Immediate nephrectomy versus preoperative chemotherapy in the management of non-metastatic Wilms' tumour: results of a randomised trial (UKW3) by the UK Children's Cancer Study Group.

PURPOSE: To determine if patients receiving preoperative chemotherapy with vincristine and actinomycin D for non-metastatic Wilms' tumour have a more advantageous stage distribution and so need less treatment compared to patients who have immediate nephrectomy, without adversely affecting outcome. METHODS: Between 1991 and 2001, a total of 205 patients with newly diagnosed non-metastatic renal tumours, of which 186 had Wilms' histologies, were randomly assigned either to immediate surgery or to 6 weeks preoperative chemotherapy and then delayed surgery. Both groups of children received postoperative chemotherapy according to tumour stage and histology determined at the time of nephrectomy. RESULTS: There was a significant improvement in the stage distribution for patients with Wilms' histologies receiving delayed surgery compared to those having immediate nephrectomy (stage I: 65.2% versus 54.3%; stage II: 23.9% versus 14.9%; stage III: 9.8% versus 29.8%, chi2 test for trend=7.02, p=0.008). This improvement resulted in 20% fewer children receiving radiotherapy or doxorubicin yet event-free and overall survivals at 5 years of 79.6% and 89.0%, respectively, were similar in the two groups. CONCLUSION: Six weeks of preoperative chemotherapy with vincristine and actinomycin D results in a significant shift towards a more advantageous stage distribution and hence reduction in therapy, while maintaining excellent event free and overall survival in children with non-metastatic Wilms' tumour. Around 20% of survivors were therefore spared the late-effects of doxorubicin or radiotherapy. Our results suggest that all children with non-metastatic Wilms' tumour should receive chemotherapy prior to tumour resection.

Surgical complications after immediate nephrectomy versus preoperative chemotherapy in non-metastatic Wilms' tumour: findings from the 1991-2001 United Kingdom Children's Cancer Study Group UKW3 Trial.

PURPOSE: To compare surgical complication rates after immediate nephrectomy versus delayed nephrectomy following preoperative chemotherapy in children with non-metastatic Wilms' tumour enrolled in UKW3, both in randomised patients and in those for whom the treatment approach was defined by parental or physician choice. METHODS: Records for all patients enrolled into UKW3 were reviewed. Any record of tumour rupture or surgical complication was extracted and comparisons made between the two treatment strategies in both populations of randomised and non-randomised patients. RESULTS: Of 525 children enrolled, 205 patients were randomised to either immediate nephrectomy (n=103) or pre-operative chemotherapy followed by delayed nephrectomy (n=102). Of the 320 children not randomised, data were available on 189 cases treated with immediate nephrectomy and 103 treated with pre-operative chemotherapy. There were significantly fewer surgical complications in randomised children given pre-operative chemotherapy before surgery compared to children undergoing immediate nephrectomy (1% vs. 20.4%, P<0.001); this difference was most marked for tumour rupture (0% vs. 14.6%, P<0.001). CONCLUSIONS: Delayed nephrectomy for Wilms' tumour, preceded by pre-operative chemotherapy was associated with fewer surgical complications compared with immediate nephrectomy.

Mature and immature extracranial teratomas in children: the UK Children's Cancer Study Group Experience.

PURPOSE: The purpose of this article is to describe the features, treatment, and risk factors for relapse of children with mature teratoma (MT) and immature teratoma (IT) to assist future treatment plans. PATIENTS AND METHODS: Patients were younger than 16 years of age and referred to the UK Children's Cancer Study Group centers with biopsy-proven extracranial MT and IT and no prior chemotherapy. Complete excision, with the coccyx in sacrococcygeal patients, and follow-up, including serum alpha-fetoprotein monitoring for early detection of malignant yolk sac tumor (YST) recurrence, were recommended. Carboplatin, etoposide, and bleomycin (JEB) were given for YST relapse, whereas relapsed MT and IT were treated at clinicians' discretion, usually surgically. Pathology was reviewed and treatments, outcome, and prognostic features assessed. RESULTS: There were 351 patients, 227 with MT, 124 with IT. Tumor sites were: testis (n = 53), ovary (n = 130), sacrococcygeal region (n = 98), thorax (n = 23), and other (n = 47). Surgical resection was incomplete in 26% of MT and 40% of IT patients; 5-year event-free survival was 92.2% and 85.9%, respectively, and 5-year overall survival was 99% and 95.1%. Poorer outcome occurred with incomplete resection, tumor rupture, nongonadal site (particularly sacrococcygeal), young age, higher stage and grade, and gliomatosis peritonei, but not with cyst fluid aspiration/spillage, tumor enucleation, nodal gliomatosis, or microfoci of YST in the tumor (Heifetz lesions). JEB was effective for YST recurrence, but not for MT or IT. CONCLUSION: Treatment remains primarily surgical, with JEB chemotherapy for YST relapse. No definite response followed JEB for pure MT and IT. Adjuvant chemotherapy after surgery for sacrococcygeal patients is not advocated.

