CHAPTER X CHAPTER 4 - Cancer et environnement

A
Fig. 4.73 A Sertoli cell tumour. B Sertoli cell tumour mimicking seminoma.
B
(30%) {2575,2894}. Tumour cells are
invariably negative for placental alkaline
phosphatase, alpha-fetoprotein, human
chorionic gonadotropin.
Ultrastructure
Charcot-Böttcher crystals, composed of
filaments, are rarely seen but are considered
to be typical of Sertoli cells.
Variants
In addition to Sertoli cell tumours NOS
two variants are recognized: large cell
calcifying Sertoli cell tumour, and sclerosing
Sertoli cell tumour. There are not
enough data to determine whether the
proposed variants such as "lipid rich variant"
and "Sertoli cell tumour with heterologous
sarcomatous component" {875}
warrant separation from the Sertoli cell
tumour NOS.
Large cell calcifying Sertoli cell tumour
(LCCST)
Large cell calcifying Sertoli cell tumour
(LCCST) can be sporadic, but occur also
as parts of the Carney and Peutz-
Jeghers syndromes {1391}. Only about
50 cases of this neoplasm have been
reported so far.
Sporadic tumours account for 60% of
cases, whereas the remaining 40% are
associated with genetic syndromes or
have endocrine disorders {1391}.
Endocrine symptoms, including precocious
puberty and gynecomastia are
found in a significant number of cases. In
contrast to Sertoli cell tumours NOS,
most patients harbouring LCCST are
young and the average age is 16 years.
The youngest patient on record was 2
years old. In most cases the tumours are
benign, but 20% are malignant. In 40% of
cases the tumours are bilateral.
Microscopic features of LCCST include
nests and cords of relatively large polygonal
cells with eosinophilic cytoplasm
embedded in myxohyaline stroma.
Tumour cells have vesicular and relatively
large nuclei and prominent nucleoli,
but mitoses are rare. The stroma may be
hyalinized, often with abundant neutrophils,
and typically shows broad areas
of calcification, though a substantial proportion
lack calcification. Intratubular
spread of the tumour cells is typically
found in most cases {366}.
Sclerosing Sertoli cell tumour (SSCT)
Sclerosing Sertoli cell tumour (SSCT) is
rare and less than 20 cases of this variant
are recorded {929,2951}. They occur
in adults and the average age at the time
of diagnosis is 35 years.
Most tumours on record are relatively
small (0.4-1.5 cm). Microscopically, features
of SSCT include small neoplastic
tubules surrounded by dense sclerotic
stroma. The tubules may be solid or hollow,
and may be discrete or anastomosing.
Typically the tumours contain
entrapped non neoplastic tubules.
Differential diagnosis
Sertoli cell tumours NOS need to be dis-
A
B
Fig. 4.74 Sertoli cell tumour A Large cell calcifiying variant. Cords and nests of cells in a fibrous stroma with focal ossification. B Large cell calcifiying Sertoli cell tumour.
254 Tumours of the testis and paratesticular tissue