solitary fibrous tumor

孤立性纤维瘤
  • 文章类型: Journal Article
    背景:颅内孤立性纤维性肿瘤(SFT)是罕见的间充质肿瘤,由于它们与脑膜瘤和其他中枢神经系统肿瘤相似,通常难以诊断。虽然分子遗传学的进步有助于分类,诊断细微差别和优化管理策略仍然是人们感兴趣的领域。
    方法:这项回顾性研究分析了2020年2月至2024年1月在印度神经外科中心治疗的11例颅内SFT。临床数据,放射学发现,组织病理学特征,和随访细节进行了审查。免疫组织化学,特别是STAT6,促进诊断确认。
    结果:就诊年龄中位数为32岁,男性占主导地位。头痛是最常见的症状,常导致影像学误诊为脑膜瘤。组织学上,SFT表现为梭形至卵形细胞,鹿角状血管和胶原基质,在鉴别诊断中提出了挑战。WHO分级主要显示1级肿瘤,虽然复发,强调长期跟进的重要性。免疫组织化学,特别是STAT6,在区分SFT与其他实体方面发挥了关键作用。
    结论:颅内SFT由于与其他肿瘤的重叠特征而存在诊断挑战,保证一个综合的方法,整合临床,放射学,和组织病理学发现。免疫组织化学,特别是STAT6,成为一种有价值的诊断工具。长期随访对于监测复发和潜在的恶性转化至关重要。需要进一步的研究来描述最佳的治疗策略,包括放疗在SFT管理中的作用。
    BACKGROUND: Intracranial solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms, often challenging to diagnose due to their resemblance to meningiomas and other central nervous system tumors. While advancements in molecular genetics have aided in classification, diagnostic nuances and optimal management strategies remain areas of interest.
    METHODS: This retrospective study analyzed 11 cases of intracranial SFTs treated at a neurosurgical centre in India between February 2020 and January 2024. Clinical data, radiological findings, histopathological features, and follow-up details were reviewed. Immunohistochemistry, particularly STAT6, facilitated diagnosis confirmation.
    RESULTS: The median age of presentation was 32 years, with a male predominance. Headache was the most common presenting symptom, often leading to misdiagnosis as meningiomas on radiological imaging. Histologically, SFTs exhibited spindle to ovoid cells with staghorn vessels and collagenized stroma, posing challenges in differential diagnosis. WHO grading predominantly revealed grade 1 tumors, though recurrence occurred, emphasizing the importance of long-term follow-up. Immunohistochemistry, particularly STAT6, played a pivotal role in distinguishing SFTs from other entities.
    CONCLUSIONS: Intracranial SFTs present diagnostic challenges due to overlapping features with other tumors, warranting a comprehensive approach integrating clinical, radiological, and histopathological findings. Immunohistochemistry, particularly STAT6, emerges as a valuable diagnostic tool. Long-term follow-up is essential for monitoring recurrence and potential malignant transformation. Further research is needed to delineate optimal treatment strategies, including the role of radiotherapy in SFT management.
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  • 文章类型: Case Reports
    孤立性纤维性肿瘤(SFT)是一种罕见的间叶性肿瘤,可能是成纤维细胞起源的,主要在四肢和胸膜。肾上腺的原发性SFT在临床上更为罕见。这里,我们报道了一名47岁的女性在体检中发现左肾上腺肿块的病例,没有任何症状,也没有实验室异常.肾上腺的计算机断层扫描(CT)检查提示左肾上腺区域有圆形软组织密度阴影。未增强的扫描显示质量密度不均匀,里面有一个分散的圆形囊状低密度阴影,增强扫描显示明显的不均匀增强。我们认为它是肾上腺嗜铬细胞瘤。最终,患者接受了腹腔镜左肾上腺切除术。病理检查提示肾上腺SFT。我们回顾了以往肾上腺SFT的病例报道,并结合相关文献总结了肾上腺SFT的临床特点。对于具有不均匀低密度阴影和不均匀CT增强特征的肾上腺肿瘤,应考虑肾上腺SFT的鉴别诊断。
    Solitary fibrous tumor (SFT) is a rare mesenchymal tumor, probably of fibroblastic origin, mainly in the extremities and pleura. Primary SFT of the adrenal gland is clinically more rare. Here, we report the case of a 47-year-old woman who detected a left adrenal mass on physical examination, without any symptoms, and no laboratory abnormalities. A computed tomography (CT) examination of the adrenal gland suggested a round-like soft tissue density shadow in the left adrenal area. An unenhanced scan showed uneven density of the mass, with a scattered circular-like cystic low-density shadow inside, and an enhanced scan showed obvious uneven enhancement. We considered it to be adrenal pheochromocytoma. Ultimately, the patient was treated with laparoscopic left adrenalectomy. A pathological examination suggested an adrenal SFT. We reviewed previous case reports of adrenal SFTs and summarized the clinical characteristics of adrenal SFT combined with the relevant literature. For adrenal tumors with uneven low-density shadow and uneven CT enhancement features, we should consider the differential diagnosis of adrenal SFT.
