers AI. Primary gastric melanoma presenting as a nonhealing ulcer. Gastrointest Endosc 2003; 57:431-3. [PMID: 12612539 DOI: 10.1067/mge.2003.120] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Affiliation(s)
- Waleed M Alazmi
- Division of Gastroenterology, University of Miami, Miami Beach, Florida 33136, USA
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28
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Herman J, Duda M, Lovecek M, Svach I. Primary malignant melanoma of the esophagus treated by endoscopic ablation and interferon therapy. Dis Esophagus 2002; 14:239-40. [PMID: 11869328 DOI: 10.1046/j.1442-2050.2001.00192.x] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Primary malignant melanoma of the esophagus is a rare illness accounting for 0.1-0.2% of malignant diseases of the esophagus; however, the incidence of the disease appears to be rising. The average survival time is between 10 and 15 months. The authors describe the 25 month follow up of a patient with primary malignant melanoma of the esophagus which was treated with endoscopic ablation followed by interferon therapy. No other focus was found and the patient is undergoing regular endoscopic check-ups, currently without any problems.
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Affiliation(s)
- J Herman
- Second Department of Surgery, Teaching Hospital --Medical Faculty, Palacký University Olomouc, Svatoplukova, Olomouc, Czech Republic.
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29
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Abstract
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.
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Affiliation(s)
- H A Archer
- Department of Surgery, Guy's and St Thomas' Hospital Trust, St Thomas' Hospital, Lambeth Palace Road, London SE1 EH7, UK
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30
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Mikami T, Fukuda S, Shimoyama T, Yamagata R, Nishiya D, Sasaki Y, Uno Y, Saito H, Takaya S, Kamata Y, Munakata A. A case of early-stage primary malignant melanoma of the esophagus. Gastrointest Endosc 2001; 53:365-7. [PMID: 11231405 DOI: 10.1016/s0016-5107(01)70420-8] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Affiliation(s)
- T Mikami
- First Department of Internal Medicine, the First Department of Surgery, and Central Pathology, Hirosaki University School of Medicine, Hirosaki, Japan
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31
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Abstract
A case of primary malignant melanoma of the esophagus is reported, followed by a review of the literature. The clinical presentation of this uncommon tumor is similar to esophageal carcinoma and the preoperative diagnosis may be difficult. A total esophagectomy is the treatment of choice. The prognosis remains poor despite aggressive treatment.
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Affiliation(s)
- M de Perrot
- Thoracic Surgery Unit, Department of Surgery, University Hospital of Geneva, Switzerland
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32
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Abstract
Primary melanoma originating in the gastrointestinal tract is very rare and the majority of these tumors arise in the mucosa of the anus or rectum. A case of solitary colonic melanoma in a 79-year-old man is described with a review of pertinent literature. The surgically excised neoplasm was evaluated by routine histology and immunohistochemistry stains. Pathologic examination of the excised cecal mass revealed an 8 x 5-cm tan-pink mass with a central green-black necrotic area. Histologically, there were solid sheets of S100- and HMB-45-positive pigmented cells extending from the mucosal ulcerated surface through the bowel wall. The patient had no evidence of cutaneous or ocular primary melanoma. He remained free of recurrent disease 5 years after surgical resection of the colonic melanoma. The unique pathologic features and clinical outcome support the diagnosis of primary colonic melanoma in this patient.
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Affiliation(s)
- S H Poggi
- Section of Surgical Oncology, Yale School of Medicine, New Haven, Connecticut 06520-8062, USA
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33
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Schuchter LM, Green R, Fraker D. Primary and metastatic diseases in malignant melanoma of the gastrointestinal tract. Curr Opin Oncol 2000; 12:181-5. [PMID: 10750731 DOI: 10.1097/00001622-200003000-00014] [Citation(s) in RCA: 124] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
In this review, the gastrointestinal (GI) manifestations of malignant melanoma including primary mucosal melanoma of the GI tract and metastatic melanoma to the GI tract are discussed. Although malignant melanoma most commonly arises in the skin, primary melanomas can also arise from the mucosal epithelial lining of the gastrointestinal tract. The vast majority of gastrointestinal melanoma is metastatic from a cutaneous primary; however, there is evidence that melanoma can arise de novo from within certain areas of the gastrointestinal system. The sporadic nature and small numbers of patients reported in the literature with mucosal melanomas have prevented a good understanding of the pathogenesis, natural history, and optimal treatment of this uncommon presentation of melanoma.
