Cervical chordoma: a case report

International Nuclear Information System (INIS)

Romera, C.; Wiehoff, A.; Candela, V. P.; Perera, J.

2002-01-01

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

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.

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.)

Carbon-11-methionine positron emission tomography imaging of chordoma

International Nuclear Information System (INIS)

Zhang, Hong; Yoshikawa, Kyosan; Tamura, Katsumi; Sagou, Kenji; Kandatsu, Susumu; Tian, Mei; Suhara, Tetsuya; Suzuki, Kazutoshi; Tanada, Shuji; Tsujii, Hirohiko

2004-01-01

Chordoma is a rare malignant bone tumor that arises from notochord remnants. This is the first trial to investigate the utility of 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.)

Magnetic resonance (MR) imaging of chordoma and chondroma in the skull base

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Tashiro, Takahiko; Inoue, Yuichi; Nemoto, Yutaka

1992-01-01

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)

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

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.

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

International Nuclear Information System (INIS)

Lee, Young Uk; Youn, Eun Kyung

1991-01-01

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

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.

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

Intracranial chordomas: para and intrasellar localization - report of two cases

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Cerioni Junior, M.; Romero, P.C.; Peres, A.J.; Cecilio, S.; Botelho, R.V.; Caldas, J.G.; Settanni, F.

1992-01-01

Two patients with chordomas are reported. One 39-year-old woman with intrasellar chordoma which suffered of galactorrhea, amenorrhea and bi temporal hemianopsia. Another patient, a 50-year-old woman with left parasellar chordoma with proptosis, progressive blindness and left sided facial pain. Clinical and radiological findings, including CT-scan and MRI, are discussed. The MRI in one patient showed a persistent tumor. (author)

From notochord formation to hereditary chordoma: the many roles of Brachyury.

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Nibu, Yutaka; José-Edwards, Diana S; Di Gregorio, Anna

2013-01-01

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.

Chordoma with postoperative subcutaneous implantation and meningeal dissemination: MRI

International Nuclear Information System (INIS)

Kinoshita, T.; Okudera, T.; Shimosegawa, E.; Hatazawa, J.; Yoshida, Y.; Yasui, N.; Ogawa, T.

2001-01-01

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.)

The top 50 cited articles on chordomas.

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Ikpeze, Tochukwu; Mesfin, Addisu

2018-03-01

Chordomas are rare malignant primary tumors of the spine. In the mobile spine and sacrum an en-bloc resection is associated with decreased rates of recurrence. Our objective was to identify the top cited articles in chordoma research and to further analyze characteristics of these articles. In March 2017, we used ISI Web of Science (v5.11, Thomas Reuter, Philadelphia, Pennsylvania, USA) to search for the following key word: "chordoma". Articles were searched from 1900 to 2017. Articles were ranked based on number of citations. The results were evaluated to determine articles most clinically relevant to the management of chordomas. The top 50 articles that met the search criteria were further characterized on the basis of: title, author, citation density, journal of publication, year (and decade) of publication, institution and country of origin and paper topic. A total of 1,043 articles matched the search criteria. The most influential 50 articles were cited 65 to 290 times. The articles were published between 1926 and 2012, and all articles were published in English. Thirty-three publications (66%) originated from the United States and seven (14%) from Italy. Cancer accounted for the most frequent (n=9) destination journal followed by Journal of Bone and Joint Surgery (n=4). A total of 41 institutions contributed to the top 50 articles. The most common article types were: clinical 44% (n=22), papers that combined clinical and pathology findings 18% (n=9) and basic science research 14% (n=7). The top 50 cited articles on chordomas are predominantly clinical papers, arising from the United States and most frequently published in Cancer and Journal of Bone and Joint Surgery .

Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas

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Pamir, M. Necmettin; Ozduman, Koray

2006-01-01

Chordomas and chondrosarcomas are malignant tumors that are reported to have similar clinical presentations and radiological features but different behaviors and outcomes. The aim of this retrospective study was to determine whether specific radiological features of skull-base chordomas or chondrosarcomas are correlated with histopathology, and thus allow preoperative diagnosis. The study involved 32 classic chordomas, 6 chondroid chordomas and 4 chondrosarcomas (42 tumors total). For each case, tumor size and extent, the detailed anatomy involved, and magnetic resonance imaging and computed tomography findings were analyzed. Tumor extent was assessed using a novel method that assessed presence/absence in 18 defined skull-base zones. The chondrosarcomas presented significantly earlier in life than the chordomas (means, 20.5 years versus 36 years, respectively). At time of diagnosis, the median tumor volume was 23 cm 3 (range, 1.2-78.8 cm 3 ) and the mean tumor extent was 6.7 ± 2.9 zones. There were no differences between chordomas and chondrosarcomas, or between the two chordoma subgroups, with respect to lesion volume or extent. Comparison of other imaging findings revealed no features that were diagnostic for either chordoma or chondrosarcoma. The data support previous claims that chondrosarcomas present earlier in life than chordomas, but this finding is not diagnostic. There is wide variation in the extent of skull-base chordomas and chondrosarcomas, and in the specific anatomical structures these tumors involve. None of the MRI or CT features of these tumors appear to be useful for differentiating chordomas from chondrosarcomas preoperatively. For surgical planning, specific, area-oriented definition of tumor extent might provide more useful information than tumor-type classification schemes

Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas

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Pamir, M. Necmettin [Marmara University Faculty of Medicine, Department of Neurosurgery, Istanbul (Turkey)]. E-mail: koray.ozduman@yale.edu; Ozduman, Koray [Marmara University Faculty of Medicine, Department of Neurosurgery, Istanbul (Turkey)

2006-06-15

Chordomas and chondrosarcomas are malignant tumors that are reported to have similar clinical presentations and radiological features but different behaviors and outcomes. The aim of this retrospective study was to determine whether specific radiological features of skull-base chordomas or chondrosarcomas are correlated with histopathology, and thus allow preoperative diagnosis. The study involved 32 classic chordomas, 6 chondroid chordomas and 4 chondrosarcomas (42 tumors total). For each case, tumor size and extent, the detailed anatomy involved, and magnetic resonance imaging and computed tomography findings were analyzed. Tumor extent was assessed using a novel method that assessed presence/absence in 18 defined skull-base zones. The chondrosarcomas presented significantly earlier in life than the chordomas (means, 20.5 years versus 36 years, respectively). At time of diagnosis, the median tumor volume was 23 cm{sup 3} (range, 1.2-78.8 cm{sup 3}) and the mean tumor extent was 6.7 {+-} 2.9 zones. There were no differences between chordomas and chondrosarcomas, or between the two chordoma subgroups, with respect to lesion volume or extent. Comparison of other imaging findings revealed no features that were diagnostic for either chordoma or chondrosarcoma. The data support previous claims that chondrosarcomas present earlier in life than chordomas, but this finding is not diagnostic. There is wide variation in the extent of skull-base chordomas and chondrosarcomas, and in the specific anatomical structures these tumors involve. None of the MRI or CT features of these tumors appear to be useful for differentiating chordomas from chondrosarcomas preoperatively. For surgical planning, specific, area-oriented definition of tumor extent might provide more useful information than tumor-type classification schemes.

Surgical Consideration for Adolescents and Young Adults With Cervical Chordoma.

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Zhong, Nanzhe; Yang, Xinghai; Yang, Jian; Meng, Tong; Yang, Cheng; Yan, Wangjun; Xiao, Jianru

2017-05-15

Retrospective study. The aim of this study was to compare the clinical outcomes between adolescent and young adult (AYA) patients and old adult patients with cervical chordoma who were treated surgically and present the surgical consideration for adolescents and young adults with cervical chordoma. With predominance in senior patients, chordoma is distinctively rare in AYAs. Because of the rarity of AYA chordoma, individual case report represents most of the literature on this disease entity on mobile spine and lack of long-term follow up, which leads to the paucity of clinical evidence for treatment planning and prognosis prediction. A retrospective study was conducted to investigate the prognosis of AYA patients with cervical chordoma who were treated surgically. We collected the clinical data of these patients and their older counterparts, and further compared the prognosis of the patients in different age groups. To estimate survival curves, Kaplan-Meier method was used, and significance was assessed using a log-rank test. Forty consecutive patients with chordoma of the cervical spine treated in our institution were included in the study. Two groups were identified according to age. Group 1 comprised children and adolescents (age ≤ 25 yrs; n = 9) and Group 2 comprised adults (age > 25 years; n = 31). In comparison, Group 1 was featured by significantly higher rate of recurrence and shorter overall survival, although no difference found in the surgical modality between two groups. There is a dismal prognosis in young patients with chordoma, and thus support the notion that as radical a total en bloc spondylectomy (TES) of the lesions as possible may benefit the overall survival of these young patients. Although the ensuing neurological deficits may be devastating, it will be worth sacrificing if the life expectancy of these young patients is prolonged. 4.

Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity

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Kelvin Manuel Pińa Batista

2017-12-01

Full Text Available Chordomas are rare and low-grade malignant solid tumours, despite their histologically benign appearance, that arise in the bone from embryonic notochordal vestiges of the axial skeleton, a mesoderm-derived structure that is involved in the process of neurulation and embryonic development. Chordomas occurring in the skull base tend to arise in the basiocciput along the clivus. Three major morphological variants have been described (classical, chondroid, and atypical/dedifferentiated. The pathogenesis and molecular mechanisms involved in chordoma development remain uncertain. From a pathological standpoint, the microenvironment of a chordoma is heterogeneous, showing a dual epithelial-mesenchymal differentiation. These tumours are characterised by slow modality of biologic growth, local recurrence, low incidence of metastasis rates, and cancer stem cell (CSC phenotype. The main molecular findings are connected with brachyury immunoexpression and activation of the downstream Akt and mTOR signalling pathways. The differentiation between typical and atypical chordomas is relevant because the tumoural microenvironment and prognosis are partially different. This review provides an insight into the recent and relevant concepts and histochemical markers expressed in chordomas, with special emphasis on dedifferentiated chordomas and their prognostic implications.

Metastatic carcinoma of breast or a chordoma? A case report and clinical perspectives.

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Trivedi, Sachin; Odrazka, Karel

2015-01-01

We present a case of chordoma in a patient who had been previously treated for ductal carcinoma of the breast. The initial clinical findings and radiological studies suggested a possibility of metastases. However, the findings also adhered to the classical presentations and findings of the chordoma of the base of skull. It was only after the surgical resection and immunohistochemical confirmation that the diagnosis of chordoma could be established. Here, we discuss chordoma with the analysis of our clinical intrigue.

Gamma Knife surgery for intracranial chordoma and chondrosarcoma: radiosurgical perspectives and treatment outcomes.

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Kim, Ji Hee; Jung, Hyun Ho; Chang, Jong Hee; Chang, Jin Woo; Park, Yong Gou; Chang, Won Seok

2014-12-01

Intracranial chordomas and chondrosarcomas are histologically low-grade, locally invasive tumors that are reported to be similar in terms of anatomical location, clinical presentation, and radiological findings but different in terms of behavior and outcomes. The purpose of this study was to investigate and compare clinical outcomes after Gamma Knife surgery (GKS) for the treatment of intracranial chordoma and chondrosarcoma. The authors conducted a retrospective review of the results of radiosurgical treatment of intracranial chordomas and chondrosarcomas. They enrolled patients who had undergone GKS for intracranial chordoma or chondrosarcoma at the Yonsei Gamma Knife Center, Yonsei University College of Medicine, from October 2000 through June 2007. Analyses included only patients for whom the disease was pathologically diagnosed before GKS and for whom more than 5 years of follow-up data after GKS were available. Rates of progression-free survival and overall survival were analyzed and compared according to tumor pathology. Moreover, the association between tumor control and the margin radiation dose to the tumor was analyzed, and the rate of tumor volume change after GKS was quantified. A total of 10 patients were enrolled in this study. Of these, 5 patients underwent a total of 8 sessions of GKS for chordoma, and the other 5 patients underwent a total of 7 sessions of GKS for chondrosarcoma. The 2- and 5-year progression-free survival rates for patients in the chordoma group were 70% and 35%, respectively, and rates for patients in the chondrosarcoma group were 100% and 80%, respectively (log-rank test, p = 0.04). The 2- and 5-year overall survival rates after GKS for patients in the chordoma group were 87.5% and 72.9%, respectively, and rates for patients in the chondrosarcoma group were 100% and 100%, respectively (log-rank test, p = 0.03). The mean rates of tumor volume change 2 years after radiosurgery were 79.64% and 39.91% for chordoma and

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

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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.

The importance of preoperative tissue sampling for mobile spine chordomas: literature review and report of two cases.

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Zuccato, Jeffrey A; Witiw, Christopher D; Keith, Julia; Dyer, Erin; Saghal, Arjun; da Costa, Leodante

2018-01-01

Pre-operative biopsy and diagnosis of chordomas of the mobile spine is indicated as en bloc resections improve outcomes. This review of the management of mobile spine chordomas includes two cases of unexpected mobile spine chordomas where a preoperative tissue diagnosis was decided against and may have altered surgical decision-making. Two lumbar spine chordomas thought to be metastatic and primary bony lesions preoperatively were not biopsied before surgery and eventual pathology revealed chordoma. Preoperative diagnoses were questioned during surgery after an intraoperative tissue diagnosis of chordoma in one case and unclear pathology with non-characteristic tumor morphology in the other. The surgical plan was altered in these cases to maximize resection as en bloc resection reduces the risk of local recurrence in chordoma. Mobile spine chordomas are rare and en bloc resection is recommended, contrary to the usual approach to more common spine tumors. Since en bloc resection of spine chordomas improves disease free survival, it has been recommended that tissue diagnosis be obtained preoperatively when chordoma is considered in the differential diagnosis, in order to guide surgical planning. We present two cases where a preoperative biopsy was considered but not obtained after neuroradiology consultation and imaging review, which may have been managed differently if the diagnosis of spine chordomas were known pre-operatively.

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.).

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.

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

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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.

Sacrococcygeal chordomas with wide-spread metastases: report of two cases and review of literature

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Suh, Hyun Suk; Shin, Young Ju; Joo, Mee; Kim, Byung Jik [College of Medicine, Inje Univ., Pusan (Korea, Republic of)

1999-03-01

Chordomas are rare tumors arising from the primitive notochord. The commonest affected segment is the sacrum and these chordomas frequently follow a progressive course with multiple recurrences and metastases and eventual death due to tumor. This report describes two cases of sacrococcygeal chordomas with widespread metastases treated by surgery and adjuvant radiation therapy.

Sacrococcygeal chordomas with wide-spread metastases: report of two cases and review of literature

International Nuclear Information System (INIS)

Suh, Hyun Suk; Shin, Young Ju; Joo, Mee; Kim, Byung Jik

1999-01-01

Chordomas are rare tumors arising from the primitive notochord. The commonest affected segment is the sacrum and these chordomas frequently follow a progressive course with multiple recurrences and metastases and eventual death due to tumor. This report describes two cases of sacrococcygeal chordomas with widespread metastases treated by surgery and adjuvant radiation therapy

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

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Zhang, Linlin; Guo, Shang; Schwab, Joseph H; Nielsen, G Petur; Choy, Edwin; Ye, Shunan; Zhang, Zhan; Mankin, Henry; Hornicek, Francis J; Duan, Zhenfeng

2013-01-01

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+ (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.

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)

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

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

2014-07-01

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. © 2014. Published by The Company of Biologists Ltd.

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.)

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

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Choi, Guk Myeong; Han, Moon Hee; Chang, Kee Hyun; Kim, Hong Dae; Yeon, Kyung Mo; Kim, Sam Soo

1998-01-01

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

Chordoma

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Saxton, J.P.

1981-01-01

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

Treatment of Recurrent Chordomas by Percutaneous Ethanol Injection Therapy and Radiation Therapy

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Nakajo, M.; Ohkubo, K.; Fukukura, Y.; Nandate, T.; Nakajo, M.

2006-01-01

We report a case of recurrent sacral chordomas that have been successfully controlled by the combination therapy of percutaneous ethanol injection therapy (PEIT) and radiation therapy in a 71-year-old man. PEIT may be one of the adjuvant therapies for recurrent chordomas

Characteristics and Patterns of Metastatic Disease from Chordoma

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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.

Magnetic resonance imaging (MRI) of intracranial chordomas

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Fukuda, Teruo; Inoue, Yuichi; Shakudo, Miyuki and others

1988-03-01

MR images of 5 patients with intracranial chordoma were evaluated and compared with those of other clival lesions (1 clival osteomyelitis, 1 metastatic clival tumor, 3 clival meningiomas). The MR examination was performed using a 0.5 T superconductive magnet, with approximately 10 mm section thickness, one average and a 256 x 256 matrix. T1 weighted images were obtainned by inversion recovery (IR) with TR 2100 - 2500 msec, TI 600 msec and TE 40 msec. T2 weighted images were obtained by spin echo pulse sequence with TR 1800 - 2500 msec and TE 120 msec (long SE). In several cases, the spin echo pulse sequences with TR 1000 msec and TE 40 msec (short SE) were also done. Multiplaned images were obtained. Four of 5 intracranial chordomas were low in intensity compared to cerebral gray matter on T1 weighted images, and all of 5 chordomas were as high in intensity as cerebrospinal fluid or higher than that of cerebrospinal fluid on T2 weighted images. Clival fatty marrow is high intensity on T1 weighted images. Clival involvement by a tumor was a clearly demonstrated as disappearance of this high intensity in all cases. In two cases, the tumor extended to the retropharyngeal space and this was detected clearly on short SE image. Although clival fatty marrow was disappeared, osteomyelitis and metastatic tumor in clivus were iso-intense to cerebral gray matter on both T1 and T2 weighted images. All of 3 clival meningiomas showed iso-intensity to cerebral gray matter on T1 weighted images and slightly high intensity to brain on T2 weighted images, and clival fatty marrow was normal in all 3 cases. Although our experiences are limited in number, intracranial chordoma appeared to be differentiated from other clival lesions.

Computer Navigation-aided Resection of Sacral Chordomas

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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.

Benign chordoma of the sacral bone. Radiologic appearance and differential dignosis

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Pegios, W.; Vogl, T.J.; Rausch, M.; Klein, U.; Balzer, J.O.; Hammerstingl, R.; Mack, M.G.; Felix, R.

1994-01-01

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.) [de

On a Rare Cutaneous Metastasis from a Sacrococcygeal Chordoma

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Alessandro D’Amuri

2017-01-01

Full Text Available Chordomas are rare malignant tumors of notochordal origin and are rare locally aggressive ones with a metastatic potential. The skin rarely is seen as metastatic site. We describe a case of an adult woman with cutaneous metastasis of a primary sacral chordoma excised ten years before, which appeared as a painless cutaneous mass located in the dorsal region. Once removed, the surgical specimen was formalin fixed and in paraffin embedded. Sections were stained with haematoxylin-eosin, and histochemical and immunohistochemical investigations were performed. Histologically, the neoplasia was characterized by cords or single tumor cells with an abundant myxoid stroma, conspicuous pale vacuolated cytoplasm (the classic “physaliphorous cells”, and mild nuclear atypia. Mitotic activity was scanty. At immunohistochemistry, the tumor cells were diffusely positive for S-100 protein, pan-keratins, EMA, and vimentin. A diagnosis of cutaneous metastasis of chordoma was performed. This case illustrates a diagnostic challenge because of the unusual presentation of an already rare tumor.

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

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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.

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

International Nuclear Information System (INIS)

Cho, Hyoun; Yu, In Kyu; Kim, Seung Min; Kim, Han Kyu

2012-01-01

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

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

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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.

Endoscopic Resection of Clival Chordoma. A Case Report

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Ines Gamboa

2018-01-01

Full Text Available Introduction: Chordoma is a rare malignant tumor that arises from remnants cells of the primitive notochord, which are located at caudal and cephalic ends of the vertebral column. It represents 2 to 5 % of all primary bone tumors. Description: We report the case of a patient with a clival chordoma, asymptomatic, diagnosed as an accidental finding in a paranasal sinus. imaging study. Discussion: The imaging findings were suggestive of a potentially malignant lesion given the underlying bone lysis. Once the diagnosis is histological, biopsy of clival suspicious lesions should be promptly carried out. In this case report, the surgical approach and the postoperative follow-up are presented.

Proton radiation therapy for clivus chordoma

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Yoshii, Yoshihiko; Tsunoda, Takashi; Hyodo, Akio; Nose, Tadao; Tsujii, Hirohiko; Tsuji, Hiroshi; Inada, Tetsuo; Maruhashi, Akira; Hayakawa, Yoshinori.

1993-01-01

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)

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

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

2016-06-07

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.

Chordoma of the petrous apex - a case report and review of the literature

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Loureiro, Ricardo; Leal Junior, Osvaldo S.; Loureiro, Lautonio Junior; Buril, Marlus V.M.

1998-01-01

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)

Chordomas of the Skull Base, Mobile Spine, and Sacrum: An Epidemiologic Investigation of Presentation, Treatment, and Survival.

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Zuckerman, Scott L; Bilsky, Mark H; Laufer, Ilya

2018-05-01

Chordomas are rare primary bone tumors that arise from the axial skeleton. Our objective was to analyze trends in radiation and surgery over time and determine location-based survival predictors for chordomas of the skull base, mobile spine, and sacrum. A retrospective cohort study of the SEER (Surveillance Epidemiology and End Results) database from 1973 to 2013 was conducted. All patients had histologically confirmed chordomas. The principal outcome measure was overall survival (OS). The cohort included 1616 patients: skull base (664), mobile spine (444), and sacrum (508). Skull base tumors presented earliest in life (47.4 years) and sacral tumors presented latest (62.7 years). Rates of radiation remained stable for skull base and mobile spine tumors but declined for sacral tumors (P = 0.006). Rates of surgical resection remained stable for skull base and sacral tumors but declined for mobile spine tumors (P = 0.046). Skull base chordomas had the longest median survival (162 months) compared with mobile spine (94 months) and sacral tumors (87 months). Being married was independently associated with improved OS for skull base tumors (hazard ratio, 0.73; 95% confidence interval, 0.53-0.99; P = 0.044). Surgical resection was independently associated with improved OS for sacral chordomas (hazard ratio, 0.48; 95% confidence interval, 0.34-0.69; P mobile spine chordomas and radiation for sacral chordomas decreased over time. Patients with skull base tumors survived longer than did patients with mobile spine and sacral chordomas, and surgical resection was associated with improved survival in sacral chordomas only. Understanding the behavior of these tumors can help cranial and spinal surgeons improve treatment in this patient population. Copyright © 2018 Elsevier Inc. All rights reserved.

Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins' Law.

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Rassi, Marcio S; Hulou, M Maher; Almefty, Kaith; Bi, Wenya Linda; Pravdenkova, Svetlana; Dunn, Ian F; Smith, Timothy R; Al-Mefty, Ossama

2018-05-01

Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined. To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins' law for embryonal tumors. Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263). Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival. Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins' law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

Carbon ion radiotherapy for chordomas and low-grade chondrosarcomas of the skull base. Results in 67 patients

International Nuclear Information System (INIS)

Schulz-Ertner, D.; Wannenmacher, M.; Nikoghosyan, A.; Thilmann, C.; Jaekel, O.; Karger, C.; Haberer, T.; Scholz, M.; Kraft, G.; Debus, J.

2003-01-01

Purpose: To prospectively evaluate outcome and toxicity after carbon ion radiotherapy (RT) in chordomas and low-grade chondrosarcomas. Patients and Methods: Between September 1998 and December 2001, 74 patients were treated for chordomas and chondrosarcomas with carbon ion RT at the ''Gesellschaft fuer Schwerionenforschung'' (GSI). Seven patients reirradiated with reduced carbon ion doses after conventional RT were excluded from the analysis, leaving 67 evaluable patients (44 chordomas and 23 chondrosarcomas) who received a full course of carbon ion therapy. Tumor-conform application of carbon ion beams was realized by intensity-controlled raster scanning with active energy variation. Three-dimensional treatment planning included intensity modulation and biological plan optimization. A median dose of 60 GyE was applied to the target volume within 20 consecutive days at a dose of 3.0 GyE per fraction. Results: Median follow-up was 15 months (range 3-46 months). At 3 years, actuarial local control was 100% for chondrosarcomas and 87% for chordomas, respectively. Partial tumor remission was observed in 14/44 (31%) chordoma patients and in 4/23 (17%) chondrosarcoma patients. At 3 years, actuarial overall survival was 100% for chondrosarcomas and 89% for chordomas, respectively. No severe side effects > CTC III have been observed. Conclusions: These data demonstrate the clinical efficiency and safety of scanning beam delivery of carbon ion beams in patients with skull base chordomas and chondrosarcomas. The observation of tumor regressions at a dose level of 60 GyE may indicate that the biological effectiveness of carbon ions in chordomas and chondrosarcomas is higher than initially estimated. (orig.)

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

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

2014-07-30

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.

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

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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.

