Eur J Pediatr Surg 2011; 21(1): 33-37
DOI: 10.1055/s-0030-1263195
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Morbidity After Ganglioneuroma Excision: Is Surgery Necessary?

G. Retrosi1 , M. Bishay1 , E. M. Kiely1 , N. J. Sebire2 , J. Anderson3 , M. Elliott4 , D. P. Drake1 , P. de. Coppi1 , S. Eaton1 , A. Pierro1
  • 1UCL – Institute of Child Health and Great Ormond Street Hospital for Children, Department of Surgery, London, United Kingdom
  • 2UCL – Institute of Child Health and Great Ormond Street Hospital for Children, Department of Histopathology, London, United Kingdom
  • 3UCL – Institute of Child Health and Great Ormond Street Hospital for Children, Department of Molecular Haematology and Cancer Biology, London, United Kingdom
  • 4UCL – Institute of Child Health and Great Ormond Street Hospital for Children, Department of Cardiology, London, United Kingdom
Further Information

Publication History

received May 18, 2010

accepted after revision July 26, 2010

Publication Date:
15 October 2010 (online)

Abstract

Introduction: Ganglioneuroma (GN), the benign form of peripheral neuroblastic tumour, is often asymptomatic and the diagnosis can be incidental. Our aim was to evaluate the incidence of complications after surgical treatment following diagnosis of this tumour.

Material and Methods: 24 consecutive children were diagnosed with GN in our centre between January 1989 and December 2009. All patients had negative urinary catecholamines and/or biopsy confirming the diagnosis of GN. Data are reported as mean±SD.

Results: Age at diagnosis was 73±43 months. The most common presentation was respiratory symptoms and/or abdominal pain; 9 (38%) patients were asymptomatic. Tumour location was in the chest (n=14), abdomen (n=7), or pelvis (n=3). 23 children (9 asymptomatic) were operated on; 1 child with a thoracic mass did not undergo surgery because of severe neurological impairments from birth unrelated to GN. 13 children (4 asymptomatic) had a thoracotomy, 8 children (4 asymptomatic) had laparotomy, and 2 (1 asymptomatic) underwent perineal resection. A macroscopically complete surgical excision was performed in 17 cases (74%) and a macroscopically near-complete excision in 6 (26%). At histological examination, resection margins contained tumour in 10 patients (43%) and were free of tumour in the remaining 13 (57%). 7 children (30%) had complications after surgery including 3 patients with Horner's syndrome (which persisted in 2), 1 with chylothorax, 1 with pneumothorax, 1 with pain in the arm, and 1 who developed adhesive intestinal obstruction. 2 children received adjuvant chemotherapy. We re-evaluated the histology specimens according to the International Neuroblastoma Pathology Classification and found that the diagnosis of GN was confirmed in 20 cases (83%), while intermixed ganglioneuroblastoma (iGNB) was diagnosed in 4 patients (17%). At 33.5±40 months (range 1–137) follow-up, all 24 patients, including the child not operated on and the children with incomplete resection or iGNB, are alive with no tumour progression or recurrence.

Conclusions: GN excision is associated with postoperative complications which can be persistent and may affect the quality of life of survivors. In our series we did not observe tumour progression in spite of incomplete excision. The rationale for GN excision should be revisited.

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Correspondence

Prof. Agostino Pierro

UCL – Institute of Child Health

and Great Ormond Street

Hospital for Children

Department of Surgery

30 Guilford Street

WC1N 1EH London

United Kingdom

Phone: +44 020 7905 2641

Fax: +44 020 7404 6181

Email: pierro.sec@ich.ucl.ac.uk