内脏位置不明确伴多脾畸形并合并肾腺癌。

Situs ambiguous with polysplenia complicated by renal adenocarcinoma.

作者信息

Hirohata S, Isobe H, Mitamura T, Yoshinoya S, Miyamoto T, Motoki T, Koide T

出版信息

Arch Intern Med. 1985 Jun;145(6):1134-7.

Abstract

Two men developed renal adenocarcinoma in association with situs ambiguous off with polysplenia (SAP) (also known as the polysplenia syndrome). Features of their diseases included (1) no normal spleen--just splenuli, (2) interruption of the inferior vena cava with azygos or hemiazygos continuation, (3) bilateral hyparterial bronchi, (4) cardiac malformations, (5) renal adenocarcinomas originating from the kidneys, ipsilateral to the anomalous spleens. The association of renal adenocarcinomas and SAP has not been previously reported, to our knowledge. We suggest that renal adenocarcinoma and SAP may share a common pathogenetic link.

摘要

两名男性患肾腺癌，并伴有多脾综合征（也称为多脾症）的内脏转位异常。他们疾病的特征包括：（1）无正常脾脏，仅有多个脾小结；（2）下腔静脉中断，奇静脉或半奇静脉延续；（3）双侧动脉下支气管；（4）心脏畸形；（5）肾腺癌起源于与异常脾脏同侧的肾脏。据我们所知，肾腺癌与多脾综合征的关联此前尚未见报道。我们认为肾腺癌和多脾综合征可能存在共同的发病机制联系。