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. 2021 Nov 28;46(11):1310–1314. [Article in Chinese] doi: 10.11817/j.issn.1672-7347.2021.200966

克罗恩病合并腹壁炎性肌纤维母细胞瘤1例

A case of Crohn's disease combined with inflammatory myofibroblastoma of abdominal wall

LIU Zhongcheng 1,2, CHEN Qian 2, LONG Meichun 1, HE Tian 1, GUO Qin 1,3,✉
Editor: 彭 敏宁
PMCID: PMC10929853  PMID: 34911868

Abstract

Inflammatory myofibroblastoma (IMT) is a rare solid tumor, and its etiology and pathogenesis are unclear. Crohn's disease is a non-specific intestinal inflammatory disease. The clinical manifestations, laboratory examinations, and imaging examinations of IMT are not specific, making diagnosis difficult. A case of Crohn's disease combined with IMT of abdominal wall was admitted to the Department of Gastroenterology at the Third Xiangya Hospital, Central South University, on Nov. 21, 2017. This patient was admitted to our hospital because of repeated right lower abdominal pain for 4 years. A 6 cm×5 cm mass was palpated in the right lower abdomen. After completing the transanal double-balloon enteroscopy and computed tomographic enterography for the small intestinal, the cause was still unidentified. The patient underwent surgery due to an abdominal wall mass with intestinal fistula on Sept. 12, 2018 and recovered well currently. According to histopathology and immunohistochemistry, he was diagnosed with Crohn's disease combined with IMT. Up to July 2020, the patients still took azathioprine regularly, without abdominal pain, abdominal distension, and other discomfort, and the quality of his life was good.

Keywords: inflammatory myofibroblastoma, Crohn's disease, abdominal wall

炎性肌纤维母细胞瘤(inflammatory myofibro-blastoma，IMT)是一种少见间的叶性肿瘤，主要由分化的肌纤维母细胞性梭形细胞组成，常伴大量炎细胞浸润，根据世界卫生组织(WHO)对软组织肿瘤的分类可划分为中间成纤维细胞或肌成纤维细胞肿瘤类[1]。其病因和发病机制尚不清楚。克罗恩病(Crohn's disease)是一种非特异性肠道炎症性疾病。国外仅报道了5例与克罗恩病相关的IMT病例，其中肝3例，胰腺1例，小脑1例[2-5]。国内尚无此类病例的报道。中南大学湘雅三医院(以下简称我院)收治了1例克罗恩病合并腹壁IMT的病例，现报告如下。

1. 病例资料

患者，男，46岁，2013年开始无明显诱因出现右下腹痛，就诊于当地医院，诊断为急性阑尾炎，予以手术治疗，术中发现大网膜与腹壁、乙状结肠系膜粘连，术后病理提示异物肉芽肿伴感染、坏死。2017年11月21日患者因过量饮酒后出现右下腹阵发性隐痛1个多月，收入我院消化内科。入院时专科体格检查：生命体征平稳，腹稍韧，右下腹可见一长约5 cm的手术疤痕，右下腹可触及一6 cm×5 cm大小包块，质地硬，界清，位置固定，触之压痛且皮温高，全身浅表淋巴结未触及肿大；肛查未见肛瘘及肛周脓肿。既往史无特殊。入院时血常规：白细胞8.35×1012/L，血红蛋白128 g/L，血小板437×109/L(↑)，中性粒细胞百分比81.5%(↑)，淋巴细胞百分比10.6%。肝功能：谷丙转氨酶88 U/L(↑)、白蛋白34.1 g/L。免疫全套：IgM 2.95 g/L(↑)，余正常。粪便钙卫蛋白132.57 μg/g(↑)；红细胞沉降率44 mm/h(↑)；C反应蛋白35.58 mg/L(↑)；超敏C反应蛋白>5 mg/L；神经元特异性烯醇化酶活性升高；肾功能、凝血功能、乙肝两对半、大小便常规、大便细菌及真菌培养、结核抗体、结核感染T细胞斑点试验(T-SPOT)、结缔组织全套、EB-DNA、巨细胞病毒(cytomegalovirus，CMV)-DNA、病毒全套、甲胎蛋白、癌胚抗原、糖类抗原125和糖类抗原19-9均无明显异常。体表包块彩超示腹壁皮下低回声结节，考虑炎性改变并瘘管形成。小肠CT造影(2017年11月15日)示：下腹可见节段性小肠管壁水肿增厚，正常肠管呈混杂密度包块形成，局部皮下脂肪间隙见少许索条影；肿块周围索条状渗出。经肛双气囊小肠镜(2017年11月17日)示：回肠中段及下段多个纵行溃疡，回肠中段疡周黏膜纠集并稍狭窄，至回肠中段后因肠腔扭曲粘连反复尝试外套管未能继续进镜，余所见小肠未见明显异常(图1)。病理检查结果为小肠黏膜有糜烂，部分绒毛变宽大，间质有大量淋巴浆细胞及少量嗜酸性粒细胞浸润。入院予以抗感染、护胃、护肠、护肝等治疗后，患者右下腹包块较前缩小，大小约 3 cm×2 cm，质地硬，界清，位置固定，局部皮温正常。根据腹部体格检查结果、炎症指标升高情况及彩超提示，考虑患者腹壁包块为炎性病变。结合多学科会诊意见，患者充分考虑后未行进一步治疗并出院。

