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Invasive breast carcinoma - IARC

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A<br />

Fig. 1.144 Mammary osteosarcoma. A The sectioned surface shows a well delineated, solid mass with focal haemorrhage. B The spindle cell component of the<br />

tumour invades the adipose tissue and is admixed with abundant bony frabeculae.<br />

B<br />

Prognosis and predictive factors<br />

Both the myxoid and pleomorphic variants<br />

of liposarcoma can recur and<br />

metastasize. Axillary node metastases<br />

a re exceptionally rare. Recurre n c e s<br />

generally develop within the first year<br />

and patients who die from their disease<br />

usually do so within a year of the diagnosis.<br />

Because of the high frequency of<br />

marginal irre g u l a r i t y, complete excision<br />

with tumour free margins is necessary.<br />

L i p o s a rcomas behave part i c u l a r l y<br />

a g g ressively when associated with<br />

p re g n a n c y.<br />

R h a b d o m y o s a r c o m a<br />

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

A tumour composed of cells showing<br />

v a rying degrees of skeletal muscle diff<br />

e re n t i a t i o n .<br />

ICD-O codes<br />

R h a b d o m y o s a rc o m a 8900 / 3<br />

Alveolar type 8920 / 3<br />

Pleomorphic type 8901 / 3<br />

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

P u re primary rhabdomyosarcoma of<br />

the <strong>breast</strong> is very uncommon, and,<br />

although primary mammary rhabdom<br />

y o s a rcoma has been described,<br />

it usually re p resents a metastasis from<br />

a soft tissue rhabdomyosarcoma<br />

occuring in children, young females<br />

or males {2402}. More fre q u e n t l y, rhabd<br />

o m y o s a rcomatous diff e rentiation may<br />

be observed in older women as an<br />

h e t e rologous component of a malignant<br />

phyllodes tumour or a metaplastic<br />

c a rc i n o m a .<br />

Pathology<br />

Primary rhabdomyosarcoma has been<br />

reported in adolescents {773,1166,1198,<br />

2402}, when it is predominantly of the<br />

alveolar subtype; the pleomorphic subtype<br />

has been reported in older women<br />

over forty {2871}.<br />

Metastatic rhabdomyosarcoma to the<br />

<strong>breast</strong> is again predominantly of the alveolar<br />

subtype {1166,1248}. The primary<br />

lesion is usually located on the extremities,<br />

in the nasopharynx/paranasal sinuses<br />

or on the trunk {1166}. A metastasis<br />

from an embryonal rhabdomyosarcoma<br />

to the <strong>breast</strong> is less frequent {1166,<br />

2531}.<br />

Metastatic <strong>breast</strong> tumours may occur as<br />

part of disseminated disease or as an<br />

isolated lesion.<br />

Mammary osteosarcoma<br />

Definition<br />

A malignant tumour composed of spindle<br />

cells that produce osteoid and/or bone<br />

together with cartilage in some cases.<br />

ICD-O code 9180/3<br />

Synonym<br />

Mammary osteogenic sarcoma.<br />

Epidemiology<br />

Accounting for about 12% of all mamm<br />

a ry sarcomas, pure osteosarc o m a s<br />

must be distinguished from those originating<br />

in phyllodes tumours or carc i-<br />

n o s a rcomas. Absence of connection to<br />

the skeleton, which should be confirm e d<br />

by imaging studies, is re q u i red for a<br />

diagnosis of a primary mammary<br />

o s t e o s a rc o m a s .<br />

O s t e o s a rcomas occur mainly in older<br />

women with a median age of 64.5 years;<br />

the age range is 27–89 years {2681}.<br />

The vast majority of patients are women<br />

who are predominantly Caucasian. A<br />

prior history of radiation therapy or trauma<br />

has been noted in some women<br />

{ 3 3 1 } .<br />

Clinical features<br />

The tumour presents as an enlarging<br />

mass which is associated with pain in<br />

one-fifth of cases. Bloody nipple discharge<br />

or nipple retraction occurs in<br />

12% of the women. Mammographically,<br />

o s t e o s a rcomas present as a well circumscribed<br />

mass with focal to extensive<br />

coarse calcification. Because of their<br />

p redominantly circumscribed nature ,<br />

they may be misinterpreted as a benign<br />

lesion {3072}.<br />

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

O s t e o s a rcomas vary in size from 1.4 to<br />

13 cm; most are about 5 cm in size and<br />

a re sharply delineated. The consistency<br />

varies from firm to stony hard depending<br />

on the pro p o rtion of osseous diff e re n t i a-<br />

tion. Cavitation and necrosis are seen in<br />

larger tumours.<br />

Histopathology<br />

The histopathological appearance and<br />

immunophenotype are similar to that of<br />

Mesenchymal tumours<br />

97

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