Biomedical subjects
Publications and source records attributed to H B Marsden.
A 2 1/2-year-old boy presented with an illness resembling glandular fever. He was found to have T-cell deficiency with lack of red and white cell purine nucleoside phosphorylase enzyme activity. A spastic tetraparesis was noted. The patient subsequently died of a malignant lymphoma of the B-immunoblastic type.
One hundred and thirty-seven germ cell tumours occurring in 134 children included in the Manchester University Children's Tumour Registry over a 25-year period have been studied. The incidence and sites of these tumours as seen in a comprehensive population-based registry are presented and the relation between the primary site and histological type is assessed. There were 48 deaths in this series of which 34 were directly due to the tumour. The cause of death in the other 14 cases is given. The behaviour of the germ cell tumours was closely related to the presence of yolk sac elements.
The histopathological spectrum of a large series of a recently described tumour entity is presented. Seven diagnostic features which may be encountered are described and their frequency discussed. The most striking clinical feature was the marked male preponderance (M:F = 7.6:1). It is suggested that an appreciation of the full histopathological spectrum is necessary to ensure adequate diagnosis.
Analysis of data from 1434 children with primary renal tumours revealed 57 who developed bone metastases. Of these, 54 were initially recorded as nephroblastoma. Fifty-two of the 57 cases were reviewed histologically, and only 18 were found to be Wilms' tumours. Twenty-three were classified as "Bone-Metastasizing Renal Tumour of Childhood" (BMRTC), and a high male incidence was found for these tumours (M:F=6.7:1). Differences in the pattern of metastasis and the one-year survival between BMRTC and nephroblastoma are discussed. The rarity of bone metastases from true Wilms' tumours is emphasized.
The Manchester Children's Tumour Registry data for the period 1954-1977 have been analysed. The overall incidence of malignant disease in children aged 0-14 years in the north-west of England is estimated to be 100 per million person-years. The most common disease group is leukaemia, which forms about one third of the total number of cases. Among solid tumours, by far the most common presenting site is the central nervous system, representing nearly a quarter of all neoplasms. Wilms' tumour, neuroblastoma and soft-tissue sarcomas comprise approximately 5%, 6.5% and 6% respectively of the total. The tumours most frequently seen in adults (e.g. carcinoma of colon, lung and breast) are extremely rare in childhood. A significant excess of males was seen in acute lymphoid leukaemia, non-Hodgkin's lymphoma, Hodgkin's disease, medulloblastoma and hepatoblastoma. A female excess was found among germ-cell tumours. During the study period significant increases in incidence were seen among acute lymphoid leukaemia and epithelial tumours, and an increase in germ cell tumours approached significance.
Fifty-six primary childhood renal tumours, 14 normal and 12 fetal kidneys were examined for their staining reaction with xenogeneic anti-Wilms' antiserum. The antiserum was raised by injecting Wilms' tumour extracts into 6-month-old rabbits which had been rendered tolerant in utero with pooled normal kidney extracts. Renal carcinomas, mesoblastic nephroma and a large proportion of tubular wilms' tumours were stained by the antiserum. In contrast, 6 of 7 bone-metastasising renal tumours of childhood (BMRTC) failed to fluoresce when treated with the antiserum, suggesting that the BMRTC has a different origin from the other childhood renal tumours studied.
In a postmortem survey of children with leukaemia and lymphoma, the overall incidence of testicular infiltration was 65% and of ovarian infiltration 66%. In acute lymphoblastic leukaemia, testicular infiltration was found in 25 of 39 cases and ovarian infiltration in 6 of 12 cases. The majority of boys with acute lymphoblastic leukaemia and testicular infiltration also had infiltration in other organs; furthermore, in most cases without testicular infiltration leukaemic infiltration was found in other organs. The findings of this survey support the view that testicular infiltration is indicative of widespread disease.
