Surgical treatment of Lhermitte–Duclos disease (dysplastic gangliocytoma of the cerebellum)
https://doi.org/10.17650/1683-3295-2020-22-1-77-82
Abstract
The study objective is to describe a clinical case of a rare disorder of the cerebellum, Lhermitte–Duclos disease.
Clinical case. The Federal Neurosurgical Center (Novosibirsk) admitted a male patient, 40 years old. Medical history showed that in 2017 he underwent ventriculoperitoneal shunt due to hypertensive hydrocephalus syndrome caused by space-occupying mass of the right hemisphere of the cerebellum. In June of 2017, the patient was consulted by a neurosurgeon. Later, an increase in the mass, partial atrophy of the optic disks were observed. Due to the patient’s complaints of headache, ataxia, vertigo, as well as insufficient effect of the shunt, microsurgical removal of the space-occupying mass in the cerebellum by right paramedian suboccipital craniotomy was performed. Gangliocytoma removal was performed under constant neurophysiological control which allowed to fully resect the gangliocytoma inside intact brain tissue. Histological and immunohistological examinations allowed to diagnose grade I dysplastic gangliocytoma of the cerebellum (Lhermitte–Duclos disease). In the postoperative period, brain symptoms regressed fully, vestibulocerebellar syndrome persisted. The patient was discharged in satisfactory condition. No negative dynamics were observed in 7 months of follow-up.
Conclusion. Due to the rareness of Lhermitte–Duclos disease, currently there are no established approaches to treatment, however, in symptomatic course there are indications for surgical removal of the gangliocytoma. Radicality of resection should be correlated with the risk of neurological defects. In our case, surgical treatment allowed to improve the patient’s condition, prevent further progression of the gangliocytoma and obtain an accurate histological diagnosis.
About the Authors
A. A. Bimurzin
Federal Neurosurgical Center (Novosibirsk), Ministry of Health of Russia
Russian Federation
Russian Federation
132 / 1 Nemirovicha-Danchenko St., Novosibirsk 630087
A. V. Kalinovsky
Federal Neurosurgical Center (Novosibirsk), Ministry of Health of Russia
Russian Federation
Russian Federation
132 / 1 Nemirovicha-Danchenko St., Novosibirsk 630087
References
1. Rainov N., Holzhausen H., Burkert W. Dysplastic gangliocytoma of the cerebellum (Lhermitte–Duclos disease). Clin Neurol Neurosurg 1995;97(2):175–80. DOI: 10.1016/0303-8467(95)00017-E.
2. Nowak D.A., Trost H.A. Lhermitte– Duclоs disеase (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm. Acta Neurologica Scandinavica 2002;105(3):137–45. DOI: 10.1034/j.1600-0404.2002.1r127.
3. Shimansky V.N., Karnaukhov V.V., Shishkina L.V., Vinogradov E.V. The successful treatment of a patient with Lhermitte–Duclos disease (a case report and literature rewiew). Zhurnal “Voprosy neyrokhirurgii” im. N.N. Burdenko = Burdenko’s Journal of Neurosurgery 2015;79(4):78–83. (In Russ.). DOI: 10.17116/neiro201579478-83.
4. Nieuwenhuis M., Kets C., Murphy-Ryan M. et al. Cancer risk and genotype-phenotype correlations in PTEN hamartoma tumor syndrome. Fam Cancer 2014;13(1):57–63. DOI: 10.1007/s10689-013-9674-3.
5. Lhermitte J., Duclos P. Sur un ganglio-neurome diffus du cortex du cervelet. Bull Assoc Franç Etude Cancer 1920;9:99–107.
6. Demaerel P., Van Calenbergh F., Wilms G. Lhermitte–Duclоs disease: a tumour or not a tumour. Acta Neurol Scand 2003;108(4):294–5. DOI: 10.1034/j.1600-0404.2003.00156.
7. Padberg G.W., Schot D.L., Vielvoye G.J. et al. Lhermitte–Duclos disease and Cowden disease: a single phakomatosis. Ann Neurol 1991;29(5):517–23. DOI: 10.1002/ana.410290511.
8. Murray C., Shipman P., Khangure M. et al. Lhermitte–Duclоs disease associated with Cowden’s syndrome: case report and literature review. Australas Radiol 2001;45(3):343–6. DOI: 10.1046/j.1440-1673.2001.00933.x.
9. Ambler M., Pogacar S., Sidman R. Lhermitte–Duclos disease (granule cell hypertrophy of the cerebellum) pathologcal analysis of the first familial cases. J Neuropathol Exp Neurol 1969;28(4);622–47. DOI: 10.1097/00005072-196910000-00005.
