Invasive breast carcinoma - IARC

Fig. 2.113 Mixed germ cell-sex cord-stromal tumour associated with dysgerminoma. The former is composed of clusters of germ cells and small sex-cord type cells
in a dense fibrous stroma. Note the dysgerminoma in the right upper portion of the field.
finding in gonadoblastoma, the sex cord
derivatives also show mitotic activity
{2847,2849,2850}.
The composition of a mixed germ cellsex
cord-stromal tumour varies, and in
some areas the sex cord elements may
predominate, whereas in others there is a
predominance of germ cells. The cystic
spaces seen in some tumours resemble
the cystic spaces seen in cystic and retiform
Sertoli cell tumours and should not
be confused with cysts and papillae
seen in ovarian serous tumours, which
they may resemble superficially {2849,
2850}.
Although originally mixed germ cell-sex
c o rd - s t romal tumours were found to
occur in pure form, it was later noted
that approximately 10% of cases are
associated with dysgerminoma or other
malignant germ cell elements. This finding
is by far less common than in
gonadoblastoma.
The tumour is always found in normal
ovaries, and whenever the unaffected
contralateral gonad is examined, it is a
normal ovary.
Genetic susceptibility
Familial clustering of these rare tumours
has not been reported.
Prognosis and predictive factors
In the majority of cases the mixed germ
cell-sex cord-stromal tumour occurs in
pure form. Mixed germ cell-sex cordstromal
tumours are generally benign
and are treated by unilateral oophorectomy.
Preservation of fertility should be a
priority in those patients that appear to
have a unilateral mixed germ cell-sex
cord-stromal tumour.
The association with other neoplastic
germ cell elements is more common in
postmenarchal subjects, but it may be
seen in children in the first decade
{2849,2850}. One case of mixed germ
cell-sex cord-stromal tumour was associated
with para-aortic lymph node and
abdominal metastases {1556}. Another
patient developed intra-abdominal metastatic
disease two years following the
excision of a large ovarian tumour {124}.
Both patients are well and disease free
following surgery and chemotherapy. It is
of interest that the tumour associated
with the intra-abdominal re c u r re n c e
showed an unusual histological pattern
of sex cord tumour with annular tubules,
but differed from the latter by the presence
of numerous germ cells {124}.
In those cases with metastatic disease,
aggressive surgical cytoreduction is performed,
and the BEP regimen is routinely
used postoperatively.
Mixed germ cell-sex cord-stromal tumours 179