Hepatocellular carcinoma, syncytial giant cell: a novel variant in children: a case report.

Hepatocellular carcinoma (HCC) is the second most common primary malignant hepatic tumor in children. It often develops in patients with underlying liver disease. We report the clinicopathologic features of an unusual HCC occurring in an infant who presented with features of Cushing's syndrome due to bilateral adrenal hyperplasia. The tumor is characterized by epithelial syncytial giant cells. Giant cell carcinoma of the liver has been previously reported, but the cells were osteoclast-like (ie, mesenchymal type) and not epithelial type as it is in this patient. We propose to use the term HCC, syncytial giant cell type, to denote this apparently novel lesion.

A new autosomal recessive syndrome. Early onset of pancytopenia, distinct facial features, growth retardation and developmental delay.

The association of dysmorphic features and failure of one or more bone marrow cell lines is well known. Examples are Fanconi's anemia and Diamond-Blackfan anemia. This report describes 3 similarly affected children from consanguineous parents, all showing low birth weight, severe growth retardation, distinct facial features, microcephaly, mental retardation and onset of severe pancytopenia in infancy without increased chromosomal breakage. We conclude that these cases represent a new familial autosomal recessive bone marrow failure syndrome.

Isolated hepatic mucormycosis in an immunocompetent child.

A case of isolated localized hepatic mucormycosis in an immunocompetent 3 1/2-yr-old girl with concomitant acute toxoplasmosis is described. Mucormycosis is rare in immunocompetent patients, and hepatic mucormycosis has so far been described only in the context of disseminated disease. The infection resolved spontaneously without surgical debridement and/or appropriate medical therapy with amphotericen B.

Extramedullary blastic transformation in a child with adult chronic myelocytic leukemia.

We report a case of Philadelphia chromosome positive (Ph+) chronic myelocytic leukemia (CML) in a 4-year-old child presenting with a one-sided cervical chloroma (granulocytic sarcoma) of 5 months duration preceded by an inflammatory reaction in the same area. Blood and bone marrow were consistent with CML in chronic phase. Cytogenetic analysis of blood, bone marrow and chloroma showed, in addition to the classical Ph+ cell line, another clone with additional aberrations: 50,XY,+Y,+8,t(9;22)(q34;q11), +19,+21, present predominantly in the chloroma. In conclusion, this is the first report of a Ph+ CML in a young child with a chloroma as an isolated extramedullary localization of blastic transformation. It is hypothesized that local events such as inflammation might be inductive of extramedullary blastic transformation.

Cutaneous granuloma with ataxia telangiectasia--a case report and review of literature.

A patient is reported who had ataxia telangiectasia with multiple cutaneous lesions mainly on the limbs, which showed atrophy and scarring. Histopathology of these skin lesions showed tuberculoid granulomas without frank collagen necrobiosis in the dermis. Chromosomal analysis revealed a translocation between 7 and 14 as well as deletion of chromosomes 4 and 6.

Causes of death in a paediatric oncology unit.

The records of 101 patients (64 males and 37 females) registered at Bristol Children's Hospital who died between January 1986 and December 1989 were reviewed to determine the cause of death. Nineteen patients (19%) died without obtaining remission and 6 (6%) in first remission. Seventy-six (75%) died after relapse; three during re-induction and two in second remission. The causes of death were active disease in 85 patients (84%), active disease and infection (4%), active disease and other factors (4%), infection only (3%), toxic cardiomyopathy (2%), graft versus host disease (2%), and second malignancy (1%).