Objective: To report the first case of Multinodular and Vacuolating Posterior Fossa Lesions of Unknown Significance (MV-PLUS) with atypical supratentorial temporal extension, expanding the known radiological spectrum of this entity.

Background: MV-PLUS is a rare benign lesion first described in 2019. The diagnosis is radiological, characterized by T2-FLAIR-hyperintense nodular clusters, T1-hypointense, without gadolinium enhancement or diffusion restriction, exclusively located in the posterior fossa. Till now, fewer than 25 cases worldwide have been reported. No prior case of supratentorial extension has been documented. Recognition of their typical imaging pattern is essential to avoid misdiagnosis and unnecessary surgical intervention.

Method: A 32-year-old female presented with chronic refractory headache, vertigo, photophobia, dysphasia, amaurosis fugax, and Uhthoff phenomenon. Neurological examination revealed bilateral grade II papilledema, left APD with anisocoria, left temporal hemianopia, bilateral Hoffmann’s and Marinescu’s, left limb monoparesis, ataxic gait and distal polymyoclonus. Brain MRI demonstrated multiple (>10) round T2-hyperintense, T1-hypointense, non-enhancing, non-restricting lesions (2–9 mm) in the cerebellar vermis with minimal parasagittal extension, alongside two small oval lesions in the deep left temporal region sharing identical signal characteristics representing a supratentorial distribution not previously described in MV-PLUS.

Results: Patient’s management was conservative with symptomatic pharmacological therapy and followed up for five years. No lession growth, changes in number, volume or malignant transformation were observed, confirming the long-term radiological stability.

Conclusion: This is the first reported case of MV-PLUS in Peru and the first worldwide with supratentorial temporal extension. This unique presentation broadens the radiological profile of MV-PLUS and adds to the evidence that it usually follows a benign, nonprogressive course. Identifying these imaging patterns is important for avoiding misdiagnosis and unnecessary procedures.

References: 1. Lecler A, Bailleux J, Carsin B, Adle-Biassette H, Baloglu S, Bogey C, et al. Multinodular and Vacuolating Posterior Fossa Lesions of Unknown Significance. AJNR Am J Neuroradiol. 2019;40(10):1689-94. doi: 10.3174/ajnr.A6223.
2. Yasuoka Y, Yamasaki F, Ozono I, et al. Multinodular and vacuolating posterior fossa lesions of unknown significance: a long-term observational study and literature review. Childs Nerv Syst. 2025;41:63. doi: 10.1007/s00381-024-06721-0.
3. Calandrelli R, Mallio CA, Bernetti C, Pilato F. Multinodular and Vacuolating Neuronal Tumors: Imaging Features, Diagnosis, and Management Challenges. Diagnostics. 2025;15:334. doi: 10.3390/diagnostics15030334.
4. Nunes RH, Hsu CC, da Rocha AJ, do Amaral LLF, Godoy LFS, Watkins TW, 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;38(10):1899-904. doi: 10.3174/ajnr.A5281.
5. Passanisi M, Marrone S, Scalia G, Cicero S, Chaurasia B, Umana GE. Vermian multinodular and vacuolating lesion of unknown significance discovered following syncope: a case report. Ann Med Surg (Lond) [Internet]. 2024;86(10):6231–5. Disponible en: http://dx.doi.org/10.1097/MS9.0000000000002493

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MDS Abstracts - https://www.mdsabstracts.org/abstract/redefining-anatomical-boundaries-an-atypical-case-of-mv-plus-with-supratentorial-extension/