Invasive breast carcinoma - IARC

in all grade I and most grade II angiosarcomas
these markers may be lost in
m o re poorly diff e rentiated tumours or
areas of tumour.
Prognosis and predictive factors
If well diff e rentiated angiosarc o m a s
(Grade I) were excluded, this bre a s t
tumour is usually lethal {457}.
Grading systems highlight the relative
benignity of well differentiated angiosarcomas.
The survival probability for grade
I tumours was estimated as 91% at 5
years and 81% at 10 years. For grade III
tumours the survival probability was 31%
at 2 years and 14% at 5 and 10 years.
Grade II lesions had a survival of 68% at
5 and 10 years. Recurrence free survival
at 5 years was 76% for grade I, 70% for
grade II and 15% for grade III angiosarcomas
{2436}. Metastases are mainly to
lungs, skin bone and liver. Very rarely
axillary lymph nodes show metastases at
presentation {457}. The grade can vary
between the primary tumour and its
metastases {2876}. Radio and
chemotherapy are ineffective.
Angiosarcoma of the skin of the
arm after radical mastectomy
followed by lymphoedema
Stewart and Treves in 1949 gave a lucid
description of a condition subsequently
named S-T syndrome {2793}. They
reported six patients who had: 1) undergone
mastectomy for breast cancer
including axillary dissection; 2) developed
an “immediate postmastectomy
oedema” in the ipsilateral arm; 3)
received irradiation to the breast area
together with the axilla; 4) developed
oedema which started in the arm and
extended to the forearm and finally the
dorsum of the hands and digits.
The patients ranged in age from 37-60
years, with a mean age of 64 years {3149}.
The angiosarcomatous nature of S-T
s y n d rome has been conclusively pro v e d
by ultrastructure and immunohistochemi
s t ry in most of the cases studied {1049,
1462,1862,2690}.
The oedema is preceded by radical mastectomy
for breast carcinoma including
axillary dissection {275} and develops
within 12 months. Nearly 65% of patients
also had irradiation of the chest wall and
axilla {2425}. The interval to tumour appearance
varies from 1-49 years {2425},
but most become evident about 10 years
following mastectomy {2752,3149}.
S-T syndrome is a lethal disease with a
median survival of 19 months {3149}.
Lungs are the most frequent site of
metastasis.
Post-radiotherapy angiosarcoma
A n g i o s a rcoma can manifest itself after
radiotherapy in two separate settings.
1) In the chest wall when radiotherapy
has been administered after mastectomy
for invasive breast carcinoma with a
latency time ranging from 30 to 156
months (mean 70 months). The age is
m o re advanced than that of de novo
a n g i o s a rcoma ranging from 61 to 78
years {2223}. In these cases the neoplastic
endothelial proliferation is necessarily
confined to the skin {392}.
2) In the breast after conservation tre a t-
ment for breast carcinoma. Fifty two
cases had been re p o rted as of
December 1997. The first case was
described in 1987 {1764}. This type of
a n g i o s a rcoma involves only the skin in
m o re than half the cases, while exclusive
involvement of breast parenchyma is
v e ry rare. Most tumours (81%) are multifocal
and a large majority of patients harbour
grade II to III angiosarc o m a s .
Radiotherapy and chemotherapy are
i n e ffective {2876}.
Liposarcoma
Definition
A variably cellular or myxoid tumour containing
at least a few lipoblasts.
ICD-O code 8850/3
Epidemiology
P r i m a ry liposarcoma should be distinguished
from liposarcomatous diff e-
rentiation in a phyllodes tumour. It occurs
p redominantly in women ranging in age
f rom 19-76 years (median, 47 years) {116,
138}. The tumour only rarely occurs in the
male breast {3027}. Liposarcoma following
radiation therapy for breast carc i n o m a
has been re p o rt e d .
Clinical features
Patients present most often with a slowly
enlarging, painful mass. In general, skin
changes and axillary node enlargement
a re absent. Rarely the tumour is bilateral
{ 3 0 2 7 } .
Macroscopy
L i p o s a rcomas are often well circ u m-
scribed or encapsulated, about one-third
have infiltrative margin. With a median
size of 8 cm, liposarcomas may become
enormous exceeding 15 cm {116,138}.
Necrosis and haemorrhage may be present
on the cut surface of larger tumours.
Histopathology
The histopathology and immunophenotype
is identical to that of liposarcoma at
other sites. The presence of lipoblasts
establishes the diagnosis. Practically
e v e ry variant of soft tissue liposarc o m a
has been re p o rted in the breast, including
the pleomorphic, dediff e rentiated and
myxoid variants. Despite the well delineated
gross appearance, many mammary
liposarcomas have at least partial infiltrative
margins on histological examination.
Atypia is often present at least focall
y. The well diff e rentiated and myxoid
variants have a delicate arborizing vascular
network and few lipoblasts. These
may assume a signet-ring appearance in
the myxoid variant. The pleomorphic variant
is composed of highly pleomorphic
cells and bears significant re s e m b l a n c e
to malignant fibrous histiocytoma; the
p resence of lipoblasts identifies the
lesion as a liposarcoma. Mitotic figure s
a re readily identifiable in this variant.
Differential diagnosis
Vacuolated cells in a variety of lesions
may be confused with lipoblasts. Ty p i c a l
lipoblasts have scalloped irregular nuclei
with sharply defined vacuoles that contain
lipid rather than glycogen or mucin.
Clear nuclear pseudo-inclusions are a
characteristic of the bizarre large cells in
atypical lipomatous tumours and help
distinguish these atypical cells from true
lipoblasts that are diagnostic of a liposarc
o m a .
Fig. 1.143 Liposarcoma. Note the highly pleomorphic
nuclei and multiple lipoblasts.
96 Tumours of the breast