Case report: EBV-related eye orbits and sinuses lymphohistiocytic infiltration responsive to rituximab in a patient with X lymphoproliferative syndrome type 1.

Background and aims: X lymphoproliferative syndrome type 1 (XLP1) is a rare inborn error of immunity due to mutations of SH2D1A, encoding for slam-associated protein (SAP). The clinical phenotype includes severe mononucleosis, hemophagocytic lymphohistiocytosis (HLH), and B-cell lymphomas. Methods: We report the case of a child affected with XLP1 who presented with an incomplete HLH, triggered by Epstein-Barr virus (EBV) and treated with rituximab, involving orbits and paranasal sinuses. Results: The lesion was indistinguishable from lymphoma, complicating diagnosis and treatment. In addition, considering the high incidence of lymphoma in patients with XLP1, histology helped define its nature, driving therapeutic choices. Conclusion: We described an unusual presentation of incomplete HLH in a patient affected with XLP1: an EBV-driven infiltration of the orbits and paranasal sinuses. This led us to a challenging differential diagnosis of lymphoma-associated hemophagocytic syndrome, which can be frequently observed in patients with XLP1. Considering the extremely poor prognosis of this clinical finding, we sought for a prompt diagnosis and managed to obtain it and to immediately establish the right treatment on the basis of the pathological finding.

Primary Orbital Respiratory Epithelial Cyst: A Systematic Review and Two Case Reports.

A primary orbital respiratory cyst is a congenital choristoma that presents in the orbit and with different signs and symptoms depending on the location, which might also change the surgical approach. The aim of this report is to describe 2 new cases of primary respiratory epithelial cysts and to review the literature on presentation, management, and risk factors with different surgical approaches and complications. Two cases presenting with gradually increased proptosis had a confirmed diagnosis of a respiratory epithelial cyst. CT and MRI imaging revealed a thin-walled intraconal cystic lesion. Complete surgical removal was not possible and an incisional biopsy of the wall was performed. Rarely, orbital respiratory epithelium cysts are primary lesions. The high risk of breaking the capsule during surgery and the presence of residual epithelial cells within the orbit may cause cyst's recurrence. Indeed, we recommend careful attention during dissection maneuvers and a long-term follow-up.

Management of the skull base invasion in spheno-orbital meningiomas.

BACKGROUND: The tumor invasion of the skull base structures is very frequent in spheno-orbital meningiomas. The aim of the present study is to evaluate the invasion rate of skull base structures and the best surgical approach and management. METHODS: The surgical series of 80 spheno-orbital meningiomas was reviewed. The tumors were classified according to the intraorbital location with respect to the optic nerve axes into three types: I-lateral: II-medial; III-diffuse. The invasion of the orbital apex, optic canal, superior orbital fissure, anterior clinoid, ethmoid-sphenoid sinuses, and infratemporal fossa was evaluated. The rate and extension of involvement of these structures was correlated with the intraorbital location and the surgical approach. The preoperative ophtalmological symptoms and signs and their outcome were also evaluated. RESULTS: Proptosis was found in 79 patients (97%), variable decrease of the visual function in 47 patients (59%), and deficits of the eye movements in 28(35%). The invasion of the optic canal (74%), superior orbital fissure (65%), anterior clinoid (60%), and orbital apex (59%) was more frequently found, whereas the tumor extension into the ethmoid-sphenoid sinuses (4%) and infratemporal fossa (4%) was rare. Types II and III meningiomas showed significantly higher involvement of the skull base structures than type I ones, which only had 15% invasion of the optic canal. Remission or significant improvement of the visual function occurred postoperatively in 24 among 47 cases (51%), with a higher rate for type I meningiomas vs. other types (p = 0.021, p = 0.019) and worsening in 7 (15%). CONCLUSIONS: Spheno-orbital meningiomas growing in the lateral orbital compartment show no involvement of the skull base structures excepting the optic canal as compared to those growing medially or diffusely. The surgical resection of tumor invading the skull base structures should be more extensive as possible, but the risk of optic and oculomotor deficits must be avoided.

