title: Specificity of splenopancreatic field abnormality in trisomy 13 syndrome: macroscopic and histological analysis in 21 autopsy cases.
abstract: Splenopancreatic field abnormalities were carefully examined macroscopically and microscopically in 21 trisomy 13 (TR13) subjects, and the results were compared with those of non-TR13 control cases. Of the 21 TR13 subjects, 12 had intrapancreatic splenic tissue (IPST) and 17 had fusion of the pancreatic tail and splenic hilus and/or accessory spleen (FPS/FPAS). All 21 had IPST and/or FPS/FPAS. Five of 1060 controls (non-TR13) had IPST, while two had FPS/FPAS. On histology the pancreata of TR13 subjects had intralobular ducts, with goblet cells (ILDGC) and microcysts (MC) in 17 and 18 subjects, respectively. All 21 TR13 subjects had ILDGC and/or MC. Two of 360 age-matched controls (stillbirths and neonates) had ILDGC and nine had MC. IPST, FPS/FPAS, ILDGC, and MC were considered to be highly specific for TR13, and could therefore be useful in differentiating TR13 from other malformation diseases.
authors.affiliation: Division of Pathology, Kanagawa Children's Medical Center, Yokohama, Japan. gomikiyoshi@yahoo.co.jp
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Gomi
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Sato
authors.forename: Mio
authors.initials: M
authors.lastname: Tanaka
authors.forename: Rieko
authors.initials: R
authors.lastname: Ijiri
authors.forename: Keisuke
authors.initials: K
authors.lastname: Kato
authors.forename: Ichiro
authors.initials: I
authors.lastname: Aoki
authors.forename: Yukichi
authors.initials: Y
authors.lastname: Tanaka
mesh_terms: D001344:Autopsy; D002675:Child, Preschool; D002828:Choristoma / Q000151:congenital / Q000235:genetics / Q000473:pathology*; D002882:Chromosomes, Human, Pair 13 / Q000235:genetics*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010179:Pancreas; D010182:Pancreatic Diseases / Q000151:congenital / Q000235:genetics / Q000473:pathology*; D013154:Spleen / Q000473:pathology*; D050497:Stillbirth; D014314:Trisomy / Q000473:pathology*