Understanding esophageal neurofibroma: A case series and systematic review

Esophageal neurofibroma is a rare benign esophageal neoplasm. With very few cases documented in the literature, not much is known about the demographics and clinicopathologic features of this tumor. This study was aimed at presenting a case report of an esophageal neurofibroma, and to conduct a systematic review of published cases.

This review was performed according to the PRISMA guidelines. Literature search was conducted through PubMed, SCOPUS, and Cochrane Databases from inception until May 2020 for all histologically confirmed cases of esophageal neurofibroma.

28 cases, including the newly reported case, were included in the review. The mean age at diagnosis was 53.3 years ±12.1. 53.6% were male. Dysphagia was the most common presenting symptom (53.6%). Most of the reported cases involved the upper esophagus (39.3%). The most utilized diagnostic test was esophagogastroduodenoscopy (57.1%). The mean tumor size was 6.1 cm ± 5.1. Preoperative biopsy was done for 9 cases, out of which seven were negative or inconclusive. In 17 cases (60.7%), immunohistochemical (IHC) staining of the resected tumor was not performed. S100 was the most utilized IHC stain. Enucleation (39.3%) was the most common treatment, followed by esophagectomy (28.6%).

Esophageal neurofibroma should be considered in the setting of dysphagia caused by a subepithelial tumor. Accurate preoperative histologic diagnosis by using a well-defined biopsy algorithm, in conjunction with IHC analysis, will favor less aggressive surgical treatment and surveillance of asymptomatic lesions. Minimally invasive surgical treatment is feasible and should be considered when the expertise is available.