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Affiliation(s)
- L M Schuchter
- University of Pennsylvania Cancer Center, Philadelphia 19104, USA
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34
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Mosnier JF, Balique JG. Pleomorphic giant cell carcinoma of the esophagus with coexpression of cytokeratin and vimentin and neuroendocrine differentiation. Arch Pathol Lab Med 2000; 124:135-8. [PMID: 10629146 DOI: 10.5858/2000-124-0135-pgccot] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
A pleomorphic (giant cell) carcinoma of the esophagus is reported in a 52-year-old man who had dysphagia and weakness. The 8-cm-high vegetating tumor consisted of solid sheets of poorly cohesive epithelioid cells broken into clusters by strands of stroma. Numerous giant cells showing phagocytic phenomenon were present. Immunochemical analyses demonstrated the epithelial origin of the neoplasm, although most of the tumor cells strongly expressed vimentin. Numerous tumor cells expressed synaptophysin. Neurosecretory granules were detected in some tumor cells on electron microscopic examination. The patient died 4 months after he became symptomatic. As far as we can ascertain, this is the first case report describing a pleomorphic carcinoma arising in the esophagus. This poorly differentiated carcinoma might be of neuroendocrine differentiation. In the esophagus, pleomorphic carcinoma must be distinguished from polypoid tumors such as carcinosarcoma and malignant melanoma.
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Affiliation(s)
- J F Mosnier
- Departments of Pathology, Hôpital de Bellevue, CHU de Saint-Etienne, France
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35
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Naomoto Y, Perdomo JA, Kamikawa Y, Haisa M, Yamatsuji T, Kenzo A, Taguchi K, Hara K, Tanaka N. Primary malignant melanoma of the esophagus: report of a case successfully treated with pre- and post-operative adjuvant hormone-chemotherapy. Jpn J Clin Oncol 1998; 28:758-61. [PMID: 9879295 DOI: 10.1093/jjco/28.12.758] [Citation(s) in RCA: 23] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
Primary malignant melanoma of the esophagus is a very rare and deadly disease, with a survival of 2.2% at 5 years and a median survival of about 10 months. The aggressive biological behavior of this disease and advanced stage at the time of diagnosis together with the lack of effective treatment have contributed to its poor prognosis. We present the case of a 45-year-old Japanese man diagnosed as having a primary melanoma of the esophagus by clinical evaluation and a histological examination of endoscopic biopsy specimens. A novel approach consisting of pre- and post-operative chemo-hormone therapy with dacarbazine, nimustine, cisplatin and tamoxifen in conjunction with radical esophagectomy accompanied by lymph node dissection was carried out. The tumor size was decreased to 70% by the pre-operative chemo-hormone therapy. During the post-operative 32 months of follow-up, no evidence of recurrence or metastatic disease has been found. Although this is only one case, the outcome observed suggests that the combination of pre- and post-operative chemo-hormone therapy and radical esophagectomy with lymph node dissection is a modality that can increase the possibility of curability or at least improve the survival of patients with primary melanoma of the esophagus.
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Affiliation(s)
- Y Naomoto
- First Department of Surgery, Okayama University Medical School, Japan
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36
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Abstract
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.
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Affiliation(s)
- P DeMatos
- Department of Surgery, Duke University Medical Center, Durham, North Carolina 27710, USA.
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37
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Affiliation(s)
- S Kinuya
- Department of Nuclear Medicine, Kanazawa University School of Medicine, Japan
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38
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39
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Wade TP, Goodwin MN, Countryman DM, Johnson FE. Small bowel melanoma: extended survival with surgical management. Eur J Surg Oncol 1995; 21:90-1. [PMID: 7851566 DOI: 10.1016/s0748-7983(05)80078-2] [Citation(s) in RCA: 23] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/27/2023] Open
Abstract
Small intestinal melanoma is rare, and primary vs metastatic origin is often unclear. A patient with the longest reported survival (21 years) after resection of a melanoma in the small intestine is presented, and the debate regarding primary small bowel melanoma is reviewed. Multiple resections of neck recurrence (or second primaries) in this patient with prolonged survival validates the aggressive pursuit and excision of localized melanoma when possible.
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Affiliation(s)
- T P Wade
- Department of Surgery, USAF Medical Center Scott Air Force Base, IL
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40
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Haga Y, Iwanaga Y, Matsumura F, Nakamura T, Ohtsu S, Yagi Y, Nakamura Y, Tanimura M. Curatively resected primary malignant melanoma of the esophagus: report of a case. Surg Today 1993; 23:820-4. [PMID: 8219617 DOI: 10.1007/bf00311627] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2023]
Abstract
We report herein a rare case of primary esophageal malignant melanoma in a 49-year-old Japanese woman who presented with a 3-week history of dysphagia. Esophagogram and esophagoscopy demonstrated a polypoid tumor in the upper to mid-thoracic portion of the esophagus, and a radical subtotal esophagectomy was performed through a right thoracotomy. Histological and immunohistochemical studies proved this tumor to be a primary esophageal malignant melanoma. She received no postoperative adjuvant therapy and no signs of recurrence have been clinically observed for the 22 months since her operation.
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Affiliation(s)
- Y Haga
- Department of Surgery, Mitsui Ohmuta Hospital, Fukuoka, Japan
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