Carbon ion radiotherapy for chordomas and low-grade chondrosarcomas of the skull base. Results in 67 patients

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Schulz-Ertner, D.; Wannenmacher, M. [Dept. of Clinical Radiology, Univ. of Heidelberg (Germany); Nikoghosyan, A.; Thilmann, C.; Jaekel, O.; Karger, C. [German Cancer Research Center (dkfz), Heidelberg (Germany); Haberer, T.; Scholz, M.; Kraft, G. [Dept. of Biophysics, German Ion Research Center (GSI), Darmstadt (Germany); Debus, J. [Dept. of Clinical Radiology, Univ. of Heidelberg (Germany); German Cancer Research Center (dkfz), Heidelberg (Germany)

2003-09-01

Purpose: To prospectively evaluate outcome and toxicity after carbon ion radiotherapy (RT) in chordomas and low-grade chondrosarcomas. Patients and Methods: Between September 1998 and December 2001, 74 patients were treated for chordomas and chondrosarcomas with carbon ion RT at the ''Gesellschaft fuer Schwerionenforschung'' (GSI). Seven patients reirradiated with reduced carbon ion doses after conventional RT were excluded from the analysis, leaving 67 evaluable patients (44 chordomas and 23 chondrosarcomas) who received a full course of carbon ion therapy. Tumor-conform application of carbon ion beams was realized by intensity-controlled raster scanning with active energy variation. Three-dimensional treatment planning included intensity modulation and biological plan optimization. A median dose of 60 GyE was applied to the target volume within 20 consecutive days at a dose of 3.0 GyE per fraction. Results: Median follow-up was 15 months (range 3-46 months). At 3 years, actuarial local control was 100% for chondrosarcomas and 87% for chordomas, respectively. Partial tumor remission was observed in 14/44 (31%) chordoma patients and in 4/23 (17%) chondrosarcoma patients. At 3 years, actuarial overall survival was 100% for chondrosarcomas and 89% for chordomas, respectively. No severe side effects > CTC III have been observed. Conclusions: These data demonstrate the clinical efficiency and safety of scanning beam delivery of carbon ion beams in patients with skull base chordomas and chondrosarcomas. The observation of tumor regressions at a dose level of 60 GyE may indicate that the biological effectiveness of carbon ions in chordomas and chondrosarcomas is higher than initially estimated. (orig.)

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

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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

2016-04-01

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. 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. 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 mobile spine.

Gain of chromosome 7 by chromogenic in situ hybridization (CISH) in chordomas is correlated to c-MET expression.

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Walter, Beatriz A; Begnami, Maria; Valera, Vladimir A; Santi, Mariarita; Rushing, Elisabeth J; Quezado, Martha

2011-01-01

Chordomas are low to intermediate grade malignancies that arise from remnants of embryonic notochord. They often recur after surgery and are highly resistant to conventional adjuvant therapies. Recently, the development of effective targeted molecular therapy has been investigated in chordomas that show receptors for tyrosine kinase (RTKs) activation. Expression of specific RTKs such as Epidermal Growth Factor Receptor (EGFR) and Mesenchymal-epithelial transition factor (c-MET) in chordomas may offer valuable therapeutic options. We investigated changes in copy number of chromosome 7 and correlated it with EGFR gene status and EGFR and c-MET protein expression in 22 chordoma samples. Chromosome 7 copy number was evaluated by chromogenic in situ hybridization (CISH) and protein expression of EGFR and c-MET by immunohistochemistry. Tumors mostly showed conventional histopathologic features and were found mainly in sacral (41%) and cranial sites (54.5%). Aneusomy of chromosome 7 was seen in 73% of the samples, 62% of primary tumors and in all recurrent chordomas. EGFR and c-MET were both expressed, but only c-MET protein expression was significantly correlated with chromosome 7 aneusomy (P ≤ 0.001). c-MET overexpression may represent an early chromosome 7 alteration that could play an important role during chordoma pathogenesis. c-MET overexpression shows promise as a molecular marker of response to targeted molecular therapy in the treatment of chordomas.

Radiotherapy for chordomas and low-grade chondrosarcomas of the skull base with carbon ions

International Nuclear Information System (INIS)

Schulz-Ertner, Daniela; Haberer, Thomas; Jaekel, Oliver; Thilmann, Christoph; Kraemer, Michael; Enghardt, Wolfgang; Kraft, Gerhard; Wannenmacher, Michael; Debus, Juergen

2002-01-01

Purpose: Compared to photon irradiation, carbon ions provide physical and biologic advantages that may be exploited in chordomas and chondrosarcomas. Methods and Materials: Between August 1998 and December 2000, 37 patients with chordomas (n=24) and chondrosarcomas (n=13) were treated with carbon ion radiotherapy within a Phase I/II trial. Tumor conformal application of carbon ion beams was realized by intensity-controlled raster scanning with pulse-to-pulse energy variation. Three-dimensional treatment planning included biologic plan optimization. The median tumor dose was 60 GyE (GyE Gy x relative biologic effectiveness). Results: The mean follow-up was 13 months. The local control rate after 1 and 2 years was 96% and 90%, respectively. We observed 2 recurrences outside the gross tumor volume in patients with chordomas. Progression-free survival was 100% for chondrosarcomas and 83% for chordomas at 2 years. Partial remission after carbon ion radiotherapy was observed in 6 patients. Treatment toxicity was mild. Conclusion: These are the first data demonstrating the clinical feasibility, safety, and effectiveness of scanning beam delivery of ion beams in patients with skull base tumors. The preliminary results in patients with skull base chordomas and low-grade chondrosarcomas are encouraging, although the follow-up was too short to draw definite conclusions concerning outcome. In the absence of major toxicity, dose escalation might be considered

Lumbar vertebra chordoma | Erlank | SA Journal of Radiology

African Journals Online (AJOL)

Abstract. Spinal chordomas in the lumbar region are rare and can easily be overlooked in the differential diagnosis of vertebral column tumours. South African Journal of Radiology Vol. 10 (3) 2006: pp. 37-38 ...

Base of skull and cervical spine chordomas in children treated by high-dose irradiation

International Nuclear Information System (INIS)

Benk, Veronique; Liebsch, Norbert J.; Munzenrider, John E.; Efird, John; McManus, Patricia; Suit, Herman

1995-01-01

Purpose: To evaluate the outcome of children with base of skull or cervical spine chordomas treated by high dose irradiation. Methods and Materials: Eighteen children, 4 to 18 years of age, with base of skull or cervical spine chordomas, received fractionated high-dose postoperative radiation using mixed photon and 160 MeV proton beams. The median tumor dose was 69 Cobalt Gray-equivalent (CGE) with a 1.8 CGE daily fraction. Results: The median follow-up was 72 months. The 5-year actuarial survival was 68% and the 5-year disease-free survival (DFS) was 63%. The only significant prognostic factor was the location: patients with cervical spine chordomas had a worse survival than those with base of skull lesions (p = 0.008). The incidence of treatment-related morbidity was acceptable: two patients developed a growth hormone deficit corrected by hormone replacement, one temporal lobe necrosis, and one fibrosis of the temporalis muscle, improved by surgery. Conclusion: Chordomas in children behave similarly to those in adults: children can receive the same high-dose irradiation as adults with acceptable morbidity

Base of skull and cervical spine chordomas in children treated by high-dose irradiation

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Benk, Veronique; Liebsch, Norbert J; Munzenrider, John E; Efird, John; McManus, Patricia; Suit, Herman

1995-02-01

Purpose: To evaluate the outcome of children with base of skull or cervical spine chordomas treated by high dose irradiation. Methods and Materials: Eighteen children, 4 to 18 years of age, with base of skull or cervical spine chordomas, received fractionated high-dose postoperative radiation using mixed photon and 160 MeV proton beams. The median tumor dose was 69 Cobalt Gray-equivalent (CGE) with a 1.8 CGE daily fraction. Results: The median follow-up was 72 months. The 5-year actuarial survival was 68% and the 5-year disease-free survival (DFS) was 63%. The only significant prognostic factor was the location: patients with cervical spine chordomas had a worse survival than those with base of skull lesions (p = 0.008). The incidence of treatment-related morbidity was acceptable: two patients developed a growth hormone deficit corrected by hormone replacement, one temporal lobe necrosis, and one fibrosis of the temporalis muscle, improved by surgery. Conclusion: Chordomas in children behave similarly to those in adults: children can receive the same high-dose irradiation as adults with acceptable morbidity.

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

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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.

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

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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

2015-06-01

We report our preliminary outcomes following high-dose image-guided intensity modulated radiotherapy (IG-IMRT) for skull base chordoma and chondrosarcoma. 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. 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. 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. © The Author(s) 2014. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Clinical results of proton beam therapy for skull base chordoma

International Nuclear Information System (INIS)

Igaki, Hiroshi; Tokuuye, Koichi; Okumura, Toshiyuki; Sugahara, Shinji; Kagei, Kenji; Hata, Masaharu; Ohara, Kiyoshi; Hashimoto, Takayuki; Tsuboi, Koji; Takano, Shingo; Matsumura, Akira; Akine, Yasuyuki

2004-01-01

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

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

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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.)

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

International Nuclear Information System (INIS)

O'Donnell, Paul; Tirabosco, Roberto; Vujovic, Sonja; Henderson, Stephen; Boshoff, Chris; Bartlett, William; Briggs, Timothy W.R.; Flanagan, Adrienne M.

2007-01-01

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.)

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

International Nuclear Information System (INIS)

Matsumoto, J.; Towbin, R.B.; Ball, W.S. Jr.; Children's Hospital Medical Center, Cincinnati, OH; Cincinnati Univ., OH

1989-01-01

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.)

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

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Jun-qi Liu

2015-09-01

Full Text Available 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. 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. Keywords: p16, Cyclin D1, Rb, MIB-1, Skull base chordoma, Skull base chondrosarcoma

Analysis of prognostic factors for survival in patients with primary spinal chordoma using the SEER Registry from 1973 to 2014.

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Pan, Yue; Lu, Lingyun; Chen, Junquan; Zhong, Yong; Dai, Zhehao

2018-04-06

Spinal chordomas are rare primary osseous tumors that arise from the remnants of the notochord. They are commonly considered slow-growing, locally invasive neoplasms with little tendency to metastasize, but the high recurrent rate of spinal chordomas may seriously affect the survival rate and quality of life of patients. The aim of the study is to describe the epidemiological data and determine the prognostic factors for decreased survival in patients with primary spinal chordoma. The Surveillance, Epidemiology, and End Results (SEER) Registry database, a US population-based cancer registry database, was used to identify all patients diagnosed with primary spinal chordoma from 1973 to 2014. We utilized Kaplan-Meier method and Cox proportional hazards regression analysis to evaluate the association between patients overall survival and relevant characteristics, including age, gender, race, disease stage, treatment methods, primary tumor site, marital status, and urban county background. In the data set between 1973 and 2014, a total of 808 patients were identified with primary spinal chordoma. The overall rate of distant metastatic cases in our cohort was only 7.7%. Spinal chordoma was more common occurred in men (62.6%) than women (37.3%). Majority of neoplasms were found in the White (87.9%), while the incidence of the Black is relatively infrequent (3.3%). Three hundred fifty-seven spinal chordomas (44.2%) were located in the vertebral column, while 451 patients' tumor (55.8%) was located in the sacrum or pelvis. Age ≥ 60 years (HR = 2.72; 95%CI, 1.71 to 2.89), distant metastasis (HR = 2.16; 95%CI, 1.54 to 3.02), and non-surgical therapy (HR = 2.14; 95%CI, 1.72 to 2.69) were independent risk factors for survival reduction in analysis. Survival did not significantly differ as a factor of tumor site (vertebrae vs sacrum/pelvis) for primary spinal chordoma (HR = 0.93, P = 0.16). Race (P = 0.52), gender (P = 0.11), marital status (P = 0.94), and

Proton radiation therapy for clivus chordoma; Case report

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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).

Sacrococcygeal chordoma: increased 99mTc methylene diphosphonate uptake on single photon emission computed tomography/computed tomography bone scintigraphy

International Nuclear Information System (INIS)

Kamaleshwaran, Koramadai Karuppuswamy; Bhattacharya, Anish; Harisankar, Chidambaram Natarajan Balasubramaniam; Mittal, Bhagwant Rai; Goni, Vijay

2012-01-01

Chordoma is a malignant tumor arising from the remnants of the notochord, and is the most frequent primitive tumor of the sacrum. While most sacral tumors show increased concentration of bone-seeking radiopharmaceuticals, chordomas usually exhibit decreased uptake. The authors present an image of a sacrococcygeal chordoma with osteolysis and increased uptake of 99m Tc methylene diphosphonate on planar and single photon emission computed tomography/computed tomography bone scintigraphy. (author)

High-resolution Whole-Genome Analysis of Skull Base Chordomas Implicates FHIT Loss in Chordoma Pathogenesis12

OpenAIRE

Diaz, Roberto Jose; Guduk, Mustafa; Romagnuolo, Rocco; Smith, Christian A; Northcott, Paul; Shih, David; Berisha, Fitim; Flanagan, Adrienne; Munoz, David G; Cusimano, Michael D; Pamir, M Necmettin; Rutka, James T

2012-01-01

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 ...

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

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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.

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

International Nuclear Information System (INIS)

Smolders, D.; Wang, X.; Vanhoenacker, F.; De Schepper, A.M.; Drevelengas, A.

2003-01-01

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.)

Lymphocyte-Related Inflammation and Immune-Based Scores Predict Prognosis of Chordoma Patients After Radical Resection

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Wenhao Hu

2018-04-01

Full Text Available The inflammatory microenvironment plays a critical role in the development and progression of malignancies. In the present study, we aimed to evaluate the prognostic value of lymphocyte-related inflammation and immune-based prognostic scores in patients with chordoma after radical resection, including the neutrophil-lymphocyte ratio (NLR, platelet-lymphocyte ratio (PLR, monocyte-lymphocyte ratio (MLR, and systemic immune-inflammation index (SII. A total of 172 consecutive patients with chordoma who underwent radical resection were reviewed. R software was used to randomly select 86 chordoma patients as a training set and 86 chordoma patients as a validation set. Potential prognostic factors were also identified, including age, sex, tumor localization, KPS, Enneking stage, tumor size, and tumor metastasis. Overall survival (OS was calculated using the Kaplan–Meier method and multivariate Cox regression analyses. NLR, PLR, SII, Enneking stage, tumor differentiation and tumor metastasis were identified as significant factors from the univariate analysis in both the training and validation sets and were subjected to multivariate Cox proportional hazards analysis. The univariate analysis showed that NLR ≥1.65, PLR ≥121, and SII ≥370×109/L were significantly associated with poor OS. In the multivariate Cox proportional hazard analysis, SII, Enneking stage and tumor metastasis were significantly associated with OS. As noninvasive, low-cost, reproducible prognostic biomarkers, NLR, PLR and SII could help predict poor prognosis in patients with chordoma after radical resection. This finding may contribute to the development of more effective tailored therapy according to the characteristics of individual tumors.

Predicting clinical outcomes in chordoma patients receiving immunotherapy: a comparison between volumetric segmentation and RECIST

International Nuclear Information System (INIS)

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

2016-01-01

The Response Evaluation Criteria in Solid Tumors (RECIST) are the current standard for evaluating disease progression or therapy response in patients with solid tumors. RECIST 1.1 calls for axial, longest-diameter (or perpendicular short axis of lymph nodes) measurements of a maximum of five tumors, which limits clinicians’ ability to adequately measure disease burden, especially in patients with irregularly shaped tumors. This is especially problematic in chordoma, a disease for which RECIST does not always adequately capture disease burden because chordoma tumors are typically irregularly shaped and slow-growing. Furthermore, primary chordoma tumors tend to be adjacent to vital structures in the skull or sacrum that, when compressed, lead to significant clinical consequences. Volumetric segmentation is a newer technology that allows tumor burden to be measured in three dimensions on either MR or CT. Here, we compared the ability of RECIST measurements and tumor volumes to predict clinical outcomes in a cohort of 21 chordoma patients receiving immunotherapy. There was a significant difference in radiologic time to progression Kaplan-Meier curves between clinical outcome groups using volumetric segmentation (P = 0.012) but not RECIST (P = 0.38). In several cases, changes in volume were earlier and more sensitive reflections of clinical status. RECIST is a useful evaluation method when obvious changes are occurring in patients with chordoma. However, in many cases, RECIST does not detect small changes, and volumetric assessment was capable of detecting changes and predicting clinical outcome earlier than RECIST. Although this study was small and retrospective, we believe our results warrant further research in this area

[Application of neuroendoscope in the treatment of skull base chordoma].

Science.gov (United States)

Zhang, Ya-Zhuo; Wang, Zong-Cheng; Zong, Xu-Yi; Wang, Xin-Sheng; Gui, Song-Bai; Zhao, Peng; Li, Chu-Zhong; He, Yue; Wang, Hong-Yun

2011-07-05

To further explore the application, approach, indication and prognosis of neuroendoscope treatment for skull base chordoma. A total of 101 patients of skull base chordoma were admitted at our hospital from May 2000 to April 2010. There were 59 males and 42 females. Their major clinical manifestations included headache, cranial nerve damage and dyspnea. They were classified according to the patterns of tumor growth: Type I (n = 13): tumor location at a single component of skull base, i. e. clivus or sphenoid sinus with intact cranial dura; Type II (n = 56): tumor involving more than two components of skull e. g clivus, sphenoid and nasal/oral cavity, etc. But there was no intracranial invasion; Type III (n = 32) : tumor extending widely and intradurally forming compression of brain stems and multiple cranial nerves. Based on the types of chordoma, different endoscopic approaches were employed, viz. transnasal, transoral, trans-subtemporal fossa and plus microsurgical craniotomy for staging in some complex cases. Among all patients, total resection was achieved (n = 19), subtotal (n = 58) and partial (n = 24). In partial resection cases, 16 cases were considered to be subtotal due to a second-stage operation. Most cases had conspicuous clinical improvements. Self-care recovery within one week post-operation accounted for 58.4%, two weeks 30.7%, one month 6.9% and more than one month 1.9%. Postoperative complications occurred in 13 cases (12.8%) and included CSF leakage (n = 4) cranial nerve palsy (n = 5), hemorrhagic nasal wounds (n = 3) and delayed intracranial hemorrhage (n = 1). All of these were cured or improved after an appropriate treatment. A follow-up of 6 - 60 months was conducted in 56 cases. Early detection and early treatment are crucial for achieving a better outcome in chordoma. Neuroendoscopic treatment plays an important role in managing those complicated cases. Precise endoscopic techniques plus different surgical approaches and staging procedures

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.

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

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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.)

Proton Therapy for Skull Base Chordomas: An Outcome Study from the University of Florida Proton Therapy Institute

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Deraniyagala, Rohan L.; Yeung, Daniel; Mendenhall, William M.; Li, Zuofeng; Morris, Christopher G.; Mendenhall, Nancy P.; Okunieff, Paul; Malyapa, Robert S.

2013-01-01

Objectives Skull base chordoma is a rare, locally aggressive tumor located adjacent to critical structures. Gross total resection is difficult to achieve, and proton therapy has the conformal advantage of delivering a high postoperative dose to the tumor bed. We present our experience using proton therapy to treat 33 patients with skull base chordomas.

On the cost-effectiveness of Carbon ion radiation therapy for skull base chordoma

International Nuclear Information System (INIS)

Jaekel, Oliver; Land, Beate; Combs, Stephanie Elisabeth; Schulz-Ertner, Daniela; Debus, Juergen

2007-01-01

Aim: The cost-effectiveness of Carbon ion radiotherapy (RT) for patients with skull base chordoma is analyzed. Materials and Methods: Primary treatment costs and costs for recurrent tumors are estimated. The costs for treatment of recurrent tumors were estimated using a sample of 10 patients presenting with recurrent chordoma at the base of skull at DKFZ. Using various scenarios for the local control rate and reimbursements of Carbon ion therapy the cost-effectiveness of ion therapy for these tumors is analyzed. Results: If local control rate for skull base chordoma achieved with carbon ion therapy exceeds 70.3%, the overall treatment costs for carbon RT are lower than for conventional RTI. The cost-effectiveness ratio for carbon RT is 2539 Euro per 1% increase in survival, or 7692 Euro per additional life year. Conclusion: Current results support the thesis that Carbon ion RT, although more expensive, is at least as cost-effective as advanced photon therapies for these patients. Ion RT, however, offers substantial benefits for the patients such as improved control rates and less severe side effects

Malignant transformation of clival chordoma after gamma knife surgery. Case report

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Tsuboi, Yoshifumi; Hayashi, Nakamasa; Kurimoto, Masanori; Nagai, Shoichi; Sasahara, Masakiyo; Endo, Shunro

2007-01-01

A 54-year-old woman presented a midline clival tumor manifesting as right abducens palsy in May 1997. Magnetic resonance (MR) imaging revealed a midline clival tumor. She underwent surgery twice with the transsphenoidal approach and gamma knife surgery for residual tumor. The histological diagnosis was chordoma. MR imaging revealed that the tumor had extended to the right cerebellopontine angle, with spinal seeding in February 2002. She underwent partial removal of the right cerebellopontine angle tumor. The histological diagnosis was chordoma with slight nuclear atypism. She died 5 years and 5 months after the first gamma knife surgery. Autopsy revealed multiple areas of spinal seeding. Histological examination confirmed malignant transformation with unique epithelial characteristics, possibly caused by gamma knife surgery. (author)

Is there a role for conventional MRI and MR diffusion-weighted imaging for distinction of skull base chordoma and chondrosarcoma?

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Müller, Uta; Kubik-Huch, Rahel A; Ares, Carmen; Hug, Eugen B; Löw, Roland; Valavanis, Antonios; Ahlhelm, Frank J

2016-02-01

Chordoma and chondrosarcoma are locally invasive skull base tumors with similar clinical symptoms and anatomic imaging features as reported in the literature. To determine differentiation of chordoma and chondrosarcoma of the skull base with conventional magnetic resonance imaging (cMRI) and diffusion-weighted MR imaging (DWI) in comparison to histopathological diagnosis. This retrospective study comprised 96 (chordoma, n = 64; chondrosarcoma, n = 32) patients with skull base tumors referred to the Paul Scherrer Institute (PSI) for proton therapy. cMRI signal intensities of all tumors were investigated. In addition, median apparent diffusion coefficient (ADC) values were measured in a subgroup of 19 patients (chordoma, n = 11; chondrosarcoma, n = 8). The majority 81.2% (26/32) of chondrosarcomas displayed an off-midline growth pattern, 18.8% (6/32) showed clival invasion, 18.8% (6/32) were located more centrally. Only 4.7% (3/64) of chordomas revealed a lateral clival origin. Using cMRI no significant differences in MR signal intensities were observed in contrast to significantly different ADC values (subgroup of 19/96 patients examined by DWI), with the highest mean value of 2017.2 × 10(-6 )mm(2)/s (SD, 139.9( )mm(2)/s) for chondrosarcoma and significantly lower value of 1263.5 × 10(-6 )mm(2)/s (SD, 100.2 × 10(-6 )mm(2)/s) for chordoma (P = 0.001/median test). An off-midline growth pattern can differentiate chondrosarcoma from chordoma on cMRI in a majority of patients. Additional DWI is a promising tool for the differentiation of these skull base tumors. © The Foundation Acta Radiologica 2015.

Radioresistance of chordoma cells is associated with the ATM/ATR pathway, in which RAD51 serves as an important downstream effector.

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Zhang, Chao; Wang, Bing; Li, Lei; Li, Yawei; Li, Pengzhi; Lv, Guohua

2017-09-01

Surgery followed by radiotherapy is the standard treatment for chordomas, which are a rare but low-grade type of bone cancer arising from remnants of the embryonic notochord. However, disease recurrence following radiotherapy is common, most likely due to endogenous DNA repair mechanisms that promote cell survival upon radiation strikes. The ataxia telangiectasia mutated/ataxia telangiectasia mutated and Rad3 related (ATM/ATR)-mediated pathway has a critical role in DNA repair mechanisms; however, it has rarely been investigated in chordomas. In the present study, the expression of signal molecules related to the ATM/ATR pathway in chordoma tissues and adjacent normal tissues were initially examined using immunohistochemistry and western blot analysis. Chordoma U-CH1 and U-CH2 cells were subsequently used to investigate cell responses to ionizing radiation and the potential protective actions mediated by the ATM/ATR pathway. Phosphorylated (p)-ATM, p-ATR, γ-H2A histone family, member X (H2AX) and RAD51 were significantly upregulated in chordoma tissues relative to adjacent normal tissues (PATM, γ-H2AX and RAD51 expression in U-CH1 cells (PATM, p-ATR and RAD51 levels in U-CH2 cells (PATM/ATR pathway, in which RAD51 serves as an important downstream effector. Thus, RAD51 presents a promising therapeutic target for improving the outcome of radiotherapy treatment in chordomas.

