Yeoman Andrew, Young Joanne, Arnold Julie, Jass Jeremy, Parry Susan
Department of Gastroenterology and Hepatology, Middlemore Hospital, Otahuhu, Auckland, New Zealand.
N Z Med J. 2007 Nov 30;120(1266):U2827.
The hyperplastic polyposis syndrome (HPS) has been described in a subset of patients with multiple or large hyperplastic polyps. HPS is associated with an increased risk of colorectal cancer (CRC). In this report, we review the presentation and management of a series of individuals with HPS.
From 2001, gastroenterologists, surgeons, and histopathologists at Middlemore Hospital were asked to report cases of HPS. Clinical records were retrospectively reviewed to confirm the number and size of polyps and the age at diagnosis, site of involvement in the colon, and nature of surgical procedures performed in cases with CRC.
HPS was identified in 24 patients: 14 females and 10 males. Though 46% of patients attending our gastroenterology department are non-Europeans, all HPS cases had European ancestry. A family history of CRC was identified in four patients (16.6%). All patients had small polyps (<5mm) however 15 (63%) had at least one polyp > or =10 mm, the largest being 45 mm. There were 21 CRCs in 14 patients with a mean age at diagnosis of 61 years. The tumour site was known in 19 CRC, and 16 of these (84%) occurred in the proximal colon. Synchronous cancers were identified in four patients and metachronous tumours in two patients. Twenty-two surgical procedures were performed in 17 patients. Three patients underwent prophylactic surgery due to polyp burden or dysplasia.
HPS is rarely encountered but is associated with a significant risk of CRC and is found in the European component of the New Zealand population. Identification of this syndrome has implications regarding management and surveillance for both the individual patient and their first-degree relatives.
增生性息肉病综合征(HPS)已在患有多个或大型增生性息肉的部分患者中被描述。HPS与结直肠癌(CRC)风险增加相关。在本报告中,我们回顾了一系列HPS患者的临床表现及治疗情况。
从2001年起,米德尔莫尔医院的胃肠病学家、外科医生和组织病理学家被要求报告HPS病例。对临床记录进行回顾性分析,以确认息肉的数量和大小、诊断时的年龄、结肠受累部位以及CRC患者所行手术的性质。
共识别出24例HPS患者,其中女性14例,男性10例。尽管在我们胃肠病科就诊的患者中有46%为非欧洲裔,但所有HPS病例均有欧洲血统。4例患者(16.6%)有CRC家族史。所有患者均有小息肉(<5mm),然而15例(63%)至少有一个息肉≥10mm,最大息肉为45mm。14例患者中有21例CRC,诊断时的平均年龄为61岁。19例CRC的肿瘤部位已知,其中16例(84%)发生在近端结肠。4例患者为同时性癌,2例患者为异时性肿瘤。17例患者共进行了22次手术。3例患者因息肉负荷或发育异常接受了预防性手术。
HPS很少见,但与CRC的显著风险相关,且在新西兰人群的欧洲裔中发现。识别该综合征对个体患者及其一级亲属的管理和监测具有重要意义。