solitary fibrous tumor

孤立性纤维瘤
  • 文章类型: Case Reports
    肺孤立性纤维瘤(SFT)是一种罕见的肿瘤,通常起源于肺胸膜。我们提供了一例无明显病史的57岁男性的病例报告,该男性在胸部计算机断层扫描扫描中偶然被诊断为肺实质的SFT。放射学成像显示肺左下叶有明确的肿块。活检和组织病理学检查证实了孤立性纤维瘤的诊断。该病例强调了在肺部肿块的鉴别诊断中考虑SFT的重要性,因为它的临床表现和放射学特征可以模仿更常见的肺部恶性肿瘤。
    Solitary fibrous tumor (SFT) of the lung is a rare neoplasm, usually originating from lung pleura. We present a case report of a 57-year-old male with no significant medical history who was incidentally diagnosed with an SFT of lung parenchyma on chest computed tomography scan. Radiological imaging revealed a well-defined mass in the left lower lobe of the lung. Biopsy and histopathological examination confirmed the diagnosis of solitary fibrous tumor. This case highlights the importance of considering SFT in the differential diagnosis of lung masses, as its clinical presentation and radiological features can mimic those of more common pulmonary malignancies.
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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
    简介-梭形细胞肿瘤是鳞状细胞癌的一种变体。其亚型之一是孤立性纤维性肿瘤。它在头颈部的发生非常罕见,在硬腭中也很少见。但如果发生了,根治性切除是唯一的选择,因为它具有恶性潜力,但覆盖如此大的中面部缺损在整形外科医生面前提出了挑战,因为它需要软组织覆盖和骨骼支持。病例报告-一名33岁男性就诊于我科,左侧面部肿胀累及前腭,上颌骨,鼻子,和上唇。在肿瘤外科团队的帮助下,肿瘤的广泛局部切除以及双侧基础设施上颌骨切除术,全鼻切除术,进行了上唇全切除术和全硬腭切除术。这在中部面部形成了大的缺损,上面覆盖着游离的股前外侧皮瓣。进行了活检,发现肿胀为硬腭的孤立性纤维瘤。所有边缘均无肿瘤。襟翼沉降良好。鼻孔用鼻支架固定。三个月后,将扩张器放置在患者的前额,用于将来的鼻重建。三个月后,使用扩张的前额皮瓣和肋软骨进行鼻重建。21天后进行皮瓣脱离和内陷。还完成了白卷创作。由于患者抱怨鼻塞,对皮瓣变薄进行了另一项辅助手术。6个月后,引入了血管化的游离腓骨骨移植物以重建上颌骨,以供将来进行牙科康复。患者正在定期随访,他对结果感到满意。讨论-涉及口周和鼻孔区域的面部缺损非常罕见,需要复合重建。在这种情况下,微血管游离皮瓣覆盖是不可替代的选择。可能必须进行多个阶段以进行进一步改进。结论-肿瘤切除术后的重建总是非常苛刻。通过适当的术前计划和熟练的执行,患者可以从功能上受益,审美和社会心理。
    Introduction- Spindle cell neoplasm is a variant of squamous cell carcinoma. One of its subtypes is solitary fibrous tumor. Its occurrence in head and neck is very rare and rarer in hard palate. But if occurs, radical excision is the only choice as it has malignant potential but coverage of such large mid face defects imposes a challenge in front of a Plastic Surgeon as it demands both soft tissue coverage and skeletal support. Report of the case- A 33 year male presented to our department with swelling of left side face involving the anterior palate, maxilla, nose, and upper lip. With the help of the surgical oncology team, wide local excision of the neoplasm along with bilateral infrastructure maxillectomy, total rhinectomy, total upper lip resection and total hard palatectomy was done. This created large defect in the mid face which was covered with free anterolateral thigh flap. Biopsy was done which revealed the swelling as a solitary fibrous tumor of hard palate. All the margins were free of tumour. The flap settled well. Nostrils were secured with nasal stents. After 3 months, an expander was placed in forehead of the patient for future nasal reconstruction. After 3 months, nasal reconstruction was done using expanded forehead flap and costal cartilage. After 21 days flap detachment and insetting was done. White roll creation was also done. One more secondary procedure was done for flap thinning as patient had complain of nasal obstruction. After 6 months vascularised free fibula bone graft was introduced to reconstruct maxilla for future dental rehabilitation. The patient is in regular follow up and he is satisfied with the results. Discussion- Mid face defects involving perioral and nasomaxillar areas are very uncommon and require composite reconstruction. In such cases, microvascular free flap coverage is an irreplaceable option. Multiple stages might have to be done for further refinement. Conclusion- Reconstruction after oncological resection is always very demanding. With proper preoperative planning and skilled execution, the patient can be benefited functionally, aesthetically and psychosocially.
