Nephroblastoma in infants, 1969-75: variations in treatment and survival.

In a series of 79 infants aged under 1 year with nephroblastoma diagnosed during 1969-75 all the patients underwent nephrectomy, 33 (42%) received a course of radiotherapy, and 49 (62%) received chemotherapy. The overall three-year survival rate for patients who survived at least one week after diagnosis was 65%. The corresponding rate for infants with stage I tumours was 76%. The survival rate in children with early-stage tumours was significantly higher in those who were treated by nephrectomy and chemotherapy alone compared with those who also received radiotherapy. In a large proportion of cases nephrectomy and chemotherapy together constituted sufficient treatment for the cure of infants with nephroblastoma, and in some instances nephrectomy alone proved adequate. There was no general tendency for children under 1 year old to be unable to withstand chemotherapy.

Childhood medulloblastoma in Britain 1971-77: analysis of treatment and survival.

In a population-based series of 368 children undergoing surgery for medulloblastoma, 304 (83%) survived to complete a course of radiotherapy. Among those patients who completed radiotherapy, the short-term survival rates were lower for young children (those aged under 5 years) than for older children, but by 6 years the survival rates were very similar (approximately 35%) for children in both age groups. Higher survival rates were obtained in the young children where total macroscopic excision of the tumour was achieved. For older children there was no difference in survival rates between those with total or partial excision, though the survival rate was lower for those whose surgery was limited to biopsy. In young children radiotherapy dose had no effect on survival rates. In older children, survival rates were appreciably higher where doses had been at least 45 Gy to the posterior fossa and 30 Gy to the spinal cord, and there were also fewer spinal cord metastases among those who received a higher spinal cord dose. Ninety (30%) of the 304 children also received chemotherapy as part of their initially planned treatment; a wide variety of protocols was used and no conclusions could be drawn as to the effects on survival rates.

Retinoblastoma: a study of natural history and prognosis of 268 cases.

The natural history and prognosis of retinoblastoma were analysed using data relating to the 268 cases registered during 1962-8 in England, Scotland, and Wales. The children were followed up for a minimum of four years; the proportion surviving for four years was 86%. The most important factors affecting survival rate were the stage of the tumour at diagnosis and the hospital of treatment. Of children surviving for three years after treatment only three died during the subsequent period of follow-up, which varied from one to seven years. Among children with retinoblastoma treated between 1949 and 1968 nine died between seven and 13 years later of other cancers: seven from osteosarcomas, one from angiosarcoma, and one from fibrosarcoma.

Group characteristics of children with cerebral and spinal cord tumours.

A study of 2072 children who developed cerebral or spinal cord tumours of varying degrees of malignancy before 15 years of age has shown that there is equally good representation of fatal and non-fatal cases in official registrations. Attack rates are higher for boys than girls and the prognosis is better for girls than boys. The risk of an early death is negatively correlated with age at diagnosis, and the risk of a late death shows the opposite relationship. These observations and a relatively high incidence of hindbrain tumours are suggestive of an embryonic origin for most of the cases.

Incidence of cardiac septal defects in children with Wilms' tumour and other malignant diseases.

In a population-based series of 8045 children with malignant neoplasms in Great Britain, the incidence of septal defects was 0.40%. The high rate of 19% in Down's syndrome children and the overall rate of 0.28% in children without Down's syndrome were both comparable with rates found in previous large prospective studies. The incidence of septal defects in children with Wilms' tumour was 1.82%, greater than 10 times that for non-Down's children with other neoplasms. The presence of this association in Wilms' tumour patients with and without aniridia and the recent finding of a loss of heterozygosity in chromosome 11 in many Wilms' tumours taken from patients without aniridia suggest the possibility of a link between Wilms' tumour, some septal defects and chromosomal abnormalities.

Carcinomas in childhood. A registry-based study of incidence and survival.

During a 10 year period, 1971-80, there were 234 children in Great Britain younger than 15 years with a diagnosis of carcinoma registered in Great Britain. These cases represented approximately 2% of all childhood malignant disease. The most common primary site was the thyroid, followed by the nasopharynx and the adrenal cortex. With the exception of adrenocortical tumors, most of the carcinomas occurred in children older than 10 years. In some patients there was a genetic predisposition to neoplasia. Children with carcinomas of the thyroid and female genital tract have an excellent prognosis with 5-year survival rates of over 90%. The prognosis for other sites was generally less favorable; 60% of children with nasopharyngeal tumors were alive at 5 years from diagnosis but less than 20% of those with carcinomas of the gastrointestinal tract or the adrenal cortex.

Bone metastases in childhood renal tumours.

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.

Patterns of childhood cancer among siblings.

The National Registry of Childhood Tumours contains over 51000 records of children born in Great Britain who developed cancer under the age of 15 years. Patterns of childhood cancer among families containing more than one child with cancer have been studied. A total of 225 "sib pair' families have been ascertained from interviews with parents of affected children, from hospital and general practitioner records and from manual and computer searches of names and addresses of patients. A number of special groups have been identified, including those with a known genetic aetiology such as retinoblastoma, twins and families with three or more affected children. A further 148 families not in any of the above groups contain two children with cancer: in 46 families the children had tumours of the same type, most commonly leukaemia. Some of the families are examples of the Li-Fraumeni syndrome; some are associated with other conditions, including Down's syndrome. There is clearly a genetic element in the aetiology of cancer in some families discussed here; shared exposure to environmental causes may account for others and some will be simply due to chance.

Nephroblastoma: treatment during 1970-3 and the effect on survival of inclusion in the first MRC trial.

In 1970-3 313 children were diagnosed as having nephroblastoma in Great Britain. From the start of the first Medical Research Council nephroblastoma study in October 1970 until the end of 1973, 98 children (57% of all eligible children) were included in the trial. Of the 313 children, 288 (92%) had a nephrectomy, 248 (79%) received a course of radiotherapy, and 267 (85%) were given at least four days' chemotherapy. The three-year survival rate was 58%; the rate among children in the trial (77%) was significantly better than that among children who were eligible for the trial but not included (58%). Children who had nephrectomies at specialised children's and teaching hospitals had a higher survival rate than those treated elsewhere. All children with nephroblastoma should be treated according to well-defined protocols which take into account the age of the child and the stage of the tumour and include a full course of maintenance chemotherapy.