Originally described in pleura and other serosal surfaces, later also described in somatic tissues and visceral organs including prostate.
Considered as same spectrum with hemangiopericytoma(cellular SFT).
Exceedingly rare, only a handful was reported.
Patients are 21 to 75 years old (mean 58 years) and most present with obstructive symptoms and abnormal DRE.
Gross: tumor is well circumscribed, solid, firm or rubbery, with white-tan nodular cut surface.
Characteristically shows spindle cells with scant cytoplasm admixed with thick "ropey" collagen in a haphazard (patternless) pattern.
Degree of cellularity varies with hypo- and hypercellular areas (image A).
Vessels are abundant with characteristic larger staghorn vessels (image B).
Immunohistochemistry: CD34+, CD99+ and Bcl-2+.
Larger tumors (>10 cm) with increase mitosis, pleomorphism, infiltrative borders and necrosis are considered malignant.
(So far) has favorable outcome even those considered to be histologically malignant.
DDX: other spindle cell lesions of the prostate such as stroma-predominant BPH (nodules multiple with spindle cells following small vessels), stromal tumors, smooth muscle tumor (actin+), sarcomatoid carcinoma (keratin+) and GIST (c-kit+).