Henry Basil Marsden

Bone metastasizing renal tumor of childhood: morphological and clinical features, and differences from Wilms' tumor.

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 Childrens Tumor Registry, the incidence of this tumor was found to be 2.3% of all primary renal neoplasms in childhood. Cancer 42:1922–1928, 1978.

Histopathological study of the first Medical Research Council Nephroblastoma Trial.

A histological analysis of the tumors from the first Medical Research Council (M.R.C.) Nephroblastoma Trial was carried out. Material from 104 out of the 111 included cases was suitable for assessment. The degree of tubular differentiation and the presence or absence of glomeruli, cysts and papillary structures were correlated with clinical stage and survival. A significant association was found with regard to tubular differentiation. Papillary structures appeared to be important, but the number of cases was small. The presence of either glomeruli or cysts was not a statistically significant feature as regards prognosis.

Methods of classifying and ascertaining children's tumours.

Several methods of ascertaining and classifying childhood neoplasms for epidemiological study have been evaluated using material from the University of Manchester Childrens Tumour Registry (CTR), which includes data from several sources on children with neoplasms first seen in the period 1954-73 who were under 15 years old and living in the Manchester Regional Hospital Board area at the time. Two systems of classification-the International Classification of Diseases (ICD) and the Morphology Section of the Manual of Tumor Nomenclature and Coding (MOTNAC; Percy, Berg and Thomas, 1968)-were tested. No major problems arose with the Morphology Section of MOTNAC, and we recommend that the revised version of this section, in the proposed International Classification of Diseases for Oncology, should be used in epidemiological reports on childrens tumours whenever possible. The ICD discriminates less well between the commoner types of childhood neoplasms, but must be retained as a supplementary classification to facilitate international comparisons. A comparison of the completeness of ascertainment achieved in recent years by each source of data showed that more than 98% of the serious cases (neoplasms that were malignant and/or lay within the craniovertebral canal) could have been identified using a combination of Hospital Activity Analysis (HAA) and cancer registration records, and more than 95% using HAA and death records. But in an analysis of 2 years HAA returns and 6 years cancer registrations of serious cases, nearly one quarter of the former and one fifth of the latter were shown to record diagnoses which differed from those finally assigned at the CTR. It is concluded that, in epedimiological studies based on routine records, the diagnoses given should always be checked centrally, by experts, in the light of all the available clinical and pathological material (including histological preparations).

Problems of Children’s Tumours in Britain

With the steady improvement in the prevention and treatment of bacterial disease during the past 30 years, their importance as a cause of death in children has greatly diminished. During the same period the practical benefits resulting from cancer research have not been so striking and consequently neoplasms have assumed a relatively greater importance in paediatrics. In 19 70 they were the second commonest cause of death between the ages of 1 and 15 years in England and Wales (Registrar-General), being exceeded only by accidents (Table 1).

Tumours of the Sympathetic System

The sympathetic nervous system is the fourth commonest site of tumour formation. Since this system consists of cells which are scattered over a wide area, sympathetic tumours could theoretically start almost anywhere, but in practice they usually arise where there are concentrations of sympathetic cells in or beside the vertebral column or near the kidneys.

Connective Tissue Tumours

One hundred and ninety-one children with connective tissue tumours were included in the Registry between 1954 and 1968. This figure certainly is less than the actual number of cases in the Region during this period since many children with these tumours are not treated by paediatricians or radiotherapists and therefore are less likely to be reported to the Registry. Although connective tissue tumours are an important group in children there has been considerable confusion about their clinical management and classification. There are two reasons for this: n na) n nThe children initially often come under the care of a variety of specialists (such as ophthalmologists, otologists, orthopaedic or genitourinary surgeons) because the tumours arise in situations such as the orbit, ear, bones or bladder. Consequently there is little uniformity of treatment and clinical trials can rarely be organised.

Intracranial and Spinal Tumours

The central nervous system is the second commonest site of tumour formation in children. In contrast with lethal neoplasms elsewhere, those in the central nervous system very rarely spread to other organs but are dangerous because they invade and destroy adjoining vital centres. Thus the prognosis depends as much on site as on histological type. The fact that in children these tumours often arise close to the midline structures at the base of the brain, limits the extent of local treatment which can be given safely.

Non-Leukaemic Reticuloendothelial Tumours and Leucosarcomas

During the past 10 years there has been a great change in the clinical management of patients with non-leukaemic reticuloendothelial tumours. Three factors have contributed to this: firstly, the greater use of chemotherapy; secondly, greater precision in assessing the extent of the disease so that both local and general treatment can be planned more rationally and, thirdly, the classification of these tumours in histological subgroups which reflect more closely their natural history.

Epithelial and Other Rare Tumours

Since epithelial tumours are much less common in children than in adults, individual experience of their behaviour and management is difficult to obtain. Although a wide range of these neoplasms occurs, the number of each type included in the Registry is insufficient to provide a satisfactory picture either of their true incidence or their natural histories. This also applies to other rare non-epithelial tumours and therefore it is proposed to discuss both these groups in this chapter. Craniopharyngiomas, pituitary adenomas, pinealomas, and meningeal melanomas which are considered in Chapter 6 are not included in this section.

Ewing’s Tumours

JAMES EWING (1922) selected a small-celled non-osteogenic tumour of bone with a number of clinical features, including slow growth rate, and classified it as an endothelial myeloma or diffuse endothelioma of bone. All the patients in his series were under 20 years old and in 7 of the 10 cases the long bones of limbs were affected. PARKER and JACKSON (1939) described a somewhat similar tumour occurring mainly in adults, called it reticulum cell sarcoma of bone, and noted that, in contrast with EWING’s cases, it appeared to have a good prognosis.