Low grade ganglioglioma rapidly progressing to a WHO grade IV tumor showing malignant transformation in both astroglial and neuronal cell components.
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Slide 1 :
Low grade ganglioglioma rapidly progressing to a WHO grade IV tumor showing malignant transformation in both astroglial and neuronal cell components. email@example.com
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Patient history: 47 year old female patient left trigeminal neuralgia in 2001 further neurological examination in 2001 was normal incidental finding of a left frontal precentral mass lesion (MRI)
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Neuroradiology: In 2001, MRI revealed a left frontal mass with a diameter of 2cm with no edema or contrast enhancement (A: T1 with contrast media (CM); B: T2)
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Neuroradiology: In 2004, a slight tumor progression was observed (C: T1 with CM; D: T2-FLAIR).
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Neuropathology: Histology and immunohistochemistry of the tumor resected in 2004. (A) Routine HE stain revealed a tumor of low cell density consisting of large well-differentiated neuronal (arrows) and small glial cells. (B) GFAP immunohistochemistry showed the astroglial differentiation but was absent in the neuronal component (arrows). (C) Synaptophysin immunohistochemistry displayed a staining pattern complementary to GFAP exhibiting immunoreactivity of the larger cells which were sporadically binucleated (arrows). (D) MIB-1 index was very low throughout the whole tumor. Original magnification A-D: 400x. (Diagnosis: Low grade ganglioglioma)
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Neuroradiology: Preoperative MRI in April 2006 revealed a marked contrast enhancement (E: T1 with CM) and progressive tumor growth located at the site of the primary tumor (F; T2-FLAIR)
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Neuropathology: Histology and immunohistochemistry of the tumor resected in 2006. HE stainings revealed a pleomorphic tumor with (A) high cell density, (B) eosinophilic granular bodies, (C) necrosis, (D) large vascular proliferations, (E) multinucleated cells and (F) frequent mitoses (arrow). (G) Glial cells as well as large polynucleated (arrow) and (H) mononucleated neuronal cells displayed strong MAP2 immunoreactivity. (I) Strong GFAP expression was noted on astroglial tumor cells, but was absent on large polynucleated neuronal tumor cells (arrow). (J) Synaptophysin was mainly found on polynucleated pleomorphic neuronal tumor cells. (K) MIB-1 index was highly elevated, escpecially nuclei of polynucleated tumor cells were positively stained. Original magnification A: 200x; B-K: 400x. (Diagnosis: Ganglioglioma, WHO grade IV).
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Neuroradiology: Control MRI in September 2006 showed the resection defect without any signs of residual tumor parts or recurrence (F: T1 with CM; G: T2-FLAIR).
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Background/Discussion -> Gangliogliomas are rare benign CNS neoplasms, usually classified as WHO grade I tumors consisting of a mixture of neoplastic mature ganglion and glial cells. -> Gangliogliomas are considered to originate in a dysontogenic way from a glioneuronal precursor lesion with a clonal proliferating neoplastic glial component. -> 7.5-year recurrence-free survival rate of ganglioglioma patients is 97%. Malignant progression of gangliogliomas is only observed in about 2% of all cases. About 7% of the gangliogliomas present with histological features of higher malignancy and were therefore classified as WHO grade II or III tumors. -> WHO grade II was assigned to tumors with increased cellularity and a MIB-1 proliferation index of more than 5%. -> Only in 1999, the first case of a primary ganglioglioma, WHO grade IV, was described however malignancy was restricted to the glial component. -> Not only the astrocytic, but also the neuronal tumor component is capable of undergoing malignant neuroblastomatous transformation in single cases. -> Malignant transformation is thought to be associated with radiotherapy. -> The presented case is the first reported low grade ganglioglioma rapidly progressing to a WHO grade IV glio-neuronal tumor not being associated with radiotherapy and showing malignant transformation in both astroglial and neuronal tumor cell component.
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