Preoperative transarterial embolization of a recurrent orbital solitary fibrous tumor with significant hypervascularity: a case report.

BACKGROUND: Orbital solitary fibrous tumors (SFTs) are rare neoplasms. Recurrent, hypervascular, malignant variations of orbital SFTs have recently been noted and can present a surgical challenge. CASE PRESENTATION: We describe a case of a 53-year-old Chinese woman with a history of a resected orbital SFT. She presented with proptosis, limited eyeball movement, and visual loss in the right eye, suggestive of a recurrent SFT. Ocular examination with multimodal imaging revealed a large, nonpulsatile, noncompressible, hypervascular mass behind the eyeball. The patient underwent preoperative transarterial embolization of the main blood supply to the tumor in order to control intraoperative blood loss, followed by ocular enucleation to optimize exposure and enable complete resection of the tumor. Embolization of the right ophthalmic artery and the distal branch of the right internal maxillary artery caused an immediate, substantial reduction of vascular flow, which allowed us to enucleate the eyeball and resect the tumor with minimal blood loss and no complications. CONCLUSIONS: Our case is so far the first Chinese case of successful preoperative embolization of the main blood supply to a large, recurrent, hypervascular orbital SFT. This case also described a different surgical approach to achieve total removal of an orbital SFT without osteotomy.

Preoperative embolisation of orbital solitary fibrous tumour.

Solitary fibrous tumours (SFT) is an encompassing terminology comprising of tumours with proliferating CD34 positive specialised fibroblasts. Orbital SFTs are rare slowly progressive highly vascular neoplasms. Complete surgical excision is considered the mainstay treatment. Incomplete resection is a known risk factor for recurrence and malignant transformation. Recently preoperative embolisation of SFT has shown promising results in reducing the vascularity of these tumours rendering them amenable to complete surgical excision. Less than 10 cases of embolisation of orbital solitary fibrous tumours have been described in literature. Our patient underwent an attempted surgical excision elsewhere with significant intraoperative haemorrhage which precluded its complete excision. Herein, we report successful outcome in a case of hypervascular orbital SFT managed with preoperative embolisation, surgical resection and adjuvant radiotherapy along with a review of relevant literature.

Symptomatic Intraosseous Vascular Malformation of Infraorbital Rim: A Case Report With Literature Survey.

BACKGROUND/AIM: Intraosseous orbital hemangiomas or vascular malformations (VM) are rare. This report is intended to complement the experience of diagnosing and treating a rare vascular lesion at this site. Special attention is paid to three-dimensional imaging and the morphological distinction between the two entities in this location. CASE REPORT: A 54-year-old female was examined and surgically treated for an exophytic firm mass of the infraorbital, which had become palpable as a hard mass due to growth in size. At first, a bone tumor, for example, an osteoma, was suspected. Intraoperatively, an osseous expansion with distinct fenestrations of the newly grown bone's surface, was detected. The lesion was firmly attaching to the orbital rim. The densely vascularized tumor was well defined to the soft tissues but had grown in continuity from the orbital floor and rim. Vascularized cavities caused the tumor to have a slightly reddish color. The histological examination confirmed the suspicion of the lesion's vascular origin. The lesion's immunohistochemical expression profile approved the final diagnosis of intraosseous VM. CONCLUSION: The symptoms of intraosseous vascular lesions of the orbit are determined by location and size. Modern imaging techniques facilitate the estimation of tumor-like expansion of lesions. However, the imaging characteristics of intraosseous vascular lesions are very variable. The symptoms of the patient presented herein show that growth phases of a vascular orbital malformation can occur in later stages of life and are initially indistinguishable from a neoplasm. In individual cases, patient care necessitates advanced diagnostic measures to establish the diagnosis and determine surgical therapy.

Characterization of Skull Base Lesions Using Pseudo-Continuous Arterial Spin Labeling.

