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Biomedical subjects

H Oliva

Publications and source records attributed to H Oliva.

At least 73 records · Page 4Linked to original sources

Porphyria cutanea tarda and hepatocellular carcinoma. Frequency of occurrence and related factors.

In order to assess the incidence of hepatocellular carcinoma (HCC) in porphyria cutanea tarda (PCT), 83 patients (77 males, 6 females, mean age 57.4 years) were studied. Thirteen patients (15.7%) had HCC, all of whom were male and cirrhotics with a mean age of 58.5 years. HCC patients showed a statistically significant (P less than 0.0005) longer evolution time (23 years since onset of the cutaneous disease) than patients without HCC (9.4 years), while the age of onset was similar in both groups. Differences in alcohol intake and hepatitis B virus (HBV) markers were non-significant, although high prevalence (54%) of past HBV infection was found in both groups. In HCC development, attributable risks of 100% were found for cirrhosis (P less than 0.001), male sex (P = NS) and for age over 51 (P less than 0.025). Therefore, PCT harbours a high incidence of HCC; evolution time, cirrhosis and age over 51 appear to be the most important contributing factors.

Adult↗

The pathology of the liver in porphyria cutanea tarda.

We report the findings in 53 biopsies from 45 patients with porphyria cutanea tarda (PCT). Red autofluorescence and birefringent acicular cytoplasmic inclusions were constant findings in all untreated cases. Autofluorescence occurs in other hepatic porphyrias, but acicular inclusions appear to be specific for PCT; we have seen them in subclinical porphyria and before development of cutaneous symptoms. They are probably uroporphyrins and they trend to disappear during rinsing by water during most staining procedures. We recommend unstained paraffin sections for their demonstration. Liver damage in PCT has features distinct from other liver diseases, including alcoholic liver disease. These include constant but mild periportal siderosis, focal lipofuscin deposition, focal lobular hepatocyte necrosis associated with groups of pigment-laden macrophages, focal steatosis, marked hepatocyte hyperplasia and the presence of periductal lymphocyte aggregates. The latter have not been previously described in PCT and were present in 43% of our cases. There is a direct relationship between increasing age and progressive distortion of liver architecture, with fibrosis present at a mean age of 48 years, cirrhosis at 57 and hepatocellular carcinoma at 66. The characteristic liver histology and the natural history of PCT are against this being the result of any non-specific liver damage and favour instead a specific liver disease whose pathogenesis may be mainly the result of the metabolic defect of PCT.

Adult↗

Vascular leiomyosarcoma: the malignant counterpart of vascular leiomyoma.

The clinical and pathological findings of six cases of leiomyosarcoma arising from blood vessels of different caliber are described. The term vascular leiomyosarcoma, having both a topographic and morphologic significance, is proposed for these tumors. The histologic pattern is characterized by a proliferation of atypical smooth muscle cells with a large number of intermingled blood vessels. Mitoses were counted per 10 high power field (hpf) and tumors were divided in three groups I, 10 to 20 mitoses, group II, 20 to 35 mitoses, and group III, more than 35 mitoses per 10 hpf. The mitotic index seems to be the most important pathological feature on which a prognostic evaluation for vascular leiomyosarcoma can be based. Tumors in group I had neither local recurrences nor metastases; the one tumor in group II had one local recurrence, but the patient is free of disease 6 years after surgical treatment; the three tumors in group III developed distant metastases and constitutional symptoms. Vascular leiomyoma, bizarre leiomyoma, and hemangiopericytoma are included in the differential diagnosis of vascular leimyosarcoma. The possibility that vascular leiomyosarcoma arising from small vessels represents the malignant counterpart of vascular leiomyoma is proposed.

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