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Case No. 618
2022-03-01~2022-03-31
출제자 : 장진희
Hit : 392
| Period | 2022-03-01~2022-03-31 |
|---|---|
| Diagnosis | Dysplastic cerebellar gangliocytoma |
| Gender |
|
| Age | 62 |
| Clinical information | 62/F 임상정보 Rt breast cancer s/p mastectomy (1991) s/p thyroidectomy (2002) For evaluation of malignancy No neurologic symptom |
| Discussion | Dysplastic cerebellar gangliocytoma - occurs when in association with Lhermitte-Duclos disease (LDD) - Hereditary (AD), Sporadic (new mutations) • Hamartomatous cerebellar lesion characterized by enlarged cerebellar folia and replacement of internal granular layer by dysplastic ganglion cells - Increased risk of malignancy : 89% lifetime risk of cancer Breast cancer: 30-50% Thyroid cancer: 5%, usually follicular • Typically present in young adults (20~40s) • encountered at all ages • Lifetime risk in Cowden syndrome : 32% • Can be symptomatic : ataxia, headache, seizure, mass effect, IICP Radiology finding: CT Non-specific low density cerebellar mass± calcification MR Widened cerebellar folia with striated/tigroid appearance; coduroy/laminated apperance T1WI low, T2WI high, DWI (similar to GM or T2 shine through), No enhancement (rare) |
| Correct answer |
Correct Answer
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