图1.

图1

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经肛双气囊小肠镜下表现

Figure 1 Endoscopic manifestation under the transanal double-balloon enteroscopy

A: Ulcer: In the lower part of the ileum, longitudinal ulcer (arrow) can be seen; B: Narrow: In the middle part of the ileum, the ileum slightly narrowed; C: Ulcers and narrow: In the middle part of the ileum, longitudinal ulcer (arrows) can be seen, the outer tube was repeated tried because of the twisted adhesion of the intestinal cavity.

2018年9月10日患者因腹壁包块并发肠瘘形成再入我院，2018年9月12日行回盲部及回肠末端切除+回肠、横结肠侧吻合术。术中见右下腹肠管粘连成团，腹壁肌肉增厚水肿。术后病理检查结果示：(腹壁肿块及乙状结肠肿块)呈慢性炎症，有变性，肠壁浆膜面及(腹壁肿块)脓肿及异物肉芽肿形成，组织细胞增生，周边大量梭形细胞瘤样增生伴明显胶原化(图2)。免疫组织化学结果示：CKpan(-)，波形蛋白(Vimentin)(+)，S100(-)，SMA(+)，H-Caldeson(-)，Desmin(部分+)，ALK(-)，β-catenin(浆+)，CD34(-)，Bcl-2(-)，CD117(-)，Myogenin(-)，Ki-67(热点区30%)(图3)。结合临床病史及免疫组织化学结果倾向诊断为炎性包块伴肌纤维母细胞瘤样增生，不能排除克罗恩病。出院后予以口服美沙拉嗪缓释片2 g，每日3次。

图2.

图2

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病理检查结果

Figure 2 Results of pathological examination

A: Histopathological image of surgical resection (HE, ×40): Abdominal wall mass and sigmoid colon mass have chronic inflammation, degeneration. Intestinal wall serous surface and abdominal wall mass have abscess and foreign body granuloma formation, histiocytosis, a large number of shuttles around protocytoma-like hyperplasia with obvious collagenization, flattening of some small intestinal villi distortion and branches of crypts, pyloric gland metaplasia, and local ulcer formation. A small non-foreign body granuloma on the serosal surface of the small intestinal wall and individual lymph nodes granuloma formation can be seen inside, no necrosis is seen. B: Granuloma outside the intestinal wall (HE, ×200). C: Granuloma of lymph node (HE, ×40).

图3.

图3

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免疫组织化学检测结果(HE，×100)

Figure 3 Results of immunohistochemistry (HE, ×100) A: ALK; B: SMA; C: Viementin.

2019年11月19日患者再发腹痛，就诊于我院消化内科。复查小肠CT造影，结果示：回盲部及回肠末端切除+回肠及横结肠侧侧吻合术后改变；右中下腹部部分小肠管壁增厚，伴周围多发淋巴结，符合炎性肠病的特征。结肠镜示：回肠末端及吻合口多发溃疡。病理科复检2018年9月12日手术标本，结果示：送检小肠呈慢性肠炎改变，部分小肠绒毛变扁平，可见隐窝扭曲及分支，幽门腺化生，局部溃疡形成，小灶肠壁外浆膜面见一处非异物肉芽肿(图2B)，个别淋巴结内可见肉芽肿形成，未见坏死，结合临床病史综合分析倾向合并有克罗恩病。免疫组织化学结果示：EB病毒编码的小RNA(-)，CMV(-)。结合临床病史及病理结果诊断：1)克罗恩病(回肠型、狭窄型、中度活动期)；2)IMT。

