Atrio-ventricular nodal tumor associated with polyendocrine anomalies.
We report the clinical and autopsy findings of a young female suffering of complete heart block since the age of 11 and who suddenly died at the age of 32 because of a polycystic atrio-ventricular node tumor. In association with this tumor, we observed a hyperplasia of the islets of Langherans, a true thymic hyperplasia, bilateral multicystic ovaries, adrenal tissue heterotopia and clear cell adenomatosis in the right kidney. We suggest that the polycystic atrio-ventricular node tumor may be part of a complex syndrome with endocrine disorders and heterotopias.
Declich
P
P
Department of Pathology, Legnano General Hospital, Milano, Italy.
Sironi
M
M
Isimbaldi
G
G
Bana
R
R
D000236:Adenoma / Q000473:pathology; D000311:Adrenal Glands / Q000473:pathology; D000328:Adult; D001283:Atrioventricular Node; D002828:Choristoma / Q000473:pathology; D004702:Endocrine Glands / Q000473:pathology*; D005260:Female; D006327:Heart Block / Q000150:complications; D006338:Heart Neoplasms / Q000150:complications* / Q000473:pathology; D006801:Humans; D006965:Hyperplasia / Q000473:pathology; D007150:Immunohistochemistry; D007515:Islets of Langerhans / Q000473:pathology; D007674:Kidney Diseases / Q000473:pathology; D010048:Ovarian Cysts / Q000150:complications / Q000473:pathology; D010179:Pancreas / Q000473:pathology; D013952:Thymus Hyperplasia / Q000473:pathology