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  • 文章类型: Journal Article
    目的:孤立性纤维性肿瘤(SFT)是一种少见的良性肿瘤。然而,多达20%的病例显示出局部浸润或转移的恶性趋势。通常出现在胸腔,文献中只有少数病例报道了直肠系膜组织的SFT。完整的手术切除,传统上通过后路,是治疗的选择。这篇综述的目的是证明经肛门微创手术(TAMIS)作为切除良性直肠旁实体瘤的手术方法的安全性和适用性。
    方法:我们报告一例52岁男性患者,该患者偶然诊断为远端直肠系膜SFT。进行TAMIS切除。基于这个案子,我们描述了该程序的步骤和潜在益处,并对文献进行了全面回顾.
    结果:组织病理学证实SFT完全切除。在顺利的术后过程和第四天出院后,多学科委员会建议通过临床检查和MRI进行随访,其中显示愈合良好的瘢痕,在切除后3年内没有复发。
    结论:直肠系膜的SFT是一种非常罕见的实体。据我们所知,这是关于SFT的TAMIS切除术的第一份报告,被证明是完全切除良性直肠旁实体瘤的安全方法。
    OBJECTIVE: Solitary fibrous tumors (SFT) are a rare entity of in majority benign neoplasms. Nevertheless, up to 20% of cases show a malignant tendency with local infiltration or metastasis. Commonly arising in the thoracic cavity, only few cases of SFT of the mesorectal tissue have been reported in the literature. Complete surgical resection, classically by posterior approach, is the treatment of choice. The purpose of this review is to demonstrate the safety and suitability of transanal minimally invasive surgery (TAMIS) as a surgical approach for the resection of benign pararectal solid tumors.
    METHODS: We report the case of a 52-year-old man who was diagnosed incidentally with SFT of the distal mesorectum. Resection by TAMIS was performed. Based on this case, we describe the steps and potential benefits of this procedure and provide a comprehensive review of the literature.
    RESULTS: Histopathology confirms the completely resected SFT. After uneventful postoperative course and discharge on day four, follow-up was recommended by a multidisciplinary board by clinical examination and MRI, which showed a well-healed scar and no recurrence up to 3 years after resection.
    CONCLUSIONS: SFT of the mesorectum is a very rare entity. To our knowledge, this is the first report on a TAMIS resection for SFT, demonstrated as a safe approach for complete resection of benign pararectal solid tumors.