Effectiveness and Safety of Spot Scanning Proton Radiation Therapy for Chordomas and Chondrosarcomas of the Skull Base: First Long-Term Report

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Ares, Carmen; Hug, Eugen B.; Lomax, Antony J.; Bolsi, Alessandra; Timmermann, Beate; Rutz, Hans Peter; Schuller, Jan C.; Pedroni, Eros; Goitein, Gudrun

2009-01-01

Purpose: To evaluate effectiveness and safety of spot-scanning-based proton radiotherapy (PT) in skull-base chordomas and chondrosarcomas. Methods and Materials: Between October 1998 and November 2005, 64 patients with skull-base chordomas (n = 42) and chondrosarcomas (n = 22) were treated at Paul Scherrer Institute with PT using spot-scanning technique. Median total dose for chordomas was 73.5 Gy(RBE) and 68.4 Gy(RBE) for chondrosarcomas at 1.8-2.0 Gy(RBE) dose per fraction. Local control (LC), disease specific survival (DSS), and overall survival (OS) rates were calculated. Toxicity was assessed according to CTCAE, v. 3.0. Results: Mean follow-up period was 38 months (range, 14-92 months). Five patients with chordoma and one patient with chondrosarcoma experienced local recurrence. Actuarial 5-year LC rates were 81% for chordomas and 94% for chondrosarcomas. Brainstem compression at the time of PT (p = 0.007) and gross tumor volume >25 mL (p = 0.03) were associated with lower LC rates. Five years rates of DSS and OS were 81% and 62% for chordomas and 100% and 91% for chondrosarcomas, respectively. High-grade late toxicity consisted of one patient with Grade 3 and one patient with Grade 4 unilateral optic neuropathy, and two patients with Grade 3 central nervous system necrosis. No patient experienced brainstem toxicity. Actuarial 5-year freedom from high-grade toxicity was 94%. Conclusions: Our data indicate safety and efficacy of spot-scanning based PT for skull-base chordomas and chondrosarcomas. With target definition, dose prescription and normal organ tolerance levels similar to passive-scattering based PT series, complication-free, tumor control and survival rates are at present comparable.

Brachyury, SOX-9, and Podoplanin, New Markers in the Skull Base Chordoma Vs Chondrosarcoma Differential: A Tissue Microarray Based Comparative Analysis

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Oakley, GJ; Fuhrer, K; Seethala, RR

2014-01-01

The distinction between chondrosarcoma and chordoma of the skull base/head and neck is prognostically important; however, both have sufficient morphologic overlap to make distinction difficult. As a result of gene expression studies, additional candidate markers have been proposed to help in this distinction. Hence, we sought to evaluate the performance of new markers: brachyury, SOX-9, and podoplanin alongside the more traditional markers glial fibrillary acid protein, carcinoembryonic antigen, CD24 and epithelial membrane antigen. Paraffin blocks from 103 skull base/head and neck chondroid tumors from 70 patients were retrieved (1969-2007). Diagnoses were made based on morphology and/or whole section immunohistochemistry for cytokeratin and S100 protein yielding 79 chordomas (comprising 45 chondroid chordomas and 34 conventional chordomas), and 24 chondrosarcomas. A tissue microarray containing 0.6 mm cores of each tumor in triplicate was constructed using a manual array (MTA-1, Beecher Instruments). For visualization of staining, the ImmPRESS detection system (Vector Laboratories) with 2 - diaminobenzidine substrate was used. Sensitivities and specificities were calculated for each marker. Core loss from the microarray ranged from 25-29% yielding 66-78 viable cases per stain. The classic marker, cytokeratin, still has the best performance characteristics. When combined with brachyury, accuracy improves slightly (sensitivity and specificity for detection of chordoma 98% and 100%, respectively). Positivity for both epithelial membrane antigen and AE1/AE3 had a sensitivity of 90% and a specificity of 100% for detecting chordoma in this study. SOX-9 is apparently common to both notochordal and cartilaginous differentiation, and is not useful in the chordoma-chondrosarcoma differential diagnosis. Glial fibrillary acid protein, carcinoembryonic antigen, CD24, and epithelial membrane antigen did not outperform other markers, and are less useful in the diagnosis of

Incidence, treatment, and survival patterns for sacral chordoma in the United States, 1974-2011

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Esther Yu

2016-09-01

Full Text Available IntroductionSacral chordomas represent one half of all chordomas, a rare neoplasm of notochordal remnants. Current NCCN guidelines recommend surgical resection with or without adjuvant radiotherapy, or definitive radiation for unresectable cases. Recent advances in radiation for chordomas include conformal photon and proton beam radiation. We investigated incidence, treatment, and survival outcomes to observe any trends in response to improvements in surgical and radiation techniques over a near 40 year time period.Materials and Methods345 microscopically confirmed cases of sacral chordoma were identified between 1974 and 2011 from the Surveillance, Epidemiology, and End Results (SEER program of the National Cancer Institute. Cases were divided into three cohorts by calendar year, 1974-1989, 1990-1999, and 2000-2011, as well as into two groups by age less than or equal to 65 versus greater than 65 to investigate trends over time and age via Chi-square analysis. Kaplan-Meier analyses were performed to determine effects of treatment on survival. Multivariate Cox regression analysis was performed to determine predictors of overall survival.Results5-year overall survival for the entire cohort was 60.0%. Overall survival correlated significantly with treatment modality, with 44% surviving at 5 years with no treatment, 52% with radiation alone, 82% surgery alone, and 78% surgery and radiation (p<.001. Age greater than 65 was significantly associated with non-surgical management with radiation alone or no treatment (p<.001. Relatively fewer patients received radiation between 2000 and 2011 compared to prior time periods (p=.03 versus surgery, for which rates which did not vary significantly over time (p=.55. However, 5-year overall survival was not significantly different by time period. Age group and treatment modality were predictive for overall survival on multivariate analysis (p<.001. ConclusionSurgery remains an important component in the

Endoscopic Transoral Resection of an Axial Chordoma: A Case Report

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Taran S

2015-11-01

Full Text Available Upper cervical chordoma (UCC is rare condition and poses unique challenges to surgeons. Even though transoral approach is commonly employed, a minimally invasive technique has not been established. We report a 44-year old Malay lady who presented with a 1 month history of insidious onset of progressive neck pain without neurological symptoms. She was diagnosed to have an axial (C2 chordoma. Intralesional resection of the tumour was performed transorally using the Destandau endoscopic system (Storz, Germany. Satisfactory intralesional excision of the tumour was achieved. She had a posterior fixation of C1-C4 prior to that. Her symptoms improved postoperatively and there were no complications noted. She underwent adjuvant radiotherapy to minimize local recurrence. Endoscopic excision of UCC via the transoral approach is a safe option as it provides an excellent magnified view and ease of resection while minimizing the operative morbidity.

Proton beam therapy in the management of skull base chordomas: systematic review of indications, outcomes, and implications for neurosurgeons.

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Matloob, Samir A; Nasir, Haleema A; Choi, David

2016-08-01

Chordomas are rare tumours affecting the skull base. There is currently no clear consensus on the post-surgical radiation treatments that should be used after maximal tumour resection. However, high-dose proton beam therapy is an accepted option for post-operative radiotherapy to maximise local control, and in the UK, National Health Service approval for funding abroad is granted for specific patient criteria. To review the indications and efficacy of proton beam therapy in the management of skull base chordomas. The primary outcome measure for review was the efficacy of proton beam therapy in the prevention of local occurrence. A systematic review of English and non-English articles using MEDLINE (1946-present) and EMBASE (1974-present) databases was performed. Additional studies were reviewed when referenced in other studies and not available on these databases. Search terms included chordoma or chordomas. The PRISMA guidelines were followed for reporting our findings as a systematic review. A total of 76 articles met the inclusion and exclusion criteria for this review. Limitations included the lack of documentation of the extent of primary surgery, tumour size, and lack of standardised outcome measures. Level IIb/III evidence suggests proton beam therapy given post operatively for skull base chordomas results in better survival with less damage to surrounding tissue. Proton beam therapy is a grade B/C recommended treatment modality for post-operative radiation therapy to skull base chordomas. In comparison to other treatment modalities long-term local control and survival is probably improved with proton beam therapy. Further, studies are required to directly compare proton beam therapy to other treatment modalities in selected patients.

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

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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.

A potential therapy for chordoma via antibody-dependent cell-mediated cytotoxicity employing NK or high-affinity NK cells in combination with cetuximab.

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Fujii, Rika; Schlom, Jeffrey; Hodge, James W

2018-05-01

OBJECTIVE Chordoma is a rare bone tumor derived from the notochord and is resistant to conventional therapies such as chemotherapy, radiotherapy, and targeting therapeutics. Expression of epidermal growth factor receptor (EGFR) in a large proportion of chordoma specimens indicates a potential target for therapeutic intervention. In this study the authors investigated the potential role of the anti-EGFR antibody cetuximab in immunotherapy for chordoma. METHODS Since cetuximab is a monoclonal antibody of the IgG1 isotype, it has the potential to mediate antibody-dependent cell-mediated cytotoxicity (ADCC) employing natural killer (NK) cells as effectors. Polymorphisms in the CD16 allele expressed on NK cells have been shown to influence the degree of ADCC of tumor cells, with the high-affinity valine (V)/V allele being responsible for more lysis than the V/phenylalanine (F) or FF allele. Unfortunately, however, only approximately 10% of the population expresses the VV allele on NK cells. An NK cell line, NK-92, has now been engineered to endogenously express IL-2 and the high-affinity CD16 allele. These irradiated high-affinity (ha)NK cells were analyzed for lysis of chordoma cells with and without cetuximab, and the levels of lysis observed in ADCC were compared with those of NK cells from donors expressing the VV, VF, and FF alleles. RESULTS Here the authors demonstrate for the first time 1) that cetuximab in combination with NK cells can mediate ADCC of chordoma cells; 2) the influence of the NK CD16 polymorphism in cetuximab-mediated ADCC for chordoma cell lysis; 3) that engineered haNK cells-that is, cells transduced to express the CD16 V158 FcγRIIIa receptor-bind cetuximab with similar affinity to normal NK cells expressing the high-affinity VV allele; and 4) that irradiated haNK cells induce ADCC with cetuximab in chordoma cells. CONCLUSIONS These studies provide rationale for the use of cetuximab in combination with irradiated haNK cells for therapy for

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

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Shahab Kamali Ardekani

2012-02-01

Full Text Available Introduction: notochord. Although histologically benign, these tumors are locally aggressiveand present significant managment challenges . There arew some studies onevaluated the location, age and gender of the patients with Chordoma in tworeferral centers in Tehran.chordoma cases but there was no study about Iranian cases. In this study weSkull base chordomas are rare neoplasms arising from theMethods: (Shariati and Imam Hospitals, Tehran from 2001 to 2011 was retrospectivelyreviewed.A database of patients with chordoma tumors referred to two centersResults: women, and they are most commonly diagnosed in middle-aged (mean age was50.6. Tumors typically occur in the axial skeleton and have a tendency for thespheno-occipital region of the skull base and sacral region. In adults 33.3% ofchordomas involve the sacrococcygeal region, 53% occured at the base of theskull near the spheno-occipital area, and near 14% were found in the vertebralcolumn. The cranial nerves mostly affected were abducens, oculomotor andtrochlear, with some overlaps. All patients were treated with surgery and somecases referred for gamma-knife radiosurgery (GKS.In our subjects tumors affect men nearly twice as frequently asDiscussion: to females that is different from other studies, however, few studies reportedmore male to female ratio. Despite the progress in current surgical techniquesand some encouraging results with the use of targeted therapy, disease controland long-term prognosis of patients are still poor.Findings of this study showed more involvement of males compare

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

International Nuclear Information System (INIS)

Yamaguchi, Takehiko; Yamato, Minoru; Saotome, Koichi

2002-01-01

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.)

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

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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.

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

Proton Therapy for Reirradiation of Progressive or Recurrent Chordoma

International Nuclear Information System (INIS)

McDonald, Mark W.; Linton, Okechuckwu R.; Shah, Mitesh V.

2013-01-01

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 3 (range, 0-701 cm 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 toxicities may

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

International Nuclear Information System (INIS)

Kondziolka, D.; Lunsford, L.D.; Flickinger, J.C.

1991-01-01

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

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

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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.

Clival chordomas: considerations after 16 years of endoscopic endonasal surgery.

Science.gov (United States)

Zoli, Matteo; Milanese, Laura; Bonfatti, Rocco; Faustini-Fustini, Marco; Marucci, Gianluca; Tallini, Giovanni; Zenesini, Corrado; Sturiale, Carmelo; Frank, Giorgio; Pasquini, Ernesto; Mazzatenta, Diego

2018-02-01

OBJECTIVE In the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach. METHODS All consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter. Postoperative adjuvant therapies were considered. RESULTS Sixty-five patients (male/female ratio 1:0.9) were included in this study. The median age was 48 years (range 9-80 years). Gross-total resection (GTR) was achieved in 47 cases (58.7%). On univariate analysis, primary procedures (p = 0.001), location in the superior or middle third of the clivus (p = 0.043), extradural location (p = 0.035), and histology of conventional chordomas (p = 0.013) were associated with a higher rate of GTR. The complication rate was 15.1%, and there were no perioperative deaths. Most complications did not result in permanent sequelae and included 2 CSF leaks (2.5%), 5 transient cranial nerve VI palsies (6.2%), and 2 internal carotid artery injuries (2.5%), which were treated with coil occlusion of the internal carotid artery without neurological deficits. Three patients (3.8%) presented with complications resulting in permanent neurological deficits due to a postoperative hematoma (1.2%) causing a hemiparesis, and 2 permanent ophthalmoplegias (2.5%). Seventeen patients (26.2%) have died of tumor progression over the course of follow-up (median 52 months, range 7-159 months). Based on Kaplan-Meier analysis, the survival rate was 77% at 5 years and 57% at 10

Imaging features of posterior mediastinal chordoma in a child

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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.)

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.

Sacral chordomas: Impact of high-dose proton/photon-beam radiation therapy combined with or without surgery for primary versus recurrent tumor

International Nuclear Information System (INIS)

Park, Lily; De Laney, Thomas F.; Liebsch, Norbert J.; Hornicek, Francis J.; Goldberg, Saveli; Mankin, Henry; Rosenberg, Andrew E.; Rosenthal, Daniel I.; Suit, Herman D.

2006-01-01

Purpose: To assess the efficacy of definitive treatment of sacral chordoma by high-dose proton/photon-beam radiation therapy alone or combined with surgery. Methods and Materials: The records of 16 primary and 11 recurrent sacral chordoma patients treated from November 1982 to November 2002 by proton/photon radiation therapy alone (6 patients) or combined with surgery (21 patients) have been analyzed for local control, survival, and treatment-related morbidity. The outcome analysis is based on follow-up information as of 2005. Results: Outcome results show a large difference in local failure rate between patients treated for primary and recurrent chordomas. Local control results by surgery and radiation were 12/14 vs. 1/7 for primary and recurrent lesions. For margin-positive patients, local control results were 10 of 11 and 0 of 5 in the primary and recurrent groups, respectively; the mean follow-up on these locally controlled patients was 8.8 years (4 at 10.3, 12.8, 17, and 21 years). Radiation alone was used in 6 patients, 4 of whom received ≥73.0 Gy (E); local control was observed in 3 of these 4 patients for 2.9, 4.9, and 7.6 years. Conclusion: These data indicate a high local control rate for surgical and radiation treatment of primary (12 of 14) as distinct from recurrent (1 of 7) sacral chordomas. Three of 4 chordomas treated by ≥73.0 Gy (E) of radiation alone had local control; 1 is at 91 months. This indicates that high-dose proton/photon therapy offers an effective treatment option

Whole-Body MRI Virtual Autopsy Using Diffusion-weighted Imaging With Background Suppression (DWIBS) at 3 T in a Child Succumbing to Chordoma.

Science.gov (United States)

Andronikou, Savvas; Kemp, Marnie L; Meiring, Michelle

2017-03-01

We report the use of diffusion-weighted imaging with background suppression (DWIBS) in pediatric virtual magnetic resonance imaging (MRI) autopsy of a child who succumbed to chordoma. A 10-year-old girl who succumbed to relapse of a chordoma underwent whole-body virtual MRI autopsy 12 hours postmortem with short Tau inversion recovery (STIR) and DWIBS on 3 T, which demonstrated the primary mass, local and cardiac invasion, and metastatic disease to the thorax, abdomen, head/neck, and musculoskeletal system. Postmortem virtual MRI autopsy including DWIBS successfully demonstrated the transthoracic spread of chordoma and invasion of the heart, resulting in blood-borne metastases. Motion and respiratory artifact were not factors during virtual autopsy using DWIBS on 3 T, making ideal use of this technology.

Skull base chordomas: analysis of dose-response characteristics

International Nuclear Information System (INIS)

Niemierko, Andrzej; Terahara, Atsuro; Goitein, Michael

1997-01-01

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

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

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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.

Osseous metastases of chordoma: imaging and clinical findings

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Chang, Connie; Torriani, Martin; Bredella, Miriam [Massachusetts General Hospital, Division of Musculoskeletal Imaging and Intervention, Department of Radiology, Boston, MA (United States); Chebib, Ivan [Massachusetts General Hospital, Department of Pathology, Boston, MA (United States)

2017-03-15

To describe the imaging and clinical characteristics of chordoma osseous metastases (COM). Our study was IRB approved and HIPAA compliant. A retrospective search of our pathology database for pathology-proven COM yielded 15 patients who had undergone MRI, CT, bone scan, and/or FDG-PET/CT. The imaging and clinical features of the COMs were recorded. A control group of age and gender matched chordoma patients without osseous metastasis was evaluated. The COM mean maximal dimension was 6.4 ± 4.0 cm. The majority (60%) of patients had one lesion. Extra-osseous soft tissue component was present in 85% and was larger than intra-osseous component in 76%. On MRI the lesions were heterogeneous but predominantly T2 hyperintense with hypointense septae, and with variable enhancement. On CT the lesions were typically destructive or permeative; calcifications were rare. The extent of the soft tissue component was isodense to muscle on CT and therefore better evaluated on MRI. COM was in a body part contiguous to the site of the primary tumor. Compared to the controls, COM patients were more likely to have local recurrence (P = 0.0009) and positive resection margins (P = 0.002). At 1 year, 33% of COM patients were deceased and 13% had progressive metastases. COM are associated with large extra-osseous soft tissue components, which are better visualized by MRI. They are often located in a body part contiguous to the site of the primary tumor, portend poor prognosis, and are associated with positive resection margins and local recurrence. (orig.)

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

OpenAIRE

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

2016-01-01

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 ch...

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.

Cervical chordoma with vertebral artery encasement mimicking neurofibroma: MRI findings

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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.)

Cervical chordoma with vertebral artery encasement mimicking neurofibroma: MRI findings

International Nuclear Information System (INIS)

Mortele, B.; Lemmerling, M.; Mortele, K.; Verstraete, K.; Defreyne, L.; Kunnen, M.; Vandekerckhove, T.

2000-01-01

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.)

Chordoma: review of clinico radiological features and factors affecting survival

International Nuclear Information System (INIS)

Soo, M.Y.S.

2001-01-01

This study reviews the clinico radiological features of cranial and sacrospinal chordomas and identifies factors affecting survival. Nineteen patients seen between January 1980 and December 2000 with histopathological diagnosis of chordomas were retrospectively reviewed with reference to clinical presentation, imaging features, treatment modalities and post-therapy status. Eight had tumours in the skull base while 11 patients had spinal and sacro-coccygeal lesions. Surgical resection was performed in 16 patients whose subsequent natural history was used to identify clinical indicators that may influence survival. Completeness of resection, age, gender and postoperative irradiation were subjected to analysis using the Cox proportional hazard models. Kaplan-Meir survival curves illustrate the survival distributions. Diplopia and facial pain are prime clinical presentations in cranial lesions, while extremity weakness and a sacrogluteal mass are common complaints in the sacrospinal group. Lesional calcifications are present in 40% while an osteolytic soft tissue mass is detectable by CT in all cases. Heterogeneous signals and internal septations on T 2 -weighted MRI are predominant features. In sacrospinal tumours, complete excision with adjuvant radiotherapy achieves the best results with a disease-free survival of more than 5 years. The clinical and imaging findings in this study are in accordance with those of other series. Except for complete surgical excision followed by radiotherapy in the subset of patients with sacrospinal tumours, none of the other clinical indicators show a statistical significant influence on survival. Copyright (2001) Blackwell Science Pty Ltd

Multidisciplinary management of clival chordomas; long-term clinical outcome in a single-institution consecutive series

DEFF Research Database (Denmark)

Förander, Petter; Bartek, Jiri; Fagerlund, Michael

2017-01-01

with an intracranial chordoma were treated at the Karolinska University Hospital, Stockholm, Sweden. Sixteen of 22 were treated with Gamma Knife radiosurgery (GKRS) for tumour residual or progression during the disease course. Seven of 22 received adjuvant fractionated radiotherapy and 5 of these also received proton...

Natural history and surgical treatment of chordoma: a retrospective cohort study

Directory of Open Access Journals (Sweden)

Samuel Aguiar Júnior

Full Text Available CONTEXT AND OBJECTIVE: Chordoma is a rare tumor with a high risk of locoregional recurrences. The aim of this study was analyze the long-term results from treating this pathological condition.DESIGN AND SETTING: Cohort study in a single hospital in São Paulo, Brazil.METHODS: This was a retrospective cohort study on 42 patients with chordoma who were treated at Hospital A. C. Camargo between 1980 and 2006. The hospital records were reviewed and a descriptive analysis was performed on the clinical-pathological variables. Survival curves were estimated using the Kaplan-Meier method and these were compared using the log-rank test.RESULTS: Nineteen patients were men and 23 were women. Twenty-five tumors (59.5% were located in the sacrum, eleven (26.2% in the skull base and six (14.3% in the mobile spine. Surgery was performed on 28 patients (66.7%. The resection was considered to have negative margins in 14 cases and positive margins in 14 cases. The five-year overall survival (OS was 45.4%. For surgical patients, the five-year OS was 64.3% (82.2% for negative margins and 51.9% for positive margins. In the inoperable group, OS was 37.7% at 24 months and 0% at five years.CONCLUSION: Complete resection is related to local control and definitively has a positive impact on long-term survival.

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

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

International Nuclear Information System (INIS)

Ferraresi, Virginia; Biagini, Roberto; Nuzzo, Carmen; Zoccali, Carmine; Marandino, Ferdinando; Vidiri, Antonello; Salducca, Nicola; Zeuli, Massimo; Giannarelli, Diana; Cognetti, Francesco

2010-01-01

Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported. We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008. 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. 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, however, essential to succeed in improving patients

Combination of photon and proton radiation therapy for chordomas and chondrosarcomas of the skull base: the Centre de Protontherapie D'Orsay experience

International Nuclear Information System (INIS)

Noeel, Georges; Habrand, Jean-Louis; Mammar, Hamid; Pontvert, Dominique; Haie-Meder, Christine; Hasboun, Dominique; Moisson, Patricia; Ferrand, Regis; Beaudre, Anne; Boisserie, Gilbert; Gaboriaud, Genevieve; Mazal, Alexandre; Kerody, Katia; Schlienger, Michel; Mazeron, Jean-Jacques

2001-01-01

Purpose: Prospective analysis of local tumor control, survival, and treatment complications in 44 consecutive patients treated with fractionated photon and proton radiation for a chordoma or chondrosarcoma of the skull base. Methods and Materials : Between December 1995 and December 1998, 45 patients with a median age of 55 years (14-85) were treated using a 201-MeV proton beam at the Centre de Protontherapie d'Orsay, 34 for a chordoma and 11 for a 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 the gross tumor volume was 67 cobalt Gray equivalent (CGE) (range: 60-70). Results: With a mean follow-up of 30.5 months (range: 2-56), the 3-year local control rates for chordomas and chondrosarcomas were 83.1% and 90%, respectively, and 3-year overall survival rates were 91% and 90%, respectively. Eight patients (18%) failed locally (7 within the clinical tumor volume and 1 unknown). Four patients died of tumor and 2 others of intercurrent disease. In univariate analysis, young age at time of radiotherapy influenced local control positively (p < 0.03), but not in multivariate analysis. Only 2 patients presented Grade 3 or 4 complications. Conclusion: In skull-base chordomas and chondrosarcomas, the combination of photons with a proton boost of one-third the total dose offers an excellent chance of cure at the price of an acceptable toxicity. These results should be confirmed with a longer follow-up

Extended maxillotomy for skull base access in contemporary management of chordomas: Rationale and technical aspect.

Science.gov (United States)

Abdul Jalil, Muhammad Fahmi; Story, Rowan D; Rogers, Myron

2017-05-01

Minimally invasive approaches to the central skull base have been popularized over the last decade and have to a large extent displaced 'open' procedures. However, traditional skull base surgery still has its role especially when dealing with a large clival chordoma where maximal surgical resection is the principal goal to maximize patient survival. In this paper, we present a case of a 25year-old male patient with chordoma in the inferior clivus which was initially debulked via a transnasal endoscopic approach. He unfortunately had a large recurrence of tumor requiring re-do resection. With the aim to achieve maximal surgical resection, we then chose the technique of a transoral approach with Le Fort 1 maxillotomy and midline palatal split. Post-operative course for the patient was uneventful and post-operative MRI confirmed significant debulking of the clival lesion. The technique employed for the surgical procedure is presented here in detail as is our experience over two decades using this technique for tumors, inflammatory lesions and congenital abnormalities at the cranio-cervical junction. Copyright © 2017 Elsevier Ltd. All rights reserved.

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

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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

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

International Nuclear Information System (INIS)

Hauptman, Jason S.; Barkhoudarian, Garni; Safaee, Michael; Gorgulho, Alessandra; Tenn, Steven; Agazaryan, Nzhde; Selch, Michael; De Salles, Antonio A.F.

2012-01-01

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

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

International Nuclear Information System (INIS)

Noel, G.; Jauffret, E.; Mammar, H.; Ferrand, R.; Habrand, J.L.; Crevoisier, R. de; Haie-Meder, C.; Beaudre, A.; Dederke, S.; Hasboun, D.; Boisserie, G.; Pontvert, D.; Gaboriaud, G.; Guedea, F.; Petriz, L.; Mazeron, J.J.

