Primary Synovial Sarcoma of the Prostate Gland: A Review of the Literature | Chapter 03 | New Insights into Disease and Pathogen Research Vol. 4
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), E-cadherin (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.
Author(s) Details
Mr. Anthony Kodzo-Grey
Venyo MB ChB FRCS(Ed) FRCSI FGCS Urol. LLM
Department of Urology, North
Manchester General Hospital, Delaunays Road Manchester, United Kingdom.
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