Ollier disease is a rare condition presenting with enchondromas in an irregular distribution within the medullary cavity of bones. The disease is well known for sarcomatous transformation to chondrosarcomas. It also increases the risk of other malignancies like leukemia, ovarian tumors, and glial tumors. Central nervous system malignancies associated with Ollier disease are thought to arise by somatic IDH mosaicism with their atypical features of distribution, multifocality, and age of onset. We present a case with imaging consistent with diffuse midline glioma in a patient with Ollier disease. We conclude with a brief review of the literature on Ollier Disease with a focus on central nervous system malignancies, tumorigenesis and pathophysiology.

Objective: This study aimed to observe the preventive effect of prophylactic treatment on joint health in people with hemophilia (PwH) and to investigate the importance of integration of ultrasonographic examination into clinical and radiological evaluation of the joints. Materials and Methods: This national, multicenter, prospective, observational study included male patients aged ≥6 years with the diagnosis of moderate or severe hemophilia A or B from 8 centers across Turkey between January 2017 and March 2019. Patients were followed for 1 year with 5 visits (baseline and 3 rd , 6 th , 9 th , and 12 th month visits). The Hemophilia Joint Health Score (HJHS) was used for physical examination of joints, the Pettersson scoring system was used for radiological assessment, point-of-care (POC) ultrasonography was used for bilateral examinations of joints, and the Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score was used for evaluation of ultrasonography results. Results: Seventy-three PwH, of whom 62 had hemophilia A and 11 had hemophilia B, were included and 24.7% had target joints at baseline. The HJHS and HEAD-US scores were significantly increased at the 12 th month in all patients. These scores were also higher in the hemophilia A subgroup than the hemophilia B subgroup. However, in the childhood group, the increment of scores was not significant. The HEAD-US total score was significantly correlated with both the HJHS total score and Pettersson total score at baseline and at the 12 th month. Conclusion: The HEAD-US and HJHS scoring systems are valuable tools during follow-up examinations of PwH and they complement each other. We suggest that POC ultrasonographic evaluation and the HEAD-US scoring system may be integrated into differential diagnosis of bleeding and long-term monitoring for joint health as a routine procedure.

Objective: This study aimed to (1) compare the oncological results of patients who underwent re-excision after unplanned excision with those who underwent planned excision and ( 2) analyze the impact of local recurrences on oncological outcomes.Methods: Patients with soft tissue sarcoma who had been treated in our center between 2000 and 2018 were retrospectively reviewed. Patients were divided into two groups: Group PE (Planned excision; n = 345) and group UE (Unplanned excision; n = 145). Two groups were compared in terms of local recurrence-free survival (LRFS), metastasis-free survival (MFS), and overall survival (OS). Local recurrences effects over MFS and OS were also analyzed.Results: There were 26 (17.9%) local recurrences in the UE group and 30 (8.7%) local recurrences in the PE group (P = 0.005). There was no difference in MFS and OS between study groups (P = 0.278 and P = 0.848, respectively). Five years MFS rates of UE and PE groups were 76.4% and 73.6%, and five-year OS rates of UE and PE groups were 70.3% and 73.9%, respectively (P = 0.417, P = 0.656). Patients with local recurrence had a 1.96 times higher risk of metastasis than patients without local recurrence (P = 0.008). Patients with local recurrence had 1.65 times higher risk of mortality than patients without local recurrence (P = 0.047). Conclusion:Although local recurrence is much more common in the UE group, this outcome does not seem to affect MFS or OS. These results indicate that similar outcomes can be achieved if UE patients are referred and appropriately treated with wide re-resections.

PURPOSE\AIM Hemophilia affects the blood clotting process, is a genetic disease characterized by recurrent bleeding. The hemophilia early arthropathy detection with ultrasound (HEAD‐US) procedure and scoring method were designed for the detection of early changes in affected joints of patients. In this article, it was aimed to detect early arthropathic changes in the joints of hemophilia patients with the HEAD US scoring system and to investigate its clinical contribution. It was aimed to investigate the effectiveness of HEAD‐US scoring in showing early joint damage in subclinical hemophilia cases and its contribution to treatment. METHODS The present study included 50 hemophilia patients who were admitted to Departments of Pediatric and Adult Hematology for routine follow‐up. During routine follow‐up controls, patients were scored by physical examination and HJHS 2.1 and by ultrasonography and HEAD US. Statistical tests were used to analyze joint health status and the results of US examination in the patient group. RESULTS A total of 294 joints (elbow n = 100, knee n = 94, ankle n = 100) were evaluated by ultrasonography. The mean HJHS and HEAD‐US scores of the patients were 14.94 ± 15.18 and 15.6 ± 12.6, respectively. CONCLUSIONS HEAD‐US is accepted to be more sensitive than HJHS in detecting early signs of arthropathy. Detection of early abnormalities by ultrasonography will enable the development of individualized treatment protocols and to the prevention of arthropathy development.

Pachydermodactyly is a rare digital fibromatosis, characterized by painless, fusiform swelling in proximal interphalangeal joints. Since it may be misdiagnosed as inflammatory arthritis, it is important to call physicians’ attention to this rarely seen condition to avoid unnecessary immunosuppressive treatment. Hereby, we present a case of a pachydermodactyly that had been previously followed-up and treated as an inflammatory arthritis for 4 years. We describe the clinical, radiological, and dermoscopic features of the case.