Ann Thorac Surg 1996;62:1848-1850
© 1996 The Society of Thoracic Surgeons
Case Report
Primary Inflammatory Fibrosarcoma of the Esophagus
Christopher J. Magovern, MD,
Charles A. Mack, MD,
Mai Gu, MD, PhD,
Syed Hoda, MD,
Nasser K. Altorki, MD
Departments of Department ofCardiothoracic Surgery and Pathology, The New York Hospital-Cornell Medical Center, New York, New York
Accepted for publication June 22, 1996.
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Abstract
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Primary sarcomas of the esophagus are rare. We report the radiologic, surgical, and pathologic findings of a primary inflammatory fibrosarcoma of the esophagus in a 33-year-old woman, and review the prognostic features and management options of this tumor.
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Introduction
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Soft tissue sarcomas are relatively uncommon malignancies, and primary sarcoma of the esophagus is rare. Since the first description of a primary esophageal sarcoma by Chapman [1] in 1877, 138 cases have been reported in the English-language literature [2], and only 18 of these cases have been identified to be fibrosarcomas [3–5]. We report a case with radiologic, surgical, and pathologic findings.
A 33-year-old woman presented with a 7-year history of heartburn and dysphagia. The diagnosis of a benign distal esophageal stricture had been made previously, and although initially responsive to esophageal dilatation, her symptoms progressed. Multiple endoscopic biopsies had consistently demonstrated no evidence of malignancy. Physical examination was unremarkable. Barium swallow revealed a narrowing of the distal esophagus with no mucosal abnormalities. Computed tomography of the chest revealed circumferential thickening of the distal esophagus with extension through the gastroesophageal junction to the cardia of the stomach (Fig 1A
). Flexible esophagoscopy and endoscopic ultrasonography revealed an 8-cm distal esophageal stricture and a submucosal mass originating at 32 cm from the incisors with extension to the gastroesophageal junction (Fig 1B). Endoscopic biopsies revealed no evidence of malignancy.
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Fig 1. . Computed tomogram and endoscopic ultrasonogram of a distal esophageal fibrosarcoma. (A) Computed tomographic scan demonstrating circumferential thickening of the distal esophagus. (B) Endoscopic ultrasonogram demonstrating a submucosal mass at 32 cm from the incisors. (A = aorta; E = esophagus; T = tumor.)
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On July 27, 1994, an exploratory left thoracotomy revealed prominent celiac lymphadenopathy and extensive tumor infiltration of the distal esophagus and gastroesophageal junction with invasion of the diaphragm at the level of the hiatus. Biopsies of multiple lymph nodes were determined to show benign reactive lymphoid hyperplasia on frozen section examination. An esophagogastrectomy was performed with a short segment colonic interposition.
Gross examination revealed a well-circumscribed, firm tumor on the serosal surface of the gastroesophageal junction measuring 4.2 x 3.5 x 2.5 cm. No mucosal invasion was identified. The esophageal wall was diffusely thickened with luminal narrowing at the level of the gastroesophageal junction. Histologically, the tumor consisted of two components: fibroblastic and inflammatory cells (Fig 2). The fibroblasts were loosely arranged in sweeping fascicles; using immunohistochemical analysis, these cells were shown to be positive for vimentin and CD34, and negative for keratin, S-100 protein, histiocyte marker KP-1, desmin, smooth muscle antigen, and actin, further confirming their fibroblastic nature. The inflammatory cell component of the tumor consisted of mature lymphocytes and plasma cells. All lymph nodes and surgical margins were negative for tumor. The pathologic diagnosis was low-grade inflammatory fibrosarcoma [6].
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Fig 2. . Photomicrograph of the resected tumor demonstrating a mixed proliferation of fibroblasts and inflammatory cell infiltrate. (x200 before 46% reduction.)
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Eighteen months postoperatively the patient had no evidence of residual disease and was symptom-free. A computed tomogram of the chest performed at that time demonstrated no evidence of tumor recurrence.
