Fibromatosis, Abdominal

纤维瘤病,腹部
  • 文章类型: Letter
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    文章类型: Case Reports
    纤维瘤病是一种相对罕见的疾病,常伴有家族性腺瘤性息肉病和腹部手术史。一名43岁的男性患者出现腹痛,对比增强CT显示下腹部有肿块。肿块呈4×4×3厘米白色,具有嗜酸性粒细胞纺锤形细胞的花环状排列的致密肿瘤。免疫染色显示KIT(-),CD34(-),desmin(-),β-连环蛋白(+),SMA(几个+),诊断为纤维瘤样纤维化。手术后六个月,无明显复发.
    Desmoid-type fibromatosis is a relatively rare disease, often associated with familial adenomatous polyposis and a history of abdominal surgery. A 43-year-old male patient presented with abdominal pain and contrast-enhanced CT showed a mass in the lower abdomen. The mass was a 4×4×3 cm white, dense tumor with a wreath-like arrangement of eosinophilic spindle-shaped cells. Immunostaining showed KIT(-), CD34(-), desmin(-), β-catenin(+), SMA(few+), and the diagnosis was desmoid-type fibrosis. Six months after surgery, there was no apparent recurrence.
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  • 文章类型: Case Reports
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    文章类型: Review
    病人是一名27岁的男子。他被转诊到我们医院,因为他知道腹部有肿块。腹部超声显示70毫米的肿块病变。增强的计算机断层扫描显示70毫米的肿块,边缘清晰,近端空肠附近的内部增强不均匀。患者被诊断为十二指肠或小肠原发性粘膜下肿瘤,并计划手术来诊断和治疗肿瘤。肿瘤位于空肠上肠系膜,肿瘤切除和部分小肠切除。组织病理学检查显示梭形细胞增殖,无核分裂,和组织中混合的胶原纤维。免疫组织化学显示β-catenin(+),SMA(+),AE1/AE3(-),KIT(-),CD34(-),S-100(-)。基于这些发现,我们诊断为原发性小肠系膜纤维瘤病。在这份报告中,我们描述了一例原发性小肠系膜纤维瘤病,并回顾了文献。
    The patient was a 27-year-old man. He was referred to our hospital because he was aware of a mass in his abdomen. An abdominal ultrasound showed a 70-mm mass lesion. Enhanced computed tomography showed a 70-mm mass with well- defined margins and heterogeneous internal enhancement near the proximal jejunum. The patient was diagnosed with a suspected primary submucosal tumor of the duodenum or small intestine, and surgery was planned to diagnose and treat the tumor. The tumor was located in the upper jejunal mesentery, and tumor resection and partial small bowel resection were performed. Histopathological examination revealed proliferation of spindle-shaped cells without karyomitosis, and mixed collagen fibers in the tissue. Immunohistochemistry showed β-catenin(+), SMA(+), AE1/AE3(-), KIT(-), CD34(-), and S-100(-). Based on these findings, we diagnosed primary desmoid fibromatosis of the small intestinal mesentery. In this report, we describe a case of primary desmoid fibromatosis of the small intestinal mesentery with a review of the literature.
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  • 文章类型: Review
    纤维瘤是罕见的软组织肿瘤,在初次切除后表现出局部侵袭性和高局部复发率。关于怀孕期间增大的硬纤维瘤的治疗时机和方法,尚无固定的建议。纤维瘤在怀孕期间倾向于增大,而且大多数产后不会自发消退。因此,即使在怀孕期间也可能需要手术。我们报告了一例腹壁硬纤维瘤,在怀孕期间生长到90毫米,并在妊娠17周时切除。进行了边缘切除,手术切缘镜下呈阳性。术后病程和妊娠顺利,在15个月的随访中没有观察到复发.
    Desmoid tumors are rare soft-tissue tumors that exhibit locoregional aggressiveness and a high local recurrence rate following initial resection. No fixed recommendations have been established with regard to the timing and method of treatment for desmoid tumors that enlarge during pregnancy. Desmoid tumors tend to enlarge during pregnancy, and most do not regress spontaneously postpartum. Thus, surgery may be required even during pregnancy. We report a case of an abdominal wall desmoid tumor that grew to 90 mm during pregnancy and was resected at 17 weeks of gestation. Marginal resection was performed, and the surgical margin was microscopically positive. The postoperative course and the pregnancy were uneventful, and no recurrence was observed at the 15-month follow-up visit.
