title: Mucinous adenocarcinoma arising from supratentorial intramedullary neuroenteric cyst with broncho-pulmonary differentiation.
abstract: Neuroenteric cysts are benign intradural endoderm cysts lined by gastrointestinal (GI) or tracheobronchial epithelial cells. Their malignant transformation is extremely rare and only six cases have been reported. In these cases, tissue lineage of the cystic endoderm cells giving rise to carcinoma was not clearly identified either as respiratory or as GI type. Herein, we report a case of mucinous adenocarcinoma arising from the neuroenteric cyst with broncho-pulmonary differentiation in the right cerebral hemisphere of a Japanese woman in her late 50s. The cyst wall was entirely lined by the following respiratory epithelial components: stratified bronchial ciliated columnar epithelium with basal cells positive for CK5 and p63, terminal bronchiolar Clara cells positive for thyroid transcription factor (TTF)-1, surfactant B and negative for surfactant C, type I pneumocytes positive for TTF-1, negative for surfactant B and C, and type II pneumocytes positive for TTF-1 and surfactant B and C. In addition, nests of hyperplastic single layered mucinous epithelial cells with bronchial goblet cell phenotype were also demonstrated, and histologic features were almost the same as the pulmonary type I congenital adenomatoid malformation (CCAM) with mucinous epithelial proliferation. The mucinous epithelial nests of type I CCAM are liable to develop mucinous adenocarcinoma and frequently accompany K-ras mutation and expression of p16. However, K-ras mutation and p-16 expression were not detected in this case.
authors.affiliation: Division of Diagnostic Pathology, Department of Laboratory Medicine, Shiga University of Medical Science, Otsu, Japan; Department of Pathology, Japanese Red Cross Kyoto Daini Hospital, Kyoto, Japan.
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Okabe
authors.forename: Kanade
authors.initials: K
authors.lastname: Katsura
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Yamano
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tenjin
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Nakahara
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Ishida
authors.forename: Toshikazu
authors.initials: T
authors.lastname: Kato
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000473:pathology*; D001932:Brain Neoplasms / Q000473:pathology*; D002454:Cell Differentiation; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008875:Middle Aged; D009436:Neural Tube Defects / Q000473:pathology*; D015173:Supratentorial Neoplasms / Q000473:pathology*
keywords: TTF-1; congenital cystic adenomatoid malformation; malignant transformation; neuroenteric cyst; surfactant