Electronic Clinical Challenges and Images in GI

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• Answer to the Clinical Challenges in GI Question: Image 3: Granular Cell Tumor or Abrikossoff Tumor of the Esophagus
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Question: A 39-year-old woman was referred for endoscopic evaluation of heartburn with no significant improvement after proton-pump inhibitor treatment. Mild constipation was reported; otherwise, she was free of symptoms. The patient denied nausea, vomiting, fever, dysphagia, weight loss, or the passage of blood. Her records stated a normal upper endoscopy 17 years ago. Clinical examination was unremarkable but for mild obesity. All laboratory tests were within normal limits. Long-standing hypertension was controlled by an angiotensin-converting enzyme inhibitor; no other medications were taken. Endoscopy revealed a nonobstructive, yellowish, submucosal mass of about 8 mm in the mid esophagus (Figure A). On biopsy, the mass seemed to be solid. The remaining upper endoscopy was normal without signs of erosive acid reflux disease. Endoscopic ultrasound revealed a homogenous, well-circumscribed, hypoechogenic tumor 7 mm in diameter extending from the submucosal layer. The muscularis propia, however, was not involved (Figure B).

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What might the diagnosis of this tumor be?

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Answer to the Clinical Challenges in GI Question: Image 3: Granular Cell Tumor or Abrikossoff Tumor of the Esophagus 

Histopathologic examination revealed subepithelial, sheet-like cell formations with intersections of smooth muscles (Figure D). The cells contained a small, round–oval nucleus without atypia and an eosinophilic, periodic acid-Schiff–positive, bright cytoplasma. These cell formations were covered with a normal squamous epithelium without dysplasia. Immunostains for S-100 (Figure C) and vimentin were positive and negative for CD 117, desmin, Lu-5, and actin. The diagnosis of a granular cell tumor (GCT) was made.

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GCTs were first described 1926 by Abrikossoff.¹ This uncommon tumor can occur throughout the body. Only 4%–10 % of GCTs are located in the gastrointestinal tract, mainly in the esophagus. Of the different benign esophageal tumors, 1% are GCTs. Most GCTs are identified as a painless mass by incidental finding on endoscopy as in our case. Some esophageal GCTs cause dysphagia or, on rare occasions, chest pain.

The endoscopic appearance is a yellow-grayish, intramural tumor covered with normal squamous epithelium. The firm consistency of the tumor gave rise to the description as a “submucosal pill.”² The endoscopic ultrasound picture shows a homogenous, hypoechogenic tumor with a sharp border and a normal mucosa. Histologic diagnosis is based on the criteria mentioned.

Only about 2% of all GCTs fulfill the histologic criteria for malignancy.³ Therefore, strong evidence for resection does not exist. In the gastrointestinal tract small, asymptomatic tumors can safely be observed. Large and/or symptomatic GCTs can be removed either during endoscopy or operatively. Because of the comparatively high risk of perforation, tumors >2 cm in diameter should be removed operatively.

Having discussed these options with our patient, she decided to have the tumor removed. After submucosal injection snare resection was performed without complications. We do not see any causal correlation between the tumor and heartburn.

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References 

1. Abrikossoff A. Über Myome ausgehend von der quergestreiften willkürlichen Muskulatur. Virchows Arch Pathol Anat. 1926;260:215–233
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2. Harikumar R. Abrikossoff‘s tumor of the esophagus: case report and review of the literature. Trop Gastroenterol. 2006;24:52–53
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3. Fanburg-Smith JC. Malignant granular-cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. 1998;22:779–794
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