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Biomedical subjects

Luigi Maria Terracciano

Publications and source records attributed to Luigi Maria Terracciano.

5 recordsLinked to original sources

Chronic hepatitis C in childhood: an 18-year experience.

BACKGROUND: The long-term outcome of chronic hepatitis C (CHC) has not been well studied, both for untreated and interferon-treated children. The aim of this study was to evaluate the long-term outcome of disease in a large series of children with CHC. METHODS: Clinical, biochemical, virological, and histological features were evaluated in all children (age, 2-18 years) with CHC who did not have concomitant disease and who attended at our hospital's liver unit during the period of 1986-2004. RESULTS: One hundred twenty-five children with CHC were studied. All patients remained free of symptoms throughout the period of observation. On the basis of transaminase levels during the first year of positivity for antibodies to hepatitis C virus (HCV), children were divided into 2 groups: patients with hypertransaminasemia (100 patients, all of whom had detectable HCV RNA), and those with normal transaminases (25 patients; 16 had viremia and 9 did not have viremia). Sustained clearance of viremia was achieved in 38% of the patients treated with interferon, compared with 12% of untreated children (P<.05). A sustained response to therapy was obtained in 64.7% of children infected with an HCV genotype other than genotype 1 and in 24.2% of those infected with HCV genotype 1 (P<.05). Histological lesions were mild in all 64 patients who underwent liver biopsy. No linear correlation was found between duration of disease and progression of fibrosis. Examination of a follow-up liver biopsy specimen revealed cirrhosis only in 1 (4.7%) of 21 children. CONCLUSIONS: Children with CHC were symptom free and had a morphologically mild liver disease. Interferon therapy may be effective for patients infected with HCV genotypes other than genotype 1, whereas lower response rates are expected for HCV genotype 1-infected children. The real impact of therapy on long-term outcome remains to be established.

Adolescent↗

Hepatocyte paraffin 1 expression in human normal and neoplastic tissues: tissue microarray analysis on 3,940 tissue samples.

Hepatocyte paraffin 1 (Hep Par 1) is a monoclonal antibody developed from hepatic tissue from a failed liver allograft. Several studies have shown that Hep Par 1 is a useful marker to differentiate hepatocellular carcinoma (HCC) from other types of adenocarcinoma metastatic to the liver. The aim of our study was the systematic investigation of the epidemiology of Hep Par 1 expression in 3,940 tissue samples using the tissue microarray technique. Strong Hep Par 1 expression was found most frequently in 35 (73%) of 48 HCCs. In nonhepatic tumors, strong Hep Par 1 expression was detected in adenocarcinoma of the lung (2/50), gallbladder (3/31), pancreas (2/48), stomach (3/74), small intestine (1/11), adenoma of the colon with high-grade dysplasia (1/49), adrenal gland carcinoma (1/6), paraganglioma (1/9), and malignant melanoma (2/48). Our data suggest that Hep Par 1 is a highly specific marker for HCC, although several nonhepatic tumors occasionally can show some Hep Par 1 positivity.

Adenocarcinoma↗

Fibrolamellar hepatocellular carcinoma occurring 5 years after hepatocellular adenoma in a 14-year-old girl: a case report with comparative genomic hybridization analysis.

An unusual case of fibrolamellar carcinoma of the liver developed 5 years after removal of a hepatocellular adenoma in a 14-year-old girl belonging to a family with Carney syndrome. Both tumors were studied by light and electron microscopy, flow cytometry, and comparative genomic hybridization. The first tumor, removed at the age of 9, was a bulky well-circumscribed liver mass composed of large eosinophilic cells with a focal pseudoglandular pattern but without cytologic atypia or sclerosis. A diagnosis of hepatocellular adenoma was rendered. Five years later, another hepatic tumor was removed from the right lobe. Microscopic examination revealed polygonal cells, each with a large amount of eosinophilic cytoplasm and a round nucleus with a conspicuous nucleolus. These cells were arranged in nests and strands and separated by bands of dense fibrous tissue, leading to a diagnosis of fibrolamellar carcinoma. Comparative genomic hybridization analysis revealed no genetic alteration in the adenoma; however, several chromosomal aberrations (loss of chromosome regions 1p and 4p and gains of chromosome regions 6q, 13q, and Xq) were detected in the fibrolamellar carcinoma. To our knowledge, this is the first report of an association between hepatocellular adenoma and fibrolamellar carcinoma.

