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Ear, Nose, & Throat Journal Dec 2023Ganglioneuroma is a rare benign tumor originating in the sympathetic ganglia, composed of differentiated ganglion cells, nerve sheath cells, and nerve fibers, which tend... (Review)
Review
Ganglioneuroma is a rare benign tumor originating in the sympathetic ganglia, composed of differentiated ganglion cells, nerve sheath cells, and nerve fibers, which tend to occur in the posterior mediastinum, adrenal gland, retroperitoneal, and other locations, occurring in the head and neck is relatively rare, and parapharyngeal space involvement is extremely rare. In our report, we present 2 adult male patients whose preoperative imaging and fine needle cytology did not confirm the diagnosis of a parapharyngeal space mass and who completely resected the tumor through a combined cervical and oral approach. Finally, pathology confirmed ganglioneuroma; we also reviewed the English articles on parapharyngeal ganglioneuroma over the past 40 years, and summarized the diagnostic and treatment characteristics of parapharyngeal ganglioneuroma in combination with our cases to improve understanding of the disease.
Topics: Adult; Humans; Male; Parapharyngeal Space; Ganglioneuroma; Neck; Needles
PubMed: 36450599
DOI: 10.1177/01455613221142658 -
Frontiers in Immunology 2023Neuroblastoma(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,...
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. A complete transcriptional profile of FNB was constructed from 18,394 cells from FNB, and we found that may be genetically associated with FNB and identified a prognostic related CAF subtype in FNB: Fib-4. Single-cell flux estimation analysis (scFEA) results showed that malignant cells were associated with arginine spermine, oxaloacetate and hypoxanthine, and that malignant cells metabolize lactate at lower levels than T cells. Our study provides new resources and ideas for the development of the genomics of family NB, and the mechanisms of cell-to-cell interactions and communication and the metabolic landscape will provide new therapeutic targets.
Topics: Humans; Transcriptome; Neuroblastoma; Ganglioneuroblastoma; Prognosis; Genetic Predisposition to Disease
PubMed: 37790931
DOI: 10.3389/fimmu.2023.1197773 -
International Journal of Surgery Case... Oct 2023Retroperitoneal ganglioneuroma is an infrequent, benign tumor originating from the sympathetic nervous system. The diagnosis predominantly relies on histological...
INTRODUCTION AND IMPORTANCE
Retroperitoneal ganglioneuroma is an infrequent, benign tumor originating from the sympathetic nervous system. The diagnosis predominantly relies on histological assessment, often as an incidental discovery. Surgical removal stands as the primary treatment modality, and the overall prognosis tends to be favorable.
CASE PRESENTATION
Objective: In order to comprehensively examine the diagnostic, therapeutic, and progressive aspects of retroperitoneal ganglioneuroma, this study aims to analyze five cases of retroperitoneal ganglioneuroma that were surgically managed between 1993 and 2013.
PATIENTS AND METHODS
We present a series of five cases involving retroperitoneal ganglioneuromas that underwent surgical intervention in the urology department of Charles Nicolle Teaching Hospital of Tunis between the years 1993 and 2013. The study cohort comprised three females and two males, with an average age of 36.4 years (ranging from 10 to 88 years). Among these cases, four instances showcased incidental tumor detection, while one case manifested with cruralgia due to nerve compression. Complete tumor excision was achieved in all cases, with a single instance necessitating concurrent nephrectomy. Accurate diagnosis was ascertained through meticulous histological examination.
CLINICAL DISCUSSION
Results: The case series encompassed three women and two men, with an average age of 36.4 years (ranging from 10 to 88 years). The majority of cases (four out of five) unveiled tumors incidentally, whereas one case presented with cruralgia. Surgical removal resulted in complete resection of all tumors, although nephrectomy was necessary in one instance. Postoperative complications were minimal, and effective anticoagulant treatment addressed one thrombotic event. While recurrence was noted in a solitary case, subsequent imaging confirmed its stability. The average follow-up duration extended to 23.2 months (ranging from 6 to 72 months).
CONCLUSION
Retroperitoneal ganglioneuroma, being a rare and benign neoplasm, mandates meticulous pathological assessment and precise imaging for precise localization. Unquestionably, complete surgical resection remains paramount to forestall recurrence and mitigate the likelihood of malignant transformation. Overall, the prognosis associated with retroperitoneal ganglioneuroma tends to be favorable.
