Invasive breast carcinoma - IARC

A
Fig. 2.77 A Retiform Sertoli-Leydig cell tumour. Note the retiform spaces surrounded by oedematous stroma
at the periphery of a cellular nodule. B Keratin stains the retiform areas and shows limited staining of
adjacent sex cord areas and stroma.
B
Fig. 2.78 Sertoli-Leydig cell tumour with retiform
elements. The sex cord areas stain strongly for
inhibin with weaker staining in retiform areas.
Genetic susceptibility
A familial occurrence of SLCTs in association
with thyroid disease has been
re p o rted {1344} with occasional re p o rt s
of other families since then. The thyro i d
a b n o rmalities are usually adenomas or
nodular goitres. Autosomal dominant
inheritance with variable penetrance
has been suggested as the method of
genetic transmission. SLCT has been
re p o rted in association with cervical sarcoma
botryoides in three cases {1026}.
Prognosis and predictive factors
The mortality from SLCTs as a group is
low and is confined to those of interm e-
diate and poor diff e rentiation. Poor diff
e rentiation, tumour rupture and hetero l-
ogous mesenchymal elements were
identified as features correlating with
the development of metastases {302,
2459}. In one large series none of the
well diff e rentiated tumours, 11% of
those of intermediate diff e rentiation and
59% of those that were poorly diff e re n t i-
ated behaved in a clinically malignant
fashion {3217}. Presentation with stage
II or higher disease is also associated
with a poor outcome. However, tumours
without any apparent poor pro g n o s t i c
factors may behave in an aggre s s i v e
fashion {1903}.
Sertoli-Leydig tumour with
heterologous elements
D e f i n i t i o n
A SLCT that contains either macro s c o p-
ic or histological quantities of a tissue
not re g a rded as intrinsic to the sex cord -
s t romal category. Such elements
include epithelial (mostly mucinous)
and/or mesenchymal tissues (most
commonly chondroid and rhabdomyoblastic)
and tumours arising from these
e l e m e n t s .
Clinical features
The presence of heterologous elements
does not alter the presentation, but 20%
of patients have a slightly raised serum
a l p h a - f e t o p rotein (AFP) due in some
cases to hepatocytes as a hetero l o g o u s
e l e m e n t .
M a c r o s c o p y
P a rt or the entire cystic component of a
SLCT may be mucinous in type; howeve
r, heterologous elements are only
occasionally diagnosed macro s c o p i-
c a l l y.
H i s t o p a t h o l o g y
H e t e rologous elements are seen in
a p p roximately 20% of SLCTs. They
occur only in those of intermediate or
poor diff e rentiation or in re t i f o rm
tumours but are not identified in well-diff
e rentiated tumours. Heterologous mesenchymal
elements occur in 5% of
S L C Ts and usually consist of cart i l a g e ,
skeletal muscle or rhabdomyosarc o m a .
They may be admixed with the sex cord
a reas of the tumour or present as disc
rete areas. Both cartilage and skeletal
A
Fig. 2.79 A Sertoli cell tumour, simple tubular pattern. Note the hollow and obliterated tubules in cross section. B Sertoli cell tumour, complex tubular pattern. Islands
of Sertoli cells are arranged around multiple round hyaline bodies.
B
Sex cord-stromal tumours 155