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Invasive breast carcinoma - IARC

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A<br />

Fig. 1.145 Leiomyoma. A The well circumscribed margin (left) is apparent. B The bland smooth muscle cells proliferate in whorls and fascicles.<br />

B<br />

extraosseous osteosarcoma at other<br />

sites of the body. Despite the pre d o m i-<br />

nantly circumscribed margins, charact<br />

e r i s t i c a l l y, at least focal infiltration is<br />

p resent. The tumour is composed of a<br />

spindle to oval cell population with variable<br />

amounts of osteoid or osseous tissue;<br />

cartilage is present in over a third<br />

of the cases {2681} but no other diff e r-<br />

entiated tissues.<br />

The appearance of the tumours varies<br />

depending on the cellular composition<br />

( f i b roblastic, osteoblastic, osteoclastic)<br />

as well as the type and amount of matrix<br />

(osteoid, osseous, chondroid).<br />

The osteoclastic giant cells are immun<br />

o reactive with the macrophage marker<br />

CD68 (clone KP1) while the spindle<br />

cells fail to immunoreact with either<br />

e s t rogen receptor (ER) or pro g e s t e ro n e<br />

receptor (PR) or epithelial markers.<br />

Prognosis and predictive factors<br />

M a m m a ry osteosarcomas are highly<br />

a g g ressive lesions with an overall fiveyear<br />

survival of 38% {2681}. Rec<br />

u r rences develop in over two-thirds of<br />

the patients treated by local excision<br />

and 11% of those treated by mastectom<br />

y. Metastases to the lungs and<br />

absence of axillary node involvement are<br />

typical of osteosarcomas. Many of the<br />

patients who develop metastases die of<br />

the disease within 2 years of initial diagnosis<br />

{2681}.<br />

F i b roblastic osteosarcomas are associated<br />

with a better survival compared to<br />

the osteoblastic or osteoclastic variants.<br />

Large tumour size at pre s e n t a t i o n ,<br />

p rominent infiltrating margins and necrosis<br />

are associated with more aggre s s i v e<br />

b e h a v i o u r.<br />

Leiomyoma and<br />

leiomyosarcoma<br />

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

Benign and malignant tumours composed<br />

of intersecting bundles of smooth<br />

muscle which is mature in benign lesions.<br />

Malignant lesions are larger in size and<br />

show more mitotic activity in the neoplastic<br />

cells.<br />

ICD-O codes<br />

L e i o m y o m a 8890 / 0<br />

L e i o m y o s a rc o m a 8890 / 3<br />

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

Benign and malignant smooth muscle<br />

tumours of the <strong>breast</strong> are uncommon and<br />

re p resent less than 1% of <strong>breast</strong> neoplasms.<br />

The majority of leiomyomas originate<br />

from the are o l a r-nipple complex and<br />

a minority occur within the <strong>breast</strong> pro p e r<br />

{1981}. Leiomyosarcomas arise mainly<br />

within the <strong>breast</strong> {821}.<br />

The age at presentation of leiomyomas<br />

and leiomyosarcomas overlaps, extending<br />

from the fourth to the seventh<br />

decades. However, cases of both have<br />

been re p o rted in adolescents {2000} and<br />

patients over eighty years old {821}.<br />

Clinical features<br />

Both leiomyomas and leiomyosarc o m a s<br />

usually present as a slowly growing palpable<br />

mobile mass that may be painful.<br />

Incidental asymptomatic leiomyomas disc<br />

o v e red in mastectomy specimens have<br />

been re p o rted {1981}.<br />

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

These lesions appear as well circ u m-<br />

scribed firm nodules with a whorled or<br />

lobulated cut surface. Their size ranges<br />

f rom 0.5 to 15 cm {770,2000}.<br />

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

The histopathology and immunophenotype<br />

are identical to that seen in smooth<br />

muscle tumours elsewhere in the body.<br />

These neoplasms may be well circ u m-<br />

scribed {1981} or show irregular infiltrative<br />

borders {2000}. Both are composed<br />

of spindle cells arranged in interlacing<br />

fascicles.<br />

In leiomyomas, these cells have elongated<br />

cigar-shaped nuclei and eosinophilic<br />

cytoplasm without evidence of atypia.<br />

Mitoses are sparse and typically fewer<br />

than 3 per 10 high power fields {458}. In<br />

l e i o m y o s a rcomas, nuclear atypia and<br />

mitotic activity are more prominent {821}.<br />

Tumour necrosis may also be observed.<br />

Infiltrating margins may not be evident in<br />

some leiomyosarc o m a s .<br />

D i ff e rential diagnosis<br />

A diagnosis of a smooth muscle tumour of<br />

the <strong>breast</strong> should be considered only after<br />

excluding other <strong>breast</strong> lesions that may<br />

show benign or malignant smooth muscle<br />

d i ff e rentiation i.e. fibroadenoma, muscular<br />

h a m a rtoma and sclerosing adenosis<br />

should be distinguished from leiomyoma;<br />

spindle cell myoepithelioma and sarc o-<br />

matoid <strong>carcinoma</strong> from leiomyosarc o m a .<br />

Prognosis and predictive factors<br />

Leiomyomas are best treated by complete<br />

excision whereas, wide excision<br />

with tumour- f ree margins is re c o m m e n d-<br />

ed for leiomyosarcomas. Late local re c u r-<br />

rence and metastatasis have been re p o r-<br />

ted in cases of mammary leiomyosarc o-<br />

ma {458,2014}.<br />

98 Tumours of the <strong>breast</strong>

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