extra-pleural

  • 文章类型: Journal Article
    孤立性纤维性肿瘤(SFT)属于一组不同的间充质肿瘤,最初由Klemper和Rabin于1931年在胸膜中描述。然而,它也可以是胸膜外来源。舌头是这个地区最常见的部位,会厌,喉部,甲状腺,外耳道,泪囊,舌下神经,腮腺,舌下腺,咽旁空间,鼻咽部,头皮,牙龈,眼眶和颞下窝以及鼻旁窦和鼻腔也可能受累。但是涉及鼻腔和鼻咽的SFT并不常见,占所有鼻部肿瘤的<0.1%。到目前为止,文献报道的鼻SFT只有40例。我们报告了从鼻腔延伸到蝶窦的血管外孤立性纤维瘤的病例,这种类型的更罕见的介绍。我们的病例报告是其中一种,强调需要对疾病的性质和管理进行进一步研究。
    Solitary fibrous tumors (SFTs) belonging to a distinct group of mesenchymal tumors, was originally described by Klemper and Rabin in the pleura in year 1931. However, it can also be extra-pleural in origin. With tongue being the most common site involved in this region, epiglottis, larynx, thyroid, external auditory canal, lacrimal sac, hypoglossal nerve, parotid gland, sublingual gland, Para pharyngeal space, nasopharynx, scalp, gingiva, orbit and infratemporal fossa as well as paranasal sinuses and nasal cavities can also get involved. But SFTs involving nasal cavities and nasopharynx are quite uncommon, accounting for < 0.1% of all Sino-nasal neoplasms. Until now there have only been 40 cases of nasal SFT reported in literature. We report the case of an extraserosa solitary fibrous tumor arising from the nasal cavity with extension to the sphenoid sinus, a much rarer presentation of its type. Our case report is one of its type, emphasizing the need conducting further studies on the nature and management of the disease.
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  • 文章类型: Case Reports
    孤立性纤维瘤(SFTs)是罕见的肿瘤,通常由胸膜引起。文献已经建立了多个胸膜外事件。CT扫描和MRI静脉造影作为早期发现和诊断的垫脚石,并评估疾病负担。手术切除大部分时间是治愈性的。SFT具有恶性潜能,可能复发或转移,因此长期随访对于诊断为该肿瘤的患者至关重要。我们讨论了一名47岁的男性患者的尿潴留,由大型盆腔肿瘤引起,经病理证实为孤立性纤维肿瘤。
    Solitary fibrous tumors (SFTs) are rare neoplasms commonly arising from the pleura. Multiple extra-pleura occurrences have been established by the literature. CT scan and MRI with IV contrast serve as the steppingstone for early detection and diagnosis, and to evaluate disease burden. Surgical resection is most of the time curative. SFTs carry a malignant potential and may recur or metastasize thus long-term follow-up is of utmost importance for patients diagnosed with this tumor. We discuss the case of a 47-year-old male patient who presented with urinary retention, caused by a large pelvic tumor pathologically proven to be a solitary fibrous tumor.
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  • 文章类型: Case Reports
    目的:眼眶原发性恶性孤立性纤维性肿瘤(SFT)是一种罕见的梭形细胞肿瘤,需要切除活检进行组织病理学诊断。我们提出了一个使用当代免疫组织化学染色的临床病例,世界卫生组织最新分类报告,并提供文献综述。
    方法:报告一例65岁男性右侧突出,有限的内收,上睑下垂,地球横向位移,脸颊上的花彩。神经成像显示2.2厘米,弥漫性增强的外异质质量。基于核异型性的整体肿瘤切除证实了SFT恶性肿瘤,细胞过多,和增加的有丝分裂活性(13个有丝分裂图/10个高功率场)。Ki-67在良性肿瘤中显示2%的核染色,在恶性对应物中显示10-15%的染色。免疫组织化学分析显示弥漫性Stat6阳性,CD34阳性,恶性部分部分缺乏染色,恶性部分的S-100阳性,而CAM5.2、Desmin、肌动蛋白,CD31和CD117。
    结论:免疫分析有助于诊断眼眶恶性孤立性纤维性肿瘤。完全切除肿瘤仍然是首选治疗方法。胸外SFT的行为是不可预测的,所有地方的SFT患者都需要小心,长期随访。
    OBJECTIVE: Primary malignant solitary fibrous tumor (SFT) of the orbit is a rare spindle cell neoplasm that requires excisional biopsy for histopathological diagnosis. We present a clinical case using contemporary immunohistochemical stains, report on the latest World Health Organization classification, and provide a review of the literature.
    METHODS: Report of a single case of a 65 year old male who presented with right-sided proptosis, limited adduction, ptosis, lateral globe displacement, and cheek festooning. Neuroimaging revealed a 2.2 cm, extraconal heterogeneous mass that diffusely enhanced.En-bloc tumor resection confirmed SFT malignancy based upon nuclear atypia, hypercellularity, and increased mitotic activity (13 mitotic figures/10 high powered fields). Ki-67 showed 2% nuclear staining in the benign tumor and 10-15% staining in the malignant counterpart. Immunohistochemical analysis revealed diffuse Stat6 positivity, CD 34 positivity with partial lack of staining within the malignant portion, S-100 positivity in the malignant portion, and overall negativity for CAM 5.2, desmin, actin, CD 31, and CD 117.
    CONCLUSIONS: Immunoprofiling is helpful to making the diagnosis of malignant solitary fibrous tumor of the orbit. Complete tumor resection continues to be the preferred treatment. The behavior of extrathoracic SFT is unpredictable, and patients with SFT in all locations require careful, long-term follow-up.
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  • 文章类型: Journal Article
    孤立性纤维瘤(SFT)是一种不寻常的梭形细胞肿瘤,通常发生在胸膜,但最近也有报道称起源于胸膜外。肾脏表现非常罕见。高达90%的肿瘤具有良性特征。使用成像技术很难将其与肾细胞癌区分开。可以通过做详细的病理检查来做出明确的诊断,其中包括免疫组织化学。我们正在报告一例肾脏的大型孤立性纤维瘤,该肿瘤发生在一名70岁的男性中。切除标本的组织学检查证实了诊断,通过揭示肿瘤细胞对CD34,bcl-2,波形蛋白的强烈阳性反应和对上皮膜抗原(EMA)的阴性反应,平滑肌肌动蛋白(SMA),S-100蛋白和Ki-67。患者心脏骤停,手术后第七天死亡。
    Solitary Fibrous Tumour (SFT) is an unusual spindle cell tumour that usually occurs in the pleura, but has recently also been reported to be extra-pleural in origin. A renal presentation is very rare. Upto 90% of the tumours have benign characteristics. It is difficult to differentiate it from renal cell carcinoma by using imaging techniques. A definitive diagnosis can be made by doing a detailed pathological examination, which includes immunohistochemistry. We are reporting a case of a large solitary fibrous tumour of the kidney which here occurred in a 70-years-old male. Histological examination of the resected specimen confirmed the diagnosis, by revealing strongly positive reactions of the neoplastic cells for CD34, bcl-2, vimentin and negativity for Epithelial Membrane Antigen (EMA), Smooth Muscle Actin (SMA), S-100 protein and Ki-67. The patient suffered a cardiac arrest and died on the seventh day after his surgery.
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