Harzem Ozger

Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1.

We report 4 cases of malignant peripheral nerve sheath tumors (MPNST) with neurofibromatosis type 1 (NF1). Mean age was 29.5. Two of them had a family history. Three of them were male. All of them had enlarging mass and pain in the background of neurofibromas. Locations were popliteal, thigh and forearm. The masses were greater than 5 cm in diameter in each case. In two cases the mass was showing continuity with a nerve. One patient had a nonossifying fibroma as well as a MPNST. Wide excision and radiotherapy were applied to three of the patients. One of them did not take any therapy after surgical resection. Two of the patients died of lung metastases after a mean period of 12.5 months. In a majority of NF1 patients MPNST emerges from a preexisting neurofibroma. The patients with NF1 are at greatest risk for developing sarcomas, so they should be followed closely.

CT and MR characteristics of hibernoma: six cases

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.

Neuropathic arthropathy of the shoulder associated with syringomyelia: a report of six cases

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.

A Phase II Study of Cisplatin, Ifosfamide and Epirubicin Combination Chemotherapy in Adults with Nonmetastatic and Extremity Osteosarcomas

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/m2, cisplatin 100 mg/m2 on day 1 and ifosfamide 2.0 g/m2/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.

Surgical treatment of malignant tumors of the foot and ankle

BackgroundThe 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.MethodsFour 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.ResultsThe 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.ConclusionWide 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.

Childhood Osteosarcoma: Multimodal Therapy in a Single-Institution Turkish Series

Between January 1990 and December 2006, 123 patients<or=16 years with the histopathologic diagnosis of osteosarcoma were treated with a chemotherapy regimen comprising epirubicin, cisplatin, and ifosfamide. The mean follow-up time was 36 months (range 2-219 months). Among the 94 patients analyzed, 68 patients (72.3%) were alive at the time of the analysis. A total of 26 patients (13 each with nonmetastatic and metastatic disease) died; 20 of these (9 with nonmetastatic disease and 11 with metastatic disease) died of disease; 5, of chemotherapy toxicity, and 1, of nonmetastatic disease from acute nonlymphoid leukemia 13 months following the cessation of osteosarcoma therapy. The estimated 5- and 10-year Overall Survival (OS) rates for all patients were 64.7% (95% confidence interval [95% CI] 74.8-52.94%) and 62.2% (95% CI 74.6-49.9%), respectively. The Event Free Survival (EFS) rate for all patients was 51.8% (95% CI 40.2-63.4%) at both 5 and 10 years. The estimated 5- and 10-year Overall Survival (OS) rates for patients with nonmetastatic disease were 78.3% (95% CI 66.9-89.7%) and 75.1 (95% CI 62.6-87.6%), respectively; this 5-year rate was significantly superior to that of patients with metastatic disease, 13.5% (95% CI 0-30.8%) (p<0.001). The estimated EFS rate for patients with nonmetastatic disease was 62.4% (95% CI 49.9-79.9%) at both 5 and 10 years and was significantly better than the 5-year EFS of 6.9% (95% CI 0-19.9%) in patients with metastatic disease (p<0.001). Progression during preoperative chemotherapy was encountered in 18 patients (19.1%), 11 of whom had metastatic disease at diagnosis. Four patients (three with nonmetastatic disease and one with metastatic disease) underwent salvage treatment consisting of early surgical intervention and preoperative radiation. The estimated 5- and 10-year OS rates were 13% (95% CI 0-29.7%) for patients who had progression during treatment; this rate was significantly inferior to both the 5- and 10-year OS rates for patients without progressive disease, which were 78.2% (95% CI 66.1-90.4%) and 75% (95% CI 61.9-83.1%), respectively (p<0.001). A total of 33 patients experienced relapse and/or progression at a median time of 9 months (range 0-40 months). Histologic response (<90% necrosis vs. >or=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.

Avulsion fractures involving the straight and reflected heads of the rectus femoris

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.

Biological reconstruction of the femur using double free vascularized fibular autografts in a vertical array because of a large defect following wide resection of an osteosarcoma: a case report with 7 years of follow-up.

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.

Forequarter amputation as a life-saving procedure

Forequarter amputation is performed for high-grade malignant tumours in the proximal part of the upper extremity with palliative or curative intentions. Two cases are included in this report of patients who presented in need of urgent surgical intervention. Both patients had an actively bleeding, ulcerated or fungating mass and were haemodynamically unstable. The purpose of this report is to highlight the importance of emergency surgical intervention for such selected patients.

Applications of external fixation for management of complications associated with musculoskeletal tumors and related surgery

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), Ewings sarcoma (n=3), hereditary multiple exostosis (n=3), chondrosarcoma (n=2), synovial sarcoma (n=2), Olliers 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 Olliers 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.