Recurrences of Spheno-Orbital Meningiomas: Risk Factors and Management.

BACKGROUND: The extent of resection constitutes one of the most important predictive risk factors of recurrence for spheno-orbital meningiomas; although gross total resection represents the gold standard, it is not always achievable, with a consequent high rate of recurrence. Management of these tumors is a surgical challenge and is represented by maximal safe resection with preservation of function. The aim of the present study is to discuss the risk factors for recurrence and the best management of the recurrent tumors. METHODS: Eighty patients operated on for spheno-orbital meningiomas were retrospectively reviewed. Two groups were identified: group 1 comprised 30 patients (37.5%) who experienced recurrence and group 2 comprised 50 patients with no recurrence from 5 to 28 years after the initial surgery. The analyzed factors in both groups include the involvement of the skull base structures, the extent of resection, and World Health Organization grade. In the recurrence group, the pattern of tumor regrowth, the entity of resection, and the management were also analyzed. RESULTS: The invasion of the whole orbit, the involvement of the orbital apex, superior orbital fissure, infratemporal fossa, and sphenoidal-ethmoidal sinuses, and World Health Organization grade II are risk factors of recurrence. All 30 patients with recurrence underwent reoperation, 9 of whom had 2 or 3 reoperations. Overall, 70 of the 80 patients (88%) had tumor control and no progression after one or more reoperations. CONCLUSIONS: We suggest re-surgery for spheno-orbital meningioma recurrences to prevent worsening of visual function and proptosis. Because of their slow natural course, even multiple reoperations may be performed, resulting in long overall survival with stable symptoms and good quality of life.

Outcomes and risk factors of surgical management of thyroid eye disease-related diplopia.

OBJECTIVES: To evaluate the outcomes of strabismus surgery in patients with thyroid eye disease (TED) and to assess the influence of preceding treatments such as steroid administration, orbital decompression and radiotherapy. METHODS: Retrospective study on a cohort of patients with TED undergoing strabismus surgery. Data collection included clinical features, smoking status, preceding steroid therapy, orbital radiation therapy and orbital decompression. Qualitative evaluation, assessing diplopia and quality of life and quantitative evaluation, assessing the eye misalignment were analysed 1 year after the last squint surgical procedure. RESULTS: Seventy-nine patients were identified. Ninety-five surgical procedures were performed on 92 eyes. A significant overall improvement of ocular motility was registered (p < 0.001, paired t-test). Forty-five (57%) patients had excellent outcomes, twenty-three (29%) had good outcomes and eleven (14%) had poor outcomes. The average number of muscles operated was significantly greater in patients who underwent orbital bone decompression (1.58 ± 0.63; p = 0.0082; 95% CI 1.413-10.214). Quantitative and qualitative outcomes were not associated with the preceding therapy: steroid (p = 0.75), radiotherapy (p = 0.95) or orbital bone decompression (p = 0.25). The success rate was no different between adjustable and fixed sutures (p = 0.8). CONCLUSION: Strabismus surgery in TED patients resulted in a high success rate in reducing diplopia and improving ocular motility. The success rate was not negatively associated with previous therapies. Indeed, resolution of diplopia required more squint procedures in patients who have previously undergone to orbital decompression.

Conjunctival Carcinomas Arising in the Anophthalmic Socket.

PURPOSE: The aim of this study was to report management and outcomes of patients in which conjunctival carcinomas arose in the anophthalmic socket. METHODS: The authors retrospectively analyzed the data of patients which presented the anophthalmic socket after surgery. The clinical records of 4 patients with histological diagnosis of conjunctival carcinoma in the anophthalmic socket, referred to our Department, between January 2014 and December 2019 was collected. RESULTS: The study included 4 men (median age 58 years). A previous enucleation surgery had been performed in all patients due to previous ocular trauma for three patients (Case 1, 3, and 4) and due to a retinoblastoma for 1 patient (Case 2). All patients underwent surgical excision of the lesion and only 1 patient (case 3) underwent orbital exenteration due to the high-grade of the cancer. The most common tumor was the squamous cell carcinoma (2 cases) followed by 1 case of high-grade mucoepidermoid carcinoma and 1 case of carcinoma in situ. Systemic work-up was performed for all the patients. Conjunctival TNM, according to the American Joint Committee on Cancer TNM staging system was used to stage the lesions. The median follow-up time was 15 months. CONCLUSION: Considering the severity and rarity of these tumors, a careful ophthalmological examination of the cavity and a correct revision of the prosthesis should be mandatory in order to ensure the elimination of any macro and microscopic irregularities that can then in the long-term cause chronic inflammation of the tissues and consequently promote the growth of carcinomas.

