Distal femoral replacement with the MML system: a single center experience with an average follow-up of 86 months.

BACKGROUND: The aim of this study was to compare the functional outcomes and complication rates after distal femoral replacement (DFR) performed with the modular Munich-Luebeck (MML) modular prosthesis (ESKA/Orthodynamics, Luebeck, Germany) in patients being treated for malignant disease or failed total knee arthroplasty. METHODS: A retrospective review of patient charts and a functional investigation (involving Musculoskeletal Tumor Society Score [MSTS], American Knee Society Score [AKSS], Oxford Knee Score [OKS], Western Ontario and McMaster Universities Osteoarthritis Index [WOMAC], Toronto Extremity Salvage Score [TESS], the 12-Item Short-Form [SF-12] Health Survey, and a failure classification system developed by Henderson et al.) of DFR cases from 2002 to 2015 were conducted. The indications for DFR were malignant tumor resection in the femur (n = 20, group A) or failure of revision total knee arthroplasty without a history of malignant disease (n = 16, group B). RESULTS: One-hundred and twenty-nine patients were treated during the study period. Of these, 82 were analyzed for complications and implant-survival. Further, 36 patients were available for functional assessment after a mean follow-up of 86 months (range: 24-154). There were 75 complications in total. The overall failure rate for DFR was 64.6% (53/82 patients). The most common failure mechanisms were type III (mechanical failure), followed by type I (soft tissue) and type II (aseptic loosening). The mean MSTS score (out of 30) was 17 for group A and 12 for group B. All the clinical outcome scores revealed an age-dependent deterioration of function. CONCLUSION: DFR is an established procedure to restore distal femoral integrity. However, complication rates are high. Post-procedure functionality depends mainly on the patient's age at initial reconstruction.

Management of extremity arterial pseudoaneurysms associated with osteochondromas.

Arterial pseudoaneurysms associated with osteochondromas are rare and most publications on this topic are case reports. The management of this double entity is not standardized. We wanted to update it. Literature searches on MEDLINE and EMBASE were performed using the keywords "artery pseudoaneurysm" and "osteochondroma". Patient demographics, clinical presentations, diagnostic and therapeutic modalities were reviewed. In sum, 101 cases were analyzed. Overall, young adults represented the majority of the affected population with a masculine preponderancy (86%). Painful swelling (51%) was the most commonly physical finding. Distal femur was the most common site of the osteochondroma (86%). Multiple hereditary exostosis was seldom reported (36%). Diagnostic confirmation was dominated by arteriography (55%). Popliteal artery (77%) was the most commonly injured vessel. The treatment was open surgery with vascular repair and optimal exostectomy. Arterial repair was performed with saphenous vein grafting (40%) or lateral suture (39%). Postoperative courses were often uneventful (97%). Arterial pseudoaneurysms resulted from osteochondromas were dominated by those involving the popliteal artery. The use of arteriography to confirm the diagnosis may be limited to the benefit of non-invasive radiological methods because endovascular treatment is not relevant in the setting of osteochondroma-induced arterial pseudoaneurysm.

Distribution and evaluation of bone and soft tissue tumors in the middle Black Sea Region.

OBJECTIVE: The aim of this study was to evaluate the characteristics of bone and soft tissue tumors operated on at the Department of Orthopedics and Traumatology at Ondokuz Mayis University Faculty of Medicine Hospital between January 1987 and January 2012. METHODS: This descriptive study retrospectively evaluated 1,925 patients hospitalized with a preliminary diagnosis of tumor. Patients were analyzed for age, gender, tumor incidence and localization. Three hundred and forty-nine patients found to have non-tumor causes. The 94 patients discharged at their own request or deceased during follow-up were not included in the tumor group. RESULTS: Of the 1,482 (76.9%) patients diagnosed with tumor, 687 (46.4%) were bone tumors, 586 (39.5%) soft tissue tumors and 209 (14.1%) metastatic tumors. The most common benign bone tumor was osteochondroma (118; 25%), followed by enchondroma (68; 14.4%) and giant cell tumor (59; 12.5%), and the most common malignant bone tumor was osteosarcoma (58; 27%), followed by chondrosarcoma (36; 16.7%) and Ewing's sarcoma (33; 15.3%). The most common benign soft tissue tumor was cystic hygroma (96; 22%), followed by lipoma (75; 17.2%) and hemangioma (52; 11.9%), and the most common malignant soft tissue tumors were pleomorphic cell tumor (29; 19.3%) and liposarcoma (29; 19.3%), followed by pleomorphic undifferentiated sarcoma (21; 14%). Seventy (33.5%) of the metastatic tumors were of pulmonary origin, 36 (17.2%) were of breast origin and the primary site of the tumor was not clearly determined in 58 (27.8%) patients. CONCLUSION: The distribution of bone and soft tissue tumors appear to have certain characteristics but can show regional differences. We believe that the establishment of a larger series through the collection of these types of studies from centers in which bone and soft tissue tumor surgery is performed will provide important information on the epidemiological features of bone and soft tissue tumors.

