Invasive breast carcinoma - IARC

A
Fig. 7.16 Epithelioid sarcoma of the vulva. A The tumour forms a multinodular mass beneath the skin with areas of haemorrhage. B The neoplasm is composed of
large epithelioid cells with pleomorphic nuclei, prominent nucleoli and frequent mitotic figures.
B
Synonym
Malignant rhabdoid tumour, adult type.
Histopathology
This tumour, which has histological and
immunological features similar to epithelioid
sarcoma of the extremities, has a
predilection for the vulva {1078}. The
growth pattern is frequently nodular, and
the tumour cells are large with abundant
amphophilic cytoplasm. The nuclei are
either large and pleomorphic with small
nucleoli or vesicular with pro m i n e n t
nucleoli. Keratin and vimentin stains are
positive in essentially all tumours, and
CD34 is positive in approximately onehalf
of the cases.
Prognosis and predictive factors
F requent re c u r rences and a high incidence
of metastasis mark the clinical course.
Alveolar soft part sarcoma
Definition
A sarcoma characterized by solid and
alveolar groups of large epithelial-like
cells with granular, eosinophilic cytoplasm.
Histopathology
The rare cases of alveolar soft part sarcoma
re p o rted in the vulva have the
same distinctive histology as those
neoplasms occurring in more conventional
soft tissue locations {2639}. The
tumour is composed of large uniform
cells with abundant granular to vacuolated
eosinophilic cytoplasm; the cells
a re compartmentalized into packets by
thin-walled often sinusoidal vessels.
Most of the tumours contain characteristic
intracytoplasmic PA S - p o s i t i v e ,
diastase resistent, rod-shaped cry s-
t a l s .
Liposarcoma
Liposarcomas are extremely rare in this
location {354,2062}. Both atypical lipomatous
tumours (well diff e re n t i a t e d
liposarcomas) and myxoid liposarcomas
have been reported.
Dermatofibrosarcoma
protuberans
Dermatofibrosarcoma protuberans is a
highly recurrent low grade cutaneous
sarcoma that is usually located on the
trunk. Although rare, more than 10 cases
have been reported in the vulva, and in
one such case a supernumerary ring
c h romosome with the characteristic
COL1A1/PDGFB fusion gene was found
{3004}.
Benign soft tissue tumours
Definition
Benign soft tissue tumours that arise in
the vulva.
ICD-O codes
Deep angiomyxoma 8841/1
Superficial angiomyxoma 8841/0
Angiomyofibroblastoma 8826/0
Cellular angiofibroma 9160/0
Leiomyoma 8890/0
Granular cell tumour 9580/0
A
B
Fig. 7.17 Deep angiomyxoma. A The tumour forms a large bulging mass with a pale myxoid surface. B The neoplasm contains vessels of variable calibre, some of
which are thick-walled. C The tumour is sparsely cellular and composed of uniform stellate cells set in a myxoid matrix.
C
Mesenchymal tumours 327