Ann Thorac Surg 2006;82:340-342
© 2006 The Society of Thoracic Surgeons
Case report
Giant Benign Esophageal Schwannoma Requiring Esophagectomy
Bernard J. Park, MD,
Julian Carrasquillo,
Manjit S. Bains, MD,
Raja M. Flores, MD
*
Thoracic Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York
Accepted for publication September 20, 2005.
* Address correspondence to Dr Flores, Memorial Sloan-Kettering Cancer Center, 1275 York Ave, Room C-879, New York, NY 10021 (Email: floresr{at}mskcc.org).
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Abstract
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Benign esophageal schwannomas are rare tumors with only 14 cases reported in the English literature. All previous reports describe excision without major esophageal resection. We report the case of a 33-year-old woman with a giant esophageal schwannoma that required total thoracic esophagectomy.
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Introduction
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The majority of submucosal tumors of the gastrointestinal tract are benign leiomyomas. Neurogenic tumors of the digestive tract are uncommon, and schwannomas are rare with the majority occurring in the stomach or colon. None of the previously reported cases of esophageal schwannoma required esophagectomy. We report the first case of a patient with a giant esophageal schwannoma that required esophagectomy for removal.
A 33-year-old otherwise healthy, never-smoking Peruvian woman underwent a routine chest roentgenogram showing a large right-sided mass in the chest. The patient gave a 6-month history of shortness of breath, fatigue, and right shoulder pain without dysphagia. Physical examination revealed a healthy young woman with evidence of only diminished breath sounds over the right chest and mild right upper extremity weakness.
Computed tomographic scan of the chest revealed a large (12 x 10 cm) low-attenuation posterior mediastinal mass extending into the right lung and the subcarinal space (Fig 1). Positron emission tomographic scan showed mild, diffuse uptake in the mass, and a computed tomographic-guided core needle biopsy suggested a diagnosis of schwannoma. Barium esophagram demonstrated dilatation of the proximal esophagus, and esophagoscopy showed extrinsic compression from 22 to 38 cm from the incisors without mucosal ulceration. A magnetic resonance imaging scan of the chest showed no osseous, neural foraminal, vascular, or brachial plexus involvement.
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Fig 1. Computed tomographic scan of the chest showing a large, right-sided posterior mediastinal mass.
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The patient underwent a right posterolateral thoracotomy. The tumor was found to be arising from within the muscular layers of the esophagus and extending from the carina down to the gastroesophageal junction (Fig 2). Local excision was attempted but would have resulted in a greater than 75% circumferential defect in the remaining esophagus for a distance of at least 10 cm. A total thoracic esophagectomy with cervical esophagogastrostomy, pyloroplasty, and feeding jejunostomy was performed.
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Fig 2. Intraoperative appearance of a giant, plexiform esophageal schwannoma arising from the thoracic esophagus.
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Grossly, the tumor was encapsulated, which measured 15 x 15 x 4.5 cm and weighed 720 g. The cut surface showed variegated soft tan and yellow areas involving the esophageal wall along a length of 9 cm. Microscopically, the tumor was composed of compact bundles of spindle cells within loose myxoid areas in a plexiform or multinodular growth pattern. There was little mitotic activity (1/10 per high-powered field) and no necrosis. Immunohistochemical staining was positive for S100 and laminin, and was negative for CD117 and CD34.
The patient's hospital course was notable only for a local wound infection. She was discharged in good condition on postoperative day 10. The patient was well and without evidence of disease 1 year postoperatively, and she has since permanently returned to Peru.
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Comment
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The first report of a benign neurinoma of the esophagus was by Sanchez [1] in the Argentinian literature in 1956 followed by seven reports of similar lesions in the Japanese and European literature. The ability to accurately diagnose neurogenic tumors of the gastrointestinal tract did not become available until 1988 when Daimaru and colleagues [2] defined the morphologic features and immunohistochemical findings of a series of 24 benign gastrointestinal schwannomas. The authors found these lesions to be generally well-defined, confined to the muscularis propria contiguous with the myenteric plexus with compact bundles of spindle cells admixed with loose myxoid regions. Unlike soft tissue schwannomas, these tumors are characterized by peripheral lymphoid cuffing and show benign nuclear atypia defined as nuclei that vary in shape and size, but with uniform chromatin distribution. There are scant, if any, mitoses present. Immunohistochemical staining shows positivity for S-100 protein and Leu 7, and absence of smooth muscle markers such as actin, desmin and CD-34.
The series by Daimaru and colleagues [2] did not include an esophageal schwannoma. The first report in the English literature was by Eberlein and colleagues [3] in 1992, and the total number of cases in the world literature including this one, is only 18 with 15 benign esophageal schwannomas reported in English (Table 1). None were in association with von Recklinghausen's disease. The sizes and location within the esophagus vary widely. However, prior to this report all have been able to be excised without esophagectomy. In one case (Naus and colleagues), the tumor was excised endoscopically. The follow-up of these reports is inconsistent, but it seems that patients do well with complete resection. In our patient the tumor involved an extensive area of the esophagus intramurally, making it not feasible for local excision. This is the only reported case of a benign esophageal schwannoma requiring esophagectomy. There have been three other reports of esophagectomy for nerve sheath tumors involving the esophagus [4–6]. In one report, a patient was believed to have a malignant melanoma of the esophagus, but on final pathology it was found that the patient may have had a melanocytic schwannoma [4]. The remaining two esophagectomies were performed for malignant schwannomas [5, 6].
Benign esophageal schwannoma is a rare entity but must be a consideration when faced with a submucosal esophageal mass. The diagnosis can be made on morphologic and immunohistochemical grounds, and complete excision should yield good results. The overwhelming majority of patients will not require esophagectomy, but for large lesions suspected of diffuse esophageal involvement, patients should be prepared for the possibility.
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References
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- Sanchez ZJ. Benign neurinoma of the esophagus Bol Trab Soc Cir B Aires 1956;40:610-611.[Medline]
- Daimaru Y, Kido H, Hashimoto H, et al. Benign schwannoma of the gastrointestinal tracta clinicopathologic and immunohistochemical study. Hum Pathol 1988;19:257-264.[Medline]
- Eberlein TJ, Hannan R, Josa M, et al. Benign schwannoma of the esophagus presenting as a giant fibrovascular polyp Ann Thorac Surg 1992;53:343-345.[Abstract/Free Full Text]
- Brown RM, Darnton SJ, Papdaki L, et al. A primary tumour of the oesophagus with both melanocytic and schwannian differentiation. Melanocytic schwannoma or malignant melanoma? J Clin Pathol 2002;55:318-320.[Abstract/Free Full Text]
- Manger T, Pross M, Haeckel C, et al. Malignant peripheral nerve sheath tumor of the esophagus Dig Surg 2000;17:627-631.[Medline]
- Sanchez A, Mariangel P, Carrasco C, et al. Malignant nerve sheath tumor of the esophagus (malignant esophageal schwannoma) Gastroenterol Hepatol 2004;27:467-469.[Medline]
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Abstract
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