Pilomatrixoma--accuracy of clinical diagnosis.

BACKGROUND/PURPOSE: Pilomatrixoma is a common tumor of skin appendages in children. The aim of the study was to assess the accuracy of clinical diagnosis and factors contributing to misdiagnosis. METHODS: A retrospective case note review of patients who had pilomatrixoma excised during a 5-year period in a tertiary referral children's hospital in the UK. RESULTS: From 75 patients, 78 pilomatrixomata were excised. The diagnosis was achieved preoperatively in 46% of patients. Other diagnoses included sebaceous and dermoid cysts, foreign body reaction, calcification in lymph gland, and fat necrosis. CONCLUSION: Factors contributing to misdiagnosis include cystic lesions with varying consistency, punctum-like appearance, atypical location, and absence of clinically recognizable calcification. Despite close excision, the recurrence rate is low.

Wilms' tumor with intracaval thrombus in the UK Children's Cancer Study Group UKW3 trial.

BACKGROUND/PURPOSE: To define the clinical characteristics and surgical management of intracaval involvement in patients enrolled in the UKW3 trial (1991-2001), which recommended elective preoperative chemotherapy for such cases. METHODS: Cases were identified from preoperative imaging and surgical trial forms. These asked specific questions about whether the surgeon suspected intracaval extension at diagnosis or found it at nephrectomy. For tumors with Wilms' histology, original case notes were examined. RESULTS: Of 842 patients registered in UKW3, 730 (87%) had Wilms' tumor. Among them, 59 (8.1%) had evidence of intracaval extension, either documented at diagnosis (53) or found unexpectedly at nephrectomy (6). Intracaval extension was also seen in tumors of other histology. The level of thrombus was intraatrial (10), suprahepatic (9), retrohepatic (8), infrahepatic (26), and unknown (6). The median age at diagnosis was 3.75 years compared to 2.97 years in patients without inferior vena cava thrombus (P < .0001). Fifty-two of 59 received preoperative chemotherapy. Thirty-one (52%) needed cavotomy, and 3 (30%) with intraatrial extension required cardiopulmonary bypass. The commonest operative complication was significant hemorrhage and resulted in mortality in 3 cases. CONCLUSIONS: Preoperative chemotherapy is a useful adjunct to shrink the tumor and thrombus. This reduces the requirement for cavotomy and cardiopulmonary bypass. Intraoperative hemorrhage remains a significant cause of operative morbidity and mortality.

Rhabdomyosarcoma relapse in an unusual site.

Lower genitourinary tract rhabdomyosarcoma is the most common soft tissue sarcoma in children. Recurrence is common but usually occurs at the original site. A case of a young boy with recurrent rhabdomyosarcoma at an unusual site (glans penis) is presented.

The long-term outcome of the endoscopic subureteric implantation of polydimethylsiloxane for treating vesico-ureteric reflux in children: a retrospective analysis of the first 195 consecutive patients in two European centres.

OBJECTIVE: To determine the long-term efficacy of endoscopically implanting polydimethylsiloxane (PDS, Macroplastique, Uroplasty BV, Geleen, the Netherlands) for treating vesico-ureteric reflux (VUR) in children. PATIENTS AND METHODS: We retrospectively reviewed the records of 195 children treated with PDS over the past 10 years in two European centres (Netherlands and UK) and conducted follow-up investigations where deemed necessary. In the Dutch centre patients were treated according to a prospective approved study protocol. RESULTS: Of the 195 treated children, 155 had a successful outcome; in all, 311 refluxing renal units were treated, with an overall success rate of 82.3% in a mixed group of patients with unilateral or bilateral VUR and duplex systems. CONCLUSION: The long-term data from these two independent centres confirms the viability of PDS and the technique for treating VUR in children. We conclude that the material is safe and effective and can be used in VUR grades II-V. Treating patients with grade I VUR is recommended in those cases where there is no spontaneous resolution and 'breakthrough' infections.

Foregut duplications.

Twenty-one children were treated for foregut duplications at the Royal Hospital for Sick Children over a 42-year period. There were 11 males and 10 females. The mean age at diagnosis was 1 year 6 months; antenatal diagnosis was made in 1 case. Respiratory and gastrointestinal (GI) presentation predominated. The diagnosis was made by chest radiograph (10), barium swallow (5), at operation (4), and by ultrasound (2). All patients were treated surgically; the mortality was 10%. The histology of the epithelial lining of the duplications revealed gastric mucosa in 15 cases; in 5 the duplication cyst was lined by more than one cell type. Nine patients had other associated congenital anomalies, the majority of these were vertebral abnormalities. The size of this series emphasises that clinicians should be aware that duplication cysts occur and should be considered in a child with unusual respiratory or GI symptoms.