2003-01-01

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 indicators

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.

Degenerative Pannus Mimicking Clival Chordoma Resected via an Endoscopic Transnasal Approach.

Science.gov (United States)

Khaldi, Ahmad; Griauzde, Julius; Duckworth, Edward A M

2011-05-01

Lesions of the lower clivus represent a technically challenging subset of skull base disease that requires careful treatment. A 75-year-old woman with tongue atrophy was referred for resection of a presumed clival chordoma. The lesion was resected via an endoscopic transnasal transclival approach with no complications. Pathology revealed only chronic inflammatory tissue consistent with a degenerative pannus. Degenerative pannus should be included in the differential diagnosis of lower clival extradural lesions. The endoscopic transnasal transclival corridor should be considered for resection of such lesions as an alternative to larger, more morbid, traditional skull base approaches.

The analysis of the effective of preserving sacral nerve root during surgical treatment of chordoma

International Nuclear Information System (INIS)

Ji Yiming; Chen Kangwu; Yang Huilin; Zhu Lifan

2010-01-01

Objective: To analyze the effective of preserving sacral nerve root during surgical treatment of sacral chordoma. Methods: This retrospective study included 30 cases of sacral chordomas. All the cases were operated with posterior approach. The blood loss and blood transfusion during operation, the drainged blood after operation were reviewed. The sphincter muscle function of bladder and bowl were observed. Results: Tremendous reduction of blood loss during surgery was found in all cases, the blood loss was 1280 ml in average, the blood transfusion was 1080 ml in average, the drainged blood after ope-ration was 650 ml. Nine patients whose sacral nerve roots had been reserved bilaterally at and above S 3 level, the sphincter muscle function of bladder and bowl was good, whereas the function of sphincter muscle impaired in the other 21 patients and in one case colostomy and ureterocutaneostomy were used. Conclusion: Preoperative arterial embolization is effective method and can lead to excellent results. Even if the tumor is relatively huge and the upper resection margin is as high as at S 1 or S 2 level, the tumor can be removed successfully by posterior approach. Sacral nerve should be preserved as possible. (authors)

Struniak w okolicy kości krzyżowej u 47 – letniej pacjentki – opis przypadku = Chordoma in the sacral spine in an 47-year-old woman – case report

Directory of Open Access Journals (Sweden)

Kamila Woźniak

2016-09-01

• 2.        Katedra i Klinika Rehabilitacji, Collegium Medicum im. Ludwika Rydygiera w Bydgoszczy, Uniwersytet Mikołaja Kopernika w Toruniu     Streszczenie   Struniaki (łac. chordoma są rzadkimi, histologicznie łagodnymi, lecz klinicznie złośliwymi nowotworami szkieletu osiowego. Mogą powstać w dowolnym miejscu w linii środkowej ciała, gdzie istniała struna grzbietowa (np. stok, nosogardziel, siodło i okolica okołosiodłowa, otwór wielki kręgi i okolica krzyżowo-ogonowa. Struniaki to wolno rosnące nowotwory, wykazujące miejscową agresywność w stosunku do kości i tkanek miękkich. Struniaka po raz pierwszy opisał w 1856 roku Virchow i Luschke. Z kolei w 1858 roku Muller stwierdził, że nowotwór może pochodzić z pozostałości embrionalnych prymitywnej struny grzbietowej tzw. chorda dorsalis. Autorzy przedstawiają opis przypadku klinicznego leczonego operacyjne w Klinice Neurochirurgii, Neurotraumatologii i Neurochirurgii Dziecięcej Szpitala Uniwersyteckiego nr 1 im. dr A. Jurasza w Bydgoszczy z rozpoznanym w badaniu histopatologicznym struniakiem.   Słowa kluczowe: struniak, chrodoma, nowotwór, szkielet osiowy, neurochirurgia.     Abstract   Chordomas are rare, histologically benign but clinically malignant tumors of the axial skeleton. They can occur anywhere in the midline of the body where there notochord (clivus, nasopharynx, saddle and surroundings parasellar, the foramen vertebrae and surroundings sacral area. Chordomas are slow-growing cancer, showing local aggressiveness relative to bone and soft tissue. Chordoma was first described in 1856 by Virchow and Luschke. In turn, in 1858 Muller found that the cancer can come from embryonic remnants of the primitive notochord called chorda dorsalis. The authors present a clinical case operating treated in the Department of Neurosurgery, Neurotraumatology and Pediatric Neurosurgery, Dr Antoni Jurasz University Hospital No.1 in Bydgoszcz histopathological diagnosed

A Prospective Outcomes Study of Proton Therapy for Chordomas and Chondrosarcomas of the Spine

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Indelicato, Daniel J., E-mail: dindelicato@floridaproton.org [Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida (United States); Rotondo, Ronny L.; Begosh-Mayne, Dustin [Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida (United States); Scarborough, Mark T.; Gibbs, C. Parker [Department of Orthopedics and Rehabilitation, University of Florida College of Medicine, Gainesville, Florida (United States); Morris, Christopher G.; Mendenhall, William M. [Department of Radiation Oncology, University of Florida College of Medicine, Jacksonville, Florida (United States)

2016-05-01

Purpose: To evaluate the effectiveness of definitive or adjuvant external beam proton therapy on survival in patients with chordomas and chondrosarcomas of the spine. Methods and Materials: Between March 2007 and May 2013, 51 patients with a median age of 58 years (range, 22-83 years) with chordoma (n=34) or chondrosarcomas (n=17) of the sacrum (n=21), the cervical spine (n=20), and the thoracolumbar spine (n=10) were treated with external beam proton therapy to a median dose of 70.2 Gy(RBE) [range, 64.2-75.6 Gy(RBE)] at our institution. Distant metastases, overall survival, cause-specific survival, local control, and disease-free survival were calculated. Results: The mean follow-up time was 3.7 years (range, 0.3-7.7 years). Across all time points, 25 patients experienced disease recurrence: 18 local recurrences, 6 local and distant recurrences, and 1 distant metastasis. The 4-year rates of overall survival and cause-specific survival were 72%; disease-free survival was 57%, local control was 58%, and freedom from distant metastases was 86%. The median time to local progression was 1.7 years (range, 0.2-6.0 years), and the median time to distant progression was 1.6 years (range, 0.2-6.0 years). The risk factors for local recurrence were age ≤58 years (62% vs 26%; P=.04) and recurrence after prior surgery (29% vs 81%; P=.01). Secondary cancers developed in 2 patients: B-cell lymphoma 5.5 years after treatment and bladder cancer 2 years after treatment. We observed the following toxicities: sacral soft tissue necrosis requiring surgery (n=2), T1 vertebral fracture requiring fusion surgery (n=1), chronic urinary tract infections (n=1), surgery for necrotic bone cyst (n=1), and grade 2 bilateral radiation nephritis (n=1). Conclusion: High-dose proton therapy controls more than half of spinal chordomas and chondrosarcomas and compares favorably with historic photon data. Local progression is the dominant mode of treatment failure and may be reduced by

Carbon Ion Radiation Therapy for Unresectable Sacral Chordoma: An Analysis of 188 Cases

Energy Technology Data Exchange (ETDEWEB)

Imai, Reiko, E-mail: r_imai@nirs.go.jp [Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba (Japan); Kamada, Tadashi [Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba (Japan); Araki, Nobuhito [Department of Orthopedic Surgery, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Abe, Satoshi; Iwamoto, Yukihide; Ozaki, Toshifumi; Kanehira, Chihiro; Kaya, Mitsunori; Takahashi, Kazuhisa; Chuman, Hirokazu; Tsujii, Hirohiko; Tsuneyoshi, Masazumi; Nishida, Yoshihiro; Hiraga, Hiroaki; Hiruma, Toru; Machinami, Rikuo; Matsumine, Akihiko; Matsumoto, Seiichi; Morioka, Hideo; Yamaguchi, Takehiko; and others

2016-05-01

Purpose: To evaluate the results of carbon ion radiation therapy administered to 188 patients with unresectable primary sacral chordomas. Patients and Methods: One hundred eighty-eight patients were treated with carbon ion radiation therapy at a single institute between 1996 and 2013 and retrospectively analyzed. The median age was 66 years. The highest proximal invasion reached past S2 level in 137 patients. The median clinical target volume was 345 cm{sup 3}. One hundred six patients received 67.2 gray equivalents (GyE)/16 fractions (fr), 74 patients received 70.4 GyE/16 fr, 7 patients received 73.6 GyE/16 fr, and 1 patient received 64.0 GyE/16 fr. Results: The median follow-up period was 62 months (range, 6.8-147.5 months). Seventy percent of patients were followed for 5 years or until death. The 5-year local control, overall survival, and disease-free survival rates were 77.2%, 81.1%, and 50.3%, respectively. Forty-one patients had a local recurrence. Sex, tumor volume, level of proximal invasion, and irradiated dose were unrelated to local control. There was grade 3 toxicity of the peripheral nerves in 6 patients and grade 4 toxicity of the skin in 2 patients. Ambulation remained in 97% of patients. Conclusions: Carbon ion radiation therapy was safe and effective for unresectable chordoma and provided good local control and survival while preserving ambulation.

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

Updated Outcome and Analysis of Tumor Response in Mobile Spine and Sacral Chordoma Treated With Definitive High-Dose Photon/Proton Radiation Therapy

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Kabolizadeh, Peyman, E-mail: peyman.kabolizadeh@beaumont.org [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Chen, Yen-Lin; Liebsch, Norbert [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Hornicek, Francis J.; Schwab, Joseph H. [Department of Orthopedic Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Choy, Edwin [Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Rosenthal, Daniel I. [Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States); Niemierko, Andrzej; DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts (United States)

2017-02-01

Purpose: Treatment of spine and sacral chordoma generally involves surgical resection, usually in conjunction with radiation therapy. In certain circumstances where resection may result in significant neurologic or organ dysfunction, patients can be treated definitively with radiation therapy alone. Herein, we report the outcome and the assessment of tumor response to definitive radiation therapy. Methods and Materials: A retrospective analysis was performed on 40 patients with unresected chordoma treated with photon/proton radiation therapy. Nineteen patients had complete sets of imaging scans. The soft tissue and bone compartments of the tumor were defined separately. Tumor response was evaluated by the modified Response Evaluation Criteria in Solid Tumors (RECIST) and volumetric analysis. Results: With a median follow-up time of 50.3 months, the rates of 5-year local control, overall survival, disease-specific survival, and distant failure were 85.4%, 81.9%, 89.4%, and 20.2%, respectively. Eighty-four computed tomographic and magnetic resonance imaging scans were reviewed. Among the 19 patients, only 4 local failures occurred, and the median tumor dose was 77.4 GyRBE. Analysis at a median follow-up time of 18 months showed significant volumetric reduction of the total target volume (TTV) and the soft tissue target volume (STTV) within the first 24 months after treatment initiation, followed by further gradual reduction throughout the rest of the follow-up period. The median maximum percentage volumetric regressions of TTV and STTV were 43.2% and 70.4%, respectively. There was only a small reduction in bone target volume over time. In comparison with the modified RECIST, volumetric analysis was more reliable, more reproducible, and could help in measuring minimal changes in the tumor volume. Conclusion: These results continue to support the use of high-dose definitive radiation therapy for selected patients with unresected spine and sacral chordomas

Updated Outcome and Analysis of Tumor Response in Mobile Spine and Sacral Chordoma Treated With Definitive High-Dose Photon/Proton Radiation Therapy

International Nuclear Information System (INIS)

Kabolizadeh, Peyman; Chen, Yen-Lin; Liebsch, Norbert; Hornicek, Francis J.; Schwab, Joseph H.; Choy, Edwin; Rosenthal, Daniel I.; Niemierko, Andrzej; DeLaney, Thomas F.

2017-01-01

Purpose: Treatment of spine and sacral chordoma generally involves surgical resection, usually in conjunction with radiation therapy. In certain circumstances where resection may result in significant neurologic or organ dysfunction, patients can be treated definitively with radiation therapy alone. Herein, we report the outcome and the assessment of tumor response to definitive radiation therapy. Methods and Materials: A retrospective analysis was performed on 40 patients with unresected chordoma treated with photon/proton radiation therapy. Nineteen patients had complete sets of imaging scans. The soft tissue and bone compartments of the tumor were defined separately. Tumor response was evaluated by the modified Response Evaluation Criteria in Solid Tumors (RECIST) and volumetric analysis. Results: With a median follow-up time of 50.3 months, the rates of 5-year local control, overall survival, disease-specific survival, and distant failure were 85.4%, 81.9%, 89.4%, and 20.2%, respectively. Eighty-four computed tomographic and magnetic resonance imaging scans were reviewed. Among the 19 patients, only 4 local failures occurred, and the median tumor dose was 77.4 GyRBE. Analysis at a median follow-up time of 18 months showed significant volumetric reduction of the total target volume (TTV) and the soft tissue target volume (STTV) within the first 24 months after treatment initiation, followed by further gradual reduction throughout the rest of the follow-up period. The median maximum percentage volumetric regressions of TTV and STTV were 43.2% and 70.4%, respectively. There was only a small reduction in bone target volume over time. In comparison with the modified RECIST, volumetric analysis was more reliable, more reproducible, and could help in measuring minimal changes in the tumor volume. Conclusion: These results continue to support the use of high-dose definitive radiation therapy for selected patients with unresected spine and sacral chordomas

Influence of Residual Tumor Volume and Radiation Dose Coverage in Outcomes for Clival Chordoma

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); Moore, Michael G.; Ting, Jonathan Y. [Department of Otolaryngology, Head and Neck Surgery, Indiana University School of Medicine, Indianapolis, Indiana (United States); Cohen-Gadol, Aaron A.; Shah, Mitesh V. [Department of Neurological Surgery, Indiana University School of Medicine, Indianapolis, Indiana (United States); Goodman Campbell Brain and Spine, Indianapolis, Indiana (United States)

2016-05-01

Purpose: The purpose of this study was to evaluate factors associated with tumor control in clival chordomas. Methods and Materials: A retrospective review of 39 patients treated with surgery and proton therapy for clival chordomas between 2004 and 2014 was performed. The median prescribed dose was 77.4 Gy (relative biological effectiveness [RBE]); range was 70.2-79.2 Gy (RBE). Minimum and median doses to gross tumor volume (GTV), radiation dose received by 1 cm{sup 3} of GTV (D1cm{sup 3}), and the equivalent uniform dose were calculated. Receiver operating characteristics curves evaluated the predictive sensitivity and specificity for local failure of potential cutpoint values for GTV and D1cm{sup 3}. Results: After a median follow-up of 51 months, the 5-year estimate of local control (LC) was 69.6% (95% confidence interval [CI] 50.0%-89.2%), and overall survival (OS) was 81.4% (95% CI: 65.3%-97.5%). Tumor histology, GTV at the time of radiation, and prescribed radiation dose were significantly associated with local control on multivariate analysis, whereas D1cm{sup 3} was associated with overall survival. Compared to those patients whose conditions remained controlled, patients experiencing tumor failure had statistically significant larger GTVs and lower D1cm{sup 3}, and prescribed and median doses to GTV. A subset of 21 patients with GTV of ≤20 cm{sup 3} and D1cm{sup 3} of >67 Gy (RBE) had a median follow-up of 47 months. The 5-year estimate of local control in this subset was 81.1% (95% CI: 61.7%-100%; P=.004, overall comparison by GTV ≤20 cm{sup 3} stratified by D1cm{sup 3}). A D1cm{sup 3} of 74.5 Gy (RBE) had 80% sensitivity for local control and 60% specificity, whereas a GTV of 9.3 cm{sup 3} had 80% sensitivity for local control and 66.7% specificity. Conclusions: Local control of clival chordomas was associated with both smaller size of residual tumor and more complete high-dose coverage of residual tumor. Multidisciplinary care should seek

Clinical Outcome of Sacral Chordoma With Carbon Ion Radiotherapy Compared With Surgery

International Nuclear Information System (INIS)

Nishida, Yoshihiro; Kamada, Tadashi; Imai, Reiko; Tsukushi, Satoshi; Yamada, Yoshihisa; Sugiura, Hideshi; Shido, Yoji; Wasa, Junji; Ishiguro, Naoki

2011-01-01

Purpose: To evaluate the efficacy, post-treatment function, toxicity, and complications of carbon ion radiotherapy (RT) for sacral chordoma compared with surgery. Methods and Materials: The records of 17 primary sacral chordoma patients treated since 1990 with surgery (n = 10) or carbon ion RT (n = 7) were retrospectively analyzed for disease-specific survival, local recurrence-free survival, complications, and functional outcome. The applied carbon ion dose ranged from 54.0 Gray equivalent (GyE) to 73.6 GyE (median 70.4). Results: The mean age at treatment was 55 years for the surgery group and 65 years for the carbon ion RT group. The median duration of follow-up was 76 months for the surgery group and 49 months for the carbon ion RT group. The local recurrence-free survival rate at 5 years was 62.5% for the surgery and 100% for the carbon ion RT group, and the disease-specific survival rate at 5 years was 85.7% and 53.3%, respectively. Urinary-anorectal function worsened in 6 patients (60%) in the surgery group, but it was unchanged in all the patients who had undergone carbon ion RT. Postoperative wound complications requiring reoperation occurred in 3 patients (30%) after surgery and in 1 patient (14%) after carbon ion RT. The functional outcome evaluated using the Musculoskeletal Tumor Society scoring system revealed 55% in the surgery group and 75% in the carbon ion RT group. Of the six factors in this scoring system, the carbon ion RT group had significantly greater scores in emotional acceptance than did the surgery group. Conclusion: Carbon ion RT results in a high local control rate and preservation of urinary-anorectal function compared with surgery.

Chordoma in the jugular foramen area: a review of 16 cases%颈静脉孔区脊索瘤16例临床分析

Institute of Scientific and Technical Information of China (English)

田凯兵; 王亮; 郝淑煜; 李达; 王科; 吴震; 张力伟; 张俊廷

2013-01-01

Objective To analysis the clinical characteristics,surgical treatment and outcome of chordoma in the jugular foramen area.Methods The author retrospectively studied data from 16 patients with chordoma in the jugular foramen area who had undergone surgeries in the department of skullbase and brain stem of Beijing Tiantan Hospital between January 2006 and June 2011,and follow-up was performed through recheck in out-patient department,telephone and letter.Results There are 8 females and 8 males in this group and all of them underwent surgical procedures.The mean age is 39-year-old and the mean symptomatic history time was 18.3 months.The main clinical presentation is paralysis of the lower cranial nerves (Ⅸ,Ⅹ and Ⅺ,12 cases),6 patients also suffered from deficits in cranial nerve Ⅷ,other presentations are deficits in eyesight (4 cases),ataxia (3 cases),numbness of limbs (2 cases),facial paralysis (2 cases) and deficits of abducent nerve (1 case).Far lateral approach was used in 10 cases,retrosigmoidsuboccipital approach was used in 2 cases,infratemporal trans-tentorium of cerebellum approach in 2 cases and expanded middle cranial fossa approach in 2 cases.Gross total removal was achivevd in 5 cases,subtotal removal in 10 cases and partial resection 1 case.The pathologic type of 5 cases in this group is typical chordoma,and other 11 cases are chondroid chordoma.Follow-up revealed obvious improvement in 5 cases,permanent paralysis of the lower cranial nerves in 7 cases and facial paralysis in 1 case.Conclusions The main clinical presentation of patients with jugular foramen chordoma is paralysis of the lower cranial nerves and the main pathology of the chordoma in this area is chondroid type,choosing an appropriate approach and the protection of lower cranial nerve are important for a good prognosis.%目的 探讨颈静脉孔区脊索瘤的临床特点和手术疗效.方法 回顾性分析16�

Analysis of the relationship between tumor dose inhomogeneity and local control in patients with skull base chordoma

International Nuclear Information System (INIS)

Terahara, Atsuro; Niemierko, Andrzej; Goitein, Michael; Finkelstein, Dianne; Hug, Eugen; Liebsch, Norbert; O'Farrell, Desmond; Lyons, Sue; Munzenrider, John

1999-01-01

Purpose: When irradiating a tumor that abuts or displaces any normal structures, the dose constraints to those structures (if lower than the prescribed dose) may cause dose inhomogeneity in the tumor volume at the tumor-critical structure interface. The low-dose region in the tumor volume may be one of the reasons for local failure. The aim of this study is to quantitate the effect of tumor dose inhomogeneity on local control and recurrence-free survival in patients with skull base chordoma. Methods and Materials: 132 patients with skull base chordoma were treated with combined photon and proton irradiation between 1978 and 1993. This study reviews 115 patients whose dose-volume data and follow-up data are available. The prescribed doses ranged from 66.6 Cobalt-Gray-Equivalent (CGE) to 79.2 CGE (median of 68.9 CGE). The dose to the optic structures (optic nerves and chiasma), the brain stem surface, and the brain stem center was limited to 60, 64, and 53 CGE, respectively. We used the dose-volume histogram data derived with the three-dimensional treatment planning system to evaluate several dose-volume parameters including the Equivalent Uniform Dose (EUD). We also analyzed several other patient and treatment factors in relation to local control and recurrence-free survival. Results: Local failure developed in 42 of 115 patients, with the actuarial local control rates at 5 and 10 years being 59% and 44%. Gender was a significant predictor for local control with the prognosis in males being significantly better than that in females (P 0.004, hazard ratio = 2.3). In a Cox univariate analysis, with stratification by gender, the significant predictors for local control (at the probability level of 0.05) were EUD, the target volume, the minimum dose, and the D 5cc dose. The prescribed dose, histology, age, the maximum dose, the mean dose, the median dose, the D 90% dose, and the overall treatment time were not significant factors. In a Cox multivariate analysis, the

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

Outcomes following attempted en bloc resection of cervical chordomas in the C-1 and C-2 region versus the subaxial region: a multiinstitutional experience.

Science.gov (United States)

Molina, Camilo A; Ames, Christopher P; Chou, Dean; Rhines, Laurence D; Hsieh, Patrick C; Zadnik, Patricia L; Wolinsky, Jean-Paul; Gokaslan, Ziya L; Sciubba, Daniel M

2014-09-01

Chordomas involving the mobile spine are ideally managed via en bloc resection with reconstruction to optimize local control and possibly offer cure. In the cervical spine, local anatomy poses unique challenges, limiting the feasibility of aggressive resection. The authors present a multi-institutional series of 16 cases of cervical chordomas removed en bloc. Particular attention was paid to clinical outcome, complications, and recurrence. In addition, outcomes were assessed according to position of tumor at the C1-2 level versus the subaxial (SA) spine (C3-7). The authors reviewed cases involving patients who underwent en bloc resection of cervical chordoma at 4 large spine centers. Patients were included if the lesion epicenter involved the C-1 to C-7 vertebral bodies. Demographic data and details of surgery, follow-up course, exposure to adjuvant therapy, and complications were obtained. Outcome was correlated with presence of tumor in C1-2 versus subaxial spine via a Student t-test. Sixteen patients were identified (mean age at presentation 55 ± 14 years). Seven cases (44%) cases involved C1-2, and 16 involved the subaxial spine. Median survival did not differ significantly different between the C1-2 (72 months) and SA (60 months) groups (p = 0.65). A combined (staged anteroposterior) approach was used in 81% of the cases. Use of the combined approach was significantly more common in treatment of subaxial than C1-2 tumors (100% vs 57%, p = 0.04). En bloc resection was attempted via an anterior approach in 6% of cases (C1-2: 14.3%; SA: 0%; p = 0.17) and a posterior approach in 13% of cases (C1-2: 29%; SA: 0%; p = 0.09). The most commonly reported margin classification was marginal (56% of cases), followed by violated (25%) and wide (19%). En bloc excision of subaxial tumors was significantly more likely to result in marginal margins than excision of C1-2 tumors (C1-2: 29%; SA: 78%; p = 0.03). C1-2 tumors were associated with significantly higher rates of

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

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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.

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

International Nuclear Information System (INIS)

Mirra, J.M.; Brien, E.W.

2001-01-01

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.)

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

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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).

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

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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

Solitary intracranial plasmacytoma located in the spheno-clival region mimicking chordoma: a case report.

Science.gov (United States)

Liu, Z Y; Qi, X Q; Wu, X J; Luo, C; Lu, Y C

2010-01-01

Solitary intracranial plasmacytoma (SIP) is very rare. This case report presents serial findings of SIP located in the spheno-clival region in a 54-year old female who presented with an inferior hemianopia in the right eye and an enlarged physiological blind spot in both eyes. Based on the initial diagnosis of a spheno-clival region chordoma, the tumour was partially resected by the nasal-sphenoidal sinus approach. Subsequently, the correct diagnosis of SIP was made based on the pathology and immunohistochemical staining of the tumour. The patient was treated using a whole skull-base radiation therapy protocol with 45 Gy and she was in good physical condition during the subsequent 22 months. The findings of a series of similar case reports documenting SIP in 20 cases published from 1976 to 2008 are also reviewed. Based on these case reports, the key features of SIP, including their clinical manifestations, clinical imaging characteristics, treatment and prognosis, are described.

