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J V Bond

Publications and source records attributed to J V Bond.

14 recordsLinked to original sources

Wilm's tumour, hypospadias, and cryptorchidism in twins.

Twin boys, both of whom had hypospadias and bilateral cryptorchidism, each developed a left-sided Wilms's tumour. The first twin was found to have an advanced multifocal tumour at the age of 15 months and died with local recurrence and pulmonary metastases. The diagnosis was made in the second twin one month later and at nephrectomy the tumour was found to be encapsulated without metastases; he is disease-free 12-years later. Although the histological appearances were similar in each twin, the tumours did not develop at the same rate and did not show the 'mirror-image' pattern suggested for embryonal tumours in identical twins.

Cryptorchidism

Pulmonary metastases in Wilms' tumour.

In a series of 87 patients with a diagnosis of Wilms' tumour seen at the Great Ormond Street Hospital for Sick Children between 1960 and 1973, 15 patients had pulmonary metastases at the time of diagnosis and a further 22 patients developed pulmonary metastases within 18 months of their initial nephrectomy. The incidence of later development of metastases was very much greater in those patients with locally advanced primary tumours than in those with a completely resectable, encapsulated tumour. In both groups of patients the disease-free survival rate was closely related to initial local tumour staging. In the whole series there were 11/37 (42-5%) patients with pulmonary metastases who survived disease-free for two or more years following treatment. The survival rate was highest in those patients who had a solitary pulmonary metastasis which was surgically resected (5/6); this type of lesion was seen only in those patients who had a localised primary tumour. In those patients with multiple pulmonary metastases, treated with irradiation to the whole lungs and single-agent chemotherapy, there were only 6/31 disease-free survivors; this group of patients all had advanced primary tumours and there was a high incidence of associated abdominal recurrence. The need for multiple agent chemotherapy in conjunctin with surgery and radiotherapy, both as prophylaxis against and in the treatment of metastases from Wilms' tumour is stressed.

Adolescent

Neuroblastoma metastatic to the liver in infants.

Four infants are described who presented with rapid enlargement of the liver. This was found to be due to neuroblastoma which had metastasized to the liver; the condition was associated with high levels of urinary vanillylmandelic acid (VMA). In 3 infants the primary tumour was in the adrenal gland and in one it was not identified. One infant died after laparotomy and 2 infants survive disease free with normal VMA levels, one after adrenalectomy and hepatic irradiation and one after a short course of chemotherapy. The fourth patient responded initially to hepatic irradiation and chemotherapy, but relapsed 2 years later with recurrent disease; at the same time the VMA level which had been normal, again rose. The importance of screening for an abnormal VMA level in any infant with a rapidly enlarging liver in order to obtain an early diagnosis is stressed. Careful follow-up, with serial VMA estimations, is essential to detect recurrent disease. The prognosis for some infants with this distribution of neuroblastoma which has metastasized to the liver, but not to the bones or oribt, is good.

Adrenal Gland Neoplasms

Wilms' tumour.

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Child

Bilateral Wilms' tumour. Age at diagnosis, associated congenital anormalies, and possible pattern of inheritance.

A series of 87 patients with Wilms' tumour seen during the period 1960-73 included 11 (13%) with bilateral tumours. 6 patients presented with simultaneous bilateral tumours, 2 had tumours in each side of a horseshoe kidney, and 3 later developed a tumour in the remaining kidney. There was no reported familial incidence of Wilms' tumour. Maternal age at birth of the patients with simultaneous bilateral tumours was over 30 years in 7/8 cases. The average of patients with bilateral tumours was 15 months, whereas that of patients with unilateral tumours was 31/2 years. All the simultaneously occurring bilateral tumours and those within a horseshoe kidney were multifocal, whilst the sequentially occurring bilateral tumours and the unilateral tumours all developed as a single tumour mass within the affected kidney. Associated congenital anomalies were found in 5 (45%) of 11 patients with bilateral tumours, several of whom had more than one defect. Of 76 patients with a unilateral tumour, only 3 (4%) had congenital anomalies.

Age Factors

Prognosis and treatment of Wilms' tumor at Great Ormond Street Hospital for Sick Children--1960-1972.

Eighty-one children with a diagnosis of Wilms' tumor who were treated at the Great Ormond Street Hospital for Sick Children between 1960 and 1972 are reviewed. The National Wilms' Tumor Study group staging system is used. Distribution by age and sex in each stage together with survival rate is given. Treatment and prognostic factors are discussed. Wilms' tumor is most common in children below the age of 3 years. There was a survival rate of 87% in Stage I, 36% in Stage II, 8% in Stage III, and 33% in Stage IV. The survival was worse in patients with locally advanced tumor than in patients who had pulmonary metastases in association with a locally resectable tumor. Whole abdominal irradiation is necessary in all patients in whom the tumor has breached the renal capsule. Single agent chemotherapy has not proved effective in preventing relapse in patients with advanced tumors. More intensive therapy is needed in this group to prevent both local recurrence and distant metastases. The addition of multiple drug chemotherapy to nephrectomy and whole abdominal irradiation is already improving the disease-free survival.

Adolescent

Bone metastases in Wilms' tumour.

In a series of 81 patients with a diagnosis of Wilms' tumour seen at the Hospital for Sick Children, Great Ormond Street, between 1960-1972, two patients developed histologically proven bone metastases. In one patient a lump on the head was the presenting symptom of his Wilms' tumour. In the second patient an isolated metastasis developed in the iliac bone 2 3/4 years after complete removal of an encapsulated Wilms' tumour. The bone lesion was outside the previously irradiated volume. Response to treatment was poor and both patients died with further widespread disease. A review of other series confirms the low incidence of bone metastases from Wilms' tumour and the poor prognosis of this complication. This contrasts with a high incidence of pulmonary metastases in which the response to treatment and subsequent survival is good. The value of skeletal surveys and scans in detecting this relatively rare site of metastases from Wilms' tumour is discussed.

Bone Neoplasms

Clinical significance of catecholamine excretion levels in diagnosis and treatment of neuroblastoma.

Bond, J.V. (1975). Archives of Disease in Childhood, 50, 691. Clinical significance of catecholamine excretion levels in diagnosis and treatment of neuroblastoma. The pattern of urinary excretion of vanillylmandelic acid (VMA) was studied in 50 children with neuroblastoma. There were 10 disease-free survivors, and 4 children who survive for 3 or more years with residual nonmetastatic disease, 36 children died with widespread metastic disease. The urinary VMA level was raised at diagnosis in 8 of the 10 disease-free survivors but rapidly returned to normal after treatment. In 3 out of 4 children with residual tumour, VMA remains persistently raised. In 28 of the 36 children who died VMA was raised at diagnosis and remained so throughout the course of their disease. In the majority of the children with metastatic disease the finding of a raised VMA revealed the nature of the primary tumour. Measurement of urinary VMA is a simple diagnostic test which confirms the presence of neurblastoma and avoids the need for more complex investigations. The prompt return to normal levels in the disease-free survivors confirms the importance of serial VMA estimations in assessing response to treatment and ultimate prognosis. The other good prognostic features in the surviving patients were young age at diagnosis, primary tumour in the thorax, and histologically well-differentiated tumours which had not metastasized.

Age Factors