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Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

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A<br />

B<br />

Fig. 3.87 A Malignant phyllodes tumour. High cellularity and cellular pleomorphism are obvious even at this magnification. B Leiomyosarcoma. Fascicular arrangement,<br />

high cellularity and mitotic activity are characteristic.<br />

ma. Both STUMP and stromal sarcomas<br />

characteristically express progesterone<br />

receptors (PR) and uncommonly express<br />

estrogen receptors (ER), supporting the<br />

concept that STUMP and stromal sarcomas<br />

are lesions involving hormonally<br />

responsive prostatic mesenchymal cells,<br />

the specialized prostatic stroma.<br />

STUMPS typically react positively with<br />

actin, whereas prostatic stromal sarcomas<br />

react negatively, suggesting that the<br />

expression of muscle markers in these<br />

lesions is a function of differentiation.<br />

Leiomyosarcoma<br />

Leiomyosarcomas are the most common<br />

sarcomas involving the prostate in adults<br />

{443}. The majority of patients are<br />

between 40 and 70 years of age, though<br />

in some series up to 20% of leiomyosarcomas<br />

have occurred in young adults.<br />

Leiomyosarcomas range in size between<br />

2 cm and 24 cm with a median size of 5<br />

cm. Histologically, leiomyosarcomas<br />

range from smooth muscle tumours<br />

showing moderate atypia to highly pleomorphic<br />

sarcomas. As with leiomyosarcomas<br />

found elsewhere, these tumours<br />

immunohistochemically can express<br />

cytokeratins in addition to muscle markers.<br />

There have been several well circumscribed<br />

lesions with a variable<br />

amount of nuclear atypia and scattered<br />

mitotic activity which have been referred<br />

to as atypical leiomyoma of the prostate<br />

{2233}, giant leiomyoma of the prostate<br />

{2162}, or circumscribed leiomyosarcoma<br />

of the prostate {2505}. Following<br />

either local excision or resection of prostatic<br />

leiomyosarcomas, the clinical<br />

course tends to be characterized by multiple<br />

recurrences. Metastases, when<br />

present, are usually found in the lung.<br />

The average survival with leiomyosarcoma<br />

of the prostate is between 3 and 4<br />

years. Because smooth muscle tumours<br />

of the prostate are rare, the criteria for<br />

distinguishing between leiomyosarcoma<br />

and leiomyoma with borderline features<br />

have not been elucidated. Although most<br />

"atypical leiomyomas" have shown no<br />

evidence of disease with short follow-up,<br />

a few have recurred.<br />

Rhabdomyosarcoma<br />

Rhabdomyosarcoma is the most frequent<br />

mesenchymal tumour within the prostate<br />

in childhood {1522}. Rhabdomyosarcomas<br />

of the prostate occur from<br />

infancy to early adulthood with an average<br />

age at diagnosis of 5 years. Most<br />

present with stage III disease, in which<br />

there is gross residual disease following<br />

incomplete resection or biopsy. A smaller,<br />

but significant proportion of patients<br />

present with distant metastases.<br />

Localized tumour that may be complete-<br />

A<br />

B<br />

Fig. 3.88 A Rhabdomyosarcoma. Note strap cells. B Angiosarcoma with slit-like spaces lined by atypical cells.<br />

210 Tumours of the prostate

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