WorldWideScience

Sample records for chondrosarcomas

  1. Testicular chondrosarcoma

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    Yalçinkaya Ulviye

    2003-01-01

    Full Text Available A case of primary chondrosarcoma of the testis is reported. A 40-year-old man presented a painless swelling of the right testis that he has been observing for 3 years. Gross examination of the resected specimen showed an encapsulated, gray to tan colored, roughly rounded tumor. Histologically, the tumor revealed a well-differentiated chondrosarcoma.

  2. Intracortical chondrosarcoma: a case report.

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    Khodamorad Jamshidi

    2014-02-01

    Full Text Available Chondrosarcoma is the second most common primary mesenchymal malignant tumor of the bone. The most common form is central chondrosarcoma and the rarest is intracortical chondrosarcoma. Here, we describe the clinical, pathological, and imaging features of a case of intracortical chondrosarcoma as well as the outcome of surgical treatment. This is the third case reported in the literature.

  3. Clear cell chondrosarcoma

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    Kumar, R.; David, R.; Cierney, G. III

    1985-01-01

    The clinical, radiologic, and histopathologic features of three cases of clear cell chondrosarcoma are described. On radiographs, this rather benign-appearing tumor resembles a chondroblastoma when it occurs at the end of a long bone, and may occasionally show a calcified matrix. However, it has distinctive tumor cells with a centrally placed vesicular nucleus surrounded by clear cytoplasm. The lesion has a low-grade malignancy and is amenable to en bloc surgical resection, which results in a much better prognosis than that of conventional chondrosarcoma.

  4. Chondrosarcoma of the Mobile Spine and Sacrum

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    Ryan M. Stuckey

    2011-01-01

    Full Text Available Chondrosarcoma is a rare malignant tumor of bone. This family of tumors can be primary malignant tumors or a secondary malignant transformation of an underlying benign cartilage tumor. Pain is often the initial presenting complaint when chondrosarcoma involves the spine. In the mobile spine, chondrosarcoma commonly presents within the vertebral body and shows a predilection for the thoracic spine. Due to the resistance of chondrosarcoma to both radiation and chemotherapy, treatment is focused on surgery. With en bloc excision of chondrosarcoma of the mobile spine and sacrum patients can have local recurrence rates as low as 20%.

  5. Primary myxoid chondrosarcoma of the breast.

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    Farahat, A; Magdy, N; Elaffandi, A

    2014-01-01

    Primary breast chondrosarcoma has been rarely reported in the literature. Conservative breast surgery has never been part of the management of previously reported cases. Surgery remains the mainstay management of such a disease as it is resistant to chemotherapy and radiotherapy. In this report, we present a case of rare primary myxoid chondrosarcoma of the breast that was managed successfully with a conservative approach.

  6. Intracranial extra-skeletal mesenchymal chondrosarcoma.

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    Reyaz, Nadeem; Ashraf, Muhammad

    2006-01-01

    Intracranial Mesenchymal Chondrosarcoma is a very rare and uncommon entity that affects young adults. We came across one such patient who presented with severe headache and intermittent nausea and vomiting. The clinical, radiological preoperative diagnosis was a meningioma, on histological examination it turned out to be mesenchymal chondrosarcoma of tentorial region in posterior fossa, uncommon site for this entity.

  7. Chondrosarcoma of the mandible. Case report.

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    George Rallis, Panagiotis Stathopoulos, ,Constantinos Mourouzis, Hussein Al Momani, Nicholas Zachariades

    2010-08-01

    Full Text Available Chondrosarcoma is a malignant tumour derived from cartilaginous cells. It accounts for 10-12% of all malignant bone tumours and it is the second more common bone tumour after osteosarcoma. However, it rarely occurs in the bones of the visceralcranium. The majority of head and neck chondrosarcomas occur in the upper or lower jaw, with a slightly higher incidence in the upper jaw. Especially, the temporomandibular joint (TMJ is a rare location. Case report: Α rare case of a large chondrosarcoma of the mandible, developed on an osteochondroma of the mandibular condyle is presented, and the most interesting and unusual pathology results are discussed. A 70-year-old woman presented with a hard painless swelling on the right side of her face, related to the mandibular condyle. A biopsy of the lesion revealed an osteochondroma. The patient refused to undergo the proposed surgical operation, but returned to our hospital after 18 months, with a large tumour mass resultingin facial asymmetry and difficulties in chewing and speaking. The tumour was surgically removed. Microscopically, it was a chondrosarcoma, grade II to III. In acertain part, foci of undifferentiated chondrosarcoma were also present. The undifferentiated component displayed histological features of a malignant fibrous histiocytoma developed on a large osteochondroma.

  8. Extraskeletal myxoid chondrosarcoma of the orbit

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    Abdullah M Al-Osaily

    2016-01-01

    Full Text Available Extraskeletal myxoid chondrosarcoma (EMC is a rare soft tissue tumor. Numerous cases of EMC have been reported in different anatomical locations. There is currently only a single case of EMC of the orbit and that was reported in 1985. We report a second case of orbital EMC in a 34-year-old healthy male.

  9. Chondrosarcoma of the larynx: a therapeutic challenge.

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    Shinhar, S; Zik, D; Issakov, J; Rappaport, Y

    2001-08-01

    The diagnosis of laryngeal chondrosarcoma is likely to be missed because of its infrequent occurrence and its indolent pattern of growth. A 53-year-old woman came to our service with an 18-year history of hoarseness and increasing dyspnea. She had been previously documented as having left vocal fold paralysis and a bulging laryngeal mass. Computed tomography revealed the presence of a large calcified tumor that had caused a deformity of the larynx and an erosion of the arytenoid and cricoid cartilages. Direct laryngoscopy detected a large supraglottic mass with a normal-appearing mucosa. Total excision of the tumor was achieved through a lateral neck incision that spared the larynx. This case emphasizes the importance of a high index of suspicion for laryngeal chondrosarcoma in a patient who has unexplained vocal fold paralysis and a submucosal subglottic mass. Every effort should be made to take a conservative surgical approach that preserves laryngeal function when possible.

  10. Functional roles of CSPG4/NG2 in chondrosarcoma.

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    Jamil, Nuor S M; Azfer, Asim; Worrell, Harrison; Salter, Donald M

    2016-04-01

    CSPG4/NG2 is a multifunctional transmembrane protein with limited distribution in adult tissues including articular cartilage. The purpose of this study was to investigate the possible roles of CSPG4/NG2 in chondrosarcomas and to establish whether this molecule may have potential for targeted therapy. Stable knock-down of CSPG4/NG2 in the JJ012 chondrosarcoma cell line by shRNA resulted in decreased cell proliferation and migration as well as a decrease in gene expression of the MMP (matrix metalloproteinase) 3 protease and ADAMTS4 (aggrecanase). Chondrosarcoma cells in which CSPG4/NG2 was knocked down were more sensitive to doxorubicin than wild-type cells. The results indicate that CSPG4/NG2 has roles in regulating chondrosarcoma cell function in relation to growth, spread and resistance to chemotherapy and that anti-CSPG4/NG2 therapies may have potential in the treatment of surgically unresectable chondrosarcoma.

  11. MRI features of extraskeletal myxoid chondrosarcoma

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    Tateishi, Ukihide; Arai, Yasuaki [National Cancer Center Hospital, Division of Diagnostic Radiology, Tokyo (Japan); Hasegawa, Tadashi [Sapporo Medical University School of Medicine, Department of Clinical Pathology, Sapporo (Japan); Nojima, Takayuki [Kanazawa Medical University, Department of Pathology, Ishikawa (Japan); Takegami, Tsutomu [Kanazawa Medical University, Medical Research Institute, Ishikawa (Japan)

    2006-01-01

    To describe the MRI features of extraskeletal myxoid chondrosarcoma in comparison with clinicopathologic findings. The study comprised 12 male subjects and seven female subjects with a mean age of 53 years (range 16-76 years). MRI findings, evaluated by two radiologists with agreement by consensus, were compared for histopathologic features. The tumor size ranged from 2.0 cm to 20.0 cm (mean 8.9 cm). Fusion gene transcripts could be detected in 13 (68%) of the 19 cases: EWS-CHN in nine cases, TAF2N-CHN in three, and TFG-TCH in one. There were six fusion-negative cases. Signal characteristics on T1-weighted and T2-weighted MR images were non-specific with regard to each cytogenetic variant. Peripheral enhancement was seen more frequently in tumors with the EWS-CHN variant than in those with other cytogenetic variants. The characteristic pattern of enhancement corresponded to the presence of fibrous septa and peripheral areas of high cellularity within lobules, by correlation with pathologic findings. All cases with TAF2N-CHN or TFG-TCH variants showed invasion of extracompartmental structure, bone, or vessels. Extraskeletal myxoid chondrosarcoma is an uncommon soft-tissue malignancy that may be recognized by MRI features of multi-lobular soft-tissue mass often invading extracompartmental, bony, and vascular structures. (orig.)

  12. Managing chondrosarcoma of the epiglottis: a case report.

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    Maughan, E; Pankhania, M; Shah, K; Gurr, P

    2012-11-01

    Laryngeal chondrosarcomas are a very rare malignancy with less than 150 cases reported in the literature. Of these, the epiglottis is the most unusual primary neoplastic subsite. Uncertainties arise owing to the extremely rare nature of the condition with regard to treatment and investigation for metastases in overtly low grade cases. We present the case of a 62-year-old woman with a low grade chondrosarcoma, arising from the tip of the epiglottis, presenting with dysphagia but no other symptoms.

  13. Intracranial meningeal chondrosarcoma--probable mesenchymal type.

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    Rodda, R A; Franklin, C I

    1984-08-01

    A 12 year old girl with episodes of left hemiparesis for 9 months was found to have a large, partly calcified brain tumour which at craniotomy presented on the parasagittal and medial surfaces of the right frontal lobe. No dural or falx attachment could be found and naked eye removal of the tumour was achieved. At a second craniotomy 10 weeks later there was recurrent tumour attached to the falx and involving the sagittal sinus. She died 5 months later. Pathologically, almost all this malignant intracranial neoplasm comprised differentiated cartilaginous tumour. Although only a very small amount of undifferentiated mesenchymal tissue was found in the surgical material available for histological study, it is suggested the tumour can be regarded as a predominantly mature mesenchymal chondrosarcoma of the meninges.

  14. Clear-cell chondrosarcoma of the maxilla Report of a case

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    Slootweg, P.J.

    1980-01-01

    Clear-cell chondrosarcoma is a variant of chondrosarcoma which is characterized by a typical histomorphology and a very slow rate of growth. A case is presented in which the tumor was located in the maxilla.

  15. Primary Extraskeletal Mesenchymal Chondrosarcoma Arising from the Pancreas

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    Oh, Bae Geun; Han, Yoon Hee; Lee, Byung Hoon; Kim, Su Young; Hwang, Yoon Joon; Seo, Jung Wook; Kim, Yong Hoon; Cha, Soon Joo; Hur, Gham; Joo, Mee [Inje University, School of Medicine, Goyang (Korea, Republic of)

    2007-12-15

    The CT scans showed a heterogeneously enhancing necrotic mass with numerous areas of coarse calcification, and this was located in the left side of the retroperitoneal space and involved the body and tail of the pancreas. Portal venography via the celiac axis also showed invasion of the splenic vein. It represents approximately 1% of all chondrosarcomas and it carries a poor prognosis. It can occur in extraskeletal locations and mainly in the soft tissues of the orbit, the cranial and spinal meningeal coverings and the lower limbs. To the best of our knowledge, there has been no reported case of primary extraskeletal mesenchymal chondrosarcoma of the pancreas. Only two instances of metastatic chondrosarcomas in the pancreas have been reported in the literature. We report here on a case of primary mesenchymal chondrosarcoma arising from the pancreas in a 41-year-old man. In summary, we present here a case of primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas. Radiologically, it manifested as a necrotic soft tissue mass with chondroid calcifications.

  16. Chondrosarcoma in a dromedary camel (Camelus dromedarius).

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    Janardhan, Kyathanahalli S; Ganta, Chanran K; Andrews, Gordon A; Anderson, David E

    2011-05-01

    A 4-year 10-month-old, intact female dromedary camel had progressive left carpal joint swelling and lameness for 7 months. Radiographs showed multifocal lytic lesions in the carpal and proximal metacarpal bones. Surgical biopsy of the synovial capsule and carpal bones suggested neoplasia, and the camel was subsequently euthanized. At necropsy, a white to pale pink, firm, multilobulated, soft tissue mass was located on the palmar aspect of the left carpal joint. Two smaller masses were present on the dorsal aspect of the carpal joint. The masses infiltrated all the carpal bones and the proximal region of the metacarpal bone. The joint capsule was diffusely thickened. The articular surfaces of the carpal bones and the metacarpal bone were multifocally eroded. The lungs contained multiple, firm, raised, gray, randomly distributed nodules. The neoplastic cells stained positive for vimentin and S-100. Chondrosarcoma arising from around the carpal joint with infiltration of carpal and metacarpal bones, and pulmonary metastasis, was diagnosed based on the histopathological and immunohistochemical evaluation.

  17. [Chondrosarcoma of the upper jaw. Apropos of a clinical case].

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    Smatt, V

    1977-01-01

    Initially it has been a tendency to place chondrosarcoma in the general category of osteogenic sarcoma. Jaffe et Lichtenstein claimed that the concept of chondrosarcoma as a separate entity has a firm clinical and histological basis. Chondrosarcomas may arise in peripheral long and flat bones. The maxillary location is very rare, it represents about 0,7% of the whole body. 40 well documented instances are yet counted in the literature. Reporting a personnal case, the author proceeds to a comparative study between the three varieties of maxillary sarcomas, evokes the special histogenesis in this location, he reminds the interest of bone electrocoagulation as curative treatment, and discusses the difficulty in making a prognosis 3 years after surgery without local recurrence or metastasis in this case.

  18. Extraskeletal myxoid chondrosarcoma: tumor response to sunitinib

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    Stacchiotti Silvia

    2012-10-01

    Full Text Available Abstract Background Extraskeletal myxoid chondrosarcoma (EMCS is a rare soft tissue sarcoma of uncertain differentiation, characterized in most cases by a translocation that results in the fusion protein EWSR1-CHN (the latter even called NR4A3 or TEC. EMCS is marked by >40% incidence of metastases in spite of its indolent behaviour. It is generally resistant to conventional chemotherapy, and, to the best of our knowledge, no data have been reported to date about the activity of tirosin-kinase inhibitor (TKI in this tumor. We report on two consecutive patients carrying an advanced EMCS treated with sunitinib. Methods Since July 2011, 2 patients with progressive pretreated metastatic EMCS (Patient1: woman, 58 years, PS1; Patient2: man, 63 years, PS1 have been treated with continuous SM 37.5 mg/day, on an individual use basis. Both patients are evaluable for response. In both cases diagnosis was confirmed by the presence of the typical EWSR1-CHN translocation. Results Both patients are still on treatment (11 and 8 months. Patient 1 got a RECIST response after 4 months from starting sunitinib, together with a complete response by PET. An interval progression was observed after stopping sunitinib for toxicity (abscess around previous femoral fixation, but response was restored after restarting sunitinib. Patient 2 had an initial tumor disease stabilization detected by CT scan at 3 months. Sunitinib was increased to 50 mg/day, with evidence of a dimensional response 3 months later. Conclusions Sunitinib showed antitumor activity in 2 patients with advanced EMCS. Further studies are needed to confirm these preliminary results.

  19. Curcumin blocks interleukin-1 signaling in chondrosarcoma cells.

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    Thomas Kalinski

    Full Text Available Interleukin (IL-1 signaling plays an important role in inflammatory processes, but also in malignant processes. The essential downstream event in IL-1 signaling is the activation of nuclear factor (NF-κB, which leads to the expression of several genes that are involved in cell proliferation, invasion, angiogenesis and metastasis, among them VEGF-A. As microenvironment-derived IL-1β is required for invasion and angiogenesis in malignant tumors, also in chondrosarcomas, we investigated IL-1β-induced signal transduction and VEGF-A expression in C3842 and SW1353 chondrosarcoma cells. We additionally performed in vitro angiogenesis assays and NF-κB-related gene expression analyses. Curcumin is a substance which inhibits IL-1 signaling very early by preventing the recruitment of IL-1 receptor associated kinase (IRAK to the IL-1 receptor. We demonstrate that IL-1 signaling and VEGF-A expression are blocked by Curcumin in chondrosarcoma cells. We further show that Curcumin blocks IL-1β-induced angiogenesis and NF-κB-related gene expression. We suppose that IL-1 blockade is an additional treatment option in chondrosarcoma, either by Curcumin, its derivatives or other IL-1 blocking agents.

  20. Novel Strategies for the Treatment of Chondrosarcomas: Targeting Integrins

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    Jui-Chieh Chen

    2013-01-01

    Full Text Available Chondrosarcomas are a heterogeneous group of malignant bone tumors that are characterized by the production of cartilaginous extracellular matrix. They are the second most frequently occurring type of bone malignancy. Surgical resection remains the primary mode of treatment for chondrosarcomas, since conventional chemotherapy and radiotherapy are largely ineffective. Treatment of patients with high-grade chondrosarcomas is particularly challenging, owing to the lack of effective adjuvant therapies. Integrins are cell surface adhesion molecules that regulate a variety of cellular functions. They have been implicated in the initiation, progression, and metastasis of solid tumors. Deregulation of integrin expression and/or signaling has been identified in many chondrosarcomas. Therefore, the development of new drugs that can selectively target regulators of integrin gene expression and ligand-integrin signaling might hold great promise for the treatment of these cancers. In this review, we provide an overview of the current understanding of how growth factors, chemokines/cytokines, and other inflammation-related molecules can control the expression of specific integrins to promote cell migration. We also review the roles of specific subtypes of integrins and their signaling mechanisms, and discuss how these might be involved in tumor growth and metastasis. Finally, novel therapeutic strategies for targeting these molecules will be discussed.

  1. Radical resection of giant chondrosarcoma of the anterior chest wall

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    Stanić Vojkan

    2008-01-01

    Full Text Available Background. Chondrosarcomas represent approximately 30% of primary malignant bone tumors, the most frequent of which is on anterior thoracic wall. Case report. We presented a case of 50-year-old man suffering from a slowgrowing, painless giant chondrosarcoma of the anterior chest wall. A wide resection was performed to excise the tumor including attached skin, right breast, ribs, sternum, soft tissues and parietal pleura. Mediastinum was not affected by the tumor. After resecting a 26 × 20 × 22 cm segment, the chest wall defect was reconstructed with a Marlex mesh and extensive latissimus dorsi myocutaneous flap pedicled on the right thoracodorsal vessels. Histopatology diagnosis was chondrosarcoma G 2−3. The mechanics of ventilation was not altered and respiratory function was normal from the immediate postoperative period. Three years after the operation postoperative results showed no local recurrence and excellent functional and aesthetic results were evident. Respiratory function remained unaltered. Conclusion. According to the results it can be concluded that the use of Marlex mash and myocutaneous flap is good method for stabilization of the chest wall and enough to avoid paradoxical respiratory movements in managing giant chondrosarcoma of the anterior chest wall.

  2. Pulmonary extraskeletal myxoid chondrosarcoma: A case report and literature review

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    Ricardo Balanzá, MD

    2016-01-01

    Conclusion: EMC is an intermediate-grade neoplasm, characterized by a long clinical course with high potential for local recurrence and distant metastasis. Treatment for EMC is surgical and non-surgical treatment is reserved for recurrence or metastatic disease. Pulmonary extraskeletal myxoid chondrosarcoma is a rare neoplasm with only isolated case reports found in the literature.

  3. Chondrosarcoma of the Mandibular Condyle: A Case Report

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    Keshani F.

    2012-12-01

    Full Text Available Chondrosarcomas are slow-growing, malignant mesenchymal neoplasms characterized by formation of cartilage by the tumoral cells. They display a wide range of morphological features from a well-differentiated growing mass resembling a benign cartilage tumour to a high-grade malignancy with aggressive local invasion. Only 5% to 10% of this neoplasm is confined to the head and neck region. Chondrosarcomas of the mandibular condyle may manifest the typical symptoms of the temporomandibular joint dysfunction syndrome. Tumours of the condyle can reach a large size without producing clinically obvious swellings. A rare case of chondrosarcoma of the mandibular condyle in a 34-years old woman is presented in this report. Patient’s chief complaint was pain in the right temporomandibular joint when her mouth was in a maximum opening position. Mild malocclusion, figured as an occlusal discrepancy, was also detected. Radiographs illustrated erosion in the head of condyle. After condylectomy, the excised mass was histologically diagnosed as a grade II chondrosarcoma.

  4. Clinical and radiologic features of extraskeletal myxoid chondrosarcoma including initial presentation, local recurrence, and metastases

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    Kapoor Neena

    2014-09-01

    Full Text Available Background. The aim of the study was to evaluate the clinical and imaging features of extraskeletal myxoid chondrosarcoma (EMC including initial presentation, recurrence, and metastases.

  5. MRI differentiation of low-grade from high-grade appendicular chondrosarcoma

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    Douis, Hassan; Singh, Leanne; Saifuddin, Asif [The Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, Stanmore, Middlesex (United Kingdom)

    2014-01-15

    To identify magnetic resonance imaging (MRI) features which differentiate low-grade chondral lesions (atypical cartilaginous tumours/grade 1 chondrosarcoma) from high-grade chondrosarcomas (grade 2, grade 3 and dedifferentiated chondrosarcoma) of the major long bones. We identified all patients treated for central atypical cartilaginous tumours and central chondrosarcoma of major long bones (humerus, femur, tibia) over a 13-year period. The MRI studies were assessed for the following features: bone marrow oedema, soft tissue oedema, bone expansion, cortical thickening, cortical destruction, active periostitis, soft tissue mass and tumour length. The MRI-features were compared with the histopathological tumour grading using univariate, multivariate logistic regression and receiver operating characteristic curve (ROC) analyses. One hundred and seventy-nine tumours were included in this retrospective study. There were 28 atypical cartilaginous tumours, 79 grade 1 chondrosarcomas, 36 grade 2 chondrosarcomas, 13 grade 3 chondrosarcomas and 23 dedifferentiated chondrosarcomas. Multivariate analysis demonstrated that bone expansion (P = 0.001), active periostitis (P = 0.001), soft tissue mass (P < 0.001) and tumour length (P < 0.001) were statistically significant differentiating factors between low-grade and high-grade chondral lesions with an area under the ROC curve of 0.956. On MRI, bone expansion, active periostitis, soft tissue mass and tumour length can reliably differentiate high-grade chondrosarcomas from low-grade chondral lesions of the major long bones. (orig.)

  6. The diagnosis and treatment of the laryngeal chondrosarcoma

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    Wenwei Zhang; Jie Qiu; Xinyu Zhang

    2012-01-01

    Chondrosarcoma (CS) of larynx is uncommon and predominantly affects the cricoid cartilage. A 70% to 75% of these tumors arise on the endolaryngeal surface of the posterior lamina of the cricoid cartilage. The clinical presentation include hoarseness, stridor, dyspnea, or a neck mass etc. The diagnosis is suggested when calcification is identified in a laryngeal tumor. Computed tomography (CT) is the best noninvasive technique for imaging the lesion. Surgical extirpation is the standard therapy with no role for radiation therapy or chemotherapy.

  7. Chondrosarcoma of the hand: is a wide surgical resection necessary?

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    Mittermayer, Florian; Dominkus, Martin; Krepler, Petra; Schwameis, Eva; Sluga, Maria; Toma, Cyril; Lang, Susanna; Grampp, Stephan; Kotz, Rainer

    2004-07-01

    Chondrosarcomas of the hand are rare and generally treated with surgical resection. Thirteen patients with Grade 1 chondrosarcoma of the small bones of the hand were followed up for a mean of 99.8 months (range, 26-293 months). In eight patients (Group 1) curettage and reconstruction with cancellous bone was done and in five patients (Group 2) a wide resection was done. No patient experienced relapse in Group 2. In Group 1 one patient had a local relapse 18 months after intralesional resection. Using the Musculoskeletal Tumor Society score for evaluation, the clinical results showed an average of 98% and 95% of the normal function in Groups 1 and 2, respectively. None of the patients had evidence of systemic spread of the disease. With a relapse rate of 12.5% and no distant metastases after curettage, intralesional resection is the preferred method of treatment in Grade 1 chondrosarcoma of the hand, allowing the patient to avoid amputation and major loss of function.

  8. Laryngeal chondrosarcoma diagnosed by core-needle biopsy.

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    Miyamaru, Satoru; Haba, Koichi

    2014-01-01

    We report a case of chondrosarcoma of the larynx, diagnosed by a percutaneous core-needle biopsy (CNB). Cartilaginous tumors of the larynx are usually diagnosed by biopsy with direct laryngomicroscopy under general anesthesia. However, patients find it difficult to undergo a biopsy under general anesthesia, for physical, economic, and social reasons. Instead, we can readily detect and sample tumors of the larynx using ultrasound under local anesthesia with reduced stress. Concerning needle-puncture biopsies, including fine-needle aspiration cytology (FNAC) and CNB, some studies have reported needle track dissemination, a possible complication in patients with malignant tumors. Thus, in the head and neck region, we generally use FNAC for biopsies, not CNB. However, it can be difficult to diagnose bone tumors by cytology alone. Regarding primary bone tumors, only one study has reported needle track dissemination by CNB, in osteosarcoma of the femur. Additionally, this complication has not been reported before with chondrosarcoma anywhere in the body. To our knowledge, this is the first report concerning chondrosarcoma of the larynx diagnosed by percutaneous CNB. We recommend CNB as a useful and safe diagnostic technique for primary bone tumors in the head and neck region.

  9. Laryngeal Chondrosarcoma as a Rare Cause of Subglottic Stenosis

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    Kerem Kökoğlu

    2014-01-01

    Full Text Available Laryngeal chondrosarcoma (CS is a very rare entity. It is usually seen in 50–80-year olds. It is developed from cricoid cartilage largely. Patients have laryngeal CS complaint of respiratuvar distress, dysphonia, and dysphagia generally. A submucous mass is usually seen in physical examination with an intact mucosa. Distant metastasis is rare in CSs. Main treatment is surgical excision. An 82-year-old patient who has respiratuvar distress is presented in this paper and laryngeal CS is reviewed in the light of the literature.

  10. Desmoid tumor of bone with enchondromatous nodules, mistaken for chondrosarcoma

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    Bahk, Won-Jong [Musculoskeletal Oncology Study Group, Catholic University of Korea (Korea); Department of Orthopaedic Surgery, Uijongbu St. Mary' s Hospital, 65-1 Geumohdong, Uijongbu, Gyunggido, 480-130 (Korea); Kang, Yong-Koo; Lee, An-Hee [Musculoskeletal Oncology Study Group, Catholic University of Korea (Korea); Mirra, Joseph M. [Orthpaedic Oncology, Orthopaedic Hospital, Los Angeles, CA (United States)

    2003-04-01

    Desmoid tumor of bone, also termed desmoplastic fibroma or aggressive fibromatosis, is a rare, locally aggressive fibroblastic tumor. We present a 16-year-old male with a huge desmoid tumor involving the iliac wing. It was associated with enchondromatous nodules mimicking malignancy. The tumor in this patient was mistaken for chondrosarcoma and hemipelvectomy was performed. To our knowledge, such a case has not previously been documented fully in the English literature. The radiographic and pathologic findings and a possible mechanism of enchondromatous nodule formation in fibrous bone tumors are discussed. (orig.)

  11. Trichodermin induces cell apoptosis through mitochondrial dysfunction and endoplasmic reticulum stress in human chondrosarcoma cells

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    Su, Chen-Ming [Graduate Institute of Basic Medical Science, China Medical University, Taichung, Taiwan (China); Wang, Shih-Wei [Department of Medicine, Mackay Medical College, New Taipei City, Taiwan (China); Lee, Tzong-Huei [Graduate Institute of Pharmacognosy, Taipei Medical University, Taipei, Taiwan (China); Tzeng, Wen-Pei [Graduate Institute of Sports and Health, National Changhua University of Education, Changhua, Taiwan (China); Hsiao, Che-Jen [School of Respiratory Therapy, College of Medicine, Taipei Medical University, Taipei, Taiwan (China); Liu, Shih-Chia [Department of Orthopaedics, Mackay Memorial Hospital, Taipei, Taiwan (China); Tang, Chih-Hsin, E-mail: chtang@mail.cmu.edu.tw [Graduate Institute of Basic Medical Science, China Medical University, Taichung, Taiwan (China); Department of Pharmacology, School of Medicine, China Medical University, Taichung, Taiwan (China); Department of Biotechnology, College of Health Science, Asia University, Taichung, Taiwan (China)

    2013-10-15

    Chondrosarcoma is the second most common primary bone tumor, and it responds poorly to both chemotherapy and radiation treatment. Nalanthamala psidii was described originally as Myxosporium in 1926. This is the first study to investigate the anti-tumor activity of trichodermin (trichothec-9-en-4-ol, 12,13-epoxy-, acetate), an endophytic fungal metabolite from N. psidii against human chondrosarcoma cells. We demonstrated that trichodermin induced cell apoptosis in human chondrosarcoma cell lines (JJ012 and SW1353 cells) instead of primary chondrocytes. In addition, trichodermin triggered endoplasmic reticulum (ER) stress protein levels of IRE1, p-PERK, GRP78, and GRP94, which were characterized by changes in cytosolic calcium levels. Furthermore, trichodermin induced the upregulation of Bax and Bid, the downregulation of Bcl-2, and the dysfunction of mitochondria, which released cytochrome c and activated caspase-3 in human chondrosarcoma. In addition, animal experiments illustrated reduced tumor volume, which led to an increased number of terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL)-positive cells and an increased level of cleaved PARP protein following trichodermin treatment. Together, this study demonstrates that trichodermin is a novel anti-tumor agent against human chondrosarcoma cells both in vitro and in vivo via mitochondrial dysfunction and ER stress. - Highlights: • Trichodermin induces chondrosarcoma apoptosis. • ER stress is involved in trichodermin-induced cell death. • Trichodermin induces chondrosarcoma death in vivo.

  12. Chondrosarcoma of the Proximal Phalanx of the Fourth Digit: A Rare Location

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    Thivi Vasilakaki

    2012-10-01

    Full Text Available Introduction: Cartilaginous tumors involving the small bones of the hands and feet are usually benign such as enchondroma, chondromyxoid fibroma and chondroblastoma. The small bones of the hands and feet are rarely involved by primary chondrosarcoma. Proximal phalanges are the most common sites in the hands, but the fourth digit is the least common site. Case Presentation: We report a case of a 76-year-old Greek female who presented to our hospital with a painful swollen mass measuring 4.5 × 2.6 cm on the fourth digit of the left hand. The radiograph showed a destructive, permeative lytic tumor of the proximal phalanx with extension into soft tissue. The patient underwent curettage, and the microscopic examination of the specimen revealed grade 2 chondrosarcoma. Conclusion: Cartilaginous tumors involving the small bones of the hands and feet are usually benign such as enchondroma, chondromyxoid fibroma and chondroblastoma. Primary chondrosarcoma is the third most common malignancy of bone after myeloma and osteosarcoma, but the small bones of the hands and feet are very rarely involved by chondrosarcoma (1% of all chondrosarcoma. However, in these cases differentiation between a benign lesion and chondrosarcoma may be difficult. Occasionally chondrosarcoma of the hands and feet is associated with multiple recurrences or distal metastasis.

  13. Is bone scintigraphy necessary in the initial surgical staging of chondrosarcoma of bone?

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    Douis, Hassan; James, Steven L.; Davies, Mark A. [Royal Orthopaedic Hospital, Department of Radiology, Birmingham (United Kingdom); Grimer, Robert J. [Royal Orthopaedic Hospital, Department of Orthopaedic Oncology, Birmingham (United Kingdom)

    2012-04-15

    To assess the value of whole-body bone scintigraphy in the initial surgical staging of chondrosarcoma of bone. A retrospective review was conducted of the bone scintigraphy reports of a large series of patients with peripheral or central chondrosarcoma of bone treated in a specialist orthopaedic oncology unit over a 13-year period. Abnormal findings were correlated against other imaging, histological grade and the impact on surgical staging. A total of 195 chondrosarcomas were identified in 188 patients. In 120 (63.8%) patients the reports of bone scintigraphy noted increased activity at the site of one or more chondrosarcomas. In one patient the tumour was outside the field-of-view of the scan, and in the remaining 67 (35.6%) cases, there was increased activity at the site of the chondrosarcoma and further abnormal activity in other areas of the skeleton. Causes of these additional areas of activity included degenerative joint disease, Paget's disease and in one case a previously undiagnosed melanoma metastasis. No cases of skeletal metastases from the chondrosarcoma were found in this series. Multifocal chondrosarcomas were identified in three cases. In two it was considered that all the tumours would have been adequately revealed on the initial MR imaging staging studies. In only the third multifocal case was an unsuspected, further presumed low-grade, central chondrosarcoma identified in the opposite asymptomatic femur. Although this case revealed an unexpected finding the impact on surgical staging was limited as it was decided to employ a watch-and-wait policy for this tumour. There is little role for the routine use of whole-body bone scintigraphy in the initial surgical staging in patients with chondrosarcoma of bone irrespective of the histological grade. (orig.)

  14. Spinal meningeal mesenchymal chondrosarcoma. Report of a new case and review of the literature.

    Science.gov (United States)

    Platania, N; Nicoletti, G; Lanzafame, S; Albanese, V

    2003-06-01

    A case of T12-L2 intraspinal extradural meningeal mesenchymal chondrosarcoma is described in a patient presenting with a clinical history of low-back pain and weakness of the lower limbs. Magnetic resonance T1-weighted Gadolinium enhanced imaging, showed an intraspinal extradural mass, extending from T12 to L2, located anterolaterally into the spinal canal and pushing posteriorly the conus medullaris. The mass was completely removed and postoperative histological diagnosis was of mesenchymal chondrosarcoma. The patient was completely symptoms-free after surgery. Intraspinal meningeal mesenchymal chondrosarcoma and treatment options are reviewed.

  15. Mesenchymal chondrosarcoma of maxilla: a rare case report.

    Science.gov (United States)

    Jaetli, Vipul; Gupta, Sunita

    2011-07-01

    Mesenchymal chondrosarcoma (MC) is a rare variant of chondrosarcoma (CS) that accounts for upto 3-9% of all CS and has high predilection for the head and neck region. It is usually seen in younger age group compared to conventional CS and maxillary anterior alveolus is the most common site. The tumor is most unusual as it has been described as a particularly aggressive neoplasm with a high tendency for late recurrence and delayed metastasis. It is a biphasic tumor with areas comprising of spindle cell mesenchyme interspread with areas of chondroid differentiation. A 75 year old male presented to us as a painless mass in maxilla. Contrast enhanced computed tomography (CECT) revealed a lytic expansile lesion in the left maxillary bone with foci of calcification within soft tissue lesion. Fine needle aspiration cytology (FNAC) and incisional biopsy was performed which confirmed the diagnosis of maxillary MC. The patient underwent right and left subtotal maxillectomy with 2 cm margins. The review of literature shows that very few cases of maxillary MC have been reported so far. Thus an attempt is made to add this rare case of MC of maxillary alveolus in the English literature.

  16. Managing Epiglottal Chondrosarcoma of a Dog: A case report

    Directory of Open Access Journals (Sweden)

    Ahmed M Shoieb

    2014-08-01

    Full Text Available A primary chondrosarcoma was found in the epiglottis of a 6-year-old, neutered, male Boxer cross-breed dog. Clinically, there was upper respiratory noise, and a 3.2 cm and times; 2.8 cm and times; 2.7 cm, ovoid mass involving the epiglottis was observed. No abnormalities were detected upon radiographic examinations (X-ray of the chest or abdomen. Grossly, the excised mass was hard. On cut section, it displayed a lobular pattern of translucent gray-white color (interpreted as cartilage. Histologically, the epiglottal submucosa contained a non-encapsulated, fairly demarcated multi-nodular neoplasm composed of streams of cells admixed with moderate to abundant amounts of a pale amphophilic to eosinophilic matrix (chondroid. The neoplastic cells stained were immunohistochemically positive for vimentin and S-100 protein, and negative for pancytokeratin. The matrix stained deeply with Alcian Blue (pH 2.5-periodic acid Schiff, which often separated cells into individual lacunae. To the best of our knowledge, this is the first report of extra-skeletal chondrosarcoma primarily arising in the epiglottis of a dog. [J Interdiscipl Histopathol 2014; 2(4.000: 224-227

  17. Chondroblastoma and clear cell chondrosarcoma: radiological and MRI characteristics with histopathological correlation

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    Kaim, Achim H.; Huegli, Rolf [Institute of Diagnostic Radiology, University Hospital Basle (Switzerland); Bonel, Harald M. [Institute of Clinical Radiology, University Hospital, Munich-Grosshadern (Germany); Jundt, Gernot [Institute of Pathology, University Hospital Basle (Switzerland)

    2002-02-01

    Objective: To analyze and compare the radiological and magnetic resonance imaging (MRI) appearances of chondroblastoma and clear cell chondrosarcoma with histopathological correlation. Design and patients: Twelve patients with histologically proven chondroblastoma and of another four patients with clear cell chondrosarcoma were investigated by radiographs and MRI (T1-, T2-weighted sequences, intravenous gadolinium application). Additionally, the clinical and radiologic data of seven cases of clear cell chondrosarcoma without available MRI were considered. The localization, calcification of tumor matrix, periosteal reaction, cortical bone and patterns of bone destruction were analyzed according to the Lodwick radiological grading system (LRGS). The signal intensity on T1- and T2-weighted sequences, characteristics of contrast enhancement, associated bone marrow edema, soft tissue reaction and joint involvement were evaluated. Histopathological specimens were available in all cases. Results: The age of patients with chondroblastoma (range 15-59 years, mean 22.3 years) was lower than that of those with clear cell chondrosarcoma (range 19-61 years, mean 36.6 years), and the lesions were smaller in the chondroblastoma group (range 1-4 cm, mean 2.3 cm) than in patients with clear cell chondrosarcoma (range 3-7.5 cm, mean 5.2 cm). The chondroblastomas were more confined to the epiphysis (10/12) than the clear cell chondrosarcomas. All chondroblastomas and clear cell chondrosarcomas except one were classified as grade 1A or 1B according to the LRGS; one clear cell chondrosarcoma was judged as grade 2. Signal intensity of the tumors on MRI was very heterogeneous in both groups. High signal intensity on T2-weighted MR images in chondroblastoma mostly corresponded to areas with aneurysmal bone cyst components and in clear cell chondrosarcoma to islands of hyaline cartilage. Contrast enhancement occurred in all tumors and tended to be more intense with clear cell

  18. Microenvironment alters epigenetic and gene expression profiles in Swarm rat chondrosarcoma tumors

    Directory of Open Access Journals (Sweden)

    Hamm Christopher A

    2010-09-01

    Full Text Available Abstract Background Chondrosarcomas are malignant cartilage tumors that do not respond to traditional chemotherapy or radiation. The 5-year survival rate of histologic grade III chondrosarcoma is less than 30%. An animal model of chondrosarcoma has been established - namely, the Swarm Rat Chondrosarcoma (SRC - and shown to resemble the human disease. Previous studies with this model revealed that tumor microenvironment could significantly influence chondrosarcoma malignancy. Methods To examine the effect of the microenvironment, SRC tumors were initiated at different transplantation sites. Pyrosequencing assays were utilized to assess the DNA methylation of the tumors, and SAGE libraries were constructed and sequenced to determine the gene expression profiles of the tumors. Based on the gene expression analysis, subsequent functional assays were designed to determine the relevancy of the specific genes in the development and progression of the SRC. Results The site of transplantation had a significant impact on the epigenetic and gene expression profiles of SRC tumors. Our analyses revealed that SRC tumors were hypomethylated compared to control tissue, and that tumors at each transplantation site had a unique expression profile. Subsequent functional analysis of differentially expressed genes, albeit preliminary, provided some insight into the role that thymosin-β4, c-fos, and CTGF may play in chondrosarcoma development and progression. Conclusion This report describes the first global molecular characterization of the SRC model, and it demonstrates that the tumor microenvironment can induce epigenetic alterations and changes in gene expression in the SRC tumors. We documented changes in gene expression that accompany changes in tumor phenotype, and these gene expression changes provide insight into the pathways that may play a role in the development and progression of chondrosarcoma. Furthermore, specific functional analysis indicates that

  19. MR features of multiple enchondromas with associated chondrosarcoma in the lower extremities

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    Yang, Kyung Yoon; Sung, Mi Sook; Lee, Hee Jeong; Park, Il Joong; Lee, Jae Young; Yu, Won Jong; Lee, Gook Jin; Jeon, Sang Hoon; Yu, Mi Na; Yoon, Se Cheol [College of Medicine, The Catholic University of Korea, Bucheon St. Mary' s Hospital, Bucheon (Korea, Republic of)

    2016-02-15

    Multiple enchondromas are well described in the literature, however, the associated spectrum of MR imaging findings remains unclear. Secondary chondrosarcoma of the hand and feet associated with multiple enchondromas is extremely rare. Herein, we reported a case of multiple enchondromas of intramedullary, intracortical, and periosteal location with associated low-grade chondrosarcomas in the lower extremities on MR imaging in a 57 year old woman.

  20. Enchondroma vs. chondrosarcoma: A simple, easy-to-use, new magnetic resonance sign

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    Vanel, Daniel, E-mail: daniel.vanel@ior.it [Department of Research, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Kreshak, Jennifer [Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Larousserie, Frédérique [Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Université Paris Descartes, Sorbonne Paris Cité, Paris (France); Alberghini, Marco; Mirra, Joe [Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); De Paolis, Massimiliano [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Picci, Piero [Department of Research, Istituto Ortopedico Rizzoli, Bologna (Italy)

    2013-12-01

    Introduction: There is no clear radiologic or pathologic agreement on the differences between enchondroma and conventional chondrosarcoma, which has huge therapeutic consequences. Microscopically, an enchondroma is composed of “islands of intramedullary hyaline cartilage surrounded by marrow fat”, and a chondrosarcoma a “diffuse cartilaginous replacement (invasion) of the marrow which leads to complete ‘trapping’ of host lamellar bone trabeculae.” The marrow around islands of cartilage should be detectable on magnetic resonance imaging (MR). Enchondroma may be the precursor of chondrosarcoma; benign cartilaginous islands are often seen microscopically at the periphery of chondrosarcoma. We attempted to detect these islands at the periphery of chondrosarcomas on MR and correlate them microscopically. Materials and methods: We examined our database for all patients with a chondrosarcoma of the long and flat bones between 1990 and 2007. Only those with a preoperative MR who underwent an en bloc resection were included, yielding 32 patients. We looked for low-signal islands surrounded by high (fat) signal on T1-weighted images, and high-signal islands surrounded by low signal on T2-weighted fat saturated images at the periphery of the main tumour mass. Microscopic correlation was performed in all cases. Results: On microscopy, there were 23 conventional chondrosarcomas, nine dedifferentiated. Peripheral islands surrounded by fat were detected on MR in 19 cases, corresponding to benign cartilage in 18 cases and to the benign scar of a needle biopsy tract in one. There were no peripheral islands detected radiographically or microscopically in 13 cases. Conclusion: Cartilaginous islands microscopically detected at the periphery of some chondrosarcomas are easily and reliably diagnosed on MR.

  1. Clonal evolution through loss of chromosomes and subsequent polyploidization in chondrosarcoma.

    Directory of Open Access Journals (Sweden)

    Linda Olsson

    Full Text Available Near-haploid chromosome numbers have been found in less than 1% of cytogenetically reported tumors, but seem to be more common in certain neoplasms including the malignant cartilage-producing tumor chondrosarcoma. By a literature survey of published karyotypes from chondrosarcomas we could confirm that loss of chromosomes resulting in hyperhaploid-hypodiploid cells is common and that these cells may polyploidize. Sixteen chondrosarcomas were investigated by single nucleotide polymorphism (SNP array and the majority displayed SNP patterns indicative of a hyperhaploid-hypodiploid origin, with or without subsequent polyploidization. Except for chromosomes 5, 7, 19, 20 and 21, autosomal loss of heterozygosity was commonly found, resulting from chromosome loss and subsequent duplication of monosomic chromosomes giving rise to uniparental disomy. Additional gains, losses and rearrangements of genetic material, and even repeated rounds of polyploidization, may affect chondrosarcoma cells resulting in highly complex karyotypes. Loss of chromosomes and subsequent polyploidization was not restricted to a particular chondrosarcoma subtype and, although commonly found in chondrosarcoma, binucleated cells did not seem to be involved in these events.

  2. Chondrosarcoma of the hand secondary to multiple enchondromatosis; report of two cases.

    Science.gov (United States)

    Goto, Takahiro; Motoi, Toru; Komiya, Keita; Motoi, Noriko; Okuma, Tomotake; Okazaki, Hiroshi; Takatori, Yoshio; Tange, Tsuyoshi; Nakamura, Kozo

    2003-02-01

    Although malignant transformation to chondrosarcoma may occur in some patients with multiple enchondromatosis, this event rarely occurs in the hand. We encountered two patients with chondrosarcoma of the hand secondary to multiple enchondromatosis. One patient was a 27-year-old man and the other, a 76-year-old man. Both patients manifested multiple osteolytic lesions in the hand on the plain radiographs. Severe bone destruction associated with a large soft-tissue swelling of the proximal and middle phalanges of the little finger was seen in case 1. In case 2, tremendous expansion and bone destruction of the middle phalanx of the ring finger was seen. Magnetic resonance images of the tumour in both patients showed low signal intensity on T1-weighted and high signal intensity on T2-weighted images. Amputation was performed in each patient. Histological examination revealed that the tumour was a grade 2 chondrosarcoma in case 1 and a grade 1 chondrosarcoma in case 2 accompanied by enchondromata. From these findings, the diagnosis of chondrosarcoma secondary to multiple enchondromatosis was made. Because quite a few patients with multiple enchondromatosis develop secondary chondrosarcoma, although rarely in the hand, the enchondromata should be curetted, unless impractical, before malignant transformation occurs.

  3. ADAMTS1 is regulated by interleukin-1beta, not by hypoxia, in chondrosarcoma.

    Science.gov (United States)

    Kalinski, Thomas; Krueger, Sabine; Sel, Saadettin; Werner, Kerstin; Röpke, Martin; Roessner, Albert

    2007-01-01

    Angiogenesis is characteristic of cartilage tumors, not of normal cartilage tissue. In addition to our previous report on differential expression of proangiogenic vascular endothelial growth factor A (VEGF-A) in cartilage tumors, we analyzed the expression of a disintegrin and metalloproteinase with thrombospondin motifs 1 (ADAMTS1), which has been identified as a potent inhibitor of VEGF-A. We further used a chondrosarcoma cell line to study the effect of interleukin (IL)-1beta and hypoxia on the regulation of ADAMTS1 and VEGF-A expression. ADAMTS1 was detected by reverse transcriptase-polymerase chain reaction and immunohistochemistry in all analyzed samples from enchondromas, conventional chondrosacromas, and dedifferentiated chondrosarcomas without exception. In contrast to previous reports on other cancers, we did not detect a consistent decrease in ADAMTS1 expression in chondrosarcomas. Interleukin-1beta stimulation, not hypoxia, transcriptionally downregulated ADAMTS1 in chondrosarcoma cells, whereas VEGF-A expression was upregulated either by hypoxia or IL-1beta. We conclude that ADAMTS1 and VEGF-A in chondrosarcoma cells are regulated independently from each other. We believe that IL-1beta has a stronger impact on vascularization in chondrosarcomas than hypoxia, as both factors, ADAMTS1 and VEGF-A, are regulated in a way that favors angiogenesis.

  4. Clear cell chondrosarcoma mimicking chondroblastoma in a skeletally immature patient

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    Cannon, Christopher P. [Department of Orthopaedic Surgery, Madigan Army Medical Center, Ft. Lewis, WA (United States); Nelson, Scott D. [Department of Pathology and Laboratory Medicine, University of California, Los Angeles School of Medicine, CA (United States); Seeger, Leanne L. [Department of Radiological Sciences, University of California, CA (United States); Eckardt, Jeffrey J. [Department of Orthopaedic Surgery, University of California, Los Angeles School of Medicine, CA (United States)

    2002-06-01

    We report the case of a clear cell chondrosarcoma (CCCS) occurring in the femoral head of a 14-year-old skeletally immature boy. Radiographic examination revealed a well-defined, osteolytic lesion in the epiphysis of the femoral head. Given the patient's age and the radiographic appearance of the lesion, chondroblastoma was high on the differential diagnosis. A frozen section was performed at the time of open biopsy was felt to be consistent with either chondroblastoma or CCCS. CCCS in a skeletally immature patient was felt to be unlikely, so curettage and bone grafting was performed. Final pathology review, however, confirmed the diagnosis of CCCS. The patient was taken back to surgery 4 weeks later for a wide resection and hemiarthroplasty. (orig.)

  5. The expression of SIRT1 regulates the metastaticplasticity of chondrosarcoma cells by inducing epithelial-mesenchymal transition

    Science.gov (United States)

    Feng, Helin; Wang, Jin; Xu, Jianfa; Xie, Congcong; Gao, Fulu; Li, Zhiyong

    2017-01-01

    SIRT1 belongs to the mammalian sirtuin family and plays an important role in deacetylating histone and nonhistone proteins. It is reported that SIRT1 is associated with tumor metastasis in several kinds of tumors. However, the effect of SIRT1 on the metastasis of chondrosarcoma cells is still unknown. In this study, we demonstrated that up and down-regulation of SIRT1 expression could significantly change the invasive and metastatic potential in chondrosarcoma cell line. Besides that, the result from the nude mice confirmed the effect of SIRT1 on metastasis of chondrosarcoma cells. Furthermore, we also found that SIRT1 effectively enhanced the metastasis by inducing epithelial-mesenchymal transition (EMT) in chondrosarcoma cells. Inhibition the expression of SIRT1 could block the incidence of metastasis and EMT in chondrosarcoma cells. In addition, we also observed that SIRT1 could enhance the expression of Twist which is a key transcriptional factor of EMT. A clinicopathological analysis showed that SIRT1 expression was significantly correlated with the poor prognosis of pelvis chondrosarcoma. Kaplan-Meier survival curves revealed that positive SIRT1 expression was associated with poor prognosis in patients with pelvis chondrosarcoma. Taken together, these results indicate that SIRT1 may promote the metastasis of chondrosarcoma by inducing EMT and can be a potential molecular target for chondrosarcoma therapy. PMID:28112277

  6. Temporo-mandibular joint chondrosarcoma: Case report and review of the literature.

    Science.gov (United States)

    Giorgione, C; Passali, F M; Varakliotis, T; Sibilia, M; Ottaviani, F

    2015-06-01

    Chondrosarcoma is a malignant mesenchymal tumour of cartilaginous origin. It represents 11% of all malignant primary bone tumours, and the pelvis, ribs, femur and humerus are most frequently involved. Chondrosarcoma of the head and neck region is a rare disease, and represents approximately 0.1% of all head and neck neoplasms. This report describes a rare localisation of chondrosarcoma in a 56-year-old man who presented with swelling in the right preauricular area and mild limitation and pain in the mouth opening. Since 1959, just a few cases of temporomandibular joint (TMJ) chondrosarcoma have been described. Computed tomography revealed a large mass (39 x 46 x 40 mm) in the right preauricular and parotid region with morpho-structural alterations of the condyle and an intense periostotic reaction. The tumour was treated by total parotidectomy and condylotomy. The VII cranial nerve was preserved. Histopathologic examination revealed a low grade chondrosarcoma with a 50% proliferation index. At present, the patient is still receiving routine follow-up after radiotherapy and physiotherapy.

  7. Primary Spinal Chondrosarcoma: Radiologic Findings with Pathologic Correlation

    Energy Technology Data Exchange (ETDEWEB)

    Lloret, I.; Server, A. [The Norwegian Radium Hospital, Oslo (Norway). Depts. of Radiology and Pathology; Bjerkehagen, B. [Ullevaal Univ. Hospital, Oslo (Norway). Dept. of Neuroradiology

    2006-02-15

    Purpose: To describe the radiologic appearance of the four types of primary spinal chondrosarcoma (CHS) (conventional intramedullary, juxtacortical, clear cell, and mesenchymal) and to correlate with histopathologic findings. Material and Methods: A retrospective review was carried out of 5 patients with histopathologically confirmed primary spinal CHS; 3 F and 2 M ranging in age between 27 and 66 years (mean 40.2; median 39). Charts, conventional radiographs, computed tomography scans, and magnetic resonance images were reviewed. All the patients underwent surgical excision, followed by postoperative chemotherapy (1 patient) and radiotherapy (3 patients). Follow-up was available for all patients but one. The mean follow-up was 42 months (14-120 months). Histopathological specimens for all patients were available for review. Results: Vertebral column distribution was 3 thoracic (60%), 1 cervical (20%), and 1 lumbar (20%). Neurological deficits were present in 3 (60%) cases. The radiological appearance of the four types of primary spinal CHS varies with specific lesion type. Imaging findings suggest diagnosis of the conventional intramedullary and juxtacortical types. While the clear cell and mesenchymal types show some distinctive features, these do not allow confident radiologic diagnosis. Conclusion: The radiologist must be aware of imaging features of these tumors in order to improve diagnostic accuracy, treatment planning, and prognosis.

  8. Extraskeletal myxoid chondrosarcoma metastatic to the pancreas: A case report

    Institute of Scientific and Technical Information of China (English)

    C Fotiadis; A Charalambopoulos; S Chatzikokolis; GC Zografos; M Genetzakis; R Tringidou

    2005-01-01

    Extraskeletal myxoid chondrosarcoma (EMC) is a lowgrade sarcoma characterized by developing metastases and local recurrence in high rate. It is mainly deep seated in the proximal extremities. The most common metastatic sites are the lungs, soft tissues, lymph nodes, bones and the brain. To our knowledge, no case of clearly defined EMC has been reported to date developing a metastasis in the pancreas. We describe a case of a man suffering from EMC who developed a single pancreatic metastasis 20 years after the initial diagnosis. A 49-year-old man was submitted to surgical excision of an EMC, in left thigh, 20 years ago. Fourteen years after the initial diagnosis a local recurrence in left thigh occurred. Multiple lesions of metastatic origin, in both lungs, were excised via thoracotomies until the time being. In 2003, as a part of a periodically performed imaging control, an abdominal CT scan was performed revealing a solid lesion in the pancreas. Distal pancreatectomy was performed. The histopathology of the excised specimen proved to be the one of metastatic lesion of EMC. The above-mentioned case of EMC is, as far as we know, the first one described developing a certain pancreatic metastasis.

  9. Dedifferentiated Chondrosarcoma of Proximal Tibia and Fibula with an Infected Ulcer: A Case Report

    Science.gov (United States)

    Pattanashetty, O.B.; B.B, Dayanand; Pillai, Arravind; Endigeri, Preetish

    2016-01-01

    Introduction: Dedifferentiated chondrosarcoma (DDCS) is a rare and malignant form of primary bone tumor refractive to chemotherapy and radiotherapy. It accounts for 1-2% of all primary bone tumors. Surgical resection, limb-salvage surgeries and amputation remain the mainstay of treatment. The prognosis of dedifferentiated chondrosarcoma is poor. Case Report: We report a case of a 51-year-old male having a swelling and an ulcer distal to the left knee later diagnosed with dedifferentiated chondrosarcoma of proximal tibial and fibular metaphysis. Although DDCS is a highly malignant tumor, no metastasis was found and the patient was treated with transfemoral amputation. Post-operative histopathology showed nuclear atypia, binucleated and multinucleated chondrocytes with spindling and neoplastic cells arranged in storiform pattern and giant cells which confirmed the diagnosis of DDCS. Conclusion: Early diagnoses and treatment are important while treating these tumors in order to prevent morbidity and mortality.

  10. A Rare and Interesting Case of a Massive Secondary Spinal Chondrosarcoma and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Anthony Adimonye

    2015-01-01

    Full Text Available Chondrosarcoma, the second most common primary malignancy of the bone, is malignant cartilage forming tumour that very rarely involves the axial skeleton. It may arise as a primary bone tumour or as a secondary lesion from a preexisting benign cartilaginous neoplasm such as osteochondroma or enchondroma. A rare case of a massive secondary lumbar spine chondrosarcoma is presented. Management consisted of an initial posterior spinal stabilization and fusion and then a curative radical en bloc tumour resection. A review of the literature is also presented.

  11. Chondromyxoid fibroma of the rib mimics a chondrosarcoma on 18F-FDG PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Makis, William (Dept. of Nuclear Medicine, Brandon Regional Health Centre, Brandon (Canada)), email: makisw79@yahoo.com; Ciarallo, Anthony; Lisbona, Robert (Dept. of Nuclear Medicine, Royal Victoria Hospital, McGill Univ. Health Centre, Montreal (Canada))

    2011-06-15

    Chondromyxoid fibroma (CMF) is a rare benign bone tumor of chondroid origin that occurs mostly in the metaphyses of long bones. CMF can occasionally mimic a chondrosarcoma on CT, and the literature on the 18F-FDG PET/CT imaging characteristics of CMF tumors is limited. In the presented case, a large histologically proven CMF chest wall mass was initially misinterpreted as a chondrosarcoma. This case highlights a potential pitfall in the PET/CT evaluation of these rare benign bone tumors

  12. Titanium internal fixation system used for sternum reconstruction after resection of chondrosarcoma

    Institute of Scientific and Technical Information of China (English)

    LIU Zheng-cheng; ZHAO Heng

    2010-01-01

    @@ Chondrosarcoma is the most common malignant primary ches t wall tumor, only 20% of these cases involve sternum.1 Current therapy for chondrosarcoma requires adequate surgical excision and radiation therapy,2chemotherapy have not been yet proved to be effective.However, reconstruction of the defect is difficult, the problems of providing soft tissue coverage additionally complicating the surgery and even potentially limit the ability to achieve clear margin. Titanium internal fixation system can provide proper chest stability, ensure wide surgical margin and reduce hurts.

  13. Brain-derived neurotrophic factor increases vascular endothelial growth factor expression and enhances angiogenesis in human chondrosarcoma cells.

    Science.gov (United States)

    Lin, Chih-Yang; Hung, Shih-Ya; Chen, Hsien-Te; Tsou, Hsi-Kai; Fong, Yi-Chin; Wang, Shih-Wei; Tang, Chih-Hsin

    2014-10-15

    Chondrosarcomas are a type of primary malignant bone cancer, with a potent capacity for local invasion and distant metastasis. Brain-derived neurotrophic factor (BDNF) is commonly upregulated during neurogenesis. The aim of the present study was to examine the mechanism involved in BDNF-mediated vascular endothelial growth factor (VEGF) expression and angiogenesis in human chondrosarcoma cells. Here, we knocked down BDNF expression in chondrosarcoma cells and assessed their capacity to control VEGF expression and angiogenesis in vitro and in vivo. We found knockdown of BDNF decreased VEGF expression and abolished chondrosarcoma conditional medium-mediated angiogenesis in vitro as well as angiogenesis effects in vivo in the chick chorioallantoic membrane and Matrigel plug nude mouse models. In addition, in the xenograft tumor angiogenesis model, the knockdown of BDNF significantly reduced tumor growth and tumor-associated angiogenesis. BDNF increased VEGF expression and angiogenesis through the TrkB receptor, PLCγ, PKCα, and the HIF-1α signaling pathway. Finally, we analyzed samples from chondrosarcoma patients by immunohistochemical staining. The expression of BDNF and VEGF protein in 56 chondrosarcoma patients was significantly higher than in normal cartilage. In addition, the high level of BDNF expression correlated strongly with VEGF expression and tumor stage. Taken together, our results indicate that BDNF increases VEGF expression and enhances angiogenesis through a signal transduction pathway that involves the TrkB receptor, PLCγ, PKCα, and the HIF-1α. Therefore, BDNF may represent a novel target for anti-angiogenic therapy for human chondrosarcoma.

  14. Osteosarcoma and chondrosarcoma: radiographic differentiation by means of computed tomography; Osteossarcoma e condrossarcoma - diferenciacao radiografica por meio da tomografia computadorizada

    Energy Technology Data Exchange (ETDEWEB)

    Tossato, Patricia dos Santos; Pereira, Amanda Caceres; Cavalcanti, Marcelo Gusmao Paraiso [Sao Paulo Univ., SP (Brazil). Faculdade de Odontologia. Dept. de Estomatologia

    2002-03-01

    The purpose of this paper was to associate and compare the radiographic patterns of osteosarcoma and those of chondrosarcoma by means of computed tomography (CT). Computed tomographs of five cases of osteosarcoma and five cases of chondrosarcoma of the head and neck region were analyzed. The sensitivity of CT was calculated based on the results of histopathological examinations. We concluded that CT can facilitate the establishment of the final diagnosis since it is possible to distinguish the individual characteristics of osteosarcomas and chondrosarcomas in that exam. (author)

  15. Clinical outcome for patients with dedifferentiated chondrosarcoma: a report of 9 cases at a single institute

    Directory of Open Access Journals (Sweden)

    Yokota Kazuya

    2012-12-01

    Full Text Available Abstract Background Dedifferentiated chondrosarcomas consist of two distinguishable components: low-grade chondrosarcoma components and high-grade dedifferentiated components. Materials and methods Nine cases (4 males, 5 females of dedifferentiated chondrosarcoma were treated in our institute. The average age was 58.6 (range, 37–86 years. The tumor location was the long bone in 7 cases (femur, n=5; humerus, n=1; tibia, n=1 and the pelvic bone in 2 cases. The average time from appearance of symptoms to treatment was 9.4 (range, 1–40 months. Results and discussion On plain radiographs, matrix mineralization was seen in all 9 cases (100%. Bone destruction was observed in 5 of 9 cases (56%, while pathological fracture was seen in one femur case (11%. Lung metastasis was observed in all cases (initially in 5 cases; during the treatment course in 4 cases. Surgery was performed in 8 cases, with local recurrence occurring in 2 of those cases (time to recurrence, 2 and 10 months. Chemotherapy was administered in 4 cases, but did not result in significant improvement. All 9 cases died of lung metastases, with a median survival time of 10 (range, 3.4-18.8 months. The presence of initial metastasis at diagnosis was a significant unfavorable prognostic factor. Conclusion The prognosis of dedifferentiated chondrosarcoma is dismal. With the lack of convincing evidence of the benefit of chemotherapy, complete surgical excision is the initial recommended treatment.

  16. Alendronate inhibits cell invasion and MMP-2 secretion in human chondrosarcoma cell line

    Institute of Scientific and Technical Information of China (English)

    Te-jen LAI; Yi-chin FONG; Sheng-feng HSU; Temao LI; Horng-chaung HSU; Jaung-geng LIN; Chin-jung HSU; Ming-chih CHOU; Meng-chih LEE; Shun-fa YANG

    2007-01-01

    Aim: Chondrosarcoma is a malignant primary bone tumor that responds poorly to both chemotherapy and radiation therapy. The aim of the present study was to investigate the effect of alendronate, a bisphosphonate, on the invasion and migration of human chondrosarcoma cells (JJ012). Methods: JJ012 cells were treated with alendronate of various concentrations up to 100 μmol/L for a speci-fied period, and then gelatin zymography and matrigel invasion assay was per-formed to study the effects of alendronate on matrix metalloproteinase (MMP)-2 activity and the invasion ability of JJ012 cells, respectively. Results: Our data showed that alendronate exerted a dose- and time-dependent inhibitory effect on the invasion and migration of JJ012 cells. Furthermore, gelatin zymography and RT-PCR showed that alendronate treatment decreased the activity and mRNA levels of MMP-2 in a concentration-dependent manner. Conclusion: Our find-ings suggest that alendronate may reduce MMP-2 secretion at the transcriptional and translational levels, and inhibit the invasion of chondrosarcoma cell. Therefore,alendronate may be a potential candidate for the systemic therapy of chondro-sarcomas, as well as other malignant diseases.

  17. Chondrosarcoma of the epiglottis: report of a case treated with CO2 laser epiglottectomy.

    Science.gov (United States)

    Mesolella, M; Motta, G; Galli, V

    2004-01-01

    Chondrosarcoma of the larynx is a rare slow-growing malignant tumour with approximately 240 cases reported in the literature: the cricoid cartilage is the most commonly affected site (72-75%), in rare case was described of epiglottis (1-2%). We report a case of a chondrosarcoma of the epiglottis treated with CO2 laser epiglottectomy. The patient was referred with dysphonia, dysphagia and halitosis that started four months before. Indirect laryngoscopy revealed a large smooth mass with a roundish appearance and a pearly-grey colour, as big as a nut, apparently arising from the laryngeal surface of the epiglottis, causing obliteration of the piriform fossa. The definitive histopathological report showed microscopically, the tumour was low-grade (grade I) chondrosarcoma. A literature review regarding chondrosarcomas of the epiglottis is presented. The diagnosis, histology and treatment of these tumours are discussed. In particular we examine the controversy of conservative surgery vs. total laryngectomy. A conservative surgical approach is typically appropriate in light of this tumour's low-aggressive nature.

  18. [Chondrosarcoma of the femur neck simulating a normal variant. A difficult diagnostic pitfall].

    Science.gov (United States)

    Lerais, J M; Baudrillard, J C; Hulin, P H; Laredo, J D; Devillebichot, C

    1991-05-01

    We report a case of chondrosarcoma of the femoral neck wrongly considered as a normal variant during a long time due to the initial aspect on the plain films and its location. Precise radiological criteria must allow the differential diagnosis and will be detailed.

