Immunotherapy for Solitary Fibrous Tumor (Hemangiopericytoma): A Unique Treatment Approach for a Rare Central Nervous System Tumor.

INTRODUCTION: Solitary fibrous tumors (SFTs) of the central nervous system represent a unique entity with limited data on best treatment practices. CASE REPORT: Here, we present a case of multiply recurrent central nervous system SFT treated with radiation and immunotherapy. Immunotherapy was chosen based on mutations of genes encoding DNA repair enzymes detected through next-generation sequencing of the tumor, DNA polymerase epsilon catalytic subunit ( POLE ) and mutL homolog 1. The use of radiation and immunotherapy led to slight shrinkage and no recurrence of the tumor for over 2 years. CONCLUSION: The presence of somatic DNA repair enzyme gene mutations in SFT may suggest a benefit from a combination of radiotherapy and immunotherapy. This may serve as a biomarker for guiding management in patients with this rare tumor.

Intracranial Solitary Fibrous Tumor Mimicking Meningioma.

Solitary fibrous tumor (SFT) is a clinically rare tumor derived from mesenchymal spindle cells. Central nervous system SFT represents only 0.09% of tumors occurring on the meninges, while intracranial solitary fibrous tumors (ISFT) are even more rare. Due to the similar genetic characteristics it shares with hemangiopericytoma, in 2016, the World Health Organization (WHO) classified it as a single disease called solitary fibrous tumor (SFT)/hemangiopericytoma. We reported a case of a 60-year-old female with an intracranial solitary fibrous tumor (ISFT). The patient's magnetic resonance imaging showed a mass adhering extensively to the dura mater, with adjacent thickening of the meninges and evidence of a meningeal tail sign. These radiologic findings suggested a meningioma. The tumor was surgically removed and sent for pathologic examination, which confirmed that the tumor was consistent with a solitary fibrous tumor(WHO III). Due to its rarity and similarities with meningioma, ISFT is often misdiagnosed as other types of brain tumors. ISFT is poorly understood and poses a diagnostic challenge. Our case report presents several features suggestive of meningioma, but histopathological examination after surgery confirmed the diagnosis of SFT. Knowledge of these tumors is crucial for neurosurgeons to include them in preoperative differential diagnosis.

68 Ga-Prostate-Specific Membrane Antigen PET/CT in Imaging of Hemangiopericytoma.

ABSTRACT: Hemangiopericytoma is a mesenchymal neoplasm that derives from pericytes surrounding the capillaries presenting overexpression of PSMA, which can be a source of pitfall in 68 Ga-PSMA-11 PET/CT. We reported 2 cases with recurrent hemangiopericytoma grade III with high expression of 68 Ga-PSMA-11 in PET/CT. Based on the performed examination, one of them received targeted α-therapy with the IV injection of 225 Ac-PSMA-617.

Treatment and Management of a Sinonasal Hemangiopericytoma.

Sinonasal Hemangiopericytoma (HPC) is a rare vascular tumor originated from Zimmermann's pericyte with not well valuable course. The diagnosis needs a careful ENT endoscopic examination, radiological study and histopathological analysis with immunohistochemistry for confirmation of diagnosis. We describe a case of a male patient, 67 years old, with history of repeated monolateral right epistaxis. Endoscopic and radiological examination showed an ethmoid-sphenoidal lesion which occupied the entire nasal fossa extending towards the choana, with blood supply coming from the posterior ethmoidal artery. The patient performed an extemporaneous biopsy with subsequent en-bloc removal in operating room, without prior embolization, with the Centripetal Endoscopic Sinus Surgery technique (CESS). The histopathologic analysis led to the diagnosis of sinus HPC. The patient performed close endoscopic follow-ups every 2 months, without performing radio or chemotherapy, and with no evidence of recurrence after 3 years of follow-up. Analyzing the recent literature, a more indolent course with low recurrence rates after total endoscopic surgery remotion was described. Preoperative embolization can be useful in determinate cases, but cause to different complications, it should not be ordinarily performed.

Uterine hemangiopericytoma with cardiac involvement and pulmonary metastasis: A case report and literature review.

Uterine hemangiopericytoma is extremely rare. This article describes a case of uterine hemangiopericytoma. The tumor involved the parauterine vein; extended into the inferior vena cava, right cardiac cavity, and pulmonary artery; and metastasized to the lungs. It was irregular in shape and exhibited the string-of-beads sign on echocardiography, and it was tightly attached to the right ventricular surface and pulmonary artery wall. The patient underwent tumor resection without adjuvant treatment. A pelvic nodule was found 3 months postoperatively and was considered a recurrent lesion.

Postoperative progression of intracranial grade II-III solitary fibrous tumor/hemangiopericytoma: predictive value of preoperative magnetic resonance imaging semantic features.

BACKGROUND: Preoperative prediction of postoperative tumor progression of intracranial grade II-III hemangiopericytoma is the basis for clinical treatment decisions. PURPOSE: To use preoperative magnetic resonance imaging (MRI) semantic features for predicting postoperative tumor progression in patients with intracranial grade II-III solitary fibrous tumor/hemangiopericytoma (SFT/HPC). MATERIAL AND METHODS: We retrospectively analyzed the preoperative MRI data of 42 patients with intracranial grade II-III SFT/HPC, as confirmed by surgical resection and pathology in our hospital from October 2010 to October 2017, who were followed up for evaluation of recurrence, metastasis, or death. We applied strict inclusion and exclusion criteria and finally included 37 patients. The follow-up time was in the range of 8-120 months (mean = 57.1 months). RESULTS: Single-factor survival analysis revealed that tumor grade (log-rank, P = 0.024), broad-based tumor attachment to the dura mater (log-rank, P = 0.009), a blurred tumor-brain interface (log-rank, P = 0.008), skull invasion (log-rank, P = 0.002), and the absence of postoperative radiotherapy (log-rank, P = 0.006) predicted postoperative intracranial SFT/HPC progression. Multivariate survival analysis revealed that tumor grade (P = 0.009; hazard ratio [HR] = 11.42; 95% confidence interval [CI] = 1.832-71.150), skull invasion (P = 0.014; HR = 5.72; 95% CI = 1.421-22.984), and the absence of postoperative radiotherapy (P = 0.001; HR = 0.05; 95% CI = 0.008-0.315) were independent predictors of postoperative intracranial SFT/HPC progression. CONCLUSION: Broad-based tumor attachment to the dura mater, skull invasion, and blurring of the tumor-brain interface can predict postoperative intracranial SFT/HPC progression.

The Natural History of Parapharyngeal Solitary Fibrous Tumor/Hemangiopericytoma: A Case Report.

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is extremely rare, and most of them are immediately treated for radical resection. However, the information concerning its natural history remains unclear. In this report, we presented a patient with parapharyngeal SFT/HPC, who was not immediately treated with surgical resection at first diagnosis. After approximately 3 years, the tumor volume doubling time (TVDT) and specific growth rate (SGR) could be measured through 3 serial magnetic resonance imagings. The TVDTs in the early and late pretreatment stages were 350 and 180 days, respectively, while the SGRs were 0.002 and 0.003, respectively. The growth rate of this disease entity is generally slow and may accelerate in the disease process.

Malignant Progression of a Superior Cerebellar Tentorium Solitary Fibrous Tumor in the Pineal Region with Intracranial Metastases: A Case Report and a Literature Review.

BACKGROUND: Pineal region solitary fibrous tumors (SFT) incorporate a histologic spectrum of rarely metastasizing mesenchymal neoplasms that include tumors formerly classified as hemangiopericytoma. CASE REPORT: Here, we describe a rare case of SFT of the pineal region in a 25-year-old man with a literature review. After the first surgery, the tumor reappeared as a local low-grade recurrence, followed by metastasis to the right parietal lobe, and then hyperacute intraparenchymal hematoma at the metastatic site, and later presentation of widespread intracranial intra-axial and extra-axial metastases during the follow-up period. Systemic metastases were not detected. The histopathological evaluation of the resected tissues confirmed the malignant progression of the tumor. CONCLUSION: The diagnosis of SFT of the pineal region through clinical and imaging features can be considerably challenging. Large size, intratumoral cystic areas, and intense contrast enhancement are the main conventional imaging characteristics of the tumor. Surgery is the first preferential treatment. All recurrent or metastatic cases were grade II or grade III tumors. Adjuvant radiotherapy should be added to surgical treatment in high-grade tumors. Gamma knife radiosurgery is a treatment option for intracranial metastases.

Radiological Differentiation Between Intracranial Meningioma and Solitary Fibrous Tumor/Hemangiopericytoma: A Systematic Literature Review.

BACKGROUND: Intracranial solitary fibrous tumor (SFT) is characterized by aggressive local behavior and high post-resection recurrence rates. It is difficult to distinguish between SFT and meningiomas, which are typically benign. The goal of this study was to systematically review radiological features that differentiate meningioma and SFT. METHODS: We performed a systematic review in accordance with PRISMA guidelines to identify studies that used imaging techniques to identify radiological differentiators of SFT and meningioma. RESULTS: Eighteen studies with 1565 patients (SFT: 662; meningiomas: 903) were included. The most commonly used imaging modality was diffusion weighted imaging, which was reported in 11 studies. Eight studies used a combination of diffusion weighted imaging and T1- and T2-weighted sequences to distinguish between SFT and meningioma. Compared to all grades/subtypes of meningioma, SFT is associated with higher apparent diffusion coefficient, presence of narrow-based dural attachments, lack of dural tail, less peritumoral brain edema, extensive serpentine flow voids, and younger age at initial diagnosis. Tumor volume was a poor differentiator of SFT and meningioma, and overall, there were less consensus findings in studies exclusively comparing angiomatous meningiomas and SFT. CONCLUSIONS: Clinicians can differentiate SFT from meningiomas on preoperative imaging by looking for higher apparent diffusion coefficient, lack of dural tail/narrow-based dural attachment, less peritumoral brain edema, and vascular flow voids on neuroimaging, in addition to younger age at diagnosis. Distinguishing between angiomatous meningioma and SFT is much more challenging, as both are highly vascular pathologies. Tumor volume has limited utility in differentiating between SFT and various grades/subtypes of meningioma.

Differentiation of intracranial solitary fibrous tumor/hemangiopericytoma from atypical meningioma using apparent diffusion coefficient histogram analysis.

This study aimed to investigate the value of apparent diffusion coefficient (ADC) histogram analysis in differentiating intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC) from atypical meningioma (ATM). Retrospective analyzed the clinical, magnetic resonance imaging, and pathological data of 20 and 25 patients with SFT/HPC and ATM, respectively. Histogram analysis was performed on the axial ADC images using MaZda software, and nine histogram parameters were obtained, including mean, variance, skewness, kurtosis, and the 1st (ADC1), 10th (ADC10), 50th (ADC50), 90th (ADC90), and 99th (ADC99) percentile ADC. Differences in ADC histogram parameters between SFT/HPC and ATM were compared by an independent t test or Mann-Whitney U test, while the statistically significant histogram parameters were further analyzed by drawing receiver operating characteristic (ROC) curves to evaluate the differential diagnostic performance. Among the nine ADC histogram parameters we extracted, the mean, ADC1, ADC10, ADC50, and ADC90 in the SFT/HPC group were greater than those of ATM, and significant differences were observed (all P < 0.05). ROC analysis showed that the ADC1 generated the highest area under the curve (AUC) value of 0.920 in distinguishing the two tumors, when using 91.00 as the optimal threshold. The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value in distinguishing between SFT/HPC and ATM were 84.00%, 85.00%, 84.44%, 87.50%, and 81.00%, respectively. ADC histogram analysis can be a reliable tool to differentiate between SFT/HPC and ATM, with the ADC1 being the most promising potential parameter.

Rare hypervascular pancreatic tumors diagnosed as asynchronous metastases of central nervous system solitary fibrous tumor/hemangiopericytoma.

A 70-year-old woman was referred to our hospital for asymptomatic pancreatic tumors. She had a history of hemagiopericytoma (HPC) about 20 years ago, and no apparent recurrence has been observed. Contrast-enhanced computed tomography revealed two hypervascular tumors in the head and uncinate process of the pancreas, and no obvious neoplastic lesions were found in other organs. Endoscopic ultrasound guided fine-needle aspiration cytology was performed and histopathology showed that spindle-shaped tumor cells were arranged in a hemangiopericytoma-like pattern and positive for STAT6, which was a characteristic feature of solitary fibrous tumors (SFTs). Immunohistochemical staining for surgical pathology specimens from past HPC showed positive expression of STAT6, which was Grade 2 central nervous system solitary fibrous tumor/hemagiopericytoma (CNS SFT/HPC) according to the current WHO classification. From these findings, the pancreatic tumors were preoperatively diagnosed as pancreatic metastases of CNS SFT/HPC. She underwent pancreaticoduodenectomy. Histopathological examination of the surgically resected specimen proved that the both pancreatic tumors were SFT/HPC. Thus, pancreatic tumors were finally diagnosed as asynchronous pancreatic metastases from CNS SFT/HPC. Although extremely rare, metastatic pancreatic tumors derived from SFT/HPC should be considered as a differential diagnosis for hypervascular pancreatic tumors, especially when having a past history of brain tumors.

Skull base hemangiopericytomas.

OBJECTIVE: To retrospectively evaluate the clinical outcome of six patients with skull base hemangiopericytomas (HPCs) and that of a cohort of 37 similar patients identified by a systematic review of the literature. METHODS: The series constitutes of three men and three women with newly diagnosed skull base HPC who underwent multimodal treatment including surgery, external beam radiotherapy (EBRT) and pre-operative embolization. Furthermore, a systematic review off the literature identified 37 reports of primarily intracranial skull base HPCs. RESULTS: Four patients had a gross total resection (GTR) and two patients had a near total resection. Five patients were referred for adjuvant EBRT with a survival ranging from 15 to 47 months. All patients had an excellent outcome and resumed their previous activities. Literature review identified 37 additional patients with skull base HPC. Altogether, tumors were unevenly distributed above and below tentorium. GTR was achieved in half the patients, and 72.1% were referred to EBRT. Out of 37 reported patients in the literature, survival longer than 1 year was described in only 24. Within the combined cohort including the present series, survival was 83.6 months. CONCLUSIONS: The present series shows that a radical resection of HPC can be achieved under the difficult anatomical conditions of skull base surgery. Pre-operative arterial embolization may be instrumental to maintain a clear visual field and prevent excessive blood loss. Finally, the results of the present cohort suggest that EBRT may be useful for local growth control, as an effective palliative measure for skull base HPCs.

The relationship between the apparent diffusion coefficient and the Ki-67 proliferation index in intracranial solitary fibrous tumor/hemangiopericytoma.

This study evaluated the value of the apparent diffusion coefficient (ADC) in distinguishing grade II and III intracranial solitary fibrous tumors/hemangiopericytomas and explored the correlation between ADC and Ki-67. The preoperative MRIs of 37 patients treated for solitary fibrous tumor/hemangiopericytoma (grade II, n = 15 and grade III, n = 22) in our hospital from 2011 to October 2020 were retrospectively analyzed. We compared the difference between the minimum, average, maximum, and relative ADCs based on tumor grade and examined the correlation between ADC and Ki-67. Receiver operating characteristic curve analysis was used to analyze the diagnostic efficiency of the ADC. There were significant differences in the average, minimum, and relative ADCs between grade II and III patients. The optimal cutoff value for the relative ADC value to differentiate grade II and III tumors was 0.998, which yielded an area under the curve of 0.879. The Ki-67 proliferation indexes of grade II and III tumors were significantly different, and the average (r = - 0.427), minimum (r = - 0.356), and relative (r = - 0.529) ADCs were significantly negatively correlated with the Ki-67 proliferation index. ADC can be used to differentiate grade II and III intracranial solitary fibrous tumors/hemangiopericytomas. Our results can be used to formulate a personalized surgical treatment plan before surgery.

Sinonasal Glomangiopericytoma: Review of Imaging Appearance and Clinical Management Update for a Rare Sinonasal Neoplasm.

Introduction: Glomangiopericytoma (GPC) is a rare tumor in the nasal cavity or paranasal sinuses with low malignant potential. Initially deemed a hemangiopericytoma, in 2005 it was classified as a distinct entity by the World Health Organization (WHO). Case Presentation: A male patient in his early 60s presented with new-onset right arm and leg weakness/numbness, who was incidentally found to have a left ethmoid sinus mass with extension in the olfactory fossa. On CT and MRI, the mass enhanced with well-defined borders and eroded the bone, but without dural enhancement. The mass was surgically excised, and pathology confirmed the diagnosis of glomangiopericytoma by microscopic appearance and staining. Discussion: Glomangiopericytoma has less than 0.5% incidence of all neoplasms of the sinonasal cavity, making it rare. Most diagnosed patients are in their 6th or 7th decade of age, with a slight female predominance. Treatment is complete surgical excision, with excellent prognosis, although there is up to 17% local recurrence. Despite the non-specific appearance on CT and MRI, imaging can help provide differential diagnosis, tumor extent, size, and reassuring non-aggressive characteristics of the tumor prior to surgery. GPC tumors are relatively resistant to radiation and chemotherapy. Conclusion: It is important to recognize glomangiopericytoma in the differential of masses of the nasal cavities or paranasal sinuses, as they rarely warrant aggressive treatment beyond local excision. Each reported case of glomangiopericytoma helps to build guidance for imaging and treatment since GPC is rare and not well-represented in the medical literature.

Primary endodermal hemangiopericytoma/solitary fibrous tumor of the cervical spine: a case report and literature review.

BACKGROUND: Hemangiopericytoma (HPC), also known as solitary fibrous tumor (SFT), is a type of soft tissue sarcoma with a special aggressive behavior. The HPC/SFT is locally aggressive with possibility of late recurrence locally or distant extraneural metastasis. The most common location of this HPC/SFT is the lower extremities. The HPC/SFT in the central nervous system (CNS) is very rare, and compared with the brain, it is rarer in the spinal region. However, clinicians also lack an overall understanding of the diagnosis of HPC/SFT in the spinal cord. CASE PRESENTATION: In this study, we report a rare case of primary cervical spine HPC/SFT in a 53-year-old woman. Two to three weeks before admission, she experienced pain and numbness in her left upper extremity. After computerized tomography (CT) and magnetic resonance imaging (MRI), a gross total resection was performed. Obvious neurological improvement was observed postoperatively. The pain and numbness in the patient's left upper limb were relieved subsequently. We then reviewed the literature on HPC/SFT, such as its clinical presentation, imaging characteristics, treatment, and follow-up. CONCLUSIONS: Diagnosis of HPC/SFT relies on magnetic resonance spectroscopy, enhanced CT, and MRI. Postoperative radiotherapy is strongly recommended to reduce the HPC/SFT recurrence. Immunohistochemical analysis can also help in the differential diagnosis. However; early and long-term follow-up is necessary for patients.

A Rare Case of Metastatic Solitary Fibrous Tumor (Hemangiopericytoma) of the Dura on 18F-FDG PET/CT.

ABSTRACT: Solitary fibrous tumor is a rare neoplasm of mesenchymal origin. It can arise in various locations, most commonly in the pleura. It encompasses a heterogeneous group of histological patterns with different biological behavior. In some cases, metastases occur, sometimes years after the initial diagnosis. We report a case of metastatic meningeal hemangiopericytoma, also called solitary fibrous tumor of the dura, exhibiting liver metastases and muscle metastases 5 years after resection and adjuvant radiotherapy of the primary tumor.