Neuroectodermal differentiation of the gastrointestinal tumors in the Carney triad. An ultrastructural and immunohistochemical study.
A 16-year-old female with mediastinal paraganglioma and multicentric gastroduodenal "leiomyoblastomas" was thought to have an incomplete form of the Carney triad. The histologic, ultrastructural, and immunohistochemical findings of the gastroduodenal tumors revealed features of neuroectodermal differentiation. The architecture of the smallest duodenal tumors suggested an origin from the myenteric autonomic ganglia.
Perez-Atayde
A R
AR
Department of Pathology, Children's Hospital, Boston, MA 02115.
Shamberger
R C
RC
Kozakewich
H W
HW
D000293:Adolescent; D004379:Duodenal Neoplasms / Q000473:pathology*; D004475:Ectoderm / Q000502:physiology; D005260:Female; D005725:Ganglia, Autonomic / Q000196:embryology; D005770:Gastrointestinal Neoplasms / Q000196:embryology / Q000378:metabolism / Q000648:ultrastructure; D006801:Humans; D007150:Immunohistochemistry; D007889:Leiomyoma / Q000473:pathology*; D008479:Mediastinal Neoplasms / Q000473:pathology*; D008854:Microscopy, Electron; D008954:Models, Biological; D009197:Myenteric Plexus / Q000196:embryology; D010235:Paraganglioma / Q000473:pathology*; D013274:Stomach Neoplasms / Q000473:pathology*; D013577:Syndrome