Desmoid-type fibromatosis

纤维样型纤维瘤病
  • 文章类型: Letter
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  • 文章类型: Case Reports
    纤维瘤,也被称为侵袭性纤维瘤病,代表一种罕见的成纤维细胞增殖形式。这些肿瘤可能出现在整个身体的任何肌肉筋膜结构中。由于几个独特的特征,它们被归类为良性的:组织学上,它们表现出规则的有丝分裂活性,并且没有转移潜力。计算机断层扫描(CT)仍然是精确诊断的最终方式,强烈建议手术切除。此帐户详细介绍了位于31岁女性患者前腹壁的硬纤维状肿瘤的表现,该患者明显缺乏任何先前的手术干预措施。手术干预需要切除肿瘤并随后使用聚丙烯网片重建腹壁。术后,病人在三天后从医疗机构获释,没有经历术后并发症。随后是六个月的间隔,没有任何不良事件。
    Desmoid tumors, also referred to as aggressive fibromatosis, represent an uncommon form of fibroblastic proliferation. These neoplasms may arise within any musculoaponeurotic structure throughout the body. They are classified as benign due to several distinctive features: histologically, they exhibit regular mitotic activity and are devoid of metastatic potential. Computed tomography (CT) remains the definitive modality for precise diagnosis, and surgical excision is strongly advised. This account details the manifestation of a desmoid tumor located in the anterior abdominal wall of a 31-year-old female patient who notably lacks any prior surgical interventions. The surgical intervention entailed the excision of the neoplasm and subsequent reconstruction of the abdominal wall utilizing a polypropylene mesh. Postoperatively, the patient was released from the medical facility after a period of three days, having experienced no post-surgical complications. This was followed by a six-month interval free of any adverse events.
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  • 文章类型: Case Reports
    纤维瘤病(DF)的特征是罕见的单克隆成纤维细胞增殖,表现出可变且不可预测的临床表现。DF可分为散发性和遗传性。尽管进行了广泛的研究,DF的确切病因仍然难以捉摸。
    一名31岁的男性患者出现在医院,右下腹部肿块逐渐增大,伴有腹部不适。入院前1周发现症状。肠镜检查没有发现结肠异常,血液检查没有任何异常。由于手术过程中质量的不确定性,部分切除回肠和盲肠,然后是回肠结肠端对端吻合术,术后无并发症。最终的病理诊断证实远端回肠的原发性纤维样型纤维瘤病(侵袭性纤维瘤病)。为了有效地管理DF,我们建议对患者进行随访.这包括手术后第一年每3个月预约一次,其次是每6个月的任命,直到第五年,此后每年一次.随访检查应包括收集患者的病史,体检,验血,超声波,CT扫描,以及其他相关评估。在后续行动的第一年,没有进行进一步的治疗,患者保持无病。
    起源于小肠的纤维瘤病(DF)是一种极为罕见的疾病,表现出局部侵袭性,可能危及生命。尽管它的组织学是良性的,DF具有高的局部复发率并且缺乏转移潜力。DF的诊断仍然具有挑战性,特别是在由于无症状患者或部分器官受损而导致手术干预不可行的情况下。在这种情况下,建议采用“观察等待”方法作为初始治疗策略。然而,当术前诊断困难时,手术通常被认为是最好的选择。鉴于局部复发的可能性和不确定的长期预后,定期跟进是必要的。
    UNASSIGNED: Desmoid-type fibromatosis (DF) is characterized by a rare monoclonal fibroblast proliferation that exhibits variable and unpredictable clinical presentation. DF can be classified into sporadic and hereditary types. Despite extensive research efforts, the exact etiology of DF remains elusive.
    UNASSIGNED: A 31-year-old male patient presented to the hospital with a progressively growing mass in the right lower abdomen, accompanied by abdominal discomfort. Symptoms are discovered 1 week before admission. Enteroscopy revealed no evidence of colonic abnormalities, and blood tests did not indicate any abnormalities. Due to the indeterminate nature of the mass during surgery, a partial resection of the ileum and cecum was performed, followed by ileocolonic end-to-end anastomosis, with no postoperative complications. The final pathological diagnosis confirmed primary desmoid-type fibromatosis of the distal ileum (invasive fibromatosis). To effectively manage DF, we recommend a follow-up schedule for patients. This includes appointments every 3 months in the first year following surgery, followed by appointments every 6 months up to the fifth year, and then once a year thereafter. The follow-up examinations should include collection of the patient\'s medical history, physical examination, blood tests, ultrasounds, CT scans, and other relevant assessments. During the first year of the follow-up period, no further treatment was administered, and the patient remained disease-free.