  19. New clinically relevant, orthotopic mouse models of human chondrosarcoma with spontaneous metastasis

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    Dass Crispin R

    2010-06-01

    Full Text Available Abstract Background Chondrosarcoma responds poorly to adjuvant therapy and new, clinically relevant animal models are required to test targeted therapy. Methods Two human chondrosarcoma cell lines, JJ012 and FS090, were evaluated for proliferation, colony formation, invasion, angiogenesis and osteoclastogenesis. Cell lines were also investigated for VEGF, MMP-2, MMP-9, and RECK expression. JJ012 and FS090 were injected separately into the mouse tibia intramedullary canal or tibial periosteum. Animal limbs were measured, and x-rayed for evidence of tumour take and progression. Tibias and lungs were harvested to determine the presence of tumour and lung metastases. Results JJ012 demonstrated significantly higher proliferative capacity, invasion, and colony formation in collagen I gel. JJ012 conditioned medium stimulated endothelial tube formation and osteoclastogenesis with a greater potency than FS090 conditioned medium, perhaps related to the effects of VEGF and MMP-9. In vivo, tumours formed in intratibial and periosteal groups injected with JJ012, however no mice injected with FS090 developed tumours. JJ012 periosteal tumours grew to 3 times the non-injected limb size by 7 weeks, whereas intratibial injected limbs required 10 weeks to achieve a similar tumour size. Sectioned tumour tissue demonstrated features of grade III chondrosarcoma. All JJ012 periosteal tumours (5/5 resulted in lung micro-metastases, while only 2/4 JJ012 intratibial tumours demonstrated metastases. Conclusions The established JJ012 models replicate the site, morphology, and many behavioural characteristics of human chondrosarcoma. Local tumour invasion of bone and spontaneous lung metastasis offer valuable assessment tools to test the potential of novel agents for future chondrosarcoma therapy.

  20. 1-Benzyl-2-Phenylbenzimidazole (BPB, a Benzimidazole Derivative, Induces Cell Apoptosis in Human Chondrosarcoma through Intrinsic and Extrinsic Pathways

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    Ju-Fang Liu

    2012-12-01

    Full Text Available In this study, we investigated the anticancer effects of a new benzimidazole derivative, 1-benzyl-2-phenyl -benzimidazole (BPB, in human chondrosarcoma cells. BPB-mediated apoptosis was assessed by the MTT assay and flow cytometry analysis. The in vivo efficacy was examined in a JJ012 xenograft model. Here we found that BPB induced apoptosis in human chondrosarcoma cell lines (JJ012 and SW1353 but not in primary chondrocytes. BPB induced upregulation of Bax, Bad and Bak, downregulation of Bcl-2, Bid and Bcl-XL and dysfunction of mitochondria in chondrosarcoma. In addition, BPB also promoted cytosolic releases AIF and Endo G. Furthermore, it triggered extrinsic death receptor-dependent pathway, which was characterized by activating Fas, FADD and caspase-8. Most importantly, animal studies revealed a dramatic 40% reduction in tumor volume after 21 days of treatment. Thus, BPB may be a novel anticancer agent for the treatment of chondrosarcoma.

  1. Clinicopathological Features of Extraskeletal Myxoid Chondrosarcoma:An Analysis of 9 Cases

    Institute of Scientific and Technical Information of China (English)

    YE Xiu-feng; MI Can; LI Yu; LIU Qiong

    2008-01-01

    Objective:To investigate the Clinicopathological characteristics of extraskeletal myxoid chondrosarcoma(EMC).Methods:Nine cases of extraskeletal myxoid chondrosarcoma were studied.Extensive immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas.Follow-up information was available for seven patients.Results:There were 7 males and 2 females whose ages ranged from 31 to 69 years(median 52.78 years).Local pain or tenderness and the presence of a palpable mass were the main complaints of the patients.The tumors were located mainly in the lower extremities(66.7%).Most tumors were deep-seated.They usually had a distinct multinodular configuration delineated by fibrous connective tissue.The tumor cells were arranged in delicate intersecting strands,rings,and garlands for the most part.The myxoid matrix was abundant in most cases.Immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas.EMC expressed vimentin(100%,9/9),neuron-specific enolase(77.8%,7/9),S-100 protein(66.7%,6/9),synaptophysin and chromogranin A(22.2%,2/9).None of the tumors expressed EMA and desmin.Ultrastructurally:EMC was characterized by distinct cords of cells immersed in a glycosaminoglycan rich matrix.The cells were rich in mitochondria,had well-developed Golgi apparatus and there were numerous smooth vesicles.In many cells,there were also prominent glycogen deposits and lipid droplets.Some tumor cells had intracisternal microtubules.In one of the 2 extraskeletal myxoid chondrosarcomas there were 140-180 nm diameter membrane-bound dense-core secretory granules in cell bodies.Conclusion:Extraskeletal myxoid chondrosarcoma(EMC)is a rare soft tissue sarcoma characterized by distinctive morphological and cytogenetical features.However,the chondroid nature has been a subject of controversy,and its line of differentiation remains to be

  2. Chondrosarcoma of the mandibular condyle in a patient with Werner syndrome: a case report.

    Science.gov (United States)

    Goutzanis, Labros; Kalfarentzos, Evagelos F; Petsinis, Vassilis; Papadogeorgakis, Nick

    2013-10-01

    Werner syndrome, also called progeria of the adult and pangeria is a rare autosomal recessive disorder that affects connective tissue throughout the body. It is associated with premature ageing and an increased risk of cancer and other diseases. The mean survival for patients with Werner syndrome is 47 years. Death usually occurs when patients are aged 30-65 years because of atherosclerosis or malignant tumours. The purpose of this paper is to present a patient with Werner syndrome exhibiting a chondrosarcoma of the left temporomandibular joint and ramus. To the best of our knowledge this is the first case, of a Werner syndrome patient with an associated head and neck chondrosarcoma being reported. The diagnostic procedure followed and management of the patient are outlined in the paper as well.

  3. Multicentric extraskeletal myxoid chondrosarcoma of uterine adnexa in a young female: An unusual presentation

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    Surbhi Goyal

    2012-01-01

    Full Text Available Extraskeletal myxoid chondrosarcoma is a rare soft-tissue sarcoma, mostly occurring in the proximal extremities and limb girdle. Majority of the patients are in fifth and sixth decades of life with male preponderance. We report here a case of primary extraskeletal myxoid chondrosarcoma of the uterine adnexa involving the broad ligament and fallopian tube synchronously without any evidence of uterine/ovarian involvement in a young multiparous female of 27 years. After the histopathological diagnosis, re-excision of the tumor bed with wide local margins was recommended. Since the tumor has an aggressive course, with propensity for late recurrence and metastases to lungs, the patient must be considered for long-term follow-up.

  4. Rare Case of a Chondrosarcoma of the Mandible in a Child

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    Hendryk Vieweg

    2013-01-01

    Full Text Available Chondrosarcoma of the mandible is rare, especially in children. The available literature consists mostly of a few case reports which are partly integrated in small studies. Growing this small pool of literature is helpful in solidifying knowledge about this disease and facilitating appropriate treatment for children. Therefore, we present such a case in a 12-year-old boy, exhibit comprehensive and relevant information concerning this entity, and discuss our findings in the context of other publications.

  5. Intraoral diode laser epiglottectomy for treatment of epiglottis chondrosarcoma in a dog.

    Science.gov (United States)

    De Lorenzi, D; Bertoncello, D; Dentini, A

    2015-11-01

    Laryngeal tumours are rare in dogs. Surgery is the treatment of choice, but it is usually palliative in malignant conditions, due to advanced stage of the tumour at the time of diagnosis. In veterinary medicine, little information is available about the use of diode laser in laryngeal oncological surgery. In the case reported here, a dog with an epiglottic chondrosarcoma was successfully treated with diode laser epiglottectomy. The surgical technique and follow up are described.

  6. Mesenchymal Chondrosarcoma in Children and Young Adults: A Single Institution Retrospective Review

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    Michael W. Bishop

    2015-01-01

    Full Text Available Background. Mesenchymal chondrosarcoma is an aggressive, uncommon histologic entity arising in bone and soft tissues. We reviewed our institutional experience with this rare diagnosis. Methods. We conducted a retrospective chart review on patients with mesenchymal chondrosarcoma over a 24-year period. Clinicopathologic and radiographic features were reviewed. Results. Twelve patients were identified. Nine were females; median age was 14.5 years (1.2–19.7 years. The most common site was the head/neck (7/12. Disease was localized in 11/12 patients (one with lung nodules. Six with available tissue demonstrated NCOA2 rearrangement by FISH. Six underwent upfront surgical resection, and six received neoadjuvant therapy (2 chemotherapy alone and 4 chemotherapy and radiation. All patients received adjuvant chemotherapy (most commonly ifosfamide/doxorubicin and/or radiation (median dose 59.4 Gy. At a median follow-up of 4.8 years, 5-year disease-free survival and overall survival were 68.2% (95% CI 39.8%, 96.6% and 88.9% (95% CI 66.9%, 100%. Two patients had distant recurrences at 15 and 42 months, respectively. Conclusion. Aggressive surgical resection of mesenchymal chondrosarcoma with chemoradiotherapy yields excellent local control and may reduce likelihood of late recurrence. Characterization of downstream targets of the HEY1-NCOA2 fusion protein, xenograft models, and drug screening are needed to identify novel therapeutic strategies.

  7. Clear cell chondrosarcoma calcaneum - a case report and review of literature.

    Science.gov (United States)

    Nagmani, Singh; Rakesh, John; Aditya, Aggarwal; Sandeep, Patel; Arjun, R H H; Debasis, Gochhait

    2015-03-01

    Chondrosarcoma is the second most common primary bone malignancy accounting for 20-25% of all bone sarcomas. However chondrosarcoma of the foot is rare with just a handful of cases being described. Among the subtypes clear cell variant is the rarest and has never been documented in the foot. We present a rare case of clear cell chondrosarcoma of the calcaneum with multiple metastases that was treated at our institute. The patient was a 62-year old male who presented to us with pain and mass in the left hindfoot with difficulty in walking for 2 years and a discharging ulcer over the lateral aspect for 4 months. Radiography showed aggressive, destructive, lytic lesion in the calcaneum with cortical breach and soft tissue invasion. Bone scan and PET-CT revealed multiple bony metastases and lung metastasis. After initial biopsy, patient underwent below knee amputation and has been in remission since the last 18 months. Given the rarity of this tumor in the calcaneum, this report highlights the importance to consider the possibility of this tumor in the calcaneum as an early diagnosis; complete metastatic workup and expeditious management can thus significantly improve prognosis.

  8. Slug signaling is up-regulated by CCL21/CCR7 [corrected] to induce EMT in human chondrosarcoma.

    Science.gov (United States)

    Li, Guosong; Yang, Yanjun; Xu, Siliang; Ma, Lifeng; He, Mingtang; Zhang, Ziqing

    2015-02-01

    In recent decades, the CXC chemokine receptor 7 (CCR7) [corrected] and its ligand CCL21 have been extensively reported to be associated with tumorigenesis. Meanwhile, Slug signaling induces the epithelial-mesenchymal transition (EMT) process in chondrosarcoma development. In the present study, we explored the functions of CCL21/CCR7 [corrected] in Slug-mediated EMT in the chondrosarcoma. We analyzed protein expression of CCR7 [corrected] and Slug in human chondrosarcoma samples. Effects of CCR7 [corrected] on chondrosarcoma cells were assessed by in vitro assays. Additionally, CCR7 [corrected] pathways were further investigated by pharmacological and genetic approaches. We found that the altered CCR7 [corrected] (81.7 %) and Slug (85.0 %) expression in human chondrosarcoma tissues were significantly associated with grade, recurrence, and 5-year overall survival. According to in vitro assays, CCL21 stimulation induced the expression of phosph-ERK, phosph-AKT, Slug and N-cadherin in SW1353 cells, while the expression of E-cadherin was down-regulated. Furthermore, Slug signaling modulated E- to N-cadherin switch, which was influenced by the kinase inhibitor PD98059 and LY294002. In addition, the genetic silencing of Slug inhibited the capacity of migration and invasion of SW1353 cells. In conclusion, CCL21/CCR7 [corrected] pathway activates ERK and PI3K/AKT signallings to up-regulate Slug pathway, leading to the occurrence of EMT process in human chondrosarcoma. This study lays a new foundation for molecule-targeted therapy of human chondrosarcoma.

  9. Condrossarcoma primário de língua em cão Primary chondrosarcoma in the tongue of a dog

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    A.E. Silva

    2007-04-01

    Full Text Available A 10 year old female Boxer was taken dead to veterinary hospital for a necropsy without any clinical history, showing a growth on the dorsal part of the tongue. Based on the histopathological findings mesenchymal chondrosarcoma was diagnosed. This report describes the first case of chondrosarcoma on the base of the tongue in veterinary medicine.

  10. Screening for potential targets for therapy in mesenchymal, clear cell, and dedifferentiated chondrosarcoma reveals Bcl-2 family members and TGFβ as potential targets

    DEFF Research Database (Denmark)

    van Oosterwijk, Jolieke G; Meijer, Danielle; van Ruler, Maayke A J H

    2013-01-01

    The mesenchymal, clear cell, and dedifferentiated chondrosarcoma subtypes are extremely rare, together constituting 10% to 15% of all chondrosarcomas. Their poor prognosis and lack of efficacious treatment emphasizes the need to elucidate the pathways playing a pivotal role in these tumors. We co...

  11. Paeonol suppresses chondrosarcoma metastasis through up-regulation of miR-141 by modulating PKCδ and c-Src signaling pathway.

    Science.gov (United States)

    Horng, Chi-Ting; Shieh, Po-Chuen; Tan, Tzu-Wei; Yang, Wei-Hung; Tang, Chih-Hsin

    2014-07-02

    Chondrosarcoma, a primary malignant bone cancer, has potential for local invasion and distant metastasis, especially to the lungs. Patients diagnosed with it show poor prognosis. Paeonol (2'-hydroxy-4'-methoxyacetophenone), the main active compound of traditional Chinese remedy Paeonia lactiflora Pallas, exhibits anti-inflammatory and anti-tumor activity; whether paeonol regulates metastatic chondrosarcoma is largely unknown. Here, we find paeonol do not increase apoptosis. By contrast, at non-cytotoxic concentrations, paeonol suppresses migration and invasion of chondrosarcoma cells. We also demonstrate paeonol enhancing miR-141 expression and miR-141 inhibitor reversing paeonol-inhibited cell motility; paeonol also reduces protein kinase C (PKC)d and c-Src kinase activity. Since paeonol inhibits migration and invasion of human chondrosarcoma via up-regulation of miR-141 via PKCd and c-Src pathways, it thus might be a novel anti-metastasis agent for treatment of metastatic chondrosarcoma.

  12. Paeonol Suppresses Chondrosarcoma Metastasis through Up-Regulation of miR-141 by Modulating PKCδ and c-Src Signaling Pathway

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    Chi-Ting Horng

    2014-07-01

    Full Text Available Chondrosarcoma, a primary malignant bone cancer, has potential for local invasion and distant metastasis, especially to the lungs. Patients diagnosed with it show poor prognosis. Paeonol (2'-hydroxy-4'-methoxyacetophenone, the main active compound of traditional Chinese remedy Paeonia lactiflora Pallas, exhibits anti-inflammatory and anti-tumor activity; whether paeonol regulates metastatic chondrosarcoma is largely unknown. Here, we find paeonol do not increase apoptosis. By contrast, at non-cytotoxic concentrations, paeonol suppresses migration and invasion of chondrosarcoma cells. We also demonstrate paeonol enhancing miR-141 expression and miR-141 inhibitor reversing paeonol-inhibited cell motility; paeonol also reduces protein kinase C (PKCd and c-Src kinase activity. Since paeonol inhibits migration and invasion of human chondrosarcoma via up-regulation of miR-141 via PKCd and c-Src pathways, it thus might be a novel anti-metastasis agent for treatment of metastatic chondrosarcoma.

  13. Subglottic Chondrosarcoma Presenting Only Mild Acute-Onset Dyspnea: A Case Report and Review of the Literature

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    Su-wei Tsai

    2014-01-01

    Full Text Available Chondrosarcoma is categorized as a malignant cartilaginous tumor, which occurs rarely in the craniofacial region. We report the case of a 68-year-old man with chondrosarcoma in the subglottic area. His chief symptoms were hoarseness and mild dysphagia. A computed tomography scan revealed a lesion with expansion of the cricoid cartilage and marked reduction of the airway. After biopsy, histological inspection showed that chondrocytes are multi-nucleus, their size does not differ much and mitosis is not obvious. These are all characteristics of a low-grade chondrosarcoma. We performed an organ-preserving operation by debulking the low-grade malignant tumor in order to keep a patent airway. No further metastasis or airway compromise was evident during the 1-year follow-up visit.

  14. Swarm rat chondrosarcoma cells as an in vivo model: lung colonization and effects of tissue environment on tumor growth.

    Science.gov (United States)

    Morcuende, Jose A; Stevens, Jeff W; Scheetz, Todd E; de Fatima Bonaldoc, Maria; Casavant, Thomas L; Otero, Jesse E; Soares, Marcelo B

    2012-01-01

    Swarm rat chondrosarcoma cells have been used extensively for biochemical studies of extra-cellular matrix metabolism in cartilage. However, these cells also possess tumor-like behavior in vivo and are useful in investigation of chondrosarcoma biology. the current study was designed to develop a metastatic model using swarm rat chondrosarcoma cells, and to assess the effect of tissue-environment on tumor behavior in vivo. Tumors were implanted subcutaneously or into bone, and animals were assessed radiographically and microscopically for tumor growth and metastasis. The subcutaneous tumor grew to an average mass of 35 g, while tumor implanted into bone grew 75 mg. Transplantation of the cells into the bone led to extensive bone remodeling with invasion of the medullary cavity and destruction of the bone cortex. Light microscopy demonstrated no significant differences in the number of mitoses, cellular atypia or extracellular matrix staining between the two sites of tumor implantation. Interestingly, lung colonization was observed in none of the animals in the subcutaneous tumor injection group, while tumors colonized the lungs in 95% of the rats with tumor injected into bone. Analysis of cDNA libraries from subcutaneous and bone-transplanted tumors demonstrated a complex and diverse array of expressed transcripts, and there were significant differences in gene expression between tumors at different sites. The results of this study suggest swarm rat chondrosarcoma is a model that resembles human chondrosarcoma mimicking its ability to infiltrate and remodel local bone and to colonize the lungs. Furthermore, the interaction between host-tissue and tumor cells plays a major role in the tumor behavior in this model. Identifying these interactions will lead to further understanding of chondrosarcoma and contribute to therapeutic targets in the future.

  15. Chondrosarcoma arising within a radiation-induced osteochondroma several years following childhood total body irradiation: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Nagata, Shuji [Kurume University Hospital, Department of Radiology, Fukuoka (Japan); Shen, Robert K. [Mayo Clinic, Department of Surgery, Rochester, MN (United States); Laack, Nadia N. [Mayo Clinic, Department of Radiation Oncology, Rochester, MN (United States); Inwards, Carrie Y. [Mayo Clinic, Department of Pathology, Rochester, MN (United States); Wenger, Doris E.; Amrami, Kimberly K. [Mayo Clinic, Department of Radiology, Rochester, MN (United States)

    2013-08-15

    Malignant degeneration arising in radiation-induced osteochondromas is extremely rare. We report a case of a 34-year-old man with a chondrosarcoma arising from an osteochondroma of the left posterior eighth rib that developed following total body irradiation received as part of the conditioning regimen prior to bone marrow transplantation at age 8. To our knowledge, this is only the fourth reported case of a chondrosarcoma arising within a radiation-induced osteochondroma and the first case occurring following childhood total body irradiation. (orig.)

  16. UNUSUAL PRESENTATION OF EXTRASKELETAL MESENCHYMAL CHONDROSARCOMA OF ABDOMEN IN 10 YR OLD GIRL- A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Ramani

    2013-01-01

    Full Text Available ABSTRACT: Mesenchymal chondrosarcoma (MSC is rare form of ch ondrosarcoma which usually arises in bone. Extraskeletal mesenchymal c hondrosarcomas are far less common and accounts approximately 30–50% of all mesenchymal chon drosarcomas. We report a rare case of intra-abdominal extraskeletal MSC in a 10 yr old gi rl who presented with biliary vomitings and abdominal pain. Ultrasound abdomen showed 42x24 mm h ypoechoic mass in right iliac fossa. CT abdomen showed calcified granulomas in spleen, Soft tissue dense lesion in right iliac fossa, suggestive of lymphadenopathy. Histopathology and immu nohistochemistry confirmed the diagnosis of MSC

  17. Chondrosarcoma secondary to hereditary multiple exostosis treated by extended internal hemiplevectomy

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    Ademar Lopes

    Full Text Available The authors report on the case of a 28-year-old patient with extensive chondrosarcoma of the left ischium and pubis involving hip joint, skin, and soft tissue of the gluteal region, secondary to hereditary multiple exostosis submitted to an extended internal Enneking type II and Ill hemipelvectomy. No prosthesis or arthrodesis was used. A few years ago, patients with extensive tumors like this one were treated with interilioabdominal amputation, resulting in a loss of quality of Iife.Two years after the limb-preserving surgery, this patient was disease free, with good functional results, including bipedal ambulation with support.

  18. Dedifferentiated chondrosarcoma arising in fibrous dysplasia: A case report and review of the current literature

    Directory of Open Access Journals (Sweden)

    Nicole MD Riddle

    2009-06-01

    Full Text Available Nicole MD Riddle1, Hideko Yamauchi2, Jamie T Caracciolo4, David Johnson2, G Douglas Letson2, Ardeshir Hakam1,3, Prudence V Smith1,2,3, Marilyn M Bui1,2,31Department of Pathology and Cell Biology, University of South Florida, Tampa, FL, USA; 2Department of Sarcoma, 3Department of Anatomic Pathology, 4Department of Radiology, Moffitt Cancer Center, Tampa, FL, USABackground: Fibrous dysplasia is an uncommon bone disease that has rare but clear potential for malignant transformation. The frequency is increased in polyostotic forms, McCune–Albright syndrome, Mazabraud’s syndrome, and previously irradiated sites. Rapidly progressing pain unrelated to trauma is the most concerning symptom. The early radiological features of sarcomatous transformation are moth-eaten or cystic areas of osteolysis, cortical destruction, and gradual formation of a soft tissue mass. The prognosis is unfavorable as most of the cases are in an advanced stage at the time of diagnosis.Methods: This case was diagnosed at a large cancer center in Florida. Pertinent clinical findings were obtained from chart review and inter-departmental consultation.Results: Histopathological examination revealed dysplastic lamellar bone with no osteoblastic rimming and “Chinese letter” shapes, areas composed of lobulated hyaline cartilage with mild to severe nuclear atypia, and areas of poorly differentiated cells with a spindled appearance, consistent with chondrosarcoma arising within fibrous dysplasia.Conclusions: Sarcomatous transformation of fibrous dysplasia is an uncommon occurrence, yet has significant importance for those with the disease. There may be difficulty with diagnosis given the symptoms and radiologic findings of benign fibrous dysplasia. We report a case of chondrosarcoma rising in fibrous dysplasia and review the current literature. This case is of interest due to the fact that the diagnosis of monostotic fibrous dysplasia was first made at the age of 59 and

  19. Spontaneous massive hemothorax secondary to chest wall chondrosarcoma: a case report.

    Science.gov (United States)

    Rad, Mohammad Ghasemi; Mahmodlou, Rahim; Mohammadi, Afshin; Mladkova, Nikol; Noorozinia, Farahnaz

    2011-01-01

    We present the case of a 30-year-old man with no past history of disease or recent trauma, who was seen in the emergency room after developing sharp pain in the left hemithorax. Chest roentgenogram showed costopherenic angle blunting and an oval mass in the left mediastinum. A computed tomographic scan showed extrapleural mass with coarse calcifications and pleural effusion, confirmed by magnetic resonance imaging. The tumor was biopsied and removed during thoracotomy. The pathology reported revealed chondrosarcoma, which is a rare cause for a spontaneous massive hemothorax. Invasion of the intercostals vessels by the tumor was the probable cause of hemothorax in this patient.

  20. Berberine Reduces the Metastasis of Chondrosarcoma by Modulating the αvβ3 Integrin and the PKCδ, c-Src, and AP-1 Signaling Pathways

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    Chi-Ming Wu

    2013-01-01

    Full Text Available Chondrosarcoma is a primary malignant bone cancer, with a potent capacity to invade locally and cause distant metastasis, especially to the lungs. Patients diagnosed with chondrosarcoma have poor prognosis. Berberine, an active component of the Ranunculaceae and Papaveraceae families of plant, has been proven to induce tumor apoptosis and to prevent the metastasis of cancer cells. However, the effects of berberine in human chondrosarcoma are largely unknown. In this study, we found that berberine did not induce cell apoptosis in human primary chondrocytes and chondrosarcoma cells. However, at noncytotoxic concentrations, berberine reduced the migration and invasion of chondrosarcoma cancer cells. Integrins are the major adhesive molecules in mammalian cells and have been associated with the metastasis of cancer cells. We also found that incubation of chondrosarcoma cells with berberine reduced mRNA transcription for, and cell surface expression of, the αvβ3 integrin, with additional inhibitory effects on PKCδ, c-Src, and NF-κB activation. Thus, berberine may be a novel antimetastasis agent for the treatment of metastatic chondrosarcoma.

  1. Apoptosis signal-regulating kinase 1 is involved in brain-derived neurotrophic factor (BDNF)-enhanced cell motility and matrix metalloproteinase 1 expression in human chondrosarcoma cells.

    Science.gov (United States)

    Lin, Chih-Yang; Chang, Sunny Li-Yun; Fong, Yi-Chin; Hsu, Chin-Jung; Tang, Chih-Hsin

    2013-07-25

    Chondrosarcoma is the primary malignancy of bone that is characterized by a potent capacity to invade locally and cause distant metastasis, and is therefore associated with poor prognoses. Chondrosarcoma further shows a predilection for metastasis to the lungs. The brain-derived neurotrophic factor (BDNF) is a small molecule in the neurotrophin family of growth factors that is associated with the disease status and outcome of cancers. However, the effect of BDNF on cell motility in human chondrosarcoma cells is mostly unknown. Here, we found that human chondrosarcoma cell lines had significantly higher cell motility and BDNF expression compared to normal chondrocytes. We also found that BDNF increased cell motility and expression of matrix metalloproteinase-1 (MMP-1) in human chondrosarcoma cells. BDNF-mediated cell motility and MMP-1 up-regulation were attenuated by Trk inhibitor (K252a), ASK1 inhibitor (thioredoxin), JNK inhibitor (SP600125), and p38 inhibitor (SB203580). Furthermore, BDNF also promoted Sp1 activation. Our results indicate that BDNF enhances the migration and invasion activity of chondrosarcoma cells by increasing MMP-1 expression through a signal transduction pathway that involves the TrkB receptor, ASK1, JNK/p38, and Sp1. BDNF thus represents a promising new target for treating chondrosarcoma metastasis.

  2. Case of intracranial mesenchymal chondrosarcoma. Changes observed by computed tomography before and after radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Hoshino, M.; Tanji, H.; Watanabe, M. (Fukushima Medical Coll. (Japan))

    1981-06-01

    Intracranial mesenchymal chondrosarcoma is very rare, only 14 cases being reported in Europe and in the United States of America. Recently we experienced a case in which the follow-up indicating computed tomograms (CT) demonstrated interesting data on the radiosensitivity of this tumor. The patient, a 14-year-old female was admitted to our hospital with the complaint of left hemiplegia which had gradually progressed. CT revealed an area spreading upward from the right median base of the skull and consisted of two components showing (A) a density as high as that of calcium and (B) a density higher than that of surrounding brain tissue, but much lower than that of calcium. Temporoparietal craniotomy was performed to resect approximately one-half of the tumor. Histological finding revealed mesenchymal chondrosarcoma. The component-A was though to be a cartilaginous tissue, and-B to be an undifferentiated mesenchymal tissue. Postoperative irradiation of 7,000 rad was initiated. The effect of radiotherapy as seen on computed tomograms is as follows, (1) decrease in the volume of the tumor by 26%, (2) decrease in density and enhancement of the area which is considered to be the undifferentiated mesenchymal cells, (3) mild reduction of the area which is considered to be the caltilaginous tissue, and (4) a very high density of the entire tumor similar in degree to that of the bone one year later. These results suggested that radiotherapy is effective for this tumor.

  3. Enchondroma versus Low-Grade Chondrosarcoma in Appendicular Skeleton: Clinical and Radiological Criteria

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    Eugenio M. Ferrer-Santacreu

    2012-01-01

    Full Text Available Objectives. To determine the validity of clinical and radiological features of enchondroma and low grade chondrosarcoma, and contrast the biopsy results with the clinical diagnosis based on the history and imaging. Material and Method. The study included 96 patients with cartilage type lesions suggestive of an enchondroma (E or an low grade chondrosarcoma (LGC according to the clinical and imaging data. The hypotheses were contrasted with the biopsy. Results. Of the 82 patients studied completely, 56 were considered E (68.29%, 8 as LGC (8.33% and in 18 (18.75% were doubtful cases and considered as suspected LGC. Of these, the biopsy showed 4 E (25%, 10 LGC (50% and 4 were not definitive. On the other hand, of the 56 cases diagnosed as E, 15 were biopsied, 5 of these biopsies turned out to be LGC (33.3%. The 8 cases diagnosed as LGC, were also biopsied and only 4 biopsies (50% confirmed the initial diagnosis. Features analyzed in the study showed no statistically significant difference. Correlation analysis between the diagnosis issued initially and the biopsy result gave a value of 0.69 (kappa coefficient, which was considered a good correlation. Conclusion. Features analyzed did not have any statistical significance. However, there was a good correlation between initial diagnosis and biopsy's result.

  4. Meningitis caused by Enterococcus casseliflavus with refractory cerebrospinai fluid leakage following endoscopic endonasal removal of skull base chondrosarcoma

    Institute of Scientific and Technical Information of China (English)

    2011-01-01

    To the Editor:Meningitis caused by Enterococcus casseliflavus (E.casseliflavus) is extremely rare.Here we report an unusual case of meningitis caused by E.casseliflavus coexisting with refractory cerebrospinal fluid (CSF) leakage following endoscopic endonasal resection of skull base chondrosarcoma.

  5. Secondary chondrosarcoma arising from a solitary enchondroma at the index finger of the right hand: A case report

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    Peyker DEMİRELİ

    2006-01-01

    Full Text Available Enchondromas are benign cartilaginous tumors and tend to present in small bones of hands and feet. Malignant transformation, though rare, is seen more often in multiple rather than solitary enchondromatous. A 70- year-old woman presented with swelling and pain lasting for years at the index finger of the right hand. Excisional curettage material of the mass revealed to be entirely an enchondroma. Seven months later, the lesion recurred at the same localization and amputation of the finger was performed. This time the tumor was found to be transformed into an intermediate grade (grade II which demonstrated chondrosarcoma intermingled with classical enchondromatous areas. Enchondromas arising at the hands and feet very rarely transform into chondrosarcomas. Since enchondromas at this site often show histological and clinical features suggestive of malignancy, it is often is difficult to make a histological distinction between benign cartilaginous tumors and chondrosarcomas. Detection of radiological as well as pathological findings are essential for differential diagnosis. This case is remarkable for she has a solitary enchondroma located at the index finger of the right hand that transformed into chondrosarcoma in seven months.

  6. Internal hemipelvectomy with intraoperative and external beam radiotherapy in the limb-sparing treatment of a pelvic girdle chondrosarcoma

    NARCIS (Netherlands)

    Hoekstra, HJ; Szabo, BG

    1998-01-01

    The case of a patient with an extensive pelvic girdle chondrosarcoma treated with internal hemipelvectomy and intraoperative radiotherapy, followed by adjuvant high-dose external beam radiotherapy, with a successful attempt in achieving long-term local tumor control and limb-sparing treatment is des

  7. Clinicopathological significance of p16, cyclin D1, Rb and MIB-1 levels in skull base chordoma and chondrosarcoma

    Institute of Scientific and Technical Information of China (English)

    Jun-qi Liu; Qiu-hang Zhang; Zhen-lin Wang

    2015-01-01

    Objective: To investigate the expression of p16, cyclin D1, retinoblastoma tumor suppressor protein (Rb) and MIB-1 in skull base chordoma and chondrosarcoma tissues, and to determine the clinicopathological significance of the above indexes in these diseases.Methods: A total of 100 skull base chordoma, 30 chondrosarcoma, and 20 normal cartilage tissue samples were analyzed by immunohistochemistry.The expression levels of p16, cyclinD1,Rb and MIB-1 proteins were assessed for potential correlation with the clinicopathological features.Results: As compared to normal cartilage specimen (control), there was decreased expression of p16, and increased expression of cyclin D1, Rb and MIB-1 proteins, in both skull base chordoma and chondrosarcoma specimens.MIB-1 LI levels were significantly increased in skull base chordoma specimens with negative expression of p16, and positive expression of cyclin D1 and Rb (P < 0.05).Significantly elevated MIB-1 LI was also detected in skull base chondrosarcoma tissues, while there was negative expression of p16, cyclin D1 and Rb (P < 0.05).In skull base chordoma, p16 negatively correlated with cyclin D1 and Rb, while cyclin D1 positively correlated with Rb.Additionally, p16, cyclin D1, Rb, or MIB-1 expression showed no correlation with age, gender, or pathological classification of patients with skull base chordoma (P > 0.05).However, p16 and MIB-1 levels correlated with the intradural invasion, and expression of p16, Rb and MIB-1 correlated with the number of tumor foci (P < 0.05).Further, the expression of p16 and MIB-1 appeared to correlate with the prognosis of patients with skull base chordoma.Conclusions: The abnormal expression of p16, cyclin D1 and Rb proteins might be associated with the tumorigenesis of skull base chordoma and chondrosarcoma.

  8. Global demethylation of rat chondrosarcoma cells after treatment with 5-aza-2'-deoxycytidine results in increased tumorigenicity.

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    Christopher A Hamm

    Full Text Available Abnormal patterns of DNA methylation are observed in several types of human cancer. While localized DNA methylation of CpG islands has been associated with gene silencing, the effect that genome-wide loss of methylation has on tumorigenesis is not completely known. To examine its effect on tumorigenesis, we induced DNA demethylation in a rat model of human chondrosarcoma using 5-aza-2-deoxycytidine. Rat specific pyrosequencing assays were utilized to assess the methylation levels in both LINEs and satellite DNA sequences following 5-aza-2-deoxycytidine treatment. Loss of DNA methylation was accompanied by an increase in invasiveness of the rat chondrosarcoma cells, in vitro, as well as by an increase in tumor growth in vivo. Subsequent microarray analysis provided insight into the gene expression changes that result from 5-aza-2-deoxycytidine induced DNA demethylation. In particular, two genes that may function in tumorigenesis, sox-2 and midkine, were expressed at low levels in control cells but upon 5-aza-2-deoxycytidine treatment these genes became overexpressed. Promoter region DNA analysis revealed that these genes were methylated in control cells but became demethylated following 5-aza-2-deoxycytidine treatment. Following withdrawal of 5-aza-2-deoxycytidine, the rat chondrosarcoma cells reestablished global DNA methylation levels that were comparable to that of control cells. Concurrently, invasiveness of the rat chondrosarcoma cells, in vitro, decreased to a level indistinguishable to that of control cells. Taken together these experiments demonstrate that global DNA hypomethylation induced by 5-aza-2-deoxycytidine may promote specific aspects of tumorigenesis in rat chondrosarcoma cells.

  9. rAAV Vectors as Safe and Efficient Tools for the Stable Delivery of Genes to Primary Human Chondrosarcoma Cells In Vitro and In Situ

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    Henning Madry

    2012-01-01

    Full Text Available Treatment of chondrosarcoma remains a major challenge in orthopaedic oncology. Gene transfer strategies based on recombinant adenoassociated viral (rAAV vectors may provide powerful tools to develop new, efficient therapeutic options against these tumors. In the present study, we tested the hypothesis that rAAV is adapted for a stable and safe delivery of foreign sequences in human chondrosarcoma tissue by transducing primary human chondrosarcoma cells in vitro and in situ with different reporter genes (E. coli lacZ, firefly luc, Discosoma sp. RFP. The effects of rAAV administration upon cell survival and metabolic activities were also evaluated to monitor possibly detrimental effects of the gene transfer method. Remarkably, we provide evidence that efficient and prolonged expression of transgene sequences via rAAV can be safely achieved in all the systems investigated, demonstrating the potential of the approach of direct application of therapeutic gene vectors as a means to treat chondrosarcoma.

  10. Paeonol Suppresses Chondrosarcoma Metastasis through Up-Regulation of miR-141 by Modulating PKCδ and c-Src Signaling Pathway

    OpenAIRE

    Chi-Ting Horng; Po-Chuen Shieh; Tzu-Wei Tan; Wei-Hung Yang; Chih-Hsin Tang

    2014-01-01

    Chondrosarcoma, a primary malignant bone cancer, has potential for local invasion and distant metastasis, especially to the lungs. Patients diagnosed with it show poor prognosis. Paeonol (2'-hydroxy-4'-methoxyacetophenone), the main active compound of traditional Chinese remedy Paeonia lactiflora Pallas, exhibits anti-inflammatory and anti-tumor activity; whether paeonol regulates metastatic chondrosarcoma is largely unknown. Here, we find paeonol do not increase apoptosis. By contrast, at n...

  11. Treatment with a Small Molecule Mutant IDH1 Inhibitor Suppresses Tumorigenic Activity and Decreases Production of the Oncometabolite 2-Hydroxyglutarate in Human Chondrosarcoma Cells.

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    Luyuan Li

    Full Text Available Chondrosarcomas are malignant bone tumors that produce cartilaginous matrix. Mutations in isocitrate dehydrogenase enzymes (IDH1/2 were recently described in several cancers including chondrosarcomas. The IDH1 inhibitor AGI-5198 abrogates the ability of mutant IDH1 to produce the oncometabolite D-2 hydroxyglutarate (D-2HG in gliomas. We sought to determine if treatment with AGI-5198 would similarly inhibit tumorigenic activity and D-2HG production in IDH1-mutant human chondrosarcoma cells. Two human chondrosarcoma cell lines, JJ012 and HT1080 with endogenous IDH1 mutations and a human chondrocyte cell line C28 with wild type IDH1 were employed in our study. Mutation analysis of IDH was performed by PCR-based DNA sequencing, and D-2HG was detected using tandem mass spectrometry. We confirmed that JJ012 and HT1080 harbor IDH1 R132G and R132C mutation, respectively, while C28 has no mutation. D-2HG was detectable in cell pellets and media of JJ012 and HT1080 cells, as well as plasma and urine from an IDH-mutant chondrosarcoma patient, which decreased after tumor resection. AGI-5198 treatment decreased D-2HG levels in JJ012 and HT1080 cells in a dose-dependent manner, and dramatically inhibited colony formation and migration, interrupted cell cycling, and induced apoptosis. In conclusion, our study demonstrates anti-tumor activity of a mutant IDH1 inhibitor in human chondrosarcoma cell lines, and suggests that D-2HG is a potential biomarker for IDH mutations in chondrosarcoma cells. Thus, clinical trials of mutant IDH inhibitors are warranted for patients with IDH-mutant chondrosarcomas.

  12. Adiponectin promotes VEGF-A-dependent angiogenesis in human chondrosarcoma through PI3K, Akt, mTOR, and HIF-α pathway

    OpenAIRE

    Lee, Hsiang-Ping; Lin, Chih-Yang; Shih, Jhao-Sheng; Fong, Yi-Chin; Wang, Shih-Wei; Li, Te-Mao; Tang, Chih-Hsin

    2015-01-01

    Chondrosarcoma is a type of highly malignant tumor with a potent capacity to invade locally and cause distant metastasis. Adiponectin is a protein hormone secreted predominantly by differentiated adipocytes. On the other hand, angiogenesis is a critical step in tumor growth and metastasis. However, the relationship of adiponectin with vascular endothelial growth factor-A (VEGF-A) expression and angiogenesis in human chondrosarcoma is mostly unknown. In this study we first demonstrated that th...

  13. An Evaluation of the Diagnostic Accuracy of the Grade of Preoperative Biopsy Compared to Surgical Excision in Chondrosarcoma of the Long Bones

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    Robert Jennings

    2010-01-01

    Full Text Available Chondrosarcoma is the second most common primary malignant bone tumour. Distinguishing between grades is not necessarily straightforward and may alter the disease management. We evaluated the correlation between histological grading of the preoperative image-guided needle biopsy and the resection specimen of 78 consecutive cases of chondrosarcoma of the femur, humerus, and tibia. In 11 instances, there was a discrepancy in histological grade between the biopsy and surgical specimen. Therefore, there was an 85.9% (67/78 accuracy rate for pre-operative histological grading of chondrosarcoma, based on needle biopsy. However, the accuracy of the diagnostic biopsy to distinguish low-grade from high-grade chondrosarcoma was 93.6% (73/78. We conclude that accurate image-guided biopsy is a very useful adjunct in determining histological grade of chondrosarcoma and the subsequent treatment plan. At present, a multidisciplinary approach, comprising experienced orthopaedic surgeons, radiologists, and pathologists, offers the most reliable means of accurately diagnosing and grading of chondrosarcoma of long bones.

  14. Antitumor effect of resveratrol on chondrosarcoma cells via phosphoinositide 3-kinase/AKT and p38 mitogen-activated protein kinase pathways.

    Science.gov (United States)

    Dai, Zixun; Lei, Pengfei; Xie, Jie; Hu, Yihe

    2015-08-01

    Chondrosarcoma is one of the most common types of primary bone cancer that develops in cartilage cells. Resveratrol (Res), a natural polyphenol compound isolated from various fruits, has a suppressive effect on various human malignancies. It has been shown that Res inhibits matrix metalloproteinase (MMP)-induced differentiation in chondrosarcoma cells. However, the effects of Res on cell proliferation, apoptosis and invasion of chondrosarcoma cells, as well as the underlying mechanisms, remain largely unknown. To the best of our knowledge, the present study showed for the first time that Res inhibited proliferation and induced apoptosis in chondrosarcoma cells in a dose-dependent manner. Furthermore, it was shown that Res also suppressed chondrosarcoma cell invasion in a dose-dependent manner, probably via the inhibition of MMP2 and MMP9 protein expression. Molecular mechanism investigations revealed that Res could inhibit the activity of phosphoinositide 3-kinase/AKT and p38 mitogen-activated protein kinase signaling pathways, which has been demonstrated to be important in the regulation of proliferation, apoptosis and invasion in various cancer cell types. In conclusion, this study suggests that Res may serve as a promising agent for the treatment of chondrosarcoma.

  15. Ultrasonographic findings of mesenchymal chondrosarcoma of the mandible: report of a case

    Energy Technology Data Exchange (ETDEWEB)

    Shakibafard, Alireza [TABA Medical Imaging Center, Shiraz (Iran, Islamic Republic of); Shahidi, Shoaleh; Zamiri, Barbod; Houshyar, Maneli; Amanpour, Sara [Dental School, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of); Houshyar, Maral [Dental Center of Dastgheib Hospital, Shiraz(Iran, Islamic Republic of)

    2012-06-15

    Today, ultrasound imaging is being widely used to assess soft tissue lesions in the maxillofacial region. However, ultrasound investigations of intra-osseous lesions are rare, especially for tumors of the jaws. This report emphasized the capability of this useful imaging modality in identification of the characteristics of malignant conditions involving the bone. Mesenchymal chondrosarcoama, one of the unusual malignant conditions of the jaw, was presented in a young male with significant facial swelling. Different imaging modalities parallel with the histopathologic investigation confirmed the diagnosis. Interestingly, destruction of the bony cortex and new bone formation with a characteristic 'sun ray appearance', highly suggestive of sarcomas, was manifested on the ultrasonograph. Thus, this report presented the ultrasonographic features of chondrosarcoma of mandible and considered the ultrasonography to be a useful imaging modality to evaluate intra-osseous jaw lesions.

  16. Extraskeletal myxoid chondrosarcoma in the lung: asymptomatic lung mass with severe anemia

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    Zhou Qianjun

    2012-08-01

    Full Text Available Abstract Extraskeletal myxoid chondrosarcoma (EMC is a rare soft-tissue sarcoma, which primarily occurs deep in the extremities, especially in skeletal muscle, or tendon. EMC of the pleura has been described, however, no case of primary EMC arising from lung has been previously reported. We describe herein, a 51-year-old Asian female initially manifested with signs of severe anemia who presented with a lung mass unrelated to pleura that was morphologically typical EMC, with strong immunoreactivity for vimentin and NSE. Two weeks after resection, the anemia was cured. The patient continued with follow-up, without sign of abnormality 32 months after operation. Virtual slides The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2882199847396682

  17. [Chest Wall Reconstruction Using Titanium Plates Sandwiched Between Sheets after Resection of Chest Wall Chondrosarcoma].

    Science.gov (United States)

    Endoh, Makoto; Oizumi, Hiroyuki; Kato, Hirohisa; Suzuki, Jun; Watarai, Hikaru; Hamada, Akira; Suzuki, Katsuyuki; Takahashi, Ai; Nakahashi, Kenta; Sugawara, Masato; Tsuchiya, Takashi; Sadahiro, Mitsuaki

    2016-07-01

    Extensive chest wall resection carries the risk of difficult reconstruction and surgical complications. We report our experience on chest wall reconstruction using titanium plates for a wide thoracic defect after tumor resection. A 74-year-old man was diagnosed with chondrosarcoma of the 6th rib on the right. He needed extensive chest wall resection because of skip lesions on 4th rib noted on operative inspection, leaving a defect measuring 33 × 20 cm. Reconstruction using 5 transverse titanium plates sandwiched between an expanded polytetrafluoroethylene patch and a polypropylene mesh sheet stabilized the chest wall. This reconstruction allowed successful separation from ventilatory support after operation. The postoperative course was uneventful, and he was discharged on postoperative day 20. The advantages of this form of reconstruction over conventional prostheses are rigidity, and stability and usability.

  18. Transsacral colon fistula: late complication after resection, irradiation and free flap transfer of sacral chondrosarcoma

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    Schildhauer Thomas A

    2008-11-01

    Full Text Available Abstract Background Primary sacral tumors are rare and experience related to accompanying effects of these tumors is therefore limited to observations on a small number of patients. Case presentation In this case report we present a patient with a history of primary sacral chondrosarcoma, an infection of an implanted spinal stabilization device and discuss the challenges that resulted from a colonic fistula associated with large, life threatening abscesses as late complications of radiotherapy. Conclusion In patients with sacral tumors enterocutaneous fistulas after free musculotaneous free flaps transfer are rare and can occur in the setting of surgical damage followed by radiotherapy or advanced disease. They are associated with prolonged morbidity and high mortality. Identification of high-risk patients and management of fistulas at an early stage may delay the need for subsequent therapy and decrease morbidity.

  19. Thyroid, Renal, and Breast Carcinomas, Chondrosarcoma, Colon Adenomas, and Ganglioneuroma: A New Cancer Syndrome, FAP, or Just Coincidence

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    Ihab Shafek Atta

    2016-01-01

    Full Text Available We are presenting a case associated with papillary thyroid carcinoma, renal cell carcinoma, invasive mammary carcinoma, chondrosarcoma, benign ganglioneuroma, and numerous colon adenomas. The patient had a family history of colon cancer, kidney and bladder cancers, lung cancer, thyroid cancer, leukemia, and throat and mouth cancers. She was diagnosed with colonic villous adenoma at the age of 41 followed by thyroid, renal, and breast cancers and chondrosarcoma at the ages of 48, 64, 71, and 74, respectively. Additionally, we included a table with the most common familial cancer syndromes with one or more benign or malignant tumors diagnosed in our case, namely, FAP, HNPCC, Cowden, Peutz-Jeghers, renal cancer, tuberous sclerosis, VHL, breast/other, breast/ovarian, Carney, Werner’s, Bloom, Li-Fraumeni, xeroderma pigmentosum, ataxia-telangiectasia, osteochondromatosis, retinoblastoma, and MEN2A.

  20. A “Proteoglycan Targeting Strategy” for the Scintigraphic Imaging and Monitoring of the Swarm Rat Chondrosarcoma Orthotopic Model

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    Caroline Peyrode

    2011-01-01

    Full Text Available Our lab developed 99mTc-NTP 15-5 radiotracer as targeting proteoglycans (PGs for the scintigraphic imaging of joint. This paper reports preclinical results of 99mTc-NTP 15-5 imaging of an orthotopic model of Swarm rat chondrosarcoma (SRC. 99mTc-NTP 15-5 imaging of SRC-bearing and sham-operated animals was performed and quantified at regular intervals after surgery and compared to bone scintigraphy and tumoural volume. Tumours were characterized by histology and PG assay. SRC exhibited a significant 99mTc-NTP 15-5 uptake at very early stage after implant (with tumour/muscle ratio of 1.61 ± 0.14, whereas no measurable tumour was evidenced. As tumour grew, mean tumour/muscle ratio was increased by 2.4, between the early and late stage of pathology. Bone scintigraphy failed to image chondrosarcoma, even at the later stage of study. 99mTc-NTP 15-5 imaging provided a suitable set of quantitative criteria for the in vivo characterization of chondrosarcoma behaviour in bone environment, useful for achieving a greater understanding of the pathology.

  1. The Use of a Custom-made Prosthesis in the Treatment of Chondrosarcoma of Distal Radius.

    Science.gov (United States)

    Guzik, Grzegorz

    2016-01-01

    The most common neoplasms of the distal radius are primary tumors, of which aneurysmal bone cysts and giant cell tumors are seen almost exclusively. Chondrosarcomas are most commonly located in the pelvis, ribs and proximal segments of the extremities; they rarely occur in forearm bones. Bone defects after distal radial resection can be replaced with bone grafts, both autogenous and allogenic. There is always a risk of failure of the bones to mend or slower synostosis, which necessitates the search for new treatments. Recently, custom-made prostheses have been used with increasing frequency. In early 2015, a 25-year-old male patient was admitted to the Department of Orthopedic Oncology in Brzozów on account of a tumor involving the epiphysis and metaphysis of the right distal radius. Imaging studies confirmed that the lesion was a neoplasm and a biopsy revealed a chondrosarcoma. Radical resection of the tumor was attempted and a custom-made prosthesis was inserted in the place of the bone defect. The prosthesis was designed and manufactured over 4 weeks. No complications occurred during the surgery or in the postoperative period. After the surgery, the forearm and wrist were in a plaster splint for 6 weeks and then rehabilitation was started. The treatment outcome was good. Now, three months after the surgery, the patient has good wrist mobility and efficient grip. Surgical treatment of malignant tumors of the distal radius with extensive bone resection poses the challenge of bone replacement and recovery of fair hand function. Commonly known and practised, reconstructions with autogenous or allogenic bone grafts enable partial restoration of the radiocarpal joint surface and DRUJ. The use of large bone grafts is associated with a risk of non-union and limited hand function even if the grafts are vascularized. Arthrodesis of the radiocarpal joint is currently performed less and less frequently. Custom-made prostheses appear to be a good solution. This method makes

  2. Gliotoxin Isolated from Marine Fungus Aspergillus sp. Induces Apoptosis of Human Cervical Cancer and Chondrosarcoma Cells

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    Van-Tinh Nguyen

    2013-12-01

    Full Text Available Gliotoxin, a secondary metabolite produced by marine fungus Aspergillus sp., possesses various biological activities including anticancer activity. However, the mechanism underlying gliotoxin-induced cytotoxicity on human cervical cancer (Hela and human chondrosarcoma (SW1353 cells remains unclear. In this study, we focused on the effect of gliotoxin induction on apoptosis, the activating expressions of caspase family enzymes in the cells. Apoptotic cell levels were measured through DAPI and Annexin V/Propidium Iodide (PI double staining analysis. The apoptotic protein expression of Bcl-2 and caspase family was detected by Western blot in Hela and SW1353 cells. Our results showed that gliotoxin treatment inhibited cell proliferation and induced significant morphological changes. Gliotoxin induced apoptosis was further confirmed by DNA fragmentation, chromatin condensation and disrupted mitochondrial membrane potential. Gliotoxin-induced activation of caspase-3, caspase-8 and caspase-9, down-regulation of Bcl-2, up-regulation of Bax and cytochromec (cyt c release showed evidence for the gliotoxin activity on apoptosis. These findings suggest that gliotoxin isolated from marine fungus Aspergillus sp. induced apoptosis in Hela and SW1353 cells via the mitochondrial pathway followed by downstream events leading to apoptotic mode of cell death.

  3. Condrossarcoma de epiglote: relato de caso e revisão da literatura Epiglottis chondrosarcoma: review of the literature and report of one case

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    José V. Tagliarini

    2001-09-01

    Full Text Available Condrossarcoma é o sarcoma mais freqüente da laringe. Sua incidência é maior na cartilagem cricóide do que nas outras cartilagens da laringe, sendo raro que ele se origine na epiglote. Relatamos no texto um caso de condrossarcoma originado na epiglote, no qual foi realizada laringectomia subtotal com crico-hioidopexia - e realizamos revisão da literatura.Chondrosarcoma is the most frequent sarcoma of the larynx. It is more prevalent in the cricoid and less prevalent in the other laryngeal cartilages. Chondrosarcoma is rarely located in the epiglottis. We reported a case of epiglottis chondrosarcoma that was treated with a supracricoid laryngectomy with cricohyoidopexy.

  4. Adiponectin promotes VEGF-C-dependent lymphangiogenesis by inhibiting miR-27b through a CaMKII/AMPK/p38 signaling pathway in human chondrosarcoma cells.

    Science.gov (United States)

    Huang, Chun-Yin; Chang, An-Chen; Chen, Hsien-Te; Wang, Shih-Wei; Lo, Yuan-Shun; Tang, Chih-Hsin

    2016-09-01

    Chondrosarcoma is the second most frequently occurring type of bone malignancy characterized by distant metastatic propensity. Vascular endothelial growth factor-C (VEGF-C) is the major lymphangiogenic factor, and makes crucial contributions to tumour lymphangiogenesis and lymphatic metastasis. Adiponectin is a protein hormone secreted predominantly by differentiated adipocytes. In recent years, adiponectin has also been indicated as facilitating tumorigenesis, angiogenesis and metastasis. However, the effect of adiponectin on VEGF-C regulation and lymphangiogenesis in chondrosarcoma has remained largely a mystery. In the present study, we have shown a clinical correlation between adiponectin and VEGF-C, as well as tumour stage, in human chondrosarcoma tissues. We further demonstrated that adiponectin promoted VEGF-C expression and secretion in human chondrosarcoma cells. The conditioned medium from adiponectin-treated cells significantly induced tube formation and migration of human lymphatic endothelial cells. In addition, adiponectin knock down inhibited lymphangiogenesis in vitro and in vivo We also found that adiponectin-induced VEGF-C is mediated by the calmodulin-dependent protein kinase II (CaMKII), AMP-activated protein kinase (AMPK) and p38 signaling pathway. Furthermore, the expression of miR-27b was negatively regulated by adiponectin via the CaMKII, AMPK and p38 cascade. The present study is the first to describe the mechanism of adiponectin-promoted lymphangiogenesis by up-regulating VEGF-C expression in chondrosarcomas. Thus, adiponectin could serve as a therapeutic target in chondrosarcoma metastasis and lymphangiogenesis.

  5. "Chondrosarcoma Arising in the Posterior Part of a Rib with Dumb-Bell Shaped Extension to the Adjacent Intervertebral Foramen; A Rare and Unusual Presentation "

    Directory of Open Access Journals (Sweden)

    M. Bakhshayesh Karam

    2004-06-01

    Full Text Available Chondrosarcomas of thorax are unusual tumors categorized as axial malignancies that also invade and destroy the adjacent bone. These tumors are known to displace soft tissue structures coming in their way without invasion. They destroy adjacent organs violently. In this report, we present a 44-year-old man with a large, expansive mass in the posterior mediastinum invading the spinal canal and epidural space through an intervertebral foramen and displacing the thecal sac. To our knowledge, this is the second case in the literature describing a chondrosarcoma of a rib with dumb-bell-shaped extension to the adjacent intervertebral foramen.

  6. Laryngeal chondrosarcoma: a very unusual pathology CONDROSARCOMA LARÍNGEO: UNA PATOLOGÍA POCO USUAL

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    Enrique Cadena-Piñeros

    2011-06-01

    Full Text Available Chondrosarcoma is the third most frequently occurring bone tumor, following myeloma and osteosarcoma; it accounts for around 20% of all malign bone tumors. They are usually localized in the long bones and the bones of the pelvis; only 2% to 5% are located in the head and neck, mainly in the maxillary bone. Initial symptoms are usually very bizarre and sometimes only a sensation of having a neck mass means that a patient goes to see a doctor. Due to the larynx's intimate relationship with the thyroid gland, this tumor may give a false diagnostic impression. As happened in this case, a patient consulted due to level 4 mass (central neck area, echography of the thyroid nodule and fine needle aspiration (FNA having proved positive for thyroid goiter. The presence of an invasive thyroid carcinoma was suspected in the TAC due to infiltration of the cricoid cartilage; a well-differentiated diagnosis of chondrosarcoma (grade 1 was only made during surgical intervention orientated by biopsy following intraoperation exposure and the definitive study of the pathology. This case has been reported and the topic reviewed as it was difficult case to diagnose and involved a very infrequently occurring pathology.El condrosarcoma es el tercer tumor más frecuente de los huesos, luego del mieloma y el osteosarcoma, y constituye aproximadamente el 20% de todos los tumores óseos malignos. Generalmente se localiza en los huesos largos y de la pelvis y tan sólo 2 a 5% se ubican en la cabeza y el cuello, principalmente en el maxilar. Los síntomas iniciales suelen ser muy bizarros y en ocasiones sólo la sensación de masa del cuello hace que el paciente acuda al médico. Por la íntima relación de la laringe y de la glándula tiroides, este tumor puede llevar a una falsa impresión diagnóstica. Como ocurrió en este caso, una paciente que consultó por masa del nivel VI (zona central del cuello, con ecografía de nódulo tiroideo y aspiración con aguja fina (ACAF

  7. NR4A3 rearrangement reliably distinguishes between the clinicopathologically overlapping entities myoepithelial carcinoma of soft tissue and cellular extraskeletal myxoid chondrosarcoma

    NARCIS (Netherlands)

    Flucke, Uta; Tops, Bastiaan B. J.; Verdijk, Marian A. J.; van Cleef, Patricia J. H.; van Zwam, Peter H.; Slootweg, Pieter J.; Bovee, Judith V. M. G.; Riedl, Robert G.; Creytens, David H.; Suurmeijer, Albert J. H.; Mentzel, Thomas

    2012-01-01

    Myoepithelial carcinoma of soft tissue (MEC) and cellular extraskeletal myxoid chondrosarcoma (cEMC) share striking similarities. In this paper, we compare ten MECs with five cEMCs. MEC patients had an equal gender distribution. The age range was 15-76 years (mean, 42 years). Tumours were located on

  8. NR4A3 rearrangement reliably distinguishes between the clinicopathologically overlapping entities myoepithelial carcinoma of soft tissue and cellular extraskeletal myxoid chondrosarcoma.

    NARCIS (Netherlands)

    Flucke, U.; Tops, B.B.J.; Verdijk, M.A.J.; Cleef, P.J. van; Zwam, P.H. van; Slootweg, P.J.; Bovee, J.V.; Riedl, R.G.; Creytens, D.H.; Suurmeijer, A.J.H.; Mentzel, T.

    2012-01-01

    Myoepithelial carcinoma of soft tissue (MEC) and cellular extraskeletal myxoid chondrosarcoma (cEMC) share striking similarities. In this paper, we compare ten MECs with five cEMCs. MEC patients had an equal gender distribution. The age range was 15-76 years (mean, 42 years). Tumours were located on

  9. Extraskeletal myxoid chondrosarcoma presenting as an intradural spinal mass: report of a rare clinical presentation with an emphasis on differential diagnostic considerations

    Directory of Open Access Journals (Sweden)

    Priya Rao

    2014-12-01

    Full Text Available Extraskeletal myxoid chondrosarcoma is a rare soft tissue neoplasm that occurs predominantly in the soft tissues of the lower extremities. Herein we present a case of a 29 year old male who presented with bilateral femoral numbness believed to be the result of prior injury to his back. A magnetic resonance imaging revealed a mass in the T4-T5 epidural space compressing the spinal cord. Laminectomy was performed and the lesion removed piecemeal. The pathology specimen consisted of multiple fragments of dura involved by a myxoid neoplasm with a nodular growth pattern. The tumor cells were arranged in anastomosing cords and strands. Individual tumor cells were small, of uniform size and shape, with small hyperchromatic nuclei and scant eosinophilic cytoplasm. Immunohistochemical stains were performed which showed the tumor cells were diffusely positive for vimentin and focally positive for EMA, S-100 protein and cytokeratin, whereas they were negative for CD34 and CD99. Fluorescence in situ hybridization (FISH studies showed a clonal population of cells with re-arrangement of the EWSR1 locus, confirming the histologic impression of extraskeletal myxoid chondrosarcoma. This is the first report of a case of an extraskeletal myxoid chondrosarcoma arising from the dura, confirmed to have rearrangement of the EWSR1 gene by FISH. There have only been two other cases of dural based extraskeletal myxoid chondrosarcoma reported prior to our case. We also briefly review the published literature and discuss differential diagnostic considerations for this rare tumor.

  10. Gamma knife radiosurgery for skull base chondrosarcoma%伽玛刀治疗颅底软骨肉瘤

    Institute of Scientific and Technical Information of China (English)

    高德智; 罗斌; 孙时斌; 鲍恩萌; 刘鹏; 王美华; 潘剑; 刘阿力

    2014-01-01

    Objective To assess outcomes after gamma knife radiosurgery for chondrosarcomas of the skull base.Methods Six patients (one female and five male) with skull base chondrosarcomas were reviewed who underwent gamma knife treatment between 2007 and 2013.The mean patient age was 45.6 years (range 28-65 years).All patients were diagnosed as chondrosarcoma by histological confirmation.All patients had no radiation therapy experience.The mean tumor volume was 16.8 cm(range 4.7-62.4 cm) and mean/median margin dose was 13.3/13.5 Gy (range 10-14 Gy),and the mean/median tumor maximum dose was 29.1/28.6 Gy(range 26-31 Gy).The mean/median followed up time was 29.3/27.0 months (range 6-51 months).Results One paitent who was diagnosisd as mesenchymal chondrosareoma dead at 15 months after gamma knife treatment because of tumor progression at 27 months,one patient underwent repeat gamma knife treatment,and the tumor progression free until now.Other 4 patient keep tumor progression free.No symptomatic adverse radiation effects occurred.Conclusion Stereotactic radiosurgery provide a benefit to patients as a adjuvant therapy for skull base chondrosarcoma.%目的 探讨伽玛刀治疗颅底软骨肉瘤的临床效果.方法 回顾分析2007年10月至2013年12月6例软骨肉瘤患者资料,男5例,女1例;年龄28 ~ 65岁,平均45.6岁.所有患者均经手术病理证实为软骨肉瘤.其中间叶型软骨肉瘤1例,标准型软骨肉瘤5例.肿瘤体积4.7 ~62.4 cm3,平均16.8 cm3.周边剂量10~ 14 Gy(平均13.3 Gy,中位13.5 Gy),中心剂量26~31 Gy(平均29.1 Gy,中位28.6 Gy),伽玛刀治疗后随访时间6~51个月(平均29.3个月,中位27.0个月).结果 1例间叶型软骨肉瘤患者术后15个月死亡,1例术后27.0个月复发,再次行伽玛刀治疗,随访至今肿瘤控制良好.4例肿瘤控制良好,且无放射相关并发症出现.结论 伽玛刀作为颅底软骨肉瘤的术后辅助治疗安全且有效.

  11. The EWSR1/NR4A3 fusion protein of extraskeletal myxoid chondrosarcoma activates the PPARG nuclear receptor gene.

    Science.gov (United States)

    Filion, C; Motoi, T; Olshen, A B; Laé, M; Emnett, R J; Gutmann, D H; Perry, A; Ladanyi, M; Labelle, Y

    2009-01-01

    The NR4A3 nuclear receptor is implicated in the development of extraskeletal myxoid chondrosarcoma (EMC), primitive sarcoma unrelated to conventional chondrosarcomas, through a specific fusion with EWSR1 resulting in an aberrant fusion protein that is thought to disrupt the transcriptional regulation of specific target genes. We performed an expression microarray analysis of EMC tumours expressing the EWSR1/NR4A3 fusion protein, comparing their expression profiles to those of other sarcoma types. We thereby identified a set of genes significantly overexpressed in EMC relative to other sarcomas, including PPARG and NDRG2. Western blot or immunohistochemical analyses confirm that PPARG and NDRG2 are expressed in tumours positive for EWSR1/NR4A3. Bioinformatic analysis identified a DNA response element for EWSR1/NR4A3 in the PPARG promoter, and band-shift experiments and transient transfections indicate that EWSR1/NR4A3 can activate transcription through this element. Western blots further show that an isoform of the native NR4A3 receptor lacking the C-terminal domain is very highly expressed in tumours positive for EWSR1/NR4A3, and co-transfections of this isoform along with EWSR1/NR4A3 indicate that it may negatively regulate the activity of the fusion protein on the PPARG promoter. These results suggest that the overall expression of PPARG in EMC may be regulated in part by the balance between EWSR1/NR4A3 and NR4A3, and that PPARG may play a crucial role in the development of these tumours. The specific up-regulation of PPARG by EWSR1/NR4A3 may also have potential therapeutic implications.

  12. Condrossarcoma laríngeo: relato de caso e revisão de literatura Laryngeal chondrosarcoma: a case report and review of literature

    Directory of Open Access Journals (Sweden)

    Giordania Gomes Campos

    2004-12-01

    Full Text Available Os tumores cartilaginosos da laringe são extremamente raros e correspondem a aproximadamente 1% dos tumores que acometem este órgão. Menos que 0,1% destes tumores correspondem aos condrossarcomas. Os condromas e os condrossarcomas de baixo grau são os mais freqüentemente encontrados e 70-75% destes tumores localizam-se na face laríngea da lâmina posterior da cartilagem cricóidea. O diagnóstico do condrossarcoma da laringe pode ser esquecido devido a sua baixa ocorrência e sua forma indolente de crescimento. A apresentação clínica é variada e diretamente dependente do tamanho e localização do tumor: estridor, cornagem, dispnéia, disfagia ou massa cervical são os sinais mais freqüentes. O objetivo deste estudo é apresentar um caso incomum de condrossarcoma laríngeo de origem na cartilagem tireóidea, discutindo o quadro clínico, o diagnóstico, tratamento e os fatores prognósticos.Cartilaginous tumors of the larynx are extremely rare neoplasms that account for approximately one per cent of all tumors of this organ. Less than 0.1% correspond to chondrosarcomas. Chondroma and low-grade chondrosarcoma are the most common, 70-75% of these tumors arise on the endolaryngeal surface of the posterior lamina of the cricoid cartilage. The diagnosis of laryngeal chondrosarcoma is likely to be missed because of its infrequent occurrence and its indolent pattern of growth. The clinical presentation is varied and directly dependent on size and location of tumor: stridor, hoarseness, dyspnea or neck mass are commonly presented signs. The objective of this study was to show an unusual case of laryngeal chondrosarcoma originating from thyroid cartilage, discussing its clinical presentation, diagnosis, treatment and prognosis.

