Diverse imaging findings of Lhermitte-Duclos disease

AIM: To evaluate the diverse clinical and imaging features of Lhermitte-Duclos disease (LDD) and its subgroup comparison.MATERIALS AND METHODS: Clinical data from 21 patients with LDD were collected, including eight patients with LDD without other tumours and 13 LDD with other tumours. Redefined diagnostic criteria are used to evaluate Cowden Syndrome. Imaging indicators were analysed retrospectively to extract typical and atypical features. Imaging findings and preoperative diagnostic accuracy were compared between the subgroups.RESULTS: None of these patients met the redefined diagnostic criteria. The typical tiger stripe sign was seen in most LDD lesions (13/29, 61.9%), with lower density (29.66 f 2.51 versus 37.81 f 2.76 HU, p<0.001) and higher apparent diffusion coefficient (ADC) value (1.04 f 0.05 x 10-3 versus 0.74 f 0.03 x 10-3 mm2/s, p<0.001) than that of the normal cerebellum. Atypically, some lesions showed abnormal vessels (8/21, 38.1%), intratumoural calcification (3/21, 14.29%), intratumoural haemorrhage (4/21, 19.05%), peritumoural oedema (6/21, 28.57%), and heterogeneous enhancement (5/21, 23.81%). The typical tiger stripe sign was more common in LDD with other tumours (84.62% versus 25%, p=0.018). Although LDD without other tumours was more common with abnormal vessels (75% versus 15.38%, p=0.018), intratumoural calcification (37.5% versus 0, p=0.042), intratumoural haemorrhage (50% versus 0, p=0.012), peritumoural oedema (62.5% versus 7.69%, p=0.014) and heterogeneous enhancement (50% versus 7.69%, p=0.047). Preoperative diagnostic accuracy was higher in LDD with other tumours than LDD without other tumours (76.92% versus 25%, p=0.032).CONCLUSION: The tiger stripe sign of LDD is characteristic, but not unique. With or without other tumours, it may be associated with the imaging diversity. Combining typical and atypical signs can improve the imaging assessment of LDD.(c) 2022 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

Automated summary

This study evaluated the clinical and imaging features of Lhermitte-Duclos disease (LDD) and its subgroup comparison. The typical tiger stripe sign was commonly observed in LDD lesions, and it had lower density and higher apparent diffusion coefficient values compared to the normal cerebellum. Atypical features such as abnormal vessels, intratumoural calcification, intratumoural haemorrhage, peritumoural oedema, and heterogeneous enhancement were also found in some lesions. The presence of other tumours was associated with a higher prevalence of the tiger stripe sign and a higher preoperative diagnostic accuracy.