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Comment
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The incidence of primary sarcoma of the esophagus ranges from 0.1% to 1.5% of all malignant esophageal tumors [7]. Sarcomas arise from mesenchymal cell elements within the esophagus, and the most common histologic entities encountered are leiomyosarcoma, fibrosarcoma, rhabdomyosarcoma, and liposarcoma, in decreasing order of frequency. It is extremely rare for inflammatory fibrosarcoma to be described in this location, although 1 case has been reported in the diaphragm and others have been reported in the stomach [6]. This relatively newly described entity is characterized by intersecting fascicles of spindle cells accompanied by a chronic inflammatory cell infiltrate and immunohistochemical reactivity for vimentin and CD34. Inflammatory fibrosarcoma more commonly presents in younger female patients with anemia and fever. The tumor is locally aggressive and can metastasize and recur locally after resection [6].
Patients with primary esophageal sarcoma generally present with dysphagia of several years' duration. Diagnosis is suggested by contrast radiography, computed tomography, and endoscopy, and is confirmed by biopsy. Although this lesion can be found throughout the course of the esophagus, it is slightly more common in the lower third. Grossly, these tumors vary from an infiltrating ulcerating mass to a pedunculated submucosal lesion with normal overlying mucosa [2]. A review of our case and all previously reported cases of primary fibrosarcoma of the esophagus for which data are available in the literature indicates a male predominance of 3.5:1, a median age at the time of diagnosis of 63 years, and an average duration of symptoms of 16 months before diagnosis. Seventy-eight percent of lesions were located in the distal esophagus, and all patients were treated by surgical resection. After resection, one-third of patients were dead within 1 year, one-third of patients were alive and well at an average follow-up of 7 years, and one-third of patients were lost to follow-up [4, 5].
Because of the rarity of esophageal sarcomas and paucity of data in the literature, the impact of treatment on survival is difficult to evaluate. In a pooling of the raw data of 73 patients with primary esophageal sarcoma reported in the literature, Burt [2] reported a 36% actuarial 5-year survival for patients undergoing surgical resection compared with an 11% and 0% 5-year survival after radiation therapy and no therapy, respectively. Radiation therapy may offer palliation of dysphagia in patients with advanced disease, but the role of chemotherapy as either an adjuvant or primary treatment strategy remains unclear [8]. Surgical resection is considered the treatment of choice for localized primary esophageal sarcomas, and the addition of postoperative radiation therapy should be considered for incomplete resections.
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Acknowledgments
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We thank Dr Juan Rosai of the Department of Pathology at Memorial Sloan Kettering Cancer Center, New York, NY, for graciously reviewing the case.
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Footnotes
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Address reprint requests to Dr Altorki, Department of Cardiothoracic Surgery, The New York Hospital-Cornell Medical Center, 525 E 68th St, F2212, New York, NY 10021.
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References
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- Chapman E. Sarcoma of inferior constrictor of pharynx and inlet of esophagus. Am J Med Sci 1877;100:433–7.
- Burt M. Unusual malignancies. In: Pearson FG, Deslauriers J, Ginsberg RJ, Hiebert CA, McKneally MF, Urschel HC, eds. Esophageal surgery. New York: Churchill Livingstone, 1995;629–47.
- Thorek P, Neiman BM. Rhabdomyosarcoma of the esophagus. J Thorac Surg 1950;20:77–89.
- Borrie J. Sarcoma of the esophagus: surgical treatment. J Thorac Cardiovasc Surg 1959;37:413–26.
- Goodner JT, Miller TR, Watson WL. Sarcoma of the esophagus. AJR 1963;89:132–9.
- Meis JM, Enzinger FM. Inflammatory fibrosarcoma of the mesentery and retroperitoneum. Am J Surg Pathol 1991;15:1146–56.[Medline]
- Stout AP, Lattes RL. Tumors of the esophagus. In: Atlas of tumor pathology, Section V, Fascicle 20. Washington DC: Armed Forces Institute of Pathology, 1957:83–7.
- 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]
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Abstract
Introduction
