Articles published on Ganglioneuroma
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- Research Article
- 10.1002/dc.70016
- Dec 1, 2025
- Diagnostic cytopathology
- Shruti Gupta + 5 more
Ganglioneuromas (GNs) are the rare benign tumors belonging to the spectrum of the neuroblastic tumors and can be found anywhere across the distribution of the sympathetic nervous system, including the retroperitoneum. The majority of retroperitoneal GNs are asymptomatic and are diagnosed incidentally. We present a case of a retroperitoneal GN presenting as a palpable lumbar swelling, scoliosis, and inability to walk in a 6-year-old child, diagnosed on fine needle aspiration cytology (FNAC) and confirmed on histopathology. This case report discusses the rare presentation and challenges in FNAC diagnosis of these lesions.
- Abstract
- 10.1210/jendso/bvaf149.253
- Oct 22, 2025
- Journal of the Endocrine Society
- Gunjan Umarji + 4 more
Disclosure: G. Umarji: None. K. Desai: None. N.V. Kagita: None. M. Stezzi: None. S.A. Jabbour: None.Adrenal ganglioneuromas constitute <2% of all adrenal incidentalomas. Ganglioneuromas belong to the neuroblastic group of tumors. They originate from sympathetic ganglia, including the adrenal medulla, and are benign. A 23-year-old female was referred to endocrinology for a workup of an adrenal incidentaloma discovered during an emergency room visit for abdominal pain. CT scan of the abdomen with contrast reported a right 3.7 x 2.5 x 3 cm adrenal nodule without any further details. History was significant for intermittent palpitations, for which she reduced her caffeine intake. She denied sweating, pallor, easy bruising, weakness, and unintentional weight changes. Abdominal pain had resolved, which was thought to be from irritable bowel syndrome (IBS). Past medical history included class 3 obesity, cholelithiasis, migraine, and IBS. There was no history of hypertension and hypokalemia and no family history of adrenal disorders. Vital signs were BP 128/84 mmHg, HR 70 beats/min, and body mass index (BMI) 45.35 kg/m2. Physical examination was significant for moon facies, slight dorsocervical hump with no skin atrophy or wide violaceous striae. Hormonal workup was as follows: Plasma-free metanephrines - 10.7 pg/mL (0-88), plasma normetanephrine - 30.9 pg/mL (0-210.1), 24-hour urine-free cortisol - 36 microg/24h (6-42). Magnetic Resonance Imaging (MRI) of the abdomen with and without contrast showed 4.2 x 2.8 cm heterogenous R adrenal nodule, the posterior aspect of the mass demonstrated heterogenous increased T2-weighted signal posteriorly with mildly hypointense signal anteriorly. Opposed phase imaging was restricted due to respiratory motion artifact. The patient underwent a robotic-assisted laparoscopic radical right adrenalectomy. Pathology showed a 5.7 cm adrenal ganglioneuroma, extending beyond the adrenal into the peri-adrenal adipose tissue. Tumor cells were positive for S100, Neurofilament and focally for synaptophysin, predominantly in ganglion cells. Ganglioneuroma (GN) is a rare tumor comprised of ganglion cells and Schwannian stroma. 21% of GN can be seen in the adrenal glands. Adrenal GN are more commonly seen in females. They are hormonally silent but sometimes can produce catecholamines. The gold standard for diagnosis is histological features, including mature, large neuronal ganglion cells in a Schwannian stroma. Immunohistochemical staining is positive for S-100, vimentin, synaptophysin, and neuron-specific enolase. We present a fascinating case of adrenal incidentaloma in a young female that turned out to be an adrenal ganglioneuroma.Presentation: Monday, July 14, 2025
- Research Article
- 10.1186/s43054-025-00385-4
- Aug 8, 2025
- Egyptian Pediatric Association Gazette
- Asya Eylem Boztas + 2 more