Adenoma, Liver Cell↗

Macrofollicular variant of papillary carcinoma of the thyroid: a histologic, cytologic, and immunohistochemical study of 3 cases and review of the literature.

CONTEXT: The macrofollicular variant of papillary carcinoma of the thyroid is a rare entity described by J. Albores-Saavedra and colleagues in 1991. It is characterized histologically by a predominance of macrofollicles and clinically by a low incidence of metastasis. This entity may represent a source of diagnostic error, since it can be easily misinterpreted as a macrofollicular adenoma or nodular goiter. DESIGN: In this study, we describe 3 cases of papillary carcinoma of the thyroid with a macrofollicular growth pattern and review the literature. RESULTS: The fine-needle aspiration biopsies in 2 cases showed large cells with optically clear nuclei and nuclear grooves, suggestive of papillary carcinoma of the thyroid. In one case, the cytology showed no signs of malignancy. In all cases, the tumors showed a combination of the conventional follicular variant of papillary carcinoma of the thyroid and macrofollicles (diameter, >250 microm) occupying more than 50% of the cross-sectional area. Cytologic features were large, cuboidal cells with optically clear, ground-glass nuclei with smooth outlines, a fine chromatin pattern, nuclear grooves, and pseudoinclusions. The colloid was dense and more eosinophilic than in adjacent normal follicles. In 2 cases, there was capsular or blood vessel infiltration, and one tumor had metastasized to a cervical lymph node. One tumor recurred 1 year later as an anaplastic carcinoma. Immunohistochemical staining showed a positivity of the tumor cells for cytokeratins 7, 17, and 19, thyroid transcription factor-1, and galectin-3 and a negativity for cytokeratin 20 and p53. CONCLUSIONS: Although it has been suggested that this tumor is a highly differentiated variant with a favorable prognosis, our study shows that its biologic behavior is not conclusive because metastases and recurrences with dedifferentiation may occur.

Adult↗

Comparative genomic hybridization analysis of hepatoblastoma reveals high frequency of X-chromosome gains and similarities between epithelial and stromal components.

Hepatoblastoma (HB) is the most common liver tumor in childhood and differs in its environmental risk factors and genetic background from hepatocellular carcinoma. HB is associated with inherited conditions such as familial adenomatous polyposis and Beckwith-Wiedemann syndrome, suggesting the importance of genetic abnormalities in the pathogenesis and progression of this disease. It has a very polymorphous morphology. A diverse range of cytogenetic alterations has been reported to date, the most frequent being trisomy 2 and trisomy 20. Thirty-five HB specimens from 31 patients (22 purely epithelial, 4 purely mesenchymal, 9 mixed) were examined by comparative genomic hybridization (CGH), a technique that enables us to screen the entire tumor genome for genetic losses and gains. Our aims were as follows: (1) to characterize chromosome abnormalities that appear in this tumor and (2) to identify possible differences between different histologic subtypes of HB. We found significant gains of genetic material, with very little difference in the number and type of alterations between the different histologic components of HB. The most frequent alterations were gains of Xp (15 cases, 43%) and Xq (21 cases, 60%). This finding was also confirmed by fluorescent in situ hybridization performed on nuclei extracted from 6 specimens. Other common alterations were 1p-, 2q+, 2q-, 4q-, and 4q+. We found no difference between different histologic subtypes, a finding that may be in agreement with the hypothesis of a common clonal origin for the different components. An hitherto-unreported high frequency of X chromosome gains may support the assumption that X-linked genes are involved in the development of this neoplasm.

Cell Nucleus↗