Morbidity After Ganglioneuroma Excision: Is Surgery Necessary?

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.

ganglioneuroma - surgical complications - peripheral neuroblastic tumours

References

• 1 Brodeur GM. Neuroblastoma: biological insights into a clinical enigma.  Nat Rev Cancer. 2003;  3 203-216
• 2 Peuchmaur M, d'Amore ES, Joshi VV. et al . Revision of the International Neuroblastoma Pathology Classification: confirmation of favourable and unfavourable prognostic subsets in ganglioneuroblastoma, nodular.  Cancer. 2003;  98 2274-2281
• 3 De Bernardi B, Gambini C, Haupt R. et al . Retrospective study of childhood ganglioneuroma.  J Clin Oncol. 2008;  26 1710-1716
• 4 Geoerger B, Hero B, Harms D. et al . Metabolic activity and clinical features of primary ganglioneuromas.  Cancer. 2001;  91 1905-1913
• 5 Cannady SB, Chung BJ, Hirose K. et al . Surgical management of cervical ganglioneuromas in children.  Int J Pediatr Otorhinolaryngol. 2006;  70 287-294
• 6 Leuthardt R, Petralli C, Lütschg J. et al . Cortical blindness: an unusual complication after removal of a ganglioneuroma of the neck.  Childs Nerv Syst. 17 356-358
• 7 Duhem-Tonnelle V, Vinchon M, Defachelles AS. et al . Mature neuroblastic tumors with spinal cord compression: report of five pediatric cases.  Childs Nerv Syst. 2006;  22 500-505
• 8 Mosiello G, Gatti C, De Gennaro M. et al . Neurovesical dysfunction in children after treating pelvic neoplasms.  BJU Int. 2003;  92 289-292
• 9 Guye E, Lardy H, Piolat C. et al . Thoracoscopy and solid tumors in children: a multicenter study.  J Laparoendosc Adv Surg Tech A. 2007;  17 825-829
• 10 Shimada H, Ambros IM, Dehner LP. et al . The International Neuroblastoma Pathology Classification (the Shimada system).  Cancer. 1999;  86 364-372
• 11 Takeda S, Miyoshi S, Minami M. et al . Intrathoracic neurogenic tumors – 50 years' experience in a Japanese institution.  Eur J Cardiothorac Surg. 2004;  26 807-812
• 12 Demir HA, Yalçın B, Büyükpamukçu N. et al . Thoracic neuroblastic tumors in childhood.  Pediatr Blood Cancer. 2010;  54 885-889
• 13 Kulkarni AV, Bilbao JM, Cusimano MD. et al . Malignant transformation of ganglioneuroma into spinal neuroblastoma in an adult. Case report.  J Neurosurg. 1998;  88 324-327
• 14 Moschovi M, Arvanitis D, Hadjigeorgi C. Late malignant transformation of dormant ganglioneuroma?.  Med Pediatr Oncol. 1997;  28 377-381
• 15 Drago G, Pasquier B, Pasquier D. et al . Malignant peripheral nerve sheath tumor arising in a “de novo” ganglioneuroma: a case report and review of the literature.  Med Pediatr Oncol. 1997;  28 216-222
• 16 Kimura S, Kawaguchi S, Wada T. et al . Rhabdomyosarcoma arising from a dormant dumbbell ganglioneuroma of the lumbar spine: a case report.  Spine. 2002;  27 E513-E517
• 17 Keller SM, Papazoglou S, McKeever P. et al . Late occurrence of malignancy in a ganglioneuroma 19 years following radiation therapy to a neuroblastoma.  J Surg Oncol. 1984;  25 227-231
• 18 Ricci Jr A, Parham DM, Woodruff JM. et al . Malignant peripheral nerve sheath tumors arising from ganglioneuromas.  Am J Surg Pathol. 1984;  8 19-29
• 19 Chandrasoma P, Shibata D, Radin R. et al . Malignant peripheral nerve sheath tumor arising in an adrenal ganglioneuroma in an adult male homosexual.  Cancer. 1986;  57 2022-2025
• 20 de Chadarévian JP, MaePascasio J, Halligan GE. et al . Malignant peripheral nerve sheath tumor arising from an adrenal ganglioneuroma in a 6-year-old boy.  Pediatr Dev Pathol. 2004;  7 277-284

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