Abstract Introduction Ganglioneuromas (GNs) are rare, benign tumors originating from undifferentiated neural crest cells, especially the autonomic nervous system (sympathetic). Ganglioneuromas, located in the gastrointestinal tract, are classified into three groups: single polypoid lesion, ganglioneuromatous polyposis, and the rarest, diffuse intestinal ganglioneuromatosis. In this report, we present the case of an 11-year-old girl who presented with complete distal intestinal obstruction and was diagnosed with isolated diffuse ganglioneuromatosis. Case Presentation An 11-year-old girl was consulted by our emergency department with complaints of not passing stool for 4 days, abdominal distension, and vomiting. She previously underwent a laparotomy for an acute abdomen three years prior. Severe malnutrition, anemia, and growth retardation were observed. Due to significant abdominal distention and shortness of breath, the patient was taken to surgery with a preliminary diagnosis of ileus. The cecum had a mass-like appearance and was immobile, adhering to the upper right quadrant and the lower corner of the ascending colon. The colon appeared to be unused. Milking of the ileal loops showed no passage through the cecum. A resection was performed, including 2 cm of ileum and an ascending colon, and an ileo-colic anastomosis was performed. The pathology report highlights hyperplasia of ganglion cells and nerve fibers. NSE and S-100 immunohistochemical staining were positive. It is interpreted as consistent with diffuse ganglioneuromatosis. The patient exhibited improvement in malnutrition status and correction of anemia. Genetic analysis has not revealed any mutations. Patient is followed up without complications. Conclusions Since it is a very rare entity, we aimed to draw attention to the importance of the diagnosis. Ileus is a relatively common surgical pathology that needs intervention. The diagnosis is very important, as it can be both a differential diagnosis of surgical pathologies and the first sign of possible related genetic syndromes.
- Research Article
1
- 10.24287/1726-1708-2020-19-4-133-142
- Jul 8, 2025
- Pediatric Hematology/Oncology and Immunopathology
- S B Babakhanova + 6 more
Ganglioneuroma (GN) represents a mature, well-differentiated tumor arising from the sympathetic nervous system. Mostly developing de novo, GN can appear during the treatment course of poorly differentiated or undifferentiated tumors of the sympathetic nervous system, such as neuroblastoma, or as a result of their spontaneous maturation. In this article we report three clinical cases of spontaneous and induced maturation of neuroblastoma (primary tumor and metastatic lesion) to GN. Histological verification of long-lasting stable or progressing residual tumor mases in patients with neuroblastoma stratified to the observation group plays a pivotal role as it may significantly affect the treatment course. The patients' parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.
- Research Article
- 10.3390/cancers17081303
- Apr 12, 2025
- Cancers
- Rebecca Rothe + 11 more
Background/Objectives: Peripheral neuroblastic tumors (pNT) are a biologically heterogeneous group of embryonal tumors that derive from the neural crest and affect the sympathetic nervous system. So far, little is known about the complex immune landscape in these rare childhood cancers. Methods: We focused on the immune cell infiltrate of treatment-naïve pNT from 24 patients, including high-risk neuroblastoma (HR-NBL), non-high-risk neuroblastoma (NHR-NBL), ganglioneuroblastoma (GNBL), and rare ganglioneuroma (GN). To gain novel insights into the immune architecture of these pNT subtypes, we used multiplex immunohistochemistry, multispectral imaging, and algorithm-based data evaluation to detect and characterize T cells, B cells, neutrophils, macrophages, and tertiary lymphoid structures (TLS). Results: The majority of the investigated tumor-infiltrating immune cells were macrophages and T cells. Their detailed phenotypic characterization revealed high proportions of M2-like macrophages as well as activated GrzB+ CD8+ and PD-1+ T lymphocytes. Proportions of these T cell phenotypes were significantly increased in GN compared to HR-NBL, NHR-NBL, or GNBL. In addition, TLS occurred in 11 of 24 patients, independent of immune cell frequencies in the whole tissues. Interestingly, all GN, most GNBL, but only a few NBL contained TLS. We distinguished between three TLS maturation stages that were present irrespective of the pNT subtype. The majority belonged to mature TLS of the primary follicle state. Mature LAMP3+ dendritic cells were also found, predominantly in T cell zones of TLS. Furthermore, TLS presence identified pNT patients with significantly prolonged progression-free survival in contrast to all other analyzed immunological features. Conclusions: We propose TLS to be a potential prognostic marker for pNT to predict patient outcomes.