A rare case of bilateral conjunctival Kaposi's sarcoma in a HIV-negative patient.

PURPOSE: Kaposi's sarcoma (KS) is a rare multi-centric vascular neoplasm, first described by Moritz Kaposi in 1872. It can appear in four different forms: classic, endemic, HIV-related and post-transplant form. We present an uncommon case of seronegative HIV infection patient with skin KS involving conjunctiva of both eyes. OBSERVATIONS: Firstly, right eye lesions were surgically removed. Subsequently, the patient was administered with systemic chemotherapy (doxorubicin) to treat both skin and left eye lesions. No signs of recurrence were observed at 20 months' follow-up. CONCLUSIONS: Both eyes involvement in KS is rarely described in scientific literature. Both surgery and chemotherapy could be considered valid treatment options for conjunctival KS.

Risks and Benefits of Surgical Excision of Orbital Cavernous Venous Malformations (So-Called Cavernous Hemangioma): Factors Influencing the Outcome.

PURPOSE: To assess risks and benefits associated with surgical excision of orbital cavernous venous malformation and analyze factors influencing the outcome. METHODS: Design: cohort study. Participants: 164 consecutive patients undergoing surgical removal of orbital cavernous venous malformation. Intervention: lateral or anterior orbitotomy. Outcome measures: visual acuity, visual field, pupillary function, ocular motility, and fundus examination. Influence of the mass volume and tumor location on the outcome was evaluated. The threshold value and risk group for the volume were evaluated using the receiver operating characteristic analysis; 8 orbital different quadrants, extraconal/intraconal position, and orbital apex extension were considered as variables to evaluate the location. RESULTS: Postoperatively overall visual acuity (p = 0.0084), visual field (p < 0.0001), diplopia (p < 0.0001), proptosis (p < 0.0001), ocular motility (p = 0.02), ptosis (p = 0.033), choroidal fold (p < 0.0001), disk edema (p = 0.0004) were significantly improved. The commonest location was the lateral quadrant (28%). Choroidal folds were associated with tumor greater than 1100 mm3 (p = 0.001). Visual loss occurred in 2 patients (1.2%) and tonic pupil in 5 (3%,) Visual deterioration was associated with the apical extension (p = 0.001). Pupil efferent defect was associated with lateral quadrant location (p = 0.011) and apical extension (p = 0.05). CONCLUSIONS: Surgical removal of orbital cavernous venous malformation is a useful treatment modality, particularly for large size tumors. Removal of cavernous venous malformations located at the lateral aspect of the orbit carries an increased risk of permanent tonic pupil. Postoperative blindness is rarely seen and is often associated with the apical extension. Tailoring the surgical approach, depending on the relative position to the optic nerve, may have an important bearing on outcome.

The role of ultrasonography in differential diagnosis of orbital lesions.

AIMS: The aim of this study is to assess the value of ultrasound in the differential diagnosis of orbital lesions. METHODS: One hundred and thirty-eight patients with clinical features of an orbital mass were examined by orbital ultrasound prior to undergoing surgery, from January 2000 to January 2017. All patients underwent excisional or incisional orbital biopsy. The results of orbital ultrasonography were compared with the final histological diagnosis. RESULTS: Orbital lesions were localized by ultrasonography in 133/138 cases (96.4%); the false-negative rate of orbital echography was 3.6% (5/138). The nature of the orbital lesions was correctly determined by ultrasonography in 54.3% of the cases (75/138) preoperatively (true positives). In 58/138 (42%) patients, there was no correspondence between the ultrasound diagnosis and the histological diagnosis (false positives). The sensitivity of orbital ultrasonography for the detection of an orbital mass was 93.75% (CI 87.87-99.63%), while the specificity yielded no meaningful result (CI 0.00-60.24%). Moreover, the specificity of orbital ultrasonography to identify a malignant tumor falls within a CI of 0-62.72%. CONCLUSIONS: Orbital ultrasonography is a rapid and noninvasive test that is highly sensitive in displaying an orbital mass; however, the specificity in the differential diagnosis of orbital lesions is not meaningful, particularly if malignancy is suspected. The assessment of orbital diseases requires multiple diagnostic approaches to balance the strengths and weaknesses of each method.

