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Spilled tumour

Stage IV

Metastatic disease

oping fetal liver. These cells contain a small round nucleus with fine nuclear chromatin and an indistinct nucleolus. The cytoplasm varies from finely granular to clear, reflecting variable amounts of glycogen and lipid which can impart a 'light and dark' pattern to the lesion when viewed at lower magnifications. Cana-liculi may be seen between hepatocytes of the 2-3 cell layer trabeculae, but only rarely is bile stasis present. In biopsies taken before preoperative chemotherapy, foci of extramedullary haemato-poiesis (EMH) composed of clusters of erythroid and myeloid precursors may be present in the sinusoids {2023}. Sinusoids are lined by endothelial and Kupffer cells which show a more diffuse staining with UEA-1 and anti-CD34 than the focal staining of the sinusoidal endothelial cells of normal liver {1630}. The fetal phenotype has been significantly associated with both diploid DNA nuclear content and low proliferative activity assessed by flow cytometry and PCNA labeling index {1640}.

Combined fetal and embryonal epithelial

Approximately 20% of cases display a pattern combining fetal epithelial cells and sheets or clusters of small, ovoid to angulated cells with scant amounts of dark granular cytoplasm surrounding a nucleus with increased nuclear chromatin. The cells display little cohesive-ness but may cluster into pseudorosette, glandular or acinar structures. These small, round, blue cells resemble the blastemal cells seen in nephroblastomas, neuroblastomas and other 'embryonal' tumours in children. While often intermixed with the fetal epithelial cells, the foci of embryonal cells, which are devoid of glycogen and lipid, can be identified by their absence of staining with PAS or oil red-O stains. Mitotic activity is more pronounced in the embryonal areas, and associated with a low TGF-alpha expression. EMH, in the absence of preopera-tive chemotherapy, may also be noted {925}.

Macrotrabecular

In about 3% of cases of fetal or fetal and embryonal epithelial hepatoblastomas, areas containing broad trabeculae (6-12 or more cells in thickness) are present. These macrotrabeculae are composed of fetal and embryonal epithelial cells and a third, larger cell type characterized by more abundant cytoplasm and larger nuclei. Although the trabeculae resemble those seen in the pseudoglandular type of hepatocellular carcinoma, the cells display only mild hyperchromasia and anisocytosis, and mitotic activity is low. The term 'macrotrabecular' is applied to only those cases in which macrotrabecu-lae are a prominent feature of the lesion. If only an isolated focus is present, the

Table 8.04

Clinical syndromes, congenital malformations and other conditions that have been associated with hepatoblastoma.

Absence of left adrenal gland Acardia syndrome Alcohol embryopathy Beckwith-Wiedemann syndrome Beckwith-Wiedemann syndrome with opsoclonus, myoclonus Bilateral talipes Budd-Chiari syndrome

Cleft palate, macroglossia, dysplasia of ear lobes

Cystothioninuria

Down syndrome, malrotation of colon, Meckel diverticulum, pectum excavatum, intrathoracic kidney, single coronary artery

Duplicated ureters

Fetal hydrops

Gardner syndrome

Goldenhar syndrome - oculoauriculovertebral dysplasia, absence of portal vein

Hemihypertrophy

Heterotopic lung tissue

Heterozygous a1-antitrypsin deficiency

HIV or HBV infection

Horseshoe kidney

Hypoglycemia

Inguinal hernia

Isosexual precocity

Maternal clomiphene citrate and Pergonal

Meckel diverticulum

Oral contraceptive, mother

Oral contraceptive, patient

Osteoporosis

Persistent ductus arteriosus Polyposis coli families Prader-Willi syndrome Renal dysplasia

Right-sided diaphragmatic hernia Schinzel-Geidion syndrome Synchronous Wilms tumour Trisomy 18

Type 1a glycogen storage disease Umbilical hernia Very low birth weight

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