Invasive breast carcinoma - IARC

Tumours unassociated with PJS form
masses of simple and complex tubules
separated by sparse fibrous stro m a .
Extensive hyalinization may develop.
The neoplastic cells may spill over
beyond the confines of the tubules and
infiltrate the surrounding stroma. Mitotic
f i g u res occasionally exceed 4 per 10
high power fields and rarely exceed 10
per 10 high power fields. Areas of well
d i ff e rentiated Sertoli cell tumour characterized
by elongated solid tubules
and/or microfollicular granulosa cell
tumour are often present. Calcification
of the hyaline bodies is typically found in
over half of the tumours associated with
P J S .
E l e c t ron micro s c o p y
Ultrastructural assessment has shown
C h a rcot-Böttcher filaments in several
cases {2882}. While not re q u i red for
diagnosis, their presence confirms the
identification of the sex cord component
as Sertoli cells.
H i s t o g e n e s i s
Although there is ultrastructural evidence
supporting diff e re n t i a t i o n
t o w a rds Sertoli cells in SCTAT, the histological
and clinical features are suff i-
ciently distinctive to merit its classification
as a specific form of sex cord - s t romal
tumour.
Prognosis and predictive factors
All PJS-associated tumourlets have
been benign. Up to 25% of SCTATs that
occur in the absence of the PJS have
been clinically malignant. Tumours with
an infiltrative growth pattern and mitotic
f i g u res beyond the usual 3-4 per 10 high
power fields are more likely to recur or
otherwise behave aggre s s i v e l y. It is difficult,
however, to predict the behaviour
of individual cases. Some tumours produce
müllerian inhibiting substance
and/or alpha-inhibin, and these tumour
markers may be useful in monitoring the
course of disease in those cases
{1091,2304}. Recurrences are often late
and may be multiple. Spread thro u g h
lymphatics may result in regional and
distant lymph node involvement.
Somatic genetics
G e rmline mutations in a gene encoding
s e r i n e - t h reonine kinase have been identified
in a SCTAT associated with PJS
but not in sporadic cases {548}.
Fig. 2.83 Steroid cell tumour. The tumour is composed of large polygonal cells with eosinophilic cytoplasm
containing lipofuscin pigment.
A
Fig. 2.84 A Steroid cell tumour composed of large polygonal cells with vacuolated cytoplasm. B Tumour
cells show intense cytoplasmic immunoreactivity for alpha-inhibin.
G y n a n d r o b l a s t o m a
D e f i n i t i o n
A tumour composed of an admixture of
well diff e rentiated Sertoli cell and granulosa
cell components with the second
cell population comprising at least 10%
of the lesion.
Clinical features
An extremely rare tumour, gynandro b-
lastoma generally occurs in young
adults, though it may be encountered in
a wide age range {96,432,1820,1996}.
Nearly all tumours present in stage I and
may have either estrogenic or androgenic
manifestations. Variable in size,
they may be massive (up to 28 cm) with
a predominantly solid sectioned surf a c e
showing a few cysts.
B
H i s t o p a t h o l o g y
Well formed hollow tubules lined by
Sertoli cells are generally admixed with
rounded islands of granulosa cells growing
in a microfollicular pattern. Variation
from this typical histology with a juvenile
granulosa cell pattern or an intermediate
or poorly differentiated Sertoli-Leydig cell
tumour with or without heterologous elements
has been reported {1820}. The
tumours are alpha-inhibin positive.
Prognosis and predictive factors
Almost all tumours are stage I at initial
p resentation and clinically benign. It is
i m p o rtant to mention the components of
the tumour in the diagnosis, in part i c u l a r
whether the granulosa cell component
is of adult or juvenile type and also the
subtype of Sertoli-Leydig cell tumour.
Sex cord-stromal tumours 159