Improvement of the local control of spinal chordomas treated by surgery and targeted irradiation (CyberKnife{sup R}) on hypoxic cells marked with {sup 18}F-FMiso; Amelioration du controle local des chordomes du rachis traites par chirurgie et une irradiation (CyberKnife{sup R}) ciblee sur les cellules hypoxiques marquees au {sup 18}F-FMiso

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Mammar, H.; Kerrou, K.; Bondiau, P.Y.; Angellier, G.; Thariat, J.; Benezery, K.; Heroult, J.; Leysalle, A.; Gerard, J.P. [Centre Antoine-Lacassagne, Nice (France); Talbot, J.N. [Service de medecine nucleaire, Hopital Tenon, AP-HP, Paris (France)

2011-10-15

The authors report and comment the treatment of two women suffering from a recurring cervical spine chordoma. Each patient had a first PET (positron emission tomography) with {sup 18}F-fluorodeoxyglucose to assess the hyper-metabolic component and PET with {sup 18}F-FMiso to quantify the hypoxic component within the lesion. This last PET allows a non-invasive quantification of the hypoxic component which is potentially radio-resistant in cervical spine chordomas. It also allows an intelligent dose increase to improve the local control rate. Short communication

Skull base chordomas: treatment outcome and prognostic factors in adult patients following conformal treatment with 3D planning and high dose fractionated combined proton and photon radiation therapy

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Munzenrider, J E; Hug, E; McManus, P; Adams, J; Efird, J; Liebsch, N J

1995-07-01

Purpose: To report treatment outcome and prognostic factors for local recurrence-free survival and overall survival in adult patients with skull base chordomas treated with 3D planning and high dose fractionated combined proton and photon radiation therapy. Methods and Materials: From 1975 through 1993, 132 adult patients with skull base chordomas were treated with fractionated combined proton and photon radiation therapy. Seventy five patients (57%) were male and 57 (43%) female. Age ranged from 19 to 80 years (median 45.5 years). All pathology was verified at MGH by a single pathologist. Ninety six had non-chondroid (NCC) and 36 chondroid chordomas (CC), respectively. Median prescribed dose was 68.7 CGE (CGE, Cobalt Gray-equivalent: proton Gy X RBE 1.1 + photon Gy), ranging from 36 to 79.2 CGE; 95% received {>=} 66.6 CGE. Between 70 and 100% of the dose was given with the 160 MeV proton beam at the Harvard Cyclotron. 3D CT-based treatment planning has been employed in all patients treated since 1980. Median follow-up was 46 months (range 2-158 months). Results: Treatment outcome was evaluated in terms of local recurrence-free survival (LRFS) and disease specific survival (DSS), as well as treatment-related morbidity. Local failure (LF), defined as progressive neurological deficit with definite increase in tumor volume on CT or MRI scan, occurred in 39 patients (29.5%). LF was more common among women than among men:(26(57)) (46%) vs (13(75)) (17%), respectively. Thirty three of the 39 LF were seen in non-chondroid chordoma patients, with 6 occurring in patients with the chondroid variant (34% of NCC and 17% of CC), respectively. Distant metastasis was documented in 8 patients. LRFS was 81 {+-} 5.8%, 59 {+-} 8.3%, and 43 {+-} 10.4%, and DSS was 94 {+-} 3.6%, 80 {+-} 6.7%, and 50 {+-} 10.7% at 36, 60, and 96 months, respectively, for the total group. LRFS and DSS were not significantly different for patients with NCC than those with CC (p > .05). Gender was

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

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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.

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

International Nuclear Information System (INIS)

Kim, Joo Young; Choi, Myung Sun

1988-01-01

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

SU-F-T-221: An Assessment of the Potential for Improved Local Control of Skull- Base Chordomas Via Reduction of the Proton Beam Range Uncertainty

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Muller, L; Soldner, A; Kirk, M; Fager, M; Solberg, T; Robert, L; Dolney, D [University of Pennsylvania, Philadelphia, PA (United States)

2016-06-15

Purpose: The beam range uncertainty presents a special challenge for proton therapy. Novel technologies currently under development offer strategies to reduce the range uncertainty [1,2]. This work quantifies the potential advantages that could be realized by such a reduction for dosimetrically challenging chordomas at the base of skull. Therapeutic improvement was assessed by evaluating tumor control probabilities (TCP) and normal tissue complication probabilities (NTCP). Methods: Treatment plans were made for a modulated-scanned proton delivery technique using the Eclipse treatment planning system. The prescription dose was 7920 cGy to the CTV. Three different range uncertainty scenarios were considered: 5 mm (3.5% of the beam range + 1 mm, representing current clinical practice, “Curr”), 2 mm (1.3%), and 1 mm (0.7%). For each of 4 patients, 3 different PTVs were defined via uniform expansion of the CTV by the value of the range uncertainty. Tumor control probability (TCP) and normal tissue complication probabilities (NTCPs) for organs-at-risk (OARs) were calculated using the Lyman-Kutcher-Burman[3] formalism and published model parameters [ref Terahara[4], quantec S10, Burman Red Journal v21 pp 123]. Our plan optimization strategy was to achieve PTV close to prescription while maintaining OAR NTCP values at or better than the Curr plan. Results: The average TCP values for the 5, 2, and 1 mm range uncertainty scenarios are 51%, 55% and 65%. The improvement in TCP for patients was between 4 and 30%, depending primarily on the proximity of the GTV to OAR. The average NTCPs for the brainstem and cord were about 4% and 1%, respectively, for all target margins. Conclusion: For base of skull chordomas, reduced target margins can substantially increase the TCP without increasing the NTCP. This work demonstrates the potential significance of a reduction in the range uncertainty for proton beams.

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

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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

[Usefulness of neuroendoscopy and a neuronavigator for removal of clival chordoma].

Science.gov (United States)

Miyagi, A; Maeda, K; Sugawara, T

1998-02-01

We report a case of large clival chordoma. The patient was a 56-year-old male who was admitted to our hospital with left eye ptosis and diplopia of 2 months duration. On admission, neurological examinations revealed oculomotor nerve palsy of the left eye. Skull radiographs with polytomographs demonstrated marked destruction of the clivus. A plain computed tomography (CT) scan revealed a large iso-attenuated mass in the clivus, extending anteriorly into the sphenoidal sinus, superiorly into the suprasellar cistern, bilaterally into the petrous apex, posteriorly into the prepontine cistern and caudally into the foramen magnum. An enhanced CT scan demonstrated a slightly enhanced tumor. A high-resolution bone-window CT scan revealed marked destruction of the clivus and bilateral petrous apex. Magnetic resonance imaging (MRI) scans disclosed a large enhanced mass extending superiorly into the suprasellar cistern, bilaterally into the petrous apex and inferiorly into the foramen magnum. The tumor extended so widely that we decided on a one-stage operation via a transsphenoidal sublabial transseptal approach and transoral transpalatal approach. At surgery, we employed a neuronavigator and Codman 4-mm rigid neuroendoscope with 0 degree, 30 degrees and 70 degrees angled lenses. The tumor was very soft and suckable, and could be easily removed by applying CUSA, a pituitary curette and suction. The neuronavigator was particularly useful because the surgeon had a real-time two-dimensional representation of the position of the tip of this device in the corresponding imaging space intraoperatively. The neuroendoscope also proved useful, since remnant tumor tissues that could not be seen under an operating microscope were frequently recognized near or around the entrance of the tumor cavity, cavernous sinus region and petroclival junction area. The surgeon was able to remove these remnants safely by checking on the neuroendoscope monitor. The tumor was excised completely. The

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

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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.

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

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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.

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

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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

Chordome extra-axial : a propos d'une localisation latero tracheale ...

African Journals Online (AJOL)

Most chordomas arise in the sacrococcygeal and spheno-occipital region. Extranotochordal chordomas are extremely unusual. Case report : A 50 year women suffering from a left compressive cervical mass with a acute respiratory detress. in cervicotomy, it was an inextirpable tumor in contact with the left thyroid lobe and ...

Chordoma

African Journals Online (AJOL)

is bound to result in relief of pain and may even prolong life expectancy. .... regrowth of the tumour, and with the patients all asympto- matic. ..... Ten days later the tumour was much ... visible, his speech had improved, and he was back to doing.

Chondrosarcoma of the temporal bone. Diagnosis and treatment of 13 cases and review of the literature

International Nuclear Information System (INIS)

Coltrera, M.D.; Googe, P.B.; Harrist, T.J.; Hyams, V.J.; Schiller, A.L.; Goodman, M.L.

1986-01-01

Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with multiple sclerosis, glomus jugulare tumors, meningioma, and chordomas. The cranial nerve palsies frequently observed with the tumors are related to the anatomic locations of the tumors. Thirteen patients with this entity are presented and the eleven other cases in the literature are reviewed. Histologically the tumors are low grade and exhibit myxoid features. The myxoid features must be differentiated from chordoma and chondroid chordoma. The tumor locations preclude surgical excision and conventional radiation therapy can cause unacceptable neurologic sequelae. Proton beam therapy has been effective in short-term results and appears capable of avoiding serious neurologic side effects

Proton therapy for tumors of the skull base

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

1999-06-01

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

Proton therapy for tumors of the skull base

International Nuclear Information System (INIS)

Munzenrider, J.E.; Liebsch, N.J.

1999-01-01

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

Giant Vertebral Notochordal Rest: Magnetic Resonance and Diffusion Weighted Imaging Findings

International Nuclear Information System (INIS)

Oner, Ali Yusuf; Akpek, Sergin; Tali, Turgut; Ucar, Murat

2009-01-01

A giant vertebral notochordal rest is a newly described, benign entity that is easily confused with a vertebral chordoma. As microscopic notochordal rests are rarely found in adult autopsies, the finding of a macroscopic vertebral lesion is a new entity with only seven previously presented cases. We report here radiological findings, including diffusion weighted images, of a patient with a giant notochordal remnant confined to the L5 vertebra, with an emphasis on its distinction from a chordoma

Benign notochordal lesions of the axial skeleton: a review and current appraisal

International Nuclear Information System (INIS)

Kyriakos, Michael

2011-01-01

At the 1996 meeting of the International Skeletal Society, an idea was put forth that there existed symptomatic lesions of the axial skeleton, morphologically different from chordoma, that were consistent with benign notochordal remnants (rests). A review of the embryological basis for this concept is made, along with an analysis of these lesions, termed giant notochordal rests or benign notochordal cell tumors, that have been reported in the intervening 15 years, with a commentary on their relationship, if any, to chordoma. (orig.)

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

Distribution and retention of 35S-sodium sulfate in man

International Nuclear Information System (INIS)

Woodard, H.Q.; Pentlow, K.S.; Mayer, K.; Laughlin, J.S.; Marcove, R.C.

1976-01-01

Measurements were made of the 35 S content of tissues obtained from biopsies and autopsies made during and up to 6 months after treatment of chondrosarcoma or chordoma with carrier-free Na 2 35 SO 4 . Usually 70 to 90 percent of an intravenous dose was excreted in the urine during the first 3 days. The major component of the blood concentration had a biologic half-time of 0.4 to 0.7 days. The initial uptakes in chondrosarcoma, chordoma, and red bone marrow were high and nearly equal, but the rates of loss differed greatly. Uptake in epiphyseal cartilage was comparable to that in chondrosarcoma; uptake in other types of cartilage was lower, but subsequent loss was very slow. For an administered dose of 1 mCi per kilogram of body weight, the integrated radiation doses were 2.4 rads for blood, 33 rads for red bone marrow, 155 rads for chondrosarcoma, 49 rads for chordoma, and 135 rads for normal cartilage. Doses to muscle, skin, and fibrous tissue were 7 to 15 rads

Advanced chordoma treated by first-line molecular targeted therapies: Outcomes and prognostic factors. A retrospective study of the French Sarcoma Group (GSF/GETO) and the Association des Neuro-Oncologues d'Expression Française (ANOCEF).

Science.gov (United States)

Lebellec, Loïc; Chauffert, Bruno; Blay, Jean-Yves; Le Cesne, Axel; Chevreau, Christine; Bompas, Emmanuelle; Bertucci, François; Cupissol, Didier; Fabbro, Michel; Saada-Bouzid, Esma; Duffaud, Florence; Feuvret, Loïc; Bonneville-Levard, Alice; Bay, Jacques-Olivier; Vauleon, Elodie; Vinceneux, Armelle; Noel, Georges; Penel, Nicolas; Mir, Olivier

2017-07-01

To assess the role of first-line Molecular Targeted Therapies (MTTs) in Advanced chordoma (AC) patients. Retrospective study of 80 patients treated between January 2004 and December 2015 at 15 major French Sarcoma or Neurooncology Centres. The sex ratio M/F was 46/34. The median age was 59 (6-86) years. The primary sites were the sacrum (50, 62.5%), mobile spine (12, 15.0%), and skull base (18, 22.5%). Metastases were present in 28 patients (36.0%). The first line of MTTs consisted of imatinib (62, 77.5%), sorafenib (11, 13.7%), erlotinib (5, 6.3%), sunitinib (1, 1.2%) and temsirolimus (1, 1.2%). The reported responses were: partial response (5, 6.3%), stable disease (58, 72.5%), or progressive disease (10, 12.5%). Symptomatic improvement was seen in 28/66 assessable patients (42.4%) and was associated with an objective response occurrence (p = 0.005), imatinib (p = 0.020) or erlotinib use (p = 0.028). The median progression-free survival (PFS) was 9.4°months (95% CI, [6.8-16.1]). Two independent factors of poor prognosis for PFS were identified: a skull-based primary location (HR = 2.5, p = 0.019), and the interval between diagnosis and MTT of guide treatment decisions and design further clinical trials. Copyright © 2017 Elsevier Ltd. All rights reserved.

Myxoid chondrosarcoma of sphenoid bone

Directory of Open Access Journals (Sweden)

Amit K Chowhan

2012-01-01

Full Text Available The myxoid variant of chondrosarcoma is usually seen in soft tissues where it is known as chordoid sarcoma or parachordoma. Rarely, it involves bone and when it does, cranial bones are the preferred location. This tumor is frequently amalgamated with the chondroid variant of chordoma, especially when the lesion occurs in the sphenoid bone/spheno-occipital region, because of their similar clinical presentations, anatomical locations, radiological findings, and mistaken histopathological features. It is essential to distinguish myxoid chondrosarcoma from the chondroid variant of chordoma, because of the different treatment protocol and prognostic importance. We present such a location-based diagnostic dilemma, solved successfully with ancillary immunohistochemistry.

Notochord isolation using laser capture microdissection.

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Santegoeds, R G C; Yakkioui, Y; Jahanshahi, A; Raven, G; Van Overbeeke, J J; Herrler, A; Temel, Y

2017-03-01

Chordoma are malignant tumors of the axial skeleton, which arise from remnants of the notochord. The Notochord (chorda dorsalis) is an essential embryonic structure involved in the development of the nervous system and axial skeleton. Therefore, the notochord seems to be the most biologically relevant control tissue to study chordoma in molecular biology research. Nevertheless, up to now mainly different tissues but not the notochord have been used as control for chordoma, due to difficulty of isolating notochordal tissue. Here, we describe a fast and precise method of isolating notochordal cells. Examination of human fetuses, with a gestation of 9, 11 and 13 weeks, using (immuno)histochemical methods was performed. To isolate pure notochord cells for further molecular biology investigation five flash frozen fetuses between 9 and 10 weeks of gestation were dissected by microtome slicing. Thereafter pure notochord cells for further molecular biology investigation where harvested by using laser capture microdissection (LCM). RNA was extracted from these samples and used in quantitative PCR. This study illustrates notochord of embryonic spines in three different stages of gestation (9-11-13 weeks). Immunohistochemical staining with brachyury showed strong staining of the notochord, but also weak staining of the intervertebral disc and vertebral body. LCM of notochord slices and subsequent total RNA extraction resulted in a good yield of total RNA. qPCR analysis of two housekeeping genes confirmed the quality of the RNA. LCM is a fast and precise method to isolate notochord and the quality and yield RNA extracted from this tissue is sufficient for qPCR analysis. Therefore early embryo notochord isolated by LCM is suggested to be the gold standard for future research in chordoma development, classification and diagnosis. Copyright © 2016 Elsevier B.V. All rights reserved.

[Intracranial collision tumor--A case report (author's transl)].

Science.gov (United States)

Fukaya, T; Yoshida, J; Banno, T; Kageyama, N; Ito, M

1976-06-01

A 21-year-old man with nasopharyngeal tumor was first admitted to the Nagoya University Hospital on April 15, 1972. He had difficulty in speaking and swallowing, and developed double vision prior to admission. A soft and yellow tumor was found in the nasopharynx and revealed typical features of chordoma. The patient underwent Co60 irradiation after the operation. On January 25, 1973, the patient developed double vision of severe degree. Microscopic examination of the specimen which was obtained at the time of the second operation in February 9, 1973, disclosed a coexistence (collision) of chordoma and hemangioblastoma. The two different tumors were situated in juxtaposition on histological examination. Co60 irradiation was added during his second hospitalization. Three months after the second operation, he developed symptoms of meningitis and was hospitalized for the third time on June 3, 1973, at which time the tumor tissue extended through the right frontal and middle fossa. The third operation was done with frontal craniotomy and tumor was partially removed. The histological diagnosis was hemangioblastoma. Postoperatively the patient went downhill and died on September 19, 1973. The report of a collision tumor of intracranial chordoma and hemangioblastoma is not found in the previous literature. There have been many theories as to the origin of collision tumor. Some investigators have proposed that the existence of hyperplastic blood vessels within the glioblastoma is responsible for the collision tumor of sarcoma and glioblastoma. Since the advent of radiotherapy, several examples of sarcoma have been discovered at postmortem examination in patient irradiated for treatment of cerebral neoplasm, both gliogeneous and nongliogenous, suggesting a possible relationship between the tumor and the radiation therapy. In our case, the chordoma showed neither hyperplastic blood vessels nor malignant pattern on histological examination. It was suspected that post

Diagnosis of pelvic wall tumor on multislice CT

International Nuclear Information System (INIS)

Zhang Keyun; Deng Lequn; Lei Hongwei

2011-01-01

Objective: To evaluate the value of multi-slice CT (MSCT) in diagnosing pelvic wall tumors. Methods: MSCT of 21 cases of pelvic wall tumors including metastasis (10), neurogenic tumor (5), chondrosarcoma (2), chordoma (1), aneurysmal bone cyst (1), giant cell tumor (1), and osteochondroma (1) was retrospectively analyzed. Results: CT appearances of pelvic wall tumors include bony destruction and soft tissue masses. Common features were bone destruction in metastasis, expansion of the neuroforamen in neurogenic tumor, pleomorphic calcification in chondrosarcoma, lower sacral vertebral location of chordoma, iliac crest bone destruction in giant cell tumor, cauliflower-like nodules in osteochondroma. Conclusion: MSCT with three-dimensional volume rendering demonstrates well the tumor shape, size, extent, internal structure and relationship with the surrounding organs to aid diagnosis of pelvic wall tumors. (authors)

Acquired myospherulosis secondary to gluteal augmentation on fine needle aspiration cytology: A diagnostic challenge.

Science.gov (United States)

Alperstein, Susan; Dilcher, Thomas; Viswanathan, Kartik; Rao, Rema A; Siddiqui, Momin T; Giorgadze, Tamara

2018-05-01

A 30-year-old female presented with a three-month history of a multilocular cystic lesion over the lumbosacral spine. Fine-needle aspiration biopsy (FNA) of the lesion was performed at an outside institution, and a cytologic diagnosis, suspicious for chordoma, was rendered. The patient presented for surgical consultation at our institution. Repeat FNA demonstrated an unusual fat-like material. Upon further inquiry, the patient provided a recent history of gluteal contour improvement with fibroadipose tissue implants. A diagnosis of myospherulosis was made with a concurrent surgical pathology correlation. No evidence of chordoma was identified. To date, this is the first reported case of acquired myospherulosis in the context of gluteal contour enhancement and represents an important diagnostic pitfall to consider on cytology preparations. © 2017 Wiley Periodicals, Inc.

Stereotactic Body Radiotherapy for Head and Neck Tumors

Science.gov (United States)

2016-04-18

Squamous Cell Carcinoma of the Head and Neck; Nasopharyngeal Carcinoma; Salivary Gland Cancer; Head and Neck Sarcoma; Paraganglioma of Head and Neck; Chordoma of Head and Neck; Chondrosarcoma of Head and Neck; Angiofibroma of Head and Neck

Stereotactic gamma radiosurgery of brain tumors

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Kobayashi, Tatsuya; Kida, Yoshihisa; Tanaka, Takayuki; Oyama, Hirofumi; Yoshida, Kazuo; Maesawa, Satoshi; Kai, Osamu; Nakamura, Mototoshi; Arahata, Masashige [Komaki City Hospital, Aichi (Japan)

1996-06-01

One thousand cases with various head and neck diseases have been treated by gamma radiosurgery at Komaki City Hospital since May 1991. Five hundred and sixty-eight out of 1,000 cases were neoplastic lesions which consisted of 173 cases of neurinoma, 108 of metastatic tumors, 103 of meningioma, 69 of gliomas, 27 of pituitary adenoma, 26 of craniopharyngioma, 13 of pineal tumors, 11 of chordoma, 6 of malignant lymphoma, 5 of hemangioblastoma and so on. The most effective result has been shown in metastatic brain tumors. The complete response (disappearance of the lesion) was obtained in more than 50% of the treated lesions, and the control rate of 85% was maintained for more than 12 months. Next effective results were shown in craniopharyngioma, malignant pineal tumors and malignant lymphoma. There was a group which showed moderate response but no tumor disappearance. Those were pituitary adenoma, acoustic neurinoma, meningioma and chordoma. Gliomas showed less response and even progression of tumor at relatively higher rate. It has been found that malignant gliomas showed difficult control of the tumor and progression rate of 70%, while benign gliomas showed the control rate of more than 90%. Besides intracranial lesions, malignant skull base tumors such as chordoma, naso-pharyngeal cancer, adenoid cystic cancer showed better response to gamma radiosurgery and higher control rate for longer period of time with high QOL compaired to conventional irradiation. (author)

Experience with carbon ion radiotherapy at GSI

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Jaekel, O. [Division of Medical Physics in Radiation Therapy (E040), German Cancer Research Center, Deutsches Krebsforschungszentrum, Im Neuenheimer Feld 280, 69120 Heidelberg (Germany)]. E-mail: o.jaekel@dkfz.de; Schulz-Ertner, D. [Department of Radiation Oncology, University of Heidelberg, Heidelberg (Germany); Karger, C.P. [Division of Medical Physics in Radiation Therapy (E040), German Cancer Research Center, Deutsches Krebsforschungszentrum, Im Neuenheimer Feld 280, 69120 Heidelberg (Germany); Heeg, P. [Division of Medical Physics in Radiation Therapy (E040), German Cancer Research Center, Deutsches Krebsforschungszentrum, Im Neuenheimer Feld 280, 69120 Heidelberg (Germany); Debus, J. [Department of Radiation Oncology, University of Heidelberg, Heidelberg (Germany)

2005-12-15

At GSI, a radiotherapy facility was established using beam scanning and active energy variation. Between December 1997 and April 2004, 220 patients have been treated at this facility with carbon ions. Most patients are treated for chordoma and chondrosarcoma of the base of skull, using a dose of 60 Gye (Gray equivalent) in 20 fractions. Carbon ion therapy is also offered in a combination with conventional radiotherapy for a number of other tumors (adenoidcystic carcinoma, chordoma of the cervical spine and sacrum, atypical menningeoma). The patients treated for skull base tumors showed an overall local control rate after two years of 90%. The overall treatment toxicity was mild. This shows that carbon ion radiotherapy can safely be applied using a scanned beam and encouraged the Heidelberg university hospital to build a hospital based facility for ion therapy.

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

International Nuclear Information System (INIS)

Weber, A.L.

1991-01-01

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

[The efficacy of desmopressin in the treatment of central diabetes insipidus after resection of chiasmo-sellar region tumors].

Science.gov (United States)

Astaf'eva, L I

Central diabetes insipidus (CDI) is a neuroendocrine disease, the pathogenesis of which is associated with abnormal secretion of the antidiuretic hormone. One of the specific causes of CDI is neurosurgical resection of chiasmatic-sellar region tumors. to study the efficacy and safety of desmopressin in CDI patients after resection of chiasmatic-sellar region (CSR) tumors. Examination and treatment of patients were performed at a hospital for 7-14 days after surgery and then were continued after discharge. During treatment, the following tests were performed: a daily fluid intake and excretion volume, serum levels of sodium, potassium, and glucose twice a day, morning urine specific gravity, and Zimnitsky's test. Twenty-three patients with CSR tumors (11 craniopharyngiomas, 10 pituitary adenomas, 1 skull base chordoma, and 1 CSR meningioma) and CDI after neurosurgical treatment received desmopressin. On treatment, a thirst decrease, a reduced rate of diuresis, a reduced amount of excreted urine, and normalization of the sodium level were observed in all patients. In 12 patients (with pituitary adenoma, skull base chordoma, and meningioma) with transient CDI, desmopressin therapy was discontinued upon regression of symptoms 7-30 days after surgery. Eleven patients with permanent CDI continued to receive the drug at a dose of 1 to 4 doses per day. All patients well tolerated the drug without significant adverse effects. Therapy with desmopressin in the form of a nasal spray (vazomirin) in patients with transient and permanent CDI after resection CSR tumors of various histological nature (craniopharyngiomas, pituitary adenomas, meningiomas, and chordomas) was effective and safe in the early postoperative and long-term postoperative periods.

