Hemangiopericytoma

A tumor composed of spindle cells with a rich vascular network, which apparently arises from pericytes, cells of smooth muscle origin that lie around small vessels. Benign and malignant hemangiopericytomas exist, and the rarity of these lesions has led to considerable confusion in distinguishing between benign and malignant variants. (From Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1364)

Also Known As:

Networked: 399 relevant articles (20 outcomes, 29 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Meningioma (Meningiomas)
3.	Solitary Fibrous Tumors
4.	Sarcoma (Soft Tissue Sarcoma)
5.	Acoustic Neuroma (Acoustic Neurinoma)

Experts

1.	Park, Sung-Hye: 3 articles (01/2022 - 12/2016)
2.	Hornick, Jason L: 3 articles (03/2014 - 02/2006)
3.	McCutcheon, Ian E: 3 articles (10/2013 - 04/2004)
4.	Araujo, Dejka M: 3 articles (09/2012 - 07/2010)
5.	Park, Min S: 3 articles (09/2012 - 07/2010)
6.	Wang, Wei-Lien: 3 articles (09/2012 - 07/2009)
7.	Jeon, Seung Hyuck: 2 articles (01/2022 - 05/2018)
8.	Kim, Il Han: 2 articles (01/2022 - 05/2018)
9.	Park, Chul-Kee: 2 articles (01/2022 - 05/2018)
10.	Panigrahi, Souvagya: 2 articles (11/2020 - 01/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hemangiopericytoma:

1.	Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA 09/01/2013 - "Animals with hemangiopericytoma showed higher serum VEGF levels compared to the patients with malignant peripheral nerve sheath. " 01/01/2010 - "Greater intratumoral VEGF was expressed in hemangiopericytomas (P = 0.04). " 07/01/2010 - "This review highlights the current body of knowledge regarding the role of the vascular endothelial growth factor (VEGF) and its receptor (VEGFR) in angiosarcoma, epithelioid hemangioendothelioma (EHE), and hemangiopericytoma/solitary fibrous tumor (HPC/SFT). " 07/01/2010 - "Inhibiting the VEGF-VEGFR pathway in angiosarcoma, epithelioid hemangioendothelioma, and hemangiopericytoma/solitary fibrous tumor." 03/01/1996 - "VEGF mRNA was up-regulated in all of the hemangiopericytomas studied and highly expressed in the stromal cells of hemangioblastomas. "
2.	Bevacizumab (Avastin)FDA Link 01/01/2013 - "Antitumor effect of bevacizumab in a xenograft model of canine hemangiopericytoma." 09/15/2012 - "We report the development of a skin rash with two different patterns in a patient with a hemangiopericytoma of the meninges, a rare aggressive sarcoma, who was treated with repeated intravenous injections of bevacizumab." 09/01/2019 - "Cases from June 2006 through October 2016 were identified for patients receiving bevacizumab and temozolomide for locally advanced, recurrent or metastatic solitary fibrous tumour or hemangiopericytoma, which is sometimes used to describe tumours arising from the meninges. " 11/01/2020 - "Solitary fibrous tumor/hemangiopericytoma treated with temozolomide plus bevacizumab: a report of four cases and literature review." 11/01/2011 - "Activity of temozolomide and bevacizumab in the treatment of locally advanced, recurrent, and metastatic hemangiopericytoma and malignant solitary fibrous tumor."
3.	Monoclonal AntibodiesIBA 07/15/1991 - "Monoclonal antibody 29-13 exhibited significant binding to membranes isolated from fibrosarcoma and aggressive fibromatosis; moderate binding to osteosarcoma, hemangiopericytoma, and malignant fibrous histiocytoma; and minimal to no binding to other soft tissue sarcoma plasma membranes. " 07/01/1996 - "The immunohistochemical expression of muscle actin has been studied in 45 canine hemangiopericytomas (CHP) using a monoclonal antibody (HHF35) and formalin-fixed, paraffin-embedded specimens. " 01/01/2022 - "This phase II study investigates the efficacy of bevacizumab (BEV), a VEGF binding monoclonal antibody, in patients with progressive Grade I (G1M), Grade II (G2M), Grade III (G3M) meningioma, and other non-parenchymal tumors including vestibular schwannoma (n = 4) and hemangiopericytoma (n = 4) with the primary endpoint of progression-free survival rate at 6-months (PFS-6). "
4.	pazopanibFDA Link 01/01/2014 - "Pazopanib may be useful for treatment of metastatic hemangiopericytoma, though further studies are needed to confirm the efficacy of this medication and to investigate its molecular mechanism of action. " 09/01/2018 - "Pazopanib efficacy in recurrent central nervous system hemangiopericytomas." 01/01/2014 - "Successful use of pazopanib for treatment of refractory metastatic hemangiopericytoma." 09/15/2021 - "We herein report a case of symptomatic bradycardia of 35 beats per minute in a patient with solitary fibrous tumor/hemangiopericytoma (SFT/HPC) treated with pazopanib for 1 month. " 09/15/2021 - "Symptomatic Sinus Bradycardia in a Patient with Solitary Fibrous Tumor/Hemangiopericytoma Treated with Pazopanib."
5.	Keratins (Keratin)IBA 08/01/2018 - "The morphology (presence of branching thin-walled, hemangiopericytoma-like blood vessels) and the immunohistochemical profile (including STAT6 and GRIA2 positivity) were very useful to differentiate this very challenging case of a cytokeratin-positive dedifferentiated solitary fibrous tumor with heterologous rhabdomyosarcomatous differentiation from a broad list of differential diagnoses." 02/01/2021 - "Synovial sarcoma should be considered in the differential diagnosis of monomorphous spindle cell tumors, especially if the tumors have a hemangiopericytoma-like vasculature or express keratins. " 04/01/1989 - "We conclude that (a) meningiomas express both epithelial and mesenchymal markers as do arachnoid granulations, (b) that angioblastic meningiomas demonstrate only mesenchymal markers, (c) that angioblastic meningiomas express identical markers to peripheral hemangiopericytoma and should thus be considered a variant thereof, (d) among meningiomas, CEA and keratin appear to be relatively specific markers for the "secretory" variant, and (e) because of overlap in S-100 and EMA reactivity, these markers are unreliable in differentiating meningioma from acoustic schwannoma." 01/01/1997 - "Two atypical cases which presented scarce pseudocalcifications and tendency to concentric cellular structures did not differ from other hemangiopericytomas by their intrinsic immunohistochemical properties such as negative reaction to epithelial membrane antigen and cytokeratin. " 01/01/1987 - "Soft tissue hemangiopericytomas and normal pericytes were negative for both vimentin and keratin. "
6.	Antineoplastic Agents (Antineoplastics)IBA 01/01/1981 - "Animal pretreatment with PB or with PB + MC markedly enhanced the antineoplastic activity of ftorafur in Rauscher leukemia-, leukemia La-, or hemangiopericytoma-bearing mice but seemed unlikely to afford any therapeutic advantage over this drug because the lethal toxicity of ftorafur was increased." 09/01/2010 - "We report a case of a heavily pretreated metastatic meningeal hemangiopericytoma in which fourth-line chemotherapy with trabectedin, a marine-derived antineoplastic agent effective in treating advanced soft tissue sarcomas, resulted in clinical benefit."
7.	Human Growth Hormone (Saizen)FDA LinkGeneric 05/01/1985 - "Total removal of the suprasellar hemangiopericytoma resulted in normalization of the plasma human growth hormone (GH) level and a marked decrease in size of the pulmonary hemangiopericytoma. "
8.	GlucocorticoidsIBA 08/01/2009 - "Hemangiopericytoma-associated hypoglycemia improved by glucocorticoid therapy: a case report."
9.	TrabectedinIBA 09/01/2010 - "Response to trabectedin treatment in a highly pretreated patient with an advanced meningeal hemangiopericytoma." 09/01/2010 - "We report a case of a heavily pretreated metastatic meningeal hemangiopericytoma in which fourth-line chemotherapy with trabectedin, a marine-derived antineoplastic agent effective in treating advanced soft tissue sarcomas, resulted in clinical benefit."
10.	VimentinIBA 03/01/1989 - "Vimentin was the only intermediate filament expressed in the normal pericytes of human fetal and adult tissues and in the neoplastic pericytes of all of the hemangiopericytomas. " 03/01/1996 - "A second control group of 7 mesotheliomas was positive for cytokeratins and vimentin but not for CD 34 and Faktor VIII. Diagnosis of SFT might be difficult because of its variability in histopathology, sometimes mimicking a hemangiopericytoma or endothelioma. " 01/01/2001 - "In contrast, infantile myofibromatosis, malignant hemangiopericytomas, and fibrous histiocytomas expressed CTGF in both endothelial cells and in vimentin-positive tumor cells, particularly those around the blood vessels. " 03/01/2007 - "Immunohistochemically, the tumor cells were focally positive for CD-68 and negative for S-100, smooth muscle actin, vimentin, and CD-34, which ruled out neurofibroma, leiomyoma, solitary fibrous tumor, and hemangiopericytoma. " 05/01/1988 - "In all of the three hemangiopericytoma cell cultures, the matrix stained positively for vimentin and desmin. "

