PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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%.
UNASSIGNED: 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.
UNASSIGNED: 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%.
UNASSIGNED: 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.
摘要：
■肠系膜纤维瘤病（腹内硬纤维瘤）很少见，文献中只有少数病例报道。临床症状范围从无症状，恶心,早期饱腹感,腹痛,和消化道出血.尽管组织学上是良性的，这样的肿瘤可能会变得局部侵入性，和侵略性形式有助于显著的发病率和死亡率。
■我们报告一例52岁的西非男性，有1年的间歇性便血和间歇性腹胀病史。结肠镜检查显示4毫米直肠息肉和内痔。食管胃十二指肠镜检查显示，壶腹远端4-5厘米处有严重的十二指肠狭窄。腹部和骨盆的对比增强计算机断层扫描进一步检查显示肠系膜内有5.0×3.7×4.3厘米的肿块，包围十二指肠的远端部分。进行了剖腹探查术，从空肠切除肿块。组织病理学结果和免疫组化分析显示诊断为肠系膜纤维瘤病（硬纤维瘤），核β-连环蛋白和SMA阳性，STAT6、desmin、Caldesmon,泛细胞角蛋白，或者c-kit.Ki67指数<1%。
■本病例报告强调了肠系膜纤维瘤病由于其非特异性临床表现的诊断挑战。认识到肠系膜纤维瘤病的罕见表现和危险因素有助于早期诊断，管理,和治疗。重要的是,这也有助于预防并发症，如肠梗阻，肠缺血,和瘘管形成。
■我们报告一例52岁的西非男性，有1年的间歇性便血和间歇性腹胀病史。结肠镜检查显示4毫米直肠息肉和内痔。食管胃十二指肠镜检查显示，壶腹远端4-5厘米处有严重的十二指肠狭窄。腹部和骨盆的对比增强计算机断层扫描进一步检查显示肠系膜内有5.0×3.7×4.3厘米的肿块，包围十二指肠的远端部分。进行了剖腹探查术，从空肠切除肿块。组织病理学结果和免疫组化分析显示诊断为肠系膜纤维瘤病（硬纤维瘤），核β-连环蛋白和SMA阳性，STAT6、desmin、Caldesmon,泛细胞角蛋白，或者c-kit.Ki67指数<1%。
■本病例报告强调了肠系膜纤维瘤病由于其非特异性临床表现的诊断挑战。认识到肠系膜纤维瘤病的罕见表现和危险因素有助于早期诊断，管理,和治疗。重要的是,这也有助于预防并发症，如肠梗阻，肠缺血,和瘘管形成。