Management of infants with Pierre Robin sequence.

OBJECTIVE: Several methods of treating babies with Pierre Robin sequence have been described since the condition itself was first documented in 1923. The main aim of treatment has been to relieve upper airway obstruction. Treatment methods used range from positioning of the baby to invasive surgery. The aim of this article was to describe the assessment, treatment, and monitoring methods used for babies referred with Pierre Robin sequence (PRS). SETTING/PATIENTS: From December 1995 to May 2000, 22 consecutive patients were admitted to Birmingham Children's Hospital with PRS. Their airway and nutritional status were assessed and continuously monitored. INTERVENTIONS: Treatment concentrated on the relief of airway obstruction with a nasopharyngeal airway (NPA) and nutritional support of the babies until they grew out of their respiratory and feeding difficulties. MAIN OUTCOME MEASURES: Outcome measures were oxygen saturation, growth of the babies, and the need for surgery. RESULTS: All babies were managed successfully with an NPA and nutritional support. No baby required surgery, and the majority showed good weight gain. CONCLUSION: Relieving airway obstruction by NPA is an effective and safe treatment for babies with PRS until they have grown out of their respiratory and feeding difficulties. It avoids the need for surgery and can be used on neonatal wards using the monitoring described.

The role of biopsy in the diagnosis of renal tumors of childhood: Results of the UKCCSG Wilms tumor study 3.

BACKGROUND: The United Kingdom Children's Cancer Study Group (UKCCSG) Wilms Tumor Study 3 has adopted preoperative chemotherapy for Wilms tumors (WT), but required prechemotherapy biopsy for histologic diagnosis. The aims of this review were to assess the usefulness and safety of prechemotherapy biopsy and to compare histologic features of WT before and after chemotherapy. PROCEDURE: There were 286 eligible patients but only 241 biopsies and 226 nephrectomy case slides were submitted for panel review. The presence of different histologic components of WT before and after chemo therapy was retrospectively assessed. RESULTS: Among the 241 cases, the biopsy material in 9 (4%) was not diagnostic, in 28 (12%) that were clinically and radiologically consistent with WT, the biopsy revealed tumors other than WT, and in the remaining 204 (85%) WT was confirmed. Of 188 WT suitable cases, blastema was found in 89% of tumors at biopsy, but in only 50% at nephrectomy; the remainder were either completely necrotic (17%) or showed only epithelial and/or stromal elements (33%). Of 182 children who had percutaneous cutting needle biopsy (PCNB), a fall in haemoglobin (20% of cases) and local pain (19%) were the most common complications. One child required emergency nephrectomy due to massive intratumoral bleeding, another had tumor rupture and subsequently died, and a third developed a needle track recurrence 8 months after the biopsy. CONCLUSIONS: A number of renal tumors (12%) can have the correct histologic diagnosis made by PCNB. Preoperative chemo therapy markedly decrease in the number of samples with preserved blastema. The morbidity associated with PCNB is small. Needle biopsy of any renal mass prior to initiation of chemotherapy is recommended.

Management of pyopagus conjoined twins.

INTRODUCTION: Approximately 18% of conjoined twins (1 in 200,000 live births) are joined at the sacrum ("pyopagus"). As the joined structures are not life-threatening, there is generally a good prognosis, with time for investigation and planning of operative separation. CASE REPORT: This paper reports on the management of pyopagus twin girls, the first in the UK delivered at 36 weeks by Caesarean section. The diagnosis had been made by ultrasound at 12 weeks' and confirmed by MRI scan at 26 weeks' gestation. Each twin had wasting and weakness below one knee, but no deficit in the other leg. The perineum had two urethras but only one anus. One infant had a colostomy in the immediate neonatal period. With appropriate further imaging, surgery was planned in two stages. First, a balloon expander was inserted in the bridging area. At 3 months of age a team comprising Plastic, Paediatric and Neurosurgeons undertook the definitive separation. DISCUSSION: Details of the multidisciplinary planning, operative separation and follow-up of these two children will be discussed.