Solitary fibrous

  • 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: Journal Article
    血管外皮细胞瘤和孤立性纤维瘤是发病率较低的肿瘤。它们有局部复发和转移的趋势。WHO将这两种肿瘤整合到一个新实体中,但悬而未决的问题之一是证明手术加辅助放射治疗(RT)的有效性并标准化其使用。我们回顾了10年的数据。我们评估了病理和放射学特征。评估手术记录以确定肿瘤切除的特征和程度。我们比较了使用或不使用RT的患者的结果。平均随访时间为74.8个月,范围为12个月和210个月。人口包括3名男性(30%)和7名女性(70%)。最常见的位置是大脑凸度(30%),剩下的是颈椎和腰椎,骶骨,心室内,环形的,蝶骨脊和眶内。7例(70%)患者接受术后外照射放疗,标准为部分切除或WHOII和III组织学分级.2例患者在初次手术后12个月和19个月出现局部复发。1例患者接受了2次手术,另一个,4个手术平均无复发生存率为15.5个月。4例患者发现远处转移。10例患者中有3例死亡。5年总生存率为66%,平均总生存期为76个月。首次手术中安全完整的切除是最重要的预后因素。补充RT可能会有所帮助,即使在WHO低级别完全切除的情况下。
    Hemangiopericytoma and Solitary Fibrous Tumor are tumors with low incidence. They have a tendency to recur locally and to metastasize. The WHO integrated both tumors into a new entity but one of the pending issues is to demonstrate the effectiveness of surgery plus complementary radiotherapy (RT) and standardize the use of it. We reviewed the data from 10 years. We assessed pathologic and radiologic characteristics. The operation records were evaluated to determine the features and extent of tumor resection. We compared the outcomes in patients using or not RT. The mean follow-up was 74.8 months, with a range of 12 and 210 months. The population included 3 males (30%) and 7 females (70%). The most common location was brain convexity (30%), the remaining were cervical and lumbar spine, sacrum, intraventricular, torcular, sphenoid ridge and intraorbital. Postoperative external beam radiotherapy was delivered in 7 patients (70%), the criteria were a partial resection or WHO II and III histological grades. 2 patients developed local recurrences at 12 and 19 months after initial surgery. 1 patient underwent 2 surgeries, and the other, 4 surgeries. The mean recurrence free survival rate was 15.5 months. Distant metastases were found in 4 patients. 3 of the 10 patients died. Five-year overall survival rate was 66% and mean overall survival was 76 months. A safe and complete resection in the first surgery is the most important prognostic factor. Complementary RT can be helpful, even in cases of complete resection in WHO low-grade.
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  • 文章类型: Journal Article
    BACKGROUND: Solitary fibrous tumor of the pleura is a rare, usually benign, and slow-growing neoplasm. Complete surgical resection for giant tumor of the pleura is challenging because of poor exposure and a large blood supply. We report the case of a giant hypervascular fibrous tumor that filled nearly the entire left hemithorax and anterior mediastinum, and its preoperative management.
    METHODS: A 59-year-old woman presented to us with exertional dyspnea and chest pain. A chest radiograph showed the right hemithorax completely opaque and a mediastinal shift to the left hemithorax. A tomography scan of the thorax showed a giant mass that almost completely filled the right hemithorax and compressed the mediastinum to the left. Because of excessive bleeding during dissection, the operation was terminated after a biopsy specimen was obtained. The biopsy was diagnosed as a benign fibrous tumour. A thoracic computed tomography angiogram showed that the mass was supplied by multiple intercostal arteries as well as an aberrant artery that branches off the celiac trunk in the subdiaphragmatic region. Due to the many arteries that needed to be embolized, the final decision was to control the bleeding following resection by inducing total circulatory arrest with the help of cardiopulmonary bypass. The bleeding could not be controlled under cardiopulmonary bypass and the patient\'s death was confirmed.
    CONCLUSIONS: We report this case to emphasize the necessity of preoperative embolization; the use of cardiopulmonary bypass and total circulatory arrest is not a valid alternative method to control the bleeding.
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