Invasive breast carcinoma - IARC

A
B
Fig. 2.108 Strumal carcinoid. A Sectioned surface of the ovarian tumour shows a spongy brown area reflecting the strumal component and a solid yellow region
reflecting the carcinoid component. B The strumal and carcinoid tumour patterns are located side by side, with the struma on the right. C Immunoreactivity for cytokeratin
7 is present in the strumal elements but not in the carcinoid trabeculae.
C
bling neoplasms of the nervous system
with a similar spectrum of differentiation.
Epidemiology
Less than 40 cases are re p o rted in
patients 6-69 years old (average 28),
{1077,1418,1476}.
Clinical features
The tumours usually present as a pelvic
mass.
Macroscopy
Tumours are unilateral and 4-20 cm in
diameter, averaging 14 cm {1476}. The
sectioned surface varies from solid with
friable, gray-pink tissue to cystic with
papillary excrescences in their inner or
outer surface {1077}.
Tumour spread and staging
The majority of patients have stage II or
III disease at laparotomy usually in the
form of peritoneal implants {1476}.
Histopathology
These tumours are morphologically identical
to their nervous system counterparts.
They may be divided into three
categories as follows:
(1) Well diff e rentiated forms such as
ependymoma.
(2) Poorly differentiated tumours such as
primitive neurectodermal tumour (PNET),
and medulloepithelioma.
(3) Anaplastic forms such as glioblastoma
multiforme.
Whilst ependymomas are not found in
association with teratoma, other neuroectodermal
tumours in the ovary may be
associated with elements of mature or
immature teratoma {2605}. Cases previously
re p o rted as neuroblastoma or
medulloblastoma would now most likely
be classifed as PNETs since the morphology
of all three tumours is similar
with the term medulloblastoma being
reserved for cerebellar and neuroblastoma
for adrenal neoplasms {1474}.
Medulloepithelioma, on the other hand,
has a distinctive appearance characterized
by papillary, tubular or trabecular
arrangements of neoplasiic neuroepithelium
mimicking the embryonic neural
tube {1474}.
Ependymomas and anaplastic tumours
a re immunoreactive for glial fibrillary
acidic protein. The characteristic
immunoprofile of PNETs, vimentin and
MIC2 protein (CD99) positive and GFAP,
cytokeratin, desmin. chromogranin, and
inhibin negative, help to distinguish
these tumours from small cell carcinoma
and juvenile granulosa cell tumour.
Somatic genetics
Reverse transcription-polymerase chain
reaction in a case of ovarian PNET led to
the detection of EWS/FLI1 chimeric transcript,
originating from the characteristic
t(11;22)(q24;q12) translocation of the
PNET/Ewing tumour family {1418}.
Prognosis and predictive factors
Most patients with ovarian ependymomas
survive despite multiple re c u r-
rences, whereas patients with PNET and
anaplastic tumours have a poor outcome
{1476}.
Carcinomas
Definition
A dermoid cyst in which a secondary
carcinoma develops.
Epidemiology
Malignancy arising within a mature cystic
teratoma is a rare complication (1-2% of
cases), mostly re p o rted in postmenopausal
women (mean 51-62 years)
{1214,1429,2164}.
Clinical features
The tumour may present as a dermoid
cyst or as an advanced ovarian cancer
depending on tumour stage {2605}. The
tumour may show adherence to surrounding
pelvic structures {1214,
1429,2164}.
Macroscopy
On macroscopic examination cauliflower
exophytic growth, infiltrative grey-white
plaques or thickenings of the cyst wall
with necrosis and haemorrhage may be
seen {1214,1429,2164}.
Histopathology
The malignancy may be detectable only
after histological examination, thus dermoid
cysts in postmenopausal women
must be adequately sampled. Any component
of a mature teratoma may undergo
malignant transform a t i o n .
Carcinomas are the most common malignancy,
with squamous cell carcinomas
accounting for 80% of cases and 51% of
all primary ovarian squamous cell carcinomas
{1214,2255}. Adenocarcinoma is
the second most common lmalignancy
arising in dermoid cysts {1456}.
A d e n o c a rcinoma of intestinal type
{2970}, Paget disease, adenosquamous
c a rcinoma, transitional cell carc i n o m a
{1456}, undifferentiated carcinoma, small
cell carcinoma, basal cell carcinoma and
c a rc i n o s a rcoma {123} have been
described {2605}. The malignant component
invades other parts of the dermoid
cyst and its wall.
Somatic genetics
Selective tissue microdissection and
genetic analyses of malignant tumours
174 Tumours of the ovary and peritoneum