(Radiology. 1999;213:97-100.)
© RSNA, 1999
Primary Melanoma of the Esophagus: Radiologic and Clinical Findings in Six Patients1
Marc J. Gollub, MD and
Joan C. Prowda, MD
1 From the Department of Radiology, Memorial Sloan-Kettering Cancer Center, 1275 York Ave, New York, NY 10021 (M.J.G.) and New York Presbyterian Hospital–Weill Cornell Medical College, NY (J.C.P.). Received September 10, 1998; revision requested November 3; final revision received January 12, 1999; accepted March 1. Address reprint requests to M.J.G. (e-mail: gollubm@mskcc.org).
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Abstract
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PURPOSE: To evaluate the clinical and radiologic findings of primary melanoma of the esophagus.
MATERIALS AND METHODS: A computer search of pathology, radiology, and cancer registry records from 1973 to 1998 revealed six patients with primary malignant melanoma of the esophagus whose radiographs were available for review. Six esophagograms, three contrast material–enhanced chest computed tomographic (CT) scans, and four chest radiographs were reviewed. Medical records were reviewed for presenting symptoms and clinical course.
RESULTS: Six patients (age range, 63–78 years; mean age, 70 years) had histopathologically proved primary malignant melanoma of the esophagus. All patients presented with dysphagia or odynophagia of 6 weeks duration or less. Esophagography and chest CT showed polypoid, nonobstructing esophageal masses, which were mucosal (n = 5) or submucosal (n = 1) and which were located in the middle (n = 3), distal (n = 2), or proximal (n = 1) third of the esophagus. Five patients underwent esophagogastrectomy: Three died a mean of 5 months afterward, two were lost to follow-up, and one was alive 7 months later.
CONCLUSION: Primary melanoma of the esophagus is rare. It is usually polypoid, intraluminal, and nonobstructive. As with other esophageal malignancies, the prognosis is dismal despite resection.
Index terms: Esophagus, neoplasms, 71.329 • Melanoma, 71.329 • Thorax, CT, 71.12112
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Introduction
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Primary melanoma of the esophagus is a rare tumor that accounts for less than 0.1%–0.2% (seven of 4,995) of esophageal malignancies (1). Although it was once thought that primary melanoma could not arise in the esophagus because of a lack of precursor cells, it has since been shown in autopsy series that 4%–8% of individuals have melanoblasts in the esophageal mucosa. To our knowledge, as of 1995, there were 180 cases reported in the world literature; these were mainly derived from surgical or gastroenterologic series (2). Only individual case reports have appeared in the radiology literature (1,3). The goal of this study was to describe the radiologic and clinical findings in six patients with this disease, all of whom were evaluated and treated at our institution.
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MATERIALS AND METHODS
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A computer search of pathology, radiology, and cancer registry records in our institution from 1973 to 1998 revealed six patients with primary esophageal melanoma whose radiographs were available for review. No patient had evidence of a primary melanoma elsewhere in the body. Six esophagograms, three contrast material–enhanced chest computed tomographic (CT) scans, and four chest radiographs were reviewed retrospectively. Both authors at the same sitting simultaneously reviewed each patient's images by consensus; images included chest radiographs, esophagograms, and CT scans.
Chest radiographs were evaluated for mediastinal or hilar masses or widening and for parenchymal masses or infiltrates. Barium esophagograms were evaluated for the size and configuration of any mass, for the presence of ulceration or obstruction, and for the appearance of the mass (infiltrative, polypoid or annular, mucosal, or mural or extramural). Contrast-enhanced CT scans were evaluated for the appearance of the primary esophageal lesion, if seen, and its size, extent (intraluminal, invasive into the mediastinum, or multifocal), and location (upper, middle, or lower third of the esophagus). CT scans were also evaluated for the presence of obstruction and for associated wall thickening. Nodal and distant organ metastatic diseases (eg, liver or lung metastases) were also evaluated. Medical records were reviewed for presenting symptoms and clinical course, including findings at histopathologic examination. All patients underwent endoscopy with biopsy. Five patients underwent esophagogastrectomy; two patients were lost to follow-up.
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RESULTS
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Clinical Findings
Six patients (four women and two men) with a mean age of 70 years (age range, 63–78 years) had pathologically or histopathologically proved primary malignant melanoma of the esophagus. All patients presented with dysphagia of 6 weeks duration or less. Other complaints included odynophagia in one patient, melena in one patient, and a palpable 3-cm right supraclavicular lymph node in one patient (proved to be melanoma at surgery).
Five patients underwent esophagogastrectomy. Two patients were lost to long-term follow-up; one patient underwent removal of a node-negative T2 (involvement of the muscularis propria) tumor with no metastases, and the other was referred for laser ablation. The four remaining patients underwent surgical excision; three died at a mean of 5 months after surgery (range, 1–12 months), and one was alive at 7 months. The causes of death were myocardial infarction (n = 2) and gastrointestinal bleeding with recurrent primary and disseminated tumor (n = 1). Metastases were present in three patients and involved the liver (n = 1); axillary lymph node (n = 1, the patient lost to follow-up); and cervical and mediastinal lymph node, pulmonary, and esophagogastric anastomosis (n = 1). Results are given in the Table.
