Invasive breast carcinoma - IARC

Sex cord-stromal tumours
F.A. Tavassoli
S. Fujii
E. Mooney T. Kiyokawa
D.J. Gersell
P. Schwartz
W.G. McCluggage R.A. Kubik-Huch
I. Konishi L.M. Roth
Definition
Ovarian tumours composed of granulosa
cells, theca cells, Sertoli cells, Leydig
cells and fibroblasts of stromal origin,
singly or in various combinations. Overall,
sex cord - s t romal tumours account for
about 8% of ovarian neoplasms.
Granulosa-stromal cell
tumours
Definition
Tumours containing granulosa cells, theca
cells or stromal cells resembling fibroblasts
or any combination of such cells.
Granulosa cell tumour group
Definition
A neoplasm composed of a pure or at the
least a 10% population of granulosa cells
often in a fibrothecomatous background.
Two major subtypes are recognized, an
adult and a juvenile type.
ICD-O codes
Granulosa cell tumour group
Adult granulosa cell tumour 8620/1
Juvenile granulosa cell tumour 8622/1
Epidemiology
Granulosa cell tumours account for app
roximately 1.5% (range, 0.6-3%) of all
ovarian tumours. The neoplasm occurs in
a wide age range including newborn infants
and postmenopausal women. About
5% occur prior to pubert y, whereas almost
60% occur after menopause {284,2588}.
Aetiology
The aetiology of these tumours is unknown.
Several studies suggest that inf
e rtile women and those exposed to ovulation
induction agents have an incre a s e d
risk for granulosa cell tumours {2458,
2 9 8 2 , 3 1 2 5 } .
Clinical features
Signs and symptoms
Granulosa cell tumours may present as
an abdominal mass, with symptoms suggestive
of a functioning ovarian tumour or
both. About 5-15% present with symptoms
suggestive of haemoperitoneum
secondary to rupture of a cystic lesion
{3195}. Ascites develops in about 10% of
the cases. The tumour is clinically occult
in 10% of the patients {829}. Granulosa
cell tumours produce or store a variety of
steroid hormones. When functional, most
are estrogenic, but rarely androgenic activity
may occur. The symptoms and clinical
presentation vary depending on the
patient’s age and reproductive status. In
prepubertal girls, granulosa cell tumours
frequently induce isosexual pseudoprecocious
puberty. In women of reproductive
age, the tumour may be associated
with a variety of menstrual disord e r s
related to hyperoestrinism. In postmenopausal
women, irregular uterine bleeding
due to various types of endometrial hyperplasia
or, rare l y, well diff e rentiated adenocarcinoma
is the most common manifestation
of hyperoestrinism. A rare unilocular
thin-walled cystic variant is often
androgenic when functional {1971,2059}.
Imaging
Cross sectional imaging, i.e. computed
tomography and magnetic re s o n a n c e
imaging is of value in the surgical planning
and preoperative determination of
resectability of patients with granulosa
cell tumours {859,1480,1728,1915,2131}.
In contradistinction to epithelial ovarian
tumours, granulosa cell tumours have
been described as predominantly solid
adnexal lesions; variable amounts of cystic
components may, however, be present.
Enlargement of the uterus and
endometrial thickening might be seen as
a result of the hormone production of the
tumour {859,1480,1728,1915,2131}.
Adult granulosa cell tumour
Epidemiology
More than 95% of granulosa cell tumours
are of the adult type, which occurs in
middle aged to postmenopausal women.
Macroscopy
Adult granulosa cell tumours (AGCTs) are
typically unilateral (95%) with an average
size of 12.5 cm and are commonly encapsulated
with a smooth or lobulated surface.
The sectioned surface of the tumour
Fig. 2.55 Granulosa cell tumour. Axial contrastenhanced
computed tomography image of the
pelvis shows a large, well defined, multicystic mass.
A
Fig. 2.56 Adult granulosa cell tumour, microfollicular pattern. A An aggregate of neoplastic granulosa cells
contains numerous Call-Exner bodies. B The Call-Exner bodies contain fluid and/or pyknotic nuclei; the
tumour cells have scant cytoplasm and longitudinal nuclear grooves.
B
146 Tumours of the ovary and peritoneum