Biomedical subjects
Publications and source records attributed to R D Spicer.
We describe two cases of rhabdomyosarcoma with a translocation involving 12q13 as the primary cytogenetic abnormality. Literature review of 35 cases has identified 3 other cases with this abnormality, and we speculate that this may be another nonrandom rearrangement in rhabdomyosarcoma.
We report a case of fibrosarcoma, which presented in a two week old boy. Excision was not performed because it would have required mutilating surgery. The tumour regressed and was impalpable by 7 months of age. The patient is tumour free at 4 years of age. This is the first reported case of spontaneous regression of a fibrosarcoma. The literature is reviewed. We conclude that the chances of metastasis are low, tumours are likely to respond to chemotherapy, and mutilating surgery is not appropriate initial treatment for infantile fibrosarcomas.
Thirty-five different soft tissue tumours occurring in the first month of life are described and classified into five Clinical Groups. A. Excellent prognosis with no treatment or simple surgical excision. B. Good prognosis. Treatment depends upon anatomical site. C. Good prognosis. Treatment usually surgical but chemotherapy may be indicated in certain situations. D. Intermediate prognosis. Treatment as for older child, usually surgery or chemotherapy. E. Poor prognosis. Treatment palliative or experimental. The relatively good prognosis of tumours in this age group and the importance of avoiding over-treatment are emphasised. Treatment guidelines are given and the value of obtaining tissue for biological studies to improve our understanding of these rare tumours is stressed.
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The haemolytic uraemic syndrome (HUS) is a well recognized cause of acute renal failure in children. Gastrointestinal involvement is common, and may result in rectal bleeding which can be an important presenting symptom. Previous publications have stressed the importance of correct diagnosis to avoid unnecessary surgery. Occasionally serious gastrointestinal complications do occur. We present five children with life-threatening gastrointestinal complications of the HUS and discuss the indications for laparotomy.
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A case of proliferative fascitis in the forearm of a 7-year-old child is presented. The lesion is composed of spindle cells and large bizarre ganglionlike cells in a collagenous matrix with some myxoid areas. The cells contain intracytoplasmic inclusions of collagen. The cytoplasm stains for vimentin, and the cells have ultrastructural features of myofibroblasts. This is the second case reported of proliferative fasciitis occurring in a child, and the importance of distinguishing this lesion from childhood neoplasms with a similar appearance is discussed.
Two infants presenting with vomiting were thought to have palpable pyloric tumours. At operation hypertrophic pyloric stenosis was not present, but other conditions causing duodenal obstruction were, and these required surgical treatment. The cases illustrate that other abnormalities may masquerade as pyloric stenosis.
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Experience in undiversion of the renal tract in thirteen patients with a neurogenic bladder is presented. All cases had what was intended to be a permanent diversion and many were grossly handicapped. Upper renal tract deterioration continued after surgery in only one patient. The commonest indication for surgery was the inability to achieve a satisfactory stoma. All were either dry or only damp postoperatively.
A child with bilateral Wilms' tumors is reported. The left renal tumor showed nephroblastoma with several tissues of apparent mesenchymal derivation and tubules with diverse epithelial differentiation. The right-sided tumor showed the more familiar triphasic pattern of nephroblastoma. Initial percutaneous renal biopsy of the left tumor did not reveal nephroblastoma but showed tubules with various epithelia and mesenchymal elements, and led to a diagnosis of teratoma. The pathogenesis of this complex neoplasm is discussed, and argument is presented that primitive renal blastema may be capable of more diverse differentiation than has previously been realized. A comparison is drawn between this neoplasm and some cases of hepatoblastoma. Certain cases reported as renal teratoma may be similar in nature.
Infantile hypertrophic pyloric stenosis is a relatively common condition and there is some recent evidence that the incidence is increasing in this country. Gastric outlet obstruction is caused by hypertrophy of the pyloric smooth muscle and the clinical presentation is with non-bilious vomiting starting at the age of 3-4 weeks. The diagnosis can usually be made by palpation of the hypertrophied pylorus. Operation has now superseded medical treatment as the treatment of choice but meticulous assessment and correction of fluid and electrolyte imbalance is essential preoperatively. The operation of pyloromyotomy as described by Ramstedt in 1912 is simple and effective, but attention to detail is necessary if minimal morbidity and zero mortality are to be achieved. Untreated, the mortality is high but, after successful treatment, these babies are healthy and normal, so that treatment is both worth while and gratifying. The aetiology is obscure; a polygenic pattern of inheritance has been shown to be a predisposing factor, but the postnatal precipitating factors are less defined. Early hopes that the hormone gastrin might prove to be the key have not been sustained by more recent research, but the role of other gastrointestinal hormones awaits clarification.
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Doppler ultrasound was used to detect flow through 160 C.S.F. shunt systems, 94 without suspected blockage, 62 with suspected blockage. An accuracy of 87% was achieved and we found this test helpful in clinical management in a significant number of cases. The test can distinguish between blockage of proximal and distal catheters and in certain situations can indicate infection of the distal catheter. Follow-up is continuing to determine the reliability of this technique in deciding whether asymptomatic patients are valve-dependent or not. The test is simple to learn and noninvasive.
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