Ann Thorac Surg 2001;72:596-597
© 2001 The Society of Thoracic Surgeons
Case report
Long-term survival after resection for small cell carcinoma of the esophagus
Shinichi Yachida, MDa,
Kohtaroh Matsushita, MDa,
Hisashi Usuki, MDa,
Hideki Wanibuchi, MDa,
Takashi Maeba, MDa,
Hajime Maeta, MDa
a Department of Surgery, Faculty of Medicine, Kagawa Medical University, Kagawa, Japan
Accepted for publication June 30, 2000.
Address reprint requests to Dr Yachida, Department of Surgery, National Cancer Center Hospital, Tsukiji 5-chome, Chuo-ku, Tokyo 104-0045, Japan
e-mail: syachida{at}ncc.go.jp
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Abstract
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We describe the rare case of a patient with esophageal small cell carcinoma who was completely cured. A 77-year-old man had small cell carcinoma of the esophagus with extensive lymph node metastases. Treatment comprised a subtotal esophagectomy and extended lymph node dissection. He has survived for more than 7 years with no evidence of recurrent disease. We suggest that radical operations should be considered for future patients if curative resection can be expected.
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Introduction
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Since its original description by McKeown [1] in 1952, primary small cell carcinoma of the esophagus has been recognized as a rare but aggressive malignant tumor characterized by early metastases and a rapidly fatal course [2]. The optimal clinical management of this entity is uncertain. Here we report a case of successful surgical resection with subsequent survival for more than 7 years. To our knowledge, this is the longest survival time documented for a patient with advanced small cell carcinoma of the esophagus.
A 77-year-old Japanese man was admitted to a local hospital for evaluation of dysphagia. He was referred to our hospital in May 1992 because of a smoothly marginated, sessile mass 10 cm in length containing a relatively flat area of central ulceration of the lower esophageal wall detected by a barium study. He had smoked 20 cigarettes a day for 57 years, but he had no respiratory symptoms. The chest, abdomen, and lymph node–bearing areas were unremarkable on physical examination. Blood values were all normal on admission. Upper gastrointestinal endoscopic study confirmed the presence of a sessile mass with a central ulceration and a stricture from the proximal margin of the tumor, 30 cm from the upper incisors. A thoracoabdominal computed tomographic scan showed abnormal thickening of the lower esophageal wall and extensive mediastinal and perigastric lymph node enlargement (3.0 cm). An advanced esophageal cancer was suspected.
The patient underwent a subtotal esophagectomy with a gastric tube reconstruction through the antesternal route and an extended systematic dissection of the mediastinal and perigastric lymph nodes in June 1992. The operation appeared macroscopically to be a curative resection. Pathological examination revealed a 9.5 x 7.5-cm raised, ulcerated lower esophageal lesion extending to the deep margins of the specimen (Fig 1). Sections through the esophageal tumor showed a location primarily in the lamina propria, covered by a thin, stratified squamous epithelium at the margin. Superficial ulceration was present in the center. On light microscopy, the tumor was composed entirely of anaplastic small fusiform to polygonal cells possessing strongly hyperchromatic nuclei and scanty cytoplasm that had invaded the adventitia and permeated the local lymphatics and blood vessels (Fig 2). The tumor did not contain foci of either squamous or glandular differentiation. Metastatic tumors were present in two paraesophageal lymph nodes and three perigastric lymph nodes. Resection margins were negative for tumor cells. Immunohistochemically, they showed positive staining for neuron-specific enolase but were negative for cytokeratin, serotonin, and chromogranin. Grimelius stain was negative. Electron microscopy revealed neither identifiable cytoplasmic neurosecretory granules nor well-formed desmosomes. The final pathological diagnosis was small cell carcinoma of the esophagus with lymph node metastases (TNM classification, T3 N1 M0: stage III).
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Fig 2. Photomicrograph of esophageal tumor. The tumor cells show hyperchromatic nuclei, inconspicuous nucleoli, thin nuclear membranes, scanty cytoplasm, and ill-defined borders. (Hematoxylin and eosin; x200 before 49% reduction.)