- Research Article
- 10.14341/serg12995
- Mar 13, 2025
- Endocrine Surgery
- M M Gadzhimuradova + 10 more
Ganglioneuromas (GN) are rare benign neoplasms originating from neural crest tissue. These tumors are typically asymptomatic and are often detected incidentally during diagnostic imaging studies. Although the radiological characteristics of adrenal GNs are relatively well-studied, establishing an accurate diagnosis still remains a challenging task. Currently, the definitive diagnosis is established based on the morphological examination of postoperative material.This publication presents a rare clinical case of an adrenal GN identified in a 35-year-old male. А mass in the right adrenal gland measuring 8.4×5.8×5.8 cm with a malignant CT phenotype and indirect signs of possible invasion was detected at an abdominal MSCT scan. Hormonal evaluation showed no evidence of excessive hormone secretion. Taking into account the set of characteristics of the tumor, which indicated a high risk of malignancy, the patient underwent surgical treatment, including thoracophrenolaparotomy and right adrenalectomy. Morphological examination revealed that the tumor consisted of interwoven bundles of mature Schwann cells with ganglion cell inclusions confirming the diagnosis of GN. The postoperative period was uneventful.This clinical case highlights the difficulty in preoperative diagnosis of GN and underscores the need for comprehensive and thorough evaluation using all available diagnostic methods.
- Research Article
3
- 10.1186/s12967-025-06298-5
- Feb 26, 2025
- Journal of Translational Medicine
- Zan Liu + 6 more
BackgroundNeuroblastoma (NB) is the most common solid tumor in children, characterized by high recurrence rates, drug resistance, and significant mortality.MethodsIn this study, we analyzed the proteomic profiles of NB tissue samples alongside other pathological categories, including ganglioneuroma (GN) and ganglioneuroblastoma (GNB). Using weighted gene co-expression network analysis (WGCNA), the core prognostic gene models associated with histopathology of NB were identified. Furthermore, by mapping our core prognostic gene models onto drug-perturbed transcriptome profiles from the L1000FWD and CMap databases, repurposing drug candidates were screened and validated for NB.ResultsOur proteomic analysis reveals that pathways associated with the cell cycle and DNA replication are significantly upregulated in NB, while oxidative phosphorylation, pyruvate metabolism, and the TCA cycle are notably downregulated compared to GNB and GN. By applying WGCNA, we identified a core prognostic gene model strongly associated with the unfavorable subtype and high MKI of NB and primarily related to chromatin binding and mRNA metabolic process. Protein–protein interaction network analysis identified 15 hub genes in this core prognostic module: SMARCA4, SMARCA5, SMARCC2, SMARCC1, PBRM1, BRD3, ARID1A, BRD2, ARID1B, KDM1A, TP53BP1, ALYREF, CBX1, SF3B1, and ADNP, which mainly related to chromatin remodeling. Notably, SMARCA4 and ALYREF are also high-risk genes of mortality and validated as potential prognostic biomarkers for NB. Through repurposing drugs screening, mocetinostat and clofarabine were validated as effective treatments in two NB cell lines.ConclusionMocetinostat and clofarabine offer valuable insights for the development of novel targeted therapies in neuroblastoma.
- Research Article
1
- 10.1210/jendso/bvaf036
- Feb 26, 2025
- Journal of the Endocrine Society
- Shubham Agarwal + 9 more
Cells derived from neural crest populate several organs. A particular precursor cell, sympathogonia, gives rise to pheochromoblasts and neuroblasts. Due to common origin, tumors originating from pheochromoblasts, such as pheochromocytoma (PHEO) and paraganglioma (PGL), may rarely coexist with ganglioneuroma (GN). We evaluated clinical, biochemical, and radiological characteristics of patients with composite PHEO/PGL and GN (PPGL-GN) and compared them to patients with PHEO. In this retrospective, dual-center, observational, case-control study, we identified patients with PPGL-GN. Similarly, we identified a control group of patients with PHEO who underwent laparoscopic adrenalectomy. All diagnoses were confirmed on histology. Descriptive statistics were used to summarize demographic and clinical data. We identified 19 consecutive patients with PPGL-GN and 86 patients with PHEO. Patients with PPGL-GN, compared to those with PHEO, were younger (aged 46.0 vs 50.8 years; P = .03), had higher rate of underlying genetic disorders (47.4% vs 23.2%; P = .03), and had fewer functioning tumors (89.5% vs 100%; P = .002). There was no difference in the median radiological tumor size or the precontrast computed tomography density. Disease recurrence (at another site) was noted in 15.8% of PPGL-GN patients who had a median follow up of 14.6 months, as opposed to no disease recurrence in patients with PHEO. There was no documented recurrence at the tumor bed and no metastasis in both groups. Patients with PPGL-GN were younger and had a higher occurrence of underlying genetic disorders compared to PHEO. However, PPGL-GN was radiologically indistinguishable from PHEO. The higher observed disease recurrence of PPGL-GN reinforces vigilant postoperative follow-up.
