[Surgically Resected Cases of Mediastinal Ganglioneuroma Detected in Adults].

A ganglioneuroma is a rare, benign, neurogenic tumor originating from the sympathetic ganglion. Mediastinal ganglioneuroma are mostly detected in children, typically around 10 years of age, and are rarely identified in adults. Herein, we report two surgically resected cases of mediastinal ganglioneuroma in adults. In Case 1, a 53-year-old man, without any symptom, underwent a computed tomography, revealing a 3.2 cm well-defined paravertebral superior mediastinal tumor with long craniocaudal axis. In case 2, a 29-year-old woman presented with newly-developed ptosis and a history of left-sided facial hypohidrosis since the age of 10. Chest computed tomography (CT) revealed a 7.8 cm well-defined paravertebral superior mediastinal tumor with long craniocaudal axis. Both patients were initially suspected to have neurogenic tumors, particularly schwannomas. They underwent mediastinal tumor resections, requiring sympathetic nerve trunk dissection. Pathological examination confirmed the diagnosis of ganglioneuromas in both cases. Mediastinal ganglioneuroma must be differentiated from schwannoma, the most common neurogenic tumor in adults. Unlike schwannoma, ganglioneuroma cannot be enucleated, therefore attention should be focused on complications associated with sympathetic nerve trunk dissection, such as Horner's syndrome, hyperhidrosis, and arrhythmia. Identifying this rare entity and its characteristic imaging aids in preoperative differentiation, strategizing surgical approaches, and predicting complications.

En Bloc Resection of a Giant Ganglioneuroma of the Chest Through Clamshell Thoracotomy.

Ganglioneuroma is a benign tumor requiring subtotal resection as a primary mode of treatment. There are several surgical approaches. A giant ganglioneuroma of the chest cavity may be approached via a clamshell thoracotomy. This manuscript presents a case of giant ganglioneuroma resected en bloc via clamshell thoracotomy in a seven-year-old child.

Diagnostic imaging of a rare case of incidental adrenal ganglioneuroma.

A 53-year-old man complaining of pain in the right hypochondrium underwent an abdominal ultrasound that showed a left adrenal lesion. Further instrumental investigations (CT and MRI, both with contrast medium) were performed which diagnosed an adrenal ganglioneuroma, confirmed by the histological examination. The patient also underwent an endocrinological examination. The treatment was surgical and consisted of an adrenalectomy through video-laparoscopic access. Adrenal ganglioneuromas are rare tumors but well described and known in the literature. For this reason, this case report has primarily an educational purpose: the totality of the data collected (clinical, laboratoristic, instrumental, and histopathological) constituted a multidisciplinary case, with the focus on imaging.

Preoperative differentiation of mediastinum and retroperitoneum ganglioneuroma from schwannoma with clinical data and enhanced CT: developing a multivariable prediction model.

AIM: To develop a multivariable prediction model for preoperative differentiation of ganglioneuroma (GN) from schwannoma in mediastinum and retroperitoneum based on clinical data and enhanced computed tomography (CT). MATERIALS AND METHODS: This was a retrospective diagnostic study. Patients diagnosed with mediastinum or retroperitoneal GN or schwannoma at Zhongshan Hospital between July 2006 and March 2022 were divided into a training cohort and a validation cohort at a ratio of 7:3. Clinical information and CT features were collected. Histopathology was the reference standard for diagnosis. The model was developed using binary logistic regression. The predictive performance of the model was assessed using receiver operating characteristic (ROC) curves, calibration curves, and decision curve analysis (DCA). RESULTS: A total of 105 patients (47 men and 58 women; mean age of 41 ± 15 years) were enrolled. There were significant differences in symptoms (p=0.006), location (p=0.008), ratio of the craniocaudal diameter (CC) to the major axis on axial images (CC/M; p=0.025), ratio of the CC to the diameter on axial images (CC/D; p<0.001), density homogeneity (p=0.001), enhancement homogeneity (p<0.001), enhancement degree (p<0.001), venous phase CT attenuation value (V; p=0.011), and blood vessels changes (p=0.045) between GN and schwannoma. The area under the ROC curve (AUC) and accuracy in the validation cohort were 0.841 (95% confidence interval [CI] 0.672, 1.000) and 0.839 (95% CI: 0.674, 0.929), respectively. Calibration curves and DCA showed that the model was beneficial for patients. CONCLUSION: The multivariable prediction model exhibited good predictive performance and may facilitate preoperative planning.

Image-defined risk factors in localized thoracic neuroblastoma and ganglioneuroma.