10. Takei H., Dauser R., Su J. et al. Anaplastic ganglioglioma arising from a Lhermitte– Duclos like lesion. J Neurosurg 2007;107(2):137–42. DOI: 10.3171/ped-07/08/137.
11. Murata J., Tada M., Sawamura Y. et al. Dysplastic gangliocytoma (Lhermitte–Duclos disease) associated with Cowden disease: report of a case and review of the literature for the genetic relationship between the two diseases. J Neurooncol 1999;41(2):129–36. DOI: 10.1023/a:1006167421100.
12. Robinson S., Cohen A. Cowden disease and Lhermitte–Duclos disease: an update. Case report and review of the literature. Neurosurg Focus 2006;20(1):E6. DOI: 10.3171/foc.2006.20.1.7.
13. Di Cristofano A., Pesce B., Cordon-Cardo C., Pandolfi P.P. PTEN is essential for embryonic development and tumor suppression. Nat Genet 1998;19(4):348–55. DOI: 10.1038/1235.
14. Saito H., Ogasawara K., Beppu T. et al. Biological characteristics of a cerebellar mass regrowing after removal in a patient with Lhermitte–Duclos disease: emission tomography studies. Case Rep Neurol 2014;6(1):96–100. DOI: 10.1159/000362118.
15. Roessmann U., Wongmongkolrit T. Dysplastic gangliocytoma of cerebellum in a newborn. Case report. J Neurosurg 1984;60(4):845–7. DOI: 10.3171/jns.1984.60.4.0845.
16. Pritchett P.S., King T.I. Dysplastic gangliocytoma of the cerebellum – an ultrastructural study. Acta Neuropathol 1978;42(1):1–5. DOI: 10.1007/bf01273258.
17. Cessaga E.C. Lhermitte–Duclos disease (diffuse hypertrophy of the cere bellum). Report of two cases. Neurosurg Rev 1980;3(2):151–8. DOI: 10.1007/bf01644067.
18. Maran O.R., Johnson P.C., Spetzler R.F. Recurrent Lhermitte–Duclos disease in a child. Case report. J Neurosurg 1988;69(4):599–603. DOI: 10.3171/jns.1988.69.4.0599.
19. Marcus C.D., Galeon M., Peruzzi P. et al. Lhermitte–Duclos disease associated with syringomyelia. Neuroradiology 1996;38(6): 539–31. DOI: 10.1007/bf00626089.
20. Wolansky L.J., Malantic G.P., Hear Y.R. et al. Preoperative MRI diagnosis of Lhermitte–Duclos disease: case report with associated enlarged vessels and syrinx. Surg Neurol 1996;45(5):470–5. DOI: 10.1016/0090-3019(95)00244-8.
21. Kulkantrakorn K., Awwad E.E., Levy B. et al. MRI in Lhermitte–Duclos disease. Neurology 1997;48(3):725–31. DOI: 10.1212/WNL.48.3.725.
22. Reznik M., Schoenen J. Lhermitte– Duclos disease. Acta Neuropathol 1983;59(2):88–94. DOI: 10.1007/bf00691592.
23. Prestor В. Dysplastic gangliocytoma of the cerebellum (Lhermitte–Duclos disease). J Clin Neurosci 2006;13(8):877–81. DOI: 10.1016/j.jocn.2005.07.018.
24. Golden N., Tjokorda M.G., Sri M. et al. Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte–Duclos disease) in a developing country: case report and review of the literature. Asian J Neurosurg 2016;11(2):170. DOI: 10.4103/1793-5482.145091.
25. Yağci-Küpeli B., Oguz K.K., Bilen A. et al. An unusual cause of posterior fossa mass: Lhermitte–Duclos disease. J Neurol Sci 2010;290(1–2):138–41. DOI: 10.1016/j.jns.2009.12.010.
26. Huang S., Zhang G., Zhang J. Similar MR imaging characteristics but different pathological changes: a misdiagnosis for Lhermitte–Duclos disease and review of the literature. Int J Clin Exp Pathol 2015;8(6): 7583–7.
27. Afshar-Oromieh A., Linhart H., Podlesek В. et al. Postoperative cerebellar mutism in adult patients with Lhermitte– Duclos disease. Neurosurg Rev 2010;33(4):401–8. DOI: 10.1007/s10143-010-0278-1.
Review
For citations:
Bimurzin A.A., Kalinovsky A.V. Surgical treatment of Lhermitte–Duclos disease (dysplastic gangliocytoma of the cerebellum). Russian journal of neurosurgery. 2020;22(1):77-82. (In Russ.) https://doi.org/10.17650/1683-3295-2020-22-1-77-82
Views:
1944
Email this article 