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Invasive breast carcinoma - IARC

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mitotic rate may be present in these<br />

t u m o u r s .<br />

Stromal-Leydig cell tumour<br />

Definition<br />

An ovarian stromal tumour composed of<br />

f i b romatous stroma and clusters of<br />

Leydig cells containing crystals of<br />

R e i n k e .<br />

Clinical features<br />

This tumour is virilizing in appro x i m a t e l y<br />

one-half of the cases.<br />

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

These extremely rare neoplasms are usually<br />

well circumscribed {302,2165,2842}.<br />

The sectioned surface has been<br />

described as lobulated with a yellowwhite<br />

appearance. They may be bilateral.<br />

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

S t romal-Leydig cell tumours have two<br />

components. Spindle-shaped or ovoid<br />

s t romal cells identical to those of a fibroma<br />

or thecoma are present together with<br />

Leydig cells containing Reinke cry s t a l s<br />

{2789,3252}. Ty p i c a l l y, in these neoplasms<br />

the fibrothecomatous element<br />

p redominates with the Leydig cell component<br />

comprising small nodular aggreg<br />

a t e s .<br />

Definitive diagnosis re q u i res the pre s-<br />

ence of Reinke crystals, otherwise the<br />

neoplasm would be categorized as<br />

luteinized thecoma. Since Reinke cry s-<br />

tals may be difficult to identify and since<br />

sampling errors may occur, it has been<br />

suggested that stromal-Leydig cell<br />

tumours are more common than the lite<br />

r a t u re would suggest.<br />

Prognosis and predictive factors<br />

The clinical behaviour of stro m a l - L e y d i g<br />

cell tumours is benign, and neither clinical<br />

re c u r rence nor metastasis has been<br />

d o c u m e n t e d .<br />

Sex cord-stromal tumours<br />

of mixed or unclassified<br />

cell types<br />

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

Sex cord - s t romal tumours that do not fall<br />

in the granulosa-stromal, Sert o l i - s t ro m a l<br />

or steroid cell categories.<br />

ICD-O codes<br />

Sex cord tumour with annular<br />

t u b u l e s 8 6 2 3 / 1<br />

Variant associated with<br />

Peutz-Jeghers syndrome 8 6 2 3 / 0<br />

G y n a n d ro b l a s t o m a 8 6 3 2 / 1<br />

Sex cord - s t romal tumour, NOS 8 5 9 0 / 1<br />

Sex cord tumour with<br />

annular tubules<br />

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

A tumour composed of sex cord (Sert o l i )<br />

cells arranged in simple and complex<br />

annular tubules {2599}.<br />

S y n o n y m<br />

S e rtoli cell tumour, annular tubular varia<br />

n t .<br />

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

Patients with this tumour most commonly<br />

present in the third or fourth decades,<br />

but the age ranges from 4-76 years.<br />

About one-third of cases occur in women<br />

with Peutz-Jeghers syndrome (PJS). The<br />

average age of patients with PJS is in the<br />

mid-twenties and of those unassociated<br />

with PJS in the mid-thirties.<br />

Clinical features<br />

Nearly all women without PJS pre s e n t<br />

with a palpable mass. Isosexual<br />

p s e u d o p recocity or other features of<br />

aberrant estrogen occurs in about 40%<br />

of cases, and, occasionally, there are<br />

p ro g e s t e rone effects. Those tumours<br />

that are associated with PJS are found<br />

either incidentally at autopsy or in<br />

ovaries removed as part of treatment for<br />

other gynaecological disease.<br />

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

These are unilateral neoplasms except<br />

for those occurring in the PJS, which are<br />

usually bilateral. PJS-associated lesions<br />

a re usually macroscopically undetectable;<br />

when visible, the tumourlets<br />

a re multiple and

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