Solitary-fibrous tumor/hemangiopericytoma of the central nervous system: a population-based study

Abstract

The World Health Organization (WHO) classification of tumors of the central nervous system (CNS) was recently updated, restructuring solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) into one combined entity. This is the first population-based study to examine outcomes of SFT/HPC based on the new WHO guidelines. The Surveillance, Epidemiology, and End Results (SEER) database (1998-2013) was queried to examine age-adjusted incidence and prognostic factors associated with overall survival in 416 surgically resected cases. Age-adjusted incidence was calculated to be 3.77 per 10,000,000 and was rising. Median survival was 155 months, with 5- and 10-year survival rates of 78 and 61%, respectively. Younger age, Asian/Pacific Islander versus white race, benign histology, tumor location, gross-total resection (GTR), and GTR plus radiation (RT) versus subtotal resection were significantly associated with survival. In multivariable analysis, older age (HR = 1.038, p < 0.0001), infratentorial location (HR = 2.019, p = 0.038), GTR (HR = 0.313, p = 0.041), and GTR + RT (HR = 0.215, p = 0.008) were independent prognostic factors. In the HPC and borderline/malignant subgroups, GTR + RT was associated with significantly increased survival compared with GTR alone (HR = 0.537, p = 0.039 and HR = 0.525, p = 0.038). After eliminating patients that died within 3 months of diagnosis, GTR + RT was still associated with an incremental increase in survival (HR = 0.238, p = 0.031) over GTR alone (HR = 0.280, p = 0.054). GTR + RT may be optimal in the management CNS HPC and SFT/HPC tumors with borderline/malignant features. This study, in combination with existing literature, warrants further investigation of adjuvant radiation through a prospective clinical trial.

Spinal location is prognostic of survival for solitary-fibrous tumor/hemangiopericytoma of the central nervous system

“…Although these tumors are understood to share a biologic basis [7], they remain behaviorally distinct: SFTs occurring in the CNS tend to be classified as benign and are usually treated with resection alone [8,9], while HPCs are aggressive, known to metastasize systemically, and commonly recur [10][11][12][13]. HPCs of the CNS are frequently treated with adjuvant radiotherapy after resection [14,15].…”

“…The annual incidence rate of CNS SFT/HPC in the United States is 3.77 per 10,000,000 people and is rising [14]. The rarity of this disease and accompanying paucity of clinical trials has made optimal management difficult to establish [16].…”

Extrameningeal solitary fibrous tumors—surgery alone or surgery plus perioperative radiotherapy: A retrospective study from the global solitary fibrous tumor initiative in collaboration with the Sarcoma Patients EuroNet

“…Furthermore, detailed conclusions on subgroups (eg, by site) may lack statistical power. Because SFT is a rare disease with an estimated 230 cases per year in the United States, it is unlikely that prospective, randomized, clinical trials will ever be conducted 28 …”

Clear Cell Adenocarcinoma of the Urinary Bladder Is a Glycogen-Rich Tumor with Poorer Prognosis

“…Incidence and mortality were age-adjusted by standardizing to the 2000 United States Census population. All other data collection and analysis were conducted as described previously [16,25]. We obtained the November, 2015 submission [26] and November, 2017 submission [27] from the SEER Program database and merged all identified cases of malignant cancers of the bladder identified by International Classification of Diseases-O-3 (ICD-O-3) codes C67.0-C67.9 from January 2004 to December 2015.…”