Desmoid Tumor

硬纤维瘤
  • 文章类型: Case Reports
    纤维瘤(DT)是一种罕见的局部侵袭性但非转移性间充质软组织肿瘤,主要发生在腹壁,腹腔,和四肢。它在肠系膜的发生相对罕见。
    本文报道了胃肠外科治疗的两例硬纤维瘤,潍坊市人民医院.第一个病例是一名59岁的男性患者,他之前曾接受过食管胃结合部癌的手术。术后,他的腹内肿块在三个月内迅速增大。第二例是一名60岁的男性患者,偶然发现左下腹部有肿块。两名患者都接受了手术治疗,术后病理诊断为肠系膜纤维瘤。
    硬纤维瘤的治疗仍然具有挑战性。简单的手术切除通常会产生不令人满意的结果,辅助放疗和化疗的疗效也有限。进一步的研究和临床实践是必要的,以改善诊断和治疗策略,旨在提高患者的生存和生活质量。
    UNASSIGNED: Desmoid tumor (DT) is a rare locally aggressive but non-metastatic mesenchymal soft tissue neoplasm that predominantly occurs in the abdominal wall, abdominal cavity, and extremities. Its occurrence in the mesentery is relatively uncommon.
    UNASSIGNED: This article reports two cases of desmoid tumor treated at the Department of Gastrointestinal Surgery, Weifang People\'s Hospital. The first case was a 59-year-old male patient who had previously undergone surgery for esophagogastric junction cancer. Postoperatively, he developed an intra-abdominal mass that rapidly increased in size within three months. The second case was a 60-year-old male patient who incidentally discovered a mass in the left lower abdomen. Both patients underwent surgical treatment, and the postoperative pathological diagnosis was mesenteric desmoid tumor.
    UNASSIGNED: The treatment of desmoid tumor remains challenging. Simple surgical resection often yields unsatisfactory outcomes, and the efficacy of adjuvant radiotherapy and chemotherapy is also limited. Further research and clinical practice are necessary to improve diagnostic and therapeutic strategies, aiming to enhance patient survival and quality of life.
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  • 文章类型: Case Reports
    纤维增生性纤维瘤(DF)是一种罕见的良性骨肿瘤,采用攻击行为,代表了临床和影像学诊断的挑战。此病例报告集中于一名31岁的男子,该男子下颌病变较大,下颌牙齿严重移位。只有副临床发现的组合才能做出明确的诊断。颈面部MRI显示低T1信号强度,钆后周围增强,和T2高强度信号,而PET扫描显示中等代谢。骨活检和免疫组织化学分析允许在消除主要鉴别诊断后明确诊断DF(纤维发育不良,纤维肉瘤,硬纤维瘤,和骨肉瘤)。该患者通过下颌骨大切除和游离腓骨骨瓣重建成功治疗。”
    Desmoplastic fibroma (DF) is a rare benign bone tumor adopting an aggressive behavior, representing a challenge for clinical and radiographic diagnosis. This case report focused on a 31-year-old man with a large mandibular lesion with severe displacements of the mandibular teeth. Only a combination of paraclinical findings allows a definitive diagnosis to be made. Cervicofacial MRI revealed a low T1 signal intensity with peripheral enhancement after Gadolinium, and T2 hyperintense signal, while PET scan showed a moderate metabolism. Bone biopsy with immunohistochemical analysis allowed for definitive diagnosis of DF after eliminating the main differential diagnosis (fibrous dysplasia, fibrosarcoma, desmoid tumor, and osteosarcoma). The patient was successfully treated by large mandibular resection and reconstruction with a free-fibular bone flap\".
