C. Marques, Cátia Ferreira, Ricardo Vaz Pereira, G. Guidi, João A. Pinto-de-Sousa
{"title":"克罗恩病中的隐匿性小肠腺癌--不仅仅是简单的狭窄","authors":"C. Marques, Cátia Ferreira, Ricardo Vaz Pereira, G. Guidi, João A. Pinto-de-Sousa","doi":"10.18203/2349-2902.isj20240582","DOIUrl":null,"url":null,"abstract":"Small-bowel adenocarcinoma (SBA) accounts for less than 5% of all gastrointestinal cancers. It is generally linked to genetic disorders and immune-mediated inflammatory conditions like Crohn's disease (CD). Despite advances in oncology, SBA has a poor prognosis and high relative risk in this subgroup of patients. Risk factors for the development of SBA in patients with CD include a stricture phenotype and a long- standing disease. This article aims to expose a case of association between CD and SBA and its management. A 66-year-old woman with long-standing terminally ileum-affected CD with multiple admissions due to sub-occlusive episodes and weight loss. CT and MRI revealed intestinal thickening in the small bowel, suggesting an inflammatory stenosis and entero-enteric fistulous tracts. An ileocolectomy was performed, and the patient's histopathological evaluation revealed a mucinous invasive adenocarcinoma of the terminal ileum. The patient was treated with adjuvant chemotherapy and has been under surveillance for two years, without malignant recurrence. Although it is a relatively rare neoplasm, CD patients have a significant risk of developing SBA, when compared to the general population. Diagnosis is challenging due to the occult nature of CD-associated SBA, and imaging and endoscopy alone make it difficult to detect the pathology. Treatment involves a high index of suspicion for the diagnosis and a balance between extended mesenteric resection and CD surgery's primary idea of bowel length preservation. Despite recent advances in oncology, the survival rate in CD-SBA patients remains low. Long-standing CD patients should have the terminal ileum monitored regularly and surgeons should be aware for occult SBA. Post-resection patient surveillance involves regular abdominal exams, serial surveillance, cross-sectional imaging, and monitoring for obstructive symptom recurrence. There is a lack of clear guidelines for primary prevention and surveillance of SBA, with a focus on inflammation management. Preoperative diagnosis techniques are scarce, and patients risk suboptimal treatment if incidental cancer is found. Strategies include right mesenteric-based surgical techniques and/or frozen section exam, always balancing cancer treatment and bowel preservation which is of high relevance in this subgroup of patients.","PeriodicalId":14372,"journal":{"name":"International Surgery Journal","volume":"28 3","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-02-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Occult small bowel adenocarcinoma in Crohn's disease-more than a simple stricture\",\"authors\":\"C. Marques, Cátia Ferreira, Ricardo Vaz Pereira, G. Guidi, João A. Pinto-de-Sousa\",\"doi\":\"10.18203/2349-2902.isj20240582\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Small-bowel adenocarcinoma (SBA) accounts for less than 5% of all gastrointestinal cancers. It is generally linked to genetic disorders and immune-mediated inflammatory conditions like Crohn's disease (CD). Despite advances in oncology, SBA has a poor prognosis and high relative risk in this subgroup of patients. Risk factors for the development of SBA in patients with CD include a stricture phenotype and a long- standing disease. This article aims to expose a case of association between CD and SBA and its management. A 66-year-old woman with long-standing terminally ileum-affected CD with multiple admissions due to sub-occlusive episodes and weight loss. CT and MRI revealed intestinal thickening in the small bowel, suggesting an inflammatory stenosis and entero-enteric fistulous tracts. An ileocolectomy was performed, and the patient's histopathological evaluation revealed a mucinous invasive adenocarcinoma of the terminal ileum. The patient was treated with adjuvant chemotherapy and has been under surveillance for two years, without malignant recurrence. Although it is a relatively rare neoplasm, CD patients have a significant risk of developing SBA, when compared to the general population. Diagnosis is challenging due to the occult nature of CD-associated SBA, and imaging and endoscopy alone make it difficult to detect the pathology. Treatment involves a high index of suspicion for the diagnosis and a balance between extended mesenteric resection and CD surgery's primary idea of bowel length preservation. Despite recent advances in oncology, the survival rate in CD-SBA patients remains low. Long-standing CD patients should have the terminal ileum monitored regularly and surgeons should be aware for occult SBA. Post-resection patient surveillance involves regular abdominal exams, serial surveillance, cross-sectional imaging, and monitoring for obstructive symptom recurrence. There is a lack of clear guidelines for primary prevention and surveillance of SBA, with a focus on inflammation management. Preoperative diagnosis techniques are scarce, and patients risk suboptimal treatment if incidental cancer is found. 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引用次数: 0
摘要
在所有胃肠道癌症中,小肠腺癌(SBA)的发病率不到 5%。它通常与遗传疾病和免疫介导的炎症(如克罗恩病(CD))有关。尽管肿瘤学取得了进展,但 SBA 的预后较差,在这一亚组患者中的相对风险较高。CD 患者发生 SBA 的风险因素包括狭窄表型和长期患病。本文旨在揭示一例 CD 与 SBA 相关的病例及其治疗方法。一位 66 岁的女性患者长期受回肠影响,多次因次闭塞发作和体重减轻而入院。CT 和 MRI 显示小肠增厚,提示有炎症性狭窄和肠道瘘道。对患者进行了回肠切除术,组织病理学评估显示其回肠末端患有黏液浸润性腺癌。患者接受了辅助化疗,两年来一直接受监测,没有恶性复发。虽然这是一种相对罕见的肿瘤,但与普通人群相比,CD 患者罹患 SBA 的风险很大。由于 CD 相关 SBA 具有隐匿性,因此诊断具有挑战性,仅凭影像学检查和内窥镜检查很难发现病变。治疗时需要高度怀疑诊断,并在肠系膜扩大切除术和 CD 手术保留肠管长度的主要理念之间取得平衡。尽管肿瘤学取得了最新进展,但 CD-SBA 患者的存活率仍然很低。病程较长的 CD 患者应定期监测回肠末端,外科医生应注意隐匿性 SBA。切除术后患者监测包括定期腹部检查、连续监测、横断面成像以及监测阻塞性症状复发。目前缺乏明确的 SBA 初级预防和监测指南,重点是炎症管理。术前诊断技术匮乏,如果偶然发现癌症,患者将面临治疗效果不佳的风险。策略包括基于右肠系膜的手术技术和/或冷冻切片检查,始终在癌症治疗和肠道保护之间保持平衡,这对该亚群患者具有重要意义。
Occult small bowel adenocarcinoma in Crohn's disease-more than a simple stricture
Small-bowel adenocarcinoma (SBA) accounts for less than 5% of all gastrointestinal cancers. It is generally linked to genetic disorders and immune-mediated inflammatory conditions like Crohn's disease (CD). Despite advances in oncology, SBA has a poor prognosis and high relative risk in this subgroup of patients. Risk factors for the development of SBA in patients with CD include a stricture phenotype and a long- standing disease. This article aims to expose a case of association between CD and SBA and its management. A 66-year-old woman with long-standing terminally ileum-affected CD with multiple admissions due to sub-occlusive episodes and weight loss. CT and MRI revealed intestinal thickening in the small bowel, suggesting an inflammatory stenosis and entero-enteric fistulous tracts. An ileocolectomy was performed, and the patient's histopathological evaluation revealed a mucinous invasive adenocarcinoma of the terminal ileum. The patient was treated with adjuvant chemotherapy and has been under surveillance for two years, without malignant recurrence. Although it is a relatively rare neoplasm, CD patients have a significant risk of developing SBA, when compared to the general population. Diagnosis is challenging due to the occult nature of CD-associated SBA, and imaging and endoscopy alone make it difficult to detect the pathology. Treatment involves a high index of suspicion for the diagnosis and a balance between extended mesenteric resection and CD surgery's primary idea of bowel length preservation. Despite recent advances in oncology, the survival rate in CD-SBA patients remains low. Long-standing CD patients should have the terminal ileum monitored regularly and surgeons should be aware for occult SBA. Post-resection patient surveillance involves regular abdominal exams, serial surveillance, cross-sectional imaging, and monitoring for obstructive symptom recurrence. There is a lack of clear guidelines for primary prevention and surveillance of SBA, with a focus on inflammation management. Preoperative diagnosis techniques are scarce, and patients risk suboptimal treatment if incidental cancer is found. Strategies include right mesenteric-based surgical techniques and/or frozen section exam, always balancing cancer treatment and bowel preservation which is of high relevance in this subgroup of patients.