摘要
十二指肠错构瘤（BGH）是十二指肠良性肿瘤中比较少见的类型，因其临床表现不典型、缺乏特异性，导致术前诊断困难，往往易被漏诊或误诊，主要依靠术后病理确诊。巨大BGH的病例临床更是罕见。本文报告1例巨大BGH致慢性贫血的诊治过程，并结合既往文献对本病特点进行复习，以期为临床工作提供经验借鉴。
患者，女性，49岁；因腹痛伴恶心、呕吐就诊西安交通大学附属咸阳市中心医院肝胆外科。患者既往有慢性贫血病史，月经规律，偶有柏油样便。入院上腹部B超检查见胰头内侧十二指肠部包块；上腹部CT检查见十二指肠降部及水平部肠壁弥漫性增厚，结构不清；上腹部MRI+MRCP检查见十二指肠降部及水平部与邻近空肠分界不清，肠管明显扩张扭曲，肠壁分界不清，肠壁水肿明显，可见同心圆样改变。胃镜检查考虑十二指肠占位，并镜下活检提示符合胃黏膜异位。上消化道造影提示十二指肠降段与水平段移行区近圆形充盈缺损，考虑良性占位可能性大。最终考虑十二指肠占位性病变并引起梗阻，剖腹探查术后行胰十二指肠切除术，切除标本送病理学检查，最终诊断为BGH，免疫组化MUC5AC（+），术后恢复顺利出院。术后监测血常规，红细胞及血红蛋白逐渐恢复正常，随访12个月，患者一般状况良好，未诉不适，复查各项指标正常，继续随访。
十二指肠错构瘤，也称为十二指肠腺瘤或Brunner腺错构瘤（Brunner's gland hamartoma，BGH），是临床上一种罕见的十二指肠良性肿瘤，在十二指肠良性肿瘤中发病率约为5.0%~10.0%，常规内镜镜检率为0.01%~0.07
患者 女，49岁。腹痛伴恶心、呕吐1 d就诊，患者自诉1 d前进食后出现上腹部疼痛，呈持续性，并进行性加重，伴恶心、呕吐，呕吐物为胃内容物，伴发热，体温最高38.0 ℃。体格检查：贫血貌，右上腹压痛阳性，余未见明显异常。实验室检查：白细胞14.77×1
图1 入院影像学资料 A：CT示十二指肠降部及水平部肠壁弥漫性增厚，结构不清；B：轴位MRI示十二指肠降部及水平部与邻近空场分界不清，肠管明显扩张扭曲，肠壁分界不清，肠壁水肿明显，可见同心圆样改变；C：冠状位MRI示低位胆道梗阻并胆囊积液，扩张胆总管下段受牵拉向左移位
Figure 1 Imaging data on admission A: CT showing diffuse thickening of the intestinal wall in the descending and horizontal parts of the duodenum, with unclear structure; B: Axial MRI imaging showing that indistinct sight of the descending and horizontal parts of the duodenum and the adjacent jejunum, with obviously distended and twisted intestinal tube, indistinct intestinal wall, obvious intestinal wall edema and presence of concentric circles change; C: Coronal CT imaging showing lower biliary obstruction and gallbladder collections, with a left dislocation of the lower common bile duct due to traction
图2 胃镜资料 A：十二指肠降部；B：十二指肠水平部；C：胃镜活检胃型黏膜（HE ×100）
Figure 2 Gastroscopic data A: View of the descending part of the duodenum; B: View of the horizontal part of the duodenum; C: Biopsy showing gastric mucosa (HE ×100)
术前考虑十二指肠占位并引起梗阻，经患者及家属同意，于2021年2月3日在全麻下行剖腹探查术，术中探查腹腔未见明显异常，常规游离十二指肠，切开十二指肠降部，见肿物位于幽门至十二指肠乳头间，大小约7 cm×6 cm（
图3 术后资料 A：大体标本；B：组织病理示增生的导管及腺体形成分叶状结构（HE×100）；C：术后复查CT
Figure 3 Postoperative data A: Gross specimen; B: Histopathological examination showing the lobulated structures of the hyperplastic ducts and glands (HE×100); C: Postoperative CT review
错构瘤形成的主要原因是机体各种组织成分比例、分布、分化异于正常组织。BGH主要由Brunner腺体、平滑肌、纤维结缔组织及血管构成。其病理表现为十二指肠腺增生伴小叶结构消失，间质内可见淋巴组织、脂肪组织及平滑肌束穿插，有时可见被覆柱状或纤毛柱状上皮的导管，可呈囊性或有黏液潴
大多数BGH是孤立的带蒂息肉，少数不带蒂，直径大多介于1~2 cm，很少超过5 cm。大多位于十二指肠近端，距幽门越远，发病率越低，十二指肠球部占57.0%；十二指肠降部占27.0%；十二指肠水平部占7.0
目前，BGH的诊断常依靠内镜及影像学检查。内镜表现为黏膜下隆起性病变，表面光滑，表面可伴有散在出血点。值得注意的是，内镜活检大多为阴性，这是因为病灶通常被覆正常十二指肠黏膜，活检深度难以到达黏膜下层的病灶。刘健
对于BGH较小、无症状的患者，可以保守治疗，如果病灶大、出现症状时，建议切除，可以考虑内镜切除及外科手术。秦榕
