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Harzem Ozger,1 Bugra Alpan,1 Mehmet Salih Söylemez,2 Korhan Ozkan,3 Ahmet Salduz,4 Bilge Bilgic,5 Basak Kumbasar Sirin6 1Department of Orthopaedics and Traumatology, Maslak Acıbadem Hospital, Istanbul, 2Department of Orthopaedics and Traumatology, Bingöl State Hospital, Bingöl, 3Department of Orthopaedics and Traumatology, Faculty of Medicine, Istanbul Medeniyet University, 4Department of Orthopaedics and Traumatology, 5Department of Pathology, Faculty of Medicine, Istanbul University, 6Department of Radiology, Maslak Acıbadem Hospital, Istanbul, Turkey Abstract: Osteoblastoma-like osteosarcoma, a rare form of osteosarcoma, is a malignant lesion associated with risks of both local recurrence and distant metastasis. The differential diagnosis of osteoblastoma-like osteosarcoma from osteoblastoma and aggressive osteoblastoma remains controversial and challenging. Previous studies suggest that these three types of tumor are distinct entities. However, working out a precise diagnosis may not always be possible immediately on the basis of initial clinical, radiological, and histopathological features. On the other hand, the importance of a correct diagnosis cannot be overemphasized since the treatment strategies change dramatically according to the nature of the lesion. In all of our cases, initial Tru-Cut biopsies revealed osteoblastic features with minimal atypia, but further biopsies confirmed malignancy. A high index of suspicion and considerable experience are prerequisites for accurate diagnosis in case of clinicopathological and radiological discordance. Keywords: osteoblastoma-like osteosarcoma, differential diagnosis, biopsy

Background: Excessive inflammatory immune response during SARS-CoV-2 infection contributes to severe disease in COVID-19 patients. Recently, some researchers hypothesized that dysregulation of the bradykinin (BK) system may also play a role in the pathogenesis of severe disease. Des-Arg(9)-bradykinin (DABK), an active metabolite of BK, is responsible for vasodilatation and increased permeability in the lungs and regulated by angiotensin converting enzyme 2 (ACE-2). Viral inhibition of ACE-2 by SARS-CoV-2 increases DABK levels. Serum levels of this metabolite may be linked to disease severity in COVID-19 patients. In this study, it is aimed to investigate the prognostic value of serial measurement of serum DABK levels in severe COVID-19 patients. Methods: This prospective cohort study was conducted in hospitalized severe COVID-19 patients. Serum DABK levels of patients were serially measured on day 0, day 3 and day 5. Patients were categorized as cases with poor or good prognosis and critical or non-critical cases. Serum DABK levels of these patient groups were compared with paired sample t-test. Serum DABK levels on different days in the same patients were compared with repeated measures ANOVA tests. Results: There was no statistically significant difference in serum DABK levels measured at day 0, day 3, and day 5 between good and poor prognosis groups. DABK levels in critical and non-critical COVID-19 patients also did not show any significant difference. Conclusions: According to our results serially measured serum DABK levels did not correlate with outcome of severe COVID-19 and do not have prognostic value in severe COVID-19 patients. [ABSTRACT FROM AUTHOR]

Coronavirus disease-2019 (COVID-19) has become a serious health problem in Turkey and the world. The diagnosis stage of many chronic diseases, the treatment process and the status of being affected by COVID-19 have become the focus of attention in the medical community during the pandemic, which has been continuing for nine months. We will discuss the course of COVID-19 infection over a 32-year-old and 76-year-old female patient with Cushing syndrome who applied to our clinic as a tertiary referral centre. [ABSTRACT FROM AUTHOR]

Background: In our study, we evaluated the in vitro activities of plazomicin, amikacin, gentamicin, and tobramycin among fifty carbapenem resistant Klebsiella pneumoniae (CR-Kp) isolates. Aminoglycoside resistance genes in selected CR-Kp strains were also examined. Methods: Minimum inhibitory concentration (MIC) of meropenem, plazomicin, tobramycin, gentamicin, and amikacin were determined by gradient test (G-test) method. In all strains carbapenemase activity was assessed by polymerase chain reaction (PCR). Aminoglycoside modifying enzyme (AME) genes in 14 CR-Kp strains that are resistant to at least one of tobramycin, gentamicin, and amikacin among fifty CR-Kp isolates, and 16S ribosomal methylase genes in 6 CR-Kp strains with plazomicin MIC = 128 mg/L were investigated by PCR method. Methods: Minimum inhibitory concentration (MIC) of meropenem, plazomicin, tobramycin, gentamicin, and amikacin were determined by gradient test (G-test) method. In all strains carbapenemase activity was assessed by polymerase chain reaction (PCR). Aminoglycoside modifying enzyme (AME) genes in 14 CR-Kp strains that are resistant to at least one of tobramycin, gentamicin, and amikacin among fifty CR-Kp isolates, and 16S ribosomal methylase genes in 6 CR-Kp strains with plazomicin MIC = 128 mg/L were investigated by PCR method. Conclusions: In our study, increased in vitro efficacy of plazomicin was shown in CR-Kp isolates comparing to other aminoglycosides. Plazomicin is an effective treatment option against CR-Kp isolates and needs to be supported by clinical studies. [ABSTRACT FROM AUTHOR]

Objective: The synergistic activity of eravacycline in combination with colistin on Oxacillinase (OXA) producing carbapenem-resistant Klebsiella pneumoniae isolates (CR-Kp) was evaluated in this study. Methods: Minimum inhibitory concentrations of meropenem were determined by the broth microdilution method. All strains screened for carbapenemase activity by PCR. Chequerboard assay and time-kill analysis were used to assess potential synergy. Results: Synergistic activity was found in 40 % of the strains by chequerboard assay. No antagonism was detected. Comparing to colistin alone at subinhibitory concentrations synergistic and bactericidal activity was observed when it combined with eravacycline. A Similar activity was also observed in colistinresistant CR-Kp isolates. Conclusion: Our results indicate that Eravacycline and colistin combination may be a potential therapeutic option for the treatment of CR-Kp infections. [ABSTRACT FROM AUTHOR]

Endocarditis caused by Bartonella strains has been increasingly reported. While a few sporadic case reports associated with B. henselae were published in Turkey, no endocarditis cases caused by Bartonella spp. have been reported yet. Herein, a case of first B. quintana endocarditis in Turkey, diagnosed using molecular methods, was presented. [ABSTRACT FROM AUTHOR]

Chondrosarcoma is the second most common surgically treated primary bone sarcoma. Despite a large number of scientific papers in the literature, there is still significant controversy about diagnostics, treatment of the primary tumour, subtypes, and complications. Therefore, consensus on its day-to-day treatment decisions is needed. In January 2024, the Birmingham Orthopaedic Oncology Meeting (BOOM) attempted to gain global consensus from 300 delegates from over 50 countries. The meeting focused on these critical areas and aimed to generate consensus statements based on evidence amalgamation and expert opinion from diverse geographical regions. In parallel, periprosthetic joint infection (PJI) in oncological reconstructions poses unique challenges due to factors such as adjuvant treatments, large exposures, and the complexity of surgery. The meeting debated two-stage revisions, antibiotic prophylaxis, managing acute PJI in patients undergoing chemotherapy, and defining the best strategies for wound management and allograft reconstruction. The objectives of the meeting extended beyond resolving immediate controversies. It sought to foster global collaboration among specialists attending the meeting, and to encourage future research projects to address unsolved dilemmas. By highlighting areas of disagreement and promoting collaborative research endeavours, this initiative aims to enhance treatment standards and potentially improve outcomes for patients globally. This paper sets out some of the controversies and questions that were debated in the meeting., Competing Interests: A. Puri is a member of the editorial board of The Bone & Joint Journal. P. Ruggieri reports consulting fees from Exactech and Stryker, unrelated to this study. M. T. Houdek reports consulting fees from Link Orthopedics, unrelated to this study. E. Botello reports consulting fees, payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events, and support for attending meetings and/or travel from Zimmer Biomet, all of which are unrelated to this study. G. V. Morris reports consulting fees, payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events, and support for attending meetings and/or travel from Implantcast, all of which are unrelated to this study., (© 2024 Jeys et al.)

Objective: The aim of the study is to investigate the role of whole-body magnetic resonance imaging (MRI) in assessing extrapulmonary metastases in primary osteosarcoma staging., Methods: We retrospectively reviewed medical data to identify primary osteosarcoma patients with available preoperative whole-body MRI obtained in the staging or restaging. Histopathology was the reference test for assessing the diagnostic performance, if available. Otherwise, oncology board decisions were used as the reference. In addition, the benefits of whole-body MRI to F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) and bone scintigraphy were investigated., Results: In all, 36 patients with osteosarcoma (24 staging, 12 restaging) with a mean age of 16.36 ± 5.63 years (range, 9-29 years) were included in the study. The median follow-up duration was 26.61 months (interquartile range, 33.3 months). Of 36 patients, 8 had skeletal, 1 had a lymph node, and 1 had a subcutaneous metastasis. Whole-body MRI correctly identified all patients with metastatic disease but incorrectly classified a bone infarct in one patient as a skeletal metastasis, equating a scan-level sensitivity, specificity, accuracy, negative predictive value, and positive predictive value of 100%, 96.3%, 97.3%, 100%, and 90.91%. Whole-body MRI contributed to bone scintigraphy by identifying a skeletal metastasis in one patient and positron emission tomography-computed tomography by ruling out a skeletal metastasis in another., Conclusions: Whole-body MRI could accurately identify extrapulmonary metastases in primary osteosarcoma patients for staging or restaging. In addition, it might contribute to the standard whole-body imaging methods., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: Accurate determination of life expectancy becomes very important when determining the treatment of patients with pathologic fractures. We aimed to investigate the predictive role of the PATHFx model in Turkish patients by estimating the area under curve (AUC) of the receiver operator characteristic (ROC) and externally validating the results of PATHFx on the Turkish population., Methods: The data of 122 patients who presented to one of four orthopaedic oncology referral centres in Istanbul (2010-2017) and underwent surgical management of pathologic fractures were retrospectively collected. Patients were evaluated according to age, sex, type of pathologic fracture, presence of organ metastasis, presence of lymph node metastasis, haemoglobin concentration at presentation, primary oncologic diagnosis, number of bone metastases, and Eastern Cooperative Oncology Group (ECOG) status. Estimations of the PATHFx program by months were statistically evaluated using ROC analysis., Results: In our study population (122 patients), all survived the first month, 102 survived the third month, 89 were alive at 6 months, and 58 patients survived at 12 months. At 18 and 24 months, 39 and 27 patients were alive, respectively. The AUC value was 0.677 at 3 months, 0.695 at 6 months, 0.69 at 12 months, 0.674 at 18 months, and 0.693 at 24 months. The 3-, 6-, 12-, 18-, and 24-month survival rates were statistically significant (P < 0.01 and P < 0.05). ECOG performance status was 0-2 points in 33 patients (Memorial Sloan-Kettering Cancer Center (MSKCC) data set: 93 cases, our data set: 33 cases). ECOG performance status was 3-4 points in 89 patients (MSKCC data set: 96 cases, our data set: 89 cases)., Conclusions: The objective data used by PATHFx for prediction provided statistically accurate estimates on Turkish patients, who are presumed to have mixed genomes through history from both Europe and Asia, and demonstrates its applicability to the Turkish population., Competing Interests: None

Osteosarcoma as a secondary malignancy after hematopoietic stem cell transplantation (HSCT) is very rare. We present a case and review of 18 other cases reported to date. Our patient underwent HSCT for myelodysplastic syndrome at the age of 4 years. She developed osteosarcoma 13 years later. She underwent surgery after three courses of neoadjuvant chemotherapy followed by chemotherapy and mifamurtide. She has no evidence of disease 28 months after termination of chemotherapy. In 18 other cases of secondary osteosarcoma in the literature, 15 had received total body irradiation, eight had received alkylating agents, and six had received etoposide. The median interval from HSCT to the onset of osteosarcoma was 6.5 years (range 2.5-15.3), which confirms that children undergoing HSCT should be followed up for many years. In conclusion, osteosarcoma must be included in the differential diagnosis among solid tumors that may develop following HSCT., (© 2016 Wiley Periodicals, Inc.)

Osteosarcoma (OSA) is the most common adolescence cancer among all primary bone tumors next only to multiplemyeloma. It has a substantially worse prognosis and ability to metastasize to lung. MMPs (matrix metalloproteinases) are among the major proteases that take part in regulation of ECM (extracellular matrix). MMPs play an active role in the formation of the osteoid tissue, rich in collagens and other ECM proteoglycans. They also take part in pro-osteoclast, osteoclast, osteoblast, and osteoid formation. Many members of the MMP gene family have been linked to human cancers. It has been shown that MMPs particularly play a role in the tumor's acquisition of an invasive and metastatic character. In our study, the E45K and T102T polymorphisms of MMP-3 were studied using the PCR-RFLP method in 135 Turkish subjects (54 subjects with osteosarcoma and 81 healthy controls). We found that frequencies of E45K G allele (p:0,010, χ²:6,710, OR:1,429, 95% Cl: 1,019-1,858) and AG genotype (p:0,001, χ²:14,753, OR:2,32, 95% Cl: 1,491-3,626) were elevated in patients compared to controls. Besides, there was a significant difference in.E45K AA genotype between study groups (p:0,004, χ²:8,182, OR: 2,929, 95% Cl: 1,38-6,19). There were no significant differences between any genotypes or allele in the control and patient groups for MMP-3 T102T polymorphism. Our findings indicate that the G allele and AG genotype of MMP-3 E45K polymorphism is associated with increased risk of osteosarcoma in adolescent population of Turkey.

Metastasis fingers (acral metastasis) are finding a poor prognosis. Past medical history should be questioned and metastasis from primary tumor should be kept in mind in patients with pain, swelling, and hyperemia in fingers. Successful surgical treatment on acral metastasis does not extend the life expectancy; however, it reduces the patient's pain during his terminal period, saves the functions of the limb, and increases life comfort.

Aim: The aim of the present study was to evaluate the results of treatment and prognostic factors in adult patients with recurrent or refractory Ewing's sarcoma family tumors (ESFT)., Patients and Methods: We retrospectively evaluated treatment outcomes of 54 consecutive patients with ESFT (aged 15 years or more) with complete medical records, who were treated with multimodal therapies after recurrence at the Istanbul University, Institute of Oncology., Results: The commonly used chemotherapy regimens at relapse were ifosfamide and etoposide (IE), ifosfamide and etoposide plus carboplatin (ICE), and oral etoposide. The median progression-free survival and overall survival for the entire group were 6.3 (95% confidence interval, 3.08-9.60) and 8.6 (95% confidence interval CI, 4.7-12.4) months, respectively. Multivariate analysis using a Cox proportional hazards model showed that non-IE/ICE chemotherapy regimens (p=0.003, hazard ratio=2.38) and the presence extrapulmonary metastases (p=0.045, hazard ratio=2.15) were associated with worse overall survival., Conclusion: In primary refractory or relapsed ESFT, the presence of extrapulmonary metastases and treatment with salvage regimens other than ifosfamide and etoposide and/or carboplatin correlate with a poor prognosis.

Tumours and sport injuries can both affect active, young patients, and the symptoms of the two often overlap. Neglected avulsion injuries sometimes cause a mass to develop which can mimic a tumour. We discuss the diagnosis, treatment, and results following nine anterior inferior iliac spine (AISS) avulsion fractures.

Desmoid tumors (DTs) are benign tumors that exhibit fibroblastic proliferation, which arises from fascial or musculoaponeurotic structures. The aim of this study was to investigate the characteristics and outcomes of patients with resectable DTs. A total of 21 patients were included and their clinicopathological characteristics were retrospectively analyzed. The 21 patients (16 females and 5 males) were identified through reviewing the patient charts at our institute. The tumor was located in the lower extremities in 7 cases, in the upper extremities in 4 cases, in the abdominal region in 9 cases and in the neck region in 1 case. Patients who had been initially treated by surgical excision were included in the study. Of these 21 patients, a positive surgical margin (SM) was reported in 11 patients, 7 of whom received postoperative radiotherapy (RT). Ten patients had a negative SM and 6 received RT. A total of 5 patients (46%) in the positive SM group and 4 (40%) in the negative SM group had documented disease relapse (P>0.05). The median relapse-free survival (RFS) was 20.5 months for the patients treated by surgery alone and 50 months for those treated with surgery followed by adjuvant RT (P>0.05). Age, gender, SM and adjuvant RT were not identified as predictors of recurrence. No predictive factors appeared to indicate local DT recurrence following surgery.

Free vascularized fibular autograft transfer to the defect area after wide resection of bone sarcoma is one of many biological reconstruction methods. We report on an 8-year-old girl with 7 years of follow-up treated for right femur osteosarcoma. A 26 cm long tumor was resected with clear margins. Because the length of one fibular autograft was shorter than the defect length, bilateral free vascularized fibular autografts were used in a vertical array on top of one another, and placed inside a fresh-frozen femoral allograft. The original length of the femur was maintained by this technique.

Aneurysmal bone cysts are non-neoplastic, expansile, osteolytic tumor-like conditions. All bones may be involved, but the most commonly affected is the metaphysis of the long bones, especially the tibia, humerus and femur. We present a 13-year-old female patient with a cystic lesion with cortical continuity and a large bone cyst in the coronoid process of the ulna. The bone cyst was treated with curettage, phenol application and cement implantation following cyst debulking. Aneurysmal bone tumors are rare tumor-like conditions and localization at the coronoid process of the ulna with mechanical block of the elbow motion has yet to be reported. Debulking and curettage of the lesion and bone cement implantation are useful methods for local control of aneurysmal bone cysts.

We present two cases of tuberculosis osteomyelitis mimicking subacute osteomyelitis and treated without extensive debridement. Tuberculous osteomyelitis should be considered in the differential diagnosis of proximal tibial infections in children and early diagnosis and treatment is possible without extensive debridement.

Malignant foot tumours are often treated with amputations due to anatomical difficulties. Limb salvage techniques are difficult to perform, as a stable, sensible and plantigrade foot should be obtained to prevent further problems.In this report, we present a midfoot reconstruction with a vascularized free fibula, osteomized in a V-shape after wide resection of a midfoot synovial sarcoma. We describe the reconstruction of both longitudinal arches in which using a vascularized autograft facilitated union, remodeling of the bone, and obtaining a functional foot.

Paraoxonase is an HDL-associated enzyme that plays a preventive role against oxidative stres. Previous studies suggested that involved an amino acid substitution at position 192 gives rise to two alloenzymes with a low activity (Q allele) and a high activity (R allele) towards paraoxon. There also exists a second polymorphism of the human PON1 gene affecting amino acid 55, giving rise to a leucine (L-allele) substitution for methionine (M-allele). PON1 gene polymorphisms were studied in 50 patients with osteosarcoma and 50 healthy controls. Paraoxonase genotypes were determined by PCR-RFLP. We found a reduction in the frequency of PON1 192 R allele in patients (P=0.015). Besides, PON1 192 wild type QQ genotype (P=0.015) and PON1 55 wild type L allele (P=0.001) were higher in patients compared to healthy controls. PON1 192 QQ genotype was associated with osteosarcoma in multivariate logistic regression analysis. Our findings have suggested that PON1 192 wild type genotypes may be associated with a risk of developing osteosarcoma.

Background: Enchondromatosis is characterized by the presence of multiple benign cartilage lesions in bone. While Ollier disease is typified by multiple enchondromas, in Maffucci syndrome these are associated with hemangiomas. Studies evaluating the predictive value of clinical symptoms for development of secondary chondrosarcoma and prognosis are lacking. This multi-institute study evaluates the clinical characteristics of patients, to get better insight on behavior and prognosis of these diseases., Method: A retrospective study was conducted using clinical data of 144 Ollier and 17 Maffucci patients from 13 European centers and one national databank supplied by members of the European Musculoskeletal Oncology Society., Results: Patients had multiple enchondromas in the hands and feet only (group I, 18%), in long bones including scapula and pelvis only (group II, 39%), and in both small and long/flat bones (group III, 43%), respectively. The overall incidence of chondrosarcoma thus far is 40%. In group I, only 4 patients (15%) developed chondrosarcoma, in contrast to 27 patients (43%) in group II and 26 patients (46%) in group III, respectively. The risk of developing chondrosarcoma is increased when enchondromas are located in the pelvis (odds ratio, 3.8; p = 0.00l)., Conclusions: Overall incidence of development of chondrosarcoma is 40%, but may, due to age-dependency, increase when considered as a lifelong risk. Patients with enchondromas located in long bones or axial skeleton, especially the pelvis, have a seriously increased risk of developing chondrosarcoma, and are identified as the population that needs regular screening on early detection of malignant transformation.

Treatment results of 17 patients who were diagnosed with sacral chordoma between 1993 and 2007, were analyzed retrospectively. The mean duration of symptoms was 30.2 months. The mean tumour size was 10.7 cm; the location was S2 or more proximal in ten-patients. A wide resection was achieved in 14 patients, a marginal resection in one patient and two patients had intralesional excision. Seven patients had a recurrence after a mean time interval of 36.3 months. Wound dehiscence and infection were the major problems. Four patients required continuous urinary catheterization because of incontinence, and ten patients had colostomy or ileostomy simultaneously with tumour resection. This study showed that tumour size and a wide surgical margin are importance factors for survival in chordoma patients. Use of a combined anterior and posterior approach could increase the chance of achieving a wide margin. A multidisciplinary approach is required to achieve this goal.

Here, we report a series of 5 patients (6 shoulders) diagnosed with neuropathic arthropathy of the shoulder joint in our clinic between 2005 and 2008. Initial diagnosis, previous treatment, and radiological and clinical follow-up findings were reviewed. The mean age at diagnosis was 44.2 years. Four patients had unilateral and 1 patient had bilateral involvement. The presenting symptoms were pain, swelling, and loss in range of motion. Active forward flexion and abduction ranged from 0° to 90°. Hypoesthesia and loss of temperature sense was evident in 3 patients. Radiographs showed massive osteolysis of humeral head and glenoid process, and magnetic resonance imaging showed periarticular fluid collection, and degeneration at the rotator cuff and shoulder joint, resembling chronic septic arthritis or sarcoma. Biopsy was performed in 4 patients before definitive diagnosis, and synovial hypertrophy and necrotic bone was found. Two patients had a history of operated cervical syringomyelia, and the remaining 3 patients were later diagnosed to have syringomyelia and referred to neurosurgery clinic, where 2 of those were operated. Four patients were followed-up with symptomatic therapy, and 1 patient underwent an unsuccessful shoulder arthroplasty in another clinic. As a conclusion, neuropathic arthropathy of the shoulder is rare, and correct diagnosis is possible by careful physical and neurological examination and pathologic evaluation when needed.

We retrospectively evaluated the results after ulnar lengthening and radial deformity correction using an external fixator for forearm deformities caused by osteochondromas. Eight forearms were treated surgically in seven patients with multiple hereditary osteochondroma. The mean follow-up time was 40 months (range, 20 to 60 months). The average radial articular angle improved from 43 degrees to 35.5 degrees (range, 28 to 56 degrees) and the carpal slip improved from 69.5% to 55% (range, 40 to 60%) postoperatively. The average shortening of the ulna was reduced from 2.06 cm to 0.44 cm (range 0 to 1 cm) after the treatment. There were no serious complications associated with the surgery; two minor pin track infections were successfully treated by local wound care and antibiotics. Although technically demanding, ulnar osteotomy and gradual lengthening by an external fixator provided promising results in the treatment of forearm deformities in children with multiple osteochondroma.

Objectives: We evaluated the results of, and the course of treatment with, external fixation (EF) in treating complications associated with bone tumors and related surgery., Methods: Eighteen patients (9 males, 9 females; mean age 19 years; range 6 to 35 years) who were treated with EF were evaluated in three groups. Histologic diagnoses were osteosarcoma (n=3), Ewing's sarcoma (n=3), hereditary multiple exostosis (n=3), chondrosarcoma (n=2), synovial sarcoma (n=2), Ollier's disease, giant cell tumor of bone, desmoid fibroma, chondromyxoid fibroma, and enchondroma. Complications secondary to bone tumors (n=4) and occurring following limb salvage surgery (n=14) were treated with Ilizarov circular EF in nine patients, unilateral EF in six patients, and both in three patients., Results: The first group included eight patients who were treated with EF for infection and nonunion or deformity following surgery. The mean shortening was 10.6 cm, the mean lengthening was 9.7 cm, and the mean external fixator index was 48.8 days/cm. One patient developed fracture of the free vascularized fibula graft after EF removal, and amputation was required in two patients. The second group consisted of six patients who had shortening secondary to tumor surgery. The mean shortening was 7.5 cm, the mean lengthening was 6.5 cm, and the mean external fixator index was 28 days/cm. In this group, the major complications were implant failure and knee stiffness. The third group included four patients with deformity and shortening secondary to multiple exostosis (n=3) and Ollier's disease. The mean shortening was 7.5 cm, the mean lengthening was 6.5 cm, and the mean external fixator index was 57.2 days/cm. One patient developed ulnar shortening of 2 cm after growth., Conclusion: The use of EF in the management of complications associated with bone tumors and related surgery yields successful results especially in young patients.

Objectives: Despite the developments in chemotherapy protocols, improvement in the survival rates of osteosarcoma has been limited. We evaluated the effect of certain prognosis-related proteins on survival of patients with osteosarcoma., Methods: Data from 45 patients (24 males, 21 females) who were treated and followed-up for osteosarcoma were reviewed. Following neoadjuvant chemotherapy, 41 patients underwent extremity saving surgery, and four patients underwent amputation. The most frequent localization was the lower end of the femur (n=23, 51.1%), followed by the upper end of the tibia (n=10, 22.2%). Three patients had metastasis on admission. Surgical resection samples were retrieved from the pathology archive and analyzed immunohistochemically for the expression of p-glycoprotein p170, p53, heat-shock protein 27 (HSP27), HSP90, and nm23. The effect of these proteins on prognosis and survival was assessed with survival analysis using the Kaplan-Meier method. The mean follow-up was 49.7 months (range 6 to 185 months)., Results: Three patients with metastasis on admission died within five years due to pulmonary metastasis. New metastases developed in 29 patients. Total 5-year and 10-year survival rates were 60% and 43%, respectively. The corresponding disease-free survival rates were 41% and 24%. Five-year survival was 29% in patients who developed metastasis. Among clinical factors, survival was influenced only by the presence of metastasis on admission (p=0.044). Five-year and 10-year survival rates were significantly different between patients with and without p53 positivity (p=0.04), while the other proteins were not significantly associated with survival., Conclusion: Our data suggest that p53 may be used as a prognostic marker in osteosarcoma due to its significant association with survival.

Between January 1990 and December 2006, 123 patientsor=90%) was significantly correlated with the 5-year EFS (31% vs. 67.6%, respectively, p=0.023) but not with OS (57.7% vs. 76.5%, respectively, p=0.13). The presence of metastases at diagnosis was found to be the most significant single characteristic influencing the outcome. The rate of histologically good response to preoperative chemotherapy was 64.5%, which is comparable with the 28-85% response rates given in the literature. Our results demonstrate that the combination of epirubicin, cisplatin, and ifosfamide is an active and reasonably well-tolerated regimen for childhood osteosarcoma.

Purpose: The purpose of this study was to describe the computed tomography (CT) and magnetic resonance (MR) imaging findings of hibernoma., Materials and Methods: We retrospectively evaluated imaging findings of CT and MR examinations of six patients (three men and three woman, aged 27-48 years) with histopathological diagnosis of hibernoma., Results: On CT examination, the lesions were slightly hyperdense, and on T1- and T2-weighted MR images, they were isointense or slightly hypointense compared to the subcutaneous fat. All of these lesions showed contrast enhancement and one out of the six lesions had internal linear septations., Conclusion: Hibernoma has a wide spectrum of CT and MR imaging findings, which should be considered in differential diagnosis, especially with other lipomatous lesions.

This study was designed to investigate the association of MTHFR C677T polymorphism and the risk of two common musculoskeletal sarcomas, osteosarcoma and chondrosarcoma. MTHFR genotypes were determined in 56 patients (44 osteosarcoma, 12 chondrosarcoma) and 44 controls using the PCR-RFLP technique. In the gender subgroup analysis, wild-type A allele frequency was higher in male osteosarcoma patients than in male control subjects (P = 0.064). Mutant V allele and mutant VV genotype were similar in the control group compared to the sarcoma groups (P > 0.05). No correlation could be proved between patient tumour site, presence of metastasis, and local tumour relapse and MTHFR polymorphism. The MTHFR C677T polymorphism may not be important in an individual's susceptibility to osteosarcoma and chondrosarcoma in Turkey and may not be a useful marker for identifying patients at high risk of developing sarcomas.

Rhabdomyosarcoma (RMS) is rare disease in adults (age >or= 16 years). The data from randomized prospective trials are scarce; the clinical outcome of these patients seems poor with the currently available treatment strategies. In this study, we report a single institution's experience in the treatment of adult RMS. We reviewed the medical records of patients with RMS who were >or= 16 years and have been treated in our institution between 1988 and 2003 retrospectively. We analyzed the survival outcome of these patients and the prognostic impact of clinical/pathological factors on their survival. In total, 23 patients with RMS were identified. Median age was 26 years (range, 16-72 years). Majority of patients were male (n: 17, 73.9%), and had large tumors (>or= 5 cm, n: 13, 56.5%), localized disease (N0, M0, n: 12, 52.2%), and embryonal histology (n: 10, 43.5%). Median overall survival was 31.3 months, and the 3-year progression-free survival and overall survival rates were 19.9% and 34.94%, respectively. Patients with smaller tumors (< 5 cm) (p < 0.04), local disease (p < 0.01), and normal lactic dehydrogenase (LDH) level (p < 0.01) at the time of diagnosis were found to have better survival outcome. The tumor size, serum LDH level, and metastatic disease at the time of diagnosis are potential predictors of outcome in patients with adult RMS. Adult RMS is an aggressive disease with poor survival despite treatment. The data from prospective, randomized multicenter trials are necessary in order to improve the clinical outcome of adult RMS patients.

Neurofibromatosis may be associated with ossifying subperiosteal hematoma resulting in lower extremity hypertrophy. We presented two girls, aged 10 and 13 years, with neurofibromatosis type 1, who exhibited increased thigh girth on the right side. Plain radiographs showed an ossifying cystic formation. Magnetic resonance scans demonstrated a fluid-fluid level. Diagnosis of ossifying subperiosteal hematoma was confirmed with a biopsy in both cases. In the older patient, radiologic studies after 1.5 years showed an increase in the wall thickness of the femoral cyst from 3 mm to 5 mm. In the other patient, cystic formation surrounding the tibia became more prominent at the end of five years. This patient died from multiple lung metastases following chemotherapy and wide resection for Ewing's sarcoma that developed in the right shoulder.

Objectives: We investigated treatment results and the role of potential prognostic factors in patients treated by surgery with or without adjuvant radiotherapy for primary or recurrent extra-abdominal desmoid tumors., Methods: The study included 38 patients (23 females, 15 males; mean age 24 years; range 5 to 61 years) who underwent surgical treatment for extra-abdominal desmoid tumors. Of these, eight patients (21.1%) already had recurrences before treatment. Involvement was in the upper extremity in 12 cases (31.6%), in the lower extremity in 22 cases (57.9%), and in the axial region in four cases (10.5%). Twenty-two patients received adjuvant radiotherapy following surgical resection. Survival was analyzed by the Kaplan-Meier method. The mean follow-up period was 7.3 years (2.5 to 228 months)., Results: Twenty patients (52.6%) developed recurrences after treatment. Of these, recurrences were already present in six patients, and adjuvant radiotherapy was administered to 11 patients (55%). Recurrences developed at the irradiated site in eight patients, and in other regions in three patients. The mean disease-free survival was 38+/-8 months, and eight-year disease-free survival was 35.7+/-8.5%. Disease-free survival did not differ significantly between patients receiving adjuvant radiotherapy (47.9+/-7.9 months) and those treated with surgery alone (37.9+/-12.4 months), and between patients who developed a recurrence at the resection site (12.1+/-4.7 months) or at a different site (24.3+/-1.0 months) (p>0.05). None of the potential prognostic factors including gender, age, localization, surgical margin, or adjuvant irradiation were found to affect disease-free survival., Conclusion: In our series, no prognostic factor could be identified as having an association with the high recurrence rate.

Objectives: The hand is a very rare localization for sarcomas. We evaluated patients who underwent surgical treatment for sarcoma of the hand., Methods: Between 1990 and 2006, eight patients (5 males, 3 females; mean age 39.6 years; range 26 to 67 years) with hand sarcoma received treatment at two centers. The lesions arose from soft tissue in five cases, and from bone in three cases. Localizations were fingers (n=3), carpal region (n=2), and the wrist (n=3). Radiologic assessments included plain radiographs, magnetic resonance imaging, and scintigraphy in all the patients, with addition of computed tomography for bone tumors. All the patients underwent biopsies for precise diagnosis. Staging was made according to the Enneking classification. Tumors involving the fingers were treated with digital amputation, while the rest were treated with wide resection. The mean follow-up was 48 months (range 6 to 123 months)., Results: Mortality occurred in two patients due to multiple metastases, who had synovial sarcoma in the carpal region and clear cell sarcoma in the wrist, respectively. Both patients had stage III disease on presentation. Of three patients with tumors localized in the fingers, two had stage IA, and one had stage IB disease. Apart from clear cell sarcoma, diagnoses of other wrist tumors were stage IIB epithelioid sarcoma and malignant fibrous histiocytoma. Of six patients who were alive, hand functions were sufficient in four, fair in one, and poor in one. None of the patients developed local recurrences., Conclusion: Due to rarity of malignant hand tumors, diagnosis and treatment require considerable attention. It was observed that patients maintained a good psychological status by avoiding complete amputation of the hand and performing an appropriate wide resection with reconstruction.

We evaluated the results of polygonal triple (Kotz) osteotomy for the treatment of acetabular dysplasia over 10 years. This study included 31 hips of 27 patients who had the Kotz osteotomy for acetabular dysplasia. The mean age was 21.5 years. We performed the original Kotz osteotomy for the first 22 hips (group I), while the modified Kotz osteotomy through an intra-pelvic approach without damage to the abductor muscle was applied for the last 9 hips (group II). Patients were evaluated by clinically and radiologically. The average follow-up was 106 months in group I, and 18 months in group II. The Trendelenburg gait was unchanged for four patients in group I and for one patient in group II. The Harris Hip Score improved in all patients postoperatively. Radiographic assesment showed improvement in both groups in terms of the angle of CE, VCE, and Sharp postoperatively(P<0.05). The complication rate per hip was 0.29. The original Kotz osteotomy achieves adequate coverage for the treatment of acetabular dysplasia, and patients are generally satisfied by this procedure. Nonetheless, the modified Kotz osteotomy provides recovery of the abductor muscle strength in the early postoperative period and subsequently decreases the rate of the Trendelenburg gait compared to the original Kotz osteotomy.

Background: Osteosarcoma is a rare malignancy, and patients with this disease benefit from adjuvant chemotherapy. While cisplatin, anthracyclines and ifosfamide are the most commonly used agents in the treatment of osteosarcoma, a search for the best combination with higher efficacy and minimal toxicity continues. We planned to evaluate the efficacy of epirubicin combined with cisplatin and ifosfamide in patients with localized primary osteosarcoma., Methods: Patients with nonmetastatic extremity osteosarcoma who were older than 15 years were included in the study. The preoperative chemotherapy regimen consisted of epirubicin 90 mg/m(2), cisplatin 100 mg/m(2) on day 1 and ifosfamide 2.0 g/m(2)/day with an equivalent dose of mesna on days 2-4, repeated every 21 days. Six cycles of this combination regimen were administered (3 cycles prior to surgery and 3 cycles postoperatively)., Results: Forty-five patients with localized osteosarcoma entered this phase II trial. Median follow-up was 64 months. Thirty-two patients (84%) received the assigned 6 cycles of chemotherapy. Complete and good histological response to neoadjuvant chemotherapy was 26 and 37%, respectively. The 5-year disease-free and overall survival rates were 41.9% (95% CI 33.6-50.2) and 48.2% (95% CI 39.6-56.8). The most prominent grade 4 toxicity was neutropenia occurring in 32% of patients. The lungs were the most frequent site of relapse (32%)., Conclusions: The combination of cisplatin, ifosfamide and epirubicin is an active, reasonably well-tolerated regimen without grade 3 or 4 cardiac toxicity in patients with nonmetastatic extremity osteosarcoma and deserves further investigation in the context of prospective phase III trials., ((c) 2008 S. Karger AG, Basel)

