Ganglioneuromas

OBJECTIVES: To assess the efficacy of thoracoscopy and the outcome for children with thoracic neurogenic tumors. METHODS: We performed a retrospective review of 15 European centers between 2000 and 2020 with patients who underwent thoracoscopy for a neurogenic mediastinal tumor. We assessed preoperative data, complications, and outcomes. Results were expressed with the median and range values. RESULTS: We identified 119 patients with a median age of 4 years old (3 months-17 years)...

Neuroblastomas have shed light on the differentiation disorder that is associated with spontaneous regression or differentiation in the same tumor at the same time. Long noncoding RNAs (lncRNAs) actively participate in a broad spectrum of biological processes. However, the detailed molecular mechanisms underlying lncRNA regulation of differentiation in neuroblastomas remain largely unknown. Here, we sequenced clinical samples of ganglioneuroma, ganglioneuroblastoma, and neuroblastoma. We compared transcription profiles of neuroblastoma cells, ganglion cells, and intermediate state cells; verified the profiles in a retinoic acid-induced cell differentiation model and clinical samples; and screened out the lncRNA ADAMTS9 antisense RNA 2 (ADAMTS9-AS2), which contributed to neuroblastoma differentiation...

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PURPOSE: Peripheral neuroblastic tumors are the most common extracranial cancers found in children, and they are characterized by a diverse spectrum of clinical manifestations and heterogeneous behaviors. This study aimed to investigate the epidemiological and clinical characteristics of children with peripheral neuroblastic tumors admitted to the Department of Pediatric Hematology and Oncology of the Hospital August 20 in Casablanca. METHODS: The medical files of 48 children with peripheral neuroblastic tumors addressed to our department between February 2018 and February 2023 were reviewed...

Pheochromocytomas and paragangliomas (PPGLs) are rare tumors that secrete catecholamines and arise from the adrenal medulla or extra-adrenal sympathetic ganglia. These tumors secrete adrenaline and noradrenaline, but paragangliomas usually produce only noradrenaline because of the lack of phenylethanolamine N-methyltransferase (PNMT) expression. Composite paragangliomas, which are complex tumors consisting of multiple types of neuroblastic cells, are extremely rare. We present the case of a 46-year-old woman with an atypical catecholamine profile who was preoperatively diagnosed with pheochromocytoma...

BACKGROUND: Gangliocytoma is an uncommon disease. In this report, we report a patient who was admitted to the hospital with a mediastinal tumor which is giant rarely. CASE DESCRIPTION: A 30-year-old male patient was found to have a mediastinal mass 2 weeks ago during a routine examination. The patient occasionally had chest pain before 1 month. Computed tomography of the chest showed a mass occupying the posterior mediastinum and located anterior to the spine, above the diaphragm...

BACKGROUND: Neuroblastoma is the most common extra-cranial solid tumor in children. The survival rate of relapsed/refractory neuroblastoma is dismal. Late recurrence may occur rarely. CASE PRESENTATION: We have, herein, presented a case with stage IV neuroblastoma who relapsed after 11 years and had a subsequent relapse after 15 years from the initial diagnosis, and reviewed cases with late relapsed (after >5 years) neuroblastoma in the literature. The case presented with recurrent disease at the T7 vertebra after 11 years from the initial diagnosis...

Paraneoplastic neurological disorders are rare in children, with paraneoplastic cerebellar degeneration (PCD) considered highly atypical. We describe a 13-year-old girl with progressive neurobehavioral regression, cerebellar ataxia, and intractable epilepsy presenting in super-refractory status epilepticus. After an extensive evaluation, her clinical picture was suggestive of probable autoimmune encephalitis (AE). Further diagnostic testing revealed a molecularly undefined neural-restricted autoantibody in both serum and CSF, raising suspicion over an adrenal mass previously considered incidental...

BACKGROUND: Multiple endocrine neoplasia type 2B (MEN 2B) is a rare hereditary syndrome caused mainly by Met918Thr germline RET mutation and characterized by medullary thyroid carcinoma (MTC), pheochromocytoma (PHEO), and typical phenotypic features. MEN 2B cases previously reported in the literature have variable clinical course. OBJECTIVES: We aimed to discuss the characteristics of four MEN 2B cases with unusual presentations,clinical course and review the recent clinical data on MEN2B...

Multifocal ganglioneuromas are characterized by the presence of multiple benign neuroepithelial tumor nodules and are less common than solitary tumors. A small percentage of ganglioneuromas present with a fatty appearance. Only a few cases of multifocal ganglioneuromas have been reported, due to both their rarity and minimal symptomatic presentation; therefore, generalizations about risk factors and predictive markers are very difficult. Here, we report a case of multifocal retroperitoneal ganglioneuroma with an infiltrative appearance on computed tomography (CT)...