Eyelid sebaceous gland carcinoma: An assessment of the T classification of the American Joint Committee of Cancer TNM staging system 8th versus 7th edition.

PURPOSE: To assess the prognostic values of the T classification of the 8th edition of the American Joint Committee of Cancer staging system and compare it to the 7th edition. METHODS: Multicenter retrospective study of patients with eyelid sebaceous gland carcinoma. The primary outcome measure was the differences between outcomes when tumors were staged with either 7th or 8th edition. The measures evaluated included presenting features, management, histopathology, metastasis, recurrence, and mortality. RESULTS: Of the 60 patients (median age 73 years), 31 (51.7%) were females. A change in T staging occurred in 39 patients (65%) when the 8th edition was applied. Advanced categories (T3/T4) were significantly associated with nodal metastasis (p = 0.037) using the 8th edition criteria but not with the 7th edition (p = 0.066). The 8th edition T categorization significantly correlated with eye survival (p = 0.022) while the 7th edition did not (p = 0.058). Applying the 8th edition, category T4 at presentation was associated with a higher risk of nodal metastasis (p = 0.037) but not associated with local recurrence, distant metastasis, or tumor-related death (p = 0.281, p = 0.737, p = 0.319, respectively). T3/T4 category tumors were significantly associated with poor tumor differentiation (p = 0.001), and papillary histologic pattern (p = 0.024) but not with pagetoid spread (p = 0.056). CONCLUSION: The application of the 8th edition AJCC staging system for eyelid SGC may accurately predict nodal metastasis. Local recurrence and distant metastasis were not significantly associated with T classification, using either edition. Poor tumor differentiation and papillary pattern were associated with T3/T4 categories suggesting that pathological features may assist in determining prognosis.

Relation of the eighth edition of the American Joint Committee on Cancer staging system with histological risk classification for primary eyelid basal cell carcinoma.

PURPOSE: To investigate the relation between the eighth edition of the American Joint Committee on Cancer staging system and histological risk classification for primary eyelid basal cell carcinoma. METHODS: Retrospective, observational case series of patients undergoing excisional biopsy for primary eyelid basal cell carcinoma in two tertiary centres between 2008 and 2018. Patients with <6 months of follow-up were excluded. Outcomes measured included histological subtype, American Joint Committee on Cancer 7 and 8 staging. RESULTS: A total of 222 cases were included over a 10-year period, with a mean (range) follow-up of 25.74 (6-120) months and a median (range) age of 70 (28-93) years. According to American Joint Committee on Cancer 8, the most common T category was T1a (64%), followed by T1b (18%) and T2a (8%). Of the 222 specimens, 183 (82.43%), 17 (7.66%), 19 (8.56%) and 3 (1.35%) were staged as IA, IB, IIA and IIB, respectively. The most common histological subtype was nodular in IA category and infiltrative in categories IB and IIA. Histologically, low-risk basal cell carcinomas were related to lower American Joint Committee on Cancer staging (IA), whereas high-risk basal cell carcinomas were related to American Joint Committee on Cancer stages IB and IIA (p < 0.001). No significant relation was found between T categorisation and risk stratification when adopting American Joint Committee on Cancer 7. CONCLUSION: American Joint Committee on Cancer 8 staging system is strongly related to primary eyelid basal cell carcinoma histological risk classification.

Primary cutaneous anaplastic large-cell lymphoma of the eyelid: report of two cases and review of the literature.

