Granular Cell Tumor of the Neurohypophysis: 3 Cases and a Systematic Literature Review of 98 Cases

World Neurosurg. 2018 Oct:118:e621-e630. doi: 10.1016/j.wneu.2018.07.004. Epub 2018 Jul 11.


Background: Granular cell tumors (GCTs) of the neurohypophysis are rare neoplastic diseases of the pituitary. Because of the rare nature of the disease, previous descriptions have been limited to single-case studies, small series, or simple reviews.

Methods: We reported 3 cases in our medical center and systematically reviewed 98 cases who met inclusion criteria. Data were gathered on different aspects of GCTs' characteristics, including demographics, clinical features, laboratory features, histological features, immunocytochemical features, radiologic features, and treatment to elucidate any associations useful in determining pathogenesis and appropriate treatment.

Results: Female sex was significantly associated with GCTs (P < 0.001). The most common presenting symptom and tentative diagnosis were ophthalmologic defects in 61.2% and pituitary adenoma preoperatively in 18.4%, respectively. Most neurohypophysial GCTs possess immunopositivity for S-100 protein (59.1%, 26/44) and negative staining for glial fibrillary acidic protein (69.8%, 30/43).

Conclusions: These findings lend strong support to the suggestion that tumors probably remain in the transitional cell type. To our knowledge, our systematic review presents the largest number of symptomatic cases ever enumerated, with 98 cases meeting the inclusion criteria.

Keywords: Granular cell tumors; Neurohypophysis; Systematic literature review.

Publication types

  • Case Reports
  • Review
  • Systematic Review

MeSH terms

  • Adenoma / diagnostic imaging*
  • Adenoma / surgery
  • Adolescent
  • Adult
  • Aged
  • Child
  • Child, Preschool
  • Female
  • Granular Cell Tumor / diagnostic imaging*
  • Granular Cell Tumor / surgery
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Middle Aged
  • Pituitary Gland, Posterior / diagnostic imaging*
  • Pituitary Gland, Posterior / surgery
  • Pituitary Neoplasms / diagnostic imaging*
  • Pituitary Neoplasms / surgery
  • Young Adult