Journals / Türk Patoloji Dergisi / 2018 / Cilt: 34 - Sayı: 1

Lhermitte-Duclos Disease: A Rare Lesion with Variable Presentations and Obscure Histopathology

Pages
92–99
Publication Date
2022-11-05
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Pathological diagnosis of Lhermitte-Duclos disease can be extremely difficult in the absence of proper clinical information and the pathologist should be watchful for any irregularity in the internal granular layer in evaluating the cerebellar tissue which is otherwise normal

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Since Lhermitte-Duclos is a quite rare disorder with both neoplastic and hamartomatous features, clinical and pathological diagnosis can sometimes be challenging.
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Key Findings & Data:Although enlargement of the internal granular layer of the cerebellum is a consistent finding in our cases, morphological severity was highly variable and in some cases the enlargement was insignificant.
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Conclusion & Impact:Pathological diagnosis of Lhermitte-Duclos disease can be extremely difficult in the absence of proper clinical information and the pathologist should be watchful for any irregularity in the internal granular layer in evaluating the cerebellar tissue which is otherwise normal
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Since Lhermitte-Duclos is a quite rare disorder with both neoplastic and hamartomatous features, clinical and pathological diagnosis can sometimes be challenging. For the pathologist it is of extreme importance to be aware of variable clinical and histopathological presentations of such a rare lesion particularly to differentiate it from the low-grade glial and neuronal tumors. We present four cases of Lhermitte-Duclos in a histopathological perspective. Although enlargement of the internal granular layer of the cerebellum is a consistent finding in our cases, morphological severity was highly variable and in some cases the enlargement was insignificant. Frozen sections of one case did not reveal diagnostic findings. The vacuolar change observed in the paraffin sections was obscure in the frozen. Pathological diagnosis of Lhermitte-Duclos disease can be extremely difficult in the absence of proper clinical information and the pathologist should be watchful for any irregularity in the internal granular layer in evaluating the cerebellar tissue which is otherwise normal

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Cite This Article

ARIKÖK, A. T., ALPER, M., TÜRKOĞLU, E., ÖNDER, E. (2022). Lhermitte-Duclos Disease: A Rare Lesion with Variable Presentations and Obscure Histopathology. Türk Patoloji Dergisi, Cilt: 34 - Sayı: 1, 92–99. https://ajindex.com/articles/640477

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References (12)

  1. WHO Classification of Tumours of the Central Nervous System. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. 4th ed. Lyon: IARC; 2007
  2. Nowak DA, Trost HA. Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): A malformation, hamartoma or neoplasm? Acta Neurol Scand. 2002;105:137-45
  3. Lhermitte J, Duclos P. Sur un ganglioneurome diffus du cortex du cervelet. Bull Assoc Fr Etud Cancer. 1920;9:99-107
  4. Robinson S, Cohen AR. Cowden disease and Lhermitte-Duclos disease: An update. Case report and review of the literature. Neurosurg Focus. 2006;20(1):E6
  5. Zhou XP, Marsh DJ, Morrison CD, Chaudhury AR, Maxwell M, Reifenberger G, Eng C. Germline inactivation of PTEN and dysregulation of the phosphoinositol-3-kinase/Akt pathway cause human Lhermitte-Duclos disease in adults. Am J Hum Genet. 2003;73:1191-8
  6. Abel TW, Baker SJ, Fraser MM, Tihan T, Nelson JS, Yachnis AT, Bouffard JP, Mena H, Burger PC, Eberhart CG. Lhermitte-Duclos disease: A report of 31 cases with immunohistochemical analysis of the PTEN/AKT/mTOR Pathway. J Neuropathol Exp Neurol. 2005;64:341-9
  7. Nowak DA, Trost HA, Porr A, Stölzle A, Lumenta CB. LhermitteDuclos disease (Dysplastic gangliocytoma of the cerebellum). Clin Neurol Neurosurg. 2001;103:105-10
  8. Wei G, Zhang W, Li Q, Kang X, Zhao H, Liu X, Tang X, Wu Y, Han J, Yin H. Magnetic resonance characteristics of adultonset Lhermitte-Duclos disease: An indicator for active cancer surveillance? Mol Clin Oncol. 2012; 2: 415-20
  9. Thomas B, Krishnamoorthy T, Radhakrishnan VV, Kesavadas C. Advanced MR imaging in Lhermitte-Duclos disease: Moving closer to pathology and pathophysiology. Neuroradiology 10. Bozbuga M, Gulec I, Suslu HT, Bayindir C. Bilateral LhermitteDuclos Disease. Neurol India. 2010;58:309-11
  10. Douglas-Akinwande AC, Payner TD, Hattab EM. Medulloblastoma mimicking Lhermitte-Duclos disease on MRI and CT. Clin Neurol Neurosurg. 2009;111:536-9
  11. Savardekar A, Salunke P, Ahuja CK, Rane S, Singla N. Unusual presentation in adult medulloblastomas: Imaging features mimicking cerebellar dysplastic gangliocytoma (LhermitteDuclos disease). Neurol India. 2012;60:555-7
  12. Capone Mori A, Hoeltzenbein M, Poetsch M, Schneider JF, Brandner S, Boltshauser E. Lhermitte-Duclos disease in 3 children: A clinical long-term observation. Neuropediatrics. 2003;34:30-5