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The Turkish Journal of Gastroenterology
2007, Volume 18, No 3, Page(s) 188-191
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Granular cell tumor of esophagus removed with endoscopic submucosal dissection
Sadettin HÜLAGÜ, Ömer ŞENTÜRK, Cem AYGÜN, Yeflim GÜRBÜZ, Orhan KOCAMAN, Altay ÇELEBİ, Tolga KONDUK
Department of Gastroenterology, Kocaeli University, Faculty of Medicine, Kocaeli
Keywords: Esophagus, granular cell tumor, endoscopic submucosal dissection.
Summary
Granular cell tumors of the esophagus are rare neoplasms and their diagnosis is mainly based on histopathologic examination of endoscopic biopsies. With the development of endoscopy and iodine staining, there has been a marked increase in local treatment alternatives for early esophageal neoplasms. In this case report, we describe a granular cell tumor of the esophagus in an adult patient and briefly discuss the literature concerning clinicopathologic aspects and management of these uncommon tumors. In this patient, we describe the removal of the tumor by endoscopic submucosal dissection technique.
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  • Summary
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • Introduction
    Granular cell tumors (GCT), first described by Abrikossoff in 1926, occur throughout the body, most commonly in the tongue and skin, but also in the breast, respiratory tract, biliary tree, nervous system and gastrointestinal (GI) tract (1,2). Tumors involving the GI tract account for 8% of all GCT (1). Among the GCTs involving the GI tract, only 2% are found in the esophagus (2,3). The first case of esophageal GCT was also described by Abrikossoff in 1931 (4) and, since that time, over 200 cases have been reported in the literature (5). In this case report, we aimed to describe a GCT of the esophagus in an adult patient and its removal by endoscopic submucosal dissection technique. We also briefly discuss the literature concerning clinicopathologic aspects and management of these uncommon tumors.
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  • Summary
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • Case Presentation

    A 51-year-old Caucasian male had a 5-year history of progressively worsening epigastric discomfort. Additionally, he had a 6-month history of intermittent dysphagia with epigastric pain radiating to the retro-sternum. He was treated with proton pump inhibitors; however, ongoing dysphagia and pain led to his referral to the gastroenterology outpatient clinic.

    An upper GI endoscopic examination was performed which revealed a yellow submucosal tumor with central depression, located 37 cm distal from the incisor teeth (Figure 1). Microscopical examination of biopsy specimens from the lesion demonstrated a neoplasia consisting of nests and cords of elongated and polygonal cells containing small dark nuclei and abundant granular eosinophilic cytoplasm. Overlying epithelium had pseudoepitheliomatous hyperplasia. Based on these histopathological findings, the lesion was diagnosed as a GCT (Figure 2). Computed tomography scan of his chest and abdomen revealed no additional pathology except the solid mass in the distal esophagus. Esophageal endosonography revealed a 10 x 14 mm thick hypoechoic mass in the esophageal wall. The mass was located in the submucosa, and the muscularis propria was intact. There were no periesophageal or celiac lymph nodes. The patient subsequently underwent an endoscopic submucosal dissection (ESD) using sodium hyaluronate, a small-caliber-tip transparent hood and a needle knife according to the method developed by Yamamoto (6). ESD procedure was carried out by a single- channel upper GI endoscope (Fujinon Co, Japan) and a high frequency generator with an automatically controlled system (Endocut mode; Erbotom, ERBE Elektromedizin GmbH, Germany). A transparent cap was fitted on the tip of the endoscope to obtain a constant view and to create a tension on the connective tissue for dissection. Lugol chromoendoscopy was done to mark the margins of lesion. With the use of an electrosurgical needle knife, dots were placed about 5 mm outside of the margins at 2 mm intervals. Following an initial injection of 2 ml saline, a submucosal fluid cushion was created using a solution prepared with hyaluronic acid, saline, indigo carmine and epinephrine mixture. About 2 ml of the solution was injected into the submucosal layer at a time and repeated until the mucosa was lifted. After lifting the lesion, the mucosa was gently cut with the electrosurgical needle knife, using the Endocut mode. En bloc resection with tumor-free lateral/basal margins was accomplished. To control minor bleedings, hemostatic forceps were used in soft coagulation mode. The patient was discharged after one week without any complication. This method enabled submucosal incision under direct visualization and thus allowed precise determination of both the lateral and vertical margins to be resected (Figures 3, 4). Microscopically, the surgical margin was free of tumor cells. Follow-up endoscopy 6 weeks after removal of tumor showed scar tissue from prior mucosal dissection; no masses or nodules were detected (Figure 5).

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  • Summary
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • Discussion

    Granular cell tumors are uncommon tumors in the GI tract and are very rare in the esophagus. Most esophageal GCTs are found incidentally during endoscopy, upper GI contrast studies, or at autopsy. Although patients usually have no symptoms, those with tumors larger than 1 cm may report dysphagia (5). Nausea, vomiting, and retrosternal or epigastric pain occur less frequently (7).

    The endoscopic appearance of a GCT is characteristic. The tumor usually appears as a yellow, firm, polypoid submucosal mass. Differential diagnosis should include esophageal cyst, epithelial lesions such as glycogenic acanthosis, inflammatory polyp, and squamous papilloma, and other submucosal tumors, such as leiomyoma, lipoma, and hamartoma.

