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Invasive breast carcinoma - IARC

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aged women (average age of presentation<br />

is 40-50 years) around 15-20 years<br />

older than for FAs.<br />

In Asian countries, PTs occur at a younger<br />

age (average 25-30 years) {487}.<br />

Malignant PTs develop on average 2-5<br />

years later than benign PTs. Among<br />

Latino whites, especially those born in<br />

Central and South America, malignant<br />

phyllodes is more frequent {254}.<br />

Isolated examples of PTs in men have<br />

been recorded {1424a,2023}.<br />

Aetiology<br />

P Ts are thought to be derived fro m<br />

intralobular or periductal stroma. They<br />

may develop de novo or from FAs. It is<br />

possible, in rare cases, to demonstrate<br />

the presence of a pre-existing FA adjacent<br />

to a PT.<br />

Clinical features<br />

Usually, patients present with a unilateral,<br />

firm, painless <strong>breast</strong> mass, not attached<br />

to the skin. Very large tumours (>10 cm)<br />

may stretch the skin with striking distension<br />

of superficial veins, but ulceration is<br />

very rare. Due to mammographic screening,<br />

2-3 cm tumours are becoming more<br />

common, but the average size remains<br />

around 4-5 cm {775,2425}. Bloody nipple<br />

discharge caused by spontaneous<br />

i n f a rction of the tumour has been<br />

described in adolescent girls {1781,<br />

2833}. Multifocal or bilateral lesions are<br />

rare {1932}.<br />

Imaging reveals a rounded, usually<br />

sharply defined, mass containing clefts<br />

or cysts and sometimes coarse calcifications.<br />

Macroscopy<br />

P Ts form a well circumscribed firm ,<br />

bulging mass. Because of their often<br />

clearly defined margins, they are often<br />

shelled out surgically.<br />

The cut surface is tan or pink to grey and<br />

may be mucoid. The characteristic<br />

whorled pattern with curved clefts<br />

resembling leaf buds is best seen in<br />

large lesions, but smaller lesions may<br />

have an homogeneous appearance.<br />

Haemorrhage or necrosis may be present<br />

in large lesions.<br />

Histopathology<br />

PTs typically exhibit an enhanced intracanalicular<br />

growth pattern with leaf-like<br />

p rojections into dilated lumens. The<br />

epithelial component consists of luminal<br />

A<br />

B<br />

Fig. 1.152 Phyllodes tumour, borderline. A A predominantly pushing margin in a borderline tumour.<br />

B Periductal stromal condensation. C Dense spindle-cell stroma with a few mitotic figures.<br />

C<br />

epithelial and myoepithelial cells.<br />

Apocrine or squamous metaplasia is<br />

occasionally present and hyperplasia is<br />

not unusual. In benign phyllodes<br />

tumours, the stroma is more cellular than<br />

in FAs, the spindle cell nuclei are<br />

monomorphic and mitoses are rare. The<br />

stromal cellularity may be higher in zones<br />

in close contact with the epithelial component.<br />

Areas of sparse stromal cellularity,<br />

hyalinisation or myxoid changes are<br />

not uncommon. Necrotic areas may be<br />

seen in very large tumours. The presence<br />

of occasional bizarre giant cells<br />

should not be taken as a mark of malignancy.<br />

Lipomatous, cartilagenous and<br />

osseous metaplasia have been reported<br />

{2057,2730}. The margins are usually<br />

well delimited, although very small<br />

tumour buds may protrude into the surrounding<br />

tissue. Such expansions may<br />

be left behind after surgical removal and<br />

are a source of local recurrence.<br />

Malignant PTs have infiltrative rather than<br />

pushing margins. The stroma shows<br />

frankly sarcomatous, usually fibrosarcomatous<br />

changes. Heterologous differentiation<br />

such as liposarcoma, osteosarcoma,<br />

chondrosarcoma or rhabdomyosarcoma<br />

may occur {536,1161,2057,2249,<br />

2308}. Such changes should be indicated<br />

in the diagnostic report. Due to overgrowth<br />

of the sarcomatous components,<br />

the epithelial component may only be<br />

identified after examining multiple sections.<br />

Borderline PTs (or low grade malignant<br />

PTs) display intermediate features and<br />

the stroma often resembles low-grade<br />

fibrosarcoma.<br />

Malignant epithelial transformation (DCIS<br />

or LIN and their invasive counterparts) is<br />

uncommon {2136}.<br />

Differential diagnosis<br />

Benign PTs may be difficult to distinguish<br />

from fibroadenomas. The main<br />

f e a t u res are the more cellular stro m a<br />

and the formation of leaf-like pro c e s s e s .<br />

H o w e v e r, the degree of hyperc e l l u l a r i t y<br />

that is re q u i red to qualify a PT at its<br />

lower limit is difficult to define. Leaf-like<br />

Fibroepithelial tumours 101

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