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  • 文章类型: Journal Article
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    文章类型: Case Reports
    病人22岁,female.她有家族性腺瘤性息肉病(FAP)的家族史,并且在18岁时对FAP进行了预防性全结直肠切除术。她出现发烧和腹胀,并在右下腹部触诊有压痛的肿块。腹部对比增强CT扫描显示肿瘤边缘周围的对比效果不均匀。随着腹内硬纤维瘤的诊断,十二指肠部分切除术,小肠肿块切除术,右输卵管切除术和肿瘤一起进行,用空肠制造了一个人造肛门。硬纤维瘤切除后16个月,腹部增强CT扫描显示肠系膜有6.5厘米长的硬纤维瘤复发。她接受了5个疗程的阿霉素(DOX)加达卡巴嗪(DTIC)治疗,然后继续使用NSAIDs。手术七年后,她已经能够维持复发性肿瘤的缩小,并且仍在服药。由于将来可能出现其他相关病变,因此需要长期监测。
    The patient is a 22-year-old, female. She had a family history of familial adenomatous polyposis(FAP)and a prophylactic total colorectal resection was performed for FAP at age of 18. She presented with fever and abdominal distention and palpated a mass with tenderness in the right lower abdomen. Contrast-enhanced CT scan of the abdomen showed a heterogeneous contrast effect around the tumor margins. With the diagnosis of intra-abdominal desmoid tumor, a partial duodenal resection, small bowel mass resection, and right fallopian tube resection were performed along with the tumor, and an artificial anus was created with the jejunum. Contrast-enhanced CT scan of the abdomen 16 months after resection of desmoid tumor showed a 6.5 cm long desmoid tumor recurrence in the mesentery. She received 5 courses of doxorubicin (DOX)plus dacarbazine(DTIC)therapy followed by continued NSAIDs. Seven years after the operation, she has been able to maintain the shrinkage of the recurrent tumor and is still on medication. Long-term surveillance is necessary because of the possibility of the appearance of other associated lesions in the future.
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    文章类型: Case Reports
    纤维瘤是一种罕见的软组织肿瘤。发生频率为每百万人每年2.4至4.3例,这是一种非常罕见的疾病。我们经历了一个巨大的腹内硬纤维瘤,被认为是原发性肠系膜。此案是一名20多岁的男性。他去看了附近的医生,主诉腹胀和腹痛。腹部造影CT显示腹部巨大肿块,从右上腹部延伸至骨盆,边界清晰,大小为35×25cm。手术诊断为腹内肿块。开腹肿瘤切除术。由于渗入十二指肠,横结肠,还有胰腺,行右半结肠切除术和十二指肠联合切除术。病理诊断为硬纤维瘤。
    Desmoid tumor is a rare tumor of the soft tissue. The frequency of occurrence is 2.4 to 4.3 cases per year per million people, which is a very rare disease. We experienced a huge intra-abdominal desmoid tumor which is thought to be the primary mesentery. The case was a male in his 20s. He visited a nearby doctor with a complaint of abdominal bloating and abdominal pain. Abdominal contrast CT revealed a huge abdominal mass with a clear boundary of 35×25 cm in size extending from the upper right abdomen to the pelvis. Surgery was performed with a diagnosis of an intra-abdominal mass. Open abdominal tumor resection. Due to infiltration into the duodenum, transverse colon, and pancreas, right hemicolectomy and duodenal combined resection were performed. The pathological diagnosis was a diagnosis of desmoid tumor.
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  • DOI:
    文章类型: Case Reports
    1例71岁病理Ⅰ期(pT1bN0M0)患者行腹腔镜乙状结肠癌切除术。术后18个月,随访计算机断层扫描(CT)显示吻合部位附近30mm增强的软组织肿瘤。考虑磁共振成像(MRI)和正电子发射断层扫描(PET)的结果,我们诊断乙状结肠癌局部复发。腹腔镜结肠和肠根治术,包括肿瘤,已执行。病理上,肿瘤由具有胶原纤维的梭形细胞组成,通过免疫染色诊断为硬纤维瘤(β-catenin,c-kit-,CD34-,α-SMA-,和狗-1-)。我们报告了一例腹腔镜乙状结肠癌切除术后吻合口附近的腹内硬纤维瘤。
    A 71-year-old man with pathological Stage Ⅰ(pT1bN0M0)underwent laparoscopic sigmoid colon cancer resection. After 18 months postoperatively, follow-up computed tomography(CT)showed a 30 mm enhanced soft tissue tumor near the anastomotic site. Considering the magnetic resonance imaging(MRI)and positron emission tomography(PET)results, we diagnosed sigmoid colon cancer with local recurrence. Laparoscopic radical resection of the colon and intestine, including the tumor, was performed. Pathologically, the tumor comprised spindle-shaped cells with collagen fibers and was diagnosed as a desmoid tumor by immunostaining(β-catenin+, c-kit-, CD34-, α-SMA-, and DOG-1-). We report a case of intra-abdominal desmoid tumor near the anastomotic site after laparoscopic sigmoid colon cancer resection.
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  • 文章类型: Case Reports
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