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Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e3182446b6a
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Granular Cell Tumor of the Esophagus in an Adolescent Girl

Mohammad, Saeed; Naiditch, Jessica A.; Jaffar, Reema; Rothstein, David; Bass, Lee M.

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Feinberg School of Medicine, Northwestern University, Children's Memorial Hospital, Chicago, IL

Address correspondence and reprint requests to Saeed Mohammad, MD, 2300 Children's Plaza, Box 65, Children's Memorial Hospital, Chicago, IL 60614.

Submissions for the Image of the Month should include high-quality TIF endoscopic images of unusual or informative findings. In addition, 1 or 2 other associated photographs, such as radiological or pathological images, can be submitted. A brief description of no more than 200 words should accompany the images. Submissions are to be made online at www.jpgn.org, and will undergo peer review by members of the NASPGHAN Endoscopy and Procedures Committee, as well as by the Journal.

A 16-year-old girl presented with a 1-year history of dysphagia. She had a history of granular cell tumor (GCT) of the esophagus diagnosed 2 years earlier and eosinophilic esophagitis treated with an elimination diet. A barium esophagram was normal, and on endoscopy, her esophagus appeared grossly normal with a 9-mm tan-yellow lesion in the lower esophagus, 35 cm from the mouth (Fig. 1). Histology confirmed the diagnosis of GCT (Fig. 2) and revealed increased eosinophils suggestive of partially treated eosinophilic esophagitis. Because of increasing chest discomfort, she was referred to surgery for removal of the tumor for symptom relief and confirmation of a benign cause. Initial thoracoscopic dissection revealed that the tumor was intimate with the mucosa, precluding enucleation, and a right lower lateral thoracotomy was performed to enable a full esophageal wall resection. The tumor was excised, resulting in a circumferential defect, which was repaired primarily in 2 layers and then buttressed with mediastinal pleura. A postoperative esophagram revealed no anastomotic leak and a mild narrowing of the distal esophagus. Two months later, endoscopy for continued dysphagia revealed a distal esophageal stricture, which was successfully dilated with a 12-F balloon. She has had no further evidence of stricture and her dysphagia has resolved.

Figure 1
Figure 1
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Figure 2
Figure 2
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GCTs are rare tumors of the head and neck, with 4% to 6% located in the gastrointestinal tract (1). One-third are found in the esophagus with a female predilection (1,2). There are few reported cases in children (3,4). The endoscopic appearance is of a yellow intramural lesion covered by normal mucosa and is described as a “submucosal pill appearance.” They are usually benign, with only 2% reported as malignant (5). Most patients are asymptomatic; however, in those with symptoms, dysphagia is common (6). Endoscopic ultrasound is useful for follow-up and treatment guidance, whereas surgical excision may be performed if malignancy is suspected or for symptomatic relief (7).

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REFERENCES

1. Johnston J, Helwig EB. Granular cell tumors of the gastrointestinal tract and perianal region: a study of 74 cases. Dig Dis Sci 1981; 26:807–816.

2. Morrison JG, Gray GF Jr, Dao AH, et al. Granular cell tumors. Am Surg 1987; 53:156–160.

3. Buratti S, Savides TJ, Newbury RO, et al. Granular cell tumor of the esophagus: report of a pediatric case and literature review. J Pediatr Gastroenterol Nutr 2004; 38:97–101.

4. Finck C, Moront M, Newton C, et al. Pediatric granular cell tumor of the tracheobronchial tree. J Pediatr Surg 2008; 43:568–570.

5. 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; 22:779–794.

6. Narra SL, Tombazzi C, Datta V, et al. Granular cell tumor of the esophagus: report of five cases and review of the literature. Am J Med Sci 2008; 335:338–341.

7. Palazzo L, Landi B, Cellier C, et al. Endosonographic features of esophageal granular cell tumors. Endoscopy 1997; 29:850–853.

Copyright 2012 by ESPGHAN and NASPGHAN

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