Hemangiopericytoma, Malignant

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Retrieved

2019-09-22

Source

Omim

Trials

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Genes

STAT6, NAB2, CD34, UBE2D1, IGF2, LOC110806263, BCL2, TP53, NCOA2, H3P10, PAX8, CDKN2A, MIB1, TERT, S100B, S100A1, VIM, SMARCA1, AHSA1, AIMP2, GRAP2, RELA, CIC, SATB2, DSTYK, RNF19A, POLDIP2, PCSK4, BCOR, AHRR, ROS1, ACTB, PTEN, MAPK3, APAF1, CCND1, BCL6, CD38, ATF2, CRK, MAPK14, CSF1, CTNNB1, EGR1, ETV4, EWSR1, FGFR1, FOXO1, GRIA2, KIT, MET, CD99, MKI67, NF1, NTRK3, PDGFRA, AKT1, MAPK1

Drugs

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Malignant tumors of the vascular system are relatively uncommon and usually have an unfavorable, rapid clinical course. Metastases usually occur early by hematogenous routes to the lung and bone as well as to the regional lymph nodes. These vascular tumors may develop anywhere and can be difficult to diagnose, both clinically and histologically. Histogenetically they are of 2 main types: malignant hemangioendotheliomas originating from intimal endothelial cells, and malignant hemangiopericytomas arising from adventitial perivascular cells or pericytes. Plukker et al. (1988) reported malignant hemangiopericytoma in a brother and sister, aged 19 and 22 years, respectively, and in a 25-year-old fifth cousin of theirs. In 1 patient the tumor originated in the scalp, in the second in the right lower quadrant of the abdomen, and in the third in the right orbit.