Tumours and tumour-like lesions of the hip in the paediatric age: a review of the Rizzoli experience.

Bone tumours and tumour-like lesions of the hip in children are rare. Signs and symptoms of these tumours are generally nonspecific. Delay of diagnosis is not uncommon. A high index of suspicion in young patients presenting with persistent pain and without history of trauma, that is unresolved with conservative therapy should prompt further investigation, including radiographs or computed tomography scan of the pelvis. In the experience of the Istituto Rizzoli, in patients less than 14 years (mean 9 years, ranged from 6 months to 14 years), 752 tumours and tumours-like lesions occurred in the pelvis or proximal femur, involving the hip. Tumour-like lesions accounted for 322 cases (simple bone cyst in 255, eosinophilic granuloma in 43, aneurismal bone cyst in 34), benign tumours for 340 cases (osteoid osteoma in 229, fibrous dysplasia in 63, exostosis in 48) and malignant tumours for 80 cases (Ewing's sarcoma in 53 and osteosarcoma in 27). The epidemiology, pathology, clinical presentation, and radiograph findings are discussed for each of these tumours.Treatment of these tumours differs from observation or minimally invasive treatment for most pseudotumoural lesions, intralesional excision or termoablation for benign bone tumours and wide resection for malignant bone tumours. In this latter group, chemotherapy is required and often administered pre- and postoperatively.

[Conservative surgery as an alternative treatment of a knee osteosarcoma in the presence of a pathological fracture]. / Cirugía de salvamento como alternativa en el tratamiento de osteosarcoma de rodilla ante la presencia de fractura en terreno patológico.

INTRODUCTION: classical osteosarcoma is defined as a very malignant spindle cell sarcoma, characterized by the production of osteoid matrix, it is the most common primary malignant bone tumor. It is most common among males in their twenties. Factors involved include those related with the skeletal growth and development, pathological fractures, and very young patients who are still growing. Conservative surgery is inappropriate; however, preoperative chemotherapy and a good choice of conservative treatment are an alternative. MATERIAL AND METHODS: a 15-year-old male patient presented at the orthopedics service with a diagnosis of a pathological fracture in the distal third of the right femur and a tumor in the lateral aspect of the knee, with pain and limitation of gait. Based on X-rays, a bone scan and a biopsy, the diagnosis of a fibroblastic osteosarcoma, an Enneking IIB lesion, was made. Treatment consisted of conservative surgery with broad tumor resection involving 18 cm of the femur, resection of the vastus medialis and vastus lateralis, release of the femoral bundle, the popliteal and sciatic nerves, as well as placement of a modular stryker tumor prosthesis, with knee replacement. RESULTS: the clinical and radiological course was appropriate. After a 3-week rehabilitation period the patient was able to walk and the lower limb was salvaged. At 5 months there is no evidence of tumor relapse, the patient can walk properly and has recovered the strength. DISCUSSION: this is a case salvage surgery to treat a pathological fracture resulting from a malignant bone tumor. We think that staging is essential to select the treatment. In the case presented herein a comprehensive management is fundamental to the success of conservative treatment.

[The results of curettage and bone cementing for enchondromas of the distal femur]. / Femur alt uç enkondromlarinin küretaj ve kemik çimentosu ile tedavi sonuçlari.

OBJECTIVES: We evaluated patients who were treated with curettage and cementing for enchondromas localized in the distal femur. METHODS: The study included 13 patients (7 females, 6 males; mean age 54 years; range 43 to 65 years) who underwent surgery for enchondromatosis in the distal femur. Enchondromas were detected incidentally in 10 patients; of these, complaints of pain appeared afterwards in seven patients. Surgery involved curettage and cement filling of the defects. Preoperative biopsies were obtained in seven patients. Clinical evaluations were made with a visual analog scale. The mean follow-up period was four years (range 1 to 5 years). RESULTS: Radiographically, the size of the lesions ranged from 3 cm to 6 cm (mean 4.5 cm). Computed tomography showed cortical extension in five patients and erosion leading to narrowing in the posterior cortex in one patient. The mean pain score decreased from 3.63 (range 0 to 7) to 0.54 (range 0 to 3) postoperatively (p<0.05). Histopathological diagnoses of surgical specimens were enchondroma in 12 patients and grade 1 chondrosarcoma in one patient. Postoperatively, only one patient who had erosion and narrowing in the posterior cortex required cast immobilization for three weeks. None of the patients had recurrence, sarcomatous changes, or infection. No functional loss developed after surgical treatment. CONCLUSION: Treatment of femoral enchondromas with curettage and cementing yields successful functional and radiologic results.