本文报道了1例巨大BGH致慢性贫血病例并回顾性分析相关文献，BGH患者缺乏特异性临床表现，常见的症状有腹痛、腹胀、恶心、呕吐、黑便等，影像学检查常无阳性发现，并且易误诊为胆道系统疾病，内镜、EUS以及EUS下穿刺活检有助于诊断，但难度较大。当临床上出现消化道梗阻或慢性出血时，胃镜检查发现十二指肠肿物，肿物边界清楚、表面光滑，黏膜呈暗红色、水肿时，应考虑BGH可能。BGH恶性者罕见，但当病变增大并且有形态学改变时，应警惕恶变可能。病灶较大者可考虑外科手术切除。本例患者在手术探查过程中发现瘤体大、且无法排除十二指肠恶性肿瘤可能，故决定行胰十二指肠切除术，手术创伤虽大，但取得较好效果，长期随访，患者恢复良好，未见复发，长期的慢性贫血得到改善，对临床有一定的参考价值。
利益冲突
所有作者均声明不存在利益冲突。
参考文献
Bakir MA, AlYousef MY, Alsohaibani FI, et al. Brunner's glands hamartoma with pylorus obstruction: a case report and review of literature[J]. J Surg Case Rep, 2020, 2020(8):rjaa191. doi: 10.1093/jscr/rjaa191. [百度学术]
Zhu MH, Li HY, Wu YY, et al. Brunner's gland hamartoma of the duodenum: a literature review[J]. Adv Ther, 2021, 38(6):2779-2794. doi: 10.1007/s12325-021-01750-6. [百度学术]
Bush A, Francis J, Laczek J. Brunner's gland hamartomas: two cases of this (sometimes) distinctive lesion[J]. Mil Med, 2021, 186(9/10):e1040-1042. doi: 10.1093/milmed/usaa489. [百度学术]
Hussain I, Tan M, Veeraraghavan MA, et al. Endosonographic appearances of a rare duodenal tumour, brunner's gland hamartoma[J]. Case Rep Gastroenterol, 2019, 13(2):310-320. doi: 10.1159/000501674. [百度学术]
Rana R, Sapkota R, Kc B, et al. Giant Brunner's gland adenoma presenting as upper gastrointestinal bleeding in 76 years old male: a case report[J]. JNMA J Nepal Med Assoc, 2019, 57(215):50-52. doi: 10.31729/jnma.4013. [百度学术]
Olivares LO, Gómez PC, Tamayo JS, et al. Endoscopic approach to Brunner's gland hamartoma: A c-ase report[J]. Rev Gastroenterol Mex (Engl Ed), 2022. doi: 10.1016/j.rgmxen.2022.05.019. [Online ahead of print] [百度学术]
Nath S, Panda G, Karna R, et al. Brunner's gland hamartoma presenting as large duodenal polyp[J]. Indian J Pathol Microbiol, 2020, 63(2):334-335. doi: 10.4103/IJPM.IJPM_650_18. [百度学术]
Coskun A, Erkan N. Giant Brunner's gland adenoma as an unusual cause of anaemia: report of a case[J]. Radiol Oncol, 2011, 45(2):129-131. doi: 10.2478/v10019-010-0053-5. [百度学术]
Yi LZ, Cheng ZY, Qiu HR, et al. A giant Brunner's gland hamartoma being treated as a pedunculated polyp: a case report[J]. BMC Gastroenterol, 2019, 19(1):151. doi: 10.1186/s12876-019-1074-1. [百度学术]
Zhou SR, Ullah S, Liu YY, et al. Brunner's gland adenoma: lessons learned for 48 cases[J]. Dig Liver Dis, 2021, 53(1):134-136. doi: 10.1016/j.dld.2020.10.028. [百度学术]
Sakurai T, Sakashita H, Honjo G, et al. Gastric foveolar metaplasia with dysplastic changes in Brunner gland hyperplasia: possible precursor lesions for Brunner gland adenocarcinoma[J]. Am J Surg Pathol, 2005, 29(11):1442-1448. doi: 10.1097/01.pas.0000180449.15827.88. [百度学术]