PURPOSE: Pseudo-continuous arterial spin labeling (pCASL) is a non-invasive magnetic resonance (MR) perfusion technique. Our study aimed at estimating the diagnostic performance of the pCASL sequence in assessing the perfusion of skull base lesions both qualitatively and quantitatively and at providing cut-off values for differentiation of specific skull base lesions. METHODS: In this study 99 patients with histopathologically confirmed skull base lesions were retrospectively enrolled. Based on a pathological analysis, the lesions were classified as hypervascular and non-hypervascular. Patients were divided into two subgroups according to the anatomical origin of each lesion. The MRI study included pCASL and 3D T1-weighted fat-saturated post-contrast sequences. Of the patients seven were excluded due to technical difficulties or patient movement. The lesions were classified by two raters, blinded to the diagnosis as either hyperperfused or non-hyperperfused, based on the pCASL sequence. The normalized tumor blood flow (nTBF) of each lesion was determined. Qualitative and quantitative characteristics of hypervascular and non-hypervascular lesions were compared. RESULTS: Visual assessment enabled correct classification of 98% of the lesions to be performed. Quantitatively, we found significant differences between the nTBF values for hypervascular and non-hypervascular lesions (p < 0.001) and provided cut-off values, allowing meningioma and schwannoma to be distinguished from meningioma and adenoma. Significant differences were also found within the hypervascular group, namely, paraganglioma was more hyperperfused than meningioma (p = 0.003) or metastases (p = 0.009). CONCLUSION: The present study demonstrates the high diagnostic performance of pCASL in characterizing skull base lesions by either visual assessment or nTBF quantification. Adding the pCASL sequence to the conventional protocol of skull base assessment can be recommended.

[Orbital hemangiomas: capabilities of modern neuroradiological diagnostics]. / Gemangiomy orbity: vozmozhnosti sovremennoi neiroradiologicheskoi diagnostiki.

MATERIAL AND METHODS: In the period from 2010 to 2016. 14 patients with cavernous hemangioma (CH) and 2 patients with capillary hemangioma (CapH) of the orbit were examined. The age of CH patients varied from 17 to 67 years (median, 53 years); 8 females and 6 males. The age of CapH patients was 35 and 54 years. All patients underwent surgery with subsequent histological verification. CT-perfusion was performed in 10 CH patients and 2 CapH patients according to a developed low-dose protocol (80 kV, 200 mAs, tscan=40 s) with allowance for a target localizer (80 kV, 120 mAs) and at a maximum radiation dose of not more than 4.0 mZv. Neoplasm microcirculation was quantitatively assessed by calculating hemodynamic parameters: blood flow velocity (BFV), blood volume (BV), and mean transit time (MTT). MRI without and with contrast enhancement was performed in 11 CH patients and 2 CapH patients according to the ophthalmologic protocol (Signa GE, 3.0 T) accepted at the Institute: without contrast enhancement - T1, T2, and T2-FLAIR modes, T1 and T2 with a Fat Sat technique at a scan thickness of 3 mm, and DWI MRI; contrast enhancement - T1 (three projections) mode, including the Fat Sat technique. SWAN (n=2) and non-contrast MR perfusion ASL (n=3) were also used. Diffusion-weighted images (DWI) were processed with calculation of the apparent diffusion coefficient (ACD). RESULTS: In all CH patients, CT-perfusion revealed low perfusion parameters of blood flow: BVCH=0.86±0.37 mL/100 g, BFVCH= 4.89±2.01 mL/100 g/min with a high mean transit time MTTCH=10.13±3.05 s compared to the same parameters of blood flow in the normal white matter: CBVNormWM=1.63±2.22 mL/100 g, CBFVNormWM=9.72±3.13 mL/100 g/min, and MTTNormWM=6.76±2.78 s. In CapH cases, significantly increased blood flow velocity and volume values and a low MTT value in the tumor were observed: BVCapH=10.30±4.10 mL/100 g, BFVCapH=119.72±53.13 mL/100 g/min, and MTTCapH=4.35±1.79 s. In the case of orbital hemangiomas, optimal MRI modes were T1 and T2 with the Fat Sat technique, a scan thickness of 3 mm, and intravenous contrast enhancement. The revealed pattern of contrast agent accumulation by CH, initially in the central part and then in the periphery, may be a useful radiographic sign in the differential diagnosis with other orbital tumors. CONCLUSION: Modern CT- and MRI-based diagnostics of orbital hemangiomas provides not only the exact location, size, and spread of the lesion but also reveals the characteristic structural features of these tumors, and the use of perfusion techniques visualizes hemodynamics of the tumors. CT-perfusion-based hemodynamic parameters of cavernous hemangiomas typical of this type of hemangiomas may be used in the differential diagnosis with other tumors of this location. The use of contrast enhancement and the Fat Sat technique with a scan thickness of not more than 3 mm is optimal for MRI diagnostics of orbital hemangiomas.

Orbital pulsatile metastasis as initial presenting sign of metastatic clear cell renal carcinoma.

CASE REPORT: We present the case of a 56 year-old male with a pulsating lesion in the temporal region of the left orbit as presenting sign of a renal cell carcinoma. A review is also presented of all cases of orbital metastases diagnosed in our hospital between 1957 and 2012. DISCUSSION: Carcinoma is the most common malignancy involving the kidney but, only rarely does it metastasize to the orbit. As these tumors can be confused with other amelanotic or vascular tumors, a high index of suspicion is required for early detection and management of the primary tumor.

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.

A case of MPO- and PR3-ANCA-positive hypertrophic cranial pachymeningitis with elevated serum IgG4.

We report a case of orbital tumor and hypertrophic cranial pachymeningitis in a 64-year-old woman, who was initially suspected to have IgG4-related disease because of an elevated level of serum IgG4 at onset. However, her condition was resistant to glucocorticoid therapy, and additional cyclophosphamide was necessary to control the disease activity. Additional features included elevated levels of serum myeloperoxidase (MPO) and proteinase-3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and biopsy specimens from the orbital mass revealed very few infiltrating IgG4-positive cells. Instead, rupture of the elastic layer of the arterial walls with neovascularization and a small number of giant cells were observed. Considering these findings and the clinical course, the disease was considered more likely to be ANCA-associated pachymeningitis with elevation of the serum IgG4 level.

An analysis of flow dynamics in cerebral cavernous malformation and orbital cavernous angioma using indocyanine green videoangiography.

BACKGROUND: Cerebral cavernous malformations (CCMs) are known to be vascular anomalies with low perfusion because of being angiographically occult. We attempted direct visualization of blood flow within CCMs and orbital cavernous angiomas (CAs), and analyzed flow dynamics using indocyanine green videoangiography (ICG-VAG). METHODS: This series included seven CCMs and two orbital CAs. ICG-VAG was performed to visualize blood flow of the lesions before resection. Time to peak of staining was evaluated by reviewing recorded ICG-VAG. RESULTS: In five of seven CCMs, stain was identified. CCMs were seen as avascular areas in both arterial and venous phases, and were stained gradually. Stain was maximized late after venous phase. The orbital CAs were also stained lately, but more intensely than CCMs. CONCLUSIONS: The present study directly demonstrated slow and low perfusion within CCM and orbital CA using ICG-VAG. On the basis of characteristic flow dynamics of CCMs, intraoperative ICG-VAG provides useful information in microsurgical resection.

High-flow hemangioblastoma of the orbit.

A 62-year-old Chinese man was found to have a mass in the right superior orbit and mild fullness in right cavernous sinus associated with minimal proptosis. Carotid artery angiography revealed a vascular tumor in the superior orbit with supply from the internal and external carotid arteries. Selective embolization of the feeder vessel was performed before surgical excision of the tumor. Histopathologic examination with light microscopy and immunohistochemistry confirmed the tumor to have characteristics of a hemangioblastoma. Hemangioblastoma involving the orbit is exceedingly uncommon and to date only one such case has been documented in the literature.

Natural history of periocular capillary haemangiomas: changes in internal blood velocity and lesion volume.

AIM: Although the clinical characteristics of childhood periocular capillary haemangiomas are well known, serial measurements of blood velocity and lesion size are unknown. This investigation was designed to measure the changes in maximum blood velocity and estimated size of lesion in children with capillary haemangioma not requiring active intervention. STUDY DESIGN: Retrospective case-note review for a cohort of children with capillary haemangioma involving the eyelid and orbit. PATIENTS AND METHODS: Children with periocular capillary haemangioma, under the care of the Orbital unit at Moorfields Eye Hospital between 1996 and 2005, were monitored clinically and with repeated ultrasonographic examination. Volume estimates were calculated as an ovoid based on the three maximum orthogonal measurements for the haemangioma, and blood velocity was assessed by Colour Flow Mapping, Colour Doppler Energy Imaging, and Spectral Doppler techniques using a Sequoia 512 Acuson scanner. RESULTS: Twenty-four children (12 boys) had initial assessment by 18 months of age, and the haemangioma increased in size in 14/24 (58%), the increase being between 4 and 931% of initial volume estimate. The largest measured size for an individual haemangioma appears inversely related to the child's age at measurement, this mirroring a similar trend in measurements for the maximum blood velocity. Blood velocity measurements also tend to decrease with time, the peak velocity being before 1 year of age in the majority (15/24; 62%). In many children, both volume estimates and blood velocities show a cyclic variation-this occurring with increasing intervals between the maxima, before a final decay in both parameters. Although, for the whole group, there was no correlation (correlation coefficient=0.29) between estimated size and measured blood velocity, some individual children showed a significant correlation between the two parameters. The age at maximum blood velocity appeared to precede the age at maximum volume in most children, and in many there was an orbital anomaly detectable on ultrasonographic examination, even with complete clinical resolution of the haemangioma. CONCLUSIONS: Ultrasonographic examination of periocular capillary haemangiomas show that these lesions have a very high blood velocity in feeding vessels-about 2-3 orders of magnitude greater than normal capillary beds-and that the velocity and volume of such lesions undergo a cyclic variation during their natural history. Evidence suggests that both velocity and volume decrease with time, although often not returning to zero on ultrasonography (unlike the clinical resolution of the lesions). In most children, blood velocity peaks before volume estimates and this might suggest that decreasing perfusion leads to later tissue atrophy and involution of the haemangioma.

Sclerosing therapy as first line treatment for low flow vascular lesions of the orbit.

PURPOSE: To evaluate the outcome of sodium morrhuate 5% injections in patients with low flow vascular lesions, which consist of orbital lymphangiomas, and in one patient with intraosseous cavernous hemangioma. DESIGN: Prospective, interventional consecutive case series. METHODS: Intralesional sodium morrhuate 5% was injected under direct visualization or under radiographic guidance to six patients with orbital lymphangiomas and one patient with intraosseous cavernous hemangioma. Comprehensive eye examination and follow-up imaging studies were performed. main outcome measures: Lesion size was evaluated by orbital imaging and clinical examination, visual acuity, exophthalmos, and posttreatment complications. RESULTS: Seven patients (four female, three male; average age, 33 years) were included. Six patients were diagnosed with orbital lymphangioma, and one patient was diagnosed with intraosseous cavernous hemangioma. Patients received an average of 2.6+/-2 intralesional injections of sodium morrhuate, with a range of one to six injections and a mean volume of 0.9+/-0.8 ml (range, 0.2 to 2.1 ml). Lesions showed a decrease in size an average of 50% (33%) and ranged from minimal (10%) to near total resolution (85%). Visual acuity and intraocular pressure remained unchanged; exophthalmos decreased an average of 1.5+/-1.8 mm. Complications included one case of orbital hemorrhage that resolved spontaneously and transient keratopathy in all patients with anterior orbital lesions. CONCLUSION: Intralesional sclerosing therapy with sodium morrhuate 5% is effective in tumor debulking in patients with orbital lymphangioma and is not associated with vision-threatening complications. It may be a better alternative to surgery for low flow orbital tumors, which includes lymphangioma.

Metastatic tumor of orbit presenting as pulsatile proptosis.

Pulsatile proptosis is caused by vascular lesion of orbit or by herniation of brain through bony defect in orbit. Pulsatile proptosis secondary to a metastatic tumor of orbit is very uncommon. We report a 69-year-old male who presented with pulsatile proptosis owing to metastasis of an asymptomatic renal cell carcinoma.

Technetium tc 99m-labeled red blood cells in the preoperative diagnosis of cavernous hemangioma and other vascular orbital tumors.

