Radiographic Findings of Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in a Woman with Cowden Syndrome

Posted By Gloria Joo
Radiographic Findings of Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) in a Woman with Cowden Syndrome

Gender, Age

Female, 56

Diagnosis

Lhermitte-Duclos disease; Dysplastic Cerebellar Gangliocytoma

History

A 56-year-old female patient with a complicated medical history of Cowden syndrome, Hashimoto’s thyroiditis, nephrolithiasis, and ductal carcinoma in situ came to the hospital with gradual onset of diplopia, flashing lights, and intractable headaches of four-weeks’ duration with no apparent cause.

Leave A Comment

You need to be logged in to leave comments.


Comments
  • Roland Talanow 2019-09-19 11:37:59

    Thank you for your case presentation.
    Can you please tell a little bit more about this case and entity?

    Gloria Joo 2019-09-21 23:01:16

    Hi Dr. Talanow,

    This case is an interesting one because it features a patient with Cowden syndrome, a rare genetic disorder involving a PTEN mutation with an estimated prevalence of 1 in 250,000. The most common type of brain tumor arising adult patients with Cowden syndrome is Lhermitte-Duclos disease (LDD). This is a mass of hamartomatous origin that usually grows unilaterally in one of the cerebellar hemispheres.

    Axial MRI of the brain is usually the best way to diagnose LDD because the mass presents in a striated pattern with T2 imaging- this pattern usually isn't seen in other posterior fossa tumors. I have some additional images (that failed to upload properly when I was submitting this case) if you're interested in looking at them!

    Reply