A case report of an intracaval extrathoracic solitary fibrous tumour

Solitary fibrous tumours are infrequent neoplasms based in the pleura that are predominantly benign with malignant pathology and behaviour described in 10–36% of cases. Extrathoracic solitary fibrous tumours (ESFTs) have been considered separately to their intrathoracic counterparts and comprise a third of all solitary fibrous tumours. The extrathoracic location was identified as an adverse prognostic factor for local recurrence but not for metastatic disease. So far, there have not been any reports of solitary fibrous tumours demonstrating caval infiltration. We present a case of a benign ESFT infiltrating into the perirenal inferior vena cava. Together with extrauterine leiomyomas, ESFTs should also be considered as a differential diagnosis for the rare benign lesions invading the inferior vena cava.     

Clinicopathological analysis of a case with mesenteric solitary Castleman's disease: diagnostic value of radiological findings

We report a case with rare solitary mesenteric Castleman's disease (CD). A 45-year-old woman complaining of nausea was presented. A round-shaped, smooth margin, and hypoechoic mass was seen on screening abdominal ultrasonography. Computed tomography showed a markedly enhanced tumor anterior to the left iliopsoas muscle. Selective jejunal arteriography revealed an extreme hypervascularity without vascular invasion. These results retrospectively seem to differ from those of malignant lymphoma or sarcoma. The tumor was surgically resected, and hyaline-vascular type CD was pathologically diagnosed. We postulate that these radiological findings might suggest hyaline-vascular type CD as one of the differential diagnoses in similar cases, although more clinical data should be evaluated.     

Extra-thoracic,extra-meningeal solitary fibrous tumours - A case series and service review

BackgroundSolitary fibrous tumours (SFT) are a type of mesenchymal tumour. Whilst the majority of cases follow an indolent course a significant proportion of patients suffer metastases or disease recurrence post-surgical excision. Due to the unpredictable clinical course follow up duration and intensity remains contentious.AimsWe aimed to determine current outcomes of management of this tumour, apply and assess current risk recurrence models to determine if our standard of care could be improved upon.Methods and patientsA prospective database of patients treated at a regional musculoskeletal oncology service was assessed. Only extra-pleural, extra-meningeal SFTs were included in the study. Surgical outcome and post-operative investigations were scrutinised and the Pasquali and Demicco recurrence risk models were applied and assessed.ResultsFrom 2009 to 2019 12 patients were identified, 8 female and 4 males. Their age at diagnosis ranged from 21 to 76 years. 11 patients underwent surgery with curative intent and no patient suffered disease progression or recurrence, with a mean follow up time of 41 months. One patient presented with metastatic disease and was managed palliatively.ConclusionsFollowing this review of our case series and utilising risk recurrence models published in the literature we have changed our follow up protocol. In new cases of SFT the Pasquali prognostic model, with the addition of the presence or absence of necrosis, will be utilised. If a patient has benign features on initial biopsy we propose to not perform staging. Furthermore, if biopsy and final pathology results remain concordant, with no concerning features, and the patient has undergone complete excision reduced intensity follow up could be considered.Level of evidence Level IV, retrospective case series.     

颅内孤立性纤维瘤的显微外科治疗

目的：提高颅内孤立性纤维瘤疾病的诊治水平。方法总结2008年至2013年手术后经病理组织学证实的22例颅内孤立性纤维瘤,结合其术前MRI等影像学检查,术后免疫组化结果和肿瘤标志物,分析其预后。结果17例行肿瘤全切,5例因术中肿瘤血供丰富行肿瘤次全切除术,术后病理免疫组化肿瘤标志物CD34,VIM及BCL-2为阳性证实为孤立性纤维瘤。结论颅内孤立性纤维瘤诊断最终依据病理结果,全切除术后一般可以达到良好的预后。     

Clinical behavior of solitary fibrous tumors of the pleura.

BACKGROUND: Solitary fibrous tumors of the pleura are rare and present unpredictable clinical behavior. METHODS: Between 1981 and 1998, 11 solitary fibrous tumors of the pleura were resected in 10 patients at the University Hospital of Geneva. Their clinical behavior and outcome were reviewed. RESULTS: Seven tumors arose from the visceral pleura, and three arose from the parietal pleura. Tumors arising from the parietal pleura were revealed to be more difficult to resect than those from the visceral pleura because of their size and adhesion to the chest wall requiring extrapleural resection. Eight tumors showed benign features, whereas two showed distinct features of malignity. One additional patient presented marked pleomorphism that could represent an intermediate form before frank malignity. Four tumors had been followed expectantly for 2 to 10 years before surgery. Although three enlarged rapidly, no signs of malignity were observed on histological examination. All patients are alive, from 2 months to 14 years after surgery (mean 55 months). In one case, however, a malignant tumor recurred 6 years after resection of a benign variant. CONCLUSIONS: Although histologically benign, solitary fibrous tumors of the pleura may enlarge rapidly and occasionally transform into malignant variants after several years. Therefore, complete surgical resection and long-term follow-up is recommended for all patients.     

肾孤立性纤维瘤1例报告并文献复习

目的 分析肾孤立性纤维瘤的临床病理特征及诊治方式.方法 回顾性分析我院诊治的1例肾孤立性纤维瘤的病理特征、临床表现、影像学检查、治疗方式和术后随访状况.结果 肾孤立性纤维瘤是肾脏较罕见的一种间叶性肿瘤,良性居多,少数为恶性.病理特征多为VIM(+)、CD34(+)、BcL-2(+)、CD99(+).影像学检查显示为类圆形、包膜完整的肿物,与周围组织界限清楚,CT增强后呈现不同程度欠均匀强化.本例患者行机器人辅助腹腔镜下肾部分切除术,术后病理诊断为肾孤立性纤维瘤(中间性),术后随访3月余,未见肿瘤复发、进展和转移.结论 肾孤立性纤维瘤属临床上较罕见的肿瘤,确诊主要通过病理诊断,治疗上首选手术切除肿瘤.     

下肢孤立性纤维性肿瘤影像学表现 (附2例报告及文献复习)

目的分析下肢孤立性纤维性肿瘤（SFT）的影像学表现。方法报告2例经病理证实的下肢SFT的影像学表现,并复习相关文献。2例患者均接受MR检查,1例接受X线检查。结果2例病变均主要位于大腿股二头肌内,MR多表现为边界清楚的椭圆形或类圆形肿块,T1WI呈等低或等信号,T2WI信号混杂,可表现为高、稍高和低信号;增强扫描多呈不均匀强化,肿瘤实质强化明显。X线平片1例未见骨质破坏。结论下肢SFT影像学表现有一定的特点,MR是其最佳检查方法。     

Malignant solitary fibrous tumor of the pleura; report of a case

A 68-year-old female was admitted to our hospital for further examination of abnormal shadow on chest X-ray. Needle biopsy could not establish pathological diagnosis. Three years later, chest computed tomography (CT) revealed the tumor was enlarged. We suspected it was a malignant tumor, and resected by video-assisted thoracoscopy. The tumor occurred from the right middle lobe, and intraoperative diagnosis was malignant tumor. We added middle lobectomy. Histological examination revealed that tumor was malignant solitary fibrous tumor.     

脊髓髓内孤立性纤维性肿瘤1例报道

正孤立性纤维性肿瘤(solitary fibrous tumors,SFT)是一种临床罕见的来源于间叶组织的梭形细胞肿瘤,1931年Klemperer和Rabin首次将其描述为一种来源于间叶细胞的纤维增殖性肿瘤~([1])。其主要发生在脏层胸膜,也可发生在全身各处~([2、3])。多数表现为良性,一般无明显临床症状~([4])。近期我院收治了1例脊髓髓内SFT,报告如下。患者女,62岁,因"左下肢无力11月余"于2016年     

肠系膜纤维瘤、孤立性纤维性肿瘤和胃肠道间质瘤的免疫组织化学观察

目的：探讨肠系膜纤维瘤（MF）、孤立性纤维性肿瘤（SFT）和胃肠道间质瘤（GIST）的免疫组织化学鉴别诊断。方法：运用组织芯片结合免疫组织化学技术对3种肿瘤进行CD117、CD34、β-catenin、S-100、Desmin、Bcl-2和Dog-1的免疫组织化学分析。结果：75%的MF为β-catenin阳性表达,而13例GIST和7例SFT均为阴性表达;85%的GIST为CD117阳性表达,92%的GIST为Dog-1阳性表达,25%的MF为Dog-1阳性表达,8例MF均为CD117阴性表达,7例SFT则CD117和Dog-1均为阴性表达;71%的SFT表达CD34和Bcl-2,而23%的GIST表达为CD34阳性。结论：组合应用CD117（或Dog-1）、CD34和β-catenin并结合HE染色切片的形态特点可以有效地鉴别深部MF、SFT和GIST。配合应用S-100和Desmin,可以进一步排除肌源性和外周神经源性肿瘤。     

The myxoid tumors of somatic soft tissues

Tumors and tumorous conditions of superficial and deep soft tissues may owe their naked-eye appearance and much of their bulk to the accumulation of mucopolysaccharide material or, on rare occasions, to edema. The so-called myxoid tumors may be chondroblastic, fibroblastic, lipoblastic, myoblastic, or neurogenic. Benign and malignant tumors may be encountered. Naked-eye and microscopic appearances are described. Pseudotumors such as bursae, ganglia, cutaneous mucinoses, fasciitis, and myxoid cysts may enter the differential diagnosis. The value of accurate documentation is stressed. Histochemistry can make a limited contribution to diagnosis. The entities described have been examined with regard to alcian blue staining with and without pretreatment with hyaluronidase. Alcian blue staining in the presence of increasing concentrations of magnesium chloride has been investigated and found to be of value in the identification of tumors of chondroblastic origin.     

Trans-sacral resection of a solitary fibrous tumor in the pelvis: Report of a case

Solitary fibrous tumors (SFTs) develop most commonly in the pleura, although they have occasionally been reported to arise in the pelvic cavity. We report a case of an SFT presenting as a painless nodule in the pelvis of a 56-year-old woman. Histologically, the tumor was composed of spindle-shaped cells arranged without pattern, with short and narrow fascicles and interspersed bundles of thick collagen, and numerous blood vessels with a focally hemangiopericytoma-like appearance. Immunohistochemically, the tumor cells strongly expressed vimentin, CD34, and bcl-2. The tumor was excised via a trans-sacral approach, without preoperative transcatheter embolization, and the patient remains well more than 2 years after her operation. To our knowledge, this is the first case of an SFT in the pelvis, which was excised completely via a trans-sacral approach.     

前列腺孤立性纤维性肿瘤一例报告

孤立性纤维性肿瘤是一种罕见的起源于间充质梭形细胞的肿瘤,发生于前列腺者很少见。本院收治1例,年龄30岁,以排尿困难就诊,行经尿道前列腺钬激光剜除术,术后病理诊断为前列腺孤立性纤维性肿瘤,术后患者排尿症状得到明显改善。     

Unusual presentations of cerebral solitary fibrous tumors: report of four cases

OBJECTIVE AND IMPORTANCE: Four rare cases of central nervous system solitary fibrous tumors (C-SoFTs) are described. This tumor has not previously been reported in children or in deep cortical structures. Three of these tumors occurred in the posterior fossa. Only four cases in the posterior fossa have been described previously. Nine years after its debulking from the posterior fossa, one tumor disseminated to the spine, lung, and liver. Only one such aggressive C-SoFT has been described previously. CLINICAL PRESENTATION: A 7-year-old child had had 1 year of right-sided weakness at presentation. Magnetic resonance imaging scans revealed a left basal ganglia lesion. A 49-year-old woman and a 30-year-old man presented after experiencing headaches for months. Magnetic resonance imaging scans in these patients revealed a tumor in the fourth ventricle and right jugular foramen, respectively. A 55-year-old man had spinal, liver, and lung dissemination of a previous posterior fossa tumor at presentation. INTERVENTION AND TECHNIQUE: All four patients underwent craniotomy for resection or subtotal removal of the tumor. Intraoperative observations noted solid well-encapsulated tumors. Immunohistochemistry confirmed C-SoFTs in all four cases. CONCLUSION: C-SoFTs are rare central nervous system, typically dural-based, tumors. They frequently are overlooked in the differential diagnosis of solid central nervous system tumors. Our findings suggest that these tumors can occur at any age and in most locations, regardless of proximity to the meninges (basal ganglia and ventricle), suggesting that the cells of origin are not meningothelial, but rather the mesenchyme of the cerebral vasculature. T1- and T2-weighted magnetic resonance imaging was notable for areas of hypointensity and of hyperintensity best described as patchy or a ying-yang appearance in all cases. Delayed extracranial metastasis may be noted.     

A resected case of solitary fibrous tumor of the pleura

A 45-year-old female with solitary fibrous tumor (SFT) of the pleura was reported. Chest X-ray and CT scan on admission showed a large tumor in the right thoracic cavity. A preoperative needle biopsy was performed. The microscopic appearance of biopsied specimen revealed many spindle cells. And an immunohistochemical study was positive for CD 34 and negative for keratin, epithelial membrane antigen, alpha-smooth muscle actin, S-100 protein. So SFT was strongly suspected and an operation was performed. A tumor arised from the visceral pleura of right middle lobe and was pedunculated. The tumor was 13 x 10 x 7.5 cm in size. An immunohistochemical study of the tumor was positive for CD 34, and negative for SMA, ki-67. From these immunohistochemical stainings and microscopic findings, the tumor was diagnosed as SFT. An immunohistochemical study of the tumor seems to be very useful for the diagnosis of SFT.