[Neurophysiological identification of the cranial nerves in endoscopic endonasal surgery of skull base tumors].

Science.gov (United States)

Shkarubo, A N; Ogurtsova, A A; Moshchev, D A; Lubnin, A Yu; Andreev, D N; Koval', K V; Chernov, I V

2016-01-01

Intraoperative identification of the cranial nerves is a useful technique in removal of skull base tumors through the endoscopic endonasal approach. Searching through the scientific literature found one pilot study on the use of triggered electromyography (t-EMG) for identification of the VIth nerve in endonasal endoscopic surgery of skull base tumors (D. San-Juan, et al, 2014). The study objective was to prevent iatrogenic injuries to the cranial nerves without reducing the completeness of tumor tissue resection. In 2014, 5 patients were operated on using the endoscopic endonasal approach. Surgeries were performed for large skull base chordomas (2 cases) and trigeminal nerve neurinomas located in the cavernous sinus (3). Intraoperatively, identification of the cranial nerves was performed by triggered electromyography using a bipolar electrode (except 1 case of chordoma where a monopolar electrode was used). Evaluation of the functional activity of the cranial nerves was carried out both preoperatively and postoperatively. Tumor resection was total in 4 out of 5 cases and subtotal (chordoma) in 1 case. Intraoperatively, the IIIrd (2 patients), Vth (2), and VIth (4) cranial nerves were identified. No deterioration in the function of the intraoperatively identified nerves was observed in the postoperative period. In one case, no responses from the VIth nerve on the right (in the cavernous sinus region) were intraoperatively obtained, and deep paresis (up to plegia) of the nerve-innervated muscles developed in the postoperative period. The nerve function was not impaired before surgery. The t-EMG technique is promising and requires further research.

Putative oncogene Brachyury (T) is essential to specify cell fate but dispensable for notochord progenitor proliferation and EMT.

Science.gov (United States)

Zhu, Jianjian; Kwan, Kin Ming; Mackem, Susan

2016-04-05

The transcription factor Brachyury (T) gene is expressed throughout primary mesoderm (primitive streak and notochord) during early embryonic development and has been strongly implicated in the genesis of chordoma, a sarcoma of notochord cell origin. Additionally, T expression has been found in and proposed to play a role in promoting epithelial-mesenchymal transition (EMT) in various other types of human tumors. However, the role of T in normal mammalian notochord development and function is still not well-understood. We have generated an inducible knockdown model to efficiently and selectively deplete T from notochord in mouse embryos. In combination with genetic lineage tracing, we show that T function is essential for maintaining notochord cell fate and function. Progenitors adopt predominantly a neural fate in the absence of T, consistent with an origin from a common chordoneural progenitor. However, T function is dispensable for progenitor cell survival, proliferation, and EMT, which has implications for the therapeutic targeting of T in chordoma and other cancers.

Brachyury Essential for Notochord Cell Fate, Not Proliferation or EMT | Center for Cancer Research

Science.gov (United States)

The Brachyury or T gene encodes a transcription factor that is essential for body axis elongation during embryonic development. T is also highly expressed in chordomas, rare sarcomas derived from notochord cells, and a number of additional tumor types, including lung, prostate, and colon cancers. 

Chondrosarcoma of the temporal bone: a case report

International Nuclear Information System (INIS)

Park, Man Soo; Lee, Sang Youl; Chung, Jae Gul; Lee, Deok Hee; Jung, Seung Mun; Ryu, Dae Sik

2001-01-01

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

Chondrosarcoma of the temporal bone: a case report

Energy Technology Data Exchange (ETDEWEB)

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

2001-07-01

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

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

Science.gov (United States)

DAUGAARD, SØREN; CHRISTENSEN, LISE H; HØGDALL, ESTRID

2009-01-01

Chondroid tumors comprise a heterogenous group of benign to overt malignant neoplasms, which may be difficult to differentiate from one another by histological examination. A group of 43 such tumors was stained with nine relevant antibodies in an attempt to find consistent marker profile(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) and extraskeletal myxoid chondrosarcomas (n=3) were positive for bcl-2. In contrast to all other tumors, two of three extraskeletal myxoid chondrosarcomas were also positive for CD17 and negative for osteonectin, cox-2, mdm-2 and actin. All five chordomas were negative for D2-40 and positive for mdm-2 and YKL-40. The diagnosis of chondrosarcoma may be aided by its positivity for D2-40 and YKL-40 and its lack of reactivity for actin and CD117. This should be seen in the light of no reaction for D2-40 in chordomas and a corresponding lack of reaction for osteonectin, cox-2, mdm-2 and actin in extraskeletal myxoid chondrosarcomas. A convincing immunoreactivity for calponin and/or actin in chondromyxoid fibromas and chondroblastomas may also be helpful in differentiating these tumors from chondrosarcomas. PMID:19594492

Transformed chest chardomas in malignant fibrous histiocytorme: presentation of case and reviewing of literature

International Nuclear Information System (INIS)

Capelastegui, A.; Mateos, B.; Astigarraga, E.; Pastor, A.; Pomposo, I.; Egurbide, M.V.

1994-01-01

Chest chordomas are rare neoplasms, and their transformation into malignant fibrous histiocytoma (MFH) is even more exceptional. We present a new case, including magnetic resonance (MR) images. The literature on the subject is reviewed, focussing especially on the dorsal location of these neoplasms and their possible malignant transformation, as well as the role of MR in the assessment of these lesions. (Author)

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.

Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma

Science.gov (United States)

2017-12-11

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

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

Isolated Petroclival Craniopharyngioma with Aggressive Skull Base Destruction

Science.gov (United States)

Lee, Young-Hen; Lim, Dong-Jun; Park, Jung-Yul; Chung, Yong-Gu; Kim, Young-Sik

2009-01-01

We report a rare case of petroclival craniopharyngioma with no connection to the sellar or suprasellar region. MRI and CT images revealed a homogenously enhancing retroclival solid mass with aggressive skull base destruction, mimicking chordoma or aggressive sarcoma. However, there was no calcification or cystic change found in the mass. Here, we report the clinical features and radiographic investigation of this uncommon craniopharyngioma arising primarily in the petroclival region. PMID:19881982

Case report: Greater meningeal inflammation in lumbar than in ventricular region in human bacterial meningitis

OpenAIRE

Naija, Walid; Matéo, Joaquim; Raskine, Laurent; Timsit, Jean-François; Lukascewicz, Anne-Claire; George, Bernard; Payen, Didier; Mebazaa, Alexandre

2004-01-01

Differences in the composition of ventricular and lumbar cerebrospinal fluid (CSF) based on single pairs of samples have previously been described. We describe a patient that developed post-surgical recurrent meningitis monitored by daily biochemical and bacteriological CSF analysis, simultaneously withdrawn from lumbar space and ventricles. A 20-year-old Caucasian man was admitted to the ICU after a resection of a chordoma that extended from the sphenoidal sinus to the anterior face of C2. C...

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.)

Radiological diagnostics of skeletal tumors

International Nuclear Information System (INIS)

Uhl, M.; Herget, G.W.

2008-01-01

The book contains contributions concerning the following topics: 1. introduction and fundamentals: WHO classification of bone tumors, imaging diagnostics and their function; localization, typical clinical and radiological criteria, TNM classification and status classification, invasive tumor diagnostics; 2. specific tumor diagnostics: chondrogenic bone tumors, osseous tumors, connective tissue bony tumors, osteoclastoma, osteomyelogenic bone tumors, vascular bone tumors, neurogenic bone tumors, chordoma; adamantinoma of the long tubular bone; tumor-like lesions, bony metastases, bone granulomas, differential diagnostics: tumor-like lesions

Distinguishing benign notochordal cell tumors from vertebral chordoma

International Nuclear Information System (INIS)

Yamaguchi, Takehiko; Iwata, Jun; Sugihara, Shinsuke; McCarthy, Edward F.; Karita, Michiaki; Murakami, Hideki; Kawahara, Norio; Tsuchiya, Hiroyuki; Tomita, Katsuro

2008-01-01

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.)

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.

The presence and absence of lymphatic vessels in the adult human intervertebral disc: relation to disc pathology

International Nuclear Information System (INIS)

Kliskey, Karolina; Williams, Kelly; Yu, J.; Urban, Jill; Athanasou, Nick; Jackson, David

2009-01-01

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.)

Single-energy intensity modulated proton therapy.

Science.gov (United States)

Farace, Paolo; Righetto, Roberto; Cianchetti, Marco

2015-10-07

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.

Single-energy intensity modulated proton therapy

International Nuclear Information System (INIS)

Farace, Paolo; Righetto, Roberto; Cianchetti, Marco

2015-01-01

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. (note)

Skull base chordoid meningioma: Imaging features and pathology

International Nuclear Information System (INIS)

Soo, Mark Y.S.; Gomes, Lavier; Ng, Thomas; Cruz, Malville Da; Dexter, Mark

2004-01-01

The clinical, imaging and pathological features of a skull base chordoid meningioma (CM) are described. The huge tumour resulted in obstructive hydrocephalus and partial erosion of the clivus such that a chordoma was suspected. The lesion's MRI findings were similar to those of a meningioma. Light microscopic, immunohistochemistry and ultrastructural features were diagnostic of CM. Chordoid meningioma is a rare subtype of meningioma and has a great tendency to recur should surgical resection be incomplete Copyright (2004) Blackwell Publishing Asia Pty Ltd

Locally challenging osteo- and chondrogenic tumors of the axial skeleton: results of combined proton and photon radiation therapy using three-dimensional treatment planning

Energy Technology Data Exchange (ETDEWEB)

Hug, Eugen B; Fitzek, Markus M; Liebsch, Norbert J; Munzenrider, John E

1995-02-01

Purpose: Tumors of the axial skeleton are at high risk for local failure. Total surgical resection is rarely possible. Critical normal tissues limit the efficacy of conventional photon therapy. This study reviews our experience of using combined high dose proton and photon radiation therapy following three-dimensional (3D) treatment planning. Methods and Materials: Between December 1980 and September 1992, 47 patients were treated at the Massachusetts General Hospital and Harvard Cyclotron Laboratory for primary or recurrent chordomas and chondrosarcomas (group 1, 20 patients), osteogenic sarcomas (group 2, 15 patients) and giant cell tumors, osteo- or chondroblastomas (group 3, 12 patients). Radiation treatment was given postoperatively in 23 patients, pre- and postoperatively in 17 patients, and 7 patients received radiation therapy as definitive treatment modality following biopsy only. The proton radiation component was delivered using a 160 MeV proton beam and the photon component using megavoltage photons up to 23 MV energy with 1.8-2.0 Cobalt Gray Equivalent (CGE) per fraction, once a day. Total external beam target dose ranged from 55.3 CGE to 82.0 CGE with mean target doses of 73.9 CGE (group 1), 69.8 CGE (group 2), and 61.8 CGE (group 3). Results: Group 1 (chordoma and chondrosarcoma): Five of 14 patients (36%) with chordoma recurred locally, and 2 out of 5 patients developed distant metastasis, resulting in 1 death from disease. A trend for improved local control was noted for primary vs. recurrent tumors, target doses > 77 CGE and gross total resection. All patients with chondrosarcoma achieved and maintained local control and disease-free status. Five-year actuarial local control and overall survival rates were 53% and 50% for chordomas and 100% and 100% for chondrosarcomas, respectively. Group 2 (osteogenic sarcoma): Three of 15 patients (20%) never achieved local control and died within 6 months of completion of radiation treatment. Only 1 out of 12

Imaging findings of sacral tumors

International Nuclear Information System (INIS)

Kim, Seung Ho; Hong, Sung Hwan; Choi, Ja Young; Koh, Sung Hye; Chung, Hye Won; Choi, Jung Ah; Kang, Heung Sik

2003-01-01

The various pathologic conditions detected at CT and MRI and subsumed by the term 'sacral tumor' include primary bone tumors, sacral canal tumors and metastases. Among these, metastases are much more common than primary bone tumors, of which chordoma is the most common. Although the imaging findings of sacral tumors are nonspecific, a patient's age and sex, and specific findings such as calcification or fluid-fluid levels, can help radiologists in their differential diagnosis. We describe the imaging findings of primary sacral tumors, emphasizing the MRI findings

Imaging of painful solitary lesions of the sacrum

International Nuclear Information System (INIS)

Peh, W. C. G.; Koh, W. L.; Kwek, J. W.; Htoo, M. M.; Tan, P. H.

2007-01-01

Full text: In patients with sacral pain, the painful symptoms may be caused by a variety of bony and soft tissue lesions. Benign lesions include giant cell tumour, neurogenic tumour, insufficiency fracture, infection and giant bone island. Malignant lesions include primary bone tumours, Ewing sarcoma, plasmacytoma, lymphoma and chordoma. Soft tissue tumours adjacent to or involving the sacrum may cause painful symptoms. A multimodality approach to imaging is required for full assessment of these lesions. This pictorial essay describes a range of common solitary sacral lesions that may cause pain, with emphasis on imaging features

L5 vertebrectomy for the surgical treatment of tumoral and traumatic lesions of L5 vertebra

Directory of Open Access Journals (Sweden)

Tuncay Kaner

2012-02-01

Full Text Available We retrospectively reviewed the clinical characteristics and the surgical results of seven patients treated with L5 vertebrectomy. The pathologies, clinical characteristics, preoperative and postoperative radiological findings, surgical techniques, and instrumentation for seven patients operated on between 1998 and 2009 are presented in this article. Biopsies were performed on all patients except those involving trauma. Patients were followed up at three-month intervals in the first year, at 6- month intervals in the second year, and on a regular basis afterward. One patient had a traumatic L5 burst fracture; the other six had tumoral pathologies in the L5 vertebrae. One tumoral lesion was a chordoma, another was a hemangioma, and the remaining four were metastatic lesions. Radiotherapy and chemotherapy were performed for the metastatic tumor patients during the postoperative period. Patients with renal cancer and chordoma survived for 3 years; patients with lung cancer and bladder cancer survived for 1 year; and patients with breast cancer survived for 16 months. The lumbosacral region presents significant stabilization problems because of the presence of sacral slope. In our opinion, if the lesion involves only the L5 vertebra, anterior cage-filled bone cement or bone graft should be performed, as dictated by the pathology and posterior transpedicular instrumentation. If the lesion involves the L4 vertebra or the sacrum and the L5 vertebra, the instrumentation can be extended to cover other segments with sacral attachments. The present cases involved only L5 vertebra and treatment with short-segment stabilization covering the anterior and posterior columns.

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

Directory of Open Access Journals (Sweden)

2006-01-01

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

The progress in radiotherapy techniques and it's clinical implications

International Nuclear Information System (INIS)

Reinfuss, M.; Walasek, T.; Byrski, E.; Blecharz, P.

2011-01-01

Three modem radiotherapy techniques were introduced into clinical practice at the onset of the 21 st century - stereotactic radiation therapy (SRT), proton therapy and carbon-ion radiotherapy. Our paper summarizes the basic principles of physics, as well as the technical reqirements and clinical indications for those techniques. SRT is applied for intracranial diseases (arteriovenous malformations, acoustic nerve neuromas, brain metastases, skull base tumors) and in such cases it is referred to as stereotactic radiosurgery (SRS). Techniques used during SRS include GammaKnife, CyberKnife and dedicated linacs. SRT can also be applied for extracranial disease (non-small cell lung cancer, lung metastases, spinal and perispinal tumors, primary liver tumors, breast cancer, pancreatic tumors, prostate cancer, head and neck tumors) and in such cases it is referred to as stereotactic body radiation therapy (SBRT). Eye melanomas, skull base and cervical spine chordomas and chordosarcomas, as well as childhood neoplasms, are considered to be the classic indications for proton therapy. Clinical trials are currently conducted to investigate the usefulness of proton beam in therapy of non-small cell lung cancer, prostate cancer, head and neck tumors, primary liver and oesophageal cancer Carbon-ion radiotherapy is presumed to be more advantageous than proton therapy because of its higher relative biological effectiveness (RBE) and possibility of real-time control of the irradiated volume under PET visualization. The basic indications for carbon-ion therapy are salivary glands neoplasms, selected types of soft tissue and bone sarcomas, skull base chordomas and chordosarcomas, paranasal sinus neoplasms, primary liver cancers and inoperable rectal adenocarcinoma recurrences. (authors)

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

Energy Technology Data Exchange (ETDEWEB)

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

2003-10-01

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

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.

Sacral Ewing's Sarcoma and Challenges in it's Diagnosis on MRI

Directory of Open Access Journals (Sweden)

Albert D'Souza

2009-01-01

Full Text Available A 15-yr old boy presented with low backache for 4 months associated with weakness of left lower limb. MRI of lumbosacral spine showed a sacral lesion with intraspinal and presacral soft tissue extension with neural compression. A diagnosis of tuberculosis was considered in the view of high prevalence in this part of the world, however biopsy revealed Ewing's sarcoma. Ewing's tumor of sacrum is rare, but should be suspected in low backache in children. Differential diagnosis for a sacral lesion includes tuberculosis, pyogenic osteomyelitis, lymphoma, chordoma, osteosarcoma and Ewing's sarcoma. MRI is sensitive in detecting these lesions but is nonspecific requiring histopathological examination for confirmation.

Case report 351: Aggressive osteoblastoma of the third lumbar vertebra

Energy Technology Data Exchange (ETDEWEB)

Abdelwahab, I.F.; Frankel, V.H.; Klein, M.J.

1986-02-01

In summary, a case is presented of an aggressive osteoblastoma affecting the third lumbar vertebra in a 65-year-old man. Because of the age of the patient, the aggressive, destructive nature of the tumor and the extensive involvement of the vertebral body, metastatic carcinoma as well as several primary malignancies were entertained in the differential diagnosis (e.g. plasmacytoma, chordoma, primary lymphoma of bone). However, the destructive, expanding nature of the lesion, the focal calcification within the area of destroyed bone, the production of new bone with dense sclerosis, raised the possibility of an osteoblastoma, despite the advanced age of the patient. Without the histological material, the type of osteoblastoma (aggressive) could not be diagnosed. (orig./SHA).

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. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Case report 351: Aggressive osteoblastoma of the third lumbar vertebra

International Nuclear Information System (INIS)

Abdelwahab, I.F.; Frankel, V.H.; Klein, M.J.

1986-01-01

In summary, a case is presented of an aggressive osteoblastoma affecting the third lumbar vertebra in a 65-year-old man. Because of the age of the patient, the aggressive, destructive nature of the tumor and the extensive involvement of the vertebral body, metastatic carcinoma as well as several primary malignancies were entertained in the differential diagnosis (e.g. plasmacytoma, chordoma, primary lymphoma of bone). However, the destructive, expanding nature of the lesion, the focal calcification within the area of destroyed bone, the production of new bone with dense sclerosis, raised the possibility of an osteoblastoma, despite the advanced age of the patient. Without the histological material, the type of osteoblastoma (aggressive) could not be diagnosed. (orig./SHA)

[Limitation of medical treatment and ethics in chronic recurrent Clivus chordoma].

Science.gov (United States)

Egger, Alexandra; Müller-Busch, H Christof

2008-01-01

In this paper ethical questions concerning the limitation and termination of medical treatment of comatose patients will be discussed on the basis of a case study. The team is confronted with extremely high communicative and ethical demands, since every person engaged in the treatment and care of the patient should take part in this decision making process. The final responsibility regarding the medical decisions, however, lies with the doctor in charge. In such cases advance directives or living wills are important and should be taken into consideration.

Stereotactic radiosurgery using the gamma knife

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Kawamoto, Shunsuke; Sasaki, Tomio; Matsutani, Masao; Takakura, Kintomo; Terahara, Atsuro (Tokyo Univ. (Japan). Faculty of Medicine)

1992-03-01

Since stereotactic radiosurgery using a gamma knife was developed in 1968 by Leksell, it has been used with increasing frequency in Japan. During the period from June 19, 1990 through December 20, 1991, 218 patients have been treated with stereotactic radiosurgery using a gamma knife. Of them, 116 had vascular lesions (116), including arteriovenous malformation (114), dural arteriovenous malformation (one), and cerebral aneurysm (one); and the other 102 had tumorous lesions, including acoustic neurinoma (48), meningioma (26), pituitary tumor (11), metastatic tumor (7), germ cell tumor (3), glioma (2), hemangioblastoma (2), chordoma (one), craniopharyngioma (one), and trigeminal neurinoma (one). In this article, candidates of stereotactic radiosurgery using a gamma knife are discussed, with particular attention to clinical results of the aforementioned 218 patients. (N.K.) 54 refs.

Imaging of sacral tumours

International Nuclear Information System (INIS)

Gerber, S.; Ollivier, L.; Brisse, H.; Neuenschwander, S.; Leclere, J.; Vanel, D.; Missenard, G.; Pinieux, G. de

2008-01-01

All components of the sacrum (bone, cartilage, bone marrow, meninges, nerves, notochord remnants, etc.) can give rise to benign or malignant tumours. Bone metastases and intraosseous sites of haematological malignancies, lymphoma and multiple myeloma are the most frequent aetiologies, while primary bone tumours and meningeal or nerve tumours are less common. Some histological types have a predilection for the sacrum, especially chordoma and giant cell tumour. Clinical signs are usually minor, and sacral tumours are often discovered in the context of nerve root or pelvic organ compression. The roles of conventional radiology, CT and MRI are described and compared with the histological features of the main tumours. The impact of imaging on treatment decisions and follow-up is also reviewed. (orig.)

Molecular pathology of bone tumours: diagnostic implications.

Science.gov (United States)

Puls, Florian; Niblett, Angela J; Mangham, D Chas

2014-03-01

Alongside histomorphology and immunohistochemistry, molecular pathology is now established as one of the cornerstones in the tissue diagnosis of bone tumours. We describe the principal molecular pathological techniques employed, and each of the bone tumour entities where their identified characteristic molecular pathological changes can be detected to support and confirm the suspected histological diagnosis. Tumours discussed include fibrous dysplasia, classical and subtype osteosarcomas, central and surface cartilaginous tumours, Ewing's sarcoma, vascular tumours, aneurysmal bone cyst, chordoma, myoepithelioma, and angiomatoid fibrous histiocytoma. This is a rapidly evolving field with discoveries occurring every few months, and some of the newer entities (the Ewing's-like sarcomas), which are principally identified by their molecular pathology characteristics, are discussed. © 2013 John Wiley & Sons Ltd.

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....

Imaging of sacral tumours

Energy Technology Data Exchange (ETDEWEB)

Gerber, S.; Ollivier, L.; Brisse, H.; Neuenschwander, S. [Institut Curie, Department of Radiology, Paris (France); Leclere, J. [Institut Gustave Roussy, Department of Radiology, Villejuif (France); Vanel, D. [The Rizzoli Institute, Department of Radiology, Bologna (Italy); Missenard, G. [Institut Gustave Roussy, Comite de pathologie tumorale de l' appareil locomoteur, Villejuif (France); Pinieux, G. de [CHRU de Tours, Department of Pathology, Hopital Trousseau, Tours (France)

2008-04-15

All components of the sacrum (bone, cartilage, bone marrow, meninges, nerves, notochord remnants, etc.) can give rise to benign or malignant tumours. Bone metastases and intraosseous sites of haematological malignancies, lymphoma and multiple myeloma are the most frequent aetiologies, while primary bone tumours and meningeal or nerve tumours are less common. Some histological types have a predilection for the sacrum, especially chordoma and giant cell tumour. Clinical signs are usually minor, and sacral tumours are often discovered in the context of nerve root or pelvic organ compression. The roles of conventional radiology, CT and MRI are described and compared with the histological features of the main tumours. The impact of imaging on treatment decisions and follow-up is also reviewed. (orig.)

Uterine carcinosarcoma associated with pelvic radiotherapy for sacral chordoma: A case report

Directory of Open Access Journals (Sweden)

Korhan Kahraman

2012-03-01

Conclusion: In uterine masses seen in patients with history of irradiation to the pelvic field, the probability of uterine sarcomas should always be kept in mind. These tumors may occur simultaneously with recurrence of primary tumor previously treated by adjuvant radiation therapy.

