Expression of beta-catenin and p53 are prognostic factors in deep aggressive fibromatosis

AIMS: To determine the prognostic significance of beta-catenin in aggressive fibromatosis and to identify potential molecular markers for new targeted therapies. METHODS AND RESULTS: A tissue microarray of 37 cases of deep aggressive fibromatosis was constructed and subjected to immunohistochemical analysis for beta-catenin, p53, smooth muscle actin (SMA), desmin, Ki67, c-erbB2, epidermal growth factor receptor (EGFR), c-kit, CD34 and S100. Complete clinical follow-up was available for 23 patients. Nuclear beta-catenin expression was associated with an increased rate of local tumour recurrence (60.0% 1-year and 0% 5-year event-free survival; P < 0.05). Furthermore, p53 expression was associated with an increased risk of tumour recurrence (50% 1-year event-free survival rate and 0% 5-years event-free survival rate, P < 0.05). The coexpression of p53 and beta-catenin was significantly correlated (P < 0.05). No statistically significant association was seen between MIB1 and p53 or beta-catenin expression, respectively. No expression of EGFR, c-erbB2 or c-kit was seen. CONCLUSIONS: The overexpression of beta-catenin and p53 is associated with a decreased event-free survival in deep aggressive fibromatosis. Further studies are required to establish whether these findings can lead to an improvement in the treatment of this rare neoplasm.     

Komplikationsmanagement bei Megaprothesen

Background

Megaprostheses are frequently used after segmental resection of bone sarcomas, bone metastases, and in large osseous defects in revision arthroplasty.

Objectives

The incidence of the most common complications associated the use of megaprostheses are reported. The management of complications including therapeutic recommendations are described.

Materials and methods

The current knowledge and our own experience of complication management with the use of megaprostheses are presented.

Results

Prospective, randomized studies or meta-analyses on this topic are lacking. An analysis of the literature shows that beside the occurrence of a local recurrence, periprosthetic infection remains the most serious complication. Two-stage revision remains the gold standard, but a single-stage exchange of the prosthesis without removing the stems might be possible in selected cases. Infection is associated with a higher risk of secondary amputation. In contrast, mechanical failures (e.g., wear of the bushings in knee replacements and aseptic loosening of the stems) can be treated more easily. Dislocation of a proximal femur replacement can mostly be prevented by using bi- or tripolar cups.

Conclusions

Complications with the use of megaprostheses can be successfully treated by revision surgery in most cases.     

Palliation und Frakturprävention

Sind Skelettmetastasen nachweisbar, so ist dies ein untrügliches Zeichen dafür, dass sich die Tumorerkrankung im gesamten Organismus ausgebreitet hat. Eine Heilung ist nur in seltenen F?llen zu erwarten. Die Therapie ist in diesem Stadium eher palliativ. Bei konservativer Therapiestrategie sollte wegen des Risikos von pathologischen Frakturen eine Abstimmung mit dem Chirurgen und Orthop?den erfolgen, um ggf. rechtzeitig operativ zu intervenieren. W?hrend eine pr?ventive operative Stabilisierung im Bereich der langen R?hrenknochen relativ unproblematisch ist, kann sie im Becken- und Wirbels?ulenbereich Komplikationen mit sich bringen.     

Chondroblastoma of the acromion mimicking fibrous dysplasia

The authors report the case of a 65-year-old man who presented with an expansive osteolytic lesion in the right acromion, mimicking cystic fibrous dysplasia. Magnetic resonance imaging showed a lesion with intermediate-signal intensity on T1-weighted images and a high-signal intensity on fat suppressed T2-weighted images. The biopsy led to the diagnosis of chondroblastoma. This tumour is rare in flat bones, and may mimic other benign or malignant lesions. It is therefore essential to perform a biopsy in order to obtain a definite diagnosis. The acromion was excised, and replaced with an iliac crest graft.     

Die Pathologische Fraktur aus Sicht des Orthopäden und Unfallchirurgen

Owing to the demographic development, the number of patients in Germany with cancer has been steadily rising over recent decades. While primary bone tumours remain rare, the number of skeletal metastases is clearly increasing. When diagnosis of skeletal metastasis is late, pathologic fractures are not uncommon; prediliction sites for fracture are the vertebral column, the pelvis, the femur and the humerus. This review gives an overview of the necessary diagnostic steps when a pathologic fracture is suspected and of the different treatment strategies. The type and extent of the treatment depends for example on the tumour entity, the time of occurrence of skeletal metastasis, the stage of disease and the site of the fracture. The aim of surgical treatment is full weight-bearing on the involved extremity until the patient’s death. Revision surgery due to tumour progression, pseudarthrosis and / or implant failures should be avoided because of an additional limitation of life quality in the palliative situation.     

Lack of toxicological side-effects in silver-coated megaprostheses in humans

Deep infection of megaprostheses remains a serious complication in orthopedic tumor surgery. Furthermore, reinfection gets a raising problem in revision surgery of patients suffering from infections associated with primary endoprosthetic replacement of the knee and hip joint. These patients will need many revision surgeries and in some cases even an amputation is inevitable. Silver-coated medical devices proved their effectiveness on reducing infections, but toxic side-effects concerning some silver applications have been described as well. Our study reports about a silver-coated megaprosthesis for the first time and can exclude side-effects of silver-coated orthopedic implants in humans. The silver-levels in the blood did not exceed 56.4 parts per billion (ppb) and can be considered as non-toxic. Additionally we could exclude significant changes in liver and kidney functions measured by laboratory values. Histopathologic examination of the periprosthetic environment in two patients showed no signs of foreign body granulomas or chronic inflammation, despite distant effective silver concentrations up to 1626 ppb directly related to the prosthetic surface. In conclusion the silver-coated megaprosthesis allowed a release of silver without showing any local or systemic side-effects.     

The treatment of locally recurrent chondrosarcoma: Is extensive further surgery justified?

The aim of this study was to define the treatment criteria for patients with recurrent chondrosarcoma. We reviewed the data of 77 patients to examine the influence of factors such as the intention of treatment (curative/palliative), extent of surgery, resection margins, status of disease at the time of local recurrence and the grade of the tumour. A total of 70?patients underwent surgery for recurrent chondrosarcoma. In seven patients surgery was not a viable option. Metastatic disease occurred in 41 patients, appearing synchronously with the local recurrence in 56% of cases. For patients without metastasis at the time of local recurrence, the overall survival at a mean follow-up after recurrence of 67 months (0 to 289) was 74% (5 of 27) compared with 19% (13 of 50) for patients with metastasis at or before the development of the recurrence. Neither the type/extent of surgery, site of tumour, nor the resection margins for the recurrent tumour significantly influenced the overall survival. With limited survival for patients with metastatic disease at the time of local recurrence (0% for patients with grade III and de-differentiated chondrosarcoma), palliative treatment, including local radiation therapy and debulking procedures, should be discussed with the patients to avoid long hospitalisation and functional deficits. For patients without metastasis at the time of local recurrence, the overall survival of 74% justifies an aggressive approach including wide resection margins and extensive reconstruction.