  13. Intraparenchymal mesenchymal chondrosarcoma of the frontal lobe--a case report and molecular detection of specific gene fusions from archival FFPE sample.

    Science.gov (United States)

    Sajjad, Emir Ahmed; Sikora, Katarzyna; Paciejewski, Tomasz; Garbicz, Filip; Paskal, Wiktor; Szacht, Milena; Grajkowska, Wieslawa; Włodarski, Pawel Krzysztof

    2015-01-01

    Mesenchymal chondrosarcoma is a rare tumor of cartilaginous origin characterized by its bimorphic pattern composed of highly undifferentiated small round cells separated by islands of well-differentiated hyaline cartilage. It exhibits higher malignancy and earlier occurrence in comparison to classic chondrosarcomas. Recently identified HEY1-NCOA2 and IRF2BP2-CDX1 gene fusions confirm their distinct molecular origin and pose a promising diagnostic marker. The majority of cases arise from craniofacial bones. In this study, we present a rare case of mesenchymal chondrosarcoma encompassed within the brain parenchyma of the frontal lobe without any dural or bone attachment. We demonstrate histopathological findings and confirm the HEY1-NCOA2 gene fusion in a formalin-fixed paraffin-embedded archival sample using simple reverse transcription polymerase chain reaction (RT-PCR) method. IRF2BP2-CDX1 gene fusion was absent in the analyzed sample. The clinical follow-up is also presented with a review of treatment modalities for this entity.

  14. Epigenetics: methylation-associated repression of heparan sulfate 3-O-sulfotransferase gene expression contributes to the invasive phenotype of H-EMC-SS chondrosarcoma cells.

    Science.gov (United States)

    Bui, Catherine; Ouzzine, Mohamed; Talhaoui, Ibtissam; Sharp, Sheila; Prydz, Kristian; Coughtrie, Michael W H; Fournel-Gigleux, Sylvie

    2010-02-01

    Heparan sulfate proteoglycans (HSPGs), strategically located at the cell-tissue-organ interface, regulate major biological processes, including cell proliferation, migration, and adhesion. These vital functions are compromised in tumors, due, in part, to alterations in heparan sulfate (HS) expression and structure. How these modifications occur is largely unknown. Here, we investigated whether epigenetic abnormalities involving aberrant DNA methylation affect HS biosynthetic enzymes in cancer cells. Analysis of the methylation status of glycosyltransferase and sulfotransferase genes in H-HEMC-SS chondrosarcoma cells showed a typical hypermethylation profile of 3-OST sulfotransferase genes. Exposure of chondrosarcoma cells to 5-aza-2'-deoxycytidine (5-Aza-dc), a DNA-methyltransferase inhibitor, up-regulated expression of 3-OST1, 3-OST2, and 3-OST3A mRNAs, indicating that aberrant methylation affects transcription of these genes. Furthermore, HS expression was restored on 5-Aza-dc treatment or reintroduction of 3-OST expression, as shown by indirect immunofluorescence microscopy and/or analysis of HS chains by anion-exchange and gel-filtration chromatography. Notably, 5-Aza-dc treatment of HEMC cells or expression of 3-OST3A cDNA reduced their proliferative and invading properties and augmented adhesion of chondrosarcoma cells. These results provide the first evidence for specific epigenetic regulation of 3-OST genes resulting in altered HSPG sulfation and point to a defect of HS-3-O-sulfation as a factor in cancer progression.

  15. Mesenchymal chondrosarcoma of bone and soft tissue: a systematic review of 107 patients in the past 20 years.

    Directory of Open Access Journals (Sweden)

    Jie Xu

    Full Text Available Mesenchymal chondrosarcoma(MCS is a rare high-grade variant of chondrosarcoma. Consensus has not been reached on its optimal management. Resection with wide margins is usually recommended, but the effect of margins has been demonstrated by little positive evidence. Moreover, the effectiveness of adjuvant chemo- and/or radiotherapy remains controversial.To describe the clinical characteristics and outcomes of MCS of bone and soft tissue, to assess the efficacies of surgery, chemotherapy and radiation, and finally to deliver a more appropriate therapy.We reviewed EMBASE-, MEDLINE-, Cochrane-, Ovid- and PubMed-based to find out all cases of MCS of bone and soft tissue described between April 1994 and April 2014. Description of treatment and regular follow-up was required for each study. Language was restricted to English and Chinese. Issues of age, gender, location, metastasis, and treatment were all evaluated for each case. Kaplan-Meier Method and Cox Proportional Hazard Regression Model were used in the survival analysis.From the 630 identified publications, 18 meeting the inclusion criteria were selected, involving a total of 107 patients. Based on these data, the 5-, 10-and 20-year overall survival are 55.0%, 43.5% and 15.7% respectively. The 5-, 10-, 20- year event-free survival rates are 45.0%, 27.2% and 8.1%, respectively. Treatment without surgery is associated with poorer overall survival and event-free survival. Negative surgical margins could significantly bring down the local-recurrence rate and are associated with a higher event-free survival rate. Chemotherapy regime based on anthracyclines does not benefit the overall survival. The addition of radiation therapy is not significantly associated with the overall or event-free survival. However, we recommend radiation as the salvage therapy for patients with positive margin so as to achieve better local control.This review shows that surgery is essential in the management of MCS of bone

  16. Enchondroma versus Chondrosarcoma in Long Bones of Appendicular Skeleton: Clinical and Radiological Criteria—A Follow-Up

    Directory of Open Access Journals (Sweden)

    Eugenio M. Ferrer-Santacreu

    2016-01-01

    Full Text Available As of today two types of cartilage tumors remain a challenge even for the orthopedic oncologist: enchondroma (E, a benign tumor, and chondrosarcoma (LGC, a malignant and low aggressiveness tumor. A prospective study of 133 patients with a cartilaginous tumor of low aggressiveness in the long bones of the appendicular skeleton was done to prove this difficult differential diagnosis. Parameters including medical history and radiological and nuclear imaging were collected and compared to the result of the biopsy. A scale of aggressiveness was applied to each patient according to the number of aggressiveness episodes present. A comparison of the results of the biopsy with the initial diagnosis made by the orthopedic oncologist based solely on clinical data and imaging tests was also made. Finally, a management algorithm for these cases was proposed. A statistical significance for LGC resulted from the parameter as follows: pain on palpation, involvement of cortical in either the CT or MRI, and Tc99 bone scan uptake equal or superior to anterosuperior iliac crest. In our series, a tumor scoring 5 points or higher in the scale of aggressiveness can have 50% more chance of being LGC. When compared with the gold standard (the biopsy, surgeon’s initial judgement showed a sensitivity of 73.5% and a specificity of 94.1%.

  17. {sup 99m}Tc-N.T.P. 15-5 imaging for the early and specific diagnosis of chondrosarcoma: proof of concept in rats

    Energy Technology Data Exchange (ETDEWEB)

    Miot-Noirault, E.; Vidal, A.; Rapp, M.; Madelmont, J.C.; Maublant, J.; Moins, N. [Institut National de la Sante et de la Recherche Medicale (INSERM), UMR 484, 63 - Clermont Ferrand (France); Redini, F.; Gouin, F.; Heymann, D. [Institut National de la Sante et de la Recherche Medicale (INSERM), ERI 7, EA 3822, 44 - Nantes (France)

    2008-02-15

    Aim. - The U.M.R. 484 I.N.S.E.R.M. develops a 'cartilage imaging strategy' with the {sup 99m}Tc-N.T.P. 15-5 tracer that selectively binds to cartilage proteoglycans, allowing a highly specific cartilage imaging. Since chondro-genic tumours are characterized by the presence of cartilaginous matrix, we hypothesized that the {sup 99m}Tc-N.T.P. 15-5 tracer would allow chondrosarcoma imaging, which is currently lacking in clinics. In the rat model of grade II para-tibial chondrosarcoma, we evaluated the relevance of {sup 99m}Tc-N.T.P. 15-5 imaging for an early and specific diagnosis of chondrosarcoma. Methods. - {sup 99m}Tc-N.T.P. 15-5 longitudinal imaging of animals was performed during two months after para-tibial ortho-topic tumour implantation in the right paw, the left being used as control. At regular intervals, animals were submitted to {sup 99m}Tc-M.D.P. bone imaging, the only examination used for SPECT diagnosis of chondrosarcoma in patients. Tumour volume was monitored for two months when the tumours became palpable, with the two perpendicular diameters measured. For both cartilage and bone imaging, the scans were considered positive when areas of tracer uptake were present at sites consistent with the sites of inoculation. For each animal, positive scans were analyzed at each stage using the semiquantitative method of the target to background ratio (T.B.R.), with the target R.O.I. being delineated over the tumour and background R.O.I. over vertebra and muscle. T.B.R. time-course was followed as a function of tumour growth.At study ending, each animal was sacrificed for histopathological control. Results. - {sup 99m}Tc-N.T.P. 15-5 scans were positive in 100% of the animals at very early stage (three days) after implantation, while no palpable nor measurable tumour could be assessed. Quantitative analysis of {sup 99m}Tc-N.T.P. 15-5 scans evidenced a significant uptake of the tracer at the implantation site at early stage. The time-course of T

  18. Genome-wide mRNA and miRNA expression data analysis to screen for markers involved in sarcomagenesis in human chondrosarcoma cell lines

    Directory of Open Access Journals (Sweden)

    Biju Issac

    2014-12-01

    Full Text Available Genes and miRNAs involved in sarcomagenesis related pathways are unknown and therefore signaling events leading to mesenchymal cell transformation to sarcoma are poorly elucidated. Exiqon and Illumina microarray study on human chondrosarcoma JJ012 and chondrocytes C28 cell lines to compare and analyze the differentially expressed miRNAs and their gene targets was recently published in the Journal Tumor Biology in 2014. Here we describe in details the contents and quality controls for the miRNA and gene expression data associated with the study that is relevant to this dataset.

  19. 低级别软骨肉瘤7例临床病理分析%THE CLINICOPATHOLOGICAL ANALYSIS OF 7-CASE LOW GRADE CHONDROSARCOMA

    Institute of Scientific and Technical Information of China (English)

    杨海平; 许丽娟; 吴骏; 刘辉

    2012-01-01

    目的 分析低级别软骨肉瘤的临床病理特点,提高其诊断和鉴别诊断的能力,为临床治疗提供帮助.方法 回顾分析低级别软骨肉瘤7例的临床病理资料并复习相关文献.结果 低级别软骨肉瘤主要发生在中轴骨或接近中轴骨的部位.肉眼观察肿瘤呈灰白色软骨样或灰白间灰褐色,有囊性变5例;显微镜下为低级别软骨肉瘤.结论 低级别软骨肉瘤的诊断必须将临床症状、影像资料及病理改变进行综合分析.尽管为低度恶性,但明确诊断后根治切除尚能取得满意的临床疗效.%Objective To analyze the clinicopathological characteristics of low grade chondrosarcoma, enhance the ability of diagnosis and differential diagnosis of the tumor, and provide help for clinical treatment. Methods The clinical data and pathological changes of 7 cases of low grade chondrosarcoma were retrospectively studied and the relevant literature was reviewed. Results The tumors were mainly located in axial skeleton, or near site. Macroscopic observation of tumors were gray cartilage - like, some of which were gray - brown, and there were cystic changes in 5 cases; Low grade chondrosarcomas were proved under the microscope. Conclusion Diagnosis of the low grade chondrosarcoma must be made by comprehensive analysis of clinical symptoms, radiological imaging and pathological changes. The clinical effect of the tumor is satisfactory by the radical resection of the tumor in spite of low - grade malignancy.

  20. Epigenetic regulation of embryonic stem cell marker miR302C in human chondrosarcoma as determinant of antiproliferative activity of proline-rich polypeptide 1.

    Science.gov (United States)

    Galoian, Karina; Qureshi, Amir; D'Ippolito, Gianluca; Schiller, Paul C; Molinari, Marco; Johnstone, Andrea L; Brothers, Shaun P; Paz, Ana C; Temple, H T

    2015-08-01

    Metastatic chondrosarcoma of mesenchymal origin is the second most common bone malignancy and does not respond either to chemotherapy or radiation; therefore, the search for new therapies is relevant and urgent. We described recently that tumor growth inhibiting cytostatic proline-rich polypeptide 1, (PRP-1) significantly upregulated tumor suppressor miRNAs, downregulated onco-miRNAs in human chondrosarcoma JJ012 cell line, compared to chondrocytes culture. In this study we hypothesized the existence and regulation of a functional marker in cancer stem cells, correlated to peptides antiproliferative activity. Experimental results indicated that among significantly downregulated miRNA after PRP-1treatment was miRNAs 302c*. This miRNA is a part of the cluster miR302‑367, which is stemness regulator in human embryonic stem cells and in certain tumors, but is not expressed in adult hMSCs and normal tissues. PRP-1 had strong inhibitory effect on viability of chondrosarcoma and multilineage induced multipotent adult cells (embryonic primitive cell type). Unlike chondrosarcoma, in glioblastoma, PRP-1 does not have any inhibitory activity on cell proliferation, because in glioblastoma miR-302-367 cluster plays an opposite role, its expression is sufficient to suppress the stemness inducing properties. The observed correlation between the antiproliferative activity of PRP-1 and its action on downregulation of miR302c explains the peptides opposite effects on the upregulation of proliferation of adult mesenchymal stem cells, and the inhibition of the proliferation of human bone giant-cell tumor stromal cells, reported earlier. PRP-1 substantially downregulated the miR302c targets, the stemness markers Nanog, c-Myc and polycomb protein Bmi-1. miR302c expression is induced by JMJD2-mediated H3K9me2 demethylase activity in its promoter region. JMJD2 was reported to be a positive regulator for Nanog. Our experimental results proved that PRP-1 strongly inhibited H3K9 activity

  1. BMP-7 enhances cell migration and αvβ3 integrin expression via a c-Src-dependent pathway in human chondrosarcoma cells.

    Directory of Open Access Journals (Sweden)

    Jui-Chieh Chen

    Full Text Available Bone morphogenic protein (BMP-7 is a member of the transforming growth factor (TGF-beta superfamily, which is originally identified based on its ability to induce cartilage and bone formation. In recent years, BMP-7 is also defined as a potent promoter of cell motility, invasion, and metastasis. However, there is little knowledge of the role of BMP-7 and its cellular function in chondrosarcoma cells. In the present study, we investigated the biological impact of BMP-7 on cell motility using transwell assay. In addition, the intracellular signaling pathways were also investigated by pharmacological and genetic approaches. Our results demonstrated that treatment with exogenous BMP-7 markedly increased cell migration by activating c-Src/PI3K/Akt/IKK/NF-κB signaling pathway, resulting in the transactivation of αvβ3 integrin expression. Indeed, abrogation of signaling activation, by chemical inhibition or expression of a kinase dead form of the protein attenuated BMP-7-induced expression of integrin αvβ3 and cell migration. These findings may provide a useful tool for diagnostic/prognostic purposes and even therapeutically in late-stage chondrosarcoma as an anti-metastatic agent.

  2. The cucurbitacins E, D and I: investigation of their cytotoxicity toward human chondrosarcoma SW 1353 cell line and their biotransformation in man liver.

    Science.gov (United States)

    Abbas, Suzanne; Vincourt, Jean-Baptiste; Habib, Lamice; Netter, Patrick; Greige-Gerges, Hélène; Magdalou, Jacques

    2013-02-04

    Cucurbitacins are a class of natural compounds known for their numerous potential pharmacological effects. The purpose of this work was to compare the cytotoxicity of three cucurbitacins I, D, E on the chondrosarcoma SW 1353 cancer cell line and to investigate their biotransformation in man. Cucurbitacins I and D showed a very strong cytotoxicity, which was higher than that of cytochalasin D, used as a drug reference. Almost 100% of the cells were apoptotic as observed by DNA fragmentation (TUNEL assay) after 12 h with cucurbitacins I and D (1 μM) and cucurbitacin E (10 μM). In terms of IC(50) values, cucurbitacins I and E presented a higher toxicity compared to that of cucurbitacin D (MTT assay). Cucurbitacin E was readily hydrolyzed by human hepatic microsomes, leading to cucurbitacin I (K(m) 22 μM, V(max) 571 nmol/mg proteins/min). On the other hand, the three cucurbitacins were hydroxylated at a very low extent, but they were sulfated and glucuronidated. In terms of V(max)/K(m), the cucurbitacin E was the best substrate of UDP-glucuronosyltransferases. This study shows that cucurbitacins I, D and E present a potent cytotoxic activity toward the chondrosarcoma SW 1353 cell line and are metabolized as sulfate and glucuronide conjugates.

  3. Femur chondrosarcoma misdiagnosed as acute knee arthritis and osteomyelitis--further developing a hitherto unreported complication of tumor embolic ischemic ileal perforation after arthroscopic lavage.

    Science.gov (United States)

    Chow, Louis Tsun Cheung

    2014-12-01

    The differentiation between osteomyelitis and bone tumor may be difficult due to their overlapping clinical and radiological features. A 25-year-old lady presented with left knee pain and joint effusion associated with redness and hotness. A sub-optimally taken plain radiograph showed mixed osteolytic and osteoblastic lesion in the left lower femur with surrounding soft tissue swelling. Since the clinical diagnosis was acute osteomyelitis and arthritis, arthroscopic lavage was performed as a diagnostic and therapeutic procedure. The removed loose bodies and fibrinous tissue showed pathological features suspicious of chondrosarcoma. Subsequent MRI revealed an infiltrative tumor eroding through the cortex and joint cartilage. En bloc excision of the left lower femur, upper tibia including the knee joint and patella was performed, and the final diagnosis was grade 2 chondrosarcoma. The patient developed bilateral pulmonary metastasis 33 months after operation. Five months later, she suffered from a hitherto undescribed complication of ischemic perforation of the terminal ileum secondary to tumor embolic arterial obstruction with no macroscopic intestinal or peritoneal tumor deposit. The patient developed multiple brain metastases and died 43 months after initial presentation. Our case illustrates that malignant bone tumor as a differential diagnosis of acute osteomyelitis and arthritis merits recognition and exclusion before arthroscopic lavage, which may enhance tumor dissemination and in our patient results in embolic ischemic ileal perforation.

  4. Spot-Scanning Proton Radiation Therapy for Pediatric Chordoma and Chondrosarcoma: Clinical Outcome of 26 Patients Treated at Paul Scherrer Institute

    Energy Technology Data Exchange (ETDEWEB)

    Rombi, Barbara [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); ATreP (Provincial Agency for Proton Therapy), Trento (Italy); Ares, Carmen, E-mail: carmen.ares@psi.ch [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); Hug, Eugen B. [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); ProCure Proton Therapy Center, Somerset, New Jersey (United States); Schneider, Ralf; Goitein, Gudrun; Staab, Adrian; Albertini, Francesca; Bolsi, Alessandra; Lomax, Antony J. [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); Timmermann, Beate [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); WestGerman Proton Therapy Center Essen (Germany)

    2013-07-01

    Purpose: To evaluate the clinical results of fractionated spot-scanning proton radiation therapy (PT) in 26 pediatric patients treated at Paul Scherrer Institute for chordoma (CH) or chondrosarcoma (CS) of the skull base or axial skeleton. Methods and Materials: Between June 2000 and June 2010, 19 CH and 7 CS patients with tumors originating from the skull base (17) and the axial skeleton (9) were treated with PT. Mean age at the time of PT was 13.2 years. The mean prescribed dose was 74 Gy (relative biological effectiveness [RBE]) for CH and 66 Gy (RBE) for CS, at a dose of 1.8-2.0 Gy (RBE) per fraction. Results: Mean follow-up was 46 months. Actuarial 5-year local control (LC) rates were 81% for CH and 80% for CS. Actuarial 5-year overall survival (OS) was 89% for CH and 75% for CS. Two CH patients had local failures: one is alive with evidence of disease, while the other patient succumbed to local recurrence in the surgical pathway. One CS patient died of local progression of the disease. No high-grade late toxicities were observed. Conclusions: Spot-scanning PT for pediatric CH and CS patients resulted in excellent clinical outcomes with acceptable rates of late toxicity. Longer follow-up time and larger cohort are needed to fully assess tumor control and late effects of treatment.

  5. Interleukin-1β induced nuclear factor-κB binds to a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 9 promoter in human chondrosarcoma cells.

    Science.gov (United States)

    Altuntas, Aynur; Halacli, Sevil Oskay; Cakmak, Ozlem; Erden, Gonul; Akyol, Sumeyya; Ugurcu, Veli; Hirohata, Satoshi; Demircan, Kadir

    2015-07-01

    Nuclear factor-κB (NF-κB) is involved in the regulation of inflammation‑associated genes. NF-κB forms dimers which bind with sequences referred to as NF-κB sites (9-11 bp). A disintegrin-like and metalloproteinase with thrombospondin type 1 motif 9 (ADAMTS9) is a type of proteoglycanase, which proteolytically cleaves versican and aggrecan. ADAMTS9 is a cytokine-inducible gene that contains binding sites for NF-κB within its promoter region. Interleukin-1β (IL-1β) affects cartilage metabolism and is involved in the NF-κB pathway. It is therefore hypothesized that NF-κB binding with ADAMTS9 promoters may activate IL-1β, thereby promoting chondrocytic cell growth. In the present study, the OUMS-27 chondrocytic human chondrosarcoma cell line was treated with IL-1β with or without inhibitors of NF-κB signaling pathways. Chromatin immunoprecipitation (ChIP) and electromobility shift assays (EMSA) were conducted order to analyze the binding of NF-κB with the ADAMTS9 promoter region. NF-κB-p65 subunit phosphorylation was promoted in IL-1β-treated cells, which were not treated with inhibitors of NF-κB signaling pathways. By contrast, NF-κB-p65 subunit phosphorylation was inhibited in cells that had been treated with BAY-117085, an NF-κB pathway inhibitor. ChIP and EMSA assays demonstrated that, following treatment with IL-1β, NF-κB‑p65 bound to elements located at -1177 and -1335 in the ADAMTS9 promoter region, in contrast to the untreated samples. The results of the present study suggested that NF-κB may be involved in IL-1β-induced activation of ADAMTS9 in human chondrocytes.

  6. Resveratrol inhibits phosphorylation within the signal transduction and activator of transcription 3 signaling pathway by activating sirtuin 1 in SW1353 chondrosarcoma cells.

    Science.gov (United States)

    Jin, Haidong; Chen, Hui; Yu, Kehe; Zhang, Jingdong; Li, Bin; Cai, Ningyu; Pan, Jun

    2016-09-01

    The present study assessed the mechanism by which resveratrol (Res) inhibits the growth of SW1353 chondrosarcoma cells and examined whether sirtuin 1 (Sirt1) activation affects phosphorylation within the signal transduction and activator of transcription 3 (STAT3) signaling pathway. The present study used SW1353 chondrosarcoma cells in the logarithmic phase of growth (control and treatment groups). The latter group was treated with Res at 25 and 50 µmol/l for 24 h, and cell viability, proliferation and apoptosis were analyzed using the cell counting kit‑8 assay, colony counting and Hoechst staining, respectively. The expression levels of caspase‑3, cleaved caspase‑3, B‑cell lymphoma‑2 (BCL‑2), BCL-2 associated X protein (Bax), STAT3 and phosphorylated (p‑)STAT3) were measured by Western blotting. SW1353 cells were transfected with small interfering (si)RNA targeting Sirt1 and the expression levels of Sirt1, STAT3 and p-STAT3 were assessed. Exposure of SW1353 cells to Res reduced cell viability in a dose‑dependent manner (P<0.01). Additionally, cell proliferation was significantly inhibited and the cell nuclei exhibited apoptotic characteristics. Cleaved caspase‑3, Sirt1 and Bax levels were upregulated. The expression levels of BCL‑2 and p‑STAT3 were downregulated. Additionally, the BCL‑2/Bax ratio was reduced compared with the control group. The total STAT3 level was unaffected. Res treatment activated Sirt1, however, in cells transfected with Sirt1‑siRNA, the ability of resveratrol to suppress p‑STAT3 expression was compromised. Overall, it was revealed that Res treatment induced apoptosis, inhibited proliferation and affected phosphorylation within the STAT3 signaling pathway by activating Sirt1 in SW1353 chondrosarcoma cells.

  7. Management of primary spinal chondrosarcoma: report of two cases causing cord compression Manejo dos condrosarcomas espinhais primários: relato de dois casos causando compressão medular

    Directory of Open Access Journals (Sweden)

    Daniel Monte-Serrat Prevedello

    2004-09-01

    Full Text Available Chondrosarcomas are malignant tumors that rarely grow inside the spinal canal. Prognosis depends on histological features, patient's age and surgical margins free from tumor. Response to radio and chemotherapy is poor. Ideal treatment consists of total " en-block" resection, not always achievable due to limitation of location, compromise of stability and risk of inducing neurological deficits. Two cases of spinal chondrosarcoma causing cord compression are reported, located in the cervical and thoracic spine. Microsurgical technique consisted of initial debulking followed by removal of margins until limits free from tumor were obtained. Total resection was accomplished and neurological function improved in both cases. Follow-up has been seven and one year respectively, with no evidence of recurrence and preserved neurological functions. Association between chondrosarcoma and estrogen-dependent tumor has been confirmed in this report. Although " en-block" resection of a chondrosarcoma should be tried whenever possible, tumor fragmentation should be considered in difficult cases, as in the present report, in which a long period free from recurrence with good quality of life can be obtained.Os condrosarcomas são tumores malignos, raramente localizados no interior do canal espinhal, com prognóstico dependente do grau histológico do tumor, idade do paciente e margens cirúrgicas livres. Esses tumores apresentam pouca resposta à radio e quimioterapia. O tratamento ideal consiste em ressecção tumoral em bloco, condição particularmente difícil em se tratando de tumores causando compressão medular, devido à localização da lesão, comprometimento da estabilidade axial e necessidade da manutenção ou recuperação da integridade da função neurológica do paciente. Relatamos dois casos de condrossarcomas causando compressão medular, um na coluna cervical e outro na torácica, submetidos a cirurgia com esvaziamento tumoral seguido de remo

  8. Significance of Sox9 mRNA in dedifferentiation process of chondrosarcoma%Sox9 mRNA在软骨肉瘤去分化过程中的意义

    Institute of Scientific and Technical Information of China (English)

    刘百奇; 孟祥龙; 张昊; 车德馨; 王国文; 韩金刚

    2016-01-01

    目的:探讨Sox9 mRNA在软骨肉瘤去分化过程中所起的作用,并分析其与各临床病理参数的关系。方法利用免疫组织化学方法检测Sox9 mRNA在人普通软骨肉瘤与去分化软骨肉瘤间的差异表达及其与临床病理参数的关系。结果与正常软骨组织比较,Sox9 mRNA 在普通软骨肉瘤中表达上调,在去分化软骨肉瘤中表达下调;Sox9 mRNA在软骨肉瘤各分级之间表达水平比较,差异有统计学意义(P0.05),而与肺转移、有无复发有关,且肺转移及复发者阳性率明显高于无肺转移、无复发者,差异有统计学意义(P0.05),while had relation with the lung metastasis and recurrence,moreover the positive rate in the patients with lung metastasis and recurrence was significantly higher than that in the patients without lung metastasis and recurrence ,the difference was statistically significant(P<0.01). Conclusion Sox9 mRNA is related with occurrence,progression,metastasis and prognosis of chondrosarcoma.

  9. Atypical giant chondroblastoma mimicking a chondrosarcoma.

    Science.gov (United States)

    Dhanda, Sunita; Menon, Santosh; Gulia, Ashish

    2015-01-01

    Chondroblastoma is a rare, benign tumor derived from chondroblasts, commonly presenting in the second decade of life. It is usually found in the epiphysis or apophysis of long bones; however, it may rarely affect flat bones like scapula. Occasionally a histologically typical chondroblastoma may exhibit an aggressive behavior that is not normally associated with benign tumors such as a large size, pulmonary metastases, joint and soft-tissue infiltration and local recurrence. We present a case report of a patient with chondroblastoma showing atypical radiological presentation and non-concordance with age.

  10. Chondrosarcoma in Childhood: The Radiologic and Clinical Conundrum

    Science.gov (United States)

    2012-12-01

    internal matrix. The differential included a simple bone cyst, intraosseous lipoma, enchondroma or sequestrae from infection or osteoid osteoma...sensitive extension of the radiographic findings. CT accurately defines the intraosseous portion of the tumor, better detects subtle tumor matrix

  11. Fluoroquinolone's effect on growth of human chondrocytes and chondrosarcomas. In vitro and in vivo correlation

    DEFF Research Database (Denmark)

    Multhaupt, H A; Alvarez, J C; Rafferty, P A

    2001-01-01

    droplets, rough endoplasmic reticulum, and prominent Golgi apparatus; and a proteoglycan layer surrounding the cells. With prolonged ciprofloxacin treatment and with increased doses, there was an increase in dilated rough endoplasmic reticulum, the appearance of phagosomes, and disintegrated bundles...

  12. A fatal case of primary cardiac chondrosarcoma presenting with amaurosis fugax

    DEFF Research Database (Denmark)

    Sundbøll, Jens; Hansson, Nils Henrik Stubkjær; Baerentzen, Steen

    2015-01-01

    A 64-year-old previously healthy woman consulted her general practitioner because of recurrent episodes of right-sided monocular transient visual loss (ie, amaurosis fugax). At first, these symptoms were followed over time, but as the attacks worsened, and were accompanied by dizziness and general...

  13. L’implication du système immunitaire dans la progression et la réponse thérapeutique du chondrosarcome : Est-ce que l’immunothérapie peut jouer un rôle dans le traitement du chondrosarcome ?

    OpenAIRE

    Simard, François

    2016-01-01

    Chondrosarcoma is highly resistant to chemotherapy and radiation and there is an urgent need in developing new therapeutic strategies for this malignancy; among these, some immunotherapy approaches could be of great interest. We are currently investigating the immune system implication in chondrosarcoma progression and therapeutic response both on human samples and in rat chondrosarcoma model (SRC). In human and rat chondrosarcoma, immune infiltrates composed of lymphocytes and macrophages we...

  14. Trial of Dasatinib in Advanced Sarcomas

    Science.gov (United States)

    2016-10-12

    Rhabdomyosarcoma; Malignant Peripheral Nerve Sheath Tumors; Chondrosarcoma; Sarcoma, Ewing's; Sarcoma, Alveolar Soft Part; Chordoma; Epithelioid Sarcoma; Giant Cell Tumor of Bone; Hemangiopericytoma; Gastrointestinal Stromal Tumor (GIST)

  15. Analysis of angiogenic factors and cyclooxygenase-2 expression in cartilaginous tumors: clinical and histological correlation

    Directory of Open Access Journals (Sweden)

    Francisco Fontes Cintra

    2011-01-01

    Full Text Available OBJECTIVES: To study the role of angiogenesis and cyclooxygenase-2 expression in cartilaginous tumors and correlate these factors with prognosis. INTRODUCTION: For chondrosarcoma, the histological grade is the current standard for predicting tumor outcome. However, a low-grade chondrosarcoma can follow an aggressive course-as monitored by sequential imaging techniques-even when it is histologically indistinguishable from an enchondroma. Therefore, additional tools are needed to help identify the biological potential of these tumors. The degree of angiogenesis that is induced by the tumor could assist in this task. Angiogenesis can be quantified by measuring the expression of vascular endothelial growth factor and CD34, and cyclooxygenase-2 can induce angiogenesis by stimulating the production of proangiogenic factors. METHODS: In total, 21 enchondromas and 58 conventional chondrosarcomas were studied by examining the clinical and histopathological findings in conjunction with the immunostaining markers of angiogenesis and cyclooxygenase- 2 expression. RESULTS: The significant variables that were associated with poor outcome were 1 higher-grade chondrosarcomas, 2 tumors that developed in flat bones, and 3 over-expression of CD34 (with a median count that was higher than 5.9 vessels in 5 high power fields. Moreover, CD34 expression (measured using the Chalkley method revealed significantly higher microvessel density in flat bone chondrosarcomas. DISCUSSION: Previous studies have shown a positive correlation between Chalkley microvessel density and histological grade; however, in our sample, we found that the former is predictive of the outcome. Chondrosarcomas in flat bones have been shown to correlate with a poor prognosis. We also found that CD34 microvessel density values were significantly higher in flat-bone chondrosarcomas. This could explain-at least in part-the more aggressive biological course that is taken by these tumors. CONCLUSIONS

  16. Chondroblastoma with Secondary Aneurysmal Bone Cyst

    Science.gov (United States)

    2008-02-01

    dysplasia, chondroblastoma, chondromyxoid fibroma , osteochondroma, giant cell tumors, or enchondroma. Chondrosarcoma, osteoblastoma–aggressive variant and...mass appears lobulated with scalloped cortical margins. Calcifications and cysts are often described.2,3,5 Chondromyxoid fibromas present in the

  17. Effects of recombinant adenovirus Ad-PERK siRNA on the proliferation and apoptosis of human chondrosarcoma SW1353 cells in endoplasmic reticulum stress%内质网应激时重组腺病毒Ad-PERK siRNA对人软骨肉瘤SW1353细胞增殖和凋亡的影响

    Institute of Scientific and Technical Information of China (English)

    李美玲; 夏飞; 邓莉; 伍志盟; 郭风劲

    2014-01-01

    目的:探讨靶向蛋白激酶样内质网激酶(protein kinase-like endoplasmic reticulum kinase,PERK]基因的小干扰RNA (small interfering RNA,siRNA)重组腺病毒在内质网应激(endoplasmic reticulum stress,ERS)时对人软骨肉瘤SW1353细胞增殖和凋亡的影响.方法:构建靶向PERK基因的siRNA重组腺病毒Ad-PERKsiRNA,并将其感染SW1353细胞后,RT-PCR和蛋白质印迹法检测SW1353细胞中PERK mRNA和蛋白的表达.应用衣霉素(tunicamycin,TM)建立ERS模型;在ERS状态下,MTT法检测Ad-PERKsiRNA感染后SW1353细胞的增殖情况,FCM法检测感染后SW1353细胞的细胞周期和细胞凋亡率,透射电子显微镜下观察感染后SW1353细胞超微结构的变化,蛋白质印迹法检测感染后SW1353细胞中cleaved caspase 3、caspase 12、CCAAT/增强子结合蛋白同源蛋白(CCAAT/enhancer-binding protein homologous protein,CHOP)和磷酸化c-Jun氨基端激酶(phospho-c-Jun-N-terminal kinase,p-JNK)蛋白的表达.结果:成功构建获得重组腺病毒AdPERKsiRNA.Ad-PERK siRNA感染后,SW1353细胞中PERK mRNA和蛋白的表达水平下调(P<0.05).在ERS状态下,Ad-PERKsiRNA可促进SW1353细胞的增殖(P<0.05),Ad-PERK siRNA感染组S期细胞所占比例高于感染空载体腺病毒Ad-RFP的阴性对照组和未感染的空白对照组(P<0.05),Ad-PERK siRNA感染组SW1353细胞的凋亡率低于阴性对照组和空白对照组(P<0.05),透射电子显微镜下观察发现阴性对照组和空白对照组SW1353细胞存在明显的凋亡,Ad-PERK siRNA感染组SW1353细胞中cleaved caspase 3、caspase 12、CHOP和p-JNK蛋白的表达水平均明显低于阴性对照组和空白对照组(P<0.05).结论:重组腺病毒Ad-PERKsiRNA可以促进ERS状态下SW1353细胞的增殖,并抑制其凋亡.

  18. 一氧化氮合成酶、环氧化酶-2、硝基酪氨酸及血管生成与软骨肉瘤预后的相关性研究%Nitric Oxide Synthases,Cyclooxygenase-2,Nitrotyrosine,and Angiogenesis in Chondrosarcoma and Their Relation to Prognosis

    Institute of Scientific and Technical Information of China (English)

    Suely Akiko Nakagawa; 张伟滨; Ademar Lopes; André Lopes de Carvalho; Benedito Mauro Rossi; Isabela Werneck da Cunha; Fernando Augusto Soares; Wu Tu Chung; Lucíola Assun(c)(a)o Alves; 刘沛宜

    2010-01-01

    @@ 背景:免疫组织化学标记物有助于肿瘤诊断及预后评估.通过对硝基酪氨酸分析及采用一氧化氮合成酶1(NOS1)、一氧化氮合成酶2(NOS2)、一氧化氮合成酶3(NOS3)检测分析可了解一氧化氮(NO)与肿瘤的相关性.NO与超氧阴离子反应产生的过氧亚硝酸对基因有毒性作用,过氧亚硝酸传递一个硝基基团到酪氨酸的苯环上形成硝基酪氨酸.

  19. 采用病灶内刮除石碳酸、乙醇灭活及骨移植技术治疗长骨低度恶软骨肉瘤%Low-Grade Chondrosarcoma of Long Bones Treated with Intralesional Curettage Followed by Application of Phenol, Ethanol, and Bone-Grafting

    Institute of Scientific and Technical Information of China (English)

    Suzan H.M.Verdegaal; Hugo F.G.Brouwers; Erik W.van Zwet; Pancras C.W.Hogendoorn; Antonie H.M.Taminiau; 张伟滨

    2012-01-01

    背景:低度恶性的软骨病变常采用病灶内刮除及局部辅助治疗.由于该疾病诊断及治疗所涵盖的范围十分广泛,因而对于长骨的Ⅰ级中央型软骨肉瘤,采用局部石碳酸辅助治疗的有效性,还缺乏足够的认识.方法:对1994至2005年间采用病灶内刮除,并应用石碳酸、乙醇灭活及骨移植技术进行治疗的85例患者,进行回顾性研究,评估这些患者的临床及肿瘤学疗效.纳入标准为组织学确诊为Ⅰ级中央型软骨肉瘤且病灶位于长骨内.手术时的平均年龄为47.5岁(范围,15.6~72.3岁).平均随访时间为6.8年(范围,0.2~14.1年).利用常规X线及增强磁共振(Gd-MRI),对患者进行定期评估.如果MRI上发现可疑病灶,患者将进行再次治疗干预.根据复发病灶的大小,采用活组织检查,并行局部放疗(病灶< 10mm)或再次刮除(病灶≥10 mm).结果:85例患者中,有11例患者因随访期间的增强磁共振提示可疑病灶,而进行再次手术治疗.这11例患者中有5例患者经组织学确诊为局部复发[复发率为5.9%,(95%可信区间,0.9%~10.9%)],且全部为Ⅰ级软骨肉瘤.常见并发症包括1例表面感染及2例术后6周内发生股骨骨折.结论:本回顾性案例分析未设置对照组,因而存在局限性,但对长骨低度恶性的软骨肉瘤采用病灶内刮除术并应用石碳酸作为局部辅助治疗的方法是安全且有效的,术后平均生存时间为6.8年,复发率<6%.

  20. BMP2 mRNA 在颌骨骨肉瘤及软骨肉瘤中的扩增及其对肿瘤增殖活性的影响%Expression of BMP2 mRNA in osteosarcoma and chondrosarcoma of jaw and it's effect on tumor proliferating activity

    Institute of Scientific and Technical Information of China (English)

    岳文; 杨连甲; 金岩; 董绍忠

    2000-01-01

    目的研究BMP2 mRNA 在颌骨骨肉瘤及软骨肉瘤中的扩增及其对肿瘤预后和增殖活性的影响.方法应用 BMP 2 的 cDNA探针,原位杂交检测 mRNA 的扩增.结果 32例标本中有2 1例表达BMP2 mRNA,有BMP2 mRNA表达的病例更容易发生淋巴结转移,且有较高增殖活性. 结论 BMP2 可能在骨肿瘤的发生和发展过程中起一定作用,可以作为估计骨肉瘤预后的参考指标.

  1. Markers aiding the diagnosis of chondroid tumors: an immunohistochemical study including osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit), and YKL-40

    DEFF Research Database (Denmark)

    Daugaard, Søren; Christensen, Lise H; Høgdall, Estrid

    2009-01-01

    (s) for the different subgroups. Archival material from three extraskeletal myxoid chondrosarcomas, five chordomas, five chondromyxoid fibromas, five chondroblastomas and 25 chondrosarcomas was stained with antibodies against osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit) and YKL......) and extraskeletal myxoid chondrosarcomas (n=3) were positive for bcl-2. In contrast to all other tumors, two of three extraskeletal myxoid chondrosarcomas were also positive for CD17 and negative for osteonectin, cox-2, mdm-2 and actin. All five chordomas were negative for D2-40 and positive for mdm-2 and YKL-40....... The diagnosis of chondrosarcoma may be aided by its positivity for D2-40 and YKL-40 and its lack of reactivity for actin and CD117. This should be seen in the light of no reaction for D2-40 in chordomas and a corresponding lack of reaction for osteonectin, cox-2, mdm-2 and actin in extraskeletal myxoid...

  2. Transtornos factícios por procuração: discussão de um caso Factitious disorders by proxy: discussion of one case

    Directory of Open Access Journals (Sweden)

    Nelson Caldas

    2001-09-01

    Full Text Available Condrossarcoma é o sarcoma mais frequente da laringe. Sua incidência é maior na cartilagem cricóide do que nas outras cartilagens da laringe, sendo raro que ele se origine na epiglote. Relatamos no texto um caso de condrossarcoma originado na epiglote, no qual foi realizada laringectomia subtotal com circo-hioidopexia ¾e realizamos revisão da literatura.Chondrosarcoma is the most frequent sarcoma of the larynx. It is more prevalent in the cricoid and less prevalent in the other laryngeal cartilages. Chondrosarcoma is rarely located in the epiglottis. We reported a case of epiglottis chondrosarcoma that was treated with a supracricoid laryngectomy with cricohyoidopexy.

  3. Sarcomatous transformation of osteochondroma of the coronoid process forming pseudoarthrosis with zygomatic arch mistaken for Jacob disease.

    Science.gov (United States)

    Jha, Abhishek; Gupta, Prakhar; Khalid, Mohd; Naseem, Iqbal; Gupta, Gagan

    2014-01-01

    Osteochondromas are the most common benign osseous neoplasms, with a propensity to involve long bones. The involvement of the coronoid process is extremely rare, and osteochondromas of the coronoid process may form pseudoarticulations with the zygomatic arch when large, an entity that is extremely rare and termed as Jacob disease. Sarcomatous degeneration of osteochondroma of the coronoid process is an extremely rare entity and has not been described to date. Only 1 case of primary mesenchymal chondrosarcoma has been described. We present a case of a 24-year-old man with a chondrosarcoma of the coronoid process, who was wrongly diagnosed with Jacob disease on the basis of radiologic findings.

  4. Osteosarcoma of the sternum

    NARCIS (Netherlands)

    Douglas, YL; Meuzelaar, KJ; vanderLei, B; Pras, B; Hoekstra, HJ

    1997-01-01

    Primary malignant sternal tumours are very rare. The most common malignant sternal tumour is a chondrosarcoma. Until now, controversies in the management of malignant sternal tumours were mainly caused by limited clinical experience. However, treatment of malignant sternal tumours should not differ

  5. MFH Mimic in Breast: A High-Grade Malignant Phyllodes Tumor

    OpenAIRE

    Hemalatha, A. L.; V. Sumana Sindhuram; Asha, U.

    2012-01-01

    Malignant phyllodes tumor is usually diagnosed by the presence of benign duct-like epithelium and malignant mesenchymal tissue. In addition to the usual fibrosarcomatous features, the mesenchymal component may show areas resembling osteogenic sarcoma, chondrosarcoma, liposarcoma, leiomyosarcoma, rhabdomyosarcoma, malignant mesenchymoma, and, very rarely, malignant fibrous histiocytoma. We present one such rare case of malignant phyllodes tumor with malignant fibrous histiocytoma-like stromal ...

  6. THE USE OF THE SADDLE PROSTHESIS FOR RECONSTRUCTION OF THE HIP-JOINT AFTER TUMOR RESECTION OF THE PELVIS

    NARCIS (Netherlands)

    VANDERLEI, B; HOEKSTRA, HJ; VETH, RPH; HAM, SJ; OLDHOFF, J; KOOPS, HS

    1992-01-01

    Reconstruction of the hip joint by a saddle prosthesis after excision of a malignant pelvic tumor is a relatively new method, which thus far has been mainly used for revision of infected hip arthroplasties. One patient with a metastatic cystosarcoma phyllodes and one patient with a chondrosarcoma of

  7. Liposarcoma of the thigh with mixed calcification and ossification

    Directory of Open Access Journals (Sweden)

    Jeremy R. Child, MD

    2016-09-01

    Full Text Available Liposarcoma is one of the most common soft-tissue sarcomas. Calcification and ossification can occur in liposarcoma; however, the presence of both ossification and calcification is a very rare entity. We present a case of a partially calcified and ossified dedifferentiated liposarcoma of the thigh in a 76-year-old woman, which contained heterologous elements of chondrosarcoma and rhabdomyosarcoma.

  8. [Subperiosteal (juxtacortical) chondroma of the hand. Apropos of 2 cases. Review of the literature].

    Science.gov (United States)

    Baudrillard, J C; Lerais, J M; Schernberg, F; Gillier, P; Toubas, O; Durot, J F

    1985-03-01

    Subperiosteal chondroma (juxta-cortical) is unusual benign cartilaginous neoplasm with characteristic radiographic features. We report two cases occurring in uncommon location: the hand. Radiologic differential diagnosis with others bone tumors is easy but it could be difficult with some soft-tissue tumors, especially chondrosarcoma and extraarticular chondromatosis.

  9. [The combination treatment of malignant bone tumors using fast neutrons].

    Science.gov (United States)

    Chernichenko, V A; Tolstopiatov, B A; Konovalenko, V F; Monich, A Iu; Palivets, A Iu

    1990-01-01

    The study deals with results of a clinical trial evaluating treatment efficacy of a 6 MeV neutron beam produced by Y-120 cyclotron (Kiev). Procedures of preoperative radiotherapy and radical treatment are discussed. Radiotherapy was administered to 52 patients suffering chondrosarcoma (30 cases), osteogenic sarcoma (15) or chordoma (7). Combined treatment (radiation + surgery) was given to 22 patients whereas neutron beam therapy--to 30. All patients with osteogenic sarcoma received adjuvant combination chemotherapy. Three-year survival rate was compared to that observed in controls in whom combined treatment had included gamma-therapy. A significant increase in three-year survival rate was observed for osteogenic sarcoma and chordoma whereas for chondrosarcoma the improvement in survival proved insignificant. The use of fast neutrons in combined treatment of bone tumors was considered promising.

  10. Proton therapy for tumors of the skull base

    Energy Technology Data Exchange (ETDEWEB)

    Munzenrider, J.E.; Liebsch, N.J. [Dept. of Radiation Oncology, Harvard Univ. Medical School, Boston, MA (United States)

    1999-06-01

    Charged particle beams are ideal for treating skull base and cervical spine tumors: dose can be focused in the target, while achieving significant sparing of the brain, brain stem, cervical cord, and optic nerves and chiasm. For skull base tumors, 10-year local control rates with combined proton-photon therapy are highest for chondrosarcomas, intermediate for male chordomas, and lowest for female chordomas (94%, 65%, and 42%, respectively). For cervical spine tumors, 10-year local control rates are not significantly different for chordomas and chondrosarcomas (54% and 48%, respectively), nor is there any difference in local control between males and females. Observed treatment-related morbidity has been judged acceptable, in view of the major morbidity and mortality which accompany uncontrolled tumor growth. (orig.)

  11. Exuberant callus formation misdiagnosed as osteosarcoma: a case report

    Institute of Scientific and Technical Information of China (English)

    Fariba Binesh; Mohammad Sobhan; Reza Nafisi Moghadam; Ali Akhavan

    2013-01-01

    Reactive lesions of bone and soft tissue can appear alarming on histologic examination because they are often cellular and have atypical cytologic features, such as distinct nucleoli, mild hyperchromasia, and mitotic activity. Reactive lesions of bone and periosteum also produce bone and cartilage matrix, resulting in confusion with osteosarcoma or chon-drosarcoma. Careful attention to key cytomorphological features such as the pattern of bone formation, uniform appearance of cells, and absence of atypical mitoses should help identify the reactive nature of a lesion. Correlation with clinical and radiological findings is also imperative to avoid misclassification of the tumor because reactive lesions often arise at sites where osteosarcoma and chondrosarcoma are rare (eg, the hand) and lack aggressive radiological features. Here we pres-ent a case of exuberant callus formation after avulsion fracture of tibia in a three year-old Iranian girl which misdiagnosed as osteosarcoma.

  12. Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma

    Science.gov (United States)

    2016-06-09

    Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Unclassified Pleomorphic Sarcoma; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Conjunctival Kaposi Sarcoma; Dermatofibrosarcoma Protuberans; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult Unclassified Pleomorphic Sarcoma of Bone; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Kaposi Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Corpus Sarcoma; Small Intestine Leiomyosarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Unclassified Pleomorphic Sarcoma of Bone

  13. First realisation of a labelling kit of N.T.P. 15-5 ligand by {sup 99m}Tc in view of a clinical application in cartilage functional imaging; Premiere realisation d'une trousse de marquage du ligand NTP 15-5 par le 99mTc en vue d'une application clinique en imagerie fonctionnelle du cartilage

    Energy Technology Data Exchange (ETDEWEB)

    Miot-Noirault, E.; Cachin, F.; Vidal, A.; Auzeloux, P.; Chezal, J.M.; Gaumet, V.; Askienazy, S. [Inserm, EA4231, UMR 990, 63 - Clermont-Ferrand (France); Guenu, S. [UFR de pharmacie, laboratoire de chimie analytique, 63 - Clermont-Ferrand (France); Askienazy, S. [Laboratoires Cyclopharma, 63 - Saint-Beauzire (France)

    2010-07-01

    We are working on a SPECT tracer for functional imaging of articular cartilage, the {sup 99m}Tc-NTP 15-5. This molecule has its application in degenerative diseases of cartilage (arthrosis, arthritis and chondrosarcoma). Excellent reports of cartilage versus tissues fixing ratios are obtained in different animal models as well as human anatomical parts. For clinical application, we present the development of a labelling kit by the technetium of the ligand NTP 15-5. (N.C.)

  14. Multicentric malignant transformation of multiple exostoses

    Energy Technology Data Exchange (ETDEWEB)

    Ozaki, T.; Hillmann, A.; Winkelmann, W. [Department of Orthopaedics, Westfaelische Wilhelms-University, Albert-Schweitzer Strasse 33, D-48129 Muenster (Germany); Blasius, S. [Department of Pathology, Westfaelische Wilhelms-University, Albert-Schweitzer Strasse 33, D-48129 Muenster (Germany); Link, T. [Department of Radiology, Westfaelische Wilhelms-University, Albert-Schweitzer Strasse 33, D-48129 Muenster (Germany)

    1998-04-01

    We treated a patient with large multiple chondrosarcomas derived from multiple cartilaginous exostoses. One sarcoma originated in the left pubic bone and the other sarcoma in the posterior aspect of the greater trochanter of the left femur. Thirty months after hindquarter amputation, the patient is alive without relapse. This is the first report of a patient with synchronous multiple malignant transformation of multiple cartilaginous exostoses. (orig.) With 7 figs., 14 refs.

  15. Chondromyxoid fibroma of the second rib.

    Science.gov (United States)

    Long, Kristin L; Absher, Kimberly J; Draus, John M

    2013-06-01

    Chondromyxoid fibromas are benign tumors which are found most frequently in the metaphyses of long bones. They comprise less than 1% of primary bone neoplasms and display a hypermetabolic appearance on PET imaging. Oftentimes, they are misdiagnosed as chondrosarcomas and are excised due to concern for malignancy. We present a case of a condromyxoid fibroma originating from the second rib of a 15-year-old girl.

  16. Congenital chondromyxoid fibroma of the ethmoid: case report

    Energy Technology Data Exchange (ETDEWEB)

    Mendoza, M.; Gonzalez, I.; Aperribay, M.; Nogues, A. [Servicio de Radiologia, Hospital Ntra. Sra. de Aranzazu/Arantzazuko Amaren Ospitalea, Donostia-San Sebastian, Gipuzkoa (Spain); Hermosa, J.R. [Servicio de Anatomia Patologica, Hospital Ntra. Sra. de Aranzazu/Arantzazuko Amaren Ospitalea, Donostia-San Sebastian, Gipuzkoa (Spain)

    1998-05-01

    This report describes a congenital case of chondromyxoid fibroma (CMF) arising from the ethmoid bone. We believe it to be the second case of congenital CMF that has been documented, and the third case of CMF arising in the ethmoid. We describe the radiographic features of this rare entity and indicate the necessity for careful correlation between radiographic and histological findings to distinguish CMF from chondrosarcoma. (orig.) With 3 figs., 5 refs.

  17. TUMOR CONTAMINATION IN THE BIOPSY PATH OF PRIMARY MALIGNANT BONE TUMORS

    OpenAIRE

    Oliveira,Marcelo Parente; Lima, Pablo Moura de Andrade; Mello,Roberto José Vieira de

    2015-01-01

    Objective: To study factors possibly associated with tumor contamination in the biopsy path of primary malignant bone tumors. Method: Thirty-five patients who underwent surgical treatment with diagnoses of osteosarcoma, Ewing's tumor and chondrosarcoma were studied retrospectively. The sample was analyzed to characterize the biopsy technique used, histological type of the tumor, neoadjuvant chemotherapy used, local recurrences and tumor contamination in the biopsy path. Results: Among the 35 ...

  18. MFH Mimic in Breast: A High-Grade Malignant Phyllodes Tumor

    Directory of Open Access Journals (Sweden)

    A. L. Hemalatha

    2012-01-01

    Full Text Available Malignant phyllodes tumor is usually diagnosed by the presence of benign duct-like epithelium and malignant mesenchymal tissue. In addition to the usual fibrosarcomatous features, the mesenchymal component may show areas resembling osteogenic sarcoma, chondrosarcoma, liposarcoma, leiomyosarcoma, rhabdomyosarcoma, malignant mesenchymoma, and, very rarely, malignant fibrous histiocytoma. We present one such rare case of malignant phyllodes tumor with malignant fibrous histiocytoma-like stromal differentiation.

  19. Proximal femoral reconstruction with custom mega prosthesis

    OpenAIRE

    2003-01-01

    Seventy-seven patients with proximal femoral tumours underwent limb salvage surgery with custom mega prostheses between July 1989 and January 2002. We describe 44 cases with a mean follow-up of 57.8 months. Forty-one patients presented with malignant neoplasms of which 11 were malignant giant cell tumours, eight were chondrosarcoma and five were metastases. Wide margins were achieved for all malignant tumours, and marginal resection was done for all benign and metastatic lesions. Six patients...

  20. Prevalence of cartilaginous tumours as an incidental finding on MRI of the knee

    Energy Technology Data Exchange (ETDEWEB)

    Stomp, Wouter; Reijnierse, Monique; Bloem, Johan L. [Leiden University Medical Center, Department of Radiology, Leiden (Netherlands); Kloppenburg, Margreet [Leiden University Medical Center, Department of Rheumatology, Leiden (Netherlands); Leiden University Medical Center, Department of Clinical Epidemiology, Leiden (Netherlands); Mutsert, Renee de; Heijer, Martin den [Leiden University Medical Center, Department of Clinical Epidemiology, Leiden (Netherlands); Bovee, Judith V.M.G. [Leiden University Medical Center, Department of Pathology, Leiden (Netherlands); Collaboration: NEO study group

    2015-12-15

    The purpose was to determine prevalence of enchondromas and atypical cartilaginous tumour/chondrosarcoma grade 1 (ACT/CS1) of the knee on MRI in a large cohort study, namely the Netherlands Epidemiology of Obesity (NEO) study. Participants aged 45 to 65 years were prospectively included, oversampling overweight and obese persons. Within a subgroup of participants, MRI of the right knee was performed and screened for incidental cartilaginous tumours, as defined by their characteristic location and appearance. Forty-nine cartilaginous tumours were observed in 44 out of 1285 participants (estimated population prevalence 2.8 %, 95 % CI 2.0-4.0 %). Mean largest tumour diameter was 12 mm (range 2-31 mm). Eight participants with a tumour larger than 20 mm or a tumour with aggressive features were referred to rule out low-grade chondrosarcoma. One was lost to follow-up, three had histologically proven ACT/CS1 and four had dynamic contrast MRI findings consistent with benign enchondroma. Incidental cartilaginous tumours were relatively common on knee MRI and may be regarded as a normal concurrent finding. However, more tumours than expected were ACT/CS1. Because further examination was performed only when suspicion of chondrosarcoma was high, the actual prevalence might be even higher. (orig.)

  1. Extreme lateral transcondylar approach to the skull base.

    Directory of Open Access Journals (Sweden)

    Banerji D

    1999-01-01

    Full Text Available In this study, the authors present their experience of using extreme later transcondylar approach (ELTC for treating 7 patients with lesions in the anterolateral foramen magnum, upper cervical spine and cerebellopontine angle reaching upto jugular foramen. The tumours included meningiomas, neurofibromas (2 cases each, chondrosarcoma, epidermoid and aneurysmal bone cyst (one case each. The approach was used alone, in combination with retrolabyrinthine presigmoid approach in a patient with lower cranial nerve neurofibroma extending extracranially through the jugular foramen, or in combination with partial C1-C3 laminectomy in two patients with meningiomas situated anterolateral to the cord from the foramen magnum to C3. In two patients with extradural vertebral artery (VA entrapment by a chondrosarcoma and aneurysmal bone cyst respectively, the vertebral artery was ligated distal to the tumour. The tumours were totally excised in five cases and partially in two. There was no preoperative mortality. The major complications included cerebrospinal fluid leak from the wound (3 cases and increase in lower cranial nerve paresis (2 cases. At follow up, ranging from 6 months to 2 years, 5 patients showed no tumour recurrence. There was improvement in neurological status. One patient, with a partially excised aneurysmal bone cyst, showed no added deficits or increase in the tumour size. However, there was a massive regrowth in the patient with chondrosarcoma after 6 months. This technique provided a wide surgical exposure with direct visualization of the tumour-anterior cord interface, early proximal control of the VA and preservation of lower cranial nerves.

  2. Multiple osteochondromas

    Directory of Open Access Journals (Sweden)

    Bovée Judith VMG

    2008-02-01

    Full Text Available Abstract Multiple osteochondromas (MO is characterised by development of two or more cartilage capped bony outgrowths (osteochondromas of the long bones. The prevalence is estimated at 1:50,000, and it seems to be higher in males (male-to-female ratio 1.5:1. Osteochondromas develop and increase in size in the first decade of life, ceasing to grow when the growth plates close at puberty. They are pedunculated or sessile (broad base and can vary widely in size. The number of osteochondromas may vary significantly within and between families, the mean number of locations is 15–18. The majority are asymptomatic and located in bones that develop from cartilage, especially the long bones of the extremities, predominantly around the knee. The facial bones are not affected. Osteochondromas may cause pain, functional problems and deformities, especially of the forearm, that may be reason for surgical removal. The most important complication is malignant transformation of osteochondroma towards secondary peripheral chondrosarcoma, which is estimated to occur in 0.5–5%. MO is an autosomal dominant disorder and is genetically heterogeneous. In almost 90% of MO patients germline mutations in the tumour suppressor genes EXT1 or EXT2 are found. The EXT genes encode glycosyltransferases, catalyzing heparan sulphate polymerization. The diagnosis is based on radiological and clinical documentation, supplemented with, if available, histological evaluation of osteochondromas. If the exact mutation is known antenatal diagnosis is technically possible. MO should be distinguished from metachondromatosis, dysplasia epiphysealis hemimelica and Ollier disease. Osteochondromas are benign lesions and do not affect life expectancy. Management includes removal of osteochondromas when they give complaints. Removed osteochondromas should be examined for malignant transformation towards secondary peripheral chondrosarcoma. Patients should be well instructed and regular

  3. Components of the RANK/RANKL/OPG system, IL-6, IL-8, IL-16, MMP-2, and calcitonin in the sera of patients with bone tumors.

    Science.gov (United States)

    Kushlinskii, N E; Timofeev, Yu S; Solov'ev, Yu N; Gerstein, E S; Lyubimova, N V; Bulycheva, I V

    2014-08-01

    Serum levels of sRANKL, RANK, OPG, IL-8, IL-6, IL-16, MMP-2, and calcitonin were measured by ELISA in patients with malignant, borderline, and benign bone tumors and in healthy individuals (control). Serum levels of RANK, OPG, IL-8, IL-6, and the OPG/sRANKL ratio were significantly higher, while the level of MMP-2 was significantly lower in patients with bone tumors than in controls. Serum concentration of IL-16 in osteosarcoma patients was significantly lower than in chondrosarcoma patients. No significant differences between bone sarcomas of different differentiation were detected for any of the studied markers. Calcitonin level depended on the tumor location and type.

  4. [Treatment with charged particles beams: hadrontherapy part I: physical basis and clinical experience of treatment with protons].

    Science.gov (United States)

    Noël, G; Feuvret, L; Ferrand, R; Mazeron, J-J

    2003-10-01

    Protons have physical characteristics, which differ from those of photons used in conventional radiotherapy. Better shielding of critical organs is obtained by using their particular ballistic (Bragg peak and lateral narrow penumbra). Some indications as ocular melanoma, chordoma and chondrosarcoma of the base of skull are now strongly accepted by the radiation oncologist community. Others are still in evaluation: meningioma, locally advanced nasopharynx tumor and paediatric tumors. The aim of this review is to present the clinical results of a technic which seems "confidential" because of the rarety and the cost of equipments.

  5. Biphasic Malignant Pleural Mesothelioma Masquerading as a Primary Skeletal Tumor

    Science.gov (United States)

    Diacovo, Maria Julia

    2016-01-01

    Biphasic malignant pleural mesothelioma is a rare malignant tumor, usually presenting as a pleural-based mass in a patient with history of chronic asbestos exposure. We herein report a case of a 41-year-old man who presented with chest pain and had a chest computed tomography (CT) scan suggestive of a primary skeletal tumor originating from the ribs (chondrosarcoma or osteosarcoma), with no history of asbestos exposure. CT-guided core needle biopsies were diagnosed as malignant sarcomatoid mesothelioma. Surgical resection and chest wall reconstruction were performed, confirming the diagnosis and revealing a secondary histologic component (epithelioid), supporting the diagnosis of biphasic malignant mesothelioma. PMID:27660729

  6. An unusual case of lumbar paravertebral miositis ossificans mimicking muscular skeletal tumor.

    Science.gov (United States)

    Zoccali, C; Chichierchia, G; Covello, R

    2013-12-01

    Several lesions have clinical and radiological characteristics mimicking muscular skeletal tumor. Myositis ossificans usually presents a typical pattern making biopsy unnecessary; nevertheless, in rare cases, neoplasm must be ruled out. Biopsy is often sufficient to allow a diagnosis and a correct related treatment, but, unfortunately, sometimes it may lead to erroneous treatment. We report an unusual case of a lumbar paravertebral mass that had an MRI aspect similar to a chondrosarcoma, a histology pattern based on biopsy compatible with neurinoma and a definitive diagnosis of myosistis ossificans.

  7. Magnetic resonance imaging appearance at 1. 5 tesla of cartilaginous tumors involving the epiphysis

    Energy Technology Data Exchange (ETDEWEB)

    Fobben, E.S.; Dalinka, M.K.; Schiebler, M.L.; Burk, D.L.; Kressel, H.Y.; Fallon, M.D.; Schmidt, R.G.

    1987-11-01

    Three cases of lytic, calcified epiphyseal lesions with plain film and computed tomography features suggestive of chondroblastoma were imaged by magnetic resonance imaging. Histopathologic correlation was obtained in each case. Two cases of chondroblastoma showed low signal intensity on both short (TR600/TE20ms) and long (TR2500/TE80ms) spin echo (SE) images. The third case, a clear cell chondrosarcoma, demonstrated increased signal intensity on moderately T2 weighted (TR2500/TE40ms) images. These findings suggest that magnetic resonance imaging may be helpful in distinguishing these lesions. (orig.)

  8. [Plasmacytomas of the upper respiratory tract].

    Science.gov (United States)

    Gegova, A; Kunev, K; Popkhristova, E; Terziev, I

    1995-01-01

    Nine cases of plasmocytoma of the upper airways in the current biopsy material are studied over a three-year period (1991-1993). Initially, all nine cases are clinically diagnosed as neoplasms: hemangiofibroma--one, chondrosarcoma--one, suspected carcinoma--five, and unspecified diagnosis--two. "Plasmocytoma" diagnosis is made on the ground of histological investigation of the material, supported histochemically and electron-microscopically. Some diagnostic problems (differential diagnosis inclusive) are discussed, as well as issues relating to the clinical and morphological evolution of the disease, and predilected gender and age groups.

  9. Cartilage homeoprotein 1, a homeoprotein selectively expressed in chondrocytes.

    OpenAIRE

    Zhao, G. Q.; Zhou, X.; Eberspaecher, H; Solursh, M; de Crombrugghe, B

    1993-01-01

    We identified a rat cDNA that encodes cartilage homeoprotein 1 (Cart-1). The deduced amino acid sequence of Cart-1 contains a paired-type homeodomain. Northern blot hybridization and RNase protection assay revealed that Cart-1 RNA was present at high levels in a well-differentiated rat chondrosarcoma tumor and in a cell line derived from this tumor. Cart-1 RNA was detected in primary mouse and rat chondrocytes but not in various fibroblasts including mouse 10T1/2 cells, NIH 3T3 cells, BALB 3T...

  10. Imaging findings of sternal abnormalities

    Energy Technology Data Exchange (ETDEWEB)

    Franquet, T. [Dept. of Radiology, Hospital de Sant Pau, Universidad Autonoma de Barcelona (Spain); Gimenez, A. [Dept. of Radiology, Hospital de Sant Pau, Universidad Autonoma de Barcelona (Spain); Alegret, X. [Dept. of Radiology, Hospital de Sant Pau, Universidad Autonoma de Barcelona (Spain); Sanchis, E. [Dept. of Radiology, Hospital de Sant Pau, Universidad Autonoma de Barcelona (Spain); Rivas, A. [Dept. of Radiology, Hospital Vall d`Hebron, Universidad Autonoma de Barcelona (Spain)

    1997-05-01

    Radiographic findings in the sternal abnormalities are often nonspecific, showing appearances from a localized benign lesion to an aggressive lesion as seen with infections and malignant neoplasms. A specific diagnosis of sternal abnormalities can be suggested on the basis of CT and MR characteristics. Familiarity with the presentation and variable appearance of sternal abnormalities may aid the radiologist is suggesting a specific diagnosis. We present among others characteristic radiographic findings of hemangioma, chondrosarcoma, hydatid disease, and SAPHO syndrome. In those cases in which findings are not specific, cross-sectional imaging modalities may help the clinician in their management. (orig.)

  11. Biphasic Malignant Pleural Mesothelioma Masquerading as a Primary Skeletal Tumor

    Directory of Open Access Journals (Sweden)

    James Benjamin Gleason

    2016-01-01

    Full Text Available Biphasic malignant pleural mesothelioma is a rare malignant tumor, usually presenting as a pleural-based mass in a patient with history of chronic asbestos exposure. We herein report a case of a 41-year-old man who presented with chest pain and had a chest computed tomography (CT scan suggestive of a primary skeletal tumor originating from the ribs (chondrosarcoma or osteosarcoma, with no history of asbestos exposure. CT-guided core needle biopsies were diagnosed as malignant sarcomatoid mesothelioma. Surgical resection and chest wall reconstruction were performed, confirming the diagnosis and revealing a secondary histologic component (epithelioid, supporting the diagnosis of biphasic malignant mesothelioma.

  12. NY-ESO-1 (CTAG1B) expression in mesenchymal tumors.

    Science.gov (United States)

    Endo, Makoto; de Graaff, Marieke A; Ingram, Davis R; Lim, Simin; Lev, Dina C; Briaire-de Bruijn, Inge H; Somaiah, Neeta; Bovée, Judith V M G; Lazar, Alexander J; Nielsen, Torsten O

    2015-04-01

    New York esophageal squamous cell carcinoma 1 (NY-ESO-1, CTAG1B) is a cancer-testis antigen and currently a focus of several targeted immunotherapeutic strategies. We performed a large-scale immunohistochemical expression study of NY-ESO-1 using tissue microarrays of mesenchymal tumors from three institutions in an international collaboration. A total of 1132 intermediate and malignant and 175 benign mesenchymal lesions were enrolled in this study. Immunohistochemical staining was performed on tissue microarrays using a monoclonal antibody for NY-ESO-1. Among mesenchymal tumors, myxoid liposarcomas showed the highest positivity for NY-ESO-1 (88%), followed by synovial sarcomas (49%), myxofibrosarcomas (35%), and conventional chondrosarcomas (28%). Positivity of NY-ESO-1 in the remaining mesenchymal tumors was consistently low, and no immunoreactivity was observed in benign mesenchymal lesions. On the basis of these findings, nearly 90% of myxoid liposarcomas, as well as a significant proportion of synovial sarcomas, myxofibrosarcomas, and conventional chondrosarcomas are good candidates for immunotherapy targeting NY-ESO-1.

  13. Follow-up study of cartilaginous bone tumors.

    Directory of Open Access Journals (Sweden)

    Suzuki,Atsushi

    1986-06-01

    Full Text Available A series of clinical and pathological studies were performed on 74 cartilaginous bone tumors including osteochondromas, multiple cartilaginous exostoses, chondromas, chondromatoses, benign chondroblastomas and chondrosarcomas. Resection was adequate for the osteochondromas, and no recurrence was observed. Out of 14 multiple cartilaginous exostoses, three, all in flat bones showed malignant change. The predominant sites of chondroma were the finger and toe bones, and curettage and bone graft was adequate treatment. Neither recurrence nor malignant change was observed. Two cases of chondromatosis, one of Ollier's disease and one of Maffucci's syndrome, were included in our series. Leg length discrepancy and pathologic fracture were common problems in chondromatosis. Moreover, malignant change was suspected in a hemangioma of the Maffucci's syndrome patient. Benign chondroblastoma was treated by curettage and bone graft, with no recurrence. In our series, 4 primary and 3 secondary chondrosarcomas were observed. Metastasis was seen in only one case. Because of the discrepancy between the biological behavior and histological findings of cartilaginous bone tumors, the malignancy of tumors should be evaluated by clinical signs and symptoms as well as by histological findings.

  14. V-ATPase as an effective therapeutic target for sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Perut, Francesca, E-mail: francesca.perut@ior.it [Laboratory for Orthopaedic Pathophysiology and Regenerative Medicine, Istituto Ortopedico Rizzoli, Bologna (Italy); Avnet, Sofia; Fotia, Caterina; Baglìo, Serena Rubina; Salerno, Manuela [Laboratory for Orthopaedic Pathophysiology and Regenerative Medicine, Istituto Ortopedico Rizzoli, Bologna (Italy); Hosogi, Shigekuni [Laboratory for Orthopaedic Pathophysiology and Regenerative Medicine, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Molecular Cell Physiology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto (Japan); Kusuzaki, Katsuyuki [Department of Molecular Cell Physiology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto (Japan); Baldini, Nicola [Laboratory for Orthopaedic Pathophysiology and Regenerative Medicine, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna (Italy)

    2014-01-01

    Malignant tumors show intense glycolysis and, as a consequence, high lactate production and proton efflux activity. We investigated proton dynamics in osteosarcoma, rhabdomyosarcoma, and chondrosarcoma, and evaluated the effects of esomeprazole as a therapeutic agent interfering with tumor acidic microenvironment. All sarcomas were able to survive in an acidic microenvironment (up to 5.9–6.0 pH) and abundant acidic lysosomes were found in all sarcoma subtypes. V-ATPase, a proton pump that acidifies intracellular compartments and transports protons across the plasma membrane, was detected in all cell types with a histotype-specific expression pattern. Esomeprazole administration interfered with proton compartmentalization in acidic organelles and induced a significant dose-dependent toxicity. Among the different histotypes, rhabdomyosarcoma, expressing the highest levels of V-ATPase and whose lysosomes are most acidic, was mostly susceptible to ESOM treatment. - Highlights: • Osteosarcoma, rhabdomyosarcoma, and chondrosarcoma survive in acidic microenvironment. • At acidic extracellular pH, sarcoma survival is dependent on V-ATPase expression. • Esomeprazole administration induce a significant dose-dependent toxicity.

  15. The Findings of {sup 99m}Tc-MDP Bone Scan in Primary malignant Bone Tumors

    Energy Technology Data Exchange (ETDEWEB)

    Hyun, In Young; Lee, Kung Han; Lee, Dong Soo; Chung, June Key; Lee, Myung Chul; Koh, Chang Soon; Kang, Heung Sik; Lee, Sang Hoon; Lee, Han Koo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1995-03-15

    Tc-99m-MDP bone scan was performed in 31 patients with primary malignant bone tumors, 22 patients with osteogenic sarcoma, 5 patients with chondrosarcoma and 4 patients with Ewing's sarcoma. The findings were classified by isotope intensity of accumulation in tumor as grade 1 to 3, overall pattern of isotope distribution in tumor as grade 1 to 3, and distortion of bony outline as grade 1 to 3. Histologic classifications were correlated with scan findings in 22 patients with osteogenic sarcoma. The results were as follows. 1) In 22 patients with osteogenic sarcoma, markedly increased isotope intensity higher than sacroiliac joint with patchy areas of decreased intensity and severe bony distortion were found in 16 patients. The correlations between histologic classification and scan findings were not discovered. 2) In 5 patients with chondrosarcoma, mildly increased isotope intensity with patchy areas of increased intensity and mild bony distortion were found in 4 patients. 3) In 4 patients with Ewing's sarcoma, markedly increased homogenous intensity with moderate bony distortion were found in 3 patients. Conclusively there were common findings in each 3 primary malignant bone tumors and Tc-99m-MDP bone scan was complemented with radiologic studies in differentiating primary malignant bone tumors.

  16. Spot Scanning Proton Therapy for Malignancies of the Base of Skull: Treatment Planning, Acute Toxicities, and Preliminary Clinical Outcomes

    Energy Technology Data Exchange (ETDEWEB)

    Grosshans, David R., E-mail: dgrossha@mdanderson.org [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Zhu, X. Ronald; Melancon, Adam [Department of Radiation Physics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Allen, Pamela K. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Poenisch, Falk; Palmer, Matthew [Department of Radiation Physics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); McAleer, Mary Frances; McGovern, Susan L. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Gillin, Michael [Department of Radiation Physics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); DeMonte, Franco [Department of Neurosurgery, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Chang, Eric L. [Department of Radiation Oncology, University of Southern California Keck School of Medicine, Los Angeles, California (United States); Brown, Paul D.; Mahajan, Anita [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States)

    2014-11-01

    Purpose: To describe treatment planning techniques and early clinical outcomes in patients treated with spot scanning proton therapy for chordoma or chondrosarcoma of the skull base. Methods and Materials: From June 2010 through August 2011, 15 patients were treated with spot scanning proton therapy for chordoma (n=10) or chondrosarcoma (n=5) at a single institution. Toxicity was prospectively evaluated and scored weekly and at all follow-up visits according to Common Terminology Criteria for Adverse Events, version 3.0. Treatment planning techniques and dosimetric data were recorded and compared with those of passive scattering plans created with clinically applicable dose constraints. Results: Ten patients were treated with single-field-optimized scanning beam plans and 5 with multifield-optimized intensity modulated proton therapy. All but 2 patients received a simultaneous integrated boost as well. The mean prescribed radiation doses were 69.8 Gy (relative biological effectiveness [RBE]; range, 68-70 Gy [RBE]) for chordoma and 68.4 Gy (RBE) (range, 66-70) for chondrosarcoma. In comparison with passive scattering plans, spot scanning plans demonstrated improved high-dose conformality and sparing of temporal lobes and brainstem. Clinically, the most common acute toxicities included fatigue (grade 2 for 2 patients, grade 1 for 8 patients) and nausea (grade 2 for 2 patients, grade 1 for 6 patients). No toxicities of grades 3 to 5 were recorded. At a median follow-up time of 27 months (range, 13-42 months), 1 patient had experienced local recurrence and a second developed distant metastatic disease. Two patients had magnetic resonance imaging-documented temporal lobe changes, and a third patient developed facial numbness. No other subacute or late effects were recorded. Conclusions: In comparison to passive scattering, treatment plans for spot scanning proton therapy displayed improved high-dose conformality. Clinically, the treatment was well tolerated, and

  17. Cartilage tumors. Pathology and radiomorphology; Chondrogene Knochentumoren. Pathologie und Radiomorphologie

    Energy Technology Data Exchange (ETDEWEB)

    Uhl, M. [RKK-Klinikum Freiburg, Klinik fuer Diagnostische und Interventionelle Radiologie, Kinderradiologie und Neuroradiologie SJK, Freiburg (Germany); Herget, G. [Universitaetsklinik Freiburg, Department Orthopaedie und Traumatologie, Freiburg (Germany); Kurz, P. [Universitaetsklinik Freiburg, Pathologisches Institut, Freiburg (Germany)

    2016-06-15

    Primary cartilage-forming tumors of the bone are frequent entities in the daily work of skeletal radiologists. This article describes the correlation of pathology and radiology in cartilage-forming skeletal tumors, in particular, enchondroma, osteochondroma, periosteal chondromas, chondroblastoma and various forms of chondrosarcoma. After reading, the radiologist should be able to deduce the different patterns of cartilage tumors on radiographs, CT, and MRI from the pathological aspects. Differentiation of enchondroma and chondrosarcoma is a frequent diagnostic challenge. Some imaging parameters, e. g., deep cortical scalloping (more than two thirds of the cortical thickness), cortical destruction, or a soft-tissue mass, are features of a sarcoma. Osteochondromas are bony protrusions with a continuous extension of bone marrow from the parent bone, the host cortical bone runs continuously from the osseous surface of the tumor into the shaft of the osteochondroma and the osteochondroma has a cartilage cap. Chondromyxoid fibromas are well-defined lytic and eccentric lesions of the metaphysis of the long bones, with nonspecific MRI findings. Chondroblastomas have a strong predilection for the epiphysis of long tubular bones and develop an intense perifocal bone marrow edema. Dedifferentiated chondrosarcomas are bimorphic lesions with a low-grade chondrogenic component and a high-grade noncartilaginous component. Most chondrogenic tumors have a predilection with regard to site and age at manifestation. (orig.) [German] Primaere knorpelbildende Tumoren sind haeufige Entitaeten in der taeglichen Arbeit des Radiologen. Der Beitrag beschreibt die Korrelation von Pathologie und Radiologie knorpelbildender Skeletttumoren, insbesondere von Enchondrom, Osteochondrom, periostalem Chondrom, Chondroblastom, und verschiedenen Varianten des Chondrosarkoms. Nach Lesen des Beitrags kann der Radiologe die verschiedenen typischen Muster knorpelbildender Tumoren im Roentgenbild

  18. Vascularized fibula grafts for reconstruction of bone defects after resection of bone sarcomas

    DEFF Research Database (Denmark)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen;

    2010-01-01

    We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1-24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4......'s sarcoma had an early hip disarticulation, developed multiple metastases, and died 9 months after the operation. The remaining patients (n = 7) are all alive 50 months (range 26-75 months) after surgery. During the follow-up the following major complications were seen: 1-2 fractures (n = 4), pseudarthrosis...... (n = 2), and hip dislocation (n = 1). Limb-sparing surgery with reconstruction of bone defects using vascularized fibular grafts in BS cases is feasible with acceptable clinical results, but fractures should be expected in many patients....

  19. [Cranio-orbital zygomatic extradural approach for cavernous sinus or Meckel's cave tumors].

    Science.gov (United States)

    Kinjo, T; Mukawa, J; Mekaru, S; Koga, H

    1996-06-01

    Direct surgery to cavernous sinus (CS) lesions has become one of the optimal treatments based on advancement in microsurgical anatomy and imaging modality, and the progress of microsurgical techniques. We have removed the CS or Meckel's cave tumors extradurally when they do not extend intradurally, using modified Al-Mefty's cranio-orbital zygomatic craniotomy. Three CS tumors; trigeminal neurofibroma, squamous cell carcinoma and chondrosarcoma, and a Meckel's cave meningioma were reported. Total removal was achieved in all but one (case 4). Postoperative complications were permanent ophthalmoplegia in one, transient ophthalmoplegia in one, subcutaneous CSF accumulation in two and trigeminal dysfunction in one. The extradural approach can be the first choice of methods for total removal of tumors when they are confined to the CS or Meckel's cave.

  20. Frozen section diagnosis in ophthalmic pathology

    Directory of Open Access Journals (Sweden)

    Biswas Jyotirmay

    1993-01-01

    Full Text Available Frozen section diagnosis is extensively used in various branches of pathology, but its application in ophthalmic pathology was recognised only in the 1970s. We studied 10 sections of ocular and adenexal lesions by frozen section diagnosis, which included orbital lesions (4 cases, lid lesions (3 cases, and intraocular tissue (1 case. The time taken for processing ranged between 10 to 15 minutes. Diagnoses based on frozen section evaluation included lymphoma, mesenchymal chondrosarcoma, solar keratosis, compound naevus, silicone oil globules in cataractous lens, neurofibromatosis, pseudotumour, retinoblastoma, and chronic blepharitis. Although further histopathologic examination correlated well with the frozen section (100% observations, the diagnosis was deferred in the case of naevus and reactive lymphoid hyperplasia. Our study shows that frozen section diagnosis in ophthalmic surgery is quite reliable and is particularly useful in ocular adenexal lesions

  1. Bone marrow oedema associated with benign and malignant bone tumours

    Energy Technology Data Exchange (ETDEWEB)

    James, S.L.J. [Department of Radiology, Royal Orthopaedic Hospital, Birmingham, B31 2AP (United Kingdom)], E-mail: steven.james@roh.nhs.uk; Panicek, D.M. [Department of Radiology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10021 (United States); Davies, A.M. [Department of Radiology, Royal Orthopaedic Hospital, Birmingham, B31 2AP (United Kingdom)

    2008-07-15

    Bone marrow oedema is associated with a wide variety of pathological processes including both benign and malignant bone tumours. This imaging finding in relation to intraosseous tumours can aid in providing a more focused differential diagnosis. In this review, we will discuss the MR imaging of bone marrow oedema surrounding intraosseous neoplasms. The different pulse sequences used in differentiating underlying tumour from surrounding oedema are discussed along with the role of dynamic contrast enhanced MRI. Benign lesions commonly associated with bone marrow oedema include osteoid osteoma, osteoblastoma, chondroblastoma and Langerhan's cell histiocytosis. Metastases and malignant primary bone tumours such as osteosarcoma, Ewing's sarcoma and chondrosarcoma may also be surrounded by bone marrow oedema. The imaging findings of these conditions are reviewed and illustrated. Finally, the importance of bone marrow oedema in assessment of post chemotherapeutic response is addressed.

  2. An intraosseous lipoma of the calcaneus: a case report.

    Science.gov (United States)

    Pappas, Alexander J; Haffner, Kyle E; Mendicino, Samuel S

    2014-01-01

    Intraosseous lipomas are one of the rarest bone tumors found in the body. The incidence has been reported to be intraosseous lipoma in the calcaneus include plantar fasciitis, retrocalcaneal bursitis, gout, stress fracture, unicameral bone cyst, aneurysmal bone cyst, osteoblastoma, enchondroma, chondromyxoid fibroma, nonossifying fibroma, giant cell tumor, chondroblastoma, fibrous dysplasia, and chondrosarcoma. It has been reported that 60% to 70% of patients with an intraosseous lipoma present with symptoms. This article describes a case of a pathologic fracture secondary to a large intraosseous lipoma, the surgical treatments, and the subsequent resolution of symptoms. The purpose of our report was 3-fold: (1) to increase awareness of intraosseous lipomas and their potential to cause pathologic fractures in the calcaneus; (2) to suggest a possible treatment protocol for intraosseous lipomas in the calcaneus; and (3) to describe a rare case of an intraosseous lipoma of the calcaneus not located exclusively in the neutral triangle.

  3. [Deep fascicular fibromatoses in diagnostic material from the pathomorphology department of the Institute of Mother and Child].

    Science.gov (United States)

    Liebhart, M

    1989-01-01

    We studied 17 cases of deep fascicular fibromatoses of various location in children and adolescents from 7 months to 16 years. Basic proliferative cells consisted of mature cells of the connective tissue slightly changed cytologic features but without atypia. We observed scanty regular mitoses. Histological structure of individual tumours and fields in the same tumours differed in the degree of collagenization, vascularization and abundance of cells. In four cases proliferations were differentiated with neurofibromatosis, in one case with fibrosarcoma of high-degree differentiation. In four cases the tumours were of desmoid structure. In two cases of mesenteric location of the proliferation we found foci of cartilagous metaplasia, which in one case after several recurrences transformed the tumour into a chondrosarcoma.

  4. Carcinosarcoma of the Ureter with a Small Cell Component: Report of a Rare Pathologic Entity and Potential for Diagnostic Error on Biopsy

    Directory of Open Access Journals (Sweden)

    Kent Newsom

    2014-01-01

    Full Text Available Carcinosarcomas of the ureter are rare biphasic neoplasms, composed of both malignant epithelial (carcinomatous and malignant mesenchymal (sarcomatous components. Carcinosarcomas of the urinary tract are exceedingly rare. We report a unique case of a carcinosarcoma of the ureter with a chondrosarcoma and small cell tumor component arising in a 68-year-old male who presented with microscopic hematuria. CT intravenous pyelogram revealed right-sided hydroureter and hydronephrosis with thickening and narrowing of the right ureter. The patient underwent robot-assisted ureterectomy with bladder cuff excision and subsequent adjuvant chemotherapy. The patient is disease-free at 32 months after treatment. We provide a brief synoptic review of carcinosarcoma of the ureter and bladder with utilization of immunohistochemical (IHC stains and potential diagnostic pitfalls.

  5. Delineation of Chondroid Lipoma: An Immunohistochemical and Molecular Biological Analysis

    Directory of Open Access Journals (Sweden)

    Ronald S. A. de Vreeze

    2011-01-01

    Full Text Available Aims. Chondroid lipoma (CL is a benign tumor that mimics a variety of soft tissue tumors and is characterized by translocation t(11;16. Here, we analyze CL and its histological mimics. Methods. CL (n=4 was compared to a variety of histological mimics (n=83 for morphological aspects and immunohistochemical features including cyclinD1(CCND1. Using FISH analysis, CCND1 and FUS were investigated as potential translocation partners. Results. All CLs were strongly positive for CCND1. One of 4 myoepitheliomas, CCND1, was positive. In well-differentiated lipomatous tumors and in chondrosarcomas, CCND1 was frequently expressed, but all myxoid liposarcomas were negative. FISH analysis did not give support for direct involvement of CCND1 and FUS as translocation partners. Conclusions. Chondroid lipoma is extremely rare and has several and more prevalent histological mimics. The differential diagnosis of chondroid lipomas can be unraveled using immunohistochemical and molecular support.

  6. Myoepithelial Cell-Rich Pleormorphic Adenoma of Minor Salivary Gland of Parapharyngeal Space

    Directory of Open Access Journals (Sweden)

    Digvijay Singh Rawat

    2012-01-01

    Full Text Available Parapharyngeal space tumors are rare and constitute only 0.5–1.0% of head and neck tumors. Minor salivary gland tumor is still rare in parapharyngeal space. We are reporting a case of pleomorphic adenoma of minor salivary gland of parapharyngeal space. A 42-year-old female presented with a history of mass in the oropharynx for 3 years. She presented with “hot potato voice” and dysphagia. CECT and MRI were done, showing large parapharyngeal space tumor. FNAC was suspicious for tumor of nerve cell origin. Tumor was excised using “paramedian mandibulotomy with mandibular swing approach”. Histopathological examination was inconclusive, suggesting possibility of extraskeletal myxoid chondrosarcoma, solitary fibrous tumor, neurogenic tumor. On immunohistochemistry, tumor was positive for cytokeratin, EMA (dim, S-100, and P 63 and negative for SMA thus proving the case as myoepithelial cell-rich pleomorphic adenoma.

  7. Vascularized fibula grafts for reconstruction of bone defects after resection of bone sarcomas

    DEFF Research Database (Denmark)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen

    2010-01-01

    We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1-24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4....../3/1) operated on form 2000 to 2006. The bone defects reconstructed were proximal femoral diaphysis and epiphysis (n = 2), humeral diaphysis (n = 2), humeral proximal diaphysis and epiphysis (n = 1), femoral diaphysis (n = 1), ulnar diaphysis (n = 1), and tibial diaphysis (n = 1). One patient with Ewing......'s sarcoma had an early hip disarticulation, developed multiple metastases, and died 9 months after the operation. The remaining patients (n = 7) are all alive 50 months (range 26-75 months) after surgery. During the follow-up the following major complications were seen: 1-2 fractures (n = 4), pseudarthrosis...

  8. An overview of the clinical pharmacology of N-phosphonacetyl-L-aspartate (PALA), a new antimetabolite.

    Science.gov (United States)

    Erlichman, C

    1980-01-01

    N-Phosphonacetyl-L-aspartic acid (PALA) is new synthetic antimetabolite which inhibits de novo pyrimidine biosynthesis. Its significant activity against Lewis lung carcinoma, B16 melanoma, and glioma 26 suggested that it might be useful in the treatment of human solid tumors. Phase I trials revealed that dose-limiting toxicity included skin reactions, diarrhea, and stomatitis. Pharmacologic studies demonstrated rapid renal excretion of more than 70% of the unmetabolized drug in 24 h. Peak plasma levels correlated with dose of PALA administered. Partial responses to PALA were seen in one patient with melanoma, one with chondrosarcoma, and one with colon carcinoma. The potential for PALA's use in combination chemotherapy, particularly with 5-fluorouracil, is discussed.

  9. Chondrogenic Lesions of the Skeletal System Using Radiographs, CT and MRI

    Directory of Open Access Journals (Sweden)

    Akbar Bonakdarpour

    2011-05-01

    calcification. CT and MRI confirm"nradiographic findings. The tumor is hypodense in T1"nand hyperdense in T2 and post gadolinium injection"nfat suppressed T1 images. CT and MRI confirm"nradiographic findings."nChondromyxoid Fibroma-A benign, eccentric, ovoid,"nmetaphyseal tumor with recurrent tendency. It rarely"nshows calcification. The tumor is hypodense on T1 and"nIran J Radiol 2011, 8 (Supp.1 S65"nhyper dense on T2 and post gadolinium studies show"nhyperintensity beyond the tumor. It has a tendency"nfor recurrence. Non-ossifying fibroma is a self healing"nmetaphyseal fibrous defect and not a tumor, diamond"nshaped in one of the perpendicular radiographs."nMetaphyseal fibrous defect is usually subcortical, but"nit may be small and intracortical. CT and MRI confirm"nradiographic findings."nSynovial chondrometaplasia. (synovial"nosteochondromatosis is an arthritis resulting"nfrom chondro (or osteochondro metaplasia of the"nsynovium. They may form osteocartilaginous bodies"nin the joint. In advanced cases they may cause erosion of"nthe articular surface and malignant transportation has"nbeen reported. Radiographs may demonstrate chondroosseous"nbodies in the joints, but CT demonstrates them"nbetter. MRI shows joint fluid and filling defects in the"nfluid. Erosion may be demonstrated by these three"nmodalities."nChondrosarcoma:"nConventional Chondrosarcoma: central, peripheral"nand juxtacortical."nVariants of chondrosarcoma: clear cell, mesenchymal"nand dedifferentiated. Chondrosarcoma can be"ndifferentiated from enchondroma by the presence"nof cortical erosion more than two thirds of the"ncortical diameter and periosteal reaction. Most"nchondrosarcomas discussed here are of low grad

  10. MODIFIED TRANSCRANIAL APPROACH FOR RESECTION OF TUMORS INVOLVING THE ANTERIOR CRANIAL FOSSA

    Institute of Scientific and Technical Information of China (English)

    赵素萍; 陶正德; 肖健云

    2001-01-01

    Objective: To introduce the method of a modified transcranial approach for resection of paranasal sinuses tumors involving the anterior skull base and to address our experience with the approach. Patients and Methods: Ten cases were operated by the approach. Among them, 4 suffered from benign meningeomas, 6 with malignant tumors included one chondrosarcoma, two malignant meningeomas, two olfactory neuroblastomas, and one squamous sarcoma. Of the patients, 4 cases had primary tumor and 6 cases had recurrent tumors. Result: All of the ten cases underwent operation and no postopertion complication occurred. 7 cases have survived for one to four years without tumor recurrence. 3 cases with malignant tumor died of tumor relapse in one to two years. Conclusion: This method significantly has helped to reduce the persistence and recurrence of the disease.

  11. MR imaging of the pelvis: a guide to incidental musculoskeletal findings for abdominal radiologists.

    Science.gov (United States)

    Gaetke-Udager, Kara; Girish, Gandikota; Kaza, Ravi K; Jacobson, Jon; Fessell, David; Morag, Yoav; Jamadar, David

    2014-08-01

    Occasionally patients who undergo magnetic resonance imaging for presumed pelvic disease demonstrate unexpected musculoskeletal imaging findings in the imaged field. Such incidental findings can be challenging to the abdominal radiologist, who may not be familiar with their appearance or know the appropriate diagnostic considerations. Findings can include both normal and abnormal bone marrow, osseous abnormalities such as Paget's disease, avascular necrosis, osteomyelitis, stress and insufficiency fractures, and athletic pubalgia, benign neoplasms such as enchondroma and bone island, malignant processes such as metastasis and chondrosarcoma, soft tissue processes such as abscess, nerve-related tumors, and chordoma, joint- and bursal-related processes such as sacroiliitis, iliopsoas bursitis, greater trochanteric pain syndrome, and labral tears, and iatrogenic processes such as bone graft or bone biopsy. Though not all-encompassing, this essay will help abdominal radiologists to identify and describe this variety of pelvic musculoskeletal conditions, understand key radiologic findings, and synthesize a differential diagnosis when appropriate.

  12. Petrous apex lesions in the pediatric population

    Energy Technology Data Exchange (ETDEWEB)

    Radhakrishnan, Rupa [University of Cincinnati College of Medicine, Department of Radiology, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Son, Hwa Jung [University of Cincinnati College of Medicine, Department of Otolaryngology-Head and Neck Surgery, Cincinnati, OH (United States); Koch, Bernadette L. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States)

    2014-03-15

    A variety of abnormal imaging findings of the petrous apex are encountered in children. Many petrous apex lesions are identified incidentally while images of the brain or head and neck are being obtained for indications unrelated to the temporal bone. Differential considerations of petrous apex lesions in children include ''leave me alone'' lesions, infectious or inflammatory lesions, fibro-osseous lesions, neoplasms and neoplasm-like lesions, as well as a few rare miscellaneous conditions. Some lesions are similar to those encountered in adults, and some are unique to children. Langerhans cell histiocytosis (LCH) and primary and metastatic pediatric malignancies such as neuroblastoma, rhabomyosarcoma and Ewing sarcoma are more likely to be encountered in children. Lesions such as petrous apex cholesterol granuloma, cholesteatoma and chondrosarcoma are more common in adults and are rarely a diagnostic consideration in children. We present a comprehensive pictorial review of CT and MRI appearances of pediatric petrous apex lesions. (orig.)

  13. A case of Werner's syndrome associated with osteosarcoma.

    Science.gov (United States)

    Murata, K; Hatamochi, A; Shinkai, H; Ishikawa, Y; Kawaguchi, N; Goto, M

    1999-10-01

    We described a case of Werner's syndrome associated with osteosarcoma. A 37-year-old Japanese man was diagnosed as having Werner's syndrome by the presence of juvenile cataracts, skin sclerosis and hyperpigmentation of the feet, high-pitched voice, characteristic bird-like appearance of the face with beak-shaped nose, thinning of the entire skin and hyperkeratoses on soles, hyperlipemia, hyperuricemia, diabetes melitus, and the mutated responsible gene (WRN). He had a 3-month history of a tumor on his left forearm. Histologically, the tumor included four histological patterns; a malignant fibrous histiocytoma-like, a desmoid-like, a dermatofibrosarcoma protuberans-like, and a chondrosarcoma-like pattern. Tumoral osteoid formation was also found in the tumor. Therefore, the tumor was diagnosed as osteosarcoma.

  14. The role of imaging for the surgeon in primary malignant bone tumors of the chest wall

    Energy Technology Data Exchange (ETDEWEB)

    Rocca, M., E-mail: michele.rocca@ior.it [General and Thoracic Surgery, The Rizzoli Orthopaedic Institute, Via Pupilli 1, 40136 Bologna (Italy); Salone, M. [General and Thoracic Surgery, The Rizzoli Orthopaedic Institute, Via Pupilli 1, 40136 Bologna (Italy); Galletti, S. [Ultrasound Unit, The Rizzoli Orthopaedic Institute, Bologna (Italy); Balladelli, A. [Laboratory of Experimental Oncology, The Rizzoli Orthopaedic Institute, Bologna (Italy); Vanel, D. [Research in Imaging Musculo Skeletal Tumors, The Rizzoli Orthopaedic Institute, Bologna (Italy); Briccoli, A. [General and Thoracic Surgery, The Rizzoli Orthopaedic Institute, Via Pupilli 1, 40136 Bologna (Italy)

    2013-12-01

    Primary malignant chest wall tumors are rare. The most frequent primary malignant tumor of the chest wall is chondrosarcoma, less common are primary bone tumors belonging to the Ewing Family Bone Tumors (EFBT), or even rarer are osteosarcomas. They represent a challenging clinical entities for surgeons as the treatment of choice for these neoplasms is surgical resection, excluding EFBT which are normally treated by a multidisciplinary approach. Positive margins after surgical procedure are the principal risk factor of local recurrence, therefore to perform adequate surgery a correct preoperative staging is mandatory. Imaging techniques are used for diagnosis, to determine anatomic site and extension, to perform a guided biopsy, for local and general staging, to evaluate chemotherapy response, to detect the presence of a recurrence. This article will focus on the role of imaging in guiding this often difficult surgery and the different technical possibilities adopted in our department to restore the mechanics of the thoracic cage after wide resections.

  15. Arrested pneumatization of the sphenoid sinus mimicking intraosseous lesions of the skull base

    Energy Technology Data Exchange (ETDEWEB)

    Jalali, Elnaz; Tadinada, Aditya [Dept. of Oral and Maxillofacial Radiology, University of Connecticut School of Dental Medicine, Farmington (United States)

    2015-03-15

    Arrested pneumatization of the sphenoid sinus is a developmental variant that is not always well recognized and is often confused with other pathologies associated with the skull base. This report describes the case of a patient referred for cone-beam computed tomography (CBCT) imaging for dental implant therapy. CBCT demonstrated a well-defined incidental lesion in the left sphenoid sinus with soft tissue-like density and sclerotic borders with internal curvilinear opacifications. The differential diagnoses included intraosseous lipoma, arrested pneumatization of the sphenoid sinus, chondrosarcoma, chondroid chordoma, and ossifying fibroma. The radiographic diagnosis of arrested pneumatization was based on the location of the lesion, its well-defined nature, the presence of internal opacifications, and lack of expansion. Gray-scale CBCT imaging of the area demonstrated values similar to fatty tissue. This case highlighted the fact that benign developmental variants associated with the skull base share similar radiographic features with more serious pathological entities.

  16. Chondromyxoid fibroma of the acromium with soft tissue extension

    Energy Technology Data Exchange (ETDEWEB)

    Macdonald, D. [Departments of Anatomic and Clinical Pathology, Sunnybrook and Women' s College Health Sciences Centre, Orthopedic and Arthritic Institute, Toronto, Ontario (Canada); University of Toronto, Toronto, Ontario (Canada); Fornasier, V. [Department of Anatomical Pathology and Cytology, St. Michael' s Hospital, Wellesley-Central Site, Toronto, Ontario (Canada); Holtby, R. [Department of Surgery, Sunnybrook and Women' s College Health Sciences Centre, Orthopedic and Arthritic Institute, Toronto, Ontario (Canada)

    2000-03-30

    Chondromyxoid fibroma is an unusual, benign tumor of cartilaginous origin and represents less than 1% of all primary bone tumors. It usually involves the long bones around the knee joint or the flat bones of the pelvis or ribs. Soft tissue extension is also thought to be rare in these lesions. They are usually eccentrically located in the metaphyses of the long bones and centrally in the flat bones. The radiographic appearances are characteristically those of a single, lytic lesion with lobulated margins, septations, cortical expansion and a sclerotic rim. Histologically, they display a lobulated pattern with spindle-shaped cells lying within a myxoid matrix with areas of hyaline cartilage. The differential diagnosis includes giant cell tumor, chondroblastoma or enchondroma as well as chondrosarcoma. The rarity of these lesions may render the diagnosis difficult to make, especially when the lesion involves an unusual site such as the acromium. (orig.)

  17. Phyllodes Tumor of the Breast With Malignant Melanoma Component: A Case Report.

    Science.gov (United States)

    Vergine, Marco; Guy, Catherine; Taylor, Mark R

    2015-09-01

    Phyllodes tumors of the breast display a wide variation in histological appearance and are classified into benign, borderline, and malignant categories based on a combination of histological parameters. These tumors may include a malignant heterologous component that is believed to originate through a process of multidirectional differentiation from a cancer stem cell. In these cases, the tumor is classified as a malignant phyllodes tumor. Among the heterologous elements that have been described in malignant phyllodes tumors are rhabdomyosarcoma, chondrosarcoma, osteosarcoma, liposarcoma and angiosarcoma. We present the first case of a phyllodes tumor with a malignant melanoma component in the breast of a 71-year-old lady, discussing the clinical implications of this diagnosis.

  18. Primary bone tumors of the spine.

    Science.gov (United States)

    Cañete, A Navas; Bloem, H L; Kroon, H M

    2016-04-01

    Primary bone tumors of the spine are less common than metastases or multiple myeloma. Based on the patient's age and the radiologic pattern and topography of the tumor, a very approximate differential diagnosis can be established for an osseous vertebral lesion. This article shows the radiologic manifestations of the principal primary bone tumors of the spine from a practical point of view, based on our personal experience and a review of the literature. If bone metastases, multiple myeloma, lymphomas, hemangiomas, and enostoses are excluded, only eight types of tumors account for 80% of all vertebral tumors. These are chordomas, osteoblastomas, chondrosarcomas, giant-cell tumors, osteoid osteomas, Ewing's sarcomas, osteosarcomas, and aneurysmal bone cysts.

  19. Indian data on bone and soft tissue sarcomas: A summary of published study results

    Directory of Open Access Journals (Sweden)

    Anant Ramaswamy

    2016-01-01

    Full Text Available Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS, chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients.

  20. The radiological and histopathological differential diagnosis of chordoid neoplasms in skull base

    Directory of Open Access Journals (Sweden)

    PAN Bin-cai

    2013-07-01

    Full Text Available Background Chordoid neoplasms refer to tumors appearing to have histological features of embryonic notochord, which is characterized by cords and lobules of neoplastic cells arranged within myxoid matrix. Because of radiological and histological similarities with myxoid matrix and overlapping immunohistochemical profile, chordoma, chordoid meningioma, chordoid glioma, and rare extraskeletal myxoid chondrosarcoma enter in the radiological and histological differential diagnosis at the site of skull base. However, there is always a great challenge for histopathologists to make an accurate diagnosis when encountering a chordoid neoplasm within or near the central nervous system. The aim of this study is to investigate and summarize the radiological, histological features and immunohistochemical profiles of chordoid neoplasms in skull base, and to find a judicious panel of immunostains to unquestionably help in diagnostically challenging cases. Methods A total of 23 cases of chordoid neoplasms in skull base, including 10 chordomas, 5 chordoid meningiomas, 3 chordoid gliomas and 5 extraskeletal myxoid chondrosarcomas, were collected from the First Affiliated Hospital, Sun Yat-sen University and Guangdong Tongjiang Hospital. MRI examination was performed on the patients before surgical treatment. Microscopical examination and immunohistochemical staining study using vimentin (Vim, pan-cytokeratin (PCK, epithelial membrane antigen (EMA, S?100 protein (S-100, glial fibrillary acidic protein (GFAP, D2-40, Galectin-3, CD3, CD20, Ki-67 were performed on the samples of cases. The clinicopathological data of the patients was also analyzed retrospectively. Results Most of chordomas were localized in the clivus with heterogeneous hyperintensity on T2WI scanning. The breakage of clivus was observed in most cases. Histologically, the tumor cells of chordoma exhibited bland nuclear features and some contained abundant vacuolated cytoplasm (the so

  1. Non-pituitary origin sellar tumours mimicking pituitary macroadenomas

    Energy Technology Data Exchange (ETDEWEB)

    Abele, T.A., E-mail: travaus@gmail.com [University of Texas Southwestern Medical Center at Dallas, Dallas, TX (United States); Yetkin, Z.F.; Raisanen, J.M.; Mickey, B.E.; Mendelsohn, D.B. [University of Texas Southwestern Medical Center at Dallas, Dallas, TX (United States)

    2012-08-15

    Although the large majority of sellar tumours are pituitary adenomas, several other pituitary and non-pituitary origin tumours arise in the sellar and parasellar regions. Given their location, non-adenomatous lesions frequently mimic pituitary macroadenomas and can pose a diagnostic challenge for the radiologist. Distinguishing rare sellar lesions from the common macroadenoma helps to direct the correct surgical approach and reduce the risk of incomplete resection and/or complications such as cerebrospinal fluid leak with the potential for meningitis. The purpose of this article is to review the imaging features of non-pituitary-origin sellar tumours, focusing on characteristics that may distinguish them from pituitary macroadenomas. Lesions include meningioma, metastatic disease, epidermoid cyst, germinoma, chondrosarcoma, giant cell tumour, and giant aneurysm.

  2. Myoepithelioma of soft tissue

    Directory of Open Access Journals (Sweden)

    Pai Mukhta

    2009-01-01

    Full Text Available Myoepitheliomas and mixed tumors involving deep subcutaneous and subfascial soft tissues of limb or limb girdle are rare lesions as against salivary lesions that are well established conditions. Here, we report a 22-year-old female who presented with painful hard swelling in the left gluteal region of 1½ year duration. MRI showed a large ill-defined heterogeneous mass lesion measuring about 7-8 cm. in the left sacral region eroding the left sacroiliac region and left sacroiliac joint. With a clinical diagnosis of chondrosarcoma, the tumor with the surrounding tissue was resected in segments at surgery. Histomorphology revealed nests, sheets and cords of round to spindled cells with extensive squamous metaplasia in a myxoid to fibrous stroma. These cells extensively infiltrated muscle and bone. The tumor cells expressed immunoreactivity for cytokeratin (AE1/AE3 and S-100.

  3. Congenital high-grade sarcoma presenting as skin nodules and respiratory distress in a neonate.

    Science.gov (United States)

    Powers, J W; Teitell, M; Milisavljevic, V

    2008-02-01

    We report, to our knowledge, the first case of a congenital, widespread, aggressive high-grade sarcoma, presented as multiple skin nodules and respiratory distress in a neonate that had a t(9;22)(q22;q11-12) cytogenetic abnormality suggestive of a more indolent extraskeletal myxoid chondrosarcoma (EMC). EMC is generally thought of as a slow-growing tumor that presents between the fourth and sixth decades of life. Our patient was a 45,XY, t(13;14) newborn who presented at birth with subcutaneous nodules involving the face, scalp, back and extremities, as well as multiple intrathoracic, intraabdominal and intracranial masses. Diagnosis was made using electron microscopy and immunohistochemical and cytogenetic studies. Despite attempts to control rapid growth of lesions using high-dose steroids and cis-retinoic acid, patient's clinical status continued to deteriorate and life support was withdrawn at the 26 day of life.

  4. NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis.

    Science.gov (United States)

    Lai, Jin-Ping; Robbins, Paul F; Raffeld, Mark; Aung, Phyu Phyu; Tsokos, Maria; Rosenberg, Steven A; Miettinen, Markku M; Lee, Chyi-Chia Richard

    2012-06-01

    A promising targeted therapy against NY-ESO-1 (CTAG 1B) using genetically modified T-cells in synovial sarcomas was recently demonstrated in a clinical trial at the NCI. To investigate the role of NY-ESO-1 immunohistochemistry in patient selection and gain better insight into the incidence of NY-ESO-1 expression in synovial sarcomas and other mesenchymal tumors, we evaluated NY-ESO-1 expression by immunohistochemistry in 417 tumors. This collection of samples included: 50 SS18/SSX1/2 fusion positive synovial sarcomas, 155 gastrointestinal stromal tumors (GIST), 135 other spindle cell sarcomas as well as 77 other sarcomas (chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, rhabdomyosarcoma, angiosarcoma, malignant mesothelioma, and Ewing's sarcoma). We report that 76% of synovial sarcomas expressed NY-ESO-1 in a strong and diffuse pattern (2-3+, >50-70% of tumor cells). In contrast, only rare cases of other spindle cell mesenchymal tumor expressed NY-ESO-1 (GIST (2/155), malignant peripheral nerve sheath tumors (1/34), and dermatofibrosarcoma protuberans (2/20)). Individual cases of other sarcomas (angiosarcoma, malignant mesothelioma, chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, and Ewing's sarcoma) were positive for NY-ESO-1. However, no positive cases were identified amongst our cohort of leiomyosarcomas (0/24), hemangiopericytoma/solitary fibrous tumors (0/40), and cellular schwannomas (0/17). In summary, we find that NY-ESO-1 is strongly and diffusely expressed in a majority of synovial sarcomas, but only rarely in other mesenchymal lesions. Beyond its role in patient selection for targeted therapy, immunohistochemistry for NY-ESO-1 may be diagnostically useful for the distinction of synovial sarcoma from other spindle cell neoplasms.

  5. Initial clinical experience with scanned proton beams at the Italian National Center for Hadrontherapy (CNAO).

    Science.gov (United States)

    Tuan, J; Vischioni, B; Fossati, P; Srivastava, A; Vitolo, V; Iannalfi, A; Fiore, M R; Krengli, M; Mizoe, J E; Orecchia, R

    2013-07-01

    We report the initial toxicity data with scanned proton beams at the Italian National Center for Hadrontherapy (CNAO). In September 2011, CNAO commenced patient treatment with scanned proton beams within two prospective Phase II protocols approved by the Italian Health Ministry. Patients with chondrosarcoma or chordoma of the skull base or spine were eligible. By October 2012, 21 patients had completed treatment. Immobilization was performed using rigid non-perforated thermoplastic-masks and customized headrests or body-pillows as indicated. Non-contrast CT scans with immobilization devices in place and MRI scans in supine position were performed for treatment-planning. For chordoma, the prescribed doses were 74 cobalt grey equivalent (CGE) and 54 CGE to planning target volume 1 (PTV1) and PTV2, respectively. For chondrosarcoma, the prescribed doses were 70 CGE and 54 CGE to PTV1 and PTV2, respectively. Treatment was delivered five days a week in 35-37 fractions. Prior to treatment, the patients' positions were verified using an optical tracking system and orthogonal X-ray images. Proton beams were delivered using fixed-horizontal portals on a robotic couch. Weekly MRI incorporating diffusion-weighted-imaging was performed during the course of proton therapy. Patients were reviewed once weekly and acute toxicities were graded with the Common Terminology Criteria for Adverse Events (CTCAE). Median age of patients = 50 years (range, 21-74). All 21 patients completed the proton therapy without major toxicities and without treatment interruption. Median dose delivered was 74 CGE (range, 70-74). The maximum toxicity recorded was CTCAE Grade 2 in four patients. Our preliminary data demonstrates the clinical feasibility of scanned proton beams in Italy.

  6. Primary sarcomas of the central nervous system: UCSF experience (1985-2005

    Directory of Open Access Journals (Sweden)

    Tarık TİHAN

    2007-01-01

    Full Text Available Sarcomas constitute less than 2% of all cancers, and are a highly diverse group of neoplasms. Primary sarcomas of the central nervous system (CNS are even less common, and our experience is limited by lack of studies with sufficient size that can address the challenges in predicting behavior or management. It is critical to recognize the characteristics of these uncommon neoplasms and to develop better predictors for prognosis and behavior.We have conducted a search of the UCSF Department of Pathology and UCSF Cancer Center Registry databases for all primary CNS sarcomas that were diagnosed and treated between 1985 and 2005. Hemangiopericytomas were included, so were the solitary fibrous tumors due to their close association with the former. We excluded all cases of metastatic sarcomas, chordomas, sarcomatoid variants of all neuroepithelial neoplasms, Ewing’s sarcomas and other embryonal tumors. In addition, we have identified all soft tissue sarcomas diagnosed and treated during the same period. There were 43 primary CNS neoplasms that fulfilled the inclusion criteria. At the same time, we identified 1706 sarcomas primary to the soft tissue. Primary CNS sarcomas included 16 hemangiopericytomas, 15 chondrosarcomas, 3 solitary fibrous tumors, 3 osteosarcomas, 2 leiomyosarcomas, 2 undifferentiated sarcomas, 1 histiocytic sarcoma, and 1 fibrosarcoma. There was a distinctly higher frequency of hemangiopericytoma in the CNS compared to soft tissue. In addition, a group of low grade, parasagittal chondrosarcomas were noted for their highly indolent biological behavior. Unlike some previous series, our cohort was devoid of angiosarcoma and malignant fibrous histiocytoma. This study underscores the limitations of single institutional series, and highlights the value of multi-institutional studies to understand and better treat primary CNS sarcomas.

  7. rDlx, a novel distal-less-like homeoprotein is expressed in developing cartilages and discrete neuronal tissues.

    Science.gov (United States)

    Zhao, G Q; Zhao, S; Zhou, X; Eberspaecher, H; Solursh, M; de Crombrugghe, B

    1994-07-01

    From a rat chondrosarcoma we isolated a cDNA that encodes a novel homeoprotein rDlx. The homeodomain of rDlx shows a high degree of sequence identity with those of Drosophila Distal-less, mouse Dlx, and Xenopus Xdll proteins. Northern hybridization of rDlx revealed a 1.4- to 1.6-kb RNA species in a rat chondrosarcoma and a cell line derived from this tumor and in mouse C3H10T1/2 cells, but no rDlx RNA was detected in mouse NIH3T3 fibroblasts, rat skin fibroblasts, mouse C2 myoblasts, mouse myeloma S194 cells, human B-cell lymphoma Daudi cells, or human acute myelocytic leukemia cells. RNase protection assays showed that rDlx transcripts were present at high levels in 14-day-old rat embryos, 18-day-old rat embryo skeletal tissues, and adult rat brain. rDlx RNAs were present at lower levels in newborn rat rib cartilage, 18-day-old rat embryo soft tissues, newborn rat skin, and adult rat heart. rDlx transcripts were not detected in adult rat liver, spleen, lung, kidney, testis, or skeletal muscle. In situ hybridization of rat embryos at different stages revealed that rDlx transcripts were present in otic vesicle, branchial arches, apical ectodermal ridge of limb bud, developing cartilages, perichondria of mature cartilages, mesenchymal cells of developing membranous bones, developing teeth, ganglionic eminence of the telencephalon, diencephalon, olfactory epithelia, and epidermis of the skin. rDlx RNAs were also detected in the developing parasympathetic mesenteric ganglia of the gastrointestinal tract. Hence, rDlx RNAs are mainly expressed in several neuronal tissues and developing skeletal tissues.

  8. Hereditary multiple exostosis with secondary malignization: case report

    Energy Technology Data Exchange (ETDEWEB)

    Coutinho, A.M.N.; Pitella, F.A.; Coura Filho, G.B.; Costa, P.L.A.; Ono, C.R.; Watanabe, T.; Sapienza, M.T.; Hironaka, F.; Cerri, G.G.; Buchpiguel, C.A. [Universidade de Sao Paulo (USP), SP (Brazil). Inst. de Radiologia. Centro de Medicna Nuclear

    2008-07-01

    Full text: Introduction: Hereditary Multiple Exostosis (HME) or multiple osteochondromatosis is a skeletal development anomaly which is characterized by generalized exostoses in the bones, mainly in long bone metaphyses, appearing during childhood and adolescence. The transmission is autosomal dominant, its prevalence varies from 1/50,000 to 9/1,000,000 in Europe, and around 10% of cases show no family history. Case Report: Description of an HME case with two secondary malignization episodes. The data was taken from the patient's chart and from imaging exams from the hospital files. WASB, a 19-year-old male, hospitalized after being pre-diagnosed with HME and complaints of bone-consistent mass in the right gluteal region and a lump in the posterior region of the right leg, associated to multiple bone lumps all over the body. A magnetic resonance imaging (MRI) was performed along with a bone scintillography with {sup 99m}Tc-MDP which showed multiple osteogenic lesions in the thorax, pelvic bones and long bones with periarticular prevalence in the lower limbs. The suspicion of malignancy in the right iliac area was raised due to the MRI result and to the higher intensity captured in the scintillography, confirming chondrosarcoma grade I of malignancy in the biopsy. The patient suffered interileo abdominalis amputation of the right lower limb with good evolution and control scintillography performed after 1 and 1,5 years. In the second controlling procedure, the patient complained about pain in the left knee, and a MRI suggested a new secondary malignization. The hypothesis of a head of left fibula osteochondroma with signs of aggressiveness was confirmed following surgery. Discussion: In HME, the exostoses grow along with the individual, ceasing with the epiphyseal fusion. The growth of these formations after skeletal maturation suggests activity of exostoses and, in most times, it is a sign of malignant transformation, which turns almost every time into

  9. Uptake of 153Sm-EDTMP in normal, benign and malignant tumor tissue

    CERN Document Server

    Riegel, A

    2001-01-01

    The present study was designed to investigate and compare the uptake of 153Sm-EDTMP (153Samarium-ethylenediaminetetramethylene phosphonate)and 99mTc-DPD (99mTechnetium-dicarboxypropane diphosphonate) into different soft tissue sarcoma cell lines and various tissue specimen in vitro. After 10-120 minutes of incubation at 22 sup o C and 37 sup o C with 153Sm-EDTMP, the uptake kinetics of this tracer in human soft tissue sarcoma cells SW 684 (fibrosarcoma) and SW 1353 (chondrosarcoma) were assessed. The uptake was temperature-dependent and higher into fibrosarcoma than in chondrosarconma. Normal bone tissue samples of rat and human were incubated with 153Sm-EDTMP and 99mTc-DPD. The uptake of 99mTc-DPD was higher than that of 153Sm-EDTMP. Various benign and malignant bone and soft tissue tumors and metastases of different primaries were treated in the same way. The uptake was generally very low, in the metastatic tissue specimen in part possibly due to their osteolytic character.

  10. Differential diagnosis of pediatric tumors of the nasal cavity and paranasal sinuses: a 45-year multi-institutional review.

    Science.gov (United States)

    Holsinger, F Christopher; Hafemeister, Adam C; Hicks, M John; Sulek, Marcelle; Huh, Winston W; Friedman, Ellen M

    2010-11-01

    We conducted a retrospective case-series review to identify the various diagnoses of neoplasms of the nasal cavity and paranasal sinuses in a pediatric population. Our study group was made up of 54 children-23 boys and 31 girls, aged 8 months to 16 years (mean: 9 yr). All patients had been diagnosed with a tumor of the nasal cavity or paranasal sinuses between Jan. 1, 1955, and Dec. 31, 1999, at one of four university-based, tertiary care referral centers. We compiled data on tumoral characteristics (location, size, and histopathology), morbidity and mortality, and rates of recurrence. Lesions included adnexal neoplasm, ameloblastic fibro-odontoma, basal cell carcinoma, benign fibrous histiocytoma, blue nevus, chondrosarcoma, compound nevus, epithelioma adenoides cysticum, esthesioneuroblastoma, Ewing sarcoma, fibrosarcoma, giant cell granuloma, granulocytic sarcoma, hemangioma, hemangiopericytoma, Langerhans cell histiocytosis, lymphangioma, lymphoma, melanoma, neuroblastoma, neurofibroma, ossifying osteofibroma, osteochondroma, osteosarcoma, port wine stain, rhabdomyosarcoma, Spitz nevus, and xanthogranuloma. To the best of our knowledge, this is the largest such study of its kind to date. We believe that the large size of this study and the data on disease incidence will allow clinicians to be better informed of the differential diagnosis of neoplasms of the nasal cavity and paranasal sinuses in the pediatric population.

  11. [Strategy of combined treatment in patient with cancer of paranasal sinuses].

    Science.gov (United States)

    Kiprian, Dorota

    2007-01-01

    Cancers of pranasal sinuses are rare neoplasms in humans. In 2003, in Poland there were 132 new patients diagnosed for this disease. Squamous cell cancer is the most frequent one in this region. Other types of cancer in this region are adenocarcinoma (about 30%), carcinoma adenoides cysticum or neoplasms such as rhabdosarcoma, chondrosarcoma, lymphoma or melanoma malignum. There is a very rare neoplasm as a olfactory neuroblastoma in this localization. Cancer of the paranasal sinuses infiltrates only locoregionally. Metastases to the lymph nodes are seldom--below 30%; this is why elective lymphangiectomy or irradiation are not obligatory treatment in this case. The most important is histopathological verification performed by biopsy. For clinical staging it is obligatory to perform endoscopy, CT or MR examination. The treatment of cancers of paranasal sinuses is always surgery with adjuvant irradiation. The modern radiotherapy techniques provide the possibility to spare healthy tissues and organs at risk. The organs at risk in this localization are optical nerves and chiasm, and parotid glands. The conformal radiotherapy is used most frequently. In case of the tumour being of complex shape and located in the vicinity of the organs at risk the IMRT technique is used. The radiation treatment combined with chemotherapy is applied in cases of not radical surgery in the region of ethmoides sinuses.

  12. The Role of Hedgehog Signaling in Tumor Induced Bone Disease

    Directory of Open Access Journals (Sweden)

    Shellese A. Cannonier

    2015-08-01

    Full Text Available Despite significant progress in cancer treatments, tumor induced bone disease continues to cause significant morbidities. While tumors show distinct mutations and clinical characteristics, they behave similarly once they establish in bone. Tumors can metastasize to bone from distant sites (breast, prostate, lung, directly invade into bone (head and neck or originate from the bone (melanoma, chondrosarcoma where they cause pain, fractures, hypercalcemia, and ultimately, poor prognoses and outcomes. Tumors in bone secrete factors (interleukins and parathyroid hormone-related protein that induce RANKL expression from osteoblasts, causing an increase in osteoclast mediated bone resorption. While the mechanisms involved varies slightly between tumor types, many tumors display an increase in Hedgehog signaling components that lead to increased tumor growth, therapy failure, and metastasis. The work of multiple laboratories has detailed Hh signaling in several tumor types and revealed that tumor establishment in bone can be controlled by both canonical and non-canonical Hh signaling in a cell type specific manner. This review will explore the role of Hh signaling in the modulation of tumor induced bone disease, and will shed insight into possible therapeutic interventions for blocking Hh signaling in these tumors.

  13. New SPECT tracers: Example of tracers of proteoglycans and melanin; Nouveaux traceurs TEMP: exemple des traceurs des proteoglycanes et de la melanine

    Energy Technology Data Exchange (ETDEWEB)

    Cachin, F.; Mestas, D.; Kelly, A.; Merlin, C.; Veyre, A.; Maublant, J. [CRLCC Jean-Perrin, Service de Medecine Nucleaire, 63 - Clermont-Ferrand (France); Cachin, F.; Chezal, J.M.; Miot-Noirault, E.; Moins, N.; Auzeloux, P.; Vidal, A.; Bonnet-Duquennoy, M.; Boisgard, S.; D' Incan, M.; Madelmont, J.C.; Maublant, J. [Universite d' Auvergne, EA 4231, 63 - Clermont-Ferrand (France); Boisgard, S. [CHRU Gabriel-Montpied, Service d' Orthopedie, 63 - Clermont-Ferrand (France); D' Incan, M. [CHRU Gabriel-Montpied, Service de Dermatologie, 63 - Clermont-Ferrand (France); Redini, F. [Inserm, U957-EA3822, Faculte de Medecine, 44 - Nantes (France); Filaire, M. [Universite d' Auvergne, Lab. d' Anatomie, 63 - Clermont-Ferrand (France)

    2009-02-15

    The majority of research program on new radiopharmaceuticals turn to tracers used for positron emission tomography (PET). Only a few teams work on new non fluorine labeled tracers. However, the coming of SPECT/CT gamma cameras, the arrival of semi-conductors gamma cameras should boost the development of non-PET tracers. We exhibit in this article the experience acquired by our laboratory in the conception and design of two new non fluorine labelled compounds. The {sup 99m}Tc-N.T.P. 15-5 (N.T.P. 15-5 for N-[tri-ethyl-ammonium]-3-propyl-[15]ane-N5) which binds to proteoglycans could be used for the diagnosis and staging of osteoarthritis and chondrosarcoma. The iodo benzamides, specific to the melanin, are nowadays compared to {sup 18}F-fluorodeoxyglucose in a phase III clinical trial for the diagnosis and detection of melanoma metastasis. Our last development focus on N-[2-(diethyl-amino)ethyl]-4 and 2-iodo benzamides respectively B.Z.A. and B.Z.A.2 hetero-aromatic analogues usable for melanoma treatment. (authors)

  14. Integrated and Quantitative Proteomics of Human Tumors.

    Science.gov (United States)

    Yakkioui, Y; Temel, Y; Chevet, E; Negroni, L

    2017-01-01

    Quantitative proteomics represents a powerful approach for the comprehensive analysis of proteins expressed under defined conditions. These properties have been used to investigate the proteome of disease states, including cancer. It has become a major subject of studies to apply proteomics for biomarker and therapeutic target identification. In the last decades, technical advances in mass spectrometry have increased the capacity of protein identification and quantification. Moreover, the analysis of posttranslational modification (PTM), especially phosphorylation, has allowed large-scale identification of biological mechanisms. Even so, increasing evidence indicates that global protein quantification is often insufficient for the explanation of biology and has shown to pose challenges in identifying new and robust biomarkers. As a consequence, to improve the accuracy of the discoveries made using proteomics in human tumors, it is necessary to combine (i) robust and reproducible methods for sample preparation allowing statistical comparison, (ii) PTM analyses in addition to global proteomics for additional levels of knowledge, and (iii) use of bioinformatics for decrypting protein list. Herein, we present technical specificities for samples preparation involving isobaric tag labeling, TiO2-based phosphopeptides enrichment and hydrazyde-based glycopeptides purification as well as the key points for the quantitative analysis and interpretation of the protein lists. The method is based on our experience with tumors analysis derived from hepatocellular carcinoma, chondrosarcoma, human embryonic intervertebral disk, and chordoma experiments.

  15. A well-known lesion in an unusual location: infantile myofibroma of the eyelid: a case report and review of literature.

    Science.gov (United States)

    Asadi Amoli, Fahimeh; Sina, Amir Hossein; Kasai, Aboulfazl; Ayan, Zahra

    2010-01-01

    Myofibroma is a neoplasia of myofibroblasts that can be solitary or multiple and it is found most commonly in the head & neck region including scalp, forehead, parotid region and oral cavity. In the eyelid it is rarely reported. It has a benign course in the solitary form and fatal in its multiple form. A 4 month male infant referred to Farabi hospital -the referral center for eye diseases- with a 2 month history of a mass in his eyelid with gradual enlargement with no other complaints. The only abnormal physical finding was a 2.5 cm mass in the eyelid. This mass was excised and sent to the hospital pathology laboratory. When confronting a spindle cell lesion with a nodular or multinodular growth pattern which appears biphasic due to alteration of light and dark staining areas, the surgical pathologist should think to the possibility of myofibroma. Its pattern of growth and architecture rules out the other differential diagnoses like nodular fasciitis, fibrous histiocytoma, infantile fibromatosis, and peripheral primitive neuroectodermal tumor, mesenchymal chondrosarcoma, malignant hemangiopericytoma, juvenile fibrosarcoma and poorly differentiated synovial sarcoma. In difficult cases immunohistochemical staining is helpful that is Vimentin & Actin positivity & Desmin, CK, EMA & S100 negativity.

  16. CT and MRI of intrinsic space-occupying lesions of the bony skull base; CT und MRT bei intrinsischen raumfordernden Laesionen der knoechernen Schaedelbasis

    Energy Technology Data Exchange (ETDEWEB)

    Koesling, S.; Brandt, S. [Martin-Luther-Universitaet Halle-Wittenberg, Universitaetsklinik und Poliklinik fuer Diagnostische Radiologie, Halle (Germany); Neumann, K. [Martin-Luther-Universitaet Halle-Wittenberg, Universitaetsklinik und Poliklinik fuer Hals-, Nasen-, Ohrenheilkunde, Kopf- und Halschirurgie, Halle (Germany)

    2009-07-15

    Intrinsic bony lesions of the skull base are diseases which arise within the bones forming the skull base. Mainly they are bone tumours and tumour-like lesions. With the exception of osteomas of the paranasal sinuses and exostoses of the external auditory canal, these lesions occur rarely. This article gives an overview of the appearance of the most common primary bony skull base masses in CT and MRI. From the authors' point of view these are fibrous dysplasia, chordomas, chondrosarcomas, Langerhans cell histiocytosis and multiple myelomas, which must be differentiated from pseudolesions. The possibilities of CT and MRI in making a specific diagnosis, differential diagnosis and the kind of making the final diagnosis are described. (orig.) [German] Unter intrinsischen Laesionen der knoechernen Schaedelbasis versteht man Erkrankungen, die von den die Schaedelbasis bildenden Knochen ausgehen. In erster Linie handelt es sich um Knochentumoren und tumoraehnliche Laesionen. Mit wenigen Ausnahmen (Nasennebenhoehlenosteome, Exostosen des aeusseren Gehoergangs) sind sie selten. Dieser Beitrag gibt einen Ueberblick ueber das CT- und MRT-Erscheinungsbild der aus Sicht der Autoren unter diesem Aspekt noch am haeufigsten anzutreffenden raumfordernden Laesionen. Dazu zaehlen die fibroese Dysplasie, Chordome, Chondrosarkome, die Langerhans-Zell-Histiozytose und das multiple Myelom. Abgrenzt werden muessen Pseudotumoren. Artdiagnostische Zuordnungsmoeglichkeiten durch CT und/oder MRT, Differenzialdiagnosen und Diagnosesicherung werden dargelegt. (orig.)

  17. Imaging of the larynx and hypopharynx

    Energy Technology Data Exchange (ETDEWEB)

    Becker, Minerva [Department of Radiology, Geneva University Hospital, 24 Rue Micheli-du Crest, CH-1211 Geneva 14 (Switzerland)], E-mail: Minerva.Becker@hcuge.ch; Burkhardt, Karim [Department of Clinical Pathology, Geneva University Hospital, 24 Rue Micheli-du Crest, CH-1211 Geneva 14 (Switzerland); Dulguerov, Pavel [Department of Otorhinolaryngology, Geneva University Hospital, 24 Rue Micheli-du Crest, CH-1211 Geneva 14 (Switzerland); Allal, Abdelkarim [Department of Radiation Oncology, Geneva University Hospital, 24 Rue Micheli-du Crest, CH-1211 Geneva 14 (Switzerland)

    2008-06-15

    The purpose of this article is to review currently used imaging protocols for the evaluation of pathologic conditions of the larynx and hypopharynx, to describe key anatomic structures in the larynx and hypopharynx that are relevant to tumor spread and to discuss the clinical role of Computed Tomography (CT), Magnetic Resonance Imaging (MRI) and PET CT in the pretherapeutic workup and posttherapeutic follow-up of patients with squamous cell carcinoma of this region. A detailed discussion of the characteristic neoplastic submucosal invasion patterns, including extension to the preepiglottic space, paraglottic space and laryngeal cartilages and the implications of imaging for tumor staging and treatment planning is provided. The present article also reviews less common tumors of this region, such as chondrosarcoma, lymphoma, minor salivary gland tumors and lipoma. As the majority of non-neoplastic conditions do not require imaging the role of CT and MRI is discussed in some particular situations, such as to delineate cysts and laryngoceles, abscess formation in inflammatory conditions, to evaluate laryngeal and hypopharyngeal involvement in granulomatous and autoimmune diseases, and to evaluate the extent of laryngeal fractures due to severe blunt trauma.

  18. Role of abnormal expression of receptor for advanced glycosylation end products in carcinogenesis,tumor invasion, and metastasis%晚期糖基化终产物受体异常表达对肿瘤发生和侵袭转移的影响

    Institute of Scientific and Technical Information of China (English)

    岳志强; 王颖钰; 刘玉萍; 郑仕中; 陆茵

    2011-01-01

    以往人们将晚期糖基化终产物受体(receptor for advanced glycosylation end products,RAGE)的研究重点放在糖尿病血管病变中.近年来的临床研究发现,RAGE在人肝癌、胃癌、胰腺癌和前列腺癌等多种肿瘤组织中均高表达,而在横纹肌肉瘤和肺癌组织中低表达.RAGE的异常表达与肿瘤的发生、血管生成和侵袭转移等恶性化程度密切相关.本文对不同肿瘤组织中RAGE的异常表达及其与肿瘤发生、发展的相关性进行简要综述.%Receptor for advanced glycosylation end products (RACE) has long been a focus in studies of diabetic vascular diseases. Recently, clinical evidence has shown that the expression level of RAGE is high in liver, stomach, pancreas and prostate cancer tissues whereas the expression level of RAGE is lower in chondrosarcoma and lung cancer tissues. The abnormal expression of RAGE is closely associated with tumorgenesis, angiogenesis, tumor invasion and metastasis. This review summarizes the abnormal expression of RAGE in different tumors and its role in carcinogenesis and development of tumor.

  19. Vascularized Fibula Grafts for Reconstruction of Bone Defects after Resection of Bone Sarcomas

    Science.gov (United States)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen; Rechnitzer, Catherine; Daugaard, Søren; Muhic, Aida

    2010-01-01

    We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1–24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4/3/1) operated on form 2000 to 2006. The bone defects reconstructed were proximal femoral diaphysis and epiphysis (n = 2), humeral diaphysis (n = 2), humeral proximal diaphysis and epiphysis (n = 1), femoral diaphysis (n = 1), ulnar diaphysis (n = 1), and tibial diaphysis (n = 1). One patient with Ewing's sarcoma had an early hip disarticulation, developed multiple metastases, and died 9 months after the operation. The remaining patients (n = 7) are all alive 50 months (range 26–75 months) after surgery. During the follow-up the following major complications were seen: 1-2 fractures (n = 4), pseudarthrosis (n = 2), and hip dislocation (n = 1). Limb-sparing surgery with reconstruction of bone defects using vascularized fibular grafts in BS cases is feasible with acceptable clinical results, but fractures should be expected in many patients. PMID:20490263

  20. Modified Vertical Rectus Abdominis Musculocutaneous Flap for Limb Salvage Procedures in Proximal Lower Limb Musculoskeletal Sarcomas

    Directory of Open Access Journals (Sweden)

    Haitham H. Khalil

    2008-01-01

    Full Text Available Introduction and aim. Management of complicated wounds after tumor extipiration of pelvic and proximal lower limb musculoskeletal sarcoma represents an essential component in the outcome of these patients. The authors present modified vertical rectus abdominis musculocutaneous (VRAM flap techniques to reconstruct extensive defects after debridment of these complicated wounds. Material and Methods. Over a period of 4 years (2002–2005, 5 men and 2 women were managed. Median age was 21 years (range 15–49. The patients were managed for complicated lower trunk, groin, and upper thigh wounds after resection of three pelvic chondrosarcomas as well as two pelvic and two proximal femur osteosarcomas. The modifications included a VRAM flap with lateral and tongue-like extension design of the skin paddle (5 cases or a delayed extended VRAM flap (2 cases. Results. All flaps showed complete survival and healing with no ischemic events providing stable coverage. All patients were ambulant with good limb functions in terms of walking and gait after adequate rehabilitation, 2 needed support with crutches. Conclusion. The modified VRAM flaps offer reliable reconstructive tools for coverage of complex groin and thigh defects by providing larger well-vascularized soft tissue with acceptable donor site.

  1. CNS Metastases from Bone and Soft Tissue Sarcomas in Children, Adolescents, and Young Adults: Are They Really So Rare?

    Directory of Open Access Journals (Sweden)

    Monika Bekiesinska-Figatowska

    2017-01-01

    Full Text Available Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas’ metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas’ CNS metastases. Methods. Patients with first diagnosis in 1999–2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%, 14 boys, aged 5–22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing’s sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS, and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45% than in other reports (0.7%. Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.

  2. MR imaging for evaluation of lesions of the cranial vault: a pictorial essay

    Energy Technology Data Exchange (ETDEWEB)

    Amaral, Lazaro; Chiurciu, Miriam; Almeida, Joao Ricardo; Ferreira, Nelson Fortes; Mendonca, Renato; Lima, Sergio Santos [Hospital da Beneficiencia Portuguesa, Sao Paulo, SP (Brazil). MEDIMAGEM]. E-mail: lazden@terra.com.br

    2003-09-01

    A variety of diseases affect the calvaria. They may be identified clinically as palpable masses or incidentally in radiologic examinations. There are many diagnostic possibilities, including congenital, neoplastic, inflammatory and traumatic lesions. The purpose of this study is to illustrate the main calvarial lesions through MR imaging, their signal intensity and extension to neighboring sites. A retrospective analysis of 81 cases, from November 1996 to July 2001, was conducted. The examinations were performed on a 1.5 T equipment and each one of the cases was pathologically proven. The results were: dermoid cysts [4 cases (5%)], epidermoid cysts [2 cases (2.5%)], cephalocele [14 cases (17.5%)], sinus pericranii [3 cases (3.7%)], leptomeningeal cysts [4 cases (5%)], Langerhans cell histiocytosis [10 cases (12.5%)], lipoma [4 cases (5%)], fibrous dysplasia [13 cases (16.2%)], osteoma [8 cases (10%)], hemangioma [1 case (1.2%)], meningioma [3 cases (3.7%)], chondrosarcoma [5 cases (6.2%)], hemangiosarcoma [1 case (1.2%)], multiple myeloma [3 cases (3.7%)], sarcomatous transformation of Paget disease [1 case (1.3%)], and metastasis [5 cases (6.2%)]. MRI identifies bone marrow abnormalities and invasion of adjacent tissues at an early stage. Therefore, it is an essential method when it comes to properly evaluating calvarial lesions. (author)

  3. Intraosseous Schwannoma of the Petrous Apex.

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    Tamura, Ryota; Takahashi, Satoshi; Kohno, Maya; Kameyama, Kaori; Fujiwara, Hirokazu; Yoshida, Kazunari

    2015-07-01

    Background and Importance Intraosseous schwannoma is a relatively rare clinical entity that typically arises in vertebral and mandibular bone. Intraosseous schwannoma located entirely within the petrous bone is exceedingly rare, and only two cases have been reported to date. Clinical Presentation A 47-year-old Asian man was referred to our hospital with a chief complaint of double vision. Neurologic examination revealed left abducens nerve palsy. Radiologic imaging showed a 35-mm osteolytic expansive lesion located in the left petrous apex. We made a preoperative diagnosis of chondrosarcoma and performed surgical resection. Surgery was performed via a left subtemporal epidural approach with anterior petrosectomy. The histopathologic diagnosis of the tumor was schwannoma. Schwannoma arising from cranial nerves was excluded from intraoperative findings in conjunction with the results for cranial nerves, and intraosseous schwannoma was diagnosed. Postoperative course was uneventful, and abducens nerve palsy resolved immediately after surgery. Conclusion The differential diagnosis of intraosseous schwannoma should be considered for an osteolytic mass lesion within the petrous apex. Subcapsular tumor removal was considered ideal in terms of preservation of the cranial nerves and vessels around the tumor.

  4. Osseous Tumours of the Hand: A Review of 99 Cases in 20 Years

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    Mahmoud Farzan

    2013-12-01

    Full Text Available Background: Tumors involving the hand skeleton are rare. However, a basic knowledge of hand tumors is necessary for every clinician. This is due to the importance of distinguishing typical benign tumors from life or limb threatening malignant ones. Methods: This study is a review of 99 cases of osseous hand tumors presented to the department of orthopedic surgery, Imam Khomeini Hospital in Tehran, Iran, from December 1990 to February 2011. Results: Ninety-one cases were benign osseous tumors of the hand and eight tumors were malignant which four of them were considered as primary and four considered as metastatic type. The most common benign tumors were enchondroma and osteoid osteoma. Other benign tumors were epidermoid bone cyst, giant cell tumor of the bone, aneurysmal bone cyst, osteoblastoma, and osteochondroma. Primary malignant tumors were extremely rare and we have reported two chondrosarcomas, one osteosarcoma and one Ewing’s sarcoma involving the hand skeleton. Conclusion: This study indicates that the history, physical examination, laboratory and radiographic data as well as clinicians’ knowledge of specific hand tumors are required for the best management strategy. New techniques could lead to earlier diagnosis, prevent complications and indentify the most effective type of treatment.

  5. Bone and soft tissue tumors of hip and pelvis

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    Bloem, Johan L., E-mail: j.l.bloem@lumc.nl [Leiden University Medical Center, Department of Radiology, PO Box 9600, 2300 RC Leiden (Netherlands); Reidsma, Inge I., E-mail: i.i.reidsma@lumc.nl [Leiden University Medical Center, Department of Radiology, PO Box 9600, 2300 RC Leiden (Netherlands)

    2012-12-15

    Objective is to identify epidemiologic and radiologic criteria allowing specific diagnoses of tumors and tumor-like lesions in the hip region and pelvis, and to optimize pre-operative staging. Patients with pelvic tumors are usually older, and their tumors are larger relative to patients with tumors in extremities. The majority of tumors in the pelvis are malignant (metastases, myeloma, chondrosarcoma, Ewing-, osteo-, and MFH/fibrosarcoma), while those in the proximal femur are in majority benign (fibrous dysplasia, solitary bone cyst, and osteoid osteoma). Soft tissue masses in the thigh in the elderly are typically sarcomas without tumor specific signs. Common tumor-like lesions occurring in the hip and pelvis that can mimic neoplasm are: infections (including tuberculosis), insufficiency/avulsion fractures, cysts, fibrous dysplasia, aneurysmal bone cyst, Langerhans cell histiocytosis, and Paget's disease. Local MR staging is based on the compartmental anatomy. The psoas and gluteal muscles are easily invaded by sarcoma originating in the ileum. The pectineus muscle protects the neurovascular bundle at the level of the hip. The thigh is separated into three compartments, some structures (Sartorius muscle) cross borders between compartments. Immobile joints (SI-joints, osteoarthritic hip) are relatively easily crossed by sarcoma and giant cell tumor.

  6. Osseous Tumours of the Hand: A Review of 99 Cases in 20 Years

    Directory of Open Access Journals (Sweden)

    Mahmoud Farzan

    2013-12-01

    Full Text Available   Background: Tumors involving the hand skeleton are rare. However, a basic knowledge of hand tumors is necessary for every clinician. This is due to the importance of distinguishing typical benign tumors from life or limb threatening malignant ones. Methods: This study is a review of 99 cases of osseous hand tumors presented to the department of orthopedic surgery, Imam Khomeini Hospital in Tehran, Iran, from December 1990 to February 2011. Results: Ninety-one cases were benign osseous tumors of the hand and eight tumors were malignant which four of them were considered as primary and four considered as metastatic type. The most common benign tumors were enchondroma and osteoid osteoma. Other benign tumors were epidermoid bone cyst, giant cell tumor of the bone, aneurysmal bone cyst, osteoblastoma, and osteochondroma. Primary malignant tumors were extremely rare and we have reported two chondrosarcomas, one osteosarcoma and one Ewing’s sarcoma involving the hand skeleton. Conclusion: This study indicates that the history, physical examination, laboratory and radiographic data as well as clinicians’ knowledge of specific hand tumors are required for the best management strategy. New techniques could lead to earlier diagnosis, prevent complications and indentify the most effective type of treatment.

  7. Particle beam therapy (hadrontherapy): basis for interest and clinical experience.

    Science.gov (United States)

    Orecchia, R; Zurlo, A; Loasses, A; Krengli, M; Tosi, G; Zurrida, S; Zucali, P; Veronesi, U

    1998-03-01

    The particle or hadron beams deployed in radiotherapy (protons, neutrons and helium, carbon, oxygen and neon ions) have physical and radiobiological characteristics which differ from those of conventional radiotherapy beams (photons) and which offer a number of theoretical advantages over conventional radiotherapy. After briefly describing the properties of hadron beams in comparison to photons, this review discusses the indications for hadrontherapy and analyses accumulated experience on the use of this modality to treat mainly neoplastic lesions, as published by the relatively few hadrontherapy centres operating around the world. The analysis indicates that for selected patients and tumours (particularly uveal melanomas and base of skull/spinal chordomas and chondrosarcomas), hadrontherapy produces greater disease-free survival. The advantages of hadrontherapy are most promisingly realised when used in conjunction with modern patient positioning, radiation delivery and focusing techniques (e.g. on-line imaging, three-dimensional conformal radiotherapy) developed to improve the efficacy of photon therapy. Although the construction and running costs of hadrontherapy units are considerably greater than those of conventional facilities, a comprehensive analysis that considers all the costs, particularly those resulting from the failure of less effective conventional radiotherapy, might indicate that hadrontherapy could be cost effective. In conclusion, the growing interest in this form of treatment seems to be fully justified by the results obtained to date, although more efficacy and dosing studies are required.

  8. Imaging of primary bone tumors in veterinary medicine: which differences?

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    Vanel, Maïa; Blond, Laurent; Vanel, Daniel

    2013-12-01

    Veterinary medicine is most often a mysterious world for the human doctors. However, animals are important for human medicine thanks to the numerous biological similarities. Primary bone tumors are not uncommon in veterinary medicine and especially in small domestic animals as dogs and cats. As in human medicine, osteosarcoma is the most common one and especially in the long bones extremities. In the malignant bone tumor family, chondrosarcoma, fibrosarcoma and hemangiosarcoma are following. Benign bone tumors as osteoma, osteochondroma and bone cysts do exist but are rare and of little clinical significance. Diagnostic modalities used depend widely on the owner willing to treat his animal. Radiographs and bone biopsy are the standard to make a diagnosis but CT, nuclear medicine and MRI are more an more used. As amputation is treatment number one in appendicular bone tumor in veterinary medicine, this explains on the one hand why more recent imaging modalities are not always necessary and on the other hand, that prognostic on large animals is so poor that it is not much studied. Chemotherapy is sometimes associated with the surgery procedure, depending on the aggressivity of the tumor. Although, the strakes differs a lot between veterinary and human medicine, biological behavior are almost the same and should led to a beneficial team work between all.

  9. Radiation induced osteosarcoma of the chest wall

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    Sugimoto, Tsutomu; Yuki, Yoshihiro; Oizumi, Hiroyuki; Iijima, Yoshiyuki; Fujishima, Tsukasa; Shimazaki, Yasuhisa [Yamagata Univ. (Japan). School of Medicine

    1996-11-01

    We report a successful resection of an osteosarcoma in the chest wall developed 25 years after irradiation. A 74-year-old woman was admitted to our hospital for her swelling in the left chest wall at August 24, 1995. At 49-year-old, she had undergone an operation and postoperative irradiation for left breast cancer. A computed tomography demonstrated a mass in the left chest wall that destructed the first rib, extending into the pleural space and invaded into the left common carotid and subclavian arteries. We planned a radical resection of the mass after repeated CT scannings, since it was histopathologically diagnosed as a chondrosarcoma and showed a rapid growth. The tumor was completely removed with radical transmediastinal forequarter amputation of the partial chest wall and total left upper extremity. The left common carotid artery was partially replaced with 6 mm EPTFE vascular prosthesis. The chest wall was reconstructed with Marlex-mesh prosthesis and a myocutaneous flap. She was discharged uneventfully and has not shown any evidence of recurrence. (author)

  10. Brain Metastasis in Bone and Soft Tissue Cancers: A Review of Incidence, Interventions, and Outcomes

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    Faris Shweikeh

    2014-01-01

    Full Text Available Bone and soft tissue malignancies account for a small portion of brain metastases. In this review, we characterize their incidence, treatments, and prognosis. Most of the data in the literature is based on case reports and small case series. Less than 5% of brain metastases are from bone and soft tissue sarcomas, occurring most commonly in Ewing’s sarcoma, malignant fibrous tumors, and osteosarcoma. Mean interval from initial cancer diagnosis to brain metastasis is in the range of 20–30 months, with most being detected before 24 months (osteosarcoma, Ewing sarcoma, chordoma, angiosarcoma, and rhabdomyosarcoma, some at 24–36 months (malignant fibrous tumors, malignant peripheral nerve sheath tumors, and alveolar soft part sarcoma, and a few after 36 months (chondrosarcoma and liposarcoma. Overall mean survival ranges between 7 and 16 months, with the majority surviving < 12 months (Ewing’s sarcoma, liposarcoma, malignant fibrous tumors, malignant peripheral nerve sheath tumors, angiosarcoma and chordomas. Management is heterogeneous involving surgery, radiosurgery, radiotherapy, and chemotherapy. While a survival advantage may exist for those given aggressive treatment involving surgical resection, such patients tended to have a favorable preoperative performance status and minimal systemic disease.

  11. Analysis of 24 Patients Who Were Amputated Due to a Malignant Tumor in the Skeleton

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    Hasan Gocer

    2014-11-01

    Full Text Available Aim: The purpose of the study was to assess the cases that were amputated in our clinic due to primary malignant and metastatic bone and soft tissue tumor. Material and Method: 24 cases that were amputated due to primary malignant and metastatic bone and soft tissue tumor between January 1987 and January 2012 were examined retrospectively. The cases were assessed in terms of age, gender, pathological diagnosis, localization, type of amputation, survival and characteristics. The data obtained were transferred to SPSS 15.0 program and analyzed. Normality distributions of the data were analyzed with Shapiro-Wilk test. Results: Of the 24 cases, 17 (70% were men, while 7 (30% were women and the average age was 42 (between 12 and 68. The most common reasons for amputation were skin cancer (25%, Ewing sarcoma (20.8%, Osteosarcoma (16.6% and others (Malignant mesenchymal tumor, chondrosarcoma, synovial sarcoma, metastatic tumor. 16 of these patients had previously received an intervention in a different centre at least once. The most common type of amputation was above-knee amputation (58.3%, below-knee amputation (25% and others (hip disarticulation, below-elbow amputation. 8 patients were found to have skin problems and debridement was performed on 6. 14 cases died within the postoperative first year. Discussion: Amputation can be performed for the treatment of the patient’s other health problems and fast and local controls of advanced malignant extremity tumors.

  12. Proton beam radiotherapy: report of the first patient treated at the Centro Nazionale di Adroterapia Oncologica (CNAO) [National Center of Oncologic Hadron Therapy].

    Science.gov (United States)

    Orecchia, Roberto; Srivastava, Anurita; Fiore, Maria Rosaria; Vitolo, Viviana; Fossati, Piero; Vischioni, Barbara; Iannalfi, Alberto; Tuan, Jeffrey; Ciocca, Mario; Molinelli, Silvia; Mirandola, Alfredo; Vilches, Gloria; Mairani, Andrea; Tagaste, Barbara; Baroni, Guido; Rossi, Sandro; Krengli, Marco

    2013-01-01

    Proton beam radiotherapy, an innovative treatment modality, allows delivery of high radiation doses to the target while sparing surrounding healthy structures. The Centro Nazionale di Adroterapia Oncologica (CNAO), equipped with a synchrotron and capable of using both protons and ions, initiated its clinical activity in September 2011. The first treatment of a skull base tumor with protons is reported here. The case of a 26-year-old man with an intracranial low-grade chondrosarcoma of the right petroclival junction is discussed with emphasis on technical and clinical details. Two previous surgical interventions had achieved partial removal of the tumor and the patient was treated with protons for residual disease. The prescribed dose was 70 GyE in 35 fractions of 2 GyE. Treatment was completed with minimal acute toxicity consisting of grade 1 alopecia and nausea. Nine months after treatment the disease is locally controlled. Use of high-energy protons at CNAO is a safe and effective means of treating a tumor located near critical normal structures.

  13. Thallium-201 scintigraphy for bone and soft tissue tumors

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    Tokuumi, Yuji; Tsuchiya, Hiroyuki; Sunayama, Chiaki; Matsuda, Eizo; Asada, Naohiro; Taki, Junichi; Sumiya, Hisashi; Miyauchi, Tsutomu; Tomita, Katsuro [Kanazawa Univ. (Japan). School of Medicine

    1995-05-01

    This study was undertaken to assess the usefulness of thallium-201 scintigraphy in bone and soft tissue tumors. Pre-therapy scintigraphy was undertaken in a total of 136 patients with histologically confirmed diagnosis, consisting of 74 with malignant bone and soft tissue tumors, 39 with benign ones, 12 with diseases analogous to tumors, and 11 others. Thallium activity was graded on a scale of 0-4: 0=background activity, 1=equivocal activity, 2=definitive activity, but less than myocardium, 3=definite activity equal to myocardium, and 4=activity greater than myocardium. In the group of malignant tumors, thallium-201 uptake was found in 80%, although it was low for chondrosarcoma (2/8) and malignant Schwannoma (one/3). The group of benign tumors, however, showed it in only 41%, being restricted to those with giant cell tumors, chondroblastoma, fibromatosis, and osteoid osteoma. Thallium-201 uptake was also found in all 8 patients with metastatic tumors. In 23 patients undergoing thallium imaging before and after chemotherapy, scintigraphic findings revealed a high correlation with histopathological findings. Thus, thallium-201 scintigraphy may be potentially used to distinguish malignant from benign bone and soft tissue tumors, except for a few histopathological cases, as well as to determine loco-regional metastases and response to chemotherapy. (N.K.).

  14. CNS Metastases from Bone and Soft Tissue Sarcomas in Children, Adolescents, and Young Adults: Are They Really So Rare?

    Science.gov (United States)

    Duczkowska, Agnieszka; Duczkowski, Marek; Bragoszewska, Hanna; Romaniuk-Doroszewska, Anna; Iwanowska, Beata; Szkudlinska-Pawlak, Sylwia; Madzik, Jaroslaw; Bilska, Katarzyna; Raciborska, Anna

    2017-01-01

    Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas' metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas' CNS metastases. Methods. Patients with first diagnosis in 1999–2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI)/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%), 14 boys, aged 5–22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing's sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS), and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45%) than in other reports (0.7%). Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.

  15. The use of a modular titanium endoprosthesis in skeletal reconstructions after bone tumor resections: method presentation and analysis of 37 cases

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    Croci Alberto Tesconi

    2000-01-01

    Full Text Available We analyzed 37 patients who underwent segmental wide resection of bone tumors and reconstruction with a modular titanium endoprosthesis at the Orthopaedic Oncology Group, between 1992 and 1998. Twelve patients were male and 25 were female, with a mean age of 30 years (9 - 81. The mean follow-up was 14 months (2 - 48. The diagnoses were: osteosarcoma (14 cases, metastatic carcinoma (10, Ewing's sarcoma (4, giant cell tumor (4, malignant fibrous histiocytoma (3, chondrosarcoma (1, and aneurysmal bone cyst (1. Eleven articulated total knee, 8 partial proximal femur with bipolar acetabulum, 8 partial proximal humerus, 3 total femur, 2 partial proximal tibia, 2 diaphyseal femur, 2 diaphyseal humerus, and 1 total proximal femur with cementless acetabulum endoprosthesis implant procedures were done. The complications related to the procedure included: infection (5 cases, dislocation (3, module loosening (1, and ulnar nerve paresthesia (1. We used the following criteria for the clinical evaluation: presence of pain, range of motion, reconstruction stability, surgical and oncologic complications, and patient acceptance. The results were good in 56.8% of the cases, regular in 32.4% and poor in 10.8%.

  16. Continuous-Wave Stimulated Emission Depletion Microscope for Imaging Actin Cytoskeleton in Fixed and Live Cells

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    Bhanu Neupane

    2015-09-01

    Full Text Available Stimulated emission depletion (STED microscopy provides a new opportunity to study fine sub-cellular structures and highly dynamic cellular processes, which are challenging to observe using conventional optical microscopy. Using actin as an example, we explored the feasibility of using a continuous wave (CW-STED microscope to study the fine structure and dynamics in fixed and live cells. Actin plays an important role in cellular processes, whose functioning involves dynamic formation and reorganization of fine structures of actin filaments. Frequently used confocal fluorescence and STED microscopy dyes were employed to image fixed PC-12 cells (dyed with phalloidin- fluorescein isothiocyante and live rat chondrosarcoma cells (RCS transfected with actin-green fluorescent protein (GFP. Compared to conventional confocal fluorescence microscopy, CW-STED microscopy shows improved spatial resolution in both fixed and live cells. We were able to monitor cell morphology changes continuously; however, the number of repetitive analyses were limited primarily by the dyes used in these experiments and could be improved with the use of dyes less susceptible to photobleaching. In conclusion, CW-STED may disclose new information for biological systems with a proper characteristic length scale. The challenges of using CW-STED microscopy to study cell structures are discussed.

  17. The Role of Hedgehog Signaling in Tumor Induced Bone Disease

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    Cannonier, Shellese A.; Sterling, Julie A., E-mail: Julie.sterling@vanderbilt.edu [Department of Veterans Affairs, Tennessee Valley Healthcare System, Nashville, TN 37235 (United States); Vanderbilt Center for Bone Biology, Department of Medicine, Division of Clinical Pharmacology Vanderbilt University, Nashville, TN 372335 (United States); Department of Cancer Biology, Vanderbilt University, Nashville, TN 37235 (United States)

    2015-08-26

    Despite significant progress in cancer treatments, tumor induced bone disease continues to cause significant morbidities. While tumors show distinct mutations and clinical characteristics, they behave similarly once they establish in bone. Tumors can metastasize to bone from distant sites (breast, prostate, lung), directly invade into bone (head and neck) or originate from the bone (melanoma, chondrosarcoma) where they cause pain, fractures, hypercalcemia, and ultimately, poor prognoses and outcomes. Tumors in bone secrete factors (interleukins and parathyroid hormone-related protein) that induce RANKL expression from osteoblasts, causing an increase in osteoclast mediated bone resorption. While the mechanisms involved varies slightly between tumor types, many tumors display an increase in Hedgehog signaling components that lead to increased tumor growth, therapy failure, and metastasis. The work of multiple laboratories has detailed Hh signaling in several tumor types and revealed that tumor establishment in bone can be controlled by both canonical and non-canonical Hh signaling in a cell type specific manner. This review will explore the role of Hh signaling in the modulation of tumor induced bone disease, and will shed insight into possible therapeutic interventions for blocking Hh signaling in these tumors.

  18. Epigenetic control of cancer by neuropeptides

    Science.gov (United States)

    Galoian, Karina; Patel, Parthik

    2017-01-01

    Neuropeptides act as neurohormones, neurotransmitters and/or neuromodulators. Neuropeptides maintain physiological homeostasis and are paramount in molecular mechanisms of disease progression and regulation, including in cancer. Neuropeptides, by their definition, originate and are secreted from the neuronal cells, they are able to signal to neighboring cells or are released into the blood flow, if they act as neurohormones. The majority of neuropeptides exert their functions through G protein-coupled receptors, with certain exceptions. Although previous studies indicate that neuropeptides function in supporting proliferation of malignant cells in many types of solid tumor, the antitumorigenic action of the neuropeptides and their receptors, for example, in gastric cancers and chondrosarcoma, were also reported. It is known that epigenetically modified chromatin regulates molecular mechanisms involved in gene expression and malignant progression. The epigenetic modifications are genetically heritable, although they do not cause changes in DNA sequence. DNA methylation, histone modifications and miRNA expression are subject to those modifications. While there is substantial data on epigenetic regulation of neuropeptides, the epigenetic control of cancer by neuropeptides is considered to be uncharted territory. The aim of the current review is to describe the involvement of neuropeptides in the epigenetic machinery of cancer based on data obtained from our laboratory and from other authors.

  19. Quantitative Imaging of D-2-Hydroxyglutarate in Selected Histological Tissue Areas by a Novel Bioluminescence Technique.

    Science.gov (United States)

    Voelxen, Nadine F; Walenta, Stefan; Proescholdt, Martin; Dettmer, Katja; Pusch, Stefan; Mueller-Klieser, Wolfgang

    2016-01-01

    Patients with malignant gliomas have a poor prognosis with average survival of less than 1 year. Whereas in other tumor entities the characteristics of tumor metabolism are successfully used for therapeutic approaches, such developments are very rare in brain tumors, notably in gliomas. One metabolic feature characteristic of gliomas, in particular diffuse astrocytomas and oligodendroglial tumors, is the variable content of D-2-hydroxyglutarate (D2HG), a metabolite that was discovered first in this tumor entity. D2HG is generated in large amounts due to various "gain-of-function" mutations in the isocitrate dehydrogenases IDH1 and IDH2. Meanwhile, D2HG has been detected in several other tumor entities, including intrahepatic bile-duct cancer, chondrosarcoma, acute myeloid leukemia, and angioimmunoblastic T-cell lymphoma. D2HG is barely detectable in healthy tissue (bioluminescence assay for determining D2HG in sections of snap-frozen tissue. The assay was verified independently by photometric tests and liquid chromatography/mass spectrometry. The novel technique allows the microscopically resolved determination of D2HG in a concentration range of 0-10 μmol/g tissue (wet weight). In combination with the already established bioluminescence imaging techniques for ATP, glucose, pyruvate, and lactate, the novel D2HG assay enables a comparative characterization of the metabolic profile of individual tumors in a further dimension.

  20. Isocitrate dehydrogenase 1 (IDH1) mutation in breast adenocarcinoma is associated with elevated levels of serum and urine 2-hydroxyglutarate.

    Science.gov (United States)

    Fathi, Amir T; Sadrzadeh, Hossein; Comander, Amy H; Higgins, Michaela J; Bardia, Aditya; Perry, Ashley; Burke, Meghan; Silver, Regina; Matulis, Christina R; Straley, Kimberly S; Yen, Katharine E; Agresta, Sam; Kim, Hyeryun; Schenkein, David P; Borger, Darrell R

    2014-06-01

    Mutations in the IDH1 and IDH2 (isocitrate dehydrogenase) genes have been discovered across a range of solid-organ and hematologic malignancies, including acute myeloid leukemia, glioma, chondrosarcoma, and cholangiocarcinoma. An intriguing aspect of IDH-mutant tumors is the aberrant production and accumulation of the oncometabolite 2-hydroxyglutarate (2-HG), which may play a pivotal oncogenic role in these malignancies. We describe the first reported case of an IDH1 p.R132L mutation in a patient with hormone receptor-positive (HR+) breast adenocarcinoma. This patient was initially treated for locally advanced disease, but then suffered a relapse and metastasis, at which point an IDH1-R132 mutation was discovered in an affected lymph node. The mutation was subsequently found in the primary tumor tissue and all metastatic sites, but not in an uninvolved lymph node. In addition, the patient's serum and urine displayed marked elevations in the concentration of 2-HG, significantly higher than that measured in six other patients with metastatic HR+ breast carcinoma whose tumors were found to harbor wild-type IDH1. In summary, IDH1 mutations may impact a rare subgroup of patients with breast adenocarcinoma. This may suggest future avenues for disease monitoring through noninvasive measurement of 2-HG, as well as for the development and study of targeted therapies against the aberrant IDH1 enzyme.

  1. The driver and passenger effects of isocitrate dehydrogenase 1 and 2 mutations in oncogenesis and survival prolongation.

    Science.gov (United States)

    Molenaar, Remco J; Radivoyevitch, Tomas; Maciejewski, Jaroslaw P; van Noorden, Cornelis J F; Bleeker, Fonnet E

    2014-12-01

    Mutations in isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) are key events in the development of glioma, acute myeloid leukemia (AML), chondrosarcoma, intrahepatic cholangiocarcinoma (ICC), and angioimmunoblastic T-cell lymphoma. They also cause D-2-hydroxyglutaric aciduria and Ollier and Maffucci syndromes. IDH1/2 mutations are associated with prolonged survival in glioma and in ICC, but not in AML. The reason for this is unknown. In their wild-type forms, IDH1 and IDH2 convert isocitrate and NADP(+) to α-ketoglutarate (αKG) and NADPH. Missense mutations in the active sites of these enzymes induce a neo-enzymatic reaction wherein NADPH reduces αKG to D-2-hydroxyglutarate (D-2HG). The resulting D-2HG accumulation leads to hypoxia-inducible factor 1α degradation, and changes in epigenetics and extracellular matrix homeostasis. Such mutations also imply less NADPH production capacity. Each of these effects could play a role in cancer formation. Here, we provide an overview of the literature and discuss which downstream molecular effects are likely to be the drivers of the oncogenic and survival-prolonging properties of IDH1/2 mutations. We discuss interactions between mutant IDH1/2 inhibitors and conventional therapies. Understanding of the biochemical consequences of IDH1/2 mutations in oncogenesis and survival prolongation will yield valuable information for rational therapy design: it will tell us which oncogenic processes should be blocked and which "survivalogenic" effects should be retained.

  2. Molecular Pathways: Isocitrate Dehydrogenase Mutations in Cancer.

    Science.gov (United States)

    Clark, Owen; Yen, Katharine; Mellinghoff, Ingo K

    2016-04-15

    IDH1 and IDH2 are homodimeric enzymes that catalyze the conversion of isocitrate to α-ketoglutarate (α-KG) and concomitantly produce reduced NADPH from NADP(+) Mutations in the genes encoding IDH1 and IDH2 have recently been found in a variety of human cancers, most commonly glioma, acute myeloid leukemia (AML), chondrosarcoma, and intrahepatic cholangiocarcinoma. The mutant protein loses its normal enzymatic activity and gains a new ability to produce the "oncometabolite" R(-)-2-hydroxyglutarate (R-2-HG). R-2-HG competitively inhibits α-KG-dependent enzymes which play crucial roles in gene regulation and tissue homeostasis. Expression of mutant IDH impairs cellular differentiation in various cell lineages and promotes tumor development in cooperation with other cancer genes. First-generation inhibitors of mutant IDH have entered clinical trials, and have shown encouraging results in patients with IDH-mutant AML. This article summarizes recent progress in our understanding of the role of mutant IDH in tumorigenesis.Clin Cancer Res; 22(8); 1837-42. ©2016 AACR.

  3. Potential Mitochondrial Isocitrate Dehydrogenase R140Q Mutant Inhibitor from Traditional Chinese Medicine against Cancers

    Directory of Open Access Journals (Sweden)

    Wen-Yuan Lee

    2014-01-01

    Full Text Available A recent research of cancer has indicated that the mutant of isocitrate dehydrogenase 1 and 2 (IDH1 and 2 genes will induce various cancers, including chondrosarcoma, cholangiocarcinomas, and acute myelogenous leukemia due to the effect of point mutations in the active-site arginine residues of isocitrate dehydrogenase (IDH, such as IDH1/R132, IDH2/R140, and IDH2/R172. As the inhibition for those tumor-associated mutant IDH proteins may induce differentiation of those cancer cells, these tumor-associated mutant IDH proteins can be treated as a drug target proteins for a differentiation therapy against cancers. In this study, we aim to identify the potent TCM compounds from the TCM Database@Taiwan as lead compounds of IDH2 R140Q mutant inhibitor. Comparing to the IDH2 R140Q mutant protein inhibitor, AGI-6780, the top two TCM compounds, precatorine and abrine, have higher binding affinities with target protein in docking simulation. After MD simulation, the top two TCM compounds remain as the same docking poses under dynamic conditions. In addition, precatorine is extracted from Abrus precatorius L., which represents the cytotoxic and proapoptotic effects for breast cancer and several tumor lines. Hence, we propose the TCM compounds, precatorine and abrine, as potential candidates as lead compounds for further study in drug development process with the IDH2 R140Q mutant protein against cancer.

  4. Potential mitochondrial isocitrate dehydrogenase R140Q mutant inhibitor from traditional Chinese medicine against cancers.

    Science.gov (United States)

    Lee, Wen-Yuan; Chen, Kuan-Chung; Chen, Hsin-Yi; Chen, Calvin Yu-Chian

    2014-01-01

    A recent research of cancer has indicated that the mutant of isocitrate dehydrogenase 1 and 2 (IDH1 and 2) genes will induce various cancers, including chondrosarcoma, cholangiocarcinomas, and acute myelogenous leukemia due to the effect of point mutations in the active-site arginine residues of isocitrate dehydrogenase (IDH), such as IDH1/R132, IDH2/R140, and IDH2/R172. As the inhibition for those tumor-associated mutant IDH proteins may induce differentiation of those cancer cells, these tumor-associated mutant IDH proteins can be treated as a drug target proteins for a differentiation therapy against cancers. In this study, we aim to identify the potent TCM compounds from the TCM Database@Taiwan as lead compounds of IDH2 R140Q mutant inhibitor. Comparing to the IDH2 R140Q mutant protein inhibitor, AGI-6780, the top two TCM compounds, precatorine and abrine, have higher binding affinities with target protein in docking simulation. After MD simulation, the top two TCM compounds remain as the same docking poses under dynamic conditions. In addition, precatorine is extracted from Abrus precatorius L., which represents the cytotoxic and proapoptotic effects for breast cancer and several tumor lines. Hence, we propose the TCM compounds, precatorine and abrine, as potential candidates as lead compounds for further study in drug development process with the IDH2 R140Q mutant protein against cancer.

  5. Postradiation sarcoma of bone: review of 78 Mayo Clinic cases

    Energy Technology Data Exchange (ETDEWEB)

    Weatherby, R.P.; Dahlin, D.C.; Ivins, J.C.

    1981-05-01

    Postradiation sarcoma of bone is an uncommon but serious sequela of radiation therapy. Seventy-eight Mayo Clinic patients have been treated for sarcomas arising in irradiated bones. They received their initial radiotherapy for a wide variety of nonneoplastic and neoplastic conditions, both benign and malignant. Thirty-five sarcomas arose in bone that was normal at the time of radiotherapy, and 43 arose in irradiated preexisting osseous lesions. The latent period between radiotherapy and diagnosis of sarcoma averaged 14.3 years. Ninety percent of the postradiation sarcomas were either osteosarcomas or fibrosarcomas; chondrosarcoma, malignant (fibrous) histiocytoma, malignant lymphoma, Ewing's tumor, and metastasizing chondroblastoma also occurred. Prompt radical surgery, when feasible, is usually the treatment of choice for the sarcoma. About 30% of patients with sarcomas of the extremities or craniofacial bones survived 5 years without recurrence; there were no disease-free survivors among patients with tumors of the vertebral column, pelvis, or shoulder girdle. The low risk of sarcoma following radiotherapy for the treatment of cancer should not be a contraindication to its use in these patients; however, radiation therapy for benign bone tumors should be reserved for lesions that are not amenable to surgical treatment. An unusual case is also reported herein in which a fibrosarcoma was discovered in the humerus of a patient who had received radiotherapy 55 years previously for a verified osteosarcoma in the same site.

  6. [Chondroblastoma--current opinion].

    Science.gov (United States)

    Vukasinović, Zoran; Spasovski, Dusko; Slavković, Nemanja; Slavković, Slobodan; Zivković, Zorica

    2006-01-01

    Chondroblastoma of bone is rare bone tumor, representing around 1% of benign bone lesions. It is considered a benign lesion, although primary malignant form as well as malignant alteration in the form of chondrosarcoma has been documented. It occurs predominantly in the second decade, more commonly in males. Predilection sites include proximal humeral epiphysis, femoral and tibial condyles, but it can be found in other bones, too (skull, pelvis, posterior vertebral structures, tarsal bones). Radiographically, it appears as an ovoid lesion with thin sclerotic margin, located centrally in the epiphysis. Pathohistologically, it is described as highly cellular tissue, variably differentiated and with discrete granulated to meshy calcification of the matrix and large multinuclear cells present in 20% of cases. Secondary formation of aneurysmal bone cyst has been documented. Tumor is presented with a few nonspecific local symptoms, which makes diagnostic procedure more difficult. Definitive diagnosis is made only by pathohistological verification. A pathological fracture of weakened epiphysis is possible. The treatment of chondroblastoma is strictly surgical, with a view to counteract the propagation into the joint or adjacent soft tissue, and diminish the recurrence rate. Chemotherapy is not indicated for treatment of this tumor, and radiotherapy is contraindicated as it stimulates malignant alteration. If malignant chondroblastoma of bone is verified pathohistologically, radical treatment by surgical resection is indicated, also avoiding any adjuvant therapy.

  7. Somatic mosaic mutations of IDH1 and NPM1 associated with cup-like acute myeloid leukemia in a patient with Maffucci syndrome.

    Science.gov (United States)

    Akiyama, Masaharu; Yamaoka, Masayoshi; Mikami-Terao, Yoko; Ohyama, Wataru; Yokoi, Kentaro; Arakawa, Yasuhiro; Takita, Junko; Suzuki, Hideaki; Yamada, Hisashi

    2015-12-01

    Maffucci syndrome is a nonhereditary congenital disorder characterized by multiple enchondromas and with soft-tissue hemangiomas. Somatic mutations of the isocitrate dehydrogenase (IDH) gene have been detected in enchondroma and hemangioma tissue from patients with Maffucci syndrome. The rate of malignant transformation in Maffucci syndrome is high, with enchondromas transforming into chondrosarcomas and the development of secondary neoplasms, including pancreatic and hepatic adenocarcinoma, mesenchymal ovarian tumors, and brain tumors such as glioma. However, hematopoietic malignancies arising in Maffucci syndrome are rare. We report a 7-year-old girl with Maffucci syndrome in whom acute myeloid leukemia (AML) with cup-like nuclear invagination developed. Both leukemic cells and hemangioma had the same gene mutations: an insertion frameshift c.863_864insTCTG (p.W288 fs) in the nucleophosmin (NPM1) gene and a missense mutation c.392_395GTCG > CTCT (p.G131_R132 > AL) in the IDH1 gene. However, buccal mucosa cells and peripheral blood mononuclear cells harvested after two cycles of chemotherapy showed wild-type genotypes. These results suggest that the multiple somatic mutations of the IDH1 and NPM1 genes in hemangioblasts are related to the development of cup-like AML associated with Maffucci syndrome. However, further studies are needed to identify additional molecular events in AML but not in hemangioma.

  8. The calcified lung nodule: What does it mean?

    Directory of Open Access Journals (Sweden)

    Khan Ali

    2010-01-01

    Full Text Available The aim of this review is to present a pictorial essay emphasizing the various patterns of calcification in pulmonary nodules (PN to aid diagnosis and to discuss the differential diagnosis and the pathogenesis where it is known. The imaging evaluation of PN is based on clinical history, size, distribution and the gross appearance of the nodule as well as feasibility of obtaining a tissue diagnosis. Imaging is instrumental in the management of PN and one should strive not only to identify small malignant tumors with high survival rates but to spare patients with benign PN from undergoing unnecessary surgery. The review emphasizes how to achieve these goals. One of the most reliable imaging features of a benign lesion is a benign pattern of calcification and periodic follow-up with computed tomography showing no growth for 2 years. Calcification in PN is generally considered as a pointer toward a possible benign disease. However, as we show here, calcification in PN as a criterion to determine benign nature is fallacious and can be misleading. The differential considerations of a calcified lesion include calcified granuloma, hamartoma, carcinoid, osteosarcoma, chondrosarcoma and lung metastases or a primary bronchogenic carcinoma among others. We describe and illustrate different patterns of calcification as seen in PN on imaging.

  9. Effect of LED irradiation on the expression of MMP-3 and MMP-13 in SW1353 cells in vitro

    Science.gov (United States)

    Zeng, Chang-chun; Guo, Zhou-yi; Zhang, Feng-xue; Deng, Wen-di; Liu, Song-hao

    2007-05-01

    Matrix Metalloproteinase (MMP) plays an active role in remodeling cartilage in osteoarthritic cartilage. To find an effective method of prevention of osteoclasia, this in vitro study focuses on the expression of MMP-3 and MMP-13 in the SW1353 cells by LED irradiation. The human chondrosarcoma cell line SW1353 were stimulated with the proinflammatory cytokine IL-1beta or tumor necrosis factor-alpha (TNF-alpha), and were received the irradiation of LED (632nm, 4mW/cm2). The cell count was assessed over a 96-hour period by using Trypan blue dye exclusion assay, and the cell activity was evaluated with a Cell Counting Kit-8 Assays. The subsequent expression of MMP-3 and MMP-13 was quantified. Results of this experiment showed that the cultural cell activity was decreased, and the expression of MMP-3 and MMP-13 was increased by being stimulated with IL-1beta or TNF-alpha. After received LED irradiation, the death rate of cultural cell was increased and the expression of MMP-3 and MMP-13 was decreased significantly. The present study concluded that particular LED irradiation stimulates SW1353 cell proliferation activity and inhibit the MMP-3 and MMP-13 enzymatic activity. These findings might be clinically relevant, indicating that the low power laser irradiation treatment is likely to achieve the repair of articular cartilage in clinic.

  10. Malignant phyllodes tumor of the breast with heterologous high-grade angiosarcoma

    Directory of Open Access Journals (Sweden)

    Ghassan Tranesh

    2017-03-01

    Full Text Available Phyllodes tumors (PTs account for <3% of fibroepithelial breast lesions and for 0.3% to 1.0% of primary breast tumors. They occur predominantly in middle-aged women (mean age range, 40–50 years. PTs can be categorized into benign, borderline, and malignant; the first 2 categories are distinguished only by degree of cellular atypia and mitotic activity. Malignant PTs are more frequent among persons of Hispanic ethnicity, especially those born in Central America or South America. Heterologous sarcomatous elements may be present in malignant PTs, predominantly liposarcoma and rarely fibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, osteosarcoma, and chondrosarcoma. Breast angiosarcoma (BA is a rare heterologous, sarcomatous element that may arise secondary to malignant PT. We report a 47-year-old woman with no history of previous surgery or radiation therapy who presented to the emergency department with a painful right breast mass. She admittedly noticed the right breast mass for many years; however, recently it increased in size. Mammography and ultrasonography identified a partially cystic mass. Core needle biopsy showed dense hyalinized fibrous tissue with old blood clots, suggestive of infarcted fibroadenoma. The patient received antibiotics and analgesics; however, she reported intractable pain and a worsening skin rash of her right breast. Chest computed tomography and magnetic resonance imaging showed a doubling in mass size, with pectoralis major muscle involvement. Incisional biopsy showed malignant PT with heterologous high-grade angiosarcoma. The diagnosis of angiosarcoma was confirmed through immunoreactivity for CD31, FLI1, and ERG immunostains.

  11. Imaging of primary bone tumors in veterinary medicine: Which differences?

    Energy Technology Data Exchange (ETDEWEB)

    Vanel, Maïa, E-mail: maiavanel@yahoo.fr [Diagnostic Imaging Department, Faculty of Veterinary Medicine, University of Montreal, 3200 Rue Sicotte, PO Box 5000, Saint-Hyacinthe, QC (Canada); Blond, Laurent [Diagnostic Imaging Department, Faculty of Veterinary Medicine, University of Montreal, 3200 Rue Sicotte, PO Box 5000, Saint-Hyacinthe, QC (Canada); Vanel, Daniel [The Rizzoli Institute, Via del Barbiano 1-10, 40136, Bologna (Italy)

    2013-12-01

    Veterinary medicine is most often a mysterious world for the human doctors. However, animals are important for human medicine thanks to the numerous biological similarities. Primary bone tumors are not uncommon in veterinary medicine and especially in small domestic animals as dogs and cats. As in human medicine, osteosarcoma is the most common one and especially in the long bones extremities. In the malignant bone tumor family, chondrosarcoma, fibrosarcoma and hemangiosarcoma are following. Benign bone tumors as osteoma, osteochondroma and bone cysts do exist but are rare and of little clinical significance. Diagnostic modalities used depend widely on the owner willing to treat his animal. Radiographs and bone biopsy are the standard to make a diagnosis but CT, nuclear medicine and MRI are more an more used. As amputation is treatment number one in appendicular bone tumor in veterinary medicine, this explains on the one hand why more recent imaging modalities are not always necessary and on the other hand, that pronostic on large animals is so poor that it is not much studied. Chemotherapy is sometimes associated with the surgery procedure, depending on the agressivity of the tumor. Although, the strakes differs a lot between veterinary and human medicine, biological behavior are almost the same and should led to a beneficial team work between all.

  12. Vitamin C Protects Chondrocytes against Monosodium Iodoacetate-Induced Osteoarthritis by Multiple Pathways.

    Science.gov (United States)

    Chiu, Pu-Rong; Hu, Yu-Chen; Huang, Tzu-Ching; Hsieh, Bau-Shan; Yeh, Jou-Pei; Cheng, Hsiao-Ling; Huang, Li-Wen; Chang, Kee-Lung

    2016-12-27

    Osteoarthritis (OA) is the most prevalent joint disease. Dietary intake of vitamin C relates to a reduction in cartilage loss and OA. This study examined the efficacy of vitamin C to prevent OA with the in vitro chondrosarcoma cell line (SW1353) and the in vivo monosodium iodoacetate (MIA)-induced OA rat. Results demonstrated that, in SW1353 cells, treatment with 5 μM MIA inhibited cell growth and increased oxidative stress, apoptosis, and proteoglycan loss. In addition, the expression levels of the pro-inflammatory cytokines IL-6, IL-17A, and TNF-α and matrix metalloproteinases (MMPs) MMP-1, MMP-3, and MMP-13 were increased. All of these MIA-induced changes could be prevented with treatment of 100 μM vitamin C. In an animal model, intra-articular injection of MIA-induced cartilage degradation resembled the pathological changes of OA, and treatment of vitamin C could lessen these changes. Unexpectedly, vitamin C's effects did not strengthen with the increasing dosage, while the 100 mg/kg dosage was more efficient than the 200 or 300 mg/kg dosages. Vitamin C possessed multiple capacities for prevention of OA progress, including a decrease in apoptosis and in the expression of pro-inflammatory cytokines and MMPs in addition to the well-known antioxidation.

  13. PRMT1 mediated methylation of TAF15 is required for its positive gene regulatory function

    Energy Technology Data Exchange (ETDEWEB)

    Jobert, Laure; Argentini, Manuela [Institut de Genetique et de Biologie Moleculaire et Cellulaire (IGBMC), CNRS UMR 7104, INSERM U 596, Universite Louis Pasteur de Strasbourg, BP 10142 - 67404 Illkirch Cedex, CU de Strasbourg (France); Tora, Laszlo, E-mail: laszlo@igbmc.u-strasbg.fr [Institut de Genetique et de Biologie Moleculaire et Cellulaire (IGBMC), CNRS UMR 7104, INSERM U 596, Universite Louis Pasteur de Strasbourg, BP 10142 - 67404 Illkirch Cedex, CU de Strasbourg (France)

    2009-04-15

    TAF15 (formerly TAF{sub II}68) is a nuclear RNA-binding protein that is associated with a distinct population of TFIID and RNA polymerase II complexes. TAF15 harbours an N-terminal activation domain, an RNA recognition motif (RRM) and many Arg-Gly-Gly (RGG) repeats at its C-terminal end. The N-terminus of TAF15 serves as an essential transforming domain in the fusion oncoprotein created by chromosomal translocation in certain human chondrosarcomas. Post-transcriptional modifications (PTMs) of proteins are known to regulate their activity, however, nothing is known on how PTMs affect TAF15 function. Here we demonstrate that endogenous human TAF15 is methylated in vivo at its numerous RGG repeats. Furthermore, we identify protein arginine N-methyltransferase 1 (PRMT1) as a TAF15 interactor and the major PRMT responsible for its methylation. In addition, the RGG repeat-containing C-terminus of TAF15 is responsible for the shuttling between the nucleus and the cytoplasm and the methylation of RGG repeats affects the subcellular localization of TAF15. The methylation of TAF15 by PRMT1 is required for the ability of TAF15 to positively regulate the expression of the studied endogenous TAF15-target genes. Our findings demonstrate that arginine methylation of TAF15 by PRMT1 is a crucial event determining its proper localization and gene regulatory function.

  14. Cartilage homeoprotein 1, a homeoprotein selectively expressed in chondrocytes.

    Science.gov (United States)

    Zhao, G Q; Zhou, X; Eberspaecher, H; Solursh, M; de Crombrugghe, B

    1993-09-15

    We identified a rat cDNA that encodes cartilage homeoprotein 1 (Cart-1). The deduced amino acid sequence of Cart-1 contains a paired-type homeodomain. Northern blot hybridization and RNase protection assay revealed that Cart-1 RNA was present at high levels in a well-differentiated rat chondrosarcoma tumor and in a cell line derived from this tumor. Cart-1 RNA was detected in primary mouse and rat chondrocytes but not in various fibroblasts including mouse 10T1/2 cells, NIH 3T3 cells, BALB 3T3 cells, and rat skin fibroblasts. It was also undetectable in mouse C2 myoblasts, S194 myeloma cells, and embryonic stem cells. Cart-1 RNA was present at a very low level in tested but was not detected in other soft tissues of 8-week-old rats. In situ hybridization of rat embryos between 14.5 and 16.5 days post coitum revealed relatively high levels of Cart-1 RNA in condensed prechondrocytic mesenchymal cells and in early chondrocytes of cartilage primordia. The levels of Cart-1 RNA were lower in mature chondrocytes. No hybridization was observed in brain, spinal cord, heart, spleen, gastrointestinal tract, liver, and muscle. We speculate that Cart-1 has a role in chondrocyte differentiation.

  15. MR appearance of skeletal neoplasms following cryotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Richardson, M.L. [Dept. of Radiology SB-05, Washington Univ., Seattle, WA (United States); Lough, L.R. [Pitts Radiological Associates, Columbia, SC (United States); Shuman, W.P. [Dept. of Radiology, Medical Center Hospital of Vermont, Burlington, VT (United States); Lazerte, G.D. [Dept. of Pathology RC-72, Washington Univ., Medical Center Hospital of Vermont, Burlington, VT (United States); Conrad, E.U. [Dept. of Orthopedic Surgery RK-10, Washington Univ., Medical Center of Vermont, Burlington, VT (United States)

    1994-02-01

    Cryotherapy is an increasingly popular mode of therapy adjunctive to surgical curettage in the treatment of certain skeletal neoplasms, such as giant cell tumors or chondrosarcomas. The magnetic resonance (MR) findings following cryotherapy have not been previously reported. We reviewed the MR findings in seven patients with skeletal neoplasms following curettage and cryotherapy. In six cases we found a zone of varying thickness extending beyond the surgical margins, corresponding to an area of cryoinjury to medullary bone. This zone displayed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, consistent with the presence of marrow edema. This zone of edema almost certainly reflects underlying thermal osteonecrosis. This zone may vary in size and intensity over time as the area of cryoinjury evolves or resolves. MR is currently the imaging procedure of choice for follow-up of most musculoskeletal neoplasms. Knowledge of the MR findings following cryotherapy should help prevent confusion during the interpretation of follow-up MR examinations. (orig.)

  16. 浅谈骨肿瘤诊治中的底线%Bottom line in the treatment of bone tumors

    Institute of Scientific and Technical Information of China (English)

    韦兴

    2014-01-01

    Medicine is the science of human diseases and health. The principles of all the treatment methods and techniques should be in consistent with what has been recognized in the treatment of bone and soft tumors. Clinical Practice Guidelines for Bone Cancer ( NCCN Guidelines ) printed by National Comprehensive Cancer Network is well known, in which the treatment of chondrosarcoma, chordoma, ewing’s sarcoma, giant cell tumor of bone and osteosarcoma were discussed in detail. Most important, principles of bone cancer management were emphasized in this guideline, including: ( 1 ) Biopsy diagnosis is necessary prior to any surgical procedures. ( 2 ) Wide excision should achieve histologically negative surgical margins. ( 3 ) Lab studies such as CBC, LDH, and ALP may have relevance in the diagnosis. ( 4 ) Care for bone cancer patients should be delivered directly by physicians of a multidisciplinary team. ( 5 ) Patients should have a survivorship prescription to schedule follow-up with a multidisciplinary team. Respect on the spirits of science is the bottom line of medicine, and we should regard the principle and guideline mentioned above as the bottom line in the treatment of bone and soft tumors.

  17. Potential of Pseudoshikonin I Isolated from Lithospermi Radix as Inhibitors of MMPs in IL-1β-Induced SW1353 Cells

    Directory of Open Access Journals (Sweden)

    Dae Young Lee

    2016-08-01

    Full Text Available Pseudoshikonin I, the new bioactive constituent of Lithospermi radix, was isolated from this methanol extract by employing reverse-phase medium-pressure liquid chromatography (MPLC using acetonitrile/water solvent system as eluents. The chemical structure was determined based on spectroscopic techniques, including 1D NMR (1H, 13C, DEPT, 2D NMR (gCOSY, gHMBC, gHMQC, and QTOF/MS data. In this study, we demonstrated the effect of pseudoshikonin I on matrix-metalloproteinase (MMPs activation and expression in interleukin (IL-1β-induced SW1353 chondrosarcoma cells. MMPs are considered important for the maintenance of the extracellular matrix. Following treatment with PS, active MMP-1, -2, -3, -9, -13 and TIMP-2 were quantified in the SW1353 cell culture supernatants using a commercially available ELISA kit. The mRNA expression of MMPs in SW1353 cells was measured by RT-PCR. Pseudoshikonin I treatment effectively protected the activation on all tested MMPs in a dose-dependent manner. TIMP-2 mRNA expression was significantly upregulated by pseudoshikonin I treatment. Overall, we elucidated the inhibitory effect of pseudoshikonin on MMPs, and we suggest its use as a potential novel anti-osteoarthritis agent.

  18. Review of common conditions associated with periodontal ligament widening

    Science.gov (United States)

    Mortazavi, Hamed

    2016-01-01

    Purpose The aim of this article is to review a group of lesions associated with periodontal ligament (PDL) widening. Materials and Methods An electronic search was performed using specialized databases such as Google Scholar, PubMed, PubMed Central, Science Direct, and Scopus to find relevant studies by using keywords such as “periodontium”, “periodontal ligament”, “periodontal ligament space”, “widened periodontal ligament”, and “periodontal ligament widening”. Results Out of nearly 200 articles, about 60 were broadly relevant to the topic. Ultimately, 47 articles closely related to the topic of interest were reviewed. When the relevant data were compiled, the following 10 entities were identified: occlusal/orthodontic trauma, periodontal disease/periodontitis, pulpo-periapical lesions, osteosarcoma, chondrosarcoma, non-Hodgkin lymphoma, progressive systemic sclerosis, radiation-induced bone defect, bisphosphonate-related osteonecrosis, and osteomyelitis. Conclusion Although PDL widening may be encountered by many dentists during their routine daily procedures, the clinician should consider some serious related conditions as well. PMID:28035300

  19. The dawn of hedgehog inhibitors: Vismodegib

    Directory of Open Access Journals (Sweden)

    Selvarajan Sandhiya

    2013-01-01

    Full Text Available Cancer, one of the leading causes of death worldwide is estimated to increase to approximately 13.1 million by 2030. This has amplified the research in oncology towards the exploration of novel targets. Recently there has been lots of interest regarding the hedgehog (Hh pathway, which plays a significant role in the development of organs and tissues during embryonic and postnatal periods. In a normal person, the Hh signaling pathway is under inhibition and gets activated upon the binding of Hh ligand to a transmembrane receptor called Patched (PTCH1 thus allowing the transmembrane protein, smoothened (SMO to transfer signals through various proteins. One of the newer drugs namely vismodegib involves the inhibition of Hh pathway and has shown promising results in the treatment of advanced basal-cell carcinoma as well as medulloblastoma. It has been granted approval by US Food and Drug Administration′s (US FDA priority review program on January 30, 2012 for the treatment of advanced basal-cell carcinoma. The drug is also being evaluated in malignancies like medulloblastoma, pancreatic cancer, multiple myeloma, chondrosarcoma and prostate cancer. Moreover various Hh inhibitors namely LDE 225, saridegib, BMS 833923, LEQ 506, PF- 04449913 and TAK-441 are also undergoing phase I and II trials for different neoplasms. Hence this review will describe briefly the Hh pathway and the novel drug vismodegib.

  20. Sellar chondroid chordoma

    Directory of Open Access Journals (Sweden)

    Xia LI

    2014-02-01

    Full Text Available Objective To investigate the clinical and pathological characteristics of sellar chondroid chordoma and discuss differential diagnosis of sellar chondroid chordoma with literature review. Methods The clinical manifestations of a patient with chondroid chordoma occurring in sellar area were stated. The morphological characteristics and immune phenotype were analyzed by using HE and immunohistochemical staining, and related literatures were reviewed. Results A 66-year-old male mainly presented with repeated hyponatremia. MRI revealed a well-circumscribed, round and space-occupying mass in sellar area. The tumor was removed under endoscope via the right nasal cavity. During the resection, the tumor could be seen locating in sellar region with solid, tough quality and clear boundaries. Histologically, part of the tumor showed the chondroid differentiation in classical chordoma. The immunohistochemistry of this tumor was positive for cytokeratin (CK, epithelial membrane antigen (EMA and S-100 protein (S-100, and Ki-67 labeling index was about 1%. The pathological diagnosis was sellar chondroid chordoma. During the follow-up period of 11 months, the patient was in good condition and no tumor recurrence was found. Conclusions Despite low incidence, chondroid chordoma usually develops in the midline regions with distinctive histological features and immunohistochemical phenotypes. In general, the prognosis is better than general type of chordoma, and the diagnosis should be differentiated from general types of chordoma and chondrosarcoma.doi:10.3969/j.issn.1672-6731.2014.02.007

  1. Functional and oncologic outcomes after excision of the total femur in primary bone tumors: Results with a low cost total femur prosthesis

    Directory of Open Access Journals (Sweden)

    Ajay Puri

    2012-01-01

    Full Text Available Background: The extent of tumor may necessitate resection of the complete femur rarely to achieve adequate oncologic clearance in bone sarcomas. We present our experience with reconstruction in such cases using an indigenously manufactured, low-cost, total femoral prosthesis (TFP. We assessed the complications of the procedure, the oncologic and functional outcomes, and implant survival. Materials and Methods: Eight patients (four males and four females with a mean age of 32 years, operated between December 2003 and June 2009, had a TFP implanted. The diagnosis included osteogenic sarcoma (5, Ewing′s sarcoma (1, and chondrosarcoma (2. Mean followup was 33 months (9-72 months for all and 40 months (24-72 months in survivors. They were evaluated by Musculoskeletal Tumor Society score, implant survival as well as patient survival. Results: There was one local recurrence and five of seven patients are currently alive at the time of last followup. The Musculoskeletal Tumor Society score for patients ranged from 21 to 25 with a mean of 24 (80%. The implant survival was 88% at 5 years with only one TFP needing removal because of infection. Conclusions: A TFP in appropriately indicated patients with malignant bone tumors is oncologically safe. A locally manufactured, cost-effective implant provided consistent and predictable results after excision of the total femur with good functional outcomes.

  2. Condromatosis sinovial de la articulación temporomandibular Synovial chondromatosis of the temporomandibular joint

    Directory of Open Access Journals (Sweden)

    P. Quirós Alvarez

    2006-04-01

    Full Text Available La afectación de la ATM por una condromatosis sinovial es un hecho muy poco frecuente. Trastorno metaplásico del tejido sinovial, suele producir cuerpos libres, condromas, intraarticulares. Tiene una clínica variable y muy inespecífica. Las pruebas diagnósticas de elección son la RM y la artroscopia. La retirada de cuerpos libres y sinovectomía parcial suele ser terapéutica. Ocasionalmente puede destruir la base del cráneo y extenderse intracranealmente. Se han descrito casos de malignización secundaria. Es necesario el seguimiento a largo plazo del paciente. Presentamos un caso con osteolisis incipiente de la fosa cerebral media.Synovial chondromatosis very rarely affects the TMJ. It is a metaplastic disorder of the synovial tissue that usually produces intra-articular loose bodies, or chondromas. It has variable clinical features and the symptoms are unspecific. Examination by means of magnetic resonance imaging and arthroscopic observation are the diagnostic techniques of choice. Treatment consists in the removal the loose bodies and partial synovectomy. Occasionally the skull has been destroyed and the middle cranial fossa invaded. There are cases of malignant transformation to chondrosarcoma. A long-term follow up is necessary. We describe a case with incipient intracranial extension.

  3. Analysis on the diagnostic value of CT in diagnosing malignant tumor of sternum%恶性胸骨肿瘤CT诊断价值临床分析

    Institute of Scientific and Technical Information of China (English)

    陈和平; 龙德云; 陈明安; 邵伟新

    2013-01-01

    [Objective] To investigate the value of CT in diagnosing malignant bone tumor of sternum.[Method] 41 patients with malignant bone tumor in the sternum were confirmed clinically and pathologically,included metastasis tumor in 37 cases(from lung in 27,breast cancer in 7,and one eacb from esophagus cancer,prostate cancer and osteosarcoma),4 cases with primary bone tumor in sternum (2 cases of myeloma,1 case of chondrosarcoma,1 case of osteogenic sarcoma).[Results]The local osseous changes of sternum,were osteolytic destruction in 26 cases (including 18 cases of metastatic tumor come from lung cancer,5 cases of breast cancer,2 cases of myeloma and 1 case of osteogenic sarcoma).8 cases were osteoblastic destruction(including 6 cases of metastatic tumor come from lung cancer,1 case of esophagus cancer and 1 case of osteosarcoma).7 cases were mixed destruction of bone(including 3 cases of metastatic tumor come from lung cancer,2 cases of breast cancer,1 case of prostate cancer,1 case of chondrosarcoma).21 cases had mass of soft tissue in sternum,17 cases were infiltration in the anterior muscles of sternum,subcutaneous fattissue and in the gap of the sternum nrear the fattissue and 2 cases with several nodular protrusion in the surface of skin.[Conclusion] Most malignant bone tumors of sternum are metastatic,CT is of great value in the diagnosis of bone tumor in the sternum.%[目的]分析探讨恶性胸骨肿瘤临床CT诊断价值和检查意义.[方法]搜集分析经临床病理证实的41例恶性胸骨肿瘤CT资料,其中胸骨转移性肿瘤37例(肺癌转移27例;乳腺癌转移7例;食道癌、前列腺癌及骨肉瘤转移各1例).原发性胸骨肿瘤4例(骨髓瘤2例、软骨肉瘤1例、骨肉瘤1例).[结果]胸骨局部骨质改变,其中溶骨性骨破坏26例(肺癌转移18例、乳腺癌转移5例、骨髓瘤2例、骨肉瘤1例).成骨性骨破坏8例(肺癌转移6例、食道癌转移1例、骨肉瘤转移1例).混合性骨破坏7

  4. Quantitative imaging of D-2-hydroxyglutarate (D2HG in selected histological tissue areas by a novel bioluminescence technique

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    Nadine Fabienne Voelxen

    2016-03-01

    Full Text Available AbstractPatients with malignant gliomas have a poor prognosis with average survival of less than one year. Whereas in other tumor entities the characteristics of tumor metabolism are successfully used for therapeutic approaches, such developments are very rare in brain tumors, notably in gliomas. One metabolic feature characteristic of gliomas, in particular diffuse astrocytomas and oligodendroglial tumors, is the variable content of D-2-hydroxyglutarate (D2HG, a metabolite, which was discovered first in this tumor entity. D2HG is generated in large amounts due to various gain-of–function mutations in the isocitrate dehydrogenases IDH-1 and IDH-2. Meanwhile, D2HG has been detected in several other tumor entities including intrahepatic bile-duct cancer, chondrosarcoma, acute myeloid leukemia, and angioimmunoblastic T-cell lymphoma. D2HG is barely detectable in healthy tissue (< 0.1 mM, but its concentration increases up to 35 mM in malignant tumor tissues. Consequently, the oncometabolite D2HG has gained increasing interest in the field of tumor metabolism. To facilitate its quantitative measurement without loss of spatial resolution at a microscopical level, we have developed a novel bioluminescence assay for determining D2HG in sections of snap-frozen tissue. The assay was verified independently by photometric tests and liquid chromatography / mass spectrometry (LC/MS. The novel technique allows the microscopically resolved determination of D2HG in a concentration range of 0 – 10 µmol/g tissue (wet weight. In combination with the already established bioluminescence imaging techniques for ATP, glucose, pyruvate, and lactate, the novel D2HG assay enables a comparative characterization of the metabolic profile of individual tumors in a further dimension.

  5. The effects of insulin on the expression levels of ADAMTS6 & 19 in OUMS-27 cell

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    Veli Uğurcu

    2014-09-01

    Full Text Available Objective: A Disintegrin-like Metalloproteinase with Thrombospondin Motifs (ADAMTS proteins are kind of matrix metalloproteinase enzymes that primarily founds in the extracellular matrix (ECM. Insulin is an important anabolic hormone, which acts on many tissues. The aim of this study is to evaluate the time-dependent effects of insulin on the two functionally unknown enzyme expressions (ADAMTS6 & 19 in OUMS-27 human chondrosarcoma cell line. Methods:OUMS-27 cells were cultured in Dulbecco’s modified Eagle’ medium (DMEM alone and DMEM containing 10 μg/mL insulin. The medium was changed every other day up to 11th day. Cells were harvested at 1, 3, 7,and 11th days and RNA isolation was performed at appropriate times according to study setup. The levels of RNA expression of ADAMTS6 and 19 were estimated by qRT-PCR using appropriate primers. Results: According to qRT-PCR analysis, ADAMTS6 mRNA expression was found to be decreased as early as one day after insulin application and continued up to day 11, the last day of insulin induction (p=0.008. The ratio of ADAMTS6 in insulin-applied groups was changed between 1/2 and 1/4 of control values. The changes in ADAMTS19 mRNA levels in insulin-applied cells were not statistically significant compared to control cell group. Conclusion:Our results demonstrated that insulin has a potential effect on alleviation of loss of extracellular matrix compounds by diminishing ADAMTS6 levels. To test this hypothesis and finding, more investigations are needed to recognize the real functions of orphan ADAMTS proteins.

  6. Role of Scintigraphy with Technetium-99m Depreotide in the Diagnosis and Management of Patients with Suspected Lung Cancer

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    Axelsson, R.; Herlin, G.; Baaaath, M.; Aspelin, P.; Koelbeck, K.G. (Div. of Radiology, Dept. of Clinical Science, Intervention and Technology (CLINTEC), and Div. of Pulmonary Medicine and Allergology, Dept. of Medicine, Karolinska Inst., Stockholm (Sweden))

    2008-04-15

    Background: In Sweden, there are over 3000 new lung cancer cases every year. There are still numerous patients with undetermined lesions after routine diagnostic evaluation by clinical examination, chest radiography, computed tomography (CT) of the thorax, and bronchoscopy. An appropriate method for further diagnostic workup is therefore needed. Purpose: To evaluate the diagnostic value of the somatostatin analogue depreotide in patients with suspected lung cancer, and to determine in which clinical settings it would be beneficial to use 99mTc-depreotide scintigraphy. Material and Methods: We included 99 consecutive patients referred to our hospital with suspected lung cancer. A clinical examination, bronchoscopy, chest radiography, CT of the thorax and upper abdomen, and scintigraphy were done. Scintigraphy was performed after injection of 740 MBq 99mTc depreotide with tomographical imaging of the thorax and whole-body scanning. The diagnostic outcome of the scintigrams was compared to CT, using morphology or clinical outcome as the endpoint. Results: 99mTc-depreotide uptake was found in 62 out of 66 malignancies, including 57 of 58 primary lung cancer cases. Two cases of lung metastasis (one from a colon cancer and one from an adenoid cystic carcinoma originating in the palate) and one rib chondrosarcoma did not show depreotide uptake. There were 33 patients with benign lesions, of whom 16 displayed false-positive 99mTc-depreotide uptake, whereof 11 were pneumonias. Tc-99m-depreotide uptake was absent in 17 patients with benign lesions, including all 10 hamartomas. The sensitivity in detecting malignancy was 94%, and in detecting lung cancer 98%. The specificity was calculated based on two sets of data. When all cases were used, the specificity was 52%. If the 12 pneumonias are excluded, the specificity was 77%. Conclusion: 99mTc-depreotide scintigraphy has a high sensitivity in detecting lung cancer. The method is useful in decision-making with respect to

  7. In vitro study of intracellular IL-1beta production and beta1 integrins expression in stimulated chondrocytes--effect of rhein.

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    Gigant-Huselstein, C; Dumas, D; Payan, E; Muller, S; Bensoussan, D; Netter, P; Stoltz, J F

    2002-01-01

    The purpose of the present study was to investigate the intracellular IL-1beta production and beta1 integrins (alpha4/beta1 and alpha5/beta1) expression on chondrocytes. Chondroytes monolayer (human chondrosarcoma cell line HEM-C55) were incubated for 12, 24 and 48 hours in the presence of Tumor Necrosis Factor-alpha (TNF-alpha, Sigma, France) or recombinant human IL-1alpha (rh-IL1alpha, Becton Dickinson, France). After direct immunolabelling, cells were either analyzed on FACScan flow cytometer (Becton Dickinson, France), or observed under an epi-fluorescence inverted microscope equipped with the CellScan EPR optical scanning acquisition system (IPLab-Scanalytics, USA). We found that the IL-1beta mean fluorescence intensity in flow cytometry and in 3D microscopy was increased in the presence of TNF-alpha or rh-IL-1alpha, and alpha4/beta1 or alpha5/beta1 expression was higher on stimulated cells than on control cells. On the other hand, we have evaluated the in vitro effects of rhein (10(-5) M, Negma, France), an active metabolite of diacerein, on the intracellular IL-1beta and beta1 integrins expressed by stimulated or no-stimulated chondrocytes. The results indicated that rhein leads to a reduction of IL-1beta synthesis whereas a weak decrease of beta1 integrins receptors expression is observed. From this study, it seems that rhein partially reduce cytokine-induced intracellular IL-1beta production, and it has a weak action on alpha4/beta1 or alpha5/beta1 receptors.

  8. A Novel Regulatory Mechanism of Type II Collagen Expression via a SOX9-dependent Enhancer in Intron 6.

    Science.gov (United States)

    Yasuda, Hideyo; Oh, Chun-do; Chen, Di; de Crombrugghe, Benoit; Kim, Jin-Hoi

    2017-01-13

    Type II collagen α1 is specific for cartilaginous tissues, and mutations in its gene are associated with skeletal diseases. Its expression has been shown to be dependent on SOX9, a master transcription factor required for chondrogenesis that binds to an enhancer region in intron 1. However, ChIP sequencing revealed that SOX9 does not strongly bind to intron 1, but rather it binds to intron 6 and a site 30 kb upstream of the transcription start site. Here, we aimed to determine the role of the novel SOX9-binding site in intron 6. We prepared reporter constructs that contain a Col2a1 promoter, intron 1 with or without intron 6, and the luciferase gene. Although the reporter constructs were not activated by SOX9 alone, the construct that contained both introns 1 and 6 was activated 5-10-fold by the SOX9/SOX5 or the SOX9/SOX6 combination in transient-transfection assays in 293T cells. This enhancement was also observed in rat chondrosarcoma cells that stably expressed the construct. CRISPR/Cas9-induced deletion of intron 6 in RCS cells revealed that a 10-bp region of intron 6 is necessary both for Col2a1 expression and SOX9 binding. Furthermore, SOX9, but not SOX5, binds to this region as demonstrated in an electrophoretic mobility shift assay, although both SOX9 and SOX5 bind to a larger 325-bp fragment of intron 6 containing this small sequence. These findings suggest a novel mechanism of action of SOX5/6; namely, the SOX9/5/6 combination enhances Col2a1 transcription through a novel enhancer in intron 6 together with the enhancer in intron 1.

  9. Imaging appearances and clinical outcome following sacrectomy and ilio-lumbar reconstruction for sacral neoplasia

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    Thomas, Marianna; Davies, A.M.; James, Steven L.J. [Department of Radiology, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham (United Kingdom); Stirling, A.J.; Grainger, M. [Department of Spinal Surgery, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham (United Kingdom); Grimer, R.J. [Department of Orthopaedic Oncology, The Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham (United Kingdom)

    2014-02-15

    Sacrectomy and ilio-lumbar reconstruction is an uncommonly performed complex surgical procedure for the treatment of sacral neoplasia. There are many challenges in the post-operative period including the potential for tumor recurrence, infection, and construct failure. We present our experience of this patient cohort and describe the complications and imaging appearances that can be encountered during the follow-up period. Retrospective review of our Orthopaedic Oncology database was undertaken which has been collected over a 30-year period to identify patients that had undergone sacrectomy and ilio-lumbar reconstruction. Pre and post-operative imaging including radiographs, CT, and MRI was reviewed. These were viewed by two experienced musculoskeletal radiologists with consensus opinion if there was disagreement over the imaging findings. Data regarding patient demographics, tumor type, and dimensions was collected. Serial review of radiographs, CT, and MRI was performed to assess implant position and integrity, strut graft position and union, and for the presence of recurrence within the surgical bed. Five male and two female patients (mean age 36 years, age range 15-54 years) were treated with this procedure. Histological diagnoses included chordoma, chondrosarcoma, osteosarcoma, and spindle cell sarcoma. Mean maximal tumor size on pre-operative imaging was 10.7 cm (range, 6-16 cm). Post-operative follow-up ranged from 10-46 months. A total of 76 imaging studies were reviewed. Commonly identified complications included vertical rod and cross-connector fracture and screw loosening. Fibula strut graft non-union and fracture was also evident on imaging review. Two patients demonstrated disease recurrence during the follow-up period. This study demonstrates the spectrum and frequency of complications that can occur following sacrectomy and ilio-lumbar reconstruction for sacral neoplasia. (orig.)

  10. Long-term outcome of free fibula osteocutaneous flap and massive allograft in the reconstruction of long bone defect.

    Science.gov (United States)

    Halim, Ahmad Sukari; Chai, Siew Cheng; Wan Ismail, Wan Faisham; Wan Azman, Wan Sulaiman; Mat Saad, Arman Zaharil; Wan, Zulmi

    2015-12-01

    Reconstruction of massive bone defects in bone tumors with allografts has been shown to have significant complications including infection, delayed or nonunion of allograft, and allograft fracture. Resection compounded with soft tissue defects requires skin coverage. A composite osteocutaneous free fibula offers an optimal solution where the allografts can be augmented mechanically and achieve biological incorporation. Following resection, the cutaneous component of the free osteocutaneous fibula flaps covers the massive soft tissue defect. In this retrospective study, the long-term outcome of 12 patients, who underwent single-stage limb reconstruction with massive allograft and free fibula osteocutaneous flaps instead of free fibula osteal flaps only, was evaluated. This study included 12 consecutive patients who had primary bone tumors and had follow-up for a minimum of 24 months. The mean age at the time of surgery was 19.8 years. A total of eight patients had primary malignant bone tumors (five osteosarcomas, two chondrosarcomas and one synovial sarcoma), and four patients had benign bone tumors (two giant-cell tumors, one aneurysmal bone cyst, and one neurofibromatosis). The mean follow-up for the 12 patients was 63 months (range 24-124 months). Out of the 10 patients, nine underwent lower-limb reconstruction and ambulated with partial weight bearing and full weight bearing at an average of 4.2 months and 8.2 months, respectively. In conclusion, augmentation of a massive allograft with free fibula osteocutaneous flap is an excellent alternative for reducing the long-term complication of massive allograft and concurrently addresses the soft tissue coverage.

  11. Prevalence of Malignant Soft Tissue Tumors inExtremities: An Epidemiological Study in Syria

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    Habib Reshadi

    2014-06-01

    Full Text Available Background:   Although the majority of soft tissue masses are benign, it is important to consider malignancy in differential diagnoses. Because most soft tissue sarcomas present as a painless mass, clinicians must watch for signs suggestive of malignancy, including large size, rapid growth, and site deep into the deep fascia.The purpose of this study was to determine the relative prevalence according to sex and age, site of tumor, skeletal distribution, and treatment (surgery, chemotherapy and radiotherapy before and after surgery, and ascertain the relative frequency of these tumors in specific anatomic sites and age groups based on pathological studies. Methods: A total of 308 patients, with a musculoskeletal tumor were evaluated retrospectively. All of the patients enrolled into this study were referred to the Beirouni Hospital of Damascus University with a proven diagnosis of alignant soft tissue tumors from the beginning of January 2008 until the end of 2010. The prevalence of the malignant soft tissue tumors in these patients was analyzed. For purposes of analysis, all lesions were placed in 1 of 9 categories: hand and wrist, forearm, humorous (arm, proximal limb girdle (axilla and shoulder, foot and ankle, thigh, hip and buttocks region, trunk, and other lesions. Age and sex also were recorded. Results: Malignant tumors consisted of seven diagnostic categories: malignant fibrous histiocytoma (23%, liposarcoma (22%, rhabdomyosarcoma (9%, leiomyosarcoma (8%, malignant schwannoma (5%, dermatofibrosarcoma protuberans (5%, synovial sarcoma (10%, fibrosarcoma (13%, extraskeletal chondrosarcoma (1%, and extraskeletal Ewing sarcoma (4%. Conclusions: Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the site of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in

  12. Prevalence of Malignant Soft Tissue Tumors inExtremities: An Epidemiological Study in Syria

    Directory of Open Access Journals (Sweden)

    Habib Reshadi

    2014-06-01

    Full Text Available Background:   Although the majority of soft tissue masses are benign, it is important to consider malignancy in differential diagnoses. Because most soft tissue sarcomas present as a painless mass, clinicians must watch for signs suggestive of malignancy, including large size, rapid growth, and site deep into the deep fascia.The purpose of this study was to determine the relative prevalence according to sex and age, site of tumor, skeletal distribution, and treatment (surgery, chemotherapy and radiotherapy before and after surgery, and ascertain the relative frequency of these tumors in specific anatomic sites and age groups based on pathological studies. Methods: A total of 308 patients, with a musculoskeletal tumor were evaluated retrospectively. All of the patients enrolled into this study were referred to the Beirouni Hospital of Damascus University with a proven diagnosis of alignant soft tissue tumors from the beginning of January 2008 until the end of 2010. The prevalence of the malignant soft tissue tumors in these patients was analyzed. For purposes of analysis, all lesions were placed in 1 of 9 categories: hand and wrist, forearm, humorous (arm, proximal limb girdle (axilla and shoulder, foot and ankle, thigh, hip and buttocks region, trunk, and other lesions. Age and sex also were recorded. Results: Malignant tumors consisted of seven diagnostic categories: malignant fibrous histiocytoma (23%, liposarcoma (22%, rhabdomyosarcoma (9%, leiomyosarcoma (8%, malignant schwannoma (5%, dermatofibrosarcoma protuberans (5%, synovial sarcoma (10%, fibrosarcoma (13%, extraskeletal chondrosarcoma (1%, and extraskeletal Ewing sarcoma (4%. Conclusions: Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the site of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in

  13. Differential transactivation by orphan nuclear receptor NOR1 and its fusion gene product EWS/NOR1: possible involvement of poly(ADP-ribose) polymerase I, PARP-1.

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    Ohkura, Naganari; Nagamura, Yuko; Tsukada, Toshihiko

    2008-10-15

    In extraskeletal myxoid chondrosarcoma, a chromosomal translocation creates a gene fusion between EWS and an orphan nuclear receptor, NOR1. The resulting fusion protein EWS/NOR1 has been believed to lead to malignant transformation by functioning as a transactivator for NOR1-target genes. By comparing the gene expression profiles of NOR1- and EWS/NOR1-overexpressing cells, we found that they largely shared up-regulated genes, but no significant correlation was observed with respect to the transactivation levels of each gene. In addition, the proteins associated with NOR1 and EWS/NOR1 were mostly the same in these cells. The results suggest that these proteins differentially transactivate overlapping target genes through a similar transcriptional machinery. To clarify the mechanisms underlying the transcriptional divergence between NOR1 and EWS/NOR1, we searched for alternatively associated proteins, and identified poly(ADP-ribose) polymerase I (PARP-1) as an NOR1-specific binding protein. Consistent with its binding properties, PARP-1 acted as a transcriptional repressor of NOR1, but not EWS/NOR1, in a luciferase reporter assay employing PARP-1(-/-) fibroblasts. Interestingly, suppressive activity of PARP-1 was observed in a DNA response element-specific manner, and in a subtype-specific manner toward the NR4A family (Nur77, Nurr1, and NOR1), suggesting that PARP-1 plays a role in the diversity of transcriptional regulation mediated by the NR4A family in normal cells. Altogether, our findings suggest that NOR1 and EWS/NOR1 regulate overlapping target genes differently by utilizing associated proteins, including PARP-1; and that EWS/NOR1 may acquire oncogenic activities by avoiding (or gaining) transcription factor-specific modulation by the associated proteins.

  14. Primary intracranial soft tissue sarcomas in children, adolescents, and young adults: single institution experience and review of the literature.

    Science.gov (United States)

    Maher, Ossama M; Khatua, Soumen; Mukherjee, Devashis; Olar, Adriana; Lazar, Alexander; Luthra, Raja; Liu, Diane; Wu, Jimin; Ketonen, Leena; Zaky, Wafik

    2016-03-01

    There is a paucity of literature reporting the outcome of intracranial sarcomas (IS) in children, adolescents, and young adults (CAYA). A multimodal therapeutic approach is commonly used, with no well-established treatment consensus. We conducted a retrospective review of CAYA with IS, treated at our institution, to determine their clinical findings, treatments, and outcomes. Immunohistochemistry (PDGFRA and EGFR) and DNA sequencing were performed on 5 tumor samples. A literature review of IS was also conducted. We reviewed 13 patients (median age, 7 years) with a primary diagnosis of IS between 1990 and 2015. Diagnoses included unclassified sarcoma (n = 9), chondrosarcoma (n = 2), and rhabdomyosarcoma (n = 2). Five patients underwent upfront gross total resection (GTR) of the tumor. The 5-drug regimen (vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide) was the most common treatment used. Nine patients died due to progression or recurrence (n = 8) or secondary malignancy (n = 1). The median follow-up period of the 4 surviving patients was 1.69 years (range 1.44-5.17 years). The 5-year progression-free survival and overall survival rates were 21 and 44 %, respectively. BRAF, TP53, KRAS, KIT, ERBB2, MET, RET, ATM, and EGFR mutations were detected in 4 of the 5 tissue samples. All 5 samples were immunopositive for PDGFRA, and only 2 were positive for EGFR. IS remain a therapeutic challenge due to high progression and recurrence rates. Collaborative multi-institutional studies are warranted to delineate a treatment consensus and investigate tumor biology to improve the disease outcome.

  15. Analysis of limb function after various reconstruction methods according to tumor location following resection of pediatric malignant bone tumors

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    Tokuhashi Yasuaki

    2010-05-01

    Full Text Available Abstract Background In the reconstruction of the affected limb in pediatric malignant bone tumors, since the loss of joint function affects limb-length discrepancy expected in the future, reconstruction methods that not only maximally preserve the joint function but also maintain good limb function are necessary. We analysis limb function of reconstruction methods by tumor location following resection of pediatric malignant bone tumors. Patients and methods We classified the tumors according to their location into 3 types by preoperative MRI, and evaluated reconstruction methods after wide resection, paying attention to whether the joint function could be preserved. The mean age of the patients was 10.6 years, Osteosarcoma was observed in 26 patients, Ewing's sarcoma in 3, and PNET(primitive neuroectodermal tumor and chondrosarcoma (grade 1 in 1 each. Results Type I were those located in the diaphysis, and reconstruction was performed using a vascularized fibular graft(vascularized fibular graft. Type 2 were those located in contact with the epiphyseal line or within 1 cm from this line, and VFG was performed in 1, and distraction osteogenesis in 1. Type III were those extending from the diaphysis to the epiphysis beyond the epiphyseal line, and a Growing Kotz was mainly used in 10 patients. The mean functional assessment score was the highest for Type I (96%: n = 4 according to the type and for VFG (99% according to the reconstruction method. Conclusion The final functional results were the most satisfactory for Types I and II according to tumor location. Biological reconstruction such as VFG and distraction osteogenesis without a prosthesis are so high score in the MSTS rating system. Therefore, considering the function of the affected limb, a limb reconstruction method allowing the maximal preservation of joint function should be selected after careful evaluation of the effects of chemotherapy and the location of the tumor.

  16. RANK/RANKL/OPG LIGAND-RECEPTOR SYSTEM AND ITS ROLE IN PRIMARY BONE NEOPLASMS (LITERATURE ANALYSIS AND OWN DATA

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    E. S. Gershtein

    2015-01-01

    Full Text Available RANK/RANKL/OPG ligand-receptor system is a key player in bone homeostasis regulation directly regulating osteoclast differentiation and osteolysis. Disbalance of bone homeostasis associated with malfunctioning of RANK/RANKL/OPG system underlies such oncological processes as the destruction of bone, metastasis development, tumor progression. Involvement of RANK/RANKL/OPG system in the development of metastasis from various tumors is practically confirmed, but its influence on the development and progression of primary bone tumors still needs thorough evaluation. , In this paper experimental and clinical-laboratory data on the role of RANK/RANKL/OPG system in primary bone tumors pathogenesis available in modern literature are summarized with special attention,paid to giant-cell bone tumor (GCBT that is already treated with RANK/RANKL interaction inhibitor denosumab. Results of authors study of the levels of RANK/RANKL/OPG system,s components in blood serum of 101 malignant bone tumor (37 – osteosarcoma, 41 – chondrosarcoma, 12 – chordoma, 7 – Ewing sarcoma, 2 – pleomorhic undifferentiated sarcoma, 2 – fibrosarcoma, 32 borderline GCBT, and 30 benign bone tumor patients are also presented. The disturbances in the balance of osteolysis activators and inhibitors in patients with primary bone tumor depending both on the character of neoplasm (malignant, borderline or benign, and histological structure of malignant tumors were demonstrated. The most striking changes in RANK/ RANKL/OPG system manifesting itself in an increase of serum concentrations of all its components and strengthening of association between the levels of soluble receptor and its natural inhibitor OPG were revealed in giant-cell bone tumor patients.The study of the role of RANK/RANKL/OPG system in primary bone neoplasms is a topical goal for clinical investigations; it is also a promising tool for development of new diagnostic methods and for targeted application of

  17. Synthesis and Biological Activity of Substituted Urea and Thiourea Derivatives Containing 1,2,4-Triazole Moieties

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    David E. Wedge

    2013-03-01

    Full Text Available A series of novel thiourea and urea derivatives containing 1,2,4-triazole moieties were synthesized and evaluated for their antifungal and larvicidal activity. Triazole derivatives 3a–e and 4a–e were synthesized by reacting thiocarbohydrazide with thiourea and urea compounds 1a–e and 2a–e, respectively, in a 130–140 °C oil bath. The proposed structures of all the synthesized compounds were confirmed using elemental analysis, UV, IR, 1H-NMR and mass spectroscopy. All compounds were evaluated for antifungal activity against plant pathogens, larvicidal and biting deterrent activity against the mosquito Aedes aegypti L. and in vitro cytotoxicity and anti-inflammatory activity against some human cell lines. Phomopis species were the most sensitive fungi to these compounds. Compounds 1b, 1c, 3a and 4e demonstrated selectively good activity against Phomopis obscurans and only 1b and 4e showed a similar level of activity against P. viticola. Compound 3d, with a LD50 value of 67.9 ppm, followed by 1c (LD50 = 118.8 ppm and 3e (LD50 = 165.6 ppm, showed the highest toxicity against Aedes aegypti larvae. Four of these compounds showed biting deterrent activity greater than solvent control, with the highest activity being seen for 1c, with a proportion not biting (PNB value of 0.75, followed by 1e, 2b and 1a. No cytotoxicity was observed against the tested human cancer cell lines. No anti-inflammatory activity was observed against NF-kB dependent transcription induced by phorbol myristate acetate (PMA in human chondrosarcoma cells.

  18. Electrostatic self-assembly of multilayer copolymeric membranes on the surface of porous tantalum implants for sustained release of doxorubicin

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    Guo X

    2011-11-01

    Full Text Available Xinming Guo1,*, Muwan Chen1,2,*, Wenzhou Feng1,*, Jiabi Liang1, Huibin Zhao1, Lin Tian1, Hui Chao3, Xuenong Zou11Orthopaedic Research institute/Department of Orthopaedic Surgery, the First Affiliated Hospital and Department of Pharmacy, the Fifth Affiliated Hospital, Sun Yat-sen University, Guangzhou, People's Republic of China; 2Interdisciplinary Nanoscience Center (iNANO, Aarhus University, Denmark; 3Ministry of Education Key Laboratory of Bioinorganic and Synthetic Chemistry, School of Chemistry and Chemical Engineering of Sun Yat-sen University, Guangzhou, People's Republic of China *The first three authors contributed equally to this work as co-first authorAbstract: Many studies in recent years have focused on surface engineering of implant materials in order to improve their biocompatibility and other performance. Porous tantalum implants have increasingly been used in implant surgeries, due to their biocompatibility, physical stability, and good mechanical strength. In this study we functionalized the porous tantalum implant for sustained drug delivery capability via electrostatic self-assembly of polyelectrolytes of hyaluronic acid, methylated collagen, and terpolymer on the surface of a porous tantalum implant. The anticancer drug doxorubicin was encapsulated into the multilayer copolymer membranes on the porous tantalum implants. Results showed the sustained released of doxorubicin from the functionalized porous tantalum implants for up to 1 month. The drug release solutions in 1 month all had inhibitory effects on the proliferation of chondrosarcoma cell line SW1353. These results suggest that this functionalized implant could be used in reconstructive surgery for the treatment of bone tumor as a local, sustained drug delivery system.Keywords: self-assembly, surface modification, tantalum, drug delivery system, doxorubicin, bone tumor

  19. Endocrine function following high dose proton therapy for tumors of the upper clivus

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    Slater, J.D.; Austin-Seymour, M.; Munzenrider, J.; Birnbaum, S.; Carroll, R.; Klibanski, A.; Riskind, P.; Urie, M.; Verhey, L.; Goitein, M.

    1988-09-01

    The endocrine status of patients receiving proton radiation for tumors of the upper clivus was reviewed to evaluate the effect of high dose treatment on the pituitary gland. The fourteen patients had chordomas or low grade chondrosarcomas and were all treated by the same techniques. The median tumor dose was 69.7 Cobalt Gray Equivalent (CGE) with a range from 66.6 to 74.4 CGE. (CGE is used because modulated protons have an RBE of 1.1 compared to 60Co). The daily fraction size was 1.8-2.1 CGE. The median follow-up time is 48 months, ranging from 30 to 68 months. All treatments were planned using a computerized multi-dimensional system with the position of the pituitary outlined on the planning CT scan. Review of the dose distribution indicated that the dose to the pituitary ranged from 60.5 to 72.3 CGE, with a median of 67.6 CGE. One female patient had decreased thyroid and gonadotropin function at the time of diagnosis and has been on hormone replacement since that time. The other three females were all pre-menopausal at the time of radiotherapy. At this time four patients (3 males and 1 female) have developed endocrine abnormalities 14 to 45 months after irradiation. All four had evidence of hypothyroidism and two have also developed corticotropin deficiency. The three males had decreased testosterone levels; the female patient developed amenorrhea and hyperprolactinemia. All four are asymptomatic with ongoing hormone replacement.

  20. Occupational factors and risk of adult bone sarcomas: a multicentric case-control study in Europe.

    Science.gov (United States)

    Merletti, Franco; Richiardi, Lorenzo; Bertoni, Franco; Ahrens, Wolfgang; Buemi, Antoine; Costa-Santos, Cristina; Eriksson, Mikael; Guénel, Pascal; Kaerlev, Linda; Jöckel, Karl-Heinz; Llopis-Gonzalez, Agustin; Merler, Enzo; Miranda, Ana; Morales-Suárez-Varela, Maria M; Olsson, Håkan; Fletcher, Tony; Olsen, Jorn

    2006-02-01

    We investigated the association between occupational factors and risk of bone sarcoma, a rare tumor with a largely unknown aetiology. A multicentric case-control study was conducted in 7 European countries in 1995-97. Ninety-six cases aged 35-69 years with a centrally reviewed diagnosis of bone sarcoma (68 chondrosarcomas and 28 osteosarcomas) were compared to 2,632 population (68%) or colon cancer (32%) controls. Subjects were interviewed to obtain information on occupational, medical and reproductive history, smoking and alcohol consumption and selected exposures including use of pesticides. Response proportions were 90% among cases and 66% among controls. Odds ratios (OR) and 95% confidence intervals (CI) were estimated for selected categories of job titles and branches of industry and for use of pesticides. We found an increased OR for bone sarcoma among blacksmiths, toolmakers, machine-tool operators (OR = 2.14, 95% CI 1.08-4.26), woodworkers (OR = 2.68, 95% CI 1.36-5.29) and construction workers (OR = 1.62, 95% CI 0.92-2.87). Ever users of pesticide had an OR of 2.33 (95% CI 1.31-4.13), with similar risks for exposure to insecticides and exposure to herbicides. Neither duration of employment in any of the analyzed occupational categories nor duration of use of pesticides showed an increasing trend in the risk of bone sarcoma. ORs of bone sarcoma were 1.03 (95% CI 0.23-4.57), 3.13 (95% CI 1.26-7.76) and 1.44 (95% CI 0.43-4.85) for the first, second and third tertile of days of use of pesticides. Our study suggests that novel and previously reported (woodworking) occupational factors play a role in the aetiology of bone sarcomas.

  1. Chondroblastoma-like chondroma of soft tissue: report of the first case in the base of skull.

    Science.gov (United States)

    Raparia, Kirtee; Lin, Jerry W; Donovan, Donald; Vrabec, Jeffrey T; Zhai, Qihui Jim; Ayala, Alberto A; Ro, Jae Y

    2013-06-01

    Chondroblastoma-like chondroma (CLC) of soft tissue is a rare benign neoplasm that usually involves the soft tissues of the hand. This report describes the first case of CLC of soft tissue arising in the base of the skull. A 33-year-old man was seen with a slow growing mass in the right parotid region of his face. The noncontrast computed tomographic scans showed an 8.5-cm mass with calcifications involving the right masticator space and extending through the bone into the middle cranial fossa. The radiologic differential diagnosis included osteosarcoma, leiomyosarcoma, chondrosarcoma, and giant cell tumor. During surgery, the large lateral skull base tumor appeared to involve the middle and infratemporal fossae and eroded the surrounding bone. Although the tumor was removed piecemeal, total excision was performed. On microscopic examination, the tumor displayed lobules of mature hyaline cartilage with numerous chondroblasts, coarse calcifications including chicken wire calcifications, and scattered osteoclasts. No atypia, mitoses, necrosis, or osteoid formation was seen. The tumor was diagnosed as chondroma with chondroblastoma features of the soft tissue. His postoperative clinical course was uneventful; however, after 7 months, he had a local recurrence identified on follow-up magnetic resonance imaging. He underwent repeat surgical excision of the tumor, which showed similar histology as the previous excision. This large skull based tumor eroding the bone, which clinically and radiologically mimicked a malignant process, was an unusual presentation of a benign cartilaginous neoplasm. Pathologists should be aware that CLC may occur in the base of the skull and this lesion should be differentiated from the other benign or malignant tumors arising in this area. These lesions have a potential for local recurrence; hence, a close follow-up is recommended.

  2. Preoperative embolization of primary bone tumors: A case control study

    Science.gov (United States)

    Jha, Roushan; Sharma, Raju; Rastogi, Shishir; Khan, Shah Alam; Jayaswal, Arvind; Gamanagatti, Shivanand

    2016-01-01

    AIM: To study the safety and effectiveness of preoperative embolization of primary bone tumors in relation to intraoperative blood loss, intraoperative blood transfusion volume and surgical time. METHODS: Thirty-three patients underwent preoperative embolization of primary tumors of extremities, hip or vertebrae before resection and stabilization. The primary osseous tumors included giant cell tumors, aneurysmal bone cyst, osteoblastoma, chondroblastoma and chondrosarcoma. Twenty-six patients were included for the statistical analysis (embolization group) as they were operated within 0-48 h within preoperative embolization. A control group (non-embolization group, n = 28) with bone tumor having similar histological diagnosis and operated without embolization was retrieved from hospital record for statistical comparison. RESULTS: The mean intraoperative blood loss was 1300 mL (250-2900 mL), the mean intraoperative blood transfusion was 700 mL (0-1400 mL) and the mean surgical time was 221 ± 76.7 min for embolization group (group I, n = 26). Non-embolization group (group II, n = 28), the mean intraoperative blood loss was 1800 mL (800-6000 mL), the mean intraoperative blood transfusion was 1400 mL (700-8400 mL) and the mean surgical time was 250 ± 69.7 min. On comparison, statistically significant (P < 0.001) difference was found between embolisation group and non-embolisation group for the amount of blood loss and requirement of blood transfusion. There was no statistical difference between the two groups for the surgical time. No patients developed any angiography or embolization related complications. CONCLUSION: Preoperative embolization of bone tumors is a safe and effective adjunct to the surgical management of primary bone tumors that leads to reduction in intraoperative blood loss and blood transfusion volume. PMID:27158424

  3. Chondroma of the skull base and maxilla

    Directory of Open Access Journals (Sweden)

    Kiralj Aleksandar

    2007-01-01

    Full Text Available Introduction. Chondromas are uncommon benign tumors of cartilaginous origin. Although chonodroma of the jaw is extremely rare, the commonly involved are the anterior portion of the maxilla, condyle and coronoid process. A chondroma is a painless, slow growing tumor causing destruction and exfoliation of teeth. On a radiograph, the tumor appears as a cyst-like radiolucent lesion, while some are sclerotic. The borders are usually ill-defined. Irregular calcifications may be seen with radiolucencies and then it is an osteochondroma. Material and Methods. We present a patient with a resected and histologically proven chondroma of the skull base and maxilla. A 65-year-old female was admitted to our clinic with swelling and breathing difficulties. MRI showed a large soft tissue mass of the skull base and maxilla. Clinicopathological and radiological features were examined by computed tomography (CT and magnetic resonance imaging (MRI. Discussion and Conclusion. Chondromyxoid fibroma (CMF is a rare, benign cartilaginous tumor that often occurs in the metaphyses of proximal tibia, proximal and distal femur and small bones of the foot. The differential diagnosis is wide and includes simple or aneyrismal bone cyst, giant cell tumor, nonossifying fibroma, fibrous dysplasia, enchondroma, chondroblastoma, eosinophilic granuloma and fibrous cortical defect. Our case demonstrates an uncommon occurrence in the maxillary sinus: CMF with nasal, pterygoid and orbital infiltration. In the diagnosis of an intracranial chondrocytic tumor, it is important to distinguish it from enchondroma and chondrosarcoma. MR provides a detailed assessment of soft tissue masses of the craniofacial region, while CT offers superior analysis of bone structure involvement. The present case underlines the importance of MR examination in the diagnosis of soft tissue masses in the craniofacial region.

  4. Chondromyxoid fibroma of rib with a novel chromosomal translocation: a report of four additional cases at unusual sites

    Directory of Open Access Journals (Sweden)

    Parwani Anil V

    2007-11-01

    Full Text Available Abstract Background Chondromyxoid fibromas (CMFs are rare benign chondroid/myxoid matrix-producing tumors that occur in metaphyses of long tubular bones, and very rarely in small bones of hands and feet. Flat bone involvement is even more uncommon. Prior cytogenetic analyses have identified complex abnormalities involving chromosome 6 in the majority of cases. Methods A search for CMF over an 8-year period (1999–2006 from the surgical pathology files of our institution yielded 16 cases. Four cases occurred in relatively unusual regions, three from the small bones of distal extremities and one from the rib. The rib lesion wassubmitted forroutinecytogenetic analysis. Results Radiographic studies revealed that all four lesions were well-defined expansile radiolucent lesions which expanded the bony cortices with lobulated margins, sclerotic rim, septation, and no calcification. Morphologically, all four lesions showed typical features of CMF and had low proliferative index with Ki-67. Cytogenetic analysis on the rib lesion revealed a novel chromosomal translocation, t(1;5(p13;p13. None of the four patients had a recurrence after a mean duration of follow-up of 24 months. Conclusion CMF originating in unusual locations should be distinguished from chondrosarcomas, especially on small biopsies, and should be included in the differential diagnosis. As previously noted in the literature, the cells can be positive for actin but unlike conventional chondroid neoplasms can be negative for S-100. To our knowledge, this is the first report describing a novel chromosomal translocation, t(1;5(p13;p13 in CMF.

  5. [Ankle joint prosthesis for bone defects].

    Science.gov (United States)

    Lampert, C

    2011-11-01

    Large defects of the talus, i.e. due to tumors, large areas of osteolysis in total ankle replacement (TAR) and posttraumatic talus body necrosis are difficult to manage. The gold standard in these circumstances is still tibiocalcaneal arthrodesis with all the negative aspects of a completely rigid hindfoot. We started 10 years ago to replace the talus by a custom-made, all cobalt-chrome implant (laser sintering). The first patient with a giant cell tumor did very well but the following patients showed all subsidence of the metal talus into the tibia due to missing bony edges. Therefore, we constructed a custom-made talus (mirrored from the healthy side) and combined it with a well functioning total ankle prosthesis (Hintegra). So far we have implanted this custom-made implant into 3 patients: the first had a chondrosarcoma of the talus (1 year follow-up), the second had massive osteolysis/necrosis of unknown origin (6 months follow-up) and the third massive osteolysis following a correct TAR (2 months follow-up). The results are very encouraging as all of the patients are practically pain free and have a good range of movement (ROM): D-P flexion 15°-0-20° but less motion in the lower ankle joint: ROM P-S 5°-0-5°. No subsidence was detected in the tibia or the calcaneus. The custom-made talus combined with the Hintegra total ankle replacement will probably be an interesting alternative to a tibiocalcaneal arthrodesis in selected cases with massive defects of the talus.

  6. An epidemiological survey of tumour or tumour like conditions in the scapula and periscapular region

    Science.gov (United States)

    Khan, Zeeshan; Gerrish, Adam M.; Grimer, Robert J.

    2016-01-01

    Introduction: The scapula is not an uncommon site for bone and soft tissue tumours and can be difficult to delineate on examination. Furthermore, these lesions can be potentially challenging to biopsy due to its close anatomical relationship with important structures. We present an epidemiological survey of all the scapular and periscapular lesions presenting to our institution. Methodology: This was a retrospective study with data obtained from a prospectively held electronic database over a 30-year period. Demographic and clinical data was obtained and various subgroup analyses were performed. Results: A total of 418 scapular lesions were included in the study where 132 lesions were found to be of soft tissue origin and 286 were osseous. Fifty-eight percent (n = 241) of all these lesions were malignant, of which 47% (n = 113) were primary sarcomas. The commonest malignant lesions were bone sarcomas (n = 96) followed by metastases (n = 88). The commonest primary bone sarcoma was chondrosarcoma (45%), whereas the commonest soft tissue sarcoma was high grade undifferentiated pleomorphic sarcoma (18%). The most common benign osseous and soft tissue lesions were osteochondroma (70%) and lipoma (26%), respectively. We noted that the incidence of malignancy increased with increasing age, however, the incidence of primary bone sarcomas was fairly consistent across different age groups. Conclusion: Based on our findings we recommend that suspicious lesions arising from the scapula should be dealt with in a specialist sarcoma unit with involvement of a multidisciplinary team to offer appropriate management and advice for optimum outcome. PMID:27739400

  7. Electrophysiological Monitoring in Patients With Tumors of the Skull Base Treated by Carbon-12 Radiation Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Carozzo, Simone [Department of Neuroscience, Ophthalmology, and Genetics, University of Genova, Genova (Italy); Schardt, Dieter [Department of Biophysics, GSI Helmholtzzentrum für Schwerionenforschung, Darmstadt (Germany); Narici, Livio [Department of Physics, University of Rome Tor Vergata, Rome (Italy); Combs, Stephanie E.; Debus, Jürgen [Department of Radiation Oncology, University of Heidelberg, Heidelberg (Germany); Sannita, Walter G., E-mail: wgs@dism.unige.it [Department of Neuroscience, Ophthalmology, and Genetics, University of Genova, Genova (Italy); Department of Psychiatry, State University of New York, Stony Brook, New York (United States)

    2013-03-15

    Purpose: To report the results of short-term electrophysiologic monitoring of patients undergoing {sup 12}C therapy for the treatment of skull chordomas and chondrosarcomas unsuitable for radical surgery. Methods and Materials: Conventional electroencephalogram (EEG) and retinal and cortical electrophysiologic responses to contrast stimuli were recorded from 30 patients undergoing carbon ion radiation therapy, within a few hours before the first treatment and after completion of therapy. Methodologies and procedures were compliant with the guidelines of the International Federation for Clinical Neurophysiology and International Society for Clinical Electrophysiology of Vision. Results: At baseline, clinical signs were reported in 56.6% of subjects. Electrophysiologic test results were abnormal in 76.7% (EEG), 78.6% (cortical evoked potentials), and 92.8% (electroretinogram) of cases, without correlation with neurologic signs, tumor location, or therapy plan. Results on EEG, but not electroretinograms and cortical responses, were more often abnormal in patients with reported clinical signs. Abnormal EEG results and retinal/cortical responses improved after therapy in 40% (EEG), 62.5% (cortical potentials), and 70% (electroretinogram) of cases. Results on EEG worsened after therapy in one-third of patients whose recordings were normal at baseline. Conclusions: The percentages of subjects whose EEG results improved or worsened after therapy and the improvement of retinal/cortical responses in the majority of patients are indicative of a limited or negligible (and possibly transient) acute central nervous system toxicity of carbon ion therapy, with a significant beneficial effect on the visual pathways. Research on large samples would validate electrophysiologic procedures as a possible independent test for central nervous system toxicity and allow investigation of the correlation with clinical signs; repeated testing over time after therapy would demonstrate, and may

  8. Neurofibromin specific antibody differentiates malignant peripheral nerve sheath tumors (MPNST) from other spindle cell neoplasms.

    Science.gov (United States)

    Reuss, David E; Habel, Antje; Hagenlocher, Christian; Mucha, Jana; Ackermann, Ulrike; Tessmer, Claudia; Meyer, Jochen; Capper, David; Moldenhauer, Gerhard; Mautner, Victor; Frappart, Pierre-Olivier; Schittenhelm, Jens; Hartmann, Christian; Hagel, Christian; Katenkamp, Kathrin; Petersen, Iver; Mechtersheimer, Gunhild; von Deimling, Andreas

    2014-04-01

    Malignant peripheral nerve sheath tumors (MPNST) derive from the Schwann cell or perineurial cell lineage and occur either sporadically or in association with the tumor syndrome neurofibromatosis type 1 (NF1). MPNST often pose a diagnostic challenge due to their frequent lack of pathognomonic morphological or immunohistochemical features. Mutations in the NF1 tumor suppressor gene are found in all NF1-associated and many sporadic MPNST. The presence of NF1 mutation may have the potential to differentiate MPNST from several morphologically similar neoplasms; however, mutation detection is hampered by the size of the gene and the lack of mutational hot spots. Here we describe a newly developed monoclonal antibody binding to the C-terminus of neurofibromin (clone NFC) which was selected for optimal performance in routinely processed formalin-fixed and paraffin-embedded tissue. NFC immunohistochemistry revealed loss of neurofibromin in 22/25 (88 %) of NF1-associated and 26/61 (43 %) of sporadic MPNST. There was a strong association of neurofibromin loss with deletions affecting the NF1 gene (P < 0.01). In a series of 256 soft tissue tumors of different histotypes NFC staining showed loss of neurofibromin in 2/8 myxofibrosarcomas, 2/12 (16 %) pleomorphic liposarcomas, 1/16 (6 %) leiomyosarcomas, and 4/28 (14 %) unclassified undifferentiated pleomorphic sarcomas. However, loss of neurofibromin was not observed in 22 synovial sarcomas, 27 schwannomas, 23 solitary fibrous tumors, 14 low-grade fibromyxoid sarcomas, 50 dedifferentiated liposarcomas, 27 myxoid liposarcomas, 13 angiosarcomas, 9 extraskeletal myxoid chondrosarcomas, and 7 epitheloid sarcomas. Immunohistochemistry using antibody NFC may substantially facilitate sarcoma research and diagnostics.

  9. Tumor induction following intraoperative radiotherapy: Late results of the National Cancer Institute canine trials

    Energy Technology Data Exchange (ETDEWEB)

    Barnes, M.; Duray, P.; DeLuca, A.; Anderson, W.; Sindelar, W.; Kinsella, T. (Fox Chase Cancer Center, Philadelphia, PA (USA))

    1990-09-01

    Intraoperative radiotherapy has been employed in human cancer research for over a decade. Since 1979, trials to assess the acute and late toxicity of IORT have been carried out at the National Cancer Institute in an adult dog model in an attempt to establish dose tolerance guidelines for a variety of organs. Of the 170 animals entered on 12 studies with a minimum follow-up of 2 years, 148 dogs received IORT; 22 control animals received only surgery. Animals were sacrificed at designated intervals following IORT, usually at 1, 6, 12, 24, and 60 month intervals. 102 of 148 irradiated dogs were sacrificed less than 24 months; 46 dogs were followed greater than or equal to 24 months after IORT. To date, 34 of the 46 animals have been sacrificed; the 12 remaining animals are to be followed to 5 years. These 12 animals have minimum follow-up of 30 months. In the irradiated group followed for greater than or equal to 24 months, 10 tumors have arisen in 9 animals. One animal developed an incidental spontaneous breast carcinoma outside the IORT port, discovered only at scheduled post-mortem exam. The remaining nine tumors arose within IORT ports. Two tumors were benign neural tumors--a neuroma and a neurofibroma. One animal had a collision tumor comprised of grade I chondrosarcoma adjacent to grade III osteosarcoma arising in lumbar vertebrae. Two other grade III osteosarcomas, one grade III fibrosarcoma, and one grade III malignant fibrous histiocytoma arose in retroperitoneal/paravertebral sites. An embryonal rhabdomyosarcoma (sarcoma botryoides) arose within the irradiated urinary bladder of one animal. No sham irradiated controls nor IORT animals sacrificed less than 24 months have developed any spontaneous or radiation-induced tumors. The time range of diagnoses of tumors was 24-58 months. The IORT dose range associated with tumor development was 20-35 Gy.

  10. Aberrations of 6q13 mapped to the COL12A1 locus in chondromyxoid fibroma.

    Science.gov (United States)

    Yasuda, Taketoshi; Nishio, Jun; Sumegi, Janos; Kapels, Kayla M; Althof, Pamela A; Sawyer, Jeffrey R; Reith, John D; Bridge, Julia A

    2009-11-01

    Chondromyxoid fibroma, a rare benign bone tumor, may be mistaken for chondrosarcoma. Although cytogenetic studies of chondromyxoid fibroma are few, rearrangements of the long arm of chromosome 6, frequently expressed as an inv(6)(p25q13), are prominent. In this study, conventional cytogenetic analysis of 16 chondromyxoid fibroma samples from 14 patients revealed rearrangements of chromosome 6 in 10 of 11 clonally abnormal specimens. In addition to 6q13 rearrangements, recurrent 6p25 and 6q25 anomalies were detected. Notably, an identical t(6;9)(q25;q22) translocation was identified in two cases, suggesting that it represents a distinct translocation of chondromyxoid fibroma. In an effort to further define the aberrant 6q13 breakpoint and identify the molecular consequences, a fluorescence in situ hybridization (FISH)-based positional cloning strategy on chondromyxoid fibroma abnormal metaphase and interphase cells using a series of bacterial and plasmid artificial chromosome (BAC/PAC) probe combinations spanning a 6.1 Mb region was employed. The breakpoint on 6q13 was located within the COL12A1 gene, a collagen gene purportedly involved in another benign bone tumor, subungual exostosis. The findings of this study expand our knowledge of chromosomal alterations in chondromyxoid fibroma, identify COL12A1 as the likely gene candidate within the recurrent 6q13 breakpoint, and provide an alternative approach for detecting 6q13 anomalies in nondividing cells of chondromyxoid fibroma. The latter could potentially be utilized as an adjunct in diagnostically challenging cases.

  11. Immunostaining for SYT protein discriminates synovial sarcoma from other soft tissue tumors: analysis of 146 cases.

    Science.gov (United States)

    He, Rui; Patel, Rajiv M; Alkan, Serhan; Hammadeh, Rasheed; Weiss, Sharon W; Goldblum, John R; Venkataraman, Girish; Baila, Horea

    2007-05-01

    Synovial sarcoma in its classic biphasic form can be distinguished readily from other soft tissue lesions; however, monophasic and poorly differentiated forms are diagnostically more problematic. For this reason, we assessed the efficacy of immunostaining for SYT and SSX1 proteins, the gene products resulting from unique synovial sarcoma translocation, to distinguish synovial sarcoma from other soft tissue lesions. A total number of 146 cases were analyzed, including 47 synovial sarcoma cases (all of which were verified by FISH to have t(X; 18) translocation and SYT-SSX fusion gene) and 99 soft tissue tumors of various types. A polyclonal IgG antibody against SYT was used to stain formalin-fixed paraffin embedded tissues. Forty-one out of 47 (87%) synovial sarcoma displayed strong positive nuclear staining (ranging from 80 to 90% of the tumor cells) for SYT antibody. Nineteen of 99 (19%) non-synovial sarcoma cases showed variable nuclear and cytoplasmic staining with SYT, which ranged from 20 to 60% of tumor nuclei, and included malignant peripheral nerve sheath tumor (5/25), solitary fibrous tumor (2/14), Ewing sarcoma (2/6), low grade fibromyxoid tumor (2/4), extraskeletal mesenchymal chondrosarcoma (2/6), gastrointestinal tumor (4/17), epithelioid sarcoma (2/2). The remaining non-synovial sarcomas were negative. This is the first study demonstrating SYT protein expression in tissue sections of synovial sarcoma. This method could provide an easy, rapid and widely applicable means of assisting in the diagnosis of synovial sarcoma, particularly when material and/or resources are unavailable for PCR or FISH-based testing. However, as variable weak staining for SYT may be encountered in a small percentage of non-synovial sarcoma sarcomas, a positive interpretation should be made only when the staining is strong, nuclear and present in the majority of cells.

  12. Heidelberg Ion Therapy Center (HIT): Initial clinical experience in the first 80 patients

    Energy Technology Data Exchange (ETDEWEB)

    Combs, Stephanie E. (Univ. Hospital of Heidelberg, Dept. of Radiation Oncology, Heidelberg (Germany)), E-mail: Stephanie.Combs@med.uni-heidelberg.de; Ellerbrock, Malte; Haberer, Thomas (Heidelberger Ionenstrahl Therapiezentrum (HIT), Im Neuenheimer Feld 450, 69120 Heidelberg (Germany)) (and others)

    2010-10-15

    The Heidelberg Ion Therapy Center (HIT) started clinical operation in November 2009. In this report we present the first 80 patients treated with proton and carbon ion radiotherapy and describe patient selection, treatment planning and daily treatment for different indications. Patients and methods. Between November 15, 2009 and April 15, 2010, 80 patients were treated at the Heidelberg Ion Therapy Center (HIT) with carbon ion and proton radiotherapy. Main treated indications consisted of skull base chordoma (n = 9) and chondrosarcoma (n = 18), malignant salivary gland tumors (n=29), chordomas of the sacrum (n = 5), low grade glioma (n=3), primary and recurrent malignant astrocytoma and glioblastoma (n=7) and well as osteosarcoma (n = 3). Of these patients, four pediatric patients aged under 18 years were treated. Results. All patients were treated using the intensity-modulated rasterscanning technique. Seventy-six patients were treated with carbon ions (95%), and four patients were treated with protons. In all patients x-ray imaging was performed prior to each fraction. Treatment concepts were based on the initial experiences with carbon ion therapy at the Gesellschaft fuer Schwerionenforschung (GSI) including carbon-only treatments and carbon-boost treatments with photon-IMRT. The average time per fraction in the treatment room per patient was 29 minutes; for irradiation only, the mean time including all patients was 16 minutes. Position verification was performed prior to every treatment fraction with orthogonal x-ray imaging. Conclusion. Particle therapy could be included successfully into the clinical routine at the Dept. of Radiation Oncology in Heidelberg. Numerous clinical trials will subsequently be initiated to precisely define the role of proton and carbon ion radiotherapy in radiation oncology.

  13. One-step reconstruction with a 3D-printed, biomechanically evaluated custom implant after complex pelvic tumor resection.

    Science.gov (United States)

    Wong, K C; Kumta, S M; Geel, N V; Demol, J

    2015-01-01

    Resection of a pelvic tumor is challenging because of its complex three-dimensional (3D) anatomy and deep-seated location with nearby vital structures. The resection is technically demanding if a custom implant is used for reconstruction of the bone defect as the surgeon needs to ensure the resection margin is sufficiently wide and the orientation of intended resection planes must match that of the custom implant. We describe a novel workflow of performing a partial acetabular resection in a patient with pelvic chondrosarcoma and reconstruction with a custom pelvic implant in a one-step operation. A multi-planar bone resection was virtually planned. A computer-aided design implant that both matched the bone defect and biomechanically evaluated was prefabricated with 3D printing technology. The 3D-printed patient-specific instruments (PSIs) were used to reproduce the same planned resection. The histology of the tumor specimen showed a clear resection margin. The errors of the achieved resection and implant position were deviating (1-4 mm) from the planned. The patient could walk unaided with a good hip function. No tumor recurrence and implant loosening were noted at 11 months after surgery. The use of this novel CT-based method for surgical planning, the engineering software for implant design and validation, together with 3D printing technology for implant and PSI fabrication makes it possible to generate a personalized, biomechanically evaluated implant for accurate reconstruction after a pelvic tumor resection in a one-step operation. Further study in a larger population is needed to assess the clinical efficacy of the workflow in complex bone tumor surgery.

  14. Typical tumors of the petrous bone; Typische Tumoren des Felsenbeins

    Energy Technology Data Exchange (ETDEWEB)

    Ahlhelm, F.; Mueller, U. [Kantonsspital Baden AG, Abteilung fuer Neuroradiologie, Institut fuer Radiologie, Baden (Switzerland); Ulmer, S. [Medizinisch-Radiologisches Institut, Zuerich (Switzerland)

    2014-04-15

    In the region of the petrous bone, inner acoustic canal and cerebellopontine angle, a variety of different tissues can be found, such as bony, epithelial, neural and vascular structures. Tumorous or tumor-like lesions, vascular or bony malformations or other pathologies can therefore be found in all of these areas. We discuss various frequently occurring tumorous or tumor-like pathologies including congential lesions, such as mucoceles, inflammatory disorders including osteomyelitis, pseudotumors and Wegener's granulomatosis. Benign non-neoplastic lesions, such as cholesteatoma, cholesterol granuloma, epidermoid and benign neoplastic tumors, such as the most commonly found vestibular schwannoma, meningeoma, paraganglioma, vascular pathologies and finally malignant lesions, such as metastasis, chordoma or chondrosarcoma and endolymphatic sac tumor (ELST) are also discussed. The emphasis of this article is on the appearance of these entities in computed tomography (CT) and more so magnetic resonance imaging (MRI), it provides key facts and typical images and discusses possibilities how to distinguish these pathologies. (orig.) [German] In der Region des Felsenbein, inneren Gehoerkanals und Kleinhirnbrueckenwinkels findet sich eine Vielzahl an unterschiedlichen Gewebearten inklusive knoechernes, epitheliales, nervales und vaskulaeres Gewebe. Tumoren oder tumoraehnliche Laesionen, ossaere oder vaskulaere Pathologien koennen entsprechend dort gefunden werden. Wir diskutieren verschiedene Tumoren oder tumoraehnliche Pathologien inklusive angeborene Laesionen wie Muko- und Meningozelen, entzuendliche Veraenderungen wie die Osteomyelitis, Pseudotumoren, die Wegener-Granulomatose, nichtneoplastische Tumoren wie das Epidermoid, Cholesteatom oder Cholesterolgranulom und gutartige neoplastische Tumoren wie das am haeufigsten zu findende Vestibularisschwannom, das Paragangliom und das Meningeom, Gefaessprozesse/-pathologien und schliesslich maligne Laesionen wie Metastasen

  15. Limb salvage with microvascular free fibula following primary bone sarcoma resection

    Directory of Open Access Journals (Sweden)

    Sahasrabudhe Parag

    2016-01-01

    Full Text Available Background: Extremity sarcomas are challenging to manage. Total eradication of tumour has to be balanced with restoration of limb function to prevent mortality and morbidity. Disease-free survival with maximum limb function is the ultimate goal in these patients. Materials and Methods: We present a series of ten cases of extremity malignancies, where limb salvage was attempted with microvascular free fibula for limb reconstruction from the period of 2008 to 2015. Results: Of the ten cases in the study, there were two females and eight males. There were nine patients with lower limb malignancies and one patient with upper limb malignancy. There were four patients with Ewing's sarcoma of femur, five patients with osteosarcoma of femur and one patient with chondrosarcoma of the humerus. The follow-up period ranged from 1.2 to 6.2 years with mean follow-up of 3.1 years. There were two deaths during follow-up, both were due to distant metastasis. The assessment of the function was done on the basis of Musculoskeletal Tumour Society functional score. Maximum score was 30 and minimum score was 24, the average score being 26. Of the eight surviving patients, three patients had full weightbearing, four patients had partial weightbearing at end of 2 years and one patient of upper limb reconstruction had complete upper limb function. None of the patients had to undergo limb amputation. Conclusion: Limb salvage with vascularised fibula graft offers good functional outcome along with good disease-free survival rates.

  16. Carnosol and Related Substances Modulate Chemokine and Cytokine Production in Macrophages and Chondrocytes

    Directory of Open Access Journals (Sweden)

    Joseph Schwager

    2016-04-01

    Full Text Available Phenolic diterpenes present in Rosmarinus officinalis and Salvia officinalis have anti-inflammatory and chemoprotective effects. We investigated the in vitro effects of carnosol (CL, carnosic acid (CA, carnosic acid-12-methylether (CAME, 20-deoxocarnosol and abieta-8,11,13-triene-11,12,20-triol (ABTT in murine macrophages (RAW264.7 cells and human chondrocytes. The substances concentration-dependently reduced nitric oxide (NO and prostaglandin E2 (PGE2 production in LPS-stimulated macrophages (i.e., acute inflammation. They significantly blunted gene expression levels of iNOS, cytokines/interleukins (IL-1α, IL-6 and chemokines including CCL5/RANTES, CXCL10/IP-10. The substances modulated the expression of catabolic and anabolic genes in chondrosarcoma cell line SW1353 and in primary human chondrocytes that were stimulated by IL-1β (i.e., chronic inflammation In SW1353, catabolic genes like MMP-13 and ADAMTS-4 that contribute to cartilage erosion were down-regulated, while expression of anabolic genes including Col2A1 and aggrecan were shifted towards pre-pathophysiological homeostasis. CL had the strongest overall effect on inflammatory mediators, as well as on macrophage and chondrocyte gene expression. Conversely, CAME mainly affected catabolic gene expression, whereas ABTT had a more selectively altered interleukin and chemokine gene exprssion. CL inhibited the IL-1β induced nuclear translocation of NF-κBp65, suggesting that it primarily regulated via the NF-κB signalling pathway. Collectively, CL had the strongest effects on inflammatory mediators and chondrocyte gene expression. The data show that the phenolic diterpenes altered activity pattern of genes that regulate acute and chronic inflammatory processes. Since the substances affected catabolic and anabolic gene expression in cartilage cells in vitro, they may beneficially act on the aetiology of osteoarthritis.

  17. Is there a role for diffusion-weighted MRI (DWI) in the diagnosis of central cartilage tumors?

    Energy Technology Data Exchange (ETDEWEB)

    Douis, H. [Royal Orthopaedic Hospital, Department of Radiology, Birmingham (United Kingdom); University Hospital Birmingham, Department of Radiology, Birmingham (United Kingdom); Jeys, L.; Grimer, R. [Royal Orthopaedic Hospital, Department of Orthopaedic Oncology, Birmingham (United Kingdom); Vaiyapuri, S. [Royal Orthopaedic Hospital, Department of Musculoskeletal Pathology, Birmingham (United Kingdom); Davies, A.M. [Royal Orthopaedic Hospital, Department of Radiology, Birmingham (United Kingdom)

    2015-03-07

    To assess if diffusion-weighted MRI (DWI) can differentiate between central enchondromas and chondrosarcomas (CS) and if DWI can distinguish low-grade chondroid lesions from high-grade CS. Fifty-two patients with central cartilage tumors were included. Patients underwent conventional MRI and DWI with ADC mapping. The slice on MRI with the most aggressive imaging feature was identified. The corresponding mean and minimum ADC maps of the tumor at this position were measured. There were 24 enchondromas, five atypical cartilaginous lesions, 15 grade 1, three grade 2, two grade 3, and three dedifferentiated CS. Mean ADC values (x 10{sup -6} mm{sup 2}/s) for enchondromas, atypical cartilaginous tumors, grade 1 CS, grade 2, CS, grade 3 CS and dedifferentiated CS were 1,896, 2,048, 2,152, 2,170, 2,076, and 1,261, respectively. Minimum ADC values (x 10{sup -6} mm{sup 2}/s) for enchondromas, atypical cartilaginous tumors, grade 1 CS, grade 2, CS, grade 3 CS and dedifferentiated CS were 1,820, 1,752, 2,010, 1,829, 1,752, and 767, respectively. ANOVA test demonstrated a statistically significant difference in mean and minimum ADC values in all groups. Post hoc analysis revealed this was due to difference in mean and minimum ADC values in dedifferentiated CS. The mean ADC value in low-grade chondroid lesions was 2,001, while the ADC value for high-grade CS were 2,132. The minimum ADC value for low-grade chondroid lesions was 1,896, while the minimum ADC for high-grade CS was 1,837. The difference in minimum and mean ADC values was not statistically significant. DWI cannot differentiate between enchondromas and CS and DWI does not aid in the distinction of low-grade chondroid tumors from high-grade CS. (orig.)

  18. MR Imaging in symptomatic osteochondromas

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    Kim, Soo Young; Kim, Jee Young; Kim, Sang Heum; Chun, Kyung Ah; Park, Young Ha [Catholic University of Korea, Seoul (Korea, Republic of)

    1998-06-01

    The purpose of this study is to assess the MR findings of symptomatic osteochondromas. We evaluated 31 patients who between July, 1994 and May, 1997 underwent MR imaging for symptomatic osteochondroma. Fourteen were males and 17 were females, and their ages ranged from 8 to 49 (mean, 23) years. Using T1WI, T2WI and gadolinium-DTPA-enhanced T1WI, images were analysed according to signal intensity in the osseous component of the osteochondroma, thickness of the cartilage cap, and associated change in surrounding soft tissue. Clinical manifestation included a palpable mass or tendency to grow (n=22) and pain on movement (n=9). Complications were of three types : that which followed change in the osseous component of the tumor, associated change in surrounding soft tissue, and malignant transformation. In the osseous component, bone marrow edema or contusion was seen in 21 cases (67.7%), and in two (65%), fracture was observed. In surrounding soft tissue, muscle impingement was seen in 21 cases (67.7%), bursitis was in 7 cases (22.6 %), tenosynovitis in seven (22.6 %), and vascular compression in five (16.1 %). In three cases (9.7%), transformation to chondrosarcoma had occurred; two of these were derived from osteochondromatosis and one from a single osteochondroma. The thickness of the cartilage cap was as follow : < 5 mm (n=16), 5-10 mm (n=12), and > 10 mm (n=3). In patients with symptomatic osteochondroma, MR imaging is useful for detecting both complications and malignant transformation. (author). 21 refs., 1 tab., 6 figs.

  19. The Use of Iliac Stem Prosthesis for Acetabular Defects following Resections for Periacetabular Tumors

    Directory of Open Access Journals (Sweden)

    Massimiliano De Paolis

    2013-01-01

    Full Text Available Introduction. The management of pelvic tumors is a challenge for orthopaedic oncologists due to the complex anatomy of the pelvis and the need to have extensive exposure. Various reconstructive techniques have been proposed with poor functional results and a high percentage of complications. Our purpose is to determine the functional results and the rate of complications of iliac stem prosthesis for acetabular defects following resections for periacetabular tumors. Materials and Methods. Between 1999 and 2012, 45 patients underwent pelvic resections for periacetabular bone tumors followed by reconstruction with stem cup prosthesis. The most common diagnosis was CS (chondrosarcoma, 29 cases, followed by OS (osteosarcoma, 9 cases and metastasis (3 cases. In 33 cases, this implant was associated with massive bone allografts. Minimum follow-up required to evaluate functional outcome was 2 years. We classified pelvic resections according to Enneking and Dunham’s classification and we used MSTS (musculoskeletal tumor system score to evaluate functional outcomes. Results and Discussion. Sixteen patients died of their disease, three were lost to follow-up, four are alive with disease, and twenty-two are alive with no evidence of disease. Fifteen patients had local recurrence. Sixteen patients had bone or lung metastasis. We have had 6 infections, 2 aseptic loosening, and 2 cases of hip dislocation. Iliac sovracetabular osteotomy was fused in all cases at 10 months from surgery. Functional results were good or excellent in 25 of 31 patients with long-term follow-up (77%, with a percentage similar to that reported in the literature. Conclusion. The use of iliac stem prosthesis is a simple reconstructive technique that reduces operative times and risk of infection. It allows having good results and low rate of complications, but it should be performed in selected cases and centres of reference.

  20. Reconstructive options in pelvic tumours

    Directory of Open Access Journals (Sweden)

    Mayilvahanan N

    2005-01-01

    Full Text Available Background: Pelvic tumours present a complex problem. It is difficult to choose between limb salvage and hemipelvectomy. Method: Forty three patients of tumours of pelvis underwent limb salvage resection with reconstruction in 32 patients. The majority were chondrosarcomas (20 cases followed by Ewing sarcoma. Stage II B was the most common stage in malignant lesions and all the seven benign lesions were aggressive (B3. Surgical margins achieved were wide in 31 and marginal in 12 cases. Ilium was involved in 51% of cases and periacetabular involvement was seen in 12 patients. The resections done were mostly of types I &II of Enneking′s classification of pelvic resection. Arthrodesis was attempted in 24 patients. Customized Saddle prosthesis was used in seven patients and no reconstruction in 12 patients. Adjuvant chemotherapy was given to all high-grade malignant tumours, combined with radiotherapy in 7 patients. Results: With a mean follow up of 48.5 months and one patient lost to follow up, the recurrence rate among the evaluated cases was 16.6%. Oncologically, 30 patients were continuously disease free with 7 local recurrences and 4 deaths due to disseminated disease and 2 patients died of other causes. During the initial years, satisfactory functional results were achieved with prosthetic replacement. Long-term functional result of 36 patients who were alive at the time of latest follow up was satisfactory in 75% who underwent arthrodesis and in those where no reconstruction was used. We also describe a method of new classification of pelvic resections that clarifies certain shortcomings of the previous systems of classification. Conclusion: Selection of a procedure depends largely on the patient factors, the tumour grade, the resultant defect and the tissue factors. Resection with proper margins gives better functional and oncological results

  1. Indications of Carbon Ion Therapy at CNAO

    Science.gov (United States)

    Orecchia, Roberto; Rossi, Sandro; Fossati, Piero

    2009-03-01

    CNAO will be a dual center capable of providing therapeutic beams of protons and carbon ions with maximum energy of 400 MeV/u. At the beginning, it will be equipped with three treatment rooms with fixed horizontal and vertical beam lines. In a subsequent phase, two more rooms with a rotating gantry are foreseen. An active spot scanning dose delivery system will be employed. Initially, 80% of the treatments will be carried out with carbon ions. All patients will be treated within clinical trials to assess carbon ion indications with an evidence-based methodology. Seven disease-specific working groups have been developed: lung tumors, liver tumors, sarcomas, head and neck tumors, central nervous system lesions, eye tumors and pediatric tumors. The last two groups will be treated mainly with protons. In the first phase, CNAO will focus on head and neck cancers, treating inoperable, residual or recurrent malignant salivary gland tumors, mucosal melanoma, adenocarcinoma and unfavorably located SCC (nasal and paranasal sinuses). Carbon ions will be employed as a boost in the treatment of locally advanced, poor prognosis, SCC of the hypopharynx and tongue base. Bone and soft tissue sarcomas of the extremity will be treated with a limb-sparing approach, and trunk sarcomas will be treated with exclusive or post-operative irradiation. Skull base tumors (chordoma and chondrosarcoma), recurrent or malignant meningioma and glial tumors will be treated with carbon ions. After sufficient expertise has been gained in coping with organ motion, CNAO will start treating thoracic and abdominal targets. HCC will be treated in inoperable patients with one or more lesions that can be included in a single CTV. Early stage NSCLC will be treated. In the second phase, two more groups on gynecological malignancies and digestive tumors (esophageal cancer, rectal cancer, pancreatic cancer) will be created.

  2. MR-guided laser-induced thermotherapy in recurrent extrahepatic abdominal tumors

    Energy Technology Data Exchange (ETDEWEB)

    Mack, M.G.; Straub, R.; Eichler, K.; Boettger, M.; Woitaschek, D.; Vogl, T.J. [Dept. of Diagnostic and Interventional Radiology, University of Frankfurt (Germany); Roggan, A. [LMTB GmbH, Berlin (Germany)

    2001-10-01

    The aim of this study was to evaluate the feasibility of MR-guided laser-induced thermotherapy (LITT) for treatment of recurrent extrahepatic abdominal tumors. In 11 patients (6 women and 5 men; mean age 53 years, age range 29-67 years) with 14 lesions the following tumors were treated in this study: paravertebral recurrence of hypernephroma (n=1); recurrence of uterus carcinoma (n=1); recurrence of chondrosarcoma of the pubic bone (n=1); presacral recurrence of rectal carcinoma (n=1); recurrent anal cancer (n=1); metastases in the abdominal wall (n=1); and lymph node metastases from colorectal cancer (n=8). A total of 27 laser applications were performed. A fast low-angle shot 2D sequence (TR/TE/flip angle=102 ms/8 ms/70 ) was used for nearly real-time monitoring during treatment. All patients had no other treatment option. Seventeen LITT sessions were performed using a conventional laser system with a mean laser power of 5.2 W (range 4.5-5.7 W), and 10 LITT session were performed using a power laser system with a mean laser power of 28.0 W. In 10 lesions total destruction could be achieved. In the remaining recurrent tumors, significant reduction of tumor volume by 60-80% was obtained. All patients tolerated the procedure well under local anesthesia. No complications occurred during treatment. Laser-induced thermotherapy is a practicable, minimally invasive, well-tolerated technique that can produce large areas of necrosis within recurrent tumors, substantially reducing active tumor volume if not resulting in outright destruction of tumor. (orig.)

  3. Single-stage en bloc resection using a posterior approach for sacral tumors

    Institute of Scientific and Technical Information of China (English)

    Zhong Fang; Wei Wu; Wei Xiong; Guanghui Li; Hui Liao; Jun Xiao; Fengjing Guo; Anmin Chen; Feng Li 

    2016-01-01

    Objective This study aims to investigate the surgical results of single-stage en bloc resections using a posterior approach for sacral tumors and evaluate its benefit for these tumors. Methods A total of 32 cases involving patients with sacral tumors who were treated with single-stage en bloc resection by posterior approach from April 2004 to January 2010 were reviewed. The patient cohort included 20 males and 12 females. The average patient age was 49.1 years old (range, 18 to 75 years old). Twenty-seven patients suf ered from primary sacral tumors, including 17 patients with chordomas, 5 patients with giant cel tumors of the bone, 3 patients with chondrosarcoma, 1 patient with sarcoma of the nerve fibers, and 1 patient with neurofibromatosis. Five patients were diagnosed with sacral metastatic tumors, including 2 cases of breast cancer, 1 case of renal carcinoma, 1 case of thyroid cancer, and 1 case of lung cancer. According to the Frankel grading system, there were 3 Grade B tumors, 4 Grade C tumors, 10 Grade D tumors, and 15 Grade E tumors. Results The operation took 265 min on average (range, 130–360 min), and blood loss was 1676 mL on average (range, 800–1800 mL) during the operation. The fol ow-up period ranged from 6 months to 6.2 years. Al patients had pain prior to operation. Twenty-eight patients experienced complete pain-relief, and 4 patients experienced partial pain-relief after their operations. In al patients, neurological function was improved more than one grade using the Frankel grading system. Up to now, 5 patients experienced local recurrence after operation, and 2 patients were deceased. The remaining patients are stil alive without recurrence. Conclusion Single-stage en bloc resection through a posterior approach for sacral tumors is feasible, safe, and ef ective. It has many advantages, such as control ing local recurrence, thorough decompression of the spinal cord, relieving pain, improving quality of life, and prolonging survival.

  4. In vitro chondrocyte toxicity following long-term, high-dose exposure to Gd-DTPA and a novel cartilage-targeted MR contrast agent

    Energy Technology Data Exchange (ETDEWEB)

    Midura, Sharon; Midura, Ronald J. [Cleveland Clinic, Biomedical Engineering, Lerner Research Institute, Cleveland, OH (United States); Schneider, Erika [Cleveland Clinic, Imaging Institute, A21, Cleveland, OH (United States); NitroSci Pharmaceuticals, New Berlin, WI (United States); Rosen, Gerald M. [University of Maryland School of Pharmacy, Department of Pharmaceutical Sciences, Baltimore, MD (United States); NitroSci Pharmaceuticals, New Berlin, WI (United States); Winalski, Carl S. [Cleveland Clinic, Biomedical Engineering, Lerner Research Institute, Cleveland, OH (United States); Cleveland Clinic, Imaging Institute, A21, Cleveland, OH (United States)

    2017-01-15

    To determine the concentrations exhibiting toxicity of a cartilage-targeted magnetic resonance imaging contrast agent compared with gadopentetate dimeglumine (Gd-DT-PA) in chondrocyte cultures. A long-term Swarm rat chondrosarcoma chondrocyte-like cell line was exposed for 48 h to 1.0-20 mM concentrations of diaminobutyl-linked nitroxide (DAB4-DLN) citrate, 1.0-20 mM Gd-DTPA, 1.0 μM staurosporine (positive control), or left untreated. Cell appearance, 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assays of metabolic activity, quantitative PicoGreen assays of DNA content, and calcein-AM viability assays were compared. At 1.0-7.5 mM, minimal decrease in cell proliferation was found for both agents. At all doses of both agents, cell culture appearances were similar after 24 h of treatment. At the higher doses, differences in cell culture appearance were found after 48 h of treatment, with dose-dependent declines in chondrocyte populations for both agents. Concentration-dependent declines in DNA content and calcein fluorescence were found after 48 h of treatment, but beginning at a lower dose of DAB4-DLN citrate than Gd-DTPA. Dose-dependent decreases in MTT staining (cell metabolism) were apparent for both agents, but larger effects were evident at a lower dose for DAB-DLN citrate. Poor MTT staining of cells exposed for 48 h to 20 mM DAB4-DLN citrate probably indicates dead or dying cells. The minimal effect of the long-term exposure of model chondrocyte cell cultures to DAB4-DLN citrate and Gd-DTPA concentrations up to 7.5 mM (3x typical arthrographic administration) is supporting evidence that these doses are acceptable for MR arthrography. The findings are reassuring given that the experimental exposure to the contrast agents at sustained concentrations was much longer than when used clinically. (orig.)

  5. Dose–Volume Relationships Associated With Temporal Lobe Radiation Necrosis After Skull Base Proton Beam Therapy

    Energy Technology Data Exchange (ETDEWEB)

    McDonald, Mark W., E-mail: markmcdonaldmd@gmail.com [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Indiana University Health Proton Therapy Center, Bloomington, Indiana (United States); Linton, Okechukwu R. [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Calley, Cynthia S.J. [Department of Biostatistics, Indiana University School of Medicine, Indianapolis, Indiana (United States)

    2015-02-01

    Purpose: We evaluated patient and treatment parameters correlated with development of temporal lobe radiation necrosis. Methods and Materials: This was a retrospective analysis of a cohort of 66 patients treated for skull base chordoma, chondrosarcoma, adenoid cystic carcinoma, or sinonasal malignancies between 2005 and 2012, who had at least 6 months of clinical and radiographic follow-up. The median radiation dose was 75.6 Gy (relative biological effectiveness [RBE]). Analyzed factors included gender, age, hypertension, diabetes, smoking status, use of chemotherapy, and the absolute dose:volume data for both the right and left temporal lobes, considered separately. A generalized estimating equation (GEE) regression analysis evaluated potential predictors of radiation necrosis, and the median effective concentration (EC50) model estimated dose–volume parameters associated with radiation necrosis. Results: Median follow-up time was 31 months (range 6-96 months) and was 34 months in patients who were alive. The Kaplan-Meier estimate of overall survival at 3 years was 84.9%. The 3-year estimate of any grade temporal lobe radiation necrosis was 12.4%, and for grade 2 or higher radiation necrosis was 5.7%. On multivariate GEE, only dose–volume relationships were associated with the risk of radiation necrosis. In the EC50 model, all dose levels from 10 to 70 Gy (RBE) were highly correlated with radiation necrosis, with a 15% 3-year risk of any-grade temporal lobe radiation necrosis when the absolute volume of a temporal lobe receiving 60 Gy (RBE) (aV60) exceeded 5.5 cm{sup 3}, or aV70 > 1.7 cm{sup 3}. Conclusions: Dose–volume parameters are highly correlated with the risk of developing temporal lobe radiation necrosis. In this study the risk of radiation necrosis increased sharply when the temporal lobe aV60 exceeded 5.5 cm{sup 3} or aV70 > 1.7 cm{sup 3}. Treatment planning goals should include constraints on the volume of temporal lobes receiving

  6. NY-ESO-1 is a sensitive and specific immunohistochemical marker for myxoid and round cell liposarcomas among related mesenchymal myxoid neoplasms.

    Science.gov (United States)

    Hemminger, Jessica A; Iwenofu, O Hans

    2013-09-01

    Myxoid and round cell liposarcomas constitute approximately one-third of all liposarcomas, a relatively common group of fat-derived soft tissue sarcomas. The histomorphology is a continuum between highly differentiated myxoid and poorly differentiated round cell components. The gold standard of diagnosis is dependent on histomorphology and/or identification of t(12;16)(q13;p11) translocation by cytogenetics or demonstration of DDIT3 rearrangements by fluorescence in situ hybridization. There are currently no diagnostic immunohistochemical stains available. The broad range of myxoid neoplasms in the differential diagnosis includes a variety of sarcomas. Given the notable differences in disease biology among myxoid neoplasms, which range from benign to aggressive, an accurate diagnosis is imperative for proper treatment and prognostication. Prompted by our recent study showing frequent expression of the cancer testis antigen NY-ESO-1 in myxoid and round cell liposarcomas, we sought to evaluate the utility of NY-ESO-1 as an immunohistochemical marker for myxoid and round cell liposarcoma among mesenchymal myxoid neoplasms within the differential diagnosis. Formalin-fixed, paraffin-embedded blocks were obtained for the following mesenchymal myxoid neoplasms (n=138): myxoid and round cell liposarcoma (n=38); well-differentiated liposarcoma (n=12); lipoma (n=20; 4 with myxoid change); extra-cardiac soft tissue myxoma (n=39); extraskeletal myxoid chondrosarcoma (n=12); myxofibrosarcoma (n=10: 5 low grade, 2 intermediate grade, 3 high grade); and low-grade fibromyxoid sarcoma (n=7). Utilizing standard immunohistochemistry protocols, full sections were stained with NY-ESO-1 (clone E978), and staining was assessed for intensity (1-2+), percentage of tumor positivity, and location. In all, 36/38 (95%) of the myxoid and round cell liposarcomas demonstrated NY-ESO-1 immunoreactivity. The majority of the positive cases (34/36; 94%) showed strong, homogenous staining (>50% tumor

  7. Clinicopathological features of five unusual cases of intraosseous myoepithelial carcinomas, mimicking conventional primary bone tumours, including EWSR1 rearrangement in one case.

    Science.gov (United States)

    Rekhi, Bharat; Joshi, Sujit; Panchwagh, Yogesh; Gulia, Ashish; Borges, Anita; Bajpai, Jyoti; Jambehekar, Nirmala A; Pant, Vinita; Mandholkar, Mahesh; Byregowda, Suman; Puri, Ajay

    2016-04-01

    Primary intraosseous myoepithelial tumours, including carcinomas are rare tumours. The concept of histopathological spectrum of these tumours is evolving. We describe clinicopathological and immunohistochemical features of five myoepithelial carcinomas, including molecular cytogenetic results in one case. There were five male patients within age-range of 8-40 years (median = 26). Four tumours occurred in the long bones, including two tumours, each, in the femur and fibula, respectively, while a single tumour occurred in the proximal phalanges. Tumour size (n = 3 cases) varied from 5.6 to 8.6 cm. On radiological imaging, most tumours appeared as expansile, lytic and destructive lesions. Two tumours appeared as sclerotic lesions. Two cases were referred with diagnoses of chondrosarcomas and a single case was referred with two different diagnoses, including an adamantinoma and an osteosarcoma. Histopathological examination in all these cases showed multinodular tumours comprising mostly polygonal cells, exhibiting moderate nuclear atypia and interspersed mitotic figures within a stroma containing variable amount of myxoid, chondroid, hyalinised and osteoid-like material. Three tumours revealed prominent squamous differentiation. By immunohistochemistry, tumour cells were positive for EMA (5/5), pan CK (AE1/AE3) (3/3), CK5/6 (4/4), CK MNF116 (1/1), S100 protein (5/5) and GFAP (3/5). The first tumour revealed EWSR1 rearrangement. The first patient, 10 months after tumour resection and a simultaneous lung metastatectomy, is free-of-disease (FOD). The second patient, 11 months after tumour resection is FOD. The third and fourth patients underwent wide resections and are on follow-up. The fifth patient underwent resections, including a lung metastatectomy. Primary intraosseous myoepithelial carcinomas are rare and mimic conventional primary bone tumours. Some primary intraosseous myoepithelial carcinomas display EWSR1 rearrangement. Squamous differentiation may be

  8. SU-E-T-565: RAdiation Resistance of Cancer CElls Using GEANT4 DNA: RACE

    Energy Technology Data Exchange (ETDEWEB)

    Perrot, Y; Payno, H; Delage, E; Maigne, L [Clermont Universite, CNRS/IN2P3, Laboratoire de Physique Corpusculaire de Clermont-Ferrand, Aubiere (France); Incerti, S [Universite Bordeaux 1, CNRS/IN2P3, Centres d' Etudes Nucleaires de Bordeaux-Gradignan, Gradignan (France); Debiton, E; Peyrode, C; Chezal, J; Miot-Noirault, E; Degoul, F [Clermont Universite, Universite d' Auvergne, Imagerie Moleculaire et Therapie Vectorisee, INSERM U990, Centre Jean Perrin, Clermont-Ferrand (France)

    2014-06-01

    Purpose: The objective of the RACE project is to develop a comparison between Monte Carlo simulation using the Geant4-DNA toolkit and measurements of radiation damage on 3D melanoma and chondrosarcoma culture cells coupled with gadolinium nanoparticles. We currently expose the status of the developments regarding simulations. Methods: Monte Carlo studies are driven using the Geant4 toolkit and the Geant4-DNA extension. In order to model the geometry of a cell population, the opensource CPOP++ program is being developed for the geometrical representation of 3D cell populations including a specific cell mesh coupled with a multi-agent system. Each cell includes cytoplasm and nucleus. The correct modeling of the cell population has been validated with confocal microscopy images of spheroids. The Geant4 Livermore physics models are used to simulate the interactions of a 250 keV X-ray beam and the production of secondaries from gadolinium nanoparticles supposed to be fixed on the cell membranes. Geant4-DNA processes are used to simulate the interactions of charged particles with the cells. An atomistic description of the DNA molecule, from PDB (Protein Data Bank) files, is provided by the so-called PDB4DNA Geant4 user application we developed to score energy depositions in DNA base pairs and sugar-phosphate groups. Results: At the microscopic level, our simulations enable assessing microscopic energy distribution in each cell compartment of a realistic 3D cell population. Dose enhancement factors due to the presence of gadolinium nanoparticles can be estimated. At the nanometer scale, direct damages on nuclear DNA are also estimated. Conclusion: We successfully simulated the impact of direct radiations on a realistic 3D cell population model compatible with microdosimetry calculations using the Geant4-DNA toolkit. Upcoming validation and the future integration of the radiochemistry module of Geant4-DNA will propose to correlate clusters of ionizations with in vitro

  9. Temporal Lobe Reactions After Carbon Ion Radiation Therapy: Comparison of Relative Biological Effectiveness–Weighted Tolerance Doses Predicted by Local Effect Models I and IV

    Energy Technology Data Exchange (ETDEWEB)

    Gillmann, Clarissa, E-mail: clarissa.gillmann@med.uni-heidelberg.de [Department of Radiation Oncology and Radiation Therapy, Heidelberg University Hospital, Heidelberg (Germany); Jäkel, Oliver [Department of Radiation Oncology and Radiation Therapy, Heidelberg University Hospital, Heidelberg (Germany); Heidelberg Ion Beam Therapy Center (HIT), Heidelberg (Germany); Department of Medical Physics in Radiation Oncology, German Cancer Research Center (DKFZ), Heidelberg (Germany); Schlampp, Ingmar [Department of Radiation Oncology and Radiation Therapy, Heidelberg University Hospital, Heidelberg (Germany); Karger, Christian P. [Department of Medical Physics in Radiation Oncology, German Cancer Research Center (DKFZ), Heidelberg (Germany)

    2014-04-01

    Purpose: To compare the relative biological effectiveness (RBE)–weighted tolerance doses for temporal lobe reactions after carbon ion radiation therapy using 2 different versions of the local effect model (LEM I vs LEM IV) for the same patient collective under identical conditions. Methods and Materials: In a previous study, 59 patients were investigated, of whom 10 experienced temporal lobe reactions (TLR) after carbon ion radiation therapy for low-grade skull-base chordoma and chondrosarcoma at Helmholtzzentrum für Schwerionenforschung (GSI) in Darmstadt, Germany in 2002 and 2003. TLR were detected as visible contrast enhancements on T1-weighted MRI images within a median follow-up time of 2.5 years. Although the derived RBE-weighted temporal lobe doses were based on the clinically applied LEM I, we have now recalculated the RBE-weighted dose distributions using LEM IV and derived dose-response curves with Dmax,V-1 cm³ (the RBE-weighted maximum dose in the remaining temporal lobe volume, excluding the volume of 1 cm³ with the highest dose) as an independent dosimetric variable. The resulting RBE-weighted tolerance doses were compared with those of the previous study to assess the clinical impact of LEM IV relative to LEM I. Results: The dose-response curve of LEM IV is shifted toward higher values compared to that of LEM I. The RBE-weighted tolerance dose for a 5% complication probability (TD{sub 5}) increases from 68.8 ± 3.3 to 78.3 ± 4.3 Gy (RBE) for LEM IV as compared to LEM I. Conclusions: LEM IV predicts a clinically significant increase of the RBE-weighted tolerance doses for the temporal lobe as compared to the currently applied LEM I. The limited available photon data do not allow a final conclusion as to whether RBE predictions of LEM I or LEM IV better fit better clinical experience in photon therapy. The decision about a future clinical application of LEM IV therefore requires additional analysis of temporal lobe reactions in a

  10. 骨膜骨肉瘤的临床病理分析%A clinicopathological analysis of Periosteal osteosarcoma and review

    Institute of Scientific and Technical Information of China (English)

    伍锦凤; 桂照华; 吴景; 李晓洁; 何杰

    2015-01-01

    Objective The aim of this work is to discuss the clinicopathologic feature, imaging performance, diagnosis and differential diagnosis of the periosteal osteosarcoma.Method one case of periosteal osteosarcoma was analyzed by light microscopy and radio-graphy.Results This patient presented a growing painful mass.Radiographically, the case is located superficially at the cortex of the bone and the medullary cavity was not invaded by tumor.The histological appearance of the tumor was moderately differentiated chon-droblastic osteosarcoma,with small islands of anaplasic spindle cells that produced malignant osteoid.Conclusion Periosteal osteosar-coma is a rare intermediated-grade surface osteosarcoma, and secondary bone marrow involvement is unusual.Pathological Diagnosis should be combined with clinicopathologic feature and imaging performance.And diagnosis of Periosteal osteosarcoma should be differ-entiated from periosteal chondrosarcoma, conventional osteosarcoma, high grade surface osteosarcoma, parosteal osteosarcoma.%目的:分析研究骨膜骨肉瘤( periosteal osteosarcoma,POOS)的临床病理特征、影像学特点、诊断及鉴别诊断要点。方法对1例骨膜骨肉瘤( POOS)进行常规病理观察及影像学分析。结果该例POOS临床表现为局部肿块伴有疼痛;影像学显示骨皮质表面软组织阴影,不侵犯骨髓腔。镜下由中等分化的软骨母细胞构成,呈分叶状排列,小叶被有一定异型性的梭形细胞分割,细胞间可见少量肿瘤性骨样基质形成。结论骨膜骨肉瘤是发生于骨表面的罕见骨肉瘤,伴发髓腔浸润者极少见,诊断时需结合临床病理特征及影像学特点做综合判断,需要与骨膜软骨肉瘤、普通骨肉瘤、高级别骨表面骨肉瘤、骨旁骨肉瘤等进行鉴别。

  11. IDH1 and IDH2 mutations as novel therapeutic targets: current perspectives

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    Mondesir J

    2016-09-01

    Full Text Available Johanna Mondesir1,2 Christophe Willekens3–5 Mehdi Touat6,7 Stéphane de Botton3–5 1Service d’Immunopathologie Clinique, Hôpital Saint Louis, 2CNRS UMR8104, INSERM U1016, Institut Cochin, Université Paris Descartes, Paris, 3Gustave Roussy, Université Paris-Saclay, Service d’Hématologie Clinique, 4INSERM U1170, Gustave Roussy, Université Paris-Saclay, Villejuif, 5Faculté de médecine Paris-Sud, Kremlin-Bicêtre, 6AP-HP, Hôpitaux Universitaires La Pitié Salpêtrière – Charles Foix, Service de Neurologie 2-Mazarin, Paris, 7Gustave Roussy, Université Paris‑Saclay, Département d’Innovation Thérapeutique et d’Essais Précoces, Villejuif, France Abstract: Isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2 are key metabolic enzymes that convert isocitrate to α-ketoglutarate. IDH1/2 mutations define distinct subsets of cancers, including low-grade gliomas and secondary glioblastomas, chondrosarcomas, intrahepatic cholangiocarcinomas, and hematologic malignancies. Somatic point mutations in IDH1/2 confer a gain-of-function in cancer cells, resulting in the accumulation and secretion in vast excess of an oncometabolite, the D-2-hydroxyglutarate (D-2HG. Overproduction of D-2HG interferes with cellular metabolism and epigenetic regulation, contributing to oncogenesis. Indeed, high levels of D-2HG inhibit α-ketoglutarate-dependent dioxygenases, including histone and DNA demethylases, leading to histone and DNA hypermethylation and finally a block in cell differentiation. Furthermore, D-2HG is a biomarker suitable for the detection of IDH1/2 mutations at diagnosis and predictive of the clinical response. Finally, mutant-IDH1/2 enzymes inhibitors have entered clinical trials for patients with IDH1/2 mutations and represent a novel drug class for targeted therapy. Keywords: tumor metabolism, epigenetic, oncogene, IDH1, IDH2, glioma, acute myeloid leukemia, 2-HG, targeted therapies

  12. Aktueller Stand der plastisch-rekonstruktiven Chirurgie aus der Sicht der Thoraxchirurgie

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    Rupprecht, Holger

    2012-12-01

    Full Text Available Bronchoplastic techniques: Plastic-reconstructive techniques in thoracic surgery have led to significant reductions of morbidity and mortality. Particulary the so-called sleeve resections are now commonplace in non-small cell carcinoma in stage I–IIIA, with rather similar five-year survival as conventional resections. Even with infiltration of large vessels (T4-stage, this technique can be applied even in the curative approach, e.g., replacement of the pulmonary artery by a vascular prosthesis (“double sleeve resection”.Extended resection of the anterior and lateral chest wall: Malignant tumors of the chest wall are caused by infiltration of the so-called T3-carcinoma of the lung, by primary bone tumors (e.g., chondrosarcoma or by osseous metastases from extrathoracic malignancies (e.g., breast cancer. After “en bloc-resection” of the chest wall including the surrounding lung parenchyma, the large defect is covered by a muscle rotation flap (M. latissimus dorsi. Sometimes these muscle flaps can not be used, for example in cachexia. In these cases, the bony thoracic defect is reconstructed by a Goretex-patch (Goretex Dualmesh. Then the patient undergoes laparotomy or laparoscopy and dissection of the omentum of the great curvature of the stomach while preserving the right epiploic artery. Through a subcutaneous tunnel, the omentum is displaced to the thoracic wall to cover the Goretex-patch. To accelerate the wound healing, the omentum is covered with a polyurethane sponge, which is pressed with a suction of 125 mm Hg (vacuum therapy. At about 4 weeks, the healed omentum can be covered with a split skin graft.Intrathoracic infections: Severe thoracic infections (empyema, lung abscess are usually caused by a pneumonia (80% or are the consequence of operative complications (e.g., bronchial stump insufficiency.The infection is not curable with the conventional irrigation drains. Often the muscle rotation flaps fail, too. The combination of

  13. Seguimiento radiológico en paciente con fibroma condromixoide del seno frontal: Diagnóstico diferencial, alternativas terapéuticas y revisión de la literatura Radiological follow-up of a patient with chondromyxoid fibroma of the frontal sinus: Differential diagnosis, therapeutic alternatives and a revision of the literature

    Directory of Open Access Journals (Sweden)

    I. Zubillaga Rodriguez

    2006-12-01

    Full Text Available Introducción. El fibroma condromixoide es un tumor benigno de crecimiento lento y poco frecuente. Su incidencia en la región craneofacial es rara, especialmente en el seno frontal. Objetivos. Presentamos el seguimiento a largo plazo de un paciente intervenido en relación a un fibroma condromixoide del seno frontal. Se discuten los distintos diagnósticos diferenciales y las alternativas terapéuticas. Discusión. Desde el punto de vista de anatomía patológica es necesario hacer el diagnóstico diferencial con condrosarcoma, mixosarcoma, mixoma y cordoma. Desde el punto de vista radiológico, el diagnóstico de fibroma condromixoide ha de ser considerado en toda lesión ósea aislada con expansión u osteolisis, márgenes lobulados y septos intralesionales. El tratamiento del fibroma condromixoide incluye principalmente curetaje o resección en bloque, mientras que la radioterapia aislada no se considera de primera elección. Conclusiones. La naturaleza benigna del fibroma condromixoide implica la importancia de evitar tratamientos radicales. El abordaje subcraneal se presenta como una alternativa válida para el tratamiento de lesiones localizadas en el seno frontal. Es fundamental un seguimiento clínico-radiológico adecuado para identificar precozmente posibles recidivas.Introduction. Chondromyxoid fibroma is a slow growing and rare benign bone tumor. Its location in the craniofacial skeleton is not common, especially in the frontal sinus. Objectives. We present a long-term follow-up of a patient with a frontal chondromyxoid fibroma who was operated four years ago. We discuss the various differential diagnoses and possible treatments. Discussion. The differential diagnosis includes chondrosarcoma, myxosarcoma, myxoma and chordoma. From the radiological point of view, chondromyxoid fibroma must be always considered as a diagnostic possibility when a solitary bone lesion appears with expansion or cortical osteolysis, lobulated margins and

  14. Preoperative systemic etoposide/ifosfamide/doxorubicin chemotherapy combined with regional hyperthermia in high-risk sarcoma: a pilot study.

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    Issels, R D; Bosse, D; Abdel-Rahman, S; Starck, M; Panzer, M; Jauch, K W; Stiegler, H; Berger, H; Sauer, H; Peter, K

    1993-01-01

    From November 1990 to September 1991, 23 adults with high-risk, nonmetastatic sarcomas (20 soft-tissue sarcomas and 3 chondrosarcomas) were entered in a pilot protocol (RHT-91) involving regional hyperthermia combined with systemic chemotherapy followed by surgery. Of these patients, 12 had undergone previous surgery and/or radiation, 5 had received previous multidrug chemotherapy, and 6 were previously untreated. A tumor size of > 8 cm and/or an extracompartmental tumor location (11 patients) or local recurrence (12 patients) were defined as high-risk factors in addition to tumor grading (21 patients had grade 2 or 3 sarcomas). Regional hyperthermia was produced by an electromagnetic deep-regional-heating device. For systemic chemotherapy, all patients received etoposide/ifosfamide/doxorubicin (EIA) and mesna, with regional hyperthermia being given only on days 1 and 4 in repeated EIA/regional hyperthermia cycles every 3 weeks. Tumor temperatures (range, 40 degrees-44 degrees C) were measured by invasive thermometry in all patients during each regional hyperthermia treatment. A total of 181 regional hyperthermia treatments were applied within the pelvic region (11 patients) or extremities (12 patients) bearing relatively large tumors (mean volume, 848 cm3). By the cutoff date for this analysis (October 15, 1991), 13 patients had undergone surgery after receiving 2-6 (mean, 3.8) cycles of EIA chemotherapy combined with regional hyperthermia; all tumors except one were resected without disfiguration. In 22 evaluable patients (minimum, 2 EIA plus regional hyperthermia cycles), the clinical response rate was 27%, with 6 patients showing partial responses (PRs). In addition, a pathologic response to preoperative thermochemotherapy was evaluable in 13 patients, with 4 responders (31%) having > 50% histologic necrosis. In all, 3 of the responders (1 PR and 2 patients with > 50% histologic necrosis) relapsed within 3 months of surgical resection. The other 7 responding

  15. Ifosfamide plus etoposide combined with regional hyperthermia in patients with locally advanced sarcomas: a phase II study.

    Science.gov (United States)

    Issels, R D; Prenninger, S W; Nagele, A; Boehm, E; Sauer, H; Jauch, K W; Denecke, H; Berger, H; Peter, K; Wilmanns, W

    1990-11-01

    From July 1986 to July 1989, 40 patients (92% pretreated) with deep-seated, advanced soft tissue sarcomas (STS, 25 patients), Ewing's sarcomas (ES, eight patients), osteosarcomas (OS, three patients) and chondrosarcomas (ChS, four patients) were treated at the University of Munich in a protocol involving regional hyperthermia (RHT) combined with ifosfamide plus etoposide. A total of 265 RHT treatments (mean, 6.6 RHT per patient) were applied including 33 pelvic, four extremity, and three abdominal sites. The mean tumor volume was 537 cc (range, 50 to 2,980 cc). For systemic chemotherapy, all patients received ifosfamide (1.5 g/m2, days 1 to 5), etoposide (100 mg/m2, days 1, 3, and 5), and mesna (300 mg/m2 x 4, days 1 to 5) with RHT given only on days 1 and 5 in repeated cycles every 4 weeks. Acute toxicity consisted primarily of pain (57%) combined with local discomfort within the annular phased array applicator (AA) of the BSD hyperthermia system (BSD Medical Corp, Salt Lake City, UT). The average maximum systemic temperature was 37.4 +/- 0.5 degrees C, and there was no indication of enhanced bone marrow toxicity due to the addition of RHT to the systemic chemotherapy. Detailed thermal mapping by invasive thermometry was performed in all patients. In 38 assessable patients, the overall objective response rate was 37%: six complete responses (CRs), four partial responses (PRs), and four favorable histologic responses (FHRs) (95% confidence limits, 22% to 54%). Complete responders are alive and disease-free at 40, 35, 23, 19, 19, and 8 months. Of patients with PR and FHR, two died from metastatic disease after 4 and 17 months and one died from other disease after 27 months. The remaining five patients are stable at 37, 25, 21, 13, and 8 months. Eleven patients showed no change (NC), and 13 patients showed local tumor progression (PD). The mean observation time for all patients was 11.6 months. The time-averaged temperatures (Ts) of all RHT treatments calculated as

  16. The additional value of CT images interpretation in the differential diagnosis of benign vs. malignant primary bone lesions with 18F-FDG-PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Strobel, K.; Stumpe, K.D.M.; Hany, T.F.; Mende, K.; Veit-Haibach, P.; Schulthess, G.K. von [University Hospital Zurich, Division of Nuclear Medicine, Department of Medical Radiology, Zurich (Switzerland); Exner, U.E. [University Hospital Balgrist, Department of Orthopedic Surgery, Zurich (Switzerland); Bode, B. [University Hospital Zurich, Institute of Surgical Pathology, Zurich (Switzerland); Hodler, Juerg [University Hospital Balgrist, Department of Radiology, Zurich (Switzerland)

    2008-11-15

    To evaluate the value of a dedicated interpretation of the CT images in the differential diagnosis of benign vs. malignant primary bone lesions with 18fluorodeoxyglucose-positron emission tomography/computed tomography (18F-FDG-PET/CT). In 50 consecutive patients (21 women, 29 men, mean age 36.9, age range 11-72) with suspected primary bone neoplasm conventional radiographs and 18F-FDG-PET/CT were performed. Differentiation of benign and malignant lesions was separately performed on conventional radiographs, PET alone (PET), and PET/CT with specific evaluation of the CT part. Histology served as the standard of reference in 46 cases, clinical, and imaging follow-up in four cases. According to the standard of reference, conventional 17 lesions were benign and 33 malignant. Sensitivity, specificity, and accuracy in assessment of malignancy was 85%, 65% and 78% for conventional radiographs, 85%, 35% and 68% for PET alone and 91%, 77% and 86% for combined PET/CT. Median SUV{sub max} was 3.5 for benign lesions (range 1.6-8.0) and 5.7 (range 0.8-41.7) for malignant lesions. In eight patients with bone lesions with high FDG-uptake (SUV{sub max} {>=} 2.5) dedicated CT interpretation led to the correct diagnosis of a benign lesion (three fibrous dysplasias, two osteomyelitis, one aneurysmatic bone cyst, one fibrous cortical defect, 1 phosphaturic mesenchymal tumor). In four patients with lesions with low FDG-uptake (SUV{sub max} < 2.5) dedicated CT interpretation led to the correct diagnosis of a malignant lesion (three chondrosarcomas and one leiomyosarcoma). Combined PET/CT was significantly more accurate in the differentiation of benign and malignant lesions than PET alone (p =.039). There was no significant difference between PET/CT and conventional radiographs (p =.625). Dedicated interpretation of the CT part significantly improved the performance of FDG-PET/CT in differentiation of benign and malignant primary bone lesions compared to PET alone. PET/CT more commonly

  17. 人工肱骨头假体在治疗肱骨近端骨肿瘤中的应用%Application of artificial humeral head prosthesis in the treatment of proximal humerus bone tumor

    Institute of Scientific and Technical Information of China (English)

    刘军; 廖全明; 王志贵; 王克军

    2011-01-01

    Objective To evaluate the clinical effects of the artificial humeral head prosthesis in the treatment of proximal humerus bone tumors. Methods Fifteen patients with proximal humerus bone tumor had excision of bone tumor and application of artificial humeral head prosthesis were analyzed retrospectively. Among them,8 patients had osteosarcoma,4 patients had giant cell tumor of bone,2 patients had chondrosarcoma, 1 patient had fibrosarcoma. Results All cases were followed up for average (39 ± 5 ) months after the surgery.The duration time of operation were( 110 ± 10) minutes in average. The bleeding volume during operation were (600 ± 41 ) ml in average. One patient with osteosarcoma died of recurrence and metastasis 20 months after the surgery. The functions of the other 14 patients were assessed by Enneking system and the average score was 23.3 ± 3.7. The overall rate of excellent or good results was 84.6% ( 11/15 ). Conclusion Limb salvage surgery with the artificial humeral head prosthesis is an efficacious treatment for proximal humerus bone tumors,which can improve short-term clinical outcome.%目的 探讨人工肱骨头假体在肱骨近端骨肿瘤治疗中的临床应用效果.方法 回顾性分析15例行骨肿瘤切除和人工肱骨头假体置换术肱骨近端骨肿瘤患者,其中骨肉瘤8例,骨巨细胞瘤4例,软骨肉瘤2例,纤维肉瘤1例.结果 术后随访平均(39±5)个月,手术时间平均(110±10)min,术中出血平均(600±41)ml.1例骨肉瘤患者于术后20个月死于复发伴全身多处转移,余14例按Enneking评分标准,末次随访时评分(23.3±3.7)分,总体优良率为84.6%(11/15).结论 采用人工肱骨头假体能有效地治疗肱骨近端骨肿瘤,具有良好的近期临床效果.

  18. Accuracy of magnetic resonance imaging in planning the osseous resection margins of bony tumours in the proximal femur: based on coronal T1-weighted versus STIR images

    Energy Technology Data Exchange (ETDEWEB)

    Ahmad, Sarfraz; Stevenson, Jonathan; Mangham, Charles; Cribb, Gillian; Cool, Paul [Robert Jones and Agnes Hunt Orthopaedic Hospital, Department of Musculoskeletal Oncology, Oswestry, Shropshire (United Kingdom)

    2014-12-15

    Assessment of the extent of tumours using magnetic resonance imaging (MRI) is the basis for bone resection in limb-salvage surgery. We aimed to compare the accuracy of T1-weighted MRI and STIR sequences in measuring the extent of proximal femoral tumours, using the macroscopic specimens as the gold standard for comparison. We compared single coronal T1-weighted with STIR sequences in 34 proximal femoral tumours, using bivalved resected macroscopic tumours for comparison. After randomisation, four observers measured longitudinal osseous tumour extent using MRI and specimen photographs on two separate occasions, 3 weeks apart. There were 25 metastatic tumours, 8 chondrosarcomas and 1 myeloma. Eight patients presented with pathological fractures. The Pearson's correlation coefficient for comparison of T1 with macroscopic tumours was 0.91 (95 % confidence interval [CI]: 0.83 to 0.96) for all observers and 0.90 (95 % CI: 0.81 to 0.95) for STIR images. This difference was not statistically significant, and T1 and STIR sequence measurements had similar precision and accuracy. Bland-Altman plots showed T1-weighted imaging to be unbiased, whereas STIR sequences were biased and had systematic error. Moreover, STIR measurements overestimated tumour size by 6.4 mm (95 % CI: -26.9 to 39.7 mm) and 2 patients were outliers. T1 measurements were closer to the macroscopic measurements with a mean difference of 1.3 mm (95 % CI: -28.9 mm to 31.5 mm), with 3 patients falling outside of this. The variance was greater for STIR measurements. This difference between T1 and STIR measurements was statistically significant (p = 0.000003). The intra-observer reliability between separate measurements for MRI and specimen photographs achieved interclass correlation coefficients of 0.97, 0.96 and 0.95 (T1, STIR and macroscopic tumour respectively). T1 had greater interobserver correlation than for STIR and macroscopic tumour measurements (0.88 vs 0.85 and 0.85 respectively). These

  19. 单中心肩胛骨肿瘤166例流行病学分析%An one-center epidemiological analysis of scapular tumors:166 cases report

    Institute of Scientific and Technical Information of China (English)

    高明; 张清; 徐立辉; 刘巍峰; 牛晓辉

    2015-01-01

    133 cases of primary tumors and 33 cases metastatic tumors. One hundred and eleven cases of them were male patients and 55 were female, and the ratio of males to females was 2.02 : 1. Age of onset varied from 5 to 80 years. There were 74 cases ( 44.58% ) of primary malignancy, 33 cases ( 19.88% ) of metastatic malignancy, 18 cases ( 10.84% ) of intermediate tumors and 41 cases ( 24.70% ) of benign tumors. The median age of primary malignancy was 31 years old ( range: 6-80 years ). The most common tumor types were chondrosarcoma, Ewing’s sarcoma and osteosarcoma. The median age of metastatic malignancy was 54 years ( range: 36-79 years ). The most common primary tumor types were lung cancer, breast cancer and prostate cancer. The median age of intermediate tumors was 18 years ( range: 5-62 years ). The most common tumor types were chondroblastoma, Langerhans cell histiocytosis and giant cell tumors. The median age of benign tumors was 25 years ( range: 10-62 years ). The most common tumor types were osteochondroma, enchondroma and simple bone cyst.Conclusions Malignant tumors are more common than the benign ones among scapular tumors. Metastatic tumors, chondrosarcoma, Ewing’s sarcoma and osteosarcoma are common malignant types, and osteochondroma is the most common benign tumors located in scapula.

  20. Surgical reconstruction strategy of high level sacral tumors after tumor resection%高位骶骨肿瘤切除后的外科重建策略

    Institute of Scientific and Technical Information of China (English)

    吴强; 邵增务; 杨述华; 王佰川; 范磊

    2013-01-01

    目的探讨高位骶骨肿瘤切除后个性化重建方案。方法回顾分析自2000年9月至2011年12月,手术治疗的高位骶骨肿瘤11例,肿瘤类型包括骨巨细胞瘤、脊索瘤、软骨肉瘤、骨肉瘤及神经源性肿瘤。所有病例中,骶椎S1均受累。根据骶骨及骶髂关节侵犯范围,选择最佳手术方案,进行个性化外科重建。结果全部11例无术中死亡病例,术中平均出血量3200 ml。10例获得8个月至6年的随访,平均24个月,术后近期并发症1例为切口皮缘坏死和伤口延迟愈合;1例术后出现排尿困难,1例脑脊液漏。患者早期功能恢复良好,神经功能障碍改善率达66.7%。局部复发2例,分别为1例骨巨细胞瘤和1例软骨肉瘤,未出现远处转移病例。随访病例均未发现钉棒松动、断裂,以及同种异体腓骨植入后骨端吸收现象。结论良好的手术计划以及个性化的切除及重建方案可以保证手术的成功性。减少术中出血、合适地保留马尾神经功能以及骨盆环的重建是手术考虑的重点。%Objective The surgery of high level sacrum is a challenge in the ifeld of bone tumor therapy because of its special anatomic structure, large quantity of hemorrhage during the operation and the difficulty in reconstruction. This study is to investigate the individual reconstruction strategy of high level sacral tumor surgery. Methods The retrospective study included 11 patients from September 2000 to December 2011. The tumor type included bone giant cell tumor, chordoma, chondrosarcoma, osteosarcoma and neurogenic tumor. In all cases, the sacral vertebrae S1 was involved. The individualized reconstruction strategy was conducted according to the invasion area of the sacrum and sacroiliac joint. Results No patients died during the operation. The average amount of bleeding was 3200 ml. 10 cases were followed up for 8 months to 6 years, 24 months in average. The recent

  1. 带翼型髋臼加强杯在重建髋臼肿瘤切除后骨缺损中的应用%UTILIZATION OF REINFORCED ACETABULAR CAGES WITH CAUDAL FLANGE IN RECONSTRUCTING PELVIC DEFECT AFTER ACETABULAR TUMOR RESECTION

    Institute of Scientific and Technical Information of China (English)

    李大森; 郭卫; 杨荣利; 汤小东; 曲华毅

    2011-01-01

    Objective To evaluate the surgical procedure of reinforced acetabular cage with caudal flange in reconstruction of pelvic defect after acetabular tumor resection. Methods Between June 2003 and December 2009, 25 patients with Harrington class III pelvic defect underwent reconstruction with a reinforced acetabular cage with caudal flange and total hip arthroplasty (THA). There were 13 males and 12 females with an average age of 51.2 years (range, 13-73 years). The main clinical manifestations included hip pain and buttock pain, with a median disease duration of 6 months (range, 1-96 months). Pathological findings showed 18 cases of metastasis, 3 cases of multiple myeloma, 1 case of non-Hodgkin's lymphoma, 1 case of grade I chondrosarcoma, 1 case of giant cell tumor, and 1 case of chondroblastoma. For the patient with chondroblastoma, THA with LINK RIBBED system was used. An artificial total hip system made in China was used in 6 patients and LINK SP II system was used in the other 18 patients. Results No patients died perioperatively. Deep infection and hip dislocations occurred inI and 2 patients, respectively. At last follow-up, 8 of 18 patients with metastasis died of cancer and the average survival time wasII months. The other 10 who were alive were followed up 15 months on average. One patient with multiple myeloma died of pulmonary infection at 21 months after operation and the other 2 with multiple myeloma and 1 with lymphoma were alive with an average follow-up of 17 months. The patient with grade I chondrosarcoma and patient with chondroblastoma were followed up 58 and 12 months, respectively, without recurrence. Recurrence occurred in the patient with giant cell tumor at 19 months after operation. Loosening of implant occurred in 3 patients because of local tumor recurrence. For the 23 patients at 6 months after operation, the mean Musculoskeletal Tumor Society (MSTS) 93 score was 81% (range, 57%-93%). Conclusion Reinforced acetabular cage with caudal

  2. 肩胛带骨肿瘤的外科治疗结果及评价%Surgical treatment of bone tumors of the shoulder girdle

    Institute of Scientific and Technical Information of China (English)

    郭卫; 杨毅; 姬涛

    2008-01-01

    Objective To evaluate the surgical procedures and both oncological and functional outcome in patients with bone tumors of the shoulder girdle.Methods Seventy-one patients including 61.pts with malignant tumor and 10 pts with giant cell tumors of the shoulder girdle treated in our department from July 1998 to July 2006 were studied retrospectively.According to the location,there were 15 pts with scapula tumor and 56 pts with proximal humeral tumor.Forty-two male pts and 29 female pts were included in this study with an average age of 36.5 years old ranging from 11.to 62 years old.Surgical procedures:forequarter amputation in 10 pts;Scapulectomy in 3 pts;Scapulectomy and artificial scapular replacement in 3pts;partial scapulectomy and proximal humerus resection with prosthetic reconstruction in 8 pts;proximal humerus resection and prosthesis replacement in 47 pts.Results Due to adequate soft tissue was preserved,the mean functional score was 28 for the pts with giant cell tumor according to MSTS functional scoring system,compared with the functional score 23 for the pts with malignant tumor because the deltoid muscle insertion was resected in the latter group.Among the thirty-seven patients with osteosarcoma,local recurrence occurred in four cases(10.8%),lung metastasis in five and bone metastasis in two.The seven patients died of disease.One patient iagnosed of malignancy in giant cell tumor died after distal metastasis.thtee patients with Ewing sarcoma died of the disease.No recurrence Wag observed in both five humeral chondrosarcoma and five scapular chondrosarcoma.Conclusion Tumot resection and proximal humeral prostbesis replacement is the optimal method for the sarcoma of shoulder girdle in term of preservation of elbow and hand function.Preservation of abductor insertion is necessary for good abduction function.The metastatic rate of proximal humeral osteosarcoma was lower than that of lower extremities.also the prognosis of oncological outcome

  3. Contaminação tumoral em trajeto de biópsia de tumores ósseos malignos primários Tumor contamination in the biopsy path of primary malignant bone tumors

    Directory of Open Access Journals (Sweden)

    Marcelo Parente Oliveira

    2012-10-01

    chondrosarcoma were studied retrospectively. The sample was analyzed to characterize the biopsy technique used, histological type of the tumor, neoadjuvant chemotherapy used, local recurrences and tumor contamination in the biopsy path. RESULTS: Among the 35 patients studied, four cases of contamination occurred (11.43%: one from osteosarcoma, two from Ewing's tumor and one from chondrosarcoma. There was no association between the type of tumor and presence of tumor contamination in the biopsy path (p = 0.65. There was also no association between the presence of tumor contamination and the biopsy technique (p = 0.06. On the other hand, there were associations between the presence of tumor contamination and local recurrence (p = 0.01 and between tumor contamination and absence of neoadjuvant chemotherapy (p = 0.02. CONCLUSION: Tumor contamination in the biopsy path of primary malignant bone tumors was associated with local recurrence. On the other hand, the histological type of the tumor and the type of biopsy did not have an influence on tumor contamination. Neoadjuvant chemotherapy had a protective effect against this complication. Despite these findings, tumor contamination is a complication that should always be taken into consideration, and removal of the biopsy path is recommended in tumor resection surgery.

  4. Evaluation of Clinical Results and Complications of Structural Allograft Reconstruction after Bone Tumor Surgery

    Directory of Open Access Journals (Sweden)

    Mohammad Gharedaghi

    2016-07-01

    Full Text Available Background: Massive bone allograft is an option in cases of limb preservation and reconstruction after massive benign and malignant bone tumor resection. The purpose of this study was to analyze the outcome of these procedures at Imam Reza Hospital, Mashhad University of Medical Sciences. Methods: In this study, 113 cases have been presented. Eleven cases were excluded (patients has a traumatic defect or they passed away before the completion of the study’s two-year follow up period. Each patient completed a questionnaire, went through a physical examination and, if indicated, X-ray information was collected. The patients were divided into three groups: chemotherapy, chemotherapy plus radiation therapy, and no-adjuvant-therapy. Results: Fifty-four cases were male and the mean age was 24.5±5.39. The number of cases and indications for surgery were: 33 cases of aggressive benign tumors or low grade malignant bone tumors (large bone defects including 16 germ cell tumors, eight aneurysmal bone cysts, five low grade osteosarcomas, and four chondrosarcomas. Another 69 cases were high-grade malignant bone tumors including 42 osteosarcomas, 21 Ewing’s sarcoma, and six other high grade osteosarcomas. Patients were divided into three groups: the first group received no adjuvant therapy, the second group received chemotherapy, and the third group received chemotherapy plus radiotherapy. The location of tumors were as follows: eight cases in the pelvic bone, 12 in the proximal femur, 18 in the femoral shaft, 36 in the distal femur, 12 in the proximal tibia, and 16 in the humeral bone. The 12 cases of proximal femoral defects were reconstructed by allograft composite prosthesis, 18 diaphyseal defects with intercalary allograft, and 36 distal femoral defects were reconstructed using osteoarticular allograft. The rate of deep infection was 7:8% (eight patients and in this regard, we found a significant difference among the three groups, such that most

  5. Osteogenic tumors of bone; Osteogene Tumoren

    Energy Technology Data Exchange (ETDEWEB)

    Jobke, B. [Deutsches Krebsforschungszentrum (DKFZ), Abtl. Radiologie, Heidelberg (Germany); Werner, M. [MVZ des HELIOS Klinikum Emil von Behring, Orthopaedische Pathologie - Referenzzentrum, Institut fuer Gewebediagnostik Berlin, Berlin (Germany)

    2016-06-15

    Osteogenic tumors include malignant and benign tumors that produce tumor osteoid and/or bone tissue. Osteosarcoma is the most common malignant bone tumor, especially in children and young adults. The entities with their characteristic morphological features are described to enable the reader to come to a diagnosis and differential diagnosis on the basis of patient age, history and predominant location of the tumor. For this review we selectively used mainly large published patient cohorts. Our own and externally published data on widely accepted tumor criteria were also compared. Detection is the initial diagnostic step for an osseous lesion, and is determined by the sensitivity of the method applied. Plain X-ray films in two planes and CT are the basics in the radiological toolkit for osteogenic tumors. For evaluation of local tumor extension and biopsy planning MRI or scintigraphy should be combined. MRI as a stand-alone diagnostic tool is insufficient. For malignant bone tumors staging should be performed, applying a variable combination of thoracic CT, MRI, scintigraphy, and positron emission tomography (PET). Osteosarcoma, along with Ewing sarcoma and chondrosarcoma, are the most common malignant bone tumors; all sub-entities are significantly rarer. Among benign bone tumors, osteoid osteomas have the highest incidence, presenting with typical pain, location, and age predilection. Diagnostics and treatment of malignant bone tumors should preferably be performed in specialized centers because of significant therapeutic implications for patients. In uncertain cases, a second opinion should always be obtained. (orig.) [German] Osteogene Tumoren umfassen maligne und benigne Tumoren, die eine tumoreigene Produktion von Osteoid und/oder Knochengewebe aufweisen. Das Osteosarkom ist der haeufigste maligne Knochentumor v. a. bei Kindern und jungen Erwachsenen. Es werden die Entitaeten mit ihren morphologischen Charakteristika beschrieben, um anhand wichtiger

  6. MR imaging for evaluation of lesions of the cranial vault: a pictorial essay Avaliação por ressonância magnética das lesões da calota craniana: ensaio ilustrado

    Directory of Open Access Journals (Sweden)

    Lázaro Amaral

    2003-09-01

    Full Text Available PURPOSE: A variety of diseases affect the calvaria. They may be identified clinically as palpable masses or incidentally in radiologic examinations. There are many diagnostic possibilities, including congenital, neoplastic, inflammatory and traumatic lesions. The purpose of this study is to illustrate the main calvarial lesions through MR imaging, their signal intensity and extension to neighboring sites. METHOD: A retrospective analysis of 81 cases, from November 1996 to July 2001, was conducted. The examinations were performed on a 1.5 T equipment and each one of the cases was pathologically proven. RESULTS: The results were: dermoid cysts [4 cases (5%], epidermoid cysts [2 cases (2.5%], cephalocele [14 cases (17.5%], sinus pericranii [3 cases (3.7%], leptomeningeal cysts [4 cases (5%], Langerhans cell histiocytosis [10 cases (12.5%], lipoma [4 cases (5%], fibrous dysplasia [13 cases (16.2%], osteoma [8 cases (10%], hemangioma [1 case (1.2%], meningioma [3 cases (3.7%], chondrosarcoma [5 cases (6.2%], hemangiosarcoma [1 case (1.2%], multiple myeloma [3 cases (3.7%], sarcomatous transformation of Paget disease [1 case (1.3%], and metastasis [5 cases (6.2%]. CONCLUSION: MRI identifies bone marrow abnormalities and invasion of adjacent tissues at an early stage. Therefore, it is an essential method when it commes to properly evaluating calvarial lesions.OBJETIVO: A calota craniana é sede de diversas doenças, as quais podem ser identificadas clinicamente como massas palpáveis ou incidentalmente em estudos radiológicos. O diagnóstico diferencial é variado e inclui lesões de natureza congênita, neoplásica, inflamatória e traumática. O objetivo do nosso ensaio é ilustrar as principais lesões da calota craniana através de avalição por imagens de ressonância magnética (RM, expondo suas características de sinal e demonstrando sua extensão para os espaços adjacentes. MÉTODO: Foi realizada análise retrospectiva de 81 casos no per

  7. Comparative Analysis of CT and MRI Imaging Findings of Chondroblastoma%软骨母细胞瘤的CT及MRI影像表现对比分析

    Institute of Scientific and Technical Information of China (English)

    韩宏声

    2016-01-01

    目的:比较软骨母细胞瘤的CT及MRI影像表现。方法纳入我院2011年1月-2015年12月期间共收集36例软骨母细胞瘤患者进行研究,均行CT及MRI检查,比较二者对软骨母细胞瘤临床诊断的价值。结果 CT表现以病灶边缘硬化、病灶内钙化、骨膜反应、软组织肿胀、邻近关节腔积液为主,增强扫描后病灶内均示轻度到中度强化,CT值增高8-34HU。MRI示病灶多为卵圆形或类圆形囊状骨质破坏区、边界清晰,T1WI低信号、T2WI高低混杂信号,特殊征象包括片状、斑点状低信号钙化或骨嵴、病灶边缘低信号硬化带、T2WI病灶周围骨髓腔见长T2水肿信号。两种检查方法对膨胀性病变、窗格样改变、骺板或骺线穿越等征象的检出率无统计学意义(P>0.05);CT对边界清晰伴边缘硬化、病灶内钙化检出率分别为61.1%、66.7%,高于MRI的36.1%、41.7%,对骨髓水肿检出率仅为2.8%,低于MRI的27.8%,差异均有统计学意义(P0.05); The detection rates of CT for clear boundary with marginal sclerosis and calcification within the lesions (61.1%, 66.7%) were higher than those of MRI (36.1%, 41.7%) while the detection rate for bone marrow edema (2.8%) was lower than that of MRI (27.8%) (P<0.05).Conclusions CT and MRI in the diagnosis of chondroblastoma have their own advantages and disadvantages. CT can better show marginal sclerosis and calcification within the lesions while is poorer in displaying bone marrow edema. In clinical diagnosis, it needs to be identified from bone giant cell tumor, chondrosarcoma and chondroma etc..

  8. One-center bone tumors in the foot:an epidemiological analysis of 258 cases%单中心足部骨肿瘤258例临床流行病学分析

    Institute of Scientific and Technical Information of China (English)

    廖锋; 徐海荣; 牛晓辉

    2015-01-01

    目的:探究足部骨肿瘤流行病学特点。方法回顾1957年8月至2014年7月,北京积水潭医院骨肿瘤科诊治的258例足部骨肿瘤患者资料,分析其性别、年龄、病理诊断、发病部位及影像特点。结果男174例,女84例,年龄7~76岁,平均27.9岁。病变多位于跟骨、趾骨、距骨、跖骨,少见于足舟骨、楔骨和骰骨。恶性骨肿瘤38例,包括软骨肉瘤13例、骨肉瘤8例、Ewing 肉瘤6例、转移性肿瘤5例;中间型肿瘤81例,包括软骨母细胞瘤36例、骨巨细胞瘤29例、动脉瘤样骨囊肿10例、软骨粘液样纤维瘤3例;良性肿瘤139例,包括内生软骨瘤37例、甲下外生性骨疣27例、单纯性骨囊肿26例、骨软骨瘤19例、骨样骨瘤12例、骨脂肪瘤10例。结论足部骨肿瘤好发于中青年男性,多为良性或中间型。%Objective To explore epidemiological features of bone tumors in the foot and further to guide clinical diagnosis.Methods From August 1957 to July 2014, 258 patients were included. Gender, age, location, pathological and radiological data were reviewed.Results There were 174 males and 84 females, with a mean age of 27.9 years ( range: 7-76 years ). Bone tumors of the foot had particular predilections in the calcaneus, talus, phalanx and metatarsal bones. Thirty-eight cases of malignant tumors included chondrosarcoma ( 13 cases ), osteosarcoma ( 8 cases ), Ewing sarcoma ( 6 cases ) and metastatic tumors ( 5 cases ). Eighty-one cases of intermediate bone tumors included chondroblastoma ( 36 cases ), giant cell tumors of bone ( 29 cases ), aneurysmal bone cysts ( 10 cases ) and chondromyxoid ifbroma ( 3 cases ). One hundred and thirty-nine benign bone tumors included enchondroma ( 37 cases ), subungual exostosis ( 27 cases ), simple bone cyst ( 26 cases ), osteochondroma ( 19 cases ), osteoid osteoma ( 12 cases ) and lipoma of bone ( 10 cases ).Conclusions Benign and intermediate tumors are most common in

  9. The spectrum of EWSR1-rearranged neoplasms at a tertiary sarcoma centre; assessing 772 tumour specimens and the value of current ancillary molecular diagnostic modalities

    Science.gov (United States)

    Noujaim, Jonathan; Jones, Robin L; Swansbury, John; Gonzalez, David; Benson, Charlotte; Judson, Ian; Fisher, Cyril; Thway, Khin

    2017-01-01

    Background: EWSR1 rearrangements were first identified in Ewing sarcoma, but the spectrum of EWSR1-rearranged neoplasms now includes many soft tissue tumour subtypes including desmoplastic small round cell tumour (DSRCT), myxoid liposarcoma (MLPS), extraskeletal myxoid chondrosarcoma (EMC), angiomatoid fibrous histiocytoma (AFH), clear cell sarcoma (CCS) and myoepithelial neoplasms. We analysed the spectrum of EWSR1-rearranged soft tissue neoplasms at our tertiary sarcoma centre, by assessing ancillary molecular diagnostic modalities identifying EWSR1-rearranged tumours and reviewing the results in light of our current knowledge of these and other Ewing sarcoma-like neoplasms. Methods: We retrospectively analysed all specimens tested for EWSR1 rearrangements by fluorescence in situ hybridisation (FISH) and/or reverse transcription–PCR (RT–PCR) over a 7-year period. Results: There was a total of 772 specimens. FISH was performed more often than RT–PCR (n=753, 97.5% vs n=445, 57.6%). In total, 210 (27.9%) specimens were FISH-positive for EWSR1 rearrangement compared to 111 (14.4%) that showed EWSR1 fusion transcripts with RT–PCR. Failure rates for FISH and RT–PCR were 2.5% and 18.0%. Of 109 round cell tumours with pathology consistent with Ewing sarcoma, 15 (13.8 %) cases were FISH-positive without an identifiable EWSR1 fusion transcript, 4 (3.7%) were FISH-negative but RT–PCR positive and 4 (3.7%) were negative for both. FISH positivity for DSRCT, MLPS, EMC, AFH and CCS was 86.3%, 4.3%, 58.5%, 60.0% and 87.9%, respectively. A positive FISH result led to diagnostic change in 40 (19.0%) EWSR1-rearranged cases. 13 FISH-positive cases remained unclassifiable. Conclusions: FISH is more sensitive for identifying EWSR1 rearrangements than RT–PCR. However, there can be significant morphologic and immunohistochemical overlap between groups of EWSR1-rearranged neoplasms, with important prognostic and therapeutic implications. FISH and RT–PCR should be used as

  10. Quantification of the magnetization-transfer contrast effect: can it yield additional information in differentiation of musculoskeletal lesions particularly in separation of benign from malignant lesions; Quantifizierung des Magnetization Transfer Contrast (MTC) Effektes durch Berechnung von MT-Quotienten: Ergeben sich Zusatzinformationen fuer die Differenzierung benigner und maligner Erkrankungen des Bewegungsapparates?

