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
You also want an ePaper? Increase the reach of your titles
YUMPU automatically turns print PDFs into web optimized ePapers that Google loves.
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 metastases. The haematogenous<br />
distribution of metastases 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 metastatic<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 />
Retroperitoneal lymph nodes are<br />
commonly involved, although some<br />
patients with visceral metastases 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 heterogeneous<br />
mass, which is almost isoechoic compared to the normal parenchyma (arrow). B Chest radiograph<br />
shows multiple lung metastases. The patient presented with hemoptysis.<br />
B<br />
Germ cell tumours 241