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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 />

243

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