Multinodular and Vacuolating Neuronal Tumor

1. Multinodular and Vacuolating Neuronal Tumor
Ade Wijaya, MD – July 2022

2. Introduction
• Benign lesion of cerebrum
• Described recently in the WHO CNS tumours in
2016
• Uncommon – rare / non-fatal
• No gender predisposition
Zahra, C., & Grech, R. (2018). Multinodular and vacuolating neuronal tumor. Open Access Macedonian Journal of Medical Sciences, 6(9), 1697.
Huse JT, Edgar M, Halliday J, Mikolaenko I, Lavi E, Rosenblum MK. Multinodular and vacuolating neuronal tumours of the cerebrum:10 cases of a distinctive seizure-associated
lesion. Brain Pathol. 2013;23:515–24.

3. Key Features
• Multiple tumor nodules
• Vacuolar alteration
• Widespread immunolabeling for human
neuronal protein HuC/HuD
Shitara, S., Tokime, T., & Akiyama, Y. (2018). Multinodular and vacuolating neuronal tumor: a case report and literature review. Surgical neurology international, 9.

4. Morphology
• Non-neurocyte, glial/neuronal lesion
• Unique membrane-bound appearance of the
neuronal cytoplasm
• Round to elliptic shape
• 1 mm to 5 mm in size
• Can be found as separate entities or in clusters
• Shares features of a developmental abnormality
and neoplasm
Nunes RH, Hsu CC2, da Rocha AJ, et al. Multinodular and Vacuolating Neuronal Tumor of the Cerebrum:A New “Leave Me Alone” Lesion with a Characteristic Imaging
Pattern. AJNR Am J Neuroradiol. 2017

5. Clinical Presentation
• Adult-onset seizure
• Predominantly occur in temporal lobe
Huse JT, Edgar M, Halliday J, Mikolaenko I, Lavi E, Rosenblum MK. Multinodular and vacuolating neuronal tumours of the cerebrum:10 cases of a distinctive seizure-associated
lesion. Brain Pathol. 2013;23:515–24.

6. Diagnosis: MRI
• Non-contrast enhancing
• T2/FLAIR hyperintense with the absence of
restricted diffusion
• no mass effect or encompassing oedema nor
observable calcification
• T1 weighted imaging, lesions range from
isointense to hypointense
Zahra, C., & Grech, R. (2018). Multinodular and vacuolating neuronal tumor. Open Access Macedonian Journal of Medical Sciences, 6(9), 1697.

7. Diagnosis: MRI
Zahra, C., & Grech, R. (2018). Multinodular and vacuolating neuronal tumor. Open Access Macedonian Journal of Medical Sciences, 6(9), 1697.

8. Differential Diagnosis
• Dysembryoplastic neuroepithelial tumor (DNT)
• Low-grade glioma
• Cortical dysplasia
• Hamartoma
Shitara, S., Tokime, T., & Akiyama, Y. (2018). Multinodular and vacuolating neuronal tumor: a case report and literature review. Surgical neurology international, 9.

9. Management
• Wait and see
• Antiseizure medication
• Surgery in intractable epilepsy cases
Shitara, S., Tokime, T., & Akiyama, Y. (2018). Multinodular and vacuolating neuronal tumor: a case report and literature review. Surgical neurology international, 9.

10. Summary
• A new neuronal cytoplasmic pattern described in
the WHO of CNS tumors 2016
• Benign, multinodular, well circumscribed, non-
contrast enhancing, T2/FLAIR hyperintense,
involving the cortex and superficial white
matter, with absence of restricted diffusion and
mass effect