Gautam Dutta1, Daljit Singh2, Ravindra Kumar Saran3, Hukum Singh2, Arvind Kumar Srivastava2, Anita Jagetia2. 1. Department of Neuro-Surgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India. Electronic address: gautamblue@hotmail.com. 2. Department of Neuro-Surgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India. 3. Department of Pathology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India.
Abstract
BACKGROUND: Lhermitte-Duclos disease is an extremely rare pathologic entity characterized by a cerebellar mass composed of enlarged cerebellar folia containing abnormal ganglion cells. This entity usually presents in young and middle-aged adults and rarely in children. There is no study in the literature analyzing the long-term clinical course of this disease to assess the behavior primarily because of its rarity. CASE DESCRIPTION: We present our experience with a 7-year-old patient of Lhermitte-Duclos disease who was followed up for 5 years and found to have progressed to bilateral World Health Organization grade IV medulloblastoma. This case denotes the malignant potential of this rare disorder. CONCLUSIONS: LDD is seen rarely and demands a high degree of suspicion in patients presenting with cerebellar mass and/or imaging characteristics. It is prudent to keep these patients in close follow-up for early detection of malignant transformation.
BACKGROUND:Lhermitte-Duclos disease is an extremely rare pathologic entity characterized by a cerebellar mass composed of enlarged cerebellar folia containing abnormal ganglion cells. This entity usually presents in young and middle-aged adults and rarely in children. There is no study in the literature analyzing the long-term clinical course of this disease to assess the behavior primarily because of its rarity. CASE DESCRIPTION: We present our experience with a 7-year-old patient of Lhermitte-Duclos disease who was followed up for 5 years and found to have progressed to bilateral World Health Organization grade IV medulloblastoma. This case denotes the malignant potential of this rare disorder. CONCLUSIONS: LDD is seen rarely and demands a high degree of suspicion in patients presenting with cerebellar mass and/or imaging characteristics. It is prudent to keep these patients in close follow-up for early detection of malignant transformation.
Authors: Nahal Haddadi; Glena Travis; Najah T Nassif; Ann M Simpson; Deborah J Marsh Journal: Cold Spring Harb Perspect Med Date: 2020-05-01 Impact factor: 6.915