Luca Pio1,2, Thomas Blanc1,2, Timothé de Saint Denis2,3, Sabine Irtan1,2, Dominique Valteau-Couanet4, Jean Michon5, Hervé Brisse6,7, Louise Galmiche-Rolland8, Luc Joyeux9, Thierry Odent2,10, Caroline Harte11, Christophe Glorion2,10, Michel Zerah2,3, Sabine Sarnacki1,2. 1. Department of Pediatric Surgery and Urology, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. 2. Paris Descartes University, Université Sorbonne Paris Cité, Paris, France. 3. Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. 4. Department of Oncology for Child and Adolescents, Gustave Roussy Cancer Center, Paris, France. 5. Department of Pediatric Oncology, Oncology Center SIREDO (Care, Innovation, Research for Children, Adolescents and Young Adults with Cancer, Institut Curie, Paris, France. 6. Imaging Department, Institut Curie, Paris, France. 7. Paris Sciences et Lettres Research University, Paris, France. 8. Department of Pathology, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. 9. Department of Pediatric Surgery, Queen fabiola Children's Hospital, Université Libre de Bruxelles, Brussels, Belgium. 10. Department of Pediatric Orthopedics, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. 11. Department of Pediatric Anesthesia, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France.
Abstract
INTRODUCTION: Prognosis of dumbbell neuroblastoma (NBL) is mainly determined by the sequelae induced by the tumor itself and the neurosurgical approach. However, after primary chemotherapy, surgical management of the residual tumor, especially the spinal canal component, remains controversial. METHODS: We conducted a single-center retrospective cohort study over the last 15 years (2002-2017) including patients treated for NBL with spinal canal extension focusing on timing and type of surgery, complications, and functional and oncological follow-up. RESULTS: Thirty-two children (14 M, 18 F) were managed for NBL, with the majority (26) presenting with NBL stroma poor while four had ganglioneuroblastoma intermixed, one nodular, and one ganglioneuroma. All but two patients received neoadjuvant chemotherapy. Upfront laminotomy for spinal cord decompression was performed in two patients; nine patients had extraspinal surgery with a follow-up neurosurgical procedure in seven cases; eight patients had initial neurosurgery followed by an extraspinal procedure, while six patients underwent a combined multidisciplinary approach. With a median follow up of 3.6 years (0.1-14.9), 29 patients (90.6) are alive and two out of three (19, 65.5%) have functional sequelae. CONCLUSION: Patients with NBL with persistent spinal canal extension of the tumor after neoadjuvant chemotherapy treated at our center had outcomes that compare favorably with the literature. This is likely due to the multidisciplinary approach to optimal surgical strategy and continuous evaluation of the respective risks of tumor progression. Neurological disability results from initial spinal cord compression or the radicular sacrifice required for tumor resection.
INTRODUCTION: Prognosis of dumbbell neuroblastoma (NBL) is mainly determined by the sequelae induced by the tumor itself and the neurosurgical approach. However, after primary chemotherapy, surgical management of the residual tumor, especially the spinal canal component, remains controversial. METHODS: We conducted a single-center retrospective cohort study over the last 15 years (2002-2017) including patients treated for NBL with spinal canal extension focusing on timing and type of surgery, complications, and functional and oncological follow-up. RESULTS: Thirty-two children (14 M, 18 F) were managed for NBL, with the majority (26) presenting with NBL stroma poor while four had ganglioneuroblastoma intermixed, one nodular, and one ganglioneuroma. All but two patients received neoadjuvant chemotherapy. Upfront laminotomy for spinal cord decompression was performed in two patients; nine patients had extraspinal surgery with a follow-up neurosurgical procedure in seven cases; eight patients had initial neurosurgery followed by an extraspinal procedure, while six patients underwent a combined multidisciplinary approach. With a median follow up of 3.6 years (0.1-14.9), 29 patients (90.6) are alive and two out of three (19, 65.5%) have functional sequelae. CONCLUSION:Patients with NBL with persistent spinal canal extension of the tumor after neoadjuvant chemotherapy treated at our center had outcomes that compare favorably with the literature. This is likely due to the multidisciplinary approach to optimal surgical strategy and continuous evaluation of the respective risks of tumor progression. Neurological disability results from initial spinal cord compression or the radicular sacrifice required for tumor resection.
Authors: Simone de Campos Vieira Abib; Chan Hon Chui; Sharon Cox; Abdelhafeez H Abdelhafeez; Israel Fernandez-Pineda; Ahmed Elgendy; Jonathan Karpelowsky; Pablo Lobos; Marc Wijnen; Jörg Fuchs; Andrea Hayes; Justin T Gerstle Journal: Ecancermedicalscience Date: 2022-02-17