Radiologic Findings
Findings on barium esophagograms.—In six patients, esophagography revealed lobular masses, five of which were polypoid and predominantly intraluminal (Fig 1a, 1b). These five masses expanded the luminal caliber but did not cause obstruction. One patient had a nonobstructive, submucosal, smoothly marginated mass, without ulceration, that formed obtuse angles with the wall of the esophagus (Fig 2).
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Figure 1a. (a-c) Esophagograms in three patients with primary malignant melanoma of the esophagus show polypoid intraluminal masses (arrows in a and b, arrowheads in c) in the esophagus, without obstruction, and show linear surface collections (arrow in c), which are thought to represent ulcers or barium trapped between lobulations.
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Figure 1b. (a-c) Esophagograms in three patients with primary malignant melanoma of the esophagus show polypoid intraluminal masses (arrows in a and b, arrowheads in c) in the esophagus, without obstruction, and show linear surface collections (arrow in c), which are thought to represent ulcers or barium trapped between lobulations.
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Figure 1c. (a-c) Esophagograms in three patients with primary malignant melanoma of the esophagus show polypoid intraluminal masses (arrows in a and b, arrowheads in c) in the esophagus, without obstruction, and show linear surface collections (arrow in c), which are thought to represent ulcers or barium trapped between lobulations.
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Figure 2. Esophagogram in a patient with primary malignant melanoma of the esophagus shows a submucosal midesophageal lesion (arrows) with smooth obtuse borders and no surface pattern. There is no obstruction.
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Five patients had linear or curvilinear barium collections, which were believed to represent either linear ulcers or crevices between lobulations (Fig 1c). On average, the tumors extended over 2.2 vertebral bodies (range, 1.5–3.0 vertebral bodies). (This was the only quantitation available, since images had different magnifications and sizes.) Three lesions were located in the midesophagus, two were located in the lower third of the esophagus, and one was located in the upper third of the esophagus.
Findings on chest CT scans.—Three patients had polypoid intraluminal masses that ranged 3.2–6.3 cm in length (mean, 4.8 cm) and 2.5–4.0 cm in maximal diameter (mean, 3.3 cm) (Fig 3). A rim of air or orally administered contrast material surrounded all lesions (Fig 3). No wall thickening or extension of the mass into adjacent mediastinal structures was appreciated. Mediastinal lymphadenopathy was absent in all three patients. A 3-cm mass in the left lower lung was present in one patient (later confirmed to be squamous cell cancer of the lung), and a 3-cm right axillary lymph node was present in another patient (later confirmed to be metastatic melanoma).
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Figure 3. Contrast-enhanced CT scan of the chest in a patient with primary malignant melanoma of the esophagus demonstrates an intraluminal mass and a rim of orally administered contrast material (arrow) around the lesion.
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Findings on chest radiographs.—Chest radiographs were negative in three patients and demonstrated left paraspinal and subcarinal soft-tissue fullness in the remaining patient.
Histopathologic Findings
Findings at endoscopic biopsy were positive for malignancy in all patients. However, in one patient, epidermoid cancer was suspected, and in another patient, the histologic findings were not specified. Both cases were proved to be melanoma at surgery.
In five patients, the specimens obtained at esophagogastrectomy revealed four polypoid and one sessile mass (Fig 4). The mean maximal tumor diameter was 3.6 cm (range, 2.2–4.5 cm). Four lesions were T2 and one was T1 (limited to the submucosa). The lesion was described at esophagography as submucosal; it "deeply infiltrated the muscular wall." Four tumors were brown-black, or hemorrhagic, and one was white. In situ melanoma, lateral junctional change, or melanosis was described in three patients, and positive findings at immunohistochemical staining with HMB-45 and S-100 were described in three patients. Mediastinal lymph nodes were positive in only one of five patients.
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Figure 4. Gross specimen from a primary melanoma in a 74-year-old man shows a polypoid mass with hemorrhage. (Photograph courtesy of Dr Manjit Bains, Department of Thoracic Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY.)
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DISCUSSION
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Melanoma of the esophagus is exceedingly rare. It may be either primary or secondary. Primary esophageal melanoma has a reported prevalence of 0.1%–0.5% of all esophageal malignancies (4). The tumor is twice as common in men as in women, occurs in the 6th and 7th decades of life, and is most commonly located in the middle or distal third of the esophagus (2).
Cutaneous melanoma frequently metastasizes to the gastrointestinal tract and has been found in 48% of patients who die of malignant melanoma (5). It most commonly spreads to the small intestine; less often, it spreads to the stomach and colon (6). Metastasis to the esophagus is rare. It reportedly occurs in only 4% (five of 125) of patients who die of metastatic melanoma and who are examined at autopsy (7).
