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Sean P Scully

Publications and source records attributed to Sean P Scully.

At least 19 recordsLinked to original sources
PubMed2007

Case report. Granular cell tumors of extremities: comparison of benign and malignant variants.

Granular cell tumors are uncommon soft tissue neoplasms that arise from cells of neural origin. We present three patients with this rare tumor: one with a benign tumor, one with a malignant tumor, and one with a histologically benign tumor that later metastasized to the lung. We studied the difficulty in differentiating between the benign and malignant variants using only histology. We also compared the clinical, radiographic, and pathologic features and the criteria for malignancy, natural history, and prognosis.

Adult↗
PubMed2007

Fibular osteosarcomas: contaminated margins may not impact survival.

The fibula is an expendable bone which when affected by primary malignancy may simplify treatment and improve outcome. This concept was questioned previously in a study showing a high rate of inadequate margins at surgical resection. We asked whether margin status and other dependent variables affect survival. We analyzed the charts of 36 patients with fibular osteosarcomas treated from 1919 to 2000. We estimated survival by the Kaplan-Meier method and the prognostic significance of dependent variables, especially marginal status, with univariate association using Cox proportional hazard regression. The survival of patients with fibular osteosarcomas was limited, with a median survival of 5 years 4 months after diagnosis. At 5 years, 16 patients died of their disease. Sixteen patients were still alive at 10 years followup. The margin status at surgery was not associated with survival. A higher tumor grade at diagnosis, surgical treatment with above-knee amputation, and occurrence of metastasis were associated with poorer long-term survival. The survival of patients with fibular osteosarcomas was not better than with osteosarcomas arising in other locations. A marginal resection surprisingly did not impact on overall survival, although we had a limited dataset and heterogeneous treatment protocols.

Adolescent↗
PubMed2006

Salvage of failed massive allograft reconstruction with endoprosthesis.

UNLABELLED: A subset of osteoarticular allograft reconstructions after tumor resection ultimately will fail in patients achieving long-term survival. There are several alternative surgical approaches to revising these reconstructions. We retrospectively evaluated the outcome of 20 patients who had allograft reconstruction with a modular endoprosthesis. All patients had a failed massive allograft (average length, 15.7 cm) after tumor resection about the shoulder and knee. The reasons for original allograft failure were fracture (14 patients), infection (five patients), and both (one patient). Followup of the patients averaged 159.7 months (range, 63-293 months) after allograft reconstruction and 77 months (range, 24-234 months) after endoprosthetic revision. The average number of operative procedures each patient had was 4.1 (range, 2-15 procedures). Two patients had amputations for resistant periprosthetic infections. A successful revision procedure was accomplished in 80% of the patients, and 90% of the patients retained functional limbs at recent followup. The predicted 5-, 10-, and 15-year survivals were 92%, 55% and 28% respectively. Musculoskeletal Tumor Society scores averaged 76% (range, 60-93.3%). When used to salvage massive allograft failure from infection and fractures, endoprosthetic revision preserves limb function with minimal complications. LEVEL OF EVIDENCE: Therapeutic study, Level IV (case series). See the Guidelines for Authors for a complete description of levels of evidence.

Adolescent↗
PubMed2006

Distal femur replacement is useful in complex total knee arthroplasty revisions.

UNLABELLED: Revision total knee arthroplasty can be considerably more difficult in cases involving severe bone loss, complete absence of collateral ligaments, and persistent periprosthetic supracondylar femoral fractures. Modular segmental endoprosthetic distal femur replacement is a limb salvage option when other surgical options are unfeasible. The clinical performance of rotating hinge knee prostheses has greatly improved with the evolution of second and third generation designs. The increased freedom of rotation decreases the prosthetic bone stresses and the longevity. While designs have improved, the prostheses still do not match the function and longevity of condylar components with approximately a 90% survivorship at 20 years. The modular endoprosthesis rotating hinge knee prosthesis is useful for total knee arthroplasty revision in patients with inadequate bone stock, ligamentous instability, or difficult supracondylar femur fractures, especially in low demand elderly patients. LEVEL OF EVIDENCE: Therapeutic study, level V (expert opinion). See Guidelines for Authors for a complete description of levels of evidence.

Aged↗
PubMed2005

Ganglioneuromas of the sacrum-a report of two cases with radiologic-pathologic correlation.

