Ganglioneuromas

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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 purpose of this study was to retrospectively analyze the characteristics of contrast-enhanced ultrasound (CEUS) images and quantitative parameters of time-intensity curves (TICs) in children's peripheral neuroblastic tumors (pNTs). By comparing the imaging features and quantitative parameters of the TICs of neuroblastoma (NB) and ganglioneuroblastoma (GNB) patients, we attempted to identify the distinguishing points between NB and GNB. A total of 35 patients confirmed to have pNTs by pathologic examination were included in this study...

Ganglioneuromatous hamartoma is a benign tumour of autonomic ganglia with very few cases reported in head and neck region. In this report, we are presenting a case of ganglioneuromatous hamartoma in a 20 day old female child who presented with a tongue mass. She underwent a surgical excision and the definitive diagnosis was made by histopathology. This case reports discusses presentation and management of a case of ganglioneuromatous hamartoma.

Multiple Endocrine Neoplasia type 2B (MEN2B) is an autosomal dominant cancer syndrome caused by a mutation in rearranged during transfection (RET) proto-oncogene and includes medullary thyroid carcinoma, pheochromocytoma, gastrointestinal neuromas, and mucosal ganglioneuromas. Medullary thyroid carcinoma is the major cause of mortality in MEN2B syndrome. Medullary thyroid carcinoma can often appear during the first years of life. While mucosal neuromas in MEN2B are common, laryngeal neuromas are extremely rare...

BACKGROUND/OBJECTIVE: Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors. Here, we report an unusual case of synchronous PPGL in an asymptomatic patient with tuberous sclerosis complex (TSC). CASE REPORT: A 49-year-old woman with a history of TSC and end-stage renal disease was referred for evaluation of bilateral adrenal and retroperitoneal masses. She denied chest pain, palpitations, headaches, or previous hypertensive crisis. The laboratory test results showed a plasma normetanephrine level of 20...

Ganglioneuroma, a rare benign neuroblastic tumor, typically arises in the posterior mediastinum, but it can be found in the anterior mediastinum and thymus. Predominantly affecting the young, these asymptomatic tumors are often discovered incidentally through imaging. In our reported case, a 44-year-old woman post-hysterectomy with persistent jaundice was diagnosed with a neuroganglioma in the right posterior mediastinum via a computed tomography (CT) scan. Thoracotomy and resection revealed a 10-cm neuroganglioma untangled from mediastinal planes...

BACKGROUND: The aim of this study was to investigate the clinical features and treatment options for pediatric adrenal incidentalomas(AIs) to guide the diagnosis and treatment of these tumors. METHODS: The clinical data of AI patients admitted to our hospital between December 2016 and December 2022 were collected and retrospectively analyzed. All patients were divided into neonatal and nonneonatal groups according to their age at the time of the initial consultation...

NF1 is an autosomal dominant hereditary disease, with a prevalence of at least 1 in 4000-5000 population. The diagnosis criteria of NF1 included typical manifestations such as café-au-lait spots, frecking in the axilla or inguinal region, multiple neurofibromas, Lisch nodeules, and distinctive osseous lesions. Genetic testing shows NF1 mutation. It is essential for tumor surveillance in NF1 patients because their life expectancy is about 54 years due to malignancy. A case of NF-1 patient receive laparoscopic small bowel resection and finally diagnosed as adenocarcinoma and ganglioneuroma...

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...

Cervical ganglioneuroma combined with neurofibromatosis type I is very rare. This article reports a case of a 21-year-old male patient with a rare presentation of cervical ganglioneuroma and neurofibromatosis type I. In this patient, the tumors on both sides of the cervical spine were surgically removed with good results. The effects and advantages of surgery when both diseases coexist are discussed, as well as further investigation into possible causal relationships between these two pathologies.

