Hemangiopericytoma

Symptoms of hemangiopericytoma vary greatly depending on both tumor stage and affected organs. Most patients report pain and mass-related symptoms, while others also report vascular disease-related symptoms, and some have no symptoms until late in the disease process. Hemangiopericytomas are most commonly found in the meninges, lower extremities, retroperitoneum, pelvis, lungs, and pleura.{{cite journal | vauthors = Wang K, Mei F, Wu S, Tan Z | title = Hemangiopericytoma: Incidence, Treatment, and Prognosis Analysis Based on SEER Database | journal = BioMed Research International | volume = 2020 | pages = 2468320 | date = 2020-11-03 | pmid = 33204688 | pmc = 7655240 | doi = 10.1155/2020/2468320 | doi-access = free }}

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Hemangiopericytomas are tumors that are derived from specialized spindle shaped cells called pericytes, which line capillaries.{{cite journal | vauthors = Gerner RE, Moore GE, Pickren JW | title = Hemangiopericytoma | journal = Annals of Surgery | volume = 179 | issue = 2 | pages = 128–132 | date = February 1974 | pmid = 4359454 | pmc = 1355764 | doi = 10.1097/00000658-197402000-00002 | s2cid = 220588367 }}

{{blockquote|text=Hemangiopericytoma is an aggressive mesenchymally derived tumor with oval nuclei with scant cytoplasm. There is dense intercellular reticulin staining. Tumor cells can be fibroblastic, myxoid, or pericytic. These tumors, in contrast to meningiomas, do not stain with epithelial membrane antigen. They have a grade 2 or 3 biological behavior, and need to be distinguished from benign meningiomas because of their high rate of recurrence (68.2%) and metastases.|author=W. Sherman and J. Raizer|title="Meningiomas"|source=MedMerits (2011), E.J. Dropcho (ed.){{cite journal | vauthors = Maier H, Ofner D, Hittmair A, Kitz K, Budka H | title = Classic, atypical, and anaplastic meningioma: three histopathological subtypes of clinical relevance | journal = Journal of Neurosurgery | volume = 77 | issue = 4 | pages = 616–623 | date = October 1992 | pmid = 1527622 | doi = 10.3171/jns.1992.77.4.0616 }}{{cite journal |vauthors=Kleihues P, Burger PC, Scheithauer BW |year=1993 |title=Histological typing of tumours of the central nervous system |journal=World Health Organization. Berlin: Springer-Verlag |edition=2nd |issue=30}}{{cite journal |vauthors=Sherman W, Raizer J |title=Meningiomas |url=http://www.medmerits.com/index.php/article/meningiomas/P1 |url-status=dead |journal=Medmerits |id=Section 2, "Historical note and nomenclature"|archive-url=https://web.archive.org/web/20120331070406/http://www.medmerits.com/index.php/article/meningiomas/P1 |archive-date=2012-03-31 |access-date=2011-09-19 |veditors=Dropcho EJ|year=2011}}}}

Computerized tomography and magnetic resonance imaging are not effective methods for diagnosis of hemangiocytomas. In practice, a presumptive diagnosis is often reached through exclusion of other soft tissue tumors, and a tissue biopsy is required to confirm diagnosis.

Depending on the grade of the sarcoma, it is treated with surgery,{{cite journal | vauthors = Ozaki N, Mukohara N, Yoshida M, Shida T | title = Successful resection of giant hemangiopericytoma originating from the left atrium | journal = Interactive Cardiovascular and Thoracic Surgery | volume = 5 | issue = 2 | pages = 79–80 | date = April 2006 | pmid = 17670519 | doi = 10.1510/icvts.2005.124107 | doi-access = free }} chemotherapy, and/or radiotherapy. Though surgery is the current standard of care for hemangiopericytomas, metastasis and tumor recurrence occur in more than 30% of patients, in particular recurrence in the pelvis and retroperitoneum and metastasis in bone and lungs.{{cite journal | vauthors = Shin DW, Kim JH, Chong S, Song SW, Kim YH, Cho YH, Hong SH, Nam SJ | display-authors = 6 | title = Intracranial solitary fibrous tumor/hemangiopericytoma: tumor reclassification and assessment of treatment outcome via the 2016 WHO classification | journal = Journal of Neuro-Oncology | volume = 154 | issue = 2 | pages = 171–178 | date = September 2021 | pmid = 34417710 | doi = 10.1007/s11060-021-03733-7 | s2cid = 237245514 }} Radiotherapy does not appear to provide a significant survival benefit but is recommended for use in patients with tumors greater than 5 cm in diameter or with inadequate resection margins after surgery. Clinical benefits of chemotherapy in soft tissue tumors remains unclear. However, the combination of surgery and chemotherapy appears to worsen survival in hemangiopericytoma patients.

More research is needed to determine efficacy of different types of treatment.

In one series, the median age of affected individuals was 45 years, with a 10-year survival rate of 70 percent.{{cite journal | vauthors = Enzinger FM, Smith BH | title = Hemangiopericytoma. An analysis of 106 cases | journal = Human Pathology | volume = 7 | issue = 1 | pages = 61–82 | date = January 1976 | pmid = 1244311 | doi = 10.1016/s0046-8177(76)80006-8 }} In another study, age over 45 and female sex were associated with worse survival rates in hemangiopericytomas.

• Infantile hemangiopericytoma
• List of cutaneous conditions

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• {{cite journal | vauthors = Schiariti M, Goetz P, El-Maghraby H, Tailor J, Kitchen N | title = Hemangiopericytoma: long-term outcome revisited. Clinical article | journal = Journal of Neurosurgery | volume = 114 | issue = 3 | pages = 747–755 | date = March 2011 | pmid = 20672899 | doi = 10.3171/2010.6.JNS091660 }}

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{{Medical resources

| DiseasesDB = 29249

| ICD10 = C49 (ILDS C49.M20)

| ICD9 =

| ICDO = {{ICDO|9150|1}}

| OMIM =

| MedlinePlus =

| eMedicineSubj = orthoped

| eMedicineTopic = 500

| MeshID = D006393

}}

{{Vascular tumors}}

Category:Dermal and subcutaneous growths

Category:Vascular neoplasia

Category:Nervous system neoplasia

Category:Brain tumor