Occurrence of esophageal granular cell tumors in the Netherlands between 1988 and 1994

Granular cell tumors (GCT) of the esophagus are rare. The tumor is generall y 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 tu mor. A national survey was conducted on the incidence of GCT of the esophag us, related symptoms, management, and follow-up. A national survey was perf ormed on all newly registered esophageal GCTs in the PALGA system (Dutch re gister 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 ye ars) were registered. In 44 cases clinical data could be obtained (survey r esponse 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 est imated 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 ha d dysphagia (tumor size >1 cm). No malignancies were reported. Management o f the tumor included excisional biopsy (1/44), endoscopic polypectomy (3/44 ), and surgical excision (1/44). Endoscopic follow up (1-60 months) in 16 o ut of 17 patients left untreated showed either a stable tumor size or regre ssion of the tumor. In one case with multiple GCT's a slight tumor growth w as seen after a follow-up period of 48 months. Esophageal GCTs in the Nethe rlands are rare, and mostly diagnosed incidentally. Most patients suffer fr om nonspecific symptoms; dysphagia occurs only with tumors >1 cm. The usual clinical course of esophageal GCTs is benign. Patients without dysphagia p robably do not require routine endoscopic follow-up, provided they are inst ructed to contact their physician, once dysphagia develops.