|Lhermitte Duclos Disease
| Primary CNS Lymphoma (PCNSL)
This is the most common type of brain lesion in adult patients with Cowden syndrome.
- Very rare, benign posterior fossa brain tumour
- It is a dysplastic gangliocytoma of the cerebellum
- Can be associated with hereditary cancer syndrome called Cowden disease.
- Abnormal development and enlargement of the cerebellum
- Can lead to an increased intracranial pressure with obstructive hydrocephalus
- Genetic changes in the PTEN gene
- Seen aged 10 to 30. Males = Females
- Progressive headache, nausea
- Cerebellar dysfunction, hydrocephalus, ataxia, visual disturbances.
- Enlarged brain (megalencephaly), hydromyelia
- Extra fingers or toes (polydactyly), Partial gigantism, macroglossia
- Magnetic resonance imaging (MRI): characteristic non-enhancing gyriform patterns with enlargement of cerebellar folia. The lesion is non enhancing with hypointense on T1- and hyperintense on T2-weighted magnetic resonance images with "tiger stripes".
- Dysplastic gangliocytoma of the cerebellum
- A conservative approach as benign and slow growing and surgery not needed if asymptomatic
- Surgery is the therapeutic procedure may be performed and complete resection is attempted in cases when symptoms persist or there is severe cerebellar oedema which can occur.