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The patient’s postoperative course was good, and he was discharged on postoperative day 31. To date, he has survived for more than 7 years without further treatment, and there is no evidence of recurrence or metastasis.
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Comment
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Small cell carcinoma of the esophagus is a rare tumor histologically defined by the presence of small spindle-shaped or polygonal tumor cells with hyperchromatic nuclei and scanty cytoplasm. Intracytoplasmic argyrophilia by Grimelius staining and neurosecretory granules on electron microscopic examination are often found in esophageal small cell carcinomas [3, 4]. In our patient, cytoplasmic argyrophilia and electron-dense granules in the tumor cells were absent, but immunohistochemical staining for neuron-specific enolase was positive. The presence of such neuroendocrine characteristics is important but not essential for the diagnosis of a small cell carcinoma [3]. The true nature and the histogenesis of this tumor remain controversial.
Primary small cell carcinoma of the esophagus is an aggressive tumor that is associated with a poor prognosis (mean overall survival, 5.1 months) regardless of treatment—chemotherapy, radiation therapy, surgical intervention, or any combination of these [3]. The most effective treatment has not yet been established because of the rarity of this tumor and the overall extremely poor prognosis. In a recent comprehensive review of the world literature, Casas and associates [4] examined the treatment of and prognosis for 230 patients and concluded that chemotherapy combined with local treatment (probably radiotherapy) could be used as standard therapy for limited-stage esophageal small cell carcinomas. Chemotherapy appears to result in striking clinical remission in most patients for varying lengths of time, but it alone does not produce complete remission and disease-free long-term survival. Because of the rapidly progressive nature of this disease, Kelsen and coauthors [5] have insisted that surgical resection is not the treatment of choice. This suggestion is perhaps premature because there are reports of longer survival (> 5 years) after surgical intervention, as is the case with our patient [6]. Our view is that when the lesion is localized or macroscopically resectable with no evidence of distant metastasis, it should be resected, and thereafter, a multidrug regimen should be prescribed if necessary.
Prospective randomized trials of therapy for esophageal small cell carcinoma are unlikely in the future because of the rarity of the disease. This report indicates that curative resection achieved by radical en bloc esophagectomy with lymph node dissection may offer an increased possibility of survival, and we suggest that surgical intervention should be considered first for future patients.
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Acknowledgments
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We thank Dr Shoji Fukushima, Department of Pathology, and Dr Mitsuyo Maeda, Department of Anatomy, Osaka City University Medical School, for advice regarding the histopathological examination. We also recognize Dr Samuel M. Cohen, Department of Pathology and Microbiology, University of Nebraska Medical Center, for the critical reading of this manuscript.
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References
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McKeown F. Oat-cell carcinoma of the oesophagus. J Pathol Bacteriol 1952;64:889-891.[Medline]
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Huncharek M., Muscat J. Small cell carcinoma of the esophagus. The Massachusetts General Hospital experience, 1978 to 1993. Chest 1995;107:179-181.[Abstract/Free Full Text]
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McFadden D.W., Rudnicki M., Talamini M.A. Primary small cell carcinoma of the esophagus. Ann Thorac Surg 1989;47:477-480.[Abstract]
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Casas F., Ferrer F., Farrús B., Casals J., Biete A. Primary small cell carcinoma of the esophagus: a review of the literature with emphasis on therapy and prognosis. Cancer 1997;80:1366-1372.[Medline]
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Kelsen D.P., Weston E., Kurts R., Cvitkovic E., Lieberman P., Golbey R.B. Small-cell carcinoma of the esophagus: treatment by chemotherapy alone. Cancer 1980;45:1558-1561.[Medline]
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Craig S.R., Carey F.A., Walker W.S., Cameron E.W.J. Primary small-cell cancer of the esophagus. J Thorac Cardiovasc Surg 1995;109:284-288.[Abstract/Free Full Text]
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Abstract
Introduction
