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Last update 08 May 2025

Ganglioneuroblastoma

Last update 08 May 2025

Basic Info

Synonyms

Ganglioneuroblastoma, Ganglioneuroblastoma (disorder), Ganglioneuroblastoma (morphologic abnormality)

+ [13]

Introduction

A moderately malignant neoplasm composed of primitive neuroectodermal cells dispersed in myxomatous or fibrous stroma intermixed with mature ganglion cells. It may undergo transformation into a neuroblastoma. It arises from the sympathetic trunk or less frequently from the adrenal medulla, cerebral cortex, and other locations. Cervical ganglioneuroblastomas may be associated with HORNER SYNDROME and the tumor may occasionally secrete vasoactive intestinal peptide, resulting in chronic diarrhea.

Drugs associated with Ganglioneuroblastoma

Dinutuximab

Target

GD2

Mechanism

GD2 inhibitors [+2]

Active Org.

National Cancer Institute [+2]

Originator Org.

National Cancer Institute

Active Indication

Neuroblastoma [+7]

Inactive Indication

Recurrent Lung Small Cell Carcinoma

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

United States

First Approval Date10 Mar 2015

Temozolomide

Target

DNA

Mechanism

DNA inhibitors [+1]

Active Org.

National Cancer Institute [+18]

Originator Org.

Merck Sharp & Dohme Corp.

Active Indication

Ewing Sarcoma [+81]

Inactive Indication

Acoustic Neuroma [+101]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

European Union [+3]

First Approval Date26 Jan 1999

Cyclophosphamide

Target

DNA

Mechanism

DNA inhibitors

Active Org.

Shionogi & Co., Ltd. [+36]

Originator Org.

Baxter International, Inc.

Active Indication

Graft vs Host Disease [+238]

Inactive Indication

Acute Erythroblastic Leukemia [+207]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

United States

First Approval Date16 Nov 1959

Clinical Trials associated with Ganglioneuroblastoma

NCT06858501

/ Not yet recruitingPhase 2IIT

Concordance in Response Assessment Incorporating Meta-Iodobenzylguanidine (123I-MIBG) and Meta-[18F]Fluorobenzylguanidine (18F-MFBG, IND#146379, NSC#853868) Imaging in Neuroblastoma

This phase II trial evaluates whether an investigational scan (18F-MFBG positron emission tomography [PET]/computed tomography [CT] or PET/magnetic resonance imaging [MRI]) can accurately detect tumors in patients with newly diagnosed, high-risk neuroblastoma as well as standard of care imaging with 123 I-MIBG. 18F-MFBG is a radioactive diagnostic agent that is injected into a vein and taken up by tumor cells. The cells can then be visualized using PET/CT or PET/MRI scans. A PET scan uses radioactive material injected into the blood to show the internal workings of the body. A CT scan uses x-rays and a computer to produce a 3-dimensional image of the body. MRI uses radiofrequency waves and a strong magnetic field rather than x-rays to provide clear and detailed pictures of internal organs and tissues. Combining PET with CT or MRI may help doctors better understand the extent and the exact location of disease. Diagnostic procedures, such as 18F-MFBG PET/CT or PET/MRI, may detect tumors as well as or better than the current standard imaging with 123 I-MIBG in patients with newly diagnosed, high-risk neuroblastoma.

Start Date20 Jun 2025

Sponsor / Collaborator

The Children's Oncology Group Foundation, Inc.

NCT06798532

/ Not yet recruitingNot ApplicableIIT

Interest of Preoperative Arteriography to Identify the Adamkiewicz Artery Before Surgery for Basi-thoracic Neuroblastic Tumors

