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Retrospective Study of Childhood Ganglioneuroma

Bruno De Bernardi, Claudio Gambini, Riccardo Haupt, Claudio Granata, Antonino Rizzo, Massimo Conte, Gian Paolo Tonini, Maurizio Bianchi, Maria Giuliano, Roberto Luksch, Arcangelo Prete, Elisabetta Viscardi, Alberto Garaventa, Angela Rita Sementa, Paolo Bruzzi, Paola Angelini

From the Departments of Hematology-Oncology and Surgery, and Services of Pathology, Radiology, and Statistics, Giannina Gaslini Children Hospital, and National Institute for Cancer Research, Genova; Department of Pediatrics, University of Bologna, Bologna; Department of Pediatrics, University of Napoli, Napoli; Department of Pediatrics, University of Torino, Torino; and the Division of Pediatric Oncology, National Cancer Institute, Milano, Italy

Corresponding author: Bruno De Bernardi, MD, Department of Pediatric Hematology-Oncology, Giannina Gaslini Children Hospital, Largo Gerolamo Gaslini 5, 16148 Genova, Italy; e-mail: brunodebernardi{at}ospedale-gaslini.ge.it

Purpose To review a historical cohort of childhood ganglioneuroma (GN), the benign representative of the peripheral neuroblastic tumor (PNT) family.

Patients and Methods Of 2,286 PNTs enrolled between 1979 and 2005, 146 (6.4%) were registered as GN. Histological revision was carried out on 76 tumors. Diagnosis was confirmed in 45, while 27 were reclassified as ganglioneuroblastoma intermixed (GNBI) and four were reclassified as other PNT subtypes.

Results GNs differed from other PNTs for sex, age, tumor site, stage, tumor markers, and scintigraphic results. Characteristics of 76 reviewed and 70 nonreviewed patients were comparable. Reviewed GN and GNBI patients were comparable except for homovanillic acid excretion, metaiodobenzylguanidine scintigraphy, and DNA content. Seven patients were only biopsied and 139 underwent surgery. Twenty-two patients suffered surgery-related complications, of which two were fatal and seven were severe. Radical tumor resection and surgery-related complication rates were comparable for GN, GNBI, and nonreviewed instances. Six patients developed tumor progression but survived. Two patients developed a late malignancy but survived. None of the 146 patients received chemotherapy. Of 146 patients, two died of surgery-related complications and 144 survived.

Conclusion Diagnosis was changed to GNBI for approximately one third of 76 reviewed tumors. Patients with confirmed GN, reclassified as GNBI, and nonreviewed histology presented with comparable clinical, biochemical, and biologic features. Surgical results, complication rate, number of progressions, and outcome were similar for the three groups. Surgery was associated with significant risk of complications. Survival was not influenced by extent of tumor resection. Aggressive surgical approach should not be recommended for childhood GN and GNBI.

Supported in part by the Italian Neuroblastoma Foundation.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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