PubMed: 37734125
DOI: 10.1016/j.ijscr.2023.108840 -
Asian Journal of Surgery Nov 2023
Topics: Humans; Ganglioneuroma; Adrenal Gland Neoplasms; Tomography, X-Ray Computed
PubMed: 37474382
DOI: 10.1016/j.asjsur.2023.07.048 -
JCI Insight Nov 2023Neuroblastomas have shed light on the differentiation disorder that is associated with spontaneous regression or differentiation in the same tumor at the same time. Long...
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. ADAMTS9-AS2 upregulation in neuroblastoma cell lines inhibited proliferation and metastatic potential. Additional mechanistic studies illustrated that the interactions between ADAMTS9-AS2 and LIN28B inhibited the association between LIN28B and primary let-7 (pri-let-7) miRNA, then released pri-let-7 into cytoplasm to form mature let-7, resulting in the inhibition of oncogene MYCN activity that subsequently affected cancer stemness and differentiation. Furthermore, we showed that the observed differential expression of ADAMTS9-AS2 in neuroblastoma cells was due to N6-methyladenosine methylation. Finally, ADAMTS9-AS2 upregulation inhibited proliferation and cancer stem-like capabilities in vivo. Taken together, these results show that ADAMTS9-AS2 loss leads to malignant neuroblastoma by increasing metastasis and causing dysfunctional differentiation.
Topics: Humans; RNA, Long Noncoding; N-Myc Proto-Oncogene Protein; Cell Differentiation; Neuroblastoma; ADAMTS9 Protein; RNA-Binding Proteins
PubMed: 37991019
DOI: 10.1172/jci.insight.165703 -
Internal Medicine (Tokyo, Japan) Nov 2023
PubMed: 37981307
DOI: 10.2169/internalmedicine.2496-23 -
Urology Case Reports Nov 2023Ganglioneuromas are benign tumors arising from the neural crest. Histologically, they are composed of mature Schwann cells and ganglion cells admixed with fibrous...
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.
PubMed: 37886344
DOI: 10.1016/j.eucr.2023.102583 -
Cureus Jun 2023Adrenal ganglioneuromas are rare tumors arising from sympathetic ganglion cells that may present similarly to other adrenal tumors, making preoperative diagnosis...
Adrenal ganglioneuromas are rare tumors arising from sympathetic ganglion cells that may present similarly to other adrenal tumors, making preoperative diagnosis challenging. We present a case of a young woman with a history of Hashimoto's thyroiditis who presented with hypertension and headaches. An abdominal CT scan revealed a large left adrenal mass, and while laboratory tests for catecholamines and metanephrines were normal, the suspicion for pheochromocytoma remained high given the size of the mass and persistent hypertension. The patient was started on alpha-blockers and beta-blockers in preparation for surgical removal. Pathology revealed a mature ganglioneuroma without evidence of malignancy, and postoperative blood pressure was normalized. We hypothesize that vessel compression from the large mass created functional stenosis, resulting in persistent hypertension. This case highlights the importance of a thorough workup for hypertension in young adults and routine preventative care visits to avoid delayed management. Adrenalectomy with histopathological examination remains the gold standard for treatment and diagnosis, and patients have a good prognosis following resection, with minimal need for recurrent therapy.
PubMed: 37415991
DOI: 10.7759/cureus.39977 -
Journal of Clinical Medicine Sep 2023Nonspecific gastrointestinal symptoms remain a problem for pediatricians because, out of a thousand trivial cases, there are rare diseases that require in-depth... (Review)
Review
Nonspecific gastrointestinal symptoms remain a problem for pediatricians because, out of a thousand trivial cases, there are rare diseases that require in-depth diagnostics and extensive knowledge to identify them. These complaints may be caused by a neoplastic process. We present the case of a 5-year-old boy whose diagnostic pathway lasted about 3 months. He was admitted to hospital due to severe abdominal pain. Physical examination revealed a bloated, hard, and painful abdomen. In the standing X-ray, the features of intestinal obstruction were visualized. An ultrasound examination showed a possible malignant lesion in the location of the left adrenal gland. After the surgical removal of the pathological mass and histopathological examination, the diagnosis of ganglioneuroblastoma intermixed was made. This tumor, along with neuroblastoma, ganglioneuroma, and ganglioneuroblastoma nodular, belongs to neuroblastic tumors (NTs), which originate from primitive cells of the sympathetic nervous system. NTs are quite rare, but they are still the majority of extracranial solid tumors in children, and their symptoms often appear relatively late when the neoplastic process is already advanced. The purpose of this review is to present current information about ganglioneuroblastoma, with a special emphasis on nonspecific gastrointestinal symptoms as first sign of this tumor and its diagnostics.
PubMed: 37763032
DOI: 10.3390/jcm12186092