Prostate sarcoma

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Urinary symptoms.

Patient Data

Age: 35 years
Gender: Male
ct

CT scan demonstrates a large heterogenous mass seemingly arising from the prostate with evidence of invasion into the bladder, seminal vesicles and periprostatic soft tissues. 

Numerous hepatic and pulmonary metastases are present. 

Case Discussion

Histology

Tissue shows in areas unremarkable surface epithelium comprising urothelium.   Most of the tissue shows a malignant spindle cell tumor comprising fascicles of moderately pleomorphic spindle cells with ovoid  nuclei.  There are frequent apoptotic bodies and mitosis are plentiful  (approximately 12 per 10 high power field).  There are areas of necrosis.  Some of the tumor shows focal myxoid areas. There is no definite skeletal or smooth muscle differentiation,  nor is there a heterologous component.   There is no malignant epithelial component. The tumor infiltrates into bundles  of smooth muscle presumably muscularis propria (detrusor).  There are also foci of lymphovascular space invasion.

The neoplastic cells show diffuse strong staining with vimentin. They are negative for a  panel of cytokeratin stains  (AE1/3,  PAN and EMA), PSA (prostate specific antigen), muscle markers desmin, smooth muscle actin (SMA) and caldesmon and melanoma markers gp-100 and Mel-A. There is focal, weak equivocal staining of some cells with S-100,  and the significance of this is uncertain.

The morphology is of a  malignant sarcomatoid neoplasm, and in view of the negative keratin staining,  it is most likely a  sarcoma.

The differential diagnosis (which hasn't been entirely excluded) includes a monomorphic synovial sarcoma for which cytogenetic studies to demonstrate the diagnostic translocation t(X;18) will be necessary.   A spindle cell rhabdomyosarcoma has been considered in the differential diagnosis but this is considered less likely as most of these tumors are desmin-positive and there are morphologically identifiable rhabdomyoblasts (which are not seen in this case).

FINAL DIAGNOSIS: high-grade sarcoma with lymphvascular invasion, morphology initially suggesting a synovial sarcoma. FISH studies are  NEGATIVE for SYT (18q11.2) breakapart signals however
(X;18) translocation associated with synovial sarcoma NOT detected. 

In view of the negative  FISH study, this tumor is best considered a high-grade sarcoma, NOS.

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