Ganglioneuroma Always A Histopathological Diagnosis

Neuroblastoma, ganglioneuroblastoma and ganglioneuroma arise from sympathetic tissue in the neck, posterior mediastinum, adrenal gland, retroperitoneum and pelvis Ganglioneuromas are commonly seen in childhood. They are highly differentiated benign tumors and are compatible with long-term disease free survival. Retroperitoneal localization is relatively frequent for these tumors. Due to its rarity and lack of specific radiological findings diagnosis is always postoperative. Here, we present a case of Retroperitoneal ganglioneuroma which was undiagnosed before surgery

Importance of Magnetic Resonance Imaging With Diffusion-weighted Imaging in Guiding Biopsy of Nodular Ganglioneuroblastoma: A Case Report

BACKGROUND: Nodular ganglioneuroblastoma is a rare peripheral neuroblastic tumor of variable prognosis. Accurate diagnosis, staging, and risk categorization can be particularly challenging in patients with nodular ganglioneuroblastoma due to the inherent heterogeneity of these lesions. CASE PRESENTATION: We illustrate the use of diffusion-weighted magnetic resonance imaging to identify tumor nodules and guide tumor biopsy in an almost 5-year-old boy with a large abdominal tumor. CONCLUSIONS: Diffusion-weighted magnetic resonance imaging was successful in detecting and guiding biopsy of a poorly differentiated neuroblastoma nodule within the context of a well-differentiated ganglioneuroma, allowing the diagnosis and characterization of a ganglioneuroblastoma nodular, thus influencing the child's prognosis and treatment

Ganglioneuroblastoma of the posterior mediastinum: a case report.

Introduction:Ganglioneuroblastoma is a rare peripheral neuroblastic tumor that is derived from developing neuronal cells of the sympathetic nervous system, and is found mostly, but not exclusively, in babies and young children. Case Presentation: To the best of our knowledge, there have been no previously reported cases of ganglioneuroblastoma of the mediastinum from Pakistan. We present a case of ganglioneuroblastoma in an eight-year-old Pakistani Sindhi boy incidentally found to have a large posterior mediastinal mass that on biopsy initially looked like ganglioneuroma. He underwent successful excision of the mediastinal mass and remained stable post-operatively. Final pathology findings showed a ganglioneuroblastoma. He has remained free of symptoms on long-term follow-up. Conclusions: The rarity of this tumor along with its almost exclusive occurrence in the pediatric population necessitates a thorough investigation of Patients presenting with a symptomatic mass

Neuroblastic tumors of the adrenal gland in elderly patients: a case report and review of the Literature

Background: Neuroblastic neoplasms (NN) include ganglioneuromas (GN), ganglioneuroblastomas (GNB), and neuroblastomas (NB). They generally arise in childhood from primitive sympathetic ganglion cells. Their incidence in adults, especially among elderly, is extremely low. Case Presentation: This is the case of a 74-year-old woman with history of abdominal pain, weakness and night sweating since several months. Blood pressure was normal. CT-scan showed a 10 cm left adrenal mass, without other pathologic findings. An open left-sided adrenalectomy was performed. Recovery was uneventful with hospital length of stay of 8 days. Based on morphological, immunohistochemical, and molecular features the diagnosis was a nodular GNB. A positron emission tomography (PET) performed 6 weeks after the resection did not show any residual tumor or distant metastases. The patient was followed-up with annual clinical and radiological exams. Conclusion: This case presentation, associated with a review of the literature, illustrates the importance to include NN in the preoperative differential diagnosis of adrenal tumors in adults and highlights the need for multidisciplinary patient work-up and management

Refractory Stage M Ganglioneuroblastoma With Bone Metastases and a Favorable, Chronic Course of Disease:Description of a Patient Cohort

Refractory stage M neuroblastoma (NB) is associated with a poor prognosis and a progressive course of disease. Here, we describe a unique group of patients with a discrepant clinical course. Seven histologically confirmed ganglioneuroblastoma (GNB) (n=6) and differentiating NB (n=1) patients were identified who were diagnosed with stage M disease based on iodine-123-metaiodobenzylguanidine avid bone metastases. Six patients started on high-risk treatment, without tumor response (stable disease). Treatment was discontinued before the start of consolidation treatment because of refractory response in all patients. Unexpectedly, after cessation of treatment no progression of disease occurred. In 2 patients, the primary tumors expanded (>25%) very slowly during 1.5 and 3 years, and remained stable thereafter. Metabolically, a slow decrease of urinary homovanillic acid and vanillylmandelic acid levels and iodine-123-metaiodobenzylguanidine avidity was observed. All patients are alive with presence of metastatic disease after a median follow-up of 17 years (range: 6.7 to 27 y). Interestingly, at diagnosis, 6 patients were asymptomatic, 6 patients had GNB morphology, and 5 patients had meningeal metastases. These are all features seen in only a small minority of stage M patients. This GNB entity illustrates the clinical heterogeneity of neuroblastic tumors and can be used to further study the developmental origin of different NB subtypes

Tumores neuroblásticos de mediastino na criança.

Trabalho de Conclusão de Curso - Universidade Federal de Santa Catarina, Centro de Ciências da Saúde, Departamento de Pediatria, Curso de Medicina, Florianópolis, 200

Retroperitoneal ganglionörom

Nöroblastom, ganglionöroblastom ve ganglionörom sempatik sinir sistemini oluşturan, değişik derecelerde olgunlaşmış, primordial nöral krest hücrelerinden köken alan heterojen bir grup tümördür. Abdominal distansiyon, sol üst kadran ağrısı ve daire şikayetleriyle başvuran 12 yaşındaki erkek hastaya yapılan tetkikler sonucu rastlantısal olarak primer retroperitoneal ganglionörom saptanmıştır.Neuroblastoma, ganglioneuroblastoma and ganglioneuroma are tumors of varying maturity derived from the primordial neural crest cells that form the sympathetic nervous system. A primary extraadrenal retroperitoneal ganglioneuroma was found incidentally in a 12-year-old boy during a series of examinations for abdominal distension, left upper quadrant pain and diarrhoea