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  • 文章类型: Case Reports
    纤维瘤(DT),罕见的软组织良性肿瘤,表现出局部侵袭性和高复发率。起源于肌成纤维细胞增殖,完整的手术干预是首选治疗方法。尽管它们是良性的,这些肿瘤很少见,主要影响15至60岁的女性,青春期发病率较高。
    方法:一名44岁的女性,腿部有DT,模仿外窝坐骨神经病变。活检证实诊断,手术时保留了腓骨外神经,确保最佳的神经功能。随访两年无复发,证明了手术干预的成功。
    DTs,虽然罕见,表现出三种不同的基因组突变,与45F基因型相关的复发风险最高。一般是零星的,这些肿瘤可能与家族性腺瘤性息肉病(FAP)相关,并受激素过多症状态的影响.DTs通常表现为深层肿块,尽管完全切除,但局部复发频繁。
    结论:DTs提出了诊断和治疗挑战,通常需要完全的手术干预。管理取决于症状学,仔细监测小的无症状肿瘤和辅助放疗在不完全切除的情况下。尽管手术成功,频繁的复发强调需要深入研究以加强治疗方法.
    UNASSIGNED: Desmoid tumors (DT), rare benign neoplasms of soft tissues, exhibit local aggressiveness and high recurrence rates. Originating from myofibroblast proliferation, complete surgical intervention is the preferred treatment. Despite their benign nature, these tumors are infrequent, predominantly affecting women between 15 and 60, with a higher incidence in adolescence.
    METHODS: A 44-year-old woman with a DT in the leg mimicking external popliteal sciatic neuropathy. Diagnosis confirmed by biopsy, surgery performed with preservation of the external popliteal nerve, ensuring optimal nerve function. Two-year follow-up with no recurrence, demonstrating the success of the surgical intervention.
    UNASSIGNED: DTs, although rare, exhibit three distinct genomic mutations, with the 45F genotype associated with the highest risk of recurrence. Generally sporadic, these tumors can be linked to familial adenomatous polyposis (FAP) and influenced by states of hyperestrogenism. DTs typically present as deep-seated masses, with frequent local recurrence despite complete resection.
    CONCLUSIONS: DTs pose diagnostic and therapeutic challenges, often requiring complete surgical intervention. Management depends on symptomatology, with careful monitoring for small asymptomatic tumors and adjuvant radiotherapy in case of incomplete resection. Despite surgical success, frequent recurrence underscores the need for in-depth research to enhance therapeutic approaches.
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  • 文章类型: Case Reports
    韧带样肿瘤(DT)是一种罕见的由肌肉腱膜引起的良性肿瘤,主要与外伤或怀孕有关。DT具有渗透性和局部侵略性的生长模式,通常不会转移。然而,复发率和并发症发生率高。当它发生在怀孕时,怀孕和分娩被视为医生和助产士最佳管理的个案,在为患者寻找最佳分娩模式时需要谨慎,这取决于肿瘤的大小,location,行为,和过去的历史。作者报告了一例29岁的孕妇,该孕妇先前曾接受过大型腹壁硬纤维瘤的全身肿瘤治疗,随后怀孕。DT的历史提出了后续行动和交付挑战。在妊娠第38+4周选择选择性剖宫产的观察性管理,产后随访无并发症。作者详细介绍了临床管理和选择的治疗方法;化疗可以成为DTs患者治疗选择的一种选择,尽管大多数DTs都是通过随后的网状塑料进行手术治疗的。此外,作者提供了一个系统的文献,重点是孕妇在怀孕期间和产后期间DTs的治疗管理,因为妊娠相关的硬纤维瘤是一种特定条件,在没有建立最佳管理的地方,尽管一些针对非怀孕患者的指南。
    A desmoid tumor (DT) is a rare benign neoplasm arising from muscle aponeurosis, associated mostly with trauma or pregnancy. DT has an infiltrative and locally aggressive growth pattern and usually does not metastasize. However, it has a high recurrence and complication rate. When it occurs in pregnancy, the pregnancy and delivery is taken as an individual case for optimal management by physicians and midwifes, who need to be cautious in finding the optimal delivery mode for the patient, which depends on the tumor size, location, behavior, and past history. The authors report a case of 29-year-old pregnant woman who previously underwent systemic oncological treatment for a large abdominal wall desmoid tumor and became pregnant afterwards. The history of DT presented a follow-up and delivery challenge. Observational management was chosen with an elective cesarean section at week 38 + 4 of pregnancy with uncomplicated postpartum follow-up. The authors detail the clinical management and chosen therapeutic approach; chemotherapy can be a choice in the treatment options for patients with DTs, although the majority of DTs are treated surgically with subsequent mesh plastic. Moreover, the authors provide a systematic review of the literature focused on the treatment management of DTs in pregnant women during pregnancy and the postpartum period, as pregnancy-associated desmoid tumors are a specific condition, where the optimal management is not well established, despite some guidelines for non-pregnant patients.