- Research Article
- 10.3389/fsurg.2025.1685442
- Jan 1, 2025
- Frontiers in Surgery
- Haining Zheng + 6 more
ObjectiveThis study aims to develop and internally validate a multivariable logistic regression model and a simplified scoring system, based on standardized ultrasonographic features, for the preoperative differentiation of retroperitoneal ganglioneuroma (GN) from schwannoma (SW), and to evaluate their discrimination, calibration, and clinical utility.MethodsWe retrospectively included patients with retroperitoneal GN or SW confirmed by surgical pathology. Standardized ultrasonographic features were extracted, and candidate predictors were selected using least absolute shrinkage and selection operator (LASSO) regression, while retaining potential confounders (age, sex, lesion long diameter). A multivariable model was constructed, and a six-variable simplified score was derived. Discrimination [area under the curve (AUC)], calibration (intercept, slope, Brier score), and decision curve analysis (DCA) were evaluated using stratified fivefold cross-validation and bootstrap resampling (B = 2,000). Two task-oriented thresholds were predefined: R1 [rule-out, sensitivity (Se) ≥ 0.95] and S1 [standard diagnosis, specificity (Sp) ≥ 0.50].ResultsA total of 74 patients were included (GN, 25, 33.8%; SW, 49, 66.2%). After optimism correction, the multivariable model achieved an AUC of 0.930, and the simplified score achieved an AUC of 0.917. Independent predictors included pelvic extraperitoneal location (loc_pelvic = 1), absence of cystic/necrotic change, and lower SD/LD ratio. For R1, the model threshold of 0.149 yielded Se = 0.960, Sp = 0.837, and negative predictive value (NPV) = 0.976; the score threshold of 0.206 yielded Se = 1.000, Sp = 0.592, and NPV = 1.000. For S1, the model threshold of 0.426 yielded Se = 0.920 and Sp = 0.939, and the score threshold of 0.594 yielded Se = 0.760 and Sp = 0.918.ConclusionBoth the multivariable model and the simplified score demonstrated excellent performance in differentiating GN from SW, suggesting potential value as rapid, interpretable tools for bedside use and in resource-limited settings. Their clinical utility should be confirmed through external validation and recalibration in multicenter, prospective cohorts and further enhanced through integration with multimodal imaging such as CT, MRI, and contrast-enhanced ultrasound (CEUS).
- Research Article
- 10.1002/pbc.31445
- Nov 24, 2024
- Pediatric blood & cancer
- Katherine M Burnand + 32 more
Ganglioneuroblastoma intermixed (GNBi) and ganglioneuroma (GN) represent benign variants of peripheral neuroblastic tumours. While historically surgical resection was recommended, watchful active observation has become the accepted management for a subset of patients. To review clinical features, biology, natural history and management of a retrospective UK CCLG study cohort of GN and GNBi, and compare outcomes of patients treated with surgical resection or watchful active observation. Retrospective review of histologically confirmed non-metastatic GN and GNBi diagnosed between 1990 and 2020. A total of 259 patients were identified (163 GN, 93 GNBi, median age 62months). In all 201 (78%) had initial surgery and 58 (22%) were observed. Overall survival was 98%. Twenty-one of 58 observed (36%) required subsequent surgery due to progressive tumour growth (52%). Gross total resection (GTR) was achieved in 79% of patients with a 19% complication rate. GTR was obtained in a similar proportion of patients having initial surgery (65%) and delayed surgery (76%). Patients obtaining GTR were more likely to have complete symptom(s) control (43% vs. 24%). In 45 cases (39%), surgical pathology was different from pathology at biopsy, and in 14 (12%) the pathology changed from GN/GNBi to neuroblastoma/GNB nodular. Watchful active observation can be a safe approach, with surgical resection reserved for symptomatic patients. However, a small proportion of patients in the observation group had their diagnosis revised to malignant at surgery. Careful assessment of surgical risk and expected benefits, after considering an initial period of observation, will allow clinicians to make optimal decisions for patients and their families.
