S. P. Attard-Montalto

Quality of life in survivors of childhood cancer after megatherapy with autologous bone marrow rescue

A questionnaire study was carried out in a group of survivors of childhood cancer to assess their quality of life. The questionnaire was sent to 30 survivors who had completed treatment with megatherapy followed by autologous bone marrow rescue at St Bartholomews Hospital, London. Of the 28 respondents (93%), in 27 (96%) the quality of life was judged to be good, with 11 of these 27 (40%) having no disability whatsoever and a further 9 (33%) reporting only minimal disability. The other 7 patients had moderate to severe disabilities, with pain and depression remaining ongoing problems, and some adolescents felt that they were unable to cope with everyday life alongside their peers. Nine parents and 14 of the children themselves expressed anxiety about the previous illness. The study shows that, by using a postal method, a satisfactory assessment of quality of life in survivors of childhood cancer can be made.

Retinoblastoma in association with the chromosome breakage syndromes Fanconi's anaemia and Bloom's syndrome: clinical and cytogenetic findings

Two children presenting with sporadic unilateral retinoblastoma and exhibiting a high degree of chromosome breakage were noted to have unusual facies, microcephaly and abnormal skin pigmentation. In the first child the pattern of both spontaneous and mitomycin‐C‐induced chromosome breakage was characteristic of Fanconis anaemia although the degree of breakage was extreme. She also exhibited a striking increase in X‐ray‐induced chromosomal damage in G0 lymphocytes as measured by dicentric formation and increase in chromatid‐type aberrations. She had a number of typical clinical features, including cafe‐au‐lait patches and abnormalities involving the kidney; however, she demonstrated neither the hypoplasia of radius and thumb nor the typical aplastic phase of this disorder. At age 22 months the child became anaemic with trilineage myelo‐dysplasia, which was rapidly followed by the development of acute myeloblastic leukaemia. The early onset (at age 4 months) of retinoblastoma may have been associated with the underlying genomic instability. The second child exhibited a pattern of chromosome breakage characteristic of Blooms syndrome, in addition to a moderate increase in damage induced by mytomycin‐C. She had the typical stunted growth and malar hypoplasia of Blooms syndrome although she did not demonstrate the frequently described erythematous ‘butterfly rash’. Although patients with Fanconis anaemia and Blooms syndrome are recognised to be at an increased risk of cancer, retinoblastoma has not previously been described in patients with either condition. We suggest that underlying recessive chromosome breakage syndromes may be underdiagnosed in paediatric cancer patients, with important implications for prognosis and genetic counselling.

Non-Hodgkin's Lymphoma and Klinefelter Syndrome

Patients with a 47, XXY karyotype (Klinefelter syndrome) appear to have an increased risk of developing a malignancy in adulthood, usually cancer of the breast, extragonadal germ cell tumor, and acute nonlymphoblastic leukemia. There is growing evidence to show that these patients also have an increased risk of developing a malignancy in childhood. There are reports describing the development of acute lymphoblastic leukemia, retinoblastoma, and rhabdomyosarcoma in children with a 47, XXY or mosaic 47, XXY/46, XY karyotype. We report a child with a bone metastasizing, B-cell lineage, non-Hodgkins lymphoma (NHL) who was found to have a 47, XXY karyotype in both the tumor and constitutional cells.

Is there a danger in delaying radiotherapy in childhood medulloblastoma

Approximately 45-50% of children with medulloblastoma are cured by conventional surgery and radiotherapy, but survivors may face severe late neuropsychological toxicity. Studies showing good partial responses to platinum-based chemotherapy in relapsed patients and the theoretical possibility of a therapeutic window immediately after surgery have prompted neoadjuvant treatment studies which are ongoing. However, the absolute benefit of chemotherapy for the treatment of medulloblastoma in childhood is, as yet, not proven. There is a danger that chemotherapy may simply delay radiotherapy, and in so doing reduce the radiological impact of this known effective treatment. We report four children with medulloblastoma presenting consecutively to this unit over a 6-month period, whose management was problematic because of either failure to respond to neoadjuvant chemotherapy or their very young age. These cases are discussed in the light of the current literature and future treatment strategies that must seek to improve the therapeutic ratio of multimodality therapy.

Ganciclovir Treatment of Congenital Cytomegalovirus Infection: A report of two cases

Ganciclovir has been shown to be effective against cytomegalovirus (CMV) in vitro, but its role in the treatment of congenital cytomegalovirus infection is unknown. We describe its use in the management of two cases of congenital CMV infections. The drug was well tolerated and virus shedding in the urine was eliminated, though hepatosplenomegaly and neurodevelopmental delay persisted in both cases.

High incidence of hypertension in children presenting with acute lymphoblastic leukemia

Although hypertension is a complication of acute lymphoblastic leukemia (ALL), its true incidence in this disease is unknown. In this study the blood pressure profiles in all children newly diagnosed with ALL were reviewed over an 18-month period. Fourteen (46%) from a total of 30 patients were found to be hypertensive at presentation (n = 8) or during induction chemotherapy (n = 6). A patient with significant hypertension developed generalized convulsions; the rest were asymptomatic. Six patients were managed with antihypertensive drugs. Four patients with hypertension had renal enlargement on the initial ultrasound scan, which returned to normal when hematologic remission was achieved. One patient without hypertension had bilateral renal enlargement, but this persisted despite achieving remission. All patients with hypertension were normotensive at follow-up 2 to 18 months after induction chemotherapy. The presence of hypertension before therapy and its association with renal enlargement suggest that the leukemic process is an important etiologic factor. In all cases therapy aggravated or unmasked the elevation in blood pressure. Considering the high incidence of susceptible patients, increased awareness and prompt management may avoid possible life-threatening complications.

Central venous catheter tip position and malfunction in a paediatric oncology unit

An audit of 151 central venous catheters (CVCs) in 118 children with malignant disease was carried out over 20 months. The types included 31 valved silastic (Groshong), 58 non-valved silastic (Hickman), and 62 non-valved polyurethane (Cuff Cath) CVCs. There was no difference between the three groups with regard to the clinical diagnosis. The mean patient age at catheter insertion was 5.5 years and the mean weight 21.6 kg. None of the catheter types were associated with an increased risk of problems at insertion, migration, mechanical damage, blockage, sampling, or catheter infection. The incidence of catheter infection was 1.4/1,000 catheter days. Exit-site infection was less frequent with Groshong CVCs (P <0.05), which were in situ for the shortest period. The risk of problems with blood sampling was significantly increased in those catheters whose tip was sited outside the right atrium (P <0.005). For the 60 CVCs removed electively, the mean duration in situ was similar for all catheter types; 43 were removed following a problem. Of these, Groshong catheters were in situ for the shortest period (P = 0.05), probably as a result of delayed anchoring of the cuff. The tip position was the single most important determinant in the correct functioning of CVCs, irrespective of the type of catheter. Intraoperative screening of the tip position at catheter insertion is therefore mandatory for optimal catheter functioning.

Gastric Perforation in Non-Hodgkin'S Lymphoma

Oral steroids are an important component in the treatment of leukemia and lymphoma in children. Until recently it was widely accepted that treatment with oral steroids carried the risk of gastrointestinal complications, in particular gastric irritation, gastrointestinal ulceration, and hemorrhage. Though spontaneous intestinal perforation has been reported, to our knowledge, spontaneous perforation of the stomach in a child with lymphoma taking oral steroids has not previously been described. Furthermore, this report would appear to contradict recent reports claiming that steroid-induced ulcers do not arise unless the patient is also concurrently receiving nonsteroidal anti-inflammatory drugs.1,2,3