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  • 文章类型: Case Reports
    硬纤维瘤是间质起源的成纤维细胞增殖,没有转移潜力,但具有局部侵略性。尽管对新诊断的硬纤维瘤患者的治疗已转向观察和积极监测,腹内肠系膜肿瘤或持续生长并引发症状的肿瘤可能需要及时手术治疗.只有少数病例报告说明了巨大的散发性腹内肠系膜衍生的硬纤维瘤,其中最长直径≥19cm。在本研究中,据报道,一名青少年男性患者,患有迅速增长的38厘米长的间充质起源的散发性腹内纤维瘤。由于无反应性和症状进展,患者接受化疗,然后进行手术切除,然后用维持辅助化疗,以防止由于肿瘤体积较大而复发。尽管肿瘤的快速生长和在腹腔内的高占有率,R0切除是成功的器官保存。患者已2年无复发,未来有望进一步跟进。
    A desmoid tumor is a fibroblastic proliferation of mesenchymal origin, which has no metastasizing potential but is locally aggressive. Although treatment has shifted to observation and active surveillance for newly diagnosed patients with desmoid tumors, intra-abdominal mesenteric tumors or tumors that persistently grow and provoke symptoms may need prompt surgical treatment. There have only been a small number of case reports that illustrate large sporadic intra-abdominal mesentery-deriving desmoid tumors in which the longest diameter was ≥19 cm. In the present study, an adolescent male patient with a rapidly growing 38-cm long sporadic intra-abdominal desmoid tumor of mesenchymal origin is reported. The patient was treated with chemotherapy followed by surgical resection due to non-responsiveness and progression of symptoms, then with maintenance adjuvant chemotherapy to prevent recurrence due to the large size of the tumor. Despite the rapid growth of the tumor and its high occupancy in the intra-abdominal cavity, an R0 resection was successful with organ preservation. The patient has been recurrence-free for 2 years, and further follow-up is expected in the future.
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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: Review
    背景:甲状腺乳头状癌(PTC)是一种常见的多变异瘤。这些极其罕见且描述不佳的变体之一是具有纤维瘤病样基质的PTC(PTC-FMS),一种特殊的实体,以其主要的间充质成分为特征。本文回顾了文献,讨论诊断挑战,以及这类肿瘤的临床和手术意义,文献报道的病例不到30例。
    方法:我们报告了一例在一名41岁的意大利女性身上发现的PTC-FMS,他以颈部肿块的形式来到我们研究所。进一步的免疫组织化学检查显示,间充质细胞的细胞核和细胞质中均有β-catenin异常染色。患者接受了甲状腺全切除术,并在手术后2个月接受了放射性碘(RAI)。
    结论:考虑到PTC-FMS复发的可能性和RAI治疗无效,完整的手术切除是这类肿瘤的主要治疗方法。尽管这些病变的具体性质尚未确定,应遵循经典PTC的准则。
    BACKGROUND: Papillary thyroid carcinoma (PTC) is a common neoplasia with multiple variants. One of these extremely rare and poorly described variants is PTC with fibromatosis-like stroma (PTC-FMS), a peculiar entity distinguished by its predominant mesenchymal component. This paper reviews the literature, discusses the diagnostic challenges, and the clinical and surgical implications of this type of tumor which has fewer than 30 cases reported in the literature.
