CNS Hemangiopericytoma

Ghose, Abhimanyu; Guha, Gunjan; Kundu, Ria; Tew, John M.; Chaudhary, Rekha

doi:10.1097/coc.0000000000000146

Cited by 71 publications

(24 citation statements)

References 27 publications

(41 reference statements)

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“…In our series, the similar gender distribution was seen, which was against the idea that osseous SFT/HPC occurred with a slight predominance among males [1,15,19,20]. In previous reports [6,7], intraspinal HPCs commonly occurred in the cervical and thoracic segments of the spine and rarely in the lumbar and sacral segments.…”

Section: Discussionmentioning

confidence: 39%

Surgical management of spinal solitary fibrous tumor/hemangiopericytoma: a case series of 20 patients

et al. 2017

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recurrence being 36.6 (median 28, range 12-73) months. Our results indicate that grade III is an adverse prognostic factor for both recurrence and over survival (OS) for spinal osseous SFT/HPC, while total resection, especially TES, is a positive prognostic factor. Conclusions Spinal osseous SFT/HPC is a challenging clinical entity given its high local recurrence rate. Surgical management plays a crucial role in the whole treatment of spinal SFT/HPCs and total excision, especially TES, should be strived for whenever possible. Postoperative radiotherapy is recommended to lower the recurrent rate. This study also confirms that pathology grade III is an adverse prognostic factor for spinal osseous SFT/HPCs.

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Section: Discussionmentioning

confidence: 39%

Surgical management of spinal solitary fibrous tumor/hemangiopericytoma: a case series of 20 patients

et al. 2017

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“…[8, 12, 27] As previous studies have found, surgical resection alone is sufficient to cure patients in many cases. Although tumour site was not related to DFI in our series, 57% of cases in which GTR was not achieved were located at cranial base, a site which could possibly hamper a complete resection as reported for other CNS tumours (i.e.…”

Section: Discussionmentioning

confidence: 99%

“…[8–12] Based on these findings, the current WHO grading criteria provide that SFT/HPC with solitary fibrous tumour phenotype and mitotic count <5 mitoses/10 high-power fields (HPF) are classified as grade I, whereas tumours with hemangiopericytoma morphology are graded as grade II or III based on the mitotic count with a cut-off value of 5 mitoses/10 HPF. [1] Radiotherapy may be of benefit to patients with incomplete resection or at higher risk of recurrence.…”

Section: Introductionmentioning

confidence: 99%

Pathological prognostic markers in central nervous system solitary fibrous tumour/hemangiopericytoma: Evidence from a small series

et al. 2018

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BackgroundPrimary central nervous system (CNS) solitary fibrous tumour/hemangiopericytoma (SFT/HPC) is a rare neoplasm and its classification criteria have been redefined by the latest WHO Classification of CNS Tumours. Outcome can vary significantly among patients, thus reliable prognostic markers are warranted.MethodsPrimary CNS SFT/HPC diagnosed at the Pathology Unit of our Institution between 2006 and 2016 were retrospectively collected. Tumour grade along with immunohistochemistry for Ki67, STAT6, PHH3, CD34 and Bcl-2 were assessed. TERT promoter status was evaluated by Sanger sequencing.ResultsFifteen SFT/HPC were analysed: 9/15 (60%) female, median age at diagnosis 60 (range: 10–67). Six (40%) cases showed a SFT phenotype and mean H&E-mitotic count was 4.8/10 HPF. Tumour grade was I in 6, II in 4 and III in 5 cases. Mean PHH3-mitotic count was higher than H&E count (8.4 versus 4.8/10 HPF), but it would have determined a change in tumour grade in a sole case. Nuclear staining for STAT6 was present in 14/15 (93.3%). CD34 and Bcl-2 expression rates were lower in higher grade tumours. TERT promoter was mutated in two cases. Median follow up time was 2.4 years (6 months-7.4 years) and 5/15 (33%) patients developed local disease recurrence. Partial resection (p = 0.0185), higher WHO grade (p = 0.038), lower CD34 (p = 0.038) and Bcl-2 (p = 0.010) expressions were significantly associated with a poorer disease-free interval.ConclusionsWHO grade is the main prognostic tool in CNS SFT/HPC, but it could be integrated by other markers, like CD34 and Bcl-2, in the clinical practice. The relevance of TERT promoter mutations in this subset of CNS tumours needs further evaluation.

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“…The imaging manifestation of MHP is described as a large solitary solid or cystic-solid irregular mass with uneven density, unclear boundaries, hemorrhage and necrosis. Studies have shown that MHPs can be enhanced in a variety of ways from the early to late phase [ 19 ]. In our study, 2 patients showed obvious heterogeneous enhancement in the arterial phase, which gradually decreased in the portal venous and delayed phases, with no obvious expansion in the enhancement range.…”

Section: Discussionmentioning

confidence: 99%

Primary hepatic malignant vascular tumors: a follow-up study of imaging characteristics and clinicopathological features

et al. 2020

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Background: Owing to its low incidence, there is insufficient clinical awareness and diagnostic experience with primary hepatic malignant vascular tumors (PHMVTs). The aim of our study was to investigate the imaging and clinicopathological features of patients with PHMVTs and analyze the clinicopathological correlations. Methods: We retrospectively analyzed 42 patients who had pathologically confirmed PHMVT during the period from June 2012 to December 2019 and enrolled them in our study. The computed tomography (CT) and magnetic resonance (MR) images and pathological findings of each patient were recorded. Results: There were more female (29/42) than male patients. The imaging features of primary hepatic angiosarcoma (PHA) (n = 11) included ill-defined margins (11/11, 100%), necrosis (5/11, 45%), calcification (3/11, 27%) and "slow inslow out" centripetal enhancement (7/11, 64%). Patients with epithelioid hemangioendothelioma (EHE) (n = 15) presented with ill-defined margins (15/15, 100%), necrosis (6/15, 40%), calcification (2/15, 13%), "fast in-slow out" centripetal enhancement (10/15, 67%), halo sign (15/15, 100%), pseudocapsule sign (4/15, 27%), lollipop sign (2/15, 13%) and capsule retraction sign (2/15, 13%). Patients with malignant hemangiopericytoma (MHP) (n = 3) showed illdefined margins (3/3, 100%), necrosis (3/3, 100%) and "fast in-slow out" progressive enhancement (3/3, 100%). Infantile hemangioendotheliomas (IHEs) (n = 13) were defined by ill-defined margins (7/13, 54%), necrosis (8/13, 62%), calcification (5/13, 38%) and "fast in-slow out" centripetal enhancement (13/13, 100%). Immunohistochemistry showed strong positive expression of CD31, CD34, ERG, FaVIII and FLI-1. Patients with IHE (96 months) and EHE (88 months) had the longest survival times, followed by those with MHP (23 months), while patients with PHA (15 months) had the shortest survival time. Conclusion: On CT and MR images, most PHMVTs were ill-defined, heterogeneous, hypervascular masses with centripetal progressive enhancement and possibly calcification, especially in female patients. The prognosis of patients with PHMVT was associated with the pathological type of the tumor.

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CNS Hemangiopericytoma

Cited by 71 publications

References 27 publications

Surgical management of spinal solitary fibrous tumor/hemangiopericytoma: a case series of 20 patients

Surgical management of spinal solitary fibrous tumor/hemangiopericytoma: a case series of 20 patients

Pathological prognostic markers in central nervous system solitary fibrous tumour/hemangiopericytoma: Evidence from a small series

Primary hepatic malignant vascular tumors: a follow-up study of imaging characteristics and clinicopathological features

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