Murat Hiz

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 Ewings sarcoma. METHODS Data of patients with extraskeletal Ewings 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 Ewings 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 Ewings sarcoma.

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.

A case of brown tumor mimicking fibrous dysplasia in a patient with chronic renal failure

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.

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

IntroductionGastrointestinal 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 presentationWe 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.ConclusionThe 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.

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, Cerrahpaşa 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.

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 Ewings 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 Ewings sarcoma family of tumors. Methods and Study Design From 1992–2008, 90 adolescents and adults with Ewings 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.

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.

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.

Cerrahpaşa Tıp Fakültesi Ewing sarkom deneyimi: 1983 2003 Orijinal Araştırma

Amac: Bu calismada klinigimizde tek bir merkezde tedavi gormus Ewing sarkom olgulari geriye donuk olarak incelenmistir Gerec ve yontem: Ocak 1983 Ocak 2003 yillari arasinda Cerrahpasa Tip Fakultesi Cocuk Hematoloji Onkoloji bolumune basvuran 16 yas ve alti daha once tedavi gormemis biyopsi ile Ewing sarkom tanisi kesinlesmis 25 olgu geriye donuk olarak incelenmistir Hastalarimizin sagkalim sonuclari Kaplan Meier yontemine gore hesaplanmistir Bulgular: 25 Ewing sarkom olgunun ortanca yasi 9 yas dagilim ise 3 15 yas idi Tani aninda 16’sinda yerel tutulum 9’unda ise uzak yayilim mevcuttu Ortalama 50 aylik izlem suresi 4 108 ay sonucu 4 yillik olaysiz sagkalim ve toplam sagkalim sureleri sirasi ile 30 8 ve 31 1’dir Cikarim: Cok ilacli yogun kemoterapi uygulamalarinin ve yerel denetim yontemlerinin gundeme girmesine ragmen sagkalim sonuclarinda istenen basari saglanamamistir Anahtar Kelimeler: cocuk Ewing sarkom kemoterapi

Total agenesis of five cervical vertebrae: brief report

THE JOURNAL OF BONE AND JOINT SURGERY at five months and the right at seven months. At nine months he had protective sensation to the end ofhis toes. At one year (Fig. 2) he was walking freely and climbing stairs without an aid. He is very grateful for his surgery and has had no psychiatric relapse. Discussion. Although Chen, Yang and Chang (1983) in their series of 250 reimplants had included 35 of the lower limb, few cases have been reported in western literature. The largest detailed series of nine reimplants had five failures, but the two sharp (clean-cut) amputations were both successful (Kutz, Jupiter and Tsai 1983). In the psychiatric literature Stewart and Lowrey (1980) and De Muth, Strain and Lombards-Maher (1983) have reported on self amputation. Both groups feel that replantation is well worthwhile in the acute psychiatric patient, and Stewart reported no reamputations during a five to eight year follow-up. The psychiatrist in our case feels that reimplantation helped the patient’s psychiatric recovery. The significant factors contributing to the successful outcome were : 1) the injury was a sharp one with little crushing; 2) the feet were correctly dealt with by the emergency services ; 3) the tibial shortening avoided tension in the vessels and nerves and allowed primary skin closure ; 4) early weight bearing was protected ; and 5) bony union and recovery of sensation were obtained without skin breakdown.