Neuroblastic tumors (NBTs) develop from neural crest cells that give rise to the sympathetic nervous system. They include neuroblastomas, ganglioneuroblastomas, and ganglioneuromas. They represent approximately 10% of solid tumors in children under 15 years of age. In 15 to 20% of cases, NBTs are located in the thoracic region. These paravertebral tumors have an extracanal component and some also have an intraspinal component (dumbbell tumors) that can cause spinal cord compression. Surgery for these tumors also exposes the patient to neurological complications. In the thorax, the basi-thoracic location (T9-T12) may be particularly at risk due to the presence of the artery of Adamkiewicz (AKA), which supplies blood to the spinal cord; damage to this artery can result in spinal cord ischemia. To avoid this scenario, some teams recommend performing spinal cord arteriography to identify AKA. However, many centers do not perform arteriography and do not report more postoperative complications.
Currently, there is no consensus on the indications for performing preoperative spinal arteriography in patients undergoing surgery for basi-thoracic NBT.
This study evaluates the practice in France of preoperative arteriography to identify the AKA among patients undergoing surgery for basi-thoracic neuroblastic tumors and analyzes the incidence of postoperative neurological complications in these patients.

Start Date01 Mar 2025

Sponsor / Collaborator

Assistance Publique des Hôpitaux de Paris SA

NCT06450041

/ RecruitingPhase 2IIT

Phase II Study of Ex-Vivo Expanded Allogeneic Universal Donor TGFβi NK Cell Infusions in Combination With Temozolomide, Irinotecan, Dinutuximab, and Sargramostim in Patients With Relapsed or Refractory Neuroblastoma The STING (Sequential Temozolomide, Irinotecan, NK Cells and GD2 mAb) Trial

This is a phase II study looking at patient response to treatment with the combination dinutuximab, temozolomide, irinotecan, and GM-CSF.

Start Date16 Dec 2024

Sponsor / Collaborator

United Therapeutics Corp.

[+1]

100 Clinical Results associated with Ganglioneuroblastoma

100 Translational Medicine associated with Ganglioneuroblastoma

0 Patents (Medical) associated with Ganglioneuroblastoma

1,010

Literatures (Medical) associated with Ganglioneuroblastoma

01 May 2025·Clinical Radiology

Radiomics analysis based on 18F-fluorodeoxyglucose positron emission tomography/computed tomography for differentiating the histological classification of peripheral neuroblastic tumours

Article

Author: Yang, J ; Feng, L ; Zhang, H ; Yao, X ; Wang, W ; Wang, C

AIMTo create a radiomics nomogram based on ¹⁸F-fluorodeoxyglucose positron emission tomography/computed tomography and assess the efficacy of this nomogram in differentiating ganglioneuroblastoma from neuroblastoma in peripheral neuroblastic tumours (PNTs).MATERIALS AND METHODSOne hundred and ninety-nine patients with PNTs were retrospectively included, including 115 neuroblastoma patients and 84 ganglioneuroblastoma patients, who were randomly split into the training and test sets according to a ratio of 7:3. The 3D slicer was used to delineate the primary tumour, then radiomics features were extracted and selected, and a radiomics model was built using the optimal radiomics features. The clinical model was constructed from independent clinical risk factors. A radiomics nomogram was developed by multivariate logistic regression analysis incorporating independent clinical risk factors and radiomics features. Model performance was assessed using receiver operating characteristic curves and decision curve analysis.RESULTSThe radiomics model based on the selection of 14 radiomics features was developed. The clinical model was constructed by combining age at diagnosis and 1p aberrations. The radiomics nomogram demonstrated the optimal diagnostic value in distinguishing between ganglioneuroblastoma and neuroblastoma, with an area under the receiver operating characteristic curve of 0.857 and 0.795 in the training and test sets, respectively. The decision curve analysis and calibration curves also showed good performance for the nomogram.CONCLUSIONSThe radiomics nomogram could improve diagnostic performance by differentiating the histological classification of PNTs.

24 Mar 2025·Journal of Neurosurgery: Case Lessons

Stereotactic radiosurgery for brain metastases secondary to adrenal ganglioneuroblastoma: illustrative case

Article

Author: Harary, Paul M. ; Cardona, Juan J. ; Tayag, Armine ; Chang, Steven D. ; Park, David J. ; Hori, Yusuke S. ; Ustrzynski, Louisa ; Persad, Amit R. L. ; Emrich, Sara C.