PURPOSE: Two new cases of primary cutaneous CD30+ anaplastic large-cell lymphoma (cALCL) of the eyelid are reported; these are analysed alongside existing cases to identify challenges relating to the diagnosis and management of such rare lesions. MATERIAL AND METHODS: A review of existing literature on the PubMed database is conducted using the keywords: 'eyelid lymphoid proliferations', 'lymphoma of the eyelid', and 'primary cutaneous CD30+, ALK-anaplastic large-cell lymphoma of the eyelid'. Two new cases of cALCL are reported. Cases where patients present solely with a nodular periocular lesion are analysed for recurrence and survival rate. RESULTS: Two new patients with a painless ulcerated nodule on the upper eyelid receive a confirmed diagnosis of cALCL after undergoing an excisional biopsy. The first, elderly patient has spontaneous remission; the second patient, with a concomitant chronic infection of hepatitis C virus (HCV), presents a more diffuse disease at the onset and requires radiotherapy. Together with 13 patients a primary cALCL identified from 11 previous studies, this constitutes a cohort of 15 patients. Of these, 10 present with an exclusively nodular lesion of the eyelid and four experience disease recurrence; no deaths from cALCL are reported. CONCLUSION: Differential diagnosis between primary cALCL and lymphomatoid papulosis is essential and requires careful consideration of clinical and pathologic features. Radiologic staging examination is crucial in order to exclude systemic ALCL, particularly for patients with comorbidity. Though cALCL has the pathological features of a malignant lesion, the prognosis seems favourable for patients; a relatively high percentage even experience spontaneous resolution.

Orbital and Eyelid B-Cell Lymphoma: A Multicenter Retrospective Study.

BACKGROUND: The aim of this study was to analyze patients diagnosed, staged and treated for orbital and eyelid B-cell lymphoma (OEL). METHODS: One hundred and forty-one cases of OEL were included in this study. Primary endpoints were to analyze the histopathologic findings, the main risk factors and the type of treatment and to correlate them with recurrence of OEL. The secondary endpoint was to determine the progression-free survival (PFS) time. RESULTS: Extranodal marginal zone B-cell lymphoma was the most frequent subtype (66%), followed by small lymphocytic lymphoma (12.7%), diffuse large B-cell lymphoma (DLBCL) (9.2%), follicular lymphoma (6.6%), mantle cell lymphoma (4.3%) and Burkitt lymphoma (1.2%). The probability of relapse was influenced by the histopathologic subtype DLBCL (OR = 7.7, 95% CI 1.8-32.3) and treatment with chemotherapy (OR = 14.9, 95% CI 2.6-83.7). Multivariate analysis showed that the histopathologic subtype DLBCL and chemotherapy treatment retained statistical significance for a poorer PFS, with hazard ratios of 8.581 (p = 0.0112) and 9.239 (p = 0.0094), respectively. CONCLUSIONS: Five lymphoma subtypes were found in patients with OEL. The histopathologic subtype and the type of treatment were found to be the main factors influencing treatment outcome.

Orbital solitary fibrous tumors: a multi-centered histopathological and immunohistochemical analysis with radiological description.

BACKGROUND: Solitary fibrous tumors (SFT), formerly called hemangiopericytoma, are rare tumors derived from mesenchymal cells originally described in the pleura, but these tumors may affect extraserosal tissues including the lacrimal gland and orbit. OBJECTIVE: Conduct a multi-centered clinical, radiological and histopathological analysis of 17 orbital SFT cases. DESIGN: A retrospective case series. SETTING: Three eye centers in two countries. PATIENTS AND METHODS: The data collected from the charts of 17 adult patients presenting with tissue diagnosis of orbital hemangiopericytoma or SFT from January 2003 to December 2018 included demographics, clinical imaging and histopathological information including immunohistochemical (IHC) characteristics. MAIN OUTCOME MEASURES: The demographic characteristics, clinical presentation, and histopathological patterns or variants of SFT were analyzed. SAMPLE SIZE: 17 adult patients. RESULTS: Mean age was 45 years (range 23-80 years). Male to female ratio was 3:1. The right eye was affected in 12 (70.5%) patients. Commonest presentation was proptosis in 13/17 (76% of patients). Other symptoms were impaired motility (29%) and ptosis (11%). Lesions mostly affected the medial orbit (35%), then orbital apex in 11%. The histopathological classic pattern-less variant was the commonest. One case with aggressive behavior, multiple recurrences and atypical features was encountered. Immunohistochemical (IHC) markers used included CD34 expression in all cases, Bcl-2 expression in 10/11, CD99 in 9/9 and Vimentin in 4/4. STAT6 was used in 2 cases. CONCLUSIONS: SFTs are rare tumors affecting the orbit in both genders equally in their mid-forties, but showed male predominance in our analysis with a predominant classic histopathological pattern. Tissue diagnosis is essential and requires IHC studies for confirmation. LIMITATIONS: Sample size is relatively small owing to the rarity of this tumor in the orbit. CONFLICT OF INTEREST: None.

Multi-parametric magnetic resonance imaging characterization of orbital lesions: a triple blind study.

Background: Multi-parametric MRI used for preoperative assessment of orbital lesions does not routinely include DCE-MRI, since its accuracy in differential diagnosis of orbital mass is still under debate. Aim of this study is to characterize orbital lesions by multi-parametric MRI, analysing the incremental predictive value of DCE-MRI in differential diagnosis of orbital lesions.Methods: In this prospective triple-blind study, 43 consecutive patients with unilateral orbital lesion underwent conventional multimodal MRI and DCE-MRI before biopsy in a tertiary referral centre. Pre-operative MRI examination including conventional unenhanced MRI protocol, DWI with ADC maps, static CE 3D-T1 w and dynamic CE T1 w sequences, was performed within 1 week from surgery (anterior/lateral orbitotomy depending on location of the lesion, to carry out incisional/excisional biopsy).Results: Comparison between conventional T1 w/T2 w, DWI, CE 3D-T1 w and DCE-MRI groups showed a statistically significant difference in scores distribution (p < .001). Statistically significant difference was found between conventional T1 w/T2 w and DWI (p < .005), as well as between DWI and CE 3D-T1 w (p < .001). Conversely, no significant difference was found between CE 3D-T1 w and DCE (p < .005).Conclusions and Relevance: This study confirmed the positive effect of DWI and CE 3D-T1 w on orbital lesions diagnosis when added to conventional T1 w/T2 w sequences, whereas no substantial impact on diagnostic performance was observed with the further addition of DCE-MRI. DCE does not strongly influence diagnostic performance and inter-rater agreement in characterizing orbital lesions; therefore, it should be recommended in selected patients whose assessment of flow dynamics is particularly useful for management.Abbreviations: US = ultrasonography; MRI = magnetic resonance imaging; CT = computed tomography; STIR = Short-TI Inversion Recovery; DWI = diffusion weighted imaging; DCE-MRI = dynamic contrast-enhanced MRI; SE = Spin-Echo; TSE = Turbo Spin-Echo; THRIVE = T1-weighted high resolution Isotropic Volume Examination (dynamic contrast-enhanced ultrafast spoiled gradient echo); ROI = regions of interest; IRR = inter-rater reliability; TIC = time-intensity curve.

Upper Eyelid Isolated Arterio-Venous Malformation Treated With Embolization in a Patient With Keloid-Prone Skin.

Ocular adnexal aterio-venous malformations (AVMs) are rare congenital disabling anomalies, which may enlarge causing disfiguring deformity and rarely severe hemorrhage. These lesions are generally treated by preliminary endovascular embolization to shrink the arterio-venous malformation, followed by surgical gross total resection. The authors report a case of eyelid arterio-venous malformation in a 12-year-old girl, which progressively increased in size in few months. The patient complained mild itching, blurring of the vision, and mild tenderness. Magnetic resonance imaging showed an expansive mass with multiple arterial vessels at the left superior eyelid and left forehead. The diagnosis of arterio-venous malformation was then confirmed by digital subtraction angiography. Primary surgical excision was excluded because of the high risk of intrasurgical bleeding. Embolization through superselective cannulation of the left external carotid feeder vessels was performed resulting in flow exclusion up to the 80% of the nidus. Subsequent surgical resection was not recommended due to clinical evidence of keloid-prone skin.

