Intralesional curettage and cementation of low-grade chondrosarcomas of the appendicular skeleton: Long-term results from a single center.

OBJECTIVE: The purpose of this study was to investigate the results and complications in patients who had low-grade chondrosarcomas in the appendicular skeleton and were treated by intralesional curettage and cementation within the scope of 25 years of experience in a single center. METHODS: Ninety-one patients (72 female and 19 male) were retrospectively analyzed. The median at the time of surgery was 43 (17-78) years, and the median follow-up was 102 (26-288) months. All patients were treated by intralesional curettage followed by cementation with high-viscosity bone cement (polymethylmethacrylate). Complications and local recurrence rates, as well as clinical outcome scores were recorded. RESULTS: Five patients (5.49%) developed local recurrence at an average of 6.6 (6-9) months postoperatively. Four were treated with local wide excision and reconstruction with tumor prosthesis. One patient received recurettage and cementation. Two recurred patients were dedifferentiated into grade II chondrosarcomas in the last intervention. No major postoperative complication was identified in the series. Patients achieved an average Musculoskeletal Tumor Society scoring system of 92.4% (standard deviation 5.2; range 80-100) in the sixth postoperative month. Musculoskeletal Tumor Society scores in the recurrent patients decreased from an average of 90% to 75.3% after the final intervention. CONCLUSION: Intralesional curettage and cementation seem safe and reliable techniques with low recurrence and complication rates in treating low-grade chondrosarcomas of the appendicular skeleton. Clinical, radiological, and pathological evaluations are mandatory before surgical intervention, and a multidisciplinary approach is crucial. A strict follow-up regimen in the early postoperative period is needed and strongly recommended to detect local recurrence. LEVEL OF EVIDENCE: Level IV, Therapeutic Study.

The Effect of Preoperative Albendazole Treatment on Intramuscular Cyst Structure in a Patient with Simultaneous Cystic Echinococcusis of Liver and Vastus Lateralis Muscle.

We report a 44-years-old woman with cystic echinococcosis (CE) who presented with simultaneous involvement of liver and vastus lateralis muscle to Istanbul University-Cerrahpasa, Istanbul, Turkey in 2020. Although she underwent surgery for the liver, the intramuscular mass was ignored. While the patient was under post-operative albendazole treatment, she was investigated for the mass on her right thigh which was excised later. The comparison of magnetic resonance imaging before and after albendazole treatment showed that albendazole eliminated the intramuscular vesicle structures by accelerating the degeneration process of the cyst. The comparison of pathology samples sent from both liver and intramuscular CE also revealed that the albendazole has left the laminar membrane intact, degenerate the germinative membrane, thereby reducing the intra-vesicle pressure and also caused the scolex structures to disappear. To the best of our knowledge, this is the first paper to report the effect of preoperative albendazole treatment on the structure of intramuscular CE.

The risk factors for mechanical complication in endoprosthetic reconstruction of knee osteosarcoma.

BACKGROUND: The increased limb survival rates and higher functional demands have also increased the mechanical complication rates of megaprostheses. This study aimed to analyse possible risk factors which can predispose patients to mechanical complications. METHODS: Patients with knee osteosarcoma referred to our clinic from 1992 to 2014 were investigated retrospectively. The patients who underwent surgical resection and endoprosthetic reconstruction with at least 5 years of follow up were analysed. The revision of the megaprosthesis due to a mechanical complication was accepted as an endpoint. The possible risk factors, including cement usage, implant material, joint mechanism, neoadjuvant radiotherapy, and anatomical localization of the lesion, were analysed for any association with mechanical complication rates. RESULTS: A total of 118 patients were included for final analysis. The average age was 24.5 years (standard deviation ±10.1, range: 15-64). Mechanical complication rate was 22% which included 19 aseptic loosenings and seven implant failures. The average time to mechanical complication was 32.5 months. Overall, 5-year implant survival was 78%. Multiple regression analysis revealed that cement usage is an independent risk factor for mechanical complication (P = 0.007). Although the 5-year implant survival was higher in rotating hinge and titanium implants compared with fixed hinge and cobalt chrome, the multiple regression model did not yield a correlation with mechanical complication rates. CONCLUSION: Cemented implants showed significantly higher mechanical complication rates compared with cementless ones in this series of knee osteosarcoma patients who underwent megaprosthetic reconstruction. Hinge mechanism and implant material did not have a significant effect on mechanical complication rates.

