[Myofibroma/myofibromatosis: a clinicopathologic analysis of 9 cases].

Objective: To investigate the clinical and histological features, diagnosis and differential diagnosis of myofibroma/myofibromatosis. Methods: The clinical data and pathology features of nine cases of myofibroma/myofibromatosis were collected from August 2011 to November 2016 in Affiliated Drum Tower Hospital, Nanjing University Medical School and Children's Hospital of Nanjing Medical University. Immunohistochemistry(IHC), PDGFRB molecular analysis and ETV6-NTRK3 gene fusion were performed and relevant literature reviewed. Results: There were 7 males and 2 females, with age ranging from 3 days to 18 years (mean 5 years). The tumors were located in head and neck (eight cases) and trunk (one case). Clinically, the tumors presented as freely movable nodules. Microscopically, they appeared biphasic with alternating light- and dark-staining areas. The light-staining area consisted mainly of plump myoid spindle cells with eosinophilic cytoplasm arranged in nodules, short fascicles, or whorls.The dark-staining area was composed of round or polygonal cells with slightly hyperchromatic nuclei or small spindle cells arranged around a distinct hemangiopericytoma-like vascular pattern. IHC showed the tumor cells in the light-staining area were strongly positive for vimentin and SMA, while cells in dark-staining area were strongly positive for vimentin, and weakly for SMA. Tumor cells were negative for desmin, S-100 protein, h-Caldesmon, CD34 and STAT6. Analysis of PDGFRB mutations was performed in seven cases. Two cases showed 12 exon point mutation c. 1681 c>T(p.R561C), one case showed 14 exon point mutation c. 1998C>G (p.N666K). ETV6-NTRK3 gene fusion was not detected by fluorescence in situ hybridization in four patients under three years old. All cases were followed for 6 to 68 months, with two recurrences. Conclusions: Myofibroma/myofibromatosis is an uncommon benign myofibroblastic tumor of infancy and childhood. The tumor can appear biphasic, and may show PDGFRB point mutation which is of potential diagnostic value.

Intraoperative Transpedicular Onyx Injection to Reduce Vascularity of a Thoracic Hemangiopericytoma After Unsuccessful Preoperative Endovascular Embolization: a Technical Report.

BACKGROUND AND IMPORTANCE: Hemangiopericytoma is a rare vascular tumor with central nervous system involvement representing only 1% of central nervous system tumors. They rarely affect the vertebral column. Complete surgical resection is the treatment of choice for hemangiopericytoma given their high rates of local recurrence. However, the high vascularity of such tumors with the risk of massive bleeding during surgery represents a significant challenge to surgeons. Therefore, preoperative endovascular embolization via the transarterial route has been advocated. CLINICAL PRESENTATION: In the current study, we present a case of a T12 hemangiopericytoma that was managed by a 2-stage surgical resection, with the use of intraoperative transpedicular onyx injection to reduce intraoperative blood loss following an unsuccessful trial of preoperative endovascular embolization. CONCLUSION: Preoperative endovascular embolization is not feasible in some cases due to the location of the segmental or radiculomedullary arteries in relation to tumor feeders and, rarely, small size of these arterial feeders. Percutaneous injection of onyx is an option. In this case report, we discuss direct intraoperative injection via a transpedicular route as a safe and effective method for decreasing the vascularity of some lesions and improving intraoperative blood loss.

3D digital subtracted CT angiography to evaluate the venous anatomy in extra-axial tumors invading the major dural venous sinuses.

Background Investigation of the venous system surrounding a tumor that is invading a dural sinus is of great use for guiding the surgical excision. Non-invasive imaging is often inadequate since enhancement of the tumor causes it to blend with the contrast of the venous vascular structures. Conventional two-dimensional angiography is also often insufficient. Objective The objective of this study was to report regarding the potential of three-dimensional digital subtracted computed tomography angiography (3D DS-CTA) as a technique to preoperatively explore these tumors. Methods We retrospectively studied the radiological and surgical features of patients explored with 3D DS-CTA for a tumor invading a major dural sinus. Results Three patients were included in this study, one hemangiopericytoma and two meningiomas. 3D DS-CTA allows for accurate assessment of the patency of the sinus, the location of the secondary intra- and trans-osseous venous outlets, and surgical guidance by neuronavigation. Conclusion 3D DS-CTA could be a promising guiding and diagnostic tool for the pre- and intraoperative treatment of vascular tumors invading the dural sinuses, for which the venous morbidity and mortality is substantial.

Activation of multiple angiogenic signaling pathways in hemangiopericytoma.

Hemangiopericytoma (HPC) is a highly vascularized mesenchymal tumor. Local recurrence and distant metastasis are common features of HPC. Considering the remarkable hyper-vasculature phenotype of HPC, we assumed that dysregulated angiogenic signaling pathways were involved in HPC. The key components of angiogenic signaling pathways including VEGF-VEGF-R2, EphrinB2-EphB4 and DLL4-Notch were examined by real-time RT-PCR, Western blotting and immunostaining in 17 surgical specimens of HPC patients and in 6 controls. A significant upregulation of VEGF and VEGF-R2 associated with elevated levels of p-Akt and proliferating cell nuclear antigen (PCNA) was detected in HPC. Moreover, a dramatic increase in the mRNA and protein expression of EphB4 and its downstream factor p-Erk1/2 was found in HPC. A massive activation of core-components of DLL4-Notch signaling was detected in HPC. Double-immunofluorescent staining confirmed the expression of these upregulated key factors in the endothelial cells of tumor vessels. The present study identified the activation of multiple and crucial angiogenic signaling pathways, which could function individually and/or synergistically to stimulate angiogenesis in HPC and eventually contribute to tumor growth and progression. Our findings emphasize the importance to target multiple angiogenic signaling pathways when an anti-angiogenic therapy is considered for this highly vascularized tumor.

'Papillary' solitary fibrous tumor/hemangiopericytoma with nuclear STAT6 expression and NAB2-STAT6 fusion.

This report describes clinicopathological findings, including genetic data of STAT6, in a solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) of the central nervous system in an 83-year-old woman with a bulge in the left forehead. She noticed it about 5 months before, and it had grown rapidly for the past 1 month. Neuroradiological studies disclosed a well-demarcated tumor that accompanied the destruction of the skull. The excised tumor showed a prominent papillary structure, where atypical cells were compactly arranged along the fibrovascular core ('pseudopapillary'). There was rich vasculature, some of which resembled 'staghorn' vessels. Mitotic figures were occasionally found. Whorls, psammoma bodies, or intra-nuclear pseudoinclusions were not identified. By immunohistochemistry, CD34 was strongly positive in the tumor cells, and STAT6 was localized in their nuclei. By reverse transcription-polymerase chain reaction (RT-PCR), an NAB2-STAT6 fusion gene, NAB2 exon6-STAT6 exon17, was detected, establishing a definite diagnosis of SFT/HPC. 'Papillary' SFT/HPC needs to be recognized as a possible morphological variant of SFT/HPC, and should be borne in mind in its diagnostic practice.

Heart rate turbulence analysis in female patients with fibromyalgia

OBJECTIVE: Fibromyalgia is characterized by diffuse musculoskeletal pain and discomfort. There are several reports regarding autonomic nervous system dysfunction in patients with fibromyalgia. Heart rate turbulence is expressed as ventriculophasic sinus arrhythmia and has been considered to reflect cardiac autonomic activity. Heart rate turbulence has been shown to be an independent and powerful predictor of sudden cardiac death in various cardiac abnormalities. The aim of this study is to determine whether heart rate turbulence is changed in female patients with fibromyalgia compared with healthy controls. METHODS: Thirty-seven female patients (mean age, 40±11 years) with fibromyalgia, and 35 age- and sex-matched healthy female control subjects (mean age, 42±9 years) were included. Twenty-four hours of ambulatory electrocardiography recordings were collected for all subjects, and turbulence onset and turbulence slope values were automatically calculated. RESULTS: The baseline clinical characteristics of the two groups were similar. There were no significant differences in turbulence onset and turbulence slope measures between patients and control subjects (turbulence onset: −1.648±1.568% vs. −1.582±1.436%, p ϝ 0.853; turbulence slope: 12.933±5.693 ms/RR vs. 13.639±2.505 ms/RR, p ϝ 0.508). Although body mass index was negatively correlated with turbulence slope (r ϝ −0.258, p ϝ 0.046), no significant correlation was found between body mass index and turbulence onset (r ϝ 0.228, p ϝ 0.054). CONCLUSION: To the best of our knowledge, this is the first study to evaluate heart rate turbulence in patients with fibromyalgia. It appears that heart rate turbulence parameters reflecting cardiac autonomic activity are not changed in female patients with fibromyalgia. .

Endovascular preoperative embolization of orbital hemangiopericytoma with n-butyl cyanoacrylate glue.

Hemangiopericytoma is an uncommon neoplasm that may present in myriad locations, including the lower extremities, pelvic area, and the head and neck area, including the orbit. Orbital hemangiopericytoma is often described as synonymous with orbital solitary fibrous tumor, giant cell angiofibroma, and fibrous histiocytoma, as they all belong to a spectrum of collagen-rich fibroblastic tumors that are often CD34-positive and have overlapping histopathologic features. Many cases of orbital hemangiopericytoma have been reported in the literature along with various surgical approaches, long-term outcomes, and techniques to manage recurrence; however, few have discussed preoperative embolization., Intraoperative hemorrhage is a concern in both the congenital and the adult form of these cases, and may be an indication for preoperative embolization. A unique case of preoperative embolization was presented with n-butyl cyanoacrylate for surgical resection of a large orbital hemangiopericytoma in a 58-year-old woman.

Antitumor effect of bevacizumab in a xenograft model of canine hemangiopericytoma.

Canine hemangiopericytoma (CHP) is characterized by frequent local recurrence and increased invasiveness. Vascular endothelial growth factor (VEGF) is a key regulator of angiogenesis in tumors. The aim of the present study was to investigate the effect of a single dose of bevacizumab on a xenograft model of CHP. VEGF protein was secreted from cultured CHP cells and interacted with bevacizumab. Bevacizumab treatment suppressed tumor growth by inhibiting tumor angiogenesis, whereas no significant differences were observed in the proliferation index and apoptosis rates of treated and untreated mice. Thus, bevacizumab had antitumor effects in a xenograft model of CHP.

Comparison of CT perfusion parameters and microvessel density in intracranial hemangiopericytomas with peritumoral edema.

PURPOSE: Intracranial hemangiopericytomas (HPCs) are rare, and they have a tendency for local recurrence and metastases. The purpose of this study was to evaluate the relationship between CT perfusion (CTP) parameters and microvessel density (MVD) of HPCs and compare CTP parameters in parenchyma and peritumoral edema of HPCs. MATERIALS AND METHODS: The study was approved by the ethics committee, and written informed consent was obtained. Ten patients with HPCs and peritumoral edema, confirmed by pathological results, received 64-slice CT perfusion imaging before operation. To evaluate vascular attenuation of tumoral parenchyma, we immunostained the specimen sections for CD-34, measured the integrated optical density of all the positive stained CD-34 cells in the microscopic field, and calculated its ratio to total area of field as MVD. Perfusion analysis was calculated using the Patlak method. Using a 1-cm distance from the outer enhancing tumor margin as a boundary, the peritumoral edema was divided into an immediate and a distant part. The quantitative CTP parameters, including cerebral blood volume (CBV), permeability-surface area product (PS) of parenchyma, and immediate and distant peritumoral edemas, were compared. CBV and PS in parenchyma and immediate and distant peritumoral edemas of HPCs were also compared to their respective contralateral normal white matter. The correlations between MVD, CBV, and PS of tumoral parenchyma were analyzed. RESULTS: Positive correlations existed between CBV and MVD, PS and MVD (P < 0.05) respectively in the 10 patients. Furthermore, the values of CBV and PS in parenchyma of HPCs were significantly higher than those of the contralateral normal white matter and peritumoral edema (P < 0.05). The value of CBV in peritumoral edema of HPCs were lower than that of contralateral normal white matter (P < 0.05), while the value of PS in immediate and distant peritumoral edemas of HPCs were not significantly difference with that of contralateral normal white matter (P > 0.05). Finally, the values of CBV and PS did not show a significant difference between immediate and distant peritumoral edemas. CONCLUSIONS: CT perfusion imaging, especially determination of maximal CBV and corresponding PS values in the parenchyma, may be a useful and non-invasive technique for the preoperative evaluation of hemodynamic features of HPCs with peritumoral edema. CBV of peritumoral edema indicate that HPCs have a possibility of infiltration, this need further radiological-pathological research.

Unrecognized hemangiopericytoma of posterior cervical region with intracranial extension.

Hemangiopericytoma of the posterior cervical space and occipital bone is an uncommon lesion which should be considered in the differential diagnosis of a lumpy and highly vascular lesion of the posterior cervical space. We report the case of a 47-year-old woman who experienced sudden and painful occipital and posterior cervical swelling. She underwent a blind biopsy which was complicated by profuse bleeding. The palpable lesion was not properly diagnosed preoperatively, and the endocranial extension of the lesion was overlooked by her surgeon who performed a blind biopsy without adequate diagnostic imaging who inadvertently invaded the posterior cranial fossa during biopsy. We would like to emphasize the need for appropriate non-invasive diagnostic investigations before any biopsy of head and neck lesions that may extend deeply.

Endovascular embolization of cervical hemangiopericytoma with Onyx-18: case report and review of the literature.

Cervical hemangiopericytoma (HPC) is a rare tumor involving dura and bone. Because HPC shares some imaging features with meningioma, hemangioblastoma, schwannoma and solitary fibrous tumors; histology with appropriate immunohistochemistry is essential for its diagnosis and treatment. HPC is highly vascular and has a high rate of local recurrence following resection. Thus it can pose significant therapeutic challenges. To the best of our knowledge this is the 13th reported case of HPC of the cervical spine and the first case of cervical HPC treated using embolization with Onyx-18. In addition, a systematic review of the literature is presented describing previous experience with HPC of the cervical region. The case of a 61-year-old woman is reported who presented with a 7 month history of dizziness, mild ataxia and left-sided tinnitus when supine. MRI of the cervical spine demonstrated a gadolinium enhancing mass arising from the dorsal cervical dura, extending from the occiput to C2, with internal flow voids and extension into the C2 lamina and spinous process. Digital subtraction angiography demonstrated multiple corkscrew-shaped supplying vessels, marked hypervascularity, rapid arteriovenous shunting and delayed contrast washout. Super selective injection of Onyx-18 yielded approximately 90% embolization of the lesion, followed by subtotal resection and radiotherapy. Histology confirmed the presumptive diagnosis of HPC. Preoperative embolization with Onyx-18 should be considered for large HPC as it may decrease the risk of intraoperative hemorrhage. In this case, embolization with Onyx-18 did not preclude histopathologic diagnosis of HPC.

Congenital hemangiopericytoma presenting as rectal bleeding in a neonate: a case report.

Hemangiopericytoma is a rare soft tissue lesion originating from capillary pericytes that can occur anywhere vascular capillaries are found. It is an uncommon tumor found typically in adults but rarely in children. We describe a previously unreported case of an intraluminal lesion in a neonate.

Large hemangiopericytoma associated with arteriovenous malformations and dural arteriovenous fistulae.

BACKGROUND: Hemangiopericytomas are rare vascular tumors of the central nervous system. Although hemangiopericytomas have been associated with other vascular malformations, there is no report of an intracranial hemangiopericytoma found in association with multiple arteriovenous malformations and dural arteriovenous fistulae. CASE DESCRIPTION: We present the case of an otherwise healthy 25-year-old woman who presented with a large hemangiopericytoma involving the superior sagittal sinus. The highly vascular nature of the lesion, the total occlusion of the sinus anterior to the tumor, and the presence of multiple arteriovenous malformations and dural arteriovenous fistulae complicated resection of this tumor. CONCLUSION: To our knowledge, no prior report has described a tumor of this magnitude in association with multiple AVMs and dAVFs.

Myopericytoma of the kidney.

Myopericytoma is a rare, histologically distinctive tumor that shows evidence of differentiation toward perivascular myoid cells. Myopericytoma is largely considered a neoplasm of skin and soft tissues, with examples of this lesion involving visceral sites being extremely limited. We present the clinical and pathologic details of an unusual case of myopericytoma occurring in the kidney. Histologically, the tumor was richly vascularized and composed of a perivascular proliferation of oval to spindle-shaped cells with bland cytologic features. The neoplastic cells were arranged in a concentric fashion around vascular lumina and also surrounded dilated, branching vessels, with a glomangiopericytomatous appearance. Mitotic figures were inconspicuous, and necrosis was absent. Perivascular myoid differentiation was supported by positive immunoreactivity for muscle-specific and smooth muscle actins, and absence of reactivity for desmin. The present case serves to expand the anatomical distribution of myopericytoma and also broadens the spectrum of primary mesenchymal neoplasms that may be encountered in the kidney.