Occurrence of Esophageal Granular Cell Tumors in The Netherlands Between 1988 and 1994 JAN HENK VOSKUIL, MD,* MARTIN M. VAN DIJK, MD,† SJOERD SC. WAGENAAR, MD, PhD,‡ ADRI C.M. VAN VLIET, MD, PhD,§ ROBIN TIMMER, MD, PhD,¶ and PIET A.M. VAN HEES MD, PhD¶ Granular cell tumors (GCT) of the esophagus are rare. The tumor is generally beleived to be of neurogenic origin and shows a malignant course in 2– 4% of cases. No unanimity has been reached regarding the management of this tumor. A national survey was conducted on the incidence of GCT of the esophagus, related symptoms, management, and follow-up. A national survey was performed on all newly registered esophageal GCTs in the PALGA system (Dutch register of all pathology diagnoses) for seven consecutive years (1988 –1994). Fifty-two new cases (17 men, 35 women; median age 46 years, range 22–77 years) were registered. In 44 cases clinical data could be obtained (survey response 85%). The majority of the GCTs were solitary (42/44) and localized in the distal esophagus (33/44). At endoscopy the size of the tumor was estimated at 5 mm in 50%, 5–10 mm in 25%, and 10–30 mm in 18%. Most patients (40/44) presented with nonspecific gastrointestinal symptoms, only four had dysphagia (tumor size 1 cm). No malignancies were reported. Management of the tumor included excisional biopsy (1/44), endoscopic polypectomy (3/44), and surgical excision (1/44). Endoscopic follow up (1– 60 months) in 16 out of 17 patients left untreated showed either a stable tumor size or regression of the tumor. In one case with multiple GCT’s a slight tumor growth was seen after a follow-up period of 48 months. Esophageal GCTs in the Netherlands are rare, and mostly diagnosed incidentally. Most patients suffer from nonspe- cific symptoms; dysphagia occurs only with tumors 1 cm. The usual clinical course of esophageal GCTs is benign. Patients without dysphagia probably do not require routine endoscopic follow-up, provided they are instructed to contact their physician, once dysphagia develops. KEY WORDS: granular cell tumor; esophagus; endoscopy. Since Abrikossoff first reported five cases of granular cell tumor (GCT) of the tongue in 1926, this tumor has been reported in many different locations throughout the body (1). The gastrointestinal tract is one of the more uncommon locations of GCTs. It accounts for about 8% of all GCT locations (2). About 2% occur in the esophagus (3, 4). Although there has been much controversy about Manuscript received February 17, 2000; accepted December 28, 2000. From the *Department of Gastroenterology, De Tjongerschans Hospital, Heerenveen; †Department of Pathology, Groene Hart Hospital, Gouda; ‡Department of Pathology, Onze Lieve Vrouwe Gasthuis, Amsterdam; §Department of Internal Medicine, Albert Schweitzer Hospital, Dordrecht; and ¶Department of Gastroenter- ology, St. Antonius Hospital, Nieuwegein, The Netherlands. Address for reprint: Dr. PAM van Hees, Department of Gastro- enterology, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, the Netherlands. Digestive Diseases and Sciences, Vol. 46, No. 8 (August 2001), pp. 1610 –1614 1610 Digestive Diseases and Sciences, Vol. 46, No. 8 (August 2001) 0163-2116/01/0800-1610$19.50/0 © 2001 Plenum Publishing Corporation