Fibromyxoma of bone: a case report and review of the literature

In this study, a case of fibromyxoma of the proximal femur in a 59-year old woman is reported. The classification of this rare bone tumour is still a matter of debate and some investigators have suggested that these lesions represent a degenerative form of fibrous dysplasia. Some authors make a further distinction between fibromyxoma and myxoma of bone. In a review of 23 cases of fibromyxoma and five cases of myxoma, no differences in clinical, radiographic and biologic behaviour between fibromyxoma and myxoma were found. Apart from the age at diagnosis, the most important difference between fibromyxoma and myxoma was the degree of myxoid matrix. Therefore, we suggest that extragnathic myxoma is a regressive variant of extragnathic fibromyxoma and should be termed as the same entity. In contrast to monostotic fibrous dysplasia fibromyxoma / myxoma often causes pain and presents as a Lodwick IC lesion with a soft tissue mass. Therefore, fibromyxoma / myxoma should be distinguished from fibrous dysplasia because of its different clinical and radiographic features.     

Silver-coated megaendoprostheses in a rabbit model--an analysis of the infection rate and toxicological side effects

Deep infection of megaprostheses remains a serious complication in orthopedic tumor surgery. Despite the use of systemic and local antibiotic prophylaxis the reported infection rate is between 5% and 35%. Silver-coated medical devices proved their effectiveness in reducing infections. The objective of this study was to examine in vivo the antimicrobial efficacy and possible side-effects of a silver-coated megaprosthesis. In a first study, 30 rabbits (15 titanium versus 15 silver-coated Mutars-endoprostheses) were infected with Staphylococcus aureus. In a second study, toxicological side effects were analyzed in 10 rabbits with a silver-coated megaprosthesis. The silver group showed significantly (p<0.05) lower infection rates (7% versus 47%) in comparison with the titanium group. Measurements of the C-reactive-protein, neutrophilic leukocytes, rectal temperature and body weight showed significant (p<0.05) lower signs of inflammation in the silver group. The analysis of the silver concentration in blood (median 1.883ppb) and in organs (0.798-86.002ppb) showed elevated silver concentrations without pathologic changes in laboratory parameters and without histological changes of organs. In conclusion, the new silver-coated Mutars-megaprosthesis resulted in reduced infection rates without toxicological side effects, suggesting that this prosthesis might be a promising device in tumor surgery exhibiting antimicrobial activity.     

Primary multifocal osseous Hodgkin disease: a case report and review of the literature

Purpose Hodgkin disease (HD) typically involves the lymphatic system at one or more sites. Rarely, Hodgkin disease presents as an osseous lesion without involvement of lymph nodes. Therefore, the histologic diagnosis of osseous HD can be problematic. We present a rare case of multifocal osseous HD and a review the literature with special emphasis on treatment and prognosis.Methods Osteomyelitis and lymphoma are the main differential diagnoses and can only be excluded histologically by the presence of Sternberg Reed cells or by immunohistochemical examinations. This case reports a 21-year old man with a Hodgkin lymphoma located at the proximal femur and the proximal tibia.Results Staging studies revealed no other tumor manifestations. Regarding the Ann Arbor classification, the presented case should be a stage IV disease. The patient is without evidence of disease 4 years after curettage, local radiation therapy, and systemic chemotherapy despite the poor prognosis considering the Ann Arbor classification.Conclusion Reviewing the few reported cases, osseous HD must be distinguished from systemic HD with diffuse bone marrow involvement and from osseous metastases in advanced stage of disease because it seems to have a better prognosis.     

Risk of recurrence and survival after relapse in patients with Ewing sarcoma

Background

The prognosis in patients with relapsed Ewing sarcoma is unfavorable. Our investigation identifies factors predicting for the outcome following relapse.

Procedure

We analyzed type of relapse, time to relapse and overall survival after relapse (OSr) in 714 patients with first recurrence. All patients had been treated within the Cooperative Ewing Sarcoma Studies (CESS) 81 or 86, or the European Intergroup CESS (EICESS 92). OSr time was calculated from diagnosis of first relapse to last follow‐up or death.

Results

Median follow‐up time from diagnosis of primary disease was 2.2 years (mean = 4.0; range: 0.2–24.9). Relapse sites were local in 15%, combined local and systemic in 12%, and systemic in 73%. Among patients with a localized primary tumor, 20% relapsed locally, while 12% showed combined and 68% systemic relapse. When the primary disease was disseminated, 82% developed systemic, 13% combined, and 5% local relapse. Five‐year OSr was 0.13 (SE = 0.01). Outcome following local relapse, with a 5‐year survival rate of 0.24 (P < 0.001), was superior to outcome after systemic or combined recurrence. Five‐year OSr was 0.07 (SE = 0.01) in patients who relapsed 0–2 years after the diagnosis of primary disease, as compared to a 5‐year OSr of 0.29 (SE = 0.03) when relapse occurred later.

Conclusions

5‐year OSr in Ewing sarcoma is poor (<0.2). Prognostically favorable factors are: late onset (>2 years) and strictly localized relapse. Pediatr Blood Cancer 2011; 57: 549–553. © 2011 Wiley‐Liss, Inc.     

Cytotoxic effect of clodronate and zoledronate on the chondrosarcoma cell lines HTB-94 and CAL-78

The wide surgical tumour resection is the only effective treatment in chondrosarcoma. However, a major problem remains the high rate of local recurrences and metastases due to the lack of adjuvant therapies. In this study the cytotoxic effect of the bisphosphonate clodronate (0.1–1000 μM) and zoledronate (0.1–1000 μM) in different concentrations on two chondrosarcoma cell lines (HTB-94 and CAL-78) has been investigated. After an incubation period of 48, 72 and 96 hours the chondrosarcoma cell viability was measured as the MTT-proliferation rate. In concentrations of >1 μm zoledronate the cell activity was reduced by up to 95% for the CAL-78 cells. Further, zoledronate has been more effective in lower concentrations than clodronate in the reduction of cell viability for both cell lines. However, clodronate showed significant cytotoxic effects in high concentrations and after longer incubation periods. Further research is necessary, but in the light of these results bisphosphonates may also play a role in the treatment of chondrosarcomas.     

The Modular Universal Tumour And Revision System (MUTARS®) in endoprosthetic revision surgery

The aim of this study was to present the clinical and functional results of revision surgery after failed hip endoprostheses using the Modular Universal Tumour And Revision System (MUTARS®). Functional results of the hip endoprostheses were recorded by applying the Harris hip score. The extent of the presurgical radiological bone defect was measured according to the classification system of the German orthopaedic association (DGOOC). Indications for revision surgery on 45 patients (21 female, 24 male) were aseptic loosening (19 patients), infection (16 patients), or periprosthetic fracture (Vancouver classification B2, B3 and C, in nine patients). Revision surgery was performed after 8.6 years on average (min. 0.6; max. 14.25 years). Large defects of the proximal femur (80% medial or lateral diaphysis; 20% meta-diaphysis according to DGOOC classification) were adequately reconstructed. The average follow-up was 38.6 months. Complications occurred in eight patients: one luxation, two aseptic loosenings, and five reinfections were diagnosed. The Harris hip score (presurgical 30; postsurgical 78) showed significant improvement after revision surgery. Regarding the extent of the patients’ bone defects, good functional results were achieved. The comparatively low number of luxations and loosenings is due to the high modularity of the prosthesis with arbitrary antetorsion in the hip joint. However, high reinfection rates in mega-implants still constitute a problem and should be the subject of further studies.