@entity2698 is an uncommon upper aerodigestive tract @entity5 of uncertain cellular differentiation. We report 104 cases of sinonasal-type @entity2698 diagnosed between 1970 and 1995 from the files of the Armed Forces Institute of Pathology. There were 57 females and 47 males ranging in age from 5 to 86 years (mean 62.6 years). The most common clinical presentation was @entity1224 (n = 57) and/or @entity1415 (n = 54), with symptoms averaging 10 months in duration. The @entity5 involved the nasal cavity alone (n = 47) or also a paranasal sinus (n = 26), were polypoid, and measured an average of 3.1 cm. Histologically, the @entity5 were submucosal and unencapsulated and showed a diffuse growth with @entity714 (n = 37) to solid (n = 50) to focally whorled (n = 7) patterns. The @entity5 cells were uniform in appearance with minimal pleomorphism and had spindle-shaped (n = 82) to round/oval (n = 18) nuclei with vesicular to hyperchromatic chromatin and eosinophilic to amphophilic to clear-appearing cytoplasm with indistinct cell borders. Multinucleated ( @entity5 ) giant cells were identified in a minority of cases (n = 5). Mitotic figures were @entity39 was absent. The @entity5 were richly vascularized, including staghorn-appearing vessels that characteristically had prominent perivascular hyalinization (n = 92). An associated inflammatory cell infiltrate that included mast cells and eosinophils was noted in the majority of cases (n = 87). The immunohistochemical profile included reactivity with @entity2277 (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), and laminin (52%). Surgery was the treatment of choice for all of the @entity1 ; adjunctive radiotherapy was given to four @entity1 . Recurrences developed in 18 @entity1 within 1-12 years from diagnosis. Ninety-seven @entity1 were either alive (n = 51, mean 16.5 years) or dead (n = 46, mean 9.6 years) but free of disease. Four @entity1 had disease at the last follow-up: three died with disease (mean 3.6 years) and one @entity1 is alive with disease (28.3 years). Recurrent @entity5 (17.8%) can be managed by additional surgery. The majority of sinonasal-type @entity2698 behave in a benign manner with excellent long-term prognosis (88% raw 5-year survival) following surgery alone. @entity2698 have a characteristic light microscopic appearance with an immunophenotypic profile resembling that of @entity5 .

XXXX : a clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity714 :: ('MESH:D002037', 'Disease') :: ['fascicular'] @entity2698 :: ('MESH:D006393', 'Disease') :: ['hemangiopericytomas', 'Sinonasal-type hemangiopericytoma', 'hemangiopericytoma', 'Sinonasal-type hemangiopericytomas'] @entity1415 :: ('MESH:D004844', 'Disease') :: ['epistaxis'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'glomus tumors', 'tumors'] @entity39 :: ('MESH:D009336', 'Disease') :: ['inconspicuous and necrosis'] @entity2277 :: ('7431', 'Gene') :: ['vimentin'] @entity1224 :: ('MESH:D000402', 'Disease') :: ['airway obstruction']
@entity2698 :: (MESH:D006393,Disease) :: ['Sinonasal-type hemangiopericytoma']