The effect of preoperative radiotherapy on local control and prognosis in high-grade non-metastatic intramedullary osteosarcoma of the extremities.

INTRODUCTION: This study aimed to analyse the effect of preoperative radiotherapy on survival, recurrence and necrosis rates in osteosarcoma patients treated with neoadjuvant chemotherapy and limb-salvage surgery. MATERIALS AND METHODS: Osteosarcoma patients who referred to our clinic from 1998 to 2015 were investigated retrospectively. 141 patients with non-metastatic osteosarcoma of the limb who received neoadjuvant chemotherapy and underwent limb-sparing surgery with or without preoperative radiotherapy were included in the study. 73 patients had neoadjuvant chemotherapy only (CT group), while 68 patients had additional preoperative radiotherapy (CT + RT group). 5-year survival, disease-free survival, limb survival, local recurrence, metastasis, complication and necrosis rates were compared between the two groups. RESULTS: Overall 5-year survival, 5-year disease-free survival, 5-year limb survival rates were 68.5, 64.3, 65.7 for CT group and 72.1, 67.6, 64.7 for CT + RT group, respectively. Preoperative radiotherapy gave similar survival and disease-free survival rates. Limb survival, recurrence and metastasis rates were similar between the groups. The necrosis rate of the lesions was significantly higher in the CT + RT group compared to the CT group. CONCLUSION: Preoperative radiotherapy can provide a higher necrosis rate and may allow the resection of unresectable lesions in the treatment of osteosarcoma. CT + RT provided similar 5-year survival despite the larger tumour size compared to CT group. Due to the higher wound complication rate (8.2% in CT, 23.5% in CT + RT group, p = 0.01), CT + RT should be restricted to the lesions with large size or close proximity to neurovascular structures. LEVEL OF EVIDENCE: Level III; Therapeutic, Retrospective comparative study.

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results.

BACKGROUND: Desmoid tumors are uncommon and benign mesenchymal neoplasms. The optimal treatment of patients with desmoid tumors is still controversial. Surgery is the primary treatment for locally invasive or recurrent desmoid tumors. Also, radiotherapy is a treatment option for patients at high risk for local failure such as those with positive margins or recurrent and unresectable tumors. AIMS: To report our institutional experience and long-term results of patients with desmoid tumors who received radiotherapy. STUDY DESIGN: Retrospective cross-sectional study. METHODS: Between 1980 and 2009, 20 patients who received radiotherapy (RT) in our institution were analyzed. The majority of patients (80%) were referred with a recurrent tumor after previous surgery. Thirteen patients underwent marginal resection, 4 had wide local excision and 3 patients had only biopsy. Resection margin was positive in 15 (75%) patients. All patients received radiation therapy. The median prescribed dose was 60 Gy. Five patients received less than 54 Gy. RESULTS: The median follow-up time was 77.5 months (28-283 months). Nine patients developed local recurrence after RT. Seven local failures (78%) were in field. Time to local recurrence ranged from 3-165 months (median 33 months). The 2-5 year local control (LC) rates were 80% and 69%, respectively. On univariate analysis, the 5 year local control rate was significantly better in the patients treated with ≥54 Gy than in patients who received <54 Gy (p=0.023). The most common acute side effect was grade 1-2 skin toxicity. As a late side effect of radiotherapy, soft tissue fibrosis was detected in 10 patients and lymphangitis was seen in 1 patient. One patient developed radiation-induced sarcoma. CONCLUSION: According to our results, radiotherapy is especially effective in recurrent disease and provides a high local control rate in the patients received more than 54 Gy.

Long-term treatment results in soft tissue sarcomas of the thoracic wall treated with pre-or-postoperative radiotherapy--a single institution experience.

OBJECTIVE: To evaluate the long term results among patients with soft tissue sarcoma of the thoracic wall. MATERIALS AND METHODS: Twenty-six patients who were treated with pre-or postoperative radiotherapy between December 1980-December 2007, with a diagnosis of soft tissue sarcoma of the thoracic wall were retrospectively evaluated. RESULTS: The median age was 44 years (14-85 years) and 15 of them were male. A total of 50% of patients were grade 3. The most common histologic type of tumor was undifferentiated pleomorphic sarcoma (26.9%). Tumor size varied between 2-25 cm (median 6.5 cm). Seventeen of the cases had marginal and 9 had wide local resection. Four cases received preoperative radiotherapy and 22 postoperative radiotherapy. Six of the patients with large and high grade tumors received chemotherapy. Median follow-up time was 82 months (9-309 months). Local recurrence and metastasis was detected in 34.6% and 42.3% of patients, respectively. Five- year local control (LC), disease-free survival (DFS), overall survival (OS), and disease-specific survival (DSS) were 62%, 38%, 69%, and 76% respectively. On univariate analysis, the patients with positive surgical margins had a markedly lower 5-year LC rate than patients with negative surgical margin, but the difference was not significant (43% vs 78%, p=0.1). Five-year DFS (66% vs 17%) and DSS (92% vs 60%) rates were significantly worse for the patients who had high grade tumors (p=0.01, p=0.008 respectively). CONCLUSIONS: Tumor grade and surgical margin are essential parameters for determining the prognosis of thoracic wall soft tissue sarcoma both in our series and the literature.

Non-metastatic Ewing's sarcoma family of tumors of bone in adolescents and adults: prognostic factors and clinical outcome-single institution results.

AIMS AND BACKGROUND: There is limited data regarding outcomes of Ewing's sarcoma family of tumors in adolescents and adults compared with the same tumors in childhood. The aim of the study was to analyze prognostic factors and treatment results in a cohort of adolescents and adults with non-metastatic skeletal Ewing's sarcoma family of tumors. METHODS AND STUDY DESIGN: From 1992-2008, 90 adolescents and adults with Ewing's sarcoma family of tumors of the bone were referred to our institution. Sixty-five (72%) non-metastatic patients with analyzable data and treated in our institution were retrospectively evaluated. All patients were treated with alternated chemotherapy regimens administered every 3 weeks. The local treatment modality was selected according to tumor and patient characteristics. RESULTS: The median age was 21 years (range, 13-50). Most patients (74%) were >17 years of age. Forty-six percent of the tumors were located in the extremities. Local therapy was surgery in 45 patients and radiotherapy alone in 19 patients. Twenty-one patients received preoperative and 13 patients postoperative radiotherapy. Median follow-up was 43 months (range, 7-167). The 5-year event-free and overall survival rates for all patients were 44% and 49%, respectively. On univariate survival analysis, event-free and overall survival were worse for patients >17 years of age, tumor size >8 cm in diameter, an axial location, positive surgical margins, and poor histopathological response (<90% necrosis). Age, tumor site and tumor size on event-free and overall survival remained significant on multivariate analysis. CONCLUSIONS: We identified age, tumor size, and tumor site as independent prognostic factors, in accord with the Western literature. These patients require novel treatment modalities.

Neoadjuvant treatment with preoperative radiotherapy for extremity soft tissue sarcomas: long-term results from a single institution in Turkey.

BACKGROUND: To assess the long term clinical outcome of preoperative radiotherapy with or without chemotherapy followed by limb sparing surgery in patients with non-metastatic soft tissue sarcomas (STS) of the extremities. MATERIALS AND METHODS: Sixty patients with locally advanced STS were retrospectively analyzed. The median tumor diameter was 12 cm. All patients were treated with preoperative radiotherapy delivered with two different fractionation schedules (35Gy/10fr or 46-50Gy/23-25fr). Neoadjuvant chemotherapy was added to 44 patients with large and/or high grade tumors. Surgery was performed 2-6 weeks after radiotherapy. Chemotherapy was completed up to 6 courses after surgery in patients who had good responses. RESULTS: Median follow-up time was 67 months (8-268 months). All of the patients had limb sparing surgery. The 5-year local control (LC), disease free (DFS) and overall survival (OSS) rates for all of the patients were 81%, 48.1% and 68.3% respectively. 5-year LC, DFS and cause specific survival (CSS) were 81.7%, 47%, 69.8%, and 80%, 60%, 60% in the chemoradiotherapy and radiotherapy groups, respectively. On univariate analysis, patients who were treated with hypofractionation experienced significantly superior LC, DFS and CSS rates with similar rates of late toxicity when compared with patients who were treated with conventional fractionation and statistical significance was retained on multivariate analysis. CONCLUSIONS: Treatment results are consistent with the literature. As neoadjuvant chemoradiotherapy provides effective LC and CSS with acceptable morbidity, it should be preferred for patients with large and borderline resectable STS.

Gastrointestinal stromal tumor of the rectum with scapular metastasis: a case report.

INTRODUCTION: Gastrointestinal stromal tumors are rare tumors. They commonly metastasize within the abdominal cavity, particularly to the liver. Less commonly, metastases can be found in the bone. CASE PRESENTATION: We here present a case of metastasis to the scapula in a 54-year-old Caucasian male patient with an advanced gastrointestinal stromal tumor, which was subsequently successfully treated with resection and sunitinib. CONCLUSION: The present study is, to the best of our knowledge, the second to describe scapular metastasis of a gastrointestinal stromal tumor. Our patient was treated by scapulectomy. The overwhelming majority of scapular tumors are metastases that arise from soft tissue, hepatocellular and thyroid tumors. Gastrointestinal stromal tumor metastasis occurs rarely. Scapular surgery can successfully provide local control of the disease. After the surgery, patients should continue with medical treatment.

Extraskeletal Ewing's sarcoma family of tumors in adults: prognostic factors and clinical outcome.

OBJECTIVE: The aim of this study was to evaluate prognostic factors, survival rate and the efficacy of the treatment modalities used in patients with extraskeletal Ewing's sarcoma. METHODS: Data of patients with extraskeletal Ewing's sarcoma followed up at our center between 1997 and 2010 were retrospectively analyzed. RESULTS: The median age of 27 patients was 24 years (range, 16-54 years). The median follow-up was 31.8 months (range, 6-144 months). Tumor size was between 1.5 and 14 cm (median: 8 cm). Eighty-five percent of patients had localized disease at presentation and 15% had metastatic disease. Local therapy was surgery alone in 16% of patients, surgery combined with radiotherapy in 42% and radiotherapy alone in 27%. All patients were treated with vincristine, doxorubicin, cyclophosphamide and actinomycin-D, alternating with ifosfamide and etoposide every 3 weeks. In patients with localized disease at presentation, the 5-year event-free survival and overall survival were 59.7 and 64.5%, respectively. At univariate analysis, patients with tumor size ≥ 8 cm, high serum lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy and positive surgical margin had significantly worse event-free survival. The significant predictors of worse overall survival at univariate analysis were tumor size 8 ≥ cm, high lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy, radiotherapy only as local treatment and positive surgical margin. CONCLUSIONS: Prognostic factors were similar to primary osseous Ewing's sarcomas. Adequate surgical resection, aggressive chemotherapy (vincristine, doxorubicin, cyclophosphamide and actinomycin-D alternating with ifosfamide and etoposide) and radiotherapy if indicated are the recommended therapy for patients with extraskeletal Ewing's sarcoma.

Low-grade osteosarcoma, review of 15 cases in a series of 156 osteosarcoma cases.

OBJECTIVE: Osteosarcoma of the bone accounts for approximately 20% of all primary malignant bone tumors. Most cases (75-85%) are high-grade osteosarcomas. We aimed to introduce low-grade osteosarcomas that we found by retrospectively scanning our archives because of the diagnostic difficulties, rarity and different therapeutic approach required. MATERIAL AND METHOD: We found 156 osteosarcoma cases in the archives of the Department of Pathology of Istanbul University, Cerrahpasa Faculty of Medicine covering the period 2000 to 2010. These cases included 141 high-grade and 15 low-grade osteosarcomas. RESULTS: Low-grade osteosarcomas made up 10.4% of all osteosarcomas. Age and sex distribution were 19 to 54 (median 33.6) with 9 females and 6 males. Most cases were located in the distal femur while others were in the proximal tibia, the left wrist, the distal tibia and the right knee. The tumors were generally made up of relatively well-formed trabeculae of woven bone surrounded by a moderately cellular spindle cell proliferation entangled in collagen fibers. Thirteen cases were reported as parosteal osteosarcoma, one as intramedullary well-differentiated osteosarcoma, and one as low-grade osteosarcoma metastasis in the lung. CONCLUSION: Low-grade osteosarcomas account for approximately 3-5% of all osteosarcomas. They are more commonly seen in women and 9 cases out of 6 were female in our series. The average age was 33.6 (19-54). The distal femur was the most common localization as is typical in osteosarcomas. In contrast to high-grade lesions, surgery will be sufficient in low-grade osteosarcomas. The survival rate ranges from 90% to 100%. Differentiating low-grade osteosarcomas from high-grade osteosarcomas and benign lesions and using a multidisiplinary approach for the diagnosis, treatment and follow-up periods are of vital importance.

[A case of brown tumor mimicking fibrous dysplasia in a patient with chronic renal failure]. / Kronik böbrek yetersizligi olan bir hastada fibröz displaziye benzeyen kahverengi (brown) tümör.

Renal osteodystrophy is one of the major causes of morbidity in patients receiving long-term dialysis treatment for renal failure and after transplantation. Its clinical implications include high-turnover bone disease, low-turnover bone disease, osteomalacia, osteosclerosis, and osteoporosis. A 13-year-old boy who had been on dialysis treatment for renal failure was admitted with a pathologic supracondylar femur fracture after a minor trauma. Radiological studies showed cystic lesions in the femoral supracondyle, left acetabular roof, and right proximal and distal tibia. Based on radiologic appearances of the lesions and on histopathologic findings of the lesion excised from the right distal tibia, brown tumor and fibrous dysplasia were considered in the differential diagnosis. Initially, serum parathyroid hormone level was slightly increased and calcium level was normal, but during follow-up, serum parathyroid hormone level increased significantly, enabling the diagnosis of brown tumor.

The role of preoperative radiotherapy in nonmetastatic high-grade osteosarcoma of the extremities for limb-sparing surgery.

PURPOSE: To assess the role of preoperative radiotherapy in patients with nonmetastatic high-grade osteosarcoma of the extremities for limb-sparing surgery and to compare the response of neoadjuvant therapies, local control, and survival with the literature results. METHODS AND MATERIALS: Forty-six patients with osteosarcoma of the limbs who were treated within a limb salvage protocol including preoperative radiotherapy and chemotherapy between 1987 and 2002, were retrospectively analyzed. Median age was 17 years (range, 14-66 years). Treatment was started with neoadjuvant chemotherapy. Cisplatin, epidoxorubicin, ifosfamide, and methotrexate were used in different combinations. Preoperative radiotherapy was applied, usually between the second and third cycle of chemotherapy. Radiotherapy was given (35 Gy in 10 fractions) to 44 patients. Two patients were treated with 46 Gy at 2 Gy/day. Definitive surgery was administered after the third course of chemotherapy. Chemotherapy was complete 6 courses postsurgery. RESULTS: Median follow-up time was 44 months (range, 2-154 months). Forty-four patients had limb-sparing surgery, whereas 2 had amputation. Tumor necrosis rate was >/=90% in 87% of the patients (Huvos Grade 3-4). Two patients had local failures, and 26 patients (56.5%) had distant metastases. The 5-year local control and overall survival rates were 97.5% and 48.4%, respectively. On univariate analysis, age