    Many kinds of cells have been postulated as an origin of the tumor including myoblasts, histiocytes, perineural fibroblasts, Schwann cells, and undifferentiated mesenchymal cells (8). Theories regarding the non-neoplastic nature of the lesion and its association with inflammatory as well as degenerative processes have also been considered. More strongly, the evidence of a close association of tumor cells with peripheral nerves, and the presence of S-100 protein, neuron-specific enolase, and myelin proteins on immunohistochemical stains support a Schwann cell derivation (9). Histologically, these tumors consist of polygonal and fusiform cells disposed in compact "nests" (10). Cells have small dark nuclei and abundant, fine, granular eosinophilic acid-Schiff–positive, diastase-resistant cytoplasm (11). GCTs of the skin, larynx, and esophagus are known to induce pseudoepitheliomatous hyperplasia in the malpighian epithelium. This feature may simulate a primary squamous cell carcinoma (2, 12).

    Although the natural history of the tumor is unclear, most esophageal GCTs have a benign clinical course. The infiltrative pattern of growth and the presence of metastases are important features in differentiating between malignant and benign tumors because histologically they may appear very similar. Malignant lesions are usually larger than 4 cm, display rapid recent growth, tend to recur locally after resection, and may have such subtle histologic features as nuclear pleomorphism, increased nuclear size, tumor cell necrosis, large nucleoli, mitotic figures (2 or more/10 HPF), and tumor cell spindling (13).

    Treatment of esophageal tumors has recently included saline-assisted endoscopic mucosal resection (EMR), which is considered a safe, successful procedure, and complications are uncommon (14). In a series of 650 esophageal mucosal cancers removed with EMR, Makuuchi (15) reported a complication rate of 4.8% (perforation 0.7%, bleeding 3.1%, stricture 1.6%). ESD for esophageal pathology has been recently established and the affected mucosa is incised and removed using a variety of endoscopic electrosurgical knives. Using ESD, a wider range of the mucosa can be resected in one piece more reliably using an endoscope. In our case, after submucosal injection of sodium hyaluronate to maintain sufficient thickening of the submucosal tissue, dissection of the mucosa and submucosa with a needle knife was performed by the method developed by Yamamoto (6). We preferred ESD for its more accurate resections than conventional EMR and sodium hyaluronate solution for it ability to maintain submucosal elevation for a longer time. Additional esophagectomy with lymph node dissection was not required since no submucosal invasion or vessel permeation was seen in the endoscopically resected specimen.

    In summary, granular cell tumors of the esophagus are rare neoplasms. Endoscopic biopsies are the mainstay of diagnosis. Endoscopic and endosonographic evaluation of the lesion defines the location and extension of the tumor and its suitability for endoscopic treatment. Endoscopic submucosal dissection is a safe and accurate procedure for selected cases.

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  • Summary
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • References
    1. Abrikossoff AI. Uber Myome, augehend von der quergestreiften willkurlichen Muskulatur. Virchows Arch Pathol Anat 1926; 260: 214-33.
    2. Johnston MJ, Helwig EB. Granular cell tumors of the gastrointestinal tract and perianal region: a study of 74 cases. Dig Dis Sci 1981; 26: 807-16.
    3. Lack EE, Worsham GF, Callihan MD, et al. Granular cell tumors: a clinicopathologic study of 110 cases. J Surg Oncol 1980; 13: 301-6.
    4. Abrikossoff A. Weitere unterschunger ueber Myoblastenmyome. Virchows Arch Pathol Anat 1931; 280: 723-40.
    5. Goldblum JR, Rice TW, Zuccaro G, et al. Granular cell tumors of the esophagus: a clinical and pathologic study of 13 cases. Ann Thorac Surg 1996; 62: 860-5.
    6. Yamamoto H, Kawata H, Sunada K, et al. Successful en bloc resection of large superficial tumors in the stomach and colon using sodium hyaluronate and small-caliber-tip transparent hood. Endoscopy 2003; 35: 690-4.
    7. Brady PG, Nord HJ, Connar RG. Granular cell tumor of the esophagus: natural history, diagnosis, and therapy. Dig Dis Sci 1988; 33: 1329-33.
    8. Ordonez NG, Mackay B. Granular cell tumor: a review of the pathology and histogenesis. Ultrastruct Pathol 1999; 23: 207-22.
    9. Seo IS, Azzarelli B, Warner TF, et al. Multiple visceral and cutaneous granular cell tumors. Ultrastructural and immunocytochemical evidence of Schwann cell origin. Cancer 1984; 53: 2104-10.
    10. Giacobbe A, Facciorusso D, Conoscitore P, et al. Granular cell tumor of the esophagus. Am J Gastroenterol 1988; 83: 1398-400.
    11. Cohle SD, McKechnie JC, Truong L, et al. Granular cell tumor of the esophagus. Am J Gastroenterol 1981; 75: 431-5.
    12. Gibbons JR, Bharucha H, Soorae AS. Granular cell tumor of the esophagus. Am J Gastroenterol 1980; 74: 161-4.
    13. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, et al. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol 1998; 22: 779-94.
    14. Soetikno RM, Gotoda T, Nakanishi Y, et al. Endoscopic mucosal resection. Gastrointest Endosc 2003; 57: 567-79.
    15. Makuuchi H. Endoscopic mucosal resection for mucosal cancer in the esophagus. Gastrointest Endosc Clin North Am 2001; 11: 445-58.
  • Top
  • Summary
  • Introduction
  • Case Presentation
  • Discussion
  • References
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