Primary angiosarcoma of the prostate (PASOP) is a very rare tumour which most clinicians have not encountered and may be unaware of. Literature of PASOP was reviewed by obtaining information from various internet data bases including: Google, Google Scholar, Educus and PUB Med. Less than 20 cases of PASOP have been reported. PASOP may present in a male child or adult with lower urinary tract symptoms, dysuria, haematuria, pain and constipation. There may be in some cases a history of prior radiotherapy for adenocarcinoma of prostate. Diagnosis is based upon histological examination of prostate biopsy specimens which tend to reveal: Proliferative vascular channels that are lined by atypical multi-layered or atypical solid endothelial cells, variable pleomorphic tumour cells ranging from spindle cells to large/plump cells; nuclei which are large and pleomorphic and which contain clumped chromatin and prominent nucleoli; mitotic figures of which some may look atypical are frequently seen. PASOPs on immunohistochemical staining tend to stain positively for CD34, Factor 8 (Factor VIII), Vimentin. PASOPs on immunohistochemical staining tend to exhibit negative staining for PSA, Keratin and S-100. Surgical resection with surgical margins that are clear of tumor has been shown to be the treatment associated with a chance of long-term survival but a number of reported cases of PASOP at the time of initial diagnosis had presented with metastatic disease or locally advanced disease and curative surgery with clear surgical margins has been impossible. Various adjuvant therapies had been reported but on the whole the prognosis has been poor. There is on the whole no consensus opinion on the best management options for all stages of the disease. PASOP is a rare aggressive disease. Clinicians should report cases of PASOP they encounter and should enter them into a multi-centre trial to find the best treatment option. Perhaps if patients who develop relapse disease who had previously undergone radiotherapy for prostate cancer undergo further biopsies of prostate may be new cases of PASOP would be diagnosed in the new biopsy specimens and this could illustrate that PASOPS are not as uncommon as used to be believed.
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