It was originally thought that primary melanoma could not develop in the esophagus (because of the lack of precursor cells), until 1963 when De La Pava et al (8) demonstrated typical melanocytes in the esophageal mucosa in 4% of deceased patients at autopsy. This was later confirmed in the series by Tateishi et al (9), in which an incidence of 8% was found; this established the origin of primary esophageal melanoma.
The most common presenting symptoms are dysphagia (73%), weight loss (72%), pain (44%), and melena (10%) (4). Physical signs other than weight loss are uncommon. It is interesting that palpable supraclavicular lymph nodes were found in patients in two series (4,10).
Barium esophagography usually reveals a polypoid mass in the middle or distal third of the esophagus, a finding that was present in five of our patients. A considerable degree of obstruction is uncommon. A less common appearance may include that of a stricturing, circumferential mass. Other polypoid tumors of the esophagus include spindle cell carcinoma (carcinosarcoma), leiomyosarcoma, and, rarely, adenocarcinoma. Spindle cell carcinoma, a bulky polypoid intraluminal lesion that rarely causes obstruction, would be virtually indistinguishable from melanoma at radiographic examination. Benign polypoid filling defects include leiomyoma, squamous papilloma, fibrovascular polyp, and impacted food bolus (3). Squamous cell carcinoma is not commonly polypoid.
CT of the thorax may reveal a bulky esophageal mass, with compression of adjacent mediastinal structures. As with other esophageal malignancies, CT may be most useful in demonstrating mediastinal invasion, nodal enlargement, and distant metastatic disease. Melanoma usually involves the local-regional nodes, lung and/or pleura, and liver.
At endoscopy, lesions may appear as polypoid, intraluminal, darkly colored or nonpigmented masses with intact mucosa or occasional ulceration (2). Specimens obtained at endoscopic biopsy reveal limited data for a definitive diagnosis. Findings are often mistakenly interpreted as revealing poorly differentiated squamous cell cancer. This occurred in two of our patients. Confirmation of findings at histologic examination is usually made after en bloc resection (11).
Gross specimens are polypoid, variably sized, and pigmented in 85% of patients. Ulcerations may be present, but more often the tumor is covered with intact mucosa. In one series, "satellite" tumor nodules were present in 12% of patients and, at times, were distant from the tumor (10).
At the microscopic level, this tumor involves the mucosa and submucosa and grows in a lentiginous (nodular infiltrative), radial manner. The overlying squamous mucosa is usually intact. Lymphatic and vascular invasion is common. Electron microscopy demonstrates premelanosomes. Immunohistochemical results are positive with HMB-45 or S-100 staining but are negative for cytokeratin staining.
Melanoma of the esophagus can be considered primary when it has the characteristic structure of melanoma, when it contains pigment that is demonstrable as melanin with the appropriate staining technique, and when the adjacent epithelium has melanocytic proliferation and shows junctional change. Other sites of primary melanoma must be excluded (2,10).
The treatment of choice for primary melanoma of the esophagus is surgical resection en bloc, with dissection of the lymph nodes. Because of the potential for "satellite" mucosal tumor nodules, the surgical procedure should include a radical resection with a greater margin than that used for the usual squamous cell carcinoma. The propensity for submucosal spread may lead to incomplete excision (10). Adjuvant or neoadjuvant radiation therapy has been used, but its utility is unproved (4). Currently, it is believed that chemotherapy and immunotherapy have no major role in treatment (2).
The prognosis for patients with this tumor is dismal. Metastases are present at the time of diagnosis in 49% of cases (11). Despite curative or palliative surgical resection, patients with this aggressive malignancy usually present late in the course of disease and progress to early death, with widespread metastases. As a result, the 5-year survival rate ranges from 0% to 4% (median survival, 5–7 months) (4,6). At our institution, in a previously reported series, seven of eight primary esophageal melanomas were resected. Three patients received chemotherapy. The 3- and 5-year survival rates were 13% (one of eight patients) and 0%, respectively (median survival, 4.7 months) (4).
In summary, primary melanoma of the esophagus is a rare, aggressive malignancy that should be considered in the differential diagnosis of polypoid esophageal masses on esophagograms and chest CT scans. The appearance is more likely to be caused by spindle cell carcinoma or leiomyosarcoma. However, it is important to obtain a preoperative diagnosis at histologic examination, since more aggressive surgery may be needed to properly treat these patients.
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Footnotes
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Author contributions: Guarantors of integrity of entire study, M.J.G., J.C.P.; study concepts, M.J.G.; study design and definition of intellectual content, M.J.G., J.C.P.; literature research, J.C.P.; clinical studies, M.J.G., J.C.P.; data acquisition, J.C.P.; data analysis, M.J.G., J.C.P.; manuscript preparation and review, M.J.G., J.C.P.; manuscript editing, M.J.G.
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Abstract
Introduction
