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Sample records for chordoma

  1. Chordoma

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    Saxton, J.P.

    1981-07-01

    Nineteen patients with chordoma seen at M.D. Anderson Hospital from 1948 to 1976 received definitive treatment. Six patients presented with disease in the basisphenoid region, 2 with disease in the lumbar spine (vertebral area), and 11 with disease in the sacrococcygeal area. Nine patients were treated with a combination of surgery and postoperative radiation therapy, 6 received radiation therapy only, and 4 underwent surgery only. Although the number of patients studied is small, the results suggest that surgery only is not an effective means of treating this disease. Radiation therapy only produces palliation for large inoperable lesions, but excision followed by irradiation is the best treatment for securing prolonged local control.

  2. Chordoma

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    Reddy, E.K.; Mansfield, C.M.; Hartman, G.V.

    1981-12-01

    Clinical presentation and management of ten patients with diagnosis of chordoma seen at University of Kansas Medical Center from 1959 to 1978 is presented. The average age was 41.5 years with a female to male ratio of 7:3.Primary treatment was surgical. Eight patients received radiation therapy with a varying dose ranging from 4000 to 8000 rad in four to eight weeks. Six patients recurred (60%). The average period of recurrence was four years in the irradiated patients compared to 2 1/2 years in non-irradiated patients. Three of ten patients (33%) are alive at five years free of disease. Favorable results with long term disease-free survival may be obtained with the combined use of surgery and aggressive radiation therapy to a dose of 6500 and 7000 rad in six to seven weeks, keeping in mind normal tissue tolerance.

  3. Chordoma

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    Reddy, E.K.; Mansfield, C.M.; Hartman, G.V.

    1981-12-01

    Clinical presentation and management of ten patients with diagnosis of chordoma seen at University of Kansas Medical Center from 1959 to 1978 is presented. The average age was 41.5 years with a female to male ratio of 7:3. Primary treatment was surgical. Eight patients received radiation therapy with a varying dose ranging from 4000 to 8000 rad in four to eight weeks. Six patients recurred (60%). The average period of recurrence was four years in the irradiated patients compared to 2 1/2 years in non-irradiated patients. Three of ten patients (33%) are alive at five years free of disease. Favorable results with long term disease-free survival may be obtained with the combined use of surgery and aggressive radiation therapy to a dose of 6500 and 7000 rad in six to seven weeks, keeping in mind normal tissue tolerance.

  4. Molecular profiling of chordoma

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    SCHEIL-BERTRAM, STEFANIE; KAPPLER, ROLAND; VON BAER, ALEXANDRA; HARTWIG, ERICH; SARKAR, MICHAEL; SERRA, MASSIMO; BRÜDERLEIN, SILKE; WESTHOFF, BETTINA; MELZNER, INGO; BASSALY, BIRGIT; HERMS, JOCHEN; HUGO, HEINZ-HERMANN; SCHULTE, MICHAEL; MÖLLER, PETER

    2014-01-01

    The molecular basis of chordoma is still poorly understood, particularly with respect to differentially expressed genes involved in the primary origin of chordoma. In this study, therefore, we compared the transcriptional expression profile of one sacral chordoma recurrence, two chordoma cell lines (U-CH1 and U-CH2) and one chondrosarcoma cell line (U-CS2) with vertebral disc using a high-density oligonucleotide array. The expression of 65 genes whose mRNA levels differed significantly (p<0.001; ≥6-fold change) between chordoma and control (vertebral disc) was identified. Genes with increased expression in chordoma compared to control and chondrosarcoma were most frequently located on chromosomes 2 (11%), 5 (8%), 1 and 7 (each 6%), whereas interphase cytogenetics of 33 chordomas demonstrated gains of chromosomal material most prevalent on 7q (42%), 12q (21%), 17q (21%), 20q (27%) and 22q (21%). The microarray data were confirmed for selected genes by quantitative polymerase chain reaction analysis. As in other studies, we showed the expression of brachyury. We demonstrate the expression of new potential candidates for chordoma tumorigenesis, such as CD24, ECRG4, RARRES2, IGFBP2, RAP1, HAI2, RAB38, osteopontin, GalNAc-T3, VAMP8 and others. Thus, we identified and validated a set of interesting candidate genes whose differential expression likely plays a role in chordoma. PMID:24452533

  5. Sellar chondroid chordoma

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    Xia LI

    2014-02-01

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

  6. Cervical spine chordoma

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    Díez-González L

    2012-03-01

    Full Text Available Chordomas are neoplasms that arise from notochord embryonic remnants, been the sacrococcygeal spine the main site of involvement; the cervical spine site is uncommon and it account for less than 10% of chordomas. Because of their slow growth, the diagnosis is delayed until they reach a large size, despite which they are locally aggressive tumours due to their relation to critical neurovascular structures and present a high rate of local recurrence. Radical surgery is the elective treatment and proton radiotherapy is used when residual tumour tissue and recurrences.Because of the uncommonness of this pathology, we report a case of a patient with cervical chordoma.

  7. Lumbar spine chordoma

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    M.A. Hatem, M.B.Ch.B, MRes, LMCC

    2014-01-01

    Full Text Available Chordoma is a rare tumor arising from notochord remnants in the spine. It is slow-growing, which makes it difficult to diagnose and difficult to follow up after treatment. Typically, it occurs in the base of the skull and sacrococcygeal spine; it rarely occurs in other parts of the spine. CT-guided biopsy of a suspicious mass enabled diagnosis of lumbar spine chordoma.

  8. Lumbar spine chordoma

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    Hatem, M.A.

    2015-01-01

    Chordoma is a rare tumor arising from notochord remnants in the spine. It is slow-growing, which makes it difficult to diagnose and difficult to follow up after treatment. Typically, it occurs in the base of the skull and sacrococcygeal spine; it rarely occurs in other parts of the spine. CT-guided biopsy of a suspicious mass enabled diagnosis of lumbar spine chordoma. PMID:27186250

  9. Chordoma Of Lumbar Spine

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    Nirmala M.J.*, H.A. Parshwanath and A.M.Patil

    2010-10-01

    Full Text Available Chordoma, lesion derived from the notochord, represents about 4% of the primary malignant bone tumours. Males are affected more commonly than females and it is very rare in children;the peak incidence is in the sixth decade of life.The sacrococcygeal region accounts for 50% of cases and the spheno-occipital region or the base of the skull for 37% of cases.The remainder of the cases reported occur in the descending order of frequency in the cervical,thoracic and the lumbar spine.It is found to be rarely involving the lumbar spine(about 2%.We present a case of chordoma involving L4 and L5 vertebral body and disc.The role of epithelial cell marker study has proved a well adjunct to the histopathological diagnosis of chordoma.

  10. Primary chordoma of the nose

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    牟忠林; 刘兆华

    2003-01-01

    @@ Chordoma as a rare malignant tumor arising from remnants of the notochord, accounts for 4% of malignant bone tumors. It occurs twice as frequently in men compared with women and is uncommon in individuals less than 40 years of age.1-3 Topographic distribution is spheno-occipital (35% to 44%), vertebral (15%), and sacrococcygeal (42% to 50%).4 Unusual locations include the mandible and the maxilla, and lesions in these locations have been called ectopic or dental chordomas. Some chordomas with nasal and paranasal presentations have been reported, but primary chordoma of the nose occurs rarely. To our knowlege only two cases have been reported so far according to the literature.4,5 In this paper, we present primary chordoma of the nose, its characteristics and our experiences with it.

  11. AMBIGUITY IN DIAGNOSING CHONDROID CHORDOMA-A CASE REPORT

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    Swarnagowri

    2014-08-01

    Full Text Available "Chondroid chordoma" is a controversial and confusing entity, a biphasic malignant neoplasm possessing elements of both chordoma and cartilaginous tissue. The annual incidence of chordoma is approximately one in one million. Chondroid chordoma is a subtype of chordoma possessing elements of both chordoma and cartilaginous tissue with better prognosis than classic (non chondroid chordomas. We have come across a case of Chondroid Chordoma in a 55 year old male. Our report features particularly a rare entity, chondroid chordoma, with an exceptional localization to the nasal septum. Chondroid Chordomas are likely to recur and hence diagnostically important to institute effective treatment

  12. A vertebral extra dural chordoma at C5, possibly deriving from a clival chordoma

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    Goes, R.; van Overbeeke, J.J.

    2015-01-01

    Background: Clival chordomas are a rare type of cancer with low metastatic potential and primary metastasize to the lung or bones. Case Description: This case report describes a possible metastatic, paravertebral chordoma at level C4-C5 in a patient with a past medical history of a clival chordoma. Conclusion: Chordomas are unpredictable and may metastasise. PMID:26097773

  13. Chondroid Chordoma of the Sphenoid Sinus- a rare tumour

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    Prabodh Karnik

    2014-06-01

    Full Text Available Chordomas of the skull base are extremely rare intracranial malignancies that arise from ectopic remnants of embryonal notochord. Chondroid chordoma is a subtype of chordoma possessing elements of both chordoma and cartilaginous tissue with better prognosis than classic (nonchondroid chordomas. We herein report a case of chondroid chordoma of sphenoid sinus in a 46 years old female. We believe this is the first case of chondroid chordoma involving only the sphenoid sinus.

  14. Sarcomatoid chordoma: chordoma with a massive malignant spindle-cell component

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    Morimitsu, Yosuke; Hashimoto, H. [Univ. of Occupational and Environmental Health, Kitakyushu (Japan). Dept. of Pathology and Oncology; Aoki, Takatoshi [Dept. of Radiology, Univ. of Occupational and Environmental Health, Kitakyushu (Japan); Yokoyama, Koichiro [Dept. of Orthopedics, National Kokura Hospital, Kitakyushu (Japan)

    2000-12-01

    We report a case of chordoma containing a spindle cell sarcomatoid component with a gradual transition from conventional chordoma. Immunohistochemically, many tumor cells in both conventional chordoma and sarcomatoid components were positive for cytokeratins (AE1/AE3, CAM5.2) and epithelial membrane antigen as well as vimentin. This report provides a rare example of sarcomatoid chordoma. Familiarity with this type of bone tumor should help to avoid confusion with dedifferentiated chordoma and other spindle cell sarcomas or carcinomas. (orig.)

  15. Thoracic chordoma: CT and MR findings

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    Cha, Yoo Mi; Hwang, Hee Young; Kim, Sang Joon; Chung, Hyo Sun; Han, Heon [Gil General Hospital, Incheon (Korea, Republic of)

    1993-05-15

    Chordoma arising from the notochordal remnants is a rare primary bone tumor in the cervicosacral region and is even more unusual in the thoracic region. The authors experienced a case of thoracic chordoma and reports its CT and MR findings.

  16. Clival chordoma in an infant.

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    Goel A

    1996-04-01

    Full Text Available An unusual case of clival chordoma seen in a 7 month 16 day old infant is presented. The literature on this subject, clinical course of such tumours and the management strategy in paediatric age group is reviewed.

  17. Vaccine Therapy for Unresectable Chordoma

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    In this phase II clinical trial, adult patients with inoperable chordoma who are scheduled to undergo radiation therapy will be randomly assigned to receive a yeast-based vaccine that targets a protein called brachyury or a placebo injection.

  18. Intradural Clival Chordoma: A Case Report

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    2014-01-01

    Clival chordoma is a rare intracranial neoplasm located in the clivus with bony extension and destruction. It is difficult to resect completely and generally has a poor prognosis. However, intradural clival chordomas have been reported with good surgical outcomes. We present a rare case of intradural chordoma and a review of the literature. PMID:25408929

  19. Recurrent cervical chordoma: A case illustration

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    Jun Liu; Bin Ni; Ning Xie; Huajiang Chen; Fei Wang; Zhuangchen Zhu; Peida Lin

    2009-01-01

    A case is described of multi-recurrent cervical chordoma in a man over a 5 year period. The clinical features were of progressive spinal cord compression. The authors report a chordoma at C4 that recurred 3 times in five years. The patient underwent four operations and suffered distant metastases. This case confirms that thorough resection of the tumor during the first surgery and postoperative adjuvant treatment are the best assurance of a good prognosis with a chordoma. Multiple surgeries can stimulate biological activity of a chordoma and make its recurrence and distant metastases much more likely. The authors discuss the diagnosis, surgical treatment and the relationship between the histopathological changes and malignancy of a spinal chordoma after four operations. To our knowledge, this represents the first report of a 4th surgery for cervical chordoma.

  20. A unique presentation of retroclival chordoma.

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    Warakaulle D

    2002-10-01

    Full Text Available Chordomas are rare tumours which arise from remnants of the primitive notochord. They occur primarily in the sacrum, clivus and cervical regions. We report a case of retroclival chordoma which presented as an extradural haemorrhage following minor trauma. The underlying tumour was not apparent on imaging performed immediately following the event, and chordoma presenting in this manner has not previously been described in the literature. The tumour became apparent on subsequent imaging, and progressed despite surgical debulking and radiotherapy.

  1. Unusual Presentation of Chordoma in Nose.

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    Gupta, Divya; Rathore, Praveen Kumar; Chauhan, Anju; Khurana, Nita

    2016-09-01

    Chordomas are uncommon tumors of the bone representing 1-4 % of all primary bone tumors. These typically involve axial skeleton. Primary nasal presentation of chordoma is a rare occurrence and is usually misdiagnosed till the histopathological confirmation. We present a case of chordoma in nasal cavity treated by wide local resection and also present a review of literature discussing various facets in its presentation and management.

  2. Hand metastasis from a sacral chordoma

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    Kaya, M; Sugita, S; Soma, T; Sasaki, M; Yamashita, T

    2014-01-01

    Chordomas are rare, low grade, malignant tumours derived from the ectopic remnants of the notochord that line the axial skeleton. They are characterised by their slow growth, long disease course and propensity for local relapse. Furthermore, up to 40% of non-cranial chordomas metastasise. We describe the first reported case of a hand metastasis arising from a conventional sacral chordoma after carbon ion radiotherapy. The common occurrence of distant metastasis with chordomas makes it important to perform a systemic examination, in part because their resection might improve patient prognosis. PMID:25350167

  3. Hemorrhagic chondroid chordoma mimicking pituitary apoplexy

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    Lee, H.J.; Kalnin, A.J.; Holodny, A.I. [Dept. of Radiology, University Hospital, Newark, NJ (United States); Schulder, M.; Grigorian, A. [Dept. of Neurosurgery, University Hospital, Newark, NJ (United States); Sharer, L.R. [Dept. of Pathology, University Hospital, Newark, NJ (United States)

    1998-11-01

    We describe a hemorrhagic chondroid chordoma involving the sella turcica with suprasellar extension. The CT and MRI appearances mimiked a hemorrhagic pituitary adenoma. Chondroid chordoma is a variant composed of elements of both chordoma and cartilaginous tissue. An uncommon bone neoplasm, located almost exclusively in the spheno-occipital region, it is usually not considered in the differential diagnosis of a tumor with acute hemorrhage in the sellar region. We discuss the clinical and radiological characteristics which may allow one to differentiate chondroid chordoma from other tumors of this area. (orig.) With 3 figs., 9 refs.

  4. CHONDROID CHORDOMA OF NASAL SEPTUM : A CASE REPORT

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    Gayattre

    2015-04-01

    Full Text Available Chordomas are rare neoplasms of presumed notochordal origin that arise along the vertebral axis and show a proclivity for the spheno - occipital and sacral regions. ( 1,2 Chondroid chordoma is a variant of chordoma, with cartilaginous component . We report a histologically proven case of chondroid chordoma, with an exceptional localization to the nasal septum . Chondroid Chordomas are likely to recur and hence diagnostically important to institute effective treatment .

  5. Molecular Characterization of Putative Chordoma Cell Lines

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    Silke Brüderlein

    2010-01-01

    Full Text Available Immortal tumor cell lines are an important model system for cancer research, however, misidentification and cross-contamination of cell lines are a common problem. Seven chordoma cell lines are reported in the literature, but none has been characterized in detail. We analyzed gene expression patterns and genomic copy number variations in five putative chordoma cell lines (U-CH1, CCL3, CCL4, GB60, and CM319. We also created a new chordoma cell line, U-CH2, and provided genotypes for cell lines for identity confirmation. Our analyses revealed that CCL3, CCL4, and GB60 are not chordoma cell lines, and that CM319 is a cancer cell line possibly derived from chordoma, but lacking expression of key chordoma biomarkers. U-CH1 and U-CH2 both have gene expression profiles, copy number aberrations, and morphology consistent with chordoma tumors. These cell lines also harbor genetic changes, such as loss of p16, MTAP, or PTEN, that make them potentially useful models for studying mechanisms of chordoma pathogenesis and for evaluating targeted therapies.

  6. Radiologic findings in posterior mediastinal chordoma

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    Stratt, B.; Steiner, R.M.

    1980-07-01

    A 14-year-old girl with a posterior mediastinal chordoma is described. Computed tomography is helpful in defining the extent of the soft tissue mass. Osseous changes related to the presence of the chordoma are described. A review of the pertinent literature is presented.

  7. Secondary pulmonary conventional chordoma arising from primary sarcomatoid chordoma of the sacrum: A case report

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    CHEN, JIA-HONG; CHEN, KUAN-YU; HUENG, DUENG-YUAN; JIN, JONG-SHIAW

    2014-01-01

    Chordomas are low- to intermediate-grade malignant tumors that recapitulate the notochord. Chordomas belong to the dysontogenetic bone tumors and appear primarily in the region of the axial skeleton. Chordomas are divided into conventional, chondroid, sarcomatoid and dedifferentiated subtypes. The different subtypes of chordoma have varied survival periods. According to the literature to date, secondary pulmonary and lymph-node metastases occur most frequently, followed by liver, bone and skin metastases. To the best of our knowledge, there has been no previous report of one subtype of chordoma metastasizing or transforming into another subtype with a different histopathology. This study presents a 24-year-old man with secondary pulmonary conventional chordoma arising from a primary sarcomatoid chordoma of the sacrum. The patient was alive at the end of November, 2009 and the survival time exceeded eight years. This is the first case of a patient with primary sarcomatoid chordoma of the sacrum with complete remission in whom a secondary pulmonary conventional chordoma arose from the primary cancer. PMID:24959247

  8. Chordoma with postoperative subcutaneous implantation and meningeal dissemination: MRI

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    Kinoshita, T. [Dept. of Radiology, Tottori University, Yonago (Japan); Dept. of Radiology, Research Institute of Brain and Blood Vessels-Akita, Akita (Japan); Okudera, T.; Shimosegawa, E.; Hatazawa, J. [Dept. of Radiology, Research Institute of Brain and Blood Vessels-Akita, Akita (Japan); Yoshida, Y. [Dept. of Pathology, Research Institute of Brain and Blood Vessels-Akita, Akita (Japan); Yasui, N. [Dept. of Surgical Neurology, Research Institute of Brain and Blood Vessels-Akita, Akita (Japan); Ogawa, T. [Dept. of Radiology, Tottori University, Yonago (Japan)

    2001-09-01

    Chordomas are histologically benign tumours which are locally invasive. We present an unusual case of recurrent chordoma with subcutaneous implantation and widespread meningeal dissemination after surgery. Contrast-enhanced MRI was useful for determining the extent of the tumour. (orig.)

  9. Sacral Chordoma and Single Stage Combined Anteroposterior High Sacrectomy.

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    Vasu Reddy Challa

    2014-05-01

    Full Text Available Chordomas are locally aggressive tumors, most commonly seen in sacrum. We present a case of sacral chordoma who presented with urinary retention and treated with single stage combined anteroposterior high sacrectomy.

  10. Multifocal thoracic chordoma mimicking a paraganglioma

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    Giovanni Conzo

    2013-01-01

    Full Text Available Chordoma of thoracic vertebras is a very rare locally invasive neoplasm with low grade malignancy arising from embryonic notochordal remnants. Radical surgery remains the cornerstone of the treatment. We describe a case of multifocal T1-T2 chordoma, without bone and disc involvement, incidentally misdiagnosed as a paraganglioma, occurring in a 47-year-old male asymptomatic patient. Neoplasm was radically removed by an endocrine surgeon through a right extended cervicotomy. A preoperative reliable diagnosis of chordoma, as in the reported case, is often difficult. Radical surgery can provide a favorable outcome but, given the high rates of local recurrence of this neoplasm, a strict and careful follow-up is recommended. Although very rare, chordoma should be suggested in the differential diagnosis of the paravertebral cervical masses of unknown origin. Spine surgeon consultation and a FNB should be routinely included in the multidisciplinary preoperative work-up of these neoplasms.

  11. Multifocal thoracic chordoma mimicking a paraganglioma.

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    Conzo, Giovanni; Gambardella, Claudio; Pasquali, Daniela; Ciancia, Giuseppe; Avenia, Nicola; Pietra, Cristina Della; Napolitano, Salvatore; Palazzo, Antonietta; Mauriello, Claudio; Parmeggiani, Domenico; Pettinato, Guido; Napolitano, Vincenzo; Santini, Luigi

    2013-01-01

    Chordoma of thoracic vertebras is a very rare locally invasive neoplasm with low grade malignancy arising from embryonic notochordal remnants. Radical surgery remains the cornerstone of the treatment. We describe a case of multifocal T1-T2 chordoma, without bone and disc involvement, incidentally misdiagnosed as a paraganglioma, occurring in a 47-year-old male asymptomatic patient. Neoplasm was radically removed by an endocrine surgeon through a right extended cervicotomy. A preoperative reliable diagnosis of chordoma, as in the reported case, is often difficult. Radical surgery can provide a favorable outcome but, given the high rates of local recurrence of this neoplasm, a strict and careful follow-up is recommended. Although very rare, chordoma should be suggested in the differential diagnosis of the paravertebral cervical masses of unknown origin. Spine surgeon consultation and a FNB should be routinely included in the multidisciplinary preoperative work-up of these neoplasms. PMID:24125991

  12. Intracranial metastasis from a sacrococcygeal chordoma. Case report.

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    Kamel, Mahmoud Hamdy

    2012-02-03

    Chordoma is a locally invasive tumor of low metastatic potential. Only six cases of chordoma that metastasized to the brain are found in the English literature. Most of these lesions were clinically silent and all were associated with extraneural metastases. The authors report a case of symptomatic brain metastasis from a sacrococcygeal chordoma in the absence of other metastases. The incidence, sites, and factors predictive of chordoma metastasis are discussed.

  13. Familial chordoma: A case report and review of the literature

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    WANG, KE; WU, ZHEN; TIAN, KAIBING; WANG, LIANG; HAO, SHUYU; ZHANG, LIWEI; ZHANG, JUNTING

    2015-01-01

    Familial skull base chordoma is a rare tumor derived from the remnants of the embryonic notochord. The present study describes the clinical presentation of 4 cases of skull base chordomas in a family. A 15-year-old female received staged surgeries and was pathologically confirmed with a diagnosis of skull base chordoma. Among the patient's family, 2 members had previously undergone surgery and were pathologically confirmed with chordomas; 1 family member had also received radiation therapy. Furthermore, the patient's cousin, an 18-year-old male, was confirmed to have this condition by epipharyngoscopy. All confirmed cases within the family remained alive with the condition. A literature review of familial chordoma was undertaken and 8 chordoma pedigrees were found. Familial chordoma was rare, with an estimated rate of 0.4% in all chordomas. The skull base was the predominant location for familial chordoma. Compared with sporadic chordoma, familial chordomas were diagnosed at a younger age. The brachyury gene was strongly associated with familial chordomas, however, the exact pathogenesis and genetics mechanisms remains unclear. PMID:26722267

  14. Novel targeted therapies in chordoma: an update

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    Di Maio, Salvatore; Yip, Stephen; Al Zhrani, Gmaan A; Alotaibi, Fahad E; Al Turki, Abdulrahman; Kong, Esther; Rostomily, Robert C

    2015-01-01

    Chordomas are rare, locally aggressive skull base neoplasms known for local recurrence and not-infrequent treatment failure. Current evidence supports the role of maximal safe surgical resection. In addition to open skull-base approaches, the endoscopic endonasal approach to clival chordomas has been reported with favorable albeit early results. Adjuvant radiation is prescribed following complete resection, alternatively for gross residual disease or at the time of recurrence. The modalities of adjuvant radiation therapy reported vary widely and include proton-beam, carbon-ion, fractionated photon radiotherapy, and photon and gamma-knife radiosurgery. As of now, no direct comparison is available, and high-level evidence demonstrating superiority of one modality over another is lacking. While systemic therapies have yet to form part of any first-line therapy for chordomas, a number of targeted agents have been evaluated to date that inhibit specific molecules and their respective pathways known to be implicated in chordomas. These include EGFR (erlotinib, gefitinib, lapatinib), PDGFR (imatinib), mTOR (rapamycin), and VEGF (bevacizumab). This article provides an update of the current multimodality treatment of cranial base chordomas, with an emphasis on how current understanding of molecular pathogenesis provides a framework for the development of novel targeted approaches. PMID:26097380

  15. Cervical Vertebral Body Chordoma in a Cat.

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    Hampel, R; Taylor-Brown, F; Priestnall, S L

    2016-05-01

    A 9-year-old, neutered female Maine Coon cat with a 6-week history of progressive ataxia was diagnosed with a cervical vertebral body mass using magnetic resonance imaging. The mass displaced and compressed the cervical spinal cord. The cat was humanely destroyed and necropsy examination confirmed a mass within the second cervical vertebral body. Microscopically, the mass was composed of large, clear, vacuolated ('physaliferous') cells. Immunohistochemically, the neoplastic cells expressed both cytokeratin and vimentin and the final diagnosis was a cervical, vertebral body chordoma. This is only the third report of a chordoma in this species and the first in this location. Chordoma should be considered as a potential differential diagnosis for tumours arising from the cervical vertebrae in the cat.

  16. Frequent activation of EGFR in advanced chordomas

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    Dewaele Barbara

    2011-07-01

    Full Text Available Abstract Background Chordomas are rare neoplasms, arising from notochordal remnants in the midline skeletal axis, for which the current treatment is limited to surgery and radiotherapy. Recent reports suggest that receptor tyrosine kinases (RTK might be essential for the survival or proliferation of chordoma cells, providing a rationale for RTK targeted therapy. Nevertheless, the reported data are conflicting, most likely due to the assorted tumor specimens used for the studies and the heterogeneous methodological approaches. In the present study, we performed a comprehensive characterization of this rare entity using a wide range of assays in search for relevant therapeutic targets. Methods Histopathological features of 42 chordoma specimens, 21 primary and 21 advanced, were assessed by immunohistochemistry and fluorescent in situ hybridization (FISH using PDGFRB, CSF1R, and EGFR probes. Twenty-two of these cases, for which frozen material was available (nine primary and 13 advanced tumors, were selectively analyzed using the whole-genome 4.3 K TK-CGH-array, phospho-kinase antibody array or Western immunoblotting. The study was supplemented by direct sequencing of KIT, PDGFRB, CSF1R and EGFR. Results We demonstrated that EGFR is frequently and the most significantly activated RTK in chordomas. Furthermore, concurrent to EGFR activation, the tumors commonly reveal co-activation of alternative RTK. The consistent activation of AKT, the frequent loss of the tumor suppressor PTEN allele, the recurrent activation of upstream RTK and of downstream effectors like p70S6K and mTOR, all indicate the PI3K/AKT pathway as an important mediator of transformation in chordomas. Conclusions Given the complexity of the signaling in chordomas, combined treatment regimens targeting multiple RTK and downstream effectors are likely to be the most effective in these tumors. Personalized therapy with careful selection of the patients, based on the molecular profile of

  17. Combined PDGFR and HDAC Inhibition Overcomes PTEN Disruption in Chordoma

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    Kassam, Amin B.; Park, Myung-Jin; Gardner, Paul; Prevedello, Daniel; Henry, Stephanie; Horbinski, Craig; Beumer, Jan H.; Tawbi, Hussein; Williams, Brian J.; Shaffrey, Mark E.; Egorin, Merrill J.; Abounader, Roger; Park, Deric M.

    2015-01-01

    Background The majority of chordomas show activation of the platelet-derived growth factor receptor (PDGFR). Based on in vitro intertumoral variation in response to recombinant PDGF protein and PDGFR inhibition, and variable tumor response to imatinib, we hypothesized that chordomas resistant to PDGFR inhibition may possess downstream activation of the pathway. Methods Molecular profiling was performed on 23 consecutive chordoma primary tissue specimens. Primary cultures established from 20 of the 23 specimens, and chordoma cell lines, UCH-1 and UCH-2, were used for in vitro experiments. Results Loss of heterozygosity (LOH) at the phosphatase and tensin homolog (PTEN) locus was observed in 6 specimens (26%). PTEN disruption statistically correlated with increased Ki-67 proliferation index, an established marker of poor outcome for chordoma. Compared to wild type, PTEN deficient chordomas displayed increased proliferative rate, and responded less favorably to PDGFR inhibition. PTEN gene restoration abrogated this growth advantage. Chordomas are characterized by intratumoral hypoxia and local invasion, and histone deacetylase (HDAC) inhibitors are capable of attenuating both hypoxic signaling and cell migration. The combination of PDGFR and HDAC inhibition effectively disrupted growth and invasion of PTEN deficient chordoma cells. Conclusions Loss of heterozygosity of the PTEN gene seen in a subset of chordomas is associated with aggressive in vitro behavior and strongly correlates with increased Ki-67 proliferative index. Combined inhibition of PDGFR and HDAC attenuates proliferation and invasion in chordoma cells deficient for PTEN. PMID:26247786

  18. Combined PDGFR and HDAC Inhibition Overcomes PTEN Disruption in Chordoma.

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    Dae-Hee Lee

    Full Text Available The majority of chordomas show activation of the platelet-derived growth factor receptor (PDGFR. Based on in vitro intertumoral variation in response to recombinant PDGF protein and PDGFR inhibition, and variable tumor response to imatinib, we hypothesized that chordomas resistant to PDGFR inhibition may possess downstream activation of the pathway.Molecular profiling was performed on 23 consecutive chordoma primary tissue specimens. Primary cultures established from 20 of the 23 specimens, and chordoma cell lines, UCH-1 and UCH-2, were used for in vitro experiments.Loss of heterozygosity (LOH at the phosphatase and tensin homolog (PTEN locus was observed in 6 specimens (26%. PTEN disruption statistically correlated with increased Ki-67 proliferation index, an established marker of poor outcome for chordoma. Compared to wild type, PTEN deficient chordomas displayed increased proliferative rate, and responded less favorably to PDGFR inhibition. PTEN gene restoration abrogated this growth advantage. Chordomas are characterized by intratumoral hypoxia and local invasion, and histone deacetylase (HDAC inhibitors are capable of attenuating both hypoxic signaling and cell migration. The combination of PDGFR and HDAC inhibition effectively disrupted growth and invasion of PTEN deficient chordoma cells.Loss of heterozygosity of the PTEN gene seen in a subset of chordomas is associated with aggressive in vitro behavior and strongly correlates with increased Ki-67 proliferative index. Combined inhibition of PDGFR and HDAC attenuates proliferation and invasion in chordoma cells deficient for PTEN.

  19. Intensity-Modulated Radiotherapy of Sacral Chordoma

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    Thilmann, Christoph; Schulz-Ertner, Daniela; Zabel, Angelika; Herfarth, Klaus K.; Wannenmacher, Michael; Debus, Juergen [German Cancer Research Center, Heidelberg (Germany)

    2002-08-01

    In a case of partially resected sacral chordoma, the planning target volume (PTV) received 60 Gy and the gross target volume (GTV) 72 Gy using inversely planned, intensity-modulated, radiation therapy (IMRT). IMRT was compared with 3D-conformal radiotherapy (CRT). With IMRT, it was found that dose distribution is more homogeneous within the PTV outside the GTV and allows simultaneous dose escalation within the GTV. The volume of bowel receiving a dose higher than 40 Gy was reduced from 400cc with CRT to 220cc with IMRT. If particle therapy is not available, IMRT seems to be a promising alternative in the treatment of sacral chordomas.

  20. Giant clival chordoma causing pathological laughter

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    Gripp, Daniel Andrade; do Souto, Antonio Aversa; Gonsales, Douglas; Christiani, Marcio de Miranda Chaves; Nogueira, Janio; Lopes, Helio Ferreira; Torres, Yasmine Coura

    2014-01-01

    Background: Chordomas are rare slowly growing tumors that originate from remnants of the notochord. They have a malignant local behavior, causing symptoms due to bone infiltration and compression of neurovascular structures. Only a few cases of brain tumors associated with pathological laughter have been reported in the literature. Case Description: We report a case of a 42-year-old male patient with this atypical clinical presentation treated at our institution, and discuss the concerning literature. Conclusion: Although being a very rare presentation of chordomas, pathological laughter is usually expected to improve after brain stem decompression. PMID:24778906

  1. Giant clival chordoma causing pathological laughter

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    Daniel Andrade Gripp

    2014-01-01

    Full Text Available Background: Chordomas are rare slowly growing tumors that originate from remnants of the notochord. They have a malignant local behavior, causing symptoms due to bone infiltration and compression of neurovascular structures. Only a few cases of brain tumors associated with pathological laughter have been reported in the literature. Case Description: We report a case of a 42-year-old male patient with this atypical clinical presentation treated at our institution, and discuss the concerning literature. Conclusion: Although being a very rare presentation of chordomas, pathological laughter is usually expected to improve after brain stem decompression.

  2. Expression of MDR1, HIF-1α and MRP1 in sacral chordoma and chordoma cell line CM-319

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    Ma Baoan

    2010-12-01

    Full Text Available Abstract Background Chordoma was a typically slow-growing tumor. The therapeutic approach to chordoma had traditionally relied mainly on surgical therapy. And the main reason for therapeutic failure was resistance to chemotherapy and radiotherapy. However the refractory mechanism was not clear. The aim of this study was to investigate the expression of three genes (MDR1, HIF-1α and MRP1 associated with resistance to chemotherapy and radiotherapy in chordoma and chordoma cell line CM-319. Materials and methods Using immunohistochemical techniques, the expression of MDR1, HIF-1α and MRP1 was investigated in 50 chordoma specimen. Using RT-PCR and Western blot, the expression of MDR1, HIF-1α and MRP1 was investigated in chordoma and chordoma cell line CM-319. Results Expression of MDR1, HIF-1α and MRP1 was observed in 10%, 80% and 74% of all cases, respectively. Expression of MRP1 was correlated with HIF-1α. On the other hand, expression of MDR1 was not correlated with the expression of HIF-1α or MRP1. The expression of HIF-1α and MRP1 was observed, but MDR1 was not observed in chordoma and CM-319. Conclusion Expression of HIF-1α and MRP1 was observed in most chordoma specimen and CM-319 cell line; expression of HIF-1α correlated with MRP1. HIF-1α and MRP1 may play a role in the multidrug resistance of chordoma to chemotherapy.

  3. Combined Therapy for Distant Metastasis of Sacral Chordoma

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    Özkal, Birol; Yaldız, Can; Temiz, Peyker; Temiz, Cüneyt

    2015-01-01

    Chordomas are known as rare primary malign tumours that have formed from primitive notochord remains. Sacral chordomas grow slowly but locally and aggressively. Chordomas are locally invasive and have low tendency to metastasis and have a poor prognosis in long-term follow-up. Metastasis may be seen in a rate of 5–40% of the chordomas. Metastasis of chordomas is common in liver, lung, lymph nodes, peritoneum, and brain. The treatment approaches, including surgery, have been discussed in the literature before. Susceptibility to radiotherapy and chemotherapy is controversial in these tumours. The success of surgical treatment affects survival directly. In this report, we will report a sacral chordoma case in which an intraperitoneal distant metastasis occurred and discuss the surgical approach. PMID:25649759

  4. Combined Therapy for Distant Metastasis of Sacral Chordoma

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    Birol Özkal

    2015-01-01

    Full Text Available Chordomas are known as rare primary malign tumours that have formed from primitive notochord remains. Sacral chordomas grow slowly but locally and aggressively. Chordomas are locally invasive and have low tendency to metastasis and have a poor prognosis in long-term follow-up. Metastasis may be seen in a rate of 5–40% of the chordomas. Metastasis of chordomas is common in liver, lung, lymph nodes, peritoneum, and brain. The treatment approaches, including surgery, have been discussed in the literature before. Susceptibility to radiotherapy and chemotherapy is controversial in these tumours. The success of surgical treatment affects survival directly. In this report, we will report a sacral chordoma case in which an intraperitoneal distant metastasis occurred and discuss the surgical approach.

  5. Sacrococcygeal chordoma, a rare cause of coccygodynia

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    Gavriilidis, Paschalis; Kyriakou, Dimitrios

    2013-01-01

    Patient: Male, 73 Final Diagnosis: Sacrococcygeal chordoma • Symptoms: Coccycodynia • sacral pain Medication: — Clinical Procedure: — Specialty: Surgery Objective: Rare disease Background: Sacrococcygeal chordomas are rare and difficult to diagnose tumors. Case Report: A 73-year-old man in the last 6 months felt a pain in and around the coccyx when sitting and especially when rising from the sitting position. He consulted his family physician and was referred by him to a general surgeon with the diagnosis of pilonidal disease. During the operation, the surgeon found an unusual mass and performed a wedge biopsy of the tumor. When chordoma was returned as the diagnosis, the patient was referred to our tertiary hospital for further treatment. The sacrococcygeal chordoma was excised en bloc with the coccyx and the fifth sacral vertebra by posterior approach. Conclusions: Because of minimal response to chemo- and radio-therapy, resections with wide margins at initial surgery is the most important factor influencing local recurrence. PMID:24376906

  6. Apoplexy in an intradural clival chordoma causing intraventricular bleed

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    Mohindra, Sandeep; Kapoor, Ankur; Kursa, Gopi Krishna; Mohindra, Satyawati; Saikia, Uma

    2016-01-01

    Background: A few cases depicting apoplexy in a chordoma have been reported. Rarely, this intratumoral bleed may spillover into intracerebral or intraventricular regions. Case Description: The authors report an intradural variety of clival chordoma presenting with apoplexy and spillover of blood into lateral ventricle. Clinical presentation, radiological scans, and relevant literature is also described. Conclusions: In a stable case of clival chordoma, intratumoral bleed or apoplexy may cause rapid neurological worsening and warranting urgent surgical intervention. PMID:26862453

  7. The chordoma arised from ilium: A rare case report

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    Yang, Yongkun; Niu, Xiaohui; Li, Lan; Ding, Yi

    2015-01-01

    Chordomas are malignant tumors that originate in embryonic notochordal remnants. The sacrum and skull are the most common sites; the mobile spine and other bones are extremely rare sites. We describe a 45-year-old man who presented with a lytic lesion in his left ilium. Imaging and pathology of a biopsy specimen suggested a malignant bone tumor; wide resection was accordingly performed. The morphology and immunohistochemistry of the operative specimen showed obvious characteristics of classic chordoma. To our knowledge, this is the first reported case of a chordoma originating in the ilium. Chordoma should therefore be considered in the differential diagnosis of lytic lesions in the ilium. PMID:26730357

  8. Metastatic Chordoma: A Diagnostic Challenge on Fine Needle Aspiration

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    Ghassan Tranesh

    2016-01-01

    Full Text Available Chordomas are primary low grade malignant tumors of bone that usually arise within both ends of axial skeleton. The Notochord is a midline, ectoderm-derived structure that defines the phylum of chordates. Chordomas may pose difficult diagnostic challenges when encountered in secondary locations, such as lungs or other parenchymatous organs. We report the cytologic findings of a metastatic chordoma sampled through CT-scan guided fine needle aspiration (FNA of lower lobe lung nodule in a 54-year-old man diagnosed with recurrent chordoma involving the lumber spine and paraspinal region.

  9. Sacrococcygeal chondroid chordoma: A case report with brief literature review

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    G K Sawke

    2014-01-01

    Full Text Available Chondroid-chordoma is an uncommon variant of chordoma that originates from the remnants of notochord. These tumors have axial distribution particularly at the upper and lower ends of the vertebral column. However, they may occur in unusual sites in ectopic notochordal tissue. This paper reports a rare occurrence of chondroid variant of chordoma at sacrococcygeal region. The characteristic presentation, radiological appearance, findings at surgery, pathology and treatment of this lesion are discussed due to its predilection for occurrence at the sacrococcygeal region and its more favorable prognosis compared with that of conventional chordoma.

  10. The chordoma arised from ilium: A rare case report

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    Yongkun Yang

    2015-12-01

    Full Text Available Chordomas are malignant tumors that originate in embryonic notochordal remnants. The sacrum and skull are the most common sites; the mobile spine and other bones are extremely rare sites. We describe a 45-year-old man who presented with a lytic lesion in his left ilium. Imaging and pathology of a biopsy specimen suggested a malignant bone tumor; wide resection was accordingly performed. The morphology and immunohistochemistry of the operative specimen showed obvious characteristics of classic chordoma. To our knowledge, this is the first reported case of a chordoma originating in the ilium. Chordoma should therefore be considered in the differential diagnosis of lytic lesions in the ilium.

  11. Clival chordoma manifesting as nasal bleeding. A case report

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    Kitai, Ryuhei; Yoshida, Kazuhiko; Kubota, Toshihiko; Sato, Kazufumi; Handa, Yuji; Kasahara, Kazuma [University of Fukui, Department of Neurosurgery, Fukui (Japan); Nakajima, Hirofumi [Tsuruga Municipal Hospital, Department of Neurosurgery, Fukui (Japan)

    2005-05-01

    Chordoma is a rare cartilaginous tumor, for which bleeding presentation is unusual. We report a case of rare hemorrhaged clival chordoma, which was diagnosed correctly by magnetic resonance imaging. A 32-year-old man presented with nasal bleeding. The tumor was totally removed via a trans-sphenoidal approach, from which the surgical specimen confirmed chordoma. Epistaxis seemed to be caused by the spreading of the intratumoral hemorrhage into the sphenoid sinus. This case demonstrates the importance of an exact differential diagnostic evaluation, including chordoma, by use of modern imaging techniques for nasal bleeding. (orig.)

  12. Metastatic Chordoma: A Diagnostic Challenge on Fine Needle Aspiration

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    Tranesh, Ghassan; Nassar, Aziza

    2016-01-01

    Chordomas are primary low grade malignant tumors of bone that usually arise within both ends of axial skeleton. The Notochord is a midline, ectoderm-derived structure that defines the phylum of chordates. Chordomas may pose difficult diagnostic challenges when encountered in secondary locations, such as lungs or other parenchymatous organs. We report the cytologic findings of a metastatic chordoma sampled through CT-scan guided fine needle aspiration (FNA) of lower lobe lung nodule in a 54-year-old man diagnosed with recurrent chordoma involving the lumber spine and paraspinal region. PMID:26881166

  13. Pudendal Neuralgia as the Initial Manifestation of Infiltrative Sacrococcygeal Chordoma

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    Sira Carrasco García de León

    2016-08-01

    Full Text Available Sacrococcygeal chordoma is a malignant tumour originating from remnants of the notochord. Chordomas are slow-growing tumours whose symptoms develop insidiously. We present the case of a 72-year-old woman with a 6-month history of genital pain radiating to the perianal area and exacerbating when she was in a sitting position. MRI and PET studies revealed a large mass in the sacrococcygeal region causing bone destruction and invasion of neurovascular structures. The immunohistochemical study of the surgical specimen determined it to be chordoma. This is the first published case of pudendal neuralgia as a form of presentation of sacrococcygeal chordoma.

  14. Pudendal Neuralgia as the Initial Manifestation of Infiltrative Sacrococcygeal Chordoma

    Science.gov (United States)

    Carrasco García de León, Sira; Flores Barragán, José Manuel; Villasanti Rivas, Natalia

    2016-01-01

    Sacrococcygeal chordoma is a malignant tumour originating from remnants of the notochord. Chordomas are slow-growing tumours whose symptoms develop insidiously. We present the case of a 72-year-old woman with a 6-month history of genital pain radiating to the perianal area and exacerbating when she was in a sitting position. MRI and PET studies revealed a large mass in the sacrococcygeal region causing bone destruction and invasion of neurovascular structures. The immunohistochemical study of the surgical specimen determined it to be chordoma. This is the first published case of pudendal neuralgia as a form of presentation of sacrococcygeal chordoma. PMID:27721781

  15. Chondroid chordoma of petrous temporal bone with extensive recurrence and pulmonary metastases

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    Agrawal Amit

    2008-01-01

    Full Text Available Chondroid chordoma is a variant of chordoma; it may rarely involve the petrous temporal bone and has a high propensity for recurrence. Chordoma rarely metastasizes but it is, nevertheless, associated with a poor outcome. We report a rare case of chondroid chordoma with extensive recurrence and pulmonary metastases.

  16. High-resolution Whole-Genome Analysis of Skull Base Chordomas Implicates FHIT Loss in Chordoma Pathogenesis

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    Roberto Jose Diaz

    2012-09-01

    Full Text Available Chordoma is a rare tumor arising in the sacrum, clivus, or vertebrae. It is often not completely resectable and shows a high incidence of recurrence and progression with shortened patient survival and impaired quality of life. Chemotherapeutic options are limited to investigational therapies at present. Therefore, adjuvant therapy for control of tumor recurrence and progression is of great interest, especially in skull base lesions where complete tumor resection is often not possible because of the proximity of cranial nerves. To understand the extent of genetic instability and associated chromosomal and gene losses or gains in skull base chordoma, we undertook whole-genome single-nucleotide polymorphism microarray analysis of flash frozen surgical chordoma specimens, 21 from the clivus and 1 from C1 to C2 vertebrae. We confirm the presence of a deletion at 9p involving CDKN2A, CDKN2B, and MTAP but at a much lower rate (22% than previously reported for sacral chordoma. At a similar frequency (21%, we found aneuploidy of chromosome 3. Tissue microarray immunohistochemistry demonstrated absent or reduced fragile histidine triad (FHIT protein expression in 98% of sacral chordomas and 67%of skull base chordomas. Our data suggest that chromosome 3 aneuploidy and epigenetic regulation of FHIT contribute to loss of the FHIT tumor suppressor in chordoma. The finding that FHIT is lost in a majority of chordomas provides new insight into chordoma pathogenesis and points to a potential new therapeutic target for this challenging neoplasm.

  17. Isolated Liver Metastasis of Sacrococcygeal Chordoma: Case Report and Review of the Literature

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    Akyol, Murat; Varol, Umut; Yildiz, Ibrahim; Bayoglu, Ibrahim Vedat; Yildiz, Yasar; Demir, Lutfiye; Dirican, Ahmet; Can, Alper; Cokmert, Suna; Oztop, Mine Tunakan; Alacacioglu, Ahmet; Kucukzeybek, Yuksel; Tarhan, Mustafa Oktay

    2014-01-01

    Chordomas are rare neoplasms arising from notochordal remnants and may develop anywhere in the body while the most common anatomic site is the sacrococcygeal area. The most effective treatment of chordoma is surgery. Chordomas rarely metastasize to lung, bone, soft tissue, liver, lymph nodes, and skin. However, there is currently no standard systemic treatment for advanced stage chordoma. Here, we reported a rare presentation of chordoma patient with liver only metastases and poor prognosis. PMID:25018886

  18. Isolated Liver Metastasis of Sacrococcygeal Chordoma: Case Report and Review of the Literature

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    Murat Akyol

    2014-01-01

    Full Text Available Chordomas are rare neoplasms arising from notochordal remnants and may develop anywhere in the body while the most common anatomic site is the sacrococcygeal area. The most effective treatment of chordoma is surgery. Chordomas rarely metastasize to lung, bone, soft tissue, liver, lymph nodes, and skin. However, there is currently no standard systemic treatment for advanced stage chordoma. Here, we reported a rare presentation of chordoma patient with liver only metastases and poor prognosis.

  19. Spinal cerebrospinal fluid seeding of a clival chordoma; A case report

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    Baek, Seung Hwan; Yu, In Kyu; Kim, Seong Min; Park, Ki Seok; Son, Hyun Jin [Eulji University Hospital, Daejeon (Korea, Republic of)

    2015-07-15

    Chordomas originate from remnants of the embryonic notochord and account for < 2% of all malignant bone tumors. Chordomas have a high rate of local recurrence. However, spinal cerebrospinal fluid (CSF) seeding of a chordoma is extremely rare. Here, we present a very rare case of clival chordoma with spinal seeding. Radiologists should consider spinal CSF seeding of a clival chordoma, particularly when accompanied by signs of dural perforation or caudal extension.

  20. Novel targeted therapies in chordoma: an update

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    Di Maio S

    2015-05-01

    Full Text Available Salvatore Di Maio,1 Stephen Yip,2 Gmaan A Al Zhrani,3,4 Fahad E Alotaibi,3,4 Abdulrahman Al Turki,3,4 Esther Kong,2 Robert C Rostomily5 1Division of Neurosurgery, Jewish General Hospital, McGill University, Montreal, QC, 2Department of Pathology and Laboratory Medicine, Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada; 3National Neuroscience Institute, Department of Neurosurgery, King Fahad Medical City, Riyadh, Saudi Arabia; 4Department of Neurology and Neurosurgery, The Montreal Neurological Institute and Hospital, McGill University Health Centre, Montreal, QC, Canada; 5Department of Neurological Surgery, University of Washington, University of Washington Medical Center, Seattle, WA, USA Abstract: Chordomas are rare, locally aggressive skull base neoplasms known for local recurrence and not-infrequent treatment failure. Current evidence supports the role of maximal safe surgical resection. In addition to open skull-base approaches, the endoscopic endonasal approach to clival chordomas has been reported with favorable albeit early results. Adjuvant radiation is prescribed following complete resection, alternatively for gross residual disease or at the time of recurrence. The modalities of adjuvant radiation therapy reported vary widely and include proton-beam, carbon-ion, fractionated photon radiotherapy, and photon and gamma-knife radiosurgery. As of now, no direct comparison is available, and high-level evidence demonstrating superiority of one modality over another is lacking. While systemic therapies have yet to form part of any first-line therapy for chordomas, a number of targeted agents have been evaluated to date that inhibit specific molecules and their respective pathways known to be implicated in chordomas. These include EGFR (erlotinib, gefitinib, lapatinib, PDGFR (imatinib, mTOR (rapamycin, and VEGF (bevacizumab. This article provides an update of the current multimodality treatment of cranial base

  1. Molecular characterization of chordoma xenografts generated from a novel primary chordoma cell source and two chordoma cell lines

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    Karikari, Isaac O.; Gilchrist, Christopher L.; Jing, Liufang; Alcorta, David A.; Chen, Jun; Richardson, William J.; Gabr, Mostafa A.; Bell, Richard D.; Kelley, Michael J.; Bagley, Carlos A.; Setton, Lori A.

    2014-01-01

    Object Chordoma cells can generate solid-like tumors in xenograft models that express some molecular characteristics of the parent tumor, including positivity for brachyury and cytokeratins. However, there is a dearth of molecular markers that relate to chordoma tumor growth, as well as the cell lines needed to advance treatment. The objective in this study was to isolate a novel primary chordoma cell source and analyze the characteristics of tumor growth in a mouse xenograft model for comparison with the established U-CH1 and U-CH2b cell lines. Methods Primary cells from a sacral chordoma, called “DVC-4,” were cultured alongside U-CH1 and U-CH2b cells for more than 20 passages and characterized for expression of CD24 and brachyury. While brachyury is believed essential for driving tumor formation, CD24 is associated with healthy nucleus pulposus cells. Each cell type was subcutaneously implanted in NOD/SCID/IL2Rγnull mice. The percentage of solid tumors formed, time to maximum tumor size, and immunostaining scores for CD24 and brachyury (intensity scores of 0–3, heterogeneity scores of 0–1) were reported and evaluated to test differences across groups. Results The DVC-4 cells retained chordoma-like morphology in culture and exhibited CD24 and brachyury expression profiles in vitro that were similar to those for U-CH1 and U-CH2b. Both U-CH1 and DVC-4 cells grew tumors at rates that were faster than those for U-CH2b cells. Gross tumor developed at nearly every site (95%) injected with U-CH1 and at most sites (75%) injected with DVC-4. In contrast, U-CH2b cells produced grossly visible tumors in less than 50% of injected sites. Brachyury staining was similar among tumors derived from all 3 cell types and was intensely positive (scores of 2–3) in a majority of tissue sections. In contrast, differences in the pattern and intensity of staining for CD24 were noted among the 3 types of cell-derived tumors (p < 0.05, chi-square test), with evidence of intense

  2. Case report 544: Metastatic chordoma to humeri (originating in sacrum)

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    Resnik, C.S.; Young, J.W.R. (Maryland Univ., Baltimore, MD (USA). Dept. of Diagnostic Radiology); Levine, A.M. (Maryland Univ., Baltimore, MD (USA). Div. of Orthopaedic Surgery); Aisner, S.C. (Maryland Univ., Baltimore (USA). Dept. of Pathology)

    1989-07-01

    An elderly woman develops metastases to both humeri from a sacral chordoma leading to pathological fractures. This represents only the second reported case of such metastasis to a long bone from this primary site. The incidence of skeletal metastasis from chordoma was considered and the literature was reviewed. (orig.).

  3. Craniospinal dissemination of clival chondroid chordoma

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    Shinde S

    2005-01-01

    Full Text Available Chondroid chordoma commonly presents as clival osseous and extradural mass. A 15-year-old boy presented with progressive visual deficit, headaches and diplopia since three years. Computed tomography (CT scan showed a skull base tumour, but was wrongly reported at the time as chronic sphenoidal sinusitis and nasal polyps. In the past three months, he developed dysphagia, urinary retention and constipation. Terminally, he had weakness of all limbs. Fundoscopy showed optic atrophy. Temporal and spatial variation in symptoms led to a clinical diagnosis of multiple sclerosis with optic neuritis. Partial brain autopsy revealed small gelatinous tumour nodules in the subarachnoid space of middle cranial fossa encasing base of brain like arachnoiditis. Tumour deposits extended down into the spinal cord along the subarachnoid space as far as vision allowed. Histopathology and immunohistochemistry confirmed a diagnosis of chondroid chordoma. Awareness of this rare mode of dissemination will avoid misdiagnosis and delay in treatment

  4. Sacrococcygeal chordoma: MR imaging in 30 patients

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    Sung, Mi Sook; Chung, Myung Hee [Catholic University of Korea, Holy Family Hospital, Department of Radiology, Pucheon (Korea); Lee, Gyung Kyu; Kang, Heung Sik [Seoul National University Hospital, Department of Radiology, Seoul (Korea); Kwon, Soon Tae [Chungnam University Hospital, Department of Radiology, Taejun (Korea); Park, Jin Gyoon [Chunnam University Hospital, Department of Radiology, Kwangju (Korea); Suh, Jin Suk [Yonsei University, Severans Hospital, Department of Radiology, Seoul (Korea); Cho, Gil Ho [Yeungnam University Hospital, Department of Radiology, Taegu (Korea); Lee, Sung Moon [Kaemyung University Hospital, Department of Radiology, Taeku (Korea); Resnick, Donald [VA Medical Center, Department of Radiology, San Diego, CA (United States)

    2005-02-01

    To evaluate MR imaging of sacrococcygeal chordoma. Thirty patients (age range 22-80 years) underwent MR imaging for the diagnosis and preoperative evaluation of sacrococcygeal chordomas. Eight patients had follow-up MR examination after treatment. The MR images were performed with T1- and T2-weighted imaging, and gadolinium (Gd)-enhanced imaging. The MR images were analyzed for the signal intensity, enhancing pattern, tumor size, growth pattern of the soft tissue component, and tumor extension. T1-weighted images showed low signal masses with foci of high signal intensity in 73% of cases. Tumors enhanced in a variety of patterns after the administration of Gd. Soft tissue masses extending anteriorly were seen in all cases with posterior extension in 77% of cases. The posterior masses involved the surrounding muscles and extended toward the greater sciatic notch, appearing with pseudopodia (87%). Sacroiliac joints were involved in 23% of cases. Four lesions showed intraspinal extension and involvement of the posterior spinal muscles above the level of bony involvement. In 6 patients recurrent tumors were found at or around the surgical margin of the tumor 6 months to 5 years after resection of the sacral tumor. In two of the patients, nodular metastases to the pelvic bones and femur were found 1-4 years after initial examination. In conclusion, MR imaging is useful in the diagnosis and preoperative assessment of sacrococcygeal chordoma. Characteristic findings included sacral mass with heterogeneously high signal intensity with crisscrossing septa on long-repetition-time imaging, well-encapsulated pseudopodia-like or lobulated appearance, and gluteal muscle infiltration. Follow-up MR imaging is helpful to assess for recurrent or metastatic lesions of chordomas. (orig.)

  5. Update on the Cytogenetics and Molecular Genetics of Chordoma

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    Larizza Lidia

    2005-02-01

    Full Text Available Abstract Chordoma is a rare mesenchymal tumour of complex biology for which only histologic and immunohistochemical criteria have been defined, but no biomarkers predicting the clinical outcome and response to treatment have yet been recognised. We herein review the interdisciplinary information achieved by epidemiologists, neurosurgeons and basic scientists on chordoma, usually a sporadic tumour, which also includes a small fraction of familial cases. Main focus is on the current knowledge of the genetic alterations which might pinpoint candidate genes and molecular mechanisms shared by sporadic and familiar chordomas. Due to the scarcity of the investigated tumour specimens and the multiple chromosome abnormalities found in tumours with aberrant karyotypes, conventional cytogenetics and Fluorescence In Situ Hybridization failed to detect recurrent chordoma-specific chromosomal rearrangements. Genome-wide approaches such as Comparative Genomic Hybridization (CGH are yet at an initial stage of application and should be implemented using BAC arrays either genome-wide or targeting selected genomic regions, disclosed by Loss of Heterozygosity (LOH studies. An LOH region was shown by a systematic study on a consistent number of chordomas to encompass 1p36, a genomic interval where a candidate gene was suggested to reside. Despite the rarity of multiplex families with chordoma impaired linkage studies, a chordoma locus could be mapped to chromosome 7q33 by positive lod score in three independent families. The role in chordomagenesis of the Tuberous Sclerosis Complex (TSC genes has been proved, but the extent of involvement of TSC1 and TSC2 oncosuppressors in chordoma remains to be assessed. In spite of the scarce knowledge on the genetics and molecular biology of chordoma, recent initiation of clinical trials using molecular-targeted therapy, should validate new molecular targets and predict the efficacy of a given therapy. Comparative genetic and

  6. Clival chordoma; CT and MR findings

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    Lee, Si Kyung; Han, Chun Hwan; Lee, Moon Ok; Kim, Mie Young; Yi, Jeong Geun; Lee, Joo Hyuk [Kang Nam General Hospital Pubic Corporation, Seoul (Korea, Republic of); Bae, Sang Hoon [Hallym University College of Medicine, Seoul (Korea, Republic of)

    1993-07-15

    A retrospectively analysis of CT and MR findings was performed in five patients with histologically proved chordoma including one with chondroid chordoma. All tumors were mostly isodense to gray matter on unenhanced CT, and the single intensities were iso or low and high on T1 and T2-weighted MR images, respectively. The tumors had an increase in their density on enhanced CT and MR in four patients, but a chondroid chordoma was poorly enhanced after injection of contrast medicum on CT. Four tumors contained calcifications in CT images and two lesionsshowed hemorrhage in MR images. Cavenous sinus was involved in all patients, and brain stem and basilar artery were compressed by the tumors in three cases. Pituitary gland was only displaced upward in three patients. Clivus was destroyed in all cases, and sella turcica and sphenoid bone were involved in three patients. CT is better than MR in demonstrating calcifications and bone destruction. In defining the extension of tumor, MR appears to be superior to CT in evaluation of the relationship between the tumor and the surrounding structures.

  7. Atlantoaxial Chordoma in Two Patients with Occipital Neuralgia and Cervicalgia

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    Kim, Won Seop; Park, Jong Taek; Lee, Young Bok; Park, Woo Young

    2014-01-01

    Chordoma arises from cellular remnants of the notochord. It is the most common primary malignancy of the spine in adults. Approximately 50% of chordomas arise from the sacrococcygeal area with other areas of the spine giving rise to another 15% of chordomas. Following complete resection, patients can expect a 5-year survival rate of 85%. Chordoma has a recurrence rate of 40%, which leads to a less favorable prognosis. Here, we report two cases of chordoma presenting with occipital neuralgia and cervicalgia. The first patient presented with a C1–C2 chordoma. He rejected surgical intervention and ultimately died of respiratory failure. The second patient had an atlantoaxial chordoma and underwent surgery because of continued occipital neuralgia and cervicalgia despite nerve block. This patient has remained symptom-free since his operation. The presented cases show that the patients’ willingness to participate in treatment can lead to appropriate and aggressive management of cancer pain, resulting in better outcomes in cancer treatment. PMID:26064862

  8. Two Cases of Dedifferentiated Chordoma in the Sacrum

    Science.gov (United States)

    Kim, Seon Chun; Cho, Wonik; Chang, Ung-Kyu

    2015-01-01

    Dedifferentiated chordoma (DC) is defined as a chordoma containing sarcoma components. DC is distinguished from conventional chordoma by the rapidity of tumor growth and the potential for distant metastasis. We report two cases of DC, which are developed in the sacrum. We reviewed the medical records and imaging studies of 2 patients diagnosed with DC and the literature published. In the first case, percutaneous biopsy revealed that it was conventional chordoma in the sacrum. Patient underwent radiation therapy (RT). Six years after the RT, the tumor recurred. Surgical removal was performed and the recurrent tumor was diagnosed as DC in histopathologic examination. In the second case, a patient underwent gross total resection of sacral tumor, which was diagnosed with conventional chordoma. Aggravated tumor was detected after 4 months, and patient underwent reoperation. The second operation revealed the transformation of the tumor into DC. The survival time of the patients after the diagnosis was 10 and 31 months. Dedifferentiated chordoma is a rare and highly aggressive tumor. De novo type exists, but it usually transformed from recurrent chordoma after surgical resection or radiation. PMID:26512292

  9. Combined Transnasal and Transcranial Removal of a Giant Clival Chordoma

    Science.gov (United States)

    Koechlin, Nicolas O.; Simmen, Daniel; Briner, Hans Rudolf; Reisch, Robert

    2014-01-01

    Clival chordomas confront the surgeon with the task of resecting an aggressively invasive and destructive tumor in a critical surrounding. For many, mainly smaller, chordomas, the transnasal transclival approach is a feasible and safe surgical access. Larger tumors and especially those with extensive intradural, retrochiasmal, and/or deep cervical expansion are mostly approached by open craniotomy. Staged procedures are also commonly used in the case of expansive tumor growth. We present the first case of a single-session combined transnasal and transcranial approach to radically resect a large clival chordoma. PMID:25083400

  10. Chordoma of skull base presenting as nasopharyngeal mass

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    Sant Prakash Kataria

    2013-01-01

    Full Text Available While the nasopharynx is most commonly regarded by the otolaryngologist as a primary site of neoplastic involvement, it is also an avenue of spread of base-of-the-skull tumors presenting as bulging nasopharyngeal masses. Chordoma is a relatively rare tumor of the skull base and sacrum thought to originate from embryonic remnants of the notochord. Chordomas arising from the skull base/clivus are typically locally aggressive with lytic bone destruction. The optimal treatment may be photon/proton radiotherapy alone or combined with a gross total resection, when feasible. We report a case of intracranial chordoma presenting as nasopharyngeal mass.

  11. CT and MRI appearances of a thoracic chordoma

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    Murphy, J.M.; Wallis, F.; Toner, M.; Wilson, G.F. [Department of Diagnostic Imaging and Pathology, St James`s Hospital, Dublin (Ireland); Toland, J. [Department of Neuroradiology, Beaumont Hospital, Dublin (Ireland)

    1998-12-01

    A case of a chordoma in the thoracic spine is presented. This is a very rare tumour in this location and should be considered in the differential diagnosis of any posterior mediastinal mass. The appearances on CT and MRI were similar to chordomas described in other locations. On T2-weighted images septae of low signal intensity radiated throughout the large high-signal mass. This feature may be of use in differentiating chordomas from other posterior mediastinal masses. (orig.) With 5 figs., 13 refs.

  12. An intradural skull base chordoma presenting with acute intratumoral hemorrhage

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    Uda Takehiro

    2006-01-01

    Full Text Available We present a rare case of skull base chordoma of extraosseous intradural type that presented as acute intratumoral hemorrhage. Surgical removal of the tumor was accomplished using a skull base approach.

  13. Clival chordoma with an atypical presentation: a case report

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    Alshammari Jaber

    2012-11-01

    Full Text Available Abstract Introduction Clival chordomas present with headache, commonly VI cranial nerve palsy or sometimes with lower cranial nerve involvement. Very rarely, they present with cerebrospinal fluid rhinorrhoea due to an underlying chordoma-induced skull base erosion. Case presentation A 60-year old Caucasian woman presented with meningitis secondary to cerebrospinal fluid rhinorrhoea. At first, radiological imaging did not reveal a tumoral condition, though intraoperative exploration and tissue histology revealed a chordoma which eroded her clivus and had a transdural extension. Conclusion Patients who present with meningitis and cerebrospinal fluid rhinorrhoea could have an underlying erosive lesion which can sometimes be missed on initial radiological examination. Surgical exploration allows collecting suspicious tissue for histological diagnosis which is important for the actual treatment. A revision endoscopic excision of a clival chordoma is challenging and has been highlighted in this report.

  14. Chordoma characterization of significant changes of the DNA methylation pattern.

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    Beate Rinner

    Full Text Available Chordomas are rare mesenchymal tumors occurring exclusively in the midline from clivus to sacrum. Early tumor detection is extremely important as these tumors are resistant to chemotherapy and irradiation. Despite continuous research efforts surgical excision remains the main treatment option. Because of the often challenging anatomic location early detection is important to enable complete tumor resection and to reduce the high incidence of local recurrences. The aim of this study was to explore whether DNA methylation, a well known epigenetic marker, may play a role in chordoma development and if hypermethylation of specific CpG islands may serve as potential biomarkers correlated with SNP analyses in chordoma. The study was performed on tumor samples from ten chordoma patients. We found significant genomic instability by Affymetrix 6.0. It was interesting to see that all chordomas showed a loss of 3q26.32 (PIK 3CA and 3q27.3 (BCL6 thus underlining the potential importance of the PI3K pathway in chordoma development. By using the AITCpG360 methylation assay we elucidated 20 genes which were hyper/hypomethylated compared to normal blood. The most promising candidates were nine hyper/hypomethylated genes C3, XIST, TACSTD2, FMR1, HIC1, RARB, DLEC1, KL, and RASSF1. In summary, we have shown that chordomas are characterized by a significant genomic instability and furthermore we demonstrated a characteristic DNA methylation pattern. These findings add new insights into chordoma development, diagnosis and potential new treatment options.

  15. Primary sacrococcygeal chordoma with unusual skeletal muscle metastasis

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    Lisa Vu, MD

    2014-01-01

    Full Text Available Chordomas are rare neoplasms that do not often metastasize. Of the small percent that do metastasize, they very infrequently involve skeletal muscle. Only a few cases of skeletal muscle metastases have been reported in the literature. We report an unusual case of a patient with a primary sacrococcygeal chordoma who experienced a long period of remission but who subsequently developed recurrence and multiple metastatic lesions to skeletal muscles including the deltoid, triceps, and pectineus.

  16. Primary sacrococcygeal chordoma with unusual skeletal muscle metastasis

    Science.gov (United States)

    Vu, Lisa; Haygood, Tamara Miner

    2015-01-01

    Chordomas are rare neoplasms that do not often metastasize. Of the small percent that do metastasize, they very infrequently involve skeletal muscle. Only a few cases of skeletal muscle metastases have been reported in the literature. We report an unusual case of a patient with a primary sacrococcygeal chordoma who experienced a long period of remission but who subsequently developed recurrence and multiple metastatic lesions to skeletal muscles including the deltoid, triceps, and pectineus. PMID:27190554

  17. Genomic aberrations frequently alter chromatin regulatory genes in chordoma.

    Science.gov (United States)

    Wang, Lu; Zehir, Ahmet; Nafa, Khedoudja; Zhou, Nengyi; Berger, Michael F; Casanova, Jacklyn; Sadowska, Justyna; Lu, Chao; Allis, C David; Gounder, Mrinal; Chandhanayingyong, Chandhanarat; Ladanyi, Marc; Boland, Patrick J; Hameed, Meera

    2016-07-01

    Chordoma is a rare primary bone neoplasm that is resistant to standard chemotherapies. Despite aggressive surgical management, local recurrence and metastasis is not uncommon. To identify the specific genetic aberrations that play key roles in chordoma pathogenesis, we utilized a genome-wide high-resolution SNP-array and next generation sequencing (NGS)-based molecular profiling platform to study 24 patient samples with typical histopathologic features of chordoma. Matching normal tissues were available for 16 samples. SNP-array analysis revealed nonrandom copy number losses across the genome, frequently involving 3, 9p, 1p, 14, 10, and 13. In contrast, copy number gain is uncommon in chordomas. Two minimum deleted regions were observed on 3p within a ∼8 Mb segment at 3p21.1-p21.31, which overlaps SETD2, BAP1 and PBRM1. The minimum deleted region on 9p was mapped to CDKN2A locus at 9p21.3, and homozygous deletion of CDKN2A was detected in 5/22 chordomas (∼23%). NGS-based molecular profiling demonstrated an extremely low level of mutation rate in chordomas, with an average of 0.5 mutations per sample for the 16 cases with matched normal. When the mutated genes were grouped based on molecular functions, many of the mutation events (∼40%) were found in chromatin regulatory genes. The combined copy number and mutation profiling revealed that SETD2 is the single gene affected most frequently in chordomas, either by deletion or by mutations. Our study demonstrated that chordoma belongs to the C-class (copy number changes) tumors whose oncogenic signature is non-random multiple copy number losses across the genome and genomic aberrations frequently alter chromatin regulatory genes. © 2016 Wiley Periodicals, Inc.

  18. Infrasellar craniopharyngioma mimicking a clival chordoma : a case report.

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    Kachhara R

    2002-04-01

    Full Text Available An unusual case of entirely infrasellar craniopharyngioma mimicking a clival chordoma is described. Only 22 cases of craniopharyngioma with nasopharyngeal extension have been reported in the literature. Of the reported cases, most were primarily intracranial with secondary downward extension; only two were thought to originate from an infrasellar location. The present case is another example of an entirely infrasellar craniopharyngioma, with extensive clival destruction, mimicking a clival chordoma. Relevant literature on the subject is reviewed.

  19. Genomic aberrations frequently alter chromatin regulatory genes in chordoma.

    Science.gov (United States)

    Wang, Lu; Zehir, Ahmet; Nafa, Khedoudja; Zhou, Nengyi; Berger, Michael F; Casanova, Jacklyn; Sadowska, Justyna; Lu, Chao; Allis, C David; Gounder, Mrinal; Chandhanayingyong, Chandhanarat; Ladanyi, Marc; Boland, Patrick J; Hameed, Meera

    2016-07-01

    Chordoma is a rare primary bone neoplasm that is resistant to standard chemotherapies. Despite aggressive surgical management, local recurrence and metastasis is not uncommon. To identify the specific genetic aberrations that play key roles in chordoma pathogenesis, we utilized a genome-wide high-resolution SNP-array and next generation sequencing (NGS)-based molecular profiling platform to study 24 patient samples with typical histopathologic features of chordoma. Matching normal tissues were available for 16 samples. SNP-array analysis revealed nonrandom copy number losses across the genome, frequently involving 3, 9p, 1p, 14, 10, and 13. In contrast, copy number gain is uncommon in chordomas. Two minimum deleted regions were observed on 3p within a ∼8 Mb segment at 3p21.1-p21.31, which overlaps SETD2, BAP1 and PBRM1. The minimum deleted region on 9p was mapped to CDKN2A locus at 9p21.3, and homozygous deletion of CDKN2A was detected in 5/22 chordomas (∼23%). NGS-based molecular profiling demonstrated an extremely low level of mutation rate in chordomas, with an average of 0.5 mutations per sample for the 16 cases with matched normal. When the mutated genes were grouped based on molecular functions, many of the mutation events (∼40%) were found in chromatin regulatory genes. The combined copy number and mutation profiling revealed that SETD2 is the single gene affected most frequently in chordomas, either by deletion or by mutations. Our study demonstrated that chordoma belongs to the C-class (copy number changes) tumors whose oncogenic signature is non-random multiple copy number losses across the genome and genomic aberrations frequently alter chromatin regulatory genes. © 2016 Wiley Periodicals, Inc. PMID:27072194

  20. Aspiration cytology of metastatic chordoma. A case report.

    Science.gov (United States)

    Elliott, E C; McKinney, S; Banks, H; Fulks, R M

    1983-01-01

    A patient with previously diagnosed sacrococcygeal chordoma presented with multiple skin nodules. Cytologic examination of an aspirate from one of these nodules showed syncytial clusters of hyperchromatic cells surrounded by extracellular mucin. The characteristic physaliphorous cells, although present in a biopsy of the primary sacrococcygeal tumor, were not observed in the aspirate or on histopathologic examination of three excised skin metastases. That chordoma metastases may lack physaliphorous cells should be recognized.

  1. Genomic Aberrations Frequently Alter Chromatin Regulatory Genes in Chordoma

    Science.gov (United States)

    Wang, Lu; Zehir, Ahmet; Nafa, Khedoudja; Zhou, Nengyi; Berger, Michael F.; Casanova, Jacklyn; Sadowska, Justyna; Lu, Chao; Allis, C. David; Gounder, Mrinal; Chandhanayingyong, Chandhanarat; Ladanyi, Marc; Boland, Patrick J; Hameed, Meera

    2016-01-01

    Chordoma is a rare primary bone neoplasm that is resistant to standard chemotherapies. Despite aggressive surgical management, local recurrence and metastasis is not uncommon. To identify the specific genetic aberrations that play key roles in chordoma pathogenesis, we utilized a genome-wide high-resolution SNP-array and next generation sequencing (NGS)-based molecular profiling platform to study 24 patient samples with typical histopathologic features of chordoma. Matching normal tissues were available for 16 samples. SNP-array analysis revealed nonrandom copy number losses across the genome, frequently involving 3, 9p, 1p, 14, 10, and 13. In contrast, copy number gain is uncommon in chordomas. Two minimum deleted regions were observed on 3p within a ~8 Mb segment at 3p21.1–p21.31, which overlaps SETD2, BAP1 and PBRM1. The minimum deleted region on 9p was mapped to CDKN2A locus at 9p21.3, and homozygous deletion of CDKN2A was detected in 5/22 chordomas (~23%). NGS-based molecular profiling demonstrated an extremely low level of mutation rate in chordomas, with an average of 0.5 mutations per sample for the 16 cases with matched normal. When the mutated genes were grouped based on molecular functions, many of the mutation events (~40%) were found in chromatin regulatory genes. The combined copy number and mutation profiling revealed that SETD2 is the single gene affected most frequently in chordomas, either by deletion or by mutations. Our study demonstrated that chordoma belongs to the C-class (copy number changes) tumors whose oncogenic signature is non-random multiple copy number losses across the genome and genomic aberrations frequently alter chromatin regulatory genes. PMID:27072194

  2. Chordoma Cutis – A Diagnosis not to be Missed

    Science.gov (United States)

    Mishra, Kiran

    2014-01-01

    Chordomas are rare midline tumors of the bone usually arising from sacrum, skull bones and spine, close to neuraxis. However an extensive involvement of the soft tissues can simulate a soft tissue subcutaneous tumour of the gluteal region –a presentation called chordoma cutis. Our patient presented with a gluteal mass and a trucut biopsy was done suspecting a soft tissue tumour. The hematoxylin and eosin stained section of the biopsy closely simulated a lipomatous tumour. However, on closer inspection the clear cells were found to have very fine vacuolations.The usual myxoid background and characteristic physaliferous cells seen in chordomas were not seen. Still a differential of chordoma was entertained in view of the site and age of the patient. Immunohistochemistry for cytokeratin and S-100 was performed and both were unambiguously positive. On literature search, we came across a soft tissue tumour called parachordoma which mimics chordoma both morphologically and immunohistochemically and has also been reported in the gluteal region. An MRI was performed which showed the tumour to be arising from the sacrum and secondarily involving the gluteal soft tissues. This case highlights the importance of considering chordoma in the differential diagnosis of gluteal masses with clear cell morphology even in the absence of physaliferous cells and myxoid background before signing them out as lipomatous tumours. PMID:25120995

  3. Carbon-11-methionine positron emission tomography imaging of chordoma

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    Zhang, Hong [Department of Medical Imaging, National Institute of Radiological Sciences, Chiba (Japan); Department of Medical Imaging, Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, 4-9-1, Anagawa, Inage-ku, 263-8555, Chiba (Japan); Yoshikawa, Kyosan; Tamura, Katsumi; Sagou, Kenji; Kandatsu, Susumu [Clinical Diagnosis Section, National Institute of Radiological Sciences, Chiba (Japan); Tian, Mei; Suhara, Tetsuya; Suzuki, Kazutoshi; Tanada, Shuji [Department of Medical Imaging, National Institute of Radiological Sciences, Chiba (Japan); Tsujii, Hirohiko [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba (Japan)

    2004-09-01

    Chordoma is a rare malignant bone tumor that arises from notochord remnants. This is the first trial to investigate the utility of {sup 11}C-methionine (MET) positron emission tomography (PET) in the imaging of chordoma before and after carbon-ion radiotherapy (CIRT). Fifteen patients with chordoma were investigated with MET-PET before and after CIRT and the findings analyzed visually and quantitatively. Tumor MET uptake was evaluated by tumor-to-nontumor ratio (T/N ratio). In 12 (80%) patients chordoma was clearly visible in the baseline MET-PET study with a mean T/N ratio of 3.3{+-}1.7. The MET uptake decreased significantly to 2.3{+-}1.4 after CIRT (P<0.05). A significant reduction in tumor MET uptake of 24% was observed after CIRT. Fourteen (93%) patients showed no local recurrence after CIRT with a median follow-up time of 20 months. This study has demonstrated that MET-PET is feasible for imaging of chordoma. MET-PET could provide important tumor metabolic information for the therapeutic monitoring of chordoma after CIRT. (orig.)

  4. Humeral metastasis from a sacrococcygeal chordoma: a case report

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    Sepidbakht Sepideh

    2011-08-01

    Full Text Available Abstract Introduction Chordomas are rare tumors of the skeletal system that arise from an intra-osseous benign precursor of notochordal cells. They are mainly locally aggressive. However, metastases to other sites, including the humeri, resulting in pathological fractures have been reported. We report the case of a patient with a metastatic chordoma that produced a pathologic fracture of the humerus. Case presentation We report the case of a 60-year-old Iranian woman who presented with a fracture of her right humerus following a minor trauma. She had a history of a sacrococcygeal chordoma. Histological and immunohistochemical studies of the fracture site suggested the diagnosis of a chordoma. Conclusions Chordoma is a rare tumor and rarely metastasizes, but it should be considered in the differential diagnosis of epithelioid bone tumors. The only current effective treatment for this type of tumor is carbon ion therapy. There is currently no effective medical therapy available for advanced chordoma, and this type of tumor is not very responsive to radiotherapy.

  5. Petrous apex chordoma - a case report; Cordoma de apice petroso - relato de um caso

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    Silveira, Claudio Regis S. [Clinica Boghos Boyadjian, Fortaleza, CE (Brazil); Barreto, Cristina Marques; Rossi, Luis Antonio [Hospital do Servidor Publico Estadual de Sao Paulo, SP (Brazil). Servico de Radiologia; Michiloski, Custodio; Rotta, Jose Marcus [Hospital do Servidor Publico Estadual de Sao Paulo, SP (Brazil). Servico de Neurocirurgia; Almeida, Serguey Malaquias de

    2001-02-01

    Chordomas are rare neoplasms arising from notochordal remnants that persist along the axial skeleton. Intracranial chordomas occur more frequently in the midline. We describe a typical case of an off-midline chordoma arising from the petrous apex, and discuss the embryogenic factors which determine that location, as well as the symptoms, imaging findings, surgical treatment and evolution. (author)

  6. Intracranial chordoma presenting as acute hemorrhage in a child: Case report and literature review

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    Kenneth A Moore

    2015-01-01

    Conclusion: There are few previous reports of petroclival chordomas causing acute intracranial hemorrhage. To the authors′ knowledge, this is the first case of a petroclival chordoma presenting as acute intracranial hemorrhage in a pediatric patient. Although uncommon, it is important to consider chordoma when evaluating a patient of any age presenting with a hemorrhagic lesion of the clivus.

  7. Research progress of biological behavior of spinal chordoma in motion segment

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    Yan-da LIN

    2016-04-01

    Full Text Available Chordomas are low-grade malignant bone tumors that arise from remnants of notochord or aberrant chordal tissue, and have a predilection for the axial skeleton. They are easily recurrent, locally invasive, and have a poor prognosis. Chordomas are also not sensitive to chemotherapy or radiotherapy. Due to the special anatomy of spine, chordomas of mobile spine have their own characteristics. Therefore, this paper reviewed relevant literatures of recent 10 years and summarized the characteristics of biological behavior of mobile spine chordoma, so as to provide new ideas for treating chordoma. DOI: 10.3969/j.issn.1672-6731.2016.04.004

  8. Paraspinal chordoma mimicking a neurofibroma: a rare but important radiological pitfall

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    Kotnis, Nikhil; Goepel, John [Royal Hallamshire Hospital, Sheffield Teaching Hospitals, Sheffield (United Kingdom)

    2013-03-15

    We present an unusual case of a chordoma presenting as an extradural spinal tumour with extension through an expanded intervertebral foramen to form a large paraspinal mass. The magnetic resonance imaging appearances closely mimicked a neurofibroma; however, pre-operative biopsy confirmed the diagnosis of chordoma. This is, to our knowledge, the tenth reported case of chordoma presenting as a mass expanding the intervertebral foramen. Thus, while it is a rare form of chordoma, it can lead to a recognised radiological pitfall. Making the distinction from neurofibroma before surgery is essential, as radical dissection of chordoma is required to reduce the risk of local recurrence. (orig.)

  9. Primary chondroid chordoma arising from the petrous temporal bone: a case report

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    Lee, Young Uk; Youn, Eun Kyung [Koryo General Hospital, Seoul (Korea, Republic of)

    1991-01-15

    Chordomas are uncommon tumors which arise from remnants of the primitive notochord. They are situated chiefly in the anterior spinal axis with a predilection for the sacrococcygeal region and the basiocciput. About 50% of chordomas are sacrococcygeal, 35% are intracranial, and 15% arise from a vertebral body. As a histologic variant of chordoma, /chondroid chordoma' was first described by Heffelfinger et al. We present a rare case of primary chondroid chordoma arising from the petrous temporal bone. To our knowledge, only two other cases of this type have been reported earlier.

  10. Chordomas: Is It Possible to Predict Recurrence?

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    Banu SARSIK

    2009-06-01

    Full Text Available Objective: Chordoma is a rare tumor with an unpredictable behaviour, and can display malignant behavior because of its tendency to local invasion and recurrence. We searched the prognostic value of histologic features, growth pattern, localization and Ki-67 proliferation index to predict disease-free survival.Material and Method: Twenty-nine cases diagnosed in a single center were included in the study and evaluated with their 81 surgical specimens, (29 primary tumors, 47 recurrent lesions and five metastatic foci regarding their matrix formation (myxoid, chondroid, cellular features, (pleomorphism, necrosis, inflammatory infiltration, patterns of proliferation (solid, trabecular, mixed and Ki-67 proliferation indices.Results: Eleven of the cases were females (37.9% while 18 of them were males (62.1% with a mean age of 54.1±14.6 (ranged between age 23-78 years. Thirty-eight percent of tumors were located in sacrococcigeal region followed by skull base and vertebrae (31% for both. Skull base chordomas which occured in younger patients (p=0.048 showed more trabecular pattern (p=0.04, chondroid matrix (p=0.063, lower Ki-67 (p=0.146 and longer disease-free survival (p=0.021. In contrast, tumors located in vertebrae, showed more “atypical” morphology with solid pattern, nuclear pleomorphism and dedifferentiation, higher Ki-67 indices and shorter disease-free survivals (p=0.021. Sacral tumors were the “intermediate group” which occurred in older patients, and demonstrated average Ki-67 proliferation indices and disease-free survivals.Conclusion: Vertebral localization, probably in relation with both histologic features and failure of surgery, appeared as a significant risk factor for recurrence and Ki-67 proliferation index retained its potential to predict disease-free survival.

  11. Difficulty distinguishing benign notochordal cell tumor from chordoma further suggests a link between them

    Science.gov (United States)

    2014-01-01

    Background Much discussion about benign notochordal cell tissue in vertebrae has centered on the nature of its relationship, if any, to chordoma. Often referred to as benign notochordal cell tumors (BNCTs), these lesions have unique morphological features, however, differentiating between notochordal cells in discs, BNCT, and chordoma can be difficult. They are described as radiologically distinct from chordoma, with lysis, contrast enhancement, and a soft tissue mass indicating chordoma. Methods All chordomas diagnosed at our institution, the Istituto Ortopedico Rizzoli (Bologna, Italy), prior to 2008 were reviewed, yielding 174 cases. Five were limited to bone; one was a recurrent chordoma without original data available. The remaining four were re-evaluated in detail. Results There were three women and one man, aged 33–57 years (mean, 48 years). Two were BNCTs and two were mixed lesions containing BNCT and chordoma. On computed tomography, all were radiopaque with areas of lysis. One BNCT was heterogeneous on magnetic resonance imaging, enhancing after contrast. Microscopically, one BNCT had a well-defined cystic area with a sclerotic border. The other had a minute atypical area; it recurred as chordoma. The mixed lesions had areas of definitive BNCT, definitive chordoma, and atypical areas that did not meet the criteria for either. The atypical areas in all three cases ‘blended’ with areas of chordoma or BNCT. Conclusion These cases illustrate the ongoing challenges in differentiating between BNCT and chordoma. All had unique imaging features; three had atypical microscopic areas blending with BNCT or chordoma, strengthening the argument for a relationship between the two entities and supporting the idea that some BNCTs may progress to chordoma. Our study dispels the notion that any single radiologic criterion used to distinguish between chordoma and BNCT is reliable, opening the discussion as to whether or how to monitor BNCTs. PMID:25609192

  12. Genomic and epigenetic instability in chordoma: current insights

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    Feng Y

    2014-05-01

    Full Text Available Yong Feng,1,2 Jacson K Shen,1,3 Francis J Hornicek,1,3 Zhenfeng Duan1,3 1Department of Orthopedic Surgery, Massachusetts General Hospital, Boston, MA, USA; 2Department of Orthopedic Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, People’s Republic of China; 3Sarcoma Biology Laboratory, Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital, Boston, MA, USA Abstract: Chordoma is a malignant bone tumor, which currently can only be defined by histologic and immunohistochemical criteria. There are no prognostic biomarkers to predict the clinical outcome or response to treatment yet. Currently, chordoma pathogenesis is very poorly understood; however, recent large-scale genetic and epigenetic studies have identified some of the underlying mechanisms and pathways that may contribute to the disease. In this review, we summarize the most recent findings in the field of chordoma genomics and epigenomics, from comparative genomic hybridization to evaluate chromosomal alteration, large-scale deoxyribonucleic acid (DNA sequencing to determine the gene mutation, microarray to access messenger ribonucleic acid (RNA and microRNA gene expression, and DNA-methylation profiling. These studies may also hold valuable clinical potential in the management of chordoma. Keywords: chordoma, chromosomal alterations, sequencing, miRNA, DNA methylation

  13. Erlotinib Inhibits Growth of a Patient-Derived Chordoma Xenograft

    Science.gov (United States)

    Siu, I-Mei; Ruzevick, Jacob; Zhao, Qi; Connis, Nick; Jiao, Yuchen; Bettegowda, Chetan; Xia, Xuewei; Burger, Peter C.; Hann, Christine L.; Gallia, Gary L.

    2013-01-01

    Chordomas are rare primary bone tumors that occur along the neuraxis. Primary treatment is surgery, often followed by radiotherapy. Treatment options for patients with recurrence are limited and, notably, there are no FDA approved therapeutic agents. Development of therapeutic options has been limited by the paucity of preclinical model systems. We have established and previously reported the initial characterization of the first patient-derived chordoma xenograft model. In this study, we further characterize this model and demonstrate that it continues to resemble the original patient tumor histologically and immunohistochemically, maintains nuclear expression of brachyury, and is highly concordant with the original patient tumor by whole genome genotyping. Pathway analysis of this xenograft demonstrates activation of epidermal growth factor receptor (EGFR). In vitro studies demonstrate that two small molecule inhibitors of EGFR, erlotinib and gefitinib, inhibit proliferation of the chordoma cell line U-CH 1. We further demonstrate that erlotinib significantly inhibits chordoma growth in vivo. Evaluation of tumors post-treatment reveals that erlotinib reduces phosphorylation of EGFR. This is the first demonstration of antitumor activity in a patient-derived chordoma xenograft model and these findings support further evaluation of EGFR inhibitors in this disease. PMID:24260133

  14. Erlotinib inhibits growth of a patient-derived chordoma xenograft.

    Directory of Open Access Journals (Sweden)

    I-Mei Siu

    Full Text Available Chordomas are rare primary bone tumors that occur along the neuraxis. Primary treatment is surgery, often followed by radiotherapy. Treatment options for patients with recurrence are limited and, notably, there are no FDA approved therapeutic agents. Development of therapeutic options has been limited by the paucity of preclinical model systems. We have established and previously reported the initial characterization of the first patient-derived chordoma xenograft model. In this study, we further characterize this model and demonstrate that it continues to resemble the original patient tumor histologically and immunohistochemically, maintains nuclear expression of brachyury, and is highly concordant with the original patient tumor by whole genome genotyping. Pathway analysis of this xenograft demonstrates activation of epidermal growth factor receptor (EGFR. In vitro studies demonstrate that two small molecule inhibitors of EGFR, erlotinib and gefitinib, inhibit proliferation of the chordoma cell line U-CH 1. We further demonstrate that erlotinib significantly inhibits chordoma growth in vivo. Evaluation of tumors post-treatment reveals that erlotinib reduces phosphorylation of EGFR. This is the first demonstration of antitumor activity in a patient-derived chordoma xenograft model and these findings support further evaluation of EGFR inhibitors in this disease.

  15. Intrasellar chordoma associated with a primitive persistent trigeminal artery.

    Science.gov (United States)

    Navas, Marta; Martinez, Pedro; Shakur, Sophia F; Barbosa, Antonio; Barcena, Eduardo; Gordillo, Carlos; Fraga, Javier; Blanco, Concepcion; Sola, Rafael G

    2015-01-01

    Chordomas located primarily in the sellar region are uncommon, and may be misdiagnosed non-functioning pituitary adenoma. Furthermore, the association of a persistent primitive trigeminal artery (PPTA) with an intrasellar chordoma is extremely rare, and no similar cases have been reported in the literature to date. The coexistence of intrasellar chordoma (ISC) and PPTA makes safe and complete tumor resection challenging, and preoperative endovascular occlusion of this artery may be helpful. We report a case of a 32-year-old man who developed right hemifacial paresthesias and a cranial nerve six palsy. MRI study demonstrated the presence of a primary ISC associated with a PPTA. Angiographic balloon test occlusion of the PPTA revealed no neurological changes, so this vessel was endovascularly occluded by coiling. The lesion was subtotally removed through a sublabial transsphenoidal approach, without intraoperative bleeding complications. Histological examination of the lesion was consistent with the diagnosis of chordoma. Detailed preoperative neurovascular evaluation may be useful to detect vascular anomalies associated with intracranial chordomas, such as PPTA. In this report we emphasize the importance of appropriate treatment of vascular anomalies prior intrasellar lesions resection that may facilitate surgery and avoid potential hazardous intraoperative bleeding complications. PMID:25640561

  16. Malignant fibrous histiocytoma developing in irradiated sacral chordoma

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    Halpern, J.; Kopolovic, J.; Catane, R.

    1984-06-15

    Malignant fibrous histiocytoma (MFH), arising at the site of a sacral chordoma 8 years after massive radiotherapy, is described. Initially, the patient received 7000 rad to the sacral area and, on recurrence, 5 years later, an additional 4000 rad. Two years later, a sacral mass was noted again. Biopsy then revealed MFH; chest x-ray showed multiple lung metastases. A combination chemotherapy, consisting of cyclophosphamide, vincristine, adriamycin (doxorubicin), and DTIC, resulted in a 6 month partial response. Subsequently, the patient died because of progressive metastatic disease. At autopsy, 8 years after diagnosis, both the sacral lesion and the lung metastases proved to be MFH, and no residual chordoma was found.

  17. Chondroid Chordoma and Nasal Adenocarcinoma: An Exceptional Association

    Directory of Open Access Journals (Sweden)

    Patrice Gallet

    2012-01-01

    Full Text Available Collision tumors are exceptional, associating two independent tumoral contingents. We report a case of an association of two rare tumors: sinonasal adenocarcinoma and chondroid chordoma. Initially, only adenocarcinoma was diagnosed. The treatment consisted of endoscopic endonasal surgery followed by conventional radiotherapy. After 18 months, a local recurrence was diagnosed after a facial trauma, but the true histology was difficult to assess. The tumor was dual, associating adenocarcinoma and chondroid chordoma, with atypical localization in the ethmoid. Further evolution was particularly aggressive. We discuss the key points of this observation.

  18. Thoracic intramedullary chordoma without bone involvement: a rare clinical entity.

    Science.gov (United States)

    Faheem, Mohd; Zeeshan, Qazi; Ojha, Balkrishna; Agrawal, Preeti

    2016-01-01

    An 8-year-old boy presented with a 1-year history of low backache, followed by paraparesis and urinary incontinence. MRI of the thoracic spine revealed an intramedullary, intensely contrast-enhancing lesion extending from T11 to L1 vertebral level, consistent with astrocytoma, ependymoma or haemangioblastoma. A diagnosis of intramedullary chordoma was made on tissue biopsy and immunohistochemical study. This is the second report of an intramedullary chordoma without bone involvement in English literature. After 6 months of follow-up, patient showed good clinical outcome in terms of improvement in power in lower limbs and backache.

  19. Thoracic intramedullary chordoma without bone involvement: a rare clinical entity.

    Science.gov (United States)

    Faheem, Mohd; Zeeshan, Qazi; Ojha, Balkrishna; Agrawal, Preeti

    2016-01-01

    An 8-year-old boy presented with a 1-year history of low backache, followed by paraparesis and urinary incontinence. MRI of the thoracic spine revealed an intramedullary, intensely contrast-enhancing lesion extending from T11 to L1 vertebral level, consistent with astrocytoma, ependymoma or haemangioblastoma. A diagnosis of intramedullary chordoma was made on tissue biopsy and immunohistochemical study. This is the second report of an intramedullary chordoma without bone involvement in English literature. After 6 months of follow-up, patient showed good clinical outcome in terms of improvement in power in lower limbs and backache. PMID:27469385

  20. A Novel Chordoma Xenograft Allows In Vivo Drug Testing and Reveals the Importance of NF-κB Signaling in Chordoma Biology

    Science.gov (United States)

    Trucco, Matteo M.; Awad, Ola; Wilky, Breelyn A.; Goldstein, Seth D.; Huang, Ruili; Walker, Robert L.; Shah, Preeti; Katuri, Varalakshmi; Gul, Naheed; Zhu, Yuelin J.; McCarthy, Edward F.; Paz-Priel, Ido; Meltzer, Paul S.; Austin, Christopher P.; Xia, Menghang; Loeb, David M.

    2013-01-01

    Chordoma is a rare primary bone malignancy that arises in the skull base, spine and sacrum and originates from remnants of the notochord. These tumors are typically resistant to conventional chemotherapy, and to date there are no FDA-approved agents to treat chordoma. The lack of in vivo models of chordoma has impeded the development of new therapies for this tumor. Primary tumor from a sacral chordoma was xenografted into NOD/SCID/IL-2R γ-null mice. The xenograft is serially transplantable and was characterized by both gene expression analysis and whole genome SNP genotyping. The NIH Chemical Genomics Center performed high-throughput screening of 2,816 compounds using two established chordoma cell lines, U-CH1 and U-CH2B. The screen yielded several compounds that showed activity and two, sunitinib and bortezomib, were tested in the xenograft. Both agents slowed the growth of the xenograft tumor. Sensitivity to an inhibitor of IκB, as well as inhibition of an NF-κB gene expression signature demonstrated the importance of NF-κB signaling for chordoma growth. This serially transplantable chordoma xenograft is thus a practical model to study chordomas and perform in vivo preclinical drug testing. PMID:24223206

  1. A novel chordoma xenograft allows in vivo drug testing and reveals the importance of NF-κB signaling in chordoma biology.

    Directory of Open Access Journals (Sweden)

    Matteo M Trucco

    Full Text Available Chordoma is a rare primary bone malignancy that arises in the skull base, spine and sacrum and originates from remnants of the notochord. These tumors are typically resistant to conventional chemotherapy, and to date there are no FDA-approved agents to treat chordoma. The lack of in vivo models of chordoma has impeded the development of new therapies for this tumor. Primary tumor from a sacral chordoma was xenografted into NOD/SCID/IL-2R γ-null mice. The xenograft is serially transplantable and was characterized by both gene expression analysis and whole genome SNP genotyping. The NIH Chemical Genomics Center performed high-throughput screening of 2,816 compounds using two established chordoma cell lines, U-CH1 and U-CH2B. The screen yielded several compounds that showed activity and two, sunitinib and bortezomib, were tested in the xenograft. Both agents slowed the growth of the xenograft tumor. Sensitivity to an inhibitor of IκB, as well as inhibition of an NF-κB gene expression signature demonstrated the importance of NF-κB signaling for chordoma growth. This serially transplantable chordoma xenograft is thus a practical model to study chordomas and perform in vivo preclinical drug testing.

  2. Sacrococcygeal chordoma presenting as a retro rectal tumour

    Science.gov (United States)

    Chigurupati, Pragnya; Venkatesan, Vishnukumar; Thiyagarajan, Manuneethimaran; Vikram, A.; Kiran, Kaundinya

    2014-01-01

    INTRODUCTION Chordomas are rare, slow growing, locally destructive bone tumours arising from the notochord. PRESENTATION OF CASE Presenting a case of a 65 year old man, who presented with complaints of swelling on the right lower back for 1 year associated with pain. On, physical examination, a swelling measuring 5 cm × 4 cm was noted in the lower back with posterior wall indentation on per rectal examination. MRI revealed a mass lesion involving the sacrum (s3–s4) and coccyx. FNAC showed features of a chroma. At surgery, we excised a mass from the retrorectal space and biopsy proved it to be a chondroid chordoma, a variant of chordoma. DISCUSSION Chordomas are solid malignant tumours that arise from vestiges of the foetal notochord. Common locations are the clivus and the sacrococcygeus region. Annual incidence of these tumours is 1 in one million. MRI is the imaging modality of choice. Prognosis improves based on the age, resected margins and postoperative treatment. CONCLUSION Here, we shall discuss the literature, variants, treatment and prognosis of this rare tumour. PMID:25201478

  3. Sacral chordoma: a diagnosis not to be sat on?

    Science.gov (United States)

    Gibbins, Ruth; Evans, Guy; Grimer, Robert

    2007-01-01

    Sacral chordomas are rare, slow-growing tumours that are amenable to surgery, but unfortunately often diagnosed late. The aim of the study was to identify presenting symptoms, which may aid diagnosis and reduce the treatment time. Forty-four patients were identified with sacral chordoma between 1989 and 2006. Clinical and pathological records were reviewed retrospectively to elicit the symptoms recorded prior to diagnosis, duration of symptoms, surgical treatment, size of tumour and survival. Eleven patients were excluded, leaving 33 patients in the study group. Thirty-one patients had chordomas arising from the sacrum and two patients from the coccyx. The mean duration of symptoms prior to diagnosis was 120 weeks (2.3 years), with a median of length of 104 weeks (two years) and range of 26 to 416 weeks (0.5 to eight years). The mean maximum tumour size at resection was 8.3 cm, with a mean volume of 614 cm3 (range 9–2,113 cm3). Pain, typically dull and worse with sitting, was the most common presenting symptom in 85% of patients. The classic symptoms of cauda equina (saddle anaesthesia, bladder or bowel dysfunction) occurred in 70% patients (23 patients). Sacral chordoma should be considered in cases of back pain with coccydynia, especially with neurological symptoms. PMID:17205349

  4. Chordoma of the low cervical spine presenting with Horner's syndrome

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    Leone, Antonio [Department of Radiology, Universita Cattolica, School of Medicine, Policlinico ' ' Agostino Gemelli' ' , Largo Agostino Gemelli, 8, 00168 Rome (Italy); Cerase, Alfonso [Unit of Diagnostic and Therapeutic Neuroradiology, Azienda Ospedaliera Senese, Siena (Italy); InterDepartmental Center of Magnetic Resonance, Policlinico ' ' Le Scotte' ' , Siena (Italy); Tarquini, Elisabetta; Mule, Antonio [Department of Pathology, Universita Cattolica, School of Medicine, Policlinico ' ' Agostino Gemelli' ' , Largo Agostino Gemelli, 8, 00168 Rome (Italy)

    2002-07-01

    Chordomas arising from the low cervical spine have been reported rarely. This case report expands the knowledge of chordomas of the low cervical spine by reporting the clinical, radiological, and pathological findings of a case of chordoma arising from C6 vertebra in a 61-year-old man presenting with left Horner's syndrome. This clinical presentation is uncommon for cervical chordomas and resulted from the tumor growth in the left carotid space resulting in the total encasement of common carotid artery and involvement of sympathetic fibers ipsilaterally. (orig.)

  5. Prognostic Significance of miRNA-1 (miR-1) Expression in Patients with Chordoma

    Science.gov (United States)

    Duan, Zhenfeng; Shen, Jacson; Yang, Xiaoqian; Yang, Pei; Osaka, Eiji; Choy, Edwin; Cote, Gregory; Harmon, David; Zhang, Yu; Nielsen, G. Petur; Spentzos, Dimitrios; Mankin, Henry; Hornicek, Francis

    2014-01-01

    Reliable prognostic biomarkers for chordoma have not yet been established. Recent studies revealed that expression of miRNA-1(miR-1) is frequently downregulated in several cancer types including chordoma. The goal of this follow-up study is to investigate the expression of miR-1 as a prognostic biomarker and further confirm the functional role of miR-1 in chordoma cell growth and proliferation. We determined the relative expression levels of miR-1 and Met in chordoma tissue samples and correlated those to clinical variables. The results showed that miR-1 was downregulated in 93.7% of chordoma tissues and expression was inversely correlated with Met expression. miR-1 expression levels also correlated with clinical prognosis. To characterize and confirm the functional role of miR-1 in the growth and proliferation of chordoma cells, miR-1 precursors were stably transfected into chordoma cell lines UCH-1 and CH-22. Cell Proliferation Assay and MTT were used to evaluate cell growth and proliferation. Restoring expression of miR-1 precursor decreased cell growth and proliferation in UCH-1 and CH-22 cells. These results indicate that suppressed miR-1 expression in chordoma may in part be a driver for tumor growth, and that miR-1 has potential to serve as prognostic biomarker and therapeutic target for chordoma patients. PMID:24501096

  6. Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review

    Directory of Open Access Journals (Sweden)

    Guiramand Jérôme

    2011-10-01

    Full Text Available Abstract Background Chordomas are very rare low-grade malignant bone tumors that arise from the embryonic rests of the notochord. They are characterized by slow growth and long history with frequent local relapses, and sometimes metastases. While chemotherapy is not efficient, imatinib has shown antitumor activity. Case presentation We report on a 76-year-old patient with EGFR-overexpressing advanced chordoma that progressed on imatinib and subsequently responded to erlotinib during 12 months. Conclusions We report the fourth case of advanced chordoma treated with an EGFR inhibitor. We also review the literature concerning the rationale and potential of EGFR targeting in chordoma.

  7. Cytogenetic telomere and telomerase studies in lumbo-sacral chordoma

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    Schwartz, H.S.; Dahir, G.A.; Miller, L.K. [Vanderbilt Univ., Nashville, TN (United States)] [and others

    1994-09-01

    Lumbo-sacral chordomas are rare skeletal sarcomas that originate from the remnant notochord. There are approximately 35 lumbo-sacral chordomas reported annually in the U.S.A. The understanding of this rare human cancer is limited to observations of its clinical behavior and embryonic link. We performed chromosome and molecular analyses from five surgically harvested chordomas in an effort to document genetic abnormalities and to further understand its tumor biology. Cytogenetically, four of five patients had entirely normal chromosomes. One patient had several abnormalities seen in one of 100 cells including a translocation with breakpoints at bands 5q13 and 7q22, loss of one X chromosome and an extra chromosome 14. There was no evidence of monosomy X or trisomy 14 seen with interphase in situ hybridization using biotin-labeled alpha satellite chromosome specific probes for chromosome 14/22 and X. Telomere integrity is required to protect termini from illegitimate recombination. Typically telomeric reduction occurs in senescent fibroblasts in vivo aging and several human solid tumors. A telomeric probe (TTAGGG){sub 50} was hybridized to genomic DNA isolated from chordoma cells and digested with Hinf I which allows the telomeric DNA to remain intact. The tumor DNA was paired with leukocyte DNA from age-matched controls and revealed telomere elongation in all four patients studied with molecular genetic techniques. Telomerase activity is required to maintain telomere integrity and is not present in normal somatic cells. It is determined by visualizing the sizes of the electrophoresis gel-separated radioactive telomeric fragments assembled during incubation of cytoplasmic extracts containing telomerase. Telomerase activity was detected when compared with HeLa cells, a positive control. In addition, no telomerase activity was detected from the chordoma patient`s fibroblasts.

  8. Intracranial chordoma presenting as acute hemorrhage in a child: Case report and literature review

    Science.gov (United States)

    Moore, Kenneth A.; Bohnstedt, Bradley N.; Shah, Sanket U.; Abdulkader, Marwah M.; Bonnin, Jose M.; Ackerman, Laurie L.; Shaikh, Kashif A.; Kralik, Stephen F.; Shah, Mitesh V.

    2015-01-01

    Background: Chordomas are rare, slow-growing malignant neoplasms derived from remnants of the embryological notochord. Pediatric cases comprise only 5% of all chordomas, but more than half of the reported pediatric chordomas are intracranial. For patients of all ages, intracranial chordomas typically present with symptoms such as headaches and progressive neurological deficits occurring over several weeks to many years as they compress or invade local structures. There are only reports of these tumors presenting acutely with intracranial hemorrhage in adult patients. Case Description: A 10-year-old boy presented with acute onset of headache, emesis, and diplopia. Head computed tomography and magnetic resonance imaging of brain were suspicious for a hemorrhagic mass located in the left petroclival region, compressing the ventral pons. The mass was surgically resected and demonstrated acute intratumoral hemorrhage. Pathologic examination was consistent with chordoma. Conclusion: There are few previous reports of petroclival chordomas causing acute intracranial hemorrhage. To the authors’ knowledge, this is the first case of a petroclival chordoma presenting as acute intracranial hemorrhage in a pediatric patient. Although uncommon, it is important to consider chordoma when evaluating a patient of any age presenting with a hemorrhagic lesion of the clivus. PMID:25949851

  9. Drop metastases in a patient with a chondroid chordoma of the clivus

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    Uggowitzer, M.M.; Kugler, C.; Groell, R.; Lindbichler, F.; Ranner, G. [Dept. of Radiology, Karl-Franzens Medical School and Univ. Hospital, Graz (Austria); Radner, H. [Inst. of Pathology, Karl-Franzens Medical School and Univ. Hospita, Graz (Austria); Sutter, B. [Dept. of Neurosurgery, Karl-Franzens Medical School and Univ. Hospital, Graz (Austria)

    1999-07-01

    Metastasising chordomas are extremely rare and only four cases with drop metastases have been reported. We report a patient with an intracranial chondroid chordoma, typically involving the clivus, treated by repeated resection, percutaneous transluminal embolisation and radiosurgery. During follow-up with MRI asymptomatic intradural drop metastases were observed throughout the spine, with transgression of the intervertebral foramen, forming a `dumbbell`. (orig.)

  10. Skull base chordoma presenting as nasopharyngeal mass with lymph node metastasis

    Directory of Open Access Journals (Sweden)

    Bhawna Bhutoria Jain

    2013-01-01

    Full Text Available Spheno-occipital chordomas can rarely present as nasopharyngeal mass. Metastases occur only in advanced disease. They can pose a diagnostic dilemma when information about diagnosis of the primary tumor is not available. We present cytological findings in upper cervical lymph node of a case of nasopharyngeal chordoma and discuss possible differential in such a location.

  11. Immunotherapy as a Potential Treatment for Chordoma: a Review.

    Science.gov (United States)

    Patel, Shalin S; Schwab, Joseph H

    2016-09-01

    Chordoma is a locally aggressive primary malignancy of the axial skeleton. The gold standard for treatment is en bloc resection, with some centers now advocating for the use of radiation to help mitigate the risk of recurrence. Local recurrence is common, and salvaging local failures is quite difficult. Chemotherapy has been ineffective and small molecule targeted therapy has had only marginal benefits in small subsets of patients with rare tumor phenotypes or refractory disease. Recent successes utilizing immunotherapy in a variety of cancers has led to a resurgence of interest in modifying the host immune system to develop new ways to treat tumors. This review will discuss these studies and will highlight the early studies employing immune strategies for the treatment of chordoma.

  12. Proton radiation therapy for clivus chordoma; Case report

    Energy Technology Data Exchange (ETDEWEB)

    Yoshii, Yoshihiko; Tsunoda, Takashi; Hyodo, Akio; Nose, Tadao (Tsukuba Univ., Ibaraki (Japan). Inst. of Clinical Medicine); Tsujii, Hirohiko; Tsuji, Hiroshi; Inada, Tetsuo; Maruhashi, Akira; Hayakawa, Yoshinori

    1993-03-01

    A 57-year-old male with clival chordoma developed severe hoarseness, dysphagia, and dysphonia 1 month after a second removal of the tumor. Magnetic resonance imaging demonstrated a mass 10 cm in diameter in the region of the middle clivus enhanced inhomogeneously by gadolinium-diethylenetriaminepenta-acetic acid, and a defect in the skull base. There was evidence of compression of the anterior surface of the pons. He received proton irradiation employing a pair of parallel opposed lateral proton beams. The dose aimed at the tumor mass was 75.5 Gy, to the pharyngeal wall less than 38 Gy, and to the anterior portion of the pons less than 30 Gy. Time dose and fractionation factor was calculated at 148. Thirty-one months following treatment, he was free of clinical neurological sequelae. Proton therapy should be considered in treatment planning following initial surgical removal or for inoperable clivus chordoma. (author).

  13. Immunotherapy as a Potential Treatment for Chordoma: a Review.

    Science.gov (United States)

    Patel, Shalin S; Schwab, Joseph H

    2016-09-01

    Chordoma is a locally aggressive primary malignancy of the axial skeleton. The gold standard for treatment is en bloc resection, with some centers now advocating for the use of radiation to help mitigate the risk of recurrence. Local recurrence is common, and salvaging local failures is quite difficult. Chemotherapy has been ineffective and small molecule targeted therapy has had only marginal benefits in small subsets of patients with rare tumor phenotypes or refractory disease. Recent successes utilizing immunotherapy in a variety of cancers has led to a resurgence of interest in modifying the host immune system to develop new ways to treat tumors. This review will discuss these studies and will highlight the early studies employing immune strategies for the treatment of chordoma. PMID:27475804

  14. MicroRNA-1(miR-1) inhibits chordoma cell migration and invasion by targeting Slug

    Science.gov (United States)

    Osaka, Eiji; Yang, Xiaoqian; Shen, Jacson K.; Yang, Pei; Feng, Yong; Mankin, Henry J.; Hornicek, Francis J.; Duan, Zhenfeng

    2014-01-01

    Recent studies have revealed that expression of miRNA-1(miR-1) is frequently downregulated in several cancer types including chordoma. Identifying and validating novel targets of miR-1 is useful for understanding the roles of miR-1 in chordoma. We aimed to further investigate the functions of miR-1 in chordoma. Specifically, we assessed whether restoration of miR-1 affects cell migration and invasion in chordoma, and focused on the miR-1 potential target Slug gene. Migratory and invasive activities were assessed by wound healing and Matrigel invasion assays, respectively. Cell proliferation was determined by MTT assay. Slug expression was evaluated by Western blot, immunofluorescence, and immunohistochemistry. Restoration of miR-1 expression suppressed the migratory and invasive activities of chordoma cells. Transfection of miR-1 inhibited cell proliferation both time- and dose-dependently in chordoma. miR-1 transfected cells showed inhibited Slug expression. Slug was overexpressed in chordoma cell lines and advanced chordoma tissues. In conclusion, we have shown that miR-1 directly targets the Slug gene in chordoma. Restoration of miR-1 suppressed not only proliferation, but also migratory and invasive activities, and reduced the Slug expression in chordoma cells. These results collectively indicate that miR-1/Slug pathway is a potential therapeutic target because of its crucial roles in chordoma cell growth and migration. PMID:24760686

  15. SACROCOCCYGEAL CHORDOMA DIAGNOSED BY FNAC: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Patel

    2014-05-01

    Full Text Available Chordoma is rare, slow growing but locally aggressive neoplasms derived from primitive notochordal elements. It accounts for 1- 4% of all primary bone tumors and mainly found in the sacrococcygeal and spheno-occipital regions. Preoperative fine needle aspiration cytology (FNAC examination of the tumor is diagnostic. Treatment is in the form of surgical excision, radiation therapy or a combination of both modalities. FNAC improves prognosis and survival rate due to early diagnosis.

  16. Imaging features of posterior mediastinal chordoma in a child

    Energy Technology Data Exchange (ETDEWEB)

    Soudack, Michalle; Guralnik, Ludmilla; Engel, Ahuva [Rambam Health Care Campus, Department of Diagnostic Imaging, Haifa (Israel); Ben-Nun, Alon [Rambam Health Care Campus, Department of Thoracic Surgery, Haifa (Israel); Berkowitz, Drora [Rambam Health Care Campus, Department of Pediatrics B, Haifa (Israel); Postovsky, Sergey [Rambam Health Care Campus, Department of Pediatric Hemato-Oncology, Haifa (Israel); Vlodavsky, Eugene [Rambam Health Care Campus, Department of Pathology, Haifa (Israel)

    2007-05-15

    A 51/2-year-old boy presented with repeated episodes of stridor and cough. Chest radiography demonstrated a widened mediastinum. Evaluation by CT revealed a low-density posterior mediastinal mass initially diagnosed as benign tumor. Histopathological analysis of the resected mass disclosed a malignant chordoma. Our radiological results are described with an analysis of the imaging findings in the medical literature. We present our suggestions for preoperative evaluation of posterior mediastinal tumors. (orig.)

  17. Histochemical and immunohistochemical characterization of chordoma in ferrets

    Science.gov (United States)

    YUI, Takeshi; OHMACHI, Tetsuo; MATSUDA, Kazuya; OKAMOTO, Minoru; TANIYAMA, Hiroyuki

    2015-01-01

    Chordomas of the tip of the tail in 6 ferrets were examined using histopathological, histochemical and immunohistochemical procedures. Histopathologically, round neoplastic cells containing numerous cytoplasmic vacuoles of varying sizes, categorized as “physaliphorous cells”, were observed in the amorphous eosinophilic or pale basophilic myxoid stroma. Physaliphorous cells were arranged in lobules and in a “chordoid” or “cobblestone” manner. The neoplasms were diagnosed as benign chordoma without local invasion and metastasis. Histochemically, the cytoplasm of small neoplastic cells was positive for periodic acid-Schiff stain and alcian blue (AB) pH 2.5 and pH 1.0 stains, but negative for hyaluronidase digestion-AB pH 2.5 stain. All neoplastic cells were strongly stained with colloidal ion, negative for high iron diamine AB pH 2.5 and toluidine blue pH 2.5 stains, and positive for Mayer’s mucicarmine stain. Immunohistochemistry using antibodies directed against low-molecular-weight cytokeratins (CK18, CK19 and CK20), vimentin and mucin core protein (MUC5AC) revealed that neoplastic cells had both epithelial and mesenchymal elements. The expression of low-molecular-weight cytokeratins suggests that neoplastic cells acquired the properties of glandular epithelial cells and produced epithelial mucus. Furthermore, the expression of cytokeratins, vimentin, S100 protein, brachyury and epithelial membrane antigen indicates that the neoplasms were equivalent to the classic type of human chordoma. Therefore, immunohistochemistry using these antibodies can be useful for the characterization of ferret chordoma. PMID:25648567

  18. Computer Navigation-aided Resection of Sacral Chordomas

    Directory of Open Access Journals (Sweden)

    Yong-Kun Yang

    2016-01-01

    Full Text Available Background: Resection of sacral chordomas is challenging. The anatomy is complex, and there are often no bony landmarks to guide the resection. Achieving adequate surgical margins is, therefore, difficult, and the recurrence rate is high. Use of computer navigation may allow optimal preoperative planning and improve precision in tumor resection. The purpose of this study was to evaluate the safety and feasibility of computer navigation-aided resection of sacral chordomas. Methods: Between 2007 and 2013, a total of 26 patients with sacral chordoma underwent computer navigation-aided surgery were included and followed for a minimum of 18 months. There were 21 primary cases and 5 recurrent cases, with a mean age of 55.8 years old (range: 35-84 years old. Tumors were located above the level of the S3 neural foramen in 23 patients and below the level of the S3 neural foramen in 3 patients. Three-dimensional images were reconstructed with a computed tomography-based navigation system combined with the magnetic resonance images using the navigation software. Tumors were resected via a posterior approach assisted by the computer navigation. Mean follow-up was 38.6 months (range: 18-84 months. Results: Mean operative time was 307 min. Mean intraoperative blood loss was 3065 ml. For computer navigation, the mean registration deviation during surgery was 1.7 mm. There were 18 wide resections, 4 marginal resections, and 4 intralesional resections. All patients were alive at the final follow-up, with 2 (7.7% exhibiting tumor recurrence. The other 24 patients were tumor-free. The mean Musculoskeletal Tumor Society Score was 27.3 (range: 19-30. Conclusions: Computer-assisted navigation can be safely applied to the resection of the sacral chordomas, allowing execution of preoperative plans, and achieving good oncological outcomes. Nevertheless, this needs to be accomplished by surgeons with adequate experience and skill.

  19. MicroRNA-155 expression is independently predictive of outcome in chordoma

    Science.gov (United States)

    Osaka, Eiji; Kelly, Andrew D.; Spentzos, Dimitrios; Choy, Edwin; Yang, Xiaoqian; Shen, Jacson K.; Yang, Pei; Mankin, Henry J.; Hornicek, Francis J.; Duan, Zhenfeng

    2015-01-01

    Background Chordoma pathogenesis remains poorly understood. In this study, we aimed to evaluate the relationships between microRNA-155 (miR-155) expression and the clinicopathological features of chordoma patients, and to evaluate the functional role of miR-155 in chordoma. Methods The miRNA expression profiles were analyzed using miRNA microarray assays. Regulatory activity of miR-155 was assessed using bioinformatic tools. miR-155 expression levels were validated by reverse transcription-polymerase chain reaction. The relationships between miR-155 expression and the clinicopathological features of chordoma patients were analyzed. Proliferative, migratory and invasive activities were assessed by MTT, wound healing, and Matrigel invasion assays, respectively. Results The miRNA microarray assay revealed miR-155 to be highly expressed and biologically active in chordoma. miR-155 expression in chordoma tissues was significantly elevated, and this expression correlated significantly with disease stage (p = 0.036) and the presence of metastasis (p = 0.035). miR-155 expression also correlated significantly with poor outcomes for chordoma patients (hazard ratio, 5.32; p = 0.045). Inhibition of miR-155 expression suppressed proliferation, and the migratory and invasive activities of chordoma cells. Conclusions We have shown miR-155 expression to independently affect prognosis in chordoma. These results collectively indicate that miR-155 expression may serve not only as a prognostic marker, but also as a potential therapeutic target in chordoma. PMID:25823817

  20. Whole-transcriptome analysis of chordoma of the skull base.

    Science.gov (United States)

    Bell, Diana; Raza, Shaan M; Bell, Achim H; Fuller, Gregory N; DeMonte, Franco

    2016-10-01

    Fourteen skull base chordoma specimens and three normal specimens were microdissected from paraffin-embedded tissue. Pools of RNA from highly enriched preparations of these cell types were subjected to expression profiling using whole-transcriptome shotgun sequencing. Using strict criteria, 294 differentially expressed transcripts were found, with 28 % upregulated and 72 % downregulated. The transcripts were annotated using NCBI Entrez Gene and computationally analyzed with the Ingenuity Pathway Analysis program. From these significantly changed expressions, the analysis identified 222 cancer-related transcripts. These 294 differentially expressed genes and non-coding RNA transcripts provide here a set to specifically define skull base chordomas and to identify novel and potentially important targets for diagnosis, prognosis, and therapy of this cancer. Significance Genomic profiling to subtype skull base chordoma reveals potential candidates for specific biomarkers, with validation by IHC for selected candidates. The highly expressed developmental genes T, LMX1A, ZIC4, LHX4, and HOXA1 may be potential drivers of this disease.

  1. Radiology of sacrococcygeal chordoma. Difficulties in detecting soft tissue extension

    Energy Technology Data Exchange (ETDEWEB)

    Hudson, T.M.; Galceran, M.

    1983-05-01

    Plain radiographs of seven patients with sacrococcygeal chordomas showed sacral destruction, enlarged sacral neural foramina, and, usually, a presacral mass. Conventional tomograms clarified these bone abnormalities, which were often poorly visible on the plain radiographs. Two radionuclide bone scans showed increased peripheral uptake around the lesion, and one showed decreased uptake in the area of destroyed bone. Angiograms demonstrated only vessel displacement, and barium enemas showed only displacement of bowel by large soft tissue masses. Five myelograms were normal. Adequate surgical treatment of sacral chordomas requires a wide radical resection that avoids contaminating the wound with tumor. Therefore, thorough preoperative radiologic evaluation of the anatomic extent of a chordoma is essential. Although computed tomograms in three patients provided the best delineation of the total extent of bone and soft tissue involvement, tumor extension into gluteal muscles and other tissues eluded radiographic detection. The widespread infiltrative growth pattern along soft tissue planes and nerves and into vessels, undetectable by the above listed studies, mandates a cautious approach and a wide surgical margin, to prevent wound contamination and subsequent recurrence.

  2. Characteristics and Patterns of Metastatic Disease from Chordoma

    Science.gov (United States)

    Young, Victoria A.; Curtis, Kevin M.; Temple, H. Thomas; Eismont, Frank J.; DeLaney, Thomas F.; Hornicek, Francis J.

    2015-01-01

    Chordoma is a rare, slow-growing malignant tumor arising from notochordal remnants. A retrospective review of patient records at two major referral centers was undertaken to assess the incidence, location, and prognostic factors of metastatic disease from chordoma. 219 patients with chordoma (1962–2009) were identified. 39 patients (17.8%) developed metastatic disease, most frequently to lung (>50%). Median survival from the time of initial diagnosis was 130.4 months for patients who developed metastatic disease and 159.3 months for those who did not (P = 0.05). Metastatic disease was most common in the youngest patients (P = 0.07), and it was 2.5 times more frequent among patients with local recurrence (26.3%) than in those without (10.8%) (P = 0.003). Patient survival with metastatic disease was highly variable, and it was dependent on both the location of the tumor primary and the site of metastasis. Metastasis to distal bone was the most rapid to develop and had the worst prognosis. PMID:26843835

  3. Characteristics and Patterns of Metastatic Disease from Chordoma

    Directory of Open Access Journals (Sweden)

    Victoria A. Young

    2015-01-01

    Full Text Available Chordoma is a rare, slow-growing malignant tumor arising from notochordal remnants. A retrospective review of patient records at two major referral centers was undertaken to assess the incidence, location, and prognostic factors of metastatic disease from chordoma. 219 patients with chordoma (1962–2009 were identified. 39 patients (17.8% developed metastatic disease, most frequently to lung (>50%. Median survival from the time of initial diagnosis was 130.4 months for patients who developed metastatic disease and 159.3 months for those who did not (P=0.05. Metastatic disease was most common in the youngest patients (P=0.07, and it was 2.5 times more frequent among patients with local recurrence (26.3% than in those without (10.8% (P=0.003. Patient survival with metastatic disease was highly variable, and it was dependent on both the location of the tumor primary and the site of metastasis. Metastasis to distal bone was the most rapid to develop and had the worst prognosis.

  4. Clinical results of proton beam therapy for skull base chordoma

    International Nuclear Information System (INIS)

    Purpose: To evaluate clinical results of proton beam therapy for patients with skull base chordoma. Methods and materials: Thirteen patients with skull base chordoma who were treated with proton beams with or without X-rays at the University of Tsukuba between 1989 and 2000 were retrospectively reviewed. A median total tumor dose of 72.0 Gy (range, 63.0-95.0 Gy) was delivered. The patients were followed for a median period of 69.3 months (range, 14.6-123.4 months). Results: The 5-year local control rate was 46.0%. Cause-specific, overall, and disease-free survival rates at 5 years were 72.2%, 66.7%, and 42.2%, respectively. The local control rate was higher, without statistical significance, for those with preoperative tumors <30 mL. Partial or subtotal tumor removal did not yield better local control rates than for patients who underwent biopsy only as the latest surgery. Conclusion: Proton beam therapy is effective for patients with skull base chordoma, especially for those with small tumors. For a patient with a tumor of <30 mL with no prior treatment, biopsy without tumor removal seems to be appropriate before proton beam therapy

  5. Tissue microarray immunohistochemical detection of brachyury is not a prognostic indicator in chordoma.

    Directory of Open Access Journals (Sweden)

    Linlin Zhang

    Full Text Available Brachyury is a marker for notochord-derived tissues and neoplasms, such as chordoma. However, the prognostic relevance of brachyury expression in chordoma is still unknown. The improvement of tissue microarray technology has provided the opportunity to perform analyses of tumor tissues on a large scale in a uniform and consistent manner. This study was designed with the use of tissue microarray to determine the expression of brachyury. Brachyury expression in chordoma tissues from 78 chordoma patients was analyzed by immunohistochemical staining of tissue microarray. The clinicopathologic parameters, including gender, age, location of tumor and metastatic status were evaluated. Fifty-nine of 78 (75.64% tumors showed nuclear staining for brachyury, and among them, 29 tumors (49.15% showed 1+ (<30% positive cells staining, 15 tumors (25.42% had 2+ (31% to 60% positive cells staining, and 15 tumors (25.42% demonstrated 3+ (61% to 100% positive cells staining. Brachyury nuclear staining was detected more frequently in sacral chordomas than in chordomas of the mobile spine. However, there was no significant relationship between brachyury expression and other clinical variables. By Kaplan-Meier analysis, brachyury expression failed to produce any significant relationship with the overall survival rate. In conclusion, brachyury expression is not a prognostic indicator in chordoma.

  6. Cytopathological Dilemma of Anaplastic Sacral Chordoma with Radiological and Histological Corroboration

    Directory of Open Access Journals (Sweden)

    Arghya BANDYOPADHYAY

    2011-05-01

    Full Text Available Chordoma is a relatively rare locally invasive and potentially malignant tumor of fetal notochord origin, affecting the axial skeleton. Cytopathological diagnosis of chordoma is favored by the presence of characteristic physaliphorous cells, bearing abundant foamy cytoplasm dispersed in a myxoid matrix. Anaplastic chordoma or dedifferentiated chordoma, an even rarer variant, can cause a diagnostic confusion with chondrosarcoma from the cytopathological point of view, with similar chondromyxoid matrix and atypical cells. Hence, chordoma bearing anaplastic features needs to be identified and should be distinguished from chondrosarcoma on aspiration cytopathology. We present a case of anaplastic sacral chordoma in a man 59 years of age, causing extensive destruction of sacrum and invading the paravertebral tissues as evidenced by radiology. Fine needle aspiration cytopathology revealed few large pleomorphic hyperchromatic cells, admixed with characteristic physaliphorous cells and myxoid matrix. The cytopathological diagnosis has been confirmed by histopathology and immunohistochemistry. Since anaplastic chordoma bears an unfavorable prognosis, it should be suspected on preoperative aspiration cytopathology. Clinicoradiological correlation along with histopathological and immunohistochemical confirmation is necessary subsequently.

  7. PD-1, PD-L1, PD-L2 expression in the chordoma microenvironment

    Science.gov (United States)

    Mathios, Dimitrios; Ruzevick, Jacob; Jackson, Christopher M.; Xu, Haiying; Shah, Sagar; Taube, Janis M.; Burger, Peter C.; McCarthy, Edward F.; Quinones-Hinojosa, Alfredo; Pardoll, Drew M.

    2015-01-01

    Chordomas are rare malignant tumors that are postulated to arise from remnants of the notochord. Currently, the interaction between chordomas and the host immune system is poorly understood. The checkpoint protein, PD-1 is expressed by circulating lymphocytes and is a marker of activation and exhaustion. Its ligands, PD-L1 (B7-H1, CD274) and PD-L2 (B7-DC, CD273), are expressed on a variety of human cancers; however this pathway has not been previously reported in chordomas. We used flow cytometric and RT-PCR analysis in three established primary and recurrent chordoma cell lines (U-CH1, U-CH2, and JHC7) as well as immunohistochemical analysis of chordoma tissues from 10 patients to identify and localize expression of PD-1 pathway proteins. PD-1 ligands are not constitutively expressed by chordoma cells, but their expression is induced in the setting of pro-inflammatory cytokines in all cell lines examined. In paraffin embedded tissues, we found that tumor infiltrating lymphocytes expressed PD-1 in 3/6 cases. We also found that, although chordoma cells did not express significant levels of PD-L1, PD-L1 expression was observed on tumor-infiltrating macrophages and tumor infiltrating lymphocytes. Our study suggests that PD-1, PD-L1, and PD-L2 are present in the microenvironment of a subset of chordomas analyzed. Future studies are needed to evaluate the contribution of the PD-1 pathway to the immunosuppressive microenvironment of chordomas. PMID:25349132

  8. Intradural spinal seeding and fatal progression of a sacrococcygeal chordoma:a case report

    Institute of Scientific and Technical Information of China (English)

    JI Tao; GUO Wei; SHEN Dan-hua; YANG Yi; TANG Shun

    2008-01-01

    @@ Chordoma as a rare malignant tumor arising from remnants of the fetal notochord accounts for 1%-4% of primary bone tumors.It is usually predominant in males.Local recurrences are common (44%-78%)1-3 and distant metastases may occur years after the initial presentation.The reposed rates of metastases range from 10% to 48%,4-6 which are usually accompanied by a sacrococcygeal chordoma.Cases of surgical seeding and intradural spinal seeding also have been reported clinically.7-9 To our knowledge,the present case is the first one concerning cerebrospinal fluid metastases and fatal progression of a sacrococcygeal chordoma.

  9. Chordoma of the Lumbar Spine Presenting as Sciatica and Treated with Vertebroplasty

    International Nuclear Information System (INIS)

    The lumbar spine is a less common location for chordoma. Here we describe a 44-year-old woman presenting with pain due to a L4 vertebral expansile lesion that caused significant canal stenosis and neural foraminal compromise. Vertebroplasty was performed and resulted in immediate pain relief. For patients with painful lumbar chordoma who are unwilling to undergo surgery, vertebroplasty can play a palliative role as in patients with other vertebral lesions. Treating pain and stabilizing vertebra by way of vertebroplasty in a case of chordoma has not yet been reported.

  10. Cranial chordomas in infancy and childhood. A report of two cases and review of the literature

    International Nuclear Information System (INIS)

    Cranial chordomas are uncommon, accounting for less than 1% of all intracranial neoplasms. Although they are presumed to arise from congenital notochordal remnants, it is rare for these tumors to present in childhood. Only 35 cases of cranial chordomas have been reported in children 16 years of age or younger. We report 2 additional cases of pediatric cranial chordomas. One occurred in a 4 month old infant and to our knowledge represents the earliest age of presentation yet reported. The second case documents the value of MR imaging in delineating the extent of the tumor and defining its relationship to adjacent structures. (orig.)

  11. A common single-nucleotide variant in T is strongly associated with chordoma.

    Science.gov (United States)

    Pillay, Nischalan; Plagnol, Vincent; Tarpey, Patrick S; Lobo, Samira B; Presneau, Nadège; Szuhai, Karoly; Halai, Dina; Berisha, Fitim; Cannon, Stephen R; Mead, Simon; Kasperaviciute, Dalia; Palmen, Jutta; Talmud, Philippa J; Kindblom, Lars-Gunnar; Amary, M Fernanda; Tirabosco, Roberto; Flanagan, Adrienne M

    2012-11-01

    Chordoma is a rare malignant bone tumor that expresses the transcription factor T. We conducted an association study of 40 individuals with chordoma and 358 ancestry-matched controls, with replication in an independent cohort. Whole-exome and Sanger sequencing of T exons showed strong association of the common nonsynonymous SNP rs2305089 with chordoma risk (allelic odds ratio (OR) = 6.1, 95% confidence interval (CI) = 3.1-12.1; P = 4.4 × 10(-9)), a finding that is exceptional in cancers with a non-Mendelian mode of inheritance. PMID:23064415

  12. The Brachyury Gly177Asp SNP Is not Associated with a Risk of Skull Base Chordoma in the Chinese Population

    Directory of Open Access Journals (Sweden)

    Zhen Wu

    2013-10-01

    Full Text Available A recent chordoma cancer genotyping study reveals that the rs2305089, a single nucleotide polymorphism (SNP located in brachyury gene and a key gene in the development of notochord, is significantly associated with chordoma risk. The brachyury gene is believed to be one of the key genes involved in the pathogenesis of chordoma, a rare primary bone tumor originating along the spinal column or at the base of the skull. The association between the brachyury Gly177Asp single nucleotide polymorphism (SNP and the risk of skull base chordoma in Chinese populations is currently unknown. We investigated the genotype distribution of this SNP in 65 skull-base chordoma cases and 120 healthy subjects. Comparisons of the genotype distributions and allele frequencies did not reveal any significant difference between the groups. Our data suggest that the brachyury Gly177Asp SNP is not involved in the risks of skull-base chordoma, at least in the Chinese population.

  13. Cervical chordoma with vertebral artery encasement mimicking neurofibroma: MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Mortele, B.; Lemmerling, M.; Mortele, K.; Verstraete, K.; Defreyne, L.; Kunnen, M. [Department of Radiology, University Hospital, Gent (Belgium); Vandekerckhove, T. [Department of Neurosurgery, University Hospital, Gent (Belgium)

    2000-06-01

    A case of cervical chordoma in a 36-year-old white man with hypoesthesia in the neck and right shoulder, neck pain, and restricted neck mobility is presented. Plain radiographs of the cervical spine showed radiolucency of the body of C2 on the right side and enlargement of the right intervertebral foramen at C2-C3 level. Tumor encasement of the vertebral artery was demonstrated by MR imaging and confirmed by conventional arteriography. This proved to be particularly important for preoperative assessment. (orig.)

  14. Computer Navigation-aided Resection of Sacral Chordomas

    Institute of Scientific and Technical Information of China (English)

    Yong-Kun Yang; Chung-Ming Chan; Qing Zhang; Hai-Rong Xu; Xiao-Hui Niu

    2016-01-01

    Background:Resection of sacral chordomas is challenging.The anatomy is complex,and there are often no bony landmarks to guide the resection.Achieving adequate surgical margins is,therefore,difficult,and the recurrence rate is high.Use of computer navigation may allow optimal preoperative planning and improve precision in tumor resection.The purpose of this study was to evaluate the safety and feasibility of computer navigation-aided resection of sacral chordomas.Methods:Between 2007 and 2013,a total of 26 patients with sacral chordoma underwent computer navigation-aided surgery were included and followed for a minimum of 18 months.There were 21 primary cases and 5 recurrent cases,with a mean age of 55.8 years old (range:35-84 years old).Tumors were located above the level of the S3 neural foramen in 23 patients and below the level of the S3 neural foramen in 3 patients.Three-dimensional images were reconstructed with a computed tomography-based navigation system combined with the magnetic resonance images using the navigation software.Tumors were resected via a posterior approach assisted by the computer navigation.Mean follow-up was 38.6 months (range:18-84 months).Results:Mean operative time was 307 min.Mean intraoperative blood loss was 3065 ml.For computer navigation,the mean registration deviation during surgery was 1.7 mm.There were 18 wide resections,4 marginal resections,and 4 intralesional resections.All patients were alive at the final follow-up,with 2 (7.7%) exhibiting tumor recurrence.The other 24 patients were tumor-free.The mean Musculoskeletal Tumor Society Score was 27.3 (range:19-30).Conclusions:Computer-assisted navigation can be safely applied to the resection of the sacral chordomas,allowing execution of preoperative plans,and achieving good oncological outcomes.Nevertheless,this needs to be accomplished by surgeons with adequate experience and skill.

  15. Chordoma. Report on treatment results in eighteen cases

    Energy Technology Data Exchange (ETDEWEB)

    Lybeert, M.L.M.; Meerwaldt, J.H.

    1986-01-15

    Eighteen patients with a proven histologic diagnosis of chordoma were treated between 1949 and 1982. Four patients received only surgery, 4 patients only radiation therapy, and 10 patients received surgery and postoperative radiation therapy to a varying dose. The results suggest that a higher radiation dose gives longer recurrence-free survival, and that the best long term results can be achieved by combining surgery - as radically as possible - with radiation therapy to a dose level of 60 to 65 Gy. In view of the number of marginal recurrences (2 out of the 14 patients who received radiation therapy), the importance of choosing the right treatment volume is stressed.

  16. Cervical chordoma with vertebral artery encasement mimicking neurofibroma: MRI findings

    International Nuclear Information System (INIS)

    A case of cervical chordoma in a 36-year-old white man with hypoesthesia in the neck and right shoulder, neck pain, and restricted neck mobility is presented. Plain radiographs of the cervical spine showed radiolucency of the body of C2 on the right side and enlargement of the right intervertebral foramen at C2-C3 level. Tumor encasement of the vertebral artery was demonstrated by MR imaging and confirmed by conventional arteriography. This proved to be particularly important for preoperative assessment. (orig.)

  17. Multilevel oblique corpectomies as an effective surgical option to treat cervical chordoma in a young girl

    Science.gov (United States)

    Delfini, Roberto; Marruzzo, Daniele; Tarantino, Roberto; Marotta, Nicola; Landi, Alessandro

    2014-01-01

    Chordomas are malignant tumors arising from notochordal remnants. They are the most frequent tumors of the spine after plasmacytomas. Only 6% of chordomas are localized to the cervical level. In young patients, chordomas are rare and unpredictable. Despite this, the treatment of choice remains the total resection, as much as possible, followed by proton beam radiation. This case was managed using a precarotid and retrocarotid approach at the same time. The tumor was completely resected with the edges free from disease. The cervical spine was stabilized with an anterior plating C2-C4. Eighteen months after surgery the patient is still free from illness. Multilevel oblique corpectomies are an available and safe option for the treatment of upper cervical chordomas. PMID:24653986

  18. External radiotherapy plus intracavitary brachytherapy for recurrent chordoma of the nasopharynx

    Energy Technology Data Exchange (ETDEWEB)

    Orecchia, R. [Istituto Europeo di Oncologia, Milan (Italy). Div. of Radiotherapy]|[Milan Univ. (Italy); Leonardi, M.C. [Istituto Europeo di Oncologia, Milan (Italy). Div. of Radiotherapy; Krengli, M. [Istituto Europeo di Oncologia, Milan (Italy). Div. of Radiotherapy]|[Ospedale Maggiore, Novara (Italy). Radiotherapy Div.]|[Torino Univ. (Italy); Zurrida, S. [Istituto Europeo di Oncologia, Milan (Italy). Scientific Director`s Office; Brambilla, M.G. [Istituto Europeo di Oncologia, Milan (Italy). Physics Div.

    1998-09-01

    We report a case of recurrent nasopharyngeal chordoma treated by external beam radiotherapy plus brachytherapy, and discuss this technique in relation to treatment modalities reported in the literature. (orig.)

  19. Magnetic resonance imaging of the intradural prepontine chordoma mimicking an epidermoid cyst: Pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Hyoun; Yu, In Kyu; Kim, Seung Min; Kim, Han Kyu [Eulji Univ. Hospital, Daejeon (Korea, Republic of)

    2012-08-15

    Intracranial chordomas, originating from remnants of the primitive notochord, are extradural tumors arising mostly at the sphenooccipital synchondrosis in the clivus. We present an unusual case of intradural chordoma at the prepontine cistern, with parenchymal compressive invasion to the pons. It was excised subtotally, followed by a second operation due to the increasing remnant tumor size during 8 months. A differential diagnosis for intradural chordoma must be considered when the preoperative MRI features are not consistent with an epidermoid cyst if there are multiple fine enhancing lesions on enhanced magnetic resonance images and no bright signal intensity on diffusion weighted images. This report is concerned with the radiological findings in the intradural chordoma and the differential diagnosis focused on the epidermoid cyst.

  20. The C2 ganglion sectioning epidural approach to craniocervical junction chordoma: A technical case report

    Directory of Open Access Journals (Sweden)

    Naoshi Hagihara

    2012-01-01

    Full Text Available In chordoma, complete surgical removal of the epidural tumor should be the first choice of treatment. Numerous surgical approaches to clival chordoma have been described: anterior approaches, lateral approaches, and posterolateral approaches. A multistaged operation with a combination of these approaches is generally performed. We used three approaches to remove a clival chordoma extending from the lower clivus anteriorly to the anterior perivertebral space and inferiorly to the C2 level. The epidural posterolateral approach through the vertebral artery (VA-C2 interval space after resection of the C2 dorsal ganglion was the most effective. To our knowledge, the epidural posterolateral approach below VA, referred to as C2 ganglion sectioning epidural approach has not been reported as an independent approach in detail. We report a two-year-old girl with a lower clival chordoma which has been excised using C2 ganglion sectioning epidural approach.

  1. Multilevel oblique corpectomies as an effective surgical option to treat cervical chordoma in a young girl.

    Science.gov (United States)

    Delfini, Roberto; Marruzzo, Daniele; Tarantino, Roberto; Marotta, Nicola; Landi, Alessandro

    2014-03-16

    Chordomas are malignant tumors arising from notochordal remnants. They are the most frequent tumors of the spine after plasmacytomas. Only 6% of chordomas are localized to the cervical level. In young patients, chordomas are rare and unpredictable. Despite this, the treatment of choice remains the total resection, as much as possible, followed by proton beam radiation. This case was managed using a precarotid and retrocarotid approach at the same time. The tumor was completely resected with the edges free from disease. The cervical spine was stabilized with an anterior plating C2-C4. Eighteen months after surgery the patient is still free from illness. Multilevel oblique corpectomies are an available and safe option for the treatment of upper cervical chordomas.

  2. Magnetic resonance (MR) imaging of chordoma and chondroma in the skull base; Differential diagnosis by IR sequence

    Energy Technology Data Exchange (ETDEWEB)

    Tashiro, Takahiko; Inoue, Yuichi; Nemoto, Yutaka (Osaka City Univ. (Japan). Faculty of Medicine) (and others)

    1992-05-01

    Differential diagnosis of chordoma and chondroma in the skull base is sometimes difficult. We retrospectively reviewed the MR images of 14 patients with skull base tumors (nine chordomas, four chondromas and one chondrosarcoma). MR imaging was performed with a 0.5 Tesla system (Picker International). Inversion recovery (IR) (2500-2100/600-500/40), T1-weighted spin echo (SE) (800-600/40), and T2-weighted SE (2500-1800/120) images were obtained. On IR images, seven of eight chordomas showed heterogeneous low signal intensity, and one chordoma and all chondromas showed markedly low signal intensity similar to that of CSF. Calcified or ossified portions of the chondromas were demonstrated as areas of moderately low intensity on IR images. Chondrosarcoma showed moderately low intensity similar to that of chordoma. T1-weighted SE images of chordoma and chondroma showed no difference in signal intensity. On T2-weighted SE images, six of nine chordomas and all chondromas showed markedly high signal intensity. Three chordomas and one chondrosarcoma showed moderately high signal intensity. In the diagnosis of skull base tumors, the IR sequence seems to be useful for differentiating chondroma from chordoma. (author).

  3. Metastatic Chordoma: Report of the Two Cases and Review of the Literature

    Science.gov (United States)

    Rohatgi, Saurabh; Ramaiya, Nikhil H; Jagannathan, Jyothi P.; Howard, Stephanie A.; Shinagare, Atul B.; Krajewski, Katherine M.

    2015-01-01

    Chordomas are rare malignant bone tumours with a predilection for the axial skeleton, especially the sacrum and skull base. Median survival in patients with metastatic disease is usually dismal. Treatment is challenging due to the propensity for local recurrence, metastatic disease as well as lack of clear consensus regarding the optimal management. Our case report highlights two cases of sacral chordoma with locally recurrent and widespread metastatic disease, stable on molecular targeted therapy. PMID:26180502

  4. Giant Chordoma of the Upper Thoracic Spine with Mediastinal Involvement: A Surgical Challenge

    Science.gov (United States)

    Rena, Ottavio; Allegra, Giuliano; Casadio, Caterina; Turello, Davide

    2014-01-01

    Thoracic chordomas are very rare malignant tumours originating from notochordal remnants. These tumours develop within a vertebral body and enlarge involving the mediastinal compartment. Because of their slow-growing attitude, they become symptomatic only when they invade or compress the spinal cord and/or mediastinal organs. We present a rare case of a thoracic spine chordoma presenting with increasing paraparesis with a huge mediastinal component which was surgically debulked to decompress the spinal cord and medistinal organs. PMID:24967050

  5. Low expression of PHLPP1 in sacral chordoma and its association with poor prognosis

    Science.gov (United States)

    Chen, Hao; Zhang, Kai; Wu, Guizhong; Song, Dawei; Chen, Kangwu; Yang, Huilin

    2015-01-01

    Sacral chordoma is a rare spine tumor with a high recurrence rate even after optimal therapy. Previous studies have demonstrated that the PI3K/AKT pathway plays a pivotal role in chordoma, and high expression of pAKT is associated with poor prognosis. Recently, PHLPP was recognized to be a tumor suppressor that targets AKT. We analyzed the expression of PHLPP1 and AKT2 in 37 chordoma samples and 11 fetal nucleus pulposus samples by immunohistochemical staining. Of the chordoma cases, 40.5% (15/37) showed strong cytoplasmic staining (score ≥3) for PHLPP1, which was significantly lower than the 90.9% (10/11) of fetal nucleus pulposus samples (P = 0.004). Conversely, strong immunohistochemical staining for AKT2 was observed in 75.7% (28/37) of chordoma samples, which was significantly higher than 36.4% (4/11) of fetal nucleus pulposus (P = 0.021). Kaplan-Meier survival curves and log-rank test showed that patients with high expression of PHLPP1 experienced longer progression free survival time than those with low PHLPP1 expression (P = 0.011). Further multivariate Cox regression analysis indicated that PHLPP1 expression level and surgical approaches were independent risk factors for chordoma recurrence (P = 0.023 and P = 0.022). However, PHLPP1 expression was not statistically related to patients’ total survival time. Conclusively, our results suggest that PHLPP1 plays a crucial role in sacral chordoma, and may be a promising biomarker for prognosis. Meanwhile, manipulation of PHLPP1 expression is also a potential therapeutic approach for the treatment of sacral chordoma. PMID:26823799

  6. Mutation Analysis of Nine Chordoma Specimens by Targeted Next-Generation Cancer Panel Sequencing

    Science.gov (United States)

    Fischer, Carina; Scheipl, Susanne; Zopf, Agnes; Niklas, Norbert; Deutsch, Alexander; Jorgensen, Mette; Lohberger, Birgit; Froehlich, Elke Verena; Leithner, Andreas; Gabriel, Christian; Liegl-Atzwanger, Bernadette; Rinner, Beate

    2015-01-01

    Background: Chordoma is a rare primary malignant bone tumour. Treatment options are mainly restricted to surgical excision, since chordomas are largely resistant to conventional ionising radiation and chemotherapy. Thus, there is a strong need to gain more thorough insights into the molecular biology and genetics of chordoma to allow for the development of new therapeutic options. We performed an ultra-deep sequencing analysis to find novel mutations in cancer associated genes in chordomas to date unseen with Sanger sequencing. Material and Methods: Nine chordomas (skull base (n=3), mobile spine (n=4), and sacrum/coccyx (n=2) were screened for mutations in 48 cancer genes using the Hot Spot Cancer Panel (Illumina). All putative mutations were compared against multiple databases (e.g. NCBI, COSMIC, PolyPhen, EGB, SIFT) and published Copy Number Variation (CNV) data for chordoma. Results: Our results showed mutations with a frequency above 5% in tumorsuppressor- and onco-genes, revealing new possible driver genes for chordomas. We detected three different variants accounting for 11 point mutations in three cancer associated genes (KIT, KDR and TP53). None of the detected mutations was found in all samples investigated. However, all genes affected interact or are connected in pathway analysis. There were no correlations to already reported CNVs in the samples analysed. Conclusions: We identified mutations in the associated genes KIT, KDR, and TP53. These mutations have been described previously and have been predicted to be tolerated. Further results on a larger series are warranted. The driver mechanisms of chordoma still have to be identified. PMID:26366211

  7. Cervical chordoma: a case report; Cordoma cervical: a proposito de un caso

    Energy Technology Data Exchange (ETDEWEB)

    Romera, C.; Wiehoff, A.; Candela, V. P.; Perera, J. [Hospital Universitario Materno-Insular de Canarias. Las Palmas (Spain)

    2002-07-01

    Chordomas, lesions that develop from notochordal remnants, can arise at any site ranging from the clivus to the sacrum: they represent 3% to 4% of all primary bone tumors. We present the cases of a 45-year-old man with cervical chordoma at the C2 level, the site least frequently reported in the literature. We provide the radiological findings resulting from cervical computed tomography and magnetic resonance imaging. (Author) 11 refs.

  8. Giant Petroclival Primary Intradural Chordoma: Case Report and Systematic Review of the Literature

    Science.gov (United States)

    AlOtaibi, Fahad; Guiot, Marie-Christine; Muanza, Thierry; Maio, Salvatore Di

    2014-01-01

    Background Chordomas are rare, locally aggressive neoplasms thought to arise from notochordal remnants in the axial skeleton. Primary intradural chordomas are considered to be extremely rare. In this article a giant intradural petroclival chordoma is presented, and a synthesis of the available literature is performed to measure overall survival (OS) and recurrence-free survival (RFS) and to identify prognostic factors. Methods A systematic Medline review yielded 47 patients with purely intradural tumors from 38 publications including 39 chordomas, 8 cases of ecchordosis physaliphora, and 1 case with features of both. The 5-year OS and RFS were calculated based on the Kaplan-Meier method. Risk factors for progression or mortality were analyzed using binomial logistic regression. Results Maximal tumor diameter varied from 1.5 to 6.0 cm (mean: 3.2 cm). Tumors were located predominantly in the prepontine area (66.7%). Combined 5-year Kaplan-Meier OS and RFS were 77% ± 11% and 74% ± 11%, respectively. Incomplete surgical resection, larger tumor diameter, and an elevated Ki-67 index were statistically more frequent in cases of recurrence and mortality. Conclusions Based on a systematic literature review, the behavior of primary intradural chordomas may be closer to typical chordomas than was previously thought. PMID:25083378

  9. A zebrafish model of chordoma initiated by notochord-driven expression of HRASV12

    Science.gov (United States)

    Burger, Alexa; Vasilyev, Aleksandr; Tomar, Ritu; Selig, Martin K.; Nielsen, G. Petur; Peterson, Randall T.; Drummond, Iain A.; Haber, Daniel A.

    2014-01-01

    Chordoma is a malignant tumor thought to arise from remnants of the embryonic notochord, with its origin in the bones of the axial skeleton. Surgical resection is the standard treatment, usually in combination with radiation therapy, but neither chemotherapeutic nor targeted therapeutic approaches have demonstrated success. No animal model and only few chordoma cell lines are available for preclinical drug testing, and, although no druggable genetic drivers have been identified, activation of EGFR and downstream AKT-PI3K pathways have been described. Here, we report a zebrafish model of chordoma, based on stable transgene-driven expression of HRASV12 in notochord cells during development. Extensive intra-notochordal tumor formation is evident within days of transgene expression, ultimately leading to larval death. The zebrafish tumors share characteristics of human chordoma as demonstrated by immunohistochemistry and electron microscopy. The mTORC1 inhibitor rapamycin, which has some demonstrated activity in a chordoma cell line, delays the onset of tumor formation in our zebrafish model, and improves survival of tumor-bearing fish. Consequently, the HRASV12-driven zebrafish model of chordoma could enable high-throughput screening of potential therapeutic agents for the treatment of this refractory cancer. PMID:24311731

  10. From Notochord Formation to Hereditary Chordoma: The Many Roles of Brachyury

    Directory of Open Access Journals (Sweden)

    Yutaka Nibu

    2013-01-01

    Full Text Available Chordoma is a rare, but often malignant, bone cancer that preferentially affects the axial skeleton and the skull base. These tumors are both sporadic and hereditary and appear to occur more frequently after the fourth decade of life; however, modern technologies have increased the detection of pediatric chordomas. Chordomas originate from remnants of the notochord, the main embryonic axial structure that precedes the backbone, and share with notochord cells both histological features and the expression of characteristic genes. One such gene is Brachyury, which encodes for a sequence-specific transcription factor. Known for decades as a main regulator of notochord formation, Brachyury has recently gained interest as a biomarker and causative agent of chordoma, and therefore as a promising therapeutic target. Here, we review the main characteristics of chordoma, the molecular markers, and the clinical approaches currently available for the early detection and possible treatment of this cancer. In particular, we report on the current knowledge of the role of Brachyury and of its possible mechanisms of action in both notochord formation and chordoma etiogenesis.

  11. A zebrafish model of chordoma initiated by notochord-driven expression of HRASV12

    Directory of Open Access Journals (Sweden)

    Alexa Burger

    2014-07-01

    Full Text Available Chordoma is a malignant tumor thought to arise from remnants of the embryonic notochord, with its origin in the bones of the axial skeleton. Surgical resection is the standard treatment, usually in combination with radiation therapy, but neither chemotherapeutic nor targeted therapeutic approaches have demonstrated success. No animal model and only few chordoma cell lines are available for preclinical drug testing, and, although no druggable genetic drivers have been identified, activation of EGFR and downstream AKT-PI3K pathways have been described. Here, we report a zebrafish model of chordoma, based on stable transgene-driven expression of HRASV12 in notochord cells during development. Extensive intra-notochordal tumor formation is evident within days of transgene expression, ultimately leading to larval death. The zebrafish tumors share characteristics of human chordoma as demonstrated by immunohistochemistry and electron microscopy. The mTORC1 inhibitor rapamycin, which has some demonstrated activity in a chordoma cell line, delays the onset of tumor formation in our zebrafish model, and improves survival of tumor-bearing fish. Consequently, the HRASV12-driven zebrafish model of chordoma could enable high-throughput screening of potential therapeutic agents for the treatment of this refractory cancer.

  12. Cervical chondroid chordoma in a standard dachshund: a case report

    Directory of Open Access Journals (Sweden)

    Stigen Øyvind

    2011-10-01

    Full Text Available Abstract A ten-year-old male standard dachshund was presented with a history of neck pain and progressive gait disturbances. Following a neurological examination and diagnostic imaging, including CT, a neoplastic lesion involving the third and fourth cervical vertebrae was suspected. The lesion included an extradural mass on the right side of the spinal canal causing a local compression of the cervical cord. Surgery, using a modified dorsal laminectomy procedure, was performed in order to decompress the cervical spinal cord. Histopathological examination of the extradural mass indicated that the tumour was a chondroid chordoma. Following discharge, the quality of life for the dog was very good for a sustained period, but clinical signs recurred at 22 months. The dog was euthanased 25 months post-surgery. On post-mortem examination, a regrowth of neoplastic tissue was found to have infiltrated the bone and spinal cord at C3-C4. This is the first report to show that palliative surgery can offer successful long-lasting treatment of chondroid chordoma of the cervical spine in the dog.

  13. Local control of recurrent clival and sacral chordoma after interstitial irradiation with iodine-125: new techniques for treatment of recurrent or unresectable chordomas

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    Kumar, P.P.; Good, R.R.; Skultety, F.M.; Leibrock, L.G.

    1988-03-01

    Using new /sup 125/I brachytherapy techniques, we were able to deliver safely a tumor volume dose of 16,000 rads to a previous irradiated, large, recurrent sacral chordoma by means of the intraoperative interstitial implantation of 229 low activity /sup 125/I seeds and 40,000 rads to a previously irradiated, small, recurrent clival chordoma by means of the transnasal needle implantation of two high activity /sup 125/I seeds. Iodine-125 brachytherapy was followed by regression of tumor, lessening of symptoms, and bony recalcification in both cases.

  14. Proton Therapy for Reirradiation of Progressive or Recurrent Chordoma

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    McDonald, Mark W., E-mail: mmcdona2@iuhealth.org [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Indiana University Health Proton Therapy Center, Bloomington, Indiana (United States); Linton, Okechuckwu R. [Department of Radiation Oncology, Indiana University School of Medicine, Indianapolis, Indiana (United States); Shah, Mitesh V. [Department of Neurosurgery, Indiana University School of Medicine, Indianapolis, Indiana (United States)

    2013-12-01

    Purpose: To report the results in patients reirradiated with proton therapy for recurrent or progressive chordoma, with or without salvage surgery. Methods and Materials: A retrospective review of 16 consecutive patients treated from 2005 to 2012 was performed. All patients had received at least 1 prior course of radiation therapy to the same area, and all but 1 patient had at least 1 surgical resection for disease before receiving reirradiation. At the time of recurrence or progression, half of the patients underwent additional salvage surgery before receiving reirradiation. The median prior dose of radiation was 75.2 Gy (range, 40-79.2 Gy). Six patients had received prior proton therapy, and the remainder had received photon radiation. The median gross tumor volume at the time of reirradiation was 71 cm{sup 3} (range, 0-701 cm{sup 3}). Reirradiation occurred at a median interval of 37 months after prior radiation (range, 12-129 months), and the median dose of reirradiation was 75.6 Gy (relative biological effectiveness [RBE]) (range. 71.2-79.2 Gy [RBE]), given in standard daily fractionation (n=14) or hyperfractionation (n=2). Results: The median follow-up time was 23 months (range, 6-63 months); it was 26 months in patients alive at the last follow-up visit (range, 12-63 months). The 2-year estimate for local control was 85%, overall survival 80%, chordoma-specific survival 88%, and development of distant metastases 20%. Four patients have had local progression: 3 in-field and 1 marginal. Late toxicity included grade 3 bitemporal lobe radionecrosis in 1 patient that improved with hyperbaric oxygen, a grade 4 cerebrospinal fluid leak with meningitis in 1 patient, and a grade 4 ischemic brainstem stroke (out of radiation field) in 1 patient, with subsequent neurologic recovery. Conclusions: Full-dose proton reirradiation provided encouraging initial disease control and overall survival for patients with recurrent or progressive chordoma, although additional

  15. Extended Ultrastructural Characterization of Chordoma Cells: The Link to New Therapeutic Options

    Science.gov (United States)

    Kolb, Dagmar; Pritz, Elisabeth; Steinecker-Frohnwieser, Bibiane; Lohberger, Birgit; Deutsch, Alexander; Kroneis, Thomas; El-Heliebi, Amin; Dohr, Gottfried; Meditz, Katharina; Wagner, Karin; Koefeler, Harald; Leitinger, Gerd; Leithner, Andreas; Liegl-Atzwanger, Bernadette; Zweytick, Dagmar; Rinner, Beate

    2014-01-01

    Chordomas are rare bone tumors, developed from the notochord and largely resistant to chemotherapy. A special feature of this tumor is the heterogeneity of its cells. By combining high pressure freezing (HPF) with electron tomography we were able to illustrate the connections within the cells, the cell-cell interface, and the mitochondria-associated endoplasmic reticulum membrane complex that appears to play a special role among the characteristics of chordoma. These lipid raft-like regions are responsible for lipid syntheses and for calcium signaling. Compared to other tumor cells, chordoma cells show a close connection of rough endoplasmic reticulum and mitochondria, which may influence the sphingolipid metabolism and calcium release. We quantified levels of ceramide and glycosylceramide species by the methyl tert-butyl ether extraction method and we assessed the intracellular calcium concentration with the ratiometric fluorescent dye Fura-2AM. Measurements of the changes in the intracellular calcium concentration revealed an increase in calcium due to the application of acetylcholine. With regard to lipid synthesis, glucosylceramide levels in the chordoma cell line were significantly higher than those in normal healthy cells. The accumulation of glycosylceramide in drug resistant cancer cells has been confirmed in many types of cancer and may also account for drug resistance in chordoma. This study aimed to provide a deep morphological description of chordoma cells, it demonstrated that HPF analysis is useful in elucidating detailed structural information. Furthermore we demonstrate how an accumulation of glycosylceramide in chordoma provides links to drug resistance and opens up the field for new research options. PMID:25479055

  16. Extended ultrastructural characterization of chordoma cells: the link to new therapeutic options.

    Directory of Open Access Journals (Sweden)

    Dagmar Kolb

    Full Text Available Chordomas are rare bone tumors, developed from the notochord and largely resistant to chemotherapy. A special feature of this tumor is the heterogeneity of its cells. By combining high pressure freezing (HPF with electron tomography we were able to illustrate the connections within the cells, the cell-cell interface, and the mitochondria-associated endoplasmic reticulum membrane complex that appears to play a special role among the characteristics of chordoma. These lipid raft-like regions are responsible for lipid syntheses and for calcium signaling. Compared to other tumor cells, chordoma cells show a close connection of rough endoplasmic reticulum and mitochondria, which may influence the sphingolipid metabolism and calcium release. We quantified levels of ceramide and glycosylceramide species by the methyl tert-butyl ether extraction method and we assessed the intracellular calcium concentration with the ratiometric fluorescent dye Fura-2AM. Measurements of the changes in the intracellular calcium concentration revealed an increase in calcium due to the application of acetylcholine. With regard to lipid synthesis, glucosylceramide levels in the chordoma cell line were significantly higher than those in normal healthy cells. The accumulation of glycosylceramide in drug resistant cancer cells has been confirmed in many types of cancer and may also account for drug resistance in chordoma. This study aimed to provide a deep morphological description of chordoma cells, it demonstrated that HPF analysis is useful in elucidating detailed structural information. Furthermore we demonstrate how an accumulation of glycosylceramide in chordoma provides links to drug resistance and opens up the field for new research options.

  17. The FGFR/MEK/ERK/brachyury pathway is critical for chordoma cell growth and survival

    Science.gov (United States)

    Hu, Yunping; Mintz, Akiva; Shah, Sagar R.; Quinones-Hinojosa, Alfredo; Hsu, Wesley

    2014-01-01

    Recent evidence suggests that the expression of brachyury is necessary for chordoma growth. However, the mechanism associated with brachyury-regulated cell growth is poorly understood. Fibroblast growth factor (FGF), a regulator of brachyury expression in normal tissue, may also play an important role in chordoma pathophysiology. Using a panel of chordoma cell lines, we explored the role of FGF signaling and brachyury in cell growth and survival. Western blots showed that all chordoma cell lines expressed fibroblast growth factor receptor 2 (FGFR2), FGFR3, mitogen-activated protein kinase kinase (MEK) and extracellular signal-regulated kinase (ERK), whereas no cell lines expressed FGFR1 and FGFR4. Results of enzyme-linked immunosorbent assay indicated that chordoma cells produced FGF2. Neutralization of FGF2 inhibited MEK/ERK phosphorylation, decreased brachyury expression and induced apoptosis while reducing cell growth. Activation of the FGFR/MEK/ERK/brachyury pathway by FGF2-initiated phosphorylation of FGFR substrate 2 (FRS2)-α (Tyr196) prevented apoptosis while promoting cell growth and epithelial-mesenchymal transition (EMT). Immunofluorescence staining showed that FGF2 promoted the translocation of phosphorylated ERK to the nucleus and increased brachyury expression. The selective inhibition of FGFR, MEK and ERK phosphorylation by PD173074, PD0325901 and PD184352, respectively, decreased brachyury expression, induced apoptosis, and inhibited cell growth and EMT. Moreover, knockdown of brachyury by small hairpin RNA reduced FGF2 secretion, inhibited FGFR/MEK/ERK phosphorylation and blocked the effects of FGF2 on cell growth, apoptosis and EMT. Those findings highlight that FGFR/MEK/ERK/brachyury pathway coordinately regulates chordoma cell growth and survival and may represent a novel chemotherapeutic target for chordoma. PMID:24445144

  18. Sacrococcygeal chordoma: Magnetic resonance imaging and computed tomography

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    Rosenthal, D.I.; Scott, J.A.; Mankin, H.J.; Wismer, G.L.; Brady, T.J.

    1985-07-01

    Magnetic resonance imaging (MRI) was compared to computed tomography (CT) in four cases of sacrococcygeal chordoma. Both techniques yielded important anatomic information and represented important advances over early radiologic imaging methods. MRI provided superior contrast with surrounding soft tissues because of the prolonged T1 and T2 times of the tumors. The direct sagittal images obtained by MRI were valuable in determining the extent of lesions. Either MRI or direct CT coronal images were needed for the demonstration of tumor involving the sacral nerve roots. It was not possible to reliably distinguish between tumor adherent to bowel wall and bowel wall invasion by either technique. It is concluded that MRI is at least equal to CT for demonstration of these lesions and seems likely to become the imaging method of choice.

  19. Skull base chordomas: analysis of dose-response characteristics

    International Nuclear Information System (INIS)

    Objective: To extract dose-response characteristics from dose-volume histograms and corresponding actuarial survival statistics for 115 patients with skull base chordomas. Materials and Methods: We analyzed data for 115 patients with skull base chordoma treated with combined photon and proton conformal radiotherapy to doses in the range 66.6Gy - 79.2Gy. Data set for each patient included gender, histology, age, tumor volume, prescribed dose, overall treatment time, time to recurrence or time to last observation, target dose-volume histogram, and several dosimetric parameters (minimum/mean/median/maximum target dose, percent of the target volume receiving the prescribed dose, dose to 90% of the target volume, and the Equivalent Uniform Dose (EUD). Data were analyzed using the Kaplan-Meier survivor function estimate, the proportional hazards (Cox) model, and parametric modeling of the actuarial probability of recurrence. Parameters of dose-response characteristics were obtained using the maximum likelihood method. Results: Local failure developed in 42 (36%) of patients, with actuarial local control rates at 5 years of 59.2%. The proportional hazards model revealed significant dependence of gender on the probability of recurrence, with female patients having significantly poorer prognosis (hazard ratio of 2.3 with the p value of 0.008). The Wilcoxon and the log-rank tests of the corresponding Kaplan-Meier recurrence-free survival curves confirmed statistical significance of this effect. The Cox model with stratification by gender showed significance of tumor volume (p=0.01), the minimum target dose (p=0.02), and the EUD (p=0.02). Other parameters were not significant at the α level of significance of 0.05, including the prescribed dose (p=0.21). Parametric analysis using a combined model of tumor control probability (to account for non-uniformity of target dose distribution) and the Weibull failure time model (to account for censoring) allowed us to estimate

  20. Expression of programmed cell death ligand 1 (PD-L1) and prevalence of tumor-infiltrating lymphocytes (TILs) in chordoma

    Science.gov (United States)

    Feng, Yong; Shen, Jacson; Gao, Yan; Liao, Yunfei; Cote, Gregory; Choy, Edwin; Chebib, Ivan; Mankin, Henry; Hornicek, Francis; Duan, Zhenfeng

    2015-01-01

    Chordomas are primary malignant tumors of the notochord that are resistant to conventional chemotherapy. Expression of programmed cell death ligand 1 (PD-L1), prevalence of tumor-infiltrating lymphocytes (TILs), and their clinical relevance in chordoma remain unknown. We evaluated PD-L1 expression in three chordoma cell lines and nine chordoma tissue samples by western blot. Immunohistochemical staining was performed on a chordoma tissue microarray (TMA) that contained 78 tissue specimens. We also correlated the expression of PD-L1 and TILs with clinical outcomes. PD-L1 protein expression was demonstrated to be induced by IFN-γ in both UCH1 and UCH2 cell lines. Across nine human chordoma tissue samples, PD-L1 protein was differentially expressed. 94.9% of chordoma samples showed positive PD-L1 expression in the TMA. The expression score of PD-L1 for metastatic chordoma tumors was significant higher as compared with non-metastatic chordoma tumors. Expression of PD-L1 protein significantly correlates with the presence of elevated TILs, which correlates with metastasis. In summary, our study showed high levels of PD-L1 are expressed in chordoma, which is correlated with the prevalence of TILs. The current study suggests targeting PD-L1 may be a novel immunotherapeutic strategy for chordoma clinical trials. PMID:25871477

  1. Factors for tumor progression in patients with skull base chordoma.

    Science.gov (United States)

    Wang, Liang; Tian, Kaibing; Wang, Ke; Ma, Junpeng; Ru, Xiaojuan; Du, Jiang; Jia, Guijun; Zhang, Liwei; Wu, Zhen; Zhang, Junting

    2016-09-01

    Skull base chordoma is a rare and fatal disease, recurrence of which is inevitable, albeit variable. We aimed to investigate the clinicopathologic features of disease progression, identify prognostic factors, and construct a nomogram for predicting progression in individual patients. Data of 229 patients with skull base chordoma treated by one institution between 2005 and 2014 were retrieved and grouped as primary and recurrent. Kaplan-Meier survival of progression was estimated, taking competing risks into account. Multivariable Cox regression was used to investigate survival predictors. The primary group consisted by 183 cases, gained more benefits on 5-year progression-free survival (PFS) (51%) and mean PFS time (66.9 months) than the recurrent group (46 cases), in which 5-year postrecurrent PFS was 14%, and mean postrecurrent PFS time was 29.5 months. In the primary group, visual deficits, pathological subtypes, extent of bone invasion, preoperative Karnofsky performance scale (KPS) score, and variation in perioperative KPS were identified as independent predictors of PFS. A nomogram to predict 3-year and 5-year PFS consisted of these factors, was well calibrated and had good discriminative ability (adjusted Harrell C statistic, 0.68). In the recurrent group, marginal resection (P = 0.018) and adjuvant radiotherapy (P = 0.043) were verified as protective factors associated with postrecurrent PFS. Factors for tumor progression demonstrated some differences between primary and recurrent cases. The nomogram appears useful for risk stratification of tumor progression in primary cases. Further studies will be necessary to identify the rapid-growth histopathological subtype as an independent predictor of rapid progression.

  2. Investigating Microenvironmental Regulation of Human Chordoma Cell Behaviour

    Science.gov (United States)

    Patel, Priya; Brooks, Courtney; Seneviratne, Ayesh; Hess, David A.; Séguin, Cheryle A.

    2014-01-01

    The tumour microenvironment is complex and composed of many different constituents, including matricellular proteins such as connective tissue growth factor (CCN2), and is characterized by gradients in oxygen levels. In various cancers, hypoxia and CCN2 promote stem and progenitor cell properties, and regulate the proliferation, migration and phenotype of cancer cells. Our study was aimed at investigating the effects of hypoxia and CCN2 on chordoma cells, using the human U-CH1 cell line. We demonstrate that under basal conditions, U-CH1 cells express multiple CCN family members including CCN1, CCN2, CCN3 and CCN5. Culture of U-CH1 cells in either hypoxia or in the presence of recombinant CCN2 peptide promoted progenitor cell-like characteristics specific to the notochordal tissue of origin. Specifically, hypoxia induced the most robust increase in progenitor-like characteristics in U-CH1 cells, including increased expression of the notochord-associated markers T, CD24, FOXA1, ACAN and CA12, increased cell growth and tumour-sphere formation, and a decrease in the percentage of vacuolated cells present in the heterogeneous population. Interestingly, the effects of recombinant CCN2 peptide on U-CH1 cells were more pronounced under normoxia than hypoxia, promoting increased expression of CCN1, CCN2, CCN3 and CCN5, the notochord-associated markers SOX5, SOX6, T, CD24, and FOXA1 as well as increased tumour-sphere formation. Overall, this study highlights the importance of multiple factors within the tumour microenvironment and how hypoxia and CCN2 may regulate human chordoma cell behaviour. PMID:25541962

  3. Spot-Scanning-Based Proton Therapy for Extracranial Chordoma

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    Staab, Adrian, E-mail: adrian.staab@psi.ch [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland); Rutz, Hans Peter; Ares, Carmen; Timmermann, Beate; Schneider, Ralf; Bolsi, Alessandra; Albertini, Francesca; Lomax, Antony; Goitein, Gudrun; Hug, Eugen [Center for Proton Therapy, Paul Scherrer Institute, Villigen (Switzerland)

    2011-11-15

    Purpose: To evaluate effectiveness and safety of spot-scanning-based proton-radiotherapy (PT) for extracranial chordomas (ECC). Methods and Material: Between 1999-2006, 40 patients with chordoma of C-, T-, and L-spine and sacrum were treated at Paul Scherrer Institute (PSI) with PT using spot-scanning. Median patient age was 58 years (range, 10-81 years); 63% were male, and 36% were female. Nineteen patients (47%) had gross residual disease (mean 69 cc; range, 13-495 cc) before PT, and 21 patients (53%) had undergone prior titanium-based surgical stabilization (SS) and reconstruction of the axial skeleton. Proton doses were expressed as Gy(RBE). A conversion factor of 1.1 was used to account for higher relative biological effectiveness (RBE) of protons compared with photons. Mean total dose was 72.5 Gy(RBE) [range, 59.4-75.2 Gy(RBE)] delivered at 1.8-2.0 Gy(RBE) dose per fraction. Median follow-up time was 43 months. Results: In 19 patients without surgical stabilization, actuarial local control (LC) rate at 5 years was 100%. LC for patients with gross residual disease but without surgical stabilization was also 100% at 5 years. In contrast, 12 failures occurred in 21 patients with SS, yielding a significantly decreased 5-year LC rate of 30% (p = 0.0003). For the entire cohort, 5-year LC rates were 62%, disease-free survival rates were 57%, and overall survival rates were 80%. Rates were 100% for patients without SS. No other factor, including dosimetric parameters (V95, V80) were predictive for tumor control on univariate analysis. Conclusion: Spot-scanning-based PT at PSI delivered subsequently to function-preserving surgery for tumor debulking, decompression of spinal cord, or biopsy only is safe and highly effective in patients with ECC without major surgical instrumentation even in view of large, unresectable disease.

  4. Investigating microenvironmental regulation of human chordoma cell behaviour.

    Directory of Open Access Journals (Sweden)

    Priya Patel

    Full Text Available The tumour microenvironment is complex and composed of many different constituents, including matricellular proteins such as connective tissue growth factor (CCN2, and is characterized by gradients in oxygen levels. In various cancers, hypoxia and CCN2 promote stem and progenitor cell properties, and regulate the proliferation, migration and phenotype of cancer cells. Our study was aimed at investigating the effects of hypoxia and CCN2 on chordoma cells, using the human U-CH1 cell line. We demonstrate that under basal conditions, U-CH1 cells express multiple CCN family members including CCN1, CCN2, CCN3 and CCN5. Culture of U-CH1 cells in either hypoxia or in the presence of recombinant CCN2 peptide promoted progenitor cell-like characteristics specific to the notochordal tissue of origin. Specifically, hypoxia induced the most robust increase in progenitor-like characteristics in U-CH1 cells, including increased expression of the notochord-associated markers T, CD24, FOXA1, ACAN and CA12, increased cell growth and tumour-sphere formation, and a decrease in the percentage of vacuolated cells present in the heterogeneous population. Interestingly, the effects of recombinant CCN2 peptide on U-CH1 cells were more pronounced under normoxia than hypoxia, promoting increased expression of CCN1, CCN2, CCN3 and CCN5, the notochord-associated markers SOX5, SOX6, T, CD24, and FOXA1 as well as increased tumour-sphere formation. Overall, this study highlights the importance of multiple factors within the tumour microenvironment and how hypoxia and CCN2 may regulate human chordoma cell behaviour.

  5. Chordoma versus chondrosarcoma of the central skull base: MR and CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Guk Myeong; Han, Moon Hee; Chang, Kee Hyun; Kim, Hong Dae; Yeon, Kyung Mo [Seoul National Univ. (Korea, Republic of). Coll. of Medicine; Yu, In Kyu [Uulji Hospital, Seoul (Korea, Republic of); Kim, Sam Soo [Boramae Hospital, Seoul (Korea, Republic of)

    1998-02-01

    It is known that due to both their imaging and pathologic features, the accurate differentiation of chondrosarcoma from chordoma is difficult. Through an analysis of MR and CT finding, this study aims to determine the differential points between these two tumors. In 21 patients, CT and MR imaging studies of chordoma (n=12) and chondrosarcoma (n=9) at the base of the skull were retrospectively reviewed. Diagnosis had been established by histologic examination of surgically removed specimens. Eleven of the chordomas were subclassified as conventional and one as chondroid ; eight chondrosarcoma were conventional and one was myxoid. Four chordoma patients underwent CT and MR ; in six, only MR was in one, only CT was performed. All scans were retrospectively evaluated for the location (midline/off-midline), direction of extension, margin and shape, bony destruction and calcification, MR signal intensity and enhancement patterns of the tumors. Degree of calcification was graded from I to II. Although MR and CT findings were similar in both types of tumor, location and degree of calcification may be features which usefully distinguish chordoma from chondrosarcoma. (author). 17 refs., 2 tabs., 5 figs.

  6. WRN Cys1367Arg polymorphism is not associated with skull base chordoma

    Science.gov (United States)

    WANG, KE; WANG, LIANG; FENG, JIE; HAO, SHUYU; TIAN, KAIBING; WU, ZHEN; ZHANG, LIWEI; JIA, GUIJUN; WAN, HONG; ZHANG, JUNTING

    2014-01-01

    Skull base chordoma is a rare tumor with unknown risk factors. Werner syndrome, which is caused by a mutation in the WRN gene, is a disease of progeria, resembling the pathological process of aging. The present study aimed to provide data on the possible association between skull base chordoma and the single-nucleotide polymorphism (SNP) rs1346044 of the WRN gene. Between July, 2010 and September, 2012, a total of 65 patients with pathologically confirmed skull base chordoma and 65 control subjects were enrolled in this case-control study. The clinical data of the skull base chordoma patients were documented and the rs1346044 site in all the enrolled subjects was analyzed by sequencing and statistically compared using SPSS software. The A allele was the dominant allele of the rs1346044. The comparisons of genotype distributions and allele frequencies did not reveal any significant difference between the groups [P=0.383, 95% confidence interval (CI): 0.346–1.505]. The clinicopathological factors were assessed and no statistically significant difference was observed. In conclusion, the present study suggested that there is no association between rs1346044 SNP and skull base chordomas, at least in the population analyzed. PMID:24944800

  7. Synchronous metastatic skull base chordoma to the breast: case report and literature review

    Science.gov (United States)

    Shakir, S.I.; Pelmus, M.; Florea, A.; Boileau, J.F.; Guiot, M.C.; Di Maio, S.; Muanza, T.M.

    2016-01-01

    Clinical Scenario During routine staging work-up for a left breast mass, a 68-year-old woman complained of dysphagia and dysphonia. During further investigations, a left-sided lesion at the foramen magnum was observed on brain imaging. Both lesions were biopsied and showed a classical chordoma. Management The skull-base lesion and the breast lesion were surgically resected, and adjuvant radiotherapy was given. Summary Chordoma is a rare primary central nervous system tumour that seldom metastasizes. The lung is the most common site of metastasis. Synchronous breast metastasis from a skull-base chordoma is very rare, and a safe management option includes a maximum resection followed by adjuvant radiotherapy. PMID:27122985

  8. Sustained Response of a Clivus Chordoma to Erlotinib after Imatinib Failure

    Science.gov (United States)

    Houessinon, Aline; Boone, Mathieu; Constans, Jean-Marc; Toussaint, Patrick; Chauffert, Bruno

    2015-01-01

    Chordoma is a rare malignant axial tumour that develops from embryonic remnants of the notochord. Surgery and irradiation are the standard initial treatment. However, local recurrence is frequent and cytotoxic chemotherapy is inefficient. Transient activity of imatinib, a platelet-derived growth factor receptor inhibitor, was described in a phase II study. Activity of epidermal growth factor receptor (EGFR) inhibitors (erlotinib, gefitinib) has also been shown in a few recent case reports. We describe a 68-year-old female in whom clivus chordoma recurred after surgery and radiotherapy. The tumour progressed despite imatinib treatment. A partial and sustained response (28+ months) was obtained using erlotinib, an EGFR inhibitor. Erlotinib should be evaluated in a prospective trial investigating new potential therapies against recurrent chordoma. PMID:25762924

  9. Sustained Response of a Clivus Chordoma to Erlotinib after Imatinib Failure

    Directory of Open Access Journals (Sweden)

    Aline Houessinon

    2015-01-01

    Full Text Available Chordoma is a rare malignant axial tumour that develops from embryonic remnants of the notochord. Surgery and irradiation are the standard initial treatment. However, local recurrence is frequent and cytotoxic chemotherapy is inefficient. Transient activity of imatinib, a platelet-derived growth factor receptor inhibitor, was described in a phase II study. Activity of epidermal growth factor receptor (EGFR inhibitors (erlotinib, gefitinib has also been shown in a few recent case reports. We describe a 68-year-old female in whom clivus chordoma recurred after surgery and radiotherapy. The tumour progressed despite imatinib treatment. A partial and sustained response (28+ months was obtained using erlotinib, an EGFR inhibitor. Erlotinib should be evaluated in a prospective trial investigating new potential therapies against recurrent chordoma.

  10. Brachyury: A Diagnostic Marker for the Differential Diagnosis of Chordoma and Hemangioblastoma versus Neoplastic Histological Mimickers

    Science.gov (United States)

    Ieni, Antonio; Branca, Giovanni

    2014-01-01

    Brachyury is a transcription factor which is required for posterior mesoderm formation and differentiation as well as for notochord development during embryogenesis. Due to its expression in the neoplastic cells of chordoma, a malignant tumour deriving from notochordal remnants, but not in tumors showing a similar histology, brachyury has been proposed as a diagnostic marker of this neoplasia. Though commonly considered a hallmark of chordoma, the expression of brachyury has been also documented in the stromal cells of hemangioblastoma (HBL), a slow growing tumor which may involve the central nervous system (CNS) and, rarely, the kidney. Herein we review the role of brachyury immunohistochemical detection in the identification and differential diagnosis of chordoma and HBL towards histological mimickers and suggest that brachyury is added to the panel of immunohistochemical markers for the recognition of HBL in routinary practice, principally in unusual sites. PMID:24591762

  11. Brachyury: A Diagnostic Marker for the Differential Diagnosis of Chordoma and Hemangioblastoma versus Neoplastic Histological Mimickers

    Directory of Open Access Journals (Sweden)

    Valeria Barresi

    2014-01-01

    Full Text Available Brachyury is a transcription factor which is required for posterior mesoderm formation and differentiation as well as for notochord development during embryogenesis. Due to its expression in the neoplastic cells of chordoma, a malignant tumour deriving from notochordal remnants, but not in tumors showing a similar histology, brachyury has been proposed as a diagnostic marker of this neoplasia. Though commonly considered a hallmark of chordoma, the expression of brachyury has been also documented in the stromal cells of hemangioblastoma (HBL, a slow growing tumor which may involve the central nervous system (CNS and, rarely, the kidney. Herein we review the role of brachyury immunohistochemical detection in the identification and differential diagnosis of chordoma and HBL towards histological mimickers and suggest that brachyury is added to the panel of immunohistochemical markers for the recognition of HBL in routinary practice, principally in unusual sites.

  12. DURABLE RESPONSE OF SPINAL CHORDOMA TO COMBINED INHIBITION OF IGF-1R AND EGFR

    Directory of Open Access Journals (Sweden)

    Tamara eAleksic

    2016-05-01

    Full Text Available Chordomas are rare primary malignant bone tumors arising from embryonal notochord remnants of the axial skeleton. Chordomas commonly recur following surgery and radiotherapy, and there is no effective systemic therapy. Previous studies implicated receptor tyrosine kinases including epidermal growth factor receptor (EGFR and type 1 insulin-like growth factor receptor (IGF-1R in chordoma biology. We report an adult female patient who presented in 2003 with spinal chordoma, treated with surgery and radiotherapy. She underwent further surgery for recurrent chordoma in 2008, with subsequent progression in pelvic deposits. In June 2009 she was recruited onto the Phase I OSI-906-103 trial of EGFR inhibitor erlotinib with linsitinib, a novel inhibitor of IGF-1R/insulin receptor (INSR. Treatment with 100mg QD erlotinib and 50mg QD linsitinib was well-tolerated, and after 18 months a partial response was achieved by RECIST criteria. From 43 months a protocol modification allowed intra-patient linsitinib dose-escalation to 50mg BID. The patient remained stable on trial treatment for a total of five years, discontinuing treatment in August 2014. She subsequently experienced further disease progression for which she underwent pelvic surgery in April 2015. Analysis of DNA extracted from 2008 (pre-trial tissue showed that the tumor harbored wild-type EGFR, and a PIK3CA mutation was detected in plasma but not tumor DNA. The 2015 (post-trial tumor harbored a mutation of uncertain significance in ATM, with no detectable mutations in other components of a 50 gene panel including EGFR, PIK3CA and TP53. By immunohistochemistry the tumor was positive for brachyury, the molecular hallmark of chordoma, and showed weak-moderate membrane and cytoplasmic EGFR. IGF-1R was detected in the plasma membrane and cytoplasm and was expressed more strongly in recurrent tumor than the primary. We also noted heterogeneous nuclear IGF-1R, which has been linked with sensitivity to IGF

  13. Durable Response of Spinal Chordoma to Combined Inhibition of IGF-1R and EGFR

    Science.gov (United States)

    Aleksic, Tamara; Browning, Lisa; Woodward, Martha; Phillips, Rachel; Page, Suzanne; Henderson, Shirley; Athanasou, Nicholas; Ansorge, Olaf; Whitwell, Duncan; Pratap, Sarah; Hassan, A. Bassim; Middleton, Mark R.; Macaulay, Valentine M.

    2016-01-01

    Chordomas are rare primary malignant bone tumors arising from embryonal notochord remnants of the axial skeleton. Chordomas commonly recur following surgery and radiotherapy, and there is no effective systemic therapy. Previous studies implicated receptor tyrosine kinases, including epidermal growth factor receptor (EGFR) and type 1 insulin-like growth factor receptor (IGF-1R), in chordoma biology. We report an adult female patient who presented in 2003 with spinal chordoma, treated with surgery and radiotherapy. She underwent further surgery for recurrent chordoma in 2008, with subsequent progression in pelvic deposits. In June 2009, she was recruited onto the Phase I OSI-906-103 trial of EGFR inhibitor erlotinib with linsitinib, a novel inhibitor of IGF-1R/insulin receptor (INSR). Treatment with 100 mg QD erlotinib and 50 mg QD linsitinib was well-tolerated, and after 18 months a partial response was achieved by RECIST criteria. From 43 months, a protocol modification allowed intra-patient linsitinib dose escalation to 50 mg BID. The patient remained stable on trial treatment for a total of 5 years, discontinuing treatment in August 2014. She subsequently experienced further disease progression for which she underwent pelvic surgery in April 2015. Analysis of DNA extracted from 2008 (pre-trial) tissue showed that the tumor harbored wild-type EGFR, and a PIK3CA mutation was detected in plasma, but not tumor DNA. The 2015 (post-trial) tumor harbored a mutation of uncertain significance in ATM, with no detectable mutations in other components of a 50 gene panel, including EGFR, PIK3CA, and TP53. By immunohistochemistry, the tumor was positive for brachyury, the molecular hallmark of chordoma, and showed weak–moderate membrane and cytoplasmic EGFR. IGF-1R was detected in the plasma membrane and cytoplasm and was expressed more strongly in recurrent tumor than the primary. We also noted heterogeneous nuclear IGF-1R, which has been linked with sensitivity

  14. Therapy of multiple metastasizing chordoma of the limb: a case report

    Institute of Scientific and Technical Information of China (English)

    Jing Peng; Li Min; Xianliang Zhang; Hong Duan; Yi Wen; Chongqi Tu

    2013-01-01

    Chordoma is rare, locally aggressive malignant neoplasm originated from notochord remnants. Distant extrapul-monary metastases are rare and mostly occur in patient with local recurrence. In this case report, 2 years after the radical resection of sacral chordoma, two times successive metastases to the upper limb were diagnosed by immunohistochemistry, without local recurrence. The metastatic tumors were radical excised, and the patient gained radiotherapy postoperatively. More than 2 years after the wide resection of the metastatic focuses, no sign of recurrence and metastasis was detected.

  15. Transrectal EUS-guided FNA biopsy of a presacral chordoma-report of a case and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Klaus Gottlieb; Paul H Lin; David M Liu; Karl Anders

    2008-01-01

    Chordomas are rare tumors which originate from the remnants of the notochord.These tumors are locally aggressive and have a predilection for the ends of the axial skeleton.An important prerequisite for optimal management of these tumors is a correct preoperative diagnosis.The present case is the first report of the use of endoscopic ultrasound to obtain transrectal fine needle aspiration biopsy of a presacral chordoma.A review of the prior computer tomography (CT) scans allowed us to calculate the tumor volume doubling time (18.3 mo).Transrectal biopsy of chordomas is controversial,however we believe that such concerns are not justified.

  16. Sacrococcygeal chordoma. A clinicoradiological study of 60 patients

    Energy Technology Data Exchange (ETDEWEB)

    Smith, J.; Ludwig, R.L.; Marcove, R.C.

    1987-01-01

    Sixty patients with sacrococcygeal chordoma, who were seen at this center between 1946 and 1985, were studied with particular attention to the radiographic findings. This study was undertaken because of the large number of these cases and comparison was made between the plain films available in 39 patients and the computed tomography CT studies in 22. Bone destruction was found in 78% on plain films but in 90% on CT. A soft tissue mass was identified in plain films in 60% but in 90% on CT. Calcific debris was found in plain films in 44% but in 87% on CT. Mostly the debris consisted of coarse irregular fragments and probably represented sequestrated necrotic bone. Myelography was performed in only 15 patients. Angiography was studied in 10 cases. Of the 60 patients 88% underwent surgical resection. The tumor recurred in 80% and in only 20% was there no evidence of recurrence. Distant metastases occurred in 24% of patients. Fifty percent survived 5 years; 28% survived 10 years; mean survival 7.5 years.

  17. Prognostic factors in chordoma: role of postoperative radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Thieblemont, C.; Biron, P.; Bouhour, D.; Blay, J.-Y. [Centre Leon Berard, Lyon (France); Rocher, F.; Bobin, J.-Y.; Gerard, J.-P. [Centre Hospitalier Lyon Sud, Pierre-Benite (France)

    1995-12-31

    We have investigated prognostic factors for survival in a series of 26 patients with chordoma treated in Lyon, France, between 1979 and 1993. In this series, the median progression-free (PFS) and overall survival (OS) was 10 and 90 months, respectively. In univariate analysis, PFS, but not OS, was found significantly longer in males as compared to females (median: 19 versus 7 months, P = 0.05); and patients under 60 years of age had a longer PFS (median: 18 versus 6 months; P = 0.06) and OS (median: 108 versus 47 +, P = 0.05) than older patients. A favourable prognostic subgroup including male patients under 60 years and a poor prognostic group including female patients and male over 60 years were thus defined (median PFS: 36 versus 6 months, P = 0.001; median OS: 108 versus 55+, P = 0.15). Primary treatment combining surgery and postoperative radiotherapy associated with a longer PFS than surgery only (median: 36 versus 7 months, P 0.002) in the whole series in both prognostic subgroups. (author).

  18. Aneurysmal Bone Cyst of Sphenoid Bone and Clivus Misdiagnosed as Chordoma: A Case Report

    Science.gov (United States)

    Samanci, Cesur; Asik, Murat; Yanik, Inanc; Ozkanli, Seyma; Tutar, Onur; Hasiloglu, Zehra Isik

    2015-01-01

    Aneurysmal bone cysts (ABCs) are benign and rapidly expanding bone destructive lesions of any bone. They are commonly localized in the metaphysis of long bones, whereas skull base ABCs are rare. We report a case of a 21-year-old man who had been misdiagnosed as chordoma and undergone surgery. However, histopathological examination revealed it to be an ABC. PMID:26605267

  19. Endoscopic laser ablation of clival chordoma with magnetic resonance-guided laser induced thermal therapy

    Directory of Open Access Journals (Sweden)

    James Barrese

    2014-12-01

    Conclusion: The endoscopic endonasal approach to MRI-guided laser ablation is both technically feasible and safe. As a result, this therapy may be a useful alternative in hard-to-reach chordomas, or in recurrent cases that have failed other conventional treatment modalities.

  20. Case report 516: Lumbar vertebral chordoma causing sclerosis of affected vertebra (3rd lumbar vertebra)

    Energy Technology Data Exchange (ETDEWEB)

    Roddie, M.; Adam, A.; Lambert, H.; Pickering, D.; Barker, F.

    1989-01-01

    A case is described of a 61-year-old man with chordoma involving a lumbar vertebra, causing sclerosis of the vertebra without vertebral collapse or a soft tissue mass - a hitherto unreported appearance. CT studies yielded no additional information and the diagnosis was made following needle biopsy of the lumbar vertebra.

  1. A 69-Year-Old Presenting With Musculoskeletal Low Back Pain: A Case of Lumbosacral Chordoma

    Science.gov (United States)

    Williams, Shawn P.; Beckerman, Bernard; Piña Fonti, Maria Elena

    2014-01-01

    Objective The purpose of this case report is to describe the presentation of a patient with lumbosacral chordoma characterized by somatic chronic low back pain and intermittent sacral nerve impingement. Case report A 69-year-old male presenting to an emergency department (ED) with low back pain was provided analgesics and muscle relaxants then referred for a series of chiropractic treatments. Chiropractic treatment included manipulation, physical therapy, and rehabilitation. After 3 times per week for a total of 4 weeks, re-examination showed little relief of his symptoms. His pain symptoms worsened and he presented to the ED for the second time. Magnetic resonance imaging was performed and revealed a high intensity mass. Intervention and outcome The soft tissue mass identified on magnetic resonance imaging was surgically removed. Shortly after the surgery, the patient developed post-operative bleeding and was returned to surgery. During the second procedure, he developed a post-operative hemorrhage related to the development of disseminated intravascular coagulation and subsequently died during the second procedure. A malignant lumbosacral chordoma was diagnosed on pathologic examination. Conclusion This case report describes the presentation of a patient with lumbosacral chordoma presenting with musculoskeletal low back pain. Chordomas are rare with few prominent manifestations. An early diagnosis can potentially make a difference in morbidity and mortality. Due to its insidious nature, it is a difficult diagnosis and one that is often delayed. PMID:25685125

  2. Mobile spine chordoma: results of 166 patients from the AOSpine Knowledge Forum Tumor database

    Science.gov (United States)

    Gokaslan, Ziya L.; Zadnik, Patricia L.; Sciubba, Daniel M.; Germscheid, Niccole; Goodwin, C. Rory; Wolinsky, Jean-Paul; Bettegowda, Chetan; Groves, Mari L.; Luzzati, Alessandro; Rhines, Laurence D.; Fisher, Charles G.; Varga, Peter Pal; Dekutoski, Mark B.; Clarke, Michelle J.; Fehlings, Michael G.; Quraishi, Nasir A.; Chou, Dean; Reynolds, Jeremy J.; Williams, Richard P.; Kawahara, Norio; Boriani, Stefano

    2016-01-01

    Object A chordoma is an indolent primary spinal tumor that has devastating effects on the patient’s life. These lesions are chemoresistant, resistant to conventional radiotherapy, and moderately sensitive to proton therapy; however, en bloc resection remains the preferred treatment for optimizing patient outcomes. While multiple small and largely retrospective studies have investigated the outcomes following en bloc resection of chordomas in the sacrum, there have been few large-scale studies on patients with chordomas of the mobile spine. The goal of this study was to review the outcomes of surgically treated patients with mobile spine chordomas at multiple international centers with respect to local recurrence and survival. This multiinstitutional retrospective study collected data between 1988 and 2012 about prognosis-predicting factors, including various clinical characteristics and surgical techniques for mobile spine chordoma. Tumors were classified according to the Enneking principles and analyzed in 2 treatment cohorts: Enneking-appropriate (EA) and Enneking-inappropriate (EI) cohorts. Patients were categorized as EA when the final pathological assessment of the margin matched the Enneking recommendation; otherwise, they were categorized as EI. Methods Descriptive statistics were used to summarize the data (Student t-test, chi-square, and Fisher exact tests). Recurrence and survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and multivariate Cox proportional hazard modeling. Results A total of 166 patients (55 female and 111 male patients) with mobile spine chordoma were included. The median patient follow-up was 2.6 years (range 1 day to 22.5 years). Fifty-eight (41%) patients were EA and 84 (59%) patients were EI. The type of biopsy (p < 0.001), spinal location (p = 0.018), and if the patient received adjuvant therapy (p < 0.001) were significantly different between the 2 cohorts. Overall, 58 (35%) patients developed local

  3. Chordoma with increased prolactin levels (pseudoprolactinoma mimicking pituitary adenoma: A case report with review of the literature

    Directory of Open Access Journals (Sweden)

    Kumar Pavan

    2009-01-01

    Full Text Available The article deals with a rare case of chordoma with increased prolactin levels. It could often result in a diagnostic dilemma and problems in differentiating it from a pituitary adenoma.

  4. Total resection of the sacral chordoma through posterior approach; Calkowite usuniecie struniaka kosci krzyzowej z dojscia dolnego

    Energy Technology Data Exchange (ETDEWEB)

    Doniec, J.; Pasciak, M.; Folta, K. [Wojewodzki Szpital Chirurgii Urazowej, Piekary Slaskie (Poland)

    1993-12-31

    The case of the sacral chordoma with intrapelvic ingrowth was operated and treated with radiotherapy. Technic of total tumor resection through posterior approach was described. (author). 12 refs, 1 fig.

  5. Overexpression of adenosine deaminase acting on RNA 1 in chordoma tissues is associated with chordoma pathogenesis by reducing miR-125a and miR-10a expression

    Science.gov (United States)

    KUANG, LEI; LV, GUOHUA; WANG, BING; LI, LEI; DAI, YULIANG; LI, YAWEI

    2015-01-01

    Chordoma is a rare, slow-growing primary malignant neoplasm of the axial skeleton, which arises from the remnants of the notochord. Emerging evidence suggests that microRNAs (miRs) are dysregulated in chordoma tissues and crucially involved in chordoma pathogenesis. In the present study, the expression of 11 candidate miRs were analyzed in chordoma tissues and miR-10a and miR-125a were found to be significantly downregulated compared with controls. Notably, the expression of the primary transcripts, pri-miR-125a and pri-miR-10a was unaltered, suggesting that disturbed microRNA expression may be induced by altered pri-miRNA processing. Previous studies have indicated that disturbed adenosine deaminase acting on RNA (ADAR) expression is able to alter mRNA and miRNA adenosine to inosine (A-to-I) levels associated with cancer pathogenesis. Therefore, the expression of ADAR1 and ADAR2 was analyzed in chordoma tissues. It was found that ADAR1 was significantly overexpressed, which was accompanied by enhanced pre-miR-10a and pri-miR-125a A-to-I editing. These findings suggest that ADAR2 overexpression causes enhanced pre-miR-10a and pri-miR-125a A-to-I editing, which alters mature miR-10a and miR-125a expression and may contribute to chordoma pathogenesis. PMID:25673044

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

    Institute of Scientific and Technical Information of China (English)

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

    2015-01-01

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

  7. Overexpression of Raf-1 and ERK1/2 in sacral chordoma and association with tumor recurrence

    Science.gov (United States)

    Zhang, Kai; Chen, Hao; Zhang, Bin; Sun, Jiajia; Lu, Jian; Chen, Kangwu; Yang, Huilin

    2015-01-01

    Chordoma is a rare and low-malignant neoplasm which is considered to arise from notochord remnants. Due to its large resistance to chemotherapy and radiotherapy, surgical resection so far is the prior treatment for chordoma. However, the recurrence rate is high even after complete surgical resection. Recently, targeted cancer therapy has been demonstrated to be effective in several other tumors, while the related research on chordoma is rare. Mitogen-activated protein kinase signaling pathway is acknowledged to participate in tumor development, in which Raf-1 and extracellular regulated protein kinase 1/2 (ERK1/2) play vital roles. In this study, we evaluated the expression of Raf-1 and ERK1/2 by immunohistochemical staining in 42 chordoma tissue and 16 distant normal tissue. Moreover, we also investigated the correlations of Raf-1 and ERK1/2 expression with clinical features in sacral chordoma. Expression of Raf-1 and ERK1/2 was both significantly higher in sacral chordoma tissue than distant normal tissue (P = 0.008, P = 0.019). Raf-1 positive expression was related to surrounding muscle invasion (P = 0.032) and chordoma recurrence (P = 0.002), but the results did not indicate any association with patients’ age, gender, tumor size and location. ERK1/2 was associated with tumor size (P = 0.044) instead of other clinical factors (P > 0.05). Spearman correlation test showed close relation between ERK1/2 and Raf-1 (P = 0.001, r = 0.518). Kaplan–Meier survival Curve and log-rank test showed that Raf-1 positive expression was associated with shorter continuous disease-free survival time (CDFS) (P = 0.001), while ERK1/2 had no relation to CDFS (P = 0.961). Conclusively, Raf-1 may be an important biomarker in predicting the prognosis of chordoma patients. PMID:25755752

  8. Distribution of Age and Location of Chordoma in 39 Cases and Review of Treatment Options

    Directory of Open Access Journals (Sweden)

    Alireza Khoshnevisan

    2012-02-01

    Full Text Available Introduction:Skull base chordomas are rare neoplasms arising from the notochord.Although histologically benign, these tumors are locally aggressive and present significant management challenges. There are some studies on chordoma cases but there was no study about Iranian cases.In this study we evaluated the location, age and gender of the patients with Chordoma in two referral centers in Tehran. Methods: A database of patients with chordoma tumors referred to two centers (Shariati and Imam Hospitals, Tehran from 2001 to 2011 was retrospectively reviewed. Results: In our subjects tumors affect men nearly twice as frequently as women, and they are most commonly diagnosed in middle-aged (mean age was 50.6. Tumors typically occur in the axial skeleton and have a tendency for the spheno-occipital region of the skull base and sacral region. In adults 33.3% of chordomas involve the sacrococcygeal region, 53% occured at the base of the skull near the spheno-occipital area, and near 14% were found in the vertebral column. The cranial nerves mostly affected were abducens, oculomotor and trochlear, with some overlaps. All patients were treated with surgery and some cases referred for gamma-knife radiosurgery (GKS.Discussion:Findings of this study showed more involvement of males compare to females; that is different from other studies, however, few studies reported more male to female ratio. Despite the progress in current surgical techniques and some encouraging results with the use of targeted therapy, disease control and long-term prognosis of patients are still poor.

  9. FAS/FASL are dysregulated in chordoma and their loss-of-function impairs zebrafish notochord formation

    Science.gov (United States)

    Libera, Laura; Boari, Nicola; Mortini, Pietro; Bellipanni, Gianfranco; Giordano, Antonio; Cotelli, Franco; Riva, Paola

    2014-01-01

    Chordoma is a rare malignant tumor that recapitulates the notochord phenotype and is thought to derive from notochord remnants not correctly regressed during development. Apoptosis is necessary for the proper notochord development in vertebrates, and the apoptotic pathway mediated by Fas and Fasl has been demonstrated to be involved in notochord cells regression. This study was conducted to investigate the expression of FAS/FASL pathway in a cohort of skull base chordomas and to analyze the role of fas/fasl homologs in zebrafish notochord formation. FAS/FASL expression was found to be dysregulated in chordoma leading to inactivation of the downstream Caspases in the samples analyzed. Both fas and fasl were specifically expressed in zebrafish notochord sorted cells. fas and fasl loss-of-function mainly resulted in larvae with notochord multi-cell-layer jumps organization, larger vacuolated notochord cells, defects in the peri-notochordal sheath structure and in vertebral mineralization. Interestingly, we observed the persistent expression of ntla and col2a1a, the zebrafish homologs of the human T gene and COL2A1 respectively, which are specifically up-regulated in chordoma. These results demonstrate for the first time the dysregulation of FAS/FASL in chordoma and their role in notochord formation in the zebrafish model, suggesting their possible implication in chordoma onset. PMID:25071022

  10. Enhanced killing of chordoma cells by antibody-dependent cell-mediated cytotoxicity employing the novel anti-PD-L1 antibody avelumab.

    Science.gov (United States)

    Fujii, Rika; Friedman, Eitan R; Richards, Jacob; Tsang, Kwong Y; Heery, Christopher R; Schlom, Jeffrey; Hodge, James W

    2016-06-01

    Chordoma, a rare bone tumor derived from the notochord, has been shown to be resistant to conventional therapies. Checkpoint inhibition has shown great promise in immune-mediated therapy of diverse cancers. The anti-PD-L1 mAb avelumab is unique among checkpoint inhibitors in that it is a fully human IgG1 capable of mediating antibody-dependent cell-mediated cytotoxicity (ADCC) of PD-L1-expressing tumor cells. Here, we investigated avelumab as a potential therapy for chordoma. We examined 4 chordoma cell lines, first for expression of PD-L1, and in vitro for ADCC killing using NK cells and avelumab. PD-L1 expression was markedly upregulated by IFN-γ in all 4 chordoma cell lines, which significantly increased sensitivity to ADCC. Brachyury is a transcription factor that is uniformly expressed in chordoma. Clinical trials are ongoing in which chordoma patients are treated with brachyury-specific vaccines. Co-incubating chordoma cells with brachyury-specific CD8+ T cells resulted in significant upregulation of PD-L1 on the tumor cells, mediated by the CD8+ T cells' IFN-γ production, and increased sensitivity of chordoma cells to avelumab-mediated ADCC. Residential cancer stem cell subpopulations of chordoma cells were also killed by avelumab-mediated ADCC to the same degree as non-cancer stem cell populations. These findings suggest that as a monotherapy for chordoma, avelumab may enable endogenous NK cells, while in combination with T-cell immunotherapy, such as a vaccine, avelumab may enhance NK-cell killing of chordoma cells via ADCC.

  11. EGFR inhibitors identified as a potential treatment for chordoma in a focused compound screen.

    Science.gov (United States)

    Scheipl, Susanne; Barnard, Michelle; Cottone, Lucia; Jorgensen, Mette; Drewry, David H; Zuercher, William J; Turlais, Fabrice; Ye, Hongtao; Leite, Ana P; Smith, James A; Leithner, Andreas; Möller, Peter; Brüderlein, Silke; Guppy, Naomi; Amary, Fernanda; Tirabosco, Roberto; Strauss, Sandra J; Pillay, Nischalan; Flanagan, Adrienne M

    2016-07-01

    Chordoma is a rare malignant bone tumour with a poor prognosis and limited therapeutic options. We undertook a focused compound screen (FCS) against 1097 compounds on three well-characterized chordoma cell lines; 154 compounds were selected from the single concentration screen (1 µm), based on their growth-inhibitory effect. Their half-maximal effective concentration (EC50 ) values were determined in chordoma cells and normal fibroblasts. Twenty-seven of these compounds displayed chordoma selective cell kill and 21/27 (78%) were found to be EGFR/ERBB family inhibitors. EGFR inhibitors in clinical development were then studied on an extended cell line panel of seven chordoma cell lines, four of which were sensitive to EGFR inhibition. Sapitinib (AstraZeneca) emerged as the lead compound, followed by gefitinib (AstraZeneca) and erlotinib (Roche/Genentech). The compounds were shown to induce apoptosis in the sensitive cell lines and suppressed phospho-EGFR and its downstream pathways in a dose-dependent manner. Analysis of substituent patterns suggested that EGFR-inhibitors with small aniline substituents in the 4-position of the quinazoline ring were more effective than inhibitors with large substituents in that position. Sapitinib showed significantly reduced tumour growth in two xenograft mouse models (U-CH1 xenograft and a patient-derived xenograft, SF8894). One of the resistant cell lines (U-CH2) was shown to express high levels of phospho-MET, a known bypass signalling pathway to EGFR. Neither amplifications (EGFR, ERBB2, MET) nor mutations in EGFR, ERBB2, ERBB4, PIK3CA, BRAF, NRAS, KRAS, PTEN, MET or other cancer gene hotspots were detected in the cell lines. Our findings are consistent with the reported (p-)EGFR expression in the majority of clinical samples, and provide evidence for exploring the efficacy of EGFR inhibitors in the treatment of patients with chordoma and studying possible resistance mechanisms to these compounds in vitro and in vivo. © 2016

  12. Resolving Tumor Heterogeneity: Genes Involved in Chordoma Cell Development Identified by Low-Template Analysis of Morphologically Distinct Cells

    Science.gov (United States)

    Wagner, Karin; Meditz, Katharina; Kolb, Dagmar; Feichtinger, Julia; Thallinger, Gerhard G.; Quehenberger, Franz; Liegl-Atzwanger, Bernadette; Rinner, Beate

    2014-01-01

    The classical sacrococcygeal chordoma tumor presents with a typical morphology of lobulated myxoid tumor tissue with cords, strands and nests of tumor cells. The population of cells consists of small non-vacuolated cells, intermediate cells with a wide range of vacuolization and large heavily vacuolated (physaliferous) cells. To date analysis was only performed on bulk tumor mass because of its rare incidence, lack of suited model systems and technical limitations thereby neglecting its heterogeneous composition. We intended to clarify whether the observed cell types are derived from genetically distinct clones or represent different phenotypes. Furthermore, we aimed at elucidating the differences between small non-vacuolated and large physaliferous cells on the genomic and transcriptomic level. Phenotype-specific analyses of small non-vacuolated and large physaliferous cells in two independent chordoma cell lines yielded four candidate genes involved in chordoma cell development. UCHL3, coding for an ubiquitin hydrolase, was found to be over-expressed in the large physaliferous cell phenotype of MUG-Chor1 (18.7-fold) and U-CH1 (3.7-fold) cells. The mannosyltransferase ALG11 (695-fold) and the phosphatase subunit PPP2CB (18.6-fold) were found to be up-regulated in large physaliferous MUG-Chor1 cells showing a similar trend in U-CH1 cells. TMEM144, an orphan 10-transmembrane family receptor, yielded contradictory data as cDNA microarray analysis showed up- but RT-qPCR data down-regulation in large physaliferous MUG-Chor1 cells. Isolation of few but morphologically identical cells allowed us to overcome the limitations of bulk analysis in chordoma research. We identified the different chordoma cell phenotypes to be part of a developmental process and discovered new genes linked to chordoma cell development representing potential targets for further research in chordoma tumor biology. PMID:24503940

  13. Resolving tumor heterogeneity: genes involved in chordoma cell development identified by low-template analysis of morphologically distinct cells.

    Directory of Open Access Journals (Sweden)

    Amin El-Heliebi

    Full Text Available The classical sacrococcygeal chordoma tumor presents with a typical morphology of lobulated myxoid tumor tissue with cords, strands and nests of tumor cells. The population of cells consists of small non-vacuolated cells, intermediate cells with a wide range of vacuolization and large heavily vacuolated (physaliferous cells. To date analysis was only performed on bulk tumor mass because of its rare incidence, lack of suited model systems and technical limitations thereby neglecting its heterogeneous composition. We intended to clarify whether the observed cell types are derived from genetically distinct clones or represent different phenotypes. Furthermore, we aimed at elucidating the differences between small non-vacuolated and large physaliferous cells on the genomic and transcriptomic level. Phenotype-specific analyses of small non-vacuolated and large physaliferous cells in two independent chordoma cell lines yielded four candidate genes involved in chordoma cell development. UCHL3, coding for an ubiquitin hydrolase, was found to be over-expressed in the large physaliferous cell phenotype of MUG-Chor1 (18.7-fold and U-CH1 (3.7-fold cells. The mannosyltransferase ALG11 (695-fold and the phosphatase subunit PPP2CB (18.6-fold were found to be up-regulated in large physaliferous MUG-Chor1 cells showing a similar trend in U-CH1 cells. TMEM144, an orphan 10-transmembrane family receptor, yielded contradictory data as cDNA microarray analysis showed up- but RT-qPCR data down-regulation in large physaliferous MUG-Chor1 cells. Isolation of few but morphologically identical cells allowed us to overcome the limitations of bulk analysis in chordoma research. We identified the different chordoma cell phenotypes to be part of a developmental process and discovered new genes linked to chordoma cell development representing potential targets for further research in chordoma tumor biology.

  14. Image-guided, intensity-modulated radiation therapy (IG-IMRT) for skull base chordoma and chondrosarcoma: preliminary outcomes

    Science.gov (United States)

    Sahgal, Arjun; Chan, Michael W.; Atenafu, Eshetu G.; Masson-Cote, Laurence; Bahl, Gaurav; Yu, Eugene; Millar, Barbara-Ann; Chung, Caroline; Catton, Charles; O'Sullivan, Brian; Irish, Jonathan C.; Gilbert, Ralph; Zadeh, Gelareh; Cusimano, Michael; Gentili, Fred; Laperriere, Normand J.

    2015-01-01

    Background We report our preliminary outcomes following high-dose image-guided intensity modulated radiotherapy (IG-IMRT) for skull base chordoma and chondrosarcoma. Methods Forty-two consecutive IG-IMRT patients, with either skull base chordoma (n = 24) or chondrosarcoma (n = 18) treated between August 2001 and December 2012 were reviewed. The median follow-up was 36 months (range, 3–90 mo) in the chordoma cohort, and 67 months (range, 15–125) in the chondrosarcoma cohort. Initial surgery included biopsy (7% of patients), subtotal resection (57% of patients), and gross total resection (36% of patients). The median IG-IMRT total doses in the chondrosarcoma and chordoma cohorts were 70 Gy and 76 Gy, respectively, delivered with 2 Gy/fraction. Results For the chordoma and chondrosarcoma cohorts, the 5-year overall survival and local control rates were 85.6% and 65.3%, and 87.8% and 88.1%, respectively. In total, 10 patients progressed locally: 8 were chordoma patients and 2 chondrosarcoma patients. Both chondrosarcoma failures were in higher-grade tumors (grades 2 and 3). None of the 8 patients with grade 1 chondrosarcoma failed, with a median follow-up of 77 months (range, 34–125). There were 8 radiation-induced late effects—the most significant was a radiation-induced secondary malignancy occurring 6.7 years following IG-IMRT. Gross total resection and age were predictors of local control in the chordoma and chondrosarcoma patients, respectively. Conclusions We report favorable survival, local control and adverse event rates following high dose IG-IMRT. Further follow-up is needed to confirm long-term efficacy. PMID:25543126

  15. 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.

  16. Cordoma Sacrococcígeo gigante: relato de caso Giant Sacrococcygeal chordoma: case report

    Directory of Open Access Journals (Sweden)

    Tiago Leal Ghezzi

    2009-06-01

    Full Text Available Cordoma sacrococcígeo é uma neoplasia maligna rara que se origina de remanescentes da notocorda. A localização crítica, comportamento localmente agressivo, reconhecida resistência à radioterapia, significativa morbimortalidade cirúrgica e elevada taxa de recidiva tornam seu tratamento um desafio. Descrevemos um caso de cordoma sacrococcígeo gigante.Sacrococcygeal chordoma is a rare malignant neoplasm arised from the remmants of the notochord. The critical localization, locally aggressive behavior, well-known resistance to radiation therapy, meaningful surgical morbimortality and increased recurrence rate become its treatment a challenge. We describe a case of a giant unresectable sacrococcygeal chordoma.

  17. CT and MRI of spheno-occipital chordoma. Radiologic and pathologic correlation

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    Dohi, Michiko; Tanabe, Hiroaki; Mizunuma, Kimiyoshi; Fukuda, Kunihiko; Tada, Shimpei; Nikaidou, Takashi [Jikei Univ., Tokyo (Japan). School of Medicine

    1994-12-01

    CT and MR findings of four patients with sphenooccipital chordoma were reviewed and correlated with histopathologic findings. All cases were delineated as well-defined lobulated masses. Calcifications were seen on CT in three cases out of four. The tumor showed hypo to intermediate intensity on T{sub 1}-weighted images and hyperintensity on T{sub 2}-weighted images, which was thought to be due to rich mucinous or chondroid matrices. Vascular rich fibrous septa and multiple islands of tumor matrices caused septum-like contrast enhancement (CE) on CT and mottled CE on MRI. This difference in CE pattern was probably due to higher contrast resolution of MRI than that of CT. Differential diagnosis between chordoma and chondrosarcoma on CT and MRI is thought to be difficult if chondrosarcoma was originated in the sphenooccipital region. (author).

  18. Atypical Clival Chordoma in an Adolescent without Imaging Evidence of Bone Involvement

    Science.gov (United States)

    HASHIM, Hilwati; ROSMAN, Azmin Kass; ABDUL AZIZ, Aida; ROQIAH, Abdul Kadir; BAKAR, Nor Salmah

    2014-01-01

    Clival chordoma is a rare primary bone tumour that arises from the remnant of the notochord and typically occurs in older adults. Upon imaging, the tumour can be seen arising from the clivus and causes clival destruction. This usually provides insight for a diagnosis. Here we present a case of a non-enhancing, pre-pontine mass that was hypointense on T1W and hyperintense on T2W in an adolescent. No clival bone erosion was observed. Based on the age group, imaging findings, and lack of clival erosion, a provisional diagnosis of epidermoid cyst was made and the tumour was resected. This patient was eventually diagnosed with a clival chordoma based on histopathological examination. PMID:25977639

  19. A Parapharyngeal Soft Tissue Chordoma Presenting with Synchronous Cervical Lymph Node Metastasis: An Unusual Presentation.

    Science.gov (United States)

    Khurram, S A; Biswas, D; Fernando, M

    2016-09-01

    A 63 year old male presented with a three month history of dysphagia, neck swelling and an oropharyngeal swelling on examination. Initial fine needle aspiration cytology and magnetic resonance imaging (done at a peripheral hospital) suggested a salivary gland neoplasm with lymph node metastasis. An infra-temporal approach was employed to excise the tumour mass and a modified radical neck dissection undertaken to remove the cervical metastasis. Histopathological examination with immunohistochemistry confirmed this to be a soft tissue chordoma. To the best of our knowledge, this is the first documented report of an extra-axial soft tissue chordoma presenting with synchronous metastatic disease. Though rare, this adds to the list of differential diagnoses for complex parapharyngeal lesions. A multidisciplinary approach between head and neck surgery, histopathology, radiology and sarcoma teams is paramount for arriving at the correct diagnosis and to deliver optimal treatment.

  20. Computed tomography and magnetic resonance imaging of thoracic chordoma in a Bengal tiger (Panthera tigris tigris)

    Science.gov (United States)

    ISERI, Toshie; SHIMIZU, Junichiro; AKIYOSHI, Hideo; KUSUDA, Kayo; HAYASHI, Akiyoshi; MIE, Keiichiro; IZAWA, Takeshi; KUWAMURA, Mitsuru; YAMATE, Jyoji; FUJIMOTO, Yuka; OHASHI, Fumihito

    2015-01-01

    A Bengal tiger was presented for evaluation of weakness, ataxia and inappetance. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a mass extending from the T7-8 vertebral body to the left rib and compressing the spinal cord. On CT, the bone destruction and sequestrum were shown. On MRI, the multilobulated mass appeared hypo- to isointense in T1-weighted and hyperintense in T2-weighted images. The tiger died after imaging, most likely from renal failure. Chordoma without metastasis was diagnosed on necropsy. The imaging characteristics were similar to those found in chordoma in humans. This report describes the use of CT and MRI in an exotic species. PMID:25754498

  1. Image-guided percutaneous lipiodol-pingyangmycin suspension injection therapy for sacral chordoma

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    Huang, Dexiao; Chen, Yong; Zeng, Qingie; Li, Yanhao [Dept. of Interventional Radiology, Nanfang Hospital, Southern Medical University, Guangzhou (China); Wu, Renhua [Dept. of of Medical Imaging, the 2nd Affiliated Hospital, Medical College of Shantou University, Shantou (China)

    2013-10-15

    A 74-year-old man presented with a progressively worsening pain in sacrum and was diagnosed to have a sacral chordoma by biopsy in May, 2004. Percutaneous intratumoral injection with lipiodol-pingyangmycin suspension (LPS) was carried out under image guidance and repeated when the pain in sacrum recurred and the tumor increased. During a 6-year follow-up period, three sessions of this treatment were executed. CT imaging and Karnofsky Performance Score were used to evaluate the size of tumor and quality of life, respectively. The patient was free of pain after each procedure and had a high quality of life with a Karnofsky Performance Score above 80 points. The tumor lesion in sacral area was effectively controlled. No complications were observed. Percutaneous intratumoral injection with LPS under image guidance may be an effective and safe alternative for the patients with sacral chordoma.

  2. A Parapharyngeal Soft Tissue Chordoma Presenting with Synchronous Cervical Lymph Node Metastasis: An Unusual Presentation.

    Science.gov (United States)

    Khurram, S A; Biswas, D; Fernando, M

    2016-09-01

    A 63 year old male presented with a three month history of dysphagia, neck swelling and an oropharyngeal swelling on examination. Initial fine needle aspiration cytology and magnetic resonance imaging (done at a peripheral hospital) suggested a salivary gland neoplasm with lymph node metastasis. An infra-temporal approach was employed to excise the tumour mass and a modified radical neck dissection undertaken to remove the cervical metastasis. Histopathological examination with immunohistochemistry confirmed this to be a soft tissue chordoma. To the best of our knowledge, this is the first documented report of an extra-axial soft tissue chordoma presenting with synchronous metastatic disease. Though rare, this adds to the list of differential diagnoses for complex parapharyngeal lesions. A multidisciplinary approach between head and neck surgery, histopathology, radiology and sarcoma teams is paramount for arriving at the correct diagnosis and to deliver optimal treatment. PMID:26984125

  3. Sacrococcygeal chordoma in infancy showing an aggressive clinical course: an autopsy case report.

    Science.gov (United States)

    Shinmura, Yuichiro; Miura, Katsutoshi; Yajima, Shuhei; Tsutsui, Yoshihiro

    2003-07-01

    The autopsy case of a 3-year 6-month-old boy with chordoma arising in the sacrococcygeal region is presented. The primary lesion of the sacrococcygeal area was unresectable and lung metastasis was detected. He was treated with multi-agent systemic chemotherapy and radiation therapy, but the tumor was less responsive to these therapies. He died about one year after first admission. An autopsy revealed a massive sacrococcygeal mass and metastasis in the thoracic and lumbar vertebrae, retroperitoneal and mediastinal lymph nodes, and also in the bilateral lungs and liver. Histologically, the tumor was composed of 'pink' cells and scattered 'physaliphorous' cells with a myxoid matrix. Sacrococcygeal chordoma in infancy is very rare. Our case showed a highly aggressive clinical course.

  4. Value of MRI in the diagnosis of non-clival, non-sacral chordoma

    Energy Technology Data Exchange (ETDEWEB)

    Smolders, D.; Wang, X.; Vanhoenacker, F.; De Schepper, A.M. [Department of Radiology, University Hospital Antwerp, Wilrijkstraat 10, 2650, Edegem (Belgium); Drevelengas, A. [Department of Radiology, University of Thessaloniki, Thessaloniki (Greece)

    2003-06-01

    To determine the MR features of non-sacral, non-clival chordoma and to describe a MR prototype of the lesion.Design and patients We reviewed the MR findings of 10 patients with a histologically proven chordoma (6 cervical spine, 1 thoracic spine, 3 lumbar spine). There were three female and seven male patients. Age ranged from 12 to 66 years with a mean age of 44.6 years. The MR images were reviewed for signal intensity (SI) and morphology. All lesions showed a soft tissue extension spanning several vertebral segments. Most of the lesions exhibited a so-called collar button appearance (sagittal images). Two cases of cervical chordoma displayed a ''dumbbell morphology'' (axial images) or ''mushroom'' appearance without bone involvement and with enlargement of the neuroforamen mimicking a neurogenic tumor. Although the region of the nucleus pulposus is the last part of the fetal notochord in the adult to involute, disks were surprisingly spared in all patients. Eight of 10 patients showed heterogeneous SI on all sequences. The overall SI of all lesions was isointense or slightly higher than that of muscle on T1-weighted images. All lesions exhibited high SI on T2-weighted images. After gadolinium contrast administration there was a moderate enhancement in most cases. Although the SI on MR imaging is not specific, chordoma should be considered when a destructive lesion of a vertebral body is associated with a soft tissue mass with a collar button or mushroom appearance and dumbbell morphology, spanning several vertebral segments and sparing the disk(s). (orig.)

  5. The role of radiosurgery in the management of chordoma and chondrosarcoma of the cranial base

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    Kondziolka, D.; Lunsford, L.D.; Flickinger, J.C. (Presbyterian University Hospital, University of Pittsburgh, PA (USA))

    1991-07-01

    Despite conventional multimodality treatment (surgery and fractionated radiation therapy), recurrence and clinical progression of cranial base chordomas and chondrosarcomas are common. The malignant behavior of these tumors is a result of their critical location, locally aggressive nature, and high recurrence rate. To explore the role of radiosurgery in the treatment of these skull base neoplasms, we assessed its use in four patients with chordoma and two with chondrosarcoma. In five of the patients, radiosurgery was used as adjuvant therapy for residual or recurrent tumors after surgical debulking, and in one patient with a chordoma, it was the primary treatment. No patient received fractionated external beam radiotherapy. All tumors were less than 30 mm in diameter and were treated with 20 Gy to the tumor margin. Skull base computed tomography and magnetic resonance images were essential to define the anatomic relationships between tumor and adjacent basal structures. During follow-up (mean, 22 mo; range, 8-36 mo), the authors found no progression of the treated tumor volume in any patient. Neurological deficits before treatment improved in three patients; the other three patients remained in stable neurological condition. Serial follow-up imaging studies demonstrated that two patients showed reduction in tumor size and four patients had no tumor growth. In one patient, a metastatic parietal lobe chondrosarcoma developed and was treated by microsurgery. Another patient showed tumor progression outside of the radiosurgical treatment volume. The authors results attest to the value of stereotactic radiosurgery as an adjuvant or primary treatment for selected patients with chordoma or chondrosarcoma and demonstrate its potential advantages over standard fractionated irradiation. Analysis of the long-term clinical and imaging effects after radiosurgery is warranted.

  6. MicroRNA-608 and MicroRNA-34a Regulate Chordoma Malignancy by Targeting EGFR, Bcl-xL and MET

    Science.gov (United States)

    Zhang, Ying; Schiff, David; Park, Deric; Abounader, Roger

    2014-01-01

    Chordomas are rare malignant tumors that originate from the notochord remnants and occur in the skull base, spine and sacrum. Due to a very limited understanding of the molecular pathogenesis of chordoma, there are no adjuvant and molecular therapies besides surgical resection and radiation therapy. microRNAs (miRNAs) are small noncoding regulatory RNA molecules with critical roles in cancer. The role of miRNAs in chordomas is mostly unknown. We uncover microRNA-608 (miR-608) and microRNA-34a (miR-34a) as novel tumor suppressive microRNAs that regulate malignancy in chordoma. We find that miR-608 and miR-34a expressions are downregulated in human chordoma cell lines and primary cells at least partially via alteration of their genes’ copy numbers. We identify the commonly deregulated oncogenes EGFR and Bcl-xL as direct targets of miR-608 and the receptor tyrosine kinase MET as direct target of miR-34a. We show that EGFR and MET activations promote chordoma cell proliferation and invasion and that pharmacological inhibition of EGFR and MET inhibits chordoma cell proliferation and survival. We demonstrate that restoration of miR-608 and miR-34a inhibits cell proliferation and invasion and induces apoptosis in chordoma cells. We find that miR-34a inversely correlates with MET expression and miR-608 inversely correlates with EGFR expression in chordoma cells. These findings demonstrate for the first time that miR-608 and miR-34a regulate chordoma malignancy by regulating EGFR, MET and Bcl-xL. PMID:24621885

  7. MicroRNA-608 and microRNA-34a regulate chordoma malignancy by targeting EGFR, Bcl-xL and MET.

    Directory of Open Access Journals (Sweden)

    Ying Zhang

    Full Text Available Chordomas are rare malignant tumors that originate from the notochord remnants and occur in the skull base, spine and sacrum. Due to a very limited understanding of the molecular pathogenesis of chordoma, there are no adjuvant and molecular therapies besides surgical resection and radiation therapy. microRNAs (miRNAs are small noncoding regulatory RNA molecules with critical roles in cancer. The role of miRNAs in chordomas is mostly unknown. We uncover microRNA-608 (miR-608 and microRNA-34a (miR-34a as novel tumor suppressive microRNAs that regulate malignancy in chordoma. We find that miR-608 and miR-34a expressions are downregulated in human chordoma cell lines and primary cells at least partially via alteration of their genes' copy numbers. We identify the commonly deregulated oncogenes EGFR and Bcl-xL as direct targets of miR-608 and the receptor tyrosine kinase MET as direct target of miR-34a. We show that EGFR and MET activations promote chordoma cell proliferation and invasion and that pharmacological inhibition of EGFR and MET inhibits chordoma cell proliferation and survival. We demonstrate that restoration of miR-608 and miR-34a inhibits cell proliferation and invasion and induces apoptosis in chordoma cells. We find that miR-34a inversely correlates with MET expression and miR-608 inversely correlates with EGFR expression in chordoma cells. These findings demonstrate for the first time that miR-608 and miR-34a regulate chordoma malignancy by regulating EGFR, MET and Bcl-xL.

  8. Identification of miR-140-3p as a marker associated with poor prognosis in spinal chordoma

    Science.gov (United States)

    Zou, Ming-Xiang; Huang, Wei; Wang, Xiao-Bin; Lv, Guo-Hua; Li, Jing; Deng, You-Wen

    2014-01-01

    Objective: To investigate the expression profile of miR-140-3p in formalin-fixed paraffin-embedded (FFPE) tissues of spinal chordoma, and its correlation with the prognosis of spinal chordoma patients. Methods: Dysregulated miRNAs in FFPE tissues of spinal chordoma were identified by microarray analysis. MiR-140-3p expression in surgically removed spinal chordoma tissues of 42 spinal chordoma patients (27 males and 15 females, aged 29-76 years) and corresponding nucleus pulposus tissues of 14 patients with disc herniation as the healthy control group (8 males and 6 females, aged 24-73 years) was measured by real-time quantitative RT-PCR assay. The association of miR-140-3p expression with clinicopathologic characteristics of spinal chordoma patients was analyzed. Additionally, we investigated the prognostic significance of miR-140-3p with the use of Kaplan-Meier methods and a Cox proportional hazard model. Results: The expression of miR-140-3p was significantly higher in chordoma tissues than nucleus pulposus tissues (t = 3.530, P = 0.001). The expression of miR-140-3p positively correlated with surrounding muscle invasion. The Kapan-Meier survival analysis showed that the patients with high miR-140-3p expression had a significantly worse recurrence-free survival than those with a low expression (χ 2 = 31.270, P = 0.000, log-rank test). In addition, univariate and multivariate analyses for recurrence-free survival showed that miR-140-3p expression was an independent prognostic factor for patients with spinal chordoma (HR = 1.361, 95% CI: 1.135-1.633, P = 0.001). Conclusion: Over-expression of miR-140-3p is correlated with recurrence and tumor invasion, suggesting that miR-140-3p could be a new predictor for recurrence and prognosis in patients with spinal chordoma. PMID:25197358

  9. Clival Ectopic Pituitary Adenoma Mimicking a Chordoma: Case Report and Review of the Literature.

    Science.gov (United States)

    Karras, Constantine L; Abecassis, Isaac Josh; Abecassis, Zachary A; Adel, Joseph G; Bit-Ivan, Esther N; Chandra, Rakesh K; Bendok, Bernard R

    2016-01-01

    Background. Purely ectopic pituitary adenomas are exceedingly rare. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Case Presentation. A 41-year-old male presented with an incidental clival lesion presumed to be a chordoma. On MRI it involved the entire clivus, extended laterally to the petroclival junction, and invaded the cavernous sinuses bilaterally, encasing both internal carotid arteries, without direct extension into the sella. Intraoperatively, it was clear that the tumor originated from the clivus and that the sellar dura was completely intact. Frozen-section pathology was consistent with a pituitary adenoma. Immunostaining was positive for synaptophysin and prolactin with a low Ki-67 index, suggestive of a prolactinoma. Additional immunohistochemical stains seen in chordomas (EMA, S100, and Brachyury) and other metastatic tumors were negative. A postoperative endocrine workup revealed an elevated serum prolactin of 881.3 ng/mL (normal < 20). Conclusions. In conclusion, it is crucial to maintain an extensive differential diagnosis when evaluating a patient with a clival lesion. Ectopic clival pituitary adenomas, although rare, may warrant an endocrinological workup preoperatively as the majority may respond to medical treatment. PMID:26885420

  10. En bloc resection of a C2-C3 upper cervical chordoma: Technical note

    Directory of Open Access Journals (Sweden)

    Alexander G Weil

    2015-01-01

    Full Text Available Background: Recently, en bloc spondylectomy for upper cervical chordomas has been reported. Most authors utilize the combined approaches (e.g., transoral tumor resection with anterior column reconstruction and primary pharyngeal closure without up-front flap repair. However, the 60% incidence of posterior pharyngeal wall dehiscence delays oral intake, typically requires an additional surgery (e.g. free-flap, and delays radiation therapy. Methods: Here, we demonstrate the efficacy of en bloc C2-C3 spondylectomy for the treatment of upper cervical chordomas utilizing a combined transoral followed by posterior approach. We used a novel anterior de-epithelialized submental island flap (SIF as an underlay graft between the pharyngeal wall and cage/hardware to prevent pharyngeal wound dehiscence. Results: Despite a small pharyngeal fistula, the construct healed and the patient was disease-free 40 months later. Conclusion: En bloc C2-C3 spondylectomy for the treatment of an upper cervical chordoma typically requires a combined transoral and posterior approaches. This required utilization of an anterior SIF to promote adequate wound healing. This maneuver avoided incurring the typical complications of combined approaches (e.g. transoral tumor resection with anterior column reconstruction and primary pharyngeal closure without up-front flap repair.

  11. En bloc resection of a C2–C3 upper cervical chordoma: Technical note

    Science.gov (United States)

    Weil, Alexander G.; Shehadeh, Mohammed; Ayad, Tareck; Abboud, Olivier; Shedid, Daniel

    2015-01-01

    Background: Recently, en bloc spondylectomy for upper cervical chordomas has been reported. Most authors utilize the combined approaches (e.g., transoral tumor resection with anterior column reconstruction and primary pharyngeal closure without up-front flap repair). However, the 60% incidence of posterior pharyngeal wall dehiscence delays oral intake, typically requires an additional surgery (e.g. free-flap), and delays radiation therapy. Methods: Here, we demonstrate the efficacy of en bloc C2–C3 spondylectomy for the treatment of upper cervical chordomas utilizing a combined transoral followed by posterior approach. We used a novel anterior de-epithelialized submental island flap (SIF) as an underlay graft between the pharyngeal wall and cage/hardware to prevent pharyngeal wound dehiscence. Results: Despite a small pharyngeal fistula, the construct healed and the patient was disease-free 40 months later. Conclusion: En bloc C2–C3 spondylectomy for the treatment of an upper cervical chordoma typically requires a combined transoral and posterior approaches. This required utilization of an anterior SIF to promote adequate wound healing. This maneuver avoided incurring the typical complications of combined approaches (e.g. transoral tumor resection with anterior column reconstruction and primary pharyngeal closure without up-front flap repair). PMID:26693391

  12. Sacral chordoma in an adult showing an aggressive clinical course: A case report

    Science.gov (United States)

    ENDO, KOJI; YAMASHITA, HIDEKI; NAGASHIMA, HIDEKI; TESHIMA, RYOTA

    2014-01-01

    The current report presents a case of a 78-year-old male with sacral chordoma, showing an aggressive clinical course. The patient underwent sacral resection, however, nine months later, multiple metastases were detected by magnetic resonance imaging. The metastases progressed rapidly and 15 months following surgery the patient succumbed to respiratory dysfunction. An autopsy revealed multiple metastases of the lung, liver, heart, kidneys and vertebrae. Pathologically, the tumors did not show proliferation of anaplastic cells or dedifferentiation; however, the metastatic tumor cells were smaller than the primary tumor cells. The Ki-67 labeling indices were <5% in all of the patient’s tumors, therefore, the capacity for cellular proliferation of the tumors was considered to be low. Chordoma in adults are generally slow-growing tumors and are associated with a relatively prolonged course and frequent local recurrences. Therefore, it must be recognized that chordoma may grow rapidly and show an aggressive clinical course, even when the Ki-67 labeling index is low. PMID:24765153

  13. Development of transplantable human chordoma xenograft for preclinical assessment of novel therapeutic strategies

    Science.gov (United States)

    Bozzi, Fabio; Manenti, Giacomo; Conca, Elena; Stacchiotti, Silvia; Messina, Antonella; Dagrada, GianPaolo; Gronchi, Alessandro; Panizza, Pietro; Pierotti, Marco A.; Tamborini, Elena; Pilotti, Silvana

    2014-01-01

    Background Chordomas are rare and indolent bone tumors that arise in the skull base and mobile spine. Distant metastases occur in >20% of cases, but morbidity and mortality are mainly related to local relapses that affect the majority of patients. Standard chemotherapy has modest activity, whereas new targeted therapies (alone or in combination) have some activity in controlling disease progression. However, the scarcity of preclinical models capable of testing in vivo responses to these therapies hampers the development of new medical strategies. Methods In this study, 8 chordoma samples taken from 8 patients were implanted in nude mice. Four engrafted successfully and gave rise to tumor masses that were analyzed histologically, by means of fluorescence in situ hybridization and biochemical techniques. The data relating to each of the mouse tumors were compared with those obtained from the corresponding human tumor. Results All 4 engraftments retained the histological, genetic and biochemical features of the human tumors they came from. In one epidermal growth factor receptor(EGFR)-positive xenograft, responsiveness to lapatinib was evaluated by comparing the pre- and posttreatment findings. The treatment induced a low-level, heterogeneous switching off of EGFR and its downstream signaling effectors. Conclusions Overall, this model is very close to human chordoma and represents a new means of undertaking preclinical investigations and developing tailored therapies. PMID:24366975

  14. Clival Ectopic Pituitary Adenoma Mimicking a Chordoma: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Constantine L. Karras

    2016-01-01

    Full Text Available Background. Purely ectopic pituitary adenomas are exceedingly rare. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Case Presentation. A 41-year-old male presented with an incidental clival lesion presumed to be a chordoma. On MRI it involved the entire clivus, extended laterally to the petroclival junction, and invaded the cavernous sinuses bilaterally, encasing both internal carotid arteries, without direct extension into the sella. Intraoperatively, it was clear that the tumor originated from the clivus and that the sellar dura was completely intact. Frozen-section pathology was consistent with a pituitary adenoma. Immunostaining was positive for synaptophysin and prolactin with a low Ki-67 index, suggestive of a prolactinoma. Additional immunohistochemical stains seen in chordomas (EMA, S100, and Brachyury and other metastatic tumors were negative. A postoperative endocrine workup revealed an elevated serum prolactin of 881.3 ng/mL (normal < 20. Conclusions. In conclusion, it is crucial to maintain an extensive differential diagnosis when evaluating a patient with a clival lesion. Ectopic clival pituitary adenomas, although rare, may warrant an endocrinological workup preoperatively as the majority may respond to medical treatment.

  15. Clinical Value of Interventional Embolization for Sacral Chordoma before Surgical Treatment

    Institute of Scientific and Technical Information of China (English)

    Zhihui CHANG; Zhaoyu LIU; Jiahe Zheng; Zaiming LU; Qiyong GUO

    2011-01-01

    Objetive:To evaluate the efficacy of preoperative transcatheter arterial embolization in the treatment of sacral chordoma,and to explore the choice of embolization timing. Methods:32 patients underwent the posterior approach after TAE of the main arteries that supplied the sacral chordoma. Intraoperative bleeding amount of each patient was recorded and compared between-group differences at different operation times. Results: After embolization, 12 patients were received resection within 24 hours (group A),that of 10 cases between 24 ~ 48h (group B), of another 10 cases between 48~ 72h (group C). All of the 32 tumors were removed intact with intraoperative bleeding about (894±199)ml, without any shock or death,nor injuries on abdominal organs such as rectum and ureter. There was no statistical significance in tumor size among group A,B and C (P>0.05). Data gave statistical significance in intraoperative blood loss between group A and B (P<0.01) ,there was no statistical differences between group B and C (P>0.05), in spite of group B slightly less than group C. Conclusion: Preoperative TAE for excising the sacral tumor can significantly decrease intraoperative blood loss, make the surgical field clear,and facilitate the maximal removal of the sacral chordoma. It would be best to select the embolization timing within 24 hours before surgical operation.

  16. Cordoma de ápice petroso: relato de um caso Petrous apex chordoma: a case report

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    Cláudio Régis S. Silveira

    2001-02-01

    Full Text Available Cordomas são neoplasias raras que se originam dos remanescentes da notocorda primitiva. Estes remanescentes persistem ao longo de todo o esqueleto axial. Os cordomas intracranianos, mais freqüentemente, se localizam no clivus, próximo à sincondrose esfenooccipital, tipicamente na linha média. Nós descrevemos um caso atípico de cordoma fora da linha média, mais especificamente no ápice petroso, e discutimos as causas embriológicas que determinam esta localização, bem como sintomas, achados de imagem, tratamento cirúrgico e evolução.Chordomas are rare neoplasms arising from notochordal remnants that persist along the axial skeleton. Intracranial chordomas occur more frequently in the midline. We describe an atypical case of an off-midline chordoma arising from the petrous apex, and discuss the embryogenic factors which determine that location, as well as the symptoms, imaging findings, surgical treatment and evolution.

  17. Clival Ectopic Pituitary Adenoma Mimicking a Chordoma: Case Report and Review of the Literature

    Science.gov (United States)

    Karras, Constantine L.; Abecassis, Isaac Josh; Abecassis, Zachary A.; Adel, Joseph G.; Bit-Ivan, Esther N.; Chandra, Rakesh K.; Bendok, Bernard R.

    2016-01-01

    Background. Purely ectopic pituitary adenomas are exceedingly rare. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Case Presentation. A 41-year-old male presented with an incidental clival lesion presumed to be a chordoma. On MRI it involved the entire clivus, extended laterally to the petroclival junction, and invaded the cavernous sinuses bilaterally, encasing both internal carotid arteries, without direct extension into the sella. Intraoperatively, it was clear that the tumor originated from the clivus and that the sellar dura was completely intact. Frozen-section pathology was consistent with a pituitary adenoma. Immunostaining was positive for synaptophysin and prolactin with a low Ki-67 index, suggestive of a prolactinoma. Additional immunohistochemical stains seen in chordomas (EMA, S100, and Brachyury) and other metastatic tumors were negative. A postoperative endocrine workup revealed an elevated serum prolactin of 881.3 ng/mL (normal < 20). Conclusions. In conclusion, it is crucial to maintain an extensive differential diagnosis when evaluating a patient with a clival lesion. Ectopic clival pituitary adenomas, although rare, may warrant an endocrinological workup preoperatively as the majority may respond to medical treatment. PMID:26885420

  18. Case report: Successful treatment of recurrent chordoma and bilateral pulmonary metastases following an 11-year disease-free period

    Science.gov (United States)

    Erkmen, Cherie P.; Barth, Richard J.; Raman, Vignesh

    2014-01-01

    INTRODUCTION Chordomas are rare but aggressive tumors due to local recurrence and distant metastases. They originate commonly in the sphenooccipital and sacrococcygeal regions, and metastasize to the lungs, bone, skin, liver, and lymph nodes. They occur more frequently in men and people over the age of 40. PRESENTATION OF CASE A 28 year-old female presented with sacrococcygeal chordoma for which she received wide local excision and adjuvant radiation therapy. She enjoyed an unusual disease-free survival for 11 years until a routine surveillance scan of the pelvis identified local recurrence. Further work up revealed bilateral pulmonary metastases. She underwent local excision of the recurrent tumor and video-assisted thoracoscopic (VATS) wedge resection of pulmonary metastases. She also received adjuvant radiation therapy to the recurrent resection bed. Two years later, she remains free of disease and symptoms. DISCUSSION Chordomas are commonly insensitive to chemotherapy and radiation, making surgery the most successful therapeutic modality. However, there are few guidelines on the surveillance and treatment of recurrent chordoma. We report success with aggressive surgical resection of recurrence and metastasis as well as adjuvant radiation therapy. CONCLUSION The prolonged survival of our patient underscores the importance of (1) aggressive surgical resection of chordoma, whether primary, recurrent, or metastatic, with adjuvant radiation therapy, (2) minimization of surgical seeding of tumor, and (3) diligent cancer surveillance. PMID:24907541

  19. En bloc resection of a thoracic chordoma is possible using minimally invasive anterior access: An 8-year follow-up

    Directory of Open Access Journals (Sweden)

    Anand Goomany

    2016-01-01

    Full Text Available Thoracic spine chordomas are a rare clinical entity and present several diagnostic and management challenges. Posterior debulking techniques are the traditional approach for the resection of thoracic tumors involving the vertebral body. Anterior approaches to the thoracic spine enable complete tumor resection and interbody fusion. However, this approach has previously required a thoracotomy incision, which is associated with significant perioperative morbidity, pain, and the potential for compromised ventilation and subsequent respiratory sequelae. The extreme lateral approach to the anterior spine has been used to treat degenerative disorders of the lower thoracic and lumbar spine, and reduces the potential complications compared with the anterior transperitoneal/transpleural approach. However, such an approach has not been utilized in the treatment of thoracic chordomas. We describe the first case of an en bloc resection of a thoracic chordoma via a minimally invasive eXtreme lateral interbody fusion approach.

  20. En bloc resection of a thoracic chordoma is possible using minimally invasive anterior access: An 8-year follow-up

    Science.gov (United States)

    Goomany, Anand; Timothy, Jake; Robson, Craig; Rao, Abhay

    2016-01-01

    Thoracic spine chordomas are a rare clinical entity and present several diagnostic and management challenges. Posterior debulking techniques are the traditional approach for the resection of thoracic tumors involving the vertebral body. Anterior approaches to the thoracic spine enable complete tumor resection and interbody fusion. However, this approach has previously required a thoracotomy incision, which is associated with significant perioperative morbidity, pain, and the potential for compromised ventilation and subsequent respiratory sequelae. The extreme lateral approach to the anterior spine has been used to treat degenerative disorders of the lower thoracic and lumbar spine, and reduces the potential complications compared with the anterior transperitoneal/transpleural approach. However, such an approach has not been utilized in the treatment of thoracic chordomas. We describe the first case of an en bloc resection of a thoracic chordoma via a minimally invasive eXtreme lateral interbody fusion approach. PMID:26933363

  1. Chordomas of the upper cervical spine: clinical characteristics and surgical management of a series of 21 patients

    Institute of Scientific and Technical Information of China (English)

    Zhou Hua; Jiang Liang; Wei Feng; Yu Miao; Wu Fengliang; Liu Xiaoguang; Liu Zhongjun

    2014-01-01

    Background Chordomas of the upper cervical spine are rare and present unique surgical challenge.This study aimed to describe the clinical characteristics and surgical management of patients with chordomas of the upper cervical spine.Methods Twenty-one patients with chordomas of the upper cervical spine who were treated in Peking University Third Hospital from January 1999 to October 2012 were retrospectively analyzed.Survival was calculated by the Kaplan-Meier method and was compared between groups using the log-rank test.Results The postoperative diagnosis was classical chordoma in 20 cases and chondroid chordoma in one case.The mean operative time was 9.5 hours (range 6-17 hours),and the mean blood loss was 2 812 ml (range 700-4 800 ml).There were two postoperative deaths.Unilateral vertebral artery ligation was performed in six patients,cervical nerve roots were cut in six patients,and the external branch of the superior laryngeal nerve was repaired after being cut in one case.Two patients developed postoperative velopharyngeal incompetence,and loosening of the occipitocervical screws was observed in one patient.The recurrence rate was 66.7% (10/15) after a mean follow-up period of 46.8 months (range 14-150 months).The 5-and 10-year overall survival rates were (39.8±13.1)% and (31.9±12.7)%,respectively.There was a significant difference in survival rate between patients who underwent surgery and those who did not.Conclusion In spite of the high rates of recurrence and complications after surgical treatment of chordomas of the upper cervical spine,intralesional resection combined with adjuvant radiotherapy remains the optimal treatment to prolong survival.

  2. Upregulated human telomerase reverse transcriptase (hTERT) expression is associated with spinal chordoma growth, invasion and poor prognosis

    Science.gov (United States)

    Zou, Ming-Xiang; Lv, Guo-Hua; Li, Jing; She, Xiao-Ling; Jiang, Yi

    2016-01-01

    Altered expression or activity of human telomerase reverse transcriptase (hTERT) has been associated with human carcinogenesis. This study detected hTERT expression in spinal chordoma tissues and associated the level of hTERT expression with clinicopathological data and patient survival. Tissue samples from 54 patients and 20 controls were subjected to immunohistochemical analysis of hTERT protein levels. hTERT expression levels were then analyzed for associations with patient survival rates and clinicopathological parameters (such as age, gender, tumor size, location, tumor grade, tumor stage, muscle invasion, recurrence or not, type of resection, tumor hemorrhage, tumor necrosis, levels of tumor-infiltrating lymphocytes (TILs) and Ki-67 expression). hTERT expression was detected in all 54 spinal chordomas. Expression levels were weak in 7, moderate in 17 and strong in 30 spinal chordoma tissue samples. In contrast, hTERT was rarely expressed in nucleus pulposus tissues (20 samples). hTERT expression was significantly associated with the Ki-67-staining index (t = -6.616, p < 0.001), TIL levels (F = 5.27, p = 0.008) and tumor invasion of the surrounding muscle tissue (t = -4.49, p < 0.001). Kaplan-Meier curves indicated that high hTERT expression was significantly associated with poor local recurrence-free survival of patients (χ2 = 19.07, p < 0.001 via the log-rank test), but not associated with overall patient survival. Multivariate analysis of local recurrence-free survival demonstrated that hTERT expression was an independent prognostic factor among spinal chordoma patients (HR = 1.013, 95% CI: 1.002-1.024, p = 0.016). High hTERT expression was associated with spinal chordoma growth, invasion and poor patient prognosis. Future studies will investigate the use of hTERT as a biomarker to predict patient prognosis and disease progression or as a potential spinal chordoma therapy target. PMID:27158344

  3. Diagnosing an extra-axial chordoma of the proximal tibia with the help of brachyury, a molecule required for notochordal differentiation

    Energy Technology Data Exchange (ETDEWEB)

    O' Donnell, Paul [Royal National Orthopaedic Hospital, Department of Radiology, Stanmore, Middlesex (United Kingdom); University College London, Institute of Orthopaedics and Musculoskeletal Science, Stanmore, Middlesex (United Kingdom); Tirabosco, Roberto [Royal National Orthopaedic Hospital, Department of Histopathology, Stanmore, Middlesex (United Kingdom); Vujovic, Sonja; Henderson, Stephen; Boshoff, Chris [University College London, Wolfson Institute for Biomedical Research, London (United Kingdom); Bartlett, William; Briggs, Timothy W.R. [Royal National Orthopaedic Hospital, Department of Orthopaedic Surgery, Stanmore, Middlesex (United Kingdom); Flanagan, Adrienne M. [Royal National Orthopaedic Hospital, Department of Histopathology, Stanmore, Middlesex (United Kingdom); University College London, Institute of Orthopaedics and Musculoskeletal Science, Stanmore, Middlesex (United Kingdom)

    2007-01-15

    Chordomas are rare malignant bone tumours considered to arise from notochordal remnants that persist in the axial skeleton. Although their morphology can resemble that of a carcinoma, chondrosarcoma or malignant melanoma, the axial location and their well-defined immunophenotype, including expression of cytokeratins (CK7/20/8/18/19) and S100, generally allow the diagnosis to be made with confidence once the possibility is considered. In contrast, making a robust diagnosis of an extra-axial chordoma has been difficult in the absence of specific markers for chordomas. We have recently shown in gene expression microarray and immunohistochemistry studies that brachyury, a transcription factor crucial for notochordal development, is a specific and sensitive maker for chordomas. We now present a case of an intracortical tibial tumour, with detailed report of the imaging, and morphological features consistent with a chordoma, where notochordal differentiation was demonstrated with an antibody to brachyury. The tumour cells were also positive for cytokeratins, including CK19, and S100, CEA, EMA and HMBE1, findings which support the diagnosis of chordoma. Brachyury can be employed as a marker of notochordal differentiation and help identify confidently, for the first time, extra-axial bone and soft tissue chordomas, and tumours which may show focal notochordal differentiation. (orig.)

  4. Genotyping Cancer-Associated Genes in Chordoma Identifies Mutations in Oncogenes and Areas of Chromosomal Loss Involving CDKN2A, PTEN, and SMARCB1

    Science.gov (United States)

    Choy, Edwin; MacConaill, Laura E.; Cote, Gregory M.; Le, Long P.; Shen, Jacson K.; Nielsen, Gunnlaugur P.; Iafrate, Anthony J.; Garraway, Levi A.; Hornicek, Francis J.; Duan, Zhenfeng

    2014-01-01

    The molecular mechanisms underlying chordoma pathogenesis are unknown. We therefore sought to identify novel mutations to better understand chordoma biology and to potentially identify therapeutic targets. Given the relatively high costs of whole genome sequencing, we performed a focused genetic analysis using matrix-assisted laser desorption/ionization-time of flight mass spectrometer (Sequenom iPLEX genotyping). We tested 865 hotspot mutations in 111 oncogenes and selected tumor suppressor genes (OncoMap v. 3.0) of 45 human chordoma tumor samples. Of the analyzed samples, seven were identified with at least one mutation. Six of these were from fresh frozen samples, and one was from a paraffin embedded sample. These observations were validated using an independent platform using homogeneous mass extend MALDI-TOF (Sequenom hME Genotyping). These genetic alterations include: ALK (A877S), CTNNB1 (T41A), NRAS (Q61R), PIK3CA (E545K), PTEN (R130), CDKN2A (R58*), and SMARCB1 (R40*). This study reports on the largest comprehensive mutational analysis of chordomas performed to date. To focus on mutations that have the greatest chance of clinical relevance, we tested only oncogenes and tumor suppressor genes that have been previously implicated in the tumorigenesis of more common malignancies. We identified rare genetic changes that may have functional significance to the underlying biology and potential therapeutics for chordomas. Mutations in CDKN2A and PTEN occurred in areas of chromosomal copy loss. When this data is paired with the studies showing 18 of 21 chordoma samples displaying copy loss at the locus for CDKN2A, 17 of 21 chordoma samples displaying copy loss at PTEN, and 3 of 4 chordoma samples displaying deletion at the SMARCB1 locus, we can infer that a loss of heterozygosity at these three loci may play a significant role in chordoma pathogenesis. PMID:24983247

  5. Genotyping cancer-associated genes in chordoma identifies mutations in oncogenes and areas of chromosomal loss involving CDKN2A, PTEN, and SMARCB1.

    Directory of Open Access Journals (Sweden)

    Edwin Choy

    Full Text Available The molecular mechanisms underlying chordoma pathogenesis are unknown. We therefore sought to identify novel mutations to better understand chordoma biology and to potentially identify therapeutic targets. Given the relatively high costs of whole genome sequencing, we performed a focused genetic analysis using matrix-assisted laser desorption/ionization-time of flight mass spectrometer (Sequenom iPLEX genotyping. We tested 865 hotspot mutations in 111 oncogenes and selected tumor suppressor genes (OncoMap v. 3.0 of 45 human chordoma tumor samples. Of the analyzed samples, seven were identified with at least one mutation. Six of these were from fresh frozen samples, and one was from a paraffin embedded sample. These observations were validated using an independent platform using homogeneous mass extend MALDI-TOF (Sequenom hME Genotyping. These genetic alterations include: ALK (A877S, CTNNB1 (T41A, NRAS (Q61R, PIK3CA (E545K, PTEN (R130, CDKN2A (R58*, and SMARCB1 (R40*. This study reports on the largest comprehensive mutational analysis of chordomas performed to date. To focus on mutations that have the greatest chance of clinical relevance, we tested only oncogenes and tumor suppressor genes that have been previously implicated in the tumorigenesis of more common malignancies. We identified rare genetic changes that may have functional significance to the underlying biology and potential therapeutics for chordomas. Mutations in CDKN2A and PTEN occurred in areas of chromosomal copy loss. When this data is paired with the studies showing 18 of 21 chordoma samples displaying copy loss at the locus for CDKN2A, 17 of 21 chordoma samples displaying copy loss at PTEN, and 3 of 4 chordoma samples displaying deletion at the SMARCB1 locus, we can infer that a loss of heterozygosity at these three loci may play a significant role in chordoma pathogenesis.

  6. 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

  7. 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.)

  8. 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

  9. Reprint of "Chordoma in children: Case-report and review of literature".

    Science.gov (United States)

    Habrand, Jean-Louis; Datchary, Jean; Bolle, Stéphanie; Beaudré, Anne; de Marzi, Ludovic; Beccaria, Kévin; Stefan, Dinu; Grill, Jacques; Dendale, Rémi

    2016-01-01

    We report an exceptional case of a very late local failure in a 9-year-old boy presenting with a chordoma of the cranio-cervical junction. The child was initially treated with a combination of surgical resection followed by high dose photon-proton radiation therapy. This aggressive therapy allowed a 9-year remission with minimal side-effects. Unfortunately, he subsequently presented with a local failure managed with a second full-dose course of protons. The child died one year later from local bleeding of unclear etiology.

  10. Case report 357: Chordoma of the fourth lumbar vertebra metastasizing to the thoracic spine and ribs

    Energy Technology Data Exchange (ETDEWEB)

    Abdelwahab, I.F.; Zwass, A.; O' Leary, P.F.; Steiner, G.C.

    1986-03-01

    In summary a fascinating case is presented in a 54-year-old man who developed a chordoma of the fourth lumbar vertebra which was treated by radiotherapy, with good results. The man remained asymptomatic relatively for several years and then presented with recurrence of back pain and neurological deficits. Plain films, CT and myelography showed considerable destruction of the body of L4 with a sclerotic pattern suggesting the effects of previous radiotherapy. A large paraspinal tissue mass extending into the spinal canal was present. Most interestingly the patient developed metastatic disease in the thoracic spine and ribs but no metastases other than in the skeleton. (orig./SHA).

  11. 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.

  12. Nasopharyngeal chordoma in a patient with a severe form of sleep-disordered breathing: A case report

    Science.gov (United States)

    RADZIKOWSKA, JOANNA; GRONKIEWICZ, ZUZANNA; KUKWA, ANDRZEJ; LISIK, WOJCIECH; CZARNECKA, ANNA M.; KRZESKI, ANTONI; KUKWA, WOJCIECH

    2015-01-01

    Nasopharyngeal chordoma is a rare type of malignant neoplasm that originates in the remnants of the notochord, a primitive tissue of embryonic origin preserved outside the axial skeleton. Approximately one-third of chordomas are located in the base of the skull, in the midline of the body. The slow growth rate of the tumor, which gradually fills the nasopharyngeal cavity, contributes to a delayed oncological diagnosis. Among its isolated and non-specific symptoms, the obstruction of the nasopharynx is dominant, thus, sleep-disordered breathing (SDB) may occur. The current study presents the case of a 32-year-old female patient who was incidentally diagnosed with a nasopharyngeal chordoma during a diagnostic examination for SDB. The diagnostic examination was performed as a part of a research program for pathologically obese patients who qualified for bariatric surgery. Following tumor resection, a significant improvement in various polysomnographic parameters occurred, including a decrease in the apnea hypopnea index from 53.5 to 6.4 and an increase in the mean saturation rate from 92.5 to 95%, confirming that an association exists between tumor obstruction of the nasopharynx and SDB. The incidental diagnosis of this rare type of neoplasm drew attention to diagnostic and therapeutic problems associated with nasopharyngeal chordomas. Furthermore, it indicated the necessity for the accurate laryngological examination of patients with SDB. PMID:26622754

  13. 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

  14. Endovascular occlusion of a lacerated primitive trigeminal artery during surgical resection of clival chordoma. a case report.

    Science.gov (United States)

    Baltsavias, G; Valavanis, A

    2010-06-01

    We describe a case of a persistent primitive trigeminal artery (PPTA) coexistent with a clival chordoma. During surgery of the tumor, the partially incorporated PPTA was inadvertently traumatized and ruptured. The operation was discontinued and the PPTA was endovascularly occluded permitting further safe resection of the tumor. PMID:20642897

  15. Transformation of a cervical chordoma in an anaplastic tumor. A case report and short review of the literature. Transformation eines zervikalen Chordoms in einen anaplastischen Tumor. Eine Fallbeschreibung und Literaturueberblick

    Energy Technology Data Exchange (ETDEWEB)

    Wichmann, A.P.; Latz, D.M. (Heidelberg Univ. (Germany). Radiologische Klinik, Abt. Strahlentherapie)

    1993-02-01

    The authors describe a case of a cervical chordoma in a 28-year-old man with local recurrence and metastasis. Particular attention is paid to the fact that complete malignant transformation was observed during the follow-up. A review of the literature is undertaken chronicling the documented associations of chordoma and malignant neoplasm. Followed by a discussion of the three causes proposed to explain this phenomenon. Furtheron the current opinion on the therapy methods of chordomas is reviewed. (orig.).

  16. First histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion: differential diagnosis of benign and malignant notochordal lesions

    Energy Technology Data Exchange (ETDEWEB)

    Yamaguchi, Takehiko [Department of Anatomic and Diagnostic Pathology, Dokkyo University School of Medicine, Mibu, Tochigi (Japan); Yamato, Minoru [Department of Radiology, International Catholic Hospital, Tokyo (Japan); Saotome, Koichi [Department of Orthopaedic Surgery, Dokkyo University School of Medicine, Mibu, Tochigi (Japan)

    2002-07-01

    The first histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion is presented and the differential diagnosis between benign and malignant notochordal lesions is discussed. A 57-year-old man presented with a classic chordoma in the coccyx. The resected specimen demonstrated a small intraosseous benign notochordal lesion in the coccyx, which was adjacent to the classic chordoma. Also seen were two separate, similar benign lesions in the sacrum. The classic chordoma consisted of multiple lobules that were separated by thin fibrous septa and that showed cords or strands of atypical physaliphorous cells set within an abundant myxoid matrix. In contrast, the benign lesions consisted of intraosseous sheets of bland physaliphorous cells without any extracellular matrix. The affected bone trabeculae showed sclerotic reactions. It was concluded that benign and malignant notochordal lesions can be distinguished microscopically. (orig.)

  17. Chordoma of the petrous apex - a case report and review of the literature; Cordoma de apice petroso - relato de um caso e revisao da literatura

    Energy Technology Data Exchange (ETDEWEB)

    Loureiro, Ricardo; Leal Junior, Osvaldo S. [Pernambuco Univ., Recife, PE (Brazil). Faculdade de Medicina; Loureiro, Lautonio Junior [Universidade de Pernambuco, Recife, PE (Brazil). Faculdade de Ciencias Medicas; Buril, Marlus V.M. [Hospital das Clinicas, Recife, PE (Brazil). Centro de Terapia Intensiva

    1998-10-01

    Chordomas are rare tumours arising from remnants of the embryologic notochord, typically at a midline position. Although 35-40% of these lesions are intracranial, these tumors answer for less than 1% of all intracranial tumors. The intracranial chordomas originate most frequently from the clival region at the midline. Nevertheless eventually may arise off the midline primarily in petrous apex or, very rarely, in paranasal sinuses. The authors report a case of histopathologically proved intracranial chordoma that arose atypical site in the petrous apex. The computed tomographic and magnetic resonance imaging finding were similar to those observed in midline chordomas. The computed tomographic examination revealed a well-defined soft tissue mass associated with bone destruction and foci of calcification. The magnetic resonance imaging study demonstrated a growing extra-axial formation that appeared with hypo-intensity of signal on T1-weighted images, hyperintensity on T2-weighted images and heterogeneous enhancement after paramagnetic agent injection. (author) 8 refs., 8 figs.

  18. Sellar Chordoma Presenting as Pseudo-macroprolactinoma with Unilateral Third Cranial Nerve Palsy

    Institute of Scientific and Technical Information of China (English)

    Hai-feng Wang; Hong-xi Ma; Cheng-yuan Ma; Yi-nan Luo; Peng-fei Ge

    2012-01-01

    We described a 61-year-old female with a sellar chordoma,which presented as pseudo-macroprolactinoma with unilateral third cranial nerve palsy.Physical examination revealed that her right upper lid could not be raised by itself,right eyeball movement limited to the abduction direction,right pupil dilated to 4.5 mm with negative reaction to light,and hemianopsia in bitemporal sides.CT scanning showed a hyperdense lesion at sellar region without bone destruction.Magnetic resonance imaging (MRI) revealed the tumor was 2.3 cm×1.8 cm×2.6 cm,with iso-intensity on T1WI,hyper-intensity on T2WI and heterogeneous enhancement on contrast imaging.Endocrine examination showed her serum prolactin level increased to 1,031.49 mlU/ml.The tumor was sub-totally resected via pterional craniotomy under microscope and was histologically proven to be a chordoma.Postoperatively,she recovered uneventfully but ptosis and hemianopsia remained at the 6th month.

  19. Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients

    Directory of Open Access Journals (Sweden)

    Giannarelli Diana

    2010-01-01

    Full Text Available Abstract Background Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported. Methods We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008. Results Twenty-five consecutive patients with sacral (11 patients, spine (13 patients, and skull base (1 patient chordoma went to our observation. Six patients (24% had primary disease, 14(56% a recurrent disease, and 5(20% a metastatic spreading. Surgery was the primary option for treatment in 22 out of 25 patients. Surgical margins were wide in 5 (23% and intralesional in 17(77% patients; 3 out of 4 in-house treated patients obtained wide margins. After first surgery, radiotherapy (protons or high-energy photons were delivered to 3 patients. One out of the 5 patients with wide margins is still without evidence of disease at 20 months from surgery; 2 patients died without evidence of disease after 3 and 36 months from surgery. Sixteen out of 17 (94% patients with intralesional margins underwent local progression at a median time of 18 months with a 2-year local progression-free survival of 47%. The 5-year metastasis-free survival rate was 78.3%. Seventeen patients with locally advanced and/or metastatic disease expressing platelet-derived growth factor receptor (PDGFR β were treated with imatinib mesylate. A RECIST stabilization of the disease was the best response observed in all treated cases. Pain relief with reduction in analgesics use was obtained in 6 out of 11 (54% symptomatic patients. The 5- and 10-year survival rates of the entire series of patients were 76.7 and 59.7%, respectively. Conclusions Despite progress of surgical techniques and the results obtained with targeted therapy, more effort is needed for better disease control. Specific experience of the multidisciplinar therapeutic team is

  20. Combined use of maxillomandibular swing approach and neurosurgical ultrasonic aspirator in the management of extensive clival chordoma: A case report

    Directory of Open Access Journals (Sweden)

    Hassan Shahid

    2008-02-01

    Full Text Available Abstract Introduction Chordoma is a rare malignant tumour with an incidence of metastasis of less than 10 percent. Usually arising from clivus its posterior extension may involve the brainstem before presenting as nasal mass and obstruction. Surgery is the main mode of treatment with adjuvant radiotherapy. However surgery is rarely possible for a large intracranial lesion. Case presentation We report the case of an adolescent patient with a chordoma extending posteriorly to the brainstem and anteriorly to the nasopharynx and managed by the combination of resection using a maxillomandibular swing approach and the use of a neurosurgical ultrasonic aspirator. Conclusion Maxillomandibular swing approach provides good access for large nasopharyngeal tumour extending brainstem area.

  1. Fifty-four-month survival in a 3-year-old child presenting with an aggressive metastatic dedifferentiated clival chordoma.

    Science.gov (United States)

    Kearns, Ciléin; Kearns, Cónail

    2016-01-01

    Dedifferentiated chordoma is a rare, aggressive, chemoresistant and radioresistant malignancy arising from notochord remnants that can occur anywhere along the spine. Incidence in patients under 20 years of age is 1 per 250 million. We report a case of dedifferentiated clival chordoma presenting in a 3-year-old boy with pulmonary metastasis, which responded unusually well to chemotherapy, achieving complete metastatic clearance and debulking of the primary tumour. Proton beam therapy achieved further tumour control, with excellent quality of life for multiple years. On disease relapse, an atypical lateral transcondylar surgical approach achieved complete macroscopic clearance but there was cutaneous seeding. This, and continued primary site activity, failed to be controlled with targeted therapy, traditional chemotherapy and photon radiation, resulting in gradual neurological decline and death. Intensive management resulted in above-average survival despite diagnosis late in the disease course, which may be of value directing investigation into optimal management.

  2. Chordoma of the thoracic vertebrae in a Bengal tiger (Panthera tigris tigris)

    Science.gov (United States)

    KURAMOCHI, Mizuki; IZAWA, Takeshi; HORI, Mayuka; KUSUDA, Kayo; SHIMIZU, Junichiro; ISERI, Toshie; AKIYOSHI, Hideo; OHASHI, Fumihito; KUWAMURA, Mitsuru; YAMATE, Jyoji

    2015-01-01

    A 19-year-old female Bengal tiger (Panthera tigris tigris) was presented with hind limb weakness, ataxia and respiratory distress. Computed tomography revealed a mass between the left side of the T7 vertebra and the base of the left 7th rib. The tiger then died, and necropsy was performed. Grossly, the vertebral mass was 6 × 5.7 × 3 cm, and invaded the adjacent vertebral bone and compressed the T7 spinal cord. Histologically, the mass was composed of large, clear, vacuolated and polygonal cells with osteochondral matrix. Cellular and nuclear atypia were moderate. The vacuolated cells stained positively for cytokeratin and vimentin and negatively for S-100. Based on these findings, the present case was diagnosed as a vertebral chordoma; the first report in a tiger. PMID:25766770

  3. The Unresolved Case of Sacral Chordoma: From Misdiagnosis to Challenging Surgery and Medical Therapy Resistance

    Science.gov (United States)

    Garofalo, Fabio; Christoforidis, Dimitrios; di Summa, Pietro G.; Gay, Béatrice; Cherix, Stéphane; Raffoul, Wassim; Matter, Maurice

    2014-01-01

    Purpose A sacral chordoma is a rare, slow-growing, primary bone tumor, arising from embryonic notochordal remnants. Radical surgery is the only hope for cure. The aim of our present study is to analyse our experience with the challenging treatment of this rare tumor, to review current treatment modalities and to assess the outcome based on R status. Methods Eight patients were treated in our institution between 2001 and 2011. All patients were discussed by a multidisciplinary tumor board, and an en bloc surgical resection by posterior perineal access only or by combined anterior/posterior accesses was planned based on tumor extension. Results Seven patients underwent radical surgery, and one was treated by using local cryotherapy alone due to low performance status. Three misdiagnosed patients had primary surgery at another hospital with R1 margins. Reresection margins in our institution were R1 in two and R0 in one, and all three recurred. Four patients were primarily operated on at our institution and had en bloc surgery with R0 resection margins. One had local recurrence after 18 months. The overall morbidity rate was 86% (6/7 patients) and was mostly related to the perineal wound. Overall, 3 out of 7 resected patients were disease-free at a median follow-up of 2.9 years (range, 1.6-8.0 years). Conclusion Our experience confirms the importance of early correct diagnosis and of an R0 resection for a sacral chordoma invading pelvic structures. It is a rare disease that requires a challenging multidisciplinary treatment, which should ideally be performed in a tertiary referral center. PMID:24999463

  4. Experimental Study on Differences in Clivus Chordoma Bone Invasion: An iTRAQ-Based Quantitative Proteomic Analysis

    Science.gov (United States)

    Wang, Ke; Zhang, Yang; Tian, Kaibing; Zhang, Junting; Sun, Wei; Yu, Chunjiang

    2015-01-01

    Although a bone tumor, significant differences in the extent of bone invasion exist in skull base chordoma, which directly affect the extent of surgical resection, and have an impact on its prognosis. However, the underlying mechanism of the phenomenon is not clearly understood. Therefore, we used an iTRAQ-based quantitative proteomics strategy to identify potential molecular signatures, and to find predictive markers of discrepancy in bone invasion of clivus chordoma. According to bone invasive classification criteria, 35 specimens of clivus chordoma were calssified to be either endophytic type (Type I) or exophytic type (Type II). An initial screening of six specimens of endophytic type and six of exophytic was performed, and 250 differentially expressed proteins were identified. Through the GO and IPA analysis, we found evidence that the expression of inflammatory activity-associated proteins up-regulated in endophytic type, whereas the expression of cell motility-associated proteins up-regulated in exophytic ones. Moreover, TGFβ1 and mTOR signal pathway seemed to be related with bone invasion. Thus, TGFβ1, PI3K, Akt, mTOR, and PTEN were validated in the following 23 samples by immune histochemistry and Western blot. The expression levels of TGFβ1 and PTEN were significantly lower in the endophytic type than in the exophytic ones. It was found that TGFβ1 may play an important role in its bone invasion. The mechanisms may be related with conducting an increased inflammatory cell response and a decline in cytoskeletal protein expression. PTEN is confirmed to be associated with the degree of bone invasion. The PI3K/AKT/mTOR signaling pathway might be associated with the bone invasion, but still needs a larger sample size to be verified These results, for the first time, not only demonstrate the biological changes that occur in different growth patterns from the perspective of proteomics, but also provide novel markers that may help to reveal the mechanisms

  5. Particle therapy using carbon ions or protons as a definitive therapy for patients with primary sacral chordoma

    Science.gov (United States)

    Demizu, Y; Jin, D; Hashimoto, N; Takagi, M; Terashima, K; Fujii, O; Niwa, Y; Akagi, T; Daimon, T; Hishikawa, Y; Abe, M; Murakami, M; Sasaki, R; Fuwa, N

    2014-01-01

    Objective: This study retrospectively evaluated the efficacy and toxicity of particle therapy using carbon ions or protons for primary sacral chordomas. Methods: We evaluated 23 patients with primary sacral chordoma treated with carbon ion therapy (CIT) or proton therapy (PT) between July 2005 and June 2011 at the Hyogo Ion Beam Medical Center, Hyogo, Japan. The median patient age was 72 years. 14 patients were treated with 70.4 Gy equivalents (GyE) in 16 fractions and 9 were treated with 70.4 GyE in 32 fractions. CIT was used for 16 patients, and PT was used for 7 patients. Results: The median follow-up period was 38 months. At 3 years, local control (LC), overall survival (OS) and progression-free survival (PFS) for all patients were 94%, 83% and 68%, respectively. The log-rank test revealed that male sex was significantly related to better PFS ( p = 0.029). No other factors, including dose fractionation and ion type, were significant for LC, OS or PFS. In nine patients, ≥Grade 3 acute dermatitis was observed, and ≥Grade 3 late toxicities were observed in nine patients. The 32-fraction protocol reduced severe toxicities in both the acute and late phases compared with the 16-fraction protocol. Conclusion: Particle therapy for patients with sacral chordoma showed favourable LC and OS. Severe toxicities were successfully reduced by modifying the dose fractionation and treatment planning in the later treatment era. Thus, this therapeutic modality should be considered useful and safe. Advances in knowledge: This is the first study including both CIT and PT for sacral chordomas. PMID:24288399

  6. Experimental study on differences in clivus chordoma bone invasion: an iTRAQ-based quantitative proteomic analysis.

    Directory of Open Access Journals (Sweden)

    Zhen Wu

    Full Text Available Although a bone tumor, significant differences in the extent of bone invasion exist in skull base chordoma, which directly affect the extent of surgical resection, and have an impact on its prognosis. However, the underlying mechanism of the phenomenon is not clearly understood. Therefore, we used an iTRAQ-based quantitative proteomics strategy to identify potential molecular signatures, and to find predictive markers of discrepancy in bone invasion of clivus chordoma. According to bone invasive classification criteria, 35 specimens of clivus chordoma were calssified to be either endophytic type (Type I or exophytic type (Type II. An initial screening of six specimens of endophytic type and six of exophytic was performed, and 250 differentially expressed proteins were identified. Through the GO and IPA analysis, we found evidence that the expression of inflammatory activity-associated proteins up-regulated in endophytic type, whereas the expression of cell motility-associated proteins up-regulated in exophytic ones. Moreover, TGFβ1 and mTOR signal pathway seemed to be related with bone invasion. Thus, TGFβ1, PI3K, Akt, mTOR, and PTEN were validated in the following 23 samples by immune histochemistry and Western blot. The expression levels of TGFβ1 and PTEN were significantly lower in the endophytic type than in the exophytic ones. It was found that TGFβ1 may play an important role in its bone invasion. The mechanisms may be related with conducting an increased inflammatory cell response and a decline in cytoskeletal protein expression. PTEN is confirmed to be associated with the degree of bone invasion. The PI3K/AKT/mTOR signaling pathway might be associated with the bone invasion, but still needs a larger sample size to be verified These results, for the first time, not only demonstrate the biological changes that occur in different growth patterns from the perspective of proteomics, but also provide novel markers that may help to reveal

  7. Establishment of clival chordoma cell line MUG-CC1 and lymphoblastoid cells as a model for potential new treatment strategies

    Science.gov (United States)

    Gellner, Verena; Tomazic, Peter Valentin; Lohberger, Birgit; Meditz, Katharina; Heitzer, Ellen; Mokry, Michael; Koele, Wolfgang; Leithner, Andreas; Liegl-Atzwanger, Bernadette; Rinner, Beate

    2016-01-01

    Chordomas are rare malignant tumors that develop from embryonic remnants of the notochord and arise only in the midline from the clivus to the sacrum. Surgery followed by radiotherapy is the standard treatment. As chordomas are resistant to standard chemotherapy, further treatment options are urgently needed. We describe the establishment of a clivus chordoma cell line, MUG-CC1. The cell line is characterized according to its morphology, immunohistochemistry, and growth kinetics. During establishment, cell culture supernatants were collected, and the growth factors HGF, SDF-1, FGF2, and PDGF analyzed using xMAP® technology. A spontaneous lymphoblastoid EBV-positive cell line was also developed and characterized. MUG-CC1 is strongly positive for brachyury, cytokeratin, and S100. The cell line showed gains of the entire chromosomes 7, 8, 12, 13, 16, 18, and 20, and high level gains on chromosomes 1q21–1q24 and 17q21–17q25. During cultivation, there was significant expression of HGF and SDF-1 compared to continuous chordoma cell lines. A new, well-characterized clival chordoma cell line, as well as a non-tumorigenic lymphoblastoid cell line should serve as an in vitro model for the development of potential new treatment strategies for patients suffering from this disease. PMID:27072875

  8. Novel reconstruction of the anterior craniocervical junction using an expandable cage with integrated fixation after total C2 spondylectomy for chordoma.

    Science.gov (United States)

    Wewel, Joshua T; Nunna, Ravi S; Tan, Lee A; Kasliwal, Manish K; O'Toole, John E

    2016-08-01

    Chordoma is a locally aggressive malignant tumor that generally occurs in the clivus, mobile spine and sacrum. While en bloc resection with wide margins has been advocated as the only cure for chordomas, tumor characteristics and violation of critical anatomical boundaries may preclude pursuing this treatment option in the cervical spine. We present a C2 chordoma in a 35-year-old man with epidural and prevertebral extension that was treated with a single stage anterior-posterior total C2 spondylectomy with novel reconstruction using an expandable cage with integrated fixation followed by stereotactic radiosurgery. Single stage intralesional total C2 spondylectomy via anterior transoral and posterior approaches was performed. The anterior column was reconstructed using an expandable cage with integrated fixation from the clivus to C3. The patient maintained his intact neurological status at 6 month follow-up with full resumption of activities of daily living without any significant morbidity.

  9. Clinical features and prognostic factors of patients with chordoma in the spine: a retrospective analysis of 153 patients in a single center

    Science.gov (United States)

    Meng, Tong; Yin, Huabin; Li, Bo; Li, Zhenxi; Xu, Wei; Zhou, Wang; Cheng, Mo; Wang, Jing; Zhou, Lei; Yang, Xinghai; Liu, Tielong; Yan, Wangjun; Song, Dianwen; Xiao, Jianru

    2015-01-01

    Background Chordoma in the spine is relatively rare, and minimal information has been published in the literature regarding this subject. Moreover, there are controversies over prognostic factors of this disease. Methods A retrospective analysis of chordoma in the spine was performed by survival analysis. Local relapse-free survival (LRFS) and overall survival (OS) were analyzed from the date of surgery to the date of local recurrence and death. The LRFS and OS rates were estimated using the Kaplan–Meier method to identify potential prognostic factors. Factors with P values ≤ .1 were subjected to multivariate analysis by Cox regression analysis. P values ≤ .05 were considered statistically significant. Results A total of 153 patients with spinal chordoma were included in the study. The mean follow-up period was 72.0 months (range, 1–279 months). Local recurrence was detected in 51 cases after initial surgery in our center, while death occurred in 42 cases. The statistical analysis suggested that tumor location of C3–L5, dedifferentiated chordoma, preoperative Frankel scores A–C, and total spondylectomy were independent prognostic factors for LRFS. In addition, total en bloc spondylectomy and Karnofsky' performance status (KPS) ≥ 80% were favorable factors for OS. Conclusions Total spondylectomy, by either en bloc or piecemeal method, could significantly reduce LRFS for spinal chordoma. Location of C3–L5 is a favorable factor for LRFS, while dedifferentiated subtype and preoperative Frankel scores A–C are adverse prognostic factors. In addition, total en bloc spondylectomy and KPS ≥ 80% significantly improve overall survival of patients with spinal chordoma. PMID:25488908

  10. 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

  11. Carbon ion beam treatment in patients with primary and recurrent sacrococcygeal chordoma

    Energy Technology Data Exchange (ETDEWEB)

    Uhl, Matthias; Jensen, Alexandra; Herfarth, Klaus [University of Heidelberg, Department of Radiation Oncology, Heidelberg (Germany); Heidelberg Ion Beam Therapy Center (HIT), Heidelberg (Germany); Welzel, Thomas [University of Heidelberg, Department of Radiation Oncology, Heidelberg (Germany); Ellerbrock, Malte; Haberer, Thomas [Heidelberg Ion Beam Therapy Center (HIT), Heidelberg (Germany); Jaekel, Oliver [University of Heidelberg, Department of Radiation Oncology, Heidelberg (Germany); Heidelberg Ion Beam Therapy Center (HIT), Heidelberg (Germany); German Cancer Research Center (dkfz), Heidelberg (Germany); Heidelberg Institute of Radiation Oncology (HIRO), Heidelberg (Germany); Debus, Juergen [University of Heidelberg, Department of Radiation Oncology, Heidelberg (Germany); Heidelberg Ion Beam Therapy Center (HIT), Heidelberg (Germany); German Cancer Research Center (dkfz), Heidelberg (Germany); Heidelberg Institute of Radiation Oncology (HIRO), Heidelberg (Germany); Deutsches Konsortium fuer Translationale Krebsforschung (DKTK), Heidelberg (Germany)

    2015-07-15

    The purpose of this work was to evaluate the results of high-dose radiation treatment using carbon ion therapy, alone or combined with intensity-modulated radiation treatment (IMRT), in patients with sacral chordoma. Between 2009 and 2012, 56 patients with sacral chordoma were treated in our center. The tumor was located above S3 in 33 patients and in S3 or below in 23 patients. In all, 41 patients received radiation therapy for the primary tumor, while 15 patients were treated for the recurrent tumor. Toxicity was measured using NCI CTCAE v.4.03. Local control (LC) and overall survival (OS) were evaluated with the Kaplan-Meier method. A total of 23 patients were irradiated with carbon ions in combination with photon IMRT, while 33 received carbon ion therapy only. Forty-three patients had a macroscopic tumor at treatment start with a median tumor size (GTV) of 244 ml (range 5-1188 ml). The median total dose was 66 Gy (range 60-74 Gy; RBE). After a median follow-up time of 25 months, the 2- and 3-year local control probability was 76 % and 53 %, respectively. The overall survival rate was 100 %. Treatment for primary tumor and male patients resulted in significant better local control. No higher toxicity occurred within the follow-up time. High-dose photon/carbon ion beam radiation therapy is safe and, especially for primary sacral chordomas, highly effective. A randomized trial is required to evaluate the role of primary definitive hypofractionated particle therapy compared with surgery with or without adjuvant radiotherapy. (orig.) [German] Evaluierung der Ergebnisse nach hochdosierter Kohlenstoffionentherapie, allein oder in Kombination mit einer intensitaetsmodulierten Photonenbestrahlung (IMRT), bei Patienten mit einem sakralen Chordom. Zwischen 2009 und 2012 wurden 56 Patienten mit sakralen Chordomen in unserem Zentrum behandelt. Der Tumor war bei 33 Patienten oberhalb von S3 und bei 23 Patienten auf Hoehe von S3 oder unterhalb davon lokalisiert. Insgesamt

  12. Case Report: Familial Gastric Cancer and Chordoma in the Same Family

    Directory of Open Access Journals (Sweden)

    Weber Walter

    2005-05-01

    Full Text Available Abstract Gastric cancers are the second most common malignancy in the world and represent a major burden to all societies even though the incidence of disease is decreasing in the industrialized world. The aetiology of the disease is complex and is believed to be primarily due to environmental factors but a small proportion of cases are recognised as being associated with genetic factors. Two inherited forms of stomach cancer have been identified, one which is associated with familial clusterings of stomach cancer and the other being a subgroup of families that belong to hereditary non polyposis colorectal cancer (or Lynch syndrome. In this report we present a small nuclear family which is unusual in that there is a clustering of malignancy which includes stomach cancer, colorectal cancer and chordoma. Genetic analysis failed to reveal any causative mutation in genes associated with HNPCC or in E-cadherin. Together, the clinical picture in this family may indicate that other genetic factors are behind this family's clustering of malignancy.

  13. Treatment and Outcome of Patients with Skull Base Chordoma: A Meta-analysis

    Science.gov (United States)

    Amit, Moran; Na'ara, Shorook; Binenbaum, Yoav; Billan, Salem; Sviri, Gil; Cohen, Jacob T.; Gil, Ziv

    2014-01-01

    Objective Chordoma is a locally aggressive tumor. The aim of this study was to assess the efficacy of different surgical approaches and adjuvant radiation modalities used to treat these patients. Design Meta-analysis. Main Outcome Measures Overall survival (OS), disease-specific survival (DSS), and progression-free survival (PFS). Results The 5-year OS and PFS rates of the whole cohort (n = 467) were 86% and 65.7%, respectively. The 5-year DSS for patients who underwent open surgery and endoscopic surgery was 45% and 49%, respectively (p = 0.8); PFS was 94% and 79%, respectively (p = 0.11). The 5-year OS of patients treated with surgery followed by adjuvant radiotherapy was 90% compared with 70% of those treated by surgery alone (p = 0.24). Patients undergoing partial resection without adjuvant radiotherapy had a 5-year OS of 41% and a DSS of 45%, significantly lower than in the total-resection group (p = 0.0002 and p = 0.01, respectively). The complication rates were similar in the open and endoscopic groups. Conclusions Patients undergoing total resection have the best outcome; adjuvant radiation therapy improves the survival of patients undergoing partial resection. In view of the advantages of minimally invasive techniques, endoscopic surgery appears an appropriate surgical approach for this disease. PMID:25452895

  14. Atypical CT and MR features of chondroid chordoma at the base of the skull: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Hye Jeon; Kim, Hyun Sook; Kim, Joo Seung; Kim, Eun Kyung; Kang, Hee In; Ko, Eun Joo; Yoon, Yong Kyu [School of Medicine, Eulji University, Taejon (Korea, Republic of)

    2000-10-01

    We report a case of chondroid chordoma without calcification in which T2-weighted MR images revealed homogeneous high signal intensity. The tumor was located in the left middle cranial fossa extending to the cerebellopontine angle and with displacement of the pituitary grand to the right side. Precontrast CT scans showed a homogeneous low-density mass, without calcification. T1-weighted MR images of the mass demonstrated relatively homogeneous low signal intensity, T2-weighted images showed homogeneous high signal intensity, and post-contrast T1-weighted images revealed somewhat heterogeneous enhancement. (author)

  15. Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine. Prognostic factors and patterns of failure

    Energy Technology Data Exchange (ETDEWEB)

    Noel, G.; Jauffret, E.; Mammar, H.; Ferrand, R. [Centre de Protontherapie d' Orsay, Orsay (France); Habrand, J.L.; Crevoisier, R. de; Haie-Meder, C.; Beaudre, A. [Inst. Gustave Roussy, Villejuif (France); Dederke, S.; Hasboun, D.; Boisserie, G. [Groupe Pitie Salpetriere, AP-HP, Paris (France); Pontvert, D.; Gaboriaud, G. [Inst. Curie, Paris (France); Guedea, F.; Petriz, L. [Catalan Inst. of Oncology, Barcelona (Spain); Mazeron, J.J. [Centre de Protontherapie d' Orsay, Orsay (France); Groupe Pitie Salpetriere, AP-HP, Paris (France)

    2003-04-01

    Background: Prospective analysis of local tumor control, survival and treatment complications in 67 consecutive patients treated with fractionated photon and proton radiation for chordoma or chondrosarcoma of the base of the skull and the cervical spine. Patients and Methods: Between December 1995 and January 2000, 67 patients with a median age of 52 years (range: 14-85 years), were treated at the Centre de Protontherapie d'Orsay (CPO), France, using the 201-MeV proton beam, 49 for chordoma and 18 for chondrosarcoma. Irradiation combined high-energy photons and protons. Photons represented two thirds of the total dose and protons one third. The median total dose delivered within gross tumor volume (GTV) was 67 cobalt gray equivalents (CGE; range: 60-70 CGE). Results: Within a median follow-up of 29 months (range: 4-71 months), the 3-year local control rates were 71% and 85% for chordomas and chondrosarcomas, respectively, and the 3-year overall survival rates 88% and 75%, respectively. 14 tumors (21.5%) failed locally (eight within the GTV, four within the clinical target volume [CTV], and two without further assessment). Seven patients died from their tumor and another one from a nonrelated condition (pulmonary embolism). The maximum tumor diameter and, similarly, the GTV were larger in relapsing patients, compared with the rest of the population: 56 mm vs 44 mm (p = 0.024) and 50 ml vs 22 ml (p = 0.0083), respectively. In univariate analysis, age {<=} 52 years at the time of radiotherapy (p = 0.002), maximum diameter < 45 mm (p = 0.02), and GTV < 28 ml (p = 0.02) impacted positively on local control. On multivariate analysis, only age was an independent prognostic factor of local control. Conclusion: In chordomas and chondrosarcomas of the skull base and cervical spine, combined photon and proton radiation therapy offers excellent chances of cure. In two thirds of the cases, relapses are located in the GTV. Maximum diameter, GTV, and age are prognostic

  16. Giant notochordal hamartoma of intraosseous origin: a newly reported benign entity to be distinguished from chordoma. Report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Mirra, J.M. [Dept. of Orthopaedic Pathology, Orthopaedic Hospital, Los Angeles, CA (United States); Brien, E.W. [Dept. of Musculoskeletal Tumors, Orthopaedic Hospital, Los Angeles, CA (United States)

    2001-12-01

    Two cases are reported of a newly described intraosseous entity of vertebral bodies deemed ''giant notochordal hamartoma of intraosseous origin''. This entity is commonly mistaken for chordoma and must be distinguished from it as the consequences of misinterpretation may be serious. The clinical, radiological and histologic criteria that can be used to distinguish these two entities are emphasized. Included is a proposed pathogenesis for this lesion, its probable notochordal origin, and a review of other probable cases. (orig.)

  17. In vitro characterization of cells derived from chordoma cell line U-CH1 following treatment with X-rays, heavy ions and chemotherapeutic drugs

    Directory of Open Access Journals (Sweden)

    Uesaka Mitsuru

    2011-09-01

    Full Text Available Abstract Background Chordoma, a rare cancer, is usually treated with surgery and/or radiation. However, very limited characterizations of chordoma cells are available due to a minimal availability (only two lines validated by now and the extremely long doubling time. In order to overcome this situation, we successfully derived a cell line with a shorter doubling time from the first validated chordoma line U-CH1 and obtained invaluable cell biological data. Method After isolating a subpopulation of U-CH1 cells with a short doubling time (U-CH1-N, cell growth, cell cycle distribution, DNA content, chromosome number, p53 status, and cell survival were examined after exposure to X-rays, heavy ions, camptothecin, mitomycin C, cisplatin and bleocin. These data were compared with those of HeLa (cervical cancer and U87-MG (glioblastoma cells. Results The cell doubling times for HeLa, U87-MG and U-CH1-N were approximately 18 h, 24 h and 3 days respectively. Heavy ion irradiation resulted in more efficient cell killing than x-rays in all three cell lines. Relative biological effectiveness (RBE at 10% survival for U-CH1-N was about 2.45 for 70 keV/μm carbon and 3.86 for 200 keV/μm iron ions. Of the four chemicals, bleocin showed the most marked cytotoxic effect on U-CH1-N. Conclusion Our data provide the first comprehensive cellular characterization using cells of chordoma origin and furnish the biological basis for successful clinical results of chordoma treatment by heavy ions.

  18. In vitro characterization of cells derived from chordoma cell line U-CH1 following treatment with X-rays, heavy ions and chemotherapeutic drugs

    International Nuclear Information System (INIS)

    Chordoma, a rare cancer, is usually treated with surgery and/or radiation. However, very limited characterizations of chordoma cells are available due to a minimal availability (only two lines validated by now) and the extremely long doubling time. In order to overcome this situation, we successfully derived a cell line with a shorter doubling time from the first validated chordoma line U-CH1 and obtained invaluable cell biological data. After isolating a subpopulation of U-CH1 cells with a short doubling time (U-CH1-N), cell growth, cell cycle distribution, DNA content, chromosome number, p53 status, and cell survival were examined after exposure to X-rays, heavy ions, camptothecin, mitomycin C, cisplatin and bleocin. These data were compared with those of HeLa (cervical cancer) and U87-MG (glioblastoma) cells. The cell doubling times for HeLa, U87-MG and U-CH1-N were approximately 18 h, 24 h and 3 days respectively. Heavy ion irradiation resulted in more efficient cell killing than x-rays in all three cell lines. Relative biological effectiveness (RBE) at 10% survival for U-CH1-N was about 2.45 for 70 keV/μm carbon and 3.86 for 200 keV/μm iron ions. Of the four chemicals, bleocin showed the most marked cytotoxic effect on U-CH1-N. Our data provide the first comprehensive cellular characterization using cells of chordoma origin and furnish the biological basis for successful clinical results of chordoma treatment by heavy ions

  19. An overview of the role of cancer stem cells in spine tumors with a special focus on chordoma

    Science.gov (United States)

    Safari, Mojdeh; Khoshnevisan, Alireza

    2014-01-01

    Primary malignant tumors of the spine are relatively rare, less than 5% of all spinal column tumors. However, these lesions are often among the most difficult to treat and encompass challenging pathologies such as chordoma and a variety of invasive sarcomas. The mechanisms of tumor recurrence after surgical intervention, as well as resistance to radiation and chemotherapy, remain a pervasive and costly problem. Recent evidence has emerged supporting the hypothesis that solid tumors contain a sub-population of cancer cells that possess characteristics normally associated with stem cells. Particularly, the potential for long-term proliferation appears to be restricted to subpopulations of cancer stem cells (CSCs) functionally defined by their capacity to self-renew and give rise to differentiated cells that phenotypically recapitulate the original tumor, thereby causing relapse and patient death. These cancer stem cells present a unique opportunity to better understand the biology of solid tumors in general, as well as targets for future therapeutics. The general objective of the current study is to discuss the fundamental concepts for understanding the role of CSCs with respect to chemoresistance, radioresistance, special cell surface markers, cancer recurrence and metastasis in tumors of the osseous spine. This discussion is followed by a specific review of what is known about the role of CSCs in chordoma, the most common primary malignant osseous tumor of the spine. PMID:24567788

  20. Occlusion of the abdominal aorta by balloon dilation catheter assisting surgical excision of a sacrum chordoma: case report.

    Science.gov (United States)

    Ozgiray, Erkin; Cağli, Sedat; Zileli, Mehmet; Cinar, Celal; Oran, Ismail

    2009-07-01

    Chordoma is known to be the most common primary tumor of the sacrum. Its surgery is challenging from many aspects. A large amount of bleeding is one of the biggest issues. A 52-year-old woman was admitted to our clinic with a huge mass at sacrum. The mass was diagnosed as chordoma after a needle biopsy. Prior to the surgery, a balloon dilation catheter (BDC) was placed in the distal abdominal aorta via the femoral artery. Just after the skin incision, the BDC was inflated with contrast medium and total occlusion of the aorta was achieved. At the end of the operation we observed that total hemorrhage had decreased dramatically. No complications were recorded except hypertension during the occlusion of the aorta which was expected. The patient was discharged after two weeks of hospitalization post-operatively without any neurological deficit. Our report is the second in the neurosurgical literature to our knowledge. Although this result is preliminary and needs to be replicated and expanded upon, our first experience with this method is effective for decreasing the amount of bleeding. We believe that our method prevents hemo-dynamical problems caused by massive bleeding and complications secondary to massive transfusion. PMID:19621292

  1. Transoral vs. Endoscopic Endonasal Approach for Clival/Upper Cervical Chordoma

    Science.gov (United States)

    SHIDOH, Satoka; TODA, Masahiro; KAWASE, Takeshi; NAKAJIMA, Hideo; TOMITA, Toshiki; OGAWA, Kaoru; YOSHIDA, Kazunari

    2014-01-01

    The surgical results of 18 cases of clival/upper cervical chordoma treated in the last decade via the endoscopic endonasal approach (EEA, 9 cases) and the transoral-transpalatal approach (TO-TPA, 9 cases) were compared. Each group showed the same incidence of subdural invasion, with 5 cases each. The superior (frontal base) and lateral surgical fields were wider by EEA, but the inferior view lower than the cranio-vertebral junction (CVJ) was wider by TO-TPA. Gross total removal was achieved in 3 cases in the EEA group, but in only 1 case in the TO-TPA group. Differences in radicality might be due to the extent of the lateral and subdural overview. However for large tumors extending below the CVJ, TO-TPA was the only viable approach for surgical removal. Surgical complications were higher in the EEA (4 cases) than the TO-TPA group (1 case), and were mainly caused by aggressive management of subdural invasion in the EEA group. Post-operative oral intake was earlier and the operative time was shorter in the EEA group. The surgical results were more radical and less invasive in the EEA group than the TO-TPA group. However in tumors extending below the CVJ, the surgical field in EEA was limited, indicating the need to use the transoral route or a combination of routes. A higher complication rate following subdural management was a negative factor that requires improvement in the EEA group and two-staged EEA followed by a transcranial approach may be considered for the cases with subdural invasion. PMID:25446380

  2. Differentiation of primary chordoma, giant cell tumor and schwannoma of the sacrum by CT and MRI

    Energy Technology Data Exchange (ETDEWEB)

    Si, Ming-Jue, E-mail: smjsh@hotmail.com [Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China); Wang, Cheng-Sheng [Department of Radiology, Union Hospital, Fujian Medical University, Fuzhou 350001 (China); Ding, Xiao-Yi, E-mail: dingxiaoyi1965@hotmail.com [Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China); Yuan, Fei, E-mail: yuanfeirj@hotmail.com [Department of Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China); Du, Lian-Jun; Lu, Yong [Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China); Zhang, Wei-Bin [Department of Orthopedics, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025 (China)

    2013-12-01

    Objective: To evaluate criteria to differentiate sacral chordoma (SC), sacral giant cell tumor (SGCT) and giant sacral schwannoma (GSS) with CT and MRI. Materials and methods: CT and MR images of 22 SCs, 19 SGCTs and 8 GSSs were reviewed. The clinical and imaging features of each tumor were analyzed. Results: The mean ages of SC, SGCT and GSS were 55.1 ± 10.7, 34.3 ± 10.7 and 42.4 ± 15.7 years old. SCs (77.3%) were predominantly located in the midline of lower sacrum, while most SGCTs (73.7%) and GSSs (87.5%) were eccentrically located in upper sacrum. There were significant differences in age, location, eccentricity, morphology of bone residues, intratumoral bleeding and septations. Multiple small cysts were mainly observed in SGCTs (73.7%) with large central cysts in GSSs (87.5%). SGCTs expanded mainly inside sacrum while SCs and GSSs often extended into pelvic cavity (P = 0.0022). Involvement of sacroiliac joints and muscles were also different. Ascending extension within sacral canal was only displayed in SCs. The preservation of intervertebral discs showed difference between large and small tumors (P = 0.0002), regardless of tumor type (P = 0.095). No significant difference was displayed in gender (P = 0.234) or tumor size (P = 0.0832) among three groups. Conclusion: Age, epicenter of the lesion (midline vs. eccentric and upper vs. lower sacral vertebra), bone residues, cysts, bleeding, septation, expanding pattern, muscles and sacroiliac joint involvement can be criteria for diagnosis. Fluid–fluid level is specific for SGCTs and ascending extension within the sacral canal for SCs. The preservation of intervertebral discs is related to tumor size rather than tumor type.

  3. Expression of programmed death-1 ligand (PD-L1) in tumor-infiltrating lymphocytes is associated with favorable spinal chordoma prognosis

    Science.gov (United States)

    Zou, Ming-Xiang; Peng, An-Bo; Lv, Guo-Hua; Wang, Xiao-Bin; Li, Jing; She, Xiao-Ling; Jiang, Yi

    2016-01-01

    Aberrant expression of programmed death-1 (PD-1) receptor/PD-1 ligand (PD-L1) proteins alters human immunoresponse and promotes tumor development and progression. We assessed the expression status of PD-1 and PD-L1 in spinal chordoma tissue specimens and their association with clinicopathological characteristics of patients. Formalin-fixed paraffin-embedded tumor samples from 54 patients with spinal chordoma were collected for immunohistochemical analysis of PD-1 and PD-L1 expression. The association of the expression levels of PD-1 and PD-L1 with clinicopathological variables and survival data were statistically analyzed. Lymphocyte infiltrates were present in all 54 patient samples. Of 54 samples, 37 (68.5%) had both positive PD-1 and PD-L1 expression in tumor cell membrane. Moreover, 38 (70.4%) and 12 (22.2%) had positive PD-1 and PD-L1 expression in tumor-infiltrating lymphocytes (TILs), respectively. Tumors with positive PD-L1 expression were significantly associated with advanced stages of chordoma (p = 0.041) and TIL infiltration (p = 0.005), and had a borderline association with tumor grade (p = 0.051). However, positive tumor PD-L1 expression was not significantly associated with local recurrence-free survival (LRFS) or overall survival (OS). PD-1 expression in TILs was associated with poor LRFS (χ2 = 10.051, p = 0.002, log-rank test). Multivariate analysis showed that PD-L1 expression only in TILs was an independent predictor for LRFS (HR = 0.298, 95% CI: 0.098-0.907, p = 0.033), and OS (HR = 0.188, 95% CI: 0.051-0.687, p = 0.011) in spinal chordoma patients. In conclusion, PD-L1 expression in TILs was an independent predictor for both LRFS and OS in spinal chordoma patients. Our findings suggest that the PD-1/PD-L1 pathway may be a novel therapeutic target for the immunotherapy of chordoma. PMID:27508049

  4. Clinical treatment of sacral chordoma%骶骨脊索瘤的临床病理分析

    Institute of Scientific and Technical Information of China (English)

    孙成良; 董军; 李栋

    2011-01-01

    为了探讨骶骨脊索瘤的临床特点、外科治疗方法与疗效,回顾性分析接受手术治疗的26例骶骨脊索瘤患者的临床资料.26例患者均无术中死亡,术后平均随访3.5年,7例患者局部复发,局部复发率26.9%,肺转移1例,死亡2例,术后病理检查可见典型的“Physaliphorous”细胞.初步研究结果提示,骶骨脊索瘤早期症状不典型,诊断应重视CT和MR检查.广泛手术切除是有效的治疗方式,结合术中化疗和术后局部辅助放疗可降低肿瘤的局部复发率,延长生存期,提高患者的生活质量.%The objective of this study was to analyze the clinical characteristics and surgical treatment results of patients with sacral chordoma. Twenty-six patients with surgical treatment were analyzed retrospectivly. No intraop-erative death occurred. After average 3. 5 years follow-up, 7 patients had recurred, with a recurrence rate of 26. 9%. The most recurrence was 4 times in one patients. One patient had pulmonary metastasis, and 2 patients died. The typical "Physaliphorous cell" could be found in the oncologic examination in all patients. In conclusion, the early clinical manifestation of sacral chordoma is not typical, so the CT and MR should be paid for more attention. Complete resection of tumor are an effective way to treat the sacral chordoma. Combine with intraoperative chemothery and postoperative adjuvant radiotherapy can reduce the tumor recurrence rate, prolong the survival time, and improve life quality of patients.

  5. Advances in the Treatment of Skull Base Chordomas%颅底脊索瘤的治疗进展

    Institute of Scientific and Technical Information of China (English)

    柳义夫; 杨义

    2006-01-01

    脊索瘤(chordoma)是来源于原始脊索组织的肿瘤,其发生部位也多见于残余脊索常见部位即骶尾部(50%)、颅底斜坡区(35%)及椎体(15%).本文主要复习了颅底脊索瘤,重点讨论其手术治疗和放射治疗的适应证、治疗时机和疗效.通过复习国内外近期文献报道,我们认为,尽管脊索瘤的生物学行为及其治疗存在很多争论,争取根治性切除,术后辅以质子束放疗是目前最佳治疗方案.

  6. 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

  7. Randomised trial of proton vs. carbon ion radiation therapy in patients with chordoma of the skull base, clinical phase III study HIT-1-Study

    Directory of Open Access Journals (Sweden)

    Jensen Alexandra D

    2010-11-01

    Full Text Available Abstract Background Chordomas of the skull base are relative rare lesions of the bones. Surgical resection is the primary treatment standard, though complete resection is nearly impossible due to close proximity to critical and hence also dose limiting organs for radiation therapy. Level of recurrence after surgery alone is comparatively high, so adjuvant radiation therapy is very important for the improvement of local control rates. Proton therapy is the gold standard in the treatment of skull base chordomas. However, high-LET beams such as carbon ions theoretically offer biologic advantages by enhanced biologic effectiveness in slow-growing tumors. Methods/design This clinical study is a prospective randomised phase III trial. The trial will be carried out at Heidelberger Ionenstrahl-Therapie centre (HIT and is a monocentric study. Patients with skull base chordoma will be randomised to either proton or carbon ion radiation therapy. As a standard, patients will undergo non-invasive, rigid immobilization and target volume delineation will be carried out based on CT and MRI data. The biologically isoeffective target dose to the PTV in carbon ion treatment (accelerated dose will be 63 Gy E ± 5% and 72 Gy E ± 5% (standard dose in proton therapy respectively. Local-progression free survival (LPFS will be analysed as primary end point. Toxicity and overall survival are the secondary end points. Additional examined parameters are patterns of recurrence, prognostic factors and plan quality analysis. Discussion Up until now it was impossible to compare two different particle therapies, i.e. protons and carbon ions directly at the same facility. The aim of this study is to find out, whether the biological advantages of carbon ion therapy can also be clinically confirmed and translated into the better local control rates in the treatment of skull base chordomas. Trial registration ClinicalTrials.gov identifier: NCT01182779

  8. Positron Emission Tomography/Computed Tomography Imaging of Residual Skull Base Chordoma Before Radiotherapy Using Fluoromisonidazole and Fluorodeoxyglucose: Potential Consequences for Dose Painting

    Energy Technology Data Exchange (ETDEWEB)

    Mammar, Hamid, E-mail: hamid.mammar@unice.fr [Radiation Oncology Department, Antoine Lacassagne Center, Nice (France); CNRS-UMR 6543, Institute of Developmental Biology and Cancer, University of Nice Sophia Antipolis, Nice (France); Kerrou, Khaldoun; Nataf, Valerie [Department of Nuclear Medicine and Radiopharmacy, Tenon Hospital, and University Pierre et Marie Curie, Paris (France); Pontvert, Dominique [Proton Therapy Center of Orsay, Curie Institute, Paris (France); Clemenceau, Stephane [Department of Neurosurgery, Pitie-Salpetriere Hospital, Paris (France); Lot, Guillaume [Department of Neurosurgery, Adolph De Rothschild Foundation, Paris (France); George, Bernard [Department of Neurosurgery, Lariboisiere Hospital, Paris (France); Polivka, Marc [Department of Pathology, Lariboisiere Hospital, Paris (France); Mokhtari, Karima [Department of Pathology, Pitie-Salpetriere Hospital, Paris (France); Ferrand, Regis; Feuvret, Loiec; Habrand, Jean-louis [Proton Therapy Center of Orsay, Curie Institute, Paris (France); Pouyssegur, Jacques; Mazure, Nathalie [CNRS-UMR 6543, Institute of Developmental Biology and Cancer, University of Nice Sophia Antipolis, Nice (France); Talbot, Jean-Noeel [Department of Nuclear Medicine and Radiopharmacy, Tenon Hospital, and University Pierre et Marie Curie, Paris (France)

    2012-11-01

    Purpose: To detect the presence of hypoxic tissue, which is known to increase the radioresistant phenotype, by its uptake of fluoromisonidazole (18F) (FMISO) using hybrid positron emission tomography/computed tomography (PET/CT) imaging, and to compare it with the glucose-avid tumor tissue imaged with fluorodeoxyglucose (18F) (FDG), in residual postsurgical skull base chordoma scheduled for radiotherapy. Patients and Methods: Seven patients with incompletely resected skull base chordomas were planned for high-dose radiotherapy (dose {>=}70 Gy). All 7 patients underwent FDG and FMISO PET/CT. Images were analyzed qualitatively by visual examination and semiquantitatively by computing the ratio of the maximal standardized uptake value (SUVmax) of the tumor and cerebellum (T/C R), with delineation of lesions on conventional imaging. Results: Of the eight lesion sites imaged with FDG PET/CT, only one was visible, whereas seven of nine lesions were visible on FMISO PET/CT. The median SUVmax in the tumor area was 2.8 g/mL (minimum 2.1; maximum 3.5) for FDG and 0.83 g/mL (minimum 0.3; maximum 1.2) for FMISO. The T/C R values ranged between 0.30 and 0.63 for FDG (median, 0.41) and between 0.75 and 2.20 for FMISO (median,1.59). FMISO T/C R >1 in six lesions suggested the presence of hypoxic tissue. There was no correlation between FMISO and FDG uptake in individual chordomas (r = 0.18, p = 0.7). Conclusion: FMISO PET/CT enables imaging of the hypoxic component in residual chordomas. In the future, it could help to better define boosted volumes for irradiation and to overcome the radioresistance of these lesions. No relationship was founded between hypoxia and glucose metabolism in these tumors after initial surgery.

  9. 脊索瘤治疗的临床及基础研究进展%Advance in clinical and basic research on the treatment of chordoma

    Institute of Scientific and Technical Information of China (English)

    李晓祥; 姬振伟; 马云雷; 范清宇

    2011-01-01

    Chordoma is a rare neoplasm approximately arising in the axial skeleton. Because chordoma is not sensitive to radiotherapy and chemotherapy while there are many difficulties in the processes of surgery, it presents to be a locally aggressive tumor with a high recurrence rate. Here we present a brief review focusing on the progression clini-cal and basic research which provides new means of treatment,and improves the prognosis of patients of chordoma.%脊索瘤作为一种罕见的恶性骨肿瘤,对于传统的放化疗均不敏感,而外科手术治疗过程中也存在诸多困难.病人术后的多次复发是脊索瘤的主要临床特点.目前针对脊索瘤的临床以及基础研究有了一些新的进展,为脊索瘤的治疗提供了新的治疗手段,为改善患者预后带来了希望,本文就其进展做一简要综述.

  10. Imaging of sacral chordoma: comparison between MRI and CT; Bildgebende Diagnostik bei Chordomen des Beckens: Vergleich der Modalitaeten CT und MRT

    Energy Technology Data Exchange (ETDEWEB)

    Plathow, C.; Weber, M.-A.; Kauczor, H.-U. [Deutsches Krebsforschungszentrum (DKFZ) Heidelberg, Abteilung Radiologie (Germany); Debus, J. [Universitaet Heidelberg, Abteilung Strahlentherapie (Germany)

    2005-01-01

    Sacral chordoma is a primarily benign tumor with a high tendency to recur. A correct definition of tumor volume is a significant prognostic factor. We compared the value of CT and MRI in diagnosing a sacral chordoma. 31 patients with a histologically proven sacral chordoma were included in this study. Following parameters were analyzed by two independent radiologists: septation, signs of blood and ossification in the tumor tissue, contrast enhancement, maximal tumor diameter, infiltration of the soft tissue, the dural salc and the cauda equina and multifocality. In CT all chordomas showed a hypodensity to the normal tissue and in MRI a hyperintensity on T2w images with a low level of contrast enhancement. On the basis of the more precise soft tissue contrast of MRI compared with CT, MRI was significantly more accurate in all tested parameters (p <0.05) besides in detecting tumor ossification. In CT tumor volume was frequently underestimated. MRI in sacral chordoma is an essential tool in the pretherapeutic diagnostic regimen and in a state of relapse. (orig.) [German] Das sakrale Chordom ist ein primaer gutartiger Tumor des Knochenskeletts. Eine korrekte und ausreichende Tumorvolumendefinition zur Therapieplanung ist ein entscheidender Prognoseparameter aufgrund der hohen Rate an Rezidiven. Wir untersuchten die Wertigkeit der CT und MRT in der Diagnostik des sakralen Chordoms. Mittels CT und MRT wurden bei 31 Patienten mit histologisch gesichertem sakralem Chordom Septierung, Ossifikation, Zeichen einer Einblutung, die Kontrastmittelaufnahme des Tumors, die maximale axiale Tumorausdehnung, Weichteilinfiltration, Infiltration des Duralsackes, der Cauda equina und eine Multifokalitaet durch 2 unabhaengige Radiologen untersucht und bewertet. Alle Chordome zeigten in der CT nativ ein hypodenses und in der MRT ein T2w-hyperintenses Verhalten mit nur maessiger Kontrastmittelaufnahme. Unter Zugrundelegung des besseren Weichteilkontrastes der MRT gegenueber der CT erwies

  11. Randomized phase II trial of hypofractionated proton versus carbon ion radiation therapy in patients with sacrococcygeal chordoma-the ISAC trial protocol

    Science.gov (United States)

    2014-01-01

    Background Chordomas are relatively rare lesions of the bones. About 30% occur in the sacrococcygeal region. Surgical resection is still the standard treatment. Due to the size, proximity to neurovascular structures and the complex anatomy of the pelvis, a complete resection with adequate safety margin is difficult to perform. A radical resection with safety margins often leads to the loss of bladder and rectal function as well as motoric/sensoric dysfunction. The recurrence rate after surgery alone is comparatively high, such that adjuvant radiation therapy is very important for improving local control rates. Proton therapy is still the international standard in the treatment of chordomas. High-LET beams such as carbon ions theoretically offer biologic advantages in slow-growing tumors. Data of a Japanese study of patients with unresectable sacral chordoma showed comparable high control rates after hypofractionated carbon ion therapy only. Methods and design This clinical study is a prospective randomized, monocentric phase II trial. Patients with histologically confirmed sacrococcygeal chordoma will be randomized to either proton or carbon ion radiation therapy stratified regarding the clinical target volume. Target volume delineation will be carried out based on CT and MRI data. In each arm the PTV will receive 64 GyE in 16 fractions. The primary objective of this trial is safety and feasibility of hypofractionated irradiation in patients with sacrococygeal chordoma using protons or carbon ions in raster scan technique for primary or additive treatment after R2 resection. The evaluation is therefore based on the proportion of treatments without Grade 3–5 toxicity (CTCAE, version 4.0) up to 12 months after treatment and/or discontinuation of the treatment for any reason as primary endpoint. Local-progression free survival, overall survival and quality of life will be analyzed as secondary end points. Discussion The aim of this study is to confirm the toxicity

  12. Comparison of human chordoma cell-kill for 290 MeV/n carbon ions versus 70 MeV protons in vitro

    International Nuclear Information System (INIS)

    While the pace of commissioning of new charged particle radiation therapy facilities is accelerating worldwide, biological data pertaining to chordomas, theoretically and clinically optimally suited targets for particle radiotherapy, are still lacking. In spite of the numerous clinical reports of successful treatment of these malignancies with this modality, the characterization of this malignancy remains hampered by its characteristic slow cell growth, particularly in vitro. Cellular lethality of U-CH1-N cells in response to different qualities of radiation was compared with immediate plating after radiation or as previously reported using the multilayered OptiCell™ system. The OptiCell™ system was used to evaluate cellular lethality over a broad dose-depth deposition range of particle radiation to anatomically mimic the clinical setting. Cells were irradiated with either 290 MeV/n accelerated carbon ions or 70 MeV accelerated protons and photons and evaluated through colony formation assays at a single position or at each depth, depending on the system. There was a cell killing of approximately 20–40% for all radiation qualities in the OptiCell™ system in which chordoma cells are herein described as more radiation sensitive than regular colony formation assay. The relative biological effectiveness values were, however, similar in both in vitro systems for any given radiation quality. Relative biological effectiveness values of proton was 0.89, of 13–20 keV/μm carbon ions was 0.85, of 20–30 keV/μm carbon ions was 1.27, and >30 keV/μm carbon ions was 1.69. Carbon-ions killed cells depending on both the dose and the LET, while protons depended on the dose alone in the condition of our study. This is the first report and characterization of a direct comparison between the effects of charged particle carbon ions versus protons for a chordoma cell line in vitro. Our results support a potentially superior therapeutic value of carbon particle irradiation

  13. Randomized phase II trial of hypofractionated proton versus carbon ion radiation therapy in patients with sacrococcygeal chordoma-the ISAC trial protocol

    International Nuclear Information System (INIS)

    Chordomas are relatively rare lesions of the bones. About 30% occur in the sacrococcygeal region. Surgical resection is still the standard treatment. Due to the size, proximity to neurovascular structures and the complex anatomy of the pelvis, a complete resection with adequate safety margin is difficult to perform. A radical resection with safety margins often leads to the loss of bladder and rectal function as well as motoric/sensoric dysfunction. The recurrence rate after surgery alone is comparatively high, such that adjuvant radiation therapy is very important for improving local control rates. Proton therapy is still the international standard in the treatment of chordomas. High-LET beams such as carbon ions theoretically offer biologic advantages in slow-growing tumors. Data of a Japanese study of patients with unresectable sacral chordoma showed comparable high control rates after hypofractionated carbon ion therapy only. This clinical study is a prospective randomized, monocentric phase II trial. Patients with histologically confirmed sacrococcygeal chordoma will be randomized to either proton or carbon ion radiation therapy stratified regarding the clinical target volume. Target volume delineation will be carried out based on CT and MRI data. In each arm the PTV will receive 64 GyE in 16 fractions. The primary objective of this trial is safety and feasibility of hypofractionated irradiation in patients with sacrococygeal chordoma using protons or carbon ions in raster scan technique for primary or additive treatment after R2 resection. The evaluation is therefore based on the proportion of treatments without Grade 3–5 toxicity (CTCAE, version 4.0) up to 12 months after treatment and/or discontinuation of the treatment for any reason as primary endpoint. Local-progression free survival, overall survival and quality of life will be analyzed as secondary end points. The aim of this study is to confirm the toxicity results of the Japanese data in raster

  14. 18F-FMISO PET/CT Visualization of Tumor Hypoxia in Patients with Chordoma of the Mobile and Sacrococcygeal Spine

    Science.gov (United States)

    Cheney, Matthew D.; Chen, Yen-Lin; Lim, Ruth; Winrich, Barbara K.; Grosu, Anca L.; Trofimov, Alexei V.; Depauw, Nicolas; Shih, Helen A.; Schwab, Joseph H.; Hornicek, Francis J.; DeLaney, Thomas F.

    2014-01-01

    Summary Local recurrence (LR) rates in chordoma patients following surgery ± radiation therapy (RT) or definitive RT are high. Tumor hypoxia is associated with radioresistance and LR. In this prospective study, [18F]-FMISO-PET/CT detected hypoxic tumor sub-volumes in 60% of patients with chordoma of the mobile and sacrococcygeal spine, the majority of which were sufficiently large to allow an RT boost. Further study of hypoxia-directed, dose-escalated RT, particularly in patients at high risk for LR, is warranted. Purpose/Objectives Local recurrence (LR) rates in chordoma patients following surgery ± radiation therapy (RT) or definitive RT are high. Tumor hypoxia is associated with radioresistance and LR. [18F] fluoromisonidazole positron emission tomography/computed tomography (FMISO-PET/CT) can visualize skull base chordoma hypoxic sub-volumes (HSV) and feasibility of hypoxia-directed RT dose-escalation has been demonstrated in head and neck cancer. This study investigates FMISO-PET/CT detection of targetable HSVs in chordoma of the mobile or sacrococcygeal spine. Methods and Materials A prospective, pilot study of 20 patients with primary or locally recurrent chordoma of the mobile or sacrococcygeal spine treated with proton or combined proton/photon RT ± surgery was completed. FMISO-PET/CT was performed prior to RT and after 19.8-34.2 GyRBE (relative biologic effectiveness). Gross tumor volumes (GTV) were delineated and HSVs defined including voxels with standardized uptake values ≥ 1.4 times the muscle mean. Clinical characteristics and treatments received were compared between patients with and without HSVs. Results FMISO-PET/CT detected HSVs in 12 (60%; 12/20) patients. Baseline and interval HSV spatial concordance varied (0-94%). Eight HSVs were sufficiently large (≥ 5cc) to potentially allow an intensity modulated proton therapy boost. Patients with HSVs had significantly larger GTVs (median =410.0 cc vs. 63.4 cc; p=0.02) and were significantly more

  15. Imaging manifestations of chordoma in the cervical vertebrae%颈椎脊索瘤的影像学表现

    Institute of Scientific and Technical Information of China (English)

    许霖; 周毅; 杜希娜; 崔博; 吕秀花; 杨广夫

    2015-01-01

    Objective To analyze the X-ray,CT and MRI findings of chordoma in the cervical vertebrae,so as to improve its diagnostic accu-racy.Methods X-ray,CT and MRI findings of 7 cases of chordoma proved pathologically were analyzed retrospectively.X-ray and CT were undergone in all 7 cases,MRI in 3 cases.Results X-ray demonstrated bony destruction in 7 cases,bony collapse in 1 case. Narrowing of inter-vertebral space was in 6 cases and soft tissue mass was in 3 cases.CT demonstrated bony destruction in 7 cases, calcification within the tumors in 2 cases,involvement of the accessory of the cervical vertebra and transverse foramen in 5 cases. Soft tissue mass was around the cervical vertebra in 4 cases.MRI was undergone in 3 cases.On T1 WI middle signal in 3 cases were showed,on T2 WI low and high mixed signal in 3 cases,and soft tissue masses around the lesion in 3 cases.Conclusion Chordoma of the cervical vertebrae is uncommon.Full understanding X-ray,CT,MRI characteristics is useful to improve the diagnosis,guide clinical surgery treatment and forecast prognosis.%目的:分析颈椎脊索瘤的 X线、CT和 MRI影像学特点,提高本病的诊断水平。方法收集经手术病理证实的7例颈椎脊索瘤患者的 X线、CT和 MRI的影像学资料并对其进行回顾性分析。7例患者均行 X线、CT检查,3例行 MRI检查。结果X线检查:7例椎体呈骨质破坏,1例椎体结构塌陷,6例累及邻近椎间隙并致其变窄,3例周围见软组织肿块。CT检查:7例椎体呈溶骨性骨质破坏,2例见钙化及残骨组织,5例肿瘤累及椎附件及横突孔,4例周围见软组织肿块。3例行 MRI 检查:T1 WI 呈等信号3例,T2 WI呈高低混杂信号3例。3例周围见软组织肿块。结论颈椎脊索瘤较为少见,应充分分析影像学特点,对提高诊断、指导临床手术治疗及预后有重要的意义。

  16. Radiation Therapy of a Chordoma of the Thoracic Vertebra-a Case Report and Review of Literatures-

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Joo Young; Choi, Myung Sun [Korea University College of Medicine, Seoul (Korea, Republic of)

    1988-12-15

    Chordom is a malignant tumor arising from the primitive notochord involving the axial skeleton. It usually occurs at sacrococcygeal and basisphenoidal area but only rarely does at other vertebral areas, especially at the thoracic vertebrae. It has a slow growth rate and is locally aggressive with an extremely high rate of local recurrence. Either surgery or radiation alone often fails to cure the disease and the local failure is the main cause of treatment failure and death. Overall 5 year survival rate is less than 10%. Useful palliation or occasional cure can be obtained by the combination of surgery and radiotherapy. After incomplete resection, the tumor requires radiation dose of 7,000 cGy or more over 6-7 weeks for local control. Tumor regression is slow in response to irradiation and continuation of the regression for several months after completion of RT is not unusual. We report a case of chordoma of the thoracic vertebra, the site of extreme rarity, which showed good local control after partial resection and radiation therapy. He is well and alive without any evidence of recurrence after 13 months of treatment with near complete tumor regression.

  17. 颅底脊索瘤的诊断及治疗现状%Diagnosis and Treatment of Skull Base Chordomas

    Institute of Scientific and Technical Information of China (English)

    颜高姝; 黄丹丹

    2010-01-01

    @@ 脊索瘤(chordoma)是一种少见的,起源于错置或残留的胚胎脊索组织的肿瘤.其发生部位常见于骶尾部(50%)、颅底斜坡区(35%)及椎体(15%).其中颅底脊索瘤的位置深在,侵犯周围骨质、神经和大血管等重要结构,治疗困难.本文就该肿瘤诊断及治疗现状作一综述.

  18. Benign chordoma of the sacral bone. Radiologic appearance and differential dignosis; Benignes Chordom des Os Sacrum. Radiologische Morphologie und differential-diagnostische Aspekte

    Energy Technology Data Exchange (ETDEWEB)

    Pegios, W. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Vogl, T.J. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Rausch, M. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Klein, U. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Balzer, J.O. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Hammerstingl, R. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Mack, M.G. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany); Felix, R. [Strahlenklinik und Poliklinik, Freie Univ. Berlin (Germany)

    1994-12-31

    Chordomas constitute 3-4% of all primary bony tumors [17, 20] and they arise from remnants of the notochord [4]. They can occur anywhere along the skull base and spine, where the notochord extends. 50% arise in the sacrum, 35% in the clivus and 15% in the vertebrae [17, 20]. Chordomas usually occur after the second decade with the highest incidence between the fifth and seventh decade. There is a male predominance, with roughly a 2 to 1 male-to-female ratio. Children are rarely affected [5, 25, 34]. In this article a case of a patient with a Chordoma of the sacrum is presented. After a fall on the coccyx the patient complained of recurrent and altogether increasing pain for some years. The clinical diagnosis was fracture of the coccyx with consecutive formation of callus. Finally the MRI showed a characteristically increased signal intensity in the T2-weighted spin-echo sequence (SE). With the help of MRI guided biopsy the diagnosis of a benign highly differentiated chordoma could be confirmed. (orig.) [Deutsch] Chordome stellen 3 bis 4% aller primaeren Knochentumoren dar [17, 20] und gehen von den Resten der primitiven Chorda dorsalis aus [4]. Sie koennen ueberall dort auftreten, wo embryonale Reste des Chordagewebes vorhanden sind: 50% im Sacrum, 35% im Clivus und mit 15% an den Wirbelkoepern [17, 20]. Chordome werden in der Regel nach der zweiten Lebensdekade beobachtet und erreichen ihre hoechste Inzidenz zwischen der fuenften und der siebten Lebensdekade. Sie zeigen eine Praeferenz fuer das maennliche Geschlecht mit eine Relation von ungefaehr 2:1. Kinder sind seltener betroffen [5, 25, 34]. Im folgenden soll ein Patient mit einem Chordom des Os Sacrum vorgestellt werden. Nach Sturz auf das Steissbein klagte der Patient jahrelang ueber rezidivierende und insgesamt zunehmende Schmerzsymptomatik. Die klinische Diagnose lautete Zustand nach Steissbeinfraktur mit Kallusbildung. Die schliesslich durchgefuehrte MRT zeigte eine charakteristische erhoehte

  19. NUCLEAR BRACHYURY EXPRESSION IS CONSISTENT IN CHORDOMA, COMMON IN GERM CELL TUMORS AND SMALL CELL CARCINOMAS AND RARE IN OTHER CARCINOMAS AND SARCOMAS. AN IMMUNOHISTOCHEMICAL STUDY OF 5229 CASES

    Science.gov (United States)

    Miettinen, Markku; Wang, Zengfeng; Lasota, Jerzy; Heery, Christopher; Schlom, Jeffrey; Palena, Claudia

    2015-01-01

    Brachyury is a transcription factor of the T-box family typically expressed in notochord and chordoma. Some studies report brachyury as highly specific for chordoma, whereas others have concluded that brachyury is expressed in many types of common carcinomas by RT-PCR and immunohistochemistry and could be involved in the epithelial-mesenchymal transition and metastatic process. In this study, we immunohistochemically evaluated 5229 different tumors for nuclear brachyury expression using a new rabbit monoclonal antibody and automated immunostaining (Leica Bond Max). Only nuclear labeling was scored, and antibody dilution of 1:2000 was used. In normal tissues, only rare cells in seminiferous tubules were labeled; all other organs were negative. All chordomas (75/76), except a sarcomatous one, were positive, whereas chondrosarcomas were negative. Among epithelial tumors, positivity was often detected in embryonal carcinoma (74%) and seminoma (45%). Pulmonary small cell carcinoma was often positive (41%), whereas pulmonary and pancreatic adenocarcinomas only rarely showed nuclear brachyury-positivity (3–4%). Common carcinomas such as ductal carcinomas of breast, or adenocarcinomas of the prostate only exceptionally showed nuclear positivity (< 1%). No colorectal, hepatocellular, renal cell, squamous cell, thyroid or urothelial carcinoma, or mesothelioma showed nuclear brachyury-positivity. Among mesenchymal and neuroectodermal tumors, only isolated cases of melanoma, malignant peripheral nerve sheath tumor, rhabdomyosarcoma, synovial sarcoma, and follicular lymphoma showed nuclear expression. However, as shown previously with lung carcinoma, experiments with lower antibody dilutions (1:200–1:500) showed weak cytoplasmic and nuclear labeling in breast cancers. In addition to chordoma, we show here for the first time that nuclear brachyury expression is prevalent in embryonal carcinoma, seminoma, and small cell carcinoma of the lung but very rare in common carcinomas

  20. Ressecção de cordoma sacral com abaixamento de cólon: relato de caso Sacral chordoma resection with colo anal anastomosis: case report

    Directory of Open Access Journals (Sweden)

    Kanthya Arreguy de Sena

    2006-09-01

    Full Text Available O cordoma é uma lesão maligna da medula espinhal, que se origina nos remanescentes ectópicos de tecido notocordial. Trata-se de neoplasia rara e sua localização preferencial é o sacro. Tem crescimento lento, mas comportamento localmente agressivo. Relatamos um caso de ressecção de cordoma sacral com abaixamento de cólon. Para extirpação oncológica da lesão foi necessária a abordagem colorretal e ortopédica. Novas técnicas preconizando cirurgias mais agressivas melhoraram significativamente a expectativa de vida dos pacientes portadores de cordoma, bem como o tempo de vida livre de doença. Na busca dessa cirurgia oncológica ideal, a abordagem multidisciplinar é essencial.Chordoma is a rare malignant lesion of spinal cord originated from ectopic remnants of notocordial tissue. Its presentation is characterized by slow growth in spite of locally aggressive behaviour. We report a case of multidisciplinary oncologic resection with coloanal anastomosis. Extended and aggressive resections improved significantly the disease free survival in patients with chordoma. The multidisciplinary oncologic approach is essential in order to reach this goal.

  1. VSD技术治疗骶尾部脊索瘤一例%A case of VSD technique in the treatment of sacrococcygeal chordoma

    Institute of Scientific and Technical Information of China (English)

    徐凯; 魏永鸽; 崔正军; 黄器伟

    2015-01-01

    目的:探讨VSD技术治疗骶尾部脊索瘤伴褥疮创面的适应证、禁忌证及疗效。方法:采用在我科收治的1例骶尾部脊索瘤伴褥疮创面患者,进行治疗和观察,记录并分析整理临床相关数据。结果:经对该患者进行持续3次VSD技术治疗后,术后创面恢复效果显著。结论:该技术在外科治疗领域,尤其是难治性创面方面有显著疗效。%Objective To explore the VSD technique for the treatment of sacrococcygeal chordoma with bedsore wound disease,contraindication and curative effect. Methods In a case of sacrococcygeal chordoma treated in our department with bedsore wound patients,treatment and observation, recording and analysis of clinical data. Results The patients were 3 times of VSD after treatment,postoperative wound healing effect. Conclusion The technology in the field of surgical treatment, especially has a significant effect on refractory wounds.

  2. 颅底脊索瘤影像特征及治疗进展%Imaging characteristics and treatment progress of skull base chordoma

    Institute of Scientific and Technical Information of China (English)

    覃相志; 雷进

    2015-01-01

    脊索瘤是局部呈侵袭性生长的罕见肿瘤,起源于胚胎残余的脊索,<1/3发生于颅底。手术是颅底脊索瘤的首选治疗方案,术后常结合放射治疗,但由于其解剖位置、生物学特性、预后不良等使脊索瘤治疗相对棘手。近年来随着神经影像学技术和微创神经外科的发展,脊索瘤的诊断和预后在一定程度上得到改善。%Chordoma,originated from the spinal cord of embryonic residual,is a rare tumor whose growth is invasive lo-cally.Around one third of the cases start from the base of skull.Surgery is used as a first treatment plan for chordoma, often combined with postoperative radiotherapy,because of its anatomical location,biological characterstics and undesir-able prognosis,the treatment is relatively difficult.In recent years,with the development of neuroimaging techniques and minimally invasive neurosurgery,the diagnosis and the prognosis are improved to a certain extent.

  3. Chordoma-derived cell line U-CH1-N recapitulates the biological properties of notochordal nucleus pulposus cells.

    Science.gov (United States)

    Fujita, Nobuyuki; Suzuki, Satoshi; Watanabe, Kota; Ishii, Ken; Watanabe, Ryuichi; Shimoda, Masayuki; Takubo, Keiyo; Tsuji, Takashi; Toyama, Yoshiaki; Miyamoto, Takeshi; Horiuchi, Keisuke; Nakamura, Masaya; Matsumoto, Morio

    2016-08-01

    Intervertebral disc degeneration proceeds with age and is one of the major causes of lumbar pain and degenerative lumbar spine diseases. However, studies in the field of intervertebral disc biology have been hampered by the lack of reliable cell lines that can be used for in vitro assays. In this study, we show that a chordoma-derived cell line U-CH1-N cells highly express the nucleus pulposus (NP) marker genes, including T (encodes T brachyury transcription factor), KRT19, and CD24. These observations were further confirmed by immunocytochemistry and flow cytometry. Reporter analyses showed that transcriptional activity of T was enhanced in U-CH1-N cells. Chondrogenic capacity of U-CH1-N cells was verified by evaluating the expression of extracellular matrix (ECM) genes and Alcian blue staining. Of note, we found that proliferation and synthesis of chondrogenic ECM proteins were largely dependent on T in U-CH1-N cells. In accordance, knockdown of the T transcripts suppressed the expression of PCNA, a gene essential for DNA replication, and SOX5 and SOX6, the master regulators of chondrogenesis. On the other hand, the CD24-silenced cells showed no reduction in the mRNA expression level of the chondrogenic ECM genes. These results suggest that U-CH1-N shares important biological properties with notochordal NP cells and that T plays crucial roles in maintaining the notochordal NP cell-like phenotype in this cell line. Taken together, our data indicate that U-CH1-N may serve as a useful tool in studying the biology of intervertebral disc. © 2016 The Authors. Journal of Orthopaedic Research Published by Wiley Periodicals, Inc. on behalf of Orthopaedic Research Society. J Orthop Res 34:1341-1350, 2016.

  4. 骶骨脊索瘤的手术治疗(附15例报告)%Surgical Management of Sacral Chordoma(A Review of Fifteen Cases)

    Institute of Scientific and Technical Information of China (English)

    李帅; 杨操; 杨述华; 邵增务; 郑启新

    2011-01-01

    Objective To evaluate the surgical treatment risks and effects of surgical treatment of sacral chordoma.Methods Fifteen patients with sacral chordoma were followed up. We reviewed the clinical data and analysis of the surgical methods,the causes and treatment measures of complications,and conduct the clinical and imageological evaluation preoperatively and postoperatively. Results The average follow-up time was 49 months. Five cases had died in 5 years postoperation,another one case had died in 2 hours postoperation. Six patients occurred recurrence in 3 years after operation.The dysfunction of bladder occurred in 4 patients whereas none has rectal injury.The symptom of 13 patients in 15 patients had been relieved or disappeared. Conclusion Primary sacral chordoma and recurrence with good general conditions are operative indications. Postoperative recurrenceis related to incomplete removal of tumor. As a whole,the surgical treatments are satisfied,they can prolong the lives of patients and improve the quality of life of patients,relieve pain and symptoms,and create conditions for other adjuvant treatments.%目的 评估骶骨脊索瘤的手术风险和效果.方法 回顾15例骶骨脊索瘤的临床资料,分析手术方式、引起并发症的原因及治疗措施,术前术后作临床及影像学评价.结果 平均随访49个月,术后5年内死亡共5例,另有1例术后2 h死亡.术后3年复发6例.术后发生膀胱功能异常4例,无直肠损伤病例.13例症状缓解或消失.结论 全身状况较好的原发骶骨脊索瘤及复发者均为手术适应证,术后复发与手术局部切除不彻底有关.手术治疗满意,可以延长患者的生命,改善生活质量,减轻痛苦和症状,并为其他辅助治疗创造条件.

  5. Analysis of the MR features in chondroid chordoma: 6 cases report and review of the literature%软骨样脊索瘤6例MR表现

    Institute of Scientific and Technical Information of China (English)

    唐浩; 邹丹凤; 陈卫国

    2012-01-01

    目的 探讨软骨样脊索瘤的MR特征以提高其诊断水平.方法 回顾性分析6例经病理证实的软骨样脊索瘤的MR(均行增强扫描)表现.结果 4例发生于颅内(鞍旁、延髓、枕骨大孔区及右海绵窦各1例),2例位于骶骨;肿瘤呈广泛浸润生长5例,T1WI呈不均匀等低信号块影,其中2例可见斑片状中高信号影,T2WI呈不均匀高信号;6例增强扫描病灶均为不均匀强化;4例可见明显包膜形成,5例均可见明显软组织肿块.结论 MR可清晰显示软骨样脊索瘤的部位、范围、内部成分及与周围神经、血管的关系,具有较高的敏感性和一定特征性.%Objective To explore the MR features of chondroid chordoma with cases report and literature review. Methods A retrospective analysis was performed with MR in 6 cases with pathologically approved chondroid chordoma. All of them have enhancement scanning. Results Four cases were located in parasella, medulla, foramen magnum and right sious cavernosus separately, another 2 cases located in sacrum. 5 cases was extensive infiltration. Signal intensities of the tumor presented low intermediate in T1WI, 2 of them had speckle middle-high signal, and extremely homogeneous high intermediate on T2WI; Asymmetrical enhancement could be seen in all of the cases; 4 cases showed obvious envelope and 5 cases had large soft tissue masses. Conclusion The MR could well display the location, size, interior ingredient of the tumor, as well as its relationship with the adjacent nerves and vessels, so chondroid chordoma was finally confirmed diagnosis rely on pathological examination.

  6. [18F]-Fluoromisonidazole Positron Emission Tomography/Computed Tomography Visualization of Tumor Hypoxia in Patients With Chordoma of the Mobile and Sacrococcygeal Spine

    International Nuclear Information System (INIS)

    Purpose: To investigate [18F]-fluoromisonidazole positron emission tomography/computed tomography (FMISO-PET/CT) detection of targetable hypoxic subvolumes (HSVs) in chordoma of the mobile or sacrococcygeal spine. Methods and Materials: A prospective, pilot study of 20 patients with primary or locally recurrent chordoma of the mobile or sacrococcygeal spine treated with proton or combined proton/photon radiation therapy (RT) with or without surgery was completed. The FMISO-PET/CT was performed before RT and after 19.8-34.2 GyRBE (relative biologic effectiveness). Gross tumor volumes were delineated and HSVs defined including voxels with standardized uptake values ≥1.4 times the muscle mean. Clinical characteristics and treatments received were compared between patients with and without HSVs. Results: The FMISO-PET/CT detected HSVs in 12 of 20 patients (60%). Baseline and interval HSV spatial concordance varied (0%-94%). Eight HSVs were sufficiently large (≥5 cm3) to potentially allow an intensity modulated proton therapy boost. Patients with HSVs had significantly larger gross tumor volumes (median 410.0 cm3 vs 63.4 cm3; P=.02) and were significantly more likely to have stage T2 tumors (5 of 12 vs 0 of 8; P=.04). After a median follow-up of 1.8 years (range, 0.2-4.4 years), a local recurrence has yet to be observed. Three patients developed metastatic disease, 2 with HSVs. Conclusions: Detection of targetable HSVs by FMISO-PET/CT within patients undergoing RT with or without surgery for treatment of chordoma of the mobile and sacrococcygeal spine is feasible. The study's inability to attribute interval HSV changes to treatment, rapidly changing hypoxic physiology, or imaging inconsistencies is a limitation. Further study of double-baseline FMISO-PET/CT and hypoxia-directed RT dose escalation, particularly in patients at high risk for local recurrence, is warranted

  7. [18F]-Fluoromisonidazole Positron Emission Tomography/Computed Tomography Visualization of Tumor Hypoxia in Patients With Chordoma of the Mobile and Sacrococcygeal Spine

    Energy Technology Data Exchange (ETDEWEB)

    Cheney, Matthew D., E-mail: mcheney@lroc.harvard.edu [Harvard Radiation Oncology Program, Boston, Massachusetts (United States); Chen, Yen-Lin [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Lim, Ruth [Department of Diagnostic Radiology, Massachusetts General Hospital, Boston, Massachusetts (United States); Winrich, Barbara K.; Grosu, Anca L.; Trofimov, Alexei V. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Depauw, Nicolas [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Centre of Medical Radiation Physics, University of Wollongong, Wollongong, NSW (Australia); Shih, Helen A. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Schwab, Joseph H.; Hornicek, Francis J. [Department of Orthopedic Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States)

    2014-12-01

    Purpose: To investigate [18F]-fluoromisonidazole positron emission tomography/computed tomography (FMISO-PET/CT) detection of targetable hypoxic subvolumes (HSVs) in chordoma of the mobile or sacrococcygeal spine. Methods and Materials: A prospective, pilot study of 20 patients with primary or locally recurrent chordoma of the mobile or sacrococcygeal spine treated with proton or combined proton/photon radiation therapy (RT) with or without surgery was completed. The FMISO-PET/CT was performed before RT and after 19.8-34.2 GyRBE (relative biologic effectiveness). Gross tumor volumes were delineated and HSVs defined including voxels with standardized uptake values ≥1.4 times the muscle mean. Clinical characteristics and treatments received were compared between patients with and without HSVs. Results: The FMISO-PET/CT detected HSVs in 12 of 20 patients (60%). Baseline and interval HSV spatial concordance varied (0%-94%). Eight HSVs were sufficiently large (≥5 cm{sup 3}) to potentially allow an intensity modulated proton therapy boost. Patients with HSVs had significantly larger gross tumor volumes (median 410.0 cm{sup 3} vs 63.4 cm{sup 3}; P=.02) and were significantly more likely to have stage T2 tumors (5 of 12 vs 0 of 8; P=.04). After a median follow-up of 1.8 years (range, 0.2-4.4 years), a local recurrence has yet to be observed. Three patients developed metastatic disease, 2 with HSVs. Conclusions: Detection of targetable HSVs by FMISO-PET/CT within patients undergoing RT with or without surgery for treatment of chordoma of the mobile and sacrococcygeal spine is feasible. The study's inability to attribute interval HSV changes to treatment, rapidly changing hypoxic physiology, or imaging inconsistencies is a limitation. Further study of double-baseline FMISO-PET/CT and hypoxia-directed RT dose escalation, particularly in patients at high risk for local recurrence, is warranted.

  8. CT diagnosis of skull base chordoma%CT对颅底脊索瘤的诊断价值研究

    Institute of Scientific and Technical Information of China (English)

    罗祥斌; 杜福川

    2010-01-01

    目的:评价CT对颅底脊索瘤(Chordoma)的诊断价值.方法:422例临床拟诊颅底脊索瘤的患者采用CT检查,双盲阅片,以手术病理结果和长期随访结果作为诊断金标准,评价其诊断的特异度、灵敏度、误诊率、漏诊率、阳性预测值和阴性预测值.结果:422例入选患者确诊脊索瘤者为39例(9.2%).诊断灵敏度为0.795(31/39),95%的CI为[0.635-0.907];特异度为0.992(380/383),95%的CI为10.977-0.998];误诊率为0.008(3/383);漏诊率为0.205(8139);阳性预测值为0.912(31/34);阴性预测值为0.979(380/388);诊断正确率为0.974(411/422).脊索瘤CT扫描分为四型:鞍区型、斜坡型、中颅窝型和鼻咽型.鞍区型占51.6%,最为多见.肿瘤多为类圆形和不规则分形.瘤体内高、低密度混杂,均有钙化.造成误诊和漏诊最常见的相关疾病为颅底脑膜瘤和神经源性肿瘤.结论:①以"视力下降、头痛、吞咽困难等"为临床表现的诊断不明行CT检查者,有近10%脊索瘤的患者,应引起重视.②CT对颅底脊索瘤诊断的特异度高,灵敏度中等;而造成灵敏度降低的主要原因是颅底脊索瘤发病率低,阅片时未考虑到.③CT对颅底脊索瘤诊断造成误诊和漏诊最常见的相关疾病为颅底脑膜瘤和神经源性肿瘤.

  9. Resources Required for Semi-Automatic Volumetric Measurements in Metastatic Chordoma: Is Potentially Improved Tumor Burden Assessment Worth the Time Burden?

    Science.gov (United States)

    Fenerty, Kathleen E; Patronas, Nicholas J; Heery, Christopher R; Gulley, James L; Folio, Les R

    2016-06-01

    The Response Evaluation Criteria in Solid Tumors (RECIST) is the current standard for assessing therapy response in patients with malignant solid tumors; however, volumetric assessments are thought to be more representative of actual tumor size and hence superior in predicting patient outcomes. We segmented all primary and metastatic lesions in 21 chordoma patients for comparison to RECIST. Primary tumors were segmented on MR and validated by a neuroradiologist. Metastatic lesions were segmented on CT and validated by a general radiologist. We estimated times for a research assistant to segment all primary and metastatic chordoma lesions using semi-automated volumetric segmentation tools available within our PACS (v12.0, Carestream, Rochester, NY), as well as time required for radiologists to validate the segmentations. We also report success rates of semi-automatic segmentation in metastatic lesions on CT and time required to export data. Furthermore, we discuss the feasibility of volumetric segmentation workflow in research and clinical settings. The research assistant spent approximately 65 h segmenting 435 lesions in 21 patients. This resulted in 1349 total segmentations (average 2.89 min per lesion) and over 13,000 data points. Combined time for the neuroradiologist and general radiologist to validate segmentations was 45.7 min per patient. Exportation time for all patients totaled only 6 h, providing time-saving opportunities for data managers and oncologists. Perhaps cost-neutral resource reallocation can help acquire volumes paralleling our example workflow. Our results will provide researchers with benchmark resources required for volumetric assessments within PACS and help prepare institutions for future volumetric assessment criteria.

  10. 骶骨脊索瘤CD40、PCNA、CD31表达及其与肿瘤复发的相关性研究%Expressions of CD40,PCNA, CD31 in human chordoma and their relationship with recurrence of chordoma

    Institute of Scientific and Technical Information of China (English)

    陆俭; 施琴; 陈康武; 吴贵忠; 杨惠林

    2011-01-01

    [目的]研究骶骨脊索瘤中CD40、PCNA、CD31的表达水平及其与脊索瘤复发的相关性.[方法]采用EnVisionTM免疫组化技术检测28例骶骨脊索瘤组织、9例正常胚胎脊索组织中的CD40和PCNA的表达,并计数CD31标记的肿瘤组织微血管密度(MVD).[结果]①CD40、PCNA在骶骨脊索瘤中的强阳性表达率分别为66.1%、64.3%,明显高于正常胚胎脊索组织.两者之间的差异有统计学意义(P<0.05);骶骨脊索瘤中CD31标记的微血管密度(24.26±18.68)/mm2,正常胚胎脊索组织微血管密度值为(4.63±2.19)/mm2;差异有统计学意义(P<0.05);②28例骶骨脊索瘤,其中复发组14例,非复发组14例,两组间CD40阳性表达率分别为14.29%、85.71%;PCNA阳性表达率分别为92.86%、50.00%;CD31标记的MVD值分别为(30.66±23.75)/mm2、(17.88±8.52)/mm2;均有统计学差异(P<0.05);③CD40、PCNA表达及CD31标记的MVD值与患者的年龄、性别、肿瘤大小无统计学相关性;④PCNA阳性表达的骶骨脊索瘤组织中MVD值为(28.92+20.27)/mm2,明显高于PCNA阴性组(12.64±3.70)/mm2,差异有统计学意义(P<0.05).CD40阴性表达的骶骨脊索瘤组织中MVD值为(31.68±22.81)/mm2,明显高于CD40阳性表达组(16.86±9.28)/mm2,差异有统计学意义(P<0.05).[结论]骶骨脊索瘤中CD40、PCNA、CD31表达水平可作为判断病情进展和肿瘤复发的指标.%[Objective]To study the expressions of CD40,PCNA and CD31 in human chordoma tissues and the relationship with the recurrence of chordoma. [ Methods] CD40 ,PCNA and CD31 were detected by EnVisionTM immunohistochemistry method in 28 human chordoma tissues and 9 normal notochord tissues. The tumor microvascular density ( MVD) marked by CD31 were counted. [ Results ]?The positive expression rates of CD40 and PCNA in chordoma were 66. 1% and 64. 3%. They were significantly higher than those in the normal notochord tissues ( P < 0.05 ). The MVD marked by CD31 was 24

  11. 显微手术切除颅底沟通性脊索瘤%Microsurgical treatment of communicative skull base chordoma

    Institute of Scientific and Technical Information of China (English)

    魏伟; 刘兵; 孙庚林

    2009-01-01

    Objective To investigate the appropriate surgical approaches and microsurgical resection of communicative skull base chordoma. Methods Twenty two cases of communicative skull base chordomas were microsurgically treated from 1995 to 2005 and followed up. The tumors were removed with extended anterior skull base approach in eight cases, with modified Weber-Ferguson (transmaxillary) approach in eight cases, and with transmandibular approach in six cases. Some cases were resected with combined approaches including pterional approach in 3 eases, subtemporal-zygomatie approach in 1 case, and suboccipital-retromastoid approach in 2 cases. Results Total, subtotal and partial removal of the tumors were achieved in 10 cases, 7 and 5 cases respectively. No patients died and had severe nervous system dysfunction after surgery. However, one had CSF rhinorrhea, one with infection, three with cranial never dysfunction. Twenty patients were followed-up with average 3.4 years, 12 returned to normal or partial works, 5 took care by oneself, 1 needed help, 2 died from tumor recurrence. Conclusion The treatment of communicative skull base chordoma is a challenge to neurosurgeon. The key point is the total removal of the tumor with the microsurgical technique and the appropriate approach depend on the location of the tumor. Moreover, skull base reconstruction is also important to avoid the cerebrospinal fluid leak and infection.%目的 探讨不同颅面入路治疗颅底沟通性脊索瘤的入路选择和显微手术方法.方法 手术治疗并经病理证实的颅底沟通性脊索瘤22例.采用颅面入路或联合入路显微手术切除,包括扩大的前颅底入路8例,改良Weber-Ferguson入路8例,下颌入路6例.其中部分向颅内生长明显的沟通性肿瘤,联合经颅手术,包括翼点入路3例,颞颧入路1例,枕下乙状窦后入路2例.对患者预后进行长期随访.结果 肿瘤全切除10例,次全切除7例,大部切除4例,部分切除1例.肿

  12. 骶骨脊索瘤术后复发的相关因素分析%Analysis of recurrent factors of sacral chordoma

    Institute of Scientific and Technical Information of China (English)

    陈康武; 杨惠林; 王根林; 陆俭; 季一鸣; 朱立帆; 莫建强

    2009-01-01

    [目的]探讨影响骶骨脊索瘤患者术后复发的相关因素.[方法]对1993~2008年间完成初次手术的28例骶骨脊索瘤病例进行随访,将患者术后CDFS期作为应变量,选取患者发病年龄、性别、肿瘤大小、部位、初次手术方式、有无局部浸润以及术后是否放疗等因素作为自变量,采用Kaplan-Meier法,Log-Rank法进行分析.[结果]28例患者随访时间平均为(76.2±47.2)个月,最后随访时共复发13例(46.4%),首次复发时间平均为(35.9±27.2)个月.Kaplan-Meier法单因素变量分析显示肿瘤部位、手术方式、有无局部浸润是影响骶骨脊索瘤患者术后复发的独立预测因素(P0.05).[结论]肿瘤部位高、手术切除不彻底和术前发生局部浸润患者复发率较高,术后CDFS期较短.扩大切除术有利于降低术后的复发率,延长术后CDFS期,术前仔细评估MRI有助于选择合理的手术方案.%[Objective] To retrospectively investigate recurrent factors of sacral chordoma after surgical treatment [Methods] This retrospective study included 28 patients with sacral chordoma who underwent primary operation from 1993 to 2008. The data on gender, age, tumor size, tumor location, invasion of the surrounding muscle, surgical type, and radiation therapy were reviewed. Continuous disease -free survival time (CDFS) was calculated according to the Kaplan - Meier method and statistical analysis was performed by using Log - Rank test [Results] The mean follow - up time was 76. 2 months. At the final follow - up, 13 of 28 patients (46.4%) developed local recurrences, and the average recurrence time was 35.9 months. Kaplan - Meier single factor analysis displayed that tumor location, invasion of the surrounding muscle, and surrgical type were the risk of the recurrence of sacral chordoma after surgical treatment ( P 0.05) . [ Conclusion] The higher level of tumor involvement, incomplete excision, and invasion into the surrounding muscles will probably

  13. 建立人颅底脊索瘤细胞系HBC2%Establishment of Human chordoma cell line HBC2

    Institute of Scientific and Technical Information of China (English)

    王亮; 冯洁; 历俊华; 王科; 田凯兵; 陈学涛; 吴震; 万虹; 张俊廷

    2014-01-01

    Objective To establish human chordoma cell lines.Methods The tumor tissue samples were obtained from surgery.The tumor cells were mechanically dissociated and purified based on the different rates of attachment among various cell types.The cells were cultured in DMEM/F-12 medium and passaged in vitro.The morphology and ultrastructure of tumor cells (passage 15) were observed by microscope and electron microscope.The proteins S100,Galectin-3,Fascin-1and Brachyury were measured by histochemical staining.Four phases of the cell cycle was analyzed by the flow cytometer.The karyotype analysis of tumor cells was performed.The tumor cells were subcutaneously injected into the nude mice.Results The HBC2 cell line was characterized by prominent vacuoles of mucus pushing the nuclei to the side.The proteins S100,Galectin-3,Fascin-1 and Brachyury were positively stained in the cell line.The flow cytometer showed 51.3% of the cells were at G1 phase,23.6% at S,and 25.0% at G2-M.The value of G2/G1 was almost 2.The heteroploid karyotype of the cells was indicated.The tumor formation in nude mice was found by HBC2 cells thansplantation.Conclusions Human chordoma cell line HBC2 was successfully established and cultured in vitro on the basis of maintaining its characteristics of chordoma in the process of passage.%目的 建立人脊索瘤细胞系,为今后深入开展脊索瘤的研究奠定基础.方法 手术获得新鲜人脊索瘤组织标本,经机械法和反复贴壁法进行原代培养和细胞纯化,接种到DMEM/F-12的培养基中进行传代培养,稳定传代15代.相差显微镜观察细胞的形态变化,透射电镜观察细胞器特点,免疫组化S100、Galectin-3、Fascin-1、Brachyury染色检测脊索瘤特异性标准蛋白的表达,流式细胞术检测细胞周期、吉姆萨染色体核型分析,裸鼠皮下注射验证细胞的成瘤性.结果 相差显微镜下细胞内可见大量空泡状结构,电镜下胞质内为大量低电子密度的黏

  14. Sacrococcygeal chordoma in a 9-year-old boy Cordoma sacrococígeo em um menino de 9 anos de idade

    Directory of Open Access Journals (Sweden)

    Lúcia de Noronha

    1995-09-01

    Full Text Available A case of sacrococcygeal chordoma in a 9-year-old boy is presented. The symptoms at presentation were pain in both legs and sacrococcygeal region for the last two years that increased in the last four weeks irradiating mainly to the left leg. X-ray and CT scan examinations of the lumbar region revealed an expansive process in the coccygeal region with multiple calcifications and a partially eroded coccyx. There was no invasion of the retroperitoneum and regional lymph nodes. A biopsy was performed and showed cords and nests of cells with large cytoplasm, sometimes vacuolated, nuclei with moderate pleomorphism and clumped chromatin. Immunohistochemistry with avidin-biotin peroxidase technique showed positivity for CK, S-100 protein, CEA, vimentin and to EMA. Chordomas are a distinctly uncommon neoplasm in the first two decades of life, specially in the sacrococcygeal region. They have an aggressive behavior. Treatment of choice is complete resection.Os autores apresentam um caso de cordoma sacroccígeo em um menino de 9 anos de idade. O paciente foi admitido no hospital com história de dor na região sacral e nos membros inferiores com dois anos de evolução, piorando nas últimas quatro semanas. O exame físico revelou atrofia muscular moderada em ambos os membros inferiores, diminuição do reflexo patelar e presença do sinal de Lasègue à esquerda. Os exames de imagem da região lombar mostraram um processo expansivo na região sacrococcígea com erosão parcial do coccix e focos de calcificação, sem evidência de metástases para linfonodos regionais. Foi realizada biópsia diagnóstica que mostrou neoplasia formada por cordões e ninhos de células de citoplasma amplo, por vezes vacuolado, com núcleos moderadamente pleomórficos com cromatina grumosa. O estudo imuno-histoquímico revelou positividade para CK, proteína S-100, CEA, vimentina e EMA. Cordomas são tumores raros que representam em torno de 2% de todas as neoplasias

  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. 臀大肌肌瓣在骶尾部脊索瘤手术治疗中的应用%The glutaeus maximus musculo flap in the treatment of sacrococcygeal chordoma

    Institute of Scientific and Technical Information of China (English)

    钟雷; 冯海龙

    2012-01-01

    探讨臀大肌肌瓣在骶尾部脊索瘤手术治疗中的应用.方法 1996年12月至2011年7月间,共有20例骶尾骨脊索瘤的患者在我院接受手术治疗.均采用后方入路广泛切除肿瘤,早期12例病例未采用臀大肌肌瓣,后期8例采用臀大肌肌瓣填塞缺损.结果 两组均有数例出现膀胱控尿功能及大便控制能力受损,但后期病例复发率和切口并发症发生率明显降低.结论 骶尾部脊索瘤的最佳治疗为广泛的手术切除,但术后容易出现切口并发症及外形不佳,采用臀大肌肌瓣填塞,可大大减少切口并发症的发生,效果满意.%Objective To investigate the glutaeus maximus musculo flap in the treatment of sacroco'ccygeal chordo-ma. Methods 20 cases of sacrococcygeal chordoma patients were accepted with operation treatment. All patients Using posterior approach with wide resection of the tumor, early 12 cases did not adopt the gluteus maximus musculo flap, later in 8 patients using the glutaeus maximus musculo flap packing defect. Results The two groups are few cases of bladder urination and bowel control ability is impaired, but late recurrence rate and incision complication rate was reduced significantly. Conclusion A wide range of operation excision is the best therapy for sacrococcygeal chordoma, but postoperative prone to wound complications and poor appearance, using the glutaeus maximus musculo flap packing, the incision complications can reduce greatly, The effectis satisfactory.

  17. 颅内硬膜下脊索瘤的临床病理特点%Clinical and pathological features of intradural retroclival chordoma

    Institute of Scientific and Technical Information of China (English)

    王亮; 吴震; 田凯兵; 王科; 李桂林; 万虹; 贾桂军; 张力伟; 张俊廷

    2013-01-01

    Objective To investigate the clinical and characteristics of intracranial intradural chordorna.Method Six cases of intradural retroclival chordomas were operated in our hospital between Jan.2006 and Jun.2011.The clinical characteristics and outcome of them were observed retrospectively.There were 5 male and 1 female patients.The mean age of presentation was 40.5 years.Headache and diplopia were the first clinical symptoms.The preoperative imagings varied according to each patient.Immunohistochemical staining for Brachyury,Galectin-3 and Ki-67 was performed in all of them Results All the six lesions were resected by subtemporal,far-lateral or retrosignoid approach,and 3 lesions were totally removed with the rest being subtotally resected.Immunohistochemical patterns showed that all cases were strong positive for Brachyury and Galectin-3.Ki-67 labeling index was between 2.5% and 8.2%.Mean following up period was 58.7 months.3 cases presented no signs of recurrence or regrowth,while the other 2 cases died of this disease.One patient received two more operations after discharged because of tumor regrowth.Conclusions Intradural retroclival chordomas are rare lesions occuring in middle-aged adults.Headache and paralysis of cranial nerves are the mainly clinical presentation.Totally resection by craniotomy is recommended,which achieved 50% in this series.Posivite stain for Brachyury,Galectin-3 and Ki-67 is helpful for differential diagnosis with Ecchordosis physaliphora and chordoid meningioma.Its prognosis demonstrated significant differences.Tumors with rich blood supply,creeping cellular arrangement and Ki-67 labeling index above 5%,are prone to short-term recurrence and poorer prognosis.%目的 探讨原发颅内硬膜下脊索瘤的临床特征及病理特点,以提高对该病的认识.方法 对2006年1月至2011年6月经手术、病理及影像证实的6例颅内硬膜下脊索瘤患者进行回顾性研究.其中男5例,女1例,中位发病年龄为40.5

  18. A diagnostic trap potentially lethal: chordomas uncovered by C3 large osseous destruction inconspicuous on bis-phosphonates-({sup 99}Tc) bone scintigraphy; Un piege diagnostique potentiellement letal: chordome revele par une vaste lesion lytique de C3 normofixante a la scintigraphie osseuse aux bisphosphonates-({sup 99}Tc)

    Energy Technology Data Exchange (ETDEWEB)

    Paycha, F.; Ramadan, A.; Akrout, L. [Hopital Louis Mourier, Unite de Medecine Nucleaire, 92 - Colombes (France); Dion, E. [Hopital Louis Mourier, Service de Radiologie, 92 - Colombes (France); Grossin, M. [Hopital Louis Mourier, Service d' Anatomie Pathologique, 92 - Colombes (France); Gazals-Hatem, D. [Hopital Beaujon, Service d' Anatomie Pathologique, 92 - Clichy (France)

    2006-07-15

    The authors describe a case report of a brachialgy uncovering a solitary bone destruction of C3 associated with epidural involvement. CT and MRI properly depicted the lesion and the loco-regional extension but failed to entertain the diagnosis of the causative tumor, a chordoma. Bis-phosphonates-({sup 99m}Tc) bone scintigraphy proved unconspicuous. Diagnosis was eventually established from the resection specimen, after decision of C3 spondyl-ectomy, C4 hemi-superior spondyl-ectomy and epidural extension excision. Immunohistochemistry study of the material was decisive in identifying the tumor. Cervical chordoma is a tricky diagnosis. Discussion underscores that the crux of diagnostic process is the double discrepancy firstly between extra-osseous and intra-osseous tumoral expansion, secondly between large tumoral mass obvious on multi-slice morphological imaging and negativity of bone scintigraphy, SPECT study included. Among the armamentarium of conventional scintigraphies and PET studies, methionine-({sup 11}C) PET emerges as the most promising anatomo-metabolic procedure to assess loco-regional tumoral expansion, tumoral viability, therapy efficiency, and, potentially, to search for metastases in chordoma. (author)

  19. CT and MRI findings of the chordoma in the mobile spine%脊柱活动节段脊索瘤的 CT、MRI 征象分析

    Institute of Scientific and Technical Information of China (English)

    庞超楠; 刘晓光; 袁慧书

    2015-01-01

    Objective To study the CT and MRI features of the chordoma in the mobile spine.Methods The CT and MRI fea-tures were retrospectively analyzed in twenty-four cases of chordoma in the mobile spine.Results In all the twenty-four cases,bone lesions were solitary in eight cases and multiple in sixteen cases,mostly occurred in cervical,occasionally occurred in thoracic or lum-bar vertebra.Both vertebral body and part appendix were involved in all cases.CT imaging showed that all the lesions mainly mani-fested as osteolytic bone destruction,peripheral osteosclerosis and the cortical bone were incomplete.Soft-tissue mass involved para-vertebral and intraspinal.Five lesions appeared vertebrae compression.Eight lesions appeared intervertebral foramen expansion.Le-sions appeared honeycomb or annular enhancement after contrast media injection.Lesions were presented as heterogeneous slightly hypo-isointensity on T1 WI,hyperintensity on T2 WI.Low signal fibrous septa within the tumors were seen.Lesions appeared hon-eycomb enhancement after Gd-DTPA injection.Conclusion CT and MRI findings of chordoma in the mobile spine can be regarded as characteristic,which are helpful for clinical diagnosis.%目的:探讨脊柱活动节段脊索瘤的 CT 和 MRI 表现。方法回顾分析24例经病理证实的脊柱活动节段脊索瘤的 CT、MRI 表现。结果24例病例中单发病变8例,多发病变16例,病变多发生在颈椎,也可发生在胸椎或腰椎;全部病例均同时累及椎体及部分附件结构。CT 显示全部病例均表现为不同程度的溶骨性骨质破坏,周围可见骨质硬化,骨皮质不完整,可见软组织肿块侵犯椎管内外;5例可见病变椎体压缩,8例可见椎间孔扩大;增强扫描病变呈蜂窝状或环形强化。MR 显示病变表现为不均匀等或稍长 T1长 T2信号,病灶内可见低信号间隔;增强扫描病变呈蜂窝状强化。结论脊柱活动节段脊索瘤的 CT、MRI 表现具有一定特征性,是临床诊断的重要依据。

  20. Adaptation of proton total dose with respect to dosimetric parameters within the frame of treatment of skull base or upper cervical spine chordomas; Adaptation de la dose totale de protons en fonction des parametres dosimetriques dans le cadre du traitement des chordomes de la base du crane et du rachis cervical haut

    Energy Technology Data Exchange (ETDEWEB)

    Hemery, C.G.; Mazeron, J.J.; Feuvret, L. [Groupe hospitalier Pitie-Salpetriere (AP-HP), 75 - Paris (France); Calugaru, V.; Bolle, S.; Habrand, J.L.; Datcharty, J.; Alapetite, C.; Dendale, R.; Feuvret, L. [Institut Curie-Centre de protontherapie d' Orsay, 91 (France); Habrand, J.L.; Datcharty, J. [Institut Gustave-Roussy, 94 - Villejuif (France); Noel, G. [Centre Paul-Strauss, 67 - Strasbourg (France)

    2010-10-15

    The authors report the study of the feasibility of a photon-proton irradiation protocol with a dose adaptation with respect to dosimetric factors for patients suffering form a skull base and upper cervical spine chordoma. Sixty patients have been treated between May 2006 and June 2008 with a combination of high energy photons and protons. As five tumours have locally relapsed and one at distance, the authors comment the local control rates, the number of attained cranial nerves, the value of the macroscopic tumour volume, the survival rate without relapse in terms of multifactorial of uni-factorial analysis. Short communication

  1. Carbon Ion Radiotherapy for Skull Base Chordoma

    OpenAIRE

    Mizoe, Jun–etsu; Hasegawa, Azusa; Takagi, Ryo; Bessho, Hiroki; Onda, Takeshi; Tsujii, Hirohiko

    2009-01-01

    Objective: To present the results of the clinical study of carbon ion radiotherapy (CIRT) for skull base and paracervical spine tumors at the National Institute of Radiological Sciences in Chiba, Japan. Methods: The study is comprised of three protocols: a pilot study, a phase I/II dose escalation study, and a phase II study. All the patients were treated by 16 fractions for 4 weeks with total doses of 48.0, 52.8, 57.6, and 60.8 Gy equivalents (GyE). Results: As a result of the dose escalatio...

  2. 脊索瘤MRI诊断%MRI Diagnosis of Chordomas

    Institute of Scientific and Technical Information of China (English)

    韩月东; 杨春敏; 赵海涛; 刘满生; 张学昕; 刘凯; 葛雅丽; 徐长杰

    2002-01-01

    目的 了解脊索瘤的MRI表现特征.方法 分析39例脊索瘤的MRI表现.结果 颅底部脊索瘤16例,15例破坏颅底并有硬膜外软组织肿块,1例位于颅内右侧桥前池区,骨质无破坏.骶尾部脊索瘤23例,椎体均有破坏,22例伴骶前较大软组织肿块.肿瘤边界清楚,T1WI呈低~等信号,T2WI呈较高~高信号;36例信号不均匀,14例有条状低信号纤维间隔和高信号粘液基质.结论 MRI可清楚地显示肿瘤的部位和范围,根据肿瘤侵犯特点和信号特征,多数肿瘤可以定性诊断.

  3. Distinguishing benign notochordal cell tumors from vertebral chordoma

    Energy Technology Data Exchange (ETDEWEB)

    Yamaguchi, Takehiko [Sapporo Medical University School of Medicine, Department of Surgical Pathology, Sapporo, Hokkaido (Japan); Iwata, Jun [Kochi Health Science Center, Department of Laboratory Medicine, Kochi, Kochi (Japan); Sugihara, Shinsuke [Kochi Health Science Center, Department of Orthopaedic Surgery, Kochi, Kochi (Japan); McCarthy, Edward F. [The Johns Hopkins Hospital, Department of Pathology, Baltimore, MD (United States); Karita, Michiaki; Murakami, Hideki; Kawahara, Norio; Tsuchiya, Hiroyuki; Tomita, Katsuro [Kanazawa University, Department of Orthopaedic Surgery, Kanazawa, Ishikawa (Japan)

    2008-04-15

    The objective was to characterize imaging findings of benign notochordal cell tumors (BNCTs). Clinical and imaging data for 9 benign notochordal cell tumors in 7 patients were reviewed retrospectively. Conventional radiographs (n = 9), bone scintigrams (n = 2), computed tomographic images (n = 7), and magnetic resonance images (n = 8) were reviewed. Eight of the 9 lesions were stained with hematoxylin-eosin and microscopically examined. There were 3 male and 4 female patients with an age range of 22 to 55 years (average age, 44 years). Two patients had two lesions at different sites. The lesions involved the cervical spine in 4 patients, the lumbar spine in 2, the sacrum in 2, and the coccyx in 1. The most common symptom was mild pain. The lesions of 2 patients were found incidentally during imaging studies for unrelated conditions. Five patients underwent surgical procedures. One patient died of surgical complications. All other patients have been well without recurrent or progressive disease for 13 to 84 months. Radiographs usually did not reveal significant abnormality. Five lesions exhibited subtle sclerosis and 1 showed intense sclerosis. Technetium bone scan did not reveal any abnormal uptake. Computed tomography images had increased density within the vertebral bodies. The lesions had a homogeneous low signal intensity on T1-weighted magnetic resonance images and a high intensity on T2-weighted images without soft-tissue mass. Microscopically, lesions contained sheets of adipocyte-like vacuolated chordoid cells without a myxoid matrix. Benign notochordal cell tumors may be found during routine clinical examinations and do not require surgical management unless they show extraosseous disease. These tumors should be recognized by radiologists, pathologists, and orthopedic surgeons to prevent operations, which usually are extensive. (orig.)

  4. 软骨样脊索瘤的超微病理对比研究%Electron microscopic studies on chordomas:difference between typical chordomas and chondroid chordomas

    Institute of Scientific and Technical Information of China (English)

    郑晓刚; 姜少军; 周晓军; 陆珍凤

    2004-01-01

    目的:探讨软骨样脊索瘤的超微病理特点,为阐明此肿瘤的来源提供线索.方法:结合光镜、免疫组化及临床资料,对3例软骨样脊索瘤和4例普通的脊索瘤进行了超微结构对比观察.结果:1.在软骨样脊索瘤的软骨样区域,胞膜游离,形成棘突和微绒毛样突起;在非软骨样区域,瘤细胞排列紧密,粗面内质网发达.2.普通的脊索瘤,一些瘤细胞粗面内质网扩张、融合,挤压瘤细胞核至一侧.结论:1.软骨样脊索瘤出现了两个不同分化的层面,既有分化原始的类似胚胎脊索的瘤组织,又有分化高的软骨样区域,而普通的脊索瘤仅有单一的分化层面.2.软骨样脊索瘤的瘤细胞属同一起源,即胎儿的脊索残余.3.瘤组织的黏液性间质成分是由瘤细胞的粗面内质网合成的.液滴状细胞是部分瘤细胞由于粗面内质网高度扩张而成.

  5. CT diagnosis of skull-base chordomas%颅底脊索瘤的CT诊断

    Institute of Scientific and Technical Information of China (English)

    杨超人

    2006-01-01

    目的探讨颅底脊索瘤的CT诊断及临床应用价值.方法回顾性分析15例经手术、病理证实确诊为颅底脊索瘤的CT表现.结果 15例颅底脊索瘤中经手术和CT扫描证实,发生于斜坡5例,发生于鞍区及鞍旁7例,发生于鼻咽部1例,发生于蝶窦2例.病灶有钙化11例,颅底骨质破坏12例.结论 CT扫描能够显示颅底脊索瘤部位、大小及侵犯范围,有助于确定手术方案及对术后进行评估.

  6. MRI features and differentiation of chordomas%脊索瘤MRI表现与鉴别

    Institute of Scientific and Technical Information of China (English)

    张小玲; 刘起旺; 梁力; 张锁旺

    2004-01-01

    目的:分析脊索瘤的MRI表现及与其他疾病的鉴别,探讨MRI的诊断价值.材料和方法:搜集16例经手术病理证实的脊索瘤,分析其MRI表现及有关鉴别诊断.其中13例行头颅MR平扫和增强扫描,3例分别行颈椎、骶尾部平扫.结果:13例颅底脊索瘤,局限于斜坡者4例,累及鞍区、中颅凹、蝶窦,呈广泛浸润生长者8例,同时累及后颅凹者1例;T1WI呈不均匀等低信号块影,T2WI呈不均匀高信号,10例增强扫描病灶呈不均匀强化;骶尾部脊索瘤2例,颈椎脊索瘤1例,在T1WI和T2WI上均呈混杂信号块影,可见骨质破坏,上段颈髓受累.16例均行手术治疗,有12例术后定期复查均有病变复发征象.结论:MRI可明确显示肿瘤的生长方式、浸润范围及与邻近结构的解剖关系,对诊断和手术治疗的评估有重要参考价值.

  7. MR Imaging Manifestations of Vertebral Chordomas%脊椎脊索瘤的MRI表现

    Institute of Scientific and Technical Information of China (English)

    张国伟; 初英萍; 刘静; 张光辉; 唐小峰; 周承涛; 李滢

    2005-01-01

    目的了解脊椎脊索瘤的MRI表现特征. 资料与方法分析33例脊椎脊索瘤的MRI表现. 结果骶尾部24例,颈椎6例,胸椎2例,腰椎1例.脊椎脊索瘤表现为椎体骨质破坏,并轻度膨胀性改变,常伴椎体前方巨大软组织肿块.在骶骨正中矢状面上,其前方巨大球形肿块与骶骨常呈锐角相连.33例脊索瘤均呈长T1长T2信号,其内信号不均匀.增强扫描12例,11例呈不均匀强化,1例呈明显均匀强化. 结论 MRI可准确清楚地显示脊索瘤的部位、范围、形态、信号特点和增强表现,大多数脊索瘤可在术前做出正确的诊断.

  8. Diagnosis of Basicranial Chordoma with CT%颅底脊索瘤的CT诊断

    Institute of Scientific and Technical Information of China (English)

    陈科; 陈友良

    2002-01-01

    @@ 颅底脊索瘤是一种少见的低度恶性破坏性肿瘤.临床表现以头痛最为常见.CT表现具有一定特异性,易与颅底其他肿瘤相混淆.本组收集1997~2001年共16例经手术病理证实.

  9. A case of bosom chordoma%胸壁脊索瘤1例报告

    Institute of Scientific and Technical Information of China (English)

    王索宇; 徐仁根

    2005-01-01

    病例:男,68岁,20年前偶然发现左前胸壁有一蚕豆大小包块.无触压痛.未经任何诊治.也无明显增大、近2年包块逐渐增大,入院前左前胸壁包块大小约130cm×14.0cm.突出于左前胸壁.表断皮色、皮温正常.无发热、咳嗽、胸痛胸闷、气促等症状入院拟诊却左前胸壁纤维瘤和骨纤维异样增殖症.

  10. Sellar chondroid chordoma%鞍区软骨样脊索瘤

    Institute of Scientific and Technical Information of China (English)

    李侠; 李青; 王映梅; 张丽英; 谷雨

    2014-01-01

    目的 探讨鞍区软骨样脊索瘤的临床病理学特征,复习相关文献.方法与结果 男性患者,66岁.临床表现为反复性低钠血症,MRI显示鞍区类圆形、边界清楚占位性病变.经右侧鼻腔入路行内镜下鞍区肿瘤切除术,术中可见肿瘤呈实性,质地坚韧,边界清楚;组织形态学表现为典型的脊索瘤组成,肿瘤组织内呈灶性软骨样分化;肿瘤细胞表达细胞角蛋白、上皮膜抗原和S-100蛋白,Ki-67抗原标记指数约为1%,病理诊断为鞍区软骨样脊索瘤.术后随访11个月,一般状况良好,肿瘤无复发.结论 软骨样脊索瘤多生长于中轴骨骼,具有特殊的组织学构象和免疫表型,患者预后较典型脊索瘤好,诊断时应注意与典型脊索瘤和软骨肉瘤相鉴别.

  11. Clinical Treatment Progress of Chordoma%脊索瘤的临床治疗进展

    Institute of Scientific and Technical Information of China (English)

    陈卫东; 刘潭; 王玉名; 孙平; 商冠宁

    2014-01-01

    脊索瘤是一种罕见的低度恶性肿瘤,临床治愈率低,复发率较高.手术切除是治疗脊索瘤的首选方法,目前公认的脊索瘤治疗方法是完整(或大块)切除.术后辅助放射治疗越来越多的应用于临床,而新药物的研发、术前动脉栓塞的应用,以及射频消融等都被用于脊索瘤的临床治疗.本文综述了目前临床上应用的诊疗进展,包括手术治疗、放射治疗、药物治疗及射频消融治疗等方法.

  12. MR imaging manifestations of chordoma%脊索瘤的MRI表现

    Institute of Scientific and Technical Information of China (English)

    李宏; 史佩芝; 陈文辉

    2004-01-01

    目的:探讨脊索瘤的MRI表现及其诊断价值.方法:回顾性分析17例经临床病理证实的脊索瘤的MRI表现.结果:颅底脊索瘤8例,骶尾部脊索瘤9例,邻近颅底及椎体骨质均有溶骨性破坏,伴硬膜外及骶前较大软组织肿块.17例中16例信号不均匀,T1WI呈低、等信号,T2WI呈明显高信号,MRI增强扫描后,肿瘤均有轻度或中度不均匀性强化.结论:T2WI显著高信号是脊索瘤的特征性MRI表现,有重要鉴别诊断意义,MRI反映肿瘤的部位、侵犯范围以及显示骨质破坏作用均优于CT.

  13. 胸椎脊索瘤1例%Chordoma of the thoracic spine: case report

    Institute of Scientific and Technical Information of China (English)

    邢新博; 孙金烈; 张毅军

    2009-01-01

    患者男,24岁。以“胸背部疼痛、双下肢无力”为主诉入院。查体:左侧胸背部见8cm×7cm包块,质韧,活动度差,表皮无红肿及静脉怒张。T10以下皮肤感觉减退,左下腹壁反射消失。双侧膝腱及跟腱反射亢进,双侧踝震挛阳性,双侧巴氏征阳性,提睾反射消失,肛周皮肤感觉减退,肛门括约肌自主收缩功能减弱。

  14. One case report of rhinal chordoma%鼻腔脊索瘤一例

    Institute of Scientific and Technical Information of China (English)

    李树华; 李春秋; 宋福林; 董卫东

    2007-01-01

    患者女,33岁,2001年4月开始无明显诱因地出现右侧鼻塞,伴流清水样涕,气候变化时加重,偶有轻度头晕,头闷胀感和轻度耳鸣,自服鼻炎康,阿莫西林等药物治疗后症状无明显缓解,且鼻塞症状进行性加重。当地医院就诊,发现右侧鼻腔肿物,于2005年5月25日入住本院。

  15. 颅底脊索瘤的MRI诊断%MRI Diagnosis of Basicranial Chordoma

    Institute of Scientific and Technical Information of China (English)

    徐锐; 贺能树; 徐志宣; 郝大鹏; 徐爱德

    2003-01-01

    目的研究颅底脊索瘤的MRI表现,探讨其MRI诊断价值.资料与方法回顾性分析经手术病理证实的17例颅底脊索瘤的MRI表现,13例行CT检查.结果颅底脊索瘤发生在斜坡11例(64.7%),鞍区6例(35.3%);肿瘤形态以类圆形分叶状为主(88%);病变呈不均匀长T1、长T2信号,病灶内可见囊变、出血、钙化灶;病变侵犯邻近结构器官如:斜坡、垂体、海绵窦等;MRI增强扫描示病变呈持续性"蜂房样"不均匀强化10例,均匀强化2例.结论 MRI可清晰显示颅底部脊索瘤的部位、大小、范围、内部结构及与周围神经、血管的关系,具有较高的敏感性和一定特征性,有助于制定合适的手术方案和预后的估计.

  16. Diagnosis of clivus chordomas using MRI%斜坡脊索瘤的MRI诊断

    Institute of Scientific and Technical Information of China (English)

    何光武; 项华; 何江波; 成中意; 徐建荪; 汪守中

    2006-01-01

    目的探讨MRI在斜坡脊索瘤诊断中的价值.方法分析经手术病理证实的16例脊索瘤形态、信号特点.结果 16例中,男11例,女5例,最大年龄57岁,最小年龄13岁,平均年龄30岁.肿瘤全部位于斜坡及其周围区域,肿瘤呈不规则形、圆形或椭圆形,向周围侵润生长.12例均呈混杂不均匀信号,T WI呈等、低、高信号,T2WI呈低、高信号,以高信号明显.4例信号稍不均匀,T WI呈等、稍低信号,T2呈高信号.增强后16例均表现为不均匀轻到中度强化.结论 MRI可以准确显示斜坡脊索瘤的大小、形态、边界和信号特点,有利于该病的诊断.

  17. 颅外脊索瘤治疗研究新进展%Novel progress in treatment of extracranial chordoma

    Institute of Scientific and Technical Information of China (English)

    周子斐; 华莹奇; 左冬青; 陈泉池; 蔡郑东

    2014-01-01

    脊索瘤是一种罕见的低中度恶性骨肿瘤,具有临床症状不典型、早期诊断困难、手术彻底切除困难、术后复发率高以及传统放化疗不敏感等特点,因此临床治疗十分困难.本文从外科手术、放疗和化疗等方面介绍了颅外脊索瘤的治疗进展,包括分化疗法和刺激响应性水凝胶等新型治疗方式,以及根据肿瘤药敏检测指导临床用药.最后,本文介绍了脊索瘤新靶点治疗的最新研究进展、目前存在的问题以及未来的展望.

  18. 颈部椎管内脊索瘤1例%One case:intraspinal chordoma of the Neck

    Institute of Scientific and Technical Information of China (English)

    李莹; 任翠萍; 李贝贝; 任仙

    2012-01-01

    @@ 患者女,23岁.1个月前无明诱因出现双侧下肢无力,能坚持行走,无疼痛.大小便障碍,未行治疗.近半个月来双侧下肢无力情况呈进行性加重,已不能行走,并出现头晕、颈部酸痛感,左侧上肢无力,头晕呈眩晕状,时轻时重.颈椎MRI示:C1~C3椎体水平髓外硬膜外可见不规则形长T1长T2信号(图1),压脂呈高信号,弥散加权成像示弥散明显受限,同平面脊髓明显受压后移.病变边界清晰,累及同水平左右椎间孔(图2).

  19. 脊索瘤的影像诊断及分期%Imaging diagnosis and staging of chordoma

    Institute of Scientific and Technical Information of China (English)

    王冬梅; 孙琦; 杨献峰; 牡丹; 周正杨; 朱斌

    2010-01-01

    目的:分析脊索瘤的影像学特征及分期,探讨影像学方法在脊索瘤诊断及分期中的价值.方法:回顾性分析20例首发脊索瘤患者的21个肿瘤病灶的CT和MRI表现,术前进行影像学分期,并随访术后的复发情况.结果:发生在典型部位(颅底和脊柱)的脊索瘤17个(17/21,81.0%),少见部位的脊索瘤为4个(4/21,19.0%);90.5%(19/21)的病灶CT表现为膨胀性溶骨性骨破坏,71.4%(15/21)的病灶表现为膨胀性溶骨性骨破坏伴周围软组织肿块,具有MRI T2WI"蜂房征"的病例为90.5%;随着肿瘤分期级别的提高,复发率也提高.结论:脊索瘤的MRI表现具有特征性,可以诊断大多数脊索瘤,明确肿瘤范围,为手术切除及相关治疗提供客观依据,并且根据肿瘤的分期对肿瘤的复发及预后具有一定的预见性.

  20. Progression in treatment of sacrococcygeal chordoma%骶骨脊索瘤治疗的研究进展

    Institute of Scientific and Technical Information of China (English)

    陈康武; 杨惠林; 陆俭; 莫建强

    2009-01-01

    @@ 脊索瘤是一种较为少见的中低度恶性的原发性骨肿瘤[1],占原发恶性骨肿瘤的1%~4%[2].脊索瘤起源于胚胎残余脊索组织[3],好发于脊柱中轴的两端,其中骶尾部占40%~50%,颅底部占35%~40%,只有少数发生于其余椎体占15%~20%[4].

  1. 人脊索瘤细胞的体外侵袭性%Invasive capacity of human chordoma cells in vitro

    Institute of Scientific and Technical Information of China (English)

    常恒; 马保安; 张殿忠; 文艳华

    2001-01-01

    目的观察人脊索瘤细胞在体外侵袭性的大小, 探讨与肿瘤侵袭性密切相关的尿激酶型纤溶酶原激活物(urokinase-type plasminogen activator, uPA)在侵袭中的作用. 方法利用体外球体侵袭模型观察人脊索瘤细胞的侵袭特性和侵袭过程;应用免疫组化SABC法检测脊索瘤细胞中uPA的表达情况,并且观察用uPA抗体后肿瘤细胞侵袭性的变化. 结果肿瘤细胞球体在接触预培养的鸡胚心肌块(preculture heart fragment, PHF)后3 d即包绕PHF,并有部分侵袭,接触培养5 d,每个PHF即有一半左右被侵袭,10 d左右,PHF即完全被侵袭. 免疫组化染色,阳性细胞占63%,为uPA高表达. 在培养液中加入uPA抗体后,肿瘤细胞侵袭性减低. 结论人脊索瘤细胞具有一定的侵袭性;该细胞uPA高表达;uPA抗体可使脊索瘤细胞的侵袭性减低.

  2. 骶尾部脊索瘤的手术治疗%Surgical management of sacrococcygeal chordomas

    Institute of Scientific and Technical Information of China (English)

    郭卫; 徐万鹏; 杨荣利

    2003-01-01

    目的:回顾近年来52例脊索瘤的治疗结果及经验.方法:1996年12月至2001年7月间,共有52例骶尾骨脊索瘤的患者在我院接受手术治疗.手术时平均年龄为57岁(18~80岁);男35例,女17例. 其中,初次手术患者33例, 第2次手术者14例, 第3次手术者5例. 采用后方入路43例.前后路联合切除9例. 19例第2~3次手术的患者中,9例术前接受过放疗.结果:6例患者死亡,其中2例死于转移性脊索瘤,3例死于肿瘤复发后衰竭,1例第3次局部复发后自杀.其余46例患者中,35例无瘤生存,未见肿瘤局部复发迹象,这35例患者的平均随访时间为3年半.双侧骶3以上神经根保留的50例患者中,术后膀胱控尿功能及大便控制基本正常,2例仅保全骶1神经根或更高位神经根的患者,均出现膀胱控尿功能及大便控制能力受损,术后长期放置尿管.33例第1次手术的患者中,7例出现局部复发(21%),19例第2~3次手术的患者中,再次复发者5例(26%),均需再次手术治疗.结论:骶尾部脊索瘤的最佳治疗为广泛的手术切除,术后加用辅助放疗可降低复发率,但会影响二次手术的显露及术后切口愈合. 即使是肿瘤较大的患者,切除范围要达到骶1或骶2水平,后方入路仍能完成手术,手术并发症的发生率均可接受.虽然通过恰当的广泛切除和辅助放疗可控制局部肿瘤病灶,但目前我们对如何预防转移或如何治疗已经出现的转移灶还没有好的方法.本组1例肿瘤巨大并发肺转移的病人,通过化疗获得很好疗效.

  3. A case report of craniovertebral giant chordoma%颅底巨大脊索瘤1例报告

    Institute of Scientific and Technical Information of China (English)

    魏富鑫; 刘少喻; 万勇

    2006-01-01

    患者,男,6岁。因反复的颈项部僵硬感、活动受限1年入院。1年前无明显诱因出现颈项僵硬感,伴颈活动受限,无头晕、头痛,无四肢麻木、乏力感。于当地医院诊断为“颈部扭伤”未予特殊处理。近2个月来,患儿夜间睡眠出现打鼾及颈部疼痛感,无呼吸、吞咽困难。再次于当地医院就诊,疑为颈椎结核,胸部X线透视未见异常。颈椎X线检查示,寰椎稍向前移,寰椎前弓、枢椎齿状突及椎体前缘溶解破坏,寰、枢椎椎体前缘有明显软组织肿块隆起,气管明显受压并向前移位(见图1a)。CT检查示,枕骨斜坡后份、寰椎前弓、枢椎齿状突右份可见溶骨性骨破坏,增强扫描肿物强化,密度不均匀,CT值:38~93hu(见图1b)。

  4. Perioperation nursing of chordoma surgery patients%脊索瘤切除术病人的围术期护理

    Institute of Scientific and Technical Information of China (English)

    金晶; 李银珍; 刘秋华

    2010-01-01

    目的:探讨脊索瘤手术的围手术期护理.方法:对6例脊索瘤手术病人,术前做好心理护理,术后密切观察病情变化,加强呼吸道及口腔护理,预防感染.结果:6例病人中无1例出现上颌骨坏死、切口感染等并发症.结论:脊索瘤手术病人围手术期高质量护理是手术成功、避免并发症发生的关键.

  5. 颅内脊索瘤的MRI诊断价值%MRI diagnostic value of intracrania chordoma

    Institute of Scientific and Technical Information of China (English)

    伍建林; 林茂盛; 郎志谨; 沈天真; 陈星荣

    2000-01-01

    目的:探讨磁共振成像(MRI)技术诊断颅内脊索瘤的价值.材料与方法:回顾性分析了18例经手术和病理证实的颅内脊索瘤的MRI表现.结果:颅内脊索瘤大部分(本组16例,占88 9%)发生于颅底中线处的斜坡和/或鞍区,并广泛侵犯颅底的神经和大血管等重要结构;在T1加权像上肿瘤主要表现为低信号,T2加权像上表现为不均匀高信号,Gd-DTPA静脉注射增强后(8例)均出现较明显的不均匀强化.此外,有15例(83 3%)脊索瘤均出现斜坡形态和其内MR信号的异常改变.结论:MRI检查颅内脊索瘤的临床价值在于明确肿瘤的部位、大小与范围,尤其是精确的评价肿瘤与颅底神经和大血管等重要结构的解剖关系,有助于制定合适的手术方案和估计预后.

  6. Microsurgical treatment of skull base chordoma%颅底脊索瘤的显微外科治疗

    Institute of Scientific and Technical Information of China (English)

    张俊廷; 吴震; 贾桂军; 张力伟; 钱珂

    2006-01-01

    目的探讨脊索瘤的诊断及综合治疗.方法对105例颅底脊索瘤作回顾性分析研究.结果手术108例次,有3例病人复发后再次行手术治疗.肿瘤全切除14例次,近全切除65例次,全切和次全切除率为73.2%,大部及部分切除29例次.手术死亡2例.108例次手术中有34例次术后出现并发症.结论采用显微外科治疗方法,选择适当的手术入路,是提高肿瘤切除率,降低术后并发症是治疗的关键所在.

  7. Skull base chordoma%颅底脊索瘤的分型、诊断与手术

    Institute of Scientific and Technical Information of China (English)

    周定标; 余新光; 许百男; 魏少波; 张远征; 程东源

    2005-01-01

    目的探讨颅底脊索瘤的临床分型、诊断与手术.方法1988年9月至2002年12月收治102例颅底脊索瘤病人,临床主要表现为颅神经功能损害、运动障碍、鼻咽部症状等.根据肿瘤起源和发展方向,分为鞍区型、颅中窝型、颅后窝型、鼻(口)咽型和混合型.99例病人106次分别经枕下远外侧、乳突后、前方颅底、鼻蝶、额下、颞下、侧裂、口咽和联合入路手术.肿瘤未能全切除者术后辅以放疗或放射外科治疗.结果99例手术者,肿瘤全切除25例(25.3%),次全或大部切除32例(32.3%),部分切除41例(41.4%),1例仅作活检.死亡1例(1%).2例发生脑脊液漏,6例出现新的颅神经损害,1例偏瘫.86例平均随访5.6年,21例恢复正常工作,28例恢复部分工作,17例生活自理,9例不能自理,11例死于肿瘤复发或其他原因.结论根据临床表现和影像学资料,多数颅底脊索瘤术前可确诊,但有些病例需与侵袭性垂体腺瘤、鼻咽癌、软骨性肿瘤(尤其软骨肉瘤)鉴别.恰当的临床分型有助于手术入路的选择.治疗应首选手术,部分肿瘤可全切除,多数不能全切者术后辅以放疗,可达到缓解症状延长存活期的目的.

  8. Analysis for Imaging Characteristics of Chordoma%脊索瘤的影像学特点分析

    Institute of Scientific and Technical Information of China (English)

    马永强; 杨宁; 林光武

    2007-01-01

    目的 探讨脊索瘤的影像学表现.方法 搜集经手术病理证实的脊索瘤42例,对其影像学表现进行回顾性分析.结果 颅底脊索瘤23例(54.8%),骶尾部脊索瘤18例(42.9%),外周脊索瘤1例(2.3%).平片可见颅底及骶尾部溶骨性骨质破坏;CT扫描脊索瘤特征性地表现为斜坡和骶尾部膨胀性软组织肿块伴相应部位广泛溶骨性骨质破坏;MRI扫描对病变范围的显示优于CT.结论 脊索瘤有较为典型的好发部位,MRI表现较具特征性.

  9. Endoscopic transsphenoidal surgery of chordoma%内镜经鼻蝶手术治疗颅底脊索瘤

    Institute of Scientific and Technical Information of China (English)

    张亚卓; 王忠诚; 赵德安; 周益民; 张世渊; 宗绪毅; 宋明; 裴傲; 赵澎

    2007-01-01

    目的 探讨内镜下经鼻蝶治疗颅底脊索瘤的技术、方法和手术指征.方法 自2000年6月至2006年6月,应用神经内镜经鼻蝶入路手术治疗颅底脊索瘤30例.29例应用30°,70°硬性神经内镜,经鼻中隔和中鼻甲间入路,用高速磨钻磨除相应的骨性结构,显露肿瘤并分块切除.1例在显微外科下开颅切除颅内部分肿瘤,然后在神经内镜引导下,经鼻蝶切除斜坡、鞍区蝶筛窦、上颌窦、颞下窝的肿瘤.18例在术后6-12个月获得随访. 结果 肿瘤近全切除7例,次全切除16例,部分切除7例.24例术后获得一定程度上的临床症状和体征的改善,6例无变化.所有病人在术后7-10d可恢复日常生活.1例在出院后20d因脑脊液漏再次入院做修补手术.随访的18例中,有4例在术后10-18个月复发,这4例均为广泛性生长的病例. 结论 内镜经鼻蝶手术治疗颅底脊索瘤有明显的优势.其操作简便安全;术中视野清楚,有利于显露;术后严重并发症少,病人恢复快,住院时间短.

  10. 颅底脊索瘤的MR表现%MR features of chordoma in the skull base

    Institute of Scientific and Technical Information of China (English)

    张静; 张云亭; 李威; 张敬; 陈静; 何雅娜

    2009-01-01

    目的 分析颅底脊索瘤的MRI特征,评价肿瘤T2WI信号强度与病理分型间的关系.方法 回顾性分析16例经病理证实的颅底脊索瘤的MRI资料,包括部位、生长方式、信号特点、强化方式及与邻近结构的关系;评价肿瘤T2WI信号强度,并将其与病理分型进行对照分析.结果 16例中12例发生于斜坡,4例位于斜坡以外,其中鞍旁、颈静脉孔区各1例,蝶窦2例.病变均边界清楚、形态规则或不规则(分叶状).病变信号不均,均可见蜂窝状表现.T1WI呈明显低或稍低信号,其中8例可见局灶性高信号;T2WI均为高或稍高信号.增强后均显示不均匀强化,其中轻度强化4例,中度强化6例,缓慢渐进性强化6例.多方位成像可以清晰显示病变位置及与邻近重要结构的关系.16例中10例为经典型,6例为软骨瘤样型.两型之间T2WI的信号强度差异无统计学意义.结论 颅底脊索瘤MRI无论在发病部位、信号特点及强化方式上都具有一定的特征性表现;单纯根据病变的信号强度无法确定脊索瘤病理分型.

  11. One case of chordoma in nasal cavity%鼻中隔血管平滑肌瘤一例

    Institute of Scientific and Technical Information of China (English)

    王勤瑛; 柴亮; 汪审清; 周水洪

    2007-01-01

    患者男,73岁,因鼻塞伴流涕2年于2004年9月入院。患者无局部疼痛及鼻出血史,无鼻部外伤史,查体见左中鼻道有一淡红色肿物。CT扫描见左中鼻道有一种物,起源于鼻中隔,充满左侧鼻腔,未延及鼻咽部,窦壁完整,无明显骨质破坏和增生(图1)。

  12. Parachordoma of the sacrococcygeal region - a neuroepithelial tumor

    DEFF Research Database (Denmark)

    Wiebe, B. M.; Jensen, K.; Laursen, Henning

    1995-01-01

    Chordoma, extraskeltal myxoid chondrosarcoma, glial fibrillary acidic protein, immunohistochemical staining, parachordoma, subcutaneous sacrococcygeal myxopapillary ependymoma, neuropathology......Chordoma, extraskeltal myxoid chondrosarcoma, glial fibrillary acidic protein, immunohistochemical staining, parachordoma, subcutaneous sacrococcygeal myxopapillary ependymoma, neuropathology...

  13. The surgical management of sacral chordoma%骶骨脊索瘤的手术治疗结果评价

    Institute of Scientific and Technical Information of China (English)

    郭卫; 燕太强; 汤小东; 杨毅

    2009-01-01

    例出现局部复发(42.5%).28例第2~3次手术的患者中,再次复发者18例(64.3%).51例患者共进行79次手术,25例次(31.6%)术后出现伤口并发症.11例次(13.9%)患者术后出现脑脊液漏.结论 骶骨脊索瘤为低度恶性局部侵袭性强的肿瘤,广泛切除是治疗骶骨脊索瘤的关键.S3以下的肿瘤可行广泛切除,对于累及S3以上的肿瘤为最大限度的保留神经功能,采用切刮术加高压冲洗可以有效地降低局部复发率.%above, wide en-bloc resection plus piece-meal excision in the cephalad is performed.

  14. 脊索瘤信号通路与分子靶向治疗%Chordoma Signaling Pathways and Molecular Targeted Therapy

    Institute of Scientific and Technical Information of China (English)

    蒋依娜

    2015-01-01

    脊索瘤是一种少见的骨组织恶性肿瘤,来源于残留胚胎脊索组织,占骨原发恶性肿瘤的1%~4%.主要发生于颅底、脊柱及骶尾部的中轴骨组织.肿瘤发生部位特殊,故手术完整切除困难,而传统放疗及化疗的效果亦欠佳,因此脊索瘤常常侵犯周围组织,并多次复发.近年来关于脊索瘤的分子生物学方面研究日益深入,受体酪氨酸激酶及其下游信号通路Akt/mTOR、Ras/MEK/ERK,以及其他信号通路,如STAT3、NF-κB等在脊索瘤中相继被发现,相关分子靶向药物的研究也进入Ⅰ期和Ⅱ期临床试验阶段.本文就目前研究较多的几条脊索瘤信号通路及分子靶向治疗进展作一综述.

  15. 骶尾部脊索瘤的诊断与外科治疗%Diagnosis and surgical treatment of sacrococcygeal chordoma

    Institute of Scientific and Technical Information of China (English)

    孙才兴; 谢尚闹; 杨红健; 孟旭莉

    2005-01-01

    目的回顾性分析骶尾部脊索瘤的诊断与外科治疗体会.方法收集本院经病理证实的骶尾部脊索瘤18例,分析其临床特征和诊疗结果.结果18例手术时中位年龄为44岁(24~73岁);男性11例,女性7例,其中第2次手术者3例;肿瘤体积大小为16~126 ml,平均45.6ml;病理分期Ⅰa期1例,Ⅰ b期16例,Ⅲ期1例;肿瘤位于L51例、S13例、S23例、S3以下11例.手术采用后方入路11例,前后路联合切除7例.行瘤内切除11例,边缘性切除5例,根治性切除1例,1例因术中大出血而仅行大部分切除.3例复发者及12例初发者接受60Co 45~60Gy,其中5例加11MeV电子束.并发踝关节无力3例,尿失禁或尿潴留5例.随访14个月~13年,平均3.8年.复发6例,主要在术后3年,1例于术后11年复发死亡,1例发生肺转移,另有1例于二次术后1年出现直肠周围多个类圆形软组织阴影.本组病例3年生存率77.8%(14/18),无复发生存率38.9%(7/18),5年生存率61.1%(11/18).结论骶尾部脊索瘤治疗效果依赖于肿瘤部位、手术切除程度和辅助放疗.

  16. Clinical analysis of 20 cases of chordomas in the skull base%颅底脊索瘤的临床分析

    Institute of Scientific and Technical Information of China (English)

    魏瑞; 姜维喜; 王龙云; 廖遇平

    2005-01-01

    目的探讨颅底脊索瘤的临床特点及治疗.方法本院自1996年1月~2002年9月共收治20例颅底脊索瘤,均有病理学诊断,主要症状为头痛、视力下降、复视.20例均手术治疗,其中手术加放疗或γ-刀治疗6例.结果总的3、5年生存率分别为69%、57%.单纯手术组3、5年生存率分别为:60.5%、41%;手术+放疗或γ-刀组为3、5年生存率83.3%、83.3%.结论颅底脊索瘤易复发,手术加放疗是有效的治疗方法.

  17. 21例骶尾部脊索瘤临床分析%Clinical Analysis of 21 Patients with Sacrococcygeal Chordoma

    Institute of Scientific and Technical Information of China (English)

    梁春梅; 章文成; 刘志艳; 王军; 王平

    2008-01-01

    目的:探讨骶尾部脊索瘤的临床特点及治疗方法.方法:回顾性分析天津医科大学附属肿瘤医院1989年1月至2004年12月收治的21例骶尾部脊索瘤患者的临床资料,11例接受单纯手术治疗,10例接受手术+术后放疗,放射治疗剂量为40~50Gy.结果:9例患者死亡,6例死于局部复发,1例死于双肺转移,2例死于非肿瘤相关疾病.全组1、3、5、10年生存率分别为100%、95%、85%、41%.单纯手术组的5年生存率为81%.手术+术后放疗组的5年生存率为89%,二者无显著性差异(P=0.256>0.05).局部复发15例,单纯手术组9例,局部复发率为81.8%,手术+放疗组6例,局部复发率为60%,二者无显著性差异(X2=1.222,P=0.269>0.05).单纯手术组局部复发时间为术后2个月~38个月,中位复发时间22个月;手术+放疗组发生复发时间为术后23个月~个72月,中位复发时间48个月.结论:脊索瘤极易复发,患者就诊状态及治疗手段可能为影响复发的因素,手术切除为其主要治疗手段,术后辅以放疗未能明显提高生存率及减少局部复发,但推迟了局部复发时间.

  18. One case of nasal septum and nasopharynx chordoma%鼻中隔及鼻咽部脊索瘤一例

    Institute of Scientific and Technical Information of China (English)

    曲维东; 金峰; 孙树东

    2010-01-01

    @@ 患者女,49岁,因双侧鼻塞半年于2008年5月入院.患者鼻塞呈持续性,渐进性加重,伴嗅觉减退,偶有头痛,无脓涕及涕中带血.专科检查:鼻中隔后部双侧对称性隆起,肿物约1.8 cm×2.0 cm大小,表面黏膜光滑,无破溃.

  19. Imaging diagnosis and differential diagnosis of chordomas%脊索瘤的影像学诊断和鉴别诊断

    Institute of Scientific and Technical Information of China (English)

    田爱民; 李威; 马国林

    2013-01-01

    目的 探讨脊索瘤的影像学表现.方法 收集经手术病理证实的脊索瘤14例,其中行磁共振成像(MRI)12例,CT 3例,1例行CT+MRI,对其影像学表现进行回顾性分析.结果 14例脊索瘤中,发生在颅底5例(36%),骶尾部9例(64%).CT表现肿瘤为等或稍高密度,常见斑片样或斑点状钙化,骨质破坏以溶骨性常见;MRI表现为T1WI呈低、等、高混杂信号,T2WI则以高、低混杂信号为主.增强后病灶呈中到重度强化,强化信号不均匀.结论 脊索瘤的CT和MRI表现有一定的特征性,结合临床大多可以确诊;MRI表现较具特征性,对脊索瘤的位置、形态、浸润范围与周围组织的解剖关系优于CT.

  20. Chordoma in Parapharyngeal and Retropharyngeal Space:Case Report%咽旁间隙巨大脊索瘤1例报告

    Institute of Scientific and Technical Information of China (English)

    张治忠; 高春莲; 任婵

    2011-01-01

    @@ 脊索瘤是一种少见的先天性肿瘤,由胚胎残留的脊索组织发展而成.可以发生于沿脊柱中轴的任何部位,但以斜坡嘴侧和骶尾部最常见,颈椎部脊索瘤罕见.其生长缓慢,好发于50~60岁的中老年人.此肿瘤局部破坏性很强,手术后极易复发,属于低度恶性肿瘤.

  1. 完全囊变的斜坡脊索瘤1例%Completely cystic changed chordoma in the clivus: one case report

    Institute of Scientific and Technical Information of China (English)

    陈绪珠

    2011-01-01

    @@ 患者,女,14岁.因"阵发性头痛3月,加重2月"入院.既往体健,无恶心、呕吐、眩晕、复视及抽搐等,体检病理征阴性. 影像学检查:CT扫描示后颅窝及鞍旁巨大低密度病变,最大径约5.7 cm,CT值4HU左右,病变无钙化,分叶状,边界清晰,斜坡左上部分不规则骨质破坏(图1A,B).

  2. 逆分化脊索瘤1例及文献复习%Dedifferentiated chordoma: a report of 1 case and review of literature

    Institute of Scientific and Technical Information of China (English)

    卢志达; 李明; 张勇

    2002-01-01

    目的报告1例逆分化脊索瘤(DC),复习文献重点了解其肉瘤化后的生物学性质.方法标本做常规HE切片及免疫组化染色.结果病变有典型的脊索瘤和梭形细胞肉瘤成分,两者有移行.免疫组化显示脊索瘤S-100、AEI/AF3(+),AAT、LTS、HHF35(-);梭形细胞肉瘤成分AAT、LYS(+),HHF35部分(+);梭形细胞肉瘤成分交界区部分细胞S-100、AEI/AE3(+),中央区S-100、AE1/AF3(-),表明免疫组化也有移行.综合文献资料发现DC恶性度更高,侵袭性更大,预后更差.结论DC很少见,在脊索瘤亚型中预后最差

  3. 颅底脊索瘤的临床诊治进展%Recent advances in diagnosis and treatment of skull-base chordomas

    Institute of Scientific and Technical Information of China (English)

    吕胜青; 杨辉; 陈立华

    2005-01-01

    颅底脊索瘤是一种较少见的胚胎残余组织肿瘤,呈缓慢浸润性生长.以往因其位置深在且对颅底重要神经结构广泛侵犯,颅底脊索瘤的治疗一直是颅底外科的难题之一.近年来随着神经影像学、肿瘤病理学和颅底外科学的发展,颅底脊索瘤的诊断及治疗也取得了长足的进步.本文就该肿瘤的临床表现、病理诊断、分型和手术入路等诊治进展作一综述.

  4. Clinical and pathological study of chordoma in the skull base%颅底脊索瘤的病理与临床研究

    Institute of Scientific and Technical Information of China (English)

    农辉图; 农东晓; 农晓琳; 滕映藩; 梁裕盛; 吴鸿泉; 温文胜

    2002-01-01

    目的研究脊索瘤的组织病理及临床诊断和治疗.方法观察20例脊索瘤的临床表现,影像所见及组织病理特征;对7例脊索瘤作免疫组化标记抗原染色;对2例脊索瘤作电镜观察,19例手术治疗,其中6例鼻侧及腭间进路中以内镜辅助下完成手术,11例加放射治疗,随访观察3~5年.结果①根据症状和体征、影像所见、原发部位、扩展范围和手术所见20例临床分型:蝶鞍型5例,斜坡型9例,枕颞型2例,广泛型4例,其中1例广泛型有多发性颅神经损害,未手术死亡,尸解诊断为脊索瘤肺转移,肿瘤压迫脑干生命中枢致死;3,5年生存率分别为65%和35%;②脊索瘤的病理检查见特征性的液滴细胞;③免疫组化标记蛋白染色中上皮性抗原的阳性率比其他间叶肿瘤上皮性抗原阳性率高;透射电镜显示细胞内有多量扩张的内质网及胞浆微丝.结论脊索瘤是具有上皮性及间叶性抗原分化特性的低度恶性肿瘤,以液滴细胞为组织病理学特征,免疫组化染色有助于鉴别诊断.完全切除颅底脊索瘤在技术上是不可能的,术后易复发,可发生转移.手术及放射综合治疗可延长生存期.

  5. 鼻咽脊索瘤与鼻咽癌的鉴别诊断%Differential diagnosis between nasopharyngeal chordoma and carcinoma

    Institute of Scientific and Technical Information of China (English)

    袁响林; 胡长耀

    2001-01-01

    @@ 脊索瘤是一种临床上极少见的低度恶性肿瘤,约占颅内肿瘤的0.5%,易误诊为鼻咽癌,因而造成治疗不当.鼻咽部脊索瘤主要发生部位为斜坡,也可侵及中颅凹、鞍区及鞍旁[1].1987~1996年本院收治的7例鼻咽脊索瘤,6例误诊,误诊率85.7%.现对临床误诊原因加以分析.

  6. 斜坡脊索瘤的手术治疗(47例分析)%The Surgical Treatment of 47 Case of Clival Chordoma

    Institute of Scientific and Technical Information of China (English)

    曹晓运; 鲍伟民; 杨伯捷; 谢嵘

    2006-01-01

    目的:斜坡脊索瘤的临床特点和手术方法分析.方法:对自1994年1月-2003年6月在本院接受手术治疗的47例斜坡脊索瘤进行回顾性分析.结果:47例患者发病年龄为5~67岁,平均39岁.男女比例约为1.47:1.头痛、复视是本病最常见的症状.47例患者中,肿瘤全切16例,次全切除18例,部分切除13例.结论:目前斜坡脊索瘤手术全切率低,预后差,首次手术应尽可能达到全切.较彻底的手术切除和新型放射治疗的结合可能为斜坡脊索瘤的治疗带来新的希望.

  7. 骶骨脊索瘤手术切除及并发症的防治%Resection of sacral chordomas, prevention and treatment of its complications

    Institute of Scientific and Technical Information of China (English)

    马乐群; 徐亮; 代成浦; 段肾斌; 刘洪涛

    2004-01-01

    目的探讨骶骨脊索瘤的诊治及并发症的预防.方法本组12例骶骨脊索瘤患者,术中5例行腹主动脉暂时套扎,7例行球囊导管腹主动脉血流阻断后,再将骶骨肿瘤切除.初次手术患者11例,第二次手术患者1例.结果12例患者中,2例发生术后切口部分感染、坏死;1例出现局部巨大血肿;2例患者术后肿瘤复发.随访3个月至7年,平均3.2年.术中无一例死亡.结论骶骨脊索瘤的手术切除成功的关键在于有效地控制术中失血.球囊导管腹主动脉阻断术阻断血流安全、可靠,能明显减少术中出血.术前准备工作在保证手术的安全方面具有同样的重要性.

  8. One case report of giant chordoma in posterior nasal cavity%后鼻腔巨大脊索瘤1例

    Institute of Scientific and Technical Information of China (English)

    刁鑫伟; 叶明福; 张哉根

    2003-01-01

    患者,女性,49岁,因渐进性鼻阻伴嗅觉障碍,打鼾、耳鸣3月入院,查体:双侧下鼻甲稍大,双侧后鼻腔可见灰白色新生物填充,光滑,质软,触之不易出血,间接鼻咽镜见鼻咽新生物,副鼻窦CT示:鼻咽腔内软组织块影,左上颌窦少许粘膜增厚,蝶窦及斜坡未见占位及骨质破损,考虑息肉可能性大。术中见肿瘤位于斜坡底及蝶窦底部,基底附着于骨膜上,大小约4cm×4cm,表面灰白色,包膜完整,予以完整切除,未见瘤组织周围骨破坏。

  9. 胸腰椎脊索瘤的临床特点和治疗分析%Clinical character and cure analysis of thoracolumbar vertebral chordoma

    Institute of Scientific and Technical Information of China (English)

    王长峰; 肖建如; 魏海峰; 贾连顺; 杨兴海

    2004-01-01

    目的:探讨胸、腰椎脊索瘤的临床特点和治疗方法.方法:对7例胸、腰椎脊索瘤患者的临床资料进行总结,根据WBB分期,实施椎体或全脊椎切除,其中囊内切除3例、扩大切除4例.结果:术后5例患者症状完全消失.1例T7、T8脊索瘤患者出现切口下缘顽固性疼痛,封闭后疼痛减轻.1例L1~L3脊索瘤全瘫患者,双下肢肌力恢复至2级.病理检查镜下均可见"印章样细胞环"和"合胞体".术后平均随访5.2年,局部复发1例,无远处转移.结论:胸腰椎脊索瘤相对少见,临床表现不典型,容易误诊."印章样细胞环"和"合胞体"是胸、腰椎脊索瘤的典型病理表现.全脊椎切除肿瘤能降低局部复发率.

  10. Pathological characteristic and clinical analysis of thoracolumbar vertebral chordoma%胸腰椎脊索瘤的组织学研究和综合治疗

    Institute of Scientific and Technical Information of China (English)

    王长峰; 肖建如; 魏海峰; 杨兴海; 贾连顺

    2003-01-01

    目的对胸腰椎脊索瘤的临床特点进行总结,初步探讨脊索瘤的病理特点和综合治疗手段.方法对胸腰椎肿瘤,根据WBB分期,进行椎体或全椎节切除.术后病理均提示脊索瘤.其中5例患者细胞基质中出现软骨样细胞.结果术后病理出现软骨样基质的患者未出现复发和远处转移;胸腰椎脊索瘤总体预后好于骶骨脊索瘤.结论胸腰椎脊索瘤术前不一定行血管栓塞.术中仔细处理节段性血管,手术中出血控制较满意.扩大切除脊索瘤后,短期效果较好.预后和病理分型有一定的关系.

  11. FHIT基因与脊索瘤的相关性研究%Related Study of Fragile Histidine Triad Gene With Chordoma

    Institute of Scientific and Technical Information of China (English)

    梁德勇; 刘庆阳; 韩壮; 吕刚; 富伟能; 张维

    2002-01-01

    目的: 探讨脆性组氨酸三联体(fragile histidine triad,FHIT)基因在脊索瘤发生中的作用.方法: 选择FHIT基因内3个微卫星多态标记对18例脊索瘤进行杂合性丢失(loss of hetorozygosity, LOH)和微卫星不稳定性分析(microsatellite instability, MI).结果: 在18例脊索瘤标本中,LOH的总发生率为44.4%,其中在D3S 1300,D3S 4130和D3S 1234的LOH发生率为37.5%、26.7%、23.1%.MI总发生率为33.3%,3个位点的MI发生率分别为25%、13.3%、15.4%.微卫星多态分析表明,FHIT基因在脊索瘤中既存在杂合性丢失,又存在微卫星不稳定现象. 结论: FHIT基因参与了脊索瘤的发生,可能是脊索瘤候选抑癌基因之一.

  12. Clinical-Pathological Analysis of 29 cases of chordomas%29例脊索瘤的临床病理分析

    Institute of Scientific and Technical Information of China (English)

    刘宁; 闫庆娜

    2006-01-01

    目的:探讨脊索瘤的病理诊断和鉴别诊断.方法:分析29例脊索瘤的临床表现、X线平片及病理形态.结果:29例脊索瘤患者临床表现无特异性.发生在骶尾部者25例.病史最长者4年半,最短2个月.随访5年10例术后复发.结论:脊索瘤复发率高,组织像中出现软骨化生可能是预后良好的指标.

  13. 颅底脊索瘤的临床研究进展%Recent advances in clinical of skull base chordoma

    Institute of Scientific and Technical Information of China (English)

    李梦远

    2012-01-01

    颅底脊索瘤是一种原发低度恶性肿瘤,具有局部侵袭特性和高复发率,预后较差.作者结合文献对颅底脊索瘤的流行病学、临床表现、影像学特征及组织病理学等方面进行了精要回顾,对脊索瘤的临床分型及分期、治疗原则及生存与预后相关的因素等做了详细的综述,认为目前国内外对颅底脊索瘤的诸多方面的研究仍存在很大的分歧,需要进一步明确.

  14. 18例颅底脊索瘤临床分析%Clinical analysis of 18 cases of chordomas in the skull base

    Institute of Scientific and Technical Information of China (English)

    朱林; 史继新; 王汉东; 樊友武; 杭春华; 成惠林; 潘云曦; 孙康健; 谢(韦华); 印红霞

    2005-01-01

    目的:探讨颅底脊索瘤的诊治方法.方法:分析18例颅底脊索瘤患者的临床表现、影像学特征、外科治疗和预后情况. 结果:18例颅底脊索瘤患者中无特异性临床表现.手术全切除5例,大部分切除13例.经病理证实其中典型脊索瘤17例,软骨样脊索瘤1例.14例获得随访,其中4例死亡,10例恢复正常生活. 结论:颅底脊索瘤发病率低,手术全切除的难度大,需结合放射治疗.本病预后差.

  15. Chordoma: Imaging Diagnosis and Clinical Therapy%脊索瘤的影像诊断及临床治疗分析

    Institute of Scientific and Technical Information of China (English)

    梁艺; 邱士军

    2010-01-01

    目的 探讨脊索瘤的影像特点及临床治疗.方法 回顾性分析32例经病理证实的脊索瘤MRI、CT、病理及各种临床治疗手段及相关预后.结果 32例中,颅底19例,颈椎1例;骶尾部12例.23例行MRI检查,10例行CT检查,2例行DSA检查.MRI:肿块边界清、分叶,均有不同程度骨质破坏;T_2WI高信号为主,T_1WI低信号为主,增强后18例轻中度强化, 1例明显强化.CT:6例散点状钙化,等低密度为主7例,高密度为主2例,1例混杂密度,3例行增强扫描,呈轻度强化.病理示大量黏液样基质成分及其中的液滴状细胞为特征,免疫组化CK、Vitmentin、EMA、S-100、Ki-67、PAS等阳性.共30例进行手术,3例无明显残留,6例2次或多次手术,3例X刀定位放疗,2例经皮注射化疗药.结论 脊索瘤的影像特点具有特征性,彻底的手术切除及辅以放化疗是治疗脊索瘤的共识.

  16. Xray,CT and MRI findings of sacrococcygeal chordoma%骶尾椎脊索瘤X线、CT和MRI表现

    Institute of Scientific and Technical Information of China (English)

    廖金生; 陆勇; 丁晓毅; 杜联军; 颜凌; 娄江华; 亢延卿

    2012-01-01

    目的 分析骶尾椎脊索瘤的X线、CT和MRI表现特点,提高对其诊断和鉴别诊断的能力.方法 回顾性分析25例经病理证实的骶尾椎脊索瘤患者的X线、CT和MRI表现特点,其中X线25例,CT 20例,MRI 20例.结果 25例X线检查显示骨质破坏19例,软组织肿块14例,骶髂关节受累10例,钙化12例.20例CT检查显示骨质破坏19例,其肿瘤内均见钙化,破坏区均涉及骶尾椎前缘,同时向后破坏骶尾椎后缘、突入椎管者15例,分叶17例,臀部肌肉受累13例,骶髂关节受累9例(4例为单侧,5例为双侧).20例 MR检查显示,T1WI为低信号,其中16例见斑片状稍高信号影,T2WI呈混杂等高信号影,STIR呈高信号影,包膜19例,分叶17例,肿块内分隔20例,骶髂关节受累10例,其中单侧3例,双侧7例,侵犯臀部肌肉14例,"横板"征13例,向上侵犯骶管17例形成"反引号"征.22例病灶中心位置位于S3及以下.结论 骶尾椎脊索瘤有其典型的好发年龄、部位及影像学表现特点,充分认识和应用X线、CT和MRI检查的影像表现特点,对提高诊断,指导临床手术治疗及预测预后等具有重要作用.

  17. 右腮腺区脊索瘤临床病理观察%Chordoma in right parotid:a clinicopathological analysis

    Institute of Scientific and Technical Information of China (English)

    周峻; 金岩; 董绍忠

    2008-01-01

    目的 报道腮腺区罕见脊索瘤1例,结合文献探讨脊索瘤生物学行为、临床病理特征以及免疫组化特点.方法 分析脊索瘤的临床、组织病理学资料.应用常规HE染色及免疫组化染色行CK、Vim、S-100、EMA和CEA等检测.结果 组织学上,脊索瘤被纤维组织分隔成分叶状,以液滴或空泡细胞为显著特征.免疫组化显示Vim、CK、EMA(+),少数细胞S-100呈(+),CEA(-).结论 脊索瘤属于具有上皮性及间叶性抗原分化特性的低度恶性肿瘤,腮腺区脊索瘤极罕见,组织学形态上应与转移的黏液腺癌、黏液样软骨肉瘤及软骨瘤鉴别.此瘤侵袭性强,易复发和误诊,可通过CK、Vim、EMA、CEA和S-100联合标记进行鉴别诊断.

  18. Pathological typing and prognosis analysis of sacrococcygeal chordoma%骶骨脊索瘤病理分型与预后

    Institute of Scientific and Technical Information of China (English)

    兰斌尚; 王坤正; 张建华; 吕惠茹

    2000-01-01

    目的探讨骶骨脊索瘤临床病理分型与预后的关系.方法分析24例患者的临床表现、病理分型、手术治疗及随访结果.结果软骨型9例,平均生存5.5年;典型脊索瘤15例,平均生存3.5年.3例手术完全切除,随访2、2.5、4年未复发;不完全切除21例,2年复发13例.结论软骨型脊索瘤比典型脊索瘤预后佳.包括骶骨的肿瘤完全切除术预后良好,但常难以实现.

  19. 脊索瘤针吸细胞学诊断探讨%The Approach of Fine Needle Aspiratiopn Biopsy of Chordoma

    Institute of Scientific and Technical Information of China (English)

    潘秦镜; 张询

    1999-01-01

    8例脊索瘤(1例骶尾部,2例颈部,5例颅底部)治疗前经针吸细胞学诊断.所有病例都有共同的细胞学表现:有两种细胞成份:一种是有丰富泡沫状或空泡状胞浆的圆形、卵圆形或多边形液滴状细胞,另一种是无胞浆空泡的小圆形、短梭形或星形细胞;多数细胞核圆形或卵圆形无明显异型,核染色质轻度增多、分布均匀,核浆比例不高;细胞单个散在或排列为松散的细胞群;涂片背景中有多少不等的粘液状物.本资料分析表明针吸细胞学结合临床及X线所见可以诊断脊索瘤.

  20. 颅内脊索瘤64例临床分析%Clinical Analysis of 64 Cases of Intracranial Chordomas

    Institute of Scientific and Technical Information of China (English)

    曹晓运; 陈衔城; 毛颖; 顾宇翔

    2003-01-01

    背景与目的:颅内脊索瘤好发于颅底,侵袭范围广泛,手术全切除困难,是神经外科的难题之一.本文探讨颅内脊索瘤临床特点、诊断及手术入路的选择.方法:对自1992年1月~2002年8月间在本院手术治疗的64例颅内脊索瘤进行回顾性分析.结果:64例发病年龄为5~67岁,平均37.5岁.男女比例约为1.6:1.头痛、复视是本病最常见的症状.64例患者共接受69次手术,肿瘤全切21例,次全切除24例,部分切除19例.结论:目前颅内脊索瘤手术全切率仍低,预后差,宜行积极手术治疗,手术入路的选择需根据肿瘤的解剖位置和侵袭的方向而定.

  1. 颅底脊索瘤的CT、MRI分析%Analysis of CT and MRI features in chordoma of the skull base

    Institute of Scientific and Technical Information of China (English)

    邓利猛; 廖伟华; 王小宜; 郑发祥

    2010-01-01

    目的:分析颅底脊索瘤的CT、MRI表现,探讨CT和MRI的诊断价值.方法:回顾性分析经手术病理证实的15例颅底脊索瘤,6例同时进行CT、MRI扫描,4例仅行CT扫描,5例仅行MRI扫描,2例同时行CTA检查.结果:颅底脊索瘤发生在斜坡8例,鞍区5例,颈静脉孔区1例,1例同时跨颅中、后窝生长.形态多为类圆形或不规则形,13例边界清晰,2例边界不清晰.CT表现肿块呈不均匀等或稍高密度软组织块,7例见斑点状钙化,8例见不同程度骨质破坏.MRI肿块信号不均匀,T1WI呈稍低信号或等信号,T2WI呈明显高信号,增强扫描呈不均匀轻中度强化.MRI扫描对病变范围的显示优于CT.结论:颅底脊索瘤有典型的好发部位,T2WI明显高信号较具特征性,CT和MRI对术前诊断颅底脊索瘤有较大价值.

  2. 颅底脊索瘤的长期随访研究%A long term follow-up study of the skull base chordomas

    Institute of Scientific and Technical Information of China (English)

    吴震; 张俊廷; 张力伟; 贾桂军; 王亮

    2007-01-01

    目的 通过总结颅底脊索瘤手术后生存、复发等特点,探讨颅底脊索瘤的长期预后.方法 采用电话、信件、门诊复查方式对1993年5月至2005年6月期间接受手术治疗的106例颅底脊索瘤患者进行随访,应用远期生活质量评分(Karnofsky Performance Scale,KPS)对全部患者术前、出院前、随访时的生存状况进行评分,SPSS生命表法计算生存率、复发率.结果 79例得到随访,随访时间10~158 个月,平均63.9个月,本组1、3、5、10年复发率分别为19.1%、34.7%、52.9%和88.3%,本组1、3、5、10年生存率分别为87.2%、79.4%、67.6%、59.5%.入院时KPS平均评分71.0分,出院前KPS平均评分72.5分,最后随访KPS平均评分67.1分,与出院前评分存在显著差异(P=0.020).结论 颅底脊索瘤预后不良,术后复发率呈逐年上升趋势,术后生存率前7年呈阶梯状下降,第8年后有逐渐平稳的趋势.手术治疗可以改善多数患者的远期生活质量.

  3. Radiosurgery for post-operative chordomas of skull base%伽玛刀治疗手术后的颅底脊索瘤

    Institute of Scientific and Technical Information of China (English)

    刘阿力; 王忠诚; 孙时斌; 王美华; 罗斌; 刘鹏

    2006-01-01

    目的探讨手术后残存脊索瘤的伽玛刀治疗的相关因素.方法回顾性分析本中心自1996年6月至2004年底伽玛刀治疗的31例手术残存脊索瘤病例,其中男性20例,女性11例;年龄8~70岁,平均40.2岁.伽玛刀治疗的肿瘤体积0.47~27.6cm3,平均(11.4±7.4)cm3;射点数3~12个,平均(6.5±2.4)个;肿瘤边缘剂量10~16Gy,平均(12.7±1.2)Gy;最大剂量20.8~40Gy,平均(29.2±4.0)Gy.结果本组28例得到治疗后6-102个月(平均30.2个月)的临床随访;和6-78个月,平均28个月的影像随诊.用Kaplan-Meier生存分析,患者3年和5年的总生存率为90.9%和75.8%.而影像学随诊中3年和5年的肿瘤实际控制率为64.2%和21.4%.伽玛刀治疗后1年左右多数肿瘤体积缩小;临床症状也相应缓解.随着时间延长,肿瘤复发比例增高.本组肿瘤增大的9例中,7例为伽玛刀靶区外生长;再次伽玛刀治疗4例,再手术4例,继续临床观察1例.本组共2例死亡.尚未发现伽玛刀相关的严重并发症.结论对开颅手术残存的脊索瘤,伽玛刀是一种可选的治疗方法.治疗要及时,并要充分认知肿瘤的习性和对伽玛刀治疗的反应,有益于延长患者生存期,提高生存质量.

  4. 鼻咽脊索瘤7例临床分析%Clinical Analysis of 7 Cases of Nasopharynxgeal Chordoma

    Institute of Scientific and Technical Information of China (English)

    袁响林; 胡国清; 于世英; 胡长耀

    2001-01-01

    目的 探讨鼻咽脊索瘤的诊断方法及治疗方案。方法 回顾性分析7例鼻咽脊索瘤的临床特征、误诊情况、治疗方法及随访结果。结果 (1)7例中6例被CT及临床医师误诊为鼻咽癌;误诊率均为87.1%(6/7)。(2)全组5年生存率71%(5/7),5年无瘤生存率57%(4/7)。结论 (1)鼻咽脊索瘤易误诊为鼻咽癌。(2)手术切除加术后放疗是最佳治疗方法。

  5. 经蝶切除蝶骨斜坡区脊索瘤%Transsphenoidal approach to chordoma invading the sphenoid bone and clivus

    Institute of Scientific and Technical Information of China (English)

    魏少波; 周定标; 许百男; 余新光; 张远征

    2003-01-01

    目的探讨颅底脊索瘤的手术入路和治疗方法,以提高肿瘤的全切率和治疗效果.方法经蝶入路切除蝶骨斜坡区脊索瘤.结果肿瘤次全切除14例(73.7%),大部分切除5例(26.3%).14例大部分切除无死亡.5年生存率58%.结论经蝶入路是一种安全、有效、创伤小、省时的手术入路;适宜切除大多数蝶骨斜坡区脊索瘤;积极切除肿瘤术后结合放疗可提高生存质量并能明显延长生存率.

  6. 颅底脊索瘤患者生存因素分析%Factors influencing the survival of patients with skull base chordoma

    Institute of Scientific and Technical Information of China (English)

    张俊廷; 王亮; 吴震; 贾桂军; 张力伟

    2007-01-01

    目的 分析影响颅底脊索瘤患者生存的因素.方法 收集有完善资料的颅底脊索瘤患者79例,选取性别、年龄、首发症状、阳性体征、肿瘤体积、入院前手术、入院前放疗、肿瘤部位和分期、肿瘤的质地及粘连程度、肿瘤的病理学分型、手术切除程度等可能影响颅底脊索瘤患者生存期的13个因素,用Kaplan-Meier法、Cox回归及t检验进行分析.结果 用Kaplan-Meier法单因素分析显示,肿瘤的病理学分型、入院之前曾行手术或放疗、手术切除程度、肿瘤与周围组织粘连程度是影响颅底脊索瘤患者生存期的危险因素(均P<0.01);Cox回归多因素分析显示肿瘤的病理学分型、入院之前行手术或放疗、手术切除程度对颅底脊索瘤患者生存期的影响具有统计学意义(均P<0.01).结论 结合影响颅底脊索瘤患者预后的相关因素,进一步完善包括手术技术在内的治疗手段,合理安排综合治疗的步骤,将有助于改善颅底脊索瘤患者的预后.

  7. The diagnostic value of chordoma in low field MRI%低场MRI对脊索瘤的诊断价值

    Institute of Scientific and Technical Information of China (English)

    张铁英; 刘振玉; 周士海

    2003-01-01

    目的:旨在探讨低场MRI脊索瘤表现特点,提高其诊断水平.方法:对10例手术病理证实的脊索瘤患者的低场MRI表现进行了回顾性分析.结果:脊索瘤的低场MRI的主要表现有:肿瘤信号变化,肿瘤形态改变,骨质破坏,周围血管侵蚀及肿瘤外周侵犯等.结论:低场MRI的T2WI呈稍高信号是其表现特点,其诊断价值与中、高场强MRI无明显差异.

  8. 鼻咽部脊索瘤临床分析及治疗%Clinical analysis and treatment of nasopharyngeal chordoma

    Institute of Scientific and Technical Information of China (English)

    张建设; 郑维银; 羊书勇; 李峰; 陈军; 徐光

    2009-01-01

    目的:探讨鼻咽部脊索瘤的诊断及治疗方法.方法:回顾性分析5例鼻咽部脊索瘤的临床特征、诊断情况、治疗方法及随访结果.结果:5例患者通过CT或MRI及临床检查均诊断为鼻咽部脊索瘤,诊断准确率为100%;5例患者术后3~6年存活.结论:CT、MRI是辅助诊断鼻咽部脊索瘤的有效方法.

  9. Trial of Dasatinib in Advanced Sarcomas

    Science.gov (United States)

    2016-10-12

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

  10. Establishment of anti-chordoma hybridoma cell line and its application in the pathological diagnosis of human chordoma%抗人脊索瘤杂交瘤细胞系的建立及其单抗在病理诊断中的应用

    Institute of Scientific and Technical Information of China (English)

    刘云燕; 杨连甲; 裘秀春; 文艳华; 张殿忠; 范清宇

    2010-01-01

    目的:建立一株高特异性抗人脊索瘤单克隆抗体的杂交瘤细胞系,检测其在人脊索瘤组织中及其它骨肿瘤中的特异结合程度.方法:以人脊索瘤细胞株CM-319为抗原,免疫BALB/C小鼠,利用杂交瘤技术,研制出一株MAb-6F2杂交瘤细胞系.通过免疫组织化学方法检测了MAb-6F2在4种不同骨肿瘤石蜡切片和人正常髓核组织上的结合反应.结果:MAb-6F2在骨肉瘤组织上的结合率仅为10%;在软骨肉瘤上的结合率为10%;在骨样骨瘤组织上的结合率为10%;在脊索瘤组织上的结合率为100%;在正常髓核组织上为阴性; MAb-6F2在脊索瘤组织上的结合反应显著高于其它骨肿瘤(P<0.01).结论:MAb-6F2在人脊索瘤组织中的特异性高结合,可以为脊索瘤的诊断提供新的手段.

  11. MRI Diagnosis and Differential Diagnosis of Intracranial Chordoma and Nasopharyngeal Carcinoma%颅内脊索瘤与鼻咽癌的MRI诊断及鉴别诊断

    Institute of Scientific and Technical Information of China (English)

    张伟; 黄祖平; 谭长连; 罗良贤; 刘旋辉

    2005-01-01

    目的探讨颅内脊索瘤和鼻咽癌的MRI诊断及鉴别诊断,评价MRI诊断价值.方法回顾性分析经病理证实的8例脊索瘤、23例鼻咽癌的MRI表现.结果7例脊索瘤中心部位在颅底中线处的斜坡/鞍区,肿瘤以颅底为主,主要向颅内蔓延,广泛破坏枕骨斜坡或蝶骨体,呈边界清楚、范围较大的骨质缺损;与鼻咽部生长为主再向颅内侵犯的鼻咽癌不同.颅内脊索瘤和鼻咽癌因血供不同,强化亦有较明显区别.结论MRI是诊断颅内脊索瘤和鼻咽癌的很有效的检查方法,具有重要的诊断和鉴别诊断价值.

  12. CT、MRI对颅底脊索瘤诊断与鉴别的价值探讨%Approach of CT and MRI in diagnosis and differential diagnosis to skull base chordoma

    Institute of Scientific and Technical Information of China (English)

    陈文琴; 万玉萍; 李栋; 陈刚; 罗天扬

    2012-01-01

    目的:分析颅底脊索瘤的常见病变及其CT、MRI影像学表现,探讨CT、MRI对颅底脊索瘤诊断和鉴别诊断的价值.方法:对15例经临床病理证实的颅底脊索瘤病例临床资料及CT、MRI平扫和增强的影像学表现进行回顾性分析.结果:颅底脊索瘤MRI表现为T1WI呈低、等、高混杂信号,T2WT以高、低信号为主.MRI增强后,病灶呈不均匀轻到中度强化.CT表现病灶呈不均匀等密度或稍高密度肿块影,均见不同程度的骨质破坏.结论:MRI检查可以准确显示颅底脊索瘤的大小、形态、边界及其信号特点.CT检查在显示病灶的骨质破坏、残存骨质方面较MRI具有优势.因此,CT、MRI检查相结合在颅底脊索瘤诊断中具有重要价值,并能在与垂体瘤、颅咽管瘤、软骨肉瘤、鼻咽癌、脑膜瘤等颅内肿瘤鉴别诊断中提供帮助.

  13. Stereotactic Radiosurgery - Gamma Knife

    Science.gov (United States)

    ... that connects the ear to the brain ( acoustic neuroma ) Pituitary tumors Tumors that are not cancer (chordoma, ... M. Editorial team. Related MedlinePlus Health Topics Acoustic Neuroma Brain Tumors Radiation Therapy Browse the Encyclopedia A. ...

  14. One case report of chordoma in thoraco-lumbar spine which manifested with subtle signs of both cauda equine and spinal cord compression%表现为轻微马尾和脊髓压迫症状的胸腰椎脊索瘤:1例病例报告

    Institute of Scientific and Technical Information of China (English)

    Peter Su; Marika Hess; Dominic Foo

    2013-01-01

    1例表现为轻微马尾和脊髓压迫症状的65岁男性患者诊断为T12~L1脊索瘤,行T12~L1椎体切除融合手术后复健顺利、恢复良好(需拄拐行走),未行放、化疗处理;8个月后肿瘤复发,再次行T12肿瘤切除及T9~L3后路椎体切除融合分期手术,术后并发症包括切口感染、深静脉栓塞、心房纤颤、尿脓毒症、继发于椎旁脓肿、脑室炎的耐甲氧西林金黄色葡萄球菌菌血症,经积极对症处理于3个月后恢复出院,继续行放射治疗。本文报告此例脊索瘤的病灶位置、临床表现和复杂病程,希望为临床医师提供关于此类肿瘤病例的诊疗思路。

  15. Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas

    Science.gov (United States)

    2016-05-13

    Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Recurrent Childhood Soft Tissue Sarcoma

  16. Vascularised fibula osteocutaneous flap for cervical spinal and posterior pharyngeal wall reconstruction

    Directory of Open Access Journals (Sweden)

    Thankappan Krishnakumar

    2009-01-01

    Full Text Available We report a case of vascularised fibula osteocutaneous flap used for composite cervical spinal and posterior pharyngeal wall reconstruction, in a patient with recurrent skull base chordoma, resected by an anterior approach via median labio-mandibular glossotomy approach. Bone stability and pharyngeal wall integrity were simultaneously restored

  17. Clinical results of carbon ion radiotherapy for bone and soft tissue tumors

    International Nuclear Information System (INIS)

    First choice of treatment for bone and soft tissue tumors is surgical tumor resection, but some cases have difficulties to resect radically because of tumor size, location, or their reduction in QOL after surgery. Carbon ion radiotherapy has been reported that have both good local tumor control and high QOL for patients with bone and soft tissue tumors, especially sacral chordoma and unresectable osteosarcoma of the tract. Some articles of the results with carbon ion radiotherapy for sacral chordoma show better local control and QOL than that of surgery. Moreover, several reports show good local control and preservation of QOL for patients with unresectable osteosarcoma of the tract, retroperitoneal sarcoma, and other situations of sarcomas. Now carbon ion radiotherapy can offer a promising alternative to surgery for patients with unresectable sarcomas. We will discuss about the results of carbon ion radiotherapy for bone and soft tissue tumors in this issue. (author)

  18. CT and MR findings of chordoid meningioma: a case report

    International Nuclear Information System (INIS)

    A 25-year-old woman with focal seizure, intermittent morning headache and vomiting for 2 years showed microcytic hypochromic anemia on peripheral blood smear and a 6 x 6 x 7.5 cm sized intracranial mass with cystic and solid portions at the right temporoparietal convexity on brain CT and MRI which was hypervascular on cerebral angiography. Histopathologic findings on light microscopy suggested chordoma, but it was confirmed as a chordoid meningioma by immunohistochemical study. The present case suggests that the diagnosis of chordoid meningioma should be considered in a juvenile or young adult who is presented with an extra-axial mass with typical location of meningiomas, findings of chordomas on light microscopy, and clinical findings of Castleman syndrome

  19. 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

  20. The Petro-Occipital Trans-Sigmoid Approach for Lesions of the Jugular Foramen

    OpenAIRE

    Mazzoni, Antonio

    2009-01-01

    This study's goals were twofold: (1) to analyze the author's experience with the petro-occipital trans-sigmoid (POTS) approach for the resection of tumors arising in or adjacent to the jugular foramen, and (2) to define the anatomical sites exposed by this approach. A retrospective review was conducted of 61 patients with jugular fossa tumors that included lower cranial nerve schwannomas, paragangliomas, meningiomas, chordomas, cholesteatomas, and other benign or low-grade malignant tumors. O...

  1. Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma

    Science.gov (United States)

    2016-09-23

    Adult Rhabdomyosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent 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

  2. 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 ...

  3. Sellar tumors; Tumoren der Sellaregion

    Energy Technology Data Exchange (ETDEWEB)

    Weber, M.A.; Essig, M. [Deutsches Krebsforschungszentrum, Heidelberg (Germany). Abt. Radiologie; Zoubaa, S. [GSF - Forschungszentrum fuer Gesundheit und Umwelt, Neuherberg, Muenchen (Germany). Abt. Neuropathologie; Politi, M.; Grunwald, I. [Universitaetsklinikum Saarland, Homburg (Germany). Klinik fuer Diagnostische und Interventionelle Neuroradiologie

    2007-06-15

    Because of the complex anatomy, examination of the sella turcica and hypophysis needs a dedicated MR sequence protocol. Not every sellar lesion is a pituitary adenoma. Thus, this review article summarizes the most frequently encountered intra-, supra-, and parasellar tumors and lesions. Differential diagnoses comprise besides adenoma among others craniopharyngioma, meningioma, glioma, germinoma, hamartoma, aneurysm, trigeminal schwannoma, pituitary carcinoma, chordoma, metastasis, infection, and empty sella. Characteristic imaging findings are presented and correlated to micro- and macro-pathology. (orig.)

  4. Bone tumors of the spine and sacral bone; Primaere Tumoren der Wirbelsaeule und des Sakrums

    Energy Technology Data Exchange (ETDEWEB)

    Freyschmidt, Juergen [Klinikum Bremen-Mitte gGmbH, Bremen (Germany). Beratungsstelle und Referenzzentrum fuer Osteoradiologie

    2010-12-15

    Bone tumors and tumorlike lesions of the spine are rare entities and may harbour diagnostic problems. In this article we discuss the epidemiology, topographic aspects, clinical and radiologic features as well as the diagnostic management of vertebral bone tumors. Entities that should be more familiar to the radiologist (i.e. osteoid osteoma, osteoblastoma, hemangioma, giant cell tumor, chordoma, aneurysmal bone cyst) are considered in more detail. (orig.)

  5. Analysis of receptor tyrosine kinases (RTKs) and downstream pathways in chordomas†

    Science.gov (United States)

    Tamborini, Elena; Virdis, Emanuela; Negri, Tiziana; Orsenigo, Marta; Brich, Silvia; Conca, Elena; Gronchi, Alessandro; Stacchiotti, Silvia; Manenti, Giacomo; Casali, Paolo G.; Pierotti, Marco A.; Pilotti, Silvana

    2010-01-01

    We have previously demonstrated that chordomas express activated platelet-derived growth factor receptor (PDGFRB) and that treatment with imatinib, which is capable of switching off the activation of various receptor tyrosine kinases (RTKs) including PDGFRB, benefits a number of patients. The aim of this study was to identify the possible presence of other activated RTKs and their downstream signaling effectors. Cryopreserved material from 22 naïve sporadic chordomas was investigated for the presence of activated RTKs and their cognate ligands and downstream signaling effectors by means of human phospho-RTK antibody arrays, Western blotting, and molecular analysis; immunohistochemistry and fluorescence in situ hybridization were used to analyze the corresponding formalin-fixed and paraffin-embedded samples. We detected activated PDGFRB, FLT3, and colony stimulating factor 1 receptor (CSF1R) of the PDGFR family and highly phosphorylated EGFR, HER2/neu, and (to a lesser extent) HER4 of the EGFR family. The detection of PDGFRB/PDGFB confirmed our previous data. The presence of activated EGFR was paralleled by the finding of high levels of epidermal growth factor (EGF) and transforming growth factor α (TGFα) and PDGFB co-expression and PDGFRB co-immunoprecipitation. Of the downstream effectors, the PI3K/AKT and RAS/MAPK pathways were both activated, thus leading to the phosphorylation of mammalian target of rapamycin (mTOR) and 4E-BP1 among the regulators involved in translational control. Taken together, our results (i) provide a rationale for tailored treatments targeting upstream activated receptors, including the PDGFR and EGFR families; (ii) support the idea that a combination of upstream antagonists and mTOR inhibitors enhances the control of tumor growth; and (iii) indicate that the 4E-BP1/eIF4E pathway is a major regulator of protein synthesis in chordoma. PMID:20164240

  6. Intracranial pathology of the visual pathway

    Energy Technology Data Exchange (ETDEWEB)

    Mueller-Forell, W. E-mail: mueller-forell@neuroradio.klinik.uni-mainz.de

    2004-02-01

    Intracranial pathologies involving the visual pathway are manifold. Aligning to anatomy, the most frequent and/or most important extrinsic and intrinsic intracranial lesions are presented. Clinical symptoms and imaging characteristics of lesions of the sellar region are demonstrated in different imaging modalities. The extrinsic lesions mainly consist of pituitary adenomas, meningeomas, craniopharyngeomas and chordomas. In (asymptomatic and symptomatic) aneurysms, different neurological symptoms depend on the location of aneurysms of the circle of Willis. Intrinsic tumors as astrocytoma of any grade, ependymoma and primary CNS-lymphoma require the main pathology in the course of the visual pathway. Vascular and demyelinating diseases complete this overview of intracranial lesions.

  7. 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

  8. Augmented reality-assisted skull base surgery.

    Science.gov (United States)

    Cabrilo, I; Sarrafzadeh, A; Bijlenga, P; Landis, B N; Schaller, K

    2014-12-01

    Neuronavigation is widely considered as a valuable tool during skull base surgery. Advances in neuronavigation technology, with the integration of augmented reality, present advantages over traditional point-based neuronavigation. However, this development has not yet made its way into routine surgical practice, possibly due to a lack of acquaintance with these systems. In this report, we illustrate the usefulness and easy application of augmented reality-based neuronavigation through a case example of a patient with a clivus chordoma. We also demonstrate how augmented reality can help throughout all phases of a skull base procedure, from the verification of neuronavigation accuracy to intraoperative image-guidance.

  9. The presence and absence of lymphatic vessels in the adult human intervertebral disc: relation to disc pathology

    Energy Technology Data Exchange (ETDEWEB)

    Kliskey, Karolina; Williams, Kelly; Yu, J.; Urban, Jill; Athanasou, Nick [University of Oxford, Nuffield Department of Orthopaedic, Rheumatology and Musculoskeletal Science, Oxford (United Kingdom); Jackson, David [Weatherall Institute of Molecular Medicine, Human Immunology Unit, Oxford (United Kingdom)

    2009-12-15

    Although the normal adult human intervertebral disc is considered to be avascular, vascularised cellular fibrous tissue can be found in pathological conditions involving the disc such as disc herniation. Whether lymphatics vessels form a component of this reparative tissue is not known as the presence or absence of lymphatics in herniated and normal disc tissue is not known. We examined spinal tissues and discectomy specimens for the presence of lymphatics. The examination used immunohistochemistry to identify the specific lymphatic endothelial cell markers, podoplanin and LYVE1. Lymphatic vessels were not found in the nucleus pulposus or annulus fibrosus of intact, non-herniated lumbar and thoracic discs but were present in the surrounding ligaments. Ingrowth of fibrous tissue was seen in 73% of herniated disc specimens of which 36% contained LYVE1+/podoplanin + lymphatic vessels. Lymphatic vessels were not seen in the sacrum and coccyx or biopsies of four sacrococcygeal chordomas, but they were noted in surrounding extra-osseous fat and fibrous tissue at the edge of the infiltrating tumour. Our findings indicate that lymphatic vessels are not present in the normal adult intervertebral disc but that, when there is extrusion of disc material into surrounding soft tissue, there is ingrowth of reparative fibrous tissue containing lymphatic vessels. Our findings also indicate that chordoma, a tumour of notochordal origin, spreads to regional lymph nodes via lymphatics in para-spinal soft tissues. (orig.)

  10. 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.

  11. Single-energy intensity modulated proton therapy.

    Science.gov (United States)

    Farace, Paolo; Righetto, Roberto; Cianchetti, Marco

    2015-10-01

    In this note, an intensity modulated proton therapy (IMPT) technique, based on the use of high single-energy (SE-IMPT) pencil beams, is described.The method uses only the highest system energy (226 MeV) and only lateral penumbra to produce dose gradient, as in photon therapy. In the study, after a preliminary analysis of the width of proton pencil beam penumbras at different depths, SE-IMPT was compared with conventional IMPT in a phantom containing titanium inserts and in a patient, affected by a spinal chordoma with fixation rods.It was shown that SE-IMPT has the potential to produce a sharp dose gradient and that it is not affected by the uncertainties produced by metal implants crossed by the proton beams. Moreover, in the chordoma patient, target coverage and organ at risk sparing of the SE-IMPT plan resulted comparable to that of the less reliable conventional IMPT technique. Robustness analysis confirmed that SE-IMPT was not affected by range errors, which can drastically affect the IMPT plan.When accepting a low-dose spread as in modern photon techniques, SE-IMPT could be an option for the treatment of lesions (e.g. cervical bone tumours) where steep dose gradient could improve curability, and where range uncertainty, due for example to the presence of metal implants, hampers conventional IMPT. PMID:26352616

  12. Single-energy intensity modulated proton therapy

    Science.gov (United States)

    Farace, Paolo; Righetto, Roberto; Cianchetti, Marco

    2015-09-01

    In this note, an intensity modulated proton therapy (IMPT) technique, based on the use of high single-energy (SE-IMPT) pencil beams, is described. The method uses only the highest system energy (226 MeV) and only lateral penumbra to produce dose gradient, as in photon therapy. In the study, after a preliminary analysis of the width of proton pencil beam penumbras at different depths, SE-IMPT was compared with conventional IMPT in a phantom containing titanium inserts and in a patient, affected by a spinal chordoma with fixation rods. It was shown that SE-IMPT has the potential to produce a sharp dose gradient and that it is not affected by the uncertainties produced by metal implants crossed by the proton beams. Moreover, in the chordoma patient, target coverage and organ at risk sparing of the SE-IMPT plan resulted comparable to that of the less reliable conventional IMPT technique. Robustness analysis confirmed that SE-IMPT was not affected by range errors, which can drastically affect the IMPT plan. When accepting a low-dose spread as in modern photon techniques, SE-IMPT could be an option for the treatment of lesions (e.g. cervical bone tumours) where steep dose gradient could improve curability, and where range uncertainty, due for example to the presence of metal implants, hampers conventional IMPT.

  13. 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

  14. 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.

  15. Parachordoma of Soft Tissues of the Arm: A Very Rare Tumour

    Directory of Open Access Journals (Sweden)

    Vicente Estrems Díaz

    2013-01-01

    Full Text Available Parachordoma is an infrequent neoplasm that bears some histologic resemblance to chordoma. It affects both sexes, occurs typically during the fourth decade of life, and tends to present as a slow-growing painless mass at the level of the soft tissues of the extremities. Diagnosis should be based on immunohistochemical and cytogenetic studies, as the findings of imaging techniques are often unspecific. Although it is considered a benign lesion, its behavior tends to be locally aggressive, with reports of a recurrence rate of up to 20% and of several cases of metastasis. Fewer than 60 cases have been published in the English-speaking literature. In this paper we present the case of a 32-year-old male with a two-year history of parachordoma in the right wrist.

  16. Differential diagnosis of dumbbell lesions associated with spinal neural foraminal widening: Imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Kivrak, Ali Sami [Selcuk University, Meram Medical Faculty, Department of Radiology, 42080 Konya (Turkey)], E-mail: alisamikivrak@hotmail.com; Koc, Osman; Emlik, Dilek; Kiresi, Demet; Odev, Kemal [Selcuk University, Meram Medical Faculty, Department of Radiology, 42080 Konya (Turkey); Kalkan, Erdal [Selcuk University, Meram Medical Faculty, Department of Neurosurgery, Konya (Turkey)

    2009-07-15

    Computed tomography (CT) and magnetic resonance imaging (MRI) reliably demonstrate typical features of schwannomas or neurofibromas in the vast majority of dumbbell lesions responsible for neural foraminal widening. However, a large variety of unusual lesions which are causes of neural foraminal widening can also be encountered in the spinal neural foramen. Radiologic findings can be helpful in differential diagnosis of lesions of spinal neural foramen including neoplastic lesions such as benign/malign peripheral nerve sheath tumors (PNSTs), solitary bone plasmacytoma (SBP), chondroid chordoma, superior sulcus tumor, metastasis and non-neoplastic lesions such as infectious process (tuberculosis, hydatid cyst), aneurysmal bone cyst (ABC), synovial cyst, traumatic pseudomeningocele, arachnoid cyst, vertebral artery tortuosity. In this article, we discuss CT and MRI findings of dumbbell lesions which are causes of neural foraminal widening.

  17. Primary bone tumors of the spine.

    Science.gov (United States)

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

    2016-04-01

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

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

    DEFF Research Database (Denmark)

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

    2009-01-01

    (s) for the different subgroups. Archival material from three extraskeletal myxoid chondrosarcomas, five chordomas, five chondromyxoid fibromas, five chondroblastomas and 25 chondrosarcomas was stained with antibodies against osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit) and YKL......-40. All 25 chondrosarcomas showed a positive staining reaction for D2-40, none for actin and CD117, and a partial reactivity for bcl-2 (36%). Chondroblastomas (5/5) and chondromyxoid fibromas (2/5) were the only tumors with a positive reaction for actin, and all chondroblastomas (n=5...... chondrosarcomas. A convincing immunoreactivity for calponin and/or actin in chondromyxoid fibromas and chondroblastomas may also be helpful in differentiating these tumors from chondrosarcomas....

  19. The incidentaloma of the pituitary gland: Is neurosurgery required

    Energy Technology Data Exchange (ETDEWEB)

    Reincke, M.; Allolio, B.; Saeger, W.; Menzel, J.; Winkelmann, W. (Univ. of Cologne (West Germany))

    1990-05-23

    The authors describe a series of 18 patients with an intrasellar mass incidentally discovered by computed tomography or magnetic resonance imaging. The average size of the mass was 13 mm, with a range from 5 to 25 mm. Initial ophthalmologic examination revealed bitemporal hemianopia in 2 patients. Results of routine endocrine testing showed partial hypopituitarism in 5 patients and growth hormone hypersecretion without signs and symptoms of acromegaly in 1 patient. Four patients underwent neurosurgery. Histologically, one chondroid chordoma and three pituitary adenomas were found. In the remaining 14 patients treated conservatively, repeated computed tomography and magnetic resonance imaging revealed no significant change in tumor size at the time of follow-up. The results suggest that the incidentaloma of the pituitary gland is a benign condition that does not necessarily require neurosurgical intervention.

  20. 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.

  1. 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)

  2. Secondary EWSR1 gene abnormalities in SMARCB1-deficient tumors with 22q11-12 regional deletions: Potential pitfalls in interpreting EWSR1 FISH results.

    Science.gov (United States)

    Huang, Shih-Chiang; Zhang, Lei; Sung, Yun-Shao; Chen, Chun-Liang; Kao, Yu-Chien; Agaram, Narasimhan P; Antonescu, Cristina R

    2016-10-01

    SMARCB1 inactivation occurs in a variety of tumors, being caused by various genetic mechanisms. Since SMARCB1 and EWSR1 genes are located close to each other on chromosome 22, larger SMARCB1 deletions may encompass the EWSR1 locus. Herein, we report four cases with SMARCB1-deletions showing concurrent EWSR1 gene abnormalities by FISH, which lead initially to misinterpretations as EWSR1-rearranged tumors. Our study group included various morphologies: a poorly differentiated chordoma, an extrarenal rhabdoid tumor, a myoepithelial carcinoma, and a proximal-type epithelioid sarcoma. All cases showed loss of SMARCB1 (INI1) by immunohistochemistry (IHC) and displayed characteristic histologic features for the diagnoses. The SMARCB1 FISH revealed homozygous or heterozygous deletions in three and one case, respectively. The co-hybridized EWSR1 probes demonstrated either unbalanced split signals or heterozygous deletion in two cases each. The former suggested bona fide rearrangement, while the latter resembled an unbalanced translocation. However, all the FISH patterns were quite complex and distinct from the simple and uniform split signals seen in typical EWSR1 rearrangements. We conclude that in the context of 22q11-12 regional alterations present in SMARCB1-deleted tumors, simultaneous EWSR1 involvement may be misinterpreted as equivalent to EWSR1 rearrangement. A detailed clinicopathologic correlation and supplementing the EWSR1 FISH assay with complementary methodology is mandatory for correct diagnosis. © 2016 Wiley Periodicals, Inc. PMID:27218413

  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. 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

  5. Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions

    Energy Technology Data Exchange (ETDEWEB)

    Bonneville, Fabrice; Chiras, Jacques [Pitie-Salpetriere Hospital, Department of Neuroradiology, Paris (France); Savatovsky, Julien [Adolphe de Rothschild Foundation, Department of Radiology, Paris (France)

    2007-11-15

    Computed tomography (CT) and magnetic resonance (MR) imaging reliably demonstrate typical features of vestibular schwannomas or meningiomas in the vast majority of mass lesions responsible for cerebellopontine angle (CPA) syndrome. However, a large variety of unusual lesions can also be encountered in the CPA. Covering the entire spectrum of lesions potentially found in the CPA, these articles explain the pertinent neuroimaging features that radiologists need to know to make clinically relevant diagnoses in these cases, including data from diffusion- and perfusion-weighted imaging or MR spectroscopy, when available. A diagnostic algorithm based on the lesion's site of origin, shape and margins, density, signal intensity and contrast material uptake is also proposed. Non-enhancing extra-axial CPA masses are cystic (epidermoid cyst, arachnoid cyst, neurenteric cyst) or contain fat (dermoid cyst, lipoma). Tumours can also extend into the CPA by extension from the skull base (paraganglioma, chondromatous tumours, chordoma, cholesterol granuloma, endolymphatic sac tumour). Finally, brain stem or ventricular tumours can present with a significant exophytic component in the CPA that may be difficult to differentiate from an extra-axial lesion (lymphoma, hemangioblastoma, choroid plexus papilloma, ependymoma, glioma, medulloblastoma, dysembryoplastic neuroepithelial tumour). (orig.)

  6. RECONSTRUCTION OF THE SELLAR FLOOR FOLLOWING TRANSSPHE-NOIDAL SURGERY USING GELATIN FOAM AND FIBRIN GLUE

    Institute of Scientific and Technical Information of China (English)

    Jian Yin; Chang-bao Su; Zhi-qin Xu; Xue-wei Xia; Fei Song

    2005-01-01

    Objective To introduce a new principle of sellar reconstruction and to evaluate the effectiveness of absorbable gelatin foam and fibrin glue for sellar reconstruction. Methods A total of 176 consecutive patients who underwent surgery for pituitary adenomas, cysts, chordomas, or subdiaphragmatic craniopharyngiomas in the sella turcica between January 2001 and April 2003 at Peking Union Medical College Hospital were enrolled. Different techniques of sellar closure and indications for each specific condition were retrospectively reviewed. Results Seventy-seven (43.7%) patients developed a visible cerebrospinal fluid (CSF) leakage during surgery. Intra operative CSF leakage were repaired simply with gelatin foam and fibrin glue in 62 (35.2%) patients, and with autologous fat graft and sellar floor reconstruction in 15 (8.5%) patients. Postoperative CSF rhinorrhea occurred only in 1 case. There were no visual deterioration, allergic rhinitis, meningitis, pneumocranium, granulomas, or other complications associated with the reconstruction procedure. Conclusion The procedure of using gelatin foam and fibrin glue and principle of cranial base reconstruction is safe and effective in preventing postoperative complications following transsphenoidal surgery.

  7. Percutaneous gastrostomy -a report of twenty-seven cases-

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Tae Ho; Lee, Ho Suk; Kim, Yong Joo; Kim, Tae Hun; Suh, Kyung Jin; Kang, Duk Sik [College of Medicine, Kyungpook National University, Daegu (Korea, Republic of)

    1991-05-15

    Nutritional support by gastrostomy feeding is an important treatment adjunct when major swallowing difficulty or debilitating diseases is present. This technique for percutaneous placement of a gastric feeding tube now provides many patients with a simple, safe, and well-tolerated alternative to surgical feeding gastrostomy. We experienced 27 cases of percutaneous gastrostomy from January 1989 to February 1991 at Kyungpook National University Hospital. The underlying diseases of the patients were esophageal cancer (11), pyriform sinus cancer (4), laryngeal cancer (3), tongue cancer (2), lye stricture (1), lung cancer (1), chordoma (1), lethal midline granuloma (1), malignant lymphoma (1), maxillary cancer (1), and tonsil cancer (1). Selding method was used in all cases. In five patients it was difficult to insert the nasogastric tube for air insufflation, and one patient had subtotal gastrectomy with gastrojejunostomy, but gastrostomy was performed in all cases. The general condition improved in all patients. We experienced one case of mild peritonitis, but no other significant complications were observed. Gastrostomy tubes were patient in all patients during their survival period, except two patients who experienced obstruction 2 and 6 months after the procedure. Percutaneous gastrostomy can be used safely and effectively in such patients who have pharyngoesophageal or CNS lesions precluding oral intake.

  8. Tumors of the skull base in children: review of tumor types and management strategies.

    Science.gov (United States)

    Tsai, Eve C; Santoreneos, Stephen; Rutka, James T

    2002-05-15

    Although many treatment strategies for skull base tumors in adults have been reported, relatively little has been reported regarding such therapies in the pediatric population. Skull base tumors in children present a therapeutic challenge because of their unique pathological composition, the constraints of the maturing skull and brain, and the small size of the patients. In this review, the authors examine the pediatric skull base lesions that occur in the anterior, middle, and posterior cranial base, focusing on unique pediatric tumors such as encepahalocele, fibrous dysplasia, esthesioneuroblastoma, craniopharyngioma, juvenile nasopharyngeal angiofibroma, cholesteatoma, chordoma, chondrosarcoma, and Ewing sarcoma. They review management strategies that include radio- and chemotherapy, as well as surgical approaches with emphasis on the modifications and complications associated with the procedures as they apply in children. Evidence for the advantages and limitations of radiotherapy, chemotherapy, and surgery as it pertains to the pediatric population will be examined. With a working knowledge of skull base anatomy and special considerations of the developing craniofacial skeleton, neurosurgeons can treat skull base lesions in children with acceptable morbidity and mortality rates. Outcomes in this population may be better than those in adults, in part because of the benign histopathology that frequently affects the pediatric skull base, as well as the plasticity of the maturing nervous system.

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

    Institute of Scientific and Technical Information of China (English)

    韦兴

    2014-01-01

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

  10. 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.)

  11. 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.

  12. [An operative case of cholesterol granuloma of the petrous apex].

    Science.gov (United States)

    Saino, M; Kayama, T; Kuroki, A; Siraisi, Y; Sato, K; Nakai, O

    1996-11-01

    A 59-year-old man presented with a rare cholesterol granuloma of the petrous apex manifesting as headache, left facial dysesthesia, diplopia, left hearing impairment, and left tinnitus. Neurological examination revealed dysesthesia of territory in all divisions of the left trigeminal nerve, left incomplete abducens nerve palsy, left mixed hearing impairment, and left tinnitus. Plain CT scan showed a smoothly marginated mass involving the left petrous apex. The mass was isodense with the brain parenchyma and not enhanced by contrast medium. The mass appeared heterogeneously slightly hyperintense on the T1-weighted MR image and homogeneously hyperintense on the T2-weighted MR image except for the peripheral portion. The mass was not enhanced after intravenous gadolinium DTPA administration. Surgery via a petrosal approach totally removed the mass in the intracranial, extradural space. Histological examination showed typical features of cholesterol granuloma, with cholesterin clefts, hemosiderin deposits, and erythrocytes in non-specific granulation tissue. Cholesterol granuloma most commonly occurs in the middle ear cavity, and rarely in the petrous apex. The characteristic hyperintense appearance of cholesterol granuloma on T1- and T2-weighted MR images is very useful for differentiation from other lesions of the petrous apex and the cerebellopontine angle such as cholesteatoma, mucocele, chordoma, and meningioma. Solid cholesterol granuloma of the petrous apex should be treated by total removal via craniotomy, not by drainage which is commonly performed by otorhinologists. PMID:8934474

  13. CT diagnosis of sellar and juxtasellar lesions, 2. Tumors other than pituitary adenomas

    Energy Technology Data Exchange (ETDEWEB)

    Kobayashi, Tatsuya (Nagoya Univ. (Japan). Faculty of Medicine)

    1982-06-01

    A study is done of the usefulness and limitation of the CT diagnosis of sellar and juxtasellar tumors other than pituitary adenomas based on 96 verified cases experienced at Nagoya University Hospital from October, 1976, to December, 1981. The tumors in this study included craniopharyngiomas, meningiomas, gliomas, paranasal and nasopharyngeal tumors, germinomas, chordomas, chondromas, osteomas, teratomas and teratoid tumors, and metastatic tumors. It is found that rather characteristic CT findings are demonstrated in some tumors: a suprasellar lesion with a mixed density demonstrated by craniopharyngiomas, the radiosensitivity of germinoma, the irregularity of the low-density area and the peritumoral entrapment of metrizamide demonstrated by epidermoid tumors, bony sclerosis demonstrated by meningiomas, growth along optic pathway in optic gliomas, the mode of enhancement in hypothalamic glioma, and a high-density mass upon plain CT demonstrated by colloid cysts. However, these characteristic findings are not definitely specific to each pathological aspect of a tumor. It will be possible, however, to diagnose these tumors more specifically by a combination of clinical signs, CT, and other neuroradiological findings.

  14. Proton Radiation Therapy for Head and Neck Cancer: A Review of the Clinical Experience to Date

    Energy Technology Data Exchange (ETDEWEB)

    Holliday, Emma B.; Frank, Steven J., E-mail: sjfrank@mdanderson.org

    2014-06-01

    Proton beam radiation has been used for cancer treatment since the 1950s, but recent increasing interest in this form of therapy and the construction of hospital-based and clinic-based facilities for its delivery have greatly increased both the number of patients and the variety of tumors being treated with proton therapy. The mass of proton particles and their unique physical properties (ie, the Bragg peak) allow proton therapy to spare normal tissues distal to the tumor target from incidental irradiation. Initial observations show that proton therapy is particularly useful for treating tumors in challenging locations close to nontarget critical structures. Specifically, improvements in local control outcomes for patients with chordoma, chonodrosarcoma, and tumors in the sinonasal regions have been reported in series using proton. Improved local control and survival outcomes for patients with cancer of the head and neck region have also been seen with the advent of improvements in better imaging and multimodality therapy comprising surgery, radiation therapy, and chemotherapy. However, aggressive local therapy in the proximity of critical normal structures to tumors in the head and neck region may produce debilitating early and late toxic effects. Great interest has been expressed in evaluating whether proton therapy can improve outcomes, especially early and late toxicity, when used in the treatment of head and neck malignancies. This review summarizes the progress made to date in addressing this question.

  15. 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

  16. 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.)

  17. Dosimetric comparisons of carbon ion treatment plans for 1D and 2D ripple filters with variable thicknesses

    Science.gov (United States)

    Printz Ringbæk, Toke; Weber, Uli; Santiago, Alina; Simeonov, Yuri; Fritz, Peter; Krämer, Michael; Wittig, Andrea; Bassler, Niels; Engenhart-Cabillic, Rita; Zink, Klemens

    2016-06-01

    A ripple filter (RiFi)—also called mini-ridge filter—is a passive energy modulator used in particle beam treatments that broadens the Bragg peak (BP) as a function of its maximum thickness. The number of different energies requested from the accelerator can thus be reduced, which significantly reduces the treatment time. A new second generation RiFi with 2D groove shapes was developed using rapid prototyping, which optimizes the beam-modulating material and enables RiFi thicknesses of up to 6 mm. Carbon ion treatment plans were calculated using the standard 1D 3 mm thick RiFi and the new 4 and 6 mm 2D RiFis for spherical planning target volumes (PTVs) in water, eight stage I non-small cell lung cancer cases, four skull base chordoma cases and three prostate cancer cases. TRiP98 was used for treatment planning with facility-specific base data calculated with the Monte Carlo code SHIELD-HIT12A. Dose-volume-histograms, spatial dose distributions and dosimetric indexes were used for plan evaluation. Plan homogeneity and conformity of thinner RiFis were slightly superior to thicker RiFis but satisfactory results were obtained for all RiFis investigated. For the 6 mm RiFi, fine structures in the dose distribution caused by the larger energy steps were observed at the PTV edges, in particular for superficial and/or very small PTVs but performances for all RiFis increased with penetration depth due to straggling and scattering effects. Plans with the new RiFi design yielded for the studied cases comparable dosimetric results to the standard RiFi while the 4 and 6 mm RiFis lowered the irradiation time by 25-30% and 45-49%, respectively.

  18. 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

  19. Nivolumab and Ipilimumab in Treating Patients With Rare Tumors

    Science.gov (United States)

    2016-08-24

    Acinar Cell Carcinoma; Adrenal Cortex Carcinoma; Adrenal Gland Pheochromocytoma; Anal Canal Neuroendocrine Carcinoma; Anal Canal Undifferentiated Carcinoma; Appendix Mucinous Adenocarcinoma; Bladder Adenocarcinoma; Bronchioloalveolar Carcinoma; Cervical Adenocarcinoma; Cervical Squamous Cell Carcinoma, Not Otherwise Specified; Cholangiocarcinoma; Chordoma; Colorectal Squamous Cell Carcinoma; Endometrial Adenocarcinoma; Endometrioid Adenocarcinoma; Esophageal Neuroendocrine Carcinoma; Esophageal Undifferentiated Carcinoma; Extrahepatic Bile Duct Carcinoma; Fallopian Tube Adenocarcinoma; Fibromyxoid Tumor; Gastric Neuroendocrine Carcinoma; Gastric Squamous Cell Carcinoma; Giant Cell Carcinoma; Intestinal Neuroendocrine Carcinoma; Intrahepatic Cholangiocarcinoma; Lung Carcinoid Tumor; Lung Sarcomatoid Carcinoma; Major Salivary Gland Carcinoma; Malignant Odontogenic Neoplasm; Malignant Peripheral Nerve Sheath Tumor; Malignant Skin Neoplasm; Malignant Testicular Sex Cord-Stromal Tumor; Metastatic Malignant Neoplasm of Unknown Primary Origin; Mixed Mesodermal (Mullerian) Tumor; Mucinous Adenocarcinoma; Mucinous Cystadenocarcinoma; Nasal Cavity Adenocarcinoma; Nasal Cavity Carcinoma; Nasopharyngeal Carcinoma; Nasopharyngeal Papillary Adenocarcinoma; Nasopharyngeal Undifferentiated Carcinoma; Oral Cavity Carcinoma; Oropharyngeal Undifferentiated Carcinoma; Ovarian Adenocarcinoma; Ovarian Germ Cell Tumor; Ovarian Mucinous Adenocarcinoma; Ovarian Squamous Cell Carcinoma; Pancreatic Acinar Cell Carcinoma; Pancreatic Neuroendocrine Carcinoma; Paraganglioma; Paranasal Sinus Adenocarcinoma; Paranasal Sinus Carcinoma; Parathyroid Gland Carcinoma; Pituitary Gland Carcinoma; Placental Choriocarcinoma; Placental-Site Gestational Trophoblastic Tumor; Primary Peritoneal High Grade Serous Adenocarcinoma; Pseudomyxoma Peritonei; Scrotal Squamous Cell Carcinoma; Seminal Vesicle Adenocarcinoma; Seminoma; Serous Cystadenocarcinoma; Small Intestinal Adenocarcinoma; Small Intestinal Squamous

  20. 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

  1. A panoramic view of the skull base: systematic review of open and endoscopic endonasal approaches to four tumors.

    Science.gov (United States)

    Graffeo, Christopher S; Dietrich, August R; Grobelny, Bartosz; Zhang, Meng; Goldberg, Judith D; Golfinos, John G; Lebowitz, Richard; Kleinberg, David; Placantonakis, Dimitris G

    2014-08-01

    Endoscopic endonasal surgery has been established as the safest approach to pituitary tumors, yet its role in other common skull base lesions has not been established. To answer this question, we carried out a systematic review of reported series of open and endoscopic endonasal approaches to four major skull base tumors: olfactory groove meningiomas (OGM), tuberculum sellae meningiomas (TSM), craniopharyngiomas (CRA), and clival chordomas (CHO). Data from 162 studies containing 5,701 patients were combined and compared for differences in perioperative mortality, gross total resection (GTR), cerebrospinal fluid (CSF) leak, neurological morbidity, post-operative visual function, post-operative anosmia, post-operative diabetes insipidus (DI), and post-operative obesity/hyperphagia. Weighted average rates for each outcome were calculated using relative study size. Our findings indicate similar rates of GTR and perioperative mortality between open and endoscopic approaches for all tumor types. CSF leak was increased after endoscopic surgery. Visual function symptoms were more likely to improve after endoscopic surgery for TSM, CRA, and CHO. Post-operative DI and obesity/hyperphagia were significantly increased after open resection in CRA. Recurrence rates per 1,000 patient-years of follow-up were higher in endoscopy for OGM, TSM, and CHO. Trends for open and endoscopic surgery suggested modest improvement in all outcomes over time. Our observations suggest that endonasal endoscopy is a safe alternative to craniotomy and may be preferred for certain tumor types. However, endoscopic surgery is associated with higher rates of CSF leak, and possibly increased recurrence rates. Prospective study with long-term follow-up is required to verify these preliminary observations.

  2. 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.

  3. Indications of Carbon Ion Therapy at CNAO

    Science.gov (United States)

    Orecchia, Roberto; Rossi, Sandro; Fossati, Piero

    2009-03-01

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

  4. Encouraging Early Clinical Outcomes With Helical Tomotherapy-Based Image-Guided Intensity-Modulated Radiation Therapy for Residual, Recurrent, and/or Progressive Benign/Low-Grade Intracranial Tumors: A Comprehensive Evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, Tejpal, E-mail: tejpalgupta@rediffmail.com [Department of Radiation Oncology, ACTREC/TMH, Tata Memorial Centre, Kharghar, Navi Mumbai (India); Wadasadawala, Tabassum; Master, Zubin; Phurailatpam, Reena; Pai-Shetty, Rajershi; Jalali, Rakesh [Department of Radiation Oncology, ACTREC/TMH, Tata Memorial Centre, Kharghar, Navi Mumbai (India)

    2012-02-01

    Purpose: To report early clinical outcomes of helical tomotherapy (HT)-based image-guided intensity-modulated radiation therapy (IMRT) in brain tumors of varying shape, size, and location. Materials and Methods: Patients with residual, recurrent, and/or progressive low-grade intracranial and skull-base tumors were treated on a prospective protocol of HT-based IMRT and followed clinicoradiologically. Standardized metrics were used for plan evaluation and outcome analysis. Results: Twenty-seven patients with 30 lesions were treated to a median radiotherapy dose of 54 Gy in 30 fractions. All HT plans resulted in excellent target volume coverage with steep dose-gradients. The mean (standard deviation) dose homogeneity index and conformity index was 0.07 (0.05) and 0.71 (0.08) respectively. At first response assessment, 20 of 30 lesions were stable, whereas 9 showed partial regression. One patient with a recurrent clival chordoma though neurologically stable showed imaging-defined progression, whereas another patient with stable disease on serial imaging had sustained neurologic worsening. With a median follow-up of 19 months (interquartile range, 11-26 months), the 2-year clinicoradiological progression-free survival and overall survival was 93.3% and 100% respectively. Conclusions: Careful selection of radiotherapy technique is warranted for benign/low-grade brain tumors to achieve durable local control with minimum long-term morbidity. Large or complex-shaped tumors benefit most from IMRT. Our early clinical experience of HT-based IMRT for brain tumors has been encouraging.

  5. Dosimetric comparisons of carbon ion treatment plans for 1D and 2D ripple filters with variable thicknesses.

    Science.gov (United States)

    Ringbæk, Toke Printz; Weber, Uli; Santiago, Alina; Simeonov, Yuri; Fritz, Peter; Krämer, Michael; Wittig, Andrea; Bassler, Niels; Engenhart-Cabillic, Rita; Zink, Klemens

    2016-06-01

    A ripple filter (RiFi)-also called mini-ridge filter-is a passive energy modulator used in particle beam treatments that broadens the Bragg peak (BP) as a function of its maximum thickness. The number of different energies requested from the accelerator can thus be reduced, which significantly reduces the treatment time. A new second generation RiFi with 2D groove shapes was developed using rapid prototyping, which optimizes the beam-modulating material and enables RiFi thicknesses of up to 6 mm. Carbon ion treatment plans were calculated using the standard 1D 3 mm thick RiFi and the new 4 and 6 mm 2D RiFis for spherical planning target volumes (PTVs) in water, eight stage I non-small cell lung cancer cases, four skull base chordoma cases and three prostate cancer cases. TRiP98 was used for treatment planning with facility-specific base data calculated with the Monte Carlo code SHIELD-HIT12A. Dose-volume-histograms, spatial dose distributions and dosimetric indexes were used for plan evaluation. Plan homogeneity and conformity of thinner RiFis were slightly superior to thicker RiFis but satisfactory results were obtained for all RiFis investigated. For the 6 mm RiFi, fine structures in the dose distribution caused by the larger energy steps were observed at the PTV edges, in particular for superficial and/or very small PTVs but performances for all RiFis increased with penetration depth due to straggling and scattering effects. Plans with the new RiFi design yielded for the studied cases comparable dosimetric results to the standard RiFi while the 4 and 6 mm RiFis lowered the irradiation time by 25-30% and 45-49%, respectively.

  6. 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.

  7. 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

  8. Risk factors for postoperative cerebrospinal fluid leak and meningitis after expanded endoscopic endonasal surgery.

    Science.gov (United States)

    Ivan, Michael E; Iorgulescu, J Bryan; El-Sayed, Ivan; McDermott, Michael W; Parsa, Andrew T; Pletcher, Steven D; Jahangiri, Arman; Wagner, Jeffrey; Aghi, Manish K

    2015-01-01

    Postoperative cerebrospinal fluid (CSF) leak is a serious complication of transsphenoidal surgery, which can lead to meningitis and often requires reparative surgery. We sought to identify preoperative risk factors for CSF leaks and meningitis. We reviewed 98 consecutive expanded endoscopic endonasal surgeries performed from 2008-2012 and analyzed preoperative comorbidities, intraoperative techniques, and postoperative care. Univariate and multivariate analyses were performed. The most common pathologies addressed included pituitary adenoma, Rathke cyst, chordoma, esthesioneuroblastoma, meningioma, nasopharyngeal carcinoma, and squamous cell carcinoma. There were 11 CSF leaks (11%) and 10 central nervous system (CNS) infections (10%). Univariate and multivariate analysis of preoperative risk factors showed that patients with non-ideal body mass index (BMI) were associated with higher rate of postoperative CSF leak and meningitis (both p<0.01). Also, patients with increasing age were associated with increased CSF leak (p = 0.03) and the length of time a lumbar drain was used postoperatively was associated with infection in a univariate analysis. In addition, three of three endoscopic transsphenoidal surgeries combined with open cranial surgery had a postoperative CSF leak and CNS infection rate which was a considerably higher rate than for transsphenoidal surgeries alone or surgeries staged with open cases (p<0.01 and p=0.04, respectively) In this series of expanded endoscopic transsphenoidal surgeries, preoperative BMI remains the most important preoperative predictor for CSF leak and infection. Other risk factors include age, intraoperative CSF leak, lumbar drain duration, and cranial combined cases. Risks associated with complex surgical resections when combining open and endoscopic approaches could be minimized by staging these procedures.

  9. 内分泌腺来源的血管内皮生长因子在脊柱肿瘤组织中的表达%Study of the expression of endocrine gland-derived vascular endothelial growth factor in the spinal cord tumors

    Institute of Scientific and Technical Information of China (English)

    陈学武; 杨评山; 潘光杰

    2015-01-01

    Objective To explore the relationship of spinal tumors and endocrine gland-derived vascular endothelial growth factor( EG-VEGF) .Methods 77 cases of spinal tumors and 17 cases of vertebral compression fractures were collected.The testing group were grouped according to the pathological type of spinal tumor,the observ-ing group was consisted of vertebral compression fracture cases.The samples of various types of tumors in patients were collected during operation and the positive rate and expression of EG-VEGF mRNA were detected by RT-PCR technique,and the results of each group were statistical analysed.Results The results of relative expression of EG-VEGF mRNA in the unknown primary spinal metastases group,osteosarcoma group and chordoma group were higher, there were no statistically significant difference compared between three groups(all P>0.05).Relative expressions of that in osteoid osteoma group and giant cell tumor group were lower,so was in the observation group,there were no sig-nificant differences in three groups ( all P>0.05);The relative expressions of EG-VEGF mRNA in CUP group, osteosarcoma group and chordoma group were significantly higher than the osteoid osteoma group,with statistical sig-nificance[(0.81 ±0.21),(0.84 ±0.22),(0.79 ±0.41)vs(0.53 ±0.19),t=0.86,0.82,0.81,all P<0.05]. Similar results were also found in EG -VEGF mRNA positive expression rate in three groups.Conclusion EG-VEGF in normal spine spinal vertebrae and primary benign tumor ( osteoid osteoma,giant cell tumor) showed lower expression,which consistent with its tissue specificity,but in primary malignant spinal cancer ( osteosarcoma, chordoma) and unknown primary tumor spinal metastases showed the higher expression, indicating that it may be a measure of an important indicator of spinal cancer, and may play a key role in early diagnosis and treatment of cancer of the spine in the future.%目的:探索脊柱肿瘤与内分泌腺来源的血管内皮生长因子( EG-VEGF)的

  10. 斜坡异位泌乳素垂体腺瘤 1 例临床分析并文献复习%Clinical analysis of one patient with ectopic prolactinoma in clivus and review of literatures

    Institute of Scientific and Technical Information of China (English)

    穆林森; 张红波; 陈谦学; 桂松柏; 曾小君; 白吉伟; 李储忠; 赵鹏; 孙彦辉

    2016-01-01

    Objective To explore the diagnosis , differential diagnosis and treatment of ectopic prolactinoma in clivus .Methods The clinical data of one patient with ectopic pituitary adenoma ( EPA) in clivus underwent surgery and pathology results was retrospectively analyzed ,and the clinical features with literature review were summarized .Results The cases was diagnosed as hyperprolactinemia ,MRI showed clivus mass and preoperative diagnosis was chordoma .The tumors were totally resected through transendoscopic single nostril transsphenoidal approach .Pathological immunohistochemical test showed prolactin positive , the hyperprolactinemia not returned to normal after surgery , oral bromocriptine treatment to control it , follow-up of 1 year without recurrence . Conclusions Ectopic clivus pituitary adenoma was rarely in clinic and easily be misdiagnosed .We should continue to adopt a comprehensive treatment based on surgery .%目的 探讨斜坡异位泌乳素垂体腺瘤的诊断与鉴别诊断及治疗方法. 方法 回顾性分析1例经手术及术后病理检查确诊的斜坡异位垂体腺瘤患者的临床资料,并结合文献总结其临床特点. 结果 该例患者表现为高泌乳素血症,MRI检查发现斜坡病变,术前诊断为脊索瘤. 采用内镜下单鼻孔经鼻蝶入路显微手术全切除肿瘤. 肿瘤病理免疫组化检查泌乳素阳性,术后未完全降至正常,口服溴隐亭治疗,随访1年无复发. 结论 斜坡异位垂体腺瘤临床罕见,误诊率高;仍应采用以手术为主的综合治疗.

  11. Use of recombinant human bone morphogenetic protein-2 as an adjunct for instrumented posterior arthrodesis in the occipital cervical region: An analysis of safety, efficacy, and dosing

    Directory of Open Access Journals (Sweden)

    D Kojo Hamilton

    2010-01-01

    Full Text Available Background: There have been few reports on the use of recombinant human bone morphogenetic protein (rhBMP-2 in posterior spine. However, no study has investigated the dosing, safety, and efficacy of its use in the posterior atlantoaxial, and/or craniovertebral junction. Recent case report of the cytokine-mediated inflammatory reaction, following off label use of rhBMP-2 as an adjunct for cervical fusion, particularly in complex cases, has increased concern about complications associated with the product. Objective: To assess the safety, efficacy, and dosing of rhBMP-2 as an adjunct for instrumented posterior atlantoaxial and/or craniovertebral junction arthrodesis. Materials and Methods: We included all patients treated by the senior author that included posterior atlantoaxial and/or craniovertebral junction instrumented fusion using rhBMP-2 from 2003 to 2008 with a minimum two year follow-up. Diagnosis, levels fused, rhBMP-2 dose, complications, and fusion were assessed. Results: Twenty three patients with a mean age of 60.9 years (range 4 - 89 years and an average follow-up of 45 months (range 27 to 84 months met inclusion criteria. The indications for surgery included, atlantoaxial instability (n = 16, basilar invagination (n = 6, and kyphoscoliosis (n = 1. The specific pathologic diagnosis included type 2 dens fracture (n = 7, complex C1 and C2 ring fracture (n = 2, chordoma (n = 2, degenerative/osteoporosis (n = 3, rheumatoid disease (n = 8, and pseudogout (n = 1. The average rhBMP-2 dose was 2.38 mg/level, with a total of 76 levels treated (average 3.3 levels, SD= 1.4 levels. There were no complications. During the most recent follow-up, all patients had achieved fusion. Conclusions: In a series of patients with complex pathology and/or rheumatoid arthritis, 100% fusion rate was achieved with adjunct use of rhBMP-2, with a safe and effective average rhBMP-2 dose of 2.38 mg per level.

  12. Brain Tumors and Brain Tumor Research Progress in Image Classification%脑肿瘤及脑肿瘤图像分类的研究进展

    Institute of Scientific and Technical Information of China (English)

    俞海平; 邬立保

    2011-01-01

    Many methods of brain tumor classification,there is no uniform classification^! A variety of tumors and pathological features of the different tissue, the study of benign and malignant, and things are not the same characteristics. Usually can be classified as histological.-(l) Originated in glial tumors: astrocytoma, less support glial cell tumors, medulloblastoma, etc.(2) Originated in meningeal tumors: meningioma, meningeal sarcoma, arachnoid cyst.(3) Originated in the pituitary tumors: tired color cell adenoma, acidophilic, basophilic cell adenoma.(4) Originated in cranial nerve tumors: acoustic neuroma, trigeminal nerve sheath tumors and other tumors.(S) Originated from residual embryonic tissue: craniopharyngioma, chordoma, dermoid cyst (6) Originated in vascular cells: vascular tumors and vascular reticular cell tumor, etc.(7) Transfer or by other parts of the tumor invasion: a variety of metastatic tumors, and nasopharyn-geal carcinoma, etc.%脑肿瘤分类的方法很多,目前尚无统一的分类方法,并且各种肿瘤的组织发生与病理特征不同,其良性与恶性以及物学特性也不一样.通常按组织学可分类如下:(1)发源于神经胶质的肿瘤:星形细胞瘤、少支胶质细胞瘤、髓母细胞瘤等.(2)发源于脑膜的肿瘤:脑膜瘤、脑膜内瘤、蛛网膜囊肿等.(3)发源于垂体的肿瘤:厌色细胞腺瘤,嗜酸、嗜碱性细胞腺瘤.(4)发源于颅神经的肿瘤:听神经瘤、三叉神经瘤等各种神经鞘瘤.(5)发源于胚胎残余组织:颅咽管瘤、脊索瘤、皮样囊肿等.(6)发源于血管细胞:血管瘤及血管网织细胞瘤等.(7)由其它部位转移或侵入的肿瘤:各种转移瘤及鼻咽癌等.

  13. Helical tomotherapy. Experiences of the first 150 patients in Heidelberg

    Energy Technology Data Exchange (ETDEWEB)

    Sterzing, F.; Schubert, K.; Sroka-Perez, G.; Kalz, J.; Debus, J.; Herfarth, K. [Dept. of Radiation Oncology, Univ. of Heidelberg (Germany)

    2008-01-15

    Background and purpose: helical tomotherapy was introduced into clinical routine at the Department of Radiation Oncology, University Hospital of Heidelberg, Germany, in July 2006. This report is intended to describe the experience with the first 150 patients treated with helical tomotherapy. Patient selection, time effort, handling of daily image guidance with megavoltage (MV) CT, and quality of radiation plans shall be assessed. Patients and methods: between July 2006 and May 2007, 150 patients were treated with helical tomotherapy in the University Hospital of Heidelberg. Mean age was 60 years with a minimum of 30 years and a maximum of 85 years. 79 of these patients received radiotherapy as a part of multimodal treatment pre- or postoperatively, 17 patients received treatment as a combined radiochemotherapy. 76% were treated with curative intent. Radiotherapy sites were central nervous system (n = 7), head and neck (n = 28), thoracic (n = 37), abdominal (n = 58) and skeletal system (n = 20). Most common tumor entities were prostate cancer (n = 28), breast cancer (n = 17), gastrointestinal tumors (n = 19), pharyngeal carcinoma (n = 14), lymphoma (n = 13), metastatic disease (bone n = 14, liver n = 6, lung n = 4, lymph node n = 2), sarcoma (n = 8), malignant pleural mesothelioma (n = 5), ovarian cancer treated with whole abdominal irradiation (n = 4), lung cancer (n = 3), skin malignancies (n = 3), chordoma (n = 2), meningioma (n = 2), one ependymoma and one medulloblastoma treated with craniospinal axis irradiation (n = 2), and others (n = 4). Nine patients were treated with single-fraction radiosurgery, nine with image-guided spinal reirradiation, and twelve patients were treated at multiple targets simultaneously. A pretreatment MV-CT scan was performed in 98.2% of the 3,026 fractions applied. After matching with the kilovoltage planning CT, corrections for translations and rotation around longitudinal axis (roll) were done. Results: mean time on table was 24

  14. Correction of patient positioning errors based on in-line cone beam CTs: clinical implementation and first experiences

    Directory of Open Access Journals (Sweden)

    Häring Peter

    2006-05-01

    Full Text Available Abstract Background The purpose of the study was the clinical implementation of a kV cone beam CT (CBCT for setup correction in radiotherapy. Patients and methods For evaluation of the setup correction workflow, six tumor patients (lung cancer, sacral chordoma, head-and-neck and paraspinal tumor, and two prostate cancer patients were selected. All patients were treated with fractionated stereotactic radiotherapy, five of them with intensity modulated radiotherapy (IMRT. For patient fixation, a scotch cast body frame or a vacuum pillow, each in combination with a scotch cast head mask, were used. The imaging equipment, consisting of an x-ray tube and a flat panel imager (FPI, was attached to a Siemens linear accelerator according to the in-line approach, i.e. with the imaging beam mounted opposite to the treatment beam sharing the same isocenter. For dose delivery, the treatment beam has to traverse the FPI which is mounted in the accessory tray below the multi-leaf collimator. For each patient, a predefined number of imaging projections over a range of at least 200 degrees were acquired. The fast reconstruction of the 3D-CBCT dataset was done with an implementation of the Feldkamp-David-Kress (FDK algorithm. For the registration of the treatment planning CT with the acquired CBCT, an automatic mutual information matcher and manual matching was used. Results and discussion Bony landmarks were easily detected and the table shifts for correction of setup deviations could be automatically calculated in all cases. The image quality was sufficient for a visual comparison of the desired target point with the isocenter visible on the CBCT. Soft tissue contrast was problematic for the prostate of an obese patient, but good in the lung tumor case. The detected maximum setup deviation was 3 mm for patients fixated with the body frame, and 6 mm for patients positioned in the vacuum pillow. Using an action level of 2 mm translational error, a target point

  15. The 100 most-cited articles in spinal oncology.

    Science.gov (United States)

    De la Garza-Ramos, Rafael; Benvenutti-Regato, Mario; Caro-Osorio, Enrique

    2016-05-01

    OBJECTIVE The authors' objective was to identify the 100 most-cited research articles in the field of spinal oncology. METHODS The Thomson Reuters Web of Science service was queried for the years 1864-2015 without language restrictions. Articles were sorted in descending order of the number of times they were cited by other studies, and all titles and abstracts were screened to identify the research areas of the top 100 articles. Levels of evidence were assigned on the basis of the North American Spine Society criteria. RESULTS The authors identified the 100 most-cited articles in spinal oncology, which collectively had been cited 20,771 times at the time of this writing. The oldest article on this top 100 list had been published in 1931, and the most recent in 2008; the most prolific decade was the 1990s, with 34 articles on this list having been published during that period. There were 4 studies with Level I evidence, 3 with Level II evidence, 9 with Level III evidence, 70 with Level IV evidence, and 2 with Level V evidence; levels of evidence were not assigned to 12 studies because they were not on therapeutic, prognostic, or diagnostic topics. Thirty-one unique journals contributed to the 100 articles, with the Journal of Neurosurgery contributing most of the articles (n = 25). The specialties covered included neurosurgery, orthopedic surgery, neurology, radiation oncology, and pathology. Sixty-seven articles reported clinical outcomes. The most common country of article origin was the United States (n = 62), followed by Canada (n = 8) and France (n = 7). The most common topics were spinal metastases (n = 35), intramedullary tumors (n = 18), chordoma (n = 17), intradural tumors (n = 7), vertebroplasty/kyphoplasty (n = 7), primary bone tumors (n = 6), and others (n = 10). One researcher had authored 6 studies on the top 100 list, and 7 authors had 3 studies each on this list. CONCLUSIONS This study identified the 100 most-cited research articles in the area of

  16. Prospects of the standardized treatment and research of neoplasms%骨肿瘤规范化治疗与研究展望

    Institute of Scientific and Technical Information of China (English)

    牛晓辉; 刘巍峰

    2014-01-01

    With the introduction of chemotherapy, the improvement of surgical techniques and the advocation of comprehensive and standardized treatment, great progresses have been made in the treatment of bone and soft tissue tumors. Comprehensive treatment strategy is an ideal choice for common primary bone and soft tissue tumors with surgery as the main therapy and chemoradiotherapy as the adjuvant therapy. The standardized treatment is regarded as the best strategy. The histological reaction in the neoadjuvant chemotherapy is taken as the principal method to predict the survival rate. For the patients with osteosarcoma, surgery and chemotherapy are included in the standardized treatment. It is an unclear concept of“unresectable tumors”. The tumor that can be resected by a surgeon may be unresectable for another, and it depends on how much the patient could accept the adverse effects on his or her functions. The prognosis could be predicted by the high expressions of the interferonα/β. How many metastatic lesions are there? Is it possible to resect all these lesions completely? All the factors may affect the prognosis. The wide resection combined with the preoperative chemotherapy is recommended in the treatment of resectable osteosarcoma in its progressive stage. As to the patients with unresectable metastatic tumors, the chemotherapy is a good choice. The primary tumors are evaluated, and then a suitable approach is chosen to control the local condition. The radiotherapy is the main treatment method for the patients with tumors that could not be resected by surgery. The dedifferentiated chordoma is treated according to the National Comprehensive Cancer Network ( NCCN ) guideline. It is better for the patients with metastatic giant cell tumors to undergo the treatment of intracapsular resection. Non-surgical treatment or radiotherapy is recommended for the patients with unresectable tumors. The radiotherapy can be taken as an adjutant therapy in the comprehensive

  17. 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

  18. Small bowel toxicity after high dose spot scanning-based proton beam therapy for paraspinal/retroperitoneal neoplasms

    Energy Technology Data Exchange (ETDEWEB)

    Schneider, R.A.; Albertini, F.; Koch, T.; Ares, C.; Lomax, A.; Goitein, G. [Paul Scherrer Institute PSI, Villigen (Switzerland). Center for Proton Therapy; Vitolo, V. [Fondazione CNAO, Pavia (Italy); Hug, E.B. [Paul Scherrer Institute PSI, Villigen (Switzerland). Center for Proton Therapy; ProCure Proton Therapy Centers, New York, NY (United States)

    2013-12-15

    Purpose: Mesenchymal tumours require high-dose radiation therapy (RT). Small bowel (SB) dose constraints have historically limited dose delivery to paraspinal and retroperitoneal targets. This retrospective study correlated SB dose-volume histograms with side-effects after proton radiation therapy (PT). Patients and methods: Between 1997 and 2008, 31 patients (mean age 52.1 years) underwent spot scanning-based PT for paraspinal/retroperitoneal chordomas (81 %), sarcomas (16 %) and meningiom (3 %). Mean total prescribed dose was 72.3 Gy (relative biologic effectiveness, RBE) delivered in 1.8-2 Gy (RBE) fractions. Mean follow-up was 3.8 years. Based on the pretreatment planning CT, SB dose distributions were reanalysed. Results: Planning target volume (PTV) was defined as gross tumour volume (GTV) plus 5-7 mm margins. Mean PTV was 560.22 cm{sup 3}. A mean of 93.2 % of the PTV was covered by at least 90 % of the prescribed dose. SB volumes (cm{sup 3}) receiving doses of 5, 20, 30, 40, 50, 60, 70, 75 and 80 Gy (RBE) were calculated to give V5, V20, V30, V40, V50, V60, V70, V75 and V80 respectively. In 7/31 patients, PT was accomplished without any significant SB irradiation (V5 = 0). In 24/31 patients, mean maximum dose (Dmax) to SB was 64.1 Gy (RBE). Despite target doses of > 70 Gy (RBE), SB received > 50 and > 60 Gy (RBE) in only 61 and 54 % of patients, respectively. Mean SB volumes (cm{sup 3}) covered by different dose levels (Gy, RBE) were: V20 (n = 24): 45.1, V50 (n = 19): 17.7, V60 (n = 17): 7.6 and V70 (n = 12): 2.4. No acute toxicity {>=} grade 2 or late SB sequelae were observed. Conclusion: Small noncircumferential volumes of SB tolerated doses in excess of 60 Gy (RBE) without any clinically-significant late adverse effects. This small retrospective study has limited statistical power but encourages further efforts with higher patient numbers to define and establish high-dose threshold models for SB toxicity in modern radiation oncology. (orig.)

  19. [Operative neurosurgery: personal view and historical backgrounds (8) suboccipital craniotomy-sitting position-linear incision].

    Science.gov (United States)

    Yonekawa, Yasuhiro

    2011-08-01

    Suboccipital craniotomy (SOC) can be classified into three types: midline, paramedian and lateral according to the site of linear incision. They are subdivided horizontally into cranial, intermediate and caudal, while the latter of the lateral SOC should be included into the paramedian caudal one (Fig. 1, 19). Sitting position for the craniotomy has several advantages over other positionings in spite of several known drawbacks especially air embolism: cleanliness of the operative field, good anatomical orientation, wider operative spaces obtained by gravitational downward displacement of the cerebellar hemisphere above all. Linear incision is considered to have no definite drawbacks as compared with other incisions such as the horse shoe or the hockey-stick incision and rather have advantages such as enabling effective access to the surgical target by the use of navigation, simpleness of craniotomy in the opening and the closure, and less pseudomeningocele complication. Although cranial and intermediate lateral SOCs are mainly for lesions in the upper and middle CP angle such as acoustic neurinomas or meningioma besides MVD for trigeminal neuralgia, these are applied also for cavernomas of the tectal and cerebellar peduncle, and meningiomas or chordomas of the upper and middle 1/3 of the petroclival region (Fig. 2-5). Importance of the SCTTA by cranial paramedian SOC for the management of lesions in the temporoposteromedial region including the tentorium and its incisura was emphasized and peduncular lesions at the lamina tecti and pons as well. Caudal paramedian SOC is appropriate for lesions in the lower CP angle along with MVD for hemifacial spasm and is furthermore applicable for foramen magnum meningiomas or lower clivus meningiomas by TVDRA (Fig. 6-13). Cranial midline SOC (paraculminar approach) is applicable for tumors of pineal regions and for lesions at the midbrain, thalamus, posterior part of the IIIrd ventricle. The TFUTA by lower midline SOC enables

  20. Impact of tissue specific parameters on the predition of the biological effectiveness for treatment planning in ion beam therapy

    International Nuclear Information System (INIS)

    Treatment planning in ion beam therapy requires a reliable estimation of the relative biological effectiveness (RBE) of the irradiated tissue. For the pilot project at GSI Helmholtzzentrum fuer Schwerionenforschung GmbH and at other European ion beam therapy centers RBE prediction is based on a biophysical model, the Local Effect Model (LEM). The model version in use, LEM I, is optimized to give a reliable estimation of RBE in the target volume for carbon ion irradiation. However, systematic deviations are observed for the entrance channel of carbon ions and in general for lighter ions. Thus, the LEM has been continuously developed to improve accuracy. The recent version LEM IV has proven to better describe in-vitro cell experiments. Thus, for the clinical application of LEM IV it is of interest to analyze potential differences compared to LEM I under treatment-like conditions. The systematic analysis presented in this work is aiming at the comparison of RBE-weighted doses resulting from different approaches and model versions for protons and carbon ions. This will facilitate the assessment of consequences for clinical application and the interpretation of clinical results from different institutions. In the course of this thesis it has been shown that the RBE-weighted doses predicted on the basis of LEM IV for typical situations representing chordoma treatments differ on average by less than 10 % to those based on LEM I and thus also allow a consistent interpretation of the clinical results. At Japanese ion beam therapy centers the RBE is estimated using their clinical experience from neutron therapy in combination with in-vitro measurements for carbon ions (HIMAC approach). The methods presented in this work allow direct comparison of the HIMAC approach and the LEM and thus of the clinical results obtained at Japanese and European ion beam therapy centers. Furthermore, the sensitivity of the RBE on the model parameters was evaluated. Among all parameters the

  1. LEARNING CURVE IN ENDOSCOPIC TRANSNASAL SELLAR REGION SURGERY

    Directory of Open Access Journals (Sweden)

    Ananth G

    2016-07-01

    Full Text Available BACKGROUND The endoscopic endonasal approach for the sellar region lesions is a novel technique and an effective surgical option. The evidence thus far has been conflicting with reports in favour and against a learning curve. We attempt to determine the learning curve associated with this approach. METHODS Retrospective and prospective data of the patients who were surgically treated for sellar region lesions between the year 2013 and 2016 was collected, 32 patients were operated by the endoscopic endonasal approach at Vydehi Institute of Medical Sciences and Research Centre, Bangalore. Age, sex, presenting symptoms, length of hospital stay, surgical approach, type of dissection, duration of surgery, sellar floor repair, intraoperative and postoperative complications were noted. All the procedures were performed by a single neurosurgeon. RESULTS A total of 32 patients were operated amongst which 21 patients were non-functioning pituitary adenomas, 2 were growth hormone secreting functional adenomas, 1 was an invasive pituitary adenoma, 4 were craniopharyngiomas, 2 were meningiomas, 1 was Rathke’s cleft cyst and 1 was a clival chordoma. Headache was the mode of presentation in 12 patients, 12 patients had visual deficits, 6 patients presented with hormonal disturbances amongst which 4 patients presented with features of panhypopituitarism and 2 with acromegaly. Amongst the 4 patients with panhypopituitarism, 2 also had DI, two patients presented with CSF rhinorrhoea. There was a 100% improvement in the patients who presented with visual symptoms. Gross total resection was achieved in all 4 cases of craniopharyngiomas and 13 cases of pituitary adenomas. Postoperative CSF leak was seen in 4 patients who underwent re-exploration and sellar floor repair, 9 patients had postoperative Diabetes Insipidus (DI which was transient, the incidence of DI reduced towards the end of the study. There was a 25% decrease in the operating time towards the end of

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

    Directory of Open Access Journals (Sweden)

    I. Zubillaga Rodriguez

    2006-12-01

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

  3. Tratamento de dor em paciente com tumor sacral inoperável: relato de caso Tratamiento de dolor en paciente con tumor sacral inoperable: relato de caso Management of pain in patient with inoperable sacral tumor: case report

    Directory of Open Access Journals (Sweden)

    Juliano Rodrigues Gasparini

    2004-04-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: Diversas técnicas podem ser utilizadas no controle de dor em pacientes oncológicos. O objetivo deste relato é mostrar o uso de algumas medidas terapêuticas empregadas para tratar um paciente com quadro doloroso importante de difícil controle. RELATO DO CASO: Paciente do sexo masculino, 70 anos, com cordoma sacral e com possibilidade terapêutica muito difícil. Apresentava quadro de dor importante associado ao tumor. São relatadas diversas técnicas utilizadas no seu tratamento e os resultados obtidos. CONCLUSÕES: Os bloqueios neurolíticos, para o controle da dor em paciente com tumores cuja possibilidade terapêutica é difícil, constituem técnica eficaz quando bem indicados e realizados dentro de critérios estabelecidos.JUSTIFICATIVA Y OBJETIVOS: Diversas técnicas pueden ser utilizadas en el control de dolor en pacientes oncológicos. El objetivo de este relato es mostrar el uso de algunas medidas terapéuticas utilizadas para tratar un paciente con cuadro doloroso importante de difícil control. RELATO DE CASO: Paciente masculino, 70 años, con cordoma sacral y con posibilidad terapéutica muy difícil. Presentaba cuadro de dolor importante asociado al tumor. Se relatan diversas técnicas utilizadas en su tratamiento y los resultados obtenidos. CONCLUSIONES: Los bloqueos neurolíticos, para el control del dolor en paciente con tumores cuja posibilidad terapéutica es difícil, constituyen técnica eficaz cuando bien indicados y realizados dentro de criterios establecidos.BACKGROUND AND OBJECTIVES: Different techniques may be used to control cancer pain. This report aimed at describing some therapeutic measures used to treat a patient with a severe and bad responsive pain. CASE REPORT: A 70-year-old male patient a sacral chordoma of very difficult therapeutic. He referred major tumor-associated pain. Several techniques used to treat pain of this patient and results obtained are reported. CONCLUSIONS

  4. SU-E-P-06: A Novel Hybrid Planning Approach to Allow More Patients Benefited by the Intensity Modulated Proton Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Jiang, S [UT MD Anderson Cancer Center, Tianjin Medical University Cancer Instit, Houston, TX (United States); Liao, L [UT MD Anderson Cancer Center, University of Houston, Houston, TX (United States); Li, Y [Varian Medical Systems, Houston, TX (United States); Wang, X; Sahoo, N; Liao, Z; Grosshans, D; Frank, S [MD Anderson Cancer Center, Houston, TX (United States); Li, H [M.D. Anderson Cancer Center, Houston, TX (United States); Zhu, X; Chang, J; Zhang, X [UT MD Anderson Cancer Center, Houston, TX (United States); Gillin, M [MD Anderson Cancer Ctr., Houston, TX (United States); Hojo, Y [UT MD Anderson cancer center, Houston, TX (United States); Sun, J [UT MD Anderson cancer center, Tianjin Medical University Cancer Institute a, Houston, TX (United States)

    2014-06-01

    Purpose: We report a hybrid scattering and scanning beam delivery approach, termed as (HimpsPT), which demonstrated that majority IMPT delivery can be potentially replaced with hybrid IMPT and PSPT delivery with similar or better plan quality. Methods: Three representative clinical cases, including head and neck (HN), skull base chordoma (CNS) and lung cancer, treated in MDACC Proton Therapy Center with IMPT were retrospectively redesigned using HimpsPT. The PSPT plans are designed with the same prescriptions as those of IMPT plans. The whole treatment can be delivered by either alternating or sequential PSPT and IMPT delivery. The dosimetric data and dose distributions of HimpsPT plans are compared with those of IMPT plans. We also performed a worst-case robust analysis for all plans. Results: The target coverages for all cases are comparable. For the HN case, the mean dose of esophagus larynx, left parotid and right submandibular, oral cavity V20, the max dose of cord is 18.0, 36.1, 23.6, 47.2, 0.1, 31.7 Gy in HimpsPT plan, and 25.5, 33.8, 24.9, 49.1, 0.2, 33.8 Gy in IMPT plan. For the lung case, the lung V5, V20, V30, mean lung dose, heart V40, esophagus V70, cord maximum dose are 50.5%, 37.0%, 31.7%, 21.3 Gy, 7.2%, 4.9%, 35.5 Gy in HimpsPT plan, and 55.4%, 36.7%, 30.1%, 21.3 Gy, 7.7%, 8.4%, 36.8Gy in IMPT plans. For the CNS case, brainstem maximum dose is 50.5 Gy in HimpsPT plan and 55.4 Gy in IMPT plan due to sharp penumbra offered by the aperture of the PSPT plan in HimpsPT technique. Conclusion: For majority disease sites, the dosimetric advantage of IMPT technique can be achieved by the hybrid PSPT and IMPT technique, which enables the centers equipped with both scattering and scanning beam facilities to treat more patients which can be benefited by the scanning beam.

  5. Trombose venosa profunda e sua relação com trombofilias e neoplasias: estudo retrospectivo Deep venous thrombosis and its relationship with thrombophilias and malignancies: retrospective study

    Directory of Open Access Journals (Sweden)

    Ana Vieira Baptista

    2012-09-01

    through the review of the clinical files of all patients aged 50 or less hospitalized in our Department between January 1st 2006 and December 31st 2010 with that diagnosis, and the results obtained in the protocols were analysed. Results: From a total of 89 patients, 64 (71,9% were females and 25 (28,1% were males, and the mean age was 33,3 ±10,0 years. 14,6% of the patients had already had, at least, one previous episode of deep venous thrombosis and 78,7% had, at least, one risk factor. The protocol for screening for thrombophilia and malignancy was applied to 65 patients (73,0%, being detected congenital thrombophilias in 41 patients (63,1%, two cases of antiphospholipid antibody syndrome (APS, one case of monoclonal gammopathy, one of lung cancer and one of sacral chordoma. 49 of the patients subjected to the protocols (75,4% were referred to the Hematology Clinic for evaluation and in 40 of those patients (81,6% was suggested keeping oral anticoagulation for more than 12 months. Conclusions: Given the high prevalence of genetic polimorphisms that confer a higher thrombotic risk and the not rare cases of APS and of occult malignancies present in this population, it is considered that, despiste its elevated costs, the screening for these kind of pathologies should continue to be done.

  6. Translocación del tercio medio facial en un paciente en edad infantil: Fijación con un nuevo sistema de placas y pines reabsorbibles Mid-facial translocation in children using a new system of absorbable plates and pins: A case report

    Directory of Open Access Journals (Sweden)

    Manel Coll-Anglada

    2010-12-01

    morbidity of the procedure. Absorbable osteosynthesis material has been marketed since the 1980s as a solution to this problem. We report the case of a 13-year-old female patient diagnosed of chordoma of the clivus. A bilateral mid-facial approach was used with a new system of absorbable plates and pins affixed ultrasonically (SonicWeld®. KLS Martin, LP, Jacksonville, Florida, USA. The primary approaches to tumors located in the deep facial regions and skull base, the main variations of the mid-facial translocation technique, application of the new SonicWeld® absorbable system, and the main differences compared to traditional absorbable plates and screws are reviewed.

  7. 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

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

    Institute of Scientific and Technical Information of China (English)

    齐波; 丁晶; 徐永清; 阮默; 林月秋

    2014-01-01

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

  9. Assessment of potential advantages of relevant ions for particle therapy: A model based study

    Energy Technology Data Exchange (ETDEWEB)

    Grün, Rebecca, E-mail: r.gruen@gsi.de [Department of Biophysics, GSI Helmholtzzentrum für Schwerionenforschung, Darmstadt 64291 (Germany); Institute of Medical Physics and Radiation Protection, University of Applied Sciences Gießen, Gießen 35390 (Germany); Medical Faculty of Philipps-University Marburg, Marburg 35032 (Germany); Friedrich, Thomas; Krämer, Michael; Scholz, Michael [Department of Biophysics, GSI Helmholtzzentrum für Schwerionenforschung, Darmstadt 64291 (Germany); Zink, Klemens [Institute of Medical Physics and Radiation Protection, University of Applied Sciences Gießen, Gießen 35390, Germany and Department of Radiotherapy and Radiation Oncology, University Medical Center Giessen and Marburg, Marburg 35043 (Germany); Durante, Marco [Department of Biophysics, GSI Helmholtzzentrum für Schwerionenforschung, Darmstadt 64291, Germany and Department of Condensed Matter Physics, Darmstadt University of Technology, Darmstadt 64289 (Germany); Engenhart-Cabillic, Rita [Medical Faculty of Philipps-University Marburg, Marburg 35032, Germany and Department of Radiotherapy and Radiation Oncology, University Medical Center Giessen and Marburg, Marburg 35043 (Germany)

    2015-02-15

    Purpose: Different ion types offer different physical and biological advantages for therapeutic applications. The purpose of this work is to assess the advantages of the most commonly used ions in particle therapy, i.e., carbon ({sup 12}C), helium ({sup 4}He), and protons ({sup 1}H) for different treatment scenarios. Methods: A treatment planning analysis based on idealized target geometries was performed using the treatment planning software TRiP98. For the prediction of the relative biological effectiveness (RBE) that is required for biological optimization in treatment planning the local effect model (LEM IV) was used. To compare the three ion types, the peak-to-entrance ratio (PER) was determined for the physical dose (PER{sub PHY} {sub S}), the RBE (PER{sub RBE}), and the RBE-weighted dose (PER{sub BIO}) resulting for different dose-levels, field configurations, and tissue types. Further, the dose contribution to artificial organs at risk (OAR) was assessed and a comparison of the dose distribution for the different ion types was performed for a patient with chordoma of the skull base. Results: The study showed that the advantages of the ions depend on the physical and biological properties and the interplay of both. In the case of protons, the consideration of a variable RBE instead of the clinically applied generic RBE of 1.1 indicates an advantage in terms of an increased PER{sub RBE} for the analyzed configurations. Due to the fact that protons show a somewhat better PER{sub PHY} {sub S} compared to helium and carbon ions whereas helium shows a higher PER{sub RBE} compared to protons, both protons and helium ions show a similar RBE-weighted dose distribution. Carbon ions show the largest variation of the PER{sub RBE} with tissue type and a benefit for radioresistant tumor types due to their higher LET. Furthermore, in the case of a two-field irradiation, an additional gain in terms of PER{sub BIO} is observed when using an orthogonal field configuration

  10. Percutaneous biopsy of cavernous sinus tumor via the foramen ovale: a report of 2 cases%经卵圆孔穿刺海绵窦肿瘤活检(附二例报道)

    Institute of Scientific and Technical Information of China (English)

    易伟; A Tommy Bergenheim

    2010-01-01

    examination. Results The procedures were successfully performed without complications in the 2 patients. One had a chordoma and another had a meningioma. Conclusion Percutaneous biopsy via the foramen ovale is a safe and conclusive method to obtain histopathological diagnoses.

  11. Diagnosis and surgical approach of parapharyngeal space neoplasms%咽旁间隙肿瘤的诊断及手术入路选择

    Institute of Scientific and Technical Information of China (English)

    桑建中; 娄卫华; 张亚民

    2011-01-01

    Objective:To explore the diagnosis and reasonable surgical approach for parapharyngeal space neoplasms. Method:From July 2004 to July 2009,a retrospective review of 45 patients with neoplasms of parapharyngeal space was performed. Fourty-five cases were examined by CT.some of them were examined by MRI or DSA. Several surgical approaches were selected. The transcervical approach was used in 35 cases , the transparotid approach was used in 5 cases, the transmandible approach was used in 2 cases,and the transparotid and temporal approach was used in 3 cases. Result: Among 45 patients, 37 cases(82. 22%) were benign and 8 cases(17. 78%) were malignant. Neurogenic neoplasms and salivary glands neoplasms were the most common tumors. Using CT.MRI or DSA could obtain useful information about the location,size,shape,density and degree of enhancement of the parapharyngeal space neoplasms ,acquire their relationship with styloid and carotid,and make preoperative diagnosis. The diagnostic coincidence rate between preoperative diagnosis and pathological diagnosis was 80% (origin of tissue). All benign tumors were completely resected. A lymphangiomas had recurrence after 1 year, and the second operation had no recurrence. In the 45 cases,8 cases were malignant tumors. Of 2 patients with malignant mixed tumors, 1 survived disease free after a follow-up of 4 years,and another was still alive with disease after a follow-up of 3 years;Of 2 patients with synovial sarcomas, 1 survived disease free for a follow-up of 3 years, the other one survived disease free after a follow-up of 2 years si patient of nasopharyngeal carcinoma with metastasis in the parapharyngeal space had post-operative radical radiotherapy and survived disease free after a follow-up of 5 years. One patient with chordoma was still alive with disease after a follow-up of 3 years si patient with poorly differentiated squamous cell carcinoma,died of pulmonary metastasis after a 4 year follow-up ;1 case with follicular

  12. Organogenesis of the digestive system in Brachymystax lenok during early development%细鳞鲑早期发育过程中的消化系统发生

    Institute of Scientific and Technical Information of China (English)

    2013-01-01

    -scribed the development of the digestive tract in B. lenok larvae reared under culture conditions based on histo-logical observation. The larvae were held in one of three 0.3 m3circular tanks (12–16.2℃)for 185 d and fed an artificial diet. Based on the structural changes in the digestive system, we identified three distinct stages during embryonic development: (1) 0–201 h post-fertilization; (2) 202–480 h post-fertilization, and (3) 481–552 h post-fertilization. During the first stage, the embryo initiated the intestinal phase of development, the original base cells of the digestive tract were localized below the chordoma, in a single layer of flat cells. During the second stage, the original base cells of the digestive tract began to differentiate into a straight tube. During the third stage, the inner layer cells of the digestive tube proliferate, and the outer layer forms as two layers of cells. At the end of the embryonic period, the hatched larvae have an oil-rich yolk sac, a simple digestive system that resembles a blind tube, and lack of external connection in the anus. Between 2–7 days after hatch (DAH), the digestive system begins to rapidly differentiate but larvae remain entirely dependent on endogenous nutrition from the yolk sac. At 7 DAH, the digestive tract was fully differentiated into the buccopharynx, esophagus, non-glandular and glandular stomach, and anterior and posterior intestine. By 14 DAH, larvae were transitioning from being dependent on en-dogenous nutrition to being exotrophic. The yolk sac was completely absorbed. At 49 DAH, the pyloric caeca was completely differentiated, and the liver and pancreas were functional. Between 64–105 DAH, the oropharyngeal cavity and stomach developed to the adult form with 28–36 pyloric caeca finger branches. The optimization of formulated feeding during larval culture of B. lenok is most critical between 10–14 DAH. Feeding during this pe-riod could be enhanced by continuous feeding of palatable

  13. The surgical treatment of musculo- skeletal tumors in dangerous region%危险区骨骼肌肉系统肿瘤的外科治疗

    Institute of Scientific and Technical Information of China (English)

    张杏泉; 李开华; 陈刚; 刘军; 蔡平; 方仁义; 王民政; 范清宇

    2012-01-01

    目的:介绍一种安全处理危险区骨骼肌肉系统良性或低度恶性肿瘤的外科治疗方法.方法:对骶2脊索瘤先施行前路经腹结扎双侧髂内动脉,然后再切除肿瘤,可减少出血.对腹股沟区或腘窝区较大肿瘤与股、腘大血管及神经紧密粘连者,先从肿瘤远近端血管、神经正常部分解剖出血管神经,再向病变区解剖,容易完整切除肿瘤.对锁骨上区肿瘤,先截除一段锁骨,然后从肿瘤远近端正常锁骨下动、静脉及臂丛神经处,向肿瘤部游离并保护好胸膜,可较安全地切除肿瘤.而对于腓骨头、颈及其周围部肿瘤,先解剖出腓总神经及其各肌支,尽可能保留肌支.结果:11例危险区(紧邻大血管、神经区域)良性或低度恶性骨骼肌肉系统肿瘤,采用先从肿瘤远近端正常血管、神经处游离出神经、血管,再向肿瘤部解剖,均顺利解剖出大血管及神经,并完整切除肿瘤.所有病例无复发,亦无肢体功能障碍.结论:对紧邻重要血管及神经的较大良性或低度恶性骨骼肌肉系统肿瘤,先从正常段血管、神经向肿瘤部解剖游离,既可彻底切除肿瘤,又可避免损伤血管、神经引起肢体功能障碍,是一种较好方法.%Objective:To introduce a surgical treatment of the innocent or low - degree malignant musculo - skeletal tumors in dangerous region. Methods: For the sacral 2 chordoma,to perform (he ligation of the two — side internal iliac artery first through abdominal cavity and then excise the tumors in order to reduce bleeding. For the large tumors especially adhering tightly with important nerves and vessels in inguinal region or popliteal fossa, to dissect first the normal vascular nerve of tumors distantly and proximally and then disconnect the lesion for excision of tumors completely. For the tumors in supraelavicular region, to dissect the normal vascular nerve of infraclavicula and brachial plexus of tumors distantly and proximally

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

    Institute of Scientific and Technical Information of China (English)

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

    2014-01-01

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

  15. Total sacrectomy via posterior approach for malignant sacral tumors%单纯后路全骶骨切除治疗骶骨恶性肿瘤

    Institute of Scientific and Technical Information of China (English)

    孙伟; 陈泉池; 马小军; 华莹奇; 蔡郑东

    2014-01-01

    high level (S1,S2).Methods 5 cases of primary malignant sacral tumors treated by total sacrectomy via posterior approach and iliolumbar reconstruction from March 2010 to March 2011 were analyzed retrospectively.There were 3 males and 2 females.The mean age was 41,ranging from 32 to 55.The imageology examination showed osteolytic destruction,among which there were 4 cases of obvious soft tissue mass,1 case of obvious sacral foramina expansion with bone damage.MRI showed 1 case of the tumors in S1-S5,1 case of the tumors in S1 、S2,1 case of the tumors in S1-S3.The smallest tumor was 9.2 cm×7.6 cm×4.1 cm,while the largest was 22.0 cm× 19.0 cm× 16.0 cm.Preoperative TTNB were done on the 5 patients.Pathological diagnosis:2 cases of chordoma,1 case of malignant neurilemmoma,1 case of chondrosarcoma and 1 case of malignant hemangioendothelioma.5 patients had sacrococcygeal pain or lumbocrural pain before the surgery.Visual analogue scale (VAS):2 cases of 2,2 cases of 6 and 1 case of 8.Ilium stability reconstructions were all performed on 5 patients with spine pedicle screw-rod system.Results The mean operation time was 6.5 hours (range,4.5-11 hours),with the mean intraoperative blood loss of 3 700 ml (range,2 000-7 200 ml).There was no perioperative death.The mean follow-up time was 17 months (range,9-23 months).There were 2 cases of wound complications 2 weeks after surgery and healed by second intention with washing and drainage after debridement.There was no deep infection.1 case of rectal injury,which was performed with colostomy during the operation,and stoma returned 12 weeks after surgery.4 cases of sciatic nerve symptom of lower limbs and plantar flexion dyskinesia after bilateral S1 nerve roots resection.The patients walked with ankle brace fixed after the surgery.There was 1 case of implant breakage and no obvious spine down.5 patients had functional disability in sphincter after surgery.1 case of hemangioendothelioma recurred locally 9 months later