    Energy Technology Data Exchange (ETDEWEB)

    Vahlensieck, M.; Traeber, F.; Schild, H. [Radiologische Universitaetsklinik Bonn (Germany); Gieseke, J. [Philips Medizinsysteme (Germany)

    1999-12-01

    Purpose: To investigate the potential information of the amount of magnetization-transfer effect in musculoskeletal lesions and to compare MT ratios from benign and malignant musculoskeletal lesions. Material and Method: 49 patients with malignant tumors (3 osteosarcoma, 3 malignant fibrous histiocytoma, 4 chondrosarcoma, 2 Ewing sarcomas) and benign lesions (8 chondroma, 2 fibrous dysplasia, 3 osteoid-osteoma, 6 ganglion cyst, 3 cyst, 3 osteomyelitis, 4 tendinitis, 3 rotator cuff tear, 5 scar tissue) were scanned using routine MRI protocols including T{sub 1}- and T{sub 2}-weighted spin echo as well as T{sub 2}*-weighted gradient echo (FFE) sequences at 1.5 Tesla (ACS II, Philips Medical). Additionally MTC images were generated by combining the FFE sequence and the off-resonance MT technique (-1500 Hz off-resonance frequency, 1770 flip angle and 50 ms pulse duration). MT ratios were calculated as SI{sub o}-SI{sub m}/SI{sub o}. Results: The MT ratio of benign lesions was 26{+-}15%, that of malignant lesions was 22{+-}6%. The difference was statistically not significant. As expected muscle showed a high MT ratio of 50{+-}8%. Scar tissue demonstrated an MT ratio of 39{+-}16% which was significantly higher than the tumor MT ratios. Conclusion: MTC (MT ratios) failed to show significant differences between benign and malignant lesions as was expected due to basic differences in cellularity, rate of mitosis and chromatin content. MTC might however gain more importance in separating scar tissue from recurrent tumor in the future. (orig.) [German] Zielsetzung: Durch die Quantifizierung des Magnetization Transfer Contrastes sollte untersucht werden, ob sich Zusatzinformationen in der Magnetresonanztomographie des Stuetz- und Bewegungsapparates ergeben. Insbesondere sollte ermittelt werden, ob gut- und boesartige Laesionen unterschiedliche MT-Quotienten aufweisen. Material und Methode: 49 Patienten mit boesartigen Tumoren (3 Osteosarkom, 4 Chondrosarkom, 3 Malignes

  11. Surgical treatment for giant tumors of the pelvis%骨盆巨大肿瘤的手术治疗

    Institute of Scientific and Technical Information of China (English)

    齐波; 丁晶; 徐永清; 阮默; 林月秋

    2014-01-01

    Objective To explore the method of surgical treatment for giant tumors of the pelvis. Methods 21 cases with giant tumors of the pelvis were hospitalized in the period from Jan. ,2000 to Dec. ,2007,17 male and 4 female,the average age was 55 years old(from 35 to 65),including 8 cases of sacral chordoma,5 cases of iliac chondrosarcoma,4 cases of chondroblastoma,3 cases of giant cell tumor(GCT)of bone and 1 case of osteosarcomas;all the patients underwent surgical resection with temporary blocking of bilateral or unilateral common iliac arteries. Results Follow-up lasted from 0. 5 year to 6 years with the average of 4 years;local reoccurrence was found in 4 cases of sacral chordom,1 case died of lung metastases and systemic failure,1 case died of lung metastases 1. 5 year after surgery;no reoccurrence was found in other cases;8 cases had discomfort with pain of unknown causes in the operation sites. Conclusions Temporary blocking of bilateral or unilateral common iliac artery in operation can obviously reduce the bleeding, make a complete resection of the tumors at most and maintain the stability of the pelvis,which raises the surgical effect on patients with giant tumors of pelvis.%目的:探讨骨盆巨大肿瘤的手术治疗方法。方法我院自2000年1月~2007年12月共收治21例骨盆巨大肿瘤患者,男17例,女4例,平均55(35~65)岁。其中骶骨脊索瘤8例,髂骨软骨肉瘤5例,软骨母细胞瘤4例,骨巨细胞瘤3例,骨肉瘤1例,均采用双侧或单侧髂总动脉暂时阻断的方法行手术切除。结果所有患者术后获得0.5~6年(平均4年)随访,脊索瘤4例局部复发,1例死于肺部转移全身衰竭,1例于术后1年半死于肺部转移,其余无复发;8例出现手术部位不明原因疼痛不适。结论术中采用双侧或单侧髂总动脉暂时阻断的方法可明显减少术中出血,尽量彻底切除瘤体及维持骨盆稳定性等,可明显提高骨盆巨大肿瘤患者的手术疗效。

  12. Proton therapy - Present and future.

    Science.gov (United States)

    Mohan, Radhe; Grosshans, David

    2017-01-15

    In principle, proton therapy offers a substantial clinical advantage over conventional photon therapy. This is because of the unique depth-dose characteristics of protons, which can be exploited to achieve significant reductions in normal tissue doses proximal and distal to the target volume. These may, in turn, allow escalation of tumor doses and greater sparing of normal tissues, thus potentially improving local control and survival while at the same time reducing toxicity and improving quality of life. Protons, accelerated to therapeutic energies ranging from 70 to 250MeV, typically with a cyclotron or a synchrotron, are transported to the treatment room where they enter the treatment head mounted on a rotating gantry. The initial thin beams of protons are spread laterally and longitudinally and shaped appropriately to deliver treatments. Spreading and shaping can be achieved by electro-mechanical means to treat the patients with "passively-scattered proton therapy" (PSPT) or using magnetic scanning of thin "beamlets" of protons of a sequence of initial energies. The latter technique can be used to treat patients with optimized intensity modulated proton therapy (IMPT), the most powerful proton modality. Despite the high potential of proton therapy, the clinical evidence supporting the broad use of protons is mixed. It is generally acknowledged that proton therapy is safe, effective and recommended for many types of pediatric cancers, ocular melanomas, chordomas and chondrosarcomas. Although promising results have been and continue to be reported for many other types of cancers, they are based on small studies. Considering the high cost of establishing and operating proton therapy centers, questions have been raised about their cost effectiveness. General consensus is that there is a need to conduct randomized trials and/or collect outcomes data in multi-institutional registries to unequivocally demonstrate the advantage of protons. Treatment planning and plan

  13. Clinicopathological and molecular spectrum of ewing sarcomas/PNETs, including validation of EWSR1 rearrangement by conventional and array FISH technique in certain cases.

    Science.gov (United States)

    Rekhi, Bharat; Vogel, Ulrich; Basak, Ranjan; Desai, Sangeeta B; Jambhekar, Nirmala A

    2014-07-01

    Over the years, a wide clinicopathological spectrum has been identified within Ewing family of tumors (EFTs). As these tumors are chemosensitive, their correct and timely identification is necessary. The aims of this study were (1) to present the diverse clinicopathological and molecular profile of EFTs in our settings, (2) to identify a pragmatic approach for diagnosing EFTs, especially for application of ancillary techniques, namely RT-PCR for specific transcripts (EWS-FLI1, EWS-ERG) and FISH for EWSR1 gene rearrangement, in certain cases and (3) to show the utility of tissue microarray in establishing a new FISH test. Fifty-eight EFTs were identified in 38 males and 20 females within an age-range of 1-65 years (median, 16), mostly in lower extremities (14) (24.1 %). Therapeutically, most patients underwent neoadjuvant chemotherapy with subsequent surgery. Histopathologically, diagnosis of EFTs was initially offered in 41/58 (70.6 %) tumors. On review, 59 % tumors showed diffuse pattern, while 41 % displayed rosettes. Immunohistochemically, tumor cells were mostly diffusely positive for CD99 (48/52) (92.3 %); FLI-1 (17/18) (94.4 %); variably for BCL2 (16/18) (88.8 %), synaptophysin (6/20) (35 %), S100-P (2/7) (28.5 %), CD56 (2/5) (40 %), NSE (2/5) (40 %), calponin (3/4) (75 %), EMA (5/24) (20.8 %) and CK (3/24) (12.5 %), the latter two mostly focally. Fifty five tumors were EWS-FLI1 positive, while a single tumor was EWS-ERG positive. Sensitivity for PCR was 61 %. EWSR1 rearrangement was detected by FISH in 12/13 Ewing sarcomas/PNETs. Sensitivity for EWSR1 test was 92.3 % and specificity was 100 %. Thirty-eight tumors, including 14 molecular confirmed EFTs and 21 other tumors were tested for EWSR1 rearrangement. Among 21 unrelated tumors, EWSR1 rearrangement was detected in few myoepithelial tumors, occasional desmoplastic small round cell tumor and an extraskeletal myxoid chondrosarcoma. Further, a tissue microarray with a separate set of 8 EFTs, confirmed at

  14. Clinic and imaging analysis of primary malignant tumors of the pelvis%骨盆原发性恶性肿瘤的影像学临床分析

    Institute of Scientific and Technical Information of China (English)

    徐维敏; 胡碧莹; 陈丽君; 陈卫国; 唐浩; 罗振东

    2012-01-01

    目的:探讨骨盆原发性恶性肿瘤的病种分布及其影像学表现.方法:回顾本院11年间经活检和/或手术病理证实的骨盆原发性恶性肿瘤56例,分析其发病情况及影像学主要表现.结果:骨盆原发性恶性肿瘤共56例,按其发生比例依次为骨髓瘤(包括单发及多发性骨髓瘤)34例(占60.7%),软骨肉瘤、尤文氏肉瘤及原发性骨淋巴瘤各6例(分别占10.7%),骨肉瘤2例(占3.6%),恶性骨母细胞瘤及恶性纤维组织细胞瘤各1例(占1.8%).主要影像表现为溶骨性骨质破坏(48例)或混合性骨质破坏(6例),软组织肿块或肿胀(22例),病灶边界清楚(35例),边界模糊(21例);出现瘤骨或钙、骨化(9例).少数可出现骨膜反应(4例).结论:骨盆原发性恶性肿瘤种类较多,影像学表现缺乏特异性,综合影像检查可提高对各类病变的诊断及鉴别诊断准确率.%Objective-To explore the incidence, pattern of distribution, and imaging features of primary malignant tumors of the pelvis. Methods: 56 cases with primary malignant tumors of the pelvis cnfirmed by pathology during 11 years in our hospital were retrospectively studied. The incidence and imaging features were analyzed. Results: Among the 56 cases, there were several of primary malignant tumors of the pelvis. The type of these cases in order was myeloma (single or/and multiple) in 34 cases (60.7%), chondrosarcoma in 6 cases (10. 7%)< Ewing's sarcoma in 6 cases (10. 7%). Primary lymphoma of bone in 6 cases (10. 7%), primary osteosarcoma in 2 cases (3. 6%), aggressive osteoblastoma in 1 case (1. 8%). Primary malignant fibrous histiocytoma of bone in 1 case (1. 8%). The principal imaging features were osteolys-is cortical destruction (48 cases), mixing cortical destruction (6 cases), soft-tissue masses or swelling (22 cases), sharpness of focus border (35 cases), obscure boundary of focus (21 cases), calcification (9 cases), and periosteal reaction (4 cases). Conclusion

  15. 肿瘤型膝关节假体术后并发症214例分析%Complications after the reconstruction of bone defects in the knee with tumor endoprostheses

    Institute of Scientific and Technical Information of China (English)

    覃澍; 董扬

    2016-01-01

    Objective To retrospectively analyze the prosthesis survival and complications after custom-made tumor prosthesis replacement of the knee joint. Methods Data of 214 patients with bone tumors around knee joints who underwent custom-prosthetic replacement from January 2003 to January 2013, were retrospectively analyzed. The diagnoses were osteosarcoma ( n = 136 ), giant cell tumor ( n = 49 ), aneurysmal bone cyst ( n = 1 ), fasciculated sarcoma ( n = 3 ), malignant fibrous histiocytoma ( n = 10 ), chondrosarcoma ( n = 6 ), chondroblastoma ( n = 1 ), metastatic tumor ( n = 4 ) and other types of bone tumors ( n = 3 ). The distal femur was affected in 150 patients and the proximal tibia in 64. Results Totally 187 patients were followed up for a mean period of 47 months ( range: 16 -122 months ). Complications occurred in 47 patients: early stage infection or nonunion of wounds ( n = 15 ), peroneal palsy ( n = 3 ), late infection ( n = 5 ), aseptic loosening ( n = 15 ), breakage of the prosthesis ( n = 4 ) and periprosthetic fracture ( n = 5 ). Conclusions The complication rate of proximal tibia tumor is higher than the rate of the distal femur. Common complications of tumor prosthesis replacement of the knee joint are: aseptic loosening, infection or nonunion of wounds, peroneal palsy, late infection, aseptic loosening, breakage of prosthesis and periprosthetic fracture. Common complications of tumor prosthesis replacement of the knee joint can be treated and prevented.%目的:分析肿瘤型膝关节假体置换术后并发症的发生情况以及处理方式。方法回顾性分析2003年1月至2012年1月,在我院行膝关节假体置换的214例术后并发症发生情况;其中骨肉瘤136例,骨巨细胞瘤49例,动脉瘤样股囊肿1例,梭形细胞肉瘤3例,恶性纤维组织细胞瘤10例,软骨肉瘤6例,软骨母细胞瘤1例,软骨细胞瘤1例,转移癌4例,其它骨肿瘤3例;根据位置分类,发生于股骨远端150

  16. Rehablitation of the devitalization and replantation after resection of malignant tumor segment around the knee%膝关节周围骨恶性肿瘤瘤段切除灭活再植术的康复治疗

    Institute of Scientific and Technical Information of China (English)

    俞光荣; 蔡宣松; 梅炯; 郭获萍; 袁锋

    2000-01-01

    Objective To study the rehabilitation related to recovery of the limb function for the devitalizationand replantation after resection of malignant tumor segment around the knee. Methods Seventy nine patients with themalignant tumor around the knee (proximal tibia 21 cases, distal femur 58 cases) were reviewed. There were 35 malesand 44 females, aged ranged from 8 to 66 years (mean, 31.5 years). The tumors commonly seen were osteosarcomas(57), giant cell tumors (6), MFH (6) and chondrosarcomas (5). The devitalization and replantation after intraarticu-lar resection of tumor segment (CRIRT) was performed in 75 cases, that after extraarticular resection of tumor seg-ment (CRERT) was used in 4 cases. Functional training with different programs were performed in three periods aftersurgery. Results Seventy nine patients were followed up for 1~ 7 years (mean, 4.2 years). 5 years survival rate was42 % (33 cases). Functional assessment using Ennekings criteria was evaluated. The mean score in 44 cases of CRIRTwas 20(14~25) points and satisfactory (good or excellent results were 77% (34/44). The mean score in 4 CRERTcases was 24(22~28) points and all were satisfactory. Conclusion The devitalization and replantation after resectionof tumor segment is a good limb salvage surgery for malignant tumor segment around the knee. Rehabilitation programshelp recovery of the limb function. (Shanghai Med J, 2000,23:724-727)%目的 探讨膝关节周围骨恶性肿瘤瘤段切除灭活再植术功能恢复相关的康复治疗。方法本组79例,其中胫骨上端21例,股骨远端58例。常见肿瘤中骨肉瘤57例,巨细胞瘤6例,恶性纤维组织细胞瘤6例,软骨肉瘤5例。关节内瘤段切除灭活再植75例,关节外瘤段切除灭活再植4例。术后分3个阶段采用不同的康复计划进行功能训练。结果79例随访1~7年(平均4.2年),5年生存率42%(33例)。按Enneking法功能评定,关节内瘤段切除灭活再植44

  17. Activation of matrix metalloproteinase-13 in the osteoarthritis%基质金属蛋白酶-13在骨性关节炎发病中的活性调控研究

    Institute of Scientific and Technical Information of China (English)

    杨林; 郭艾

    2011-01-01

    Objective To investigate the activation mechanism of matrix metalloproteinase-13 zymogen (pro-MMP-13) induced by nitric oxide (NO). Methods Human chondrosarcoma cells (SW1353) were grouped and treated with the NO donor S-nitroso-N-acetyl-penicillamine (SNAP), SNAP + NO scavenger oxyhemoglobin (OxyHb), and SNAP + tissue inhibitor of metalloproteinase material -2 (TIMP-2) respectively. After stimulation, matrix metalloproteinase -13 ( a-MMP-13 ) expression level was detected. Besides, the expression levels of MT1- MMP were detected after SW1353 cells stimulated with SNAP and SNAP + OxyHb, respectively. At last, the activities of the recombinant MMP-13 (r-MMP-13) were detected after r-MMP-13 stimulated with SNAP, recombinant MT1-MMP (r-MT1-MMP) and r-MT1-MMP + TIMP-2,respectively. Results SNAP increased human chondrocytes a-MMP-13 expression, OxyHb and TIMP-2 inhibited a- MMP- 13 expression. SNAP also increased the expression of MT1- MMP levels, OxyHb inhibited the expression of MT1-MMP. r-MT1-MMP activated the r-MMP-13, but SNAP did not, TIMP-2 inhibited the activity of r-MMP-13 induced by r-MT1-MMP. Conclusions NO can not directly activate pro-MMP13 by the role of S-nitroso-(S nitrosylation). MT1-MMP mediates the activiation of pro-MMP-13 induced by NO.%目的 研究一氧化氮(NO)是否通过膜型基质金属蛋白酶-1(MT1-MMP)间接激活基质金属蛋白酶-13酶原(pro-MMP-13).方法 购买并传代人软骨肉瘤细胞(SW1353),用NO供体S-亚硝基-N-乙酰基青霉胺(SNAP),SNAP+NO清除荆氧合血红蛋白(OxyHb)和SNAP+组织金属蛋白酶抑制物-2(TIMP-2)分别刺激后检测活性基质金属蛋白酶-13(a-MMP-13)的表达水平.检测SNAP和SNAP+OxyHb分别刺激SW1353后MT1-MMP的表达水平.用SNAP重组MT1-MMP(r-MT1-MMP)和r-MT1-MMP+TIMP-2,分别刺激重组MMP-13(r-MMP-13),检测其活性变化.结果 SNAP增加了人软骨细胞a-MMP-13的表达水平,OxyHb和TIMP-2可以抑制a-MMP-13的表达水平.SNAP增加了人软骨细胞MT1-MMP的

  18. CT and MRI manifestations of sacrum tumors involving sacroiliac joint%骶骨肿瘤侵犯骶髂关节的CT及MRI表现

    Institute of Scientific and Technical Information of China (English)

    娄路馨; 程晓光; 白荣杰; 张薇; 梁伟; 张卉

    2011-01-01

    目的 分析良、恶性骶骨肿瘤侵犯骶髂关节的CT及MRI表现.方法 观察51例骶骨肿瘤对骶髂关节侵犯的CT和MRI表现.其中骨巨细胞瘤19例,神经鞘瘤6例,神经纤维瘤1例,脊索瘤7例,恶性纤维组织细胞瘤、小细胞肉瘤和梭形细胞肉瘤各1例,软骨肉瘤6例,尤因肉瘤2例,黏液乳头状室管膜瘤1例,转移癌6例.结果 骶髂关节受侵29例(29/51,56.86%),其中双侧骶髂关节受侵11例,单侧受侵18例.骶髂关节受侵发生率在良性肿瘤患者中为53.85%(14/26),在恶性患者中为60.00%(15/25).CT表现为关节双侧骨质破坏、关节面消失,软组织肿块占据骶髂关节;MRI表现为骶髂关节双侧骨质信号消失,被肿瘤信号取代.结论骶骨恶性肿瘤常侵犯骶髂关节.骶骨良性肿瘤中,骨巨细胞瘤最易侵犯骶髂关节.%Objective To observe CT and MRI manifestations of the invasion of sacroiliac joint by benign and malignant sacrum tumor.Methods CT and MR images of 51 patients with the invasion of sacroiliac joint by sacrum tumors, including 19 giant cell tumors, 6 schwannomas, 1 neurofibroma, 7 chordomas, 1 malignant fibrohistiocytoma, 1 small cell sarcoma, 1 spindle cell sarcoma, 6 chondrosarcomas, 2 Ewing sarcomas, 1 myxopapillary ependymoma and 6 metastases were analyzed.Results Sacroiliac joint invasion was found in 29 patients (29/51, 56.86%).Bilateral invasion of sacroiliac joint occurred in 11, while 18 unilateral invasion occurred in 18 cases.The rate of invasion of sacroiliac joint was 53.85 % (14/26) in patients with benign tumors and 60.00% (15/25) in patients with malignant tumors.Bilateral bone destruction of the joints, disappearance of articular surface and the replacement of sacroiliac joint by soft tissue mass were observed on CT.MRI showed that the signal of bilateral sacroiliac joint were replaced by tumor signal.Conclusion Sacroiliac joint is easily to be invaded by malignant sacrum tumors, as well as by giant cell tumor.

  19. Biopsy in Musculoskeletal Tumors

    Directory of Open Access Journals (Sweden)

    Mohammad Gharehdaghi

    2014-09-01

    proximity to the skin incision, because this tract is also contaminated and must be excised with the surgical specimen. Imaging-guided core needle biopsy is a well-established technique for the diagnosis of bone and soft tissue tumors and tumor-like lesions in specialized orthopedic oncology centers. Although large lesions of the limbs can easily be biopsied without image guidance, lesions in the spine, para spinal area, and pelvis are difficult to target, therefore taking the advantage of C.T. guidance will improve the accuracy of targeting the lesion for biopsy purposes. We can benefit from image intensifiers for targeting limb lesions rather than C.T. guidance. Also sonographic guide can be applied for soft tissue lesions (Figure D, E, F. In soft tissue tumors, the results of percutaneous CNB are relatively inferior compared to open biopsy whereas almost equal results are expected for bony tumors except for low-grade chondrosarcoma. CNB is a safe, minimally invasive, and cost effective technique for the diagnosis of bone lesions if done by an experienced orthopedic oncologic surgeon and be evaluated by an experienced anatomical bone pathologist (1, 3. For soft tissue tumors, CNB results depend on the size of the lesion, its location and amount of tumor necrosis. Guided needle biopsy have become the standard technique in most orthopedic oncologic centers. The accuracy of this method in our center is more than 90% for bone tumors. Cores should be taken in different directions including areas of central necrotic tissues but from a single well planned entrance. The procedure is quick, especially for bone CNB or soft tissue FNA and CNB, and the diagnosis can be achieved within 24 – 48 hours. The material should be sufficient for immunohistochemistry evaluations as well (1-3. Because I have seen 3 cases of tumor implantation at the towel clips puncture site including 2 chondrosarcomas and a case of malignant schowanoma, so I highly suggest that never crush the skin by towel

  20. Clinical analysis on the surgical treatment of periacetabular tumors%髋臼部位肿瘤手术治疗的临床分析

    Institute of Scientific and Technical Information of China (English)

    倪建法; 周幸; 周光新; 计忠伟; 胡斌; 陆萌; 吴苏稼; 施鑫

    2014-01-01

    discuss the methods, effects, and complications of surgical treatment of periacetabular tumors. Methods We retrospectively analyzed the clinical data of 36 cases of periacetabular tumor surgically treated in our depart-ment, including 21 males and 15 females, aged 15 to 64 (45.2 ±6.3) years.Among them, there were 8 cases of benign tumor (4 ca-ses of simple bone cyst, 1 case of desmoplastic fibromas, 1 case of osteofibrous dysplasia, and 2 cases of hemangioma) and 28 cases of malignant tumor (5 cases of osteosarcomas, 12 cases of chondrosarcomas, 2 cases of giant cell tumor of the bone, 1 case of malignant chondroblastoma, 2 cases of malignant fibrohistiocytoma, and 5 cases of metastatic tumor of the bone).The benign cases received sim-ple tumor resection plus bone graft and steel plate system internal fixation, while malignant cases underwent reconstruction by combined pedicle screw fixation system with bone cement. Results No patients died perioperatively and controllable complications occurred in 5 cases after operation.All the patients were followed up for 5 to 96 months except for 1 case of benign tumor.None of the benign cases experienced recurrence, and all of them achieved normal hip function.Lung metastases occurred in 10 malignant cases (4 cases of os-teosarcomas and 6 cases of chondrosarcomas) , who died of respiratory failure, and the other 18 remained tumor-free after surgery.Of the 2 patients with malignant fibrohistiocytoma, 1 died 2 years postoperatively and the other survived with tumor.Neither local recur-rence nor lung metastasis was found in the patients with chordoma and malignant chondroblastoma till the end of follow-up.The 5 pa-tients with metastases died within 3 years after operation.At 3 months after surgery, the Harris scores after total hip replacement were >90 in the 7 benign cases, 70-79 in 9 malignant cases, and <70 in the other 19 malignant cases. Conclusion For the treatment of malig-nant tumors, reconstruction by combined pedicle screw

  1. 关节外整块切除肩胛带恶性肿瘤的手术及术后功能研究%Limb-sparing resections for malignant tumors of shoulder girdle:11-year experience of classical or modified tikhoff-linberg procedures in a single institution

    Institute of Scientific and Technical Information of China (English)

    谢璐; 郭卫; 汤小东; 杨荣利

    2015-01-01

    to 82 y).All patients were followed up for a mean of 34 months (range,6 to 109 months).The pathologic diagnosis obtained by needle or open biopsies included 10 metastatic tumors (4 from lung cancer,3 from breast cancer,1 from thyroid cancer and 2 from unknown origin),7 chondrosarcomas,4 osteosarcomas,3 Ewing sarcoma/PNET,3 synovial sarcomas,2 malignant peripheral nerve sheath tumors,1 malignant fibrous histocytoma,1 fibrous sarcoma,1 liposarcoma and 1 malignant hemangiopericytoma.The stage and extent of the tumor were evaluated according to the Enneking system.Neoadjuvant chemotherapy was administered to patients with osteosarcoma,Ewing's sarcoma/PNET and partial malignant soft tissue sarcoma.As for other anatomical sites,limb salvage procedures for bone tumors of the shoulder girdle comprise three steps:complete resection of tumors,reconstruction of bone defects and good soft tissue covering.According to the surgical classification system proposed by Linberg and Malawer et al,classical Tikhoff-Linberg resection (also called shoulder girdle resection Malawer type Ⅳ )contained extraarticular en bloc resection of proximal humerus or humeral head,glenohumeral joint,the whole scapular and lateral clavicle.While modified Tikhoff-Linberg procedure (Malawer type Ⅴ)meant extra-articular resection of proximal humerus,glenohumeral joint,lateral clavicle and the neck of scapular glenoid.The surgical margins of the resected tumor were grossly and microscopically evaluated by two different experienced pathologists respectively.If they disagreed in opinion,the margins would be judged by another doctor who usually would be a senior pathologist.Results All those 33 patients survived the perioperative period and were followed up clinically after surgery (mean follow-up time 34 m,range 6 to 109 m).At the final follow-up,6 patients were alive without evidence of disease,2 patients remained alive but with disease,and 25 had died of their disease.In patients with metastatic bone

  2. Limb salvage surgery for malignant bone tumors of the extremities in children and adolescents%儿童及青少年保留骨骺的保肢手术临床研究

    Institute of Scientific and Technical Information of China (English)

    Zhen Wang; Zheng Guo; Xiangdong Li; Luyu Huang; Qiang Ji; Haiqiang Wang; Hongxun Sang; Jun Fu

    2009-01-01

    Objective: The aim of the study was to analyze the clinical features of limb salvage surgery with epiphyseal preservation in children and adolescents, and to evaluate the recurrence rate, metastasis, complications, and the joint func-tional results after tumor resection. Methods: Between December 1995 and January 2003, 33 cases of preserving epiphysis procedure had been done. In this group, the tumor located in distal femur in 24 cases and in proximal tibia in 9 cases. There were 23 osteosarcomas, 6 Ewing's sarcomas, 2 chondrosarcomas, and 2 aggressive osteoblastomas. The patients received 2-4 cycles of preoperative adjuvant chemotherapy, and another 6 cycles after surgery. The modified protocol T10 was taken. Radiography and MRI were used to determine the margins of the tumors preoperatively and histological examination was used to corroborate the evidence intraoperatively. The tumors were staged clinically. Among them, there were 2 cases in ⅠA, 2 cases in ⅠB 17 cases in ⅡA, and 12 cases in ⅡB. According to the MRI analysis, the metaphyseal tumors in children were classified into 3 types. Type Ⅰ, with the tumor close to but not contacting epiphyseal plate, and the distance between the two over 2 cm, was taken as absolute indication for the technique. Type Ⅱ, with the tumor near or contacting epiphysis plate, and the distance between the two less than 1 cm, was taken as relative indication for the surgery. For type Ⅲ, the tumor contacted the epiphyseal plate partially, and was over 2 cm beyond the joint, in the cohort, 18 cases were cetegorized as type Ⅰ, 13 as type Ⅱ, and 2 as type Ⅲ. The size of the residual epiphyseal bone segment differed after different excision protocols which were taken according to the clinical classifications. Bone defects after tumor resections were repaired with massive intercalary allograft bone, followed by internal fixation by intramedullary nails and cancellous screws. Results: Among the 33 cases, 3 cases were

  3. Alcohol-inactivated autograft replantation in reconstruction of bone defects in management of malignant bone tumors%酒精灭活瘤段骨在重建恶性骨肿瘤骨缺损中的应用

    Institute of Scientific and Technical Information of China (English)

    许宋锋; 刘江; 聂鑫; 于秀淳; 徐明; 王冰; 郑凯; 付志厚; 宋若先

    2015-01-01

    Objective To evaluate the clinical outcomes of alcohol-inactivated autograft replantation in reconstruction of bone defects in management of malignant bone tumors,and to analyze the related factors affecting the survival rate and functional results.Methods The clinical data of 53 patients with malignant bone tumors who were admitted and treated by alcohol-inactivated autograft replantation from January 1995 to June 2013 were retrospectively analyzed.The mean age was 17.7 years old (range: 9-49 years ).Twenty-three neoplasms were located in the distal femur,2 neoplasms in the middle femur,20 neoplasms in the proximal tibia,3 neoplasms in the distal tibia,3 neoplasms in the proximal humerus,1 neoplasm in the sacrum and 1 neoplasm in the ilium respectively.According to the Enneking system,48 patients were identiifed as stage Ⅱb and 5 patients as stage Ⅲ.Forty-four patients were diagnosed as osteosarcoma,6 patients as Ewing's sarcoma,1 patient as rhabdomyosarcoma,1 patient as chondrosarcoma,and 1 patient as non-Hodgkin's lymphoma.The postoperative results were evaluated according to the Musculoskeletal Tumor Society ( MSTS ) system and the International Society of Limb Salvage ( ISOLS ) graft evaluation method.A statistical analysis of the correlation among all the related factors,survival and postoperative function was conducted.Results The mean follow-up period was 55 months ( range: 13-216 months ).Thirty-nine patients were treated with wide resection and 14 patients with marginal resection.The average length of alcohol-inactivated autograft was 16.3 cm ( range: 5-26 cm ).The recurrence rate was 22.9%( 11/48 ) in stage Ⅱb group,and there were 9 cases of pulmonary metastases with the metastasis rate of 18.8%,6 death cases and 5 cases of survival with tumors.The recurrence rate was 15.9%( 7/44 ) in osteosarcoma group,and there were 6 cases of pulmonary metastases with the metastasis rate of 13.6%and 35 cases of survival during the follow-up of 3 years

  4. Clinicopathologic Analysis of Ovary Guvenile Granulosa Cell Tumor%卵巢幼年型粒层细胞瘤临床病理观察

    Institute of Scientific and Technical Information of China (English)

    崔华娟; 赖日权; 王卓才; 王炜; 彭大云

    2012-01-01

    abdomen three years after spealbone chondrosarcoma surgery and diagnosed as JGCT at stage FIGOm after the surgery. The patient died 26 months after surgery. Another case presented with hormonal disturbance and diagnosed as JGCT at stage FIGO I A, and followed up for 29 months without tumor recurrence. Microscopic examination showed diffused atypical follicular structures formed by granulosa cells. The granulosa cells contained round hyper-chromatic nuclei without nuclear grooves or Call-Exner body formation. The first case had severe cellular atypia and the mitotic count was more than 10/10 HPF. The second case had mild cellular atypia and the mitotic count was less than 5/ 10 HPF. Immunohistochemical study showed diffused positivity for vimentin (2/2) and dispersive positivity for CD99. The first case was positive for mucus carmine red stain. The second case was nodal staining for crinhibin and CK18. cytokeratin pan (CKpan), S-100 protein, protein gene product (PGP) 9.5, smooth muscle actin (SMA), epithelial membrane antigen (EMA), desmin, myogenin, vascular endothelial growth factor (VEGF), placental alkaline phosphatase (PLAP) , human chorionicgonagotropin( HCG) , alpha - fetoprotein ( AFP) , CD4 5 and Calretinin were all negative. Conclusion JGCT is a low-grade malignant tumor, but few cases are highly malignant. Early diagnose is the most important prognostic factor. JGCT should be differentiated from adult granulosa cell tumor, hypercalcemia small cell carcinoma and malignant melanoma.

  5. Resection pseudoarthrosis for pelvic malignant tumors around acetabular%髋臼周围恶性肿瘤切除后股骨头旷置术的临床应用

    Institute of Scientific and Technical Information of China (English)

    杨正明; 陶惠民; 叶招明; 李伟栩; 杨迪生

    2011-01-01

    目的 探讨髋臼周围恶性肿瘤切除后股骨头旷置术的临床应用.方法 1997年5月至2005年6月共有25例切除后采用股骨头旷置术的髋臼周围恶性肿瘤患者得到随访,其中男性15例,女性10例;年龄16~75岁,平均42岁;骨肉瘤4例,软骨肉瘤12例,尤文肉瘤1例,原始神经外胚层瘤1例,骨髓瘤1例,恶性纤维组织细胞瘤1例,滑膜肉瘤2例,转移性肿瘤3例.根据Enneking骨盆肿瘤分区:肿瘤累及Ⅰ、Ⅱ区12例;Ⅰ、Ⅱ、Ⅲ区5例;Ⅱ、Ⅲ区4例;Ⅱ区2例;Ⅰ、Ⅱ、Ⅳ区2例.共有7例行新辅助化疗,5例行单纯术后辅助化疗,4例行术后放疗.髋臼周围切除后,采用股骨头旷置术,术后皮牵引6~8周,牵引质量2~3 kg.以后扶拐逐渐步行活动.结果 25例股骨头旷置术中6例出现手术后并发症(24.0%).随访时间3~10年,11例由于肺转移或远处转移并发肺转移死亡;2例带瘤生存;12例无瘤生存.术后5年及10年生存率分别为60%和54%.股骨头旷置术后肢体短缩在2.5~7.5 cm,平均5 cm.本组病例术后功能评价根据Enneking1993MSTS评分系统进行,平均术后功能评分是17分(12~19分).术后3个月后,患者基本能够正常坐,扶单拐跛行,能自主上下楼梯,去拐后也能跛行行走.结论 累及髋臼周围的恶性肿瘤,切除后股骨头旷置术是一种可选择的重建方式,能取得相对满意的临床效果.髋臼周围高度恶性肿瘤、软组织重建条件不佳、具有感染高危倾向者、经济条件不佳的患者,是股骨头旷置术的适应证.%Objective To discuss the resection pseudoarthrosis for pelvic malignant tumors around acetabular. Methods From May 1997 to June 2005, 25 patients with malignant tumors around acetabular were treated surgically with resection pseudoarthrosis. The series comprised 15 males and 10 females with an average age of 42 years old (range from 16 to 75 years old ) . There were 4 osteosareomas, 12chondrosarcomas, 1 Ewing's sarcoma, 1

  6. 骨盆Ⅰ-Ⅱ-Ⅳ区肉瘤整块切除后腰盆钉棒重建系统%Enbloc resection of pelvic sarcoma involving region Ⅰ , Ⅱ, IV and reconstruction with the prosthetic acetabulum pedicle screw and rod system

    Institute of Scientific and Technical Information of China (English)

    沈靖南; 王晋; 尹军强; 黄纲; 雍碧城; 李浩淼; 邹昌业

    2012-01-01

    carried out in patients with pelvic sarcoma in region Ⅰ , Ⅱ , Ⅳ who were treated by en bloc resection and reconstruction with the prosthetic acetabulum pedicle screw and rod system in orthopaedics oncology department of the first affiliated hospital of Zhongshan University from June 2009 to December 2010. The treatment effect and postoperative complications were evaluated. Inclusion criteria were as follows: primary pelvic sarcoma; no metastasis was delected in chest CT scan; stage ⅡB according to Enneking classification; very likely to finish all courses of neo-adjuvant chemotherapy if required; external iliac vessels, sciatic nerve or pelvic viscera involvement was not detected by preoperative Mri; sarcoma was found involving at least three pelvic regions ( regions Ⅰ, Ⅱ, Ⅳ). Results Four patients with pelvic sarcoma involving region Ⅰ, Ⅱ, Ⅳ underwent en bloc resection and reconstruction with the prosthetic acetabulum pedicle screw and rod system. There were three males and one female whose average age was 29.75(from 18 to 45). Pathological classification included Ewing' s sarcoma (two cases), chondrosarcoma (one case), and osteosarcoma (one case). Patients with Ewing's sarcoma and osteosarcoma finished their neo-adjuvent chemotherapy. En bloc resection included tumor in pelvic region Ⅰ, Ⅱ, Ⅳ, the tumor-invloved iliacus muscle, gluteus medius muscle, gluteus minius muscle and part of the gluteus maximus muscle. After resection, acetahulum, pelvic ring and lumbosacro region were reconstructed with die prosthetic acetabulum pedicle screw and rod system. Rehabilitation began three weeks after operation. Three months later, patients could walk with the crutches. Average follow-up time was 14 months (range from nine to 19 months). No relapse or metastasis was found. MSTS functional evaluation score was 66.67% (from 63% to 73% ). Harris hip functional evaluation score was good in two cases and poor in two cases. Conclusions The goal of the treatment

  7. 累及腓骨的胫骨近端恶性肿瘤的保肢治疗%Limb salvage surgery for malignant tumors of the proximal tibia involving the fibula

    Institute of Scientific and Technical Information of China (English)

    汤小东; 郭卫; 杨荣利; 唐顺; 董森

    2012-01-01

    Objective To investigate technique,effect,complications of limb salvage surgery for malignant tumors of the proximal tibia involving the fibula.Methods Between November 1998 and February 2010,32 patients with malignant tumors of the proximal tibia involving the fibula underwent limb salvage surgery in our institute.There were 21 males and 11 females,aged from 10 to 66 years (average,23.4years).There were 23 cases of osteosarcoma,5 cases of chondrosarcoma,1 case of malignant giant cell tumor,and 3 cases of soft tissue sarcoma.All patients received en bloc resection of tumor including proximal tibia and fibula.During operations,ligation of anterior tibial vessels was performed in 14 patients,anastomosis of posterior tibial vessels in 1 patient,both ligation of anterior tibial vessels and anastomosis or replacement of posterior tibial vessels in 5 patients.The common peroneal nerve was resected in 4 patients,and the deep peroneal nerve was resected in 5 patients.The reconstruction methods included prosthetic replacement in 24 patients,replacement with inactivated autograft-prosthesis composite in 5 patients,and replacement with inactivated autograft in 3 patients.The caput mediale musculi gastrocnemii flap was transferred to reconstruct soft tissue defect in 14 patients,while caput laterale musculi gastrocnemii flap was transferred in 1patient.Results All patients were followed up for 11 to 159 months (average,39.4 months).Local recurrence occurred in 6 patients (18.8%).The overall 5-year survival rate was 51.2%.Fourteen patients died of tumor metastasis,2 survived with tumor,and 16 patients survived without tumor.Variant complications occurred in 15 patients (46.9%).The complications included ischemia of the lower leg in 4 patients,peroneal nerve palsy in 12 patients (permanent palsy in 9 patients and temporary palsy in 3 patients),wound healing problem in 4 patients,deep infection in 1 patient,and periprosthetic fracture in 1 patient.The mean MSTS 93 score was

  8. Anterior approach and hemisectomy of stern for upper thoracic disease%经前路部分切除胸骨上端入路手术治疗上胸椎病变

    Institute of Scientific and Technical Information of China (English)

    谢恩; 郝定均; 吴起; 张子如

    2013-01-01

    Objectives: To investigate the clinical outcome of anterior approach and hemisectomy of stern for upper thoracic disease. Methods: 17 patients suffering from upper thoracic lesions underwent anterior hemisectomy of stern from July 2005 to September 2010. There were 8 males and 9 females with the age ranging from 27 to 77 years, with an average age of 57.7 years. The pathogenesis included T3 aneurysmal bone cyst in two eases, T2-T3 tuberculosis in seven cases, T3-T4 tuberculosis in three cases, T2 bone giant cell tumor in two cases, T3 giant cell tumor in two cases and T2 chondrosarcoma in one case. Before operation, upper thoracic sagittal CT was analyzed with the aim to determine the scope of surgery. Upper sternum was resected and removed as half-moon. Both sides of the sternoclavicular ligament were retained. Through the window of the brachiocephalic artery, lesion was exposed. The debridement or excision, bone grafting or reconstruction of internal fixation were performed afterward. Intraoperative, postoperative and follow-up complications were recorded. Results: 17 patients were perfomed surgery successfully. All lesions were exposed satisfactorily. Three cases were complicated with bradycardia, low blood pressure or increase of airway resistance during operation, and all resolved quickly after the discharge of traction. 1 case was complicated with transient hoarseness, that was resolved 2 month later. The surgical time was 2-3h, and the amount of blood loss was 200-700ml, averaging 400ml. All patients were followed up for 17-27 months, with an average of 22.7 months. No fixation failure was noted, and all cases reached bony fusion. Physiological curvature of the cervical and thoracic segment recovered. 17 cases with preoperative neurological deficit recovered partially. Conclusions:The modified upper thoracic vertebral approach can effectively expose T2-T4, which can retain the sternoclav-icular joint and sternum, and is proved to be an ideal approach for

  9. The clinic application of microwave heliotherapy combined with prosthesis replacement in malignant bone tumor of limbs%微波原位灭活联合人工关节假体置换治疗四肢恶性骨肿瘤的疗效分析

    Institute of Scientific and Technical Information of China (English)

    纪经涛; 张洪; 胡永成; 夏群; 苗军

    2015-01-01

    average (ranging from 11 to 65 years).The tumors involved:primary malignant tumor 11 cases,metastases of the scapula 8 cases.Locations of tumors involved:the distal femur 8 cases,the proximal tibia 6 cases,the proximal humerus 3 cases and the proximal femur 2 cases.After the patients were examined with MRI and the level of osteotomy was determined,the prostheses were custom-made.The exposures of the all tumors were via the conventional surgical approach.The lesions were heated at 50 ℃ for 20 min by 2 450 MHz microwave,with surrounding soft tissue protected by copper mesh.The level of osteotomy was determined by the result of preoperative imaging measurement.The prosthesis was installed after the microwave heliotherapy.Results The duration of surgery was from 60 min to 150 min (100 min on average).The blood loss was from 300 ml to 1200ml (600 ml on average).All patients were followed-up for 10 months to 5 years (2.7 years on average).1 case with chondrosarcoma relapsed 18 months after surgery,and survived with tumors.1 case with malignant fibrous histiocytoma died due to multiple metastases 8 months after surgery.1 case with Ewing sarcoma died due to pulmonary metastases 23 months after surgery.The remaining 7 cases with the primary malignant bone tumors did not recur or transfer during the follow-up period.6 cases with the metastases died due to metastases 5 to 20 months after surgery.The functions of shoulder joint of 3 proximal humerus tumor patients were restricted,while 16 patient's function weren't restricted.Conclusion The clinical results demonstrated that the microwave heliotherapy combined with prosthesis replacement was an ideal treatment for the intramedullary broad dissemination of the malignant bone tumor of limbs.

  10. The clinical application of femoral head exclusion after resection of pelvic tumors around acetabulum%髋臼周围肿瘤切除股骨头旷置术

    Institute of Scientific and Technical Information of China (English)

    黄洪超; 胡永成; 伦登兴; 马军; 夏群; 纪经涛; 苏秉成

    2011-01-01

    目的 探讨股骨头旷置术治疗髋臼周围肿瘤的可行性.方法 1999年10月至2009年8月采用髋臼肿瘤切除股骨头旷置术治疗累及髋臼的Enneking Ⅱ区肿瘤患者18例,男16例,女2例;年龄18~72岁,平均54岁.软骨肉瘤4例,骨肉瘤2例,骨巨细胞瘤4例,滑膜肉瘤1例,恶性纤维组织细胞瘤1例,Ewing肉瘤1例,转移瘤5例.3例行术前选择性动脉栓塞,4例行麻醉后腹主动脉或髂总动脉球囊临时阻断.术后门诊随访观察关节功能及影像学变化.结果 全部病例随访3~118个月,平均55个月.手术时间120~350min,平均170min.出血量600~2200ml,平均1200ml.切口均愈合,无一例发生伤口感染.1例骨肉瘤、3例转移癌患者于术后6个月至2年死于多脏器转移,其余病例无肿瘤局部复发.肢体不等长2.0~7.5 cm,平均5.0 cm,所有患者均需足跟垫高2~3 cm的矫形鞋进行矫正.术后3个月Enneking评分20~27分,平均23分.影像学资料提示,至随访期末无股骨头坏死发生,8例假臼磨造较好,1例发生轻度腰椎侧凸.结论 对于累及髋臼的Enneking Ⅱ区肿瘤采用肿瘤切除股骨头矿置术术后并发症发生率低,近期功能良好,肿瘤复发率低.%Objective To study clinical application of femoral head exclusion after resection of pelvic tumors around acetabulum with less limb damage and complications.Methods From October 1999 to August 2009,18 patients with pelvic tumors around acetabalum (zone Ⅱ of Ennekingl were treated with tumor resection and femoral head exclusion,including 16 males and 2 females with an average age of 54 years(range,18-72).The diagnosis were chondrosarcoma(4 cases),osteosarcoma(2),giant cell tumor of bone(4),synoviosareoma(1),malignant fibrous histiocytoma(1),Ewing sarcoma(1),and metastatic carcinoma(5).Throe cases were treated by selective arterial embolization before operation and 4 cases by temporary block of lower abdominal aorta or common iliac arte.Regular follow-up in

  11. A comparison of planned and ifnal resection lengths in limb salvage surgery for bone tumors%瘤段截除计划长度与最终截除长度的差异研究

    Institute of Scientific and Technical Information of China (English)

    邓志平; 牛晓辉; 张清; 郝林; 丁易

    2014-01-01

    Objective The resection length should be designed before the limb salvage surgery for bone tumors. To compare the differences of planned and final resection lengths in limb salvage surgery for bone tumors. Methods Fifty-three cases receiving limb salvage surgery were enrolled in the study, including 34 cases of osteosarcomas, 5 cases of giant cell tumors, 5 cases of spindle cell sarcomas, 3 cases of chondrosarcomas, 3 cases of bone metastases, 1 case of lymphoma, 1 case of undifferentiated pleomorphic sarcoma, and 1 case of soft tissue sarcoma with the bone involvement. The tumors were located in the distal femur ( n=29 ), in the proximal tibia ( n=8 ), in the proximal humerus ( n=7 ), in the proximal femur ( n=5 ), in the distal tibia ( n=3 ) and in the distal humerus ( n=1 ). The planned and ifnal resection lengths were compared. The patients were classiifed according to the tumor location, and the differences were compared. Results The range of the length error was from-30 mm to 15 mm in the 53 cases. 95%conifdence interval ( CI ) was ( 2.0±7.7 ) mm. The length error was less than 10 mm in 40 cases ( 75.5%), which was more than 10 mm in 9 cases ( 17%), and only in 4 cases ( 7.5%) was less than-10 mm. The length error was ( 2.9±6.7 ) mm in 29 cases in the distal femur, ( 3.8±5.1 ) mm in 8 cases in the proximal tibia, ( 3.6±8.0 ) mm in 7 cases in the proximal humerus, (-0.2±6.0 ) mm in 5 cases in the proximal femur, 0 mm in 3 cases in the distal tibia and-30 mm in 1 case in the distal humerus. Conclusions The anatomical landmark and resection length are planned before the surgery. The accuracy of resection length is acceptable in this method. More precise resection could be achieved after avoiding the error-making factors.%目的:探讨骨肿瘤行瘤段截除时计划截除长度与实际截除长度的差异。方法对53例行瘤段截除的患者进行研究,骨肉瘤34例,骨巨细胞瘤5例,梭形细胞肉瘤5例,软骨肉瘤3

  12. 肿瘤假体置换在肱骨近端恶性骨肿瘤治疗中的应用%Tumor prosthesis replacement in proximal humeral application in the treatment of malignant bone tumor

    Institute of Scientific and Technical Information of China (English)

    王永贵; 刘江涛

    2014-01-01

    Objective To investigate the clinical results of tumor prosthesis replacement in the treatment of malignant bone tumors of the proximal humerus.Methods Clinical data of 32 patients with malignant bone tumors of the proximal humerus who were treated from July 2000 to January 2010 were retrospectively analyzed. There were 10 cases of osteosarcoma, 6 cases of Ewing’s sarcoma, 7 cases of giant cell tumors, 5 cases of metastatic carcinoma and 4 cases of chondrosarcoma. In functional reconstruction of the shoulder joint, the custom artificial tumor prosthesis of the shoulder joint was used. According to Enneking’s functional criteria after the muscular-skeletal tumor surgery, the clinical results were evaluated.Results All the patients were followed up for 4.2 years on average ( range: 3.1-5.3 years ) after the operation. Among them, death occurred in 2 cases, postoperative recurrence in 2 cases, chronic periprosthetic infection in 1 case and shoulder subluxation in 2 cases. The elbow, wrist and hand functions in all the patients were normal. The shoulder abduction was 75° on average ( range: 60°-90° ), the lfexion 45° on average ( range: 35°-55° ), the extension 50° on average ( range: 40°-60° ) and the adduction 17.5° on average ( range: 15°-20° ). According to Enneking’s functional criteria after the muscular-skeletal tumor surgery, the minimum score was 11 points in 2 cases and the maximum score was 28 points in 2 cases. There were 14 cases whose score ranged from 24 points to 30 points, 9 cases from 18 points to 23 points, 7 cases from 12 points to 17 points and 2 cases less than 12 points. The excellent and good rate was 71.9%.Conclusions Artiifcial shoulder joint replacement is an effective method in the treatment of malignant bone tumors of the proximal humerus, because not only the entire appearance of the affected extremity will be kept, but also the function of the upper extremity can be preserved to a great extent.%目的:探讨肿瘤假

  13. Survival and complications in total knee replacement with custom-prosthesis after bone tumors resection%定制肿瘤型铰链膝关节假体置换术后假体生存率及并发症分析

    Institute of Scientific and Technical Information of China (English)

    杨志平; 田永昊; 杨强; 李昕; 李振峰; 李建民

    2011-01-01

    目的 探讨定制肿瘤型膝关节假体置换术后的假体生存率及并发症发生率.方法 对1996年4月至2007年4月接受定制肿瘤型铰链膝关节假体置换且随访资料完整的85例进行回顾性分析,男54例,女31例;年龄11~72岁,平均(31.33±15.3)岁.骨肉瘤43例,骨巨细胞瘤31例,软骨肉瘤4例,转移瘤3例,恶性纤维组织细胞瘤2例,Ewing肉瘤1例,纤维肉瘤1例.股骨远端43例,胫骨近端42例.均行瘤段广泛切除及国产骨水泥定制肿瘤型铰链膝关节假体重建.结果 随访3~124个月,平均45.8个月.并发症发生率27.1%(23/85),股骨远端25.6%(11/43),胫骨近端28.6%(12/42);发生于术后3年内13例次,3~5年3例次,5年以上7例次.与手术相关的并发症(切口不愈合、假体周围深部感染、腓总神经损伤等)6例次,全部发生于术后3年内;与假体相关的并发症(假体松动及断裂、假体关节脱位、假体周围骨折等)17例次,发生于术后3年以内7例次、3~5年3例次、5年以上7例次.假体生存率三年79.4%、五年67.7%、七年62.3%,股骨假体分别为86.6%、79.2%、73.5%,胫骨假体分别为66.1%、53.7%、48.8%.结论 与手术相关的并发症多发生于术后3年内,与假体相关的并发症于术后3年内及5年以上高发.使用国产定制肿瘤型膝关节假体置换术后早期即可发生与假体相关的并发症.%Objective To retrospectively investigate the endoprosthetic survival and complications after custom-made tumor prosthesis replacement of knee joint.Methods From April 1996 to April 2007,85 patients with bone tumors around knee joints undergoing custom-prosthetic replacement were respectively analyzed,including 54 males and 31 females with an average of 31.33±15.3 years(range,11-72).The diagnoses were osteosarcoma(43 patients),giant cell tumor(31),chondrosarcoma(4),metastatic tumor(3),malignant fibrohistiocytoma(2),Ewing sarcoma(1),and fibrosarcoma(1).The distal femar was affected in 43

  14. Biomechanical properties of a titanium alloy pelvic prosthesis individually manufactured by 3D printing: a finite element analysis%3D打印钛合金个性化骨盆假体生物力学的初步有限元分析

    Institute of Scientific and Technical Information of China (English)

    叶堃; 王金武; 胡志刚; 王成焘; 干耀凯; 韦建和; 江川; 邓源; 李雨

    2015-01-01

    Objective To evaluate the biomechanical properties of a titanium alloy pelvic prosthesis individually manufactured by 3D printing through finite element analysis.Methods A male patient with a huge chondrosarcoma at the right ilium was recruited for the present study who had been arranged for hemipelvectomy and artificial hemi-pelvic replacement.After the patient underwent CT and MRI examinations before operation,scope of tumor invasion around the ilium,surgical margins and plane for osteotomy were determined using the 3D image fusion technique.A finite element model of the pelvis of the patient was established on the basis of the defective area after pelvic osteotomy using computer aided design (CAD).After the finite element analysis,a customized titanium alloy pelvic prosthesis was manufactured using 3D printing technology.The software Abaqus was used to conduct finite element analysis of the model of the pelvic prosthesis manufactured by 3D printing.The von Mises stress,relative displacement and stress concentration point in the finite element model of the pelvis were measured and analyzed.Results The maximum von Mises stress in the titanium alloy pelvic prosthesis manufactured by 3D printing was 25.29 MPa,far smaller than the yield strength of titanium alloy (950 MPa).The stress concentration area was near the nail holes where the prosthesis and the sacrum were connected.The patient was able to walk without crutches 3 months post-surgery.After half a year,the implant was stable and the patient could perform normal activities.Conclusions The titanium alloy pelvic prosthesis individually manufactured by 3D printing based on the results of finite element analysis met the biomechanical requirements of a pelvis.The calculation results of finite element analysis were consistent with the postoperative follow-up outcomes of the patient.This method can provide biomechanical evidence for clinical application of 3D printing implants in orthopedics.%目的 通过

  15. Types and evaluation of reconstruction approach for malignant pelvic tumors around acetabulum%髋臼及其周围肿瘤的分区与重建方法

    Institute of Scientific and Technical Information of China (English)

    郭卫; 燕太强; 汤小东; 杨毅

    2009-01-01

    目的 回顾性分析髋臼及其周围恶性肿瘤行整块切除、不同方法髋臼重建的疗效.方法 72例髋臼及其周围恶性肿瘤患者接受了肿瘤整块切除、髋臼重建手术,男42例,女30例;年龄16~78岁,平均41岁.软骨肉瘤39例、骨肉瘤10例、骨巨细胞瘤9例、Ewing肉瘤5例、恶性纤维组织细胞瘤3例、恶性神经鞘瘤2例、血管外皮瘤l例、单发转移癌3例.肿瘤累及Ⅱ区、Ⅰ+Ⅱ区、Ⅱ+Ⅲ区、Ⅰ~Ⅲ区、Ⅰ~Ⅳ区和Ⅱ+Ⅲ+Ⅴ区者分别为4、16、29、7、10和6例.髋臼重建包括组配式人工半骨盆50例、马鞍式关节置换7例、骨盆灭活再植8例、股骨近端与盆骨融合7例.结果 61例获得随访,平均随访3.5年(1~8年).11例局部复发,8例深部感染,6例脱位.45例行组配式人工半骨盆置换术患者ISOLS评分平均22分.优7例、良24例、可9例、差5例.其中优良病例均为累及Ⅱ区和Ⅱ+Ⅲ区者,评分差的病例均为累及Ⅳ区者.5例行马鞍式关节置换、5例骨盆灭活再植和6例股骨近端与盆骨融合术患者,ISOLS评分平均为10、17和14分.结论 髋臼周围肿瘤切除后重建方法以组配式人工半骨盆置换术后功能最好,骨盆灭活再植次之,马鞍式关节置换术后功能最差.组配式人工半骨盆置换对单纯Ⅱ区肿瘤切除重建功能最好,其次为Ⅱ+Ⅲ区、Ⅰ+Ⅱ区、Ⅰ~Ⅲ区、Ⅱ+Ⅲ+Ⅴ区,术后功能最差为Ⅰ~Ⅳ区.%Objective To evaluate the functional outcome of malignant periacetabular tumors treated by en bloc resection and reconstructed with different limb-salvage procedures. Methods Seventy-two pa-tients with tumors around acetabular were treated surgically in People's Hospital of Peking University be-tween July 2000 and July 2007, including 42 males and 30 females with an average age of 41 years (range,16-78). 39 patients were diagnosed with chondrosarcoma, and 10 with osteosarcoma, 9 with giant cell tumorand 5 with Ewing sarcoma

  16. An analysis of therapeutic effects of one-stage posterior total en bloc spondylectomy for primary and metastatic tumors of the thoracolumbar spine%后路I期全脊椎整块切除治疗胸腰椎原发和转移性肿瘤的疗效分析

    Institute of Scientific and Technical Information of China (English)

    栗向东; 王臻; 郭征; 李靖; 范宏斌; 付军; 陈国景; 石磊

    2014-01-01

    Objective To evaluate the clinical outcomes of one-stage posterior total en bloc spondylectomy ( TES ) in the treatment of primary and metastatic tumors of the thoracolumbar spine. Methods From January 2007 to July 2012, 21 patients with primary and metastatic tumors of the thoracolumbar spine underwent TES, whose clinical data were retrospectively analyzed. There were 11 males and 10 females, whose average age was 47.8 years old ( range;21-66 years ). There were 8 cases of primary tumors and 13 cases of solitary metastatic tumors. Among the 8 cases of primary tumors, there were 3 cases of giant cell tumors of bone, 2 cases of plasmacytoma, 1 case of osteosarcoma, 1 case of chondrosarcoma and 1 case of epithelioid hemangioendothelioma. Among the 13 cases of metastatic tumors, there were 4 cases of breast cancer, 3 cases of lung cancer, 2 cases of renal cancer, 1 case of thyroid cancer, 1 case of prostate cancer, 1 case of bladder cancer and 1 case of unknown adenocarcinoma. The tumors were located from T3 to L3, including the thoracic vertebra ( n=12 ) and the lumbar vertebra ( n=9 ). The tumors were detected in the single vertebral body in 19 cases and multiple vertebral bodies ( 3 adjacent vertebral bodies ) in 2 cases. According to Tomita surgical classiifcation system, there were 5 cases of type I, 10 cases of type II, 1 case of type III, 2 cases of type V and 3 cases of type VI. All the patients had intractable back pain or neurologic disturbances before the operation. Vertebral bone defects were repaired, and internal ifxation and reconstruction were performed with titanium mesh, allograft or autograft and pedical screws. The operation was completed in all patients through the posterior approach at the ifrst stage. Results The mean operation time was 6.4 hr ( range;4.0-8.5 hr ). The average amount of blood loss was 4500 ml ( range;1300-11 600 ml ). Perioperative complications occurred in 2 cases, including 1 case of pleural effusion and pulmonary infection

  17. Wide resection and knee joint reconstruction for malignant tumors of the proximal tibia%胫骨上端恶性骨肿瘤广泛切除与重建

    Institute of Scientific and Technical Information of China (English)

    杨志平; 杨强; 李昕; 李振峰; 李建民

    2011-01-01

    bone tumors of proximal tibia,27 males and 18 females,28 years in average (range, 12 -62 years). The tumors,including 25 osteosarcomas,7 malignant giant cell tumors,6 chondrosarcomas,3 malignant fibrous histiocytomas,3 fibrosarcomas and 1 lymphoma were treated with wide resection which performed through anteromedial incision in 30 cases and anterolateral incision in 15. The superior tibiofibuiar joints were involved and the proximal tibia resected en bloc in 13. Two knee joints were involved and extraarticuler resection were performed. Partial tumorous bone was devitalized and replanted in 5 cases and the anterior tibial vessels were ligated in 28. The involved knee joints were reconstructed with domestic-made prostheses and soft tissue coverage was improved by medial or lateral gastrocnemius flap and sagittal split anterior tibial muscle flap in some patients which tibia was removed longer. Neoadjuvant chemotherapy were performed to the patients with osteosarcoma and malignant fibrous histiocytoma. [ Result] With an average follow-up of 4.6 years ( range,8 months -9 years ),local recurrences occurred in four cases and lung metastases in six cases. One patients with malignant fibrous histiocytoma of proximal tibia had metastasis to L* after 6 years postoperatively and was treated with total en bloc spondylectomy. The complications related to the prostheses included peri-prosthetic infection in 3, prosthetic dis-location in 3, prosthesis loosening in 2, peri-prosthesis fracturein 1 and prosthetic fracture in 1. The average range of motion were 92 degrees (50 - 120 degrees) with the 4.4 degrees (0 - 20 degrees) of average extension lag. According to the Musculoskeletal Tumor Society ( MSTS ) Scoring system, the average functional score was 76.7%. [ Conclusion ] Wide resection and reconstruction for malignant tumor of proximal tib-la I- highly demanding. To achieve the goal of Limb salvage and _■■?." function, il is necessary to employ standard reseolion lech

  18. Surgical management of malignant pelvic tumors involving the sacrum%累及骶骨的骨盆恶性肿瘤的外科治疗

    Institute of Scientific and Technical Information of China (English)

    杨荣利; 郭卫; 李大森; 汤小东; 燕太强; 杨毅; 臧杰

    2013-01-01

    Objective To summarize the cases of malignant pelvic tumors involving the sacrum with surgical management, to find out the reasonable surgical resection and reconstruction strategies, and to evaluate the outcomes of different surgical methods. Methods The data of 68 patients with malignant pelvic tumors involving the sacrum who were treated with tumor resection and reconstruction from July 1999 to October 2011 were reviewed. A vertebral pedicle screw-rod fixation system was used in 39 patients who had region I+IV resection, and autogenous bone graft of the fibula and ilium was used in 10 cases. Region I+II+IV resection occurred in 22 cases, including 7 cases of hemipelvic amputation, 7 cases of screw-rod fixation combined with hemipelvic endoprosthesis reconstruction and 8 cases of femoral head autograft. Region I+II+III+IV resection occurred in 7 cases, including 1 case of hemipelvic amputation, 4 cases of screw-rod fixation combined with hemipelvic endoprosthesis reconstruction and 2 cases of femoral head autograft combined with hemipelvic endoprosthesis reconstruction. Results No patient died perioperatively. 13 of the 60 patients who underwent limb salvage reconstruction had wound complications, and loosening or breaking in the internal fixation or hip dislocation occurred in 6 patients. Marginal or wide resection of the border was obtained in 3 of the 8 patients who underwent hemipelvic amputation, and only 1 patient with chondrosarcoma was alive without evidence of disease recurrence. Marginal or wide resection of the border was obtained in 27 of the 60 patients who underwent limb salvage procedures, and local recurrence occurred in 6 cases. The mean postoperative International Society of Limb Salvage ( ISOLS ) functional score was 93%in 39 patients of the region I+IV resection group, which was 63%in 21 patients of the artificial hemipelvic endoprosthesis group. There was no statistically significant difference in the ISOLS scores after screw-rod fixation

  19. Total sacrectomy via posterior approach for malignant sacral tumors%单纯后路全骶骨切除治疗骶骨恶性肿瘤

    Institute of Scientific and Technical Information of China (English)

    孙伟; 陈泉池; 马小军; 华莹奇; 蔡郑东

    2014-01-01

    high level (S1,S2).Methods 5 cases of primary malignant sacral tumors treated by total sacrectomy via posterior approach and iliolumbar reconstruction from March 2010 to March 2011 were analyzed retrospectively.There were 3 males and 2 females.The mean age was 41,ranging from 32 to 55.The imageology examination showed osteolytic destruction,among which there were 4 cases of obvious soft tissue mass,1 case of obvious sacral foramina expansion with bone damage.MRI showed 1 case of the tumors in S1-S5,1 case of the tumors in S1 、S2,1 case of the tumors in S1-S3.The smallest tumor was 9.2 cm×7.6 cm×4.1 cm,while the largest was 22.0 cm× 19.0 cm× 16.0 cm.Preoperative TTNB were done on the 5 patients.Pathological diagnosis:2 cases of chordoma,1 case of malignant neurilemmoma,1 case of chondrosarcoma and 1 case of malignant hemangioendothelioma.5 patients had sacrococcygeal pain or lumbocrural pain before the surgery.Visual analogue scale (VAS):2 cases of 2,2 cases of 6 and 1 case of 8.Ilium stability reconstructions were all performed on 5 patients with spine pedicle screw-rod system.Results The mean operation time was 6.5 hours (range,4.5-11 hours),with the mean intraoperative blood loss of 3 700 ml (range,2 000-7 200 ml).There was no perioperative death.The mean follow-up time was 17 months (range,9-23 months).There were 2 cases of wound complications 2 weeks after surgery and healed by second intention with washing and drainage after debridement.There was no deep infection.1 case of rectal injury,which was performed with colostomy during the operation,and stoma returned 12 weeks after surgery.4 cases of sciatic nerve symptom of lower limbs and plantar flexion dyskinesia after bilateral S1 nerve roots resection.The patients walked with ankle brace fixed after the surgery.There was 1 case of implant breakage and no obvious spine down.5 patients had functional disability in sphincter after surgery.1 case of hemangioendothelioma recurred locally 9 months later