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克罗恩病中的小肠腺癌:一种罕见但极具破坏性的并发症。

Small bowel adenocarcinoma in Crohn's disease: a rare but devastating complication.

机构信息

Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Main Campus, 9500 Euclid Ave, A31, Cleveland, OH, 44122, USA.

Department of Pathology, Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA.

出版信息

Tech Coloproctol. 2020 Oct;24(10):1055-1062. doi: 10.1007/s10151-020-02269-8. Epub 2020 Jun 28.

Abstract

BACKGROUND

Small bowel adenocarcinoma (SBA) remains a rare entity but occurs at increased frequency in the setting of chronic Crohn's disease (CD). Our aim was to study the presentation, diagnosis and prognosis of SBA in patients undergoing surgery for CD at a single institution.

METHODS

We reviewed the medical records of all patients with CD complicated by adenocarcinoma of the small bowel from 2000 to 2017. Descriptive statistics and Kaplan-Meier overall survival estimates were calculated.

RESULTS

In total, 22 patients (14 males) with CD (median duration of Crohn's diagnosis 32 years) were diagnosed with SBA and underwent surgical resection (8 isolated small bowel resections, 12 ileocolic resections, and 2 total proctocolectomies). The median patient age at the time of diagnosis was 54 years (range 22-82 years). A total of 17 patients (77%) underwent cross-sectional CT imaging within 3 months of surgery, a cancer diagnosis was suggested in only one patient. In one other patient, SBA was diagnosed preoperatively on endoscopic biopsy of the terminal ileum. The remaining patients were operated on for obstruction (n = 17), abscess or fistulizing disease (n = 2), and sigmoid cancer (n = 1). For these 20 (90%) patients not suspected to have SBA on preoperative assessment, 5 (25%) were diagnosed intraoperatively on frozen section and 15 (75%) were unexpectedly diagnosed postoperatively on final pathology. T staging was characterized by more advanced tumors (T4: 59%, T3: 27%, T2: 9%, and T1: 5%). Nine patients (41%) had nodal involvement and five patients (23%) had hepatic and/or peritoneal carcinomatosis. The 1-, 3-, and 5-year survival estimates for our cohort were 84%, 30%, and 10%, respectively. Median survival was 30.5 months with median follow-up of 23 months (range 6-84 months).

CONCLUSIONS

SBA in the setting of CD is most commonly found incidentally after surgical resection for benign indications. As such, any suspicious finding at the time of surgery in a patient with chronic CD should warrant careful investigation with frozen section and/or resection. Prognosis for CD complicated by SBA remains poor even in the modern era.

摘要

背景

小肠腺癌(SBA)仍然是一种罕见的疾病,但在慢性克罗恩病(CD)的情况下发生率增加。我们的目的是研究单一机构接受 CD 手术治疗的患者中 SBA 的表现、诊断和预后。

方法

我们回顾了 2000 年至 2017 年期间所有并发小肠腺癌的 CD 患者的病历。计算了描述性统计和 Kaplan-Meier 总生存估计值。

结果

共有 22 例(14 名男性)CD 患者(克罗恩氏病诊断的中位时间为 32 年)被诊断为 SBA 并接受了手术切除(8 例孤立的小肠切除术、12 例回结肠切除术和 2 例全结肠直肠切除术)。诊断时患者的中位年龄为 54 岁(范围 22-82 岁)。共有 17 例患者(77%)在手术后 3 个月内进行了横断面 CT 成像,只有 1 例患者提示癌症诊断。另一名患者在回肠末端的内镜活检中术前诊断为 SBA。其余患者因梗阻(n=17)、脓肿或瘘管病(n=2)和乙状结肠癌(n=1)而接受手术。对于这些术前未怀疑 SBA 的 20 名患者(90%),5 名(25%)在术中冷冻切片检查时确诊,15 名(75%)在术后最终病理检查时意外确诊。T 分期的特点是肿瘤更晚期(T4:59%,T3:27%,T2:9%,T1:5%)。9 名患者(41%)有淋巴结受累,5 名患者(23%)有肝和/或腹膜转移。我们队列的 1、3 和 5 年生存率分别为 84%、30%和 10%。中位生存时间为 30.5 个月,中位随访时间为 23 个月(范围 6-84 个月)。

结论

在 CD 的背景下,SBA 最常见于因良性指征接受手术切除后偶然发现。因此,在患有慢性 CD 的患者中,任何可疑的手术发现都应进行仔细调查,包括冷冻切片检查和/或切除。即使在现代,CD 并发 SBA 的预后仍然很差。

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