title: Occurrence of esophageal granular cell tumors in The Netherlands between 1988 and 1994.
abstract: 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 nonspecific 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.
authors.affiliation: Department of Gastroenterology, De Tjongerschans Hospital, Heerenveen, The Netherlands.
authors.forename: J H
authors.initials: JH
authors.lastname: Voskuil
authors.forename: M M
authors.initials: MM
authors.lastname: van Dijk
authors.forename: S S
authors.initials: SS
authors.lastname: Wagenaar
authors.forename: A C
authors.initials: AC
authors.lastname: van Vliet
authors.forename: R
authors.initials: R
authors.lastname: Timmer
authors.forename: P A
authors.initials: PA
authors.lastname: van Hees
mesh_terms: D000328:Adult; D000368:Aged; D004938:Esophageal Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology; D016586:Granular Cell Tumor / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology; D006801:Humans; D015994:Incidence; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology