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    Evaluation of PD-L1 expression in recurrent nonmetastatic sacral chordomas – a retrospective study
    (Serbia Medical Society, 2023) Zenginkinet, T.; Okay, E.; Yıldırım, A.N.T.; Celik, A.; Turhan, Y.; Özkan, K.; Akyürek, M.
    Introduction/Objective Chordomas are rare tumors of a notochordal origin. Wide surgical resection is recommended for treatment. However, it is associated with a high risk of morbidity and mortality. Additionally, these tumors are resistant to chemotherapy. Thus, targeted therapy is needed for the treatment of chordomas. Programmed death ligand 1 (PD-L1) is a promising target for cancer treatment. Here, we investigated PD-L1 expression in patients with chordoma in a single-center study. Methods Formalin-fixed paraffin-embedded blocks were evaluated for immunohistochemical analysis to evaluate PD-L1 expression. Clinicopathological variables, such as sex, age, and follow-up data (recurrence and outcome), were retrospectively collected from the patients’ medical records. Results Ten patients diagnosed with sacral chordoma in a single institution between December 2015 and November 2021 were included in this study. The median patient age was 57 years and the median follow-up period was 40 months. The surgical margins were negative in all cases, without any preoperative medical treatment. Four of the ten patients showed PD-L1 positivity on immune cells. These patients showed local recurrence, without metastasis. In these cases, the median time to local recurrence was 15 months. All the patients with the disease were alive. Conclusion This study demonstrated that PD-L1 positivity in immune cells can be used as a predictive marker for local recurrence at the time of surgical treatment. This can potentially be used to determine the necessity to administer immunotherapy. © 2023, Serbia Medical Society. All rights reserved.
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    Hemophilic arthropathy in children: Pathophysiology, diagnosis and management
    (Duzce University Medical School, 2021) Turhan, B.; Turhan, Y.
    Arthropathy is a serious and common problem in patients with hemophilia impairing the patient’s quality of life seriously. The most commonly affected joints in hemophilic arthropathy are knees, ankles and elbows. Even a single bleeding could cause devastating effects to synovium, cartilage and also subchondral bones. Ultrasound and magnetic resonance imaging have been advocated for the studying of cartilage damage. Prophylaxis has been demonstrated as a standard choice of management to prevent hemophilic arthropathy development. Primary prophylaxis starting at early years of age is very important to prevent hemorrhages but secondary prophylaxis in adolescents has also significant success rates. As the duration of exposure to the blood increases in the joint cavity, degeneration of the cartilage matrix and resultant cartilage loss also increase, so the aspiration of the hematoma from joint plays an important role in prevention of the disease progression. Synovectomy may be required in cases where prophylaxis and aspiration does not prevent the recurrent hemorrhages. The purpose of synovectomy either with medical or surgical methods is to remove the problematic synovium to prevent the progression of hemophilic arthropathy. Medical synovectomy (synoviorthesis) has two basic types; radiosynovectomy and chemical synovectomy and the former one is appearing to be more effective with an about 85% success rates. If all of these measures fail to prevent the progressive cartilage damage, open or arthroscopic synovectomy, arthrodesis of the affected joint or even arthroplasty could be necessary. Here we tried to summarize the pathological mechanism, diagnosis and management of hemophilic arthropathy in children. © 2021, Duzce University Medical School. All rights reserved.