- Research Article
2
- 10.3390/jcm13226660
- Nov 6, 2024
- Journal of clinical medicine
- Domenica Tambasco + 8 more
Purpose: To determine the diagnostic accuracy of quantitative diffusion-weighted (DW) MRI apparent diffusion coefficient (ADC) and tumour volumes to differentiate between malignant (neuroblastoma (NB)) and benign types of neuroblastic tumours (ganglioneuroma (GN) and ganglioneuroblastoma (GNB)) using different region-of-interest (ROI) sizes. Materials and Methods: This single-centre retrospective study included malignant and benign paediatric neuroblastic tumours that had undergone DW MRI at diagnosis. The outcome was diagnostic accuracy of the tumour volume from structural and ADC DW MRI, in comparison to histopathology (reference standard). Results: Data from 40 patients (NB, n = 24; GNB, n = 6; GN, n = 10), 18 (45%) females and 22 (55%) males, with a median age at diagnosis of 21 months (NB), 64 months (GNB), and 133 months (GN), respectively, ranging from 0 to 193 months, were evaluated. The area under the receiver operating characteristic (AUROC) curve for ADC for discriminating between neuroblastic tumours' histopathology for a small ROI was 0.86 (95% CI: 0.75-0.98), and for a large ROI, 0.83 (95% CI: 0.71-0.96). An ADC cut-off value of 1.06 × 10-3 mm2/s was able to distinguish malignant from benign tumours with 83% (68-98%) sensitivity and 75% (95% CI: 54-98%) specificity. Tumour volume was not indicative of malignant vs. benign tumour diagnosis. Conclusions: In this study, both small and large ROIs used to derive ADC DW MRI metrics demonstrated high accuracy to differentiate malignant from benign neuroblastic tumours, with the ADC AUROC for the averaged multiple small ROIs being slightly greater than that of large ROIs, but with overlapping 95% CIs. This should be taken into consideration for standardisation of ROI-related data analysis by international initiatives.
- Research Article
1
- 10.1097/ju9.0000000000000227
- Nov 1, 2024
- JU Open Plus
- Ibrahim A Khalil + 6 more
Introduction: Ganglioneuromas (GNs) are rare, differentiated tumors that originate from neural crest cells and can occasionally develop in the adrenal medulla. Adrenal GNs (AGNs) are typically hormonally silent and asymptomatic. Hormone-secreting pure AGNs in adults are uncommon; however, dopamine-secreting pure AGNs are extremely rare, with only a few cases reported in the literature. Giant AGNs are usually managed with open surgical intervention. Case Presentation: We present a case of a giant dopamine-secreting pure AGN in a 19-year-old woman who complained of mild left flank pain with no other symptoms. On physical examination, a mass was felt in left upper abdomen. MRI showed a left retroperitoneal mass extending from the left suprarenal region inferiorly, anterior to and compressing the left kidney, measuring 9 × 7.5 × 14 cm. Hormonal investigations ruled out Cushing syndrome, and catecholamine studies were negative for metanephrines, normetanephrines, adrenaline, and noradrenaline but were positive for dopamine levels more than 3 times the upper normal level. The patient underwent robotic-assisted adrenalectomy with minimal morbidity. Conclusion: Robotic-assisted adrenalectomy is a feasible and effective treatment option for rare dopamine-secreting pure AGNs. Careful patient selection and thorough preoperative preparation are essential to minimize risks and ensure favorable outcomes.
- Research Article
3
- 10.1186/s40170-024-00358-y
- Oct 24, 2024
- Cancer & Metabolism
- Arsenij Ustjanzew + 5 more
BackgroundGlycosphingolipids (GSLs) are membrane lipids composed of a ceramide backbone linked to a glycan moiety. Ganglioside biosynthesis is a part of the GSL metabolism, which involves sequential reactions catalyzed by specific enzymes that in part have a poor substrate specificity. GSLs are deregulated in cancer, thus playing a role as potential biomarkers for personalized therapy or subtype classification. However, the analysis of GSL profiles is complex and requires dedicated technologies, that are currently not included in the commonly utilized high-throughput assays adopted in contexts such as molecular tumor boards.MethodsIn this study, we developed a method to discriminate the enzyme activity among the four series of the ganglioside metabolism pathway by incorporating transcriptome data and topological information of the metabolic network. We introduced three adjustment options for reaction activity scores (RAS) and demonstrated their application in both exploratory and comparative analyses by applying the method on neuroblastic tumors (NTs), encompassing neuroblastoma (NB), ganglioneuroblastoma (GNB), and ganglioneuroma (GN). Furthermore, we interpreted the results in the context of earlier published GSL measurements in the same tumors.ResultsBy adjusting RAS values using a weighting scheme based on network topology and transition probabilities (TPs), the individual series of ganglioside metabolism can be differentiated, enabling a refined analysis of the GSL profile in NT entities. Notably, the adjustment method we propose reveals the differential engagement of the ganglioside series between NB and GNB. Moreover, MYCN gene expression, a well-known prognostic marker in NTs, appears to correlate with the expression of therapeutically relevant gangliosides, such as GD2. Using unsupervised learning, we identified subclusters within NB based on altered GSL metabolism.ConclusionOur study demonstrates the utility of adjusting RAS values in discriminating ganglioside metabolism subtypes, highlighting the potential for identifying novel cancer subgroups based on sphingolipid profiles. These findings contribute to a better understanding of ganglioside dysregulation in NT and may have implications for stratification and targeted therapeutic strategies in these tumors and other tumor entities with a deregulated GSL metabolism.
- Research Article
1
- 10.3390/cancers16203492
- Oct 15, 2024
- Cancers
- Angela Gasparini + 4 more
Introduction: Ganglioneuroma (GN) is a rare, benign tumor of the autonomic nervous system. It is seldom located in the head and neck (HN) region. GN typically presents as a slow-growing, painless mass, often leading to delayed diagnosis. Case report: We report a unique case of laryngeal-originating GN in a 43-year-old female who presented with worsening dysphonia and dyspnea. Imaging revealed a large mass originating from the larynx. Histological examination confirmed the diagnosis. The tumor was surgically excised with preservation of key structures in the neck. At the follow-up, the patient experienced a significant improvement in symptoms. Material and methods: A systematic literature review following PRISMA guidelines was conducted in January 2024 to investigate the common sites of GN in the HN region and the complications associated with its treatment. Results: In a total of 58 articles, we studied 65 patients, mostly under 30 years old. Surgical excision remains the primary treatment, and post-operative complications were mostly neurological. Discussion: GNs are generally slow-growing and asymptomatic, but they can reveal themselves when compressing nearby structures, especially in the HN region. In symptomatic cases or when the tumor exhibits significant growth or hormonal activity, surgical resection is required. The lateral cervical approach is the most common one. The risk of postoperative complications and recurrence underscores the need for careful surgical planning and long-term follow-up. Conclusion: This unique laryngeal GN case highlights the importance of considering GN in the differential diagnosis of HN masses. Further large-scale studies are warranted to establish evidence-based protocols for their management, especially in the HN region.
- Research Article
- 10.1007/s12070-024-05013-y
- Sep 16, 2024
- Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
- Areena Anjum + 3 more
Ganglioneuromas (GN) are benign, slow-growing, non-invasive, and well differentiated neoplasms of neuroblastic origin. We reported a 4 year old girl who presented with a left lateral slow growing cervical neck mass. After surgical excision the tumour was sent for histopathological examination which confirmed ganglioneuroma. Ganglioneuromas should be accounted as the differential diagnosis of pediatric soft tissue tumours of the neck.
