Invasive breast carcinoma - IARC

Genetic susceptibility
JGCTs may present as a component of a
variety of non-hereditary congenital syndromes
including Ollier disease (enchondromatosis)
{2857,3015} and Maffucci
syndrome (enchondromatosis and haemangiomatosis)
{1102,2859}. Bilateral
JGCT may develop in infants with features
suggestive of Goldenhar (craniofacial
and skeletal abnormalities) {2306} or
Potter syndrome {2468}.
Prognosis and predictive factors
Despite their more primitive histological
appearance, only about 5% of JGCTs
behave aggressively, and these usually
do so within 3 years of presentation. The
overall prognosis for JGCT is good with a
1.5% mortality associated with stage IA
tumours; but it is poor in stage II or higher
tumours {3195}.
Thecoma-fibroma group
Definition
Tumours forming a continuous spectrum
from those composed entirely of fibroblasts
and producing collagen to those
containing a predominance of theca
cells.
Thecoma 8600/0
Luteinized thecoma 8601/0
Fibroma, NOS 8810/0
Cellular fibroma 8810/1
Fibrosarcoma 8810/3
Stromal tumour with minor sex
cord elements 8593/1
Sclerosing stromal tumour 8602/0
Thecoma
Definition
Thecomas are stromal tumours composed
of lipid-containing cells resembling
theca interna cells with a variable
component of fibroblasts. Luteinized thecomas
contain lutein cells in a background
of thecoma or fibroma.
A
B
Fig. 2.64 Thecoma. A Sectioned surface shows a
circumscribed bright yellow tumour compressing
the adjacent ovary. B The tumour cells have abun -
dant pale, poorly delimited cytoplasm.
with sclerosing peritonitis typically
occurs in young women less than 30
years, only rarely occurring in older
women {520}.
Clinical features
Typical thecomas may be discovere d
incidentally or produce non-specific
signs and symptoms of a pelvic mass.
Symptoms related to estrogen pro d u c-
Fig. 2.65 Luteinized thecoma. Nests of luteinized
tumour cells with eosinophilic cytoplasm and
round nucleoli occur in a background of neoplastic
spindle-shaped cells.
tion including abnormal uterine bleeding
occur in about 60% of patients, and
about 20% of postmenopausal women
with thecoma have endometrial adenoc
a rcinoma or rarely a malignant müllerian
mixed tumour or endometrial stro m a l
s a rcoma {2300}. Luteinized thecomas
have a lower frequency of estro g e n i c
manifestations than typical thecomas,
and about 10% are associated with
Epidemiology
Typical thecomas are about one-third as
common as granulosa cell tumours. The
g reat majority (84%) occur in postmenopausal
women (mean age 59
years). Thecomas are rare before puberty,
and only about 10% occur in women
younger than 30 years {283}. The rare
variant of luteinized thecoma associated
Fig. 2.66 Luteinized thecoma. There are clusters of luteinized cells with vacuolated cytoplasm dispersed
among the spindle-shaped cells
Sex cord-stromal tumours 149