Lhermitte-Duclos disease: Clinical study with long-term follow-up in a single institution.

OBJECTIVE: LDD is a rare lesion, controversy about diagnosis and treatment of LDD and CS still exists. We aimed to clarify clinical and radiological characteristics, the optimal treatment timing and strategies of LDD with long-term follow-up.

PATIENTS AND METHODS: From January 2004 to May 2017, 12 LDD patients were enrolled in our study. The clinical and radiological features, treatment strategies and long-term follow-up data were retrospectively analyzed.

RESULTS: The mean follow-up was 89.1±36.9 months (range, 31-161months). Twelve patients (7 males and 5 females, mean age 28.0±14.8 years, range 3-55 years) were included in this study. The most frequent symptoms were those related to increased intracranial pressure. Mean duration of symptoms was 15.6±9.5 months. Average tumor size was 62±9.3mm in its maximal diameter (range, 50-84mm). Tonsillar herniation and preoperative hydrocephalus were observed in 9/12 and 12/12 patients. Four of them confirmed the diagnosis of CS. Gross total resection were achieved in 3 patients, partial in 6 patients and subtotal in 3 patients. All patients experienced improvement of symptoms. Recurrence and progression were identified in only 1/12 patient.

CONCLUSIONS: LDD has pathognomonic appearance on T2-weighted MRI. We recommend surgery decision should be based on symptoms appearance or progress in imaging. Total resection is difficult due to the indistinct tumor margin, favorable outcome and rare recurrence were also observed despite of partial or subtotal resection in our series. A long-term screening for Cowden's disease and tumor recurrence is essential for every patient.