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      Giant intrathoracic ganglioneuroma with scoliosis treated by one-stage posterior resection and scoliosis correction: a case report

      case-report
      1 , * , 1 , 2 , 3
      SICOT-J
      EDP Sciences
      Giant ganglioneuroma, Ganglioneuroma with scoliosis, Spinal ganglioneuroma, Posterior approach

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          Abstract

          Spinal ganglioneuroma occurs mostly in the thoracic spine causing various manifestations including scoliosis that can be misdiagnosed as idiopathic scoliosis. Few reports exist in the literature on the diagnosis and management of scoliosis secondary to huge ganglioneuroma and usually staged treatment is preferred. In this report, we present a 17-year-old female patient presented with back pain, lower limbs numbness, spinal deformity, and shortness of breath. Plain X-rays showed a 50° right thoracic scoliotic curve. MRI and chest CT revealed a huge extra pulmonary mass shifting the mediastinum with intra spinal extension through the left neural foramina compressing the spinal cord. Percutaneous US guided needle biopsy confirmed the diagnosis of ganglioneuroma. One-stage posterior instrumented correction of scoliosis, spinal cord decompression, and excision of the whole mass from the mediastinum and the spine through posterior approach was done for the patient with smooth postoperative recovery. Chest CT scan was done 2 years after surgery and excluded any local recurrence.

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          Most cited references11

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          Metabolic activity and clinical features of primary ganglioneuromas.

          Ganglioneuroma (GN) is considered by most to be a benign tumor of neural crest origin. It may evolve from differentiating neuroblastoma or may be diagnosed as primary ganglioneuroma. The rarity of this tumor and the lack of understanding of its biology often lead to inaccurate diagnosis and treatment. The authors analyzed clinical features and biologic behavior of primary ganglioneuroma in 49 patients who were registered with but were not part of the national neuroblastoma trials. Data included age and symptoms at diagnosis, gender, tumor localization and size, (123)I-metaiodobenzylguanidine (mIBG) scintigraphy, secretion of catecholamines, histology, treatment, and outcome, whenever available. Patients with primary ganglioneuroma were significantly older than patients with neuroblastoma. Median age at diagnosis was 79 months compared with 16 months (P < 0.0001). Ganglioneuroma were equally distributed between males and females (1.13:1). A preference of thoracic (41.5%) and abdominal, nonadrenal tumors (37.5%) was observed compared with adrenal GN (21%). At diagnosis, thoracic tumors appeared larger than nonthoracic ones. Local lymph node metastases occurred in two patients. One ganglioneuroma had metastasized to soft tissues. (123)I-mIBG scintigraphy detected mIBG uptake at tumor site in 57% of the GN tumors. Levels of catecholamines in plasma and/or urine were increased in 39%. Slight immaturity of ganglion cells was observed in 93% of all ganglioneuroma tumors. None of the 22 tumors analyzed exhibited MYCN gene alterations. Although 12 patients had macroscopic residuals, no tumor progression or recurrence was observed in a median follow-up of 25 months. Ganglioneuroma may present with metabolic activity such as increased secretion of catecholamines and/or mIBG uptake. There are no specific diagnostic signs or symptoms discriminating ganglioneuroma and neuroblastoma tumors. Therefore, ganglioneuroma requires tissue investigation for diagnosis. Prognosis after surgical resection without further therapy seems to be excellent. Copyright 2001 American Cancer Society.
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            Thoracolumbar paravertebral giant ganglioneuroma and scoliosis: a case report and literature review

            Paravertebral ganglioneuroma and scoliosis is a rare clinical benign disease. The case we reported is about a 12-year-old girl who was hospitalized due to neoplasm with spinal deformity in the right abdomen for 1 month. Based on a careful preoperative evaluation and found no obvious surgery contraindications, the patient was treated with surgical resection of the tumor and correction of the deformity by surgery. Postoperative pathologic examination confirmed it was a ganglioneuroma. After the operation, the patient recovered well. Her spinal deformity was corrected, and she was 5 cm taller. Complete resection of ganglioneuroma following with a low recurrence rate and a good prognosis, patient does not need further chemotherapy, radiation therapy, or other treatments. All follow-up radiographic studies demonstrated no relapse of the tumor in the following 18 months. Combining this case with similar cases at home and aboard and reviewing related literature, we formed conclusions based on the manifestations, diagnosis, treatment, and prognosis of this disease and provided treatments for similar cases.
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              Pediatric intramedullary spinal cord tumors: is surgery alone enough?

              Pediatric intramedullary spinal cord tumors occur infrequently with limited data on treatment strategies and outcome. Over an 11-year period, 44 patients with such tumors who were primarily managed by radical surgery were retrospectively reviewed. The majority of tumors (42/44, 95%) were in the cervical or thoracic spine and were histologically low grade. Gross total resection was achieved in 73% (32/44). At a median follow-up of 45 months, 11% (5/44) had severe weakness (modified McCormick scale IV-V), 66% (29/44) had mild-moderate deficits (I-III), and 16% (7/44) had sphincter dysfunction. Tumor recurrence rate was 18% (8/44) after a mean period of 54 months. Age, number of involved spinal cord segments and extent of surgical resection did not significantly impact on tumor recurrence rates (p = 0.28, 0.44 and 0.13, respectively). The only significant prognostic factor was histological type; mixed or higher grade tumors having a higher recurrence rate (p = 0.04). Radical surgery for intraspinal tumors can achieve long tumor-free survival without requiring adjuvant therapy.
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                Author and article information

                Journal
                SICOT J
                SICOT J
                sicotj
                SICOT-J
                EDP Sciences
                2426-8887
                2020
                07 May 2020
                : 6
                : ( publisher-idID: sicotj/2020/01 )
                : 12
                Affiliations
                [1 ] Department of Orthopedic and Trauma Surgery, Assiut University Hospitals 71111 Assiut Egypt
                [2 ] Department of Spine Surgery, Helios Klinikum Erfurt 99089 Erfurt Germany
                [3 ] Department of Cardiothoracic surgery, Assiut University Hospitals 71111 Assiut Egypt
                Author notes
                [* ]Corresponding author: belalelnady83@ 123456gmail.com
                Article
                sicotj200006 10.1051/sicotj/2020012
                10.1051/sicotj/2020012
                7204781
                32378512
                37ad3372-e038-4276-8663-5e232df8838e
                © The Authors, published by EDP Sciences, 2020

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 07 January 2020
                : 17 April 2020
                Page count
                Figures: 5, Tables: 0, Equations: 0, References: 11, Pages: 5
                Categories
                Spine
                Case Report

                giant ganglioneuroma,ganglioneuroma with scoliosis,spinal ganglioneuroma,posterior approach

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