Ann Thorac Surg 1997;63:1461-1463
© 1997 The Society of Thoracic Surgeons
Case Report
Surgical Therapy of Primary Malignant Melanoma of the Esophagus
Farzin Adili, MD,
Stefan P. Mönig, MD
Department of Visceral and Vascular Surgery, Albertus-Magnus University, Cologne, Germany
Accepted for publication December 2, 1996.
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Abstract
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Primary malignantmelanoma of the esophagus is an extremely rare tumor. In the absence of prospectively sampled data in the literature, the following case report and review of the literature indicate that surgical resection of the priary tumor and solitary metastases provides the longest survival times and, to date, is the treatment of choice.
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Introduction
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Primary malignant melanoma of the esophagus (PMME) is an extremely uncommon tumor associated with poor survival. n a review of 110 patients, the 5-year survival after surgical therapy was 4% [1]. Esophageal malignant melanoma was originally considered an exclusively metastatic lesion, a view founded on the low incidence and inconsistent distribution of melanocytes in the normal esophageal epithelium. However, in 1963 the variable presence of typical melanoblasts in the esophageal mucosal epithelium was demonstrated for the first time [2], and in a series of 1,910 malignant tumors of the esophagus, 0.1% were attributed to this entity [3]. Because of its rare occurrence and limited availability of conforming data on diagnosis and treatment, we report a case of PMME with surgical, radiologic, and pathologic findings.
In June 1991, a 66-year-old man presented to the University of Cologne Department of Surgery with a 3-week history of night sweat and sore throat. Before admission, the patient consulted an ear, nose, and throat specialist who could not detect any basis for these symptoms. The patient was therefore sent to a gastroenterologist who, by esophagogastroscopy, discovered a polypoid esophageal tumor, 10 mm in diameter, 40 cm from the incisors. Histologic examination of the biopsy specimens revealed an undifferentiated malignant tumor of unknown origin. A barium swallow examination was negative. Computed tomography demonstrated absence of tumor manifestations other than the distal esophagus tumor (Fig 1
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Fig 1. . Abdominal computed tomographic scan. Arrow depicts the tumor in the distal third of the esophagus.
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The patient subsequently underwent transhiatal esophagectomy and lymphadenectomy of the lower mediastinum and superior gastric lymph nodes as well as cervical esophagogastrostomy. The unstretched surgical specimen contained a polypoid tumor measuring 3 x 1.8 x 1 cm in the lower esophagus. Microscopically, it was composed of rare tumor cells containing pigmented granules. Immunohistochemistry demonstrated focal areas that stained positive for the S-100 and HMB-45 protein (Fig 2) and verified the definite diagnosis of an R0-resected PMME analogous to a pT3 N1 M0 extent. Four of 13 regional lymph nodes were infiltrated by tumor cells.
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Fig 2. . (A) Regular esophageal mucosa infiltrated by pigmented malignant melanoma cells. Arrowheads denote pigmented granules. (Hematoxylin and eosin; x200 before 28% reduction.) (B) Immunohistochemistry of esophageal submucosa with tumor cells staining positive for the S-100 antigen (arrow). (Counterstain with hemalum; x400 before 28% reduction.)
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The postoperative course was remarkable for an anastomotic leakage, which was only treated by irrigation. In the following years, the patient repeatedly underwent bouginage of the cervical anastomosis due to recurrent strictures. However, on follow-up, metastases of the melanoma were confirmed in the greater omentum, right diaphragm, and liver (segment II), and were completely removed by an operation on April 4, 1993. Currently (June 1996), the patient is alive and disease-free.
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Comment
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A case of PMME was first reported in 1906; however, since 1952, when the first case with histologic confirmation was published, a total of 174 cases have been reported in the literature [4]. In a series of 436 patients presenting to the University of Cologne Department of Surgery between 1984 and 1995 who were diagnosed a malignant tumor of the esophagus, we discovered 3 patients (0.7%), 2 men and 1 woman, with the diagnosis of PMME and 1 patient with esophageal metastasis of a cutaneous malignant melanoma. The mean presenting age was 63 years (range, 49 to 76 years), which corresponds with the literature [4, 5]. Chalkiadakis and associates [1], in a review of 110 patients diagnosed with PMME found that men predominated by 2:1 with a mean age of 60 years. The tumors were commonly located in the middle and lower thirds of the esophagus (86%), dysphagia was present in 73%, and the tumor was localized and amenable to surgical resection in the majority of patients.
