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Ganglioneuroma (GN) is the benign representative of the Neuroblastic Tumor Family, affecting prevalently children. Few series of GN have been reported, making its natural history, treatment and outcome poorly known. Of 2062 children with neuroblastic tumors diagnosed in Italy between 1979-2002, 122 had a GN (6.1%). Compared to the malignant counterpart, GN showed female predominance (M/F 0.63 v 1.2), older age (82 v 21 months), and more frequent thoracic location (43 v 13%). In 46 cases the tumor was detected occasionally, in 17 was clinically appreciable as an abdominal or cervical mass, and in 59 was symptomatic. Symptoms included pain in 29 cases, respiratory distress in 12, scoliosis in 5, urological, gastro-intestinal, and neurological disturbances in 8, 7, and 6 cases, respectively. 15/35 cases studied by mIBG showed significant uptake of the radioelement and urinary VMA excretion was elevated in 10/52 cases. MYCN oncogene amplification and 1p36 chromosome deletion were absent in 23 and 10 cases respectively. DNA content was diploid in the 50 assayed cases. Of 120 patients evaluated for surgery, 81 (70.4%) underwent a complete, 14 a near-complete and 20 a partial tumor resection. Five cases were only biopsied. Nephrectomy was performed during the operation in 2 cases. One patient died of subarachnoideal hemorrhage shortly after surgery. Other 17 non fatal complications included severe hemorrhages in 3 cases and neurological deficits in 14, including 12 cases of Horner syndrome. Complications were more frequent with incomplete v complete and near-complete tumor resections (25%, 7.1% and 14.8%, respectively), and in cervical v thoracic, abdominal and pelvic tumor locations (57.1%, 21.6%, 4.4% and 8.3%, respectively). Growth of residual tumor was documented in 6 patients all of whom survive after tumor resection in 2 cases and no treatment in 4. Median follow-up is 52 months (range 0-228). 121/122 patients are alive, of whom 97 NED and 24 with tumor residue. 11 cases were lost to follow-up shortly after surgery. One patient developed an ovarian dysgerminoma and one a thyroid carcinoma at 9 and 17 years after diagnosis, respectively. In conclusion, prognosis of children with GN is excellent. The only death encountered was surgery-related. Other 17 patients suffered surgical complications. Since survival is not influenced by the type of tumor resection, an aggressive surgical approach is not recommended. Supported by Italian Neuroblastoma Foundation.

[Proc Amer Assoc Cancer Res, Volume 47, 2006]