Strøm E H, Skjørten F, Stokke E S
Dept. of Pathology, University of Oslo, Ullevål Hospital, Norway.
Pathol Res Pract. 1993 Sep;189(8):960-4; discussion 965-7. doi: 10.1016/S0344-0338(11)81113-1.
Benign polycystic tumor of the atrioventricular nodal region is a lesion associated with cardiac conduction defects with atrioventricular block and sudden death. We present the clinical and light microscopical, immunohistochemical and ultrastructural findings of such a lesion in a young man with X-linked recessive Emery-Dreifuss muscular dystrophy who died suddenly. The tumor has not previously been described in this group of patients, who frequently suffers from cardiac electric instability and sudden death. Possible mechanisms by which the tumor may cause arrhythmia are discussed and the presence of neuroendocrine cells in the lesion is emphasized.
房室结区良性多囊性肿瘤是一种与心脏传导缺陷相关的病变，可导致房室传导阻滞和猝死。我们报告了一名患有X连锁隐性Emery-Dreifuss肌营养不良症的年轻男性突然死亡，其病变的临床、光镜、免疫组化和超微结构检查结果。此前尚未在这类经常出现心脏电不稳定和猝死的患者中描述过该肿瘤。本文讨论了该肿瘤可能导致心律失常的机制，并强调了病变中神经内分泌细胞的存在。
