Primary Synovial Sarcoma of the Prostate Gland: A Review of the Literature

Abstract

Background: Primary synovial sarcoma of the prostate gland (PSSP) is rare and most clinicians would be unfamiliar with its biological behaviour. Aim: To review the literature on PSSP. Methods: Various internet data bases were searched. Literature Review: PSSP is extremely rare with less than 10 cases reported; affects both young and older men; its diagnosis may be made incidentally following histological and cytogenetics examinations of prostate biopsy or prostatectomy specimens which show: A specific chromosomal translocation t(X; 18; p11; q11); Uniform spindle and oval cells which have formed interlacing fascicles that mimic fibrosarcoma. The compact fascicles of tumour cells focally alternate with hypo-cellular myxoid tissue which mimic peripheral nerve sheath tumours. Focal pericytomatous pattern of polygonal cells arranged around dilated, thin-walled blood vessels. PSSP tumour cells on immunohistochemical staining, stain positively with: Vimentin (most of the cells), EMA (focal positivity), Bcl-2 (strong positivity), CD99 (strong positivity), Ecadherin (strong positivity), cytokeratin (focal positivity), CD 56 and TLE/TLE1. There is no consensus opinion on treatment of PSSP which is an aggressive tumour with poor outcome. However, an aggressive radical surgical treatment by radical prostatectomy or pelvic exenteration plus or minus adjuvant therapy would appear to be the best treatment option with curative intent to help improve prognosis. Some patients with PSSP may need palliative and supportive treatment through a multi-disciplinary team approach.    Conclusions: PSSP is a rare aggressive tumour with poor prognosis. All cases of PSSP should be entered into a multi-centre trial to ascertain the best treatment option that would improve the prognosis and to further assess its biological behaviour.