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Posterior Fossa Intra-Axial Lesion: There Are Pathologies Beyond MetastasesFunding None.
Cerebellar metastases represent the most common lesion requiring surgical treatment in the daily neurosurgical praxis. We report a rare case with Lhermitte–Duclos disease presenting to the neurosurgical outpatient department and describe our diagnostic and treatment challenges. A 57-year-old man presented with transient cerebellar manifestations, including walking difficulties, dizziness, and a persistent occipital headache. The magnetic resonance imaging demonstrated a high-intensity lesion in the right cerebellar hemisphere on T2-weighted images. We initially considered surgery to establish a histopathological diagnosis. However, the magnetic resonance spectroscopy was compatible with a dysplastic gangliocytoma of the cerebellum. Therefore, we preferred a more conservative approach. Posterior fossa contrast-enhancing lesions are much more than metastases. The role of advanced magnetic resonance in avoiding unnecessary surgical interventions is priceless.
KeywordsLhermitte–Duclos disease - dysplastic ganglioglioma of the cerebellum - magnetic resonance spectroscopy - asymptomatic cases - cerebellum
The study was approved by the institutional ethics committee.
Article published online:
29 October 2022
© 2022. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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- 1 Ambler M, Pogacar S, Sidman R. Lhermitte-Duclos disease (granule cell hypertrophy of the cerebellum) pathological analysis of the first familial cases. J Neuropathol Exp Neurol 1969; 28 (04) 622-647
- 2 Barnholtz-Sloan JS, Sloan AE, Davis FG, Vigneau FD, Lai P, Sawaya RE. Incidence proportions of brain metastases in patients diagnosed (1973 to 2001) in the Metropolitan Detroit Cancer Surveillance System. J Clin Oncol 2004; 22 (14) 2865-2872
- 3 Coeli GNM, Silva GC da, Tiengo RR, Fernandes JOM, Fernandes WLM, Lourenço DNL. Acute cerebellitis with tonsillar herniation: a case report. Radiol Bras 2012; 45: 244-246
- 4 De Cocker LJL, Lövblad K-O, Hendrikse J. MRI of cerebellar infarction. Eur Neurol 2017; 77 (3–4): 137-146
- 5 Dragoo DD, Taher A, Wong VK. et al. PTEN hamartoma tumor syndrome/Cowden syndrome: genomics, oncogenesis, and imaging review for associated lesions and malignancy. Cancers (Basel) 2021; 13 (13) 3120
- 6 Grossman R, Ram Z. Posterior fossa intra-axial tumors in adults. World Neurosurg 2016; 88: 140-145
- 7 Klisch J, Juengling F, Spreer J. et al. Lhermitte-Duclos Disease: Assessment with MR Imaging, Positron Emission Tomography, Single-photon Emission CT, and MR Spectroscopy. Am J Neuroradiol 2001; 22 (05) 824-830
- 8 Lhermitte J, Duclos P. On a diffuse ganglioneuroma of the cortex of the cerebellum. Bull Assoc Franc Cancer 1920; 9: 99-107
- 9 Lloyd II KM, Dennis M. Cowden's disease. A possible new symptom complex with multiple system involvement. Ann Intern Med 1963; 58: 136-142
- 10 Nieder C, Spanne O, Mehta MP, Grosu AL, Geinitz H. Presentation, patterns of care, and survival in patients with brain metastases: what has changed in the last 20 years?. Cancer 2011; 117 (11) 2505-2512
- 11 Padberg GW, Schot JDL, Vielvoye GJ, Bots GT, de Beer FC. Lhermitte-Duclos disease and Cowden disease: a single phakomatosis. Ann Neurol 1991; 29 (05) 517-523
- 12 Zhang HW, Zhang YQ, Liu XL. et al. MR imaging features of Lhermitte-Duclos disease: case reports and literature review. Medicine (Baltimore) 2022; 101 (04) e28667
- 13 Stapleton SR, Wilkins PR, Bell BA. Recurrent dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) presenting with subarachnoid haemorrhage. Br J Neurosurg 1992; 6 (02) 153-156