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两例伴有嗜铬细胞瘤和胃肠道间质瘤(GIST)的冯雷克林霍增氏病病例。

Two von Recklinghausen's disease cases with pheochromocytomas and gastrointestinal stromal tumors (GIST) in combination.

作者信息

Teramoto Sakiko, Ota Tomonori, Maniwa Akimitsu, Matsui Takashi, Itaya Naoshi, Aoyagi Kazumi, Kusanagi Hiroshi, Narita Makoto

机构信息

Department of Urology, Kameda Medical Canter, Chiba, Japan.

出版信息

Int J Urol. 2007 Jan;14(1):73-4. doi: 10.1111/j.1442-2042.2006.01601.x.

DOI:10.1111/j.1442-2042.2006.01601.x
PMID:17199864
Abstract

We here document two cases of von Recklinghausen's disease with both pheochromocytomas and gastrointestinal stromal tumors (GIST). It is very rare that these three disorders are found to occur simultaneously, although the fact that preoperative detection of GIST is difficult except with large tumors may be important in this respect. In the present cases, GIST were not evident on preoperative computed tomography and magnetic resonance imaging, and were identified unexpectedly during surgery. Our findings indicate that investigation of the intraperitoneal space should be performed during adrenalectomy for pheochromocytomas with von Recklinghausen's disease.

摘要

我们在此记录了两例患有冯雷克林霍增氏病且同时伴有嗜铬细胞瘤和胃肠道间质瘤(GIST)的病例。这三种病症同时出现的情况极为罕见,不过,除了较大的肿瘤外,术前难以检测到GIST这一事实在这方面可能具有重要意义。在本病例中,术前计算机断层扫描和磁共振成像均未显示出GIST,而是在手术过程中意外发现的。我们的研究结果表明,对于患有冯雷克林霍增氏病的嗜铬细胞瘤患者,在进行肾上腺切除术时,应对腹腔间隙进行探查。

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