Reconstruction Using Free Vascularized Fibular Grafts after Wide Resection of Humerus Chondrosarcoma in a Patient with Cleidocranial Dysplasia.

Cleidocranial dysplasia is characterized by hypoplasia of the clavicles, unerupted teeth, narrow pelvis, short stature, and craniofacial malformations. A cause of this skeletal dysplasia is heterozygous mutations of the runt-related transcription factor 2 gene (Runx2), a master regulator for bone and cartilage development. Chondrosarcoma is a primary malignant bone tumor that is usually treated by wide resection surgery. This report shows a case of a 25-year-old female patient with cleidocranial dysplasia who was affected with chondrosarcoma of the left humerus. We performed wide resection of the tumor and reconstruction of the large bone defect of the humerus using free vascularized fibular grafts. The patient preserved the hand function and activity of daily life as the same level as preoperative condition more than five years after the surgery.

Chondrosarcoma mimicking MRI of the osteonecrosis of the femoral head: a case report.

A 25-year-old female visited our hospital with an 8-year history of arthralgia in the right hip joint. Plain radiography of the hip revealed a well-demarcated radiolucent lesion with a thin sclerotic rim in the epiphysis of the femoral head. T 1 weighted MRI revealed the demarcation line of a low-signal-intensity band in the femoral head. We were aware that this band did not split the signal of adipose tissue in the bone marrow. In cases of osteonecrosis, we usually find a low-signal-intensity band splitting the signal of normal bone marrow. However, we could not see such a low-signal-intensity band in this case. Therefore, we decided to perform other studies. Contrast-enhanced T 1 weighted MRI showed remarkable enhancement in the segment proximal to the low-signal-intensity band, indicating that this lesion might have blood perfusion. We decided to perform a bone biopsy to clarify the diagnosis. Histopathological examination of the biopsy specimen revealed chondrosarcoma. We found that contrast-enhanced MRI plays an important role to rule out osteonecrosis of the femoral head.

Extraction of well-fixed extended porous-coated cementless stems using a femoral longitudinal split procedure.

We present a technique of posterior femoral longitudinal split (FLS) osteotomy. This technique allows the expansion of the metaphyseal-diaphyseal region of the proximal femur facilitating extraction of well-fixed extended porous-coated stems. The extractions were performed using extended transfemoral osteotomy (ETO) and FLS osteotomy between June 2002 and March 2014. The study group, which comprised patients with well-fixed extended porous-coated stems, consisted of two men and ten women with an average age of 63.2 years. The stem was successfully removed using the FLS procedure in 8 of the 10 hips. Reimplantation surgery was performed in 6 of the 12 hips with ARMD, periprosthetic infection, or metallosis. This FLS technique may allow the easy removal of well-fixed extended porous-coated stems and become an alternative method for the removal of all stems.

Prognostic impact of CD109 expression in myxofibrosarcoma.

BACKGROUND: CD109, a TGF-ß co-receptor, is reported to be preferentially expressed during the early stages of tumorigenesis in several carcinoma types. Myxofibrosarcoma is one of the most common soft-tissue sarcomas found in elderly patients. This study aimed to elucidate the impact of CD109 expression in myxofibrosarcoma on prognosis and recurrence. METHODS: Immunohistochemical staining for CD109 was performed on archival specimens from 37 patients. The Fisher exact test was used to evaluate association between CD109 expression and other clinicopathological features. Survival analysis was performed using Kaplan-Meier curves, and the prognostic significance was evaluated using the log-rank test. Multivariate analysis of factors was performed using Cox regression analysis. RESULTS: CD109 overexpression was significantly associated with surgical stage and distant metastasis (P = 0.00499, and 0.011, respectively). The frequency of CD109 overexpression was approximately 10% and CD109 overexpression was significantly associated with decreased overall survival (P = 0.004). Five-year overall survival rates 77% and 0% for CD109-negative and CD109-positive patients, respectively. In multivariate analysis, CD109 overexpression was the only independent risk factor for poor outcome (P = 0.02; hazard ratio, 10.64; 95% confidence interval, 1.47-76.91). CONCLUSIONS: Immunohistochemical CD109 expression in myxofibrosarcoma was associated with poor prognosis.

Development of non-traumatic osteonecrosis of the femoral head requires toll-like receptor 7 and 9 stimulations and is boosted by repression on nuclear factor kappa B in rats.

Non-traumatic osteonecrosis of the femoral head (ONFH) often occurs after corticosteroid therapy in patients with inflammatory diseases. Recent studies suggest that toll-like receptor (TLR) signaling may contribute to the pathogenesis of inflammatory diseases, and that the reason for corticosteroid therapy for inflammatory diseases is related to the anti-inflammatory activities of corticosteroids through the reduction of NF-κB. We hypothesized that the administration of TLR ligands in combination with corticosteroid causes ONFH and that transcription factors may contribute to the pathogenesis of ONFH. The aim of the study was to evaluate (1) the incidence of ONFH in rats after the administration of TLR7 or TLR9 ligands together with methylprednisolone (MPSL) and (2) whether transcription factors contribute to the development of ONFH. Male Wistar rats (n=148) were divided into five groups as follows: Group 1: Saline+MPSL, Group 2: Imiquimod+Saline, Group 3: Imiquimod+MPSL, Group 4: CpG-C+MPSL, Group 5: Imiquimod+BAY11-7082+MPSL. As a result, ONFH was observed in 0 of 12 rats in Group 1, in 1 of 10 in Group 2, in 6 of 12 in Group 3, in 4 of 12 in Group 4, in 0 of 9 in Group 5. MPSL treatment did not significantly affect IRF7 activity, whereas NF-κB activity was significantly repressed in Group 2 and Group 3. Furthermore, the repression in interferon regulatory factor 7 (IRF7) activity by BAY11-7082 interfered with the development of ONFH simultaneously with the MPSL treatment-induced repression in NF-κB activity. In conclusion, in the present study, corticosteroid treatment after the administration of TLR7 or TLR9 ligands caused ONFH. Repression in NF-κB activity by corticosteroid treatment boosted the development of ONFH.

Rapid multiorgan dissemination of low-grade myxofibrosarcoma: a case report.

Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly people. It is characterized by a high frequency of local recurrence due to an infiltrative growth property. In contrast, the overall risk of distant metastases is generally low. This makes the prognosis for the patients with myxofibrosarcoma definitely good. In this paper, we will report the case of a 79-year-old female with very aggressive metastatic low-grade myxofibrosarcoma. The disease progression was really unexpected and misled every possible medical interpretation, leading to rapid worsening of the patient's clinical conditions and no chance for therapy. The tumor developed diffuse infiltration in lung, spine, skeletal bone, abdomen, paravertebral muscles, and liver. The patient died 8 months after the diagnosis of remote metastases due to rapid tumor progression.

Pre-operative selective arterial embolization as a neoadjuvant therapy for proximal humerus giant cell tumor of bone: radiological and histological evaluation.

The management of giant cell tumor of the proximal humerus that extends to the joint is challenging. Here, we report a case of proximal humerus giant cell tumor with cortical bone destruction extending to the shoulder joint. Pre-operative selective arterial embolization induced peripheral tumor ossification. Subsequently, the lesion was removed by intralesional curettage, and the cavity was filled with cement. Macroscopically, the inner wall of the cavity was found to be lined with a thick fibrous membrane. Histologically, massive fibrosis and resultant remodeling of the destroyed cortical bone were induced, which was consistent with the peripheral ossification on the plain radiograph. We believe that selective arterial embolization can be an effective neoadjuvant therapy for giant cell tumors of the extremities, especially for tumors with large cortical defects or joint involvement.

Revision total hip arthroplasty due to pain from hypersensitivity to cobalt-chromium in total hip arthroplasty.

We report a case with hypersensitivity to CoCr in total hip arthroplasty coupled with conventional polyethylene and CoCr femoral head. The patient complained of left hip pain and systemic fever, and computed tomography imaging revealed a periprosthetic cystic lesion, so we performed revision total hip arthroplasty using a titanium stem and ceramic head and highly crosslinked polyethylene. Hip pain and cystic lesion disappeared 3 years after revision surgery.

Giant cell tumor of the sacrum treated with selective arterial embolization.

Giant cell tumor of the sacrum is extremely difficult to manage. Standard treatments, including surgery and radiation, are associated with significant complications and recurrence rates. In this manuscript, we report an early clinical result of a case of giant cell tumor of the sacrum successfully managed with selective arterial embolization. A 56-year-old woman underwent selective embolization for management of giant cell tumor of the sacrum. Radiologically, massive shrinkage of the extraosseous mass and increased peripheral ossification were obvious. Clinically, rapid pain relief was achieved and gait disability recovered. At final follow-up 28 months after completion of treatment, she retained normal activity in daily life. We stress the effectiveness of selective arterial embolization as a less invasive and less complicated primary treatment of giant cell tumors of the sacrum.

Primary total hip arthroplasty with Asian-type AML total hip prosthesis: follow-up for more than 10 years.

BACKGROUND: We retrospectively reviewed 137 consecutive total hip arthroplasties performed with AML-A stems and Tri-Lock cups to see whether design modifications made to these components would achieve durable biological fixation in the Japanese population in whom developmental dysplasia of the hip (DDH) is relatively common. Patients from our initial clinical series using these components are available for more than 10 years' follow-up. METHODS: Between April 1988 and June 1994, we performed 137 total hip arthroplasties using the AML-A prosthesis for the patients with osteoarthritis of the hip joint. We excluded 26 hips with less than 10 years' follow-up and five hips from patients who died before the 10-year follow-up. The mean follow-up for the 105 remaining THAs is 155.2 months (range 120-237 months). The average age of these patients at the time of surgery was 53.1 years (range 22-81 years). RESULTS: In total, 17 THAs required component revisions. In seven cases, the first revision was limited to a liner exchange for polyethylene wear or osteolysis. Another seven hips underwent revision surgery for recurrent dislocation. Three cups have been revised owing to aseptic loosening. Because of the high incidence of wear-related revisions, Kaplan-Meier survivorship at the 15-year follow-up, using acetabular component revision for any reason as an endpoint, was 75.0% (95% CI 69.4%-83.8%). In contrast, no revision of the femoral stem was performed. Severe stress shielding occurred in 12 hips. Thigh pain was mild, however, and all of the femoral stems remained stable. CONCLUSIONS: Despite revisions for wear-related complications, the fixation achieved with these porous-coated components remained durable throughout the 15-year follow-up. Acetabular osteolysis has been associated with cup reoperation, but femoral stress shielding has never resulted in stem loosening.

MRI and histological evaluation of the infiltrative growth pattern of myxofibrosarcoma.

OBJECTIVE: Myxofibrosarcoma often shows abnormal signal infiltration along the fascial plane on magnetic resonance imaging (MRI). The objective was to describe this MRI characteristic of myxofibrosarcoma with pathologic findings for comparison. MATERIALS AND METHODS: Clinical, histological, and imaging data for 21 patients with myxofibrosarcoma were reviewed retrospectively. RESULTS: Seventeen tumors showed a diffuse infiltrative pattern on MRI. All tumors with diffuse infiltrative growth pattern showed borderless extension of atypical cells with moderate nuclear atypia to the muscle fascia. Notably, the remaining four patients with focal growth pattern on MRI also demonstrated infiltrative growth pattern histologically suggesting that myxofibrosarcoma shows an infiltrative growth property even in the lack of infiltrative growth pattern on MRI. CONCLUSION: Most myxofibrosarcoma show an infiltrative growth pattern histologically. Orthopedic oncologist should pay careful attention to accurately assess tumor extension. It seems prudent to resect the entire area of abnormal signal extension seen on MRI whenever possible to obtain an adequate surgical margin of myxofibrosarcoma.