    UNASSIGNED: Desmoid-type fibromatosis (DF) originating from the small intestine is an extremely rare condition that exhibits local invasiveness and can be life-threatening. Despite its benign histology, DF has a high local recurrence rate and lacks metastatic potential. Diagnosis of DF remains challenging, especially in cases where surgical intervention is not feasible due to asymptomatic patients or partial organ impairment. In such cases, a \"watchful waiting\" approach is recommended as the initial treatment strategy. However, when preoperative diagnosis is difficult, surgery is typically considered the best option. Given the potential for local recurrence and the uncertain long-term prognosis, regular follow-up is necessary.
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  • 文章类型: Case Reports
    纤维样型纤维瘤病(DF)是一种罕见的软组织肉瘤亚型,最常见于前腹壁。当发生在腹膜后,DF通常是家族综合征的一部分,但很少偶发性。这使得必须报告DF的任何经验实例以及不同管理方法的肿瘤学结果。我们报告了两例在我们机构的腹膜后发生的散发性和严重的DF。
    第一个病例是一名男性,表现为尿路梗阻症状,并接受了延伸到左肾的肿瘤的手术切除。第二例是一名女性,有大腿复发性纤维瘤的病史,并在影像学上偶然诊断为腹膜后DF。她接受了肿瘤切除和放疗;然而,肿瘤复发并伴有尿路梗阻症状,需要再次手术切除。两种情况的组织病理学特征和放射学成像如下所述。
    纤维瘤经常复发,从而显著影响生活质量,这反映在我们的一个案例中。手术仍然是主要的治疗手段,本报告中提出的这两个病例都需要手术切除肿瘤作为对症和治愈措施。
    腹膜后DF是一种罕见的实体,我们的案例增加了关于这个主题的稀缺文献,这很可能有助于制定针对这种罕见的DF变体的实践改变建议和指南。
    Desmoid-type fibromatosis (DF) is a rare subtype of soft tissue sarcomas that most commonly occurs in the anterior abdominal wall. When occurring in the retroperitoneum, DF is usually part of familial syndromes while only rarely sporadic. This makes it imperative to report any instance of experience with DF and the oncological outcomes of the different approaches to management. We report two cases of sporadic and severe DF occurring in the retroperitoneum at our institution.
    UNASSIGNED: The first case is a male that presented with urinary obstruction symptoms and underwent surgical resection of the tumor that extended into the left kidney. The second case is a female with a history of recurrent desmoid tumors of the thigh and was incidentally diagnosed with retroperitoneal DF on imaging. She underwent tumor resection and radiotherapy; however, the tumor recurred with urinary obstruction symptoms that required another surgical resection. Histopathological characteristics and radiological imaging of both cases are described below.
    UNASSIGNED: Desmoid tumors often recur, thus significantly influencing the quality of life which is reflected in one of our cases. Surgery remains a mainstay treatment, and both cases presented in this report required surgical resection of the tumors as symptomatic and curative measures.
    UNASSIGNED: Retroperitoneal DF is a rare entity, and our cases add to the scarce literature available on the topic, which may well contribute to the formulation of practice-changing recommendations and guidelines focused on this rare variant of DF.
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  • 文章类型: Case Reports
    未经证实:侵袭性纤维瘤病是一种罕见的局部浸润性单克隆成纤维细胞增生,缺乏转移潜能。我们描述了一例罕见的年轻女性伴呕吐的腹内侵袭性纤维瘤病。
    未经证实:一名23岁女性因呕吐和体重减轻入院。
    UNASSIGNED:根据影像学发现和免疫组织学发现,制定了腹内侵袭性纤维瘤病的诊断。
    未经批准:手术后,在6个月的随访期间,没有发现局部复发的证据.
    未经评估:AF可以解释为什么孕妇会出现严重的呕吐。
    UNASSIGNED: Aggressive fibromatosis is a rare and locally infiltrative monoclonal fibroblastic proliferation with lack of metastatic potential. We describe a rare case of intra-abdominal aggressive fibromatosis on young female with hyperemesis.