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  • 文章类型: Journal Article
    孤立性纤维瘤(SFT)很少出现在甲状腺内,主要与上皮癌有关的器官。本病例报告探讨了一名70岁患者的临床叙述,该患者的甲状腺左叶有相当大的SFT,在甲状腺结节中不常见的诊断挑战。该报告深入研究了临床病史,放射学发现,病理评估,和治疗干预措施,为有限的甲状腺SFT文献做出了贡献。患者的超声检查显示大量甲状腺肿块导致气管和血管移位,分类为TIRADS3。细针穿刺提示间充质来源,促使进一步调查。对比增强计算机断层扫描描绘了一个清晰的病变,具有不同的增强,压缩周围的结构。组织病理学证实梭形细胞增殖,提示免疫组织化学显示CD34、STAT6和Bcl-2阳性,符合SFT特征。甲状腺SFT的稀有性提出了诊断挑战,必须依赖免疫组织化学才能与其他梭形细胞肿瘤准确区分。放射学调查,包括超声和磁共振成像,有助于术前规划。该案例强调了细致的病理检查的重要性,强调免疫组织化学在确认SFT诊断中的实用性。该报告增进了临床医生的理解,病理学家,和研究人员,指导提高诊断准确性,并为未来甲状腺SFT的发生量身定制的治疗策略。
    Solitary Fibrous Tumor (SFT) rarely manifests within the thyroid gland, an organ predominantly associated with epithelial carcinomas. This case report explores the clinical narrative of a 70-year-old patient presenting with a sizable SFT localized in the left lobe of the thyroid, posing diagnostic challenges uncommon in thyroid nodules. The report delves into the clinical history, radiological findings, pathological assessments, and therapeutic interventions, contributing to the limited literature on thyroidal SFTs. The patient\'s ultrasound revealed a substantial thyroid mass causing tracheal and vascular displacement, categorized as TIRADS 3. Fine needle aspiration indicated mesenchymal origin, prompting further investigation. Contrast-enhanced computed tomography depicted a well-defined lesion with varied enhancement, compressing surrounding structures. Histopathology confirmed a spindle cell proliferation, prompting immunohistochemistry revealing CD34, STAT6, and Bcl-2 positivity, aligning with SFT characteristics. The rarity of thyroidal SFTs poses diagnostic challenges, necessitating reliance on immunohistochemistry for accurate differentiation from other spindle cell neoplasms. Radiological investigations, including ultrasound and magnetic resonance imaging, contribute to preoperative planning. The case underscores the importance of meticulous pathological examination, emphasizing the utility of immunohistochemistry in confirming SFT diagnosis. The report enhances understanding among clinicians, pathologists, and researchers, guiding improved diagnostic accuracy and tailored treatment strategies for future occurrences of thyroidal SFTs.
