May occur in patients with neurofibromatosis I (von Recklinghausen disease) and probably arise from the adjacent pelvic autonomic plexus.
Mostly are young patients (1 month to 35 years old) and had neurofibromas in other organs including the skin.
Present as mass lesion or with obstructive symptoms.
Most prostate neurofibromas also involved the bladder.
Morphology of prostatic neural tumors (neurofibroma or schwannoma) are similar to those in somatic tissues (image A) and can be confirmed by S100+.
Hemangiomas may arise along the posterior urethra; very rare within the prostate.
May cause hematuria, hematospermia and/or post ejaculation hematuria.
Posterior urethral hemangiomas are seen in patients 29-80 years old (mean 57) and cystoscopically, appear as small sessile varicosities between the verumontanum and external sphincter.
Histologically, majority are cavernous hemangioma.
Prostatic angiosarcoma (AS) is also rare and consists of proliferating vascular channels lined by atypical spindle to epithelioid cells (image B).
Diagnosis of AS is confirmed by endothelial markers such as factor VIII-related antigen, CD34+ or CD31+.
Other rare sarcomas reported in the prostate include synovial sarcoma (with t(X;18)(p11.2;q11.2) or SYT-SSX fusion), primitive neuroectodermal tumor (PNET) (with t(11;22)(q23;q12) PNET or EWS/FLI1 transcript) and malignant fibrous histiocytoma (MFH).