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.     

Aneurysmal bone cyst inadvertently treated with chemotherapy—A series of three cases

Aneurysmal bone cyst (ABC) is a benign locally aggressive tumor that occurs in childhood and early adulthood. Most relevant differential diagnoses are the telangiectatic osteosarcoma and the giant cell tumor. In the present case series chemotherapy following the EURAMOS or the Euro‐Ewing 99 protocol was externally applied in three patients with the misdiagnosis of ABC as malignant bone tumor. In all three cases, a significant reduction of the volume of the ABC was achieved. This is the first report about the use of neoadjuvant chemotherapy in ABC. Chemotherapy reduces the size of an ABC and leads to progressive sclerosis.     

Is a gradual reduction of stiffness on top of posterior instrumentation possible with a suitable proximal implant? A biomechanical study

Background Context

Proximal junctional kyphosis (PJK) is a challenging complication after rigid posterior instrumentation (RI) of the spine. Several risk factors have been described in literature so far, including the rigidity of the cranial aspect of the implant.

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.     

Sweet's syndrome associated with pigmented villonodular synovitis

Sweet's syndrome (acute febrile neutrophilic dermatosis) was first described in 1964. The typical symptoms of Sweet's syndrome are high temperature, peripheral leucocytosis, painful cutaneous rashes (papules, plaques) and arthralgia. Sweet's syndrome has particularly been described in association with neoplastic, infectious and immunological diseases. The pathogenesis of Sweet's syndrome can be explained by a reaction to an antigenic structure with accumulation of immunological complexes and liberation of inflammatory mediators. For the first time we report on a patient with Sweet's syndrome and pigmented villonodular synovitis, which is believed to play the antigenic role in the Sweet syndrome.     

Infiltration of sarcomas into the hip joint: comparison of CT,MRI and histologic findings in 67 cases

We analyzed the incidence, route, and characteristics of hip joint infiltration in pelvic or proximal femoral sarcomas. 67 patients with a sarcoma that originated around the hip joint (50 pelvic and 17 femoral) were included in this study. Preoperative CT and MRI were matched with the histological findings in tumor specimens. Tumor infiltration into the hip joint was suspected on the basis of preoperative imaging in 29 patients due to articular cartilage disruption, diffuse signal changes in the acetabulum or femoral neck, signs of a tumor in the joint, or markedjoint effusion. Of these 29 patients, 15 showed tumor invasion on histological examination. 12 of 31 chondrosarcomas, none of 12 Ewing's sarcomas, and 3 of 24 osteosarcomas infiltrated into the hip joint (p = 0.008). 10 of 26 low-grade sarcomas and 5 of 41 high-grade sarcomas infiltrated into the hip joint (p = 0.02). The joint infiltration rate of the chondrosarcomas was related to their size. Of 10 tumors originating in the acetabulum, 9 penetrated through or around the osseous-ligamentous junction and one through the acetabular cartilage. In 5 proximal femur lesions, all infiltrated the joint through the femoral neck, 3 of them also through the ligamentum teres.