An intracranial malignant solitary fibrous tumor

Intracranial malignant solitary fibrous tumor (SFT) is very rare. It was identified in a 39‐year‐old female patient who underwent malignant transformation over 6 months. MRI revealed an 8 × 5 × 6 cm mass with heterogenous strong enhancement in the left occipital lobe. Histologic findings and immunophenotype (positive for CD34, bcl‐2 and vimentin, and negative for epithelial membrane antigen or S100 protein) of the primary tumor were typical of SFT. However, there was a focal area (<10% of tumor volume) showing hypercellularity, nuclear pleomorphism and increased Ki‐67 labeling index (LI) of 10% (average, 1%). At the second operation, the recurrent tumor revealed full‐blown histologic features of malignant SFT, such as infiltrative brain invasion, marked nuclear pleomorphism, frequent mitotic figures (15/10 high power fields), and high Ki‐67 LI (25%). The presence of atypical histologic finding or increased Ki‐67 LI in the typical SFT, although it is focal, needs to be mentioned in the diagnosis and also may require more aggressive surgical management.     

Primary anaplastic solitary fibrous tumor of the tentorium cerebelli

Meningeal solitary fibrous tumors (MSFT) have been described in about 80 patients as benign spindle-cell neoplasms, with few anaplastic variants. We report a 57-year-old male patient with a 4-month history of progressive headache caused by a primary anaplastic MSFT arising from the tentorium cerebelli. MRI revealed a tentorium-based tumor that extended into the occipital lobe superiorly and into the cerebellum inferiorly on the left. Following gross total resection of the tumor and postoperative radiotherapy, the patient experienced symptomatic improvement with no recurrence at the 12-month follow-up. The final tumor pathology was consistent with an anaplastic MSFT, with a Ki-67 proliferative index of 25%.     

Epithelioid solitary fibrous tumor of the central nervous system

Epithelioid solitary fibrous tumor (SFT) has recently been reported and is an extremely rare soft-tissue neoplasm. Herein we present an epithelioid SFT attached to the falx cerebri occurring in a Chinese woman. This patient underwent gross-total tumor resection at the age of 30 years and recurred 68 months following the initial total resection. Histologically, the initial lesion exhibited features of classic spindle cell SFT. In contrast, the recurrent tumor demonstrated exclusively epithelioid morphology with significant atypia. Both the original and recurrent lesions showed positivity for vimentin, CD34, Bcl-2, and CD99, whereas were negative for all the remaining antibodies. The epithelioid feature in SFT seems to be associated with a more aggressive clinical behavior in this case and more cases are awaited to verify this possibility. To the best of authors’ knowledge, the present case is the first published example of SFT with epithelioid feature in the central nervous system.     

Two patients with a solitary fibrous tumor of the thoracic spinal cord

We report two patients with thoracic spinal solitary fibrous tumor (SFT). This report includes a patient with the first secondary SFT arising in the central nervous system from a pleural origin to our knowledge. The diagnosis was confirmed by histological and immunohistochemical analysis. Both patients underwent gross total resection of their tumors and did not show signs of local recurrence. The patient with the secondary lesion later presented with visceral dissemination. We review the reports of spinal SFT and discuss the diagnosis and therapeutic management of this intriguing entity.     

Intramedullary solitary fibrous tumor of dorsal spinal cord

Solitary fibrous tumors (SFT) are rare neoplasms of mesenchymal origin involving soft tissues, mainly serosal sites; the spinal cord location is uncommon. We report a case of SFT occurring in the thoracic spinal cord, discussing histological, ultrastructural and molecular aspects. A 75‐year‐old woman with an MRI suggesting a dorsal intracanalar lesion was admitted to our institution. T5–T7 laminectomies were performed and an intramedullary tumor was discovered. The tumor arose within the spinal cord and was completely removed. Tumor samples were processed for histological, ultrastructural and molecular analysis (comparative genomic hybridization [CGH], methylation status of O6‐methylguanine–DNA methyltransferase [MGMT], p16, deleted in colorectal cancer [DCC] and death‐associated protein kinase 1 [DAPK1]). The histological examination demonstrated a proliferation of spindle‐shaped cells with a collagen‐matrix background. Immunohistochemical staining was positive for vimentin and CD34 and negative for S‐100 and epithelial membrane antigen. A histological diagnosis of SFT was made. The ultrastructural examination showed undifferentiated cells within a collagenous matrix and sparse extravascular basement membrane. CGH analysis revealed deletion of 9p21 and losses on 2q, 3p, 16q and 19q and gains on 7q; furthermore, no aberrant methylation pattern was found in the promoter region of MGMT, p16, DCC and DAPK1 genes. On the second‐year follow‐up, the patient was neurologically intact. The occurrence of SFT within the spinal cord parenchyma and its histological characteristics demonstrate that SFTs are not restricted to serosal surfaces. The course of spinal cord SFT is unknown and long‐term follow‐up is necessary. The histological, ultrastructural and molecular findings are important for the diagnosis and the authors provide a literature review of these aspects.     

Meningeal solitary fibrous tumor: report of a case and literature review

Solitary fibrous tumor is a rare neoplasm that most often involves the pleura. The increasing numbers of this neoplasm have also been reported to date in extrapleural sites. We report a case of a twenty-four-year-old female with right frontal mass. Histologically, the tumor composed of spindle cell proliferation. Tumor cells were found to be positive for CD34 and CD117 with immunohistochemical studies. Ten months follow-up was uneventful. Seventy seven cases of meningeal solitary fibrous tumor from the literature are analysed and pathological, immunohistochemical and clinical features are discussed. Solitary fibrous tumor has a slight female predominance, with a male to female ratio of 1:1.5. Age distribution is similar to meningioma ranging from 7-81 years. Approximately 23% of cases originate in the spine which is the most common meningeal location. Histopathologic examination shows uniform spindle cell proliferation with various amount of collagen. CD34-positivity usually allows discrimination from schwannomas, meningiomas and hemangiopericytomas. A differential diagnosis is important because most of the solitary fibrous tumors usually behave in a benign fashion. In this study, we also showed CD117 (Kit) expression in a case of meningeal SFT. CD117-positivity can be a good strategy for treatment in malignant and recurrent cases. Further investigations are necessary for therapeutic implication of CD117-positivity in SFT.     

颅内巨大孤立性纤维瘤一例报道及文献复习

患者 男性,48岁,无明显诱因出现右侧肢体无力伴头胀头晕1周,加重ld,恶心呕吐数次,呕吐物为内容物,量中等,于2013年1月2日人院.体检:意识模糊,言语含糊,双侧瞳孔等大等圆,对光反射迟钝,伸舌不合作,颈软,右侧上肢肌力0级,下肢肌力2级,左侧肢体肌力5级,双侧肌张力正常.     

鞍区恶性孤立性纤维瘤临床病理观察

目的探讨鞍区恶性孤立性纤维瘤(malignant solitary fibrous tumor,MSFT)的临床病理特点。方法回顾性分析1例鞍区MSFT病人的临床资料,并对肿瘤组织进行病理形态学观察及免疫组化检测。结果光镜发现:瘤细胞呈梭形及短梭形,细胞致密区与疏松区交替分布,但以细胞致密区为主,血管增多并明显胶原化。瘤细胞间含有粗细不等、形状不一的胶原纤维。部分区域细胞异型性明显,核分裂像>4/10 HPF。免疫组化结果显示:波形蛋白(vimentin)、CD34、Bcl-2及CD99阳性。结论鞍区的恶性孤立性纤维瘤极为罕见,确诊依赖病理学检查。     

CD34 and dural fibroblasts: the relationship to solitary fibrous tumor and meningioma

Intracranial solitary fibrous tumors (SFTs) are typically dural-based, CD34-positive neoplasms of uncertain histogenesis. We examined ten cases of meninges obtained at autopsy from patients with no history of neurological illness, head trauma, or neurosurgical intervention, and ten cases of typical meningiomas with attached dural margins not involved by tumor. All cases were immunostained with CD34. CD34 reactivity was noted in the long, thin delicate processes of dural fibroblasts preferentially located in the meningeal portion of the dura rather than the periosteal portion. No CD34 reactivity was identified in the arachnoid or pia mater, except in some endothelial cells. One supratentorial dural-based fibrous nodule and one SFT within the confines of the fourth ventricle showed strong and diffuse reactivity to CD34, bcl-2, and vimentin, and were negative for epithelial membrane antigen (EMA), S-100 protein, glial fibrillary acidic protein, smooth muscle actin, and desmin. We also describe a meningothelial meningioma within which a well circumscribed SFT-like nodule was embedded. The SFT-like nodule was strongly CD34 positive and EMA negative, and the meningioma was strongly EMA positive and CD34 negative. Fibroblasts of the dural border cell layer are attached to the underlying arachnoid, and their inclusion with arachnoidal stromal elements and pial-based tela choroidea during formation of choroid plexus interstitium may account for intraventricular SFTs. Our results suggest that SFTs and dural-based fibrous nodules derive from CD34-positive dural-based fibroblasts, and that CD34 reactivity in meningiomas may result from inclusion of dural fibroblasts within the neoplasm.     

Solitary fibrous tumor of the orbit: Morphological,cytogenetic and molecular features

Solitary fibrous tumor (SFT), a benign neoplasm arising in mesenchymal structures, was initially described in the pleura but subsequently has also been documented in other locations. It is uncommon in the orbit, where it closely resembles other benign spindle‐shaped mesenchymal tumors of this area such as schwannoma, meningioma or hemangiopericytoma. We present a case of orbital SFT in a 34‐year‐old woman. The radiological study showed the presence of an enhanced uptake lesion measuring 2 cm in major diameter. The histopathological evaluation revealed alternating cellular and hypocellular areas with spindle‐shaped cells. The cellular organization displayed a broad variety of irregular morphological patterns. The neoplastic cells were intensely positive for CD34 and vimentin, while S100, epithelial membrane antigen (EMA), Caldesmon, Calretinin and WT‐1 proved negative. The pericellular matrix exhibited strong positivity for CD44 and collagen IV. Scarce mitotic figures, a Ki‐67 nuclear labeling index of <5%, and focal expression of p53 were also observed. Measurement of DNA content revealed a DNA index of 1, indicating a diploid peak in 95% of the tumor cells. A normal 46,XX karyotype was present. No TP53 (exons 5–8) mutations or MDM2 and CDK4 amplifications were observed. No p14^ARF, p15^INK4B and p16^INK4A deletions or hypermethylation were observed in this benign tumor. Following surgical resection and radiotherapy, the patient showed no tumor relapse after one year of follow‐up.     

Meningeal solitary fibrous tumour in a child

Introduction Meningeal solitary fibrous tumour is a relatively recent pathological entity that has rarely been described in children. With radiological techniques, it cannot be distinguished from meningiomas, and the diagnosis has to be confirmed histologically.Case report We discuss the possible histogenesis of this tumour and the need for recognizing this lesion as a separate entity. We report the case of a 12-year-old boy who developed a meningeal solitary fibrous tumour; the main clinical symptoms were progressive headaches for a long period and recent transient hemiparesis.Conclusion This child presents an uneventful evolution without additional therapy 3.5 years after surgery.     

大脑镰旁脑膜瘤显微外科手术治疗

目的探讨大脑镰旁脑膜瘤的手术方法和技巧。方法对16例经手术治疗的大脑镰旁脑膜瘤的临床治疗进行回顾性分析。结果手术全切肿瘤13例,其中SimpsonI级切除9例,SimpsonlI级切除4例,其余3例SimpsonIII级切除。术后随访6—72月,2例复发,无手术死亡。结论大脑镰旁脑膜瘤手术治疗力争达到全切除,有效地控制术中出血、保护并妥善处理好重要回流静脉是确保手术成功的关键。     

Recurrent meningeal malignant tumor: Assessment of differences in the solitary fibrous tumor/hemangiopericytoma spectrum through a case report

Solitary fibrous tumors (SFTs) are neoplasms that grow from mesenchymal fusiform cells. In the central nervous system, meninges are the common origin of these neoplasms. Although literature reports mostly SFT as benign neoplasm, malignancy data have been described in recurrences or metastatic lesions. Definitive diagnosis includes immunohistochemical profiles assessing cellular positivity for CD34, vimentin, CD99 and Bcl-2. Recent studies have demonstrated NAB2-STAT6 gene fusion as a distinct molecular feature of SFT with overexpression of the fusion protein NAB2-STAT6 in nuclei of these cells. Since several years, pathologists have grouped SFT and hemangiopericytomas (HPC) as different phenotypes of the same entity although both neoplasms do not share numerous features. This article, based on a case of a recurrent malignant SFT, aims to emphasize differences in the SFT/HPC spectrum due to the diagnostic, therapeutic and prognostic implications.     

颅内孤立性纤维瘤侵犯颈静脉孔1例并文献复习

颅内孤立性纤维瘤(ISFT)临床上较为罕见,侵犯颈静脉孔的ISFT更少见。该文报道侵犯颈静脉孔的ISFT 1例,对其病理学、影像学特征、术后复发和转移相关研究进展进行文献复习。     

Malignant solitary fibrous tumor arising from the pineal region: case study and literature review

We report a case of malignant solitary fibrous tumor involving the pineal region in a 49‐year‐old woman. The patient presented with headache, slowly progressive weakness of the right lower extremities and upgaze palsy over the past year. Histologically, the tumor was composed of moderately hypercellular proliferated spindle cells with eosinophilic collagen bands. These cells were diffusely and strongly immunoreactive with CD34, CD99, and vimentin, but were negative with epithelial membrane antigen, S‐100 protein, Bcl‐2, smooth muscle actin, cytokeratin and glial fibrillary antigenic protein. MIB‐1 labeling indices and mitosis rates were 7.3 ± 1.8% and 5 per 10 high power fields, respectively. Ultrastructural examination revealed that the neoplastic cells had features of fibroblastic differentiation. Differential diagnoses included fibrous meningioma and hemangiopericytoma. The present case provides one unique example of a rare entity to the already diverse spectrum of the pineal region neoplasms encountered in neuropathology.     

Recurrence and increased proliferation rate of a solitary fibrous tumor in the central nervous system--case report and review of the literature

Meningeal solitary fibrous tumors (SFTs) were at first estimated as rare benign tumors which can be cured by total resection. To date, only 37 patients with intracranial SFTs have been reported. Therefore, the natural history of this tumor entity needs more enlightenment. The authors report a case of a 77-year-old female in whom a SFT with infiltration of the transversal sinus was subtotally resected. After a short time, interval tumor recurrence was seen, 2 years and 6 months later second surgery was performed. Immunohistologically, in both specimens typical features for SFT with positivity for CD34, vimentin and BCL-2 and negative for epithelial membrane antigen was seen. No signs for malignancy occurred in the second resection. Notably the MIB-1 index increased from 1 to 5%. In conclusion, consequent long-time follow-up for SFTs are necessary, especially after incomplete tumor resection.