Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc
Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc
Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc
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Teratoma<br />
Teratomas are generally well circumscribed<br />
complex masses. Cartilage, calcification,<br />
fibrosis, and scar formation<br />
result in echogenic foci, which result in<br />
variable degrees of shadowing. Cyst formation<br />
is commonly seen in teratomas<br />
and the demonstration of a predominately<br />
cystic mass suggests that it is<br />
either a teratoma or a mixed germ cell<br />
tumour with a large component of teratoma<br />
within it.<br />
Fig. 4.45 Cystic trophoblastic tumour. The cyst is lined by relatively inactive appearing mononucleated trophoblastic<br />
cells.<br />
mononucleated trophoblastic cells with<br />
abundant eosinophilic cytoplasm. The<br />
nuclei often have smudged chromatin;<br />
mitotic figures are infrequent. Focal reactivity<br />
for hCG is found {427}.<br />
Teratomas<br />
Definition<br />
A tumour composed of several types of<br />
tissue representing different germinal<br />
layers (endoderm, mesoderm and ectoderm).<br />
They may be composed exclusively<br />
of well differentiated, mature tissues<br />
or have immature, fetal-like tissues.<br />
It has been recommended to consider<br />
these morphologies as a single entity<br />
based on genetics.<br />
Teratomas in children and the dermoid<br />
cyst are benign. Tumours consisting of<br />
ectoderm, mesoderm, or endoderm only<br />
are classified as monodermal teratomas<br />
e.g. struma testis. A single type of differentiated<br />
tissue associated with seminoma,<br />
embryonal carcinoma, yolk sac<br />
tumour or choriocarcinoma is classified as<br />
teratomatous component. Teratoma may<br />
contain syncytiotrophoblastic giant cells.<br />
ICD-O codes<br />
Teratoma 9080/3<br />
Dermoid cyst 9084/0<br />
Monodermal teratoma<br />
Teratoma with somatic type<br />
malignancies 9084/3<br />
Synonyms<br />
Mature teratoma, immature teratoma,<br />
teratoma differentiated (mature), teratoma<br />
differentiated (immature).<br />
Epidemiology<br />
Teratoma occurs in two age groups. In<br />
adults, the frequency of pure teratoma<br />
ranges from 2.7-7% {804,1807} and 47-<br />
50% in mixed TGCTs {172,2753}. In children,<br />
the incidence is between 24-36%<br />
{326,2366}. A number of congenital<br />
abnormalities, predominantly of the GU<br />
tract have been observed {883,2664}. In<br />
the prepubertal testis, the presence of<br />
IGCNU is not proven, because the markers<br />
used are not specific at this period of<br />
life for IGCNU {1617,2264,2482}.<br />
Clinical features<br />
Signs and symptoms<br />
In children, 65% of teratomas occur in<br />
the 1st and 2nd year of life with a mean<br />
age of 20 months. In postpubertal<br />
patients, most are seen in young adults.<br />
Symptoms consist of swelling or are due<br />
to metastases. Occasionally, serum levels<br />
of AFP and hCG may be elevated in<br />
adult patients {1211}.<br />
Most patients present with a mass that is<br />
usually firm, irregular or nodular, nontender<br />
and does not transilluminate.<br />
Approximately 2-3% of prepubertal testis<br />
tumours may be associated with or misdiagnosed<br />
as a hydrocele, particularly if the<br />
tumour contains a cystic component.<br />
Since neither of these tumours is hormonally<br />
active, precocious puberty is not<br />
seen. Serum alpha-fetoprotein (AFP) levels<br />
are helpful in the differentiation of teratomas<br />
from yolk sac tumours {924,2264}.<br />
Imaging<br />
Epidermoid cyst<br />
The distinctive laminated morphology is<br />
reflected in ultrasound images. They are<br />
sharply marginated, round to slightly<br />
oval masses. The capsule of the lesion<br />
is well defined and is sometimes calcified.<br />
The mass may be hypoechoic but<br />
the laminations often give rise to an<br />
“onion-skin” or “target” appearance<br />
{813,2377}. Teratomas and other malignant<br />
tumours may have a similar<br />
appearance and great care should be<br />
taken in evaluating the mass for any<br />
irregular borders, which would suggest<br />
a malignant lesion {671,813}.<br />
Macroscopy<br />
The tumours are nodular and firm. The<br />
cut surfaces are heterogeneous with<br />
solid and cystic areas corresponding to<br />
the tissue types present histologically.<br />
Cartilage, bone and pigmented areas<br />
may be recognizable.<br />
Tumour spread<br />
Metastatic spread from teratomas in prepubertal<br />
children is not reported {326,<br />
330,2805}. Conversely, similar tumours found<br />
after puberty are known to metastasize.<br />
Histopathology<br />
The well differentiated, mature tissue<br />
types consist of keratinizing and nonkeratinizing<br />
squamous epithelium, neural<br />
and glandular tissues. Organoid structures<br />
are not uncommon, particularly in<br />
children such as skin, respiratory, gastrointestinal<br />
and genitourinary tract.<br />
Thyroid tissue has rarely been observed<br />
{2792}. Of the mesodermal components,<br />
muscular tissue is the most common<br />
{548}. Virtually any other tissue type can<br />
be seen. Fetal type tissue may also consist<br />
of ectodermal, endodermal and/or<br />
mesenchymal tissues. They can have an<br />
organoid arrangement resembling primitive<br />
renal or pulmonary tissues. It can be<br />
difficult to differentiate fetal-type tissues<br />
from teratoma with somatic type malig-<br />
Germ cell tumours<br />
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