    METHODS: We reported a case of PTC-FMS found in a 41-year-old Italian woman, who came to our Institute with a recent growth in the form of a mass on the neck. Further immunohistochemical examination showed β-catenin aberrant staining both in the nuclei and cytoplasm of the mesenchymal cells. The patient underwent total thyroidectomy and received radioactive iodine (RAI) 2 months after surgery.
    CONCLUSIONS: Given the possibility of recurrence of PTC-FMS and the ineffectiveness of RAI therapy, complete surgical resection represents the main treatment for this type of tumor. Despite the fact that the specific nature of these lesions has yet to be determined, guidelines for classical PTC should be followed.
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  • 文章类型: Review
    再扩张性肺水肿是在受损肺的胸外科手术后可能危及生命的情况。我们报告了一名24岁的女性,该女性计划切除大型胸内硬纤维瘤,该肿瘤在手术结束时表现为再扩张性肺水肿,并讨论了临床表现。该实体的机制和预测因素,并回顾文献中报道的类似病例。
    Re-expansion pulmonary edema is a potentially life-threatening situation following thoracic surgery of a compromised lung. We report the case of a 24-year-old female scheduled for a resection of a large intrathoracic desmoid tumor that presented with re-expansion pulmonary edema at the conclusion of her surgery and discuss the clinical presentation, mechanism and predictors of this entity and review similar cases reported in the literature.
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  • 文章类型: Journal Article
    由肌筋膜结构引起的纤维瘤病(DF)很少影响头颈部,腹部是最常见的起源部位。这些是具有局部浸润性的良性肿瘤,通常表现为快速生长的无痛肿胀。颞下窝DF是极为罕见的位置,临床报道很少。本文讨论了2岁儿童的颞下窝DF(ITF)的管理以及文献综述。
    Desmoid fibromatosis (DF) arising from musculoaponeurotic structures rarely affects the head and neck region with the abdomen being the most common site of origin. These are benign tumors with locally infiltrative nature usually presenting as painless swellings that are rapidly growing. The infratemporal fossa DF is an extremely rare location with few clinical reports. This article discusses the management of a 2-year-old child with DF of the infratemporal fossa (ITF) along with literature review.
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  • 文章类型: Journal Article
    背景:纤维瘤病是一组起源于肌肉结缔组织的良性肿瘤,上覆筋膜,骨膜,或腱膜。然而,他们可能需要多次切除,由于对相邻重要结构的压缩效应。案例报告:这里,我们讨论了一例3岁女孩的颌下纤维瘤病。她接受了保守的外科手术,没有进行广泛的下颌骨切除。她没有接受辅助治疗。术后期间顺利,具有适当的美容和功能结果。1年随访显示无复发。结论:儿童头颈部纤维瘤病是一种罕见的疾病,这需要多学科协议来进行适当的管理。完全的手术切除通常会导致适当的结果。对于不适合手术切除或残留肿瘤的复发性病变,也应牢记辅助治疗。
    Background: Fibromatosis is a group of benign tumors originating from connective tissues of muscle, overlying fascia, periosteum, or aponeurosis. However, they might need several excisional resections, owing to compressive effects on adjacent vital structures. Case Report: Here, we discussed a case of submandibular fibromatosis in a 3-year-old girl. She underwent a conservative surgical procedure without a wide mandible bone resection. She received no adjuvant therapy. The postoperative period was uneventful with an appropriate cosmetic and functional outcome. A 1-year follow-up revealed no recurrence. Conclusion: Fibromatosis of the head and neck in children is a rare condition, which needs a multidisciplinary agreement for its appropriate management. A complete surgical removal often leads to a proper outcome. Adjuvant therapy should also be kept in mind for recurrent lesions not candidate for surgical removal or in case of remnant tumors.
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