Ganglioneuromas are benign tumors arising from the neural crest. Histologically, they are composed of mature Schwann cells and ganglion cells admixed with fibrous tissue. While they frequently are seen in the abdomen and mediastinum, rare reports have highlighted their occurrences in the genitourinary system. The only prior reported prostatic ganglioneuroma arose in a patient with a history of neurofibromatosis type 1. In this report, we highlight the first reported prostatic ganglioneuroma without a known genetic linkage...

Aims: This study aimed to review our surgical experience of laparoscopic adrenalectomy (LA) for adrenal masses in the pediatric age group. Materials and Methods: The electronic medical records of all patients younger than 18 years of age who underwent LA between 2016 and 2023 were retrospectively reviewed. Children with adrenal tumors localized to the site of origin without evidence of encasement of major vasculature or involvement of adjacent organs were considered for LA. Patients with diagnosis of malignant adrenal tumor on preoperative work-up underwent open adrenalectomy...

BACKGROUND: Ganglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). We shared our institutional experience with childhood GNs. METHODS: Records of the children with PNTs between January 1995 and December 2021 were reviewed, and cases with histopathological diagnoses of GN were identified. Clinical, laboratory, radiological, and histopathological findings, image-defined risk factors (IDRFs), procedures, and overall outcomes were recorded. RESULTS: Of 668 cases with PNTs, 70 (10...

Neuroblastoma (NB) is the deadliest pediatric solid tumor due to its rapid proliferation. Aberrant expression of MYCN is deemed as the most remarkable feature for the predictive hallmark of NB progression and recurrence. However, the phenomenon that only detection of MYCN in the nearly 20% of NB patients hints that there should be other vital oncogenes in the progression of NB. Here, we firstly show that MSI2 mRNA is augmented by analyzing public GEO datasets in the malignant stage according to International Neuroblastoma Staging System (INSS) stages...

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...

INTRODUCTION AND IMPORTANCE: Ganglioneuromas are extremely rare, slow-growing, benign tumors that arising from Schwann cells, ganglion cells, and neuronal or fibrous tissue. Their malignant degeneration occurs very rarely, complete surgical removal is recommended to eliminate possible symptoms or to prevent possible malignant transformation. Reviewing the literature, there is currently insufficient data available on laparoscopic resection of retroperitoneal ganglioneuromas. CASE PRESENTATION: 20-year-old young woman with no previous medical history or regular medication use complaints of abdominal pain...

Peripheral neuroblastic tumors represent the fourth-largest group of malignant tumors in childhood. The majority of these tumors are neuroblastomas, which can be classified into undifferentiated, poorly differentiated, and differentiating subtypes. In addition, peripheral neuroblastic tumors include ganglioneuroblastoma, a composite tumor composed of Schwannian cell stroma and neuroblasts as well as benign ganglioneuroma. In this overview, histopathological diagnostic criteria and grading systems, as well as common molecular alterations that are of prognostic and therapeutic significance, are discussed...

Adrenal ganglioneuromas are mostly asymptomatic, although they may manifest with compressive local effects. We present a 27-year-old man with no medical history who was referred to the surgical oncology clinic due to the incidental finding of a left adrenal mass. The initial computed tomography (CT) abdomen revealed a large mass causing displacement of adjacent organs. A CT-guided biopsy was inconclusive, and further evaluation with an NM endo-adrenal (MIBG) medullary scan pointed to a possible diagnosis of pheochromocytoma...

INTRODUCTION: Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare multisystemic disease that is named for its constellation which can easily be mistaken for other disorders. CASE PRESENTATION: In present study we evaluated a 39-year-old man with hypercalcemia and parathyroid hormone. He underwent two failed surgeries for parathyroid adenoma but due to the presence of other features of the syndrome, a diagnosis of POEMS syndrome was made...

Neuroblastoma(NB) is the most common extracranial solid tumor in childhood, and it is now believed that some patients with NB have an underlying genetic susceptibility, which may be one of the reasons for the multiplicity of NB patients within a family line. Even within the same family, the samples show great variation and can present as ganglioneuroblastoma or even benign ganglioneuroma. The genomics of NB is still unclear and more in-depth studies are needed to reveal its key components. We first performed single-cell RNA sequencing(sc-RNAseq) analysis on clinical specimens of two family neuroblastoma(FNB) and four sporadic NB cases...