BACKGROUND: The pretreatment International Neuroblastoma Risk Group Staging System (INRGSS) discriminates localized tumors L1/L2 depending on the absence/presence of image-defined risk factors (IDRFs) at diagnosis. Referring to this new staging system, we assessed initial imaging of localized thoracic neuroblastoma (NB) and ganglioneuroma (GN) and the extent of initial tumor resection. METHODS: Patients with localized thoracic NB/GN from the German clinical trials NB97 and NB2004 were included. Imaging at diagnosis and operative reports were reviewed retrospectively. IDRFs were assessed centrally and correlated to International Neuroblastoma Staging System (INSS) stage and extent of tumor resection. Additionally, we analyzed data on surgery-related complications. RESULTS: Imaging series of 88 patients were available for central review. In 18 children, no IDRF was present, 28 exhibited one IDRF, 42 two or more IDRFs, resulting in 70 patients with L2 disease. The most frequently observed IDRF was encasement of any vessel (n = 38). Initial surgical resection was aimed for in 45 patients (L1: n = 11; L2: n = 34). Complete and gross total resection rates were higher children with L2 NB (n = 8/25 L1, n = 17/25 L2 vs. n = 2/15 L1, n = 13/15 L2, respectively). The proportion of surgical complications was very similar between INRGSS L1 and L2 (n = 4/11 vs. n = 17/34). All complications were manageable, and no surgery-related deaths were observed. CONCLUSION: In this retrospective cohort, the extent of resection and the rate of surgical complications did not differ substantially between patients classified as L1/L2, indicating that INRGSS L2 does not equate unresectability. It appeared that individual IDRFs differ in value. Larger studies are needed to assess the significance and therapeutic/prognostic impact of such findings.

A giant lobular thoracic ganglioneuroma cause skeletal erosion: A case report and literature review.

RATIONALE: Ganglioneuroma (GN) is a rare tumor that originates from neural crest cells and can manifest in any location along the sympathetic chain. It typically exhibits a circular or oval shape and does not invade the surrounding tissue destructively and the enormous lobular appearance and erosion of adjacent skeletal tissues are extremely infrequent among GN. PATIENT CONCERNS: A 15-year-old girl presented to our thoracic surgery clinic with a large intrathoracic mass that was incidentally discovered on a chest X-ray. Further imaging with computed tomography and magnetic resonance imaging revealed a lobular profile and an aggressive growth pattern of the tumor, which destroyed the vertebral and rib bones. A tissue sample obtained by needle biopsy was subjected to histopathological analysis, which confirmed the diagnosis of a GN. DIAGNOSIS: Thoracic (posterior mediastinal) GN and Hashimoto's thyroiditis. INTERVENTIONS: After thoracoscopic exploration, a thoracotomy was performed to excise the mass. OUTCOMES: The patient recovered well after surgery, had no major complications, and was discharged without any issues. Further follow-up is necessary to clarify the medium to long-term outcome. LESSONS: Based on existing reports, thoracic GN rarely erodes adjacent bone tissue. By examining previously reported cases, we speculate that the lobular morphology of the tumor may be linked to the more aggressive biological behavior of GN. We also discovered that female patients may be more susceptible to bone erosion. However, further research and additional cases are required to confirm these potential associations.

Identification and validation of radiomic features from computed tomography for preoperative classification of neuroblastic tumors in children.

BACKGROUND: To identify radiomic features that can predict the pathological type of neuroblastic tumor in children. METHODS: Data on neuroblastic tumors in 104 children were retrospectively analyzed. There were 14 cases of ganglioneuroma, 24 cases of ganglioneuroblastoma, and 65 cases of neuroblastoma. Stratified sampling was used to randomly allocate the cases into the training and validation sets in a ratio of 3:1. The maximum relevance-minimum redundancy algorithm was used to identify the top 10 of two clinical features and 851 radiomic features in portal venous-phase contrast-enhanced computed tomography images. Least absolute shrinkage and selection operator regression was used to classify tumors in two binary steps: first as ganglioneuroma compared to the other two types, then as ganglioneuroblastoma compared to neuroblastoma. RESULTS: Based on 10 clinical-radiomic features, the classifier identified ganglioneuroma compared to the other two tumor types in the validation dataset with sensitivity of 100.0%, specificity of 81.8%, and an area under the receiver operating characteristic curve (AUC) of 0.875. The classifier identified ganglioneuroblastoma versus neuroblastoma with a sensitivity of 83.3%, a specificity of 87.5%, and an AUC of 0.854. The overall accuracy of the classifier across all three types of tumors was 80.8%. CONCLUSION: Radiomic features can help predict the pathological type of neuroblastic tumors in children.

Whole-tumor radiomics analysis of T2-weighted imaging in differentiating neuroblastoma from ganglioneuroblastoma/ganglioneuroma in children: an exploratory study.

