Lhermitte-Duclos Disease: A Rare Entity With Typical Histology but Ambiguous Histogenesis.

Autor: Rajeswarie, R. T., Mallik, Dattatraya, Gopal, Swaroop
Předmět:
Zdroj: International Journal of Surgical Pathology; Sep2024, Vol. 32 Issue 6, p1113-1116, 4p
Abstrakt: Lhermitte-Duclos Disease (LDD) is a rare condition characterized by a cerebellar mass composed of dysplastic ganglion cells. It is classified as a glioneuronal and neuronal tumor. The exact nature of LDD is still unclear, with debate over whether it is hamartomatous or neoplastic in nature. LDD is associated with Cowden syndrome (CS), a rare genetic disorder that increases the risk of certain cancers. The activation of the PTEN/AKT/mTOR pathway is believed to play a role in the pathogenesis of LDD. Diagnosis of LDD may prompt further screening for CS and associated malignancies. Surgical resection is the main treatment for LDD, and long-term follow-up is important for early detection of associated malignancies. [Extracted from the article]
Databáze: Complementary Index