Therapies and Procedures

1.	Radiotherapy 06/01/1992 - "Preoperative radiation therapy is useful in the treatment of hemangiopericytoma involving considerable surgical risk. " 06/01/1984 - "Generally radiotherapy is not considered to be effective for hemangiopericytoma, but sometimes it is. " 01/01/2021 - "On the basis of the current results, stereotactic radiotherapy using CyberKnife is an effective and safe option for residual, recurrent, and metastatic intracranial hemangiopericytomas. " 09/08/2022 - "Sixteen-Year Follow-Up in a Cavernous Sinus Hemangiopericytoma: Improved Outcomes over Radiotherapy Advances." 01/01/2017 - "There is no agreement on chemo/radiotherapy as the primary treatment for hemangiopericytoma, although adjuvant radiation therapy has been found to improve local control and reduce recurrences. "
2.	Therapeutics 01/01/2017 - "A 10-year-old Labrador Retriever who had been undergoing therapy for a recurrent hemangiopericytoma of the right flank presented to the Kansas State University Ophthalmology service for evaluation of a painful left eye. " 07/01/2015 - "Combined therapy against recurrent and intracranial invasion of sinonasal hemangiopericytoma: A case report." 04/01/2014 - "Hemangiopericytoma: Radical resection remains the cornerstone of therapy." 06/01/2012 - "Combined Therapy against Recurrent Hemangiopericytoma: A Case Report." 06/06/2011 - "In a review of the Stanford radiosurgery database between 2002 and 2009, the authors found 14 patients who underwent CK therapy for recurrent, metastatic, and residual hemangiopericytomas. "
3.	Drug Therapy (Chemotherapy) 01/01/1987 - "[Possible efficacy of chemotherapy in a case of cardiac hemangiopericytoma]." 04/01/1978 - "Thirty-nine chemotherapy trials in 16 patients with metastatic hemangiopericytoma treated at MSKCC and 33 trials from the literature are reviewed. " 05/22/2012 - "Infantile hemangiopericytoma of the orbit treated with primary chemotherapy." 09/01/2010 - "The results of chemotherapy in advanced hemangiopericytoma have been disappointing, and they have been particularly poor in cases located in the meninges. " 06/01/2010 - "Preoperative chemotherapy to salvage the hand in a case of giant infantile hemangiopericytoma."
4.	Adjuvant Radiotherapy 05/01/2018 - "We retrospectively evaluated an efficacy of adjuvant radiotherapy (RT) in the intracranial hemangiopericytoma (HPC) and analyzed prognostic factors influencing treatment outcomes. " 05/01/2018 - "Efficacy of adjuvant radiotherapy in the intracranial hemangiopericytoma." 10/01/2015 - "Recent studies suggest aggressive management combining a grossly total resection (GTR) with adjuvant radiotherapy (RT) as a treatment of choice for intracranial hemangiopericytoma (HPC). " 10/01/2018 - "Emergent surgical resection of the cerebellar hemangiopericytomas was performed prior to adjuvant radiotherapy. " 01/01/2015 - "We report a rare case of recurrent IDEM hemangiopericytoma of dorsal spine in a 16-year-old boy treated with surgery and adjuvant radiotherapy. "
5.	Operative Surgical Procedures 07/01/2012 - "The postoperative pathologic records of 17,165 operations that were performed between January 1990 and December 2010 in the clinic were retrospectively searched, and the files of 6 cases with the diagnosis of primary classical hemangiopericytoma were analyzed for clinical characteristics of patients, surgical procedures, histopathologic features, treatments after surgery, and morbidity and mortality results. " 01/01/2012 - "In practice, this case highlights the mandatory need for a careful long-term follow-up for patients with hemangiopericytoma, since recurrence with a greater degree of malignancy can develop following an extended disease-free interval, as such knowledge will be helpful for planning the optimal surgical procedures."