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  • 文章类型: Journal Article
    舌肿瘤比较常见,绝大多数是上皮表型。虽然不常见,在这个解剖部位出现了多样化和独特的间充质肿瘤。为了增加我们对这些病变的了解,我们回顾了我们的经验,并描述了他们的临床病理特征。从2005-2021年的病理档案和其中一位作者的咨询文件中查询了所有舌头的MN。我们回顾了组织学切片和辅助研究,并从可用的医疗记录中获得了临床数据。确认了93例病例,他们组成了研究队列——据我们所知,这是最大的舌间充质肿瘤系列。48名患者为女性,45人是男性,平均年龄51岁(范围:1-94岁)。肿瘤包括43例(46.2%)血管瘤,14(15%)个颗粒细胞瘤,8(9%)脂肪瘤,4例(4.3%)神经鞘瘤,4例(4.3%)孤立性纤维瘤-根据风险分层标准,均具有较低的进展风险,2例(2.2%)淋巴管瘤,3(3.2%)卡波西肉瘤,2(2.2%)软骨瘤,2(2.2%)肌纤维瘤,1例(1.1%)孤立性局限性神经瘤,1(1.1%)神经鞘瘤,1(1.1%)神经纤维瘤,1(1.1%)外胚间充质软骨粘液样肿瘤,1例(1.1%)具有NOTCH2重排和TLL2突变的非典型血管球瘤,1(1.1%)梭形细胞横纹肌肉瘤,1(1.1%)多形性成纤维细胞肉瘤,1例(1.1%)恶性横纹肌样瘤,1(1.1%)平滑肌肉瘤,1(1.1%)血管肉瘤,1例(1.1%)肺泡软组织肉瘤。大多数患者接受了手术切除,1例(血管瘤)患者行栓塞治疗。关于后续行动,梭形细胞横纹肌肉瘤患者在手术部位出现术后麻木,随访17个月后无病.患有平滑肌肉瘤的患者在22个月时拒绝辅助放疗并发生肺部转移。患有肺泡软组织肉瘤的患者在诊断时已转移到肺,并接受了辅助化疗。其余患者无局部或远处复发。舌头的MNs通常是良性的,其特征是内皮,脂肪细胞,或schwannian分化。治疗的主要方法是手术切除,切除程度由肿瘤类型决定。辅助治疗保留用于高级别肉瘤。
    Neoplasms of the tongue are relatively common, and the vast majority are epithelial in phenotype. Although uncommon, a diverse and distinctive array of mesenchymal neoplasms arises in this anatomic site. To increase our understanding of these lesions, we reviewed our experience of MNs of the tongue and described their clinicopathologic features. The pathology archives from 2005 to 2021 and the consultation files of one of the authors were queried for all MNs of the tongue. We reviewed the histologic slides and ancillary studies and obtained clinical data from the available medical records. Ninety-three cases were identified, and they form the study cohort - to our knowledge, this is the largest series of mesenchymal neoplasms of the tongue. Forty-eight patients were female, and forty-five were male, with a mean age of 51 years (range: 1-94 years). The tumors included 43 (46.2%) hemangiomas, 14 (15%) granular cell tumors, 8 (9%) lipomas, 4 (4.3%) schwannomas, 4 (4.3%) solitary fibrous tumors - all with low risk of progression based on risk stratification criteria, 2 (2.2%) lymphangiomas, 3 (3.2%) Kaposi sarcomas, 2 (2.2%) chondromas, 2 (2.2%) myofibromas, 1 (1.1%) solitary circumscribed neuroma, 1 (1.1%) perineurioma, 1 (1.1%) neurofibroma, 1 (1.1%) ectomesenchymal chondromyxoid tumor, 1 (1.1%) atypical glomus tumor with a NOTCH2 rearrangement and TLL2 mutation, 1 (1.1%) spindle cell rhabdomyosarcoma, 1 (1.1%) pleomorphic fibroblastic sarcoma, 1 (1.1%) malignant rhabdoid tumor, 1 (1.1%) leiomyosarcoma, 1 (1.1%) angiosarcoma, and 1 (1.1%) alveolar soft part sarcoma. Most of the patients underwent surgical excision, and 1 patient (with hemangioma) underwent embolization. On follow-up, the patient with spindle cell rhabdomyosarcoma developed postoperative numbness at the surgical site and was disease-free through 17 months of follow-up. The patient with leiomyosarcoma declined adjuvant radiation and developed metastasis to the lung at 22 months. The patient with alveolar soft part sarcoma had metastases to the lung at the time of diagnosis and received adjuvant chemotherapy. The remaining patients had no local or distant recurrence. MNs of the tongue are usually benign and characterized by either endothelial, adipocytic, or schwannian differentiation. The mainstay of treatment is surgical excision with the extent of excision determined by tumor type. Adjuvant therapy is reserved for high-grade sarcomas.
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  • 文章类型: English Abstract
    Objective:To summarize and analyze the clinical manifestations, diagnosis and management and prognostic features of solitary fibrous tumor(SFT) in nasal cavity, sinus and skull base. Methods:The clinical data of 12 patients with STF from nasal cavity, sinus and cranial base admitted to the Affiliated Hospital of Qingdao University from April 2014 to January 2022 were retrospectively analyzed, including 4 patients admitted to the department of Otolaryngology head and neck surgery and 8 patients admitted to the department of skull base surgery The clinical characteristics, diagnosis, management and prognosis were analyzed. Results:Twelve patients were included in this research, including 7 males and 5 females. All patients received surgical treatment, and 4 patients also received postoperative adjuvant chemoradiotherapy. After follow-up for 12-60 months, 4 patients with adjuvant radiotherapy and chemotherapy had a good prognosis, and among 8 patients who did not receive radiotherapy and chemotherapy, 6 patients had good prognosis and 2 patients showed relapse. Four patients with a history of recurrence of SFT after surgery were admitted to our hospital for surgical treatment, in which 1 patient had relapse after surgery, and none had metastasis. Nasal cavity and sinus to skull base SFT is rare. The most effective treatment for this disease is surgical resection, and postoperative adjuvant chemoradiation and long-term follow-up can achieve a better prognosis. En bloc resection is the key to treatment success.
    目的:总结分析鼻腔鼻窦颅底孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床表现、诊疗方法及预后特点。 方法:回顾性分析青岛大学附属医院耳鼻咽喉头颈外科和颅底外科2014年4月-2022年1月收治的12例鼻腔鼻窦颅底SFT患者的临床资料,其中耳鼻咽喉头颈外科收治患者4例、颅底外科收治患者8例,分析其临床特点、诊疗经过及预后情况。患者均行手术治疗,其中4例术后辅助放化疗,8例未行放化疗。 结果:术后随访12~60个月,4例术后辅助放化疗的患者均预后良好;8例未行放化疗患者中6例预后良好,另2例复发;4例患者既往有SFT手术史,复发后收治于青岛大学附属医院治疗,其中1例术后复发。所有患者均无转移。 结论:鼻腔鼻窦颅底SFT较为罕见,对于该病最有效的治疗手段为手术切除,术后辅助放化疗及术后长期随访可以实现较好的预后。综合治疗中完整性手术切除肿瘤是治疗的关键。.
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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: Journal Article
    目的:成纤维细胞活化蛋白(FAP)是一种丝氨酸整合膜蛋白酶,其表达已在各种癌症类型中得到证实。胸膜孤立性纤维瘤(SFTP)是罕见的间充质成纤维细胞肿瘤。我们介绍了一例18F标记的FAP抑制剂([18F]FAPI-74)PET成像及其与组织学FAP表达的相关性,并回顾了我们机构的SFTP系列与FAP表达程度的关系。
    方法:这项回顾性研究包括2011年3月至2022年12月在我们研究所接受手术的13例患者。其中一名患者还接受了[18F]FAPI-74PET成像。我们使用免疫组织化学染色和H评分半定量评估SFTP中的FAP表达。
    结果:13例患者中有9例为男性,年龄中位数为64岁(范围,28-79岁)。中位肿瘤大小为6.6cm(1.1,16cm)。在病理发现中,13例中12例Ki67的表达水平为1-5%。此外,所有患者均有FAP表达,中位数H评分为160(范围,10-280)。13例患者中有2例FAP表达的H评分较低(均为10),13名患者中有两名患者的发病率较高(240和280)。在FAP表达的H评分为180的患者中,[18F]FAPI-74PET的SUVmax值为3.57。
    结论:SFTP在不同患者中不同程度地表达FAP,1例患者的[18F]FAPI-74PET结果反映了FAP在肿瘤组织中的表达。
    OBJECTIVE: Fibroblast activation protein (FAP) is a serine integral membrane protease, the expression of which has been confirmed in various cancer types. Solitary fibrous tumors of the pleura (SFTP) are rare mesenchymal fibroblastic neoplasms. We present a case of 18F-labeled FAP inhibitor ([18F]FAPI-74) PET imaging and its correlation with histological FAP expression and review an SFTP series at our institution in relation to the extent of FAP expression.
    METHODS: This retrospective study included 13 patients who underwent surgery between March 2011 and December 2022 at our institute. One of the patients also underwent [18F]FAPI-74 PET imaging. We semi-quantitatively evaluated FAP expression in SFTPs using immunohistochemical staining and H-scores.
    RESULTS: Nine of the 13 patients were male, with a median age of 64 years (range, 28-79 years). The median tumor size was 6.6 cm (1.1, 16 cm). In the pathological findings, expression levels of Ki67 were 1-5% in 12 of 13 cases. Furthermore, FAP expression was observed in all patients, and the median H-score was 160 (range, 10-280). The H-score of FAP expression in two of the 13 patients was low (10 in both), and that in two of the 13 patients was high (240 and 280). The SUVmax value of [18F]FAPI-74 PET was 3.57 in a patient in whom the H-score of FAP expression was 180.
    CONCLUSIONS: SFTPs expressed FAP to varying degrees in different patients and the [18F]FAPI-74 PET results in one patient reflected FAP expression in the tumor tissue.
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  • 文章类型: Letter
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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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