A transmission electron microscope study was made of eight childhood brain tumors divided up into three zones, center, edge, infiltrating zone, and also of adjacent "normal-looking" brain. In seven of eight tumors, the numbers of Weibel-Palade bodies in endothelial cells were significantly increased in peripheral zones compared with central zones. A similar significant increase was observed after treatment of chick chorioallantoic membranes with tumor angiogenesis factor. It is suggested that large numbers of Weibel-Palade bodies may be a marker for proliferating endothelial cells in vivo.
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Two neck tumours in young children with similar clinical features were compared using light and electron microscopy. Both tumours consisted of uniform sheets of epithelial-like cells and there were scattered stromal elements in one. Tonofilaments in sheets or bundles reminiscent of squamous epithelium were striking in the epithelial cells. Intercellular junctions were frequent but true desmosomes were not seen. Most cells contained a few secretory droplets. Occasional secretory cells, foam cells and cilia were observed. Since these tumours were not sited superficially they are unlikely to have been derived from skin epithelium. An origin in branchial sinus remnants capable of differentiation towards squamous and ciliated epithelia is suggested.
A survey of bone metastases in 132 children presenting with renal tumours has been carried out using material from the Manchester (England) Children's Tumour Registry during the period 1954 to 1976. Seven such cases were found. Only one of the 117 Wilms' tumours developed bone metastases. The remainder included carcinoma (1/4), 'bone metastasising renal tumour of childhood' (2/3), atypical tumours (2/2), and lymphosarcoma (1/1). Bone metastases from true nephroblastoma seem to be an exceptional finding.
A case of cytomegalic congenital adrenal hypoplasia (C.C.A.H.) is reported with similar cytomegalic changes in the hypophysis. In this case the pituitary gland was small as were the thyroid and testes. The various groups of patients with C.A.H. are presented. Associated pituitary cytomegaly does not appear to have been previously described, and this association between the adrenal and pituitary changes is discussed. It is suggested that the adrenal cytomegaly is the result of overstimulation by the pituitary and the similar change in the latter may be the result of overstimulation of a hypoplastic organ.
A case is reported of a child with fatal pulmonary fibrosis following BCNU therapy. This side effect has only once previously been reported with the nitrosourea group of compounds.
A bone metastasizing primary renal tumor of childhood is described, and five cases are presented. Although all five cases were originally diagnosed as nephroblastomata, there appear to be sufficient histological, ultrastructural and clinical differences to suggest that these tumors should comprise a separate and distinct entity. Using the data from the Manchester Children's Tumor Registry, the incidence of this tumor was found to be 2.3% of all primary renal neoplasms in childhood.
A primary renal tumour of childhood with histological appearances different from the nephroblastoma is described. This neoplasm, predominantly seen in boys, has a tendency to metastasize to bone. Such metastases are considered to be rare in nephroblastoma and this aspect in studies of Wilms' tumour series is thought to be due, for the most part, to the inclusion of a particular bone-metastasizing tumour in the material.
Three cases are reported in which thrombi were found in the aorta at postmortem. In each case the thrombus had originated within the ductus arteriosus. We believe that in recent years the attention paid to the presence of indwelling cannulae as a cause of embolic phenomena in the newborn period has led to a reduction in emphasis on the importance of the ductus in this field. We think that the ductus should still be regarded as an important source of some emboli.
A 5-year-old with adrenocortical carcinoma presented with acute paraplegia. The tumour was initially nonfunctioning but finally showed rapid dissemination and the patient then developed Cushingoid features and virilisation.
A prospective study of bone marrow fibrosis was made in a group of 40 children with acute lymphoblastic leukaemia to see whether it affected the prognosis or course of the disease. Secondary myelofibrosis (SMF) was present at diagnosis in 57% of the cases. It was not statistically significantly related to the prognosis or course of the disease. Thus, although trephine biopsy occasionally provided useful information in different diagnosis and when aspiration was difficult, it provided little information of use for management.