Fibromatosis, Abdominal

纤维瘤病,腹部
  • 文章类型: Letter
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    评估超声(US)引导下高强度聚焦超声(HIFU)消融治疗不可切除和复发的腹内纤维瘤的疗效和安全性。2014年6月至2020年3月,15例经病理证实为腹内硬纤维瘤的连续无法切除和复发的患者接受了US指导的HIFU消融治疗。所有患者在HIFU治疗前后均行对比增强磁共振成像。使用非灌注体积比评估HIFU治疗的效果。记录术中和术后不良反应和并发症,以评估治疗的安全性。在随访期间,通过系列对比增强成像检查研究了HIFU消融的结果。在15名患者中,有14名成功完成了整个治疗,1例患者无效,放弃进一步治疗。平均非灌注体积比为71.1%(95%置信区间,3%至88.2%)。在平均29个月(范围从8到61个月)的随访期间,平均肿瘤体积减少了59%(95%置信区间,+49%至-100%)。除一名患者外,所有患者均无肿瘤沿治疗区域扩散。5例(33.3%)患者出现并发症,包括肠破裂(1例),腹内脓肿(1例),股神经轻度损伤(1例),和骨损伤(2例),肠破裂患者接受了手术;其他人在随访期间已治愈。US引导的HIFU消融是一种有效的治疗方式,适用于无法切除和复发性腹内硬纤维瘤的患者。
    To assess the efficacy and safety of ultrasound (US)-guided high-intensity focused ultrasound (HIFU) ablation for treatment of unresectable and recurrent intra-abdominal desmoid tumors. From June 2014 to March 2020, 15 patients with consecutive unresectable and recurrent diseases that pathologically proven to be intra-abdominal desmoid tumors had undergone the treatment of US-guided HIFU ablation. All patients underwent contrast-enhanced magnetic resonance imaging before and after HIFU treatment. Nonperfused volume ratio was used to evaluate the effect of HIFU therapy. Intraprocedural and postprocedural adverse effects and complications are recorded to assess the safety of the therapy. Outcome of HIFU ablation has been investigated through serial contrast-enhanced imaging examinations during follow up. Out of 15 patients 14 of them have successfully completed the whole therapy, 1 patient is ineffective and gives up further treatment. The mean nonperfused volume ratio is 71.1% (95% confidence interval, 3% to 88.2%). During a mean follow up of 29 months (range from 8 to 61 months), the mean tumor volume was reduced by 59% (95% confidence interval, +49% to -100%). No tumor spreads along the treated area in all patients except one. Complications have occurred in 5 patients (33.3%), including bowel rupture (1 case), intra-abdominal abscess (1 case), slight injury to the femoral nerve (1 case), and bone injury (2 cases), the bowel rupture patient underwent surgery; the others have been cured during the follow up. US-guided HIFU ablation is an effective treatment modality for patients suffered from unresectable and recurrent intra-abdominal desmoid tumors.
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  • 文章类型: Case Reports
    背景:家族性腺瘤性息肉病(FAP)与纤维瘤(DTs)并发脓肿形成相关的情况很少见。管理并不容易,选择最佳治疗方法可能会引起争议。
    方法:一名33岁男子因腹痛入院,发烧,发冷,恶心,和呕吐。他有FAP家族史,腹部手术史.
    方法:腹部增强胸部计算机断层扫描(CT)扫描显示腹壁软组织肿块和不规则的肠系膜软组织肿块,伴有内瘘和腹内脓肿。CT引导的腹壁肿块活检显示DTs。
    方法:患者口服抗生素6个月,并最终接受了手术。
    结果:患者在随访10个月时没有复发的证据。
    结论:该病例表明,对于有腹部手术史,腹部肿块和脓肿逐渐扩大的FAP患者,有必要考虑DTs的可能性。FAP相关的DTs很少因脓肿形成而复杂化。抗生素治疗加手术切除肿瘤可能是有效的,预后良好。
    BACKGROUND: Familial adenomatous polyposis (FAP) associated with desmoids tumors (DTs) complicated by abscess formation is rare. The management is not easy and the choice of the best treatment may be controversial.
