Familial solitary fibrous tumor of the pleura: a case report

This report describes the occurrence of solitary fibrous tumors of the pleura in a mother and her daughter. No other occurrence of this rare tumor in members of the same family has ever been reported.     

A solitary fibrous tumor originating from the liver surface

The case was a 30-year-old woman found to have a 7-cm mass around the pancreatic head during a company health examination. No abnormal values other than anemia were observed in the physical findings, blood exam, and various tumor markers at the initial visit. An abdominal computed tomography (CT) revealed a tumor, 6.5 cm in diameter, accompanying a hypervascular cystic lesion in contact with the dorsolateral side of the descending portion of the duodenum and showed a dense stain in the early phase. Abdominal magnetic resonance imaging (MRI) demonstrated a lesion of isosignal intensity in a T1-weighted image and of slightly high signal intensity in a T2-weighted image. Endoscopy identified no mucosal lesion and endoscopic ultrasonography (EUS) showed an unclear border with the liver. The laparoscopic findings revealed a multilocular, elastic tumor that was prone to bleeding within the abdominal cavity. The tumor was resected immediately, as it was in contact with multiple organs but continuous with the Glisson’s capsule in the posterior portion of the liver. The tumor was positive for CD34/Bcl-2 and negative for S100 protein/c-kit/EMA, therefore a diagnosis of solitary fibrous tumor (SFT) was made. The postoperative course has been favorable. The patient was discharged on day 10 and her course is currently being monitored on an outpatient basis. SFTs are relatively uncommon as extrathoracic lesions, and those originating from the liver surface are extremely rare.     

Emergency operation of a patient with spontaneous rupture and massive hemorrhage of pleural solitary fibrous tumor

Solitary fibrous tumor (SFT) is a rare pleural disease with asymptomatic clinical course. We report a case of a patient with spontaneous rupture and massive hemorrhage of pleural SFT, which was treated by emergency operation.     

Obstructive jaundice caused by secondary pancreatic tumor from malignant solitary fibrous tumor of pleura： A case report

A 77-year-old man on systemic chemotherapy against postoperative bilateral multiple lung metastases of malignant solitary fibrous tumor of the pleura suffered from pruritus and jaundice. Blood examination showed elevated levels of hepatobiliary enzymes. Abdominal computed tomography showed a tumor with peripheral enhancement in the pancreatic head, accompanied with the dilatation of intra- and extra-hepatic bile ducts. He was diagnosed as having obstructive jaundice caused by a pancreatic head tumor. The pancreatic head tumor was presumably diagnosed as the metastasis of malignant solitary fibrous tumor of the pleura, because the findings on the pancreatic head tumor on abdominal CT were similar to those on the primary lung lesion of malignant solitary fibrous tumor of the pleura. The pancreatic tumor grew rapidly after the implantation of metallic stent in the inferior part of the common bile duct. The patient died of lymphangitis carcinomatosa of the lungs. Autopsy revealed a tumor that spread from the pancreatic head to the hepatic hilum. Microscopically, spindle-shaped cells exhibiting nuclear atypicality or division together with collagen deposition were observed. Immunohistochemically the pancreatic head tumor cells were negative for staining ofα-smooth muscle actin (α-SMA) or CD117, but positive for vimentin, CD34 and CD99. These findings are consistent with those on malignant solitary fibrous tumor of the pleura. We report the first case of obstructive jaundice caused by a secondary pancreatic tumor from malignant solitary fibrous tumor of the pleura.     

Solitary fibrous tumor in the thigh: review of the literature

Solitary fibrous tumors (SFT) of extremities, especially the thighs are very rare. Despite SFTs are generally benign, well-circumscribed soft tissue tumors new cases should be presented and followed up carefully to monitor their biological behavior. In general for tumor classification a biopsy is state of the art. Histological including immunohistochemical patterns for SFTs are defined. MRI and ultrasound are not sufficient for differential diagnosis. Once property identified and defined by size and location, resection with intact tumor capsule may result in full recovery of the patient. Reviewing the literature there are no validated reasons for a wider resection. The current patient was a 41-year-old male. Four years after an arthroscopy of the left knee the patient has been suffering an ongoing swelling of the lateral thigh. Because MRI scan data suggested a synovial sarcoma a biopsy was performed. The tumor was classified as a benign SFT. The diagnosis based on histological findings and the presence of the positive immunohistochemical markers Vimentin, CD34, and CD99. The complete tumor resection with intact capsule was achieved in a final operation. Clinical and in MRI after 54-month outcome period there were no local recurrences.     

Intracranial solitary fibrous tumor mimicking meningioma: A case report

Rationale:Solitary fibrous tumor is a rare mesenchymal tumor. This case report describes the diagnosis and treatment of this tumor.Patient concerns:A 31-year-old patient presented with epileptic seizure and headache 1 day prior to the visit and showed transient right limb hemiplegia for 6 hours that was resolved after intravenous infusion of mannitol.Diagnoses:Based on imaging, the provisional diagnosis was meningioma. Postsurgical histopathological diagnosis confirmed solitary fibrous tumor.Interventions:The lesion was totally excised. The patient improved remarkably after the operation, without any signs of associated limb movement disorder. No epileptic seizure was observed or reported after the operation.Outcomes:Postoperation computed tomography (CT) scans showed no obvious residual tumor. The patient was followed up every 3 months for a total of 1 year following the operation, during which time the patient did not complain of headache or seizure.Lessons:The manifestation of solitary fibrous tumor (SFT) through imaging methods has certain specific findings,butimmunohistochemistry is still very important for confirming the diagnosis.     

Solitary fibrous tumor of the liver expressing CD34 and vimentin： A case report

A case of a successfully treated solitary fibrous tumor （SFT） of the liver is reported. An 82-year-old female presented with left upper abdominal discomfort, a firm mass on palpation, and imaging studies revealed a large tumor, 15 cm in diameter, arising from the left lobe of the liver. A formal left hepatectomy was performed. Microscopic evaluation showed spindle and fibroblast-like cells within the collagenous stroma. Immunohistochemistry disclosed diffuse CD34 and positive vimentin, supporting the diagnosis of a benign SFT. The patient remained well 21 months after surgery. SFT of the liver is a very rare neoplasm of mesenchymal origin. In most cases it is a benign lesion, although some may have malignant histological features and recur locally or metastasize. With less than 30 reported cases in the literature, little can be said regarding its natural history or the benefitsof adjuvant radiochemotherapy. Complete surgical resection remains the cornerstone of its treatment.     

Solitary fibrous tumor of the pleura: Ultrasonographic imaging findings of 3 cases

Solitary fibrous tumor (SFT) of the pleura is a rare tumor of mesenchymal origin. Although radiographic findings of thoracic computed tomography and magnetic resonance imaging in the evaluation of SFTs of the pleura have been documented, the value of ultrasonography is uncertain. We presented the ultrasonographic findings of 3 pathologically proven cases of SFTs arising from the visceral pleura.In all the cases, thoracic ultrasonography demonstrated homogeneous, hypoechoic, hemicycle, extrapulmonary lesions, which showed respiratory movement with the adjacent lung, consistent with pedunculated tumors.Preoperative thoracic ultrasonography could be useful in the evaluation of patients with pleural tumors, especially SFTs.     

Malignant solitary fibrous tumor involving the liver

Solitary fibrous tumors are predominantly benign and are most commonly found in the thoracic cavity and pleura; while reports exist in the literature of malignant solitary fibrous tumors and those located in extrathoracic organs, these cases are considered extremely rare. Herein, a case is reported of a malignant solitary fibrous tumor involving the liver that was diagnosed and treated in a 62-year-old woman. The patient presented with complaints of upper abdominal pain and unintentional weight loss. Computed tomography scan of the abdomen revealed a remarkably large mass, measuring 15 cm × 10 cm × 20 cm, which appeared to be unrelated to any particular organ. The intraoperative finding of a wide communication with the left liver suggested hepatic origin, and served as an indicator for tumor resection via left hemihepatectomy. The diagnosis of solitary fibrous tumor and its malignant nature was confirmed by histological and immunohistochemical examination of the resected tissues. Hepatic solitary fibrous tumor is very rare, and surgery remains the mainstay of treatment. Due to limited reports of such tumors in the literature, little can be said about the benefit of adjuvant therapy and prognosis for the rare cases with malignant histological findings.     

Pancreatic metastasis from a solitary fibrous tumor of the kidney: A rare cause of acute recurrent pancreatitis

Solitary fibrous tumors are unusual spindle cell neoplasms that uncommonly originate from the kidney. We report a case of a 43-year old male who presented with acute recurrent pancreatitis secondary to a mass in the head of the pancreas. Endoscopic ultrasound with fine needle aspiration (EUS-FNA) was performed. Cytology revealed solitary fibrous tumor of the kidney. This is the first reported case of solitary fibrous tumor metastasizing to the pancreas and presenting as acute recurrent pancreatitis.     

盆腔巨大孤立性纤维性肿瘤一例

孤立性纤维性肿瘤（solitary fibrous tumor,SFT）是临床中少见的一种梭形细胞肿瘤,发生在盆腔的SFT更是十分罕见。因此,目前SFT的诊治现状仍存有很多问题和争议。现本文结合一例罕见的盆腔SFT的诊疗过程,对SFT的发病特点、诊治方案以及预后等问题进行分析讨论。     

胸膜孤立性纤维性肿瘤10例临床病理观察及文献复习

目的探讨胸膜孤立性纤维性肿瘤(SFT)的临床病理特征,提高对其临床及病理改变的认识。方法对10例胸膜SFT的临床资料和组织学形态进行回顾性分析,并采用免疫组织化学染色(Envision法)研究其形态及免疫表型特点。结果 10例患者中,男性6例,女性4例,发病年龄47~76岁,平均57.5岁。肿瘤分别位于脏层胸膜(4例),壁层胸膜(4例),叶间裂(2例),患者多因咳嗽,胸痛,胸膜腔肿块,肺部肿块就诊。影像学多显示:占位性病变和软组织团块影,其中2例显示有胸腔积液。病理组织学显示:肿瘤界限清晰,由交替性分布的细胞丰富区及稀疏区组成,部分可有粘液变。免疫组织化学显示:梭形细胞表达Bcl-2、CD34和vimentin。结论 SFT是一种较为少见的间叶性梭形细胞肿瘤,好发于胸膜。其确诊主要依赖于病理组织形态学及免疫组织化学染色。完整的手术切除是临床治疗SFT的必要手段,但少数SFT生长活跃,具有侵袭性,需长期随访。     

胸部孤立性纤维瘤的MSCT诊断分析

目的探讨胸部孤立性纤维瘤的多排螺旋CT(MSCT)影像表现,以提高对该疾病的认识和诊断。方法回顾性分析31例经手术、病理证实的胸部孤立性纤维瘤患者的MSCT表现及临床资料,4例行肺CT平扫,8例行肺CT一次性增强检查,19例行肺CT平扫+增强检查;结合后处理重建图像分析胸部孤立性纤维瘤的发病部位、形态、大小、密度、边缘、增强特点及与周围组织的关系。结果 31例胸部孤立性纤维瘤患者女性21例,男性10例,位于右侧胸腔16例,左侧胸腔13例,纵膈1例,肺内多发1例。27例病灶边界清晰,呈类圆形或长椭圆形,4例边界不清,呈大片状不规则形软组织密度块影,伴有胸腔积液及邻近肋骨破坏征象;增强扫描2例动脉期未见明显强化,延迟期轻度强化;11例动脉期呈中度不均匀强化,静脉期强化程度减低;14例动脉期轻中度不均匀强化,延迟期强化较动脉期更明显。31例患者良性27例,恶性4例。结论胸部孤立性纤维瘤的MSCT表现有一定的特征,大多呈边界清楚,血供丰富、呈中度以上不均匀强化的软组织密度结节或块影,MSCT可以清晰地显示病变特点,及时进行手术切除,可提高患者预后。     

Thoracic malignant solitary fibrous tumors: A population-based study of survival

Introduction

This study characterizes the overall survival (OS) and cause specific survival (CSS) of patients with thoracic malignant solitary fibrous tumors.

Methods

Eighty-two patients with malignant solitary fibrous tumors of the lung, pleura or mediastinum, diagnosed from 2001-2007, were retrospectively analyzed using the population-based Surveillance, Epidemiology, and End Results database.

Results

Among 77 patients with available staging information, 42% (n=32) had localized disease, 31% (n=24) had regional disease extension (without nodal involvement) and 27% had regional-nodal (n=2) or distant (n=19) metastases. Cancer-directed surgery was performed in 85%; radiation was performed in 16%. The 1-year, 5-year and median OS were 87%, 49% and 4.6 years respectively. The 1-year, 5-year and median CSS were 89%, 61% and 5.7 years respectively. Less advanced stage and undergoing cancer-directed surgery were favorable prognostic factors. For localized, regional and distant stage the median OS was: not reached at 6.3 years, 4.4 years and 2.0 years respectively (P=0.021); the median CSS was not reached at 6.3 years, 5.0 years and 2.4 years (P=0.068). For patients undergoing versus not undergoing surgery, the median OS was 4.9 vs 0.9 years (P=0.053) and median CSS was 5.7 vs 0.9 years (P=0.011). Tumor size was not significant.

Conclusions

From a population-based analysis of patients with thoracic malignant solitary fibrous tumors, stage and cancer-directed surgery had the greatest impact on OS and CSS. While being amenable to surgery likely reflects more indolent disease and/or better performance status and cardiopulmonary function, the significantly favorable impact of surgery also likely reflects a therapeutic benefit.     

Giant solitary fibrous tumor of the pleura treated by video-assisted surgery: a case report

Solitary fibrous tumors of the pleura are rare, with unpredictable clinical manifestations. By the time of diagnosis, the size and consistency of the tumor usually preclude the use of minimally invasive surgical techniques. We present a case of a 34-year-old male with a solitary fibrous tumor of the visceral pleura masquerading as a mediastinal tumor. The tumor was removed by video-assisted thoracic surgery.     

Lipomatous hemangiopericytoma of the stomach: a case report and a review of literature

Lipomatous hemangiopericytomas (LHPCs) are rare soft-tissue tumors that are histologically characterized by hemangiopericytomatous vasculature and the presence of mature adipocytes. We present the clinicopathological features of a case of gastric LHPC in a 56-year-old female, along with a literature review. Endoscopy and endoscopic ultrasound showed a submucosal tumor 0.8 cm across in the greatest dimension in the lesser curvature side of the gastric antrum. Grossly, the well-defined mass had a solid and tan-white cut surface admixed with myxoid regions and yellowish areas. Histological examination revealed a submucosal well-circumscribed lesion composed of cellular nodules with the classic appearance of an hemangiopericytoma admixed with clusters and lobules of mature adipocytes. The ill-defined tumor cells had weakly eosinophilic cytoplasm and contained spindled nuclei with occasional small nucleoli. Nuclei atypia and mitoses were absent, and no cellular atypia, necrosis or vascular invasion was observed. Immunohistochemistry showed that the tumor cells were diffusely positive for CD34, CD99, and vimentin and were focally reactive for bcl-2. This is the first known report of an LHPC in the stomach. The patient was followed for 12 mo without any evidence of metastasis or recurrence.     

胸膜孤立性纤维性肿瘤的临床病理特征和外科治疗

目的探讨胸膜孤立性纤维性肿瘤（SFTP）的临床病理特征和外科治疗效果。方法对11例外科切除的SFTP患者进行临床、影像、病理学比较分析，并进行免疫组织化学检测及术后随访。结果11例中男6例，女5例，发病年龄为35—73岁（平均53．7岁）。主要临床表现为咳嗽、胸痛等局部压迫症状，5例伴胸腔积液。3例电视胸腔镜（VATS）下切除肿块，2例VATS辅助小切口开胸切除肿块，6例开胸切除肿块。肺楔形切除8例，中下叶切除、下叶切除和胸壁肿块切除各1例。肿块位于脏胸膜9例，脏胸膜下和壁胸膜各1例。病理形态学主要表现为梭形瘤细胞弥漫分布，间质中可见丰富粗大的胶原纤维和厚壁的血管；恶性肿瘤表现为细胞密度高、细胞核异型性、坏死和出现病理性核分裂。免疫组化显示梭形瘤细胞表达波形蛋白（100％，11／11）、CD34（100％，11／11）和CD99（63．6％，7／11）及bel-2（63．6％，7／11），不表达CD31。病理诊断SFTP良性9例，富于细胞型和恶性各1例。术后并发多器官衰竭死亡1例。术后住院天数平均11d。随访10例，随访时间12—48个月。均未见复发和转移。结论X线胸片和CT表现为胸膜单个结节或巨大阴影时应考虑SFTP的可能；伴有胸腔积液时易误诊；确诊依赖于组织病理学和免疫组织化学检查；VATS或者VATS辅助小切口开胸病灶切除对于直径〈5cem的肿块是一种很好的手术方式。     

胸膜弥漫性黏液性恶性纤维组织细胞瘤一例并文献复习

目的探讨胸膜弥漫性黏液性恶性纤维组织细胞瘤（MFH）的临床和病理特点。方法分析我院收治的1例胸膜弥漫性黏液性MFH患者的临床和病理资料，并分析国内外文献报道的7例胸膜MFH病例。结果患者为男性，65岁，表现为咳嗽、胸痛、气促伴大量血性胸腔积液。胸部CT示右侧脏壁层胸膜弥漫性肿块影。开胸探查见脏壁层胸膜表面弥漫性灰白色半透明肿块，直径为1～8cm。光镜下可见黏液性基质中瘤细胞弥漫分布，呈梭形和多形性。波形蛋白、CD68、溶菌酶免疫组化染色瘤细胞呈阳性反应，细胞角蛋白、上皮膜抗原、结蛋白呈阴性反应。术后24d，因上腔静脉梗阻、循环衰竭死亡。与文献报道的非弥漫性胸膜MFH相比，本例为胸膜弥漫性多灶性MFH，起病急，病情进展快。结论胸膜弥漫性黏液性MFH是非常罕见的胸膜原发性肿瘤，恶性程度高，预后极差。