    UNASSIGNED: A 23-year-old female was admitted with hyperemesis and loss of weight.
    UNASSIGNED: According to imaging findings and immunohistology findings, a diagnosis of intra-abdominal aggressive fibromatosis was formulated.
    UNASSIGNED: After the surgery, no evidence of local recurrence was noted during the 6 months of follow-up.
    UNASSIGNED: AF may explain why pregnant women may have severe hyperemesis.
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  • 文章类型: Case Reports
    背景:纤维样型纤维瘤病(DTF)是一种罕见的良性病变,通常来自腹壁或四肢,很少来自肠系膜或腹内器官。恶性腹膜间皮瘤也是一种罕见的,但侵略性疾病。据我们所知,这是恶性腹膜间皮瘤中首例纤维瘤样纤维瘤病的病例报告。
    方法:一名30岁的早期女性因腹部大肿块而转诊到我们的中心,原因是经过先前的细胞减灭术,腹腔热灌注化疗和辅助化疗后复发的恶性腹膜间皮瘤。进一步调查显示肠系膜肿块很大,与盲肠和回肠末端一起切除。病理结果证实了纤维瘤病的惊人诊断。
    结论:由于肿瘤边缘阴性,未对该患者提供辅助治疗;然而,对恶性腹膜间皮瘤和纤维瘤样纤维瘤病的复发进行密切随访,未来可以通过活检对该患者进行区分。
    BACKGROUND: Desmoid-type fibromatosis (DTF) is a rare benign lesion that usually arises from the abdominal wall or extremities and rarely from the mesentery or intrabdominal organs. Malignant peritoneal mesothelioma is also a rare, yet aggressive disease. To our knowledge, this is the first case report of desmoid-type fibromatosis in the setting of malignant peritoneal mesothelioma.
    METHODS: An early 30-year-old female was referred to our center for large intra-abdominal mass concerning for recurrent malignant peritoneal mesothelioma after previous cytoreductive surgery with hyperthermic intraperitoneal chemotherapy and adjuvant chemotherapy. Further investigation revealed a large mesenteric mass, which was resected en bloc with the cecum and terminal ileum. Pathologic findings confirmed a surprising diagnosis of desmoid-type fibromatosis.
    CONCLUSIONS: No adjuvant therapy was offered to this patient due to negative tumor margins; however, close follow-up will be provided for recurrence of both malignant peritoneal mesothelioma and desmoid-type fibromatosis, which can be differentiated in the future via biopsy in this patient.
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  • 文章类型: Journal Article
    涉及胰腺的纤维瘤病(纤维瘤)是一种罕见的诊断,在临床上可以模拟胰腺的肿瘤性和非肿瘤性病变。松散内聚的细胞学上枯燥的(myo)成纤维细胞的细胞学特征是非特异性的,然而,长束状生长模式和β-catenin突变的存在以及核免疫组织化学染色或分子检测阳性,可以明确诊断.虽然许多先前报道的胰腺纤维状肿瘤已经通过手术切除,“观察和等待”的保守治疗方法也是治疗这些肿瘤的有效模式。在这里,我们报道了胰腺硬纤维瘤的最大病例系列,细胞病理学,和放射学相关性。
    Desmoid-type fibromatosis (desmoid tumors) which involve the pancreas is an infrequent diagnosis which clinically can mimic both neoplastic and non-neoplastic lesions of the pancreas. The cytologic features of loosely cohesive cytologically bland (myo)fibroblastic cells are non-specific, however the long fascicular growth pattern and the presence of β-catenin mutation with positive nuclear immunohistochemical staining or molecular testing allows for definitive diagnosis. While many previously reported desmoid tumors of the pancreas have been surgically resected, conservative management with a \"watch and wait\" approach is also an effective mode of management for these tumors. Herein, we report the largest case series of pancreatic desmoid tumors with clinical, cytopathologic, and radiologic correlation.
