Description:

Granular cell tumors (GCT) are relatively uncommon, but can occur through out the length of the gastrointestinal tract, particularly in the esophagus (1). Endoscopically, they appear as firm yellow nodules and can be mistaken for lipomas, carcinoid tumors and gastrointestinal stromal tumors.

GCT generally behave in a benign manner, although malignant degeneration can occur about 3% of the time (2). Tumors that are less than 1 cm in size and that are confined to the superficial parts of the gastrointestinal tract, not involving the muscularis propria, are amenable to endoscopic management (3). Endoscopic ultrasound can be used to determine the size and suitability for resection.

This case involves a 57 yr old woman who was discovered to have a small subepithelial nodule in the distal esophagus during an evaluation for reflux.

She was referred for endoscopic ultrasound evaluation.

She had no symptoms related to this finding, and had a normal physical examination.

At endoscopy, a subtle lesion was seen just above the gastroesophageal junction. This was interrogated and exposed using a cold biopsy forceps. EUS revealed a 3.5 mm lesion that was in echo layers II and III, and did not appear to involve the muscularis propria.
This was felt amenable to resection due to it's small size and superficial nature as defined by ultrasonography. Resection was felt to be indicated since the firm nature and yellow color of the lesion was concerning for a granular cell tumor.
Band ligation EMR was performed, and the histology revealed a completely resected granular cell tumor.

This case illustrates an example of a low risk granular cell tumor of the gastrointestinal tract, making her a good candidate for endoscopic management.
Features associated with poor outcome in granular cell tumors include size greater than 40 mm, rapid growth, tumor necrosis, cytological atypia, and histology showing more than two mitoses per high-power field (2), and more aggressive surgery is indicated in patients with these features.

The majority of granular cell tumors with low risk features (2) are able to be managed conservatively (3) and a benign course is anticipated in our patient. Surveillance endoscopy will be performed in two years to watch for local recurrence and to evaluate for other lesions which could be small synchronous tumors not detected at this time, or metachronous lesions forming later (4).

Reference:
1)Johnston J, Helwig EB Granular cell tumors of the gastrointestinal tract and perianal region: a study of 74 cases. Dig Dis Sci. 198126:807-16

2)Lowe DL, Chaudhary AJ, Lee JR, Chamberlain SM, Schade RR, Cuartas-Hoyos U. Four cases of patients with gastrointestinal granular cell tumors South Med J. 2007 100:298-300.

3)Orlowska J, Pachlewski J, Gugulski A, Butruk E. A Conservative Approach to Granular Cell Tumors of the Esophagus: Four Case Reports and Literature Review. Am J Gastroenterol 1993; 88(2): 311-315

4) Maekawa,H, Maekawa,T, Yabuki,K, Sato,K, Tamazaki,Y, Kudo,K, Wada,R, and

Contributed By:

John C. Deutsch, MD, St. Mary’s Duluth Clinic