Waisberg Jaques, Joppert-Netto George, Vasconcellos Cidia, Sartini Gustavo Henrique, Miranda Lucimar Sonja Villela de, Franco Maria Isete Fares
Hospital do Servidor Público Estadual (IAMSPE), São Paulo, Brazil.
Arq Gastroenterol. 2013 Jan-Mar;50(1):3-9. doi: 10.1590/s0004-28032013000100002.
Duodenal carcinoids are extremely rare, and their characteristics and biological behavior have not been fully elucidated.
To analyze the clinicopathological characteristics of patients with resected duodenal carcinoids.
Twenty patients (12 females and 8 males) were investigated. Their average age was 66.4 ± 5.8 years old (43 to 88 years old). The data corresponding to the clinical picture, diagnosis, treatment, and prognosis of patients with duodenal carcinoid tumors subjected to resection over a period of 18 years (1993-2011) were analyzed.
The most common symptoms were dyspepsia (50%) and epigastric pain (45%) followed by weight loss (10%) and vomiting (5%). Carcinoid syndrome was not observed in any patient. The lesion was located on the first part of the duodenum in 15 (75%) patients, the second part in 4 (20%) patients, and the third part in 1 (5%) patient. The diagnosis of a carcinoid tumor was established through an endoscopic excision biopsy in 19 (95%) patients and an histopathological examination of the surgical specimen in 1 (5%) patient. The average tumor size was 1.1 cm ± 0.4 cm (0.3 cm to 6.0 cm). Nineteen (95%) patients were initially treated by endoscopic resection of the duodenal lesion. One patient (5%), whose tumor was on the third part of the duodenum underwent a duodenectomy of the third and fourth duodenal parts and duodenojejunal anastomosis. The duodenal carcinoid resection margin was involved in four (20%) patients. Four (20%) patients were subjected to a partial gastrectomy to fully remove the lesion. The tumor was restricted to the submucosal layer in 16 (80%) cases, and it penetrated into the muscular layer in 4 (20%) cases. All patients exhibited positive chromogranin A, neuron-specific enolase, and/or synaptophysin immunostaining. The average duration of the follow-up period was 39.6 months (3 to 96 months). Twelve (60%) of the 20 cases in this series are alive without any evidence of active disease. Only one (5%) patient died due to liver metastases of the duodenal carcinoid.
Duodenal carcinoids are rare and indolent tumors usually associated with a benign progression. Duodenoscopy, computerized tomography, and endoscopic ultrasound should be performed to evaluate the tumor size, the level of wall invasion, and the presence of regional or distant lymphatic metastases. Endoscopic removal of tumors smaller than 1.0 cm without periampullary localization or evidence of muscular propria layer invasion assessed by histology and/or endoscopic ultrasound is recommended. The endoscopic resection with a carcinoid tumor size between 1.0 cm and 2.0 cm can be incomplete and require new endoscopic resection or even surgical removal. Duodenal carcinoid larger than 2.0 cm require full-thickness resection and concomitant lymphadenectomy.
十二指肠类癌极为罕见，其特征及生物学行为尚未完全阐明。
分析行十二指肠类癌切除术患者的临床病理特征。
对20例患者（12例女性，8例男性）进行研究。他们的平均年龄为66.4±5.8岁（43至88岁）。分析了1993年至2011年18年间接受十二指肠类癌肿瘤切除术患者的临床表现、诊断、治疗及预后相关数据。
最常见症状为消化不良（50%）和上腹部疼痛（45%），其次是体重减轻（10%）和呕吐（5%）。所有患者均未观察到类癌综合征。病变位于十二指肠第一部的有15例（75%），第二部的有4例（20%），第三部的有1例（5%）。19例（95%）患者通过内镜切除活检确诊为类癌肿瘤，1例（5%）患者通过手术标本的组织病理学检查确诊。肿瘤平均大小为1.1 cm±0.4 cm（0.3 cm至6.0 cm）。19例（95%）患者最初接受十二指肠病变的内镜切除术。1例（5%）肿瘤位于十二指肠第三部的患者接受了十二指肠第三、四部切除术及十二指肠空肠吻合术。4例（20%）患者的十二指肠类癌切除边缘受累。4例（20%）患者接受了部分胃切除术以完全切除病变。16例（80%）病例肿瘤局限于黏膜下层，4例（20%）病例肿瘤侵犯至肌层。所有患者嗜铬粒蛋白A、神经元特异性烯醇化酶和/或突触素免疫染色均为阳性。平均随访期为39.6个月（3至96个月）。本系列20例病例中有12例（60%）存活，无任何活动性疾病证据。仅1例（5%）患者因十二指肠类癌肝转移死亡。
十二指肠类癌是罕见的惰性肿瘤，通常呈良性进展。应进行十二指肠镜检查、计算机断层扫描和内镜超声检查以评估肿瘤大小、壁侵犯程度及区域或远处淋巴结转移情况。对于小于1.0 cm且无壶腹周围定位或组织学和/或内镜超声评估无固有肌层侵犯证据的肿瘤，建议内镜切除。类癌肿瘤大小在1.0 cm至2.0 cm之间的内镜切除可能不彻底，需要再次内镜切除甚至手术切除。大于2.0 cm的十二指肠类癌需要全层切除并同时进行淋巴结清扫。
