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Invasive breast carcinoma - IARC

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Clinical features<br />

F i b romas may be found incidentally, but<br />

when large, patients may present with<br />

non-specific signs and symptoms of a<br />

pelvic mass. Between 10-15% of fibromas<br />

over 10 cm are associated with<br />

ascites {2519}, and Meigs syndro m e<br />

(ascites and pleural effusion with re s o l u-<br />

tion after fibroma removal) occurs in<br />

about 1% of cases {1839}.<br />

M a c r o s c o p y<br />

F i b romas are hard white tumours averaging<br />

6 cm in diameter. Oedematous<br />

tumours may be soft, and cyst form a t i o n<br />

is common. Haemorrhage and necro s i s<br />

a re rare outside the setting of torsion.<br />

The majority of tumours are unilateral.<br />

Only 8% are bilateral, and less than 10%<br />

show focal or diffuse calcification.<br />

H i s t o p a t h o l o g y<br />

F i b romas are composed of spindleshaped<br />

cells with uniform, bland nuclei<br />

and scant cytoplasm that may contain<br />

small amounts of lipid or occasionally<br />

eosinophilic droplets. The cells are<br />

arranged in fascicles or in a storiform<br />

p a t t e rn. Mitoses are absent or rare .<br />

F i b romas are generally sparsely to moderately<br />

cellular with abundant interc e l l u-<br />

lar collagen, hyalinized plaques and<br />

variable degrees of oedema. The cellularity<br />

may vary from area to area. About<br />

10% of tumours are uniformly and<br />

densely cellular (attaining the cellularity<br />

of a diffuse granulosa cell tumour) and<br />

a re re f e r red to as cellular fibro m a s<br />

{2289}. Cellular fibromas exhibit no<br />

m o re than mild cytological atypia and<br />

an average of three or less mitoses per<br />

10 high power fields. Fibromas expre s s<br />

vimentin and may be immunore a c t i v e<br />

for alpha-inhibin {1816, 2211}.<br />

Fig. 2.69 Fibrosarcoma. Moderate to severe cytological atypia accompanied by numerous mitotic figures<br />

characterize this fibrosarcoma.<br />

generations of a kindred lacking other<br />

stigmata of the syndrome {728,1635,<br />

2 2 2 1 , 2 6 3 5 } .<br />

Prognosis and predictive features<br />

R a re l y, cellular fibromas recur in the<br />

pelvis or upper abdomen, often after a<br />

long interval, particularly if they were<br />

a d h e rent or ruptured at the time of diagnosis<br />

{2289}. Ve ry rare l y, fibro m a t o u s<br />

tumours with no atypical features may<br />

s p read beyond the ovary {1722}.<br />

F i b r o s a r c o m a<br />

D e f i n i t i o n<br />

A rare fibroblastic tumour of the ovary<br />

that typically has 4 or more mitotic figu<br />

res per 10 high power fields as well as<br />

significant nuclear atypia.<br />

per 10 high power fields) with atypical<br />

division figures, haemorrhage and<br />

n e c rosis {90,145,2289}.<br />

Somatic genetics<br />

Trisomy 12 as well as trisomy 8 have<br />

been re p o rted in an ovarian fibro s a rc o-<br />

ma {2963}.<br />

Genetic susceptibility<br />

Ovarian fibro s a rcomas are rarely associated<br />

with Maffucci syndrome {484}<br />

and the nevoid basal cell carc i n o m a<br />

s y n d rome {1517}.<br />

Prognosis and predictive factors<br />

The majority of ovarian fibro s a rc o m a s<br />

have had a malignant course.<br />

Genetic susceptibility<br />

Ovarian fibromas are common in<br />

females with the nevoid basal cell carc i-<br />

noma syndrome, occurring in about<br />

75% of patients having the syndro m e<br />

re f e r red to gynaecologists. Syndro m e -<br />

related tumours are usually bilateral<br />

(75%), frequently multinodular, almost<br />

always calcified, sometimes massively,<br />

and tend to occur at a younger age,<br />

usually in children, adolescents, or<br />

young adults {1042,1354,2603}.<br />

Additional tumours may arise after local<br />

excision. The nevoid basal cell carc i n o-<br />

ma syndrome has been re p o rted in four<br />

E p i d e m i o l o g y<br />

F i b ro s a rcomas are the most common<br />

ovarian sarcoma, occurring at any age<br />

but most often in older women.<br />

M a c r o s c o p y<br />

F i b ro s a rcomas are large, solid tumours,<br />

commonly haemorrhagic and necro t i c ,<br />

and are usually unilateral.<br />

H i s t o p a t h o l o g y<br />

F i b ro s a rcomas are densely cellular,<br />

spindle cell neoplasms with moderate to<br />

s e v e re cytological atypia, a high mitotic<br />

rate (an average of 4 or more mitoses<br />

Fig. 2.70 Stromal tumour with minor sex cord elements.<br />

Rarely, fibrothecomas contain a few tubules<br />

lined by cells resembling Sertoli cells.<br />

Sex cord-stromal tumours 151

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