Invasive breast carcinoma - IARC
Invasive breast carcinoma - IARC
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