Invasive breast carcinoma - IARC

Clinical features
M o re than 70% of the tumours are unilateral.
The age range is 11-76 years
with the majority of tumours occurring
a round the 5th and 6th decade. The
clinical symptoms do not differ fro m
those recognized for other ovarian
tumours.
M a c r o s c o p y
Most tumours are solid and firm, but
some may show variably sized cysts,
sometimes filled with mucoid or haemorrhagic
fluid or debris. The sectioned
s u rface appears yellow-white or tan,
sometimes interspersed with gre y
f i b rous bundles or septa.
Fig. 2.36 Endometrioid borderline tumour. Whereas most endometrioid borderline tumours have an adenofibromatous
appearance, the adenofibromatous lesion is rarely evident within a large cystic mass.
overlying a dominant sarcomatous mesenchyme.
Clinical features
Most of the tumours reported so far have
been unilateral, occurring in the 4th and
5th decades. Abdominal discomfort and
distension are the usual complaints.
Macroscopy and histopathology
The tumour is frequently adherent to the
s u r rounding tissue {512,604,929}. The
macroscopic and histological features
are described in detail in the uterine
counterpart (see chapter 4).
Prognosis and predictive factors
Occasional re p o rts have linked the
s p read of adenosarcomas into the
abdominal cavity with a poor clinical
outcome {510}. The stroma is often predominantly
fibrotic, oedematous or hyalized
with characteristic foci of perivascular
cuffing seen only focally (sometimes,
the foci are very small) and still
the tumours recur and kill the patient
{760}. Unfort u n a t e l y, there exist no
established morphological criteria to
p redict such biological behaviour. Howe
v e r, if during the course of the disease
s a rcomatous overgrowth develops, signifying
invasive potential, the patient
re q u i res careful monitoring. In a series
of 40 cases, the 5-year survival was
64%, the 10-year survival 46% and the
15-year survival 30% {760}. Age gre a t e r
than 53 years, tumour rupture, high
grade and the presence of high grade
s a rcomatous overgrowth appear to be
associated with re c u r rence or extraovarian
spread. Ovarian adenosarcoma has
a worse prognosis than its uterine count
e r p a rt, presumably because of the
g reater ease of peritoneal spread {760}.
T h e r a p e u t i c a l l y, an aggressive surgical
a p p roach with wide excision is most
often recommended {510}. Chemotherapy
and radiation may be applied in
individual cases; however, no established
protocols exist.
Endometrioid stromal and
undifferentiated ovarian sarcoma
Definition
Endometrioid stromal sarcoma (ESS) is
a monophasic sarcomatous tumour
characterized by a diffuse pro l i f e r a t i o n
of neoplastic cells similar to stro m a l
cells of proliferative endometrium. At its
p e r i p h e ry the tumour exhibits a typical
infiltrative growth pattern. Those neoplasms
that have moderate to marked
pleomorphism, significant nuclear anaplasia
and more cytoplasm than is
found in endometrial stromal cells
should be classified as undiff e re n t i a t e d
ovarian sarc o m a .
Histopathology
Roughly half of the cases of ESS are
associated with either endometriosis or a
similar sarcomatous lesion of the
endometrial stroma or both {2605}. The
dominant cell type of ESS consists of
small, round to oval, or occasionally spindle
shaped cells with round nuclei and
scanty, sometimes barely visible pale
cytoplasm. The cells may be arranged
haphazardly in a diffuse pattern or may
form parallel cell sheets mimicking fibroma.
Hypocellular areas with a distinct
oedematous appearance can be present.
Lipid droplets may be present within
tumour cells, which are often associated
with foam cells. A hallmark of ESS is the
presence of abundant small thick-walled
vessels resembling spiral arteries of the
late secretory endometrium. The vessels
often are surrounded by whorls of neoplastic
cells. Reticulin stain discloses
delicate fibrils characteristically enveloping
individual tumour cells. The cellularity
can vary markedly within the same
specimen. The tumour can be partly
intersected by fibrous bands form i n g
more or less distinct nodules. Sometimes,
hyaline plaques are pre s e n t .
Rarely, cord-like or plexiform arrangements
of tumour cells similar to the
growth patterns seen in ovarian sex cord
tumours such as granulosa cell tumours
or thecomas are observed. In these
areas reticulin fibrils are more or less
absent. Rarely, glandular elements are
interspersed, but they never represent a
dominant feature. At its periphery the
tumour exhibits a typical infiltrative
g rowth pattern. In cases where the
tumour has spread into extraovarian
sites, a tongue-like pattern of invasion
134 Tumours of the ovary and peritoneum