Background: Rarely, granular cell tumor (GCT) occurs in the sellar region. The natural history of this clinical entity is not well known. This study sought to determine the clinical features and long-term outcome of GCT of the sellar region.
Methods: Institutional databases, the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database, and PubMed/EMBASE were searched for GCT of the sellar region. Patient-level data were extracted where available, including age, sex, symptoms, tumor size and location, presumed diagnosis, surgical approach and result, subtotal resection (STR) and gross-total resection (GTR), use of radiation, and outcome. The primary endpoints of recurrence and survival were determined.
Results: A total of 141 cases were analyzed. The mean age at diagnosis was 48.9 (SD 15.3) with a female predominance (sex ratio 1.49:1). Almost all patients experienced either or both neurological and endocrine symptoms. The most common pre-operative diagnosis was pituitary adenoma. Approximately 60% of patients were treated with surgery, 57.7% with a craniotomy, and 39.7% with a transsphenoidal approach. The 5-year overall survival (OS) was 84.7% (standard error, SE 4.2). Patients with tumors less than 2.5 cm experienced a greater 5-year OS (100.0%) than patients with tumors 2.5 cm or larger (74.0%, SE: 11.7), (Mantel-Cox, p = 0.024). GTR resulted in a greater 5-year OS (95.7%, SE 4.3) than STR (88.8%, SE 5.5) and no surgery or biopsy alone (75.0%, SE 15.3) (Mantel-Cox, p = 0.016). The use of radiation therapy did not improve OS. The 5-year progression-free survival rate was 80.8% (SE 9.2).
Conclusions: Granular cell tumor of the sellar region is a relatively benign neoplasm with neurological and endocrine clinical manifestations. Surgical resection is appropriate for management. Tumor size and extent of resection influence overall survival.
Keywords: Granular cell tumor; Pituicytoma; Radiation therapy; Transsphenoidal surgery.