根据《炎症性肠病诊断与治疗的共识意见(2018年，北京)》[6]：泼尼松片只能用于诱导缓解，不能维持治疗；而硫唑嘌呤起效慢，用药8~12周才能起效，用药12周后才能达到最大疗效，一般用于维持治疗。因此在泼尼松片使用早期就联合使用硫唑嘌呤，能更好地保证疗效。本例患者处于疾病活动期，出院后予以泼尼松片与硫唑嘌呤联用，具体方案为：泼尼松片45 mg，每天1次，持续1个月，1个月后每周减少5 mg，共服用3个月后停用；硫唑嘌呤100 mg，每天1次维持治疗。

随访至2020年7月，患者仍规律使用硫唑嘌呤100 mg，每天1次。无腹痛、腹胀等不适，生活质量可。

2. 讨 论

IMT又称炎性假瘤、浆细胞肉芽肿和纤维黄瘤等，是罕见的实体瘤，具有复发及转移趋势[7]，发病年龄广泛，好发于20岁以下人群[8]，可发生于全身各个器官，以肺部发生率最高；消化道中最常发生于胃和回盲部，其次为小肠、结直肠。IMT主要有黏液样/血管型、致密梭形细胞型、少细胞纤维型3种组织学亚型[9]。IMT发病机制不明确，目前认为主要与感染、自身免疫及基因异常等因素有关，而EB病毒和人类疱疹病毒8型的感染[10]、ALK基因的重排[11]、DNA非整倍性及克隆染色体异常[12]也可引起IMT的发生。

IMT发病率低，临床表现、实验室检查及影像学检查均无明显特异性。胃肠道IMT症状可能与发病部位相关，如胃、十二指肠IMT以上腹不适多见；空回肠、结肠以腹痛、腹部包块多见；直肠主要表现为便血[13]。对其最终确诊要依靠镜下病理及免疫组织化学结果[8]。IMT属于局灶性增生性病变，其病理改变主要表现为大量慢性炎性细胞浸润伴纤维组织增生。在IMT患者中，SMA、MSA、Vimentin和calponin阳性率和阳性表达水平均很高，而H-Caldesmon、S100、CD34、CD117及Myogenin均呈阴性表达[14]。本例符合IMT免疫组织化学特点，其中Desmin部分阳性表达而ALK阴性表达。有研究[14]报道约40%的病例出现Desmin低表达，说明Desmin是IMT的一个表达率低并且不敏感的标志物；Coffin等[15]检测IMT组织中ALK-1的表达，结果发现56%的患者肿瘤组织ALK-1阳性。ALK阳性患者使用TKI抑制剂克唑替尼可获得部分缓解，而ALK阴性患者无好转；ROS1融合患者也可获得明显疗效[16]。对于IMT患者来说，若出现复发，再次手术仍是首选。

胃肠道IMT形态多样，在一定程度上与多种胃肠道肿瘤的组织形态相似，应与以下肿瘤相鉴别：1)胃肠道间质瘤，是起源于胃肠道间叶组织的肿瘤，其特征是酪氨酸激酶KIT蛋白的高度表达；病理检查很少出现淋巴细胞、浆细胞浸润，且免疫组织化学结果示CD117及DOG1阳性[17]。2)炎性纤维性息肉，多发生于胃，病理表现主要有嗜酸性粒细胞、纤维母细胞样梭形细胞和小血管，其中嗜酸性粒细胞是其特异性病理表现，免疫组织化学标志为波形蛋白和CD34阳性，SMA局灶阳性，ALK阴性[18-19]。3)结节性筋膜炎，是最常见的软组织假性肉瘤，好发于20~50岁的中青年人，免疫组织化学结果多有β-catenin和SMA阳性，但无Desmin、PCK或S100蛋白表达[20]。4)孤立性纤维性肿瘤，是一种树突状间叶细胞肿瘤，其镜下密集区细胞丰富，细胞异型性大，核分裂象多见，免疫组织化学结果示CD34、CD99、Bcl-2及SATA6表达[21-22]。

综上所述，克罗恩病合并IMT极为少见且尚无标准化的治疗方案。传统治疗以手术为主，当患者一般情况允许时，可适当扩大手术切除范围。IMT的预后多数良好，但其具有浸润生长倾向，且有复发或转移的可能，有必要进行长期随访。

利益冲突声明

作者声称无任何利益冲突。

原文网址

http://xbyxb.csu.edu.cn/xbwk/fileup/PDF/2021111310.pdf

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