Clinical characteristics and surgical treatment of schwannomas of the esophagus and stomach: A case series and systematic review

Abstract

Background: Gastrointestinal schwannomas are slow-growing benign mesenchymal neoplasms that originate from Schwann cells of the nerve sheath of Auerbach´s plexus or less frequently from Meissner´s plexus. The main differential diagnosis of gastric schwannomas are the gastrointestinal stromal tumors (GISTs), which are classified by their immunohistochemistry. The treatment of choice for gastric schwannomas is surgery where laparoscopy plays an important role. Wedge resection, subtotal or total gastrectomy can be done. In its counterpart, esophageal schwannomas are benign tumors of the esophagus that are very uncommon since they comprise less than 2% of all esophageal tumors. The main differential diagnosis is the leiomyoma which corresponds to the most common benign esophageal tumor, followed by GIST. The treatment consists on tumoral enucleation or esophagectomy.

Aim: To review the available literature about gastrointestinal schwannomas; especially lesions from de stomach and esophagus, including diagnosis, treatment, and follow up, as well as, reporting our institutional experience.

Methods: A systematic review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyzes guidelines. The following databases were used for reviewing process: PubMed, Ovid, MEDLINE, and Scopus. Only English language manuscripts were included. All gastrointestinal schwannomas specifically located in the esophagus and stomach were included. Cases that did not report long-term follow-up were excluded.

Results: Gastric localization showed a higher prevalence in both, the literature review and our institution: 94.95% (n = 317) and 83% (n = 5) respectively. With a follow-up with disease-free survival greater than 36 mo in most cases: 62.01% (n = 80) vs 66.66% (n = 4). In both groups, the median size was > 4.1 cm. Surgical treatment is curative in most cases.

Conclusion: Schwannoma must be taken into account in the differential diagnosis of gastrointestinal mesenchymal tumors. It has a good prognosis, and most are benign. A disease-free survival of more than 36 mo can be achieved by surgery.

Keywords: Esophagus; Schwannoma; Stomach; Surgery; Systematic review.

Figure 1

Immunohistochemistry and macroscopic images. A: The immunohistochemistry was diffusely positive for S100 (in the nucleus and cytoplasm) in the proliferative cells; which confirms its histomorphogenesis of the cells of schwann. This image corresponds to the esophagic schwannoma resected at our institute; B: Macroscopic image of the esophageal schwannoma.

Figure 2

Nulcear palisading around fibrillary process (verocay bodies) is often seen in cellular areas. This image corresponds to the esophagic schwannoma resected at our institute.

Figure 3

Flowchart.

Figure 4

Resection of a gastric schwannoma. A: Tumor of approximately 10 cm; B: Wedge resection by linear cutting stapler of the greater gastric curvature.