Artikel
Multinodular and vacuolating neuronal tumor of the cerebrum
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Veröffentlicht: | 25. August 2015 |
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Gliederung
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Introduction: Multinodular and vacuolating neuronal tumor of the cerebrum (MVNT) was first described in 2013. To date, only three reports with 13 cases of MVNT have been published.
Objectives: To report on our single institute diagnostic experience with this newly described tumor entity.
Patients & methods: Histomorphological and immunohistochemical analysis within a twelve month period (April 2014-March 2015) revealed four cases of MVNT with either temporo-mesial (n=3) or parieto-occipital (n=1) localization. Age at surgery was between 23 and 59 years. Three patients suffered from epileptic seizures. Three patients underwent surgery at the University of Bonn, Medical Center. Tumor specimens from one additional case were seen in the DGNN German Tumor Reference Center Bonn. Clinical and neuroradiological data were collected.
Results: Histomorphological analysis showed well circumscribed nodular or band-like lesions with extensive vacuolization. Cytomorphologically, the tumors cells showed a neuronal or ganglionic differentiation. Reactive astrogliosis, but no glial tumor component was observed. In all cases immunohistochemical analysis revealed expression of the neuronal antigens HuC/HuD and Map2 as well as nuclear expression of Olig2. All cases were negative for NeuN, chromogranin and neurofilament. Variable immunoreactivity was detected for synaptophysin. CD34-positive satellite-cells as well as positive tumor cells were detected in one case. Increased mitotic or proliferative activity was not observed. MR imaging hallmarks were the cortical involvement without mass effect and a multicystic appearance with scattered small cysts in the adjacent brain.
Conclusion: The detection of four MVNT cases within twelve months only suggests that MVNT is an important and not infrequently occurring differential diagnosis in the neuropathological classification of epileptogenic lesions. The distinct neuropathological appearance as well as the neuroradiological aspect allows differential diagnosis from reactive changes and low-grade epileptogenic lesions such as ganglioglioma, DNT or focal cortical dysplasia.