Clear-cell sarcoma of the soft tissue--a rare diagnosis with a fatal outcome.

Clear-cell sarcomas account for less than 1% of all soft tissue tumours. They most often occur in middle-aged adults as a deeply located lesion with predilection to the tendons and aponeuroses. The aim of the present study was to show possible influencing factors on the outcome after surgical treatment in a detailed case series. We reviewed the medical records of 11 patients with the diagnosis of a clear-cell sarcoma of the soft tissue. These cases were analysed with regard to age, gender, localisation, tumour size, recurrence free survival and overall survival. A minimum follow up of 12 months was achieved. The mean age at the point of diagnosis was 47.9 years. Metastases occurred after a mean of 19.2 months. In the cases with a tumour diameter >5 cm, metastases occurred earlier. When treated in a specialist centre, metastases occurred later. Patients died a mean of 18.4 months after developing metastatic disease. Patients with tumour size >5 cm at the point of primary diagnosis died earlier than patients with a tumour size <5 cm. It is important to detect clear-cell sarcomas as soon as possible and the final surgical treatment should be performed in a centre familiar with the treatment of soft tissue tumours not only to prolong overall survival, but also to treat the patient in a multiprofessional team.

[Recurrent hammer and clawed toes after surgical correction]. / Rezidive nach Korrektur von Hammer- und Krallenzehen.

Symptomatic lesser toe deformities should be corrected if conservative therapy is exhausted or no longer seems appropriate. Prevention of recurrence starts with the correct indication for the appropriate surgical procedure. The occasional difficult question of the cause of the deformity is crucial in this context. A correct surgical technique and appropriate after-care reduces the risk of recurrence. Due to tensed bony and soft-tissue conditions, revision surgery of the forefoot is challenging. If a revision becomes necessary, the cause of recurrence must be re-examined. This paper explains the appropriate indications and surgical techniques for correction of lesser toe deformities. Subsequently, specific postoperative complications and their management are discussed in detail.

The prevalence of acetabular anomalies associated with pistol-grip-deformity in osteoarthritic hips.

BACKGROUND: Acetabular retroversion, excessive acetabular coverage and abnormal head-neck-junction with a so-called "pistol-grip-deformity" were added to the classical description of hip dysplasia to describe pathological hip morphology. The aim of the current study was the detection of pathological acetabular geometry in patients with an abnormal head-neck-junction. HYPOTHESIS: Femoroacetabular impingement and hip dysplasia features are frequent in patients with end-stage osteoarthritis before 60 years of age. MATERIALS AND METHODS: We analysed our data bank retrospectively for all patients who received a Total Hip Arthroplasty (THA) due to end-stage osteoarthritis before the age of 60 years. The pelvic-views and the Dunn-view of these patients were screened for an abnormal head-neck-junction by measuring the head-ratio and the alpha-angle. An orthopaedic surgeon and a radiologist did this independently. These radiographies were measured for signs of acetabular dysplasia, excessive acetabular coverage and crossing sign. RESULTS: A consecutive series of 135 total hip arthroplasties were performed in patients aged less or equal to 60 years because of end-stage osteoarthritis. From these, 81 patients were classified as having an abnormal head-neck-junction. The mean head-ratio in these 81 patients was 1.52±0.35, the mean alpha-angle was 62.5°±9.3°. The mean CE-angle of these 81 patients was 35.8°±10.4°, the mean CA-angle was 36.7°±5.7°, the mean depth-width ratio was 49.1±10, the mean extrusion index was 19.1±9.2 and the mean CCD-angle was 131.7°±7.3°. Of these 81hips, 14 had isolated pistol-grip-deformity, while 11 hips had associated dysplasia, 38 had excessive acetabular coverage, and 14 had crossing sign. In addition, a crossing sign was identified in four of the 11 dysplastic hips and 19 of the 38 of the hips having excessive acetabular coverage. There was no statistically significant difference in regard to the age between the four groups (P=0.087). In contrast, the hips that had excessive acetabular coverage had increased CE-angle (44.6°±7.2°) and decreased extrusion index (12.6±6.5) (P<0.001), while dysplastic hips had increased roof obliquity (17.5°±4.5°) and increased extrusion index (29.6±9.1), as well as decreased CE-angle (20.7°±3.0°) (P<0.001). CONCLUSION: There is a high coincidence of radiographic findings associated with an abnormal head-neck-junction consisting in excessive acetabular coverage and retroversion as well as hip dysplasia. These results advocate for restoring of the normal anatomy at the early stage to prevent end-stage osteoarthritis. LEVEL OF EVIDENCE: Level IV retrospective historical study.

Indications for total hip arthroplasty in young adults - idiopathic osteoarthritis seems to be overestimated.

PURPOSE: New aspects like acetabular overcoverage, acetabular retroversion and proximal femoral head-neck dysplasia have been detected as a main cause of osteoarthritis. The study addresses the detection of reasons for osteoarthritis requiring THA in young adults. We wanted to prove the hypothesis that idiopathic reasons play an overestimated role in osteoarthritis in young patients. MATERIALS AND METHODS: 228 total hip arthroplasties in patients aged ≤ 60 years were performed at our institution. After the detection of the primary reasons for osteoarthritis of the hip, the radiographic pictures of all other patients were analyzed for radiographic signs of hip dysplasia or femoroacetabular impingement. For interobserver quality testing, this was done by two different observers. RESULTS: 132 patients were initially classified as having idiopathic osteoarthritis of the hip.  There was no pathological radiographic finding in only 5 patients. 80 patients presented a reduced head-neck offset as a sign of CAM impingement with a mean head ratio of 1.52 ±â€Š0.35 and an alpha angle of 62.8 ±â€Š9.28°. 21 patients presented a figure-8 sign as an indicator for acetabular retroversion. 68 patients presented at least one radiographic finding for "dysplasia" and 60 patients at least one radiographic finding for excessive "overcoverage". The Bland-Altman Plot for testing interobserver reliability demonstrated good interobserver agreement. CONCLUSION: Idiopathic OA in young adults is rare if you look hard enough for the underlying pathology. If treated, patients might benefit and THA could be postponed for several years.

Alteration of bone sialoprotein expression in osseous metastasized renal cell carcinomas and the tumor surrounding tissue.

Bone sialoprotein (BSP) regulates bone metabolism by directly influencing the activity of osteoblasts and osteoclasts. A significant correlation between the tissue expression of BSP in tumors and the occurrence of bone metastases was found in different cancers. Aim of this study was to identify the BSP expression in renal cell carcinomas (RCC) according to their stage of metastatic disease. Tissue samples of patients with RCC who underwent partial resection or nephrectomy were separated into three groups, each with 10 patients showing either no metastases (group I), only soft tissue metastases (group II) or bone metastases (group III) at date of surgery. Immunohistochemical analysis of BSP expression in tumor tissue and corresponding renal parenchyma was performed and evaluated with an established semiquantitative scoring system. BSP expression was detected both in tumor tissue and renal parenchyma. Concerning the expression in malignant tissue, no significant difference could be found between the three groups whereas the corresponding renal parenchyma showed a staining score of 164, 198 and 224 for group I, II and III (P = 0.07). RCC staged T3 showed only a little higher BSP expression than those staged T1/2 (P < 0.21), while the corresponding parenchyma of T3 tumors showed significantly higher expressions (P = 0.02). This pilot study revealed a correlation between expression of BSP and tumor staging and type of metastases, especially for osseous metastases in RCC. Alternation of BSP expression could be detected particularly in renal parenchyma and linked to the type of metastases.

A new classification for "pistol grip deformity" - correlation between the severity of the deformity and the grade of osteoarthritis of the hip.

PURPOSE: Two types of femoroacetabular impingement (FAI) are described as reasons for the early development of osteoarthritis of the hip. Cam impingement develops from contact between an abnormal head-neck junction and the acetabular rim. Pincer impingement is characterized by local or general overcoverage of the femoral head by the acetabular rim. Both forms might cause early osteoarthritis of the hip. A decreased head/neck offset has been recognized on AP pelvic views and labeled as "pistol grip deformity". The aim of the study was to develop a classification for this deformity with regard to the stage of osteoarthritis of the hip. MATERIALS AND METHODS: 76 pelvic and axial views were analyzed for alpha angle and head ratio. 22 of them had a normal shape in the head-neck region and no osteoarthritis signs, 27 had a "pistol grip deformity" and osteoarthritis I and 27 had a "pistol grip deformity" and osteoarthritis II°-IV°. The CART method was used to develop a classification. RESULTS: There was a statistically significant correlation between alpha angle and head ratio. A statistically significant difference in alpha angle and head ratio was seen between the three groups. Using the CART method, we developed a three-step classification system for the "pistol grip deformity" with very high accuracy. This deformity was aggravated by increasing age. CONCLUSION: Using this model it is possible to differentiate between normal shapes of the head-neck junction and different severities of the pistol grip deformity.

Close localization of DAP-kinase positive tumour-associated macrophages and apoptotic colorectal cancer cells.

The death-associated protein kinase (DAP-kinase) is a cytoskeleton-associated protein crucially involved in the induction of early apoptotic pathways. Aberrant hypermethylation of the DAP-kinase promoter plays a major role in tumorigenesis. We aimed to investigate the inactivation of DAP-kinase and its association with apoptotic cell death in 94 colorectal carcinomas. DAP-kinase promoter hypermethylation and mRNA expression were investigated using methylation-specific PCR and real-time RT-PCR, respectively. The expression of DAP-kinase, Fas, and Fas-ligand (FasL) proteins was studied by immunohistochemistry and immunofluorescence. Apoptosis of tumour cells was investigated using the TUNEL assay. DAP-kinase was expressed in tumour cells and tumour-invading macrophages and was closely associated with high numbers of apoptotic tumour cells. DAP-kinase expression co-localized with FasL overexpression in tumour-associated macrophages, and aberrant promoter hypermethylation was verified in more than 50% of carcinomas. There was a tendency for proximal tumours to show DAP-kinase promoter methylation more frequently (p = 0.07). Promoter methylation resulted in a decrease or loss of DAP-kinase protein expression in tumour cells and tumour-associated macrophages. Simultaneously, a decreased apoptotic count and loss of Fas/FasL expression was observed in tumour cells. Our study is the first to demonstrate DAP-kinase expression in invading tumour-associated macrophages in colorectal cancer. The presence of similar expression levels of DAP-kinase in tumour cells and associated macrophages, and their dependence on the promoter methylation status of the tumour cells, suggests cross talk between these cell types during apoptotic cell death.