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CHAPTER X CHAPTER 4 - Cancer et environnement

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A<br />

Fig. 4.40 Yolk sac tumour. A Pleomorphic cell type. B Polyvesicular vitelline pattern.<br />

B<br />

Fig. 4.41 Yolk sac tumour. AFP positive staining.<br />

Clinical features<br />

Signs and symptoms<br />

Patients with choriocarcinoma are young,<br />

averaging 25-30 years of age. They most<br />

commonly present with symptoms referable<br />

to m<strong>et</strong>astases. The haematogenous<br />

distribution of m<strong>et</strong>astases explains the<br />

common presenting symptoms: haemoptysis,<br />

dyspnoea, central nervous system<br />

dysfunction, haematemesis, melena,<br />

hypotension, and anaemia. Haemorrhage<br />

in multiple visceral sites represents<br />

the hallmark of a “choriocarcinoma<br />

syndrome” {1529}. Patients typically<br />

have very high levels of circulating<br />

human chorionic gonadotropin (hCG)<br />

(commonly greater than 100,000<br />

mIU/ml). Because of the cross reactivity<br />

of hCG with luteinizing hormone, the consequent<br />

Leydig cell hyperplasia causes<br />

some patients (about 10%) to present<br />

with gynecomastia. Occasional patients<br />

develop hyperthyroidism because of the<br />

cross reactivity of hCG with thyroid stimulating<br />

hormone. Clinical examination of<br />

the testes may or may not disclose a<br />

mass. This is because the primary site<br />

may be quite small, or even totally<br />

regressed, despite widespread m<strong>et</strong>astatic<br />

involvement.<br />

be surrounded by a discernible rim of<br />

white to tan tumour. In some cases with<br />

marked regression, a white/grey scar is<br />

the only identifiable abnormality.<br />

Tumour spread<br />

Choriocarcinoma disseminates by both<br />

haematogenous and lymphatic pathways.<br />

R<strong>et</strong>roperitoneal lymph nodes are<br />

commonly involved, although some<br />

patients with visceral m<strong>et</strong>astases may<br />

lack lymph node involvement.<br />

Additionally, autopsy studies have shown<br />

common involvement of the lungs<br />

(100%), liver (86%), gastrointestinal tract<br />

(71%), and spleen, brain, and adrenal<br />

glands (56%) {1800}.<br />

Histopathology<br />

Choriocarcinoma has an admixture, in<br />

varying proportions, of syncytiotrophoblastic,<br />

cytotrophoblastic and intermediate<br />

trophoblastic cells. These cellular<br />

components are arranged in varying<br />

patterns, usually in an extensively<br />

haemorrhagic and necrotic background.<br />

In some examples, the syncytiotrophoblasts<br />

"cap" nests of cytotrophoblasts in<br />

a pattern that is reminiscent of the architecture<br />

seen in immature placental villi.<br />

Most commonly, they are admixed in a<br />

more or less random fashion, usually at<br />

the periphery of a nodule that has a central<br />

zone of haemorrhage and necrosis.<br />

In occasional cases, which have been<br />

descriptively termed "monophasic"<br />

{2672}, the syncytiotrophoblastic cell<br />

component is inconspicuous, leaving a<br />

marked preponderance of cytotrophoblastic<br />

and intermediate trophoblastic<br />

cells. Blood vessel invasion is commonly<br />

identified in all of the patterns.<br />

The syncytiotrophoblastic cells are usually<br />

multinucleated with deeply staining,<br />

eosinophilic to amphophilic cytoplasm;<br />

they typically have several, large, irregularly<br />

shaped, hyperchromatic and often<br />

smudged appearing nuclei. They often<br />

Imaging<br />

Choriocarcinomas do not have distinctive<br />

imaging characteristics to differentiate<br />

them from other non-seminomatous<br />

tumours. Their appearance varies from<br />

hypoechoic to hyperechoic. They may<br />

invade the tunica albuginea.<br />

Macroscopy<br />

Choriocarcinoma most commonly presents<br />

as a haemorrhagic nodule that may<br />

A<br />

Fig. 4.42 Choriocarcinoma. A Longitudinal ultrasound image of the testis shows a small, slightly h<strong>et</strong>erogeneous<br />

mass, which is almost isoechoic compared to the normal parenchyma (arrow). B Chest radiograph<br />

shows multiple lung m<strong>et</strong>astases. The patient presented with hemoptysis.<br />

B<br />

Germ cell tumours 241

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