Abstract
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.
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Our institution has exempted the SEER database from Institutional Review Board (IRB) approval. We had IRB approval for the query of and use of data from our institutional neuropathology databases (IRB Approval #: 2015P002352).
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Ahmed, AK., Dawood, H.Y., Penn, D.L. et al. Extent of surgical resection and tumor size predicts prognosis in granular cell tumor of the sellar region. Acta Neurochir 159, 2209–2216 (2017). https://doi.org/10.1007/s00701-017-3337-3
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DOI: https://doi.org/10.1007/s00701-017-3337-3