BACKGROUNDNeuroblastic tumors, including malignant neuroblastoma, ganglioneuroblastoma (GNB), and benign ganglioneuroma, are embryonal tumors derived from neural crest cells. GNB often metastasizes to the bone marrow and bone, with lung and brain metastases (BMs) remaining extremely rare. To date, only 2 prior reports of GNB BM exist, with both cases documenting resection alone. Therefore, the management of GNB BM has yet to be fully investigated.OBSERVATIONSThe present study describes an 11-year-old male with BM secondary to primary adrenal GNB and the use of stereotactic radiosurgery (SRS) to treat these lesions. These are both without precedent in the current literature. The patient initially underwent 2 surgical interventions, with the second surgery performed due to local recurrence. He subsequently received 3 rounds of SRS treatment with favorable short-term outcomes, including complete resolution of the relatively large left temporal lesion.LESSONSA primary surgical approach enabled pathological analysis and contributed to initial prolonged survival. However, a second surgery was required due to local progression. Subsequent SRS showed favorable outcomes after the 3 interventions without impairing the clinical course with systemic therapy. Comparative studies are needed to optimize the treatment strategy between surgical management and SRS as stand-alone or adjuvant therapy.

https://thejns.org/doi/10.3171/CASE24820

01 Feb 2025·Journal of Surgical Research

A Single-Institution Analysis of Surveillance Practice for Low-Risk Neuroblastic Tumors

Article

Author: Shah, Nilay ; Schwartz, Dana ; Mallampalli, Grace ; Bricker, Josh ; Mansfield, Sara A ; Streby, Keri ; Srivatsa, Shachi ; English, Jennifer ; Cole, Lindsay ; Aldrink, Jennifer H ; Nicol, Kathleen ; Ranalli, Mark

INTRODUCTIONCurrently, few prospective guidelines exist for the surveillance of children with low-risk neuroblastic tumors (LRNBTs), including ganglioneuroma or ganglioneuroblastoma intermixed. This study aims to describe our institutional approach to LRNBT surveillance following surgical resection or nonoperative management. We hypothesize that length of surveillance can be reduced due to low recurrence risk.METHODSWe conducted a retrospective review of patients diagnosed with LRNBT at our institution between January 1, 2010, and December 31, 2022. The majority of patients were followed in our multidisciplinary surgical oncology clinic. We summarized relevant patient data including follow-up imaging modalities and recurrences.RESULTSThirty-eight patients met the inclusion criteria at a median age of diagnosis of 9 ys (interquartile range [IQR]: 5, 12.75). Thirty-seven patients underwent surgical resection, with tumors located in the posterior mediastinum (n = 18), retroperitoneum (n = 14), presacral space (n = 3), pelvis (n = 1), or combined thoracic or retroperitoneal location (n = 1). One patient underwent nonoperative management for a tumor located in the retroperitoneum. Histopathological results demonstrated 18 cases of ganglioneuroma and 20 cases of ganglioneuroblastoma intermixed. The median duration of postoperative follow-up was 27 mos (IQR: 14, 51) and the median clinical visits per year was 2.6 [IQR: 1.5, 3.4). Surveillance imaging was performed at a median frequency of 2.4 times per year (IQR: 1.6, 3.1). Chest radiographs were the most common modality used for thoracic locations (34% of all imaging) and ultrasound was used most commonly for abdominal location (24% of all imaging). During surveillance, one recurrent or residual ganglioneuroma was identified on magnetic resonance imaging at 6.7 mos from presacral resection. This patient underwent repeat surgical resection, and subsequent surveillance demonstrated no further recurrences.CONCLUSIONSRecurrence was low in this cohort of LRNBT patients. Optimal surveillance strategies for this low-risk pathology would minimize radiation exposure and burden of health-care visits to patients. Further multi-institutional studies are warranted to determine the best imaging modality, as well as the frequency and duration of follow-up visits for LRNBT patients. Future studies should evaluate the acceptability of short-term surveillance for those who achieve complete resection.

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Ganglioneuroblastoma

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