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The differential diagnosis of cystic lesions of the skeleton is multifarious. Besides patient age, the localization and radiologic morphology provide important information for a closer differentiation. Juvenile and aneurysmal bone cysts represent two frequent entities in growing patients. The fluid content of the cysts helps in distinguishing between juvenile and aneurysmal bone cysts. Whereas juvenile bone cysts contain clear fluid, the content of aneurysmal bone cysts consists of blood combined with solid tissue. With respect to progression, aneurysmal bone cysts show a higher activity than solitary bone cysts. The treatment of juvenile bone cysts usually consists of curettage including filling with bone replacement material. For aneurysmal bone cysts the additional use of adjuvants is recommended. Bone cement is preferably used for filling. It shows favorable properties for prophylaxis of recurrence and facilitates the recognition of relapses. Both juvenile and aneurysmal bone cysts often show recurrences.

The differential diagnosis of cystic lesions of the skeleton is multifarious. Besides patient age, the localization and radiologic morphology provide important information for a closer differentiation. Juvenile and aneurysmal bone cysts represent two frequent entities in growing patients. The fluid content of the cysts helps in distinguishing between juvenile and aneurysmal bone cysts. Whereas juvenile bone cysts contain clear fluid, the content of aneurysmal bone cysts consists of blood combined with solid tissue. With respect to progression, aneurysmal bone cysts show a higher activity than solitary bone cysts. The treatment of juvenile bone cysts usually consists of curettage including filling with bone replacement material. For aneurysmal bone cysts the additional use of adjuvants is recommended. Bone cement is preferably used for filling. It shows favorable properties for prophylaxis of recurrence and facilitates the recognition of relapses. Both juvenile and aneurysmal bone cysts often show recurrences.

Hillmann A, Weist R, Fromme A, Volker K, Rosenbaum D. Sports activities and endurance capacity of bone tumor patients after rotationplasty. Objectives To investigate the preferred types of sports activities of patients with rotationplasty and to measure their physiologic performance characteristics through treadmill ergometry. Design Cross-sectional, descriptive analysis and repeated measures of different velocities. Setting Biomechanics research laboratory. Participants Patients (n=61) with rotationplasty after bone tumor surgery, 30 of whom participated in a functional trial (treadmill), and a control group (n=20). Interventions Not applicable. Main Outcome Measures Patients' participation in sports compared with that of the healthy population, treadmill performance at 2 or 3 different speeds, heart rate, lactate accumulation, oxygen consumption, ventilatory equivalent, efficiency, respiratory minute volume, and respiratory quotient. Results High activity in sports participation (85%) in most common sports (8 competitive, 17 sports club members, the remaining subjects were recreational athletes). At the same treadmill speed, lactate accumulation and all cardiorespiratory functions were higher in rotationplasty patients than in the control group. Conclusions Patients can re-engage in a high level of physical activity after rotationplasty for bone tumor treatment. This physical activity is necessary if patients want to maintain or improve a desired level of sports activity.

Background Limb salvage is viable in the majority of patients with malignant bone tumors, but especially in case of extensive tumors and bad soft tissue conditions, it is challenging in upper extremity. Objectives/Method The clinical and radiological results of 21 patients, who had free vascularized fibular grafts (VFG), for diaphyseal (14), and epipyseal (7) defect reconstruction of the upper extremity, are presented. The indications for VFG were resection after osteosarcoma (9 cases), Ewings sarcoma (9 cases), chondrosarcoma (1 case), rhabdomyosarcoma (1 case), and 1 case of fibrous dysplasia. The 20 malignant tumors were staged as follows: 2a (1), 2b (18), 3 (1). The mean follow-up was 43.6 months (min 6.0–max 131.9). Functional results were described and graded quantitatively according to the MSTS-score. Results Results were satisfactory with regard to pain, emotional acceptance, manual dexterity, and function. Lifting ability was decreased in two patients. Hypertrophy index was 31% (min 13%–max 71%). Main complications were fracture (5), pseudoarthrosis (4), prolonged wound healing (4), temporary nerve irritation (2), and deep infection (1). Re-operation was required in eight patients (12 operations). Conclusion VFG offers a good possibility for biological reconstruction of large skeletal defects, with an acceptable complication and re-operation rate. When conservative treatment of complications was not successful, further surgery led to recovery in the majority of cases. J. Surg. Oncol. 2006;94:114–127. © 2006 Wiley-Liss, Inc.

Six patients with giant cell tumor of the spine had surgery between 1981 and 1995. Three lesions were located in the scrum, two lesions were in the thoracic spine, and one lesion was in the lumbar spine. Preoperatively, all patients had local pain and neurologic symptoms. Two patients had cement implanted after curettage or intralesional excision of the sacral tumor; one patient had a local relapse. After the second curettage and cement implantation, the tumor was controlled. One patient with a sacral lesion had marginal excision and spondylodesis; no relapse developed. Two patients with thoracic lesions had planned marginal excision and spondylodesis; the margins finally became intralesional, but no relapse developed. One patient with a lumbar lesion had incomplete removal of the tumor and received postoperative irradiation. At the final followup (median, 69 months), five of six patients were disease-free and one patient died of disease progression. Two of the five surviving patients had pain after standing or neurologic problems. Although some contamination occurred, planning a marginal excision of the lesion seems beneficial for vertebral lesions above the sacrum. Total sacrectomy of a sacral lesion seems to be too invasive when cement implantation can control the lesion.

Osteoblastomas and osteoid osteomas of the spine are relatively rare bone-forming tumors. Between 1980 and 1999, nine patients with osteoid osteoma and 13 patients with osteoblastoma had surgery for their tumors. Four tumors were in the cervical spine, six tumors were in thoracic spine, 10 tumors were in the lumbar spine, and two tumors were in the sacrum. The average duration between onset of pain and surgery was 16.6 months in 12 patients treated in the 1980s and 8.6 months in 10 patients treated in the 1990s. Seventeen patients had scoliosis. In nine of 10 patients with magnetic resonance imaging scans, high signal intensity areas in the muscles and bone around the lesion were seen. Two of nine patients with osteoid osteoma and nine of 13 patients with osteoblastoma had neurologic disorders before treatment. All patients had open resection of the lesions. Two patients with osteoid osteoma had relapse because of incomplete resection, necessitating a second excision. In 16 of 17 patients with preoperative spinal deformity, the deformity improved during followup. With development of modern imaging techniques, exact surgical planning may become possible; however, in some cases, intraoperative complete resection of the lesion still is difficult.

