Ann Thorac Surg 1997;64:1173-1174
© 1997 The Society of Thoracic Surgeons
Case Report
Neurofibroma of the Esophagus
Raymond Lee, MD,
Warren A. Williamson, MD
Department of Thoracic and Cardiovascular Surgery, Lahey Hitchcock Medical Center, Burlington, Massachusetts
Accepted for publication May 9, 1997.
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Abstract
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Benign esophageal tumors occur infrequently, with leiomyomas accounting for approximately 70% of cases. Benign neural tumors of the esophagus account for 200 cases reported in the literature and rarely require operative resection. The case of a 58-year-old woman with a 4-month history of progressive dysphagia and odynophagia is presented. A large intramural esophageal mass was resected through a right thoracotomy, and the esophagus was primarily repaired. Histologic examination revealed a neurofibroma.
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Introduction
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Progressive dysphagia, worse with solids, developed over a 4-month period in a 58-year-old woman, followed by the onset of odynophagia for both solids and liquids. She denied any weight loss or symptoms of reflux.
A barium swallow (Fig 1
) revealed a large intrinsic mass of the proximal esophagus, esophageal dilatation proximal to the mass, and a slightly irregular mucosal surface interpreted as showing a probable large esophageal leiomyoma. Upper gastrointestinal tract endoscopy disclosed a large submucosal mass at 20 cm, with a small area of mucosal ulceration. Multiple biopsy specimens revealed hyperplastic squamous mucosa with inflamed granulation tissue; there was no evidence of malignancy. Computed tomography of the chest outlined a large soft-tissue mass, measuring 4 x 6 cm, in the right mediastinum that was compressing the esophagus and trachea (Fig 2). No associated adenopathy was present.
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Fig 1. . Barium swallow demonstrates a large intrinsic mass of the midthoracic esophagus with proximal esophageal dilation and some mucosal irregularity.
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Fig 2. . Chest computed tomogram shows 4 x 6-cm mediastinal mass compressing the esophagus and trachea with no associated adenopathy.
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Operation consisted of right thoracotomy performed through the fourth interspace. The fourth intercostal muscle bundle was harvested for use as an onlay graft. A 4 x 6-cm intramural esophageal mass noted just above the level of the azygos vein appeared to be covered by the longitudinal muscle fibers of the esophagus. The mass was dissected free of the surrounding mediastinal structures. The longitudinal esophageal muscle was dissected free of the mass, leaving only a base of attachment to the esophageal mucosa. The adherent esophageal mucosa was excised with the mass, leaving a rim of normal esophagus. A 50F Maloney dilator was inserted into the esophagus, which appeared dilated. The esophagus was closed longitudinally in two layers: a mucosal layer of interrupted sutures of 4-0 Vicryl (Ethicon, Somerville, NJ) and an outer layer of interrupted sutures of 4-0 silk. The closure was reinforced with the intercostal muscle flap, which was sewn over the closure using interrupted sutures of 4-0 silk. A postoperative barium swallow revealed no evidence of leakage from the site of repair and no evidence of esophageal narrowing.
Histologic examination revealed a mass composed of spindle cells organized in whorls (Fig 3). Immunoperoxidase staining revealed the tumor to be positive for S-100 but negative for desmin, actin, and HMB-45.
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Comment
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Benign tumors of the esophagus are found infrequently, with leiomyomas accounting for more than 70% of cases [1]. Benign neural tumors of the esophagus are exceedingly rare, with approximately 200 cases reported in the literature [2]. Most of the reported cases of esophageal neural tumors are classified as granular cell tumors of the esophagus, which typically occur as a 3- to 8-mm submucosal nodule found incidentally in the distal esophagus during upper gastrointestinal endoscopy. After endoscopic biopsy, conservative management with observation has been recommended unless the mass measures more than 1 cm or the patient is symptomatic [3, 4]. A second, smaller group of neural tumors, consisting of either benign schwannomas or neurofibromas as in our patient, has been described. In the few reports of benign esophageal neural tumors of this type [5–7], all patients had symptoms on presentation and were found to have large intramural tumors that were completely resected. We present an additional case managed by resection and primary repair of the esophagus.
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Footnotes
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Address reprint requests to Dr Williamson, Department of Thoracic and Cardiovascular Surgery, Lahey Hitchcock Medical Center, 41 Mall Rd, Burlington, MA 01805.
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References
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- Seremetis MG, Lyons WS, de Guzman VC, Peabody JW Jr. Leiomyomata of the esophagus: an analysis of 838 cases. Cancer 1976;38:2166–77.[Medline]
- Lack EE, Worsham GF, Callihan MD, et al. Granular cell tumor: a clinicopathologic study of 110 patients. J Surg Oncol 1980;13:301–16.[Medline]
- Goldblum JR, Rice TW, Zuccaro G, Richter JE. Granular cell tumors of the esophagus: a clinical and pathologic study of 13 cases. Ann Thorac Surg 1996;62:860–5.[Abstract/Free Full Text]
- Mineo TC, Biancari F, Francioni F, Trentino P, Casciani CU. Conservative approach to granular cell tumour of the oesophagus: three case reports. Scand J Thorac Cardiovasc Surg 1995;29:141–4.[Medline]
- Eberlein TJ, Hannan R, Josa M, Sugarbaker DJ. Benign schwannoma of the esophagus presenting as a giant fibrovascular polyp. Ann Thorac Surg 1992;53:343–5.[Abstract]
- Madrid G, Pardo J, Perez RG, et al. The neurofibroma of the oesophagus: case report. Eur J Radiol 1986;6:67–9.[Medline]
- Ramirez Rodriguez JM, Deus Fombellida J, Lozano Mantecón R, Gallel Vicente P. Neurofibroma solitario de esófago. Rev Esp Enferm Dig 1992;82:47–9.[Medline]
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Abstract
Introduction