- Research Article
3
- 10.4103/ijpm.ijpm_618_23
- Jun 20, 2024
- Indian journal of pathology & microbiology
- Xia Wei + 2 more
The aim of this study was to explore the correlation between the expression of GD2 and GD3 and the histopathological types, risk groups, and chemotherapy in peripheral neuroblastic tumors (pNTs) and provide a theoretical basis for the selection of immunotargeted therapy for pNTs. The expression of GD2 and GD3 in samples of pNTs in all 87 cases, including 39 neuroblastomas (NB), 13 ganglion neuroblastomas nodular (GNBn), 19 ganglion neuroblastomas intermixed (GNBi), 16 ganglioneuroma (GN), and 16 paired NB after chemotherapy, were detected by immunohistochemistry (IHC). SPSS 20.0 statistical software was used for statistical analysis, and P < 0.05 was considered statistically significant. The expression of GD2 was higher than that of GD3 ( P < 0.001) in all samples. In NB and GNBn, the expression of GD2 was higher than that of GD3 ( P < 0.001 and P = 0.02, respectively). The expression of GD2 in NB was higher than that in GNBn ( P = 0.015), and GNBn was higher than GNBi ( P < 0.001). The expression of GD2 in the high-risk group was significantly higher than that in the medium-risk group and low-risk group ( P = 0.019). The expression of GD2 before chemotherapy was higher than that after chemotherapy ( P = 0.022). GD2 was expressed in different degrees in tumor-infiltrating lymphocytes. GD2 may be better than GD3 as a target of immunotherapy for pNTs, especially in the same pathological type. NB and GNBn may be more suitable for anti-GD2 immunotherapy. The expression of GD2 on tumor-infiltrating lymphocytes may be related to the side effects of anti-GD2 immunotherapy.
- Research Article
- 10.3171/2024.2.focvid23210
- Apr 1, 2024
- Neurosurgical Focus: Video
- Enrico Giordan + 4 more
Ganglioneuroma (GN) is a rare solid neoplasm developing from neural crest cells of sympathetic ganglia or adrenal medulla. It usually presents as an asymptomatic mass in the retroperitoneal space and mediastinum. Resection through open surgery or minimal access is recommended. The video illustrates the case of a 23-year-old female with an incidental finding of thoracic GN. The authors performed a combined, staged approach to ensure complete resection, which involved unilateral T3-4 biportal endoscopy (UBE) for rhizotomy and nerve root decompression, followed by video-assisted thoracoscopic surgery (VATS) for complete excision. The procedure was uneventful, with full recovery and no postoperative complications. The video can be found here: https://stream.cadmore.media/r10.3171/2024.2.FOCVID23210.
- Research Article
- 10.38175/phnx.1300923
- Jan 22, 2024
- Phoenix Medical Journal
- Nihal Ki̇remi̇t + 4 more
Ganglioneuroma (GN) is a rare benign, well-differentiated neoplasia originating from the neural crest. Although it is most commonly seen in the posterior mediastinum, it can be observed in many areas including the adrenal gland. Lesions located in the posterior mediastinum and retroperitoneum are mostly seen in the pediatric population and adrenal ganglioneuromas are more common in the 4-5. decade. GN is a benign neoplasia but very rarely lymph node and distant organ metastases have been reported. In this study, a case of adrenal gland ganglioneuroma showing lymph node metastasis in a 3-year-old male patient is presented.
- Research Article
- 10.51271/jocs-0023
- Nov 30, 2023
- Journal of Comprehensive Surgery
- Hilal Yaşar + 5 more
Ganglioneuromas (GN) are rare benign tumors that typically occur in the posterior mediastinum or retroperitoneal region, but can also be found in adrenal glands. Up to 50% of patients are asymptomatic, but symptoms can arise from the mass effect or excessive secretion of catecholamines. Careful evaluation is necessary to differentiate GNs from other adrenal tumors. A 19-year-old female patient with no previous comorbidities was diagnosed with a right adrenal incidentaloma. Physical examination and routine laboratory tests were normal. Imaging revealed a solid lesion in the right adrenal gland, and excisional adrenalectomy was performed. Pathological examination confirmed the diagnosis of a right adrenal GN. The patient had an uneventful recovery and showed no recurrence during the follow-up period. Conclusion: After surgical removal of a benign neurogenic tumor, our patient had no tumor recurrence after five months, indicating a positive prognosis. However, long-term follow-up is still recommended because of reported cases of metastases and recurrence in some patients.
- Research Article
2
- 10.1016/j.crad.2023.08.022
- Sep 20, 2023
- Clinical Radiology
- Z Lin + 1 more
Preoperative differentiation of mediastinum and retroperitoneum ganglioneuroma from schwannoma with clinical data and enhanced CT: developing a multivariable prediction model