Tsukagoshi H, Iwasaki Y, Toyoda M, Sato T, Takagi H, Mori M, Sasaki A, Kumakura H, Ishijima H, Aoki J
First Department of Internal Medicine, Gunma University School of Medicine, Maebashi.
Intern Med. 1998 Oct;37(10):847-52. doi: 10.2169/internalmedicine.37.847.
An unusual autopsy case of systemic hemangiomatosis associated with honeycomb-like liver and splenic sarcomatoid changes is presented. The patient suffered from high-output ventricular heart failure secondary to hepatic arteriovenous shunting with hepatic failure, ending fatally due to disseminated intravascular coagulation and pulmonary bleeding. Postmortem examination revealed the characteristic distribution of vasoformative tumors presenting in the hematopoietic system including the liver, bone marrow, and spleen, with the liver appearing characteristically honeycomb-like. While these vasoformative tumors were mostly benign-appearing, partial shifts toward sarcomatoid change were observed in the splenic lesions.
本文报告了一例罕见的系统性血管瘤病尸检病例,该病例伴有蜂窝状肝脏和脾脏肉瘤样改变。患者因肝动静脉分流继发高输出量心室心力衰竭,并伴有肝功能衰竭,最终因弥散性血管内凝血和肺出血死亡。尸检发现血管形成性肿瘤在包括肝脏、骨髓和脾脏在内的造血系统中呈现出特征性分布,肝脏表现为典型的蜂窝状。虽然这些血管形成性肿瘤大多外观良性,但在脾脏病变中观察到部分向肉瘤样改变的转变。