Melanocytes, which are melanin pigment producing cells derived from the neural crest, are believed to migrate to the esophagus during embryogenesis in the same way they migrate to the skin and other sites. In 15%, metastases of malignant melanoma cannot be associated with a primary lesion [4]. Moreover, in the absence of evident black pigmentation, histologic specimens may hardly be distinguished from other unusual malignancies of the esophagus, such as undifferentiated carcinoma and sarcoma [6]. Despite these known difficulties to properly differentiate between metastases of malignant melanoma and a primary manifestation in mucous membranes, histologic criteria have been defined to appropriately classify melanotic lesions [7]: First, stromal invasion by neoplastic melanocytes containing demonstrable melanin pigment, and second, an adjacent epidermal basal layer containing atypically proliferating melanocytes have been associated with PMME. In addition, as in our case, immunohistochemistry identifies malignant melanoma of the esophagus by avid staining with anti-S-100 cytoplasmic protein or HMB-45 antibody, but not with anticytokeratins [5].
The signs and symptoms of PMME are not different from those of other malignant tumors of the esophagus. The patients present with dysphagia, epigastric and chest pain, regurgitation, weight loss, coughing, and sialism. The diagnosis of PMME is difficult and requires a combination of several examinations including upper gastrointestinal series, endoscopy, and biopsy with histopathologic assessment. Once macroscopic evaluation and histology establish the diagnosis of malignant melanoma, the preoperative staging should be directed toward a differentiation between PMME and esophageal metastases of a malignant melanoma. Primary extracutaneous manifestations of malignant melanoma include melanoma of the corioidea, conjunctival, laryngeal, oropharyngeal, anal, urethral, and vulvovaginal mucous membranes [6].
The prognosis of PMME is poor. Of 45 patients who came to autopsy, metastases were discovered in 78% and involved liver (31%), mediastinum (29%), lung (18%), and brain (13%) [1].
Although any benefit of surgical resection in improving survival is limited at best, long-term survival has been reported after resection of the esophagus [8]. Surgical resection with reestablishment of gastrointestinal continuity is the treatment of choice and provides a mean survival of 14.8 months [4]. Moreover, as documented in our case, resection of entirely removable metastases, even at different sites, may also prove beneficial for the long-term survival of these patients. The patient with the longest survival, who stayed alive for 10 years, was treated with surgical resection only [1]. In conjunction with postoperative radiation therapy, even longer mean survival times of 15.6 months were reported in 6 patients. This finding is quite surprising because malignant melanomas are not known to be sensitive to radiation therapy. The benefit of chemotherapy for the palliation of PMME has, thus far, been questionable and needs further investigation [4].
In conclusion, once the diagnosis of a PMME is made, surgical removal of the tumor with the goal of an R0 resection remains the treatment of choice and may be attempted whenever possible.
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Acknowledgments
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We thank Dr Thomas Zirbes, Institute of Pathology, University of Cologne, for help with the processing of the histologic specimens and the preparation of the photographs. We gratefully acknowledge Drs Dirk von Mallek, Volker Wenzel, Walter Heindel, and Klaus Lackner, Director of the Institute of Radiology, for the analysis of the computed tomographic scan images, which were kindly provided by the Radiology Institute Breunsbach. We are also appreciative for many helpful discussions and the continuing support of Dr Heinz Pichlmaier and Dr Arnulf H. Hölscher, Director of the Department of Visceral and Vascular Surgery, University of Cologne.
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Footnotes
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Address reprint requests to Dr Adili, Department of Visceral and Vascular Surgery, Albertus Magnus University, Joseph Stelzmann Str 9, D-50924 Köln, Germany.
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References
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- Chalkiadakis G, Wihlm JM, Morand G, Weill-Bousson M, Witz JP. Primary malignant melanoma of the esophagus. Ann Thorac Surg 1985;39:472–5.[Abstract/Free Full Text]
- De La Pava S, Nigogosyan G, Pickren JW, Cabrera A. Melanosis of the esophagus. Cancer 1963;16:48–50.
- Turnbull AD, Rosen P, Goodner JT, Beattie ES. Primary malignant tumors of the esophagus other than typical epidermoid carcinoma. Ann Thorac Surg 1973;15:463–73.[Abstract/Free Full Text]
- Uthoff K, Jähne J, Meyer HJ, Pichlmayr R. Chirurgische Therapie des primären malignen Melanoms des Oesophagus. Chirurg 1996;67:86–9.[Medline]
- Caldwell CB, Bains MS, Burt M. Unusual malignant neoplasms of the esophagus. Oat cell carcinoma, melanoma, and sarcoma. J Thorac Cardiovasc Surg 1991;101:100–7.[Abstract]
- Pavanello M, Bonavina L, Seglin A, Chella B. Primary malignant melanoma of the esophagus. Dis Esoph 1995;8:145–7.
- Raven RW, Dawson I. Malignant melanoma of the oesophagus. Br J Surg 1964;51:551–5.[Medline]
- Hamdy FC, Smith JHF, Kennedy A, Thorpe JAC. Long survival after excision of a primary malignant melanoma of the oesophagus. Thorax 1991;46:397–8.[Abstract/Free Full Text]
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Abstract
Introduction
