Two cases of multinodular and vacuolating neuronal tumour.
An unusual multinodular and vacuolating neuronal tumour (MVNT) has been described in the cerebral hemispheres of ten patients with adult-onset seizures. We report the findings in two cases with similar features, a surgical resection and the other an autopsy specimen.Case 1, a 34-year-old female, underwent surgical resection for a multinodular non-enhancing frontal white matter lesion causing intractable epilepsy. Case 2, presented with motor neurone disease (MND) at the age of 71 and MRI scanning revealed extensive multinodular non-enhancing white matter lesions in the temporal lobe. There was no history of epilepsy and post mortem histology confirmed MND.Macroscopically multiple small grey well-formed, discrete and coalescent nodules were seen in the deep cortex and subcortical white matter. On histology, mature-looking neurons with large cytoplasmic vacuoles were distributed in a fibrillary background, where vacuoles were also noted. In the resected tumour scattered oligodendroglia-like cells were present. No ganglion cells were seen. The vacuolated cells exhibited immunopositivity for synaptophysin, HuC/HuD and p62 but were negative for NeuN, neurofilament, GFAP, IDH1, nestin and CD34. Electron microscopy showed non-membrane bound cytoplasmic vacuoles in the neurons and in some neuronal processes. The seizures recurred in Case 1.Some clinicopathological features of this lesion suggest a possible relationship with dysembryoplastic neuroepithelial tumour (DNT) although the morphological features are not typical of DNT. Case 2 demonstrates that MVNT may remain asymptomatic.
Department of Clinical Neuropathology, King's College Hospital NHS Foundation Trust, London SE5 9RS, United Kingdom. istvan.bodi@nhs.net.
Istvan
I
Bodi
Olimpia
O
Curran
Richard
R
Selway
Robert
R
Elwes
Juan
J
Burrone
Ross
R
Laxton
Safa
S
Al-Sarraj
Mrinalini
M
Honavar
D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000150:complications / Q000473:pathology*; D005260:Female; D005625:Frontal Lobe / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D006801:Humans; D008279:Magnetic Resonance Imaging; D008854:Microscopy, Electron; D016472:Motor Neuron Disease / Q000209:etiology; D018302:Neoplasms, Neuroepithelial / Q000150:complications / Q000473:pathology*; D009419:Nerve Tissue Proteins / Q000378:metabolism; D012640:Seizures / Q000209:etiology
D009419:Nerve Tissue Proteins