Ann Thorac Surg 2009;87:1603-1605. doi:10.1016/j.athoracsur.2008.09.010
© 2009 The Society of Thoracic Surgeons
Case Reports
Enucleation of a Giant Esophageal Gastrointestinal Stromal Tumor
Steven Milman, MD,
Anthony W. Kim, MD,
Erin Farlow, MD,
Michael J. Liptay, MD*
Rush University Medical Center, Chicago, Illinois
Accepted for publication September 2, 2008.
* Address correspondence to Dr Liptay, 1725 W. Harrison St, Suite 774, Chicago, IL 60612 (Email: michael_liptay{at}rush.edu).
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Abstract
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Despite development of novel chemotherapy for gastrointestinal stromal tumors (GISTs), complete resection remains the gold standard treatment. Because of the small number of reported esophageal gastrointestinal stromal tumors, the optimal extent of resection is not well defined. We present a case of an 82-year-old man with an 11-cm esophageal gastrointestinal stromal tumor who was successfully treated with enucleation.
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Introduction
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Gastrointestinal stromal tumors (GISTs) represent only 0.1% to 3% of all gastrointestinal tumors and esophageal GISTs comprise an even smaller cohort, accounting for < 1% to 5% of all GISTs [1]. These tumors most commonly cause dysphagia or are encountered incidentally, tending to remain undiagnosed until the tumor size is greater than 5 cm [2]. Predictors for poor outcome include tumor size > 10 cm and > 5 mitoses per high-power field [2, 3]. Although imatinib has become an adjuvant tool in the treatment of GIST tumors, it seems to be more useful in improving survival for unresectable disease or treating metastasis. Resection remains the optimal curative option. The following is a case of an 82 year-old man who successfully had an 11-cm esophageal GIST removed by enucleation.
An 82-year-old Caucasian man presented after a posterior mediastinal mass was found incidentally by computed tomography during work-up for nephrolithiasis. A dedicated chest computed tomographic scan then showed an 11-cm mass adjacent to the distal esophagus (Fig 1). The patient denied any history of dysphagia, odynophagia, or weight loss.His previous medical history was significant for remote tobacco use over 40 years ago.
At an outside hospital, a right thoracoscopic biopsy was attempted for diagnosis. However, this was aborted due to brisk bleeding that required conversion to a limited thoracotomy for hemostasis. Pathology from the biopsy was nondiagnostic. A follow-up magnetic resonance image showed a predominantly solid mass with some central necrosis (Fig 2). A positron emission tomographic scan demonstrated increased metabolic activity exclusively in the paraesophageal lesion with a maximum standardized uptake value of 11.
After transfer to our institution, an esophagoscopy was performed that demonstrated extrinsic compression of the esophagus at 30 cm to 38 cm from the incisors, without any intraluminal or mucosal lesions and a normal gastroesophageal junction. A right posterolateral thoracotomy was performed, at which point a large mass was immediately encountered. The mediastinal pleura were opened to better characterize the lesion. The esophagus was then encircled with a penrose above and below the mass. The mass appeared to be arising from the muscle layers of the esophagus. It was dissected off the esophagus by separating the muscular layers involved while leaving the mucosa intact. The mass was completely enucleated. The integrity of the submucosal and mucosal layers of the esophagus was tested with repeat endoscopy and insufflation of air.
The pathology revealed an 11.0 x 8.0 x 4.0 cm mass with an intact pseudocapsule and a necrotic core. The tumor was CD 117 (C-kit) and CD 34 positive, but staining for smooth muscle actin was only weakly positive (Fig 3). These findings were consistent with a GIST of the esophagus. The mass contained less than 5 mitoses per 50 high-power fields. Postoperatively, the patient underwent an esophagogram that confirmed the absence of an esophageal leak. His diet was advanced, and he was discharged after the removal of his chest tube catheter. At 1-year follow-up the patient is feeling well, and he has returned to his preoperative level of activity, although he has complaints of minor dysphagia and acid reflux while otherwise tolerating a regular diet. Follow-up computed tomographic scans have shown no evidence of recurrent disease.
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Fig 3. Microscopic appearance of the resected tumor. (A) Hematoxylin & eosin stain, x400. (B) C-kit, x100.
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Comment
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The largest description of esophageal mesenchymal tumors is from the Armed Forces Institute of Pathology [4]. This series, which spans 25 years, includes leiomyomas, leiomyosarcomas, or GISTs from 60 patients. The GISTs were distinguished from other tumors by staining positive for CD 34 and CD 117 (C-kit). Leiomyomas or sarcomas, on the other hand, were found to stain negative for these markers, but were strongly positive for smooth muscle actin. Of the 60 patients in the Armed Forces Institute of Pathology series, no patients with leiomyomas succumbed to their disease during follow-up, while all of the patients with leiomyosarcomas eventually died of their disease. Of the 17 patients (25%) who had a GIST, one half died during follow-up. Tumor size was found to be an important prognostic factor. All patients with tumors greater than 10 cm died versus all patients with tumors less than 5 cm who were still alive at last follow-up. A more recent series by Blum and colleagues [1] described 4 patients in 4 years who underwent enucleation of their esophageal GISTs. At 3-year follow-up, 2 patients were alive with no evidence of disease, and 1 patient later required esophagectomy for local recurrence. The remaining patient was still alive with metastatic disease on imatinib.
The patient presented in this case report did not have a definitive diagnosis at the time of resection, and frozen section analysis would not have allowed differentiation between GIST and leiomyoma. Because a tissue diagnosis could not be adequately made beforehand, the elderly patient in this report was not scheduled to undergo an esophagectomy in light of its attendant morbidity. After enucleation, the intended plan was to consider an esophagectomy if the tumor was to recur locally.
A review of patients at Memorial Sloan-Kettering showed a difference in survival of 66 months versus 22 months when comparing patients who achieved complete gross resection against incomplete resections [2]. Although it is clear that complete resection improves survival [1], the extent of resection is not well defined for esophageal GIST, given the small number of available cases.
The use of adjuvant imatinib in this patient was debated as its survival benefit is controversial. In a report on the ACOSOG Z9001 trial, patients with completely resected GISTs who received adjuvant imatinib immediately after surgery had lower recurrence rates. However, overall survival was not changed [5]. The patient in this report had a large, but low-grade tumor, and therefore, it was decided that imatinib would be used only if recurrence developed.
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References
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- Blum MG, Bilimoria KY, Wayne JD, et al. Surgical considerations for the management and resection of esophageal gastrointestinal stromal tumors Ann Thorac Surg 2007;84:1717-1723.[Abstract/Free Full Text]
- DeMatteo RP, Lewis JJ, Leung D, et al. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival Ann Surg 2000;231:51-58.[Medline]
- Hsu KH, Yang TM, Shan YS, Lin PW. Tumor size is a major determinant of recurrence in patients with resectable gastrointestinal stromal tumor Am J Surg 2007;194:148-152.[Medline]
- Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J. Esophageal stromal tumors Am J Surg Path 2000;24:211-222.[Medline]
- Dematteo RP, Ballman KV, Antonescu CR, et al. American College of Surgeons Oncology Group (ACOSOG) Intergroup Adjuvant GIST Study Team Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal tumour: a randomised, double-blind, placebo-controlled trial Lancet 2009Mar 18 [Epub ahead of print].
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Abstract
Introduction
