Esophageal Granular Cell Tumor in Children: A Clinicopathologic Study of 11 Cases and Review of the Literature

Am J Clin Pathol. 2023 Jul 5;160(1):106-112. doi: 10.1093/ajcp/aqad025.


Objectives: Granular cell tumor (GCT) commonly presents in the subcutaneous tissue and head and neck region, and it is uncommon in the gastrointestinal tract. Experience with esophageal GCTs in the pediatric population is limited, with only 7 cases reported in the literature, 3 with eosinophilic esophagitis (EoE).

Methods: Case information from 11 pediatric patients with GCTs of the esophagus was retrieved. H&E and immunohistochemical slides were reviewed with clinical, endoscopic, and follow-up data from all patients.

Results: In total, 7 male and 4 female patients were included, with ages ranging from 3 to 14 years. Indications for esophagogastroduodenoscopy (EGD) included EoE (n = 3), follow-up for Crohn disease, and other nonspecific complaints. Endoscopically, all patients had a single submucosal, firm mass protruding into the lumen, with normal overlying mucosa. The nodules were removed endoscopically in multiple fragments in all cases. Histologically, the tumors showed sheets and trabeculae of cells containing bland nuclei, inconspicuous nucleoli, and abundant pink granular cytoplasm without atypical features. All tumors were immunoreactive for S100, CD68, and SOX10. Follow-up showed that all patients were disease-free (median, 2 years).

Conclusions: We report the largest series of pediatric esophageal GCTs with coincidental association with EoE. These EGD findings are characteristic, and removal by biopsy is both diagnostic and therapeutic.

Keywords: Eosinophilic esophagitis; Esophagus; Granular cell tumor; Pediatric; S100; SOX10.

Publication types

  • Review

MeSH terms

  • Biopsy
  • Child
  • Esophageal Neoplasms* / complications
  • Esophageal Neoplasms* / pathology
  • Female
  • Granular Cell Tumor* / complications
  • Granular Cell Tumor* / pathology
  • Humans
  • Immunohistochemistry
  • Male