Particle Therapy Using Protons or Carbon Ions for Unresectable or Incompletely Resected Bone and Soft Tissue Sarcomas of the Pelvis

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Demizu, Yusuke, E-mail: y_demizu@nifty.com [Department of Radiology, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan); Jin, Dongcun; Sulaiman, Nor Shazrina; Nagano, Fumiko; Terashima, Kazuki; Tokumaru, Sunao [Department of Radiology, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan); Akagi, Takashi [Department of Radiation Physics, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan); Fujii, Osamu [Department of Radiation Oncology, Hakodate Goryokaku Hospital, Hakodate, Hokkaido (Japan); Daimon, Takashi [Department of Biostatistics, Hyogo College of Medicine, Nishinomiya, Hyogo (Japan); Sasaki, Ryohei [Division of Radiation Oncology, Kobe University Graduate School of Medicine, Kobe, Hyogo (Japan); Fuwa, Nobukazu [Department of Radiation Oncology, Ise Red Cross Hospital, Ise, Mie (Japan); Okimoto, Tomoaki [Department of Radiology, Hyogo Ion Beam Medical Center, Tatsuno, Hyogo (Japan)

2017-06-01

Purpose: To retrospectively analyze the treatment outcomes of particle therapy using protons or carbon ions for unresectable or incompletely resected bone and soft tissue sarcomas (BSTSs) of the pelvis. Methods and Materials: From May 2005 to December 2014, 91 patients with nonmetastatic histologically proven unresectable or incompletely resected pelvic BSTSs underwent particle therapy with curative intent. The particle therapy used protons (52 patients) or carbon ions (39 patients). All patients received a dose of 70.4 Gy (relative biologic effectiveness) in 32 fractions (55 patients) or 16 fractions (36 patients). Results: The median patient age was 67 years (range 18-87). The median planning target volume (PTV) was 455 cm{sup 3} (range 108-1984). The histologic type was chordoma in 53 patients, chondrosarcoma in 14, osteosarcoma in 10, malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma in 5, and other in 9 patients. Of the 91 patients, 82 had a primary tumor and 9 a recurrent tumor. The median follow-up period was 32 months (range 3-112). The 3-year rate of overall survival (OS), progression-free survival (PFS), and local control was 83%, 72%, and 92%, respectively. A Cox proportional hazards model revealed that chordoma histologic features and a PTV of ≤500 cm{sup 3} were significantly associated with better OS, and a primary tumor and PTV of ≤500 cm{sup 3} were significantly associated with better PFS. Ion type and number of fractions were not significantly associated with OS, PFS, or local control. Late grade ≥3 toxicities were observed in 23 patients. Compared with the 32-fraction protocol, the 16-fraction protocol was associated with significantly more frequent late grade ≥3 toxicities (18 of 36 vs 5 of 55; P<.001). Conclusions: Particle therapy using protons or carbon ions was effective for unresectable or incompletely resected pelvic BSTS, and the 32-fraction protocol was effective and relatively less toxic. Nevertheless, a

Computed tomography of the nasopharynx and related spaces. Part II: pathology

International Nuclear Information System (INIS)

Silver, A.J.; Mawad, M.E.; Hilal, S.K.; Sane, P.; Ganti, S.R.

1983-01-01

Malignant tumors of the nasopharynx were analyzed by region of origin and route of spread. Nasopharyngeal carcinomas produced early submucosal infiltration of the deglutitional muscle layer with enlargement of the levator palati muscle and lateral displacement of the parapharyngeal space. Serious otitis media was frequently associated, and the trigeminal nerve was occasionally involved. Intracranial extension via the foramen lacerum was frequent. Metastases to the infratemporal fossa produced early involvement of the masticatory muscle layer with medial displacement of the parapharygeal space. Adenoid cystic carcinomas showed late but disproportionate involvement of the sphenoid sinus. Chordomas extended into the retropharyngeal soft tissues via the petro-occipital fissure. Maxillary sinus carcinomas, which were very large at the time of presentation and impinged on the nasopharynx, showed extensive destruction of the pterygoid plates

Reconstruction of palatal defect using mucoperiosteal hinge flap and pushback palatoplasty.

Science.gov (United States)

Lee, S I; Lee, H S; Hwang, K

2001-11-01

This article describes a simple, new surgical technique to provide a complete two-layer closure of palatal defect resulting from a surgical complication of trans palatal resection of skull base chordoma. The nasal layer was reconstructed with triangular shape oral mucoperiosteal turn over hinge flap based on anterior margin of palatal defect and rectangular shaped lateral nasal mucosal hinge flaps. The oral layer was reconstructed with conventional pushback V-Y advancement 2-flaps palatoplasty. Each layer of the flaps were secured with two key mattress suture for flap coaptation. This technique has some advantages: simple, short operation time, one-stage procedure, no need of osteotomy. It can close small- to medium-sized palatal defect of palate or wide cleft palate and can prevent common complication of oronasal fistula, which could be caused by tension.

Parachordoma of Soft Tissues of the Arm: A Very Rare Tumour

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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.

The incidentaloma of the pituitary gland: Is neurosurgery required

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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.

The incidentaloma of the pituitary gland: Is neurosurgery required?

International Nuclear Information System (INIS)

Reincke, M.; Allolio, B.; Saeger, W.; Menzel, J.; Winkelmann, W.

1990-01-01

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

Radiation tolerance of the cervical spinal cord: incidence and dose-volume relationship of symptomatic and asymptomatic late effects following high dose irradiation of paraspinal tumors

International Nuclear Information System (INIS)

Liu, Mitchell C.C.; Munzenrider, John E.; Finkelstein, Dianne; Liebsch, Norbert; Adams, Judy; Hug, Eugen B.

1997-01-01

Purpose: Low grade chordomas and chondrosarcomas require high radiation doses for effective, lasting tumor control. Fractionated, 3-D planned, conformal proton radiation therapy has been used for lesions along the base of skull and spine to deliver high target doses, while respecting constraints of critical, normal tissues. In this study, we sought to determine the incidence of myelopathy after high dose radiotherapy to the cervical spine and investigated the influence of various treatment parameters, including dose-volume relationship. Methods and Materials: Between December 1980 and March 1996, 78 patients were treated at the Massachusetts General Hospital and Harvard Cyclotron Laboratory for primary or recurrent chordomas and chondrosarcomas of the cervical spine using combined proton and photon radiation therapy. In general, the tumor dose given was between 64.5 to 79.2 CGE (Cobalt Gray Equivalent). The guidelines for maximum permissible doses to spinal cord were: ≤ 64 CGE to the spinal cord surface and ≤ 53 CGE to the spinal cord center. Dose volume histograms of the spinal cord were analyzed to investigate a possible dose and volume relationship. Results: With a mean follow-up period of 46.6 months (range: 3 - 157 months), 4 of 78 patients (5.1%) developed high-grade (RTOG Grade 3 and 4) late toxicity: 3 patients (3.8%) experienced sensory deficits without motor deficits, none had any limitations of daily activities. One patient (1.2%) developed motor deficit with loss of motor function of one upper extremity. The only patient, who developed permanent motor damage had received additional prior radiation treatment and therefore received a cumulative spinal cord dose higher than the treatment guidelines. No patient treated within the guidelines experienced any motor impairment. Six patients (7.7%) experienced transient Lhermitt's syndrome and 1 patient (1.2%) developed asymptomatic radiographic MR findings only. Time to onset of symptoms of radiographic

En Bloc Resection of Primary Malignant Bone Tumor in the Cervical Spine Based on 3-Dimensional Printing Technology.

Science.gov (United States)

Xiao, Jian-Ru; Huang, Wen-Ding; Yang, Xing-Hai; Yan, Wang-Jun; Song, Dian-Wen; Wei, Hai-Feng; Liu, Tie-Long; Wu, Zhi-Peng; Yang, Cheng

2016-05-01

To investigate the feasibility and safety of en bloc resection of cervical primary malignant bone tumors by a combined anterior and posterior approach based on a three-dimensional (3-D) printing model. Five patients with primary malignant bone tumors of the cervical spine underwent en bloc resection via a one-stage combined anteroposterior approach in our hospital from March 2013 to June 2014. They comprised three men and two women of mean age 47.2 years (range, 26-67 years). Three of the tumors were chondrosarcomas and two chordomas. Preoperative 3-D printing models were created by 3-D printing technology. Sagittal en bloc resections were planned based on these models and successfully performed. A 360° reconstruction was performed by spinal instrumentation in all cases. Surgical margins, perioperative complications, local control rate and survival rate were assessed. All patients underwent en bloc excision via a combined posterior and anterior approach in one stage. Mean operative time and estimated blood loss were 465 minutes and 1290 mL, respectively. Mean follow-up was 21 months. Wide surgical margins were achieved in two patients and marginal resection in three; these three patients underwent postoperative adjuvant radiation therapy. One vertebral artery was ligated and sacrificed in each of three patients. Nerve root involved by tumor was sacrificed in three patients with preoperative upper extremity weakness. One patient (Case 3) had significant transient radiculopathy with paresis postoperatively. Another (Case 4) with C 4 and C 5 chordoma had respiratory difficulties and pneumonia after surgery postoperatively. He recovered completely after 2 weeks' management with a tracheotomy tube and antibiotics in the intensive care unit. No cerebrovascular complications and wound infection were observed. No local recurrence or instrumentation failure were detected during follow-up. Though technically challenging, it is feasible and safe to perform en

Delayed complications of radiotherapy treatment for nasopharyngeal carcinoma: imaging findings

International Nuclear Information System (INIS)

King, A.D.; Ahuja, A.T.; Yeung, D.K.; Wong, J.K.T.; Lee, Y.Y.P.; Lam, W.W.M.; Ho, S.S.M.; Yu, S.C.H.; Leung, S.-F.

2007-01-01

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

Arrested Pneumatization of the Sphenoid Sinus on Large Field-of-View Cone Beam Computed Tomography Studies

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Mehrnaz Tahmasbi-Arashlow

2015-05-01

Full Text Available Arrested pneumatization of the sphenoid sinus is a normal anatomical variant. The aim of this report is to define cone beam computed tomography (CBCT characteristics of arrested pneumatization of sphenoid sinus in an effort to help differentiate it from invasive or lytic skull base lesions. Two cases are presented with incidental findings. Both studies, acquired for other diagnostic purposes, demonstrated unique osseous patterns that were eventually deemed to be anatomic variations in the absence of clinical signs and symptoms although the pattern of bone loss and remodeling was diagnosed as pneumatization of the sphenoid sinus by a panel of medical and maxillofacial radiologists following contrasted advanced imaging. It is important to differentiate arrested pneumatization of the sphenoid sinus from lesions, such as arachnoid granulations, acoustic neuroma, glioma, metastatic lesions, meningioma, or chordoma, to prevent unnecessary biopsies or exploratory surgeries that would consequently reduce treatment costs and alleviate anxiety in patients.

Proposed Diagnostic Criteria, Classification Schema, and Review of Literature of Notochord-Derived Ecchordosis Physaliphora

Science.gov (United States)

Lagman, Carlito; Sarmiento, J. Manuel; Turtz, Alan R; Chitale, Rohan V

2016-01-01

Ecchordosis physaliphora (EP) is a benign notochordal remnant derived from ectopic nests found along the craniospinal axis. It typically presents asymptomatically and is diagnosed using classic radiologic features, particularly location, T1-hypointensity, T2-hyperintensity, and lack of enhancement following gadolinium (Gd) contrast administration. Distinguishing EP from its malignant counterpart, chordoma, is of paramount importance, given the aggressive nature of the latter. Advances in imaging and immunohistochemistry have aided in diagnosis to an extent but, to our knowledge, identification of the genetic fingerprint of EP has yet to take place. Further cytological analysis of these lesions in search of a genetic link is warranted. We propose here a set of diagnostic criteria based on features consistently cited in the literature. In this literature review, 23 case reports were identified and collated into a summary of symptomatic cases of ecchordosis physaliphora. An illustrative case report of two patients was also included.  PMID:27158576

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

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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.

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

Science.gov (United States)

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

2014-08-01

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

Unilateral duplicated abducens nerve coursing through both the sphenopetroclival venous gulf and cavernous sinus: a case report.

Science.gov (United States)

Coquet, Thomas; Lefranc, Michel; Chenin, Louis; Foulon, Pascal; Havet, Éric; Peltier, Johann

2018-03-15

In this anatomy report, we describe the first case of abducens nerve duplication limited to the sphenopetroclival venous gulf and the cavernous sinus. The objective point of division of the two duplicated roots was localized at the gulfar face of the dural porus, just distal to the unique cisternal trunk of the abducens nerve, as it pierced the petroclival dural mater. In the gulfar segment, both roots traveled through a variant of Dorello's canal called the "petrosphenoidal canal" and remained separated through the posterior half of the cavernous sinus. Both roots finally fused in the anterior half of the cavernous sinus to innervate the lateral rectus muscle as a single trunk. Although many variants of the abducens nerve have been reported over the recent decades, this anatomic variation has never been previously described and enriches the continuum of abducens nerve variations reported in the literature data. Awareness of this variation is crucial for neurosurgeons, especially during clival or petrosal surgical approaches used for resection of skull base chordomas.

Polymer Nanoparticle-Based Chemotherapy for Spinal Malignancies

Directory of Open Access Journals (Sweden)

Hongyun Ma

2016-01-01

Full Text Available Malignant spinal tumors, categorized into primary and metastatic ones, are one of the most serious diseases due to their high morbidity and mortality rates. Common primary spinal tumors include chordoma, chondrosarcoma, osteosarcoma, Ewing’s sarcoma, and multiple myeloma. Spinal malignancies are not only locally invasive and destructive to adjacent structures, such as bone, neural, and vascular structures, but also disruptive to distant organs (e.g., lung. Current treatments for spinal malignancies, including wide resection, radiotherapy, and chemotherapy, have made significant progress like improving patients’ quality of life. Among them, chemotherapy plays an important role, but its potential for clinical application is limited by severe side effects and drug resistance. To ameliorate the current situation, various polymer nanoparticles have been developed as promising excipients to facilitate the effective treatment of spinal malignancies by utilizing their potent advantages, for example, targeting, stimuli response, and synergetic effect. This review overviews the development of polymer nanoparticles for antineoplastic delivery in the treatment of spinal malignancies and discusses future prospects of polymer nanoparticle-based treatment methods.

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

Energy Technology Data Exchange (ETDEWEB)

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

2010-12-15

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

Role of the ECM in notochord formation, function and disease.

Science.gov (United States)

Trapani, Valeria; Bonaldo, Paolo; Corallo, Diana

2017-10-01

The notochord is a midline structure common to all chordate animals; it provides mechanical and signaling cues for the developing embryo. In vertebrates, the notochord plays key functions during embryogenesis, being a source of developmental signals that pattern the surrounding tissues. It is composed of a core of vacuolated cells surrounded by an epithelial-like sheath of cells that secrete a thick peri-notochordal basement membrane made of different extracellular matrix (ECM) proteins. The correct deposition and organization of the ECM is essential for proper notochord morphogenesis and function. Work carried out in the past two decades has allowed researchers to dissect the contribution of different ECM components to this embryonic tissue. Here, we will provide an overview of these genetic and mechanistic studies. In particular, we highlight the specific functions of distinct matrix molecules in regulating notochord development and notochord-derived signals. Moreover, we also discuss the involvement of ECM synthesis and its remodeling in the pathogenesis of chordoma, a malignant bone cancer that originates from remnants of notochord remaining after embryogenesis. © 2017. Published by The Company of Biologists Ltd.

TRANSORAL REMOVAL OF SKULL BASE AND C1-C2 VERTEBRAL BODY TUMOURS AND NONTUMOROUS PATHOLOGY IN THE CRANIOCERVICAL JUNCTION ACCOMPANIED BY CRANIOVERTEBRAL INSTABILITY

Directory of Open Access Journals (Sweden)

A. N. Shkarubo

2010-01-01

Full Text Available 27 patients aged 2,5-61 years with skull base and C1-C2 vertebral body tumours and nontumorous pathology in the craniocervical junction underwent surgery. All patients revealed craniovertebral instability. To perform OSD we used autobone and metallic wire in 1 case, "Ventrofix" - 2; "CCD" - 9, "Vertex" - 15. In 26 cases OSD was followed by transoral tumor removal; in 1 - removal of the skull base chordoma spreading into C1-C2 segments was followed by OSD. In our practice we used original patent instruments, devices and surgical techniques. After the tumor has been removed, the skull defect hermetic closure and plasty were performed using the original patent technique for preventing postoperative CSF leakage as well as different glue compositions. This technique proved to shorten hospitalization period and reduce treatment costs as well as launch an early rehabilitation programme - on the 3d-4th day after operation. Use of new technologies in surgical treatment of skull base tumors invading upper cervical spinal segments accompanied by craniovertebral instability allowed to improve surgical outcome and start up early rehabilitation.

Somatostatin receptor imaging in intracranial tumours

International Nuclear Information System (INIS)

Schmidt, M.; Scheidhauer, K.; Voth, E.; Schicha, H.; Luyken, C.; Hildebrandt, G.; Klug, N.

1998-01-01

The somatostatin analogue [ 111 In-DTPA-d-Phe 1 ]-octreotide ( 111 In-octreotide) allows scintigraphic visualization of somatostatin receptor-expressing tissue. While it is well known that a large variety of tissues express somatostatin receptors and 111 In-octreotide scintigraphy has a clearly defined role in various neuroendocrine diseases, the clinical value of 111 In-octreotide scintigraphy in brain tumours is still under clinical investigation. In 124 patients with 141 brain lesions (63 meningiomas, 24 pituitary adenomas, 10 gliomas WHO class I and II, 12 gliomas WHO class III and IV, 11 neurinomas and 2 neurofibromas, 7 metastases and 12 other varieties: three non-Hodgkin B-cell lymphomas, two epidermoids, one abscess, one angioleiomyoma, one chordoma, one haemangiopericytoma, one osteosarcoma, one plasmacytoma and one pseudocyst), 111 In-octreotide scintigraphy was performed 4-6 and 24 h after i.v. injection of 110-220 MBq 111 In-octreotide. Planar images of the head in four views with a 128 x 128 matrix and single-photon emission tomographic images (64 x 64 matrix) were acquired, and lesions were graded according to qualitative tracer uptake. Fifty-nine of the 63 meningiomas showed moderate to intense tracer uptake. Nine of 24 pituitary adenomas were visible; the remaining 15 did not show any tracer uptake. None of the class I and II gliomas with an intact blood-brain barrier were detected whereas 11/12 class III and IV gliomas showed 111 In-octreotide uptake. None of the neurinomas or neurofibromas were positive. Five of seven metastases were classified as positive, as were the osteosarcoma, two of three non-Hodgkin B-cell lymphomas, one abscess, one angioleiomyoma, one chordoma and one haemangiopericytoma. The other varieties (one non-Hodgkin B-cell lymphoma, two epidermoids, one plasmacytoma and one pseudocyst) did not show 111 In-octreotide uptake. The results demonstrate that a large variety of intracranial lesions express somatostatin receptors and

Somatostatin receptor imaging in intracranial tumours

Energy Technology Data Exchange (ETDEWEB)

Schmidt, M.; Scheidhauer, K.; Voth, E.; Schicha, H. [Department of Nuclear Medicine, University of Koeln (Germany); Luyken, C.; Hildebrandt, G.; Klug, N. [Department of Neurosurgery, University of Kolen (Germany)

1998-07-01

The somatostatin analogue [{sup 111}In-DTPA-d-Phe{sup 1}]-octreotide ({sup 111}In-octreotide) allows scintigraphic visualization of somatostatin receptor-expressing tissue. While it is well known that a large variety of tissues express somatostatin receptors and {sup 111}In-octreotide scintigraphy has a clearly defined role in various neuroendocrine diseases, the clinical value of {sup 111}In-octreotide scintigraphy in brain tumours is still under clinical investigation. In 124 patients with 141 brain lesions (63 meningiomas, 24 pituitary adenomas, 10 gliomas WHO class I and II, 12 gliomas WHO class III and IV, 11 neurinomas and 2 neurofibromas, 7 metastases and 12 other varieties: three non-Hodgkin B-cell lymphomas, two epidermoids, one abscess, one angioleiomyoma, one chordoma, one haemangiopericytoma, one osteosarcoma, one plasmacytoma and one pseudocyst), {sup 111}In-octreotide scintigraphy was performed 4-6 and 24 h after i.v. injection of 110-220 MBq {sup 111}In-octreotide. Planar images of the head in four views with a 128 x 128 matrix and single-photon emission tomographic images (64 x 64 matrix) were acquired, and lesions were graded according to qualitative tracer uptake. Fifty-nine of the 63 meningiomas showed moderate to intense tracer uptake. Nine of 24 pituitary adenomas were visible; the remaining 15 did not show any tracer uptake. None of the class I and II gliomas with an intact blood-brain barrier were detected whereas 11/12 class III and IV gliomas showed {sup 111}In-octreotide uptake. None of the neurinomas or neurofibromas were positive. Five of seven metastases were classified as positive, as were the osteosarcoma, two of three non-Hodgkin B-cell lymphomas, one abscess, one angioleiomyoma, one chordoma and one haemangiopericytoma. The other varieties (one non-Hodgkin B-cell lymphoma, two epidermoids, one plasmacytoma and one pseudocyst) did not show {sup 111}In-octreotide uptake. The results demonstrate that a large variety of intracranial

Evaluation of the MRI with Gd-DTPA enhancement in the diagnosis of brain and spinal tumors

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Fukui, Keiji; Sadamoto, Kazuhiko; Miki, Hitoshi; Sakaki, Saburo; Matsuoka, Kenzo.

1988-02-01

Magnetic resonance (MR) imaging was performed using saturation recovery (SR), inversion recovery (IR) and spin echo (SE) pulse sequences before and after the injection of 0.1 mmol of gadolinium diethylenetriamine pentaacetic acid dimeglumine (Gd-DTPA) per kilogram of body weight. Forty-two patients with gliomas (8), meningiomas (12), neurinomas (5), adenoma (1), craniopharyngioma (1), chordoma (1), malignant lymphomas (2), metastatic brain tumors (2), spinal tumors (4), and other tumors (6) were studied. Contrast enhancement was shown in 35 of the 38 patients with brain tumors and in all those with spinal tumors on T/sub 1/-weighted images. The T/sub 1/ relaxation time was decreased in all of these 39 tumors, while the T/sub 2/ relaxation time showed no definite tendency. No marked side effects were observed following the administration of Gd-DTPA. By using Gd-DTPA enhancement, it was possible to differentiate the tumor from the peritumoral edema on MRI, although it was difficult to do so on precontrast MRI. Among the various pulse sequences, the SR sequence provides the best diagnostic value with Gd-DTPA enhancement in the shortest examination time for screening brain and spinal tumors.

Cytokeratin positivity in myxopapillary ependymoma – a potential diagnostic pitfall

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Sur Monalisa

2008-10-01

Full Text Available Abstract Background Myxopapillary ependymomas (MPE occur in the filum terminale of the spinal cord, but also present in extra-spinal locations such as subcutaneous tissue and brain. They are slow growing grade I gliomas. Areas of solid growth pattern with aggregates of cells with "epithelioid morphology" seen in MPE can mimic metastatic carcinoma. The presence of occasional cells with clear cytoplasm and morphology can resemble Chordoma. Diagnosis can be missed due to these morphological similarities, which could affect patient management and hence, long term survival. Case presentation We describe two cases of MPE with cytokeratin (AE1 AE3, CAM 5.2, Cytokeratin 7 and cytokeratin 20 expression. Conclusion MPE can be positive for Cytokeratins (CAM 5.2, AE1 AE3, CK7 and focally for EMA, which could be misdiagnosed as metastatic carcinoma. In cases demonstrating epithelioid and clear cell morphology, the diagnosis of MPE should be made in conjunction with histology, proper immunohistochemical profile which includes co-expression of GFAP, S-100 protein and epithelial markers, radiologic findings and site. It is important to be aware of the cytokeratin profile in MPE to avoid erroneous diagnosis with other tumour entities.

CT and MR imaging findings of sphenoidal masses

Energy Technology Data Exchange (ETDEWEB)

Takahashi, Shoki; Higano, Shuichi (Tohoku Univ., Sendai (Japan). School of Medicine); Ishii, Kiyoshi (and others)

1994-07-01

CT and MR imaging findings of 57 sphenoidal masses were retrospectively reviewed to assess the possibility of differential diagnosis between them. Various kinds of masses such as pituitary adenoma, epipharyngeal cancer, mucocele, chordoma, chondroma, chondrosarcoma, distant metastasis, multiple myeloma, fibrous dysplasia, craniopharyngioma, hemangiopericytoma, giant cell tumor, primary sphenoidal cancer, malignant melanoma, leukemia, histiocytosis X, and giant cell tumor were included in this series. CT scanning was performed in all cases using a spin-echo pulse sequence. The relative density of the masses, bony changes and calcification were evaluated on CT, and on MR images, signal intensity of the masses relative to the normal gray matter, contrast enhancement and extension/contour were evaluated. Although no single feature appeared to be specific to the masses, detection of calcification on CT, identification of the normal pituitary gland as deformed or displaced on T1-weighted images, signal intensity on T2-weighted images, and extension of the masses seemed to be useful and should be examined in terms of their ability to assist in differential diagnosis. Finally, accommodative classification of sphenoidal masses primarily based on presumed origin or mode of extension was attempted. (author).

Tumores intracranianos em pacientes encaminhados para estudos por tomografia de coerência óptica como portadores de glaucoma sem hipertensão ocular: relato de dois casos Intracranial tumors in patients referred for optical coherence tomography examination as glaucoma suspects: case report

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Laurentino Biccas Neto

2009-10-01

Full Text Available A tomografia de coerência óptica (OCT tem se mostrado muito útil na avaliação de pacientes com glaucoma. São relatadas duas pacientes referidas com a suspeita de glaucoma sem hipertensão para avaliação por tomografia de coerência óptica que, na verdade, eram portadoras de tumores intracranianos - um cordoma de clivo no primeiro caso e um craniofaringeoma no segundo. Os achados à tomografia de coerência óptica - diminuição difusa da espessura da camada de fibras nervosas circumdiscais desproporcionalmente acentuada nos setores nasal e temporal - levantaram a suspeita de acometimento na região do quiasma e permitiram o diagnóstico destes importantes tumores intracranianos.Optical coherence tomography (OCT has proved to be a very valuable tool in the assessment of patients with glaucoma. In this report, intracranial tumors were discovered in two glaucoma suspects referred for diagnostic confirmation by OCT - a clivus chordoma and a craniopharyngeoma. Optical coherence tomography findings - marked asymmetrical diffuse attenuation of the peripapillary nerve fiber layer in nasal and temporal sectors - raised concerns about lesions in chiasmatic region and permitted the timely diagnosis of these intraocular tumors.

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

International Nuclear Information System (INIS)

Glosser, Guila; McManus, Pat; Munzenrider, John; Austin-Seymour, Mary; Fullerton, Barbara; Adams, Judy; Urie, Marcia M.

1997-01-01

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

Pulsed laser-induced liquid jet: evolution from shock/bubble interaction to neurosurgical application

Science.gov (United States)

Nakagawa, A.; Kumabe, T.; Ogawa, Y.; Hirano, T.; Kawaguchi, T.; Ohtani, K.; Nakano, T.; Sato, C.; Yamada, M.; Washio, T.; Arafune, T.; Teppei, T.; Atsushi, K.; Satomi, S.; Takayama, K.; Tominaga, T.

2017-01-01

The high-speed liquid (water) jet has distinctive characteristics in surgical applications, such as tissue dissection without thermal damage and small blood vessel preservation, that make it advantageous over more conventional instruments. The continuous pressurized jet has been used since the first medical application of water jets to liver surgery in the 1980s, but exhibited drawbacks partly related to the excess water supply required and unsuitability for application to microsurgical instruments intended for deep, narrow lesions (endoscopic instrumentation and catheters) due to limitations in miniaturization of the device. To solve these issues, we initiated work on the pulsed micro-liquid jet. The idea of the pulsed micro-liquid jet originated from the observation of tissue damage by shock/bubble interactions during extracorporeal shock wave lithotripsy and evolved into experimental application for recanalization of cerebral embolisms in the 1990s. The original method of generating the liquid jet was based on air bubble formation and microexplosives as the shock wave source, and as such could not be applied clinically. The air bubble was replaced by a holmium:yttrium-aluminum-garnet (Ho:YAG) laser-induced bubble. Finally, the system was simplified and the liquid jet was generated via irradiation from the Ho:YAG laser within a liquid-filled tubular structure. A series of investigations revealed that this pulsed laser-induced liquid jet (LILJ) system has equivalent dissection and blood vessel preservation characteristics, but the amount of liquid usage has been reduced to less than 2 μ l per shot and can easily be incorporated into microsurgical, endoscopic, and catheter devices. As a first step in human clinical studies, we have applied the LILJ system for the treatment of skull base tumors through the transsphenoidal approach in 9 patients (7 pituitary adenomas and 2 chordomas), supratentorial glioma (all high grade glioma) in 8 patients, including one with

Proton radiotherapy in management of pediatric base of skull tumors

International Nuclear Information System (INIS)

Hug, Eugen B.; Sweeney, Reinhart A.; Nurre, Pamela M.; Holloway, Kitty C.; Slater, Jerry D.; Munzenrider, John E.

2002-01-01

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

Morphological studies in the diagnosis of primary and secondary bone tumors

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Matveeva O.V.

2016-12-01

Full Text Available The aim: to show the possibility of morphological studies in the diagnosis of primary and secondary tumors of bones. Material and Methods. 105 (72% patients with primary bone tumors aged from 15 to 66 years and 42 (28% patients with metastatic bone lesions aged from 42 to 70 years were examined and treated for the period from 2008 till 2015. Material for morphological studies was prepared using an open biopsy tissue slices and a scraping resected tumor during surgery. Soft-tissue component is subjected to cytology. The material for histological study included changes in bone and soft tissue. Results. Giant cell tumor was verified in 45% of cases by histological examination. Multiple myeloma was diagnosed in 15% of patients. Osteogenic sarcoma was diagnosed in 14% of cases. Ewing's sarcoma was diagnosed in 3%, 2% of cases were matched by diagnosed chordoma. According to the data received, cancer metastasis of kidney and lung is mostly diagnosed in men from the group of patients with secondary bone defeat. Metastasis of cancer of the breast in women was predominated. Conclusion. The morphological (histological, cytological study plays an important role in the diagnosis of bone tumors. The coincidence of the cytological and histological diagnoses was 97%.

REVIEW OF NASA FIBROMYXOMA: Aggressive Behaviour? (Case Report

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Rr. Suzy Indharti

2015-12-01

Full Text Available Background: Myxomas are rare benign tumors arising from mesenchymal tissues throughout the body. These tumors are usually seen in the atrium of the heart and the jawbone. Involvement of the skull base with intracranial extension is extremely rare, and only a few cases of primary intracranial myxomas have been described in the literature. This article presents a rare case of primary myxoma of the nasal bone. Methods: The patient underwent a skull base surgery with a pre-diagnosis of possible fibromyxomas. The tumor pathology revealed a diagnosis of myxoma with bone and meningeal involvement. Despite the debulking surgery, the tumor showed a local recurrence in five month. A second debulking surgery in piece meal was required. In the article, the etiology, histological and radiological findings as well as treatment options of this rare entity were briefly discussed under the highlights of the relevant literature. Such a localization and intracranial extension of myxomas is extremely unusual in clinical practice; the diagnosis therefore requires a high degree of suspicion and detailed histopathological examination. The differential diagnosis frequently includes chondrosarcomas, chordoma, metastatic tumors of the skull, hemangiopericytoma, meningioma and other neoplasms of the dura and skull base in this location.

Cytomorphologic features of myxopapillary ependymoma: a review of 13 cases.

Science.gov (United States)

Takei, Hidehiro; Kosarac, Ognjen; Powell, Suzanne Z

2009-01-01

To describe the cytologic features of myxopapillary ependymoma (MPE) on intraoperative smears, to analyze cytomorphologic parameters that may help in reaching the diagnosis and to discuss differential diagnosis. Touch imprint smears of 13 MPE cases were reviewed and graded semiquantitatively for 14 cytomorphologic parameters; cellularity, myxoid background, isolated/dispersed tumor cells, "hyaline globules (HGs)," fibrillary cytoplasmic processes, papillary structures, perivascular pseudorosettes, epithelioid tumor cells (ETCs), intracytoplasmic mucin, intranuclear inclusions, nuclear grooves, mitosis, cytologic atypia and hemosiderin-laden macrophages. Cytologic examination revealed variably cellular specimens composed of isolated and loosely aggregated tumor cells with round to oval or occasionally spindle-shaped nuclei; evenly distributed, finely granular chromatin; and fibrillary processes admixed with occasional ETCs. Most of the cases showed prominent fibrillary processes and occasional ETCs with at least a focal myxoid background. HGs and hemosiderin-laden macrophages were often seen. Papillary structure, a histologic hallmark of MPE, was rarely observed. Dual glial and epithelioid properties of tumor cells, well-known features of "regular" ependymomas, and a distinctive myxoid background with HGs strongly support a diagnosis of MPE and are of great help in excluding other mimics (e.g., other variants of ependymoma, metastatic mucinous adenocarcinoma, metastatic adenoid cystic carcinoma and chordoma).

Endoscopic endonasal skull base surgery: advantages, limitations, and our techniques to overcome cerebrospinal fluid leakage: technical note.

Science.gov (United States)

Ishii, Yudo; Tahara, Shigeyuki; Teramoto, Akira; Morita, Akio

2014-01-01

In recent years, resections of midline skull base tumors have been conducted using endoscopic endonasal skull base (EESB) approaches. Nevertheless, many surgeons reported that cerebrospinal fluid (CSF) leakage is still a major complication of these approaches. Here, we report the results of our 42 EESB surgeries and discuss the advantages and limits of this approach for resecting various types of tumors, and also report our technique to overcome CSF leakage. All 42 cases involved midline skull base tumors resected using the EESB technique. Dural incisions were closed using nasoseptal flaps and fascia patch inlay sutures. Total removal of the tumor was accomplished in seven pituitary adenomas (33.3%), five craniopharyngiomas (62.5%), five tuberculum sellae meningiomas (83.3%), three clival chordomas (100%), and one suprasellar ependymoma. Residual regions included the cavernous sinus, the outside of the intracranial part of the internal carotid artery, the lower lateral part of the posterior clivus, and the posterior pituitary stalk. Overall incidence of CSF leakage was 7.1%. Even though the versatility of the approach is limited, EESB surgery has many advantages compared to the transcranial approach for managing mid-line skull base lesions. To avoid CSF leakage, surgeons should have skills and techniques for complete closure, including use of the nasoseptal flap and fascia patch inlay techniques.

Tumor Cells Surviving Exposure to Proton or Photon Radiation Share a Common Immunogenic Modulation Signature, Rendering Them More Sensitive to T Cell–Mediated Killing

Energy Technology Data Exchange (ETDEWEB)

Gameiro, Sofia R.; Malamas, Anthony S. [Laboratory of Tumor Immunology and Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (United States); Bernstein, Michael B. [Division of Radiation Oncology, M. D. Anderson Cancer Center, Houston, Texas (United States); Tsang, Kwong Y. [Laboratory of Tumor Immunology and Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (United States); Vassantachart, April; Sahoo, Narayan; Tailor, Ramesh; Pidikiti, Rajesh [Division of Radiation Oncology, M. D. Anderson Cancer Center, Houston, Texas (United States); Guha, Chandan P. [Department of Radiation Oncology, Montefiore Medical Center, Bronx, New York (United States); Hahn, Stephen M.; Krishnan, Sunil [Division of Radiation Oncology, M. D. Anderson Cancer Center, Houston, Texas (United States); Hodge, James W., E-mail: jh241d@nih.gov [Laboratory of Tumor Immunology and Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (United States)

2016-05-01

Purpose: To provide the foundation for combining immunotherapy to induce tumor antigen–specific T cells with proton radiation therapy to exploit the activity of those T cells. Methods and Materials: Using cell lines of tumors frequently treated with proton radiation, such as prostate, breast, lung, and chordoma, we examined the effect of proton radiation on the viability and induction of immunogenic modulation in tumor cells by flow cytometric and immunofluorescent analysis of surface phenotype and the functional immune consequences. Results: These studies show for the first time that (1) proton and photon radiation induced comparable up-regulation of surface molecules involved in immune recognition (histocompatibility leukocyte antigen, intercellular adhesion molecule 1, and the tumor-associated antigens carcinoembryonic antigen and mucin 1); (2) proton radiation mediated calreticulin cell-surface expression, increasing sensitivity to cytotoxic T-lymphocyte killing of tumor cells; and (3) cancer stem cells, which are resistant to the direct cytolytic activity of proton radiation, nonetheless up-regulated calreticulin after radiation in a manner similar to non-cancer stem cells. Conclusions: These findings offer a rationale for the use of proton radiation in combination with immunotherapy, including for patients who have failed radiation therapy alone or have limited treatment options.

Neurophysiological Identification of Cranial Nerves During Endoscopic Endonasal Surgery of Skull Base Tumors: Pilot Study Technical Report.

Science.gov (United States)

Shkarubo, Alexey Nikolaevich; Chernov, Ilia Valerievich; Ogurtsova, Anna Anatolievna; Moshchev, Dmitry Aleksandrovich; Lubnin, Andrew Jurievich; Andreev, Dmitry Nicolaevich; Koval, Konstantin Vladimirovich

2017-02-01

Intraoperative identification of cranial nerves is crucial for safe surgery of skull base tumors. Currently, only a small number of published papers describe the technique of trigger electromyography (t-EMG) in endoscopic endonasal removal of such tumors. To assess the effectiveness of t-EMG in preventing intraoperative cranial nerve damage in endoscopic endonasal surgery of skull base tumors. Nine patients were operated on using the endoscopic endonasal approach within a 1-year period. The tumors included large skull base chordomas and trigeminal neurinomas localized in the cavernous sinus. During the surgical process, cranial nerve identification was carried out using monopolar and bipolar t-EMG methods. Assessment of cranial nerve functional activity was conducted both before and after tumor removal. We mapped 17 nerves in 9 patients. Third, fifth, and sixth cranial nerves were identified intraoperatively. There were no cases of postoperative functional impairment of the mapped cranial nerves. In one case we were unable to get an intraoperative response from the fourth cranial nerve and observed its postoperative transient plegia (the function was normal before surgery). t-EMG allows surgeons to control the safety of cranial nerves both during and after skull base tumor removal. Copyright © 2016 Elsevier Inc. All rights reserved.

Percutaneous gastrostomy -a report of twenty-seven cases-

International Nuclear Information System (INIS)

Kim, Tae Ho; Lee, Ho Suk; Kim, Yong Joo; Kim, Tae Hun; Suh, Kyung Jin; Kang, Duk Sik

1991-01-01

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

Decisions and involvement of cancer patient survivors: a moral imperative

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Pravettoni G

2016-12-01

Full Text Available Gabriella Pravettoni,1,2 Ilaria Cutica,1,2 Simona Righetti,1 Ketti Mazzocco1,2 1Department of Oncology and Hematology, University of Milan, 2Applied Research Division for Cognitive and Psychological Science, European Institute of Oncology, Milan, Italy Purpose: The aim of this study was to review the experiences of direct involvement in patient survivorship for treatment and research. Methods: This is a narrative-focused review of the following two recent experiences of patient involvement: the Chordoma Foundation and the Triple Negative Breast Cancer Foundation. Results: These two examples represent concrete experiences that patients have built to favor a real involvement in the care and treatment of tumors. These experiences are profoundly modifying how cancer research is conducted and draw attention to the psychosocial dimensions of health care. Conclusion: These examples represent the new scenario in which modern medicine faces completely new challenges, copes with new needs, and cooperates with new health care professionals. Implications: Involving patients in a new perspective raises practical and ethical challenges for organizations to work together, for health providers to be professionally skilled and for the government to promote safeguarding policies. Keywords: patient empowerment, patients’ association, empowerment, skills, codesign ­techniques, cancer

Profile of European proton and carbon ion therapy centers assessed by the EORTC facility questionnaire.

Science.gov (United States)

Weber, Damien C; Abrunhosa-Branquinho, André; Bolsi, Alessandra; Kacperek, Andrzej; Dendale, Rémi; Geismar, Dirk; Bachtiary, Barbara; Hall, Annika; Heufelder, Jens; Herfarth, Klaus; Debus, Jürgen; Amichetti, Maurizio; Krause, Mechthild; Orecchia, Roberto; Vondracek, Vladimir; Thariat, Juliette; Kajdrowicz, Tomasz; Nilsson, Kristina; Grau, Cai

2017-08-01

We performed a survey using the modified EORTC Facility questionnaire (pFQ) to evaluate the human, technical and organizational resources of particle centers in Europe. The modified pFQ consisted of 235 questions distributed in 11 sections accessible on line on an EORTC server. Fifteen centers from 8 countries completed the pFQ between May 2015 and December 2015. The average number of patients treated per year and per particle center was 221 (range, 40-557). The majority (66.7%) of centers had pencil beam or raster scanning capability. Four (27%) centers were dedicated to eye treatment only. An increase in the patients-health professional FTE ratio was observed for eye tumor only centers when compared to other centers. All centers treated routinely chordomas/chondrosarcomas, brain tumors and sarcomas but rarely breast cancer. The majority of centers treated pediatric cases with particles. Only a minority of the queried institutions treated non-static targets. As the number of particle centers coming online will increase, the experience with this treatment modality will rise in Europe. Children can currently be treated in these facilities in a majority of cases. The majority of these centers provide state of the art particle beam therapy. Copyright © 2017 Elsevier B.V. All rights reserved.

Integrated beam orientation and scanning-spot optimization in intensity-modulated proton therapy for brain and unilateral head and neck tumors.

Science.gov (United States)

Gu, Wenbo; O'Connor, Daniel; Nguyen, Dan; Yu, Victoria Y; Ruan, Dan; Dong, Lei; Sheng, Ke

2018-04-01

Intensity-Modulated Proton Therapy (IMPT) is the state-of-the-art method of delivering proton radiotherapy. Previous research has been mainly focused on optimization of scanning spots with manually selected beam angles. Due to the computational complexity, the potential benefit of simultaneously optimizing beam orientations and spot pattern could not be realized. In this study, we developed a novel integrated beam orientation optimization (BOO) and scanning-spot optimization algorithm for intensity-modulated proton therapy (IMPT). A brain chordoma and three unilateral head-and-neck patients with a maximal target size of 112.49 cm 3 were included in this study. A total number of 1162 noncoplanar candidate beams evenly distributed across 4π steradians were included in the optimization. For each candidate beam, the pencil-beam doses of all scanning spots covering the PTV and a margin were calculated. The beam angle selection and spot intensity optimization problem was formulated to include three terms: a dose fidelity term to penalize the deviation of PTV and OAR doses from ideal dose distribution; an L1-norm sparsity term to reduce the number of active spots and improve delivery efficiency; a group sparsity term to control the number of active beams between 2 and 4. For the group sparsity term, convex L2,1-norm and nonconvex L2,1/2-norm were tested. For the dose fidelity term, both quadratic function and linearized equivalent uniform dose (LEUD) cost function were implemented. The optimization problem was solved using the Fast Iterative Shrinkage-Thresholding Algorithm (FISTA). The IMPT BOO method was tested on three head-and-neck patients and one skull base chordoma patient. The results were compared with IMPT plans created using column generation selected beams or manually selected beams. The L2,1-norm plan selected spatially aggregated beams, indicating potential degeneracy using this norm. L2,1/2-norm was able to select spatially separated beams and achieve

The role of stereotactic radiosurgery in the treatment of malignant skull base tumors

International Nuclear Information System (INIS)

Miller, Robert C.; Foote, Robert L.; Coffey, Robert J.; Gorman, Deborah A.; Earle, John D.; Schomberg, Paula J.; Kline, Robert W.

1997-01-01

Purpose: To determine the efficacy and toxicity of stereotactic radiosurgery in the treatment of malignant skull base tumors. Methods and Materials: Thirty-two patients with 35 newly diagnosed or recurrent malignant skull base tumors ≤33.5 cm 3 were treated using the Leksell Gamma unit. Tumor histologies included: adenoid cystic carcinoma, basal cell carcinoma, chondrosarcoma, chordoma, nasopharyngeal carcinoma, osteogenic sarcoma, and squamous cell carcinoma. Results: After a median follow-up of 2.3 years, 83% ± 15% (±95% confidence interval) of patients experienced a symptomatic response to treatment. Local control at the skull base was 95 ± 9% at 2 years and 78 ± 23% at 3 years. Local-regional control above the clavicles was 75 ± 15% at 1 year and 51 ± 20% at 2 years. Overall and cause specific survival were identical, 82 ± 13% at 1 year, 76 ± 14% at 2 years, and 72 ± 16% at 3 years. One patient developed a radiation-induced optic neuropathy 12 months after radiosurgery. Conclusion: Stereotactic radiosurgery using the Leksell Gamma Unit can provide durable tumor control and symptomatic relief with acceptable toxicity in the majority of patients with malignant tumors 4 cm or less in size involving the skull base. Further evaluation of more patients with longer follow-up is warranted

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

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

1988-09-01

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

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

International Nuclear Information System (INIS)

Slater, J.D.; Austin-Seymour, M.; Munzenrider, J.

1988-01-01

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

Morphometric Analysis of Bone Resection in Anterior Petrosectomies.

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

2016-06-01

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

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

International Nuclear Information System (INIS)

Gupta, Tejpal; Wadasadawala, Tabassum; Master, Zubin; Phurailatpam, Reena; Pai-Shetty, Rajershi; Jalali, Rakesh

2012-01-01

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.

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

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Gupta, Tejpal [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.

Study on blood supply of lung metastasis with trans-pulmonary arterial lipiodol infusion

International Nuclear Information System (INIS)

Zhou Jianqin; Dong Weihua; Dong Weihua; Ouyang Chang; Chang Heng; Xiao Xiangsheng

2008-01-01

Objective: To evaluate the blood supply of pulmonary metastases using small volume of lipiodol through pulmonary arterial infusion. Methods: 10 cases of lung metastasis were enroled including the primary tumors of liver cancer (n=5), renal carcinoma (n=3), chordoma (n=1) and malignant neurofibroma (n=1). Plain CT scan was performed to exclude calcification or ossification within metastasis and then pulmonary arterial DSA was undertaken to evaluate tumor vessels or staining. After pulmonary arteriovenous fistula or other anomalous circulation was excluded by lobar arterial DSA, small volume of lipiodol was infused under fluoroscopy (0.5-1.5 ml for each lobar artery, total volume less than 3.0 ml). CT scan was immediately performed. Blood supply of the pulmonary metastases was assessed according to the accumulation of lipiodol on CT scans. Results: No cases but one experienced cough, expectoration, suffocating or dyspnea. No complication of cerebral or visceral embolism occurred. Totally 27 nodules were studied including 6 nodules with cloudy lipiodol accumulation and 6 nodules with tiny granules of lipiodol accumulation. No enlarged tumor vessel or tumor stain was observed within all 27 nodules on pulmonary arterial DSA. Conclusions: Pulmonary artery supplys only parts of pulmonary metastases, especially those sited at the peripheral region of the lung. Infusion of small volume of lipiodol through pulmonary artery is safe, and the increased density of lung field could return normal after several days. (authors)

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.

Investigation of the added value of high-energy electrons in intensity-modulated radiotherapy: four clinical cases

International Nuclear Information System (INIS)

Korevaar, Erik W.; Huizenga, Henk; Loef, Johan; Stroom, Joep C.; Leer, Jan Willem H.; Brahme, Anders

2002-01-01

Purpose: Intensity-modulated radiotherapy (IMRT) with photon beams is currently pursued in many clinics. Theoretically, inclusion of intensity- and energy-modulated high-energy electron beams (15-50 MeV) offers additional possibilities to improve radiotherapy treatments of deep-seated tumors. In this study the added value of high-energy electron beams in IMRT treatments was investigated. Methods and Materials: In a comparative treatment planning study, conventional treatment plans and various types of IMRT plans were constructed for four clinical cases (cancer of the bladder, pancreas, chordoma of the sacrum, and breast). The conventional plans were used for the actual treatment of the patients. The IMRT plans were optimized using the Orbit optimization code (Loef et al., 2000) with a radiobiologic objective function. The IMRT plans were either photon or combined electron and photon beam plans, with or without dose homogeneity constraints assuming standard or increased radiosensitivities of organs at risk. Results: Large improvements in expected treatment outcome are found using IMRT plans compared to conventional plans, but differences in tumor control probability (TCP) and normal tissue complication probabilities (NTCP) values between IMRT plans with and without electrons are small. However, the use of electrons improves the dose-volume histograms for organs at risk, especially at lower dose levels (e.g., 0-40 Gy). Conclusions: This preliminary study indicates that addition of higher energy electrons to IMRT can only marginally improve treatment outcome for the selected cases. The dose-volume histograms of organs at risk show improvements for IMRT with higher energy electrons, which may reduce tumor induction but does not substantially reduce NTCP

Endoscopic endonasal transsphenoidal surgery using the iArmS operation support robot: initial experience in 43 patients.

Science.gov (United States)

Ogiwara, Toshihiro; Goto, Tetsuya; Nagm, Alhusain; Hongo, Kazuhiro

2017-05-01

Objective The intelligent arm-support system, iArmS, which follows the surgeon's arm and automatically fixes it at an adequate position, was developed as an operation support robot. iArmS was designed to support the surgeon's forearm to prevent hand trembling and to alleviate fatigue during surgery with a microscope. In this study, the authors report on application of this robotic device to endoscopic endonasal transsphenoidal surgery (ETSS) and evaluate their initial experiences. Methods The study population consisted of 43 patients: 29 with pituitary adenoma, 3 with meningioma, 3 with Rathke's cleft cyst, 2 with craniopharyngioma, 2 with chordoma, and 4 with other conditions. All patients underwent surgery via the endonasal transsphenoidal approach using a rigid endoscope. During the nasal and sphenoid phases, iArmS was used to support the surgeon's nondominant arm, which held the endoscope. The details of the iArmS and clinical results were collected. Results iArmS followed the surgeon's arm movement automatically. It reduced the surgeon's fatigue and stabilized the surgeon's hand during ETSS. Shaking of the video image decreased due to the steadying of the surgeon's scope-holding hand with iArmS. There were no complications related to use of the device. Conclusions The intelligent armrest, iArmS, seems to be safe and effective during ETSS. iArmS is helpful for improving the precision and safety not only for microscopic neurosurgery, but also for ETSS. Ongoing advances in robotics ensure the continued evolution of neurosurgery.

Proton Therapy in Children: A Systematic Review of Clinical Effectiveness in 15 Pediatric Cancers

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Leroy, Roos, E-mail: Roos.leroy@kce.fgov.be [Belgian Healthcare Knowledge Centre (KCE), Brussels (Belgium); Benahmed, Nadia; Hulstaert, Frank [Belgian Healthcare Knowledge Centre (KCE), Brussels (Belgium); Van Damme, Nancy [Belgian Cancer Registry, Brussels (Belgium); De Ruysscher, Dirk [Department of Radiation Oncology, University of Leuven, Leuven (Belgium)

2016-05-01

Because it spares many normal tissues and reduces the integral dose, proton therapy (PT) is the preferred tumor irradiation technique for treating childhood cancer. However, to the best of our knowledