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Sample records for chondrosarcomas

  1. Testicular chondrosarcoma

    Directory of Open Access Journals (Sweden)

    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. Sternal chondrosarcoma

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    Nelson Perelman Rosenberg

    2003-01-01

    Full Text Available Sternal neoplasms are extremely rare. It is difficult to make prospective evaluations due to the lack of consistent reports in the literature. The authors report the case of a woman in her seventies, who presented a chondrosarcoma of the sternum, treated by them.

  3. Chondrosarcoma of the hyoid bone

    Energy Technology Data Exchange (ETDEWEB)

    Demeyere, A.; Somer, F. de; Perdieus, D.; Lemmens, L.; Schillebeeckx, J. [Dept. of Radiology, Imeldaziekenhuis Bonheiden (Belgium); Hauwe, L. van den [Dept. of Radiology, University Hospital Antwerp (Belgium)

    2000-02-01

    The CT and MRI findings in a case of chondrosarcoma of the hyoid bone are reported. Although chondrosarcoma is the second most common primary malignant bone tumor, only 10 % of chondrosarcomas occur in the head and neck region. The hyoid bone is a rare site of involvement with only seven cases reported previously. (orig.)

  4. OCULAR CHONDROSARCOMAS IN RIVULUS MARMORATUS POEY

    Science.gov (United States)

    Only one documented case of a malignant cartilaginous tumor has been previously reported from fish, a large cranial chondrosarcoma from the paddlefish, Polyodon spathula (Walbaum). escribed here are two chondrosarcomas diagnosed from adult laboratory-reared Rivulus marmoratus Poe...

  5. Two rare cases of craniofacial chondrosarcoma

    OpenAIRE

    2012-01-01

    The craniofacial region is a rare site for chondrosarcomas. These tumors may have osseous or extraosseous origin. Extraosseous chondrosarcomas have the same histological features as osseous chondrosarcomas. Chondrosarcomas usually present in the fifth to seventh decades of life, although several cases with younger age at presentation have been reported. They usually present as a painless mass that gradually progresses to various complaints, such visual impairment, nasal obstruction, and denta...

  6. Central nervous system mesenchymal chondrosarcoma

    International Nuclear Information System (INIS)

    Central nervous system mesenchymal chondrosarcomas are rare malignant tumors that constitute a separate entity from the classical chondrosarcoma and myxoid variant. Clinical behaviour of central nervous system chondrosarcomas is still unknown. We describe two rare examples of intracranial mesenchymal chondrosarcoma with a review of the literature, in an attempt to clarify the clinical characteristics, prognosis and treatment of choice of these unusual tumors. Among the 55 reported cases, 23 had postoperative radiotherapy. Although there is no statistical significance according to the Log-Rank test (p=0.7), the patients treated with radiation therapy seem to have a better chance of survival. Patients who had adjuvant chemotherapy (only 5) showed survival times similar to those patients who had none. Although clinical behaviour of central nervous system chondrosarcomas remains to be defined, data from our series as well as literature show that radical removal is the best therapeutic choice. In addition, patients treated with postoperative radiotherapy seem to show a trend toward increased survival

  7. 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%.

  8. Retrobulbar chondrosarcoma in a dog

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    M. Ralić

    2014-04-01

    Full Text Available This paper presents a review of a dog, with a retrobulbar chondrosarcoma, which was admitted for surgery for visible changes in his eye during inspection. Orbital neoplasia in dogs may be primary and secondary. Sixty percent of orbital neoplasia in dogs are primary, ninety percent of which are malignant. Retrobulbar neoplasms are rare and in their early stage represent a diagnostic challenge. Chondrosarcoma of the skull is a slow-progressing malignant disease which occurs locally, aggressive with invasion into the surrounding tissues. Dogs with chondrosarcoma of the skull have life expectancy between 210 and 580 days - in our case it was 180 days - after the first alterations on the eye of the dog occurred.

  9. Three new chondrosarcoma cell lines: one grade III conventional central chondrosarcoma and two dedifferentiated chondrosarcomas of bone

    International Nuclear Information System (INIS)

    Chondrosarcoma is the second most common primary sarcoma of bone. High-grade conventional chondrosarcoma and dedifferentiated chondrosarcoma have a poor outcome. In pre-clinical research aiming at the identification of novel treatment targets, the need for representative cell lines and model systems is high, but availability is scarce. We developed and characterized three cell lines, derived from conventional grade III chondrosarcoma (L835), and dedifferentiated chondrosarcoma (L2975 and L3252) of bone. Proliferation and migration were studied and we used COBRA-FISH and array-CGH for karyotyping and genotyping. Immunohistochemistry for p16 and p53 was performed as well as TP53 and IDH mutation analysis. Cells were injected into nude mice to establish their tumorigenic potential. We show that the three cell lines have distinct migrative properties, L2975 had the highest migration rate and showed tumorigenic potential in mice. All cell lines showed chromosomal rearrangements with complex karyotypes and genotypic aberrations were conserved throughout late passaging of the cell lines. All cell lines showed loss of CDKN2A, while TP53 was wild type for exons 5–8. L835 has an IDH1 R132C mutation, L2975 an IDH2 R172W mutation and L3252 is IDH wild type. Based on the stable culturing properties of these cell lines and their genotypic profile resembling the original tumors, these cell lines should provide useful functional models to further characterize chondrosarcoma and to evaluate new treatment strategies

  10. Chondrosarcoma

    Science.gov (United States)

    ... Tumors Oligoastrocytoma Oligodendroglioma Pineal Tumor Pituitary Tumor PNET Schwannoma Risk Factors Brain Tumor Facts Brain Tumor Dictionary ... Tumors Oligoastrocytoma Oligodendroglioma Pineal Tumor Pituitary Tumor PNET Schwannoma Risk Factors Brain Tumor Facts Brain Tumor Dictionary ...

  11. 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.

  12. Chondrosarcoma of small bones of the hand.

    Science.gov (United States)

    Patil, S; de Silva, M V C; Crossan, J; Reid, R

    2003-12-01

    A clinicopathological review of 23 patients (mean age, 67 years; range, 42-85 years) with chondrosarcoma of the bones of the hand was done. The mean follow up was 8.5 years. Eleven patients presented with a progressive painless swelling, 26% having had symptoms for over 10 years. The proximal phalanx was the commonest site. Initial clinical misdiagnosis as ganglion, bursa, gout, rheumatoid arthritis and a cyst occurred in five patients. Radiologically most lesions showed bone expansion, cortical destruction and soft-tissue extension. The majority was of high histologic grade (Evan's grade 2 & 3) with extensive myxoid areas. Five out of eight patients who were originally treated by curettage or excision had local recurrences compared to none treated by ray resection or amputation of phalanx (P=0.002). None had metastases. The low risk of metastases despite the high histologic grade indicates that chondrosarcomas of the hand behave differently from chondrosarcomas elsewhere. PMID:14599838

  13. Chondrosarcoma of the bones of the feet.

    Science.gov (United States)

    Patil, Sanjeev; de Silva, M V Chandu; Crossan, Jim; Reid, Robin

    2003-01-01

    Twelve chondrosarcomas of the bones of the feet from 11 patients in the Scottish Bone Tumor Registry were reviewed. One patient with diaphyseal aclasis (osteochondromatosis) developed 2 chondrosarcomas. The mean age of patients was 52.3 years (range, 17 to 83 years). Men were predominantly affected. Four tumors affected the tarsal bones; the rest involved the short tubular bones. The usual clinical presentation was a painful, progressively enlarging swelling. Radiologically, most showed some bone expansion, cortical destruction with indistinct margins, and soft-tissue extension. Histologically, the majority were middle-grade tumors. Treatment included curettage or local excision for 4 tumors and amputation or ray resection for 8 tumors. Follow-up varied from 6 months to 18 years (average, 5.8 years). Local recurrence after surgery was seen in 3 patients. All 3 died because of metastases to the lungs or brain. PMID:14566721

  14. The antiapoptotic gene survivin is highly expressed in human chondrosarcoma and promotes drug resistance in chondrosarcoma cells in vitro

    International Nuclear Information System (INIS)

    Chondrosarcoma is virtually resistant to chemotherapy and radiation therapy. Survivin, the smallest member of the inhibitor of apoptosis protein family, is a critical factor for tumor progression and resistance to conventional therapeutic approaches in a wide range of malignancies. However, the role of survivin in chondrosarcoma has not been well studied. We examined the importance of survivin gene expression in chondrosarcoma and analysed its influences on proliferation, apoptosis and resistance to chemotherapy in vitro. Resected chondrosarcoma specimens from which paraffin-embedded tissues could be extracted were available from 12 patients. In vitro experiments were performed in human chondrosarcoma cell lines SW1353 and Hs819.T. Immunohistochemistry, immunoblot, quantitative PCR, RNA interference, gene-overexpression and analyses of cell proliferation and apoptosis were performed. Expression of survivin protein was detected in all chondrosarcoma specimens analyzed, while undetectable in adult human cartilage. RNA interference targeting survivin resulted in a G2/M-arrest of the cell cycle and led to increased rates of apoptosis in chondrosarcoma cells in vitro. Overexpression of survivin resulted in pronounced resistance to doxorubicin treatment. These findings indicate that survivin plays a role in the pathogenesis and pronounced chemoresistance of high grade chondrosarcoma. Survivin antagonizing therapeutic strategies may lead to new treatment options in unresectable and metastasized chondrosarcoma

  15. Chondrosarcoma of the temporal bone: a case report

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    Park, Man Soo; Lee, Sang Youl; Chung, Jae Gul; Lee, Deok Hee; Jung, Seung Mun; Ryu, Dae Sik [Kang Nung Hospital, Ulsan Univ. Kangnung (Korea, Republic of)

    2001-07-01

    Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with chordoma, glomus jugulare tumor and meningioma, among other conditions, and due to its anatomic location, cranial nerve palsy is frequently observed. We report a case involving a 50-year-old woman with chondrosarcoma of the temporal bone.

  16. Rapid malignant transformation of primary synovial chondromatosis into chondrosarcoma.

    Science.gov (United States)

    Jonckheere, J; Shahabpour, M; Willekens, I; Pouliart, N; Dezillie, M; Vanhoenacker, F; De Mey, J

    2014-01-01

    Chondrosarcoma of the synovium is rare. It may arise de novo from the synovium or pre-existing synovial chondro- matosis may undergo malignant transformation into chondrosarcoma. Diagnosing a malignant transformation of the synovium remains a big challenge. It is based on the correlation of clinical findings, imaging and histology, as illustrated in this case report. PMID:25597214

  17. The Identification of Prognostic Factors and Survival Statistics of Conventional Central Chondrosarcoma

    OpenAIRE

    Nota, Sjoerd P. F. T.; Yvonne Braun; Schwab, Joseph H.; C. Niek van Dijk; Jos A. M. Bramer

    2015-01-01

    Introduction. Chondrosarcomas are malignant bone tumors that are characterized by the production of chondroid tissue. Since radiation therapy and chemotherapy have limited effect on chondrosarcoma, treatment of most patients depends on surgical resection. We conducted this study to identify independent predictive factors and survival characteristics for conventional central chondrosarcoma and dedifferentiated central chondrosarcoma. Methods. A systematic literature review was performed in Sep...

  18. Carbon ion radiotherapy of skull base chondrosarcomas

    International Nuclear Information System (INIS)

    Purpose: To evaluate the effectiveness and toxicity of carbon ion radiotherapy in chondrosarcomas of the skull base. Patients and Methods: Between November 1998 and September 2005, 54 patients with low-grade and intermediate-grade chondrosarcomas of the skull base have been treated with carbon ion radiation therapy (RT) using the raster scan technique at the Gesellschaft fuer Schwerionenforschung in Darmstadt, Germany. All patients had gross residual tumors after surgery. Median total dose was 60 CGE (weekly fractionation 7 x 3.0 CGE). All patients were followed prospectively in regular intervals after treatment. Local control and overall survival rates were calculated using the Kaplan-Meier method. Toxicity was assessed according to the Common Terminology Criteria (CTCAE v.3.0) and Radiation Therapy Oncology Group (RTOG)/European Organization for Research and Treatment of Cancer (EORTC) score. Results: Median follow-up was 33 months (range, 3-84 months). Only 2 patients developed local recurrences. The actuarial local control rates were 96.2% and 89.8% at 3 and 4 years; overall survival was 98.2%at 5 years. Only 1 patient developed a mucositis CTCAE Grade 3; the remaining patients did not develop any acute toxicities >CTCAE Grade 2. Five patients developed minor late toxicities (RTOG/EORTC Grades 1-2), including bilateral cataract (n = 1), sensory hearing loss (n = 1), a reduction of growth hormone (n = 1), and asymptomatic radiation-induced white matter changes of the adjacent temporal lobe (n = 2). Grade 3 late toxicity occurred in 1 patient (1.9%) only. Conclusions: Carbon ion RT is an effective treatment for low- and intermediate-grade chondrosarcomas of the skull base offering high local control rates with low toxicity

  19. Radiological features of 24 periosteal chondrosarcomas

    International Nuclear Information System (INIS)

    Objective. To report the imaging findings of 24 periosteal chondrosarcomas diagnosed, staged, treated and followed in a single institution, to analyze and define their pattern, and discuss their practical consequences.Design and patients. Plain films, 16 CT examinations and four MRI examinations were reviewed, and compared with the histological evaluation.Results. There were 20 men and four women, aged from 17 to 65 years. Twelve lesions involved the distal femoral metaphyses (8 posteriorly), five the proximal humerus, two the proximal metaphyses of the femur and two of the tibia, two the humeral shafts and one the iliac wing. Size varied from 4 to 11 cm. The cortex was always involved (thick, 15; thin, 13). Typical cartilaginous calcifications and cartilaginous lobules were very frequent. Radial thick periosteal bone formations (n=6) indicated calcifications between the lobules of cartilage. Medullary involvement was rare (n=2). All patients are alive and free of disease.Conclusions. Recognizing periosteal chondrosarcoma is of paramount importance because the prognosis is excellent after adequate local surgery alone. The patterns of other surface tumors of bone are usually different. (orig.)

  20. Radiological features of 24 periosteal chondrosarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Vanel, D. [Lab. di Ricerca Oncologica, Istituto Rizzoli, Bologna (Italy); Service de Radiodiagnostic, Institut Gustave Roussy, Villejuif (France); De Paolis, M.; Mercuri, M. [Clinica Ortopedica, Universita di Bologna, Bologna (Italy); Monti, C. [Servicio di Radiologia, Istituto Rizzoli, Bologna (Italy); Picci, P. [Lab. di Ricerca Oncologica, Istituto Rizzoli, Bologna (Italy)

    2001-04-01

    Objective. To report the imaging findings of 24 periosteal chondrosarcomas diagnosed, staged, treated and followed in a single institution, to analyze and define their pattern, and discuss their practical consequences.Design and patients. Plain films, 16 CT examinations and four MRI examinations were reviewed, and compared with the histological evaluation.Results. There were 20 men and four women, aged from 17 to 65 years. Twelve lesions involved the distal femoral metaphyses (8 posteriorly), five the proximal humerus, two the proximal metaphyses of the femur and two of the tibia, two the humeral shafts and one the iliac wing. Size varied from 4 to 11 cm. The cortex was always involved (thick, 15; thin, 13). Typical cartilaginous calcifications and cartilaginous lobules were very frequent. Radial thick periosteal bone formations (n=6) indicated calcifications between the lobules of cartilage. Medullary involvement was rare (n=2). All patients are alive and free of disease.Conclusions. Recognizing periosteal chondrosarcoma is of paramount importance because the prognosis is excellent after adequate local surgery alone. The patterns of other surface tumors of bone are usually different. (orig.)

  1. 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.)

  2. Endoscopic removal of nasal septum chondrosarcoma in paediatric patient

    OpenAIRE

    Muhammad Nasri Abu Bakar; Rosdi Ramli; Zulkiflee Salahuddin; Irfan Mohamad

    2014-01-01

    Chondrosarcomas of the head and neck are uncommon malignant tumours of non-epithelial origin. They rarely occur in paediatric age group. The treatment is mainly surgical and adjuvant therapy is only reserved to certain cases. We report a boy with progressive history of nasal obstruction and epistaxis. Nasal endoscopy revealed a mass arising from nasal septum. Endoscopic excision of the tumour was successfully done followed by radiotherapy. Endoscopic excision of chondrosarcoma of the nasal...

  3. [Dedifferentiated Chondrosarcoma of the Chest Wall].

    Science.gov (United States)

    Saitoh, Genkichi; Yoneshima, Yasuto; Nakamura, Toshihiko; Kitagawa, Dai; Kinjo, Nao; Ohgaki, Kippei; Maehara, Shinichiro; Teramoto, Seiichi; Adachi, Eisuke; Ikeda, Yoichi; Mine, Mari

    2016-08-01

    A 79-year-old man complaining of an anterior chest mass with pain had an abnormal shadow on chest X-ray. A mass, 7 cm in size, with destruction of the right 4th rib was found on chest computed tomography. A F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) corresponding to the lesion showed an abnormal accumulation of FDG with the standardized uptake value(SUV) max=16.19. A malignant tumor of the chest wall origin was suspected and the tumor was resected with the 3th, 4th, and 5th ribs. Histologically, the tumor was diagnosed as dedifferentiated chondrosarcoma. He died of local recurrence about 5 months after the operation. PMID:27476566

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

    NARCIS (Netherlands)

    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.

  5. Radiological features of extraskeletal mesenchymal chondrosarcoma

    International Nuclear Information System (INIS)

    Objective: To explore CT,MRI findings of extraskeletal mesenchymal chondrosarcoma (EMC). Methods: Imaging information of all 8 cases of EMC verified by pathology were retrospectively analyzed. Results: The location of lesions included lower extremity in 4 cases, forearm in 1 case,trunk in 2 cases and right lung in 1 case. The CT examination was performed in 7 cases, and 5 cases contained different patterns of ring-and-arc, granular, clump or irregular streaky mineralization. Dense calcification was detected in 3 cases,and focal in 2 cases. The nonmineralized component had slightly lower attenuation on CT scans than adjacent muscle. Four cases of peripheral located EMC demonstrated isointense on T1WI, and mixed signal intensity on T2WI. For the cases of fine and dense calcification in 2 cases,numbers of dot-like low-intensity signals were detected resembling 'pepper sign'; while for the cases of focal mineralization in 1 case, the low intensity area was located centrally in the high intensity area. Heterogeneous enhancement was found both in the calcified and uncalcified areas. One case of central located tumor exhibited low and high intensity on T1 and T2 weighted images, and nodular enhancement was observed. Conclusion: EMC has several characteristic imaging features, including various mineralization pattern, enhancement of calcified area and signal intensity, which might have diagnostic value for this rare tumor. (authors)

  6. A chondrosarcoma in the anterior mediastinum mimicking a thymoma

    International Nuclear Information System (INIS)

    A chondrosarcoma in the anterior mediastinum is a rare finding with a relatively good prognosis. We describe a case of a 75-year-old man with a 2-year history of neck discomfort and weight loss. Imaging showed a homogenous tumor with a minor compression on the anterior part of the heart. It had close relation to the ribs, no surrounding fat, and a thymoma was suspected. Biopsy prior to surgery was impossible due to the location of the tumor. Unfortunately, final pathology from the surgical specimen revealed a chondrosarcoma

  7. A chondrosarcoma in the anterior mediastinum mimicking a thymoma

    DEFF Research Database (Denmark)

    Østergaard, Mia L.; Petersen, Rene H; Kalhauge, Anna

    2015-01-01

    A chondrosarcoma in the anterior mediastinum is a rare finding with a relatively good prognosis. We describe a case of a 75-year-old man with a 2-year history of neck discomfort and weight loss. Imaging showed a homogenous tumor with a minor compression on the anterior part of the heart. It had...... close relation to the ribs, no surrounding fat, and a thymoma was suspected. Biopsy prior to surgery was impossible due to the location of the tumor. Unfortunately, final pathology from the surgical specimen revealed a chondrosarcoma....

  8. 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.

  9. 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.

  10. 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.

  11. Clear Cell Chondrosarcoma in Association With Niemann-Pick Disease

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    K. N. Srikanth

    2005-01-01

    Full Text Available Purpose: The purpose of this case report is to bring to light this unusual combination of two rare diseases, namely Neimann-Pick disease Type B and clear cell chondrosarcoma occurring in the same patient. This has not previously been reported in the world literature.

  12. The Identification of Prognostic Factors and Survival Statistics of Conventional Central Chondrosarcoma

    Directory of Open Access Journals (Sweden)

    Sjoerd P. F. T. Nota

    2015-01-01

    Full Text Available Introduction. Chondrosarcomas are malignant bone tumors that are characterized by the production of chondroid tissue. Since radiation therapy and chemotherapy have limited effect on chondrosarcoma, treatment of most patients depends on surgical resection. We conducted this study to identify independent predictive factors and survival characteristics for conventional central chondrosarcoma and dedifferentiated central chondrosarcoma. Methods. A systematic literature review was performed in September 2014 using the Pubmed, Embase, and Cochrane databases. Subsequent to a beforehand-composed selection procedure we included 13 studies, comprising a total of 1114 patients. Results. The prognosis of central chondrosarcoma is generally good for the histologically low-grade tumors. Prognosis for the high-grade chondrosarcoma and the dedifferentiated chondrosarcoma is poor with lower survival rates. Poor prognostic factors in conventional chondrosarcoma for overall survival are high-grade tumors and axial/pelvic tumor location. In dedifferentiated chondrosarcoma the percentage of dedifferentiated component has significant influence on disease-free survival. Conclusion. Despite the fact that there are multiple prognostic factors identified, as shown in this study, there is a need for prospective and comparative studies. The resulting knowledge about prognostic factors and survival can give direction in the development of better therapies. This could eventually lead to an evidence-based foundation for treating chondrosarcoma patients.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

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

    International Nuclear Information System (INIS)

    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.)

  15. Diagnosis and Outcome of Periosteal Chondrosarcoma in Two Cats.

    Science.gov (United States)

    Thompson, Elizabeth; Fauber, Amy E; Pool, Roy R

    2016-01-01

    Two cats, both over 10 yr old, were presented for evaluation of non-painful bony proliferations on the appendicular skeleton. These proliferations were identifiable via palpation. Radiographs showed a smooth, proliferative bony lesion of the distal femur (case 1) and tarsus (case 2) with mild soft tissue swelling. Surgical debulking with incomplete resection was performed in each cat. Subsequent histopathology resulted in a diagnosis of periosteal chondrosarcoma (PC). Although both cats have experienced local recurrence, both are still alive more than 2.5 yr after mass debulking. Periosteal chondrosarcoma is a differential diagnosis in proliferative cortical bony lesions near an articular surface in older cats. Partial resection of these masses can lead to an excellent quality of life, and proper diagnosis can avoid amputation or even euthanasia. PMID:27487347

  16. Clear Cell Chondrosarcoma in Association With Niemann-Pick Disease

    OpenAIRE

    Sumathi, V. P.; Grimer, R. J.; Davies, A. M.; Kulkarni, A.; Srikanth, K. N.

    2005-01-01

    Purpose: The purpose of this case report is to bring to light this unusual combination of two rare diseases, namely Neimann-Pick disease Type B and clear cell chondrosarcoma occurring in the same patient. This has not previously been reported in the world literature. Subject: Niemann-Pick disease (NPD) is a rare autosomal recessive inborn error of metabolism. Type B NPD is even rarer. It is a lysosomal storage disorder affecting children and adolescents often causing death in early childhood,...

  17. Demonstration of the uptake of 35S by chondrosarcomas

    International Nuclear Information System (INIS)

    Described are two methods of evaluation of the uptake of 30S by chondrosarcomas, i.e. autoradiography and external monitoring of the Bremsstrahlung. A higher concentration of sulfur in the tumor area of three patients was demonstrated after the injection of aliquots of both diagnostic and therapeutic activities. Apart from the simple handling and the quickly obtained results, the external monitoring of the Bremsstrahlung permits quantitative evaluation of the degree of uptake and a simple control over the progress of therapy. (orig.)

  18. Targeting survivin as a potential new treatment for chondrosarcoma of bone.

    Science.gov (United States)

    de Jong, Y; van Oosterwijk, J G; Kruisselbrink, A B; Briaire-de Bruijn, I H; Agrogiannis, G; Baranski, Z; Cleven, A H G; Cleton-Jansen, A-M; van de Water, B; Danen, E H J; Bovée, J V M G

    2016-01-01

    Chondrosarcomas are malignant cartilage-forming bone tumors, which are intrinsically resistant to chemo- and radiotherapy, leaving surgical removal as the only curative treatment option. Therefore, our aim was to identify genes involved in chondrosarcoma cell survival that could serve as a target for therapy. siRNA screening for 51 apoptosis-related genes in JJ012 chondrosarcoma cells identified BIRC5, encoding survivin, as essential for chondrosarcoma survival. Using immunohistochemistry, nuclear as well as cytoplasmic survivin expression was analyzed in 207 chondrosarcomas of different subtypes. Nuclear survivin has been implicated in cell-cycle regulation while cytoplasmic localization is important for its anti-apoptotic function. RT-PCR was performed to determine expression of the most common survivin isoforms. Sensitivity to YM155, a survivin inhibitor currently in phase I/II clinical trial for other tumors, was examined in 10 chondrosarcoma cell lines using viability assay, apoptosis assay and cell-cycle analysis. Survivin expression was found in all chondrosarcoma patient samples. Higher expression of nuclear and cytoplasmic survivin was observed with increasing histological grade in central chondrosarcomas. Inhibition of survivin using YM155 showed that especially TP53 mutant cell lines were sensitive, but no caspase 3/7 or PARP cleavage was observed. Rather, YM155 treatment resulted in a block in S phase in two out of three chondrosarcoma cell lines, indicating that survivin is more involved in cell-cycle regulation than in apoptosis. Thus, survivin is important for chondrosarcoma survival and chondrosarcoma patients might benefit from survivin inhibition using YM155, for which TP53 mutational status can serve as a predictive biomarker. PMID:27159675

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

    International Nuclear Information System (INIS)

    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.)

  20. Laryngeal Chondrosarcoma as a Rare Cause of Subglottic Stenosis

    Directory of Open Access Journals (Sweden)

    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.

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

    International Nuclear Information System (INIS)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  3. Regulation of collagenase inhibitor production in chondrosarcoma chondrocytes

    International Nuclear Information System (INIS)

    Swarm rat chondrosarcoma chondrocytes produce an inhibitor of collagenase. This inhibitor is similar to those isolated from normal cartilage tissues. These cells will synthesize proteins in the absence of serum. Since serum contains inhibitors of collagenase, it is necessary to culture cells without serum in order to obtain accurate measurements of enzyme and inhibitor levels. They examined the effect of insulin on inhibitor secretion by cultures of Swarm rat chondrosarcoma chondrocytes. They observed a 2.5 to 3.5 fold stimulation of inhibitory activity in the presence of as little as 10 ng/ml insulin as compared to controls in serum free Dulbecco's modified Eagle's medium supplemented with 4.5 g/l glucose. The units of inhibitor were determined over a 7 day culture period. Medium was harvested daily and assayed for collagenase activity and for inhibition of a known collagenase from rabbit skin or human skin, using the 14C-glycine peptide release assay. The amount of inhibitor obtained from days 2 through 7 were: 1.4 unit (control), 3.8 units (10 ng/ml insulin), 5.2 units (1 μg/ml insulin). The addition of 1 mM dibutyryl cyclic AMP to these chondrocytes in the presence of 1 μg/ml insulin caused a decrease in the level of inhibitor, suggesting that a dephosphorylation event may be necessary for this stimulation by insulin to occur

  4. Chondrosarcoma of chest wall metastasising to the larynx: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Ashish Singhal

    2015-01-01

    Full Text Available Chondrosarcoma is the most common malignant tumor of the chest wall. Most patients present with painful progressive swelling in the anterior chest wall arising from the costochondrosternal junction. CT scan with intravenous contrast is the investigation of choice. Wide excision with adequate margins is the standard treatment for localized disease after image guided biopsy. The role of chemotherapy and radiotherapy is limited. Lung is the most common site for metastasis. Metastasis to the larynx from chondrosarcoma has not been reported in the literature though primary chondrosarcoma can occur in the larynx. We hereby report a case of laryngeal metastasis from chondrosarcoma of the chest wall as a part of disease failure.

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

    International Nuclear Information System (INIS)

    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. 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. 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. 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 thymosin-β4 may have a role in chondrosarcoma metastasis

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    International Nuclear Information System (INIS)

    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

  8. MRI of mesenchymal chondrosarcoma of the orbit: case report and review of the literature

    International Nuclear Information System (INIS)

    Extraskeletal mesenchymal chondrosarcoma is a relatively uncommon entity, an orbital location being extremely rare. A review of the literature revealed 16 reported cases of primary orbital mesenchymal chondrosarcoma demonstrated by plain film and CT. To the best of our knowledge, the MRI features of orbital extraskeletal mesenchymal chondrosarcoma have not been previously reported. We present the case of an 18-year-old man with a 2-year history of progressive proptosis of the right eye who underwent CT, dynamic CT, MRI without and with gadolinium enhancement, and magnetic resonance angiography of the orbits. CT of orbital mesenchymal chondrosarcoma demonstrates a well-defined mass with multiple areas of fine and coarse calcification and shows moderate contrast enhancement. The noncalcified portions of the mass demonstrate signal intensity lower than or equal to gray matter on T1-weighted images and are isointense to the gray matter on T2-weighted images. Dynamic CT reveals delayed contrast enhancement. MRI has proven to be a valuable diagnostic tool in the diagnosis and differentiation of well-defined intraorbital masses. By a combination of CT and MRI, it appears mesenchymal chondrosarcoma can be differentiated from other intraorbital lesions, such as cavernous hemangioma, hemangiopericytoma, orbital amyloidosis and fibrous histiocytoma. (orig.). With 6 figs

  9. 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.

  10. Zoledronic acid in metastatic chondrosarcoma and advanced sacrum chordoma: two case reports

    Directory of Open Access Journals (Sweden)

    Capasso Elena

    2009-01-01

    Full Text Available Abstract Introduction Chondrosarcomas and chordomas are usually chemoresistant bone tumors and may have a poor prognosis when advanced. They are usually associated with worsening pain difficult to control. Patients and Methods Zoledronic acid was used in a 63-year-old man with metastatic chondrosarcoma and in a 66-year-old woman with a diagnosis of sacrum chordoma both reporting severe pain related to tumor. Results In the first case, zoledronic acid was able to maintain pain control despite disease progression following chemotherapy, in the other case, zoledronic acid only produced significant clinical benefit. Conclusion Control of pain associated with bone tumors such as chondrosarcoma and chondroma may significantly improve from use of zoledronic acid, independently from tumor response to other treatments. Evaluation on larger series are needed to confirm the clinical effect of this bisphosphonate on such tumors.

  11. Tyrosine kinase inhibitor SU6668 represses chondrosarcoma growth via antiangiogenesis in vivo

    International Nuclear Information System (INIS)

    As chondrosarcomas are resistant to chemotherapy and ionizing radiation, therapeutic options are limited. Radical surgery often cannot be performed. Therefore, additional therapies such as antiangiogenesis represent a promising strategy for overcoming limitations in chondrosarcoma therapy. There is strong experimental evidence that SU6668, an inhibitor of the angiogenic tyrosine kinases Flk-1/KDR, PDGFRbeta and FGFR1 can induce growth inhibition of various primary tumors. However, the effectiveness of SU6668 on malignant primary bone tumors such as chondrosarcomas has been rarely investigated. Therefore, the aim of this study was to investigate the effects of SU6668 on chondrosarcoma growth, angiogenesis and microcirculation in vivo. In 10 male severe combined immunodeficient (SCID) mice, pieces of SW1353 chondrosarcomas were implanted into a cranial window preparation where the calvaria serves as the site for the orthotopic implantation of bone tumors. From day 7 after tumor implantation, five animals were treated with SU6668 (250 mg/kg body weight, s.c.) at intervals of 48 hours (SU6668), and five animals with the equivalent amount of the CMC-based vehicle (Control). Angiogenesis, microcirculation, and growth of SW 1353 tumors were analyzed by means of intravital microscopy. SU6668 induced a growth arrest of chondrosarcomas within 7 days after the initiation of the treatment. Compared to Controls, SU6668 decreased functional vessel density and tumor size, respectively, by 37% and 53% on day 28 after tumor implantation. The time course of the experiments demonstrated that the impact on angiogenesis preceded the anti-tumor effect. Histological and immunohistochemical results confirmed the intravital microscopy findings. SU6668 is a potent inhibitor of chondrosarcoma tumor growth in vivo. This effect appears to be induced by the antiangiogenic effects of SU6668, which are mediated by the inhibition of the key angiogenic receptor tyrosine kinases Flk-1/KDR, PDGFRbeta

  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. Magnetic resonance imaging findings of extraskeletal mesenchymal chondrosarcoma of wrist: a case report

    Directory of Open Access Journals (Sweden)

    Aysin Pourbagher

    2016-06-01

    Full Text Available Extraskeletal mesenchymal chondrosarcomas (EMC are high grade malignant tumors that rarely involves wrist. Published reports about them are mostly based on conventional radiography, tomography and conventional magnetic resonance imaging (MRI findings. We did not recognize any report of dynamic-contrast enhanced MRI so far. Herein, we present dynamic contrast enhanced and Diffusion Weighted MRI findings of a case with extraskeletal mesenchymal chondrosarcoma of the wrist. In conclusion, EMC might show peripheral enhancement at early arterial phase and restricted diffusion on MRI. The knowledge of the imaging spectrum of the EMC might help us suggesting the diagnosis. [Cukurova Med J 2016; 41(2.000: 374-378

  14. Chondrosarcoma of the hyoid bone: computed tomography findings; Condrosarcoma de hioides: hallazgos en tomografia computarizada

    Energy Technology Data Exchange (ETDEWEB)

    Saez, J.; Gallego, J. a.; Fuster, M. J. [Hospital General Universitario de Elche. Alicante (Spain)

    2001-07-01

    Chondrosarcoma of the hyoid bone is a rare entity, only 10 cases of which have been reported in the literature to date. the case we present involved a 24-year-old man who complained of progressive adynophagia and a mass in anterior neck. Computed tomography revealed a low-attenuation mas attached to the left horn of the hyoid bone. The lesion was excised and was diagnosed as a grade II chondrosarcoma. The patient remains asymptomatic 10 years after the operation. (Author) 11 refs.

  15. Mesenchymal chondrosarcoma of the orbit: CT and MRI findings

    International Nuclear Information System (INIS)

    Aim: To describe the computed tomography (CT) and magnetic resonance imaging (MRI) features of orbital mesenchymal chondrosarcomas (MCSs). Materials and methods: Six patients with histology-confirmed MCSs of the orbit were retrospectively reviewed. All six patients underwent CT and MRI. Imaging studies were evaluated for the following: (a) tumour location, (b) configuration, size, and margin, (c) CT attenuation and MRI signal intensity, and (d) secondary manifestations. Additionally, the time–intensity curve (TIC) of dynamic contrast-enhanced (DCE) MRI were analysed in five patients. Results: Two MCSs arose in the right orbit and four in the left orbit. Five MCSs were located in the retrobulbar intraconal space and one in the extraconal space. All the lesions displayed a lobulate configuration and had a well-defined margin. The mean maximum diameter was 25.8 mm (range 15–36 mm). On unenhanced CT, the lesions appeared isodense to grey matter in six patients, with calcifications in five. Two patients showed inhomogeneous, moderate enhancement on enhanced CT. Six MCSs appeared isointense on T1-weighted imaging and heterogeneously isointense on T2-weighted imaging. The lesions showed significantly heterogeneous contrast enhancement. Five patients had DCE MRI and the TICs showed a rapidly enhancing and rapid washout pattern (type III). The following features were also detected: compression of the extra-ocular muscle (six patients, 100%); displacement of the optic nerve (five patients, 83.3%); and encasing globe (three patients, 50%). Conclusions: A well-defined, lobulate orbital mass with calcification on CT and, marked heterogeneous enhancement and type III TIC on MRI are highly suspicious of orbital MCSs.

  16. Leptin promotes VEGF-C production and induces lymphangiogenesis by suppressing miR-27b in human chondrosarcoma cells

    Science.gov (United States)

    Yang, Wei-Hung; Chang, An-Chen; Wang, Shih-Wei; Wang, Shoou-Jyi; Chang, Yung-Sen; Chang, Tzu-Ming; Hsu, Shao-Keh; Fong, Yi-Chin; Tang, Chih-Hsin

    2016-01-01

    Chondrosarcoma is the second most frequently occurring type of bone malignancy that is characterized by the distant metastasis propensity. Vascular endothelial growth factor-C (VEGF-C) is the chief lymphangiogenic mediator, and makes crucial contributions to tumor lymphangiogenesis. Leptin is an adipocytokine and has been indicated to facilitate tumorigenesis, angiogenesis and metastasis. However, the effect of leptin on VEGF-C regulation and lymphangiogenesis in human chondrosarcoma has hugely remained a mystery. Our results showed a clinical correlation between leptin and VEGF-C as well as tumor stage in human chondrosarcoma tissues. We further demonstrated that leptin promoted VEGF-C production and secretion in human chondrosarcoma cells. The conditioned medium from leptin-treated chondrosarcoma cells induced lymphangiogenesis of human lymphatic endothelial cells. We also found that leptin-induced VEGF-C is mediated by the FAK, PI3K and Akt signaling pathway. Furthermore, the expression of microRNA-27b was negatively regulated by leptin via the FAK, PI3K and Akt cascade. Our study is the first to describe the mechanism of leptin-promoted lymphangiogenesis by upregulating VEGF-C expression in chondrosarcomas. Thus, leptin could serve as a therapeutic target in chondrosarcoma metastasis and lymphangiogenesis. PMID:27345723

  17. siRNA-based targeting of antiapoptotic genes can reverse chemoresistance in P-glycoprotein expressing chondrosarcoma cells

    Directory of Open Access Journals (Sweden)

    Yang Jay

    2009-05-01

    Full Text Available Abstract Background High expression of P-glycoprotein is one of the well-known mechanisms of chemoresistance in chondrosarcomas. However, the role of antiapoptotic proteins, a common mechanism responsible for chemoresistance in other tumors, has not been well studied in chondrosarcomas. We examined the importance of P-glycoprotein and antiapoptotic proteins in the chemoresistance to doxorubicin of two Grade II chondrosarcoma cell lines, JJ012 and SW1353. Results We confirmed that both chondrosarcoma cell types expressed P-glycoprotein and antiapoptotic proteins (Bcl-2, Bcl-xL and XIAP. siRNA knockdown as well as pharmacologic inhibitors of cell survival proteins (Bcl-2, Bcl-xL and XIAP enhanced apoptosis of chemoresistant chondrosarcoma cells by up to 5.5 fold at 0.1 μmol and 5.5 fold at 1 μmol doxorubicin. These chemosensitizing effects were comparable to those of P-glycoprotein inhibition by siRNA or pharmacologic inhibitor. Conclusion These findings suggest that antiapoptotic proteins play a significant role in the chemoresistance of chondrosarcoma cells independent of P-glycoprotein. Based on the results, a new siRNA-based therapeutic strategy targeting antiapoptotic genes can be designed to overcome the chemoresistance of chondrosarcomas which is often conferred by P-glycoprotein.

  18. BMP and TGFbeta pathways in human central chondrosarcoma: enhanced endoglin and Smad 1 signaling in high grade tumors

    Directory of Open Access Journals (Sweden)

    Boeuf Stephane

    2012-10-01

    Full Text Available Abstract Background As major regulators of normal chondrogenesis, the bone morphogenic protein (BMP and transforming growth factor β (TGFB signaling pathways may be involved in the development and progression of central chondrosarcoma. In order to uncover their possible implication, the aim of this study was to perform a systematic quantitative study of the expression of BMPs, TGFBs and their receptors and to assess activity of the corresponding pathways in central chondrosarcoma. Methods Gene expression analysis was performed by quantitative RT-PCR in 26 central chondrosarcoma and 6 healthy articular cartilage samples. Expression of endoglin and nuclear localization of phosphorylated Smad1/5/8 and Smad2 was assessed by immunohistochemical analysis. Results The expression of TGFB3 and of the activin receptor-like kinase ALK2 was found to be significantly higher in grade III compared to grade I chondrosarcoma. Nuclear phosphorylated Smad1/5/8 and Smad2 were found in all tumors analyzed and the activity of both signaling pathways was confirmed by functional reporter assays in 2 chondrosarcoma cell lines. Immunohistochemical analysis furthermore revealed that phosphorylated Smad1/5/8 and endoglin expression were significantly higher in high-grade compared to low-grade chondrosarcoma and correlated to each other. Conclusions The BMP and TGFβ signaling pathways were found to be active in central chondrosarcoma cells. The correlation of Smad1/5/8 activity to endoglin expression suggests that, as described in other cell types, endoglin could enhance Smad1/5/8 signaling in high-grade chondrosarcoma cells. Endoglin expression coupled to Smad1/5/8 activation could thus represent a functionally important signaling axis for the progression of chondrosarcoma and a regulator of the undifferentiated phenotype of high-grade tumor cells.

  19. BMP and TGFbeta pathways in human central chondrosarcoma: enhanced endoglin and Smad 1 signaling in high grade tumors

    International Nuclear Information System (INIS)

    As major regulators of normal chondrogenesis, the bone morphogenic protein (BMP) and transforming growth factor β (TGFB) signaling pathways may be involved in the development and progression of central chondrosarcoma. In order to uncover their possible implication, the aim of this study was to perform a systematic quantitative study of the expression of BMPs, TGFBs and their receptors and to assess activity of the corresponding pathways in central chondrosarcoma. Gene expression analysis was performed by quantitative RT-PCR in 26 central chondrosarcoma and 6 healthy articular cartilage samples. Expression of endoglin and nuclear localization of phosphorylated Smad1/5/8 and Smad2 was assessed by immunohistochemical analysis. The expression of TGFB3 and of the activin receptor-like kinase ALK2 was found to be significantly higher in grade III compared to grade I chondrosarcoma. Nuclear phosphorylated Smad1/5/8 and Smad2 were found in all tumors analyzed and the activity of both signaling pathways was confirmed by functional reporter assays in 2 chondrosarcoma cell lines. Immunohistochemical analysis furthermore revealed that phosphorylated Smad1/5/8 and endoglin expression were significantly higher in high-grade compared to low-grade chondrosarcoma and correlated to each other. The BMP and TGFβ signaling pathways were found to be active in central chondrosarcoma cells. The correlation of Smad1/5/8 activity to endoglin expression suggests that, as described in other cell types, endoglin could enhance Smad1/5/8 signaling in high-grade chondrosarcoma cells. Endoglin expression coupled to Smad1/5/8 activation could thus represent a functionally important signaling axis for the progression of chondrosarcoma and a regulator of the undifferentiated phenotype of high-grade tumor cells

  20. Chest wall secondary chondrosarcoma arising from enchondroma in a young Asian female

    Directory of Open Access Journals (Sweden)

    Chih Chiang Nieh

    2014-01-01

    CONCLUSION: Secondary chondrosarcoma in a young Asian female arising from a benign solitary enchondroma in the absence of multiple enchondromatosis, such as Ollier disease or Maffucci syndrome, is extremely rare. Regular follow up including physical assessment and radiological imaging can result in earlier detection of malignant transformation obviating the need for aggressive surgical treatment involving multiple rib resections.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  3. Effect of insulin on the mRNA expression of procollagen N-proteinases in chondrosarcoma OUMS-27 cells

    OpenAIRE

    Akyol, Sumeyya; Cömertoğlu, İsmail; FIRAT, RIDVAN; Çakmak, Özlem; YUKSELTEN, YUNUS; ERDEN, GÖNÜL; Ugurcu, Veli; Demircan,Kadir

    2015-01-01

    Chondrosarcoma is one of the most common bone tumors, and at present, there is no non-invasive treatment option for this cancer. The chondrosarcoma OUMS-27 cell line produces proteoglycan and type II, IX, and XI collagens, which constitutes cartilage tissue. A disintegrin and metalloproteinase with thrombospondin motifs (ADAMTS) proteases are a group of secreted proteases, which include the procollagen N-proteinases ADAMTS-2, -3 and -14. These procollagen N-proteinases perform a role in the p...

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

    OpenAIRE

    Chi-Ming Wu; Te-Mao Li; Tzu-Wei Tan; Yi-Chin Fong; Chih-Hsin Tang

    2013-01-01

    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 berbe...

  5. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  7. Breast cancer risk in mothers of children with osteosarcoma and chondrosarcoma.

    OpenAIRE

    Hartley, A L; Birch, J M; Marsden, H B; Harris, M

    1986-01-01

    Mothers of a population-based series of 86 children with osteosarcoma or chondrosarcoma were traced and their health status or cause of death ascertained. There were 6 cases of breast cancer among these mothers and 6 other cancers. Risk of breast cancer was approximately three times that expected, and appeared to be highest in mothers of boys and in mothers of children under the median age at diagnosis. The mothers who developed breast cancer were relatively young at diagnosis compared with p...

  8. Chondrosarcoma of chest wall metastasising to the larynx: Case report and review of literature

    OpenAIRE

    Ashish Singhal; Charu Mahajan; Rahat Hadi; Namrata P Awasthi

    2015-01-01

    Chondrosarcoma is the most common malignant tumor of the chest wall. Most patients present with painful progressive swelling in the anterior chest wall arising from the costochondrosternal junction. CT scan with intravenous contrast is the investigation of choice. Wide excision with adequate margins is the standard treatment for localized disease after image guided biopsy. The role of chemotherapy and radiotherapy is limited. Lung is the most common site for metastasis. Metastasis to the lary...

  9. Quaternary ammonium as vector of radioisotopes toward cartilage proteoglycans: in vivo imaging and monitoring of chondrosarcoma

    International Nuclear Information System (INIS)

    The full text of the publication follows. AIM: Our strategy consists in using the quaternary ammonium function, that exhibits a high affinity for proteoglycans, as a selective carrier to cartilage of (i) drugs for improving the selectivity or (ii) radioisotopes for imaging and evaluating response to treatment. For diagnosis, a radiotracer radiolabeled with 99mTc (99mTc-NTP 15-5) was selected and for therapeutic application, a quaternary ammonium derivative of melphalan (Mel-AQ) was synthesized. This study demonstrates the interest of this strategy for the diagnosis and treatment of chondrosarcoma. Methods: 99mTc-NTP 15-5 imaging was performed at regular intervals in rats bearing ortho-topic swarm chondrosarcoma, controls or treated (Mel-AQ: three intravenous doses of 10 mg/kg). 99mTc-HMDP imaging (the only radiotracer available for nuclear medicine diagnosis of chondrosarcoma) was also performed. Results: All rats exhibited a significant tumoral uptake of 99mTc-NTP 15-5 at very early stage of pathology while no palpable nor measurable tumour could be assessed. Furthermore, tumoral uptake increased as pathology progressed over time. When animals were treated with Mel-AQ, a significant tumor growth inhibition was observed with 99mTc-NTP 15-5 tumoral uptake being significantly decreased as compared to controls. 99mTc-HMDP bone scans were negative during the whole study. Conclusion: These experimental results underline (i) the potential of the proteoglycan targeting strategy for the early and specific diagnosis imaging of chondrosarcoma and its response to therapy and (ii) the efficiency of the targeted anti-tumoral therapy. In future, we could plan to substitute technetium atom for copper atom for radionuclide therapy application. Grants: INCa, CPER, Ligue contre le cancer, FRI/OSEO. (authors)

  10. Simultaneous interphase cytogenetic analysis and fluorescence immunophenotyping of dedifferentiated chondrosarcoma. Implications for histopathogenesis.

    OpenAIRE

    Bridge, J A; deBoer, J.; Travis, J; Johansson, S. L.; Elmberger, G; Noel, S. M.; Neff, J. R.

    1994-01-01

    Cytogenetic analysis of four specimens (biopsy, definitive surgical, and two separately occurring lung metastases) of a dedifferentiated chondrosarcoma with a rhabdomyosarcomatous component revealed clonal karyotypic abnormalities in each. Anomalies seen in all specimens included a structurally aberrant chromosome 17 and extra copies of chromosomes 5, 7, 12, and 20. The derivation of the chromosomally abnormal cells was determined by a combined immunocytochemical/cytogenetic approach that all...

  11. 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...

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

    Directory of Open Access Journals (Sweden)

    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.

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

    International Nuclear Information System (INIS)

    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.)

  14. Challenges in Linear Accelerator Radiotherapy for Chordomas and Chondrosarcomas of the Skull Base: Focus on Complications

    Energy Technology Data Exchange (ETDEWEB)

    Hauptman, Jason S., E-mail: jhauptman@mednet.ucla.edu [Division of Stereotactic and Functional Neurosurgery, Department of Neurosurgery, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States); Barkhoudarian, Garni; Safaee, Michael; Gorgulho, Alessandra [Division of Stereotactic and Functional Neurosurgery, Department of Neurosurgery, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States); Tenn, Steven; Agazaryan, Nzhde; Selch, Michael [Department of Radiation Oncology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States); De Salles, Antonio A.F. [Division of Stereotactic and Functional Neurosurgery, Department of Neurosurgery, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States); Department of Radiation Oncology, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA (United States)

    2012-06-01

    Purpose: Intracranial chordomas and chondrosarcomas are histologically low-grade, locally invasive tumors that infiltrate the skull base. Currently, consensus therapy includes surgical resection and adjuvant radiotherapy. Radiation delivery is typically limited by the proximity of these tumors to critical skull base structures. Methods: This is a retrospective review of 13 cases of chordomas and 2 cases of chondroid chondrosarcomas of the skull based treated with linear accelerator stereotactic radiotherapy (SRT, n = 10) or stereotactic radiosurgery (SRS, n = 5). The average time to the most recent follow-up visit was 4.5 years. The tumor characteristics, treatment details, and outcomes were recorded. Each radiation plan was reviewed, and the dosage received by the brainstem, optic apparatus, and pituitary was calculated. Results: Of the 10 patients treated with SRT, 6 were found to have unchanged or decreased tumor size as determined from radiographic follow-up. Of the 5 patients treated with SRS, 3 were found to have stable or unchanged tumors at follow-up. The complications included 1 SRT patient who developed endocrinopathy, 2 patients (1 treated with SRS and the other with SRT), who developed cranial neuropathy, and 1 SRS patient who developed visual deficits. Additionally, 1 patient who received both SRS and SRT within 2 years for recurrence experienced transient medial temporal lobe radiation changes that resolved. Conclusions: Where proton beam therapy is unavailable, linear accelerator-based SRT or radiosurgery remains a safe option for adjuvant therapy of chordomas and chondrosarcomas of the skull base. The exposure of the optic apparatus, pituitary stalk, and brainstem must be considered during planning to minimize complications. If the optic apparatus is included in the 80% isodose line, it might be best to fractionate therapy. Exposure of the pituitary stalk should be kept to <30 Gy to minimize endocrine dysfunction. Brainstem exposure should be

  15. Challenges in Linear Accelerator Radiotherapy for Chordomas and Chondrosarcomas of the Skull Base: Focus on Complications

    International Nuclear Information System (INIS)

    Purpose: Intracranial chordomas and chondrosarcomas are histologically low-grade, locally invasive tumors that infiltrate the skull base. Currently, consensus therapy includes surgical resection and adjuvant radiotherapy. Radiation delivery is typically limited by the proximity of these tumors to critical skull base structures. Methods: This is a retrospective review of 13 cases of chordomas and 2 cases of chondroid chondrosarcomas of the skull based treated with linear accelerator stereotactic radiotherapy (SRT, n = 10) or stereotactic radiosurgery (SRS, n = 5). The average time to the most recent follow-up visit was 4.5 years. The tumor characteristics, treatment details, and outcomes were recorded. Each radiation plan was reviewed, and the dosage received by the brainstem, optic apparatus, and pituitary was calculated. Results: Of the 10 patients treated with SRT, 6 were found to have unchanged or decreased tumor size as determined from radiographic follow-up. Of the 5 patients treated with SRS, 3 were found to have stable or unchanged tumors at follow-up. The complications included 1 SRT patient who developed endocrinopathy, 2 patients (1 treated with SRS and the other with SRT), who developed cranial neuropathy, and 1 SRS patient who developed visual deficits. Additionally, 1 patient who received both SRS and SRT within 2 years for recurrence experienced transient medial temporal lobe radiation changes that resolved. Conclusions: Where proton beam therapy is unavailable, linear accelerator-based SRT or radiosurgery remains a safe option for adjuvant therapy of chordomas and chondrosarcomas of the skull base. The exposure of the optic apparatus, pituitary stalk, and brainstem must be considered during planning to minimize complications. If the optic apparatus is included in the 80% isodose line, it might be best to fractionate therapy. Exposure of the pituitary stalk should be kept to <30 Gy to minimize endocrine dysfunction. Brainstem exposure should be

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

    Directory of Open Access Journals (Sweden)

    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.

  17. Expression of aromatase and estrogen receptor alpha in chondrosarcoma, but no beneficial effect of inhibiting estrogen signaling both in vitro and in vivo

    Directory of Open Access Journals (Sweden)

    Meijer Danielle

    2011-07-01

    Full Text Available Abstract Background Chondrosarcomas are malignant cartilage-forming tumors which are highly resistant to conventional chemotherapy and radiotherapy. Estrogen signaling is known to play an important role in proliferation and differentiation of chondrocytes and in growth plate regulation at puberty. Our experiments focus on unraveling the role of estrogen signaling in the regulation of neoplastic cartilage growth and on interference with estrogen signaling in chondrosarcomas in vitro and in vivo. Methods We investigated the protein expression of estrogen receptor alpha (ESR1, androgen receptor (AR, and aromatase in tumor specimens of various chondrosarcoma subtypes, and (primary chondrosarcoma cultures. Dose-response curves were generated of conventional central chondrosarcoma cell lines cultured in the presence of 17β-estradiol, dihydrotestosterone, 4-androstene-3,17 dione, 4-hydroxytamoxifen, fulvestrant and aromatase inhibitors. In a pilot series, the effect of anastrozole (n = 4 or exemestane (n = 2 treatment in 6 chondrosarcoma patients with progressive disease was explored. Results We showed protein expression of ESR1 and aromatase in a large majority of all subtypes. Only a minority of the tumors showed few AR positive cells. The dose-response assays showed no effect of any of the compounds on proliferation of conventional chondrosarcoma in vitro. The median progression-free survival of the patients treated with aromatase inhibitors did not significantly deviate from untreated patients. Conclusions The presence of ESR1 and aromatase in chondrosarcoma tumors and primary cultures supports a possible role of estrogen signaling in chondrosarcoma proliferation. However, our in vitro and pilot in vivo studies have shown no effect of estrogen-signaling inhibition on tumor growth.

  18. LARYNGEAL CHONDROSARCOMA: SUCCESSFUL USE OF VIDEO LARYNGOSCOPE IN ANTICIPATED DIFFICULT AIRWAY MANAGEMENT.

    Science.gov (United States)

    Dolinaj, Vladimir; Milošev, Sanja; Janjević, Dušanka

    2016-03-01

    Laryngeal chondrosarcoma is a rare mesenchymal tumor, most frequently affecting cricoid cartilage. The objective of this report is to present successful video laryngoscope usage in a patient with anticipated difficult airway who refused awake fiberoptic endotracheal intubation (AFOI). A 59-year-old male patient was admitted in our hospital due to difficulty breathing and swallowing. On clinical examination performed by ENT surgeon, preoperative endoscopic airway examination (PEAE) could not be performed properly due to the patient's uncooperativeness. Computed tomography revealed a spherical tumor that obstructed the subglottic area almost entirely. Due to the narrowed airway, the first choice for the anticipated difficult airway management was AFOI, which the patient refused. Consequently, we decided to perform endotracheal intubation with indirect laryngoscope using a C-MAC video laryngoscope (Karl Storz, Tuttlingen, Germany). Reinforced endotracheal tube (6.0 mm internal diameter) was placed gently between the tumor mass and the posterior wall of the trachea in the first attempt. Confirmation of endotracheal intubation was done by capnography. In a patient with subglottic area chondrosarcoma refusing PEAE and AFOI, video laryngoscope is a particularly helpful device for difficult airway management when difficult airway is anticipated. PMID:27276783

  19. Systemic therapy for selected skull base sarcomas: Chondrosarcoma, chordoma, giant cell tumour and solitary fibrous tumour/hemangiopericytoma.

    Science.gov (United States)

    Colia, Vittoria; Provenzano, Salvatore; Hindi, Nadia; Casali, Paolo G; Stacchiotti, Silvia

    2016-01-01

    This review highlights the data currently available on the activity of systemic therapy in chondrosarcoma, chordoma, giant cell tumour of the bone (GCTB) and solitary fibrous tumour, i.e., four rare sarcomas amongst mesenchymal malignancy arising from the skull base. PMID:27330421

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

    Directory of Open Access Journals (Sweden)

    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.

  1. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  3. Proton radiation therapy for chordomas and chondrosarcomas of the skull base.

    Science.gov (United States)

    Hug, E B; Slater, J D

    2000-10-01

    Most patients with conventional radiotherapy after surgery die with local disease progression. The superior local tumor control and overall survival achieved with fractionated proton RT can be attributed to improved dose localization characteristics of protons, resulting in higher doses delivered. Patients with base of skull neoplasms are increasingly considered for stereotactic radiosurgery. Recently, Muthukumar et al reported for the University of Pittsburgh group on cobalt-60 Gamma Knife (Elekta Instruments, Atlanta, GA) therapy for 15 patients with chordomas or chondrosarcomas of the base of the skull. With tumor volumes ranging between 0.98 and 10.3 mL (mean, 4.6 mL), doses to the tumor margin varying from 12 to 20 Gy (median, 18 Gy) were delivered. Two patients were treated without histologic tumor confirmation. After a median follow-up time of 40 months, 2 patients had died of disease, 2 patients had succumbed to intercurrent disease, and 1 patient surviving at the time of analysis had developed tumor progression. Neither actuarial local control nor actuarial survival data were presented. In the LLUMC series, most tumors exceeded sizes reportedly suitable for radiosurgery or were of a highly irregular configuration. Nevertheless, in 11 patients, tumors less than 15 mL in size remained locally controlled as did tumors sized between 15 and 25 mL in 11 additional patients; these patients were thus potential candidates for stereotactic radiosurgery. At present, too few reports on radiosurgery contain sufficient patient numbers and statistical analyses to permit one to draw conclusions about the feasibility of radiosurgery for chordomas and chondrosarcomas of the base of the skull. A principal difference between proton RT and radiosurgery as currently practiced in most centers concerns target definition. In proton RT, the GTV is treated. In addition, a clinical volume is defined, which is distinctly different from the GTV in size and shape, to include the

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

    International Nuclear Information System (INIS)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    Atta, Ihab Shafek; AlQahtani, Fahd Nasser

    2016-01-01

    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. PMID:27087812

  7. Dedifferentiated laryngeal chondrosarcoma: combined morphologic and functional imaging with positron-emission tomography/magnetic resonance imaging.

    Science.gov (United States)

    Purohit, Bela S; Dulguerov, Pavel; Burkhardt, Karim; Becker, Minerva

    2014-07-01

    Chondrosarcoma of the larynx is a rare, low-grade malignancy in terms of histology and clinical behavior. We present an unusual case of laryngeal chondrosarcoma, which developed a large dedifferentiated component on recurrence after primary surgery. The diagnosis of dedifferentiation was suggested in view of the morphological and metabolic findings on hybrid positron-emission tomography/magnetic resonance imaging (PET/MRI) and was subsequently confirmed surgically. Whole-organ, slice-by-slice radiologic-histologic correlation revealed excellent delineation of the well-differentiated and dedifferentiated tumor components with PET/MRI. PET/MRI can provide additional functional information to supplement the morphological mapping and histopathology of these tumors. PMID:24222068

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

    Science.gov (United States)

    Atta, Ihab Shafek; AlQahtani, Fahd Nasser

    2016-01-01

    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. PMID:27087812

  9. Dedifferentiated Chondrosarcoma of the Rib Masquerading as a Giant Chest Wall Tumor in a Teenage Girl: An Unusual Presentation

    OpenAIRE

    Abraham, Viju Joseph; Devgarha, Sanjeev; Mathur, Rajendra Mohan; Sisodia, Anula; Yadav, Amita

    2014-01-01

    Chondrosarcoma of the chest wall is a rare primary neoplasm found to occur in elderly men. Patients present with an enlarging, painful, anterior chest wall mass arising from either the vicinity of the costochondral junction or the sternum. Treatment includes wide resection with appropriate chest wall reconstruction. We report an unusual presentation of this uncommon tumor occurring as a huge chest wall mass in a young teenage girl.

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

    Directory of Open Access Journals (Sweden)

    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.

  11. The Bone Niche of Chondrosarcoma: A Sanctuary for Drug Resistance, Tumour Growth and also a Source of New Therapeutic Targets

    Directory of Open Access Journals (Sweden)

    E. David

    2011-01-01

    Full Text Available Chondrosarcomas are malignant cartilage-forming tumours representing around 20% of malignant primary tumours of bone and affect mainly adults in the third to sixth decade of life. Unfortunately, the molecular pathways controlling the genesis and the growth of chondrosarcoma cells are still not fully defined. It is well admitted that the invasion of bone by tumour cells affects the balance between early bone resorption and formation and induces an “inflammatory-like” environment which establishes a dialogue between tumour cells and their environment. The bone tumour microenvironment is then described as a sanctuary that contributes to the drug resistance patterns and may control at least in part the tumour growth. The concept of “niche” defined as a specialized microenvironment that can promote the emergence of tumour stem cells and provide all the required factors for their development recently emerges in the literature. The present paper aims to summarize the main evidence sustaining the existence of a specific bone niche in the pathogenesis of chondrosarcomas.

  12. Active raster scanning with carbon ions. Reirradiation in patients with recurrent skull base chordomas and chondrosarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Uhl, Matthias; Welzel, Thomas; Oelmann, Jan; Habl, Gregor; Hauswald, Henrik; Jensen, Alexandra; Debus, Juergen; Herfarth, Klaus [University of Heidelberg, Department of Radiation Oncology, Heidelberg (Germany); Ellerbrock, Malte [Heidelberg Ion Therapy Center (HIT), Heidelberg (Germany)

    2014-07-15

    To evaluate the safety and efficacy of reirradiation with carbon ions in patients with relapse of skull base chordoma and chondrosarcoma. Reirradiation with carbon ions was performed on 25 patients with locally recurrent skull base chordoma (n = 20) or chondrosarcoma (n = 5). The median time between the last radiation exposure and the reirradiation with carbon ions was 7 years. In the past, 23 patients had been irradiated once, two patients twice. Reirradiation was delivered using the active raster scanning method. The total median dose was 51.0 GyE carbon ions in a weekly regimen of five to six fractions of 3 GyE. Local progression-free survival (LPFS) was evaluated using the Kaplan-Meier method; toxicity was evaluated using the NCI Common Terminology Criteria for Adverse Events (CTCAE v.4.03). The treatment could be finished in all patients without interruption. In 80 % of patients, symptom control was achieved after therapy. The 2-year-LPFS probability was 79.3 %. A PTV volume of < 100 ml or a total dose of > 51 GyE was associated with a superior local control rate. The therapy was associated with low acute toxicity. One patient developed grade 2 mucositis during therapy. Furthermore, 12 % of patients had tympanic effusion with mild hypacusis (grade 2), while 20 % developed an asymptomatic temporal lobe reaction after treatment (grade 1). Only one patient showed a grade 3 osteoradionecrosis. Reirradiation with carbon ions is a safe and effective method in patients with relapsed chordoma and chondrosarcoma of the skull base. (orig.) [German] Evaluierung der Sicherheit und Wirksamkeit einer Re-Bestrahlung mittels Kohlenstoffionen bei Patienten mit Lokalrezidiv eines Chordoms und Chondrosarkoms der Schaedelbasis. Bei 25 Patienten mit einem Lokalrezidiv eines Chordoms (n = 20) oder Chondrosarkoms (n = 5) der Schaedelbasis erfolgte eine Re-Bestrahlung mittels Kohlenstoffionen. Die mediane Zeit zwischen letzter Bestrahlung und Re-Bestrahlung mit Kohlenstoffionen

  13. Human Chondrosarcoma Cells Acquire an Epithelial-Like Gene Expression Pattern via an Epigenetic Switch: Evidence for Mesenchymal-Epithelial Transition during Sarcomagenesis

    Directory of Open Access Journals (Sweden)

    Matthew P. Fitzgerald

    2011-01-01

    Full Text Available Chondrocytes are mesenchymally derived cells that reportedly acquire some epithelial characteristics; however, whether this is a progression through a mesenchymal to epithelial transition (MET during chondrosarcoma development is still a matter of investigation. We observed that chondrosarcoma cells acquired the expression of four epithelial markers, E-cadherin,desmocollin 3, maspin, and 14-3-3σ, all of which are governed epigenetically through cytosine methylation. Indeed, loss of cytosine methylation was tightly associated with acquired expression of both maspin and 14-3-3σ in chondrosarcomas. In contrast, chondrocyte cells were negative for maspin and 14-3-3σ and displayed nearly complete DNA methylation. Robust activation of these genes was also observed in chondrocyte cells following 5-aza-dC treatment. We also examined the transcription factor snail which has been reported to be an important mediator of epithelial to mesenchymal transitions (EMTs. In chondrosarcoma cells snail is downregulated suggesting a role for loss of snail expression in lineage maintenance. Taken together, these results document an epigenetic switch associated with an MET-like phenomenon that accompanies chondrosarcoma progression.

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

    Directory of Open Access Journals (Sweden)

    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.

  15. In vitro evaluation of chondrosarcoma cells and canine chondrocytes on layer-by-layer (LbL) self-assembled multilayer nanofilms

    International Nuclear Information System (INIS)

    Short-term cell–substrate interactions of two secondary chondrocyte cell lines (human chondrosarcoma cells, canine chondrocytes) with layer-by-layer self-assembled multilayer nanofilms were investigated for a better understanding of cellular-behaviour dependence on a number of nanofilm layers. Cell–substrate interactions were studied on polyelectrolyte multilayer nanofilms (PMNs) of eleven different biomaterials. Surface characterization of PMNs performed using AFM showed increasing surface roughness with increasing number of layers for most of the biomaterials. LDH-L and MTT assays were performed on chondrosarcoma cells and canine chondrocytes, respectively. A major observation was that 10-bilayer nanofilms exhibited lesser cytotoxicity towards human chondrosarcoma cells than their 5-bilayer counterparts. In the case of canine chondrocytes, BSA enhanced cell metabolic activity with increasing number of layers, underscoring the importance of the multilayer nanofilm architecture on cellular behaviour. (paper)

  16. 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. PMID:27252405

  17. Randomised trial of proton vs. carbon ion radiation therapy in patients with low and intermediate grade chondrosarcoma of the skull base, clinical phase III study

    Directory of Open Access Journals (Sweden)

    Combs Stephanie E

    2010-11-01

    Full Text Available Background Low and intermediate grade chondrosarcomas are relative rare bone tumours. About 5-12% of all chondrosarcomas are localized in base of skull region. Low grade chondrosarcoma has a low incidence of distant metastasis but is potentially lethal disease. Therefore, local therapy is of crucial importance in the treatment of skull base chondrosarcomas. Surgical resection is the primary treatment standard. Unfortunately the late diagnosis and diagnosis at the extensive stage are common due to the slow and asymptomatic growth of the lesions. Consequently, complete resection is hindered due to close proximity to critical and hence dose limiting organs such as optic nerves, chiasm and brainstem. Adjuvant or additional radiation therapy is very important for the improvement of local control rates in the primary treatment. Proton therapy is the gold standard in the treatment of skull base chondrosarcomas. However, high-LET (linear energy transfer beams such as carbon ions theoretically offer advantages by enhanced biologic effectiveness in slow-growing tumours. Methods/Design The study is a prospective randomised active-controlled clinical phase III trial. The trial will be carried out at Heidelberger Ionenstrahl-Therapie (HIT centre as monocentric trial. Patients with skull base chondrosarcomas will be randomised to either proton or carbon ion radiation therapy. As a standard, patients will undergo non-invasive, rigid immobilization and target volume definition will be carried out based on CT and MRI data. The biologically isoeffective target dose to the PTV (planning target volume in carbon ion treatment will be 60 Gy E ± 5% and 70 Gy E ± 5% (standard dose in proton therapy respectively. The 5 year local-progression free survival (LPFS rate will be analysed as primary end point. Overall survival, progression free and metastasis free survival, patterns of recurrence, local control rate and morbidity are the secondary end points. Discussion Up

  18. Randomised trial of proton vs. carbon ion radiation therapy in patients with low and intermediate grade chondrosarcoma of the skull base, clinical phase III study

    International Nuclear Information System (INIS)

    Low and intermediate grade chondrosarcomas are relative rare bone tumours. About 5-12% of all chondrosarcomas are localized in base of skull region. Low grade chondrosarcoma has a low incidence of distant metastasis but is potentially lethal disease. Therefore, local therapy is of crucial importance in the treatment of skull base chondrosarcomas. Surgical resection is the primary treatment standard. Unfortunately the late diagnosis and diagnosis at the extensive stage are common due to the slow and asymptomatic growth of the lesions. Consequently, complete resection is hindered due to close proximity to critical and hence dose limiting organs such as optic nerves, chiasm and brainstem. Adjuvant or additional radiation therapy is very important for the improvement of local control rates in the primary treatment. Proton therapy is the gold standard in the treatment of skull base chondrosarcomas. However, high-LET (linear energy transfer) beams such as carbon ions theoretically offer advantages by enhanced biologic effectiveness in slow-growing tumours. The study is a prospective randomised active-controlled clinical phase III trial. The trial will be carried out at Heidelberger Ionenstrahl-Therapie (HIT) centre as monocentric trial. Patients with skull base chondrosarcomas will be randomised to either proton or carbon ion radiation therapy. As a standard, patients will undergo non-invasive, rigid immobilization and target volume definition will be carried out based on CT and MRI data. The biologically isoeffective target dose to the PTV (planning target volume) in carbon ion treatment will be 60 Gy E ± 5% and 70 Gy E ± 5% (standard dose) in proton therapy respectively. The 5 year local-progression free survival (LPFS) rate will be analysed as primary end point. Overall survival, progression free and metastasis free survival, patterns of recurrence, local control rate and morbidity are the secondary end points. Up to now it was impossible to compare two different

  19. Isocitrate dehydrogenase 1 mutations (IDH1) and p16/CDKN2A copy number change in conventional chondrosarcomas.

    OpenAIRE

    Amary, M. F.; Ye, H; Forbes, G.; Damato, S; Maggiani, F.; Pollock, R; Tirabosco, R.; Flanagan, A M

    2015-01-01

    To determine whether IDH1 mutations are present in primary and relapsed (local and distal) conventional central chondrosarcomas; and secondly, to assess if loss of p16/CDKN2A is associated with tumour grade progression, 102 tumour samples from 37 patients, including material from presenting and relapse events, were assessed. All wild-type cases for IDH1 R132 substitutions were also tested for IDH2 R172 and R140 alterations. The primary tumour and the most recent relapse sample were tested for...

  20. Image features of two rare mediastinal tumors: schwannoma of intrathoracic phrenic nerve and clear cell chondrosarcoma of the rib

    Institute of Scientific and Technical Information of China (English)

    Ting-Kai Leung; Chien-Jui Cheng; Chi-Ming Lee; Li-Kuo Shen; Hung-Jung Wang; Ya-Yen Chen

    2005-01-01

    @@ The current report focuses on two patients of the same age who presented similar appearances on initial anteroposterior chest images. Follow-up images showed superoanterior and superoposterior mediastinal lesions. The first patient with noninvasive cystic thymoma was suspected before surgery, while the pathologic diagnosis was intrathoracic phrenic nerve schwannoma. The second patient was with an asymmetric, dumbbell-shaped paravertebral tumor over T3 and T4 on the left side. The preoperative diagnostic images were interpreted as showing a neurogenic tumor. However, the pathologic report was cell chondrosarcoma.

  1. 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

  2. Downregulation of miR-185 and upregulation of miR-218 expression may be potential diagnostic and prognostic biomarkers of human chondrosarcoma.

    Science.gov (United States)

    Goudarzi, Peyman Karimi; Taheriazam, Afshin; Asghari, Saeid; Jamshidi, Mohammad; Shakeri, Mohammadreza; Yahaghi, Emad; Mirghasemi, Alireza

    2016-05-01

    Increasing evidence has confirmed that dysregulation of microRNAs (miRNAs) can contribute to the progression and metastasis of human tumors. Chondrosarcoma is the most common primary malignant bone tumor in adults and has no effective systemic treatment, and patients with this disease have poor survival. Thus, it is important to find new diagnostic markers and improve treatment options. In the current study, we are interested to examine the role of miR-185 and miR-218 expression in patients with chondrosarcoma using real-time PCR. Moreover, the association of the two miRNAs with clinicopathological features and prognosis was evaluated. Survival and Cox proportional hazards analyses were performed to find the association of miR-185 expression and miR-218 levels with prognosis in the patients. Our results indicated that the miR-185 expression was significantly downexpressed in clinical chondrosarcoma bone tissues compared with adjacent normal tissues (P = 0.001). MiR-218 expression level was increased in clinical chondrosarcoma bone tissue than those adjacent normal tissues (P = 0.001). Decreased expression of miR-185 showed remarkable correlation with advanced tumor stage (P = 0.019), tumor grade (P < 0.001), and distant metastasis (P = 0.001). Moreover, high expression of miR-218 was strongly correlated with advanced tumor stage (P = 0.014), tumor grade (P < 0.001), and distant metastasis (P = 0.002). Kaplan-Meier survival analysis revealed that the low miR-185 expression group and the high miR-218 expression group had remarkably shorter overall survival (log-rank test P = 0.007, P = 0.004). The multivariate Cox proportional hazards model indicated that decreased expression of miR-185 and increased expression of miR-218 (P = 0.017, P = 0.012), advanced tumor stage (P = 0.006, P = 0.012), tumor grade (P = 0.032, P = 0.016), and distant metastasis (P = 0.004, P = 0.015) were independently related

  3. Clear cell chondrosarcoma: radiographic, computed tomographic, and magnetic resonance findings in 34 patients with pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Collins, Mark S.; Koyama, Takashi; Swee, Ronald G.; Inwards, Carrie Y. [Department of Radiology, Mayo Clinic, 200 First Street SW, MN 55905, Rochester (United States)

    2003-12-01

    To describe the radiographic features of clear cell chondrosarcoma (CCCS), including the computed tomographic (CT) and magnetic resonance (MR) findings, and to correlate them with the histopathologic findings. A retrospective review was carried out of 72 patients with histopathologically confirmed CCCS. Imaging studies were available for 34 patients: conventional radiographs (n=28), CT scans (n=14), and MR images (n=15). Radiographic studies were reviewed by three radiologists who rendered a consensus opinion; the studies were correlated with the histopathologic findings. Of the 34 patients with imaging studies, 30 were male and 4 were female (mean age 38.6 years; range 11-74 years). Twenty-two lesions were in long bones (15, proximal femur; 1, distal femur; 1, proximal tibia; 5, proximal humerus) and 11 were in flat bones (5, vertebra; 4, rib; 1, scapula; 1, innominate). One lesion occurred in the tarsal navicular bone. Typically, long bone lesions were located in the epimetaphysis (19/22) and were lucent with a well-defined sclerotic margin and no cortical destruction or periosteal new bone formation. More than one-third of the long bone lesions contained matrix mineralization with a characteristic chondroid appearance. Pathologic fractures were present in six long bone lesions (4, humerus; 2, femur). Lesions in the proximal humerus were more likely to have indistinct margins (4/5) and extend into the diaphysis. Flat bone lesions were typically lytic and expansile and occasionally demonstrated areas of cortical disruption. Typically, matrix mineralization, when present, was amorphous. MR imaging, when available, was superior to conventional radiographs for demonstrating the intramedullary extent of a lesion as well as soft tissue extension. CT images better delineated the presence of cortical destruction and the character of matrix mineralization patterns. CCCS lesions were typically low signal intensity on T1-weighted images and moderately or significantly

  4. Clear cell chondrosarcoma: radiographic, computed tomographic, and magnetic resonance findings in 34 patients with pathologic correlation

    International Nuclear Information System (INIS)

    To describe the radiographic features of clear cell chondrosarcoma (CCCS), including the computed tomographic (CT) and magnetic resonance (MR) findings, and to correlate them with the histopathologic findings. A retrospective review was carried out of 72 patients with histopathologically confirmed CCCS. Imaging studies were available for 34 patients: conventional radiographs (n=28), CT scans (n=14), and MR images (n=15). Radiographic studies were reviewed by three radiologists who rendered a consensus opinion; the studies were correlated with the histopathologic findings. Of the 34 patients with imaging studies, 30 were male and 4 were female (mean age 38.6 years; range 11-74 years). Twenty-two lesions were in long bones (15, proximal femur; 1, distal femur; 1, proximal tibia; 5, proximal humerus) and 11 were in flat bones (5, vertebra; 4, rib; 1, scapula; 1, innominate). One lesion occurred in the tarsal navicular bone. Typically, long bone lesions were located in the epimetaphysis (19/22) and were lucent with a well-defined sclerotic margin and no cortical destruction or periosteal new bone formation. More than one-third of the long bone lesions contained matrix mineralization with a characteristic chondroid appearance. Pathologic fractures were present in six long bone lesions (4, humerus; 2, femur). Lesions in the proximal humerus were more likely to have indistinct margins (4/5) and extend into the diaphysis. Flat bone lesions were typically lytic and expansile and occasionally demonstrated areas of cortical disruption. Typically, matrix mineralization, when present, was amorphous. MR imaging, when available, was superior to conventional radiographs for demonstrating the intramedullary extent of a lesion as well as soft tissue extension. CT images better delineated the presence of cortical destruction and the character of matrix mineralization patterns. CCCS lesions were typically low signal intensity on T1-weighted images and moderately or significantly

  5. Common and Rare Causes of Spinal Dumbbell-Shaped Lesions Including a Chondrosarcoma That Primarily Developed in the Cervical Spine: A Case Report

    Directory of Open Access Journals (Sweden)

    Amirhossein Ghazavi

    2009-01-01

    Full Text Available Various neoplastic, vascular and developmental causes may lead to spinal neural foraminal widening. Schwanomas and neurofibromas are the most common causes of spinal dumbbell-shaped lesions. Occasionally, some other slow-growing tumors may cause neural foraminal widening."nWe report an exceptional case of a spinal chondrosarcoma of the neural arcus of C5. The lesion passed through the C4-C5 foramen, producing a dumbbell mass.

  6. 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.

  7. Postoperative Spot-Scanning Proton Radiation Therapy for Chordoma and Chondrosarcoma in Children and Adolescents: Initial Experience at Paul Scherrer Institute

    International Nuclear Information System (INIS)

    Purpose: To evaluate postoperative spot-scanning proton radiation therapy (PT) and intensity-modulated PT (IMPT) for chordoma and chondrosarcoma in pediatric patients. Methods and Materials: Between 2000 and 2005, 10 patients (six male patients, four female patients; six chordomas, four chondrosarcomas), aged 10-20 years (median, 16 years), were treated at our institute. Tumor sites were in the brain (one case), skull base (five cases), cervical (three cases), and lumbar spine (one case). Three children had complete resections. In seven children, resection was incomplete, leaving residual tumor behind (range, 2.3-46.3 mL). PT was delivered using spot scanning, with (three patients) or without (seven patients) IMPT. Total dose was 74.0 cobalt Gray equivalents (CGE) for chordoma, and 63.2-68.0 CGE for chondrosarcoma (median, 66.0), depending on histopathological grading and whether the patient had concurrent chemotherapy. Results: Median follow-up time was 36 months (range, 8-77 months). Radiation treatment was well tolerated. All patients remained failure-free at their last follow-up. Late adverse events were reported in three patients and were mild (neurosensory in one patient; alopecia and hypoaccusis in one patient) to moderate (one patient, Grade 2 pituitary insufficiency). Conclusions: Postoperative spot-scanning PT, delivered in combination with and without IMPT, for chordoma and chondrosarcoma in children and adolescents was tolerated without unacceptable adverse event and initial outcome is perfectly satisfactory in this small cohort. Longer follow-up time and larger cohort are needed to more fully assess tumor control, adverse events, as well as functional and cosmetic outcome

  8. 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. PMID:25907264

  9. 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

  10. Imaging of chondrosarcoma with histopathological and prognostic correlation. An analysis of 49 cases mainly based on plain film radiography

    Energy Technology Data Exchange (ETDEWEB)

    Jurik, A.G. [Centre for Bone and Soft Tissue Tumours, Univ. Hospital, Aarhus (Denmark); Jensen, O. [Centre for Bone and Soft Tissue Tumours, Univ. Hospital, Aarhus (Denmark); Keller, J. [Centre for Bone and Soft Tissue Tumours, Univ. Hospital, Aarhus (Denmark); Nielsen, O.S. [Centre for Bone and Soft Tissue Tumours, Univ. Hospital, Aarhus (Denmark); Lundorf, E. [Centre for Bone and Soft Tissue Tumours, Univ. Hospital, Aarhus (Denmark); Daugaard, S. [Centre for Bone and Soft Tissue Tumours, Univ. Hospital, Aarhus (Denmark); Sneppen, O. [Centre for Bone and Soft Tissue Tumours, Univ. Hospital, Aarhus (Denmark)

    1995-11-01

    49 consecutive patients seen during an 11-year-period were analysed, including re-evaluation of their radiographic and histopathologic material. Forty-two patients had radiographic changes typical for cartilaginous tumours, in 37 with malignant stigmata. Seven patients had malignant changes not typical for chondrosarcoma. By histopathologic grading 16 patients had grade I, 17 grade II and 16 grade III tumours. Six of the grade II-III tumours were histopathologic variants (mesenchymal, dedifferentiated or myxoid chondrosarcomas). Surgical removal of the tumour was performed in 42 patients, 41 of whom were followed up for 0.4-11.4 years (median 3.8 years). Local recurrence occurred in 7 patients, and 11 patients developed metastases. Ten patients, 4 with local recurrence and metastases, and 6 with metastases only were dead at the end of the follow-up. The actuarial 5-year overall survival rate was 64%. The occurrence of local recurrence, metastases and death was found to be related to the histopathologic grades II and III. Atypical radiographic features only occurred in grade II-III tumours and were related to metastases and death, but not to local recurrence. (orig./MG) [Deutsch] 49 Patienten wurden waehrend eines Zeitraums von 11 Jahren analysiert, einschliesslich einer erneuten Bewertung ihrer radiologischen und histopathologischen Befunde. Bei 42 Patienten wurden radiologische Veraenderungen festgestellt, die fuer chondrogene Knochentumoren typisch sind, davon 37 mit boesartigen Anzeichen. Bei sieben Patienten bestanden boesartige Veraenderungen, die jedoch nicht typisch fuer ein Chondrosarkom waren. Das Grading bzw. die pathologische Stadienbestimmung aufgrund histologischer Kriterien ergab bei 16 Patienten den Malignitaetsgrad I, bei 17 II und bei 16 III. Sechs der Tumoren der Grade II-III waren histopathologische Varianten (mesenchymale Chondrosarkome, Atypie oder myxoide Chondrosarkome). Bei 42 Patienten wurde der Tumor chirurgisch entfernt und der

  11. Non-Hodgkin lymphoma as a secondary malignancy in a 9-year old boy after previous therapy for chondrosarcoma - a case report

    International Nuclear Information System (INIS)

    Parallel with achieving improved survivals, the number of patients who have been successfully treated for one cancer and develop a secondary malignant neoplasm (SMN) increases. Despite SMN-orientated research the exact influence of anticancer therapy on their development is still unclear The aim of this paper is to present the case report of a 9-year old boy with chondrosarcoma of the left fibula who, after treatment for the primary tumour, developed a secondary malignancy - B-cell lymphoma involving the skeletal system. (authors)

  12. Clinicopathologic and radiologic features of extraskeletal myxoid chondrosarcoma: a retrospective study of 40 Chinese cases with literature review.

    Science.gov (United States)

    Shao, Rui; Lao, I Weng; Wang, Lei; Yu, Lin; Wang, Jian; Fan, Qinhe

    2016-08-01

    The aim of this study is to describe the clinicopathologic and radiologic features of 40 cases of extraskeletal myxoid chondrosarcoma (EMC) from China. There were 25 males and 15 females (sex ratio, 1.7:1). Apart from an adolescent, all patients were adults with a median age of 49years. Twenty-four tumors (60%) occurred in the lower limb and limb girdles, especially the thigh, followed by the upper limb and limb girdles (20%) and trunk (10%). Other less commonly involved locations included the head and neck, sacrococcygeal region, and perineum. Tumors ranged in size from 1.5 to 19cm (mean, 7cm). By radiology, they appeared as hypoattenuated or isoattenuated masses on computed tomography with hyperintense signal on T2-weighted magnetic resonance imaging. Intralesional hypointense septa were present in most cases. Of the 40 tumors, 30 belonged to the classic subtype, whereas 9 cases were cellular, and 1 case had a rhabdoid phenotype. Tumor cells showed variable expression of synaptophysin (36%), S-100 protein (29%), epithelial membrane antigen (11%), and neuron-specific enolase (7%). Ki-67 index was remarkably higher in the cellular variant (mean, 30%). EWSR1-related rearrangement was detected in 12 of 14 cases tested by fluorescence in situ hybridization using break-apart probes. The overall 5- and 7-year survival was 71% and 60%, respectively. Awareness of the imaging features may help pathologists in the diagnosis of EMC. Fluorescence in situ hybridization also serves as a useful diagnostic tool for EMC, especially in the distinction from its mimics. PMID:27402218

  13. IDH1 and IDH2 mutations are frequent events in central chondrosarcoma and central and periosteal chondromas but not in other mesenchymal tumours.

    Science.gov (United States)

    Amary, M Fernanda; Bacsi, Krisztian; Maggiani, Francesca; Damato, Stephen; Halai, Dina; Berisha, Fitim; Pollock, Robin; O'Donnell, Paul; Grigoriadis, Anita; Diss, Tim; Eskandarpour, Malihe; Presneau, Nadège; Hogendoorn, Pancras Cw; Futreal, Andrew; Tirabosco, Roberto; Flanagan, Adrienne M

    2011-07-01

    Somatic mutations in isocitrate dehydrogenase 1 (IDH1) and IDH2 occur in gliomas and acute myeloid leukaemia (AML). Since patients with multiple enchondromas have occasionally been reported to have these conditions, we hypothesized that the same mutations would occur in cartilaginous neoplasms. Approximately 1200 mesenchymal tumours, including 220 cartilaginous tumours, 222 osteosarcomas and another ∼750 bone and soft tissue tumours, were screened for IDH1 R132 mutations, using Sequenom(®) mass spectrometry. Cartilaginous tumours and chondroblastic osteosarcomas, wild-type for IDH1 R132, were analysed for IDH2 (R172, R140) mutations. Validation was performed by capillary sequencing and restriction enzyme digestion. Heterozygous somatic IDH1/IDH2 mutations, which result in the production of a potential oncometabolite, 2-hydroxyglutarate, were only detected in central and periosteal cartilaginous tumours, and were found in at least 56% of these, ∼40% of which were represented by R132C. IDH1 R132H mutations were confirmed by immunoreactivity for this mutant allele. The ratio of IDH1:IDH2 mutation was 10.6 : 1. No IDH2 R140 mutations were detected. Mutations were detected in enchondromas through to conventional central and dedifferentiated chondrosarcomas, in patients with both solitary and multiple neoplasms. No germline mutations were detected. No mutations were detected in peripheral chondrosarcomas and osteochondromas. In conclusion, IDH1 and IDH2 mutations represent the first common genetic abnormalities to be identified in conventional central and periosteal cartilaginous tumours. As in gliomas and AML, the mutations appear to occur early in tumourigenesis. We speculate that a mosaic pattern of IDH-mutation-bearing cells explains the reports of diverse tumours (gliomas, AML, multiple cartilaginous neoplasms, haemangiomas) occurring in the same patient. PMID:21598255

  14. Temporal lobe (TL) damage following surgery and high-dose photon and proton irradiation in 96 patients affected by chordomas and chondrosarcomas of the base of the skull

    International Nuclear Information System (INIS)

    Purpose: To determine the temporal lobe (TL) damage rate in 96 patients treated with high-dose proton and photon irradiation for chordomas and chondrosarcomas of the base of the skull. Methods and Materials: The records of 96 consecutive patients treated at Massachusetts General Hospital (MGH) and Harvard Cyclotron Laboratory (HCL) between June 1984 and 1993, for chordomas and chondrosarcomas of the base of the skull were reviewed. All the patients had undergone some degree of resection of the tumor prior to radiation therapy. Seventy-five patients were classified as 'primary tumors' and 21 as recurrent or regrowing tumors after one or more surgical procedures. All the patients were randomized to receive 66.6 or 72 cobalt Gray equivalent (CGE) on a prospective dose-searching study by proton and photon irradiation (Radiation Therapy Oncology Group no. 85-26) with conventional fractionation (1.8 CGE/day, 5 fractions/week). All treatments were planned using the three-dimensional (3D) planning system developed at the Massachusetts General Hospital, and the dose was delivered using opposed lateral fields for the photon component and a noncoplanar isocentric technique for the proton component. Clinical symptoms of TL damage were classified into 4 grades. Computerized tomography (CT) and magnetic resonance imaging (MRI) scans were evaluated for white matter changes. Abnormalities associated with persistent or recurrent tumor were distinguished from radiation-induced changes. TLs were delineated on the original scans of the 10 patients with damage and those of a group of 33 patients with no clinical or MRI evidence of injury. Dose distributions were calculated and dose-volume histograms were obtained for these patients. Results: Of the patients, 10 developed TL damage, with bilateral injury in 2 and unilateral injury in 8. The cumulative TL damage incidence at 2 and 5 years was 7.6 and 13.2%, respectively. The MRI areas suggestive of TL damage were always separated from

  15. 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.

  16. Suppression of chondrosarcoma cells by 15-deoxy-Δ12,14-prostaglandin J2 is associated with altered expression of Bax/Bcl-xL and p21

    International Nuclear Information System (INIS)

    We previously reported that 15-deoxy-Δ12,14-prostaglandin J2 (15d-PGJ2), the most potent agonist for peroxisome proliferator-activated receptor γ (PPARγ), induces apoptosis of human chondrosarcoma cell line OUMS-27. The current study aimed to explore the mechanism of 15d-PGJ2-induced apoptosis and inhibition of cell proliferation in OUMS-27 cells. The preliminary results of cDNA microarray analysis showed the down-regulation of anti-apoptotic Bcl-xL and up-regulation of pro-apoptotic Bax in the process of 15d-PGJ2-induced apoptosis. These changes were further confirmed at mRNA and protein levels by RT-PCR and Western blot analysis, respectively. Among cyclin-dependent kinase inhibitors, p21 was induced and up-regulated by 15d-PGJ2, but p16 and p27 were not changed, suggesting that the involvement of p21 in inhibition of cell proliferation. Activation of caspase-3 by 15d-PGJ2 was partly, but not completely, blocked by PPARγ antagonist (GW9662) suggesting the 15d-PGJ2 exerted its effect by PPARγ-dependent and -independent pathways. Interestingly, immunohistochemical study on human chondrosarcoma samples revealed that Bcl-xL is frequently expressed by tumor cells. The results of the current study suggest that the potential ability of 15d-PGJ2 in regulation of cell cycle and inhibition of Bcl-xL expression might be beneficial in the development of novel pharmacological agents for chondrosarcoma

  17. CT diagnosis of larynx chondrosarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Holsbeeck, M.T. van; Oyen, R.H.; Wilms, G.E.; Baert, A.L.; Stessens, R.C.

    1985-11-01

    The authors describe a patient with a palpable anterior neck mass and a submucosal swelling at the level of the vocal cords. Computed tomography (CT) revealed a tumour of the laryngeal skeleton. Pathologic diagnosis was a moderately well differentiated chondrosacroma.

  18. Relationship between surgical procedure and outcome for patients with grade I chondrosarcomas Evolução dos pacientes com condrossarcoma grau I em relação ao tipo de tratamento cirúrgico

    Directory of Open Access Journals (Sweden)

    Maurício Etchebehere

    2005-04-01

    Full Text Available PURPOSE: To evaluate the oncological outcome of patients with grade I chondrosarcomas according to the type of surgical treatment performed, since there is still controversy regarding the need for aggressive resections to reach a successful outcome. MATERIALS AND METHODS: The records of 23 patients with grade I chondrosarcomas were reviewed. The mean age was 38.4 years, ranging from 11 to 70 years; 52% were men and 48% were women. The femur was the site of 13 tumors. The tumors were staged as IA (17, 74% and IB (6, 26%. Regarding tumor location, 74% (17 were medullary, 22% (5 were peripheral, and 4% (1 was indeterminate. Tumor size ranged from 2 to 25 cm, mean 7.9 cm. Regarding the surgical procedure, 11 patients underwent intralesional resection, 9 patients underwent wide resection, and 3 underwent radical resection. The follow-up period ranged from 24 to 192 months. RESULTS: None of the patients developed local recurrence or metastases; 7 patients had other general complications. CONCLUSIONS: This data supports the use of less aggressive procedures for treatment of low-grade chondrosarcomas.OBJETIVO: Avaliar a evolução oncológica de portadores de condrossarcomas grau I de acordo com o tipo de tratamento cirúrgico efetuado. Existe controvérsia em relação à necessidade de ressecções agressivas para obtenção de uma evolução clínica favorável. MATERIAIS E MÉTODOS: Os prontuários de 23 portadores de condrossarcoma grau I foram analisados. A idade dos pacientes variou de 11 a 70 anos com média de 38,4 anos, 52% eram homens e 48% mulheres. O local mais acometido foi o fêmur com 13 pacientes. Dezessete lesões (74% foram classificadas como IA e seis (26% como IB. Setenta e quatro por cento dos tumores eram medulares, 22% eram periféricas e uma lesão indeterminada. O tamanho dos tumores variou de 2 a 25 cm, média de 7,9 cm. Onze pacientes foram submetidos a ressecção intralesional, nove a ressecção ampla e três a ressec

  19. Intralesional curettage and electrocauterization for the treatment of grade IA chondrosarcoma of long bones%病灶残腔灭活治疗长骨ⅠA期软骨肉瘤的安全性观察

    Institute of Scientific and Technical Information of China (English)

    曲华毅; 郭卫; 杨荣利; 燕太强; 李大森; 唐顺; 杨毅

    2016-01-01

    Objective To explore the recurrence rate, the complications and functional status of 25 patients with grade I chondrosarcoma of long bones treated by intralesional curettage and electrocauterization, and to determine the feasibility and effi⁃cacy of this method for grade IA chondrosarcoma of long bones. Methods Twenty⁃five eligible patients treated in our hospital from May 2003 to December 2011, were collected in this study with a mean age of 49 years (range, 28-72 years). According to En⁃neking staging system, all the lesions were staged as IA. Patients received surgery of the lesion before were excluded. The involved bones were femur (13 patients), tibia (4 patients), and humerus (8 patients). During the operation, a large elliptical cortical window about the size of the longest dimension of the lesion was made to ensure the thorough exposure of the lesion and avoid inadequate curettage. The lesion was curettaged thoroughly after the cortical window was made, then the high speed bur drill was applied to clear away a thin layer of the reactive bone shell. After a thorough lavage of the cavity, electrocauterization was done alongside the cavity wall slowly twice. Allograft or artificial bone was used to fill in the cavity to enhance bone healing. If mechanical property of the long bone was endangered by the cortical window and the surgical procedure, plate and screws were applied to strengthen the bone to avoid post⁃operative fracture. Results All the 25 patients were followed up regularly. The period of follow⁃up was from 38 months to 142 months, with a mean time of 80 months. Calcification was observed clearly in all the lesions. The length of the le⁃sions varied from 4 cm to 11 cm, with an average length of 7 cm. Nineteen patients received a plate and screws fixation because the mechanical property of the affected bone, while the rest received no internal fixation. Deep infection occurred in 1 patient (4%, 1/25) two months after operation. Thorough

  20. Condrosarcoma Mesenquimal Sinonasal Grado Tres y Carcinosarcoma de Glándula Mamaria de un Canino: Estudios Clínico e Histopatológico Sinunasal Mesenchymal Chondrosarcoma Grade 3 and Mammary Gland Carcinosarcoma of Canine: Clinical and Histopathology Study

    Directory of Open Access Journals (Sweden)

    Anita Roque Rodríguez

    2008-12-01

    , come a 9 years oíd Chow Chow female canine, whose consultation reason was a bilateral epistaxis occurred 4 months ago, sudden loss of the visión and a mass in the nasal región of 2cm of diameter approximately, according to the report of the owner, the mass showed up 1 month the day of the consultation. To the clinical exam they were the following abnormal discoveries. Hirsute hair, opaque, presented a subdermic mass approximately in the nasal región superior of 2 cm. diameter that grew to 5 cm. diameter after 1 month of the consultation, located in the nasal región superior and zigomatic región, of hard consistency, the animal had dysnea, bilateral mydriasis, the bilateral corneal reflection was negative, increase of the infraocular pressure of the right eye, the realized test of obstacles in the clinic was negative, that which showed us a blindness. Besides a complete clinical exploration and taking of the advantages of the cytology, she was carried out an aspired with fine needle of different áreas of the masses so much neoplastic of the nose and of the región of the mammary gland, being obtained two types of samples. The cytology was evaluated to define origin of the tumors of the process. She was carried out biopsy for incisión of the sinunosal región being diagnosed wicked tumor mesenchymal Chondrosarcoma sinunasal that commits bone. After having carried out the autopsy they took samples for histopathology. Being diagnosed mesenchymal chondrosarcoma sinunasal grade 3, with metástasis to lung, and a carcinosarcoma of mammary gland.

  1. 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

    Clinical and in vitro studies have demonstrated that fluoroquinolones are toxic to chondrocytes; however, the exact mechanism of fluoroquinolone arthropathy is unknown. We investigated the toxicity of ciprofloxacin on normal cartilage and on cartilaginous tumors. Normal human cartilage, enchondroma...

  2. Disease: H00053 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available S, Hashimoto H. Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and molecula..., Gaffey TA, Rock MG, Nascimento AG. Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical

  3. Identification of a Novel, Recurrent HEY1-NCOA2 Fusion in Mesenchymal Chondrosarcoma based on a Genome-wide Screen of Exon-level Expression Data

    OpenAIRE

    Wang, Lu; Motoi, Toru; Khanin, Raya; Olshen, Adam,; Mertens, Fredrik; Bridge, Julia; Dal Cin, Paola; Antonescu, Cristina; Singer, Sam; Hameed, Meera; Bovee, Judith; Hogendoorn, Pancras C W; Socci, Nicholas; Ladanyi, Marc

    2011-01-01

    Cancer gene fusions that encode a chimeric protein are often characterized by an intragenic discontinuity in the RNA expression levels of the exons that are 5′ or 3′ to the fusion point in one or both of the fusion partners due to differences in the levels of activation of their respective promoters. Based on this, we developed an unbiased, genome-wide bioinformatic screen for gene fusions using Affymetrix Exon array expression data. Using a training set of 46 samples with different known gen...

  4. What Is Bone Cancer?

    Science.gov (United States)

    ... our document called Osteosarcoma . Chondrosarcoma: Chondrosarcoma (KON-droh-sar-KOH-muh) is a cancer of cartilage cells. ... AdditionalResources Other Resources and References Cancer Information Cancer Basics Cancer Prevention & Detection Signs & Symptoms of Cancer Treatments & ...

  5. Condrossarcoma em paciente com osteocondromatose múltipla: relato de caso e revisão da literatura Chondrosarcoma in a patient with multiple osteochondromatosis: a case report and review of the literature

    OpenAIRE

    Anna Caroline Nobre Gomes; Cláudio Régis Sampaio Silveira; Roberto Guido Santos Paiva; Antônio Gilson Monte Aragão Jr.; José Roberto Cavalcante Castro Jr.

    2006-01-01

    Os autores relatam um caso de condrossarcoma em uma paciente de 14 anos portadora de osteocondromatose múltipla, que procurou assistência médica com a queixa de tumoração na coxa esquerda. Cortes tomográficos do membro afetado evidenciaram lesão osteodestrutiva no terço superior do fêmur esquerdo. O diagnóstico foi feito através da revisão da lâmina do serviço de origem da paciente, confirmando o achado de condrossarcoma grau I em lesão condromatosa. Foi realizada desarticulação do colo femor...

  6. Cartilage tumour progression is characterized by an increased expression of heparan sulphate 6O-sulphation-modifying enzymes.

    Science.gov (United States)

    Waaijer, Cathelijn J F; de Andrea, Carlos E; Hamilton, Andrew; van Oosterwijk, Jolieke G; Stringer, Sally E; Bovée, Judith V M G

    2012-10-01

    Chondrosarcomas are malignant cartilage-forming tumours that can arise centrally (in the medulla) or peripherally (at the surface) of the bone. They are classified into three histological grades which correspond to the clinical severity. Previous studies by our group have shown altered signal transduction of the fibroblast growth factor and Wnt signalling pathways during peripheral chondrosarcoma progression. Heparan sulphate (HS) is a glycosaminoglycan that facilitates receptor binding of multiple growth factors, in which the sulphation of 6O position plays a pivotal role. 6O-Sulphation occurs through three HS 6O-sulphotransferases (HS6ST1-3) and is fine-tuned by two endosulphatases (SULF1-2) that remove 6O-sulphate groups. We have investigated whether the expression of HS6STs and SULFs changes during chondrosarcoma progression and have determined 6O-sulphation levels in two chondrosarcoma cell lines. Immunohistochemistry on tissue microarrays of chondrosarcomas showed that HS6ST3 and SULF1 were highly expressed in most chondrosarcomas, whereas SULF2 expression was absent in most cases. HS6ST1 and HS6ST2 expression are significantly increased during chondrosarcoma progression, which suggest that 6O-sulphation is increased during progression. This was confirmed in one grade III chondrosarcoma cell line, which showed a dramatically increased 6O-sulphation compared to an articular chondrocyte cell line by HPLC; another cell line showed an increased expression of one 6O-sulphated HS disaccharide. In conclusion, our results show increased HS6ST1 and HS6ST2 expression during chondrosarcoma progression and increased HS 6O-sulphation in vitro. As 6O-sulphation plays an important role in signal transduction, altered HS6ST expression might be associated with changes in signal transduction pathways in chondrosarcoma progression. PMID:22903264

  7. Metastasis-associated lung adenocarcinoma transcript 1 promotes the proliferation of chondro­sarcoma cell via activating Notch-1 signaling pathway

    Directory of Open Access Journals (Sweden)

    Xu FQ

    2016-04-01

    Full Text Available Fengqin Xu,1,* Zhi-qiang Zhang,2,* Yong-chao Fang,2 Xiao-lei Li,2 Yu Sun,2 Chuan-zhi Xiong,2 Lian-qi Yan,2 Qiang Wang2 1Department of Orthopaedics, Hongquan Hospital, 2Department of Orthopaedics, Subei People’s Hospital, Yangzhou, Jiangsu Province, People’s Republic of China *These authors contributed equally to this work Background: Metastasis-associated lung adenocarcinoma transcript 1 (MALAT-1 is identified to be overexpressed in several cancers. However, the role of MALAT-1 in chondrosarcoma is poorly understood.Methods: The expression of MALAT-1 and Notch-1 signaling pathway was detected in chondrosarcoma tissues and chondrosarcoma cells by quantitative real-time polymerase chain reaction (qRT-PCR and Western blot. 3-(4,5-Dimethyl-2-thiazolyl-2,5-diphenyl-2-H-tetrazolium bromide (MTT assay was performed to examine the cell viability of chondrosarcoma cells transfected with si-MALAT-1 or pcDNA-MALAT-1. Then the expression of Notch-1 signaling pathway was detected when MALAT-1 was upregulated or downregulated in chondrosarcoma cells. A subcutaneous chondrosarcoma cells xenograft model was used to confirm the effect of MALAT-1 on tumor growth in vivo.Results: We found the increased expression of MALAT-1 and Notch-1 signaling pathway in chondrosarcoma tissue and cells. MALAT-1 promoted the proliferation of chondrosarcoma cells. In addition, MALAT-1 activated the Notch-1 signaling pathway at posttranscriptional level in chondrosarcoma cells. Meanwhile, overexpression of Notch-1 reversed the effect of si-MALAT-1 on the proliferation of chondrosarcoma cells. Finally, we found that MALAT-1 promoted the tumor growth in a subcutaneous chondrosarcoma cells xenograft model, which confirmed the promoted effect of MALAT-1 on the tumor growth in vivo.Conclusion: Taken together, our study demonstrated that MALAT-1 promoted the proliferation of chondrosarcoma cell via activating Notch-1 signaling pathway. Keywords: MALAT-1, cell proliferation

  8. 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

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

    Science.gov (United States)

    Cintra, Francisco Fontes; Etchebehere, Mauricio; Gonçalves, José Carlos Barbi; Cassone, Alejandro Enzo; Amstalden, Eliane Maria Ingrid

    2011-01-01

    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 pro-angiogenic 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: These

  10. CT and MRI of the skull base, including the cranial nerves

    International Nuclear Information System (INIS)

    Some considerations about nuclear magnetic resonance and computerized tomography, essential for examining skull base lesions are treated here, including the cranial nerves. Neoplasms such as meningiomas, adenomas, chordomas, chondrosarcomas and others tumors are also cited, mentioning some commentaries. (author)

  11. Perioperative Management of a Jehovah's Witness Presenting for Skull Base Surgery

    OpenAIRE

    Chaney, Mark A.; Jellish, W Scott; Leonetti, John P.

    1996-01-01

    A 22-year-old, otherwise healthy, female Jehovah's Witness underwent resection of a midline skull base chondrosarcoma which had been detected after a work-up for headache and diplopia. After bilateral maxillectomies, ethmoidectomies, and a sphenoidectomy, the patient's chondrosarcoma was resected. Despite proper anesthetic management and meticulous hemostasis, significant intraoperative blood loss occurred. The initial postoperative hemoglobin level was 2.3 gm/dL. The clinical concerns pertai...

  12. 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.

  13. Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-Rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery

    Science.gov (United States)

    2016-08-03

    Adult Fibrosarcoma; Alveolar Soft Part Sarcoma; Angiomatoid Fibrous Histiocytoma; Atypical Fibroxanthoma; Clear Cell Sarcoma of Soft Tissue; Epithelioid Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma; Extraskeletal Myxoid Chondrosarcoma; Extraskeletal Osteosarcoma; Fibrohistiocytic Neoplasm; Glomus Tumor of the Skin; Inflammatory Myofibroblastic Tumor; Intimal Sarcoma; Leiomyosarcoma; Liposarcoma; Low Grade Fibromyxoid Sarcoma; Low Grade Myofibroblastic Sarcoma; Malignant Cutaneous Granular Cell Tumor; Malignant Peripheral Nerve Sheath Tumor; Malignant Triton Tumor; Mesenchymal Chondrosarcoma; Myxofibrosarcoma; Myxoid Chondrosarcoma; Myxoinflammatory Fibroblastic Sarcoma; Nerve Sheath Neoplasm; PEComa; Pericytic Neoplasm; Plexiform Fibrohistiocytic Tumor; Sclerosing Epithelioid Fibrosarcoma; Stage IB Soft Tissue Sarcoma; Stage IIB Soft Tissue Sarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Synovial Sarcoma; Undifferentiated (Embryonal) Sarcoma; Undifferentiated High Grade Pleomorphic Sarcoma of Bone

  14. 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.

  15. Perioperative Management of a Jehovah's Witness Presenting for Skull Base Surgery.

    Science.gov (United States)

    Chaney, M A; Jellish, W S; Leonetti, J P

    1996-01-01

    A 22-year-old, otherwise healthy, female Jehovah's Witness underwent resection of a midline skull base chondrosarcoma which had been detected after a work-up for headache and diplopia. After bilateral maxillectomies, ethmoidectomies, and a sphenoidectomy, the patient's chondrosarcoma was resected. Despite proper anesthetic management and meticulous hemostasis, significant intraoperative blood loss occurred. The initial postoperative hemoglobin level was 2.3 gm/dL. The clinical concerns pertaining to the perioperative management of the Jehovah's Witness are discussed along with the patient's course and management. PMID:17170989

  16. Chondromyxoid fibroma of sphenoid sinus with unusual calcifications: case report with literature review.

    Science.gov (United States)

    Morris, Luc G T; Rihani, Jordan; Lebowitz, Richard A; Wang, Beverly Y

    2009-06-01

    Chondromyxoid fibroma (CMF) is a rare benign primary tumor which usually affects the metaphyses of the long bone of the lower extremities in childhood and young adults. Rarely, CMF occurs in the skull base and parasinuses, which may be difficult to distinguish from chondrosarcoma or chordoma and other tumors in the head. It is composed of chondroid, myxoid, and fibrous tissue growth in a lobular pattern, infrequently with calcifications. We report one case of CMF involving the sphenoid sinus mimicking a chondrosarcoma. The tumor mass showed calcifications on images and histology. PMID:19644549

  17. 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.)

  18. Bone scintigraphy in the initial evaluation of dogs with primary bone tumors

    International Nuclear Information System (INIS)

    Bone scintigraphy was performed as part of an initial diagnostic evaluation of 70 dogs admitted with primary bone tumors during a 2-year period. Tumors involved major long bones of the appendicular skeleton and included 62 osteosarcomas, 6 fibrosarcomas, and 2 chondrosarcomas. All dogs were free of radiographically detectable pulmonary metastases. Bone scintigraphy was not of value in distinguishing among various types of primary tumors. One dog with an ulnar chondrosarcoma had a scintigraphically detectable occult osseous metastasis or synchronous primary tumor, and 1 dog with osteosarcoma had a scintigraphically detectable lymph node metastasis. Pulmonary metastases were not detected scintigraphically. Of the 70 dogs, 44.3% had areas of increased isotope uptake associated with nonneoplastic disease processes

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

    Science.gov (United States)

    2015-05-22

    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

  20. Melatonin Cytotoxicity Is Associated to Warburg Effect Inhibition in Ewing Sarcoma Cells

    Science.gov (United States)

    Sanchez-Sanchez, Ana M.; Antolin, Isaac; Puente-Moncada, Noelia; Suarez, Santos; Gomez-Lobo, Marina; Rodriguez, Carmen; Martin, Vanesa

    2015-01-01

    Melatonin kills or inhibits the proliferation of different cancer cell types, and this is associated with an increase or a decrease in reactive oxygen species, respectively. Intracellular oxidants originate mainly from oxidative metabolism, and cancer cells frequently show alterations in this metabolic pathway, such as the Warburg effect (aerobic glycolysis). Thus, we hypothesized that melatonin could also regulate differentially oxidative metabolism in cells where it is cytotoxic (Ewing sarcoma cells) and in cells where it inhibits proliferation (chondrosarcoma cells). Ewing sarcoma cells but not chondrosarcoma cells showed a metabolic profile consistent with aerobic glycolysis, i.e. increased glucose uptake, LDH activity, lactate production and HIF-1α activation. Melatonin reversed Ewing sarcoma metabolic profile and this effect was associated with its cytotoxicity. The differential regulation of metabolism by melatonin could explain why the hormone is harmless for a wide spectrum of normal and only a few tumoral cells, while it kills specific tumor cell types. PMID:26252771

  1. Expression and significance of TRAIL and NF-kB in osteosarcoma

    International Nuclear Information System (INIS)

    To investigate the relationship between expressions of TRAIL, NF-kB and cell proliferation in human osteosarcomas, the expressions of TRAIL and NF-kB in 16 cases of osteosarcoma, 5 cases of giant cell tumor of bone and 6 cases of chondrosarcoma were studied by flow cytometry. The expressions of TRAIL and NF-kB in osteosarcomas of different differentiation states were higher than those in other two kinds of tumors significantly in our study(P0.05). The expressions of TRAIL and NF-kB in chondrosarcoma and giant cell tumor of bone were not different significantly(P>0.05). The higher expression of TRAIL in osteosarcoma with different differentiation states could not induce apoptosis because of the higher expression of NF-kB. NF-kB may restrain the apoptosis of tumor cells by regulating the NF-kB- induced apoptosis path way in osteosarcoma. (authors)

  2. An unusual myocardial infarction

    OpenAIRE

    Di Michele, Sara; MIRABELLI, FRANCESCA; Galzerano, Domenico; Mankad, Sunil

    2014-01-01

    Summary We present a 74-year-old male with a chondrosarcoma, who presented with chest pain. The history, electrocardiogram (ECG), and biomarkers established the diagnosis of myocardial infarction (MI); angiography did not show coronary atherosclerosis and, both initial transthoracic echocardiogram and chest computed tomography (CT), did not demonstrate any cardiac abnormalities. A second echocardiogram following a routine ECG showed presence of a mass involving the right ventricle and the car...

  3. Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma

    Science.gov (United States)

    2016-04-05

    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 Undifferentiated Pleomorphic Sarcoma; Malignant Adult Hemangiopericytoma; Recurrent Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  4. Congenital chondromyxoid fibroma of the ethmoid: case report

    International Nuclear Information System (INIS)

    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.)

  5. Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma

    Science.gov (United States)

    2014-08-26

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  6. Complete transurethral bladder eversion 3 months after hemipelvectomy.

    Science.gov (United States)

    Lowe, Gregory; Mandalapu, Subbarao; Gilleran, Jason

    2010-02-01

    A 46-year-old white female underwent a left hemipelvectomy for chondrosarcoma. She presented with total incontinence and a bulging vaginal mass. Exam confirmed complete transurethral bladder eversion that was addressed with transvaginal multilayer bladder neck closure and suprapubic tube placement. Eventually she underwent abdominal hysterectomy, mesh sacral colpopexy, and catheterizable stoma creation. Patient is continent of urine 3 months postoperatively. We present the first reported case of bladder eversion after hemipelvectomy and propose possible pathophysiologic mechanisms. PMID:19629370

  7. Primary cilia attenuate hedgehog signalling in neoplastic chondrocytes

    OpenAIRE

    Ho, L.; Ali, S A; Al-Jazrawe, M; R. Kandel; Wunder, J S; Alman, B. A.

    2012-01-01

    Primary cilia can act as either a negative or positive regulator of the hedgehog (Hh) signaling pathway. Many cartilage tumors are characterized by abnormal activation of the Hh pathway. Here, we report that the presence of primary cilia occurs at a low frequency (12.4%) in neoplastic chondrocytes from malignant human chondrosarcomas, compared with chondrocytes from normal articular cartilage (67.7%). To determine the function of primary cilia in cartilaginous neoplasia, we studied benign car...

  8. Primary malignant bone tumors: diagnosis, radiological appearance and therapy

    International Nuclear Information System (INIS)

    Diagnosis, concepts of therapy and prognosis of primary malignant bone tumors require intensive cooperation between orthopedic surgeons, radiologists and pathologists with special knowledge in oncology. The present paper demonstrates relevant topics of diagnosis, radiological appearance and therapy of the osteosarcoma, Ewing's sarcoma, chondrosarcoma and malignant fibrous histiocytoma based on the material of the Vienna Bone Tumor Registry and more than 35 years of cooperation between the Institute of Pathology and Anatomy and the Department of Orthopedics at the University of Vienna. (orig.)

  9. Consolidation of massive bone allografts in limb-preserving operations for bone tumours

    OpenAIRE

    San-Julian, M.; Leyes, M.; Mora, G. (Gonzalo); Cañadell, J.M. (J. M.)

    1995-01-01

    This study analysed the influence of several factors affecting the consolidation time of 83 massive bone allografts in 79 patients with malignant bone tumours: osteosarcoma 57; Ewing's sarcoma 8; malignant fibrous histiocytoma 3; chondrosarcoma 4; fibrosarcoma 5; and giant cell tumours 2. The mean age of the patients was 19 years and the mean length of the allografts was 18 cm. The minimum follow up was for 12 months. The mean consolidation time for metaphyseal and diaphyseal osteotomies was ...

  10. Scanning electron microscopy of primary bone tumors

    International Nuclear Information System (INIS)

    Critical-point-drying of tumor tissue fixed in a glutaraldehyde-paraformaldehyde solution and viewed by scanning electron microscopy (SEM) provides a 3-dimensional view of tumor cells and their matrices. This report describes the SEM appearance of three primary bone tumors: a canine osteosarcoma of the distal radius, a feline chondrosarcoma of the proximal tibia and a canine fibrosarcoma of the proximal humerus. The ultrastructural morphology is compared with the histologic appearance of each tumor

  11. 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.)

  12. CT diagnosis of intramedulear lesions of the tubular bone

    International Nuclear Information System (INIS)

    Three cases are used to demonstrate that intramedullary masses of the tubular bones can be discovered using computered tomography even when changes in the plain film are lacking or discrete. However, it was not possible to differentiate between osteomyelitis, chondrosarcoma and enchondroma owing to the identical morphology of these three diseases. Consequently, CT does not allow any statement on type and dignity and histological clarification is indispensable. (orig./WU)

  13. Chondrocyte Senescence and Telomere Regulation: Implications in Cartilage Aging and Cancer (A Brief Review)

    OpenAIRE

    Mollano, Anthony V; Martin, James A.; Buckwalter, Joseph A

    2002-01-01

    Recent studies on osteoarthritis and the cartilage aging in our laboratory demonstrate that chronologic age correlates with molecular changes in human chondrocytes that affect cell cycle control and replicative life span. These findings indicate that age-related changes in chondrocytes may explain the heightened risk for development of primary osteoarthritis (OA) with increasing age. Concomitant studies of human chondrosarcoma suggest that these aging mechanisms may also play a role in preven...

  14. At least two regions of the viral genome determine the oncogenic potential of avian leukosis viruses.

    OpenAIRE

    Robinson, H L; Blais, B M; Tsichlis, P N; Coffin, J. M.

    1982-01-01

    Recombinants of oncogenic and nononcogenic avian leukosis viruses were tested for their oncogenic potential in chickens. The results indicate that at least two regions of the viral genome determine the oncogenic potential of these viruses. The first region contains sequences that control viral mRNA synthesis. These sequences determine the potential of a virus to induce a low incidence of lymphomas, carcinomas, chondrosarcomas, fibrosarcomas, and osteopetrosis. The second region lies outside t...

  15. Chondromyxoid Fibroma of Sphenoid Sinus with Unusual Calcifications: Case Report with Literature Review

    OpenAIRE

    Morris, Luc G. T.; Rihani, Jordan; Lebowitz, Richard A.; Wang, Beverly Y.

    2009-01-01

    Chondromyxoid fibroma (CMF) is a rare benign primary tumor which usually affects the metaphyses of the long bone of the lower extremities in childhood and young adults. Rarely, CMF occurs in the skull base and parasinuses, which may be difficult to distinguish from chondrosarcoma or chordoma and other tumors in the head. It is composed of chondroid, myxoid, and fibrous tissue growth in a lobular pattern, infrequently with calcifications. We report one case of CMF involving the sphenoid sinus ...

  16. 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.

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

    International Nuclear Information System (INIS)

    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.)

  18. Unusual dumbbell tumours of the mediastinum and thoracic spine.

    Science.gov (United States)

    Kilic, Dalokay; Erdogan, Bulent; Sener, Levent; Sahin, Ekber; Caner, Hakan; Hatipoglu, Ahmet

    2006-11-01

    Due to a lack of large clinical series in the literature of chondrosarcomas and hydatid disease presenting as mediastinal dumbbell tumours, clinicians have limited experience on this topic. We present three unusual cases of dumbbell tumour involving the spinal canal; two patients had chondrosarcoma originating from Th8-Th9 and Th10-Th12; one patient had a hydatid cyst at Th5-Th6. We performed a single-stage combined thoracic-neurosurgical approach in two patients, and a double-staged approach in one patient. During the intraspinal dissection, an operating microscope was used under electrophysiological monitoring. Spinal canal reconstruction was not required for any of the cases. Preoperative knowledge of neuroforaminal extension and the relations between the tumour and adjacent neural-vascular structures is essential to prevent spinal cord damage and plan the surgical approach. In chondrosarcomas, prognosis depends on patient age, histological grade, extent of surgery and response to radiotherapy and/or chemotherapy. In this article, the diagnostic and surgical difficulties of these unusual tumours and current treatment modalities are discussed with a review of the relevant literature. PMID:16857360

  19. 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.)

  20. 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 grade"nmalignant potential. Mesenchymal chondrosarcoma"nare high grade. Clear cell chondrosarcoma is similar"nto chondroblastoma, but it is a low grade malignant"ntumor seen in older patients.

  1. The Radiation Response of Sarcomas by Histologic Subtypes: A Review With Special Emphasis Given to Results Achieved With Razoxane

    Directory of Open Access Journals (Sweden)

    2006-01-01

    Full Text Available Purpose. Relatively few results are available in the literature about the radiation response of unresectable sarcomas in relation to their histology. Therefore, an attempt was made to summarize the present situation. Materials and methods. This report is based on a review of the literature and the author's own experience. Adult-type soft tissue sarcomas, chondrosarcomas, and chordomas were analyzed. Radioresponse was mainly associated with the degree of tumor shrinkage, that is, objective responses. Histopathologic responses, that is, the degree of necrosis, are only discussed in relation to radiation treatment reports of soft tissue sarcomas as a group. Results. Radiation therapy alone leads to major responses in about 50% of lipo-, fibro-, leiomyo-, or chondrosarcomas. The response rate is less than 50% in malignant fibrous histiocytomas, synovial, neurogenic, and other rare soft tissue sarcomas. The response rates may increase up to 75% through the addition of radiosensitizers such as halogenated pyrimidines or razoxane, or by the use of high-LET irradiation. Angiosarcomas become clearly more responsive if biologicals, angiomodulating, and/or tubulin affinic substances are given together with radiation therapy. Razoxane is able to increase the duration and quality of responses even in difficult-to-treat tumors like chondrosarcomas or chordomas. Conclusions. The available data demonstrate that the radioresponsiveness of sarcomas is very variable and dependent on histology, kind of radiation, and various concomitantly given drugs. The rate of complete sustained remissions by radiation therapy alone or in combination with drugs is still far from satisfactory although progress has been made through the use of sensitizing agents.

  2. Periosteal chondroma of the proximal phalanx of the ring finger

    International Nuclear Information System (INIS)

    Full text: Introduction: Periosteal chondroma is an uncommon benign chondroid tumour of bone that was first described by Lichtenstein and Hall in 1952. The more common sites of involvement are the metaphyseal region of a long tubular bone, especially the proximal humerus, femur, and phalanges. Roentgenographically this benign tumor can mimic chondrosarcoma. Awareness of this fact is important in order to avoid over diagnosis and resultant overtreatment of the benign lesion. Objectives and tasks: In this report, we aimed to present clinical, radiological and pathological findings of a case with periosteal chondroma. Material and methods: A 20-year-old man presented with a small painless mass on the radial side of his right ring finger of 3 months duration. Results: Physical examination revealed a slightly protuberant, subcutaneous mass, located in the radial aspect of the proximal phalanx of the right ring finger Radiographs of the ring finger showed a mild cortical irregularity in the dorso-radial aspect of the proximal phalanx. There was soft tissue calcification. There was a lucent lesion that eroded the underlying cortex and caused a reactive sclerosis at the base of the saucershaped defect in the bone. In Magnetic resonance imaging (MRI) lesion was isointense to the muscle tissue in T1WI and high intensity in T2WI. Based on the clinical, radiological and histological findings, a diagnosis of periosteal chondroma was made. Conclusion: Periosteal chondroma is a slow growing, benign, chondroid tumor that arises under or in the periosteum on the surface of cortical bone. In considering the differential diagnosis perhaps the most important tumour to distinguish from periosteal chondroma is periosteal chondrosarcoma. Treatment of these lesions is surgical, with a wide excision performed for chondrosarcoma and a local (marginal or intralesional) excision for chondroma. Differentiation from a malignant lesion is therefore crucial to prevent more extensive unnecessary

  3. Extraskeletal osteochondroma on a cat´s elbow

    Directory of Open Access Journals (Sweden)

    Robert M. Kirberger

    2012-09-01

    Full Text Available A solitary extraskeletal osteochondroma was diagnosed in a 6-year-old, castrated male Burmese cat, positive for feline leukaemia virus (FeLV. The cat presented with a rapidly growing, solid, non-painful mass on the craniolateral aspect of the left elbow. Radiographs revealed an oval, well circumscribed 2.0 cm × 1.5 cm × 1.5 cm mineralised mass separated from the underlying bone. Surgical excisional biopsy confirmed the diagnosis. Feline extraskeletal osteochondromas are benign tumours frequently seen in FeLV-positive cats which can transform into osteosarcomas or chondrosarcomas. Radiographically, they cannot be distinguished from a parosteal or an extraskeletal osteosarcoma.

  4. Fuyuan Decoction Enhances SOX9 and COL2A1 Expression and Smad2/3 Phosphorylation in IL-1β-Activated Chondrocytes

    OpenAIRE

    Yudi Zhang; Rongheng Li; Yu Zhong; Sihan Zhang; Lingyun Zhou; Shike Shang

    2015-01-01

    Fuyuan Decoction (FYD), a herbal formula in China, has been widely used for osteoarthritis (OA) treatment. Herein, we determined the effects of FYD on the expression of transcription factor SOX9 and its target gene collagen type II, alpha 1 (COL2A1) as well as the activation of Smad2/3 in interleukin- (IL-) 1β-stimulated SW1353 chondrosarcoma cells. Serum-derived FYD (FYD-CS) was prepared to treat SW1353 cells with or without SB431542, a TGF-β1 receptor inhibitor. Cell cycle progression was t...

  5. Laryngeal chondroma: report of a case and review of the literature

    International Nuclear Information System (INIS)

    Cartilaginous tumors of the larynx are very rare lesions that originate in the cricoid cartilage in 70% of cases. Their growth is slow and affects the subglottal region, although they can present intralaryngeal growth. They usually recur easily because complete resection is hard to achieve, and the histological findings can be very difficult to differentiate from those of chondrosarcoma, especially those of low grade. A histologically confirmed case of laryngeal chondroma is presented and the radiological and histological aspects are discussed. (Author) 14 refs

  6. MR imaging of uncommon soft tissue tumors in the foot: a pictorial essay

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Youn Joo; Chun, Kyung Ah; Kim, Jee Young; Sung, Mi Sook; Kim, Ki Tae [The Catholic University of Korea, Uijeongbu (Korea, Republic of)

    2007-06-15

    The large variety of masses occur in the foot. The foot is a comparatively rare site of soft tissue neoplasms. MRI has greatly improved the ability to detect and delineate soft tissue lesions and is now considered the gold-standard imaging technique in their investigation. Recently, we have encountered rare soft tissue tumors of the foot. The presented cases include benign masses such as granuloma annulare, angiomyoma, neural fibrolipoma, and giant cell tumor of tendon sheath, as well as malignant tumors such as melanoma, synovial sarcoma, rhabdomyosarcoma and extraskeletal myxoid chondrosarcoma. We wish to illustrate the MR findings of these uncommon soft tissue mors to aid in their diagnosis.

  7. Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma

    Science.gov (United States)

    2014-09-08

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  8. Bone sarcoma as a second malignant neoplasm in children: influence of radiation and genetic predisposition

    International Nuclear Information System (INIS)

    Osteosarcoma or chondrosarcoma developed as a second malignant neoplasm (SMN) in 40 of 188 individuals with SMN whose first neoplasm occurred in childhood. A genetic susceptibility to cancer was found in 23; the SMN developed in an irradiated field in 32; both factors were present in 16; neither in one. When a genetic predisposition was present, radiation shortened the interval to SMN. The intervals between tumors and the age at which the bone sarcomas developed in relation to genetic disease and therapy were analyzed by a two-mutation hypothesis

  9. Cryotherapy of skeletal neoplasms

    International Nuclear Information System (INIS)

    The authors reviewed MR examinations in six patients with giant cell tumor or chondrosarcoma who had undergone surgical curettage and subsequent cryotherapy. In five cases, the authors found a zone of varying thickness extending beyond the surgical margins consistent with cryotherapy injury to medullary bone. This zone appeared dark or intermediate in intensity on T1-weighted images and bright on T2-weighted and short inversion recovery (STIR) images, suggesting tissue edema. In one case, this marginal zone grew for 3 months as the cryotherapy injury evolved. These findings should be expected after cryotherapy and should not be confused with recurrent tumor

  10. Differential diagnosis of sacral lesions

    International Nuclear Information System (INIS)

    The authors reviewed the teaching files of Temple University Hospital and Children's Hospital of Philadelphia and selected the best representative cases of various lesions of the sacrum. They selected the following lesions: metastasis, chondrosarcoma, chrodoma, plasmacytoma, giant cell tumor, osteogenic sarcoma, Ewing sarcoma, neuroblastoma, neurofibrosarcoma, hemangiopericytoma, osteoblastoma, ossifying fibroma, eosinophilic granuloma, aneurysmal bone cyst, sacrococcygeal teratoma, anterior meningocele, endodermal sinus tumor, and stress fracture. The authors illustrate, for each lesion, the likely age range, sights of predilection, likelihood of occurrence in the sacrum, and radiographic findings helpful in limiting the differential diagnosis. They demonstrate the value of bone scintigraphy in detecting, and CT in confirming, stress fractures of the sacrum

  11. Treatment with charged particles beams: hadron therapy part 1: physical basis and clinical experience of treatment with protons; Le traitement par faisceaux de particules: hadrontherapie 1: bases physiques et experience clinique de la protontherapie

    Energy Technology Data Exchange (ETDEWEB)

    Noel, G.; Feuvret, L.; Ferrand, R.; Mazeron, J.J. [Centre de Protontherapie d' Orsay, 91 (France); Mazeron, J.J. [Centre des Tumeurs, Groupe Hospitalier Universitaire Pitie-Salpetriere, 75 - Paris (France)

    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 rarity and the cost of equipments. (authors)

  12. Bizzarre parosteal osteochondromatous proliferation: case report.

    Science.gov (United States)

    Onesti, Maria Giuseppina; Carella, Sara; Amorosi, Vittoria; Pedace, Doria; Campagna, Domenico; Fino, Pasquale; Latini, Claudio

    2013-01-01

    Nora's tumor, also known as bizzarre parosteal osteochondromatous proliferation (POPB), is an exophytic outgrowth arising from the cortical surface of the bone that consists of a mixture of bone, cartilage and fibrous tissue. It is a benign lesion with atipical microscopic features and a tendency to recur. It must be distinguishable from parosteal osteogenic sarcoma, parosteal chondrosarcoma, osteochondroma, florid reactive periostitis, turret exostosis, subungueal exostosis, myositis ossificans. The treatment is surgical, but a high rate of local relapse is described. The diagnosis is histological. We report a case of a patient with POPB involving the foot, underwent surgical excision and with no evidence of recurrence at one year. PMID:23648846

  13. Computed tomography in malignant primary bone tumours

    International Nuclear Information System (INIS)

    The importance of computed tomography is examined in malignant primary bone tumours using a strongly defined examination group of 13 Patients (six Ewing's-sarcomas, five osteosarcomas, one chondrosarcoma and one spindle-shaped cell sarcoma). Computed tomography is judged superior compared to plain radiographs in recognition of bone marrow infiltration and presentation of parosteal tumour parts as well as in analysis of tissue components of tumours, CT is especially suitable for therapy planning and evaluating response to therapy. CT does not provide sufficient diagnostic information to determine dignity and exact diagnosis of bone tumours. (orig.)

  14. Mutations in Isocitrate Dehydrogenase 1 and 2 Occur Frequently in Intrahepatic Cholangiocarcinomas and Share Hypermethylation Targets with Glioblastomas

    OpenAIRE

    Wang, Pu; Dong, Qiongzhu; Zhang, Chong; Kuan, Pei-Fen; Liu, Yufeng; Jeck, William R.; Andersen, Jesper B.; JIANG, Wenqing; Savich, Gleb L.; Tan, Ting-Xu; Auman, J. Todd; Hoskins, Janelle M; Misher, Anne D.; Moser, Catherine D.; Yourstone, Scott M

    2012-01-01

    Mutations in the genes encoding isocitrate dehydrogenase, IDH1 and IDH2, have been reported in gliomas, myeloid leukemias, chondrosarcomas, and thyroid cancer. We discovered IDH1 and IDH2 mutations in 34 of 326 (10%) intrahepatic cholangiocarcinomas. Tumor with mutations in IDH1 or IDH2 had lower 5-hydroxymethylcytosine (5hmC) and higher 5-methylcytosine (5mC) levels, as well as increased dimethylation of histone H3K79. Mutations in IDH1 or IDH2 were associated with longer overall survival (p...

  15. MR imaging of uncommon soft tissue tumors in the foot: a pictorial essay

    International Nuclear Information System (INIS)

    The large variety of masses occur in the foot. The foot is a comparatively rare site of soft tissue neoplasms. MRI has greatly improved the ability to detect and delineate soft tissue lesions and is now considered the gold-standard imaging technique in their investigation. Recently, we have encountered rare soft tissue tumors of the foot. The presented cases include benign masses such as granuloma annulare, angiomyoma, neural fibrolipoma, and giant cell tumor of tendon sheath, as well as malignant tumors such as melanoma, synovial sarcoma, rhabdomyosarcoma and extraskeletal myxoid chondrosarcoma. We wish to illustrate the MR findings of these uncommon soft tissue mors to aid in their diagnosis

  16. Chondroid lipoma of the trunk: MRI appearance and pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Boets, An; Mieghem, Isabelle M.Van; Breuseghem, Iwan Van [Catholic University Leuven Radiology, University Hospitals, Leuven (Belgium); Sciot, Raf [Catholic University Leuven, Pathology, University Hospitals, Leuven (Belgium)

    2004-11-01

    Chondroid lipoma is a rare tumour of adipose tissue, bearing a strikingly close pathologic resemblance to myxoid liposarcoma and extraskeletal myxoid chondrosarcoma. Unlike these malignant tumours, chondroid lipoma has a non-aggressive behaviour and does not require radical treatment. Although repeatedly reported in the proximal extremities and limb girdles, this rare entity may less frequently be observed in the trunk. We describe the imaging findings of a chondroid lipoma in the trunk and provide a discussion on the radiologic-pathologic correlation and differential diagnosis. (orig.)

  17. Extensive tumor reconstruction with massive allograft

    International Nuclear Information System (INIS)

    Massive deep-frozen bone allografts were implanted in four patients after wide tumor resection. Two cases were solitary proximal femur metastases, secondary to Thyroid cancer and breast cancer respectively; while the other two cases were primary in nature i.e. Chondrosarcoma proximal humerus and Osteosarcoma proximal femur. All were treated with a cemented alloprosthesis except in the upper limb where shoulder fusion was performed. Augmentation of these techniques were done with a segment 1 free vascularised fibular composite graft to the proximal femur of breast secondaries and proximal humerus Chondrosarcoma. Coverage of the wound of the latter was also contributed by lattisimus dorsi flap. The present investigations demonstrated the massive bone allografts were intimately anchored by host bone and there had been no evidence of aseptic loosening at the graft-cement interface. This study showed that with good effective tumor control, reconstructive surgery with massive allografts represented a good alternative to prosthetic implants in tumors of the limbs. No infection was seen in all four cases

  18. Comparative {sup 99m}Tc-MIBI, {sup 99m}Tc-tetrofosmin and {sup 99m}Tc-furifosmin uptake in human soft tissue sarcoma cell lines

    Energy Technology Data Exchange (ETDEWEB)

    Rodrigues, M.; Chehne, F.; Sinzinger, H. [Dept. of Nuclear Medicine, University Hospital, Vienna (Austria); Kalinowska, W.; Zielinski, C. [Clinical Div. of Oncology, Department of Internal Medicine I, University Hospital, Vienna (Austria)

    2000-12-01

    The uptake characteristics of technetium-99m hexakis-2-methoxyisobutylisonitrile (MIBI), {sup 99m}Tc-tetrofosmin and {sup 99m}Tc-furifosmin in human soft tissue sarcoma cell lines were investigated and compared. After 10-120 min of incubation at 37 C, 32 C and 22 C with {sup 99m}Tc-MIBI, {sup 99m}Tc-tetrofosmin and {sup 99m}Tc-furifosmin, the kinetics of cellular uptake of these tracers in human soft tissue sarcoma cells SW 684 (fibrosarcoma), SW 872 (liposarcoma), SW 982 (synovial sarcoma) and SW 1353 (chondrosarcoma) was assessed. The uptake of {sup 99m}Tc-MIBI, {sup 99m}Tc-tetrofosmin and {sup 99m}Tc-furifosmin was temperature dependent. The kinetics of uptake of {sup 99m}Tc-MIBI and of {sup 99m}Tc-tetrofosmin was similar between fibrosarcoma and liposarcoma cells, as well as between synovial sarcoma and chondrosarcoma cells. {sup 99m}Tc-furifosmin showed similar uptake kinetics in all cell lines. The uptake of {sup 99m}Tc-furifosmin was, however, significantly higher in liposarcoma than in the other cells. The data indicate that the cellular uptake of {sup 99m}Tc-MIBI, {sup 99m}Tc-tetrofosmin and {sup 99m}Tc-furifosmin is dependent on cellular metabolic activity. (orig.)

  19. CT findings of malignant tumors of maxillary sinus

    International Nuclear Information System (INIS)

    CT findings of histologically proven malignant tumors of maxillary sinus in 36 patients were retrospectively analyzed. A variety of malignant tumors were included; 27 cases of squamous cell carcinoma, 3 malignant lymphomas, 2 malignant melanomas, 2 rhabdomyosarcomas, and chondrosarcoma, adenoid cystic carcinoma, and malignant ameloblastoma. CT staging was T4 in 19 cases, T3 in 16 cases and T2 in 1 case. The most common involved area beyond maxillary antrum was nasal cavity (25 cases), followed by ethmoid sinus (20 cases), orbit (19 cases), infratemporal fossa (17 cases), check skin (13 cases), pterygopalatine fossa (10 cases), and nasopharynx (6 cases). Skeletal destruction was seen in medial wall of antrum (31 cases), posterior wall of antrum (25 cases), anterior wall of antrum (23 cases), roof of antrum (19 cases), lamina papyracea (6 cases), and lateral wall of orbit (5 cases) in that order of frequency. Lymph node metastases were found in 2 cases of malignant melanoma, 2 cases of malignant lymphoma, and chondrosarcoma, squamous cell carcinoma, and malignant ameloblastoma. Predilection site of lymph node involvement was submandibular (3 cases), internal jugular (2 cases), parotid (2 cases), and retropharyngeal node (1 case)

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

    International Nuclear Information System (INIS)

    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

  1. 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.

  2. Comparative 99mTc-MIBI, 99mTc-tetrofosmin and 99mTc-furifosmin uptake in human soft tissue sarcoma cell lines

    International Nuclear Information System (INIS)

    The uptake characteristics of technetium-99m hexakis-2-methoxyisobutylisonitrile (MIBI), 99mTc-tetrofosmin and 99mTc-furifosmin in human soft tissue sarcoma cell lines were investigated and compared. After 10-120 min of incubation at 37 C, 32 C and 22 C with 99mTc-MIBI, 99mTc-tetrofosmin and 99mTc-furifosmin, the kinetics of cellular uptake of these tracers in human soft tissue sarcoma cells SW 684 (fibrosarcoma), SW 872 (liposarcoma), SW 982 (synovial sarcoma) and SW 1353 (chondrosarcoma) was assessed. The uptake of 99mTc-MIBI, 99mTc-tetrofosmin and 99mTc-furifosmin was temperature dependent. The kinetics of uptake of 99mTc-MIBI and of 99mTc-tetrofosmin was similar between fibrosarcoma and liposarcoma cells, as well as between synovial sarcoma and chondrosarcoma cells. 99mTc-furifosmin showed similar uptake kinetics in all cell lines. The uptake of 99mTc-furifosmin was, however, significantly higher in liposarcoma than in the other cells. The data indicate that the cellular uptake of 99mTc-MIBI, 99mTc-tetrofosmin and 99mTc-furifosmin is dependent on cellular metabolic activity. (orig.)

  3. Evaluation of NKX2-2 expression in round cell sarcomas and other tumors with EWSR1 rearrangement: imperfect specificity for Ewing sarcoma.

    Science.gov (United States)

    Hung, Yin P; Fletcher, Christopher D M; Hornick, Jason L

    2016-04-01

    Ewing sarcoma shows considerable histologic overlap with other round cell tumors. NKX2-2, a homeodomain transcription factor involved in neuroendocrine/glial differentiation and a downstream target of EWSR1-FLI1, has been reported as an immunohistochemical marker for Ewing sarcoma. We assessed the specificity of NKX2-2 for Ewing sarcoma compared with other round cell malignant neoplasms and other soft tissue tumors with EWSR1 translocations. We evaluated whole-tissue sections from 270 cases: 40 Ewing sarcomas (4 with atypical/large cell features), 20 CIC-DUX4 sarcomas, 5 BCOR-CCNB3 sarcomas, 9 unclassified round cell sarcomas, 10 poorly differentiated synovial sarcomas, 10 lymphoblastic lymphomas, 10 alveolar rhabdomyosarcomas, 10 embryonal rhabdomyosarcomas, 10 Merkel cell carcinomas, 10 small cell carcinomas, 20 melanomas, 5 NUT midline carcinomas, 10 Wilms tumors, 10 neuroblastomas, 10 olfactory neuroblastomas, 12 mesenchymal chondrosarcomas, 10 angiomatoid fibrous histiocytomas, 10 clear cell sarcomas, 5 gastrointestinal clear cell sarcoma-like tumors, 5 desmoplastic small round cell tumors, 10 extraskeletal myxoid chondrosarcomas, 10 soft tissue and cutaneous myoepitheliomas, and 19 myoepithelial carcinomas. NKX2-2 positivity was defined as moderate-to-strong nuclear immunoreactivity in at least 5% of cells. NKX2-2 was positive in 37/40 (93%) Ewing sarcomas, including all atypical Ewing sarcomas and cases with known EWSR1-FLI1 or EWSR1-ERG fusion; 85% of Ewing sarcomas showed diffuse (>50%) staining. NKX2-2 was positive in 9 (75%) mesenchymal chondrosarcomas, 8 (80%) olfactory neuroblastomas, 1 CIC-DUX4 sarcoma, 1 poorly differentiated synovial sarcoma, 1 neuroblastoma, 2 unclassified round cell sarcomas, and 3 small cell carcinomas; all other EWSR1-associated tumors were negative for NKX2-2, apart from 1 desmoplastic small round cell tumor, 1 myoepithelioma, and 1 myoepithelial carcinoma. In summary, NKX2-2 is a sensitive but imperfectly specific marker for

  4. 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

  5. Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma

    Science.gov (United States)

    2016-05-16

    Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; 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 Undifferentiated High Grade Pleomorphic Sarcoma of Bone; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Malignant Peripheral Nerve Sheath Tumor; Childhood Pleomorphic Rhabdomyosarcoma; Childhood Rhabdomyosarcoma With Mixed Embryonal and Alveolar Features; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Malignant Adult Hemangiopericytoma; Malignant Childhood Hemangiopericytoma; Metastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Untreated Childhood Rhabdomyosarcoma

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

    International Nuclear Information System (INIS)

    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.

  7. Patterns of localization of sup(99m)Tc-diphosphonate and tetracycline in bone tumors

    International Nuclear Information System (INIS)

    In the beginning of a study on mechanism of sup(99m)Tc-phosphoric acid compounds accumulation, regions where sup(99m)Tc-methylene diphosphonate (MDP) or tetracycline accumulated were compared in 5 patients with primary bone tumors (one with osteosarcoma, one with malignant fibrous histocytoma, one with osteochondroma, and two with chondrosarcoma). In accordance with regions where tetracycline accumulated, much sup(99m)Tc-MDP accumulated in neoplastic bone, calcified and ossified cartilage, and reactive new bone around bone tumors. It was concluded from above-mentioned results and mechanism of tetracycline accumulation that much sup(99m)Tc-MDP penetrated well and accumulated in reactive new bone around bone tumors probably because in reactive new bone around bone tumors, the size of hydroxyapatite was small, the density of matrix of bone was low, and surface area of hydroxyapatite crystal was very wide. (Tsunoda, M.)

  8. Delayed complications of radiotherapy treatment for nasopharyngeal carcinoma: imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    King, A.D. [Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong (China)]. E-mail: king2015@cuhk.edu.hk; Ahuja, A.T. [Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong (China); Yeung, D.K. [Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong (China); Wong, J.K.T. [Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong (China); Lee, Y.Y.P. [Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong (China); Lam, W.W.M. [Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong (China); Ho, S.S.M. [Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong (China); Yu, S.C.H. [Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong (China); Leung, S.-F. [Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong (China)

    2007-03-15

    Radiotherapy is used to treat a wide variety of head and neck tumours that arise in and around the skull base. The delayed effects of radiation damages a range of structures, including the nervous system, bone, major vessels, mucus membranes, pituitary and salivary glands, as well as increasing the risk of radiation-induced neoplasms. In this review the complications resulting from radiation treatment for nasopharyngeal carcinoma (NPC), a cancer treated with a high dose of radiation to a fairly large region, are illustrated. Many patients with NPC have a long-term survival, so are at risk of developing delayed radiation effects, and hence may demonstrate a wide range of complications on imaging. Other tumours around the skull base treated with radiotherapy include meningiomas, chordomas, chondrosarcomas, pituitary adenomas, paranasal sinus and nasal cavity tumours. In these cases similar complications may be encountered on imaging, although the severity, incidence and location will vary.

  9. Long-term prognosis of maxillary sinus malignant tumor patients treated by fast neutron radiation therapy

    International Nuclear Information System (INIS)

    From 1976 through 1990, 19 patients with maxillary sinus malignant tumor were treated with combination therapy consisting of maxillectomy and radiation of fast neutron. Fast neutron radiotherapy was performed at National Institute of Radiological Sciences. Eight patients had adenoid cystic carcinomas, three patients squamous cell carcinomas, one patient a carcinoma in pleomorphic adenoma, four patients fibrosarcomas, one patient osteosarcoma, one patient chondrosarcoma and one patient rhabdomyosarcoma. Fast neutron therapy after/before surgery was effective in fresh cases with T2-3N0M0 adenoid cystic carcinomas and sarcomas (except for fibrosarcoma). Nine patients were alive more than three years after treatment. And serious complications of fast neutron radiation therapy appeared in six of these nine patients. Visual impairment of opposite side occurred in four patients. Bone necrosis occured in one patient and brain dysfunction in one patient. (author)

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

    International Nuclear Information System (INIS)

    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

  11. Sarcomas of the head and neck in clinical material from Clinic of Cranio-Maxillofacial and Oncological Surgery of Medical University of Lodz

    International Nuclear Information System (INIS)

    Sarcomas are the malignant neoplasms originating from mesenchymal tissue. They can arise from connective, vascular, muscular and other tissues. The most frequent localization is on the trunk and limbs, but in maxillofacial region they occur rarely and have worse survival prognosis. Initially they arise asymptotically and painlessly. Radiological findings are also untypical. The best results of treatment are obtained by the combined therapy. Applying not only surgery but also chemotherapy and radiotherapy in the treatment of patients with sarcomas enabled longer 5-years survival from 20% to almost 70%. We want to present 4 patients treated in the Department of Cranio - Maxillofacial Surgery of the Medical University in Lodz during the recent years. There were 2 patients with chondrosarcomas, 1 with osteosarcomas and 1 with leyomiosarcoma. (authors)

  12. 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

  13. 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.

  14. Primary malignant bone tumors: diagnosis, radiological appearance and therapy; Primaer maligne Knochentumoren: Klinik und Therapie

    Energy Technology Data Exchange (ETDEWEB)

    Dominkus, M.; Kotz, R. [Universitaetsklinik fuer Orthopaedie, Wien (Austria); Kainberger, F. [Abt. fuer Osteoradiologie, Universitaetsklinik fuer Radiologie, Wien (Austria); Lang, S. [Wien Univ. (Austria). Universitaetsklinik fuer Pathologische Anatomie

    1998-06-01

    Diagnosis, concepts of therapy and prognosis of primary malignant bone tumors require intensive cooperation between orthopedic surgeons, radiologists and pathologists with special knowledge in oncology. The present paper demonstrates relevant topics of diagnosis, radiological appearance and therapy of the osteosarcoma, Ewing`s sarcoma, chondrosarcoma and malignant fibrous histiocytoma based on the material of the Vienna Bone Tumor Registry and more than 35 years of cooperation between the Institute of Pathology and Anatomy and the Department of Orthopedics at the University of Vienna. (orig.) [Deutsch] Fuer die Diagnose, Therapieplanung und Prognosebeurteilung von primaer malignen Knochentumoren ist eine intensive Kooperation von onkologisch speziell ausgebildeten Fachdisziplinen wie Orthopaedie, Radiologie und Pathologie erforderlich. Die vorliegende Arbeit basiert auf dem Material des Wiener Knochengeschwulstregisters und der 35jaehrigen Zusammenarbeit des Pathologisch/Anatomischen Instituts der Universitaet Wien mit der Universitaetsklinik fuer Orthopaedie und beschreibt Diagnose, radiologisches Erscheinungsbild und Therapie der 4 haeufigsten primaer malignen Knochentumoren: Osteosarkom, Ewing`s Sarkom, Chondrosarkom und malignes fibroeses Histiozytom. (orig.)

  15. Paget's sarcoma of bone

    International Nuclear Information System (INIS)

    The most sinister complication of Paget's disease is malignant degeneration. The true etiology of Paget's disease remains unproven and unknown. The relative incidence of sarcoma ranges from 0.9% to 20% depending upon the population source. The anatomical distribution of lesions is similar to that of uncomplicated Paget's disease except for the high incidence in the humerus and rare occurrence in the spine. Approximately 50% of the lesions histologically represent osteosarcomas, with fibrosarcoma, fibrous histiocytoma, chondrosarcoma and giant cell tumor rarely occurring. The radiographic presentation is most commonly lytic, but sclerotic and mixed patterns occur. Cortical destruction and soft tissue mass are the cardinal radiographic signs. Malignant periosteal response is seldom found associated with Paget's sarcoma. The most common sites are femur, humerus, pelvis, skull and tibia. The prognosis is fatal at a very rapid rate. There are but a few isolated cases of long-term survival reported. (orig.)

  16. [Chondromyxoid fibroma of bone: a rare benign bone tumor in children].

    Science.gov (United States)

    Rouas, L; Malihy, A; Cherradi, N; Lamalmi, N; Alhamany, Z

    2004-12-01

    Chondromyxoid fibroma is a rare benign tumor that is typically found in the metaphyseal ends of long tubular bones, such as the tibia. The radiographic appearances are those of a single, lytic lesion with lobulated margins, septations, cortical expansion and a sclerotic rim. The classic histological feature of a chondromyxoid fibroma is stellate or spindle-shaped cells arranged in lobules in a myxoid or chondroid background. Two cases are presented here: 8, and 12-year-old patients, both with lesions in the proximal tibia. The first case showed an unusual feature: it was diaphyseal chondromyxoid fibroma. In the second case, the lesion was metaphyso-diaphyseal. The differential diagnosis includes chondroblastoma, myxoma, aneurysmal cyst as well as chondrosarcoma. A surgical conservative treatment with complete excision is recommended even in case of recurrence. PMID:15688891

  17. [Chondromyxoid fibroma. Morphological variations, site, incidence, radiologic criteria and differential diagnosis].

    Science.gov (United States)

    Engels, C; Priemel, M; Möller, G; Werner, M; Delling, G

    1999-07-01

    Chondromyxoid fibroma is a rare benign bone tumor, accounting for less than 1% of all bone tumors. The peak age incidence is the second and third decade of life. Chondromyxoid fibrom occurs in the metaphyseal parts of the major tubular bones, predominantly of the lower extremity. Roentgenograms show, in most cases, a well-demarcated radiolucent lesion. The classic histological feature of a chondromyxoid fibroma is stellate or spindle-shaped cells arranged in lobules in a myxoid or chondroid background. Analysis of 40 chondromyxoid fibromas demonstrates the morphological variation of this tumor. Cases were examined for age distribution, localization, and radiological and histological features. In 85% we found the typical histomorphological pattern. Recurrence rate was 12.5%. In four cases the appearance was uncharacteristic and differentiation from other tumors such as chondroblastoma or chondrosarcoma was quite difficult. By adhering to strict histomorphological criteria, definite diagnosis of chondromyxoid fibroma can be made in most cases. PMID:10478364

  18. 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.

  19. 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. PMID:24682526

  20. 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.

  1. Molecular Pathogenesis and Diagnostic, Prognostic and Predictive Molecular Markers in Sarcoma.

    Science.gov (United States)

    Mariño-Enríquez, Adrián; Bovée, Judith V M G

    2016-09-01

    Sarcomas are infrequent mesenchymal neoplasms characterized by notable morphological and molecular heterogeneity. Molecular studies in sarcoma provide refinements to morphologic classification, and contribute diagnostic information (frequently), prognostic stratification (rarely) and predict therapeutic response (occasionally). Herein, we summarize the major molecular mechanisms underlying sarcoma pathogenesis and present clinically useful diagnostic, prognostic and predictive molecular markers for sarcoma. Five major molecular alterations are discussed, illustrated with representative sarcoma types, including 1. the presence of chimeric transcription factors, in vascular tumors; 2. abnormal kinase signaling, in gastrointestinal stromal tumor; 3. epigenetic deregulation, in chondrosarcoma, chondroblastoma, and other tumors; 4. deregulated cell survival and proliferation, due to focal copy number alterations, in dedifferentiated liposarcoma; 5. extreme genomic instability, in conventional osteosarcoma as a representative example of sarcomas with highly complex karyotype. PMID:27523972

  2. 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.)

  3. Diagnosis and treatment of sarcomas and related tumors

    Energy Technology Data Exchange (ETDEWEB)

    1978-01-01

    The Cancergram focuses on clinical aspects of sarcomas involving soft tissue and bone, and also includes abstracts on related malignant and benign tumors. Soft tissues are considered as all non-epithelial extra-skeletal tissues of the body, with the exception of the reticuloendothelial system, the neuroglia, and visceral and parenchymal organs. Included, therefore, are sarcomas of the vascular system, fatty tissue, muscle tissues, connective tissues, and synovial tissues. Bone tumors included are osteosarcoma, chondrosarcoma, adamantinoma, chrondroblastoma, Ewing's sarcoma, and other benign and malignant disorders of the bone. This Cancergram excludes disorders of the bone marrow, which are the subjects of separate Cancergrams (see series CT03 for leukemias, and series CT12 for multiple myeloma). The scope includes diagnosis and staging, supportive care, evaluation, and therapy. Selected abstracts concerning epidemiology, etiology and other pre-clinical studies will also be included where they have direct clinical relevance.

  4. 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.

  5. Models of presentation of intraarticular masses

    International Nuclear Information System (INIS)

    Intraarticular masses are relatively rare, and their magnetic resonance (MR) features are little known. The purpose of this report was to asses the presentation and MR imaging findings associated with the different histological types encountered in a series of 22 patients. We review the MR imaging findings in 22 intraarticular masses (18 in knee, two in ankle and two in shoulder). The variables studied were: calcifications, edema, morphology, methemoglobin, hemosiderin, homogeneity, margins, bone changes, T1-weighted signal intensity, T2-weightes/STIR signal intensity, clinical presentation and patient age. The statistical relationships were determined by χ''2 test and ANOVA (Student-Newman-Keuls). Among the intraarticular masses, there were 14 cases of pigmented villonodular synovitis (SVNP) (63.6%), 3 cases of themangioma (13.6%), 3 cases of synovial sarcoma (13.6%) and 2 cases of chondrosarcoma (9.1%). Statistical significant relationships were observed between histological type and; calcifications (p=0.004), which were irregular in chondrosarcomas and appeared as phleboliths in hemangionas: morphology (p=0.007), which was serpiginous in hemangiomas and oval in focal SVNP; areas of hemosiderin (p=0.002)in SVNP and synovial sarcoma: clinica presentation (p=0.003), with skin changes in cases of hemangioma: and age (p=0.04), hemangioma patients being younger (mean: 21 years) and those with synovial sarcoma being older (mean: 56 years). In cases of intraarticular masses, the presence of calcification, tumor morphology, the presence of hemosiderin, patient age and clinical presentation show statistically significant differences depending on the histological type. (Author) 23 refs

  6. Models of presentation of intraarticular masses; Normas de presentacion de las masas intraarticulares

    Energy Technology Data Exchange (ETDEWEB)

    Vega, M. [Hospital Universitario Dr. Peset. Valencia (Spain); Molla, E. [Clinica Quiron. Valencia (Spain); Marti-Bonmati, L.; Galant, J. [Hospital San Juan. Alicante (Spain); Madariaga, B. [Hospital Clinico Zaragoza (Spain)

    2002-07-01

    Intraarticular masses are relatively rare, and their magnetic resonance (MR) features are little known. The purpose of this report was to asses the presentation and MR imaging findings associated with the different histological types encountered in a series of 22 patients. We review the MR imaging findings in 22 intraarticular masses (18 in knee, two in ankle and two in shoulder). The variables studied were: calcifications, edema, morphology, methemoglobin, hemosiderin, homogeneity, margins, bone changes, T1-weighted signal intensity, T2-weightes/STIR signal intensity, clinical presentation and patient age. The statistical relationships were determined by {chi}''2 test and ANOVA (Student-Newman-Keuls). Among the intraarticular masses, there were 14 cases of pigmented villonodular synovitis (SVNP) (63.6%), 3 cases of themangioma (13.6%), 3 cases of synovial sarcoma (13.6%) and 2 cases of chondrosarcoma (9.1%). Statistical significant relationships were observed between histological type and; calcifications (p=0.004), which were irregular in chondrosarcomas and appeared as phleboliths in hemangionas: morphology (p=0.007), which was serpiginous in hemangiomas and oval in focal SVNP; areas of hemosiderin (p=0.002)in SVNP and synovial sarcoma: clinica presentation (p=0.003), with skin changes in cases of hemangioma: and age (p=0.04), hemangioma patients being younger (mean: 21 years) and those with synovial sarcoma being older (mean: 56 years). In cases of intraarticular masses, the presence of calcification, tumor morphology, the presence of hemosiderin, patient age and clinical presentation show statistically significant differences depending on the histological type. (Author) 23 refs.

  7. Metastatic tumor of the pancreas: helical CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Soon Jin; Lee, Won Jae; Lim, Hyo Keun; Kim, Seung Hoon; Kim, Kyeong Ah; Choi, Sang Hee; Jang, Hyun Jung; Lee, Ji Yeon [Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul (Korea, Republic of)

    2000-04-01

    To analyze the helical computed tomographic (CT) findings of distant metastatic tumors to the pancreas and to determine the differential points between these and primary pamcreatic carcinomas. We sruveyed 22 patients with metastatic tumor of the pancreas, proven on the basis of clinical and pathological findings. Seventeen patients were men, and five were women, and their ages ranged between 36 and 83 years. Their primary conditions were lung cancer (n=3D15), rectal cancer (n=3D2), melanoma of the foot, chondrosarcoma of the sacrum, cervical cancer, leiomyosarcoma of the uterus, and extragonadal choriocarcinoma of the mediastinum. We retrospectively reviewed the abdominal helical CT findings, analysing the number, location, size and attenuation of masses, as well as secondary change, which included dilatation of the pancreatic and biliary ducts and invasion of peripancreatic tissue or vessels. We also evaluated the differential findings of primary pancreatic cancer. Sixteen patients had a solitary focal mass, while in five, two masses were present. Among the 22 patients, low-density nodular masses were present in 21; in the other, in whom multiple metastasis from chondrosarcoma had occurred, there was dense calcification. The size of metastatic masses varied, ranging from 0.6 to 6 cm in diameter. The pancreatic duct proximal to the mass was dilated in ten cases, while the bile duct was dilated in six. The metastatic masses masses demonstrated no peripancreatic or vascular invasion, though they showed a discrete margin and contour bulging. Single metastasis to the pancreas was most common, and metastatic masses had a discrete margin, with contour bulging. There was no peripancreatic or vascular invasion. If the metastasis involved a single low-attenuated mass, however, with pancreatic or biliary dilatation, it was difficult to differentiate this from primary pancreatic cancer. (author)

  8. Metastatic tumor of the pancreas: helical CT findings

    International Nuclear Information System (INIS)

    To analyze the helical computed tomographic (CT) findings of distant metastatic tumors to the pancreas and to determine the differential points between these and primary pamcreatic carcinomas. We sruveyed 22 patients with metastatic tumor of the pancreas, proven on the basis of clinical and pathological findings. Seventeen patients were men, and five were women, and their ages ranged between 36 and 83 years. Their primary conditions were lung cancer (n=3D15), rectal cancer (n=3D2), melanoma of the foot, chondrosarcoma of the sacrum, cervical cancer, leiomyosarcoma of the uterus, and extragonadal choriocarcinoma of the mediastinum. We retrospectively reviewed the abdominal helical CT findings, analysing the number, location, size and attenuation of masses, as well as secondary change, which included dilatation of the pancreatic and biliary ducts and invasion of peripancreatic tissue or vessels. We also evaluated the differential findings of primary pancreatic cancer. Sixteen patients had a solitary focal mass, while in five, two masses were present. Among the 22 patients, low-density nodular masses were present in 21; in the other, in whom multiple metastasis from chondrosarcoma had occurred, there was dense calcification. The size of metastatic masses varied, ranging from 0.6 to 6 cm in diameter. The pancreatic duct proximal to the mass was dilated in ten cases, while the bile duct was dilated in six. The metastatic masses masses demonstrated no peripancreatic or vascular invasion, though they showed a discrete margin and contour bulging. Single metastasis to the pancreas was most common, and metastatic masses had a discrete margin, with contour bulging. There was no peripancreatic or vascular invasion. If the metastasis involved a single low-attenuated mass, however, with pancreatic or biliary dilatation, it was difficult to differentiate this from primary pancreatic cancer. (author)

  9. Meclozine facilitates proliferation and differentiation of chondrocytes by attenuating abnormally activated FGFR3 signaling in achondroplasia.

    Directory of Open Access Journals (Sweden)

    Masaki Matsushita

    Full Text Available Achondroplasia (ACH is one of the most common skeletal dysplasias with short stature caused by gain-of-function mutations in FGFR3 encoding the fibroblast growth factor receptor 3. We used the drug repositioning strategy to identify an FDA-approved drug that suppresses abnormally activated FGFR3 signaling in ACH. We found that meclozine, an anti-histamine drug that has long been used for motion sickness, facilitates chondrocyte proliferation and mitigates loss of extracellular matrix in FGF2-treated rat chondrosarcoma (RCS cells. Meclozine also ameliorated abnormally suppressed proliferation of human chondrosarcoma (HCS-2/8 cells that were infected with lentivirus expressing constitutively active mutants of FGFR3-K650E causing thanatophoric dysplasia, FGFR3-K650M causing SADDAN, and FGFR3-G380R causing ACH. Similarly, meclozine alleviated abnormally suppressed differentiation of ATDC5 chondrogenic cells expressing FGFR3-K650E and -G380R in micromass culture. We also confirmed that meclozine alleviates FGF2-mediated longitudinal growth inhibition of embryonic tibia in bone explant culture. Interestingly, meclozine enhanced growth of embryonic tibia in explant culture even in the absence of FGF2 treatment. Analyses of intracellular FGFR3 signaling disclosed that meclozine downregulates phosphorylation of ERK but not of MEK in FGF2-treated RCS cells. Similarly, meclozine enhanced proliferation of RCS cells expressing constitutively active mutants of MEK and RAF but not of ERK, which suggests that meclozine downregulates the FGFR3 signaling by possibly attenuating ERK phosphorylation. We used the C-natriuretic peptide (CNP as a potent inhibitor of the FGFR3 signaling throughout our experiments, and found that meclozine was as efficient as CNP in attenuating the abnormal FGFR3 signaling. We propose that meclozine is a potential therapeutic agent for treating ACH and other FGFR3-related skeletal dysplasias.

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

    International Nuclear Information System (INIS)

    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. (author)

  11. 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

  12. Growth and behavior of chondrocytes on nano engineered surfaces and construction of micropatterned co-culture platforms using layer-by-layer platforms using layer-by-layer assembly lift-off method

    Science.gov (United States)

    Shaik, Jameel

    Several approaches such as self-assembled monolayers and layer-by-layer assembled multilayer films are being used as tools to study the interactions of cells with biomaterials in vitro. In this study, the layer-by-layer assembly approach was used to create monolayer, bilayer, trilayer, five, ten and twenty-bilayer beds of eleven different biomaterials. The various biomaterials used were poly(styrene-sulfonate), fibronectin, poly-L-lysine, poly-D-lysine, laminin, bovine serum albumin, chondroitin sulfate, poly(ethyleneimine), polyethylene glycol amine, collagen and poly(dimethyldiallyl-ammonium chloride) with unmodified tissue-culture polystyrene as standard control. Three different cell lines---primary bovine articular chondrocytes, and two secondary cell lines, human chondrosarcoma cells and canine chondrocytes were used in these studies. Chondrocyte morphology and attachment, viability, proliferation, and functionality were determined using bright field microscopy, the Live/Dead viability assay, MTT assay, and immunocytochemistry, respectively. Atomic force microscopy of the nanofilms indicated an increase in surface roughness with increasing number of layers. The most important observations from the studies on primary bovine articular chondrocytes were that these cells exhibited increasing viability and cell metabolic activity with increasing number of bilayers. The increase in viability was more pronounced than the increase in cell metabolic activity. Also, bovine chondrocytes on bilayers of poly(dimethyldiallyl-ammonium chloride, poly-L-lysine, poly(styrene-sulfonate), and bovine serum albumin were substantially bigger in size and well-attached when compared to the cells grown on monolayer and trilayers. Lactate dehydrogenase assay performed on chondrosarcoma cells grown on 5- and 10-bilayer multilayer beds indicated that the 10-bilayer beds had reduced cytotoxicity compared to the 5-bilayer beds. MTT assay performed on canine chondrocytes grown on 5-, 10

  13. The skeletal system

    International Nuclear Information System (INIS)

    The joy of diagnostic radiology is derived in great measure in its neverending variety including the unveiling of new diagnostic entities and new information concerning known disease processes. This year is no exception in the fascinating documentation of skeletal disease. In the study of disorders of the joints, CT investigation of the temporomandibular joint and arthotomography of the shoulder are gaining in popularity. New observations concerning cyst-like osseous lesions in lupus erthematosis, destructive joint lesions in renal osteodystrophy, and intra- and periarticular calcifications secondary to steroid injections have come forward. Articles discussing interesting observations concerning chondrosarcoma are included as well as one that describes the demonstration of fluid levels in aneurysmal bone cysts by CT. Ossification in soft tissues following resection of giant cell tumors as evidence of residual neoplasm is an important new sign. Marrow transplantation for treatment of mucopolysaccharidosis represents a new therapeutic breakthrough. Some of the skeletal manifestions of hypomagnesemia, 13-cis-retinoic acid, and aluminum are elucidated in this year's articles on metabolic disease. Further studies of methods of measuring bone density are also included

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

    Directory of Open Access Journals (Sweden)

    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.

  15. Neurologic abnormalities as the predominant signs of neoplasia of the nasal cavity in dogs and cats: seven cases (1973-1986)

    International Nuclear Information System (INIS)

    Neurologic abnormalities were the predominant historic and physical findings in 5 dogs and 2 cats with primary nasal cavity tumors that had invaded the cranial vault. Seizures, behavior changes, and obtundation were the most common signs. Other neurologic signs included paresis, ataxia, circling, visual deficit, and proprioceptive deficit. Although 1 dog and 2 cats had historic findings of mild respiratory disease, no physical abnormalities related to the respiratory tract were found in any of the 7 animals. Nasal cavity neoplasia was suggested by radiographic and computed tomographic studies and was confirmed histopathologically in each case. The nasal tumor types in the 5 dogs were epidermoid carcinoma (n = 1), adenocarcinoma (n = 2), solid carcinoma (n = 1), and anaplastic chondrosarcoma (n = 1). An esthesioneuroblastoma was found in each cat. Radiation therapy was effective for 3 months in palliating the clinical signs in the 2 dogs in which it was used. Neoplasia of the nasal cavity should be considered in the differential diagnosis for animals with neurologic signs suggestive of cerebral disorders

  16. The dawn of hedgehog inhibitors: Vismodegib.

    Science.gov (United States)

    Sandhiya, Selvarajan; Melvin, George; Kumar, Srinivasamurthy Suresh; Dkhar, Steven Aibor

    2013-01-01

    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. PMID:23662017

  17. 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.

  18. Nonangiogenic and nonlymphomatous sarcomas of the canine spleen: 57 cases (1975-1987)

    International Nuclear Information System (INIS)

    The case records of and histopathologic findings in 57 dogs with nonangiogenic and nonlymphomatous splenic sarcomas were reviewed. Splenic neoplasms in these dogs included leiomyosarcoma, fibrosarcoma, undifferentiated sarcoma, liposarcoma, osteosarcoma, chondrosarcoma, myxosarcoma, rhabdomyosarcoma, and fibrous histiocytoma. The clinical signs associated with splenic sarcoma included anorexia or decreased appetite, abdominal distention, polydipsia, lethargy, vomiting, weight loss, and weakness. An abdominal mass was detected in 86% of the dogs by use of abdominal palpation (63%), and/or abdominal radiography (74%). The diagnosis was based on histopathologic findings in the spleen. Abdominal exploratory surgery was performed on 43 of the 57 dogs. Twenty-seven dogs were treated by splenectomy, and 16 were euthanatized at the time of surgery because of widespread metastatic lesions. Of the 14 dogs on which surgery was not performed, 11 were euthanatized on the basis of results of preoperative diagnostic tests, and the remaining 3 dogs had splenic neoplasms that were incidental findings at necropsy. Of the 27 surgically treated dogs, 5 died in the immediate postoperative period, 12 died or were euthanatized within 1 year after splenectomy, and only 5 dogs survived greater than or equal to 1 year. Three dogs were lost to follow-up evaluation, and 2 were still alive 6 and 7 months after surgery. The median survival time of the 22 dogs for which survival was known was 2.5 months. The median survival time for 11 dogs with no obvious metastasis at the time of splenectomy was 9 months

  19. 软骨样副脊索瘤(附病例报告)

    Institute of Scientific and Technical Information of China (English)

    姚庆端; 王敏; 李培峰; 曲美婷

    2004-01-01

    To study the clinical pathological characteristics and differentiation diagnosis of the chondroid parachordoma from primary tumors or metastatic to ulna, femur and radius. Methods Three cases of primary chondroid parachordoma of the ulma, femur and radius were studied by histopathological observation and immunohisto chemical staining and by pathological consultation. Results The histological features of tumor were composed of two the chordoma ceils subtotal and the chondrosareoma ceils of small - portio. The tumor was arranged vary in sizes of lobular and brink of lobular were seplum small girder of chondroid, or the tissue fiber. The tumor cells were distributed sheet, trabecula with or island -like in the intralobular, and the tumor ceils were vary in size and the karyon heterotypic were no patent and the cytoplasm of rich shows large vacuole. The chondroidosarcomas intra - lobular were viewed ceils both nucleus and uninuclear of chondrolacunae and the stroma were hyaling cartilage with or blennoid in the mesenchyma, and that picture of typical chondrosarcoma. EMA, CK, Vimentin, S - 100 protein, NSE with immunohistochemical method (ABC) staining were positive and Desmin, CD34 negative in the all of tumor cells. The chondroid parachodoma was diagnosed in the pathological read- section- conference and consulation. Conclusion Primary ulna, femur and radius chondroid parachordoma is a rare in rate of neoplasm and is diagnosed diflqculdy. Immunohistochemical method would be helpful to diagnosis.

  20. 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. PMID:24995286

  1. C11orf95-MKL2 is the resulting fusion oncogene of t(11;16)(q13;p13) in chondroid lipoma.

    Science.gov (United States)

    Huang, Dali; Sumegi, Janos; Dal Cin, Paola; Reith, John D; Yasuda, Taketoshi; Nelson, Marilu; Muirhead, David; Bridge, Julia A

    2010-09-01

    Chondroid lipoma, a rare benign adipose tissue tumor, may histologically resemble myxoid liposarcoma or extraskeletal myxoid chondrosarcoma, but is genetically distinct. In this study, an identical reciprocal translocation, t(11;16)(q13;p13), was identified in three chondroid lipomas, a finding consistent with previously isolated reports. A fluorescence in situ hybridization (FISH)-based positional cloning strategy using a series of bacterial artificial chromosome (BAC) probe combinations designed to narrow the 16p13 breakpoint revealed MKL2 as the candidate gene. Subsequent 5' RACE studies demonstrated C11orf95 as the MKL2 fusion gene partner. MKL/myocardin-like 2 (MKL2) encodes myocardin-related transcription factor B in a megakaryoblastic leukemia gene family, and C11orf95 (chromosome 11 open reading frame 95) is a hypothetical protein. Sequencing analysis of reverse transcription-polymerse chain reaction (RT-PCR) generated transcripts from all three chondroid lipomas defined the fusion as occurring between exons 5 and 9 of C11orf95 and MKL2, respectively. Dual-color breakpoint spanning probe sets custom-designed for recognition of the translocation event in interphase cells confirmed the anticipated rearrangements of the C11orf95 and MKL2 loci in all cases. The FISH and RT-PCR assays developed in this study can serve as diagnostic adjuncts for the identification of this novel C11orf95-MKL2 fusion oncogene in chondroid lipoma. PMID:20607705

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

    Directory of Open Access Journals (Sweden)

    Tarek Mahboub

    2008-03-01

    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.

  3. Bone and soft tissue tumors of hip and pelvis

    Energy Technology Data Exchange (ETDEWEB)

    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.

  4. Is proton beam therapy the future of radiotherapy? Part I: Clinical aspects; La protontherapie: avenir de la radiotherapie? Premiere partie: aspects cliniques

    Energy Technology Data Exchange (ETDEWEB)

    Bouyon-Monteau, A.; Habrand, J.L.; Datchary, J.; Alapetite, C.; Bolle, S.; Dendale, R.; Feuvret, L.; Helfre, S.; Calugaru, V. [Centre de protontherapie d' Orsay, institut Curie, campus universitaire, 91 - Orsay (France); Bouyon-Monteau, A.; Alapetite, C.; Bolle, S.; Dendale, R.; Helfre, S.; Calugaru, V.; Cosset, J.M.; Bey, P. [Departement d' oncologie-radiotherapie, institut Curie, 75 - Paris (France); Habrand, J.L.; Datchary, J. [Departement d' oncologie-radiotherapie, institut de cancerologie Gustave-Roussy, 94 - Villejuif (France); Feuvret, L. [Departement d' oncologie-radiotherapie, hopital Pitie-Salpetriere, 75 - Paris (France)

    2010-12-15

    Proton beam therapy uses positively charged particles, protons, whose physical properties improve dose-distribution (Bragg peak characterized by a sharp distal and lateral penumbra) compared with conventional photon-based radiation therapy (X-ray). These ballistic advantages apply to the treatment of deep-sited tumours located close to critical structures and requiring high-dose levels. [60-250 MeV] proton-beam therapy is now widely accepted as the 'gold standard' in specific indications in adults - ocular melanoma, chordoma and chondrosarcoma of the base of skull - and is regarded as a highly promising treatment modality in the treatment of paediatric malignancies (brain tumours, sarcomas..). This includes the relative sparing of surrounding normal organs from low and mid-doses that can cause deleterious side-effects such as radiation-induced secondary malignancies. Other clinical studies are currently testing proton beam in dose-escalation evaluations, in prostate, lung, hepatocellular cancers, etc. Clinical validation of these new indications appears necessary. To date, over 60, 000 patients worldwide have received part or all of their radiation therapy program by proton beams, in approximately 30 treatment facilities. (authors)

  5. The diagnostic utility of reduced immunohistochemical expression of SMARCB1 in synovial sarcomas: a validation study.

    Science.gov (United States)

    Ito, Junko; Asano, Naofumi; Kawai, Akira; Yoshida, Akihiko

    2016-01-01

    Synovial sarcoma is a malignant mesenchymal neoplasm of uncertain histogenesis, characterized by a specific SS18-SSX fusion. The diagnosis of synovial sarcoma can be challenging based on morphology and conventional immunohistochemistry alone, and identification of the fusion gene by molecular genetics may be necessary for diagnosis. Several recent studies have demonstrated the diagnostic utility of the reduced expression of SMARCB1 in synovial sarcomas as measured using immunohistochemistry. Therefore, we undertook a validation study using synovial sarcomas and other spindle or round cell tumors that could enter differential diagnosis of monophasic or poorly differentiated synovial sarcomas. Among 36 synovial sarcomas that were successfully evaluated, the expression of SMARCB1 was diffusely reduced in 33 cases (92%) at variable degrees. In contrast, the expression of SMARCB1 was not reduced in any of the 93 evaluable non-synovial sarcoma tumors (5 thymomas, 5 sarcomatoid mesotheliomas, 10 schwannomas, 9 mesenchymal chondrosarcomas, 20 solitary fibrous tumors, 19 Ewing sarcomas, and 25 malignant peripheral nerve sheath tumors). A few schwannomas and malignant peripheral nerve sheath tumors showed mosaic or complete loss of SMARCB1 expression. Reduced expression of SMARCB1 immunoreactivity was therefore found to be highly sensitive and specific for synovial sarcoma, and can be useful for rapidly and accurately confirming the diagnosis of synovial sarcoma. This reduction in SMARCB1 expression likely reflects the BAF47 ejection mechanism of the SS18-SSX fusion product and can therefore be viewed as an indirect visualization of this fusion product. PMID:26520417

  6. Definitive intraoperative radiotherapy for musculoskeletal sarcomas and malignant lymphoma in combination with surgical excision

    International Nuclear Information System (INIS)

    The purpose was to estimate retrospectively the outcome of patients with musculoskeletal sarcoma or malignant lymphoma treated with intraoperative radiotherapy (IORT). Between 1988 and 1999, definitive IORT in combination with surgical excision was performed in 24 patients with musculoskeletal sarcoma (malignant fibrous histiocytoma, 10; osteosarcoma, 6; liposarcoma, 2; chondrosarcoma, 1; synovial sarcoma, 1; Ewing's sarcoma, 1; angiosarcoma, 1; epithelioid sarcoma, 1; malignant schwannoma, 1; and 3 patients with malignant lymphoma. The tumor was excised by marginal margin excision, intralesional margin excision, or wide margin excision; 15-45 Gy electrons was then delivered to the affected sites. In the 8 patients without distant metastases at the first visit, 4 patients are alive 6.5-11.5 years after IORT, and 4 patients died <4.5 years after IORT. The incidence of local recurrence was 13%. In the 19 patients with distant metastases at the first visit, 3 patients are alive 2.5-6.7 years after IORT, and 16 patients died 0.2-5.7 years after IORT. The incidence of local recurrence was 45%. Complications after IORT were found in 5 patients: neuropathy, 1 patient; skin necrosis, 1 patient; myelopathy, 1 patient; enteritis ileus, 1 patient; and edema, 1 patient. IORT with a radiation dose of 15-45 Gy in combination with surgical excision appeared to be useful for local control and to be more effective in patients without distant metastases at the first visit than in patients with distant metastases. (author)

  7. Gamma Knife Radiosurgery as a Therapeutic Strategy for Intracranial Sarcomatous Metastases

    International Nuclear Information System (INIS)

    Purpose: To determine the indication and outcomes for Gamma Knife stereotactic radiosurgery (GKSRS) in the care of patients with intracranial sarcomatous metastases. Methods and Materials: Data from 21 patients who underwent radiosurgery for 60 sarcomatous intracranial metastases (54 parenchymal and 6 dural-based) were studied. Nine patients had radiosurgery for solitary tumors and 12 for multiple tumors. The primary pathology was metastatic leiomyosarcoma (4 patients), osteosarcoma (3 patients), soft-tissue sarcoma (5 patients), chondrosarcoma (2 patients), alveolar soft part sarcoma (2 patients), and rhabdomyosarcoma, Ewing's sarcoma, liposarcoma, neurofibrosarcoma, and synovial sarcoma (1 patient each). Twenty patients received multimodality management for their primary tumor, and 1 patient had no evidence of systemic disease. The mean tumor volume was 6.2 cm3 (range, 0.07-40.9 cm3), and a median margin dose of 16 Gy was administered. Three patients had progressive intracranial disease despite fractionated whole-brain radiotherapy before SRS. Results: A local tumor control rate of 88% was achieved (including patients receiving boost, up-front, and salvage SRS). New remote brain metastases developed in 7 patients (33%). The median survival after diagnosis of intracranial metastasis was 16 months, and the 1-year survival rate was 61%. Conclusions: Gamma Knife radiosurgery was a well-tolerated and initially effective therapy in the management of patients with sarcomatous intracranial metastases. However, many patients, including those who also received fractionated whole-brain radiotherapy, developed progressive new brain disease.

  8. Four year experience of sarcoma of soft tissues and bones in a tertiary care hospital and review of literature

    Directory of Open Access Journals (Sweden)

    Ansari Tayyaba Z

    2011-05-01

    Full Text Available Abstract Background Sarcoma encompasses an uncommon group of cancer and the data is insufficient from Pakistan. We report our four years experience of Sarcoma of soft tissues and bones. Methods This cross sectional study was carried out at Aga Khan University Hospital from 2004 to 2008. The patients were divided into two groups from the outset i.e. initially diagnosed and relapsed group and separate sub group analysis was conducted. Results Out of 93 newly diagnosed patients, 58 belonged to bone sarcoma and 35 to soft tissue sarcoma group. While for relapsed patients, 5 had soft tissue sarcoma and 9 had bone sarcoma. Mean age was 32.5 years. At presentation, approximately two third patients had localised disease while remaining one third had metastatic disease. The Kaplan Meier estimate of median recurrence free survival was 25 months, 35 months, and 44 months for Osteogenic sarcoma, Ewing's sarcoma and Chondrosarcoma respectively. For Leiomyosarcoma and Synovial sarcoma, it was 20 and 19 months respectively. The grade of the tumour (p = 0.02 and surgical margin status (p = 0.001 were statistically significant for determination of relapse of disease. Conclusion The median recurrence free survival of patients in our study was comparable to the reported literature but with significant lost to follow rate. Further large-scale, multi centre studies are needed to have a more comprehensive understanding of this heterogeneous disease in our population.

  9. 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.)

  10. MR appearance of skeletal neoplasms following cryotherapy

    International Nuclear Information System (INIS)

    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.)

  11. Imaging of the larynx and hypopharynx.

    Science.gov (United States)

    Becker, Minerva; Burkhardt, Karim; Dulguerov, Pavel; Allal, Abdelkarim

    2008-06-01

    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. PMID:18495402

  12. Imaging of the larynx and hypopharynx

    International Nuclear Information System (INIS)

    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

  13. Serum- and glucocorticoid-regulated kinase 1 (SGK1) induction by the EWS/NOR1(NR4A3) fusion protein

    International Nuclear Information System (INIS)

    The NR4A3 nuclear receptor (also known as NOR1) is involved in tumorigenesis by the t(9;22) chromosome translocation encoding the EWS/NOR1 fusion protein found in approximately 75% of all cases of extraskeletal myxoid chondrosarcomas (EMC). Several observations suggest that one role of EWS/NOR1 in tumorigenesis may be to deregulate the expression of specific target genes. We have shown previously that constitutive expression of EWS/NOR1 in CFK2 fetal rat chondrogenic cells induces their transformation as measured by growth beyond confluency and growth in soft agar. To identify genes regulated by the fusion protein in this model, we have generated a CFK2 cell line in which the expression of EWS/NOR1 is controlled by tetracycline. Using the differential display technique, we have identified the serum- and glucocorticoid-regulated kinase 1 (SGK1) mRNA as being up-regulated in the presence of EWS/NOR1. Co-immunocytochemistry confirmed over-expression of the SGK1 protein in cells expressing EWS/NOR1. Significantly, immunohistochemistry of 10 EMC tumors positive for EWS/NOR1 showed that all of them over-express the SGK1 protein in contrast to non-neoplastic cells in the same biopsies and various other sarcoma types. These results strongly suggest that SGK1 may be a genuine in vivo target of EWS/NOR1 in EMC

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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. PMID:22197093

  16. Usefulness of [18F]FDG-PET in diagnosis of bone and soft tissue tumors. Study with multi-center survey by questionnaire

    International Nuclear Information System (INIS)

    In the aspect of future additional approval of [18F]fluorodeoxyglucose positron emission tomography (FDG-PET) diagnosis of the title tumors in the health insurance, its usefulness was studied by questionnaire to 18 facilities, where PET had been conducted for those tumors in the period July, 2005-February, 2006. Major questions concerned the purpose and finding of PET, findings by other imaging and by tumor markers, and judgment of PET effectiveness compared with other imaging (more useful, equally or less, and its reason). Subjects were 75 cases (42 males, 33 females; 3-82 years old) in 20 diseases, which involved 21 cases of osteosarcoma, 7 of leiomyosarcoma, 8 of Ewing sarcoma, 6 of liposarcoma, 5 of hemangiosarcoma, 4 of synovial sarcoma, each 3 of rhabdomyosarcoma, giant cell tumor, Schwannoma, malignant fibrous histiocytoma, each 2 of chondrosarcoma, alveolar soft part sarcoma, each one of epithelioid sarcoma, endometrial storomal sarcoma, hibernoma, fibrosarcoma, multiple osteochondroma, sacral chondroma, Langerhans cell histiocytosis and neurofibromatosis. Obtained were the judgments of highly useful in 5 diseases, fairly useful in 4, useful in 3, and useful/inconclusive due to the only one case in 8. FDG-PET was thus found useful in all diseases examined. (R.T.)

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

    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.

  18. 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.

  19. 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.

  20. The role of projectional radiography in the detection of primary malignant and indeterminate bone neoplasms; Detektion von primaer malignen und intermediaeren Knochentumoren: Wertigkeit der Projektionsradiographie

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, C.; Imhof, H.; Kainberger, F. [Abt. fuer Osteoradiologie, Univ. fuer Radiodiagnostik, Medizinische Univ. Wien (Austria); Huber, W. [Univ. fuer Orthopaedie, Medizinische Univ. Wien (Austria)

    2005-02-01

    Purpose: to evaluate the role of projectional radiography in the detection of primary and potentially malignant bone neoplasms. Materials and methods: we retrospectively reviewed the images and medical records of 80 patients (42 male, 38 female; range: 6 to 73 years; mean: 33 years) with pathologically proven primary or indeterminate bone tumors. Results: the most common symptom was local pain (84%). In 69 of 80 patients (86%), projectional radiography was the first imaging modality, which correctly identified the malignant character of the bone lesion in 60 of these 69 patients (87%). In 2 patients, bone neoplasms were overlooked due to diagnostically inadequate image quality. The false-diagnosis rate was not significantly different between board-certified radiologists working in hospitals and radiologists in outpatient facilities (p>0.05). Bone tumors predominantly affecting adults, such as chondrosarcomas, needed a longer time for the diagnosis than bone tumors mainly found in childhood, such as osteosarcomas. The median age of patients with delayed imaging work-up because of attempted conservative therapy (45 years{+-}20 years) was significant higher than the median age of patients with immediate radiographic work-up (27 years{+-}16 years). (orig.)

  1. Percutaneous needle aspiration biopsy of localized pulmonary lesions

    Energy Technology Data Exchange (ETDEWEB)

    Im, Chung Kie; Lim, Duck; Park, Jae Hyung; Ham, Eui Keun [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1984-12-15

    Over a period of recent two years, 100 patients who had localized pulmonary lesions and underwent percutaneous needle aspiration and biopsy, were analyzed. There were 56 malignancies and 44 benign lesions. The diagnostic accuracy of malignancy including specific cell type and benign disease are 89% and 79% respectively. Differentiation of malignancy vs. benignity was possible in 89% of cases obviating unnecessary exploratory thoracotomy for diagnostic purpose. Five cases were misinterpreted and eight cases were non-diagnostic on cytology. Inadequate material was obtained in two cases; one was due to hardness of the mass, which, later, confirmed as chondrosarcoma, and the other was too small (0.8 X 1.0 cm) to be visible on lateral view. The obtainability of the tissue was 98%. 14 (14%) patients developed pneumothorax; one of them required treatment and remainder showed spontaneous resporption. (Transient neglibigle blood tinged sputum was found in 16 (16%) cases). The method, problems and complication are discussed. Authors recommend the percutaneous needle aspiration and biopsy as the initial procedure in diagnostic work up of pulmonary coin lesions, especially when they are smaller, more peripheral and metastatic neoplasm is suspected.

  2. Elasticity, biodegradability and cell adhesive properties of chitosan/hyaluronan multilayer films

    Energy Technology Data Exchange (ETDEWEB)

    Schneider, Aurore; Richert, Ludovic; Francius, Gregory; Voegel, Jean-Claude; Picart, Catherine [Present address: Universite de Montpellier II, CNRS-UMR 5539, cc107, Place Eugene Bataillon, 34 095 Montpellier Cedex 5 (France)

    2007-03-01

    In the bioengineering field, a recent and promising approach to modifying biomaterial surfaces is the layer-by-layer (LbL) technique used to build thin polyelectrolyte multilayer films. In this work, we focused on polyelectrolyte multilayer films made of two polysaccharides, chitosan (CHI) and hyaluronan (HA), and on the control of their physico-chemical and cell adhesive properties by chemical cross-linking. CHI/HA films were cross-linked using a water soluble carbodiimide and observed by confocal laser scanning microscopy (CLSM) with a fluorescently labeled CHI. Film thicknesses were similar for native and cross-linked films. The film nanometer roughness was measured by atomic force microscopy and was found to be higher for cross-linked films. Cross-linking the films also leads to a drastic change in film stiffness. The elastic modulus of the films (Young's modulus) as measured by AFM nano-indentation was about tenfold increased for cross-linked films as compared to native ones. From a biological point of view, cross-liked films are more resistant to enzymatic degradation by hyaluronidase. Furthermore, the increase in film stiffness has a favorable effect on the adhesion and spreading of chondrosarcoma cells. Thus, the CHI/HA cross-linked films could be used for various applications due to their adhesive properties and to their mechanical properties (including stability in enzymatic media)

  3. Simvastatin inhibits CD44 fragmentation in chondrocytes.

    Science.gov (United States)

    Terabe, Kenya; Takahashi, Nobunori; Takemoto, Toki; Knudson, Warren; Ishiguro, Naoki; Kojima, Toshihisa

    2016-08-15

    In human osteoarthritic chondrocytes, the hyaluronan receptor CD44 undergoes proteolytic cleavage at the cell surface. CD44 cleavage is thought to require transit of CD44 into cholesterol-rich lipid rafts. The purpose of this study was to investigate whether statins exert a protective effect on articular chondrocytes due to diminution of cholesterol. Three model systems of chondrocytes were examined including human HCS-2/8 chondrosarcoma cells, human osteoarthritic chondrocytes and normal bovine articular chondrocytes. Treatment with IL-1β + Oncostatin M resulted in a substantial increase in CD44 fragmentation in each of the three chondrocyte models. Pre-incubation with simvastatin prior to treatment with IL-1β + Oncostatin M decreased the level of CD44 fragmentation, decreased the proportion of CD44 that transits into the lipid raft fractions, decreased ADAM10 activity and diminished the interaction between CD44 and ADAM10. In HCS-2/8 cells and bovine articular chondrocytes, fragmentation of CD44 was blocked by the knockdown of ADAM10. Inhibition of CD44 fragmentation by simvastatin also resulted in improved retention of pericellular matrix. Addition of cholesterol and farnesyl-pyrophosphate reversed the protective effects of simvastatin. Thus, the addition of simvastatin exerts positive effects on chondrocytes including reduced CD44 fragmentation and enhanced the retention of pericellular matrix. PMID:27242325

  4. Elasticity, biodegradability and cell adhesive properties of chitosan/hyaluronan multilayer films

    Science.gov (United States)

    Schneider, Aurore; Richert, Ludovic; Francius, Gregory; Voegel, Jean-Claude; Picart, Catherine

    2007-03-01

    In the bioengineering field, a recent and promising approach to modifying biomaterial surfaces is the layer-by-layer (LbL) technique used to build thin polyelectrolyte multilayer films. In this work, we focused on polyelectrolyte multilayer films made of two polysaccharides, chitosan (CHI) and hyaluronan (HA), and on the control of their physico-chemical and cell adhesive properties by chemical cross-linking. CHI/HA films were cross-linked using a water soluble carbodiimide and observed by confocal laser scanning microscopy (CLSM) with a fluorescently labeled CHI. Film thicknesses were similar for native and cross-linked films. The film nanometer roughness was measured by atomic force microscopy and was found to be higher for cross-linked films. Cross-linking the films also leads to a drastic change in film stiffness. The elastic modulus of the films (Young's modulus) as measured by AFM nano-indentation was about tenfold increased for cross-linked films as compared to native ones. From a biological point of view, cross-liked films are more resistant to enzymatic degradation by hyaluronidase. Furthermore, the increase in film stiffness has a favorable effect on the adhesion and spreading of chondrosarcoma cells. Thus, the CHI/HA cross-linked films could be used for various applications due to their adhesive properties and to their mechanical properties (including stability in enzymatic media).

  5. Reconstruction of complex thoraco-abdominal defects with extended anterolateral thigh flap

    Directory of Open Access Journals (Sweden)

    Yadav Prabha

    2010-01-01

    Full Text Available Background: The reconstruction of complex thoraco-abdominal defects following tumour ablative procedures has evolved over the years from the use of pedicle flaps to free flaps. The free extended anterolateral thigh flap is a good choice to cover large defects in one stage. Materials and Methods: From 2004 to 2009, five patients with complex defects of the thoracic and abdominal wall following tumour ablation were reconstructed in one stage and were studied. The commonest tumour was chondrosarcoma. The skeletal component was reconstructed with methylmethacrylate bone cement and polypropylene mesh and the soft tissue with free extended anterolateral thigh flap. The flaps were anastomosed with internal mammary vessels. The donor sites of the flaps were covered with split-skin graft. Result: All the flaps survived well. One flap required re-exploration for venous congestion and was successfully salvaged. Two flaps had post operative wound infection and were managed conservatively. All flap donor sites developed hyper-pigmentation, contour deformity and cobble stone appearance. Conclusion: Single-stage reconstruction of the complex defects of the thoraco-abdominal region is feasible with extended anterolateral thigh flap and can be adopted as the first procedure of choice.

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

    International Nuclear Information System (INIS)

    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

  7. Bone and soft tissue tumors of hip and pelvis

    International Nuclear Information System (INIS)

    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.

  8. High Z elements in human sarcomata: assessment by multienergy CT and neutron activation analysis

    International Nuclear Information System (INIS)

    Tumor equivalent phantoms containing inorganic salts (KH2PO4, CH3COOK, NaCl and KI) were scanned on an EMI 5005 body scanner at 140 kVp, 28 mA; 120 kVp, 33 mA; and 81 kVp, 42 mA. Significant signal gain for the detection of higher atomic number elements by multiple energy scanning was noted. Certain sarcomas are known to accumulate high Z elements. Accordingly, excised specimens of various histologies of human sarcomata (chondrosarcoma, liposarcoma, and malignant fibrous histiocytoma) were scanned at 140 kVp and 81 kVp. Using selected areas of interest in the computed tomographic (CT) image to direct the in vitro biopsy of various regions of excised tumors, intersting correlations between the CT number variation and the respective, high Z elemental composition variation, as determined by thermal neutron activation analysis were observed. Further investigation with phantoms and excised sarcomata at 62 kVp and 42 mA suggested that dual energy CT scanning (at 140 kVp and 62 kVp) may be a method of monitoring effective Z and heavy element compositional changes. The authors are also attempting to develop these same low kilovoltage techniques as a method for the noninvasive clinical monitoring of an antisarcoma chemotherapeutic agent, cis-diamminedichloroplatinum

  9. Hyperthermia combined with radiotherapy for soft tissue sarcoma

    International Nuclear Information System (INIS)

    Soft tissue sarcomas and malignant melanomas were thought to be radioresistant and few have been controlled with conventional radiotherapy. The results of the treatment of thirteen patients with seventeen lesions at Osaka City University using hyperthermia combined with radiotherapy (HT + RT) are reviewed. Four lesions were discarded due to salvage operation immediately after the treatment or to the early interruption of heat treatment under 3 sessions. Histologic tumor types included : four fibrosarcomas, three malignant teratomas, one hemangiopericytoma, embryonal cell carcinoma, malignant fibrous histiocytoma, leiomyosarcoma, chondrosarcoma and malignant melanoma. There were five complete and five partial responses and three no responses (response rate 77 %). The response rate of superficial tumors was 88 % and that of deep tumors was 60 %. The response rates of squamous cell carcinoma and adenocarcinoma treated with RT + HT were 92 % and 88 % for superficial tumors and 50 % and 64 % for deep seated tumors, respectively. This results indicate that the response rate of soft tissue sarcoma is same as squamous cell carcinoma or adenocarcinoma and HT + RT is useful for the treatment of soft tissue sarcomas. (author)

  10. 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)

  11. 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.)

  12. Malignant tumors arising in the maxillary region after radiation therapy

    International Nuclear Information System (INIS)

    Although radiotherapy has proven of great therapeutic value in the treatment of malignant tumors, it should also be borne in mind that radiation has a serious potential risk of giving rise to a secondary malignancy. We recently experienced 2 cases each of carcinoma and sarcoma arising in the irradiated areas long after radiation therapy for malignant tumors. In these 4 cases, 2 males and 2 females, the primary neoplastic diseases were squamous cell carcinoma, epidermoid carcinoma, carcinoma of unknown pathology and malignant lymphoma, and the secondary tumors were epidermoid carcinoma, squamous cell carcinoma, osteosarcoma and chondrosarcoma, respectively. The sites of occurrence of these malignancies were invariably in the maxillary region; the mean latent period was 15 years, aside from an infantile case with a latent period of 5 years. In view of the primary diseases being malignant tumors the following criteria were set up for the diagnosis of radiation-induced malignancies: (1) the site of occurrence is within the confines of a previously irradiated area, (2) the latent period is prolonged and (3) the malignancy occurs as a double tumor. Therapy was primarily by operation. The prognosis was exceedingly ominous, the average survival time being 22 months. This was probably and mainly because of rapidity of tumor growth. Thus, the secondary tumors had already spread back to inward by the time they were first discovered. This should be kept in mind during a long-term follow-up of patients receiving radiotherapy for malignancy. (author)

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

    Science.gov (United States)

    Lee, Dae Young; Choi, Soo-Im; Han, Se Hee; Lee, Ye-Joo; Choi, Jong-Gil; Lee, Young-Seob; Choi, Je Hun; Lee, Seung-Eun; Kim, Geum-Soog

    2016-01-01

    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, (13)C, 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. PMID:27548143

  14. 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

    International Nuclear Information System (INIS)

    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 T1- and T2-weighted spin echo as well as T2*-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 SIo-SIm/SIo. 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.)

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

    International Nuclear Information System (INIS)

    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

  16. New SPECT tracers: Example of tracers of proteoglycans and melanin

    International Nuclear Information System (INIS)

    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 99mTc-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 18F-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)

  17. Multifaceted impact of trichothecene metabolites on plant-microbe interactions and human health.

    Science.gov (United States)

    Kumari, Indu; Ahmed, Mushtaq; Akhter, Yusuf

    2016-07-01

    Fungi present in rhizosphere produce trichothecene metabolites which are small in size and amphipathic in nature and some of them may cross cell membranes passively. Hypocreaceae family of rhizosphere fungi produce trichothecene molecules, however it is not a mandatory characteristic of all genera. Some of these molecules are also reported as growth adjuvant, while others are reported as deleterious for the plant growth. In this review, we are exploring the roles of these compounds during plant-microbe interactions. The three-way interaction among the plants, symbiotic microbial agents (fungi and bacteria), and the pathogenic microbes (bacteria, fungi) or multicellular pathogens like nematodes involving these compounds may only help us to understand better the complex processes happening in the microcosm of rhizosphere. These metabolites may further modulate the activity of different proteins involved in the cell signalling events of defence-related response in plants. That may induce the defence system against pathogens and growth promoting gene expression in plants, while in animal cells, these molecules have reported biochemical and pharmacological effects such as inducing oxidative stress, cell-cycle arrest and apoptosis, and may be involved in maintenance of membrane integrity. The biochemistry, chemical structures and specific functional group-mediated activity of these compounds have not been studied in details yet. Few of these molecules are also recently reported as novel anti-cancer agent against human chondrosarcoma cells. PMID:27198722

  18. Occurrence of bone cancer among young adult Beagles given 239Pu

    International Nuclear Information System (INIS)

    Two hundred thirty-five young adult Beagles of both sexes were each given a single intravenous injection of 239Pu-citrate at graded dose-levels averaging about 0.026 to 106 kBq/kg when they were about 1 1/2 years of age and were maintained for lifespan observation. An additional 133 young adult Beagles of both sexes were entered into the experiment as control animals. All of these animals have now died or have been removed from the colony, and the occurrence of skeletal malignancies has been determined from histological examination. There were a total of 85 radiographically apparent malignant bone tumors in 77 dogs given 239Pu, and there was one control animal that developed a skeletal malignancy. Most of these were osteosarcomas, but there were seven chondrosarcomas of bone, one liposarcoma of bone, and in addition, there was one plasma cell myeloma and one ameloblastoma (admantinoma). Only those dogs that survived to at least the minimum latent period for death with radiation-induced bone sarcoma are included in the tabulation. There appeared to be a linear relationship between the percent of dogs with bone tumor and the average skeletal dose up to a dose of about 1 Gy. All dose-levels with skeletal doses of about 2 Gy and greater exhibited close to 100% occurrence

  19. 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)

  20. Oncogenic conversion of a novel orphan nuclear receptor by chromosome translocation.

    Science.gov (United States)

    Labelle, Y; Zucman, J; Stenman, G; Kindblom, L G; Knight, J; Turc-Carel, C; Dockhorn-Dworniczak, B; Mandahl, N; Desmaze, C; Peter, M

    1995-12-01

    A recurrent t(9;22) (q22;q12) chromosome translocation has been described in extraskeletal myxoid chondrosarcoma (EMC). Fluorescent in situ hybridization experiments performed on one EMC tumour indicated that the chromosome 22 breakpoint occurred in the EWS gene. Northern blot analysis revealed an aberrant EWS transcript which is cloned by a modified RT-PCR procedure. This transcript consists of an in-frame fusion of the 5' end of EWS to a previously unidentified gene, which was named TEC. This fusion transcript was detected in six of eight EMC studied, and three different junction types between the two genes were found. In all junction types, the putative translation product contained the amino-terminal transactivation domain of EWS linked to the entire TEC protein. Homology analysis showed that the predicted TEC protein contains a DNA-binding domain characteristic of nuclear receptors. The highest identity scores were observed with the NURR1 family of orphan nuclear receptors. These receptors are involved in the control of cell proliferation and differentiation by modulating the response to growth factors and retinoic acid. This work provides, after the PML/RAR alpha gene fusion, the second example of the oncogenic conversion of a nuclear receptor and the first example involving the orphan subfamily. Analysis of the disturbance induced by the EWS/TEc protein in the nuclear receptor network and their target genes may lead to new approaches for EMC treatment. PMID:8634690

  1. Mutations in isocitrate dehydrogenase 1 and 2 occur frequently in intrahepatic cholangiocarcinomas and share hypermethylation targets with glioblastomas.

    Science.gov (United States)

    Wang, P; Dong, Q; Zhang, C; Kuan, P-F; Liu, Y; Jeck, W R; Andersen, J B; Jiang, W; Savich, G L; Tan, T-X; Auman, J T; Hoskins, J M; Misher, A D; Moser, C D; Yourstone, S M; Kim, J W; Cibulskis, K; Getz, G; Hunt, H V; Thorgeirsson, S S; Roberts, L R; Ye, D; Guan, K-L; Xiong, Y; Qin, L-X; Chiang, D Y

    2013-06-20

    Mutations in the genes encoding isocitrate dehydrogenase, IDH1 and IDH2, have been reported in gliomas, myeloid leukemias, chondrosarcomas and thyroid cancer. We discovered IDH1 and IDH2 mutations in 34 of 326 (10%) intrahepatic cholangiocarcinomas. Tumor with mutations in IDH1 or IDH2 had lower 5-hydroxymethylcytosine and higher 5-methylcytosine levels, as well as increased dimethylation of histone H3 lysine 79 (H3K79). Mutations in IDH1 or IDH2 were associated with longer overall survival (P=0.028) and were independently associated with a longer time to tumor recurrence after intrahepatic cholangiocarcinoma resection in multivariate analysis (P=0.021). IDH1 and IDH2 mutations were significantly associated with increased levels of p53 in intrahepatic cholangiocarcinomas, but no mutations in the p53 gene were found, suggesting that mutations in IDH1 and IDH2 may cause a stress that leads to p53 activation. We identified 2309 genes that were significantly hypermethylated in 19 cholangiocarcinomas with mutations in IDH1 or IDH2, compared with cholangiocarcinomas without these mutations. Hypermethylated CpG sites were significantly enriched in CpG shores and upstream of transcription start sites, suggesting a global regulation of transcriptional potential. Half of the hypermethylated genes overlapped with DNA hypermethylation in IDH1-mutant gliobastomas, suggesting the existence of a common set of genes whose expression may be affected by mutations in IDH1 or IDH2 in different types of tumors. PMID:22824796

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  3. Neuropsychological function in adults after high dose fractionated radiation therapy of skull base tumors

    International Nuclear Information System (INIS)

    Purpose: To evaluate the long term effects of high dose fractionated radiation therapy on brain functioning prospectively in adults without primary brain tumors. Methods and Materials: Seventeen patients with histologically confirmed chordomas and low grade chondrosarcomas of the skull base were evaluated with neuropsychological measures of intelligence, language, memory, attention, motor function and mood following surgical resection/biopsy of the tumor prior to irradiation, and then at about 6 months, 2 years and 4 years following completion of treatment. None received chemotherapy. Results: In the patients without tumor recurrence or radiation necrosis, there were no indications of adverse effects on cognitive functioning in the post-acute through the late stages after brain irradiation. Even in patients who received doses of radiation up to 66 Cobalt Gy equivalent through nondiseased (temporal lobe) brain tissue, memory and cognitive functioning remained stable for up to 5 years after treatment. A mild decline in psycho-motor speed was seen in more than half of the patients, and motor slowing was related to higher radiation doses in midline and temporal lobe brain structures. Conclusion: Results suggest that in adults, tolerance for focused radiation is relatively high in cortical brain structures

  4. The clinical value of combined use of MR imaging and multi-slice spiral CT in limb salvage surgery for orthopaedic oncology patients: initial experience in nine patients

    International Nuclear Information System (INIS)

    The purpose of this prospective study was to evaluate the value of the combined use of MR imaging and multi-slice spiral CT for limb salvage surgery in orthopaedic oncology patients. Nine consecutive patients with lower/upper limb malignant bone tumours (7 osteosarcomas and 2 chondrosarcomas) were treated with limb-salvaging procedures. Preoperative planning including determination of the osteotomy plane and diameters of the prosthesis was performed basing on the preoperative CT and MR images. The histopathology was performed as golden diagnostic criteria to evaluate the accuracy of CT and MR-based determination for tumour’s boundary. The tumour extension measured on MRI was consistent with the actual extension (P>0.05, paired Student’s t test), while the extension measured on CT imaging was less than the actual extension. The length, offset and alignment of the affected limb were reconstructed accurately after the operation. An excellent functional outcome was achieved in all patients. In the present study, MRI was found to be superior to CT for determining the tumour extension, combined use of MRI and CT measurement provided high precision for the fit of the prosthesis and excellent functional results

  5. Hyperthermia combined with radiotherapy for soft tissue sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Tanaka, Masahiro; Nakajima, Toshifumi; Kida, Akio; Fukuda, Haruyuki; Tsumura, Masashi; Sugimoto, Kiyoshi; Nishio, Hiroshi; Sakashita, Mariko; Onoyama, Yasuto

    1987-12-01

    Soft tissue sarcomas and malignant melanomas were thought to be radioresistant and few have been controlled with conventional radiotherapy. The results of the treatment of thirteen patients with seventeen lesions at Osaka City University using hyperthermia combined with radiotherapy (HT + RT) are reviewed. Four lesions were discarded due to salvage operation immediately after the treatment or to the early interruption of heat treatment under 3 sessions. Histologic tumor types included : four fibrosarcomas, three malignant teratomas, one hemangiopericytoma, embryonal cell carcinoma, malignant fibrous histiocytoma, leiomyosarcoma, chondrosarcoma and malignant melanoma. There were five complete and five partial responses and three no responses (response rate 77 %). The response rate of superficial tumors was 88 % and that of deep tumors was 60 %. The response rates of squamous cell carcinoma and adenocarcinoma treated with RT + HT were 92 % and 88 % for superficial tumors and 50 % and 64 % for deep seated tumors, respectively. This results indicate that the response rate of soft tissue sarcoma is same as squamous cell carcinoma or adenocarcinoma and HT + RT is useful for the treatment of soft tissue sarcomas.

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

    International Nuclear Information System (INIS)

    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)

  7. 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.

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

    Directory of Open Access Journals (Sweden)

    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%.

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

    International Nuclear Information System (INIS)

    TAF15 (formerly TAFII68) 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.

  10. Prognosis value of Hypoxia-inducible factor-1α expression in patients with bone and soft tissue sarcoma: a meta-analysis.

    Science.gov (United States)

    Li, Yongjiang; Zhang, Wenbiao; Li, Shuangjiang; Tu, Chongqi

    2016-01-01

    The prognostic significance of Hypoxia-inducible factor-1α (HIF-1α) in patients with bone and soft tissue sarcoma remains controversial. To investigate the impact of its expression on survival outcomes, we performed a meta-analysis. Comprehensive literature searches were conducted in PubMed, Web of Science, Embase and Cochrane Library. A total of 16 studies published from 2006 to 2015 were included. We found that expression of HIF-1α was significantly associated with higher rate of metastasis (RR 3.21, 95 % CI 2.12-4.84, P < 0.001), poorer overall survival (HR 2.05, 95 % CI 1.51-2.77, P < 0.001) and poorer disease-free survival (HR 2.05, 95 % CI 1.55-2.70, P < 0.001). In addition, when subgroup analysis was conducted according to histology type, the significant correlations to poor overall survival and disease-free survival were also observed in patients with osteosarcoma, chondrosarcoma and soft tissue sarcoma. Publication bias was not found and sensitivity analysis showed the results were stable. In conclusion, HIF-1α expression might be an effective predicative factor of poor prognosis for bone and soft tissue sarcoma. PMID:27606158

  11. 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.

  12. Heavy charged particle radiotherapy trial

    International Nuclear Information System (INIS)

    Through mid-1985, a total of 49 patients received heavy-charged-particle irradiation for chordoma, chondrosarcoma, meningioma, or neurilemmoma of the base of skull or juxtaspinal area. The mean tumor dose was 68 Gray-equivalent, ranging from 26 to 80. Control within the irradiated area was obtained in 35 of 49. The median follow up in all 49 patients is 21 months, with a range from 3-90 months. Serious complications were seen in a small number of patients, with cranial nerve injury in two, transverse myelitis in one, and brain necrosis in three patients. In 42 patients with tumors of other histologies and/or sites, including tumors of paranasal sinuses, retroperitoneum, soft tissue and miscellaneous other sites, heavy charged particles were also used to deliver a higher tumor dose than possible with standard irradiation techniques. In the group, 21/42 (50%) have had local tumor control, also a good result considering the extent and the range of tumor types treated. The authors believe that there are a number of sites in addition to the juxtaspinal/base of skull tumors that will show long term benefit from treatment with heavy charged particles

  13. Percutaneous needle aspiration biopsy of localized pulmonary lesions

    International Nuclear Information System (INIS)

    Over a period of recent two years, 100 patients who had localized pulmonary lesions and underwent percutaneous needle aspiration and biopsy, were analyzed. There were 56 malignancies and 44 benign lesions. The diagnostic accuracy of malignancy including specific cell type and benign disease are 89% and 79% respectively. Differentiation of malignancy vs. benignity was possible in 89% of cases obviating unnecessary exploratory thoracotomy for diagnostic purpose. Five cases were misinterpreted and eight cases were non-diagnostic on cytology. Inadequate material was obtained in two cases; one was due to hardness of the mass, which, later, confirmed as chondrosarcoma, and the other was too small (0.8 X 1.0 cm) to be visible on lateral view. The obtainability of the tissue was 98%. 14 (14%) patients developed pneumothorax; one of them required treatment and remainder showed spontaneous resporption. (Transient neglibigle blood tinged sputum was found in 16 (16%) cases). The method, problems and complication are discussed. Authors recommend the percutaneous needle aspiration and biopsy as the initial procedure in diagnostic work up of pulmonary coin lesions, especially when they are smaller, more peripheral and metastatic neoplasm is suspected.

  14. 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.

  15. Plastic surgery in chest wall reconstruction: relevant aspects - case series

    Directory of Open Access Journals (Sweden)

    Diogo Franco

    2015-12-01

    Full Text Available Objective: to discuss the participation of Plastic Surgery in the reconstruction of the chest wall, highlighting relevant aspects of interdisciplinaryness. Methods: we analyzed charts from 20 patients who underwent extensive resection of the thoracic integument, between 2000 and 2014, recording the indication of resection, the extent and depth of the raw areas, types of reconstructions performed and complications. Results: among the 20 patients, averaging 55 years old, five were males and 15 females. They resections were: one squamous cell carcinoma, two basal cell carcinomas, five chondrosarcomas and 12 breast tumors. The extent of the bloody areas ranged from 4x9 cm to 25x40 cm. In 12 patients the resection included the muscular plane. In the remaining eight, the tumor removal achieved a total wall thickness. For reconstruction we used: one muscular flap associated with skin grafting, nine flaps and ten regional fasciocutaneous flaps. Two patients undergoing reconstruction with fasciocutaneous flaps had partially suffering of the flap, solved with employment of a myocutaneous flap. The other patients displayed no complications with the techniques used, requiring only one surgery. Conclusion: the proper assessment of local tissues and flaps available for reconstruction, in addition to the successful integration of Plastic Surgery with the specialties involved in the treatment, enable extensive resections of the chest wall and reconstructions that provide patient recovery.

  16. 骨外黏液样软骨肉瘤的临床病理分析%Extraskeletal myxoid chondrosarcoma:a clinicopathological analysis

    Institute of Scientific and Technical Information of China (English)

    方三高; 李昱; 马强; 杜娟; 林俐; 肖华亮

    2013-01-01

    Background and purpose: Extraskeletal myxoid chondrosarcoma (EMC) is a malignancy of uncertain differentiation tumor characterized by a multinodular structure and abundant myxoid matrix. Its preferred sites were the deep soft tissues of the extremities. The aim of this study was to investigate the clinicopathologic characteristics, diagnosis and differential diagnosis of EMC. Methods: Seven cases of EMC were analyzed for clinicopathological and immunohistochemical features with review of the related literature. Results: It occurred predominantly in females (male/female=2︰5). Five cases were located in low extremities and two in upper limb girdles, more commonly near the joint. Grossly, the masses showed as grey, lobular and somewhat transparent with a relatively well-deifned margin. Microscopically, the small ovary or plump spindle-shaped cells arranged in strand and cord patterns and lobular architecture which separated by delicate fibrous networks with an abundant myxoid but hypovascular background. And the tumors were immunoreactive for vimentin, and partly for S-100 and EMA, meanwhile, negative for CK. Conclusion:EMC is a rare soft tissue sarcoma with distinctive histopathological features. It should be distinguished from some mimics especially those tumors with a myxoid stroma or chondroid differentiation, such as chordoma and chondrosarcoma.%背景与目的:骨外黏液样软骨肉瘤(extraskeletal myxoid chondrosarcoma,EMC)是一种好发于四肢深部软组织分化不定的恶性肿瘤,以形成多结节样结构、富含黏液为特点。本研究旨在探讨EMC的临床病理特征、诊断及鉴别诊断。方法:对7例EMC进行病理形态学及免疫组化观察,并复习相关文献。结果:7例EMC患者病理巨检显示,灰白色多结节状半透明肿物,边界清楚。镜下卵圆形或短梭形细胞排列成条索,由纤细的纤维组织分隔,呈分叶状,富含黏液样基质但血管稀少。免疫组化肿瘤

  17. Proton radiotherapy in management of pediatric base of skull tumors

    International Nuclear Information System (INIS)

    Purpose: Primary skull base tumors of the developing child are rare and present a formidable challenge to both surgeons and radiation oncologists. Gross total resection with negative margins is rarely achieved, and the risks of functional, structural, and cosmetic deficits limit the radiation dose using conventional radiation techniques. Twenty-nine children and adolescents treated with conformal proton radiotherapy (proton RT) were analyzed to assess treatment efficacy and safety. Methods and Materials: Between July 1992 and April 1999, 29 patients with mesenchymal tumors underwent fractionated proton (13 patients) or fractionated combined proton and photon (16 patients) irradiation. The age at treatment ranged from 1 to 19 years (median 12); 14 patients were male and 15 female. Tumors were grouped as malignant or benign. Twenty patients had malignant histologic findings, including chordoma (n=10), chondrosarcoma (n=3), rhabdomyosarcoma (n=4), and other sarcomas (n=3). Target doses ranged between 50.4 and 78.6 Gy/cobalt Gray equivalent (CGE), delivered at doses of 1.8-2.0 Gy/CGE per fraction. The benign histologic findings included giant cell tumors (n=6), angiofibromas (n=2), and chondroblastoma (n=1). RT doses for this group ranged from 45.0 to 71.8 Gy/CGE. Despite maximal surgical resection, 28 (97%) of 29 patients had gross disease at the time of proton RT. Follow-up after proton RT ranged from 13 to 92 months (mean 40). Results: Of the 20 patients with malignant tumors, 5 (25%) had local failure; 1 patient had failure in the surgical access route and 3 patients developed distant metastases. Seven patients had died of progressive disease at the time of analysis. Local tumor control was maintained in 6 (60%) of 10 patients with chordoma, 3 (100%) of 3 with chondrosarcoma, 4 (100%) of 4 with rhabdomyosarcoma, and 2 (66%) of 3 with other sarcomas. The actuarial 5-year local control and overall survival rate was 72% and 56%, respectively, and the overall survival

  18. Massive chest wall resection and reconstruction for malignant disease

    Directory of Open Access Journals (Sweden)

    Foroulis CN

    2016-04-01

    Full Text Available Christophoros N Foroulis,1 Athanassios D Kleontas,1 George Tagarakis,1 Chryssoula Nana,1 Ioannis Alexiou,1 Vasilis Grosomanidis,1 Paschalis Tossios,1 Elena Papadaki,2 Ioannis Kioumis,2 Sofia Baka,3 Paul Zarogoulidis,2 Kyriakos Anastasiadis11Department of Cardiothoracic Surgery, Aristotle University School of Medicine, AHEPA University Hospital, 2Pulmonary Department-Oncology Unit, “G. Papanikolaou” General Hospital, Aristotle University of Thessaloniki, 3Oncology Department, European Interbalkan Medical Center, Thessaloniki, GreeceObjective: Malignant chest wall tumors are rare neoplasms. Resection with wide-free margins is an important prognostic factor, and massive chest wall resection and reconstruction are often necessary. A recent case series of 20 consecutive patients is reported in order to find any possible correlation between tumor histology, extent of resection, type of reconstruction, and adjuvant treatment with short- and long-term outcomes.Methods: Twenty patients were submitted to chest wall resection and reconstruction for malignant chest wall neoplasms between 2006 and 2014. The mean age (ten males was 59±4 years. The size and histology of the tumor, the technique of reconstruction, and the short- and long-term follow-up records were noted.Results: The median maximum diameter of tumors was 10 cm (5.4–32 cm. Subtotal sternal resection was performed in nine cases, and the resection of multiple ribs was performed in eleven cases. The median area of chest wall defect was 108 cm2 (60–340 cm2. Histology revealed soft tissue, bone, and cartilage sarcomas in 16 cases (80%, most of them chondrosarcomas. The rest of the tumors was metastatic tumors in two cases and localized malignant pleural mesothelioma and non-Hodgkin lymphoma in one case. The chest wall defect was reconstructed by using the “sandwich technique” (propylene mesh/methyl methacrylate/propylene mesh in nine cases of large anterior defects or by using a 2

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

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    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.

  20. The transcription factor Lc-Maf participates in Col27a1 regulation during chondrocyte maturation

    International Nuclear Information System (INIS)

    The transcription factor Lc-Maf, which is a splice variant of c-Maf, is expressed in cartilage undergoing endochondral ossification and participates in the regulation of type II collagen through a cartilage-specific Col2a1 enhancer element. Type XXVII and type XI collagens are also expressed in cartilage during endochondral ossification, and so enhancer/reporter assays were used to determine whether Lc-Maf could regulate cartilage-specific enhancers from the Col27a1 and Col11a2 genes. The Col27a1 enhancer was upregulated over 4-fold by Lc-Maf, while the Col11a2 enhancer was downregulated slightly. To confirm the results of these reporter assays, rat chondrosarcoma (RCS) cells were transiently transfected with an Lc-Maf expression plasmid, and quantitative RT-PCR was performed to measure the expression of endogenous Col27a1 and Col11a2 genes. Endogenous Col27a1 was upregulated 6-fold by Lc-Maf overexpression, while endogenous Col11a2 was unchanged. Finally, in situ hybridization and immunohistochemistry were performed in the radius and ulna of embryonic day 17 mouse forelimbs undergoing endochondral ossification. Results demonstrated that Lc-Maf and Col27a1 mRNAs are coexpressed in proliferating and prehypertrophic regions, as would be predicted if Lc-Maf regulates Col27a1 expression. Type XXVII collagen protein was also most abundant in prehypertrophic and proliferating chondrocytes. Others have shown that mice that are null for Lc-Maf and c-Maf have expanded hypertrophic regions with reduced ossification and delayed vascularization. Separate studies have indicated that Col27a1 may serve as a scaffold for ossification and vascularization. The work presented here suggests that Lc-Maf may affect the process of endochondral ossification by participating in the regulation of Col27a1 expression.

  1. 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.

  2. Morphometric Analysis of Bone Resection in Anterior Petrosectomies.

    Science.gov (United States)

    Ahmed, Osama; Walther, Jonathan; Theriot, Krystle; Manuel, Morganne; Guthikonda, Bharat

    2016-06-01

    Introduction The anterior petrosectomy is a well-defined skull base approach to lesions such as petroclival meningiomas, posterior circulation aneurysms, petrous apex lesions (chondrosarcomas, cholesteatomas), ventrolateral brainstem lesions, clival chordomas, trigeminal neurinomas, and access to cranial nerves III, IV, V, and VII. Methods and Materials Fourteen anterior petrosectomies on eight cadaveric heads were performed in a skull base dissection laboratory. Predissection and postdissection thin-cut computed tomography scans were obtained to compare the bone resection. A computer program was used (InVivo5, Anatomage, San Jose, California, United States) to measure the bone resection and the improved viewing angle. Results The average bone removed in each plane was as follows: anterior to posterior plane was 10.57 mm ± 2.00 mm, superior to inferior was 9.39 mm ± 1.67 mm, and lateral to medial was 17.46 mm ± 4.64 mm. The average increased angle of view was 13.01 ± 2.35 degrees (Table 1). The average volume was 1786.94 ± 827.40 mm(3). Conclusions Anterior petrosectomy is a useful approach to access the ventrolateral brainstem region. We present a cadaveric study quantitating the volume of bone resection and improvement in the viewing angle. These data provide useful preoperative information on the utility of this skull base approach and the gain in the viewing angle after bony removal. PMID:27175319

  3. Docosahexenoic acid treatment ameliorates cartilage degeneration via a p38 MAPK-dependent mechanism.

    Science.gov (United States)

    Wang, Zhenzhong; Guo, Ai; Ma, Lifeng; Yu, Haomiao; Zhang, Liang; Meng, Hai; Cui, Yinpeng; Yu, Fei; Yang, Bo

    2016-06-01

    Osteoarthritis (OA) is a common chronic inflammatory disease, characterized by cartilage degradation. The aberrant expression of matrix metalloproteinase-13 (MMP-13) plays a vital role in the pathogenesis of OA. The anti‑inflammatory property of docosahexenoic acid (DHA) was previously revealed and showed that DHA retards the progress of many types of inflammatory disease. To evaluate the prophylactic function of DHA in OA, the effect of DHA on cartilage degeneration was assessed in interleukin‑1β (IL‑1β) stimulated human chondrosarcoma SW1353 cells or a rat model of adjuvant‑induced arthritis (AIA). The safe concentration range (0‑50 µg/ml in vitro) of DHA was determined by flow cytometry and MTT assay. The inhibitory effects of DHA on MMP‑13 mRNA and protein expression were confirmed by RT‑qPCR, ELISA and western blotting. Furthermore, findings of an in vivo study showed that DHA can increase the thickness of articular cartilage and decrease MMP‑13 expression in cartilage matrix in a rat AIA model. We also revealed the mechanism by which DHA ameliorates cartilage degeneration from OA. The DHA-mediated inhibition of MMP‑13 expression was partially attributed to the inactivation of the p38 mitogen‑activated protein kinases pathway by suppressing p‑p38 in IL-1β-stimulated SW1353 cells and a rat AIA model. Our findings suggested that DHA is a promising therapeutic agent that may be used for the prevention and treatment of OA. PMID:27082436

  4. Chest Wall tumor: combined management

    International Nuclear Information System (INIS)

    Cancer is relatively rare disease among children and adolescents. The incidence of solid tumors other than CNS is less than 2/100,000. Tumors of the chest wall can arise either from the somatic tissue or ribs. These are rare, so either institutional reviews or multi institutional studies should determine optimal therapeutic management. Of the bony chest wall, Ewing's sarcoma or the family of tumor (peripheral neuro epithelioma, Askin tumor), are the most common. These lesions are lytic and have associated large extra pleural component. This large extra pleural component often necessitates major chest wall resection (3 or more ribs), and when lower ribs are involved, this entails resection of portion of diaphragm. Despite this resection, survival in the early 1970 was 10-20%. Since 1970 multi agent chemotherapy has increased survival rates. of importance, however, is these regimens have caused significant reduction of these extra pleural components so that major chest wall resections have become a rarity. With improved survival and decreased morbidity preoperative chemotherapy followed by surgery is now the accepted modality of treatment. Another major advantage of this regimen is that potential radiation therapy may be obviated. The most common chest wall lesion is rhabdomyosarcoma. In the IRS study of 1620 RMS patients, in 141 (9%) the primary lesion was in the chest wall. these are primarily alveolar histology. when lesions were superficial, wide local excision with supplemental radiation therapy was associated with low morbidity and good overall survival. however, a majority have significant intra- thoracic components. in these circumstances the resectability rate is less than 30% and the survival poor. Other lesions include non rhabdomyosarcomas, eosinophilic granuloma, chondrosarcoma, and osteomyelitis. The management of these lesions varies according to extent, histology, and patient characteristics

  5. 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.)

  6. The use of gold foil wrapping for radiation protection of the spinal cord for recurrent tumor therapy

    International Nuclear Information System (INIS)

    Purpose: The development of a technique to provide sufficient radiation protection to previously irradiated spinal cord in such a manner that interstitial brachytherapy can be conducted after resection of a recurrent tumor and decompression of the cord. Methods and Materials: A technique was developed that uses multiple layers of gold foil that are applied around the thecal sac and nerve root sleeves to produce an enveloping radiation shield after resection of recurrent tumor. Once the layers of gold foil are in place, interstitial I125 seeds are permanently placed in the bed of the tumor resection to prevent any recurrence from microcellular disease. The technique is described and its application in the case of a 28-year-old with a third recurrence of chondrosarcoma after external fractionated radiation therapy at the second to the fourth thoracic segments is reviewed. Results: This technique has been used in this first patient. An additional tumor dose of 120.0 Gy was delivered to the tumor bed while the spinal cord was calculated to receive only 1% of the dose over the life span of the implant. To date, this dose of radiation has prevented tumor recurrence for more than 18 months of follow-up. Conclusion: This technique of multiple layers of gold foil shielding over the spinal cord and nerve roots has the potential to be a useful tool for the shielding of a previously irradiated spinal cord in the setting of resection of recurrent tumor. It may also have a wider application to a number of other radiosensitive tumors where interstitial brachytherapy may be useful to provide additional treatment after external fractionated radiation therapy

  7. MR Imaging in symptomatic osteochondromas

    Energy Technology Data Exchange (ETDEWEB)

    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.

  8. The portrayal of bone tumours in the press.

    Science.gov (United States)

    Al-Nammari, Shafic Said; Danesh, Arash; Mussa, Mohamed; Al-Hadithy, Nawfal

    2013-04-01

    The media are pivotal in educating and informing the general public. The stories they cover and how they cover them has a powerful influence on public perceptions. There have been no previous reports of the portrayal of bone tumours in the press. LexisNexis™ Professional search engine used to retrieve articles from all United Kingdom National Newspapers over one year containing terms "bone tumour/bone tumour" and 46 other named bone and joint tumours from May 2009 to May 2010. A total of 253 relevant articles were found. Seven per cent solely bone tumour related, 41% main theme and 52% mentioned in passing. 52% mentioned tumour type. These were 51% multiple myeloma, 15% Ewing's sarcoma, 9% sarcoma unspecified, 6% clear cell sarcoma, 4.5% epithelial sarcoma, 4% synovial sarcoma, 3% osteosarcoma, 3% bony metastasis and 1.5% chondrosarcoma. Benign bone tumours not mentioned. Article focus: chemotherapy 17%, surgeon/doctor 7% and new surgical technique 2%. The overall attitudinal tone of the articles were 52% negative, 32% neutral and 16% positive. Only 13% quoted an oncologist, and 1% an orthopaedic surgeon. Quality of medical information provided was limited with 90% providing no meaningful medical information and this medical information being correct only 68% of the time. Articles with quotes from a doctor were significantly more likely to contain meaningful medical information than those without-33 versus 4%, respectively (p < 0.001 Chi-squared test)-and there was a trend for them to be more factually correct overall-68 versus 50% (p = 0.192 Fisher's exact Test). PMID:23463358

  9. Receptor-Activator of Nuclear KappaB Ligand Expression as a New Therapeutic Target in Primary Bone Tumors

    Science.gov (United States)

    Yamagishi, Tetsuro; Kawashima, Hiroyuki; Ogose, Akira; Ariizumi, Takashi; Sasaki, Taro; Hatano, Hiroshi; Hotta, Tetsuo; Endo, Naoto

    2016-01-01

    The receptor-activator of nuclear kappaB ligand (RANKL) signaling pathway plays an important role in the regulation of bone growth and mediates the formation and activation of osteoclasts. Osteoclasts are involved in significant bone resorption and destruction. Denosumab is a fully human monoclonal antibody against RANKL that specifically inhibits osteoclast differentiation and bone resorption. It has been approved for use for multiple myeloma and bone metastases, as well as for giant cell tumor of bone. However, there is no previous report quantitatively, comparing RANKL expression in histologically varied bone tumors. Therefore, we analyzed the mRNA level of various bone tumors and investigated the possibility of these tumors as a new therapeutic target for denosumab. We examined RANKL mRNA expression in 135 clinical specimens of primary and metastatic bone tumors using real-time PCR. The relative quantification of mRNA expression levels was performed via normalization with RPMI8226, a human multiple myeloma cell line that is recognized to express RANKL. Of 135 cases, 64 were also evaluated for RANKL expression by using immunohistochemistry. Among all of the tumors investigated, RANKL expression and the RANKL/osteoprotegerin ratio were highest in giant cell tumor of bone. High RANKL mRNA expression was observed in cases of aneurysmal bone cyst, fibrous dysplasia, osteosarcoma, chondrosarcoma, and enchondroma, as compared to cases of multiple myeloma and bone lesions from metastatic carcinoma. RANKL-positive stromal cells were detected in six cases: five cases of GCTB and one case of fibrous dysplasia. The current study findings indicate that some primary bone tumors present new therapeutic targets for denosumab, particularly those tumors expressing RANKL and those involving bone resorption by osteoclasts. PMID:27163152

  10. HEMIPELVECTOMY: ERASTO GAERTNER HOSPITAL'S EXPERIENCES WITH 32 CASES IN 10 YEARS

    Science.gov (United States)

    Freitas, Rosyane Rena de; Crivellaro, André Luiz Soares; Mello, Glauco José Pauka; Neto, Múrio Armani; Filho, Geraldo de Freitas; Silva, Letícia Viani da

    2015-01-01

    Objective: To show the experience of the Erasto Gaertner Hospital with hemipelvectomy surgery over a 10-year period. Methods: This was a retrospective study on 32 patients who underwent hemipelvectomy at Erasto Gaertner Hospital between 1998 and 2008, assessing clinical and surgical characteristics. Results: Among the 32 patients, 15 were female and 17 were male. The mean age was 37.94 years. Eight cases showed involvement of the neurovascular bundle: three were located in the iliac and extended to the thigh, two were in the acetabulum and extended to the thigh and three were in the acetabulum and pubis. Twenty-three cases presented a neurovascular bundle free from neoplasia: 11 were restricted to the iliac, six were in the acetabular region, two were in the pubic ramus and four extended to the whole hemipelvis bone. One case involved the iliac-femoral vessels: one in the pubic ramus. Seven cases of chondrosarcoma and four cases of Ewing's sarcoma represented the majority. Eight cases underwent external hemipelvectomy and 24 underwent internal hemipelvectomy (11 were type I; four were type II; two were type II + III; three were type III and four were type IV). Of these 24 cases, 13 did not have any reconstruction, 10 had a fibular graft and one had an iliacfemoral vein and artery prosthesis. Twenty-six surgeries were curative and six were palliative. There were 14 deaths. Survival of two and five years was seen in 11 and 10 cases, respectively. For six cases, less than two years had passed since the operation. Three cases were lost during follow-up. Conclusion: This study shows the experiences of an oncology reference service specializing in highly complex surgical treatment. PMID:27022573

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

    Directory of Open Access Journals (Sweden)

    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

  12. 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. PMID:26420474

  13. Assessment of margins in resection specimens for head and neck malignancies

    International Nuclear Information System (INIS)

    Objective: To determine the relative frequency of clear, close and involved margins in resection specimens for head and neck malignancies. Study Design: An observational study. Place and Duration of Study: The Department of Oral and Maxillofacial Surgery, Armed Forces Institute of Dentistry, Rawalpindi and the Department of Histopathology, Armed Forces Institute of Pathology, Rawalpindi, from January 2008 to December 2010. Methodology: Tumour registers and computer data bases in the department of Histopathology of Armed Forces Institute of Pathology, Rawalpindi, were analyzed for the cases of malignancies involving head and neck region that were sent for histopathological analysis after resection in the last three years. Histopathology reports were obtained. The data regarding age, gender, site, type of malignancy and margin status (clear, close or involved) was recorded on specially designed proformas for the study and later on analyzed by using SPSS version 17.0. Results were expressed. Results: A total of 319 cases were registered in the study duration. The age of the patients ranged from 22 - 90 years (mean 59.5 + 14.1 years). Male to female ratio was 1.53:1. One hundred and thirty six (42.6%) were squamous cell carcinoma (SCC), 163 were basal cell carcinomas (BCC, 51.0%); the rest included 18 salivary gland malignancies (5.7%) and one carcino-sarcoma (0.31%) and chondrosarcoma each. All margins were found clear in 137 patients (42.9%); involved in 168 cases (52.7%) and close in 14 cases (4.4%). Conclusion: Margin clearance could not be achieved in more than 50% cases, this can lead to poor prognosis. Hence, methods should be adopted to improve the margin clearance in various head and neck malignancies. (author)

  14. Wild-type ALK and activating ALK-R1275Q and ALK-F1174L mutations upregulate Myc and initiate tumor formation in murine neural crest progenitor cells

    Science.gov (United States)

    Montavon, Gisèle; Jauquier, Nicolas; Coulon, Aurélie; Peuchmaur, Michel; Flahaut, Marjorie; Bourloud, Katia Balmas; Yan, Pu; Delattre, Olivier; Sommer, Lukas; Joseph, Jean-Marc; Janoueix-Lerosey, Isabelle; Gross, Nicole; Mühlethaler-Mottet, Annick

    2014-01-01

    The anaplastic lymphoma kinase (ALK) gene is overexpressed, mutated or amplified in most neuroblastoma (NB), a pediatric neural crest-derived embryonal tumor. The two most frequent mutations, ALK-F1174L and ALK-R1275Q, contribute to NB tumorigenesis in mouse models, and cooperate with MYCN in the oncogenic process. However, the precise role of activating ALK mutations or ALK-wt overexpression in NB tumor initiation needs further clarification. Human ALK-wt, ALK-F1174L, or ALK-R1275Q were stably expressed in murine neural crest progenitor cells (NCPC), MONC-1 or JoMa1, immortalized with v-Myc or Tamoxifen-inducible Myc-ERT, respectively. While orthotopic implantations of MONC-1 parental cells in nude mice generated various tumor types, such as NB, osteo/chondrosarcoma, and undifferentiated tumors, due to v-Myc oncogenic activity, MONC-1-ALK-F1174L cells only produced undifferentiated tumors. Furthermore, our data represent the first demonstration of ALK-wt transforming capacity, as ALK-wt expression in JoMa1 cells, likewise ALK-F1174L, or ALK-R1275Q, in absence of exogenous Myc-ERT activity, was sufficient to induce the formation of aggressive and undifferentiated neural crest cell-derived tumors, but not to drive NB development. Interestingly, JoMa1-ALK tumors and their derived cell lines upregulated Myc endogenous expression, resulting from ALK activation, and both ALK and Myc activities were necessary to confer tumorigenic properties on tumor-derived JoMa1 cells in vitro. PMID:24947326

  15. 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

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

    Science.gov (United States)

    De Paolis, Massimiliano; Romagnoli, Carlo; Alì, Nikolin; Giannini, Sandro; Donati, Davide Maria

    2013-01-01

    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. PMID:24250275

  17. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  19. ZNF93 increases resistance to ET-743 (Trabectedin; Yondelis and PM00104 (Zalypsis in human cancer cell lines.

    Directory of Open Access Journals (Sweden)

    Zhenfeng Duan

    Full Text Available BACKGROUND: ET-743 (trabectedin, Yondelis and PM00104 (Zalypsis are marine derived compounds that have antitumor activity. ET-743 and PM00104 exposure over sustained periods of treatment will result in the development of drug resistance, but the mechanisms which lead to resistance are not yet understood. METHODOLOGY/PRINCIPAL FINDINGS: Human chondrosarcoma cell lines resistant to ET-743 (CS-1/ER or PM00104 (CS-1/PR were established in this study. The CS-1/ER and CS-1/PR exhibited cross resistance to cisplatin and methotrexate but not to doxorubicin. Human Affymetrix Gene Chip arrays were used to examine relative gene expression in these cell lines. We found that a large number of genes have altered expression levels in CS-1/ER and CS-1/PR when compared to the parental cell line. 595 CS-1/ER and 498 CS-1/PR genes were identified as overexpressing; 856 CS-1/ER and 874 CS-1/PR transcripts were identified as underexpressing. Three zinc finger protein (ZNF genes were on the top 10 overexpressed genes list. These genes have not been previously associated with drug resistance in tumor cells. Differential expressions of ZNF93 and ZNF43 genes were confirmed in both CS-1/ER and CS-1/PR resistant cell lines by real-time RT-PCR. ZNF93 was overexpressed in two ET-743 resistant Ewing sarcoma cell lines as well as in a cisplatin resistant ovarian cancer cell line, but was not overexpressed in paclitaxel resistant cell lines. ZNF93 knockdown by siRNA in CS-1/ER and CS-1/PR caused increased sensitivity for ET-743, PM00104, and cisplatin. Furthermore, ZNF93 transfected CS-1 cells are relatively resistant to ET-743, PM00104 and cisplatin. CONCLUSIONS/SIGNIFICANCE: This study suggests that zinc finger proteins, and ZNF93 in particular, are involved in resistance to ET-743 and PM00104.

  20. A small prospective study of chordomas treated with radiotherapy and razoxane

    Energy Technology Data Exchange (ETDEWEB)

    Rhomberg, W.; Boehler, F.K. [Dept. of Radiooncology, General Hospital, Feldkirch (Austria); Novak, H. [Dept. of Diagnostic Radiology, General Hospital, Feldkirch (Austria); Dertinger, S.; Breitfellner, G. [Dept. of Pathology, General Hospital, Feldkirch (Austria)

    2003-04-01

    Purpose: To evaluate the local effect of conventional photon irradiation in chordomas if the radiosensitizing agent razoxane is added. The rationale for this procedure were improved results previously seen in soft tissue and chondrosarcomas with this combination. Patients and Methods: Between 1988 and 1996, five patients with histologically confirmed chordomas of the skull base or the spine (three females, two males) were irradiated with 6- and 25-MeV photons under razoxane medication, one patient was treated with a telecobalt unit. Single doses of 180-200 cGy were given five times a week. The median total tumor dose was 63 Gy (range 54-67 Gy). Concomitantly, the radiosensitizer razoxane was administered at a dose of 125 mg twice daily p.o., median total dose 7.6 g. The drug was started 3-5 days before the first irradiation, and continued until the end of radiotherapy. Results: After a potential median follow-up time of 10 years, three of the five patients are alive and show neither symptoms nor signs of recurrence in CT or MR images. One patient with persistent sacral chordoma died after 8 years from cardiac insufficiency, and another patient died after 6.5 years from a bleeding complication following surgery for recurrence. The patients remained locally controlled for 5, 5.5+, 6.4, 11+, and 13+ years, respectively. Objective tumor regressions were noted in three of four patients with measurable disease. Acute side effects included mucosal reactions, two of five patients developed a leukopenia WHO grade 3 due to razoxane. Serious long-term complications were not observed. Conclusions: Although the patient series is small, there is an interesting trend in local control and survival. The cases are unselected, and the follow-up time is of considerable duration. The treatment can easily be performed at any institution and is tolerated fairly well. (orig.)

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

    Directory of Open Access Journals (Sweden)

    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.

  2. The future and progress of proton beam radiotherapy

    International Nuclear Information System (INIS)

    The advantage of proton therapy is reduction of treatment volumes relative to those feasible with conventional photon therapy. The consequence is that the radiation dose to the target can be raised, with a resultant increase in tumor control probability. Proton beams, however, yield no biological gains because their biological properties are similar to conventional low LET radiations. As more sophisticated technologies are needed, there have been many advances which are applicable to photon therapy; 3-D treatment planning, DVH analysis, and systems for positioning, etc. As of January 1994, a total of about 13,000 cases were reported as having had treatments with proton beams in 16 centers world wide. The tumor sites for those include uveal melanoma (30-40%), intra-cranial small targets (40%), and others. Uveal melanomas had been most extensively treated with 70 Gy/5 fx or 60 Gy/4 fx which resulted in local control and survival rates of >96% and 80%, respectively. For chordoma and chondrosarcoma of the skull base and cervical spine, the 5 year local control rates were 65% and 91%, respectively. Promising results are also being obtained for head and neck and pelvic tumors. Deeper-seated tumors have been treated only at Tsukuba University with successful results in some anatomic sites. Among these, inoperable primary hepatocellular carcinomas were effectively treated with a total dose of 75-85 Gy (3.0-4.5 Gy/fx). The 3 year survival rates for all patients, Child A+B patient, and Child A patients were 38%, 47%, and 60%, respectively, which compare favorably to other modalities. These successful results of world wide proton therapy have led us to the conclusion that a hospital-based proton facility will provide opportunities for additional patients to be treated with protons. Thus, new plans are proposed from more than 10 institutions to build a new treatment center or upgrade the energy of currently available proton beams. (author)

  3. Analysis of enhancement pattern of sellar and parasellar tumors using two-phase helical CT

    Energy Technology Data Exchange (ETDEWEB)

    Woo, Ji Young; Na, Dong Gyu; Roh, Hong Gee; Byun, Hong Sik; Ryoo, Jae Wook [Medical School of Sungkyunkwan Univ., Seoul (Korea, Republic of)

    2002-01-01

    To assess the enhancement patterns of sellar and parasellar tumors at two-phase helical CT. Thirty-two patients with pathologically proven sellar and parasellar tumors (meningioma (n=17), pituitary mocroadenoma (n=6), neurogenic tumor (n=5), cavernous angioma (n=1), chondrosarcoma (n=1), osteosarcoma (n=1), sphenoid carcinoma (n=1)) were included in this study. Two-phase helical CT was performed after the injection of 90 mL of contrast materials at a rate of 3 mL/sec. Transverse helical CT scans were obtained during the early and late phases, with scannin dealys of 30 and 120 seconds, respectively. Delayed coronal images were obtained after delayed axial images. Attenuation change and the enhancement patterns of the tumors were visually assessed; the former was also assessed quantitatively as the ratio of the CT number at late-phase axial and coronal scanning to that at early-phase scanning. Visual assessment of two-phase helical CT images revealed decreased attenuation in all 17 meningiomas, no change in all six pituitary macroadenomas and increased attenuation in 5 all five neurogenic tumors on late-phase axial scans as compared with early phase scans. Coronal images showed decreased attenuation in all 17 meningiomas, increased attenuation in all five neurogenic tumors and no change in four pituitary macroadenomas (66.7%). The ratio of CT numbers was significantly different between meningiomas, neurogenic tumors and pituitary macroadenomas (p< 0.05). According to their histopathology, sellar and parasellar tumors showed characteristic enhancement patterns at two-phase helical CT. An analysis of the observed enhancement patterns can be useful in the differential diagnosis of juxtasellar tumors.

  4. 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

  5. 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.)

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

    International Nuclear Information System (INIS)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    International Nuclear Information System (INIS)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  11. Clinical investigation on usefulness of MRI in the diagnosis of mediastinal masses

    Energy Technology Data Exchange (ETDEWEB)

    Oyama, Takao (Kagoshima Univ. (Japan). Faculty of Medicine)

    1994-02-01

    This study evaluated the utility of magnetic resonance imaging (MRI) in diagnosing mediastinal masses. The subjects were 96 patients with pathologically (n=82) and clinically (n=14) proven mediastinal masses, consisting of thymomas (30), teratomas (4), other thymic tumors (5), intrathoracic goiters (4), neurogenic tumors (7), congenital cysts (12), mesenchymal tumors (10), vascular masses (5), phrenic hernia (3), malignant lymphomas (6), and sarcoidosis (10). MRI findings, including marginal and capsular appearances, internal structures and signal intensities, were analyzed. Internal structure characteristic to thymomas were seen in 24 teratoma patients (80%). In 4 teratomas, MRI was capable of distinguishing benign (2) from malignant (2) ones. In the other 5 thymic tumors, MRI delineated chemical characteristics of fluids for thymic cysts (2) and internal structures for thymic cancer (2) and lipoma (one). It also delineated the continuity of intrathoracic goiter from the cervical to intrathoracic regions. For neurogenic tumors, MRI was capable of distinguishing neurofibromas from schwannomas and also benign from malignant schwannomas. A well-defined smooth margin and very high signal intensity, which were characteristic to congenital cysts, were seen on T2-weighted images. Signal intensities on T1-weighted images reflected fluid features in cysts. For mesenchymal tumors, consisting of hemangioma, hemangiosarcoma, malignant melanoma, aneurysmal bone cyst and osteosarcoma, lymphangioma and chondrosarcoma, all but osteosarcoma showed their respective characteristic MRI findings. Vascular masses were delineated as flow void structures. MRI delineated herniated contents. Malignant lymphoma was shown as irregular and inhomogeneous swollen lymph nodes, but sarcoidosis was shown as regular and homogeneous ones on MRI. In conclusion, MRI is a useful, informative modality for diagnosing mediastinal masses. (N.K.) 84 refs.

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

    Science.gov (United States)

    Schwager, Joseph; Richard, Nathalie; Fowler, Ann; Seifert, Nicole; Raederstorff, Daniel

    2016-01-01

    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. PMID:27070563

  13. 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.

  14. Importance of precise positioning for proton beam therapy in the base of skull and cervical spine.

    Science.gov (United States)

    Tatsuzaki, H; Urie, M M

    1991-08-01

    Using proton beam therapy, high doses have been delivered to chordomas and chondrosarcomas of the base of skull and cervical spine. Dose inhomogeneity to the tumors has been accepted in order to maintain normal tissue tolerances, and detailed attention to patient immobilization and to precise positioning has minimized the margins necessary to ensure these dose constraints. This study examined the contribution of precise positioning to the better dose localization achieved in these treatments. Three patients whose tumors represented different anatomic geometries were studied. Treatment plans were developed which treated as much of the tumor as possible to 74 Cobalt-Gray-Equivalent (CGE) while maintaining the central brain stem and central spinal cord at less than or equal to 48 CGE, the surface of the brain stem, surface of the spinal cord, and optic structures at less than or equal to 60 CGE, and the temporal lobes at less than or equal to 5% likelihood of complication using a biophysical model of normal tissue complication probability. Two positioning accuracies were assumed: 3 mm and 10 mm. Both proton beam plans and 10 MV X ray beam plans were developed with these assumptions and dose constraints. In all cases with the same positioning uncertainties, the proton beam plans delivered more dose to a larger percentage of the tumor volume and the estimated tumor control probability was higher than with the X ray plans. However, without precise positioning both the proton plans and the X ray plans deteriorated, with a 12% to 25% decrease in estimated tumor control probability. In all but one case, the difference between protons with good positioning and poor positioning was greater than the difference between protons and X rays, both with good positioning. Hence in treating these tumors, which are in close proximity to critical normal tissues, attention to immobilization and precise positioning is essential. With good positioning, proton beam therapy permits higher

  15. 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

  16. 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

  17. Radiation-induced pseudotumor following therapy for soft tissue sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Moore, Lacey F.; Kransdorf, Mark J. [Mayo Clinic, Department of Radiology, Jacksonville, FL (United States); Buskirk, Steven J. [Mayo Clinic, Department of Radiation Oncology, Jacksonville, FL (United States); O' Connor, Mary I. [Mayo Clinic, Department of Orthopedic Surgery, Jacksonville, FL (United States); Menke, David M. [Mayo Clinic, Department of Pathology, Jacksonville, FL (United States)

    2009-06-15

    The purpose of this study was to describe the prevalence and imaging appearance of radiation induced pseudotumors in patients following radiation therapy for extremity soft tissue sarcomas. We retrospectively reviewed the serial magnetic resonance (MR) images of 24 patients following radiation therapy for extremity soft tissue sarcomas. A total of 208 exams were reviewed (mean, 8.7 exams per patient) and included all available studies following the start of radiation therapy. Exams were analyzed for the identification of focal signal abnormalities within the surgical bed suggesting local tumor recurrence. Histopathologic correlation was available in nine patients suspected of having local tumor recurrence. Additional information recorded included patient demographics, tumor type and location, radiation type, and dose. The study group consisted of 12 men and 12 women, having an average age of 63 years (range, 39-88 years). Primary tumors were malignant fibrous histiocytoma (n = 13), leiomyosarcoma (n = 6), liposarcoma (n = 3), synovial sarcoma (n = 1), and extraskeletal chondrosarcoma (n = 1). All lesions were high-grade sarcomas, except for two myxoid liposarcomas. Average patient radiation dose was 5,658 cGy (range, 4,500-8,040 cGy). Average follow-up time was 63 months (range, 3-204 months). Focal signal abnormalities suggesting local recurrence were seen in nine (38%) patients. Three of the nine patients with these signal abnormalities were surgically proven to have radiation-induced pseudotumor. The pseudotumors developed between 11 and 61 months following the initiation of radiation therapy (mean, 38 months), with an average radiation dose of 5,527 cGy (range, 5,040-6,500 cGy). MR imaging demonstrated a relatively ill-defined ovoid focus of abnormal signal and intense heterogeneous enhancement with little or no associated mass effect. MR imaging of radiation-induced pseudotumor typically demonstrates a relatively ill-defined ovoid mass-like focus of intense

  18. Accumulation of Tc-99m-MIBI and Tc-99m-tetrofosmin in tumor cells. Uptake and washout studies

    International Nuclear Information System (INIS)

    Aim: To investigate in-vitro the uptake and washout of Tc-99m-MIBI and Tc-99m-tetrofosmin in human breast adenocarcinoma and soft tissue sarcoma cell lines. Methods: The uptake of Tc-99m-MIBI and Tc-99m-tetrofosmin (at 370C, 10, 30 and 60 minutes after incubation with 7.4x10E5 Bq each tracer) was investigated in breast adenocarcinoma MCF-7 and SK-BR-3 cells, synovial sarcoma SW 982 cells and chondrosarcoma SW 1353 cells (concentration of 1x10E6 cells/ml incubation medium). Tracer uptake in cells incubated with ouabain (Na/K-ATPase pump inhibitor; 100 μM and 1mM; 15 and 30 minutes), nigericin (increases mitochondrial potential and disrupts cell membrane potential; 5 and 50 μg/ml; 15 minutes) and carbonyl cyanide m-chlorophenylhydrazone (CCCP) (depolarizes mitochondrial membrane; 10 and 100 μM; 30 minutes) was compared to that in cells without incubation with chemical agents (control cells). The washout (at 370C, 10-60 minutes, 30 and 60 minutes after tracer incubation) of Tc-99m-MIBI and Tc-99m-tetrofosmin was studied in MCF-7 cells, SK-BR-3 cells , SW 1353 cells and fibrosarcoma SW 684 cells. Results: Cellular tracer uptake decreased with ouabain (decrease of Tc-99m-tetrofosmin > Tc-99m-MIBI in SK-BR-3 cells and SW 982 cells) and increased with nigericin (increase of Tc-99m-MIBI > Tc-99m-tetrofosmin in all cells) as compared to the uptake in control cells. With CCCP, decrease of Tc-99m-MIBI uptake in cells preincubated with nigericin was higher than that in cells under basal conditions, whereas a similar decrease of Tc-99m-tetrofosmin uptake in these two group of cells was found. Washout of Tc-99m-MIBI from all cells was lower than that of Tc-99m-tetrofosmin. No significant difference in cell-associated activity of both tracers was found between washout after 30 minutes and that after 60 minutes of incubation, in all cells. Washout of Tc-99m-MIBI from SK-BR-3 cells Tc-99m-tetrofosmin) and Na/K-ATPase pump (Tc-99m-tetrofosmin > Tc-99m-MIBI). Tc-99m-MIBI and Tc-99

  19. Radiation-induced pseudotumor following therapy for soft tissue sarcoma

    International Nuclear Information System (INIS)

    The purpose of this study was to describe the prevalence and imaging appearance of radiation induced pseudotumors in patients following radiation therapy for extremity soft tissue sarcomas. We retrospectively reviewed the serial magnetic resonance (MR) images of 24 patients following radiation therapy for extremity soft tissue sarcomas. A total of 208 exams were reviewed (mean, 8.7 exams per patient) and included all available studies following the start of radiation therapy. Exams were analyzed for the identification of focal signal abnormalities within the surgical bed suggesting local tumor recurrence. Histopathologic correlation was available in nine patients suspected of having local tumor recurrence. Additional information recorded included patient demographics, tumor type and location, radiation type, and dose. The study group consisted of 12 men and 12 women, having an average age of 63 years (range, 39-88 years). Primary tumors were malignant fibrous histiocytoma (n = 13), leiomyosarcoma (n = 6), liposarcoma (n = 3), synovial sarcoma (n = 1), and extraskeletal chondrosarcoma (n = 1). All lesions were high-grade sarcomas, except for two myxoid liposarcomas. Average patient radiation dose was 5,658 cGy (range, 4,500-8,040 cGy). Average follow-up time was 63 months (range, 3-204 months). Focal signal abnormalities suggesting local recurrence were seen in nine (38%) patients. Three of the nine patients with these signal abnormalities were surgically proven to have radiation-induced pseudotumor. The pseudotumors developed between 11 and 61 months following the initiation of radiation therapy (mean, 38 months), with an average radiation dose of 5,527 cGy (range, 5,040-6,500 cGy). MR imaging demonstrated a relatively ill-defined ovoid focus of abnormal signal and intense heterogeneous enhancement with little or no associated mass effect. MR imaging of radiation-induced pseudotumor typically demonstrates a relatively ill-defined ovoid mass-like focus of intense

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

    International Nuclear Information System (INIS)

    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 (TD5) 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 comparable

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

    International Nuclear Information System (INIS)

    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

  2. Massive chest wall resection and reconstruction for malignant disease

    Science.gov (United States)

    Foroulis, Christophoros N; Kleontas, Athanassios D; Tagarakis, George; Nana, Chryssoula; Alexiou, Ioannis; Grosomanidis, Vasilis; Tossios, Paschalis; Papadaki, Elena; Kioumis, Ioannis; Baka, Sofia; Zarogoulidis, Paul; Anastasiadis, Kyriakos

    2016-01-01

    Objective Malignant chest wall tumors are rare neoplasms. Resection with wide-free margins is an important prognostic factor, and massive chest wall resection and reconstruction are often necessary. A recent case series of 20 consecutive patients is reported in order to find any possible correlation between tumor histology, extent of resection, type of reconstruction, and adjuvant treatment with short- and long-term outcomes. Methods Twenty patients were submitted to chest wall resection and reconstruction for malignant chest wall neoplasms between 2006 and 2014. The mean age (ten males) was 59±4 years. The size and histology of the tumor, the technique of reconstruction, and the short- and long-term follow-up records were noted. Results The median maximum diameter of tumors was 10 cm (5.4–32 cm). Subtotal sternal resection was performed in nine cases, and the resection of multiple ribs was performed in eleven cases. The median area of chest wall defect was 108 cm2 (60–340 cm2). Histology revealed soft tissue, bone, and cartilage sarcomas in 16 cases (80%), most of them chondrosarcomas. The rest of the tumors was metastatic tumors in two cases and localized malignant pleural mesothelioma and non-Hodgkin lymphoma in one case. The chest wall defect was reconstructed by using the “sandwich technique” (propylene mesh/methyl methacrylate/propylene mesh) in nine cases of large anterior defects or by using a 2 mm polytetrafluoroethylene (e-PTFE) mesh in nine cases of lateral or posterior defects. Support from a plastic surgeon was necessary to cover the full-thickness chest wall defects in seven cases. Adjuvant oncologic treatment was administered in 13 patients. Local recurrences were observed in five cases where surgical reintervention was finally necessary in two cases. Recurrences were associated with larger tumors, histology of malignant fibrous histiocytoma, and initial incomplete resection or misdiagnosis made by nonthoracic surgeons. Three patients died

  3. 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

    International Nuclear Information System (INIS)

    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 differences in

  4. HSPG-deficient zebrafish uncovers dental aspect of multiple osteochondromas.

    Directory of Open Access Journals (Sweden)

    Malgorzata I Wiweger

    Full Text Available Multiple Osteochondromas (MO; previously known as multiple hereditary exostosis is an autosomal dominant genetic condition that is characterized by the formation of cartilaginous bone tumours (osteochondromas at multiple sites in the skeleton, secondary bursa formation and impingement of nerves, tendons and vessels, bone curving, and short stature. MO is also known to be associated with arthritis, general pain, scarring and occasional malignant transformation of osteochondroma into secondary peripheral chondrosarcoma. MO patients present additional complains but the relevance of those in relation to the syndromal background needs validation. Mutations in two enzymes that are required during heparan sulphate synthesis (EXT1 or EXT2 are known to cause MO. Previously, we have used zebrafish which harbour mutations in ext2 as a model for MO and shown that ext2⁻/⁻ fish have skeletal defects that resemble those seen in osteochondromas. Here we analyse dental defects present in ext2⁻/⁻ fish. Histological analysis reveals that ext2⁻/⁻ fish have very severe defects associated with the formation and the morphology of teeth. At 5 days post fertilization 100% of ext2⁻/⁻ fish have a single tooth at the end of the 5(th pharyngeal arch, whereas wild-type fish develop three teeth, located in the middle of the pharyngeal arch. ext2⁻/⁻ teeth have abnormal morphology (they were shorter and thicker than in the WT and patchy ossification at the tooth base. Deformities such as split crowns and enamel lesions were found in 20% of ext2⁺/⁻ adults. The tooth morphology in ext2⁻/⁻ was partially rescued by FGF8 administered locally (bead implants. Our findings from zebrafish model were validated in a dental survey that was conducted with assistance of the MHE Research Foundation. The presence of the malformed and/or displaced teeth with abnormal enamel was declared by half of the respondents indicating that MO might indeed be also associated

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

    International Nuclear Information System (INIS)

    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 cm3, or aV70 > 1.7 cm3. 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 cm3 or aV70 > 1.7 cm3. Treatment planning goals should include constraints on the volume of temporal lobes receiving higher dose. The EC50

  6. Neglected orthopedic oncology - Causes, epidemiology and challenges for management in developing countries

    Directory of Open Access Journals (Sweden)

    Y S Siddiqui

    2015-01-01

    Full Text Available Background: Management of malignant bone and soft tissue tumors remains an overwhelming confront to orthopedic surgeons. The challenge is discriminating in developing countries due to inadequate diagnostic and therapeutic amenities and unawareness. A lot has been discussed about the neglected orthopedic trauma, but the published literature on the causes and management of neglected bone and soft tissue tumors is sparse. Hence, current study was undertaken to highlight the causes of neglect and therapeutic challenges for managing these neglected tumors in developing countries. Aims And Objectives: To determine the causes of neglect of malignant bone and soft tissue tumors, their epidemiology (including their relative frequencies, age, gender discrimination, anatomical sites of occurrence and histological characteristics and difficult aspect of management due to neglect or delayed presentation. Materials And Methods: This was an appraisal of the neglected malignant bone and soft tissue tumors presented to J. N. Medical College and Hospital from June 2008 to May 2013. Criteria for labeling the tumor as neglected malignant bone and soft tissue tumor was delayed presentation (>3 months, locally advanced disease, ulceration, sepsis, fungating mass or metastasis at the time of presentation. All the cases were reviewed and analyzed for age, gender, histological types, educational status and socioeconomic status of the family, any prior treatment by traditional bone setters or registered medical practitioner, cause of delay for seeking medical advice. We have also analyzed the treatment given at our institute and the outcome of the tumor. Observations And Results: Eighteen patients fulfilled the criteria for neglected malignant bone and soft tissue tumors, hence were included in study. Eight cases were of osteosarcoma, five cases were of Ewing's sarcoma, three cases were of chondrosarcoma and 1 case each was of pleomorphic liposarcoma and primary lymphoma

  7. Sulphation heterogeneity in the trisaccharide (GalNAcSbeta1, 4GlcAbeta1,3GalNAcS) isolated from the non-reducing terminal of human aggrecan chondroitin sulphate.

    Science.gov (United States)

    West, L A; Roughley, P; Nelson, F R; Plaas, A H

    1999-08-15

    We report here the isolation and sulphation isomer analyses of trisaccharides GalNAcS(beta1,4)GlcA(beta1,3)GalNAcS (in which S indicates sulphate) derived from the non-reducing termini of aggrecan chondroitin sulphate. Rat chondrosarcoma and human aggrecans were digested for 1 h at 37 degrees C with 30 micro-units of endo-chondroitinase ABC per microgram of chondroitin sulphate, and trisaccharides were isolated from the digests by ToyoPearl HW40S gel-filtration chromatography. Four trisaccharide species were identified; their sulphation isomer compositions, as determined by digestion with chondroitinase ACII and fluorescence-based ion-exchange HPLC, were GalNAc4Sbeta1,4GlcAbeta1,3GalNAc4S, GalNAc4Sbeta1,4GlcAbeta1,3GalNAc6S, GalNAc4,6Sbeta1,4GlcAbeta1, 3GalNAc4S and GalNAc4,6Sbeta1,4GlcAbeta1,3GalNAc6S. The abundances of such sequences in chondroitin sulphate on aggrecan from normal (foetal to 72 years of age) and from osteoarthritic human knee cartilages were also established. The results showed that non-reducing terminal GalNAc4S or GalNAc4,6S can be linked to either a 4-sulphated or a 6-sulphated disaccharide, suggesting that the sulphation of the last disaccharide might not have a direct effect on the specificity of chondroitin sulphate terminal GalNAc sulphotransferases. Furthermore, for each aggrecan preparation examined, the 4S-to-6S ratio of all chain interior disaccharides was equivalent to that in the last repeating disaccharides at the non-reducing terminus, suggesting that neither chondroitin 4-sulphotransferase nor chondroitin 6-sulphotransferase shows preferential activity near the chain terminus. PMID:10432320

  8. 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

  9. 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

  10. 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

  11. Resecciones de pared torácica y su reconstrucción Resection of thoracic wall and its repair

    Directory of Open Access Journals (Sweden)

    Abel Nicolás Monzón Fernández

    2009-03-01

    with predominance of 6 chondrosarcoma, 5 metastatic, 3 Askin and others histological tipes. Haven`t been died in this series by complications but if by progression disease in 9.The survival was by 5 year(44,7% coincidence with the mayority of died in advace stade. Conclusions: Extensive resection of tumours of the chest wall with reconstruction using prostheses and muscle-skin flaps is a safe method that can be performed in the same surgical intervention period, it also provide a good overlife and an optimal life quality.

  12. 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

  13. 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

  14. 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.

  15. 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

  16. 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

  17. 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