BACKGROUND Due to the low incidence rate, the optimal strategy for the treatment of patients with spinal osteosarcoma is unknown. METHODS Twenty-two patients with osteosarcoma of the spine (15 with tumors of the sacrum and 7 with tumors at other sites) who received chemotherapy according to the Cooperative Osteosarcoma Study Group protocol were analyzed. Six patients presented with metastasis, and 16 patients had no evidence of metastasis at the time of entry into the protocol. Of 12 patients who underwent excision of their tumors, 2 patients underwent wide excision, 3 patients underwent marginal excision, and 7 patients underwent intralesional excision. Eight patients received irradiation: Six patients received conventional radiotherapy only, one patient received neutron beam therapy, and one patient received samarium-153-ethylene diamine tetramethylene phosphonate therapy. Follow-up ranged between 24 months and 105 months (median, 47 months). RESULTS The median survival was 23 months, and three patients have survived without disease for > 6 years. Patients with primary metastases (P = 0.004), large tumors (P = 0.010), and sacral tumors (P = 0.048) had lower overall survival compared with patients who had no metastasis, small tumors, and nonsacral tumors, respectively. There was a significant difference in overall survival between 5 patients who underwent either wide or marginal surgery and 17 patients who underwent either intralesional surgery or no surgery (P = 0.033). Among 17 patients who underwent no surgery or intralesional surgery, overall survival tended to be better in 7 patients who received irradiation compared with the overall survival in 10 patients who did not receive irradiation (P = 0.059). CONCLUSIONS Patients with metastases, a large tumors, and sacral tumors had a poor prognosis in the current study with small numbers of patients. Wide or marginal excision of the tumor improved survival. Patients with osteosarcoma of the spine should be treated with a combination of chemotherapy and at least marginal excision for those with surgically accessible tumors. Postoperative radiotherapy may be beneficial. Cancer 2002;94:1069–77. © 2002 American Cancer Society. DOI 10.1002/cncr.10258

After resection of a malignant bone tumor of the femur, rotationplasty is considered a treatment option in addition to other limb salvage procedures such as endoprosthetic replacement, allograft, or autograft reconstruction. A Type B IIIa rotationplasty is indicated when the tumor involves the total femur or when skip lesions are detected. After the total femur is resected, the lateral tibial plateau is placed into the acetabulum after rotating the tibia and foot segment 180 degrees. Eight patients at an average age of 5.9 years underwent a rotationplasty Type B IIIa at the authors' institution. After a median followup of 59.6 months (range, 24-86 months) the clinical results were excellent and the functional status according to the criteria of the Musculoskeletal Tumor Society was good (23 of 30 points). In electromyographic analysis the muscle activity of the affected leg revealed good function of the stance and the swing phase muscles according to their new function with comparable amplitudes to the unaffected limb. The kinematics (range of motion of the hip and knee) were slightly assymmetric. Gait analysis showed a slight lateral trunk lean over the ipsilateral limb with reduced joint moment. All patients had full weightbearing ability. Remodeling of the tibial plateau into a round form mimicking a new femoral head is confirmed by radiographs and magnetic resonance imaging scans. In young children with a tumor of the total femur, rotationplasty Type B IIIa is a good treatment modality with excellent clinical and good functional results. The electromyographic and gait analysis data underscore a good functional restoration of gait after rotationplasty.

The purpose of this study was to assess the diagnostic performance of computed direct magnification radiography in diagnosing bone tumors as compared with conventional radiography. Ninety-one patients with primary bone tumors and tumor-like lesions were radiographed with conventional and magnification techniques. All radiographs were analyzed by one orthopedic surgeon and two radiologists and the findings were correlated with histopathology. Two microfocal X-ray units were used for computed direct magnification radiography with a focal spot size of 20–130 μm. Using magnification versus conventional radiography, the diagnosis of benign and malignant lesions as well as the individual tumor diagnosis was obtained with higher accuracy (85% versus 71% and 69% versus 51%, respectively, P < 0.01). Margins of destruction, periosteal reactions, and matrix patterns were evaluated with higher accuracy by all observers (P < 0.01). We conclude that computed direct magnification radiography may improve evaluation and diagnosis of bone tumors.

To reduce the disability after hip disarticulation, we developed a special surgical procedure in patients having a proximal femoral tumor with a large tumor involving the sciatic nerve or neoplasms involving the tibia and femur. The hip was disarticulated, but we preserved a musculocutaneous flap. A modular endoprosthesis was then placed in the acetabulum or, in case of an extraarticular resection of the hip joint, it was placed in the iliac bone. A trevira tube was used for reconstruction of the joint capsule and fixation of soft tissues. We performed this procedure in 5 patients who had a good functional outcome.

Twelve patients aged between 10.9 and 64.7 (mean 28.5) years with a malignant tumour of the knee region underwent a rotationplasty after failed primary limb salvage procedure. The reasons for failure which finally lead to surgery were recurrent infection in 10 patients and local recurrence of the tumour in 2. The number of operations before the rotationplasty was performed was between 2 and 23 (mean 6.7). According to the primary tumour site, 9 patients underwent a rotationplasty type A1, 3 patients type A2, and 1 patient type BII. In 9 patients the rotationplasty was successful, but 3 patients finally had to undergo amputation. Intraoperative preservation of the vessels was difficult in these 3 patients due to infection and oedema of the arteries or massive fibrous tissues after the previous surgery. After rotationplasty, 3 of 9 patients had to undergo additional surgery because of thrombosis, pseudarthrosis and infection (n = 5, range 1-2). The mean follow-up after rotationplasty was 34.9 (range 13-65) months. The mean functional status according to the MSTS criteria in patients after rotationplasty scored 21.3 of 30 points. In the group of amputees, the score was 19 (range 16-22). Concerning the health-related quality-of-life, the mean score in physical functioning was 76.3 in the group with a rotationplasty versus 50.0 in the group of amputees. Patients with a rotationplasty reached a higher score of global health status (77.1 vs 58.3). Based on the present results we are convinced that rotationplasty can be recommended as a treatment option after a failed limb salvage procedure.

We treated two cases of osteosarcoma in siblings. After intensive chemotherapy and surgery, both patients are alive after 9 and 4 years without any signs of disease. We report the course of disease in these patients in detail and review the literature on 59 patients with familial osteosarcomas.

Purpose: To define MR imaging characteristics of primary aneurysmal bone cyst. Materials and Methods: MR imaging studies of 38 patients with histologically proven primary aneurysmal bone cyst were reviewed with reference to morphological features, signal characteristics, and patterns of contrast-enhancement. Result: Most lesions were well marginated towards bone and soft tissues (95%) either surrounded by a complete (84%) or incomplete (16%) rim of low signal intensity on images of all pulse sequences. Frequent features were polycyclic margins (84%), cortical expansion (87%), cystic spaces (100%), contrast-enhancing cyst walls (100%), internal septations (89%), fluid levels (71 %) and diverticula-like projections of cyst walls (68%). Solid tissue components could be identified by MR imaging in all lesions which, on pathological examination, contained larger portions of solid material (18%). Edema of surrounding soft-tissues was observed in 29% of the cases. Conclusions: Primary aneurysmal bone cysts demonstrate a relatively uniform MR imaging appearance, which reflects the patho-anatomic composition of the lesion.

Objective . The present study investigated the plantar and dorsal foot loading patterns inside the prosthesis of patients after rotationplasty. Design . In vivo foot pressure distribution measurements during free walking in rotationplasty patients at an average follow-up of 10.4 years after surgery. Methods . Fourteen subjects participated in the study and were measured during free walking. Capacitive pressure sensitive insoles were worn inside the shaft of the prosthesis either on the plantar or the dorsal aspect of the foot. Between 5 and 15 steps (mean 10.5) during full gait were selected and averaged to present a typical loading pattern for the individual patient. Results . The measurements were reproducible and indicated that the main loading areas of the rotated foot inside the prosthesis are medially on the dorsal aspect and in the heel and toe region or the heel and midfoot region on the plantar aspect. The dorsal loading area was smaller than the plantar area ( P =0.003). The force was higher on the plantar than on the dorsal aspect ( P =0.005) but the loading time was shorter ( P =0.008). In patients with callosities the high pressure areas coincided with the affected regions. Conclusions . The information gained from the pressure measurements support the understanding of foot loading characteristics in this anatomically and biomechanically unusual situation. This knowledge may be used to support the fitting process of new prosthetic designs for patients after rotationplasty. Relevance Foot loading capacity is one of the main factors limiting the activity level of rotationplasty patients. Therefore, a better understanding of foot loading characteristics might help to prevent overloading of certain foot structures and eventually improve the prosthetic fit with a direct benefit to the individual patient.

We investigated the cytotoxic responsiveness of 40 cell lines derived from representatives of the Ewing's sarcoma family of tumours (ESFT), i.e., Ewing's sarcoma (ES), peripheral primitive neuroectodermal tumour (pPNET) and Askin tumour (AT), to tumour necrosis factor–related apoptosis-inducing ligand (TRAIL). Incubation with TRAIL at 100 ng/ml induced cell death at 24 hr in 19 of 26 ES, 11 of 12 pPNET and 2 of 2 AT cell lines. Half-maximal cell death concentrations (IC50 values) varied from 0.1 to 20 ng/ml. TRAIL displayed potent cytotoxic activity against freshly derived ESFT cell isolates. Cytotoxicity was associated with phosphatidylserine expression and internucleosomal DNA fragmentation, features characteristic of apoptosis. The apoptotic programme in the sensitive ESFT VH-64 cell line revealed TRAIL-induced activation of FLICE/MACH1 (caspase-8) and CPP32/Yama/apopain (caspase-3) and processing of the prototype caspase substrate poly(ADP-ribose) polymerase. In addition, TRAIL provoked a collapse of the mitochondrial transmembrane potential (ΔΨm), parallelled by a reduction in ATP levels and release of cytochrome c from mitochondria into the cytosol. Inhibition of caspase-8 and caspase-3 by zIETDfmk and zDEVDfmk, respectively, substantially prevented TRAIL-induced apoptosis. However, zIETDfmk, but not zDEVDfmk, reduced TRAIL-mediated ΔΨm dissipation, indicating that TRAIL causes mitochondrial dysfunction through caspase-8 acting upstream of mitochondria. While macromolecule synthesis inhibitors (actinomycin D, cycloheximide) augmented susceptibility to TRAIL in TRAIL-responsive cell lines, these agents did not render TRAIL-resistant cell lines susceptible to TRAIL. However, the proteasome inhibitor MG132 sensitised to TRAIL in resistant cell lines. Collectively, these results show that TRAIL initiates effective death in the vast majority (80%) of cell lines derived from ESFT. Since TRAIL provoked cell death in ESFT ex vivo, this cytokine may be a promising drug for the treatment of ESFT in vivo.Int. J. Cancer 88:252–259, 2000. © 2000 Wiley-Liss, Inc.

BACKGROUND This article reports on 241 patients each with pelvic Ewing sarcoma registered for studies in Germany, Austria, and the Netherlands from January 1, 1981, until January 31, 1994. One hundred sixty-four patients had localized disease and 87 had metastases at diagnosis (PMP). Eighty-four patients with localized disease were entered on protocol (PP) and 80 were followed (FP). METHODS Statistics included an analysis of event free survival by the Kaplan–Meier method and a Cox regression analysis of factors influencing prognosis. RESULTS In the Kaplan–Meier analysis, on February 1, 1995, the event free survival (EFS) rate was 32% at 12 years for all patients, 54% for PP, 25% for FP, and 13% for PMP. Cox regression analysis showed that response to chemotherapy, initial metastases, and less intense therapy were significant prognostic factors. Among patients who had surgery for local control, the histologic response to chemotherapy was analyzed in the surgical specimen and had a significant influence on survival: EFS 69% for PP with good response compared with 47% (P = 0.11) for patients with poor response, and for FP 56% versus 13% (P = 0.002). All PP with small tumors had relapse free survival, compared with 69% of patients with medium-sized tumors and 36% of patients with tumors larger than 200 mL (P = 0.006).The initial tumor volume was a significant predictor of survival. CONCLUSIONS Combined modality treatment has resulted in definitive improvement of prognosis for patients with localized pelvic Ewing sarcoma. However, the results for patients with metastases at diagnosis are still discouraging, and their treatment requires new approaches. Tumor load, responsiveness to chemotherapy, and adequate surgical margins are the major factors influencing the prognosis of patients with localized Ewing sarcoma of the pelvis. Cancer 1999;85:869–77. © 1999 American Cancer Society.

We treated a patient with large multiple chondrosarcomas derived from multiple cartilaginous exostoses. One sarcoma originated in the left pubic bone and the other sarcoma in the posterior aspect of the greater trochanter of the left femur. Thirty months after hindquarter amputation, the patient is alive without relapse. This is the first report of a patient with synchronous multiple malignant transformation of multiple cartilaginous exostoses.

Nine patients with Ewing's sarcomas and seven patients with osteosarcoma of the fibula were treated surgically. The bone defect after tumour resection ranged from 5 to 25 cm (median 14 cm). Ten sarcomas were located in the proximal and six in the diaphyseal or distal fibula. Nine of ten patients with sarcomas located in the proximal fibula underwent a resection of the tumour including the common peroneal nerve. In one patient with a tumour in the proximal fibula, the peroneal nerve was preserved; however, this patient underwent amputation because of surgery with an intralesional margin. In five patients with a tumour in the distal fibula, the peroneal nerve was preserved. However, two of these five patients underwent amputation as an adequate surgical margin could not be achieved during resection. All ten patients in whom the peroneal nerve was resected achieved satisfactory function by wearing a peroneal brace. In patients with Ewing's sarcoma of the proximal fibula, preservation of the common peroneal nerve may be chosen as an alternative possibility of resection.

Background Sacrococcygeal chordomas are difficult to manage. Methods The treatment results of 12 patients with sacral chordomas were analyzed. Four patients had local relapse before they were referred to our hospital: three patients previously underwent two surgeries, and one patient one surgery and irradiation (60 Gy). In this institute, 10 patients underwent surgery alone, and one had surgery plus postoperative irradiation. One patient underwent radiotherapy alone due to an inoperable lesion that reached to the L5 spine. For seven surviving patient, the average follow-up period was 50 months. Results One of four patients with a marginal margin and one of two patients with a contaminated margin developed local relapse. Of five patients with intralesional margins, four patients developed local relapse and two had metastasis to the spine and lungs 36 and 15 months after surgery, respectively. One patient with irradiation alone had metastasis to the lungs 15 months after initiation of treatment. Three patients died due to progression of the disease, one of a heart problem, and one of apoplexy. One of 10 patients with implantation of gentamycin beads after removal of tumor, developed infection. Conclusions For local control of sacral chordomas, an adequate surgical margin is important. Gentamycin beads may be effective to control postoperative infection of the dead space. J. Surg. Oncol. 64:274–279, 1997 © 1997 Wiley-Liss, Inc.

We treated 12 patients with a primary sarcoma of the tibia by resection and centralization of the ipsilateral vascularized fibula with preserved vascularization. The reconstruction was combined with an allograft. The median bone defect after tumor resection was 14 (8-24) cm. The anastomosis between the allograft and the recipient bone was stabilized by a plate or screws or Kirschner-wires. To reconstruct the soft tissues, 8 patients underwent a gastrocnemius plasty and 2 patients a free vascularized flap of the latissimus dorsi muscle. The mean follow-up period was 2.5 (2-3.5) years. 2 years after implantation, unionor = 75% of the anastomosis area was noted in 10 patients. A deformity or fracture of the distal junction occurred in 7 patients with a screw or Kirschner-wire fixation. Neither deformity nor fracture developed in the 5 patients with plate-fixation.

The influence of preoperative irradiation on surgical complications in 42 patients with Ewing's sarcoma was analysed. After preoperative irradiation and chemotherapy, 35 of 40 patients showed a good histological response and 25 of 40 patients had no viable tumour cells in the resected specimen. Local relapse alone did not develop, local relapse and metastasis developed in 2 patients and metastasis alone in 15 patients. Surgical complications appeared in 12 of 42 patients: 9 of 19 central tumours (19 pelvic lesions), 1 of 13 proximal and 2 of 10 distal tumours. Surgical complications after preoperative irradiation are distributed as follows: delayed wound healing 8, hematoma 2, thrombosis 2, skin infection 1, and abscess 1. On the other hand, complications appeared in 2 of 28 patients without preoperative irradiation: none in 9 patients having central tumours including 2 pelvic lesions, 1 in 12 patients with proximal tumours, and 1 in 7 patients with distal tumours. The multivariate regression test showed that the tumour site (central) is an influencing factor in the appearance of surgical complications. In central tumours, the surgical complication rate increases after preoperative irradiation; however, it is affected by the increase of the ratio of patients with pelvic tumours.

Objective. To evaluate the potentials of magnification radiography as compared with conventional radiography in diagnosing bone tumors. Design and patients. Sixty-two patients with primary bone tumors and tumorlike lesions underwent radiography with both conventional (non-magnified) and magnification (fivefold) techniques. All radiographs were analyzed by four radiologists and the findings correlated with the histopathology findings. The microfocal X-ray unit used for magnification radiography had a focal spot size of 20–130 μm. Digital luminescence radiography was employed with magnification, while normal film-screen systems were used with conventional radiography. Results. The diagnosis of benign and malignant lesions as well as the individual tumor diagnosis were determined with higher accuracy using magnification compared with conventional radiography (88% vs 75% and 71% vs 52%, p