Comparisons of the skeletal locations of putative plutonium-induced osteosarcomas in humans with those in beagle dogs and with naturally occurring tumors in both species.

Osteosarcomas occur from exposures to bone-seeking, alpha-particle-emitting isotopes, particularly plutonium. The skeletal distribution of putative 239Pu-induced osteosarcomas reported in Mayak Metallurgical and Radiochemical Plutonium Plant workers is compared with those observed in canine studies, and these are compared with distributions of naturally occurring osteosarcomas in both species. In the Mayak workers, 29% and 71% of the osteosarcomas were in the peripheral and central skeleton, respectively, with the spine having the most tumors (36%). An almost identical distribution of plutonium-induced osteosarcomas was reported for dogs injected with 239Pu as young adults. This distribution of osteosarcomas is quite different from the distributions of naturally occurring osteosarcomas for both species. In the Cooperative Osteosarcoma Study Group in humans (1,736 osteosarcomas from all ages), over 91% of the tumors occurred in the peripheral skeleton. In the Mayo Clinic group of older individuals (>40 years old), over 60% of the osteosarcomas appeared in the peripheral skeleton. The distribution of naturally occurring osteosarcomas in the canine is similar to that in the adult human. The similarities of the distributions of plutonium-associated osteosarcomas in the Mayak workers with those found in experimental studies suggest that many of the reported osteosarcomas may have been associated with plutonium exposures. These results also support the experimental paradigm that plutonium osteosarcomas have a preference for well vascularized cancellous bone sites. These sites have a greater initial deposition of plutonium, but also greater turnover due to elevated bone remodeling rates.

Leiomyosarcoma of the distal femur in a patient with a history of bilateral retinoblastoma: a case report and review of the literature.

We report a case of primary leiomyosarcoma of the distal femoral shaft arising in a patient who had undergone bilateral orbital enucleation for bilateral retinoblastoma several years previously. Radiography demonstrated an osteolytic, expansive lesion with cortical destruction anteriorly in the distal femoral shaft, and these findings were confirmed on CT. MR imaging revealed an expansive intramedullary lesion with cortical breakthrough and soft tissue extension. The occurrence of a second malignancy in patients with a history of bilateral retinoblastoma is well documented. Many different histological types have been described, with osteosarcoma and leiomyosarcoma occurring with the greatest frequency.

[Pathomorphology of parosteal osteosarcoma. Experience with 125 cases in the Hamburg Register of Bone Tumors]. / Pathomorphologie des parossalen Osteosarkoms. Erfahrungen an 125 Fällen des Hamburger Knochentumor-Registers.

Parosteal osteosarcoma is a rare low-grade bone tumor. It occurs between the 2nd and 8th decade of life. In our own series of 125 parosteal osteosarcomas, most patients were aged between 30 and 40. The most frequent location was the distal dorsal femur. Until clearly proven otherwise, a bone-forming tumor in this location has to be regarded as a parosteal osteosarcoma. Of all parosteal osteosarcomas 77% are located in the lower extremity, with a female sex predominance in this location (62%); 18% are located in the upper extremity; 15% of all parosteal osteosarcomas are located in the humerus. Just 6% of all parosteal osteosarcomas are observed in the skull, the spine, and the pelvis. The tumor is characterized by hyalinized fibrous stroma with a low cell content without substantial nucleus polymorphism and variably dense bony trabeculae. The diagnosis can be difficult due to highly differentiated areas with fat tissue within the marrow and very uniformly bony structure. The operation material has to be analyzed very carefully, because the extent of dedifferentiated areas and most probably also the extent of invasion of the medullary cavity determine the prognosis and occurrence of recurrencies. The tumor is most commonly misinterpreted as osteochondroma or heterotopic ossification. Paying attention to all radiologic and histologic criteria,osteoblastic eccentrically located high-grade osteosarcomas can be clearly distinguished from parosteal osteosarcoma. An intramedullary located low-grade osteosarcoma,which is differentiated like a parosteal osteosarcoma,is histologically indistinguishable from the parosteal variant. This variant requires the synthesis of all available data to find the correct diagnosis. The parosteal osteosarcoma shows like no other tumor the necessity of close cooperation of all involved disciplines for diagnosis and therapy.

Subperiosteal schwannomas of the femur.

We report a case of two adjacent femoral subperiosteal schwannomas in a 38-year-old man. To our knowledge, this is the first report of a schwannoma arising as a surface lesion of bone. Radiographs showed focal cortical scalloping and MR imaging two small hyperintense nodules on T2-weighted and STIR images which enhanced with a gadolinium chelate.

Paget's disease of bone complicated by giant cell tumor.

