Tumeurs fibreuses solitaires et hémangiopéricytomes des méninges : immunophénotype et évaluation du grade histopronostique dans 17 cas

Savary, Caroline; Rousselet, Marie‐Christine; Michalak, Sophie; Fournier, Henri-Dominique; Taris, M.; Loussouarn, Delphine; Rousseau, Audrey

doi:10.1016/j.annpat.2016.06.002

Cited by 9 publications

(1 citation statement)

References 33 publications

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“…Around 2013, immunohistochemical staining for STAT6 was presented in the literature as a more specific marker for SFTs and HPC. [ 3 , 11 , 19 ] A NAB2-STAT6 gene fusion, results in an inversion at the 12q13 locus, produces a chimeric protein in which a repressor domain of NAB2 (EGR1-binding protein 2) (NAB2) is replaced with a carboxyterminal transactivation domain from signal transducer. The activator of transcription 6 and interleukin-4 induced (STAT6) acts as a transcriptional activator through early growth of the tumor and has been recently believed to be highly sensitive and specific for SFTs.…”

Section: Discussionmentioning

confidence: 99%

Pediatric sellar solitary fibrous tumor/ hemangiopericytoma: A rare case report and review of the literature

Ghanchi

Patchana

Christian

et al. 2020

Surgical Neurology International

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Background: Solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is a rare tumor which originates from the walls of capillaries and has historically been thought to be able to occur anywhere in the body that blood vessels are found. It is rarely found in the sellar region. Case Description: InS this report, we present the first case of this tumor occurring in the sellar region of a pediatric patient. This 12-year-old male presented with progressive vision loss which prompted surgical resection after a sellar lesion was discovered on imaging. The initial transsphenoidal approach resulted in subtotal resection and the patient experienced reoccurrence within 3 months. He underwent an orbitozygomatic craniotomy to achieve gross total tumor resection. Conclusion: We conducted a literature review of intracranial SFT/HPC in the pediatric population and found it to be an extremely rare occurrence, with <30 cases reported. The incidence of SFT/HPC occurring in the sellar region for any age group was also found to be a rare entity. Treatment recommendations for this tumor are also scarce, based on retrospective chart reviews from the adult population. The role for adjuvant radiation has mixed results.

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Section: Discussionmentioning

confidence: 99%

Pediatric sellar solitary fibrous tumor/ hemangiopericytoma: A rare case report and review of the literature

Ghanchi

Patchana

Christian

et al. 2020

Surgical Neurology International

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Meningeal haemangiopericytoma and solitary fibrous tumour: a retrospective bi centre study for outcome and prognostic factor assessment

et al. 2017

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To describe the outcome of patients diagnosed with central nervous system haemangiopericytoma (HPC) or solitary fibrous tumour (SFT) and identify factors that may influence recurrence and survival. Between January 2000 and September 2016, a retrospective search identified 55 HPCs/SFTs. The patients underwent a total of 101 surgical resections and 56.9% received radiation therapy. Median follow-up was 7.8 years. 28 patients (50.9%) were re-operated for tumour recurrence. At the end of the study, 21 patients (42%) had no residual tumour on the last scan. Surgical recurrence-free survival at 5 years was 75.2%, 95% CI [63.3-89.3] and, the median surgical recurrence-free survival was 7.4 years. In the adjusted analysis, venous sinus invasion (present vs. absent) (HR 3.39, 95% CI [1.16, 9.93], p = 0.026), completeness of resection (HR 0.38, 95% CI [0.15-0.97], p = 0.042) and tumour subtype (SFT vs. HPC) (HR 3.02, 95% CI[1.02, 8.91], p = 0.045) were established as independent prognostic factors. At the end of the study, 25 patients were deceased (45.5%). and only 15 patients (27.3%) had no residual tumour on the last scan and were alive. Overall survival at 5 years was 80.2, 95% CI [69.3-92.8] and the median overall survival was 13.1 years. None of the investigated variables was associated with overall survival. Patients who received radiation therapy demonstrated neither a reduced risk of surgical recurrence (p = 0.370) nor a longer overall survival (p = 1.000). SFTs/HPCs are associated with a significant risk of recurrence that may reduce the survival of the patients. Total tumour resection upon initial surgery is associated with a lower risk of relapse but not with a prolonged survival. We did not observe a significant improvement in any of the clinical outcomes after radiation therapy.

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

et al. 2018

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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.

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Tumeurs fibreuses solitaires et hémangiopéricytomes des méninges : immunophénotype et évaluation du grade histopronostique dans 17 cas

Cited by 9 publications

References 33 publications

Pediatric sellar solitary fibrous tumor/ hemangiopericytoma: A rare case report and review of the literature

Pediatric sellar solitary fibrous tumor/ hemangiopericytoma: A rare case report and review of the literature

Meningeal haemangiopericytoma and solitary fibrous tumour: a retrospective bi centre study for outcome and prognostic factor assessment

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

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