Background Intralesional (IL) surgery for chondrosarcoma is an inadequate procedure; however, sometimes it cannot be avoided. The outcome of 26 patients (PTS) who underwent IL surgery for chondrosarcoma was analyzed. Methods Median follow-up time was 146.5 months. Eighteen tumors were located in the axial skeleton and 8 in the extremities. The histologic grade distribution of the chondrosarcoma was: 14 Grade 1, 8 Grade 2, and 4 Grade 3. Diagnostic problems were noted in 4 of the 18 central tumors and 7 of 8 extremity tumors. Results The 20-year relapse free survival rate for the 26 PTS who underwent IL surgery was 7% and was 64% for 38 PTS who underwent wide or marginal surgery (P = 0.03144). Conversely, the 20-year overall survival rate for the 26 PTS after IL surgery was 68% and was 66% for 38 PTS who underwent wide or marginal surgery (P = 0.03144). Conversely, the 20-year overall survival rate for the 26 PTS after IL (P = 0.2266). The overall survival rates for the PTS with tumors of histologic Grade 1 and 2 or 3 tumor was 85% and 44%, respectively (P = 0.02817). The overall survival rates for PTS with central tumors and extremital tumors were 57% and 87%, respectively (P = 0.0968). Conclusions IL surgery for chondrosarcoma often leads to local relapse. However, local relapse does not always result in metastases and death. The prognosis for PTS after IL surgery tends to differ according to tumor grade. After local relapse further limb salvage becomes difficult and many complications may occur.

We performed 22 reconstructions by allografts in patients with pelvic sarcoma: 14 Ewing's sarcomas, 7 chondrosarcomas, and 1 osteosarcoma. All patients with Ewing's sarcoma and osteosarcoma received chemotherapy. No patients with chondrosarcoma had adjuvant treatment. 12 reconstructions were iliosacral arthrodesis after resection of an ilium tumor, 1 was iliofemoral arthrodesis and 9 were pelvic reconstructions with total hip prosthesis after resection of an acetabulum tumor. In the surviving patients, the mean length of follow-up was 4 (2-6) years. 2 allografts fractured and 8 allografts developed an infection. The infection was commoner in patients who had chondrosarcomas, large tumors, and a long operation time. Neither chemotherapy nor radiotherapy increased the infection rate. All infected allografts had to be removed.

31 patients with primary Ewing's sarcoma of the ribs were treated according to the protocols of CESS 81, CESS 86P and CESS 86. The results of treatment were reviewed and analysed. 24 patients presented with localised disease and 7 with regional disease. 20 of 24 localised cases and 6 of 7 regional cases underwent tumour resection. All but 2 localised cases received irradiation. The cumulative relapse-free survival (RFS) rate of 31 patients was 61% at 12.8 years. Patients with poor prognosis had tumour of the upper ribs (P = 0.0338), the posterior component of the ribs (P = 0.0597), or regional disease (P = 0.0001). Tumour size, existence of pleural effusion, type of the surgical margin and response to chemotherapy were not significant prognostic factors. Most of the localised cases could be controlled by combined treatment, but in regional cases prognosis remained poor.

Im Jahr 2015 prasentierte sich die Sektion wieder auf nationalen Kongressen mit eigenen Sitzungen. Sowohl auf dem Deutschen Kongress fur Orthopadie und Unfallchirurgie als auch auf dem Deutschen Krebskongress waren die von der Sektion initiierten Veranstaltungen sehr gut besucht. Der Tumorkurs der DGOOC im Rahmen der Zusatzweiterbildung „Spezielle Orthopadische Chirurgie“ war auch im Jahre 2015 wieder ausgebucht und sties bei den Teilnehmern auf eine sehr gute Resonanz.

Background Degenerative musculoskeletal disorders are among the most frequent diseases occurring in adulthood, often impairing patients' functional mobility and physical activity. The aim of the present study was to investigate and compare the impact of three frequent degenerative musculoskeletal disorders -- knee osteoarthritis (knee OA), hip osteoarthritis (hip OA) and lumbar spinal stenosis (LSS) -- on patients' walking ability. Methods The study included 120 participants, with 30 in each patient group and 30 healthy control individuals. A uniaxial accelerometer, the StepWatch™ Activity Monitor (Orthocare Innovations, Seattle, Washington, USA), was used to determine the volume (number of gait cycles per day) and intensity (gait cycles per minute) of walking ability. Non-parametric testing was used for all statistical analyses. Results Both the volume and the intensity of walking ability were significantly lower among the patients in comparison with the healthy control individuals (p < 0.001). Patients with LSS spent 0.4 (IQR 2.8) min/day doing moderately intense walking (>50 gait cycles/min), which was significantly lower in comparison with patients with knee and hip OA at 2.5 (IQR 4.4) and 3.4 (IQR 16.1) min/day, respectively (p < 0.001). No correlations between demographic or anthropometric data and walking ability were found. No technical problems or measuring errors occurred with any of the measurements. Conclusions Patients with degenerative musculoskeletal disorders suffer limitations in their walking ability. Objective assessment of walking ability appeared to be an easy and feasible tool for measuring such limitations as it provides baseline data and objective information that are more precise than the patients' own subjective estimates. In everyday practice, objective activity assessment can provide feedback for clinicians regarding patients' performance during everyday life and the extent to which this confirms the results of clinical investigations. The method can also be used as a way of encouraging patients to develop a more active lifestyle.

Objective Despite its impact on the overall outcome and health-related quality of life (HRQOL) after knee surgery, physical activity has not been investigated directly using accelerometry or step monitoring during the first year after total knee arthroplasty (TKA) due to osteoarthritis (OA). Therefore, the present study aimed to evaluate the development of physical activity over 12 months after surgery and its relationship to clinical outcome and HRQOL. Methods Fifty-three patients scheduled for primary TKA due to OA were measured with the DynaPort ADL monitor and a step activity monitor preoperatively and at 2, 6, and 12 months of followup. Clinical outcome and HRQOL were investigated using the American Knee Society Score (KSS) and Short Form 36 (SF-36) health survey. Results Physical activity increased significantly within 12 months of followup (from mean ± SD 4,993 ± 2,170 gait cycles preoperatively to 5,932 ± 2,111 gait cycles; P = 0.003). Clinical outcome and HRQOL improved from baseline (mean ± SD KSS 88.9 ± 21.4, mean ± SD SF-36 43.1 ± 18.4) to 12 months of followup (mean ± SD KSS 188.6 ± 10.9; P = 0.001 and mean ± SD SF-36 82.5 ± 15.9; P = 0.001). Physical activity parameters did not correlate with clinical outcome. Conclusion TKA offers profound improvements of physical activity for the majority of patients. Despite these improvements and the excellent clinical outcome, most patients do not reach the level of physical activity reported for healthy subjects. The activity level after treatment seems to be influenced by physical activity behavior prior to surgery rather than by the treatment itself.

We analysed local recurrences in young children with aneurysmal bone cysts. Nine were boys and five were girls, aged 10 years or less. The sites of the lesions were distributed as follows: five femurs, four humerus, two tibia, one sacrum, one thoracic spine and one pubis. After intensive curettage, five patients underwent cementation and five patients bone grafting. Four patients underwent resection of the lesion. No patients received irradiation. One of the five patients who underwent cementation had a relapse and the lesion was controlled after the second curettage and cementation. One of the five patients who underwent bone grafting had a relapse, and he relapsed again after the second curettage and bone graft; finally his disease was controlled by resection of the lesion and bone grafting. After 55 months (median) of follow-up, all patients were disease-free. The local recurrence rate of aneurysmal bone cysts in children was not high in this hospital.

In order to assess the changes in talocrural joint contact stress after rotationplasty, 10 lower-leg cadaver specimens were axially loaded with 600 N and investigated in two loading situations: (1) Normal loading with a plantigrade foot; (2) in an equinus position of a simulated rotationplasty. Joint contact stress in the talar facet of the talocrural joint was determined with Fuji Prescale film cut to size and analyzed with digital image analysis for joint contact area, mean and peak pressure, contact force, and location of the load application on the trochlea tali. The results demonstrate a significant transfer on the loading zone to the posterior part of the talus (p=0.005), a significant reduction of the contact area (p=0.005) and force (p=0.005), and a significant increase of the mean (p=0.022) and maximum pressures (p=0.013). These results indicate that the rotationplasty causes pronounced changes in joint loading characteristics.

Complications after pelvic sarcoma surgery are frequent; however, the reports on complications are limited. Results of the authors' experience with 110 primary pelvic tumor resections and methods to achieve low complication rates for pelvic reconstruction are reported.From 1982 to 1996, 110 patients with pelvic sarcoma (42 Ewing sarcomas, 40 chondrosarcomas, 21 osteosarcomas, and 7 other malignant tumors) underwent surgery. Sixteen patients underwent implantation of a hemipelvic megaprosthesis, 13 patients had implantation of an allograft for sacroiliac arthrodesis, 12 patients had implantation of an autograft for sacroiliac arthrodesis, and 17 patients underwent hip transposition. There were 9 hindquarter amputations, 6 implantations of allograft and total hip endoprosthesis, 1 implantation of prosthesis with autograft, and 1 implantation of allograft and autograft. No skeletal reconstruction was done in 35 patients.Postoperative function was as follows: 37% in patients with prosthesis, 60% in allograft, 66% in autograft, 66% in hip transposition, 37% in amputation, and 79% without reconstruction. In total, 10/16 patients with prosthetic replacement, 9/13 with allograft implantation, 4/12 with autograft implantation, 7/17 with hip transposition, 5/9 with amputation, 6/6 with prosthesis and allograft, and 12/35 without skeletal reconstruction had complications. Frequent complications depending on the reconstruction were infection in 6/10 prostheses and in 5/13 allografts, leg length discrepancy in 2/12 autografts and 4/17 hip transpositions, hematoma in 3/9 amputations, and infection (6) and skin problems (5) in 6 prostheses with allograft.Because of the small number of complications and good function, autograft implantation after iliac resection and hip transposition after acetabular resection are advisable.

Twelve adult patients with pelvic sarcoma had implantation of a hemipelvic prosthesis. Eight patients had hemipelvic resection, and four patients had acetabulopubic resection. The implanted prosthesis was a special Vitallium prosthesis, which was specially designed for each patient with the aid of a computer. At a median followup of 57 months, eight patients were free of disease. In four patients with local relapse, two had additional resection, one had hindquarter amputation, and one was observed. In three patients with deep infection, the prosthesis was removed; however, one patient had hindquarter amputation. One patient had dislocation of the hip and prosthesis loosening. Overall survival of patients was 70%, and the survival of prostheses was 42%. In eight patients, the functional evaluation showed that the average functional score with the prosthesis was 11.6 (39%) and without the prosthesis the functional score was 7.0 (23%). Implantation of a megaprosthesis seems to indicate a high complication rate and a poor functional result after hemipelvic resection.

In soft tissue reconstruction of megaprostheses, the reattachment of soft tissue and joint capsules is essential. Sixty-nine megaprostheses were implanted and a trevira tube was applied to support reconstruction of the capsule and soft tissue. In cases of proximal femur replacement (33 patients), total femur replacement (five patients), and proximal humerus replacement (16 patients), the trevira tube allowed for reconstruction of the capsule and refixation of the muscles and helped to minimize dislocation. In cases of proximal tibia replacement (seven patients), arthrodesis of the knee (three patients), total knee replacement (two patients), and distal femur replacement (three patients), the trevira tube allowed for attachment of muscle flaps and extensor apparatus. Dislocation was observed in two of 54 patients who had proximal femur replacements. No dislocation was observed in patients with a total femur endoprosthesis or a proximal humerus endoprosthesis. The trevira tube also was used to attach the gastrocnemius muscle in patients with a proximal tibia endoprosthesis and to reattach the rotator cuff in patients with a proximal humerus prosthesis. There was no significant increase in the rate of infection. The histopathologic findings in six patients showed tissue ingrowth into the tube.

Ewing tumors are characterized by reciprocal translocations involving the EWS gene on 22q12 fused to ETS transcription-factor family members. Little is known about further aberrations contributing to tumor development and progression. Sixty-two frozen tumors with known EWS rearrangements (52 primary tumors, 10 relapses) of ET patients registered in the EICESS protocol were analyzed by comparative genomic hybridization (CGH). The median number of changes in 52 primary and 10 relapsed cases was 2.5 and 5.0 per tumor (P = 0.153). Frequent abnormalities included gains of chromosomes 8, 12, 20, and 1q and losses of 16q and 19q. Neither number nor type of aberration was associated with histology, tumor size, disease stage, tumor localization, or histologic tumor response to chemotherapy. Among the 52 primary tumors, 26 with Type I fusion (EWS exon 7 to FLI1 exon 6) and 26 with other fusion types had a median of 2.0 and 3.0 aberrations per tumor, respectively (P = 0.031). Combinations of gains of chromosomes 8 and 12, gains of chromosome 20, and either gains of 8q or 18q and losses of 16q and 17p frequently occurred. The cumulative overall survival (OAS) was different between 35 patients with

Purpose The purpose of this study was to describe the MR characteristics of periosteal chondroma. Method MR images of 12 proven cases of periosteal chondroma were analyzed with reference to tumor morphology and size. MR features were correlated with radiographic and pathologic findings. Results Tumor size ranged from 1 to 7 cm in maximum diameter with a mean value of 2.6 cm. On MR images, a soft tissue mass at the bone surface with pressure erosion of adjacent cortical bone could be identified in all cases. All lesions were bordered by a hypointense rim (100%) and frequently showed a lobulated configuration (75%). Edema of medullary bone or soft tissues was not observed in any of the cases. Signal intensity of cartilaginous tumor tissue was typically hypo-or isointense relative to muscle on T1-weighted (100%) and hyperintense relative to fat on T2-weighted (92%) and T2*-weighted (100%) MR images. Radiographically significant calcifications of the tumor matrix, present in half of the cases, caused focal signal loss on MR images of all pulse sequences. Contrast enhancement was observed predominantly at the periphery of the lesions (100%), which on pathologic examinations typically contained fibrovascular bundles, surrounding the cartilage lobules. Conclusion Periosteal chondroma appears to have a relatively typical MR appearance, which reflects the histologic composition of the lesion. In addition to radiography, MRI therefore can substantially aid in the preoperative diagnosis of this rare bone lesion.

We report for the first time a patient who underwent a revision hip arthroplasty after a hip rotationplasty (Winkelmann type B III b) because of a chondrosarcoma in 1988. The clinical symptoms and radiographic findings (X-ray, bone scintigraphy, arthrography) of the aseptic loosening of the acetabular component are presented in detail. Furthermore, we present the functional results after hip revision.

BACKGROUND Rotationplasty is considered to be a treatment option for patients who have had a primary malignant bone tumor of the distal part of the femur or the proximal part of the tibia. The present study was performed to evaluate the muscle activity, the kinetics (range of motion of the hip and knee joints), and the kinematics (joint moments) after rotationplasty and to determine whether there was an association between these parameters and the functional outcome. METHODS Forty-three patients who had been managed with rotationplasty for the treatment of a femoral or tibial bone tumor were evaluated clinically and functionally. The mean age (and standard deviation) at the time of follow-up was 24.4 +/- 10.7 years (range, eight to sixty-eight years), the mean age at the time of the procedure was 17.8 +/- 10.2 years (range, seven to sixty-three years), and the mean duration of follow-up was 6.7 +/- 4.9 years (range, 0.7 to eighteen years). Instrumented gait and electromyographic analyses were performed. The qualitative data were compared with the functional outcome, which was determined with the functional evaluation score of the Musculoskeletal Tumor Society. RESULTS Gait analysis revealed a fairly normal walking pattern with a slight limp and a lateral lean of the trunk over the ipsilateral limb that led to a reduced joint moment in the hip (moment on involved side, 68 percent [compared with a control group]; moment on uninvolved side, 81 percent). The ranges of motion of the hips (uninvolved side, 42.0 +/- 8.2 degrees; involved side, 42.4 +/- 8.0 degrees) and the knees (uninvolved side, 59.7 +/- 5.0 degrees; involved side [former ankle joint], 58.1 +/- 11.6 degrees) were symmetrical even though the knee-motion pattern of the involved limb indicated a slightly reduced extensor mechanism in 51 percent (twenty-two) and a markedly reduced extensor mechanism in 35 percent (fifteen) of the forty-three patients. Electromyography revealed function of the muscles of the involved limb, with comparable amplitudes in the involved and uninvolved limbs. The leg muscles of the involved limb were active in the stance phase (the soleus and the lateral and medial heads of the gastrocnemius) and the swing phase (the peroneus longus and the tibialis anterior) according to their function in relation to the new knee joint. The patients had a good functional result, with a mean score of 23.9 +/- 2.7 of 30 points. With the numbers available for study, we could not show the duration of follow-up to be related to the overall outcome, but the age at the time of the operation was related to the total functional score as well as to gait and walking ability (p < 0.05). CONCLUSIONS The results of the electromyographic and gait analyses demonstrated good functional restoration of gait following rotationplasty.

Thirteen patients with aneurysmal bone cyst of the spine (excluding sacral lesions) were retrospectively reviewed. Treatment for aneurysmal bone cysts remains controversial, but surgical resection, irradiation, and embolization are common treatment modalities for those involving the spine. Of 102 patients with aneurysmal bone cysts, 15 had a lesion of the spine, including 2 sacral cases. Of the 13 patients with a lesion of the thoracic or lumbar spine, 9 underwent resection of the lesion, 2 curettage and cementation, and 2 only currettage. Eleven patients underwent segmental arthrodesis with instrumentation after treatment of the primary or recurrent lesion, while 2 patients underwent segmental arthrodesis using autogeneic bone. Nine patients did not develop a local recurrence after resection of the lesion. However, the 2 patients who underwent curettage alone developed local recurrences. None of 4 patients developed recurrences after curettage and cementation. After recurrence, 1 patient underwent additional resection with irradiation, and 1 patient underwent resection alone. At the final follow-up, all lesions were under control. In one patient, lumbar kyphosis developed after segmental arthrodesis with instrumentation, and arthrodesis was performed again. Radical resection of aneurysmal bone cysts of the spine with instrumentation is the optimal method of acquiring a high degree of local control and preventing spinal deformity.

Background: The present study was performed to determine whether there is a difference, with regard to functional outcome and quality of life, between endoprosthetic replacement and rotationplasty for the treatment of malignant tumors of the distal part of the femur or the proximal part of the tibia. Methods: Sixty-seven patients, between the ages of eleven and twenty-four years at the time of the diagnosis, had a malignant tumor of the distal part of the femur or the proximal part of the tibia. A rotationplasty was performed in thirty-three patients, and an endoprosthetic replacement was done in thirty-four patients. The median duration of follow-up was six years and one month (range, two years to sixteen years and two months). The scale developed by the Musculoskeletal Tumor Society was used to evaluate the functional results. Quality-of-life issues were assessed with the questionnaire developed by the European Organization for Research and Treatment of Cancer. Results: The patients who had had a rotationplasty had a mean functional score, according to the system of the Musculoskeletal Tumor Society, of 24 points, and the patients who had had an endoprosthetic replacement had a mean score of 25 points. This difference was not found to be significant, with the numbers available (p = 0.47). Only one patient who had had a rotationplasty used an assistive device when walking long distances, whereas six patients who had had an endoprosthetic replacement used an assistive device. This difference was significant (p < 0.001). The quality-of-life questionnaire revealed that the patients who had had a rotationplasty could participate in hobbies such as carpentry and sports as well as in other daily activities to a significantly greater degree than those who had had an endoprosthetic replacement (p = 0.001). Restriction in daily activities due to pain was significantly less common in the group that had had a rotationplasty than it was in the group that had had an endoprosthetic replacement (p = 0.047). Conclusions: Rotationplasty was not associated with any disadvantages with regard to function or quality of life in comparison with endoprosthetic replacement. It is possible that the psychosocial outcome is influenced by the fact that patients who have a rotationplasty know that additional operative intervention is not usually necessary. Despite good functional and quality-of-life results, the cosmetic appearance may be the most serious disadvantage of rotationplasty. The decision to perform this procedure must be made on a case-by-case basis.

Purpose: Treatment results and the pattern of relapse were evaluated in the multimodal treatment of Ewing’s sarcomas of the chest wall. Methods and Materials: In a retrospective analysis, 114 patients with non-metastatic Ewing’s sarcoma of the chest wall were evaluated. They were treated in the CESS 81, CESS 86, or EICESS 92 studies between January 1981 and December 1993. The treatment consisted of polychemotherapy (VACA, VAIA, or EVAIA) and local therapy, either surgery alone (14 patients), radiotherapy alone (28 patients) or a combination of both (71 patients). The median follow-up was 46.6 months (range 5–170). A relapse analysis for all patients with local or combined relapses was performed. Results: Overall survival was 60% after 5 years, event-free survival was 50%. Thirty-seven patients had a systemic relapse (32.4%), 11 patients had a local relapse alone (9.6%), and 3 patients had a combined local and systemic relapse (2.6%). The risk to relapse locally after 5 years was 0% after surgery alone, 19% after radiation alone, and 19% after postoperative irradiation. None of the 8 patients with preoperative irradiation have failed locally so far. With the introduction of central radiotherapy planning in CESS 86, local control of irradiated patients improved. Ten of 14 patients with local failure could be evaluated in the relapse analysis: 3 patients had an in-field relapse, 4 patients had a marginal relapse, 2 patients had a relapse outside the radiation fields, and 1 patient failed with pleural dissemination. Six treatment deviations were observed. Conclusion: Local control was best after surgery alone in a positively selected group of patients. Local control after radiation or combined radiation and surgery was good. With diligent performance of radiotherapy, it will be possible to further improve the results in the radiotherapy group.

Five children with pelvic Ewing's sarcoma whose age was 6-10 years underwent reconstruction after resection of the tumor. One child with a tumor of the ilium underwent an iliosacral arthrodesis with allograft. Three patients underwent a hip transposition after the resection of the ilium and the upper part of the acetabulum. One patient underwent no skeletal reconstruction after the resection of the acetabulum and the ilium. Two children are alive 96 and 60 months after surgery; however, two died 12 and 21 months after surgery of metastasis, and one is alive with metastasis 15 months after surgery. Functional evaluation was possible in four patients: one is excellent, and three are fair. After resection of the ilium, including the upper part of the acetabulum, a hip transposition can be selected. If all the acetabulum is resected, it will be optimal not to do a bony reconstruction.

Joint cartilage functions as a barrier against the extension of bone tumors. However, transarticular invasion by iliopelvic sarcomas across the sacroiliac (SI) joints into the sacrum sometimes occurs. We made a radiological analysis (CT and/or MRI) of 47 bone sarcomas which originated in the ilium and extended nearly to the SI joint. 8 of 17 chondrosarcomas and 3 of 30 other sarcomas (2 of 23 Ewing's sarcomas and 1 of 7 osteosarcomas) invaded the sacrum through the SI joint.

We compared the outcomes of 26 intramedullary cemented massive allografts with 19 allografts without cementation; all allografts were used for reconstruction after excision of bone sarcomas. In the cementation group, 12 allografts were used as osteochondral grafts (proximal humerus 4, proximal tibia 4, and distal femur 4), 7 as intercalary diaphyseal allografts of the femur, and 7 for a knee arthrodesis. In the uncemented allografts, 3 allografts were used as osteochondral grafts (proximal humerus 2, proximal tibia 1), 2 as intercalary diaphyseal allograft of the femur, and 14 for a knee arthrodesis. The average length of follow-up was 40 (25-60) months. 14 of 26 cemented allografts had an excellent (osteotomy line: not visible) or good (fusion > or = 75% of the cortical thickness) healing of the junction site. Infection developed in 1 allograft. Fracture occurred in 4 of 12 cemented osteochondral allografts due to a subchondral collapse (all in the proximal tibia). Fractures at the junction site in the lower extremity developed in 4 of 22 cemented allografts. In 19 allografts without cementation, 11 had excellent or good healing of the junction. Late infection developed in 4 allografts, fracture of the allograft in 3 cases, and junction fracture in 3 of 17 patients with reconstruction of the lower extremity. Intramedullary graft cementation seems to reduce the fracture and infection rates.

The effects of polymethylmethacrylate (bone cement) on the local control of primary aneurysmal bone cysts excluding spinal lesions were analyzed. After intensive curettage of the lesions, 30 patients underwent a bone graft, and 35 patients had the defect packed with bone cement. After cementation (median time lapse, 4.5 months), 30 of 35 patients underwent a cancellous bone graft after removal of the packed bone cement. The followup period ranged between 24 and 161 months (median, 59.5 months). The local recurrence rate after curettage and cementation was 17% and after curettage and bone graft was 37%. The difference of the cumulative 10-year relapse free survival between patients of both groups was significant. The median time between surgery and local recurrence was 17 months (range, 2-90 months).