PURPOSE: To examine the potential of whole-tumor radiomics analysis of T2-weighted imaging (T2WI) in differentiating neuroblastoma (NB) from ganglioneuroblastoma/ganglioneuroma (GNB/GN) in children. MATERIALS AND METHODS: This study included 102 children with peripheral neuroblastic tumors, comprising 47 NB patients and 55 GNB/GN patients, which were randomly divided into a training group (n = 72) and a test group (n = 30). Radiomics features were extracted from T2WI images, and feature dimensionality reduction was applied. Linear discriminant analysis was used to construct radiomics models, and one-standard error role combined with leave-one-out cross-validation was used to choose the optimal radiomics model with the least predictive error. Subsequently, the patient age at initial diagnosis and the selected radiomics features were incorporated to construct a combined model. The receiver operator characteristic (ROC) curve, decision curve analysis (DCA) and clinical impact curve (CIC) were applied to evaluate the diagnostic performance and clinical utility of the models. RESULTS: Fifteen radiomics features were eventually chosen to construct the optimal radiomics model. The area under the curve (AUC) of the radiomics model in the training group and test group was 0.940 [95% confidence interval (CI) 0.886, 0.995] and 0.799 (95%CI 0.632, 0.966), respectively. The combined model, which incorporated patient age and radiomics features, achieved an AUC of 0.963 (95%CI 0.925, 1.000) in the training group and 0.871 (95%CI 0.744, 0.997) in the test group. DCA and CIC demonstrated that the radiomics model and combined model could provide benefits at various thresholds, with the combined model being superior to the radiomics model. CONCLUSION: Radiomics features derived from T2WI, in combination with the age of the patient at initial diagnosis, may offer a quantitative method for distinguishing NB from GNB/GN, thus aiding in the pathological differentiation of peripheral neuroblastic tumors in children.

A case of ganglioneuroma of the ureter and review of the literature.

Ganglioneuroma is a rare tumor originating from neural crest tissue of the sympathetic nervous system. We report on an approximately 55-year-old woman who was admitted to hospital with abdominal pain. Surgery revealed a tumor in her right ureter, which was pathologically confirmed as a ganglioneuroma. The patient underwent transabdominal total hysterectomy, bilateral adnexal resection, release of pelvic and intestinal adhesions, right ureteroscopy, right ureter retrograde intubation, right ureteral lesion excision, and ureteral anastomosis. A literature review indicated that most ganglioneuromas are benign tumors. Clinicians may consider total or subtotal tumor resection, depending on the tumor location and patient's condition. The patient's clinical condition may improve after surgery combined with periodic long-term follow-up.

Cervical spine ganglioneuroma and its relationship with neurofibromatosis type 1.

Ganglioneuromas are slow growing tumours arising from tissues of the neural crest, mainly in autonomic nervous system. They are frequently localized in the posterior mediastinum being the intraspinal involvement very uncommon. We present the case of a female patient with bilateral cervical ganglioneuroma, together with a review of the cases published to date, emphasizing in the main characteristics of these tumours and including them as part of neurofibromatosis type 1 spectrum.

Los ganglioneuromas son tumores de lento crecimiento que se originan en tejidos derivados de la cresta neural, principalmente en el sistema nervioso autónomo. Se localizan sobre todo en el mediastino posterior, siendo la afectación intraespinal muy poco frecuente. Presentamos el caso de una mujer intervenida de ganglioneuromas localizados en la columna cervical y agrupamos los casos descritos previamente en la literatura inglesa revisando las características principales de estas neoplasias e incluyéndolas en el espectro de manifestaciones de la neurofibromatosis tipo 1.

Clinical and surgical outcome differences on the basis of pathology category in cervical neuroblastic tumors.

BACKGROUND: Cervical neuroblastic tumors (NTs) are rare but less aggressive cancer with an above-average survival rate. Little has been published regarding the management and surgical outcomes of patients with cervical NTs based on pathology category. This study compared and identified the preoperative characteristics of cervical NTs in different pathology categories and evaluated the outcomes of patients undergoing surgical resection. MATERIALS AND METHODS: Upon the institutional review board's approval, a retrospective chart review was performed at Beijing Children's Hospital from April 2013 to August 2020. Demographics of patients, imaging data, lab test results, operation details and outcomes were recorded and analyzed. RESULTS: Of 32 cervical NTs, 24(80%) were classified as neuroblastoma (NB) /ganglioneuroblastoma-nodular (GNBn) and 8(20%) as ganglioneuroblastoma-intermixed (GNBi)/ ganglioneuroma (GN). Patients with GNBi/GN were older than those with NB/GNBn (44.5 months (IQR 16-81) vs 9 months (IQR 1-47); P = 0.001). GNBi/GN patients presented more frequently with stage 1 disease compared with NB/GNBn patients (100% vs. 29.2%, P = 0.001), less frequently with tumor-related symptoms (0% vs. 70.8%, P = 0.001), artery encased tumor (0% vs. 41.7%, P = 0.035), and surgical complications (25% vs. 70.8%, P = 0.038). GNBi/GN patients were also less likely to show elevated neuron specific enolase (NSE) (12.5% vs. 79.2%, P = 0.002). CONCLUSIONS: Cervical NB/GNBn and GNBi/GN patients had distinct characteristic clinical presentations and surgical outcomes. For children with features suggestive of benign disease (older age, asymptomatic, normal serum tumor markers) and no artery image-defined risk factors (IDRFs), upfront resection can be considered.