Paget's disease is uncommon in patients younger than 50 years of age. Multifocal giant cell tumors arising in bone affected by Paget's disease have been described previously in 37 cases. A case of a 38-year-old man with polyostotic Paget's disease and multifocal giant cell tumors responsive to steroid therapy is presented.

Malignant giant cell tumour of the distal femur treated by excision, allografting and ligamentous reconstruction: an 18-year follow-up.

Extensive osteoarticular allografts have been used for knee reconstruction, but because of their composite nature and the technical difficulty of the procedure, complication and failure rates have been high. There are few records of long-term results in the literature. In this report, a 19-year-old man with a large aggressive giant cell tumour of the left distal femur was treated in 1976 by en bloc resection, massive femoral allografting and ligamentous reconstruction. Follow-up after 18 years showed no recurrence of the tumour, excellent incorporation of the graft and good knee function, which allowed the patient to work 9 hours a day on his feet without pain.

Clear cell chondrosarcoma--a report of 2 cases.

Clear cell chondrosarcoma is a form of chondrosarcoma that was first described by Unni in 1976. It is essentially a low-grade chondrosarcoma with many similar clinical, radiological and histological features as the more common chondrosarcomas. However, the distinguishing feature of this variant is in the presence of the characteristic clear cells histologically. We relate two such cases of clear cell chondrosarcoma in Singapore. A short discussion covering the incidence/prevalence of this tumour, its common presenting clinical, radiological and histological features follows. We also review the available world literature, highlight the salient and differentiating aspects of this particular tumour, and touch on its management and eventual prognosis.

Osteosarcoma in young children.

The clinicopathologic features of osteosarcoma in 12 children younger than 16 years of age treated at The Children's Hospital and Dana-Farber Cancer Institute, Boston, during a 70-year time period are presented. Only one of six children treated before 1972 is a long-term survivor. Four of six children (67%) treated after 1972 are disease-free with an average follow-up of 8.8 years. The year 1972 marked the onset of use of effective chemotherapy in osteosarcoma, namely, high-dose methotrexate and leucovorin rescue. It would appear that the pathologic features and behavior of osteosarcoma in young children is similar to that of osteosarcoma in older children and adolescents. A combination of complete (wide) surgical resection or amputation and aggressive chemotherapy offers the best chance of long-term survival.

Magnetic resonance imaging of an osteoid osteoma of the proximal femur: a potentially confusing appearance.

Osteoid osteomas are common benign pediatric bone tumors. However, radiographic diagnosis and location of the tumor nidus can be difficult. We recently diagnosed and treated an osteoid osteoma of the right proximal femur in a 9-year-old girl in whom the preoperative magnetic resonance images demonstrated a well-marginated lesion in the medullary canal but no tumor nidus. This was confusing and misleading. Correct diagnosis was made with a computed tomography (CT) scan. Care must be taken in interpretation of magnetic resonance imaging (MRI) in disorders in which secondary bone marrow changes occur to avoid erroneous diagnoses and possible incorrect operative procedures.

[Analysis of 1355 cases of tumors and tumor-like lesions in the bone].

A total of 1355 cases of tumors and tumor-like lesions in the bone seen in the three hospitals affiliated to our college in the past 32 years was analysed. Of them, 1170 (86.4%) were primary bone tumors, 31 (2.3%) metastatic and 154 (11.3%) tumor-like lesions of bone. Histologically, the primary bone tumors were mostly chondrogenic and osteogenic. The ratio of benign to malignant tumors was 2.8:1. Peak ages were 11-40 years. Patients with malignant tumors were ten years younger than those with benign tumors. The first three common benign bone tumors were osteochondroma, osteoma and chondroma. The vulnerable sites were tibia, femur and skull. The first three common malignant bone tumors were osteosarcoma, chondrosarcoma and fibrosarcoma with common sites in femur, tibia and humerus. The majority of tumor-like lesions were fibrous-dysplasia, frequently in the femur. These observations are very similar to those reported at home but quite different from those reported from other countries. In our series, the ratio of benign to malignant bone tumors was the highest (2.8:1); giant cell tumor was not among the first three common benign bone tumors; and the first vulnerable site of benign bone tumors was not femur but tibia.

Paget's sarcoma in Tasmania.

Six proven cases of Paget's sarcoma occurred in Tasmania in the decade 1965 to 1974. The incidence was 0-16 cases per 100,000 population per year. The sex ratio was two male: one female. The average age was 61-2 years. All cases occurred in the pelvis or adjacent femur. Average time of survival after onset of symptoms was one year. Two patients had lymph node metastases. One patient had no pulmonary metastases; a further patient may have had no pulmonary metastases. All patients were born in Australia (State of Tasmania) of predominantly Anglo-Saxon stock.