Hepatitis C virus in MALT-lymphoma of the ocular adnexa.

OBJECTIVE: Hepatitis C virus (HCV) has been proposed as a possible etiologic factor in ocular adnexal marginal zone lymphoma (OAML). We aimed to assess the prevalence of HCV infection in patients with OAML through a systematic review and meta-analysis. METHODS: Electronic databases were searched from their inception to August 2019 for studies assessing HCV seroprevalence in patients with OAML. Pooled prevalence of HCV infection was calculated with 95 % confidence interval (CI). Statistical heterogeneity among studies was quantified via the inconsistency index (I2). Funnel plot symmetry was used to assess the risk of bias across studies. RESULTS: Nine studies with 360 patients were included. Overall pooled prevalence of HCV in OAML was 12.7 %, with low statistical heterogeneity (I2 = 17.4 %) and with asymmetrical funnel plot. The studies clustered into two groups: 5 studies (3 from Italy and 2 multicenter with a major Italian contribution) showed a higher HCV prevalence in OAML (15.6 %), while the other 4 (from countries other than Italy) showed a lower prevalence (4.7 %); in both subgroups, statistical heterogeneity was null (I2 = 0%) and funnel plot was symmetrical. CONCLUSION: HCV might be a significant etiologic factor of OAML in Italy.

Outcomes after eye-sparing surgery vs orbital exenteration in patients with lacrimal gland carcinoma.

BACKGROUND: This study examined whether eye-sparing surgery is associated with better or worse outcomes than exenteration for the treatment of lacrimal gland carcinomas. METHODS: Forty-six patients treated for lacrimal gland carcinoma were retrospectively reviewed and compared. A statistical analysis was performed using Kaplan-Meier plots. RESULTS: The overall survival rates for eye-sparing surgery were 52% and 37% at 5 and 10 years, and those for exenteration were 37% and 25% at 5 and 10 years, respectively (P = .73). The proportion of patients with local regional control at both 5 and 10 years after eye-sparing surgery was 0.75, and that for exenteration was 0.47 (P = .30). For eye-sparing surgery, the proportions of distant metastasis-free survival at 5 and 10 years were 0.51 and 0.39 for eye-sparing surgery and 0.29 and 0.14 for exenteration (P = .50). CONCLUSION: Because the outcomes were not significantly different, the authors suggest that eye-sparing surgery can be proposed as a reasonable approach for lacrimal gland carcinomas in appropriately selected patients.

Epidemiological Analysis of Venous Malformation of the Orbit.

Orbital venous malformation (OVM) is one of the most common orbital mass of adults, accounting for approximately 5% to 15% of all vascular abnormalities in the central nervous system and representing 9% of orbital lesions. Clinical presentation is variable from asymptomatic cases to symptomatic ones. The purpose of the authors' work is to analyze OVM incidence and prevalence in a cohort of patients referred over the last 40 years to the Orbital Unit, tertiary center for orbital diseases.Records of 224 patients diagnosed with CVM in a 40-year period were retrospectively reviewed extracting prevalence/year and incidence/year based on data obtained from Eurostat population statistics. Data were aggregate and analyzed with a Microsoft Excel (Microsoft Corporation, Redmond, WA) spread sheet.The incidence rate was measured as the number of new cases of OVM observed in Orbital Unit, a tertiary center. Incidence was about 1 case out (0.94) every 10 million people. The incidence rate was measured also as the number of new cases of OVM observed in Campania, considering in the latter case patients residents in this region (112 patients). Incidence was less than 1 case out (0.58) every 10 million people.The prevalence rate was measured as the proportion of individuals with OVM observed in our Orbital Unit and is equal to 5%. This indicator resulted to increase from 1977 to 2017.The management of OVM is still under discussion, but in agreement with data present in the literature the authors still believe that the surgical indication is only for symptomatic lesions.