Lara-Torres HR, Sandoval-Balanzario MA, Rodríguez-Cervantes J, Barroso-Rodríguez N, Gómez-Ramírez AM, Becerra-Lomelí MM

Language: Spanish
References: 13
Page: 402-407
PDF size: 228.02 Kb.

ABSTRACT

Introduction. Dysplastic cerebellar gangliocytoma was first described in 1920 by Lhermitte and Duclos, authors who gave their name to the entity. It is a rare condition, which is characterized by a very slowly growing unilateral tumor mass of the cerebellar cortex, producing increased intracranial pressure. In 1991 Padberg et al. described the association of Lhermitte-Duclos disease and Cowden syndrome. The relationship between these 2 entities has been associated with mutations of the phosphatase and tensin homolog deleted on chromosome (PTEN) gene. Magnetic resonance imaging findings are the hallmark of this entity, and the diagnosis is established without hystopathologic studies.
Case report. We inform a case of a 17 years old girl with Lhermitte-Duclos of the right cerebellar hemisphere without Cowden syndrome.

REFERENCES

1. Nowak DA, Trost HA. Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? Acta Neurol Scand. 2002; 105: 137-45.

2. Kwon CH, Zhu X, Zhang J. Pten regulates neuronal soma size: a mouse model of Lhermitte-Duclos disease. Nat Genet. 2001; 26: 404-11.

3. 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): 1-6.

4. Demaerel P, Calenbergh FV, Wilms G. Lhermitte-Duclos disease: a tumour or not a tumour. Acta Neurol Scand. 2003; 108: 294-5.

5. Kleihues P, Cavenee WK. Pathology and genetics of tumours of the nervous system. WHO Classification of tumours. Lyon: IARC Press; 2000. p. 235-7.

6. Vantomme N, Calenbergh FV, Goffin J, Sciot R, Demaerel Ph, Plets C. Lhermitte-Duclos disease is a clinical manifestation of Cowden´s syndrome. Surg Neurol. 2001; 56: 201-5.

7. Barone F, Noubari BA, Torrisi A, Lanzafame S, Tropea R, Mancuso P. Lhermitte Duclos disease and Cowden disease: clinical, pathological and neuroimaging study of a case. J Neurosug Sci. 2000; 44: 234-7.

8. Derrey S, Proust F, Debono B, Langlois O, Layet A, Layet V, et al. Association between Cowden syndrome and Lhermitte-Duclos disease: report of two cases and review of the literature. Surg Neurol. 2004; 61: 447-54.

9. Lei X, Pei-yi G, Yan L. Magnetic resonance imaging findings in Lhermitte-Duclos disease: reports of three cases. CMJ. 2005; 118: 1933-6.

10. Lee, Tan SY. An unusual but distinctive cerebellar formation. Pathology. 2003; 35: 256-7.

11. Chen KS, Hung PC, Wang HS, Jung SM, Ng SH. Medulloblastoma or cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease)? Pediatr Neurol. 2002; 27: 404-6.

12. Lantos PI, Louis DN, Rosemblum MK, Kleihues P. Tumors of the nervous system. En: Graham I, Lantos P, editores. Greenfields neuropathology. 7th ed. London, Eng.: Arnold; 2002. p. 767-1052.

13. Domingo Z, Fisher-Jeffes ND, de Villiers JC. Malignant occipital astrocytoma in a patient with Lhermitte-Duclos disease (cerebellar dysplastic gangliocytoma). Br J Neurosurg. 1996; 10: 99-102.