We report a case of a robotic-assisted excision of a retrocaval ancient schwannoma. A 40-year-old female presented with generalized weakness and abdominal pain that led to the diagnosis of a retroperitoneal mass adjacent to the pancreas and inferior vena cava. Because of the clinical, imaging, and needle biopsy findings, the patient underwent an elective robotic-assisted retroperitoneal exploration. We provide an overview of the pathology and highlight the significance of utilizing a minimally invasive approach for excision of retroperitoneal masses...

This article deals with a rare occurrence of Ganglioneuroma of the left cervical sympathetic chain in a 9-year-old girl, presenting with painless paracervical swelling noticed since 7 months. Imaging studies showed a left parapharyngeal mass extending from C1 to C6, displacing the posterior pharyngeal wall and anterolaterally displacing the contents of the carotid sheath, with no significant vascular feeders. A provisional diagnosis of? Neurogenic tumour was established and the patient underwent surgical excision...

Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare immune-mediated movement disorder occurring as a paraneoplastic manifestation of neuroblastic tumours (NTs), especially neuroblastoma in infancy. Ganglioneuroma (GN), the benign tumour in the spectrum, is rarely associated with OMAS. We report the case of a child in her second year of life presenting with acute onset of progressive paraplegia and OMAS. MRI showed diffuse and infiltrating left paraspinal mass from T3-T9 levels with differentials of neuroblastoma or ganglioneuroblastoma...

A retroperitoneal ganglioneuroma is an exceptionally rare surgical entity, even more so in pancreaticoduodenal tumors. These well-differentiated neuroepithelial tumors originate in the neural crest, emerge in the sympathetic nervous system, and consist of ganglion cells and stromal Schwann cells. Generally, these tumors, despite being mostly benign, may be associated with venous or arterial vascular involvement. The symptomatology presented will depend on the mass effect due to tumor growth, and surgical excision is the only therapeutic option offered today to these patients...

Ganglioneuromas (GN) are benign neuroblastic tumors that arise from neural crest cells. Since they present with nonspecific symptoms, diagnosis is often incidental. We are reporting a case of an adult appendiceal GN incidentally found during rectal cancer surgery. A 42-year-old male was diagnosed with recurrent rectal cancer after experiencing urinary difficulties and buttock pain. A multiple-stage pelvic exenteration was carried out after neoadjuvant chemotherapy and chemoradiation. Prophylactic appendectomy was done during the course of surgery, and pathology reported an appendix with GN at the distal tip...

Robotic-assisted surgery (RAS) is increasingly adopted in the pediatric population. This retrospective multicenter study aimed to report application of RAS for gynecological indications in pediatric patients. The medical records of all girls with gynecological pathology, operated in 4 different institutions over a 3-year period, were retrospectively collected. Robot docking time, total operative time, length of stay (LOS), requirement time of pain medication, complication rate, conversion rate, and pathology were analyzed...

Retroperitoneal ganglioneuroma is a rare benign tumor that is challenging in terms of clinical diagnosis. Computed tomography and magnetic resonance imaging are usually performed for diagnosis rather than convenient and inexpensive ultrasonography. Here, we present the case of a 21-year-old female patient who was diagnosed by multimodal ultrasound imaging and whose diagnosis was confirmed by ultrasound-guided core needle biopsy before surgery. We hope that this rare case will help clinicians and radiologists realize the advantages of multimodal ultrasound imaging in the diagnosis of retropeitoneal solid tumors, and reduce misdiagnosis...

Synchronous tumors comprising of cortical as well as medullary differentiation are rare in the adrenal gland. Collision tumors comprise of two independent neoplasms arising from the cortex and the medulla without any histological admixture. Adrenal cortico-medullary mixed tumors are rare tumors comprising of adrenal cortical and chromaffin cells intermixed with each other. Ganglioneuroma is a rare benign tumor originating from the paravertebral sympathetic ganglia and is rarely seen in the adrenal medulla. The presence of a synchronous ganglioneuroma with an adrenal cortical adenoma in the ipsilateral adrenal gland is exceedingly rare...

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...