Left Adrenal Ganglioneuroma Treated by Laparoscopic Adrenalectomy in a 41-Year-Old Woman: A Case Report.

BACKGROUND Ganglioneuromas are differentiated tumors originating from the neural crest. Although their occurrence is rare, they usually involve the posterior mediastinum and retroperitoneum. However, they rarely occur in the adrenal gland. Adrenal ganglioneuromas (AGNs) are hormonally inactive tumors that are mostly discovered incidentally during abdominal imaging performed for unrelated reasons. As preoperative diagnosis is challenging owing to their heterogeneous nature, adrenalectomy is the most effective method to ascertain an AGN diagnosis. We report a case of left adrenal ganglioneuroma treated by laparoscopic adrenalectomy. In addition, we have presented a relevant literature review to provide further information about this rare tumor. CASE REPORT A 41-year-old woman presented to the Emergency Department with left flank pain associated with dysuria. She was diagnosed with renal colic, which was confirmed by computed tomography of the kidneys, ureter, and bladder. Additionally, an incidental solid lesion in the left adrenal gland was discovered. She was treated conservatively for her acute condition at the Emergency Department and discharged in a good condition. Further work-up including magnetic resonance imaging revealed a left large triangular suprarenal mixed soft tissue mass. She underwent laparoscopic left adrenalectomy. The final histopathology showed an AGN. CONCLUSIONS We present a case of a large AGN in a patient with systemic lupus erythematosus. Because it is a rare tumor with a heterogeneous presentation, its preoperative diagnosis is challenging. Thus, adrenalectomy is required to confirm the diagnosis. The prognosis is excellent and recurrence is extremely rare after tumor resection.

Scoliosis: an unusual clinical presentation of paraspinal ganglioneuroma.

PURPOSE: To comprehensively present the clinical characteristics and treatment strategies in patients with scoliosis secondary to ganglioneuroma (S-GN). METHODS: Six patients with S-GN treated surgically at a median age of 12 years were retrospectively reviewed and the median follow-up period was 6 years (4-14 years). The radiological features of GN and the associated scoliosis were evaluated. The surgical strategies and the corresponding outcomes were investigated. RESULTS: All patients had a delayed diagnosis age of GN than scoliosis (12 vs. 9 years). GN was located at the posterior mediastinum in four patients (66.7%) and at retroperitoneum in two, respectively. Tumor occupancies were frequently detected on the X-ray films for four patients (66.7%), being uniformly on the convexity of the main curve. All patients complained of rapid progressive deformities during the growth period. Five patients (83.3%) received total tumor resections, one accepted partial resection. Deformity correction was implemented for all patients with an average rate of 66.4% on the main curve. No recurrence of the GN was detected for all totally tumor-resected patients at the latest follow-up. CONCLUSION: S-GN is often misdiagnosed clinically. Paravertebral mass neighboring the apex of scoliosis can be meticulously detected from the X-ray films. Total tumor resection should be aggressively performed if possible. The deformity correction could be satisfactorily obtained and the risk of recurrence of the GN was relatively low.

Spinal Ganglioneuroma.

An otherwise healthy 57-year-old man presented with intermittent low back pain and was incidentally found to have a left-sided paraspinal mass invading the spinal canal and causing spinal cord compression. He underwent a T11-12 hemilaminectomy, facetectomy, and instrumented fusion for a gross total resection with a good clinical outcome. Pathology revealed the lesion to be a ganglioneuroma. Ganglioneuroma is a rare and interesting pathology. These tumors are benign peripheral neuroblastic tumors derived from the neural crest and found along the entire neuroaxis. Tumors come to clinical attention if they cause symptomatic compression of neural structures or are found incidentally on imaging. Additionally, as these tumors share a common lineage with pheochromocytomas, systemic symptoms can be observed resulting from secretion of vasoactive peptides. The pathologic diagnosis of ganglioneuroma is predominantly based on morphology.