OBJECTIVES: To evaluate technetium Tc 99m (99mTc) red blood cell scintigraphy as a diagnostic tool for orbital cavernous hemangioma and to differentiate between orbital masses on the basis of their vascularization. METHODS: We performed 99mTc red blood cell scintigraphy on 23 patients (8 female and 15 male; mean age, 47 years) affected by an orbital mass previously revealed with computed tomography (CT) and magnetic resonance imaging (MRI) and suggesting cavernous hemangioma. In our diagnosis, we considered the orbital increase delayed uptake with the typical scintigraphic pattern known as perfusion blood pool mismatch. The patients underwent biopsy or surgical treatment with transconjunctival cryosurgical extraction when possible. RESULTS: Single-photon emission tomography (SPET) showed intense focal uptake in the orbit corresponding to radiologic findings in 11 patients who underwent surgical treatment and pathologic evaluation (9 cavernous hemangiomas, 1 hemangiopericytoma, and 1 lymphangioma). Clinical or histologic examination of the remaining 22 patients revealed the presence of 5 lymphoid pseudotumors, 2 lymphomas, 2 pleomorphic adenomas of the lacrimal gland, 1 astrocytoma, 1 ophthalmic vein thrombosis, and 1 orbital varix. CONCLUSIONS: The confirmation of the preoperative diagnosis by 99mTc red blood cell scintigraphy shows that this technique is a reliable tool for differentiating cavernous hemangiomas from other orbital masses (sensitivity, 100%; specificity, 86%) when ultrasound, CT, and MRI are not diagnostic. Unfortunately, 99mTc red blood cell scintigraphy results were positive in 1 patient with hemangiopericytoma and 1 patient with lymphangioma, which showed increased uptake in the lesion on SPET images because of the vascular nature of these tumors. Therefore, in these cases, the SPET images have to be integrated with data regarding clinical preoperative evaluation and CT scans or MRI studies. On the basis of our study, a complete diagnostic picture, CT scans or MRI studies, and scintigraphic patterns can establish the preoperative diagnosis of vascular orbital tumors such as cavernous hemangioma, adult-type lymphangioma, and hemangiopericytoma.

CT arteriography for orbital tumors: diagnostic and surgical value.

The aim of this study is to investigate the efficacy of dynamic computed tomography (CT) during selective angiography (CT-arteriography) of orbital tumors in the evaluation of intratumoral vascular anatomy, feeding artery territory, and histological diagnosis. Among 35 consecutive cases with various orbital lesions, those cases showing tumor staining or pooling of the contrast medium on digital subtraction angiography (DSA) were evaluated by CT-arteriography (n = 14). The information obtained by CT-arteriography was compared with that provided by enhanced MRI (n = 31) and dynamic MRI (n = 21), in which the contrast medium was injected intravenously. In addition to the visualization of fine vascular anatomy, CT-arteriography emphasized areas of nodular enhancement and non-enhancing cystic/necrotic components as well as the intratumoral feeding arteries. Patterns of CT-arteriography were categorized into three subgroups: homogeneous enhancement (benign lymphoid lesion), partial enhancement (schwannomas and carcinomas), and patchy multinodular enhancement (specific for cavernous angiomas). In addition, CT-arteriography with selective arterial catheterization clearly delineated the feeding artery territories. CT-arteriography, with a minimal dose of contrast medium, can offer significant advantages over intravenously injected dynamic neuroimaging, and provides additional valuable preoperative information about the orbital tumor under investigation.

[Ultrasonic tomography of neurogenic tumors of the orbit]. / Ul'trazvukovaia tomografiia neirogennykh opukholei orbity.

The results of a comprehensive ultrasonic examination of 12 patients with tumors of the eye orbit are described. A low echogenicity as well as heterogeneity of the echo-structure and of the contours' sharpness are the main sonographic features of the mentioned tumors. An examination of a vascularization degree in patients with gliomas of the optic nerve showed no blood circulation inside tumors; while a collateral blood circulation was detected in patients with meningiomas of the optic nerve. A compression of the ocular artery by the tumor and reduced hemodynamic indices were found to be typical. Finally, a triplex examination of the orbit makes it possible to diagnose tumors of the optic nerve.