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  • 文章类型: Case Reports
    孤立性纤维瘤/脑膜血管外皮细胞瘤(SFT/M-HPC)是一种罕见的肿瘤,约占颅内肿块的1%。这种病理有很高的复发和转移到远处的地方,如肝脏的风险,肺,和骨头。精确的诊断需要详细的组织病理学检查。
    我们提供2例SFT/M-HPC病例报告。第一个病例是一名44岁的女性,她出现头痛,恶心,呕吐,额叶共济失调几个月.影像学检查结果显示,矢状旁轴外肿块很大,两个侧脑室的额角受压。她接受了大体全切除术,术后时间简单。在5年的随访期间,患者没有复发的肿瘤或远端转移。第二例是一名48岁的男性,他表现为右侧偏盲和偏瘫。计算机断层扫描(CT)扫描显示巨大的枕骨轴外肿块,上矢状窦吞噬和半球间裂隙脱位。他接受了大体全切除术,术后时间简单。六年后,他表现出右侧的弱点。CT扫描显示先前位置有多灶性复发性肿块。他接受了次全切除术,术后时间简单。
    术前影像学上出现脑膜瘤样肿瘤块时,应考虑SFT/M-HPC。免疫组织化学研究对于正确诊断至关重要。严格的长期随访检查和定期的磁共振成像扫描是防止转移和大的复发肿块出现的关键。
    UNASSIGNED: Solitary fibrous tumor/meningeal hemangiopericytoma (SFT/M-HPC) is a rare neoplasm which accounts for around 1% of the intracranial masses. This pathology has a high risk for recurrence and metastasis to distant locations such as the liver, lungs, and bones. Precise diagnosis necessitates detailed histopathological examination.
    UNASSIGNED: We present two case reports of SFT/M-HPC. The first case is a 44-year-old female who presented with headache, nausea, vomiting, and frontal ataxia for several months. Imaging findings showed a large parasagittal extra-axial mass with compression of the frontal horns of both lateral ventricles. She underwent gross total resection with an uncomplicated postoperative period. The patient had no recurrent tumors or distal metastases in the follow-up period of 5 years. The second case is a 48-year-old male who presented with right-sided hemianopsia and hemiparesis. Computed tomography (CT) scans revealed a large parieto-occipital extra-axial mass with superior sagittal sinus engulfment and dislocation of the interhemispheric fissure. He underwent gross total resection with an uncomplicated postoperative period. Six years later, he presented with right-sided weakness. CT scan showed a multifocal recurrent mass at the previous location. He underwent subtotal resection with an uncomplicated postoperative period.
    UNASSIGNED: SFT/M-HPC should be considered when presented with a meningioma-like tumor mass on preoperative imaging. Immunohistochemical study is crucial for the correct diagnosis. Strict long-term follow-up examinations and regular magnetic resonance imaging scans are key to preventing the appearance of metastases and large recurrent masses.
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  • 文章类型: Case Reports
    孤立性纤维瘤(SFT)通常累及头颈部。头颈部SFT(HNSFT)表现出不同的组织学特征,并且可以模拟具有不同治疗和行为的几种肿瘤。在这里,我们报告了3例HNSFT的临床病理特征。病例1是一名29岁的女性,其鼻腔肿块为3.5cm。患者接受了手术切除。显微镜检查显示SFT的经典组织学和免疫组织化学(IHC)特征。异常的组织学特征包括上皮样形态,透明细胞,和水肿的变化。11个月后出现局部复发,也接受了手术治疗。病例2是一名55岁的男性,在颊粘膜处出现1厘米的肿块。进行了肿瘤的手术切除。肿瘤在显微镜下被完全限定。确定了SFT的特征性组织学和IHC特征。观察到的异常组织学特征是腺瘤样,透明细胞,和粘液样变化。患者在12个月的随访中存活且无病。病例3是一名59岁的女性,表现为1.4厘米的内侧can块。患者接受了手术切除。观察到的组织学和IHC特征是SFT的诊断。发现的异常组织学特征是波状核和多核基质巨细胞。在124个月的随访中,患者还活着并且没有疾病。在诸如头部和颈部区域的不寻常位置,SFT的诊断可能是具有挑战性的。此外,HNSFT的组织学谱是多样的。因此,有关异常组织学特征和经典IHC表达的知识对于建立正确的诊断至关重要。由于HNSFT复发的风险,建议长期随访。
    Solitary fibrous tumors (SFTs) uncommonly involve the head and neck region. Head and neck SFTs (HNSFTs) exhibit diverse histological features and can mimic several neoplasms with different treatment and behavior. Herein, we report the clinicopathological features of three cases of HNSFT. Case 1 was a 29-year-old female who presented with a nasal cavity mass measuring 3.5 cm. The patient underwent surgical excision. Microscopic examination revealed classic histological and immunohistochemical (IHC) features of SFT. Unusual histological features included epithelioid morphology, clear cells, and edematous change. She developed local recurrence after 11 months, which was also treated with surgery. Case 2 was a 55-year-old male who developed a 1-cm mass at the buccal mucosa. Surgical excision of the tumor was performed. The tumor was completely circumscribed microscopically. Characteristic histological and IHC features of SFT were identified. Unusual histological features observed were an adenomatous pattern, clear cells, and myxoid change. The patient was alive and disease-free at the 12-month follow-up. Case 3 was a 59-year-old female presenting with a medial canthus mass measuring 1.4 cm. The patient underwent surgical excision. Histological and IHC features observed were diagnostic for SFT. Unusual histological features identified were wavy nuclei and multinucleated stromal giant cells. The patient was alive and disease-free at the 124-month follow-up. Diagnosis of SFT can be challenging in unusual locations like the head and neck region. In addition, the histological spectrum of HNSFT is diverse. Therefore, knowledge about unusual histological features and classic IHC expression is essential for establishing correct diagnosis. Long-term follow-up is recommended because of the risk of recurrence in HNSFT.
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  • 文章类型: Journal Article
    孤立性纤维性肿瘤(SFT)是一种罕见的间充质肿瘤,病程缓慢,但转移潜力可变。自1991年以来,记录的颈部SFT病例不到50例。我们提出了一例罕见的颈项SFT表现,代表了SFT(血管外皮细胞瘤)的高细胞变异,在治疗方面面临挑战。患者接受切除并接受辅助放射。我们得出的结论是,尽管在处理软组织肿瘤时应考虑SFT的罕见诊断,并且必须进行多学科的术前计划,以避免并发症和复发。手术切除仍然是首选治疗方法,但长期跟进是必须的。
    Solitary fibrous tumor (SFT) is a rare mesenchymal tumor with an indolent course but variable metastatic potential. Less than 50 cases of neck SFTs have been documented since 1991. We present a case report of rare presentations of SFT of nape of neck typifying the hypercellular variant of SFT (hemangiopericytoma) with challenges in treatment. Patient underwent excision and was subjected to adjuvant radiation. We concluded that SFT though a rare diagnosis should be considered while dealing with soft tissue tumors and multi-disciplinary pre-operative planning is must to avoid complications and recurrence. Surgical excision remains treatment of choice, but long follow-up is must.
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  • 文章类型: Journal Article
    孤立性纤维瘤(SFT)通常被描述为由胸膜引起的病变。很少,已在咽旁间隙(PS)中进行了描述。本研究旨在报告PS中的2例SFT,并就此主题进行文献综述。两名患者在PS中接受SFT手术切除,被报道。关于PS的SFT的文献综述,也进行了。对2例患者进行分析。两名患者都接受了手术切除,其次是辅助放疗,对于由PS产生的SFT。术后病程顺利,两名患者术后均恢复良好。随访期间未确诊复发。PS的SFT是不常见的实体。手术切除是最常用的治疗方法,对于有复发危险因素或远处转移的患者,应考虑辅助放疗。
    The solitary fibrous tumor (SFT) is usually described as a lesion arising from the pleura. Rarely, it has been described in the parapharyngeal space (PS). This study aims to report two cases of SFT in the PS and to perform a literature review on this topic. Two patients undergoing surgical resection of a SFT in the PS, were reported. A literature review on SFT of the PS, was also performed. Two patients were analyzed. Both patients underwent surgical resection, followed by adjuvant radiotherapy, for SFT arising from the PS. The postoperative course was uneventful and both patients recovered well after the procedure. No recurrences were diagnosed during the followup. SFT of the PS is an infrequent entity. Surgical resection is the most used treatment, and adjuvant radiation should be considered in patients with recurrence risk factors or distant metastases.
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  • 文章类型: Journal Article
    UNASSIGNED: Preoperative imaging for some unusual lesions in the sellar region can pose challenges in establishing a definitive diagnosis, impacting treatment strategies.
    UNASSIGNED: This study is a retrospective analysis of eight cases involving unusual sellar region lesions, all treated with endoscopic endonasal transsphenoidal surgery (EETS). We present the clinical, endocrine, and radiological characteristics, along with the outcomes of these cases.
    UNASSIGNED: Among the eight cases, the lesions were identified as follows: Solitary fibrous tumor (SFT) in one case, Lymphocytic hypophysitis (LYH) in one case, Cavernous sinus hemangiomas (CSH) in one case, Ossifying fibroma (OF) in two cases; Sphenoid sinus mucocele (SSM) in one case, Pituitary abscess (PA) in two cases. All patients underwent successful EETS, and their diagnoses were confirmed through pathological examination. Postoperatively, all patients had uneventful recoveries without occurrences of diabetes insipidus or visual impairment.
    UNASSIGNED: Our study retrospectively analyzed eight unusual lesions of the sellar region. Some lesions exhibit specific imaging characteristics and clinical details that can aid in preoperative diagnosis and inform treatment strategies for these unusual sellar diseases.
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  • 文章类型: Journal Article
    孤立性纤维瘤(SFT)是一种罕见的软组织肿瘤,存在于身体的任何部位。选择的治疗方法是手术切除,尽管10%-30%的患者经历复发性疾病。已经研究了多种风险因素和风险分层系统以预测哪些患者有复发风险。本系统评价的主要目标是创建一个最新的风险因素和风险分层系统的系统概述,预测躯干和四肢内手术切除的SFT患者的复发。
    我们根据更新的Prisma系统评价指南(PRISMA-P)编写了评价。Pubmed,Embase,科克伦图书馆,截至2022年12月,对世卫组织国际试验注册平台和ClinicalTrials.gov进行了系统搜索。包括所有描述切除SFT后复发危险因素的英文研究。我们排除了中枢神经系统和耳鼻喉区域的SFT。
    确定了81项回顾性研究。不同的风险因素,包括年龄,症状,性别,切除边缘,解剖位置,有丝分裂指数,多态性,细胞过多,坏死,尺寸,去分化,CD-34表达式,Ki67指数和TP53表达,APAF1-失活,以叙述方式研究TERT启动子突变和NAB2::STAT6融合变体。我们发现高有丝分裂指数,Ki67指数和坏死的存在增加了手术切除SFT后复发的风险,而其他因素的预后价值差异较大.我们还总结了目前可用的不同风险分层系统,并发现八个不同的系统具有不同程度的将患者分层为低的能力,中度或高度复发风险。
    有丝分裂指数,坏死和Ki67指数是复发的最可靠危险因素。TERT启动子突变似乎是未来风险分层模型中一个有希望的组成部分。Demicco风险分层系统是最有效和广泛使用的,然而,由于随访时间较长,G评分模型可能看起来更优.
    CRD42023421358。
    UNASSIGNED: Solitary fibrous tumor (SFT) is a rare soft tissue tumor found at any site of the body. The treatment of choice is surgical resection, though 10%-30% of patients experience recurrent disease. Multiple risk factors and risk stratification systems have been investigated to predict which patients are at risk of recurrence. The main goal of this systematic review is to create an up-to-date systematic overview of risk factors and risk stratification systems predicting recurrence for patients with surgically resected SFT within torso and extremities.
    UNASSIGNED: We prepared the review following the updated Prisma guidelines for systematic reviews (PRISMA-P). Pubmed, Embase, Cochrane Library, WHO international trial registry platform and ClinicalTrials.gov were systematically searched up to December 2022. All English studies describing risk factors for recurrence after resected SFT were included. We excluded SFT in the central nervous system and the oto-rhino-laryngology region.
    UNASSIGNED: Eighty-one retrospective studies were identified. Different risk factors including age, symptoms, sex, resection margins, anatomic location, mitotic index, pleomorphism, hypercellularity, necrosis, size, dedifferentiation, CD-34 expression, Ki67 index and TP53-expression, APAF1-inactivation, TERT promoter mutation and NAB2::STAT6 fusion variants were investigated in a narrative manner. We found that high mitotic index, Ki67 index and presence of necrosis increased the risk of recurrence after surgically resected SFT, whereas other factors had more varying prognostic value. We also summarized the currently available different risk stratification systems, and found eight different systems with a varying degree of ability to stratify patients into low, intermediate or high recurrence risk.
    UNASSIGNED: Mitotic index, necrosis and Ki67 index are the most solid risk factors for recurrence. TERT promoter mutation seems a promising component in future risk stratification models. The Demicco risk stratification system is the most validated and widely used, however the G-score model may appear to be superior due to longer follow-up time.
    UNASSIGNED: CRD42023421358.
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  • 文章类型: Systematic Review
    背景:颅内孤立性纤维瘤(SFT),以前的血管外皮细胞瘤(HPCs),是罕见的,侵袭性硬脑膜间质瘤。虽然辅助放射治疗已被建议改善局部肿瘤控制(LTC),尤其是在次全切除后,术后立体定向放射外科(SRS)的作用和最佳SRS给药策略仍不明确.
    方法:PubMed,EMBASE,根据PRISMA指南对描述颅内SFT术后SRS的研究进行了系统搜索和WebofScience。搜索策略在作者的PROSPERO方案(CRD42023454258)中定义。
    结果:纳入15项研究,描述293例患者有476例颅内残留或复发的SFT接受术后SRS治疗。平均随访21-77个月,SRS后的LTC率为46.4-93%,平均SRS剂量为13.5-21.7Gy,平均最大剂量为27-39.6Gy,和平均等剂量在42.5-77%线。在单个肿瘤结果的汇总分析中,18.7%的SFT表现出完整的SRS响应,31.7%有部分反应,18.9%保持稳定(整体LTC率为69.3%),进步了30.7%。当研究按边缘剂量分层时,平均边缘剂量>15Gy显示LTC率改善(74.7%对65.7%)。
    结论:SRS是颅内SFT安全有效的治疗方法。在可测量的疾病背景下,我们汇总的数据提示,随着SRS边缘剂量的增加,LTC有改善的潜在剂量反应.我们对SFT的侵袭性生物学和耐受的辅助SRS参数的更好理解支持在颅内SFT的术后治疗范例中可能更早地使用SRS。
    BACKGROUND: Intracranial solitary fibrous tumors (SFTs), formerly hemangiopericytomas (HPCs), are rare, aggressive dural-based mesenchymal tumors. While adjuvant radiation therapy has been suggested to improve local tumor control (LTC), especially after subtotal resection, the role of postoperative stereotactic radiosurgery (SRS) and the optimal SRS dosing strategy remain poorly defined.
    METHODS: PubMed, EMBASE, and Web of Science were systematically searched according to PRISMA guidelines for studies describing postoperative SRS for intracranial SFTs. The search strategy was defined in the authors\' PROSPERO protocol (CRD42023454258).
    RESULTS: 15 studies were included describing 293 patients harboring 476 intracranial residual or recurrent SFTs treated with postoperative SRS. At a mean follow-up of 21-77 months, LTC rate after SRS was 46.4-93% with a mean margin SRS dose of 13.5-21.7 Gy, mean maximum dose of 27-39.6 Gy, and mean isodose at the 42.5-77% line. In pooled analysis of individual tumor outcomes, 18.7% of SFTs demonstrated a complete SRS response, 31.7% had a partial response, 18.9% remained stable (overall LTC rate of 69.3%), and 30.7% progressed. When studies were stratified by margin dose, a mean margin dose > 15 Gy showed an improvement in LTC rate (74.7% versus 65.7%).
    CONCLUSIONS: SRS is a safe and effective treatment for intracranial SFTs. In the setting of measurable disease, our pooled data suggests a potential dose response of improving LTC with increasing SRS margin dose. Our improved understanding of the aggressive biology of SFTs and the tolerated adjuvant SRS parameters supports potentially earlier use of SRS in the postoperative treatment paradigm for intracranial SFTs.
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