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  • 文章类型: Case Reports
    一名80岁的男子因上腹痛被转诊到我们医院。腹部计算机断层扫描显示,胃胰腺区域有一个清晰的圆形腹内肿块,直径为15mm。六个月后,肿块病变呈轻度增强,在对比增强计算机断层扫描中,在肿块的下半部分发现了较弱的增强。肿块病变触及胃,而脂肪组织似乎介入肿块和胰腺之间。在磁共振成像上,在T1加权成像中,明确的肿块病变与肌肉具有等强度,在T2加权成像上对肌肉的轻微高强度,这表明一个丰富的纤维瘤。在全身麻醉下,患者接受了开放性手术。术中,肿瘤与胃分离并牢固地附着在胰腺上。因此,我们进行了胰体远端切除术和脾切除术。病理诊断为腹膜后韧带样型纤维瘤病,肿瘤边缘附着在胰腺上,脾动脉,和脾静脉.由于很少有关于在胃胰区腹膜后发生纤维瘤病的报道,很难与其他软组织肿瘤区分和确定肿瘤起源。通过放射学重新评估进行密切观察是一个有用的选择。磁共振成像信号和增强的模式可能有助于将纤维瘤病与其他软组织肿瘤区分开。放射学发现明确的纤维样型纤维瘤病可以通过肉眼或病理分析渗透到周围器官中。
    An 80-year-old man was referred to our hospital because of epigastric pain. Abdominal computed tomography revealed a well-defined circular intra-abdominal mass in the gastro-pancreatic region measuring 15 mm in diameter. After 6 months, the mass lesion was growing with mild enhancement, and weaker enhancement was found in the lower half of the mass on contrast-enhanced computed tomography. The mass lesion touched the stomach, whereas adipose tissue appeared to intervene between the mass and pancreas. On magnetic resonance imaging, the well-defined mass lesion had isointensity to muscle on T1-weighted imaging, slight hyperintensity to muscle on T2-weighted imaging, which indicated a rich fibrous tumor. Under general anesthesia, the patient underwent open surgery. Intraoperatively, the tumor was separated from the stomach and firmly attached to the pancreas. Therefore, we performed a distal pancreatomy with splenic resection. Pathological diagnosis was desmoid-type fibromatosis in the retroperitoneum, and the tumor margin was attached to the pancreas, splenic artery, and splenic vein. Since there are few reports of desmoid-type fibromatosis occurring in the retroperitoneum of the gastropancreatic region, it is difficult to distinguish from other soft tissue tumors and to identify the tumor origin. Close observation by radiological re-valuation was a useful option. Magnetic resonance imaging signals and an enhanced pattern may help distinguish a desmoid-type fibromatosis from other soft tissue tumors. A desmoid-type fibromatosis that is well-defined in radiological findings may infiltrate the surrounding organs with gross or pathological analyses.
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  • 文章类型: Case Reports
    一名65岁的男子通过联合脾切除术和部分横结肠切除术切除了巨大的肠系膜纤维瘤病(MF)。病理检查证实MF的存在,而基因检测显示肿瘤对他莫昔芬敏感。经过1年的随访,未发现腹部不适或复发的症状.
    A 65-year-old man underwent excision of a giant mesenteric fibromatosis (MF) via combined splenectomy and partial transverse colectomy. Pathological examination confirmed the presence of MF, whereas genetic testing indicated that the tumor was sensitive to tamoxifen. Over a 1-year follow-up, no symptoms of abdominal discomfort or recurrence was noted.
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  • 文章类型: Case Reports
    我们介绍了一例极为罕见的大腿深部血管粘液瘤(DAM)病例,该病例被误诊为纤维样型纤维瘤病。一名40岁的日本妇女左大腿上有一块肿块。穿刺活检的组织学诊断为纤维瘤病。肿瘤生长缓慢,并在4年后切除。切除肿瘤的组织学诊断为DAM。手术后16个月,患者没有发现任何局部复发。虽然下肢的DAM极为罕见,临床医生必须意识到它可能发生在相对靠近骨盆的区域。
    We present an extremely rare case of deep angiomyxoma (DAM) in the thigh that was misdiagnosed as desmoid-type fibromatosis. A 40-year-old Japanese woman presented with a mass on the left thigh. The histological diagnosis by needle biopsy was desmoid-type fibromatosis; the tumor grew slowly and was resected 4 years later. The histological diagnosis from the resected tumor was DAM. As of 16 months post-surgery, the patient has not noticed any local recurrence. Although DAM in a lower extremity is extremely rare, clinicians must be aware of its possible occurrence in areas relatively close to the pelvis.
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