Ganglioneuromas are rare, benign, well-differentiated, slow-growing tumors, composed of ganglion cells and Schwann cells. Ganglioneuromas are derived from the neural crest cells and can arise anywhere from the base of the skull to the pelvis. We present and discuss the clinicopathologic and radiographic features of two patients with ganglioneuroma arising from the sacrum, a rare anatomic location.

Adult↗
PubMed2005

Dysplasia epiphysealis hemimelica of the tibial tubercle.

Dysplasia epiphysealis hemimelica (DEH) is a rare skeletal dysplasia with epiphyseal involvement first described by Mouchet and Belot in 1926. Lower extremity involvement is common and might involve a single or multiple epiphyses in the affected extremity. We report an unusual case of involvement of the tibial tubercle in a girl aged 4 years 8 months, and we present the clinical, radiographic and pathologic findings. We discuss the role of MRI in the diagnosis and treatment plan.

Child, Preschool↗
PubMed2005

RNAi mediated MMP-1 silencing inhibits human chondrosarcoma invasion.

Previous work indicates that matrix metalloproteinase-1 (MMP-1) gene expression is a prognostic factor for local recurrence and metastasis in human chondrosarcoma. The current study evaluates the effects of MMP-1 knock-down via RNA interference on chondrosarcoma metastasis in vitro. Of nine siRNA oligos tested, one showed a 90% inhibitory effect on MMP-1 gene expression. Stable attenuation of MMP-1 in chondrosarcoma cells was achieved by using a plasmid vector-based siRNA system. Pronounced MMP-1 activity suppression was accompanied by marked inhibition of tumor cell invasion. Cells in which MMP-1 has been stably silenced are useful tools to elucidate the mechanism of MMP-1 mediated tumor metastasis.

Bone Neoplasms↗
PubMed2005

siRNA mediated inhibition of MMP-1 reduces invasive potential of a human chondrosarcoma cell line.

Matrix metalloproteinases (MMPs) play a crucial role in tumor cell invasion and metastasis. Expression of MMP-1 has been reported as a prognostic predictor of recurrence in human chondrosarcoma, and studies using human chondrosarcoma cell lines indicate that MMP-1 expression levels correlate with in vitro invasiveness. These observations suggest that MMP-1 activity has a central role in cell egress from the primary tumor at an early step in the metastatic cascade. In this study, siRNA was used to investigate whether knock down of the MMP-1 gene could be used to inhibit invasiveness in a human chondrosarcoma cell line. The inhibitory effect of siRNA on endogenous MMP-1 gene expression and protein synthesis was demonstrated via RT-PCR, Northern blotting, Western blotting, collagenase activity assay, and an in vitro cell migration assay. The siRNA inhibited MMP-1 expression specifically, since it did not affect the expression of endogenous glyceraldehyde phosphate dehydrogenase (GAPDH) nor other collagenases. Most importantly, the siRNA mediated reduction in MMP-1 expression correlated with a decreased ability of chondrosarcoma cells to invade a Type I collagen matrix. The reduction of invasive behavior demonstrated by human chondrosarcoma cells transfected with MMP-1 siRNA and the specificity of this inhibition supports the hypothesis that this metalloproteinase molecule is involved in initiation of chondrosarcoma metastasis.

Cell Line, Tumor↗
PubMed2005

Epithelioid sarcoma of the hand.

Twenty-eight patients were treated for a primary epithelioid sarcoma of the hand. Twenty-seven patients (96%) had excisions before evaluation, including 11 (39%) with multiple prior excisions with varying diagnoses before epithelioid sarcoma, and all had surgical treatment after referral. The patients' surgical management included three patients with amputation at the forearm, three patients with wide excision, and 21 patients with a partial amputation of the hand. The followup period averaged 120 months (range, 24-276 months). Eighteen patients have no evidence of disease at last followup. Treatment failures included one local recurrence, four regional metastases, and five distant metastases. Five patients died secondary to disease. Two patients are alive with disease, and three are alive with no evidence of disease after additional treatment. After aggressive surgical management with negative margins, 71% of the patients were alive without evidence of disease at the last followup, with a 5- and 10-year survivorship of 85%. Our goal is to review: (1) the effectiveness of preoperative imaging, (2) the role of adjuvant therapy, (3) survival after alternative resections, and (4) function after resection.

Adult↗
PubMed2005

An institutional review of clear cell chondrosarcoma.

UNLABELLED: Clear cell chondrosarcoma is a rare bone neoplasm with a slow progressive clinical course and infrequent metastasis, but with a high local recurrence rate. We sought to ascertain the long-term outcome of patients with this neoplasm and to identify possible factors predicting survival. Sixteen patients with clear cell chondrosarcomas treated at one institution and who had long-term clinical followup were identified. All patients were treated by surgical resection of the tumor, which was classified as clear (> 2 mm) in 10 patients and marginal or intralesional in six patients. Three patients had local recurrence after a median of 1.7 years. Metastatic disease developed in four patients with a median time to diagnosis of 8.1 years. Ten-year overall survival of patients with clear cell chondrosarcomas was 89%, and disease-free survival was 68%. Patients with surgical resections comprising clear margins had longer disease-free survival compared with patients with marginal and intralesional tumors. Inadequate surgical resection of clear cell chondrosarcoma leads to risk of local recurrence and metastatic disease. The malignancy has a tendency to metastasize relatively late, therefore, followup of patients is necessary after the generally accepted 5-year period. LEVEL OF EVIDENCE: Prognostic study, Level III (case control study). See the Guidelines for Authors for a complete description of levels of evidence.

Adolescent↗
PubMed2005

The radiographic morphology of foot deformities in patients with fibrodysplasia ossificans progressiva.

BACKGROUND: The most consistent deformities that allow early diagnosis of fibrodysplasia ossificans progressiva are the presence of bilateral short first rays and hallux valgus. The purpose of this study was to describe the radiographic features observed in the feet of patients with fibrodysplasia ossificans progressiva. METHODS: The radiographs of 26 feet (15 patients with fibrodysplasia ossificans progressiva) were reviewed to evaluate the radiographic changes that occur in the first ray. Variables analyzed were the hallux valgus (HV) angle, the distal metatarsal articular (DMA) angle, the proximal phalangeal articular (PPA) angle, the intermetatarsal (IM) angle, ratio of the lengths of the first and second metatarsal lengths (MT1:MT2), and the first and second ray length ratio. The length ratios were then subtracted from similar ratios in radiographs of age- and gender-matched normal patients previously reported. RESULTS: The proximal phalanx was consistently shortened but morphologically dissimilar from subject to subject. Asymmetry was noted in some patients with bilateral radiographs. The mean HV angle was 28 degrees, and the mean IM angle was 10 degrees. The mean DMA angle was 33 degrees, and the mean PPA angle was 14 degrees. The MT1:MT2 ratio was 0.89, and the mean first ray to second ray length ratio was 0.87. The mean of the differences in the MT1:MT2 and first and second ray length ratios in patients with fibrodysplasia ossificans progressiva compared to the normal controls were 0.05 and 0.01, respectively. Fusion occurred between the abnormal tibial epiphysis of the proximal phalanx and metatarsal head with advancing age, and 68% of the metatarsal heads were fused with the abnormal proximal phalangeal epiphysis. CONCLUSIONS: Foot pathology in patients with fibrodysplasia ossificans progressiva is variable but consistently involves an abnormality of the tibial aspect of the proximal phalangeal epiphysis of the hallux. This results in the clinical observation of hallux valgus in these patients. The first metatarsal is consistently shortened, and fusion between the epiphysis of the abnormal proximal phalanx and the shortened first metatarsal head occurs with advancing age.

Child↗
PubMed2005

Current treatment for Ewing's sarcoma.

Ewing's sarcoma is the second most common primary bone tumor seen in children and adolescents, and was described by James Ewing in 1921 as a diffuse endothelioma of bone. It is one of the differential diagnoses of pediatric small round blue cell tumors. This is not a single condition, but a group of morphologically and clinically closely related disorders with similar molecular biology -- expression of tumor-specific chimeric oncoproteins through balanced chromosomal translocations involving the EWS gene -- often referred to as the Ewing family of tumors. This includes Ewing's sarcoma of bone, extra-osseous Ewing's sarcoma, Askin tumor and peripheral neuroectodermal tumor. These are aggressive neoplasms with almost 25% of patients having clinically evident metastases at presentation. Ewing's sarcoma has therefore been considered as a systemic disease necessitating local as well as systemic treatment. An aggressive multidisciplinary approach has resulted in significant improvement in prognosis for patients with these tumors. Despite aggressive treatment, 20-40% of patients with localized disease and almost 80% of patients with metastatic disease at presentation succumb to the illness. Advances in understanding the molecular biology of these tumors will hopefully result in the development of novel treatment approaches. The aim of this article is to review the existing treatment methods and to highlight the more recent approaches to the treatment of this condition.

Adolescent↗
PubMed2005

Dislocation rate after hip hemiarthroplasty in patients with tumor-related conditions.

BACKGROUND: Hemiarthroplasty is frequently used to treat femoral neck insufficiency resulting from neoplastic disease in the proximal part of the femur. The authors of a recent study analyzed the dislocation rates following hemiarthroplasty but excluded patients with tumor involvement of the site of the surgery as they hypothesized that the dislocation rates would be markedly higher in such patients. The current study was performed to compare the dislocation rate following hemiarthroplasties performed in patients without tumor involvement with the rate following hemiarthroplasties in patients with tumor involvement of the surgical site. METHODS: Patients who had undergone hemiarthroplasty following resection of a tumor involving the proximal part of the femur were identified in a total joint registry, and the patients' charts were reviewed retrospectively to determine dislocation rates, preoperative conditions, and postoperative outcomes and treatments. Between 1974 and 2001, 1812 patients were treated with hemiarthroplasty for reasons other than tumor involvement and 320 hemiarthroplasties were performed because of tumor-related conditions. The patients who were treated for a tumor-related condition were younger, and a higher proportion of them were men. RESULTS: The ten-year dislocation rate after the hemiarthroplasties performed for tumor-related conditions (10.9%) was higher than that following the hemiarthroplasties performed for non-tumor-related conditions (2.1%) (p = 0.002). The median time to dislocation in the patients with a tumor-related condition (twenty-four days) was shorter than that for the patients without tumor involvement (thirty-seven days). Preservation of the greater trochanter in patients with tumor involvement did not have a significant influence on the dislocation rate, but it showed a favorable trend toward decreasing that rate (hazard ratio = 3.5, p = 0.06). CONCLUSIONS: The short-term and long-term dislocation rates associated with hemiarthroplasties performed for a tumor-related condition at the site of the surgery were significantly higher than those associated with hemiarthroplasties performed for reasons other than tumor involvement. Preservation of the greater trochanter showed a trend toward decreasing the likelihood of dislocation following the hemiarthroplasty, and it was more influential than the level of resection and the extent of soft-tissue compromise. We think that preservation of the greater trochanter should be attempted when it is justifiable according to the principles of oncologic surgery.

Adolescent↗
PubMed2004

Absence of a correlation between the presence of a single nucleotide polymorphism in the matrix metalloproteinase 1 promoter and outcome in patients of chondrosarcoma.

PURPOSE: Increased levels of matrix metalloproteinase 1 (MMP-1) expression have been associated with poor outcome in chondrosarcoma. The existence of a single nucleotide polymorphism creating an Ets-binding site in the MMP-1 promoter may be one mechanism for elevated MMP-1 transcription. The aim of our study was to identify the prevalence of this single nucleotide polymorphism (SNP) in chondrosarcoma patients, to determine its correlation with disease outcome, and to discern whether it could serve as a prognostic marker in patients with chondrosarcoma. EXPERIMENTAL DESIGN: Sixty-seven chondrosarcoma specimens were selected sequentially from an established tumor bank with a median duration of 47 months follow-up (range, 24 to 179 months). DNA was extracted, amplified with PCR, and sequenced to determine presence (GG) or absence of the Ets-binding site created by the SNP. RESULTS: Eighteen (27%) samples were homozygous for the absence of the Ets site, 34 (51%) were heterozygous for the SNP, and 15 (22%) were homozygous for the SNP. The 5-year overall survival rate for patients was 78, 80, and 84%, respectively (P = 0.5527). The disease-free survival rate was 16, 63, and 76%, respectively (P = 0.0801). The 5-year disease-free survival rate for patients with the homozygous G/G genotype was 16%, compared with 71% for patients who were either homozygous or heterozygous for the GG allele (P = 0.0444). CONCLUSIONS: Despite a statistical correlation between MMP-1 gene expression and outcome in chondrosarcoma, this study demonstrates an absence of a correlation between the presence of the SNP and prognosis in patients with chondrosarcoma.

Adult↗