Pelvic neuroblastoma: Low mortality and high morbidity*

Presented at the 31st Annual Meeting of the Canadian Association of Paediatric Surgeons, Montreal, Quebec, Canada, September 23-26, 1999.
https://doi.org/10.1053/jpsu.2000.6076Get rights and content

Abstract

Background/Purpose: Cervical, thoracic, and pelvic neuroblastomas are regarded as having a better outcome than abdominal primaries. The aim of the study was to analyze the results of treatment of pelvic neuroblastomas in our institution. Methods: The authors reviewed the records of 284 patients with neuroblastoma treated in our hospital during the period 1983 through 1998 and identified 17 (6%) with pelvic tumors. The revised International Neuroblastoma Staging System was used. Results: There were 6 patients with stage 1 disease, 8 with stage 2, 2 with stage 3, and 1 with stage 4 disease. Intraspinal extension of the tumor was present in 7 patients (41%). Except for one child with stage 4 disease, all patients underwent an attempt of tumor excision, and 6 had a complete resection. All 7 patients with intraspinal tumor survived. Permanent postoperative neurological complications occurred in 6 patients (35%). These included sciatic nerve palsy, urinary and fecal incontinence, neuropathic bladder, and leg weakness or nerve root injury L4-S1. Three of 17 patients died, but 1 fatality was unrelated to the tumor. The overall survival rate was 82% and was not influenced by the completeness of tumor resection. Conclusions: The survival of nonmetastatic pelvic neuroblastoma in our institution is good despite incomplete tumor resection. Intraspinal extension is not a negative prognostic factor. Considering the high incidence of permanent neurological damage after surgery and the generally favorable biological characteristics of these tumors, surgical treatment should not be overaggressive. J Pediatr Surg 35:724-728. Copyright © 2000 by W.B. Saunders Company.

Section snippets

Materials and methods

We reviewed the records of 284 patients with neuroblastoma treated in Great Ormond Street Hospital for Children between January 1983 and December 1998 and identified 17 (6%) with pelvic tumors. Fourteen patients were diagnosed and treated in our hospital, and the other 3 were referred after initial treatment in another hospital.

Each patient's disease was staged retrospectively using the criteria of the International Neuroblastoma Staging System.9 In this system a grossly resectable pelvic tumor

Patient characteristics

The median age of patients at diagnosis was 10 months (range, 6 weeks to 10 years). Fourteen children (82%) were less than 2 years old, of whom, 10 were less than 1 year old. The male to female ratio was 2.4:1, which is a comparable figure to that reported previously.2, 3, 4 The diagnosis of neuroblastoma was established on the basis of raised urinary catecholamine metabolites (10 patients) and/or histology of a pretreatment percutaneous biopsy (8 patients). There were 6 patients with stage 1

Discussion

The incidence of pelvic neuroblastoma in our series was 6%, which is comparable to other reports (2% to 5.6%).1, 2, 3, 4, 13 Our study confirms the excellent survival rate of children with pelvic neuroblastomas but highlights the high incidence of permanent neurological complications, often after tumor resection.

The clinical presentation of pelvic neuroblastomas is different from that of tumors arising in other parts of the abdomen.14 More than half of our patients presented with a palpable

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*

Address reprint requests to Agostino Pierro, Department of Paediatric Surgery, Institute of Child Health, 30 Guilford St, London, WC1N 1EH England.

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