    METHODS: A 33-year-old man was admitted to our hospital for abdominal pain, fever, chills, nausea, and vomiting. He had a family history of FAP, and history of abdominal surgery.
    METHODS: An abdominal enhanced chest computed tomography (CT) scan revealed a soft tissue mass in the abdominal wall and an irregular mesenteric soft tissue mass with internal fistula and intra-abdominal abscess. A CT-guided biopsy of the abdominal wall mass revealed DTs.
    METHODS: The patient was given oral antibiotics for 6 months, and ultimately underwent surgery.
    RESULTS: The patient had no evidence of recurrence on follow-up at 10 months.
    CONCLUSIONS: This case indicates that for patients with FAP who have a history of abdominal surgery and a progressively enlarging mass and abscess in the abdomen, it is necessary to consider the possibility of DTs. FAP-related DTs are rarely complicated by abscess formation. Antibiotic therapy plus surgical resection of the tumor may be effective and make good prognosis.
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  • 文章类型: Case Reports
    背景:Gardner综合征是家族性腺瘤性息肉病(FAP)的一种亚型,以腺瘤性肠息肉和结肠外病变如多发性骨瘤为特征,牙齿异常,还有软组织肿瘤.尽管12%的FAP肠息肉病患者可能发生腹内硬纤维瘤,妊娠合并巨大的腹部硬纤维瘤是一种相对罕见的病例。
    方法:一名28岁的孕妇被诊断为Gardner综合征,由于家族性腺瘤性息肉病,在接受腹腔镜全结肠切除术一年后发现腹内肿瘤。妊娠32周时,她第三次到我们部门就诊,抱怨由大约21×12cm2的巨大腹部肿块引起的上腹痛。经过多学科的协商和讨论,决定保胎治疗。一个女婴出生后,进行腹部肿块切除术,病理检查显示纤维腺瘤。患者一周后出院,半年随访顺利。
    结论:Gardner综合征是典型的综合征,包括家族性腺瘤性息肉病和肠外组织肿瘤。纤维瘤很少像胎儿一样大,局部具有侵袭性。在我们的案例中,我们选择手术切除胎儿自然分娩后的腹内纤维瘤,在6个月的随访中未观察到异常。怀孕期间的妇女患纤维瘤的风险增加,性激素可能是触发因素之一。因此,我们建议当Gardner综合征患者想要再次怀孕时,他们应该至少每3个月去医院定期复查一次。
    BACKGROUND: Gardner syndrome is a subtype of familial adenomatous polyposis (FAP), characterized by a combination of adenomatous intestinal polyps and extracolonic lesions such as multiple osteomas, dental abnormalities, and soft tissue tumors. Although 12% of patients with intestinal polyposis of FAP may occur intra-abdominal desmoid tumors, pregnancy complicating with giant abdominal desmoid tumors is a relatively rare case.
    METHODS: A 28-year-old pregnant woman was diagnosed with Gardner syndrome in whom an intra-abdominal tumor was found a year after undergoing a laparoscopic total colectomy due to family adenomatous polyposis. At 32 weeks\' gestation, she presented to our department for the third time complaining upper abdominal pain caused by the giant abdominal mass about 21 × 12 cm2 in size. After multidisciplinary consultation and discussion, the decision of fetal preservation treatment was made. After the delivery of a baby girl, abdominal mass resection was performed, and pathological examination revealed a fibrous adenoma. The patient was discharged after a week and was uneventful in the follow-up for half a year.
    CONCLUSIONS: Gardner syndrome is characterized by typical syndrome including family adenomatous polyposis and extra-intestinal tissue tumor. Were desmoid tumors rarely as large as fetus and local aggressively. In our case, we selected surgery to remove the intra-abdominal desmoid tumor after the natural delivery of the fetus and no abnormalities were observed during the 6 months follow-up. Women during pregnancy have an increased risk for the development of desmoid tumors, likely with the sex hormone to be one of the triggers. Therefore, we suggested that when a patient with Gardner syndrome desire to conceive again, they should go to the hospital for a regular review at least once every 3 months.