Maruo M, Tahara T, Inoue F, et al. A giant Brunner gland hamartoma successfully treated by endoscopic excision followed by transanal retrieval: a case report[J]. Medicine, 2021, 100(14):e25048. doi: 10.1097/MD.0000000000025048. [百度学术]
Dhali A, Ray S, Mandal TS, et al. Surgical outcome of brunner's gland hamartoma: a single-centre experience[J]. Surg J (N Y), 2022, 8(1):e14-18. doi: 10.1055/s-0041-1741510. [百度学术]
Naito S, Fukuzawa M, Nakamura S, et al. Giant Brunner's gland hamartoma diagnosed via endoscopic mucosal resection: a case report[J]. DEN open, 2021, 2(1):e65. doi: 10.1002/deo2.65. [百度学术]
Rath MM, Mohapatra D, Mohanty SK, et al. Brunner's gland hamartoma with dysplasia, presenting as multiple duodenal polyps: an unexplored entity with literature review[J]. Indian J Pathol Microbiol, 2019, 62(2):290-292. doi: 10.4103/IJPM.IJPM_69_18. [百度学术]
Kitagawa Y, Osumi H, Kawachi H, et al. Giant duodenal Brunner's gland hamartoma successfully treated via endoscopic mucosal resection[J]. Arab J Gastroenterol, 2018, 19(3):125-129. doi: 10.1016/j.ajg.2018.08.004. [百度学术]
Gupta A, Rajput D, Kumar U, et al. Brunner's gland hamartoma presenting as gastric outlet obstruction: unusual presentation and review of literature[J]. Trop Doct, 2020, 50(1):68-70. doi: 10.1177/0049475519870980. [百度学术]
Khosla M, Khosravi F, Cashman J, et al. Giant Brunner's gland hamartoma as a cause of iron deficiency Anemia[J]. ACG Case Rep J, 2017, 4:e37. doi: 10.14309/crj.2017.37. [百度学术]
Bojanapu S, Mangla V, Mehrotra S, et al. Brunner's gland hyperplasia: an unusual duodenal submucosal lesion seen in four patients[J]. J Surg Case Rep, 2018, 2018(11):rjy305. doi: 10.1093/jscr/rjy305. [百度学术]
Sudo G, Kobayashi T, Nakase H. Brunner's gland hamartoma arising from the pyloric ring[J]. Clin Gastroenterol Hepatol, 2019, 17(13):e155. doi: 10.1016/j.cgh.2018.08.076. [百度学术]
席芸, 吴伟, 程国平, 等. 十二指肠腺增生性病变18例临床病理分析[J]. 临床与实验病理学杂志, 2019, 35(7):782-786. doi: 10.13315/j.cnki.cjcep.2019.07.006. [百度学术]
Xi Y, Wu W, Cheng GP, et al. Clinicopathological analysis of 18 cases of Brunner's gland proliferating lesions[J]. Chinese Journal of Clinical and Experimental Pathology, 2019, 35(7):782-786. doi: 10.13315/j.cnki.cjcep.2019.07.006. [百度学术]
Peloso A, Viganò J, Vanoli A, et al. Saving from unnecessary pancreaticoduodenectomy. Brunner's gland hamartoma: case report on a rare duodenal lesion and exhaustive literature review[J]. Ann Med Surg, 2017, 17:43-49. doi: 10.1016/j.amsu.2017.03.034. [百度学术]
