Granular Cell Tumor of the Esophagus in an Adolescent Girl

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

Journal of Pediatric Gastroenterology & Nutrition: June 2012 - Volume 54 - Issue 6 - p 715
doi: 10.1097/MPG.0b013e3182446b6a
Image of the Month

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, and will undergo peer review by members of the NASPGHAN Endoscopy and Procedures Committee, as well as by the Journal.

Article Outline

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.

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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