Gallbladder Papilloma in a Child Unmasking Metachromatic Leukodystrophy: A Case Report With Review of Literature. (4th July 2019)

• Record Type: Journal Article
• Authors: Abdel Aziz, Marwa; Kotb, Mostafa; Abdelmeguid, Yasmine; Shehata, Sameh; Abdel-Hadi, Mona
• Is Part Of: Fetal and pediatric pathology. Volume 38:Number 4(2019)
• Page Start: 345
• Page End: 351
• Publication Date: 2019-07-04
• Subjects: Metachromatic leukodystrophy -- gallbladder mass -- tubulo-villous papilloma; Pathology, Molecular -- Periodicals; Pediatrics -- Periodicals; Molecular biology -- Periodicals; Pediatric pathology -- Periodicals; Fetal Diseases -- pathology -- Periodicals; Infant, Newborn, Diseases -- pathology -- Periodicals; Pediatrics -- Periodicals; 618.92007
• DOI: 10.1080/15513815.2019.1588442
• Languages: English
• ISSNs: 1551-3815

Abstract

Abstract: Background : Metachromatic leukodystrophy (MLD) is a lipid storage disease characterized the accumulation of sulfatides in different viscera including the gallbladder. Case report : A 2-year-old girl had upper right quadrant lesion that was preoperatively thought to be a biliary cystadenoma. Histologically, the gallbladder lesion was a tubulo-villous papilloma with multiple foci of papillary mucosal hyperplasia. Many storage histiocytes containing metachromatic granules, characteristic of MLD, were present in the tips of the papillae. MLD was later confirmed by enzyme studies. Conclusion : Gallbladder papilloma can be the presenting feature of MLD.