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Ovarian Cyst Miracle

Ovarian Cyst Miracle Handbook By Carol Foster

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Fig. 8.46 Bile duct cystadenoma. A Large peribiliary cysts in the connective tissue of the hilus; the background liver shows advanced cirrhosis. B Variably sized cysts are intermingled with peribiliary glands.

Fig. 8.46 Bile duct cystadenoma. A Large peribiliary cysts in the connective tissue of the hilus; the background liver shows advanced cirrhosis. B Variably sized cysts are intermingled with peribiliary glands.

thogranulomatous reaction, with foam cells, cholesterol clefts and pigmented lipofuscin-containing macrophages, may be present in the cyst wall. The serous type consists of multiple, small locules lined by a single layer of cuboidal cells with clear cytoplasm containing glyco-gen. The cells rest on a basement membrane but are not surrounded by the mesenchymal stroma typical of the muci-nous variety. Squamous metaplasia may also occur.

Cystadenocarcinomas are usually multi-locular and contain mucoid fluid. Malignant change may not involve all of the epithelium lining the cyst; it is usually mul-tifocal. The tumours are so well defined that complete removal can usually be achieved with good prognosis. Differentiation from intrahepatic bile duct cystade-noma depends on the demonstration of cytological (particularly nuclear) atypia, mitosis, and invasion of the underlying stroma.

Some bile duct cystadenocarcinomas may be misdiagnosed as bile duct cys-tadenomas because insufficient sampling results in tumour morphology showing no cytological features of malignancy or invasion of the underlying stro-ma {351, 809, 1268, 2096}.

Prognostic factors

The prognosis of patients with biliary duct cystadenocarcinomas is good if a curative resection is possible. The

Fig. 8.47 Bile duct cystadenocarcinoma. Papillary folding with serous and mucinous neoplastic epithelium.

course of patients with unresectable tumours seems to be better than of patients with cholangiocarcinoma {71}.

Hepatoblastoma

J.T. Stocker D. Schmidt

Definition

A malignant embryonal tumour with divergent patterns of differentiation, ranging from cells resembling fetal epithelial hepatocytes, to embryonal cells, and differentiated tissues including osteoid-like material, fibrous connective tissue and striated muscle fibers.

Epidemiology

Hepatoblastoma is the most frequent liver tumour in children. Four percent of hepatoblastomas are present at birth, 68% in the first two years of life and 90% by five years of age. Only 3% are seen in patients over 15 years of age. A recent increase in the incidence of tumours in infants with birth weights below 1500 grams has been reported {776, 777, 1899}. There is a male predominance of 1.5:1 to 2:1, but no racial predilection.

Localization

Hepatoblastomas occur as a single mass in 80% of cases, involving the right lobe in 57%, the left lobe in 15% and both lobes in 27% of patients {1838}. Multiple masses, seen in the other 20% of cases, may occur in either or both lobes.

Clinical features

Hepatoblastomas are often noted by a parent or physician as an enlarging abdomen in the infant that may be accompanied by weight loss or anorexia. Less frequently nausea, vomiting, and abdominal pain are present. Jaundice is seen in 5% of cases. Rarely, tumour cells may produce human chorionic gona-dotrophin, leading to precocious puberty with pubic hair, genital enlargement and deepening voice, noted most prominently in young boys.

Hepatoblastoma is accompanied by anemia in 70% of cases and by thrombocytosis in 50%, with platelet counts exceeding 800 x 109/L in nearly 30% of cases {1717}. Alpha fetoprotein (AFP) is elevated in about 90% of patients at the time of diagnosis. The levels of AFP parallel the course of the disease, falling to normal levels after complete removal of the tumour and rising with recurrence of the lesion. AFP levels may be normal or only slightly elevated with small cell undifferentiated hepatoblastoma. Caution must be taken in evaluating the levels of AFP in younger infants since the 'adult' level of AFP (< 25ng/mL) is not reached until approximately six months of age. Other laboratory abnormalities can include elevated levels of serum cholesterol, bilirubin, alkaline phosphatase, and aspartate aminotransferase {10}.

Imaging

Computed tomography (CT) shows single or multiple masses within the liver which in 50% of cases display calcifica-

Table 8.02

Age distribution of hepatic tumours in young patients. Data are from the Armed Forces Institute of Pathology (AFIP), Washington, DC (U.S.A.)

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