Objectives: We evaluated long-term treatment results of patients with primary osteosarcoma and the effect of prognostic factors on overall survival and disease-free survival., Methods: Between 1995 and 2005, 180 patients (111 males, 69 females; mean age 21+/-10 years; range 7 to 64 years) were treated for primary osteosarcoma. Overall and disease-free survival rates were analyzed for 165 patients with high-grade osteosarcoma with the Kaplan-Meier method. The effects of potential prognostic factors were assessed, including age, gender, localization, tumor size, primary metastasis on presentation, the presence of pathologic fractures, necrosis rate, and infection. All the patients received chemotherapy before and after surgery. The mean follow-up period was 49.7 months (range 6 to 185) months., Results: Sixty-nine patients were below 16 years of age. The most frequent involvement was in the distal femur (47.2%), followed by the proximal tibia (25%). Sixteen patients presented with a pathologic fracture, and 12 patients with metastasis. The median tumor size was 10 cm. The overall five- and 10-year survival rates were 68% and 60%, and disease-free survival rates were 50% and 44%, respectively. Only the presence of a pathologic fracture and primary metastasis on presentation were found to affect prognosis., Conclusion: The two conditions, primary metastasis and a pathologic fracture, found as the most important prognostic factors in our study are mainly associated with late presentation. As in every malignant disease, early admission would provide better survival rates.

Tumoral calcinosis is a rare condition characterized by large calcific soft tissue deposits occurring predominantly in a periarticular location. Familial tumoral calcinosis was detected in three members of a family, namely, the father and two offsprings. The father underwent many operations since age 10 for occurrences or recurrences of mass lesions in the right posterior elbow, both hips, left gluteal region, and perineal region. His 16-year-old son underwent his first operation at the age of 10 for a lesion in the posterior elbow, which recurred at the same site and required another operation two years later. He underwent subsequent surgeries for lesions that appeared in the right elbow and right dorsal foot. Finally, the 12-year-old daughter was treated with surgery for a lesion in the right dorsal foot. In all the patients, pathologic diagnoses of all surgical specimens were reported as tumoral calcinosis. All had normocalcemia, hyperphosphatemia, and D hypervitaminosis.

A 45-year-old male patient presented with paralysis of slow onset in the right forearm and hand muscles. Electromyographic assessment revealed severe denervation in the muscles innerved by the posterior interosseous nerve. Magnetic resonance imaging demonstrated a tumoral mass compressing the nerve. The patient underwent surgical excision with an initial diagnosis of lipoma. Surgical exploration and a biopsy confirmed the diagnosis. Active wrist movements and digital extension were possible after three weeks and the patient resumed full strength six weeks after the operation.

Objectives: We evaluated pediatric patients who underwent surgery for Langerhans cell histiocytosis with mono- or polyostatic involvement and an extensive soft tissue component and/or a high risk for pathologic fractures., Methods: The study included 13 children (6 boys, 7 girls; mean age 6 years; range 2 to 11 years) who were treated surgically for bone and soft tissue involvement of Langerhans cell histiocytosis with a high risk for fractures of the long bones and the pelvis. Eleven patients had only skeletal involvement while three patients had multisystem involvement. There were 17 bone lesions in the following localizations: acetabulum (n=7), femur neck and subtrochanteric region-proximal femur (n=5), and proximal (n=2) and distal (n=2) humerus. One patient had multifocal bone involvement. The risk for pathologic fractures was assessed using the scoring system of Mirels. Treatment consisted of surgery followed by chemotherapy. The results of treatment were evaluated according to the criteria by the Study Group of Langerhans Cell Histiocytosis. The mean follow-up after orthopedic surgery was 67 months (range 34 to 172 months)., Results: Only one patient developed recurrence, in which case bone marrow involvement responded well to chemotherapy. Radiographically, graft consolidation was observed in all the patients in the third postoperative month. No bone sequelae such as shortness and deformity were detected throughout the follow-up period., Conclusion: Surgical treatment combined with chemotherapy is effective in the treatment of aggressive histiocytic bone lesions associated with extensive soft tissue involvement and high risk for fractures.

Background: The current study highlights the clinical features of tumors of the foot and ankle, and presents the clinical outcomes of treatment for 16 patients who were treated at the authors' institution, between 1989 and 1998., Methods: Four tumors occurred in the ankle, three were in the hindfoot, three were in the midfoot, three were in the forefoot, and three were on the plantar surface of the foot. The median duration of symptoms before presentation was 12 months (range, 1-50 months). Fourteen patients were managed by limb-salvage procedures. Three patients were managed by ablative surgery, due to skin, neurovascular, and/or severe bone invasion. In the 9 patients who had wide local resections, local island flaps, split-thickness skin grafts, free vascularized latissimus dorsi flaps, and a free vascularized iliac crest flap were utilized to achieve wound closure., Results: The patients were followed up for a mean of 74.2 months (range, 48-121 months). The overall survival of the whole group is 71 +/- 3.7 months. Currently, 12 patients are being followed up with no evidence of disease. Of the 14 patients who were managed by limb-salvage procedures, 6 have normal functional status., Conclusion: Wide resection of malignant tumors of the foot and ankle is considered technically difficult because of technical problems of soft-tissue coverage. However, utilization of local and free flaps provides sufficient closure following wide tumor resection of the foot and ankle, thus yielding satisfactory lower extremity function and local tumor control.