Invasive breast carcinoma - IARC

Miscellaneous tumours and tumour-like
conditions of the ovary
L.M. Roth
A. Tsubura
M. Dietel
H. Senzaki
D e f i n i t i o n
A group of benign and malignant ovarian
tumours of diverse or uncertain origin.
ICD-O codes
Small cell carcinoma,
hypercalcaemic type 8041/3
Small cell carcinoma,
pulmonary type 8041/3
Large cell neuroendocrine
carcinoma 8013/3
Adenoid cystic carcinoma 8200/3
Basal cell tumour 8090/1
Hepatoid carcinoma 8576/3
Malignant mesothelioma 9050/3
Gestational choriocarcinoma 9100/3
Hydatidiform mole 9100/0
Ovarian wolffian tumour 9110/1
Wilms tumour 8960/3
Paraganglioma 8693/1
Myxoma 8840/0
Small cell carcinoma,
hypercalcaemic type
tumour has spread beyond the ovary at
the time of initial laporatomy.
Histopathology
On histological examination the tumours
typically grow diff u s e l y, but they may form
small islands, trabeculae or cords. They
f requently form follicle-like spaces that
almost always contain eosinophilic fluid,
and nuclei show easily discernible nucleoli.
Foci of either benign or malignant
mucinous epithelium are present in 10-
15% of the cases. Ty p i c a l l y, the cells of
the tumour contain scant cytoplasm, but
in approximately one-half of cases a component
of large cells with abundant
eosinophilic cytoplasm and nuclei containing
prominent nucleoli is present.
I m m u n o p ro f i l e
Small cell carcinomas generally stain for
epithelial membrane antigen but not for
inhibin {2376}. Variable staining of the
neoplastic cells for vimentin, cytokeratin
and epithelial membrane antigen is
observed {46}.
Cytometric studies
Flow cytometric studies of paraff i n -
embedded tissue has demonstrated that
the neoplastic cells are diploid {755}.
Electron microscopy
E l e c t ron microscopic examination has
shown an epithelial appearance to the
neoplasm consisting of small desmosomes
and, in some cases, tight junctions
{695}. Dilated granular endoplasmic re t i c-
ulum containing amorphous material is
characteristically present within the cytoplasm
{695,696}. Few or no neuro s e c re t o-
ry granules have been identified.
D i ff e rential diagnosis
Because of the young age of the patients
and the presence of follicle-like spaces in
the neoplasm, the diff e rential diagnosis
includes juvenile granulosa cell tumour.
D e f i n i t i o n
An undiff e rentiated carcinoma that is usually
associated with paraendocrine hypercalcaemia
and is composed primarily of
small cells.
Clinical features
This neoplasm typically occurs in young
women and is associated with paraendocrine
hypercalcaemia in appro x i m a t e l y
t w o - t h i rds of patients {3204}. Most of the
patients presented with abdominal
swelling or pain related to their tumour;
h o w e v e r, one patient had a neck exploration
for presumed parathyroid disease
with negative results before the ovarian
tumour was discovered {3204}.
M a c r o s c o p y
The tumours are usually large and predominantly
solid, pale white to gray masses.
Necrosis, haemorrhage and cystic
degeneration are common.
Table 2.08
Comparison of small cell carcinoma of the hypercalcaemic type with juvenile granulosa cell tumour.
Small cell carcinoma, hypercalcaemic type
Stage I in 50% of cases
Highly malignant
Hypercalcaemia in two-thirds of cases
Never estrogenic
Scant or non-specific stroma
Follicles often contain mucicarminophilic
basophilic secretion
Nuclei hyperchromatic
Prominent nucleoli
Mitoses frequent
Usually epithelial membrane antigen positive
Juvenile granulosa cell tumour
Stage I in greater than 97% of cases
Usually non-aggressive
Hypercalcaemia absent
Usually estrogenic
Fibrothecomatous stroma common
Follicles rarely contain mucicarminophilic
basophilic secretion
Rounded euchromatic nuclei,
Indistinct nucleoli
Mitoses variable
Epithelial membrane antigen negative
Tumour spread and staging
In approximately 50% of the patients the
Alpha-inhibin negative
Alpha-inhibin positive
182 Tumours of the ovary and peritoneum