Dhali A, Ray S, Ghosh R, et al. Massive upper gastrointestinal hemorrhage in Brunner's gland hamartoma of duodenum[J]. Cureus, 2021, 13(6):e15875. doi: 10.7759/cureus.15875. [百度学术]
Aldossary MY, Alzahir AA, Almulla LA, et al. Giant Brunner's gland hamartoma causing retrograde jejuno-duodenal intussusception: a case report[J]. Ann Med Surg (Lond), 2019, 38:37-41. doi: 10.1016/j.amsu.2018.12.007. [百度学术]
Miguel PS, Mathieu P, Mariana FF, et al. Giant Brunner's gland hamartoma of the duodenal bulb removed by endoscopic submucosal dissection[J]. Endoscopy, 2022. doi: 10.1055/a-1893-5782. Online ahead of print. [百度学术]
刘健, 赵卓, 吴双, 等. 十二指肠Brunner腺错构瘤二例[J]. 中华普通外科杂志, 2017, 32(2):111. doi: 10.3760/cma.j.issn.1007-631X.2017.02.005. [百度学术]
Liu J, Zhao Z, Wu S, et al. Two cases of duodenal Brunner's gland hamartoma[J]. Zhong Hua Pu Tong Wai Ke Za Zhi, 2017, 32(2):111. doi: 10.3760/cma.j.issn.1007-631X.2017.02.005. [百度学术]
Satoh T, Matsubayashi H, Takizawa K, et al. Giant Brunner's gland hyperplasia of the duodenum diagnosed by endoscopic ultrasonography-guided fine needle biopsy and treated by laparoscopic endoscopic cooperative surgery[J]. Intern Med, 2019, 58(14):2009-2013. doi: 10.2169/internalmedicine.2477-18. [百度学术]
Lee KJ, Park B, Kim HM. Endoscopic ultrasonography findings for Brunner's gland hamartoma in the duodenum[J]. Clin Endosc, 2022, 55(2):305-309. doi: 10.5946/ce.2020.259. [百度学术]
Cotton J, Bavi P, Ghorab Z, et al. Pre-operative diagnosis of a Brunner's gland hamartoma by endoscopic ultrasound-guided fine needle aspiration[J]. Cytopathology, 2021, 32(5):674-676. doi: 10.1111/cyt.12964. [百度学术]
仝开军, 舒荣宝, 程刘兵, 等. 巨大Brunner错构瘤一例[J]. 放射学实践, 2018, 33(5):545-546. doi: 10.13609/j.cnki.1000-0313.2018.05.024. [百度学术]
Tong KJ, Shu RB, Cheng LB, et al. A case of giant Brunner hamartoma[J]. Radiologic Practice, 2018, 33(5):545-546. doi: 10.13609/j.cnki.1000-0313.2018.05.024. [百度学术]
Destek S, Gul VO. Brunner's gland hyperplasias and hamartomas in association with Helicobacter pylori[J]. Can J Gastroenterol Hepatol, 2019, 2019:6340565. doi: 10.1155/2019/6340565. [百度学术]
Mhanna J, Francis FF, Sabatto BZ, et al. An unusual gastric polyp: brunneroma[J]. ACG Case Rep J, 2021, 8(11):e00681. doi: 10.14309/crj.0000000000000681. [百度学术]
秦榕, 孙桂芳, 高建鹏, 等. 胃镜下摘除十二指肠Brunner腺错构瘤 (附3例报告)[J]. 中国内镜杂志, 2019, 25(2):69-72. doi:10.3969/j.issn.1007-1989.2019.02.014. [百度学术]
Qin R, Sun GF, Gao JP, et al. Removal of duodenal Brunner's gland hamartoma under gastroscope(3 cases)[J]. China Journal of Endoscopy, 2019, 25(2):69-72. doi:10.3969/j.issn.1007-1989.2019.02.014. [百度学术]