Lhermitte-Duclos disease presenting with positron emission tomography-magnetic resonance fusion imaging: a case report

Ferdinando Calabria,Claudio Colonnese,Manlio Barbarisi,Giovanni Grillea,Marcello Bartolo,Maddalena Zinzi,Orazio Schillaci,Cristina Grasso,Emanuele Siravo,Giampaolo Cantore

doi:10.1186/1752-1947-6-76

Abstract

IntroductionLhermitte-Duclos disease or dysplastic gangliocytoma of the cerebellum is an extremely rare tumor. It is a slowly enlarging mass within the cerebellar cortex. The majority of cases are diagnosed in the third or fourth decade of life.Case presentationWe report the case of a 37-year-old Caucasian woman who underwent positron emission tomography-computed tomography with fluorine-18-fluorodeoxyglucose for evaluation of a solitary lung node. No pathological uptake was detected in the solitary lung node but the positron emission tomography-computed tomography of her brain showed intense tracer uptake, suggestive of a malignant neoplasm, in a mass in her left cerebellar lobe. Our patient had experienced two years of occipital headache and movement disorder. Subsequently, magnetic resonance imaging was performed with contrast agent administration, showing a large subtentorial mass in her left cerebellar hemisphere, with compression and dislocation of the fourth ventricle. Metabolic data provided by positron emission tomography and morphological magnetic resonance imaging views were fused in post-processing, allowing a diagnosis of dysplastic gangliocytoma with increased glucose metabolism. Total resection of the tumor was performed and histological examination confirmed the diagnosis of Lhermitte-Duclos disease.ConclusionsOur case indicates that increased uptake of fluorine-18-fluorodeoxyglucose may be misinterpreted as a neoplastic process in the evaluation of patients with Lhermitte-Duclos disease, but supports the usefulness of integrated positron emission tomography-magnetic resonance imaging in the exact pathophysiologic explanation of this disease and in making the correct diagnosis. However, an accurate physical examination and exact knowledge of clinical data is of the utmost importance.

Highlights

Lhermitte-Duclos disease or dysplastic gangliocytoma of the cerebellum is an extremely rare tumor
Our case indicates that increased uptake of fluorine-18-fluorodeoxyglucose may be misinterpreted as a neoplastic process in the evaluation of patients with Lhermitte-Duclos disease, but supports the usefulness of integrated positron emission tomography-magnetic resonance imaging in the exact pathophysiologic explanation of this disease and in making the correct diagnosis
Lhermitte-Duclos disease (LDD), or dysplastic cerebellar gangliocytoma, is a rare tumor arising from the cerebellar cortex, first described clinically in 1920 by Lhermitte and Duclos [1]

Summary

Conclusions

The exact pathophysiologic features of LDD are not well known, our case report indicates that morphofunctional imaging reflects an important aspect of this controversial disease: caution is needed in interpreting F18-FDG PET because the tracer can intensely accumulate in LDD, mimicking a malignant tumor. We suggest that correlative imaging with MRI and accurate knowledge of the clinical history of the patient are of the utmost importance. Simultaneous evaluation of both fused imaging modalities may provide new insight on the study of the brain and its changes in disease. Consent Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images.

Introduction

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