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A 4-cm paravertebral mediastinal tumor was resected in a 70-year-old male patient treated for hypertension. The tumor displayed both paraganglioma and ganglioneuroma areas that were in equal proportion and often merged one into the other. Paraganglioma areas contained synaptophysin and chromogranin-positive chief cells and PS100-positive sustentacular cells. Ganglioneuroma areas contained neurofilament-positive mature ganglion cells and PS100-positive Schwann cells. Such pheochromocytoma-ganglioneuroma has not been previously reported in the mediastinum and appears as the adrenal and aorticosympathetic counterpart of gangliocytic paraganglioma described in other anatomic sites.
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Ganglioneuromas are rare tumors arising from retroperitoneal and posterior mediastinal sympathetic nerves. Intracranial trigeminal nerve ganglioneuromas are even more rare, with only seven cases reported to date. We present a case of a 65-year-old male with a right-sided throbbing headache type and blindness in his right eye.
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Childs Nerv Syst
December 2024
Pediatric Neurosurgery Department, CHU de Brabois, Nancy, France.
Article Synopsis
- Spinal ganglioneuromas are rare neuroblastic tumors mostly found in children, originating from sympathetic ganglia near the spine, and can lead to radicular or medullary symptoms when they extend into the spinal canal.
- A thorough review of pediatric cases was conducted, highlighting a unique case treated in France, focusing on the thoraco-lumbar region due to its different surgical management requirements.
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Mixed pituitary adenoma/PitNET-gangliocytomas (PA/PitNET-GC) have been reported in small series over the past 20 years; some had limited immunohistochemistry (IHC) data. We interrogated our experience over 20 years, focusing on patterns of the GC component and IHC results for anterior pituitary hormones, transcription factors, NFP, and CAM5.2.
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Neuroblastic tumors (NTs) represent the most common extracranial neoplasm occurring in childhood. Although ganglioneuroblastoma intermixed (GNBI) and ganglioneuroma (GN) are classified as very low-risk tumors, neuroblastoma (NB) and ganglioneuroblastoma-nodular (GNBN) may represent a serious risk to survival. Unfortunately, areas of GNBI and GNBN can coexist in the same mass, leading to incorrect risk staging when only biopsy is performed.
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