Studies have suggested that these tumors are due to the expression of male sex hormones (i.e. androgens and progesterones), genetic factors, molecular alterations (i.e. changes in the normal characteristics of cells that lead to abnormal cell growth), and/or human papillomavirus infection.Angiofibroma of soft tissue is also named angiofibroma, not otherwise specified, by the World Health Organization, 2020. The Organization also classified these lesions as in the category of benign fibroblastic and myofibroblastic tumors. These tumors more often afflict females, typically occur in adults (median age 49 years), have a median size of ~3.5 cm, and develop in a leg near to, and may invade, a large joint. Less uncommonly, they occur in the back, abdominal wall, pelvic cavity, or breast. Angiofibroma of soft tissue tumors consist of uniform, bland, spindle-shaped cells and a prominent vascular network consisting of small thin-walled branching blood vessels in a variably collagenous tissue background. Its tumor cells contain an AHRR-NCOA2 fusion gene in 60% to 80% of cases and a GTF2I-NCOA2 or GAB1-ABL1 fusion gene in rare cases.Cellular angiofibroma is usually a small, slow-growing tumor arising in the vulva-vaginal areas of adult woman and the inguinal-scrotal areas of adult men although some of these tumors, especially in men, can grow up to 25 cm. Affected men are usually older (7th decade) than women (5th decade). Less commonly. cellular angiofibromas have occurred in various other superficial soft tissue areas throughout the body. These tumors are edematous (i.e.
Pacritinib, sold under the brand name Vonjo, is an anti-cancer medication used to treat myelofibrosis. It is a macrocyclic protein kinase inhibitor. It mainly inhibits Janus kinase 2 (JAK2) and Fms-like tyrosine kinase 3\CD135 (FLT3). Common side effects include diarrhea, low platelet counts, nausea, anemia, and swelling in legs. Medical uses
Pacritinib in indicated to treat adults who have a rare form of a bone marrow disorder known as intermediate or high-risk primary or secondary myelofibrosis and who have platelet (blood clotting cells) levels below 50,000/µL. History
The effectiveness and safety of pacritinib were demonstrated in a study that included 63 participants with intermediate or high-risk primary or secondary myelofibrosis and low platelets who received pacritinib 200 mg twice daily or standard treatment. Effectiveness was determined based upon the proportion of participants who had a 35% or greater spleen volume reduction from baseline to week 24. Nine participants (29%) in the pacritinib treatment group had a 35% or greater spleen volume reduction, compared to one participant (3%) in the standard treatment group.The U.S. Food and Drug Administration (FDA) granted the application for pacritinib priority review, fast track, and orphan drug designations. Society and culture
Names
Pacritinib is the International nonproprietary name (INN). References
External links
"Pacritinib". Drug Information Portal. U.S. National Library of Medicine.