Distal Duodenal Stricture Secondary to Mesenteric Fibromatosis (Intra-Abdominal Desmoid Tumor) of the Jejunum

IF 0.5 Q4 GASTROENTEROLOGY & HEPATOLOGY Case Reports in Gastroenterology Pub Date : 2024-04-20 DOI:10.1159/000538489
Sarah Huang, Jamil Shah, Eduardo Quintero, Philip Q. Xiao, Armand P. Asarian, Madhavi Reddy
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Abstract

Abstract Introduction Mesenteric fibromatosis (intra-abdominal desmoid tumor) is rare, with only a few cases reported in the literature. Clinical symptoms range from asymptomatic, nausea, early satiety, abdominal pain, and gastrointestinal bleeding. Although histologically benign, such a tumor may become locally invasive, and aggressive forms contribute to significant morbidity and mortality. Case Presentation We report the case of a 52-year-old West African male with a 1-year history of intermittent hematochezia and intermittent bloating. Colonoscopy revealed a 4-mm rectal polyp and internal hemorrhoids. Esophagogastroduodenoscopy revealed a severe duodenal stricture 4–5 cm distal to the ampulla. Further work-up with contrast-enhanced computed tomography of the abdomen and pelvis revealed a 5.0 × 3.7 × 4.3-cm mass within the mesentery, encasing the distal portion of the duodenum. Exploratory laparotomy was performed, and the mass was excised from the jejunum. Histopathology findings and immunohistochemical analysis revealed the diagnosis to be mesenteric fibromatosis (desmoid tumor), positive for nuclear β-catenin and SMA, and negative expression of STAT6, desmin, caldesmon, pan-cytokeratin, or c-KIT. The Ki67 index is <1%. Conclusion This case report highlights the diagnostic challenges of mesenteric fibromatosis due to its nonspecific clinical presentation. Recognizing uncommon presentations of mesenteric fibromatosis and risk factors aids in early diagnosis, management, and treatment. Importantly, this also aids in the prevention of complications such as intestinal obstruction, bowel ischemia, and fistula formation.
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十二指肠远端狭窄继发于肠系膜纤维瘤病(腹腔内蝶形瘤
摘要 引言 肠系膜纤维瘤病(腹腔内类固醇瘤)十分罕见,文献中仅有几例报道。临床症状包括无症状、恶心、早饱、腹痛和消化道出血。此类肿瘤虽然在组织学上是良性的,但可能会发生局部浸润,侵袭性肿瘤会导致严重的发病率和死亡率。病例介绍 我们报告了一名 52 岁西非男性的病例,他有 1 年的间歇性便血和间歇性腹胀病史。结肠镜检查发现了一个 4 毫米的直肠息肉和内痔。食管胃十二指肠镜检查发现,十二指肠严重狭窄,位于安瓿远端 4-5 厘米处。腹部和盆腔造影剂增强计算机断层扫描进一步检查发现,肠系膜内有一个 5.0 × 3.7 × 4.3 厘米的肿块,包裹着十二指肠的远端部分。患者接受了探查性开腹手术,从空肠中切除了肿块。组织病理学检查结果和免疫组化分析显示,诊断结果为肠系膜纤维瘤病(类肉瘤),核β-catenin和SMA阳性,STAT6、desmin、caldesmon、pan-cytokeratin或c-KIT阴性。Ki67 指数小于 1%。结论 本病例报告强调了肠系膜纤维瘤病因其非特异性临床表现而给诊断带来的挑战。认识肠系膜纤维瘤病的不常见表现和风险因素有助于早期诊断、管理和治疗。重要的是,这还有助于预防肠梗阻、肠缺血和瘘管形成等并发症。
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来源期刊
Case Reports in Gastroenterology
Case Reports in Gastroenterology Medicine-Gastroenterology
CiteScore
1.10
自引率
0.00%
发文量
99
审稿时长
7 weeks
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