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  • 文章类型: Case Reports
    背景:纤维瘤或硬纤维瘤是相对罕见的源自肌膜神经系统的肿瘤。这种肿瘤没有特定的临床症状,有时会被误诊为其他疾病,例如胃肠道间质瘤(GIST)。
    方法:一名28岁男子因腹部肿块而去北京协和医学院就诊,无腹痛或腹胀。
    方法:考虑影像学特征和临床表现,该肿块在手术前主要被诊断为GIST.在手术过程中,在回盲部肠系膜下发现了这个位置,外观呈灰白色,坚韧的质地,流动性差,这与GIST的性质不一致。手术后,病理检查和个体免疫组织化学结果表明,该病变与腹膜后纤维瘤病合并慢性淋巴结炎化脓性炎症的诊断相符。
    方法:因此,我们决定进行肿瘤切除,右半结肠切除术,十二指肠部分切除术,剖腹手术的肠吻合术,但正确的输尿管被保留了.切除肿瘤后,肠段的末端连续缝合。
    结果:患者没有经历术中或术后并发症,术后3天出院。每3个月进行定期随访体格检查,如腹部超声和计算机断层扫描,在整个12个月内没有观察到复发的证据。
    结论:总而言之,腹内纤维瘤病是一种极其罕见的肿瘤,必须与其他消化道肿瘤区分开来,病理和免疫组织化学检查是诊断的关键部分。纤维瘤病的早期诊断对于结果至关重要。广泛切除肿块可以最大程度地减少复发的风险。
    BACKGROUND: Fibromatoses or desmoid tumors are relatively rare tumors derived from the musculoaponeurotic system. This tumor has no specific clinical symptoms and it is sometimes misdiagnosed as other diseases such as gastrointestinal stromal tumors (GISTs).
    METHODS: A 28-year-old man visited Peking Union Medical College for a tangible abdominal mass without abdominal pain or distention.
    METHODS: Considering the imaging characteristics and clinical manifestation, this mass was primarily diagnosed as GIST before surgery. During the surgery, the occupancy was found under the ileocecal mesentery, with grayish white appearance, tough texture, and poor mobility, which was not consistent with the character of the GIST. After the surgery, pathological examination and individual immunohistochemistry results demonstrated that the lesion was compatible with the diagnosis of retroperitoneal fibromatosis with purulent inflammation of chronic lymphadenitis.
    METHODS: Therefore, we decided to perform tumor mass resection, right colon resection, partial duodenum resection, and intestinal anastomosis on laparotomy, but the right ureter was retained. After excision of the tumor, the ends of the intestine segment were continuously sutured.
    RESULTS: The patient experienced no intraoperative or postoperative complications, and was discharged 3 days after surgery. Periodic follow-up physical examinations such as the abdominal ultrasound and computed tomography were performed each 3 months, and no evidence of recurrence was observed during the whole 12 months.
    CONCLUSIONS: To sum up, intra-abdominal fibromatosis is an extremely rare tumor that must be differentiated from other tumors of the digestive tract, and pathological and immunohistochemical examination is a critical part of the diagnosis. Early diagnosis of fibromatosis is essential for the outcome. Extensive resection of the mass minimizes the risk of relapse.
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  • 文章类型: Case Reports
    BACKGROUND: Mesenteric fibromatosis (MF) is a rare tumor whose biological behavior is intermediate between benign fibrous neoplasms and fibrosarcomas, and the characteristic of these tumors are local aggressive lesions which is prone to local recurrence but non-metastasizing. The common symptom is abdominal distention or painless mass. We report a case of giant MF in abdominal cavity with abdominal distention as the main symptom.
    METHODS: A 26-year-old male presented with 2-month history of abdominal distention, lack of appetite, and symptoms grew progressively more debilitating with time.
    METHODS: This patient underwent a contrast-enhanced computed tomography scan which showed a giant (37 × 25 × 13 cm), inhomogeneous enhancing, well-defined, and soft tissue density mass in abdominal cavity, possibly arising in mesocolon, which suggested a high possibility of MF. The postoperative pathology showed that the tumor cells to be positive for β-catenin, vimentin, negative for CD34, CD117, DOG-1, S-100, Desmin, which confirmed the diagnosis of MF.
    METHODS: Exploratory laparotomy was performed, which revealed a large mass involving the transverse colon wall, the root of mesocolon, and encasing the middle colic vessels and the 1st branch of jejunal arteries. The complete surgical resection was performed and the mass weighted 10 kilograms (kg).
    RESULTS: The patient recovered uneventfully and was discharged 9 days after surgery. Three-month, 6-month, 12-month and 18-month on follow-up after surgery, showed no evidence of recurrence.
    CONCLUSIONS: The MF is a very rare tumor, especially a giant tumor (10 kg) involving the muscular layer of colon wall. In addition, treatment of giant MF still remains a challenge. We consider that surgical resection with negative margins is the goal but not at the expense of damaging the function of vital organs. Specific measures should be considered based on the individual patient in order to relieve symptoms and improve quality of life.
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  • 文章类型: Case Reports
    背景:腹壁侵袭性纤维瘤病(AF)也称为硬纤维瘤,韧带肿瘤,纤维组织肿瘤增生,肌腱膜纤维瘤或软组织韧带纤维瘤,等。腹壁纤维瘤病于1832年由MacFarlane首次描述,并于1838年由Muller根据其一般外观和质地首次命名。这种疾病长期以来一直被误认为是良性病变,因为当通过病理学检查细胞时,通常会显示正常的有丝分裂,临床上没有发现远处转移,但实际上这种疾病是局部侵入性的,并显示出局部侵入性的生长。是一种罕见的低度恶性软组织肿瘤。目前,这种疾病的主要治疗方法是手术,放疗和激素治疗有一定的效果,但是这些疗法并不理想。
    一个32岁的女人,三年前接受剖腹产手术的人来到医院,发现腹壁有半个月的肿块。
    腹壁肿块。
    方法:接受手术。
    结果:病理:病灶为腹壁侵袭性纤维瘤病(腹壁韧带瘤)。
    结论:我们讨论了其临床特征的特殊性,治疗策略和预后结合文献复习,我们认为外科医生需要高度关注这种疾病,让更多的患者得到及时的治疗,正确合理的治疗,从而提高生活质量。
    BACKGROUND: Aggressive fibromatosis (AF) of abdominal wall is also called desmoid tumor, ligament tumor, fibrous tissue tumor hyperplasia, tendon membrane fibroma or soft tissue ligament fibroma, etc. Aggressive fibromatosis of abdominal wall was first described by MacFarlane in 1832, and it was named for the first time by Muller according to its general appearance and texture in 1838. This disease has been mistaken for a benign lesions for a long time because when the cells were examined by pathology often show normal mitosis, and distant metastases are not found clinically, but actually the disease is locally invasive and shows a local invasive growth. So it is a rare low-grade malignant soft tissue tumor. At present, the main treatment for the disease is operation, and radiotherapy and hormone therapy have a certain effect, but these therapies are not ideal.
    UNASSIGNED: A 32-year-old woman, who underwent cesarean section three years ago came to the hospital for finding a mass on abdominal wall for half a month.
    UNASSIGNED: Mass of abdominal wall.
    METHODS: Underwent surgery.
    RESULTS: Pathology: The lesion is aggressive fibromatosis of abdominal wall (ligament tumor of abdominal wall).
    CONCLUSIONS: We discussed the particularity of its clinical characteristics, treatment strategies and prognosis combined with literature review, and we think the surgeons need to pay high attention to this disease and make more patients get timely, correct and reasonable treatment, so as to improve the quality of life.
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