S. Barrena M. Miguel C.A. de la Torre Late surgery for spinal deformities in children previously treated for neural tumors Eur J Pediatr Surg 21/1 2011 54 57

Abstract

Ganglioneuroma (GN), the benign form of peripheral neuroblastic tumor, is often asymptomatic, and the diagnosis can be incidental. The aim of this study was to evaluate the incidence of complications after surgical treatment after diagnosis of this tumor. Twenty-four consecutive children were diagnosed with GN. All patients had negative urine catecholamines and biopsies confirming the diagnosis at GN. Age at diagnosis was 73 ± 43 months. The most common presentation was respiratory symptoms and/or abdominal pain; 9 (38%) patients were asymptomatic. Tumor location was in the chest (n = 14), abdomen (n = 7), or pelvis (n = 3). Twenty-three children (9 asymptomatic) were operated on; 1 child with a thoracic mass did not undergo surgery because of severe neurologic impairments from birth unrelated to GN. Thirteen children (4 asymptomatic) had a thoracotomy, 8 (4 asymptomatic) had a laparotomy, and 2 (1 asymptomatic) underwent perineal resection. A macroscopically complete surgical excision was performed in 17 cases (74%) and a near-complete excision in 6 (26%). At histologic examination, resection margins contained tumor in 10 patients (43%) and were free of tumor in the remaining 13 (57%). Seven children (30%) had complications after surgery, including 3 patients with Horner syndrome (which persisted in 2), 1 with chylothorax, 1 with pneumothorax, 1 with pains in the arm, and 1 who developed adhesive intestinal obstruction. Two children received adjuvant chemotherapy. Histologic reevaluation according to the International Pathology Classification confirmed GN in 20 cases (83%), whereas intermixed ganglioneuroblastoma (iGNB) was diagnosed in 4 patients (17%). At 33.5 ± 40 months (range, 1-137 months) of follow-up, all 24 patients, including the child not operated on and the children with incomplete resection or iGNB, are alive with no tumor progression or recurrence. It is concluded that GN excision is associated with postoperative complications, which can be persistent and may affect the quality of life. The rationale for GN excision should be revised.