Intracranial Solitary Fibrous Tumor Research Articles

Intracranial solitary fibrous tumor

Intracranial solitary fibrous tumor

Unusual Presentations of Intracranial Solitary Fibrous Tumor with Malignant Transformation

Background: Intracranial solitary fibrous tumor was rare. However, most intracranial solitary fibrous tumors are benign, cerebral solitary fibrous tumor with malignant transformation was even more unusual. Case present: Herein, we presented a 55 year-old-male who was diagnosed intracranial solitary fibrous tumor five years ago. Partial tumor excision was performed via the pterional craniotomy. Unfortunately, one year later, cerebral solitary fibrous tumor with recurrence on latest operation was diagnosed via follow-up Magnetic Resonance imaging. Reopen the craniotomy site, subtotal removal of tumor and stereotactic radiation surgery were performed. However, four years later, a recurrent tumor was seen via follow-up computed topographic (CT) scans of brain with contrast. A third craniotomy with subtotal removal of tumor was performed. Pathological examination revealed solitary fibrous tumor with malignant transformation. Postoperative CyberKnife stereotactic radiosurgery was performed. One year after resection and CyberKnife stereotactic radiosurgery, a follow-up brain MR images showed tumor regression. Conclusion: Most of intracranial solitary fibrous tumors were benign lesions; however, physician should always keep in mind that malignant transformation may be found in intracranial solitary fibrous tumor.

Mulitmodality management of rare solitary fibrous tumor can be associated with extended survival.

Background:Little is known on the long-term course of patients treated for intracranial solitary fibrous tumors (iSFT). We therefore retrospectively reviewed the charts of our patients who underwent Gamma Knife radiosurgery (GKRS) for iSFT at Klinik Im Park in Zurich and who were treated by one of the authors. Between 1994 and 2009, two patients underwent GKRS for iSFT at Klinik Im Park.Case Description:One patient underwent altogether five radiosurgical treatments and two craniotomies for iSFT and its local recurrences. The other patient underwent two craniotomies and one radiosurgical treatment for iSFT. Both patients maintained a Karnofsky performance score 100 during follow-up and both were long-term survivors with a follow-up of 9 and 17 years, respectively.Conclusion:A close follow-up of patients with iSFT and repeat radiosurgery or surgery when indicated seems to lead to a favorable long-term outcome.

Treatment of Intracranial Solitary Fibrous Tumors With Gamma Knife Radiosurgery: Report of Two Cases and Review of Literature

Gamma knife radiosurgery (GKRS) as a treatment option has not been described in the management of typical intracranial solitary fibrous tumors. After presenting with visual decline, case A underwent a bioccipital craniotomy during which 90% of tumor was thought to have been resected. She unfortunately required re-resection 56 months later for recurrence when she again presented with progressive visual decline, altered mental status, and headaches. A subtotal resection was performed, because there was extensive tumor involvement of the torcula, straight sinus, and bilateral transverse sinuses. She was subsequently referred for GKRS. Although neurologically intact, with the exception of an upper extremity tremor, case B had undergone 7 surgeries for a posterior fossa tumor over the several decades preceding GKRS. The tumors targeted with GKRS were found on serial MRI scans and were thought to be asymptomatic at the time of treatment. At 7 and 14 months after GKRS, case A experienced tumor shrinkage, which remained stable 20 months after treatment. Effective local tumor control was seen in case B with tumor shrinkage at 3, 8, and 13 months after treatment. However, repeat GKRS was required for case B, because an out-of-field recurrence was found 15 months after the initial GKRS. Based on this report and available information in the literature, radiosurgery appears to be a reasonable approach for patients with recurrent or residual intracranial solitary fibrous tumors.

Solitary fibrous tumour extending both extracranially and intracranially

A solitary fibrous tumour (SFT) is a rare neoplasm of mesenchymal origin that occurs typically in the pleura, but can also occur several other locations.1e3 Nevertheless, SFTs occurring at the skull base and extending both extracranially and intracranially are very rare, with only two cases reported thus far.4,5 In this report, we described an atypical malignant SFT with many unusual features: rare location, unique growth pattern, lymph nodes metastasis, and atypical immunophenotype. Another unusual feature of this lesion was its high sensitivity to radiotherapy and chemotherapy of the postsurgical residual lesion. Finally, this is the first report that demonstrates a complete response of an intracranial SFT to radiotherapy and chemotherapy.

Dedifferentiation and progression of an intracranial solitary fibrous tumor: Autopsy case of a Japanese woman with a history of radiation therapy of the head during infancy

Solitary fibrous tumor (SFT) is usually an indolent neoplasm with a low rate of local recurrence and metastasis. Although dedifferentiation of low-grade sarcoma is well documented, the concept of dedifferentiated SFT was not recognized until recently. A case of intracranial SFT with seven recurrences within 5 years, showing progression and dedifferentiation during the course of disease, is reported here. A 51-year-old woman with a history of irradiation during infancy presented with a SFT in the right posterior fossa. Because of the close proximity to the brain stem, the tumor could not be removed completely. The tumor recurred 12, 16, and 28 months after the initial operation. With the repeated recurrences, cellularity, mitotic count, and Ki-67 (MIB-1) index increased gradually. The histology suddenly changed at the fourth recurrence, which occurred 16 months after postoperative radiation therapy for the third recurrence. The tumor revealed a fibrosarcoma-like appearance with necrosis and markedly increased mitotic activity. The tumor further recurred 50, 52, and 55 months after the initial operation with the same fibrosarcoma-like histology. The patient died of uncontrolled tumor 58 months after the initial operation. In this case radiation may have played some role in the tumorigenesis, progression, and dedifferentiation of the SFT.

Solitary fibrous tumors of the central nervous system: report of five cases with unusual clinicopathological and outcome patterns

This is a retrospective study of 11 patients harboring a solitary fibrous tumor (SFT) of the central nervous system (CNS), with special emphasis on unusual clinicopathological and outcomes patterns. Between 2000 and 2008, 11 patients harboring CNS SFTs were treated at our institution. Patient charts were retrospectively reviewed and tumor location, clinical presentation, imaging characteristics, extent of resection, dural origin, pathological features, adjuvant treatment, and follow-up data were collected, focusing on five atypical cases (four intracranial and one within the spine). One intracranial SFT arose from the sella turcica and relapsed threefold during the 6 years following partial removal. Disease progressed as successive isolated local recurrences treated by subsequent surgical interventions and gamma-knife radiosurgery. The MiB-1 labeling index analysis showed a steady increase in these sequential recurrences (ranging from less than 3% up to 6%) without obvious malignant transformation. The second SFT occurred in the cerebellopontine angle and exhibited a high MiB-1 index (10%) without noticeable features of malignancy. It relapsed twice during the 5 years following gross total resection without demonstrating a more aggressive histological pattern. The third SFT arose from the cerebellar tentorium, widely invaded the lateral sinus and adjacent bone, had a low MiB-1 index, and has not recurred within the 2 years after incomplete resection. The two remaining SFTs presented with unusual clinicoradiological features. We described a extremely rare case of intraventricular SFT, and a case of extradural SFT of the thoracic spine (T8-T9) radiologically consistent with a schwannoma. Immunohistochemistry confirmed that all tumors were SFTs. These atypical presentations gave us the opportunity to provide further information about the variability of the clinicoradiological patterns and natural histological course of CNS SFTs.

Intracranial solitary fibrous tumor: Imaging findings

Objective To study the neuroimaging features of intracranial solitary fibrous tumors (ISFTs). Materials and methods Retrospective study of neuroimaging features of 9 consecutive histopathologically proven ISFT cases. Location, size, shape, density, signal intensity and gadolinium uptake were studied at CT and MRI. Data collected from diffusion-weighted imaging (DWI) (3 patients), perfusion imaging and MR spectroscopy (2 patients), and DSA (4 patients) were also analyzed. Results The tumors most frequently arose from the intracranial meninges (7/9), while the other lesions were intraventricular. Tumor size ranged from 2.5 to 10 cm (mean = 6.6 cm). They presented multilobular shape in 6/9 patients. Most ISFTs were heterogeneous (7/9) with areas of low T2 signal intensity that strongly enhanced after gadolinium administration (6/8). Erosion of the skull was present in about half of the cases (4/9). Components with decreased apparent diffusion coefficient were seen in 2/3 ISFTs on DWI. Spectroscopy revealed elevated peaks of choline and myo-inositol. MR perfusion showed features of hyperperfusion. Conclusion ISFT should be considered in cases of extra-axial, supratentorial, heterogeneous, hypervascular tumor. Areas of low T2 signal intensity that strongly enhance after gadolinium injection are suggestive of this diagnosis. Restricted diffusion and elevated peak of myo-inositol may be additional valuable features.

Bifrontal solitary fibrous tumor of the meninges

Background:We report the case of a bifrontal solitary fibrous tumor (SFT) arising from the meninges. The points of interest in this case report are the particular imaging appearance, the immunohistochemical findings and the surgical features.Case Description:A 53-year-old Caucasian male presented with a 1-year history of behavioral changes, attention disorders and anterograde memory disorders. Magnetic resonance imaging revealed a bifrontal heterogeneous lesion attached to the anterior falx cerebri with a prominent multicompartmental cystic part. The patient underwent craniotomy for a sub-total resection of the tumor. At surgery, the multicystic component was highly vascularized and encased the anterior cerebral arteries. Neuropathological findings were consistent with a solitary fibrous tumor. Despite the absence of malignant features, there was a focal expression of p53.Conclusion:SFT is a pathological entity with specific immunohistochemical features; it has frequently been misdiagnosed in the past. The multicystic imaging appearance of this SFT and the particular p53 immunohistochemical staining are features that should be added to the growing data on intracranial SFTs. The surgical features described (high vascularization and partial vessel encasement) may help improve surgical planning.

Malignant Solitary Fibrous Tumor

Malignant solitary fibrous tumor (MSFT) is a rare neoplasm. Three cases of MSFT with unusual features, including 1 pleural and 2 extrapleural, are reported. A 64-year-old woman with a large right thoracic MSFT and episodes of severe hypoglycemia experienced resolution of her hypoglycemia immediately after resection of the MSFT. A 27-year-old woman with primary retroperitoneal MSFT had pulmonary metastases 10 months after resection of the primary tumor. A 54-year-old man with an intracranial solitary fibrous tumor suffered from multiple pulmonary metastases and local recurrence 21 and 28 months after resection of the primary tumor, respectively. All 3 cases of solitary fibrous tumor displayed malignant features. The tumor cells in each case were positive for CD34 and Bcl-2, but negative for cytokeratin, smooth muscle actin, S-100, and c-kit. In addition, the tumor cells in the case with concomitant hypoglycemia were strongly positive for insulin-like growth factor-II. The histopathologic diagnostic criteria for MSFT, the differential diagnosis with other spindle cell tumors, and the mechanism of MSFT-derived hypoglycemia via insulin-like growth factor-II are discussed.

Solitary fibrous tumor

Intracranial solitary fibrous tumors (SFT) are typically dural based, CD34-positive neoplasms of mesenchymal origin. Since they were first described in 1996 at the meninges, fewer than 100 SFT had been reported in both cranial and spinal compartments of the central nervous system. SFT can resemble other spindle cell tumors both radiologically and histopathologically, and differentiation can be best achieved through viewing their ultrastructure and using immunohistochemical techniques. In this report, we present four patients with SFT. Upon diagnosing two patients with SFT located in the cerebellopontine angle and parasagittal areas, we reviewed our pathological files and found two more patients; one having a parasagittal tumor and the other having a convexity tumor, that had been diagnosed with hemangiopericytoma. These tumours proved to be SFT after an immunohistochemical re-examination.

A reevaluation of the primary diagnosis of hemangiopericytoma and the clinical importance of differential diagnosis from solitary fibrous tumor of the central nervous system

Objectives Hemangiopericytomas (HPCs) are rare neoplasms with relatively high rates of recurrence and extracranial metastasis. Though the differential diagnoses from angiomatous meningiomas and from solitary fibrous tumors (SFTs) are both important, the latter diagnosis is somewhat more important in light of the benign prognosis of SFTs and the difficulties in distinguishing SFTs from HPCs. Newly developed immunohistochemical methods reveal differences in the specific immunohistochemical features of HPCs and SFTs. To elucidate whether SFTs have been misdiagnosed as HPCs in the past, our group used recent immunohistochemical methods to re-evaluate tissues that had been originally diagnosed as HPCs. We also compared the clinical features of these cases. Patients and methods Thirteen sequential cases of HPC diagnosed in Kanazawa University Hospital and Kumamoto University Hospital between 1970 and 2006 were retrospectively analyzed by immunohistochemical staining for CD34, Bcl-2, epithelial membrane antigen (EMA), vimentin, and S100 protein, and by measurement of the MIB-1 labeling index (LI). The cases were then re-evaluated and newly diagnosed based on the results of the immunohistochemical stainings. The clinical course of each case was also evaluated. Results Four of the 13 cases were newly diagnosed as SFTs and eight were reconfirmed as HPCs, based on the immunohistochemical studies for CD34, Bcl-2, and reticulin staining. One case was newly diagnosed as meningioma on the basis of a strong EMA positivity. The MIB-1 LI was less than 1% in 12 of the cases. In two cases, one case of HPC and the other of meningioma, the MIB-1 LI was relatively high, 8% and 4% respectively. All eight of the HPCs recurred, and 5 of the HPC patients died of the disease. Only one case of the SFTs recurred. Conclusion Our study suggests that a relatively high percentage of the tumors diagnosed as HPCs in the past may have in fact been intracranial SFTs. Immunohistochemical examinations of CD34, Bcl-2, and reticulin stains are keys for the differential diagnosis. Given that SFTs have a considerably better prognosis than HPCs, it is important to carry out meticulous immunohistochemical examinations for the primary diagnosis.

Intracranial solitary fibrous tumors: imaging findings in 6 consecutive patients.

Intracranial solitary fibrous tumors (ISFTs) are rare mesenchymal neoplasms originating in the meninges. The aim of this study was to describe the CT, MR imaging, and angiographic features of the solitary fibrous tumor and to identify imaging characteristics. We retrospectively reviewed CT, MR, and angiographic findings in 6 cases of ISFT. We evaluated the size, shape, and location of the tumor; the internal content and margin of the lesion; the pattern of enhancement; and the change of the adjacent structures. Density on noncontrast CT scans, signal intensity on MR images, and angiographic features were also documented. Each lesion appeared as a discrete extra-axial mass (size, 3-7 cm; mean, 5 cm). Five lesions were entirely solid, and 1 had peritumoral cyst. All 5 of the noncontrast CT scans showed hyperattenuated masses, and the tumors exhibited marked heterogeneous enhancement. No lesion contained calcification, and 2 cases showed bone invasions. On the MR images, 4 lesions showed mixed signal intensity on T2-weighted imaging. All of the lesions revealed marked heterogeneous enhancement. All of the tumors had thickening of the meninges adjacent to the tumor. Angiography showed delayed tumor blushing in all, and 3 of them had dysplastic dilation of the tumor vessels. Although there are no pathognomonic imaging findings, some imaging features, such as the "black-and-white mixed" pattern on T2-weighted images and marked heterogeneous enhancement, might be helpful in the diagnosis of intracranial solitary fibrous tumor.

Recurrent intracranial solitary fibrous tumor initially diagnosed as hemangiopericytoma

We describe a case of an intracranial solitary fibrous tumor that recurred three times consecutively in an 11-year period. A 72-year-old man presented with a headache and gait disturbance. Magnetic resonance imaging (MRI) revealed a dumbbell tumor at the left tentorium. The tumor was removed but recurred. The first diagnosis was hemangiopericytoma, but all specimens showed a "patternless pattern" and few reticulin fibers, which features were not compatible with hemangiopericytoma. All tumors showed immunoreactivity for CD34 and bcl-2. These results point to a solitary fibrous tumor (SFT) and not to hemangiopericytoma. We present here a hypercellular spindle-cell tumor that was very similar to hemangiopericytoma but is better diagnosed as SFT.

Giant solitary fibrous tumour of the meninges: MR-pathological correlation

The authors present the clinical, MR imaging and pathological features of the largest intracranial solitary fibrous tumour of the meninges ever reported in the literature. This well-circumscribed dural-based tumour strikingly demonstrated two different solid components. The first one demonstrated a suggestive T2 hypointensity that strongly enhanced after gadolinium administration while the other showed more classic homogeneous T2 hyperintensity and mild enhancement. These two components were also identifiable on the pathological examination, allowing an interesting imaging-histological correlation. Differential diagnoses of this rare extra-axial lesion are discussed.

A Highly Vascular Intracranial Solitary Fibrous Tumor Treated with Radiotherapy and Toremifene: Case Report

A case of an unusual intracranial solitary fibrous tumor with features of high vascularity and resultant difficulty at resection is presented. The use of surgery, radiotherapy, and toremifene has been successful with no recurrence after 18 months. A 33-year-old male patient presented with a 1-year history of loss of vision on the left side and a 3-month history of headache and lethargy. Magnetic resonance imaging showed a lobulated mass (7.6 x 4.5 cm) in the left temporal fossa and left parasellar, sellar, and suprasellar regions, with a "dural tail" more typical of meningioma. Vascular supply was from both the internal carotid arteries and the left middle meningeal artery, precluding embolization. At the time of the craniotomy, a highly vascular tumor was found. Intraoperative hemorrhage limited resection to 20% of the tumor. The histological diagnosis was of a solitary fibrous tumor with an unusually angiomatoid architecture. After the operation, radiotherapy and toremifene were administered. A dramatic reduction in the size of the tumor was seen with no recurrence to date. The use of surgery, radiotherapy, and toremifene in treatment of a vascular intracranial solitary fibrous tumor has been successful and warrants further research.

Atypical presentations of solitary fibrous tumors of the central nervous system: an analysis of unusual clinicopathological and outcome patterns in three new cases with a review of the literature

Central nervous system (CNS) solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms recognized less than a decade ago. Approximately 60 cases of SFT have been reported in the central nervous system. We describe three atypical SFTs of the CNS, two intracranial and one within the spine. One intracranial SFT arose from the sella turcica and expanded into the suprasellar areas. It relapsed twice during the 3 years following partial resection, and the MiB 1 labeling index steadily increased without obvious malignant transformation. The second SFT arose from the confluence of the sinuses, widely invaded the lateral sinus and adjacent bones, had a low MiB 1 index and has not recurred after 5 years. The intraspinal tumor occurred at T5-T7 in a patient with multiple café-au-lait spots, was predominantly myxoid and developed a second similar lesion at S3-S5 14 years later. The MiB 1 index was lower in the second tumor. Immunohistochemistry confirmed that all were SFTs. These atypical presentations gave us an opportunity to provide further information about the natural histological course of CNS SFTs.

Recurrent intracranial solitary fibrous tumor with cerebrospinal fluid dissemination

Solitary fibrous tumor (SFT) is a benign and rare neoplasm. To date, only 37 patients with intracranial SFTs have been reported. Although a number of the tumors were recurrent and some later underwent malignant transformation, none of these lesions progressed to cerebrospinal fluid (CSF) dissemination. In this paper the authors report a case of SFT in which the lesion recurred several times and ultimately was disseminated by the CSF. The patient was a 63-year-old woman with multiple intracranial and spinal tumors. Fifteen years before this presentation, at the age of 48 she had been hospitalized for resection of a falcotentorial tumor. During the ensuing 15 years she underwent multiple surgeries and sessions of radiation therapy for recurrent lesions. The exclusive location of her tumors in the subarachnoid space at the end of this 15-year period indicate CSF dissemination of the tumor. The tumor that was resected when the patient was 48 years old and the latest resected lesion were analyzed by performing immunohistological CD34, epithelial membrane antigen, vimentin, S100 protein, and reticulin staining, and determining the MIB-1 labeling index (LI). Most of the results were identical, and both tumors were diagnosed as SFT according to a staining pattern that showed a strong and diffuse positive reaction for CD34. Nevertheless, the authors noted that the MIB-1 LI increased from less than 1% in the original tumor to 13% in the latest tumor. The increased proliferation of MIB-1 indicates that the malignant transformation could have occurred during tumor recurrence with CSF dissemination.

Malignant intracranial solitary fibrous tumour with four recurrences over a 30‐year period

Malignant intracranial solitary fibrous tumour with four recurrences over a 30‐year period

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.