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BackgroundChordoma is a rare malignant tumor of the skull base and axial skeleton, with an incidence of less than 0.1/100,000 per year. Patients with advanced chordoma have a poor prognosis due to locoregional recurrence with infiltration and destruction of surrounding bone and soft tissue. Cytotoxic chemotherapy or other systemic therapies have not been proven to be effective for these diseases. Therefore, several molecularly targeted therapies have been proposed as potentially beneficial, including tyrosine kinase inhibitors such as imatinib, sorafenib, lapatinib, and others.Case presentationWe present three cases of advanced chordoma treated with molecular targeted therapies: a 52-year-old Caucasian man, a 72-year-old Caucasian woman, and a 38-year-old Caucasian woman.ConclusionsChordoma has few systemic treatment options and they have limited benefit. Randomized trials with large patient numbers are unfeasible in this rare disease. Targeted therapy might be a reasonable alternative treatment for chordoma. Still, new treatment strategies are needed for this rare disease.

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BackgroundBehçet's disease (BD) is a systemic disease characterized by oral aphthosis, genital ulcers, ocular lesions and systemic involvement including major vessels. Vascular Behçet's disease (VBD) is observed in up to 40% of BD. It is one of the major causes ofmorbidity and mortality in BD. Deep venous thrombosis (DVT) is the most common form of vascular involvement in Behcet's disease (BD). Post-thrombotic syndrome (PTS) develops in up to one-half of patients with DVT and is associated with impaired quality of life (QoL). There is no data on severity of DVT related PTS and its impact on quality of life in patients with Vascular Behçet's Disease (VBD).ObjectivesWe aimed to evaluate frequency of PTS and venous disease spesific QoL in VBD and to compare it with patients having DVT associated with non-Behçet's disease (NBD) causes.MethodsThis study included 50 patients (Male/Female: 41/9) with VBD and 31 age and gender-matched individuals (Male/Female: 22/9), with DVT associated with non-BD causes. Villalta scale was used to assess of PTS. Venous Disability Score (VDS) and Venous Clinical Severity Score (VCSS) were used for the assessment of venous disease. Venous disease-specific QoL was measured through Venous Insufficiency Epidemiological and Economic Study Quality of Life/Symptom questionnaire (VEINES-QoL/Sym). Behçet Syndrom Activity Score (BSAS) questionnaire was used to assess disease activity.ResultsWe observed significantly lower risk of PTS together with significantly better VEINES-QoL, VEINES-Sym and VDS in VBD group compared to NBD group. When VBD patients with PTS were compared to patients without PTS; VEINES-QoL, VEINES-Sym VCSS were significantly worse. BSAS was also significantly higher in patients with PTS. An inverse correlation was found between VEINES-QoL and BSAS in multivariate analysis. There were no differences between anticoagulant users and non-users regarding the presence of PTS and scores of all venous assessment tools.Table 1.Venous assessment and quality of life parameters in study groupsVascular Behçet disease (n=50)Non-Behçet group (n=31)P valueVEINES-QoL85.6±17.174.9±18.60.01VEINES-Sym37.7±9.433.1±10.10.04VCSS5.2±5.16.2±4.50.346CEAP2.4±1.72.3±1.50.747VDS1.1±0.61.5±0.50.002SF-36 MCS45.3±9.641.9±10.80.163SF-36 PCS42.1±10.741.2±9.60.726VEINES-QoL/Sym: Venous Insufficiency Epidemiological and Economic Study Quality of Life/Symphtom questionnaire, VCSS: Venous Clinical Severity Score, CEAP: Clinical, Etiologic, Anatomic, Pathophysiologic) classification, VDS: Venous Disability Score MCS: Mental component of SF-36, PCS: Physical component of SF-36.ConclusionsWe found lower PTS risk and better venous disease spesific QoL, symptom severity and venous disability scores in VBD compared to NBD group. Venous disease spesific QoL negatively correlated with disease activity. Effective control of vascular inflammation through treatment in VBD might explain lower PTS and better venous QoL outcomes in VBD patients. Our results suggest that successful control of disease activity might decrease devel...

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BackgroundVascular involvement is seen in up to 40% of the patients with Behcet’s Disease (BD), especially in young males and is one of the major causes of mortality and morbidity. Lower extremity vein thrombosis due to vascular inflammation is the most frequent form of vascular involvement in BD. Recently, assessment of vessel wall thickness (VWT) and venous dilatation by US is suggested to be valuable in patients with vascular inflammation.ObjectivesIn this study, we investigated whether vessel wall thickness or dilatation is present in young male BD patients prone to venous vascular disease.MethodsThirthy male patients with BD without major organ involvement and 29 male patients with Vascular BD(VBD) followed in Marmara University Behcet’s Clinics, 24 healthy male controls and 27 male patients with Ankylosing Spondylitis (AS) were included the study. Bilateral lower extremity venous doppler ultrasonography (US) was performed by an experienced radiologist blinded to cases. No patients except VBD were under immunosuppressive treatment. Bilateral common femoral vein (CFV) wall thickness and great/small saphenous vein dilatations were examined. Behçet Syndrome Activity Score (BSAS) was used for the general assessment of disease activity. In 10 patients, CFV wall thickness was measured by 2 different radiologist (RE, RA) in the same day to calculate “inter-observer reliability”. Correlation between radiologists was good. (r=0.765, p<0.001).ResultsThe mean disease duration was 9.1±6 years in patients with BD. BSAS score was 24±17. All venous measurements were significantly higher in BD compared to AS and healthy controls (p<0.001 for all, table 1). When we compared mucucutaneous BD and VBD, all measurements of patients with VBD were higher than mucocutaneous BD. But only left CFV thickness and width of right great saphenous vein reached the statistical significance (p<0.001, an p=0.028, respectively, figure 1). There were no correlations between BSAS, acute phase reactants and venous wall measurements.Abstract SAT0609 – Table 1Venous wall measurements of lower extremity in study groups. Behçet’s Disease (n=59)Ankylosing Spondylitis (n=27)Healthy Controls (n=24)P Value Age, years32.5 (23–42)32 (20–37)27.5 (25–42)0.023Body Mass Index (kg/m2)25.1 (18–33)25 (18–32)23.8 (20–29)0.213Right Common femoral VWT (mm)0.8 (0.04–1.8)0.3 (0.1–0.6)0.25 (0.06–0.4)<0.001Left Common femoral VWT (mm)0.8 (0.3–1.6)0.3 (0.1–0.5)0.2 (0.04–0.6)<0.001Right Great saphenous width (mm)3.1 (0–6.4)2.5 (1.1–3.5)2.1 (1.3–3.5)<0.001Left Great saphenous width (mm)3.1 (0–7.4)2.6 (0.3–4.8)2.4 (1.6–3.6)<0.001Right Small saphenous width (mm)2.8 (0–5.3)1.7 (1–3.1)1.4 (0.9–3.7)<0.001Left Small saphenous width (mm)2.7 (0–5.2)1.8 (1.1–3.4)1.6 (0.8–3.6)<0.001VWT: Venous wall thicknessAbstract SAT0609 – Figure 1Venous Vessel Assessments of patients with Mucocutaneous Behçet and Vascular BehçetConclusionsIn our study, an increased venous vessel wall thickness in lower extremity was shown in male BD patients with or without vascular involvement. As a similar chan...

BackgroundVascular involvement is seen in up to 40% of the patients with Behcet's Disease (BD), especially in young males and is one of the major causes of mortality and morbidity. Lower extremity vein thrombosis due to vascular inflammation is the most frequent form of vascular involvement in BD. Recently, assessment of vessel wall thickness (VWT) and venous dilatation by US is suggested to be valuable in patients with vascular inflammation.ObjectivesIn this study, we investigated whether vessel wall thickness or dilatation is present in young male BD patients prone to venous vascular disease.MethodsFifteen male patients with BD without major organ involvement followed in Marmara University Behcet's Clinics, 14 healthy male controls and 14 male patients with Ankylosing Spondylitis (AS) were included the study. Bilateral lower extremity venous doppler ultrasonography (US) was performed by an experienced radiologist blinded to cases. No patient was under immunosuppressive treatment. Bilateral common femoral vein (CFV) wall thickness and great/small saphenous vein dilatations were examined. Behçet Syndrome Activity Score (BSAS) was used for the general assessment of disease activity.ResultsThe mean disease duration was 9.1±6.3 years in patients with BD. BSAS score was 28.9±19. Bilateral CFV wall thickness was significantly higher in BD patients compared to healthy controls and AS (p=0.001, p=0.002, respectively for right CFV; p=0.001, p<0.001, respectively for left CFV) (Table 1). The width of great and small saphenous veins were also higher in patients with BD, but without reaching statistical significance. There were no correlations between BSAS and wall thickness of any vessel.Table 1.Venous wall measurements of lower extremity in study groupsBehçet's DiseaseHealthy ControlsAnkylosing SpondylitisP value (n=15)(n=14)(n=14) Age, years30.2±4.530±5.930.8±4.20.891Body Mass Index (kg/m2)23.5±3.523.8±2.826.3±3.80.080Right Common femoral VWT (mm)0.69±0.40.26±0.080.28±0.27<0.001Left Common femoral VWT (mm)0.74±0.40.31±0.130.23±0.13<0.001Right Great saphenous width (mm)2.94±2.62.1±0.712.5±0.730.436Left Great saphenous width (mm)3.1±2.22.5±0.652.4±1.10.512Right Small saphenous width (mm)2.4±1.81.4±0.31.7±0.50.126Left Small saphenous width (mm)2.1±1.51.5±0.81.8±0.60.315VWT: Venous wall thickness.ConclusionsIn preliminary results of our study, an increased venous vessel wall thickness in lower extremity was shown in male BD patients without vascular involvement. As a similar change was not observed in controls, we think, increased VWT might be an early sign of venous inflammation in patients with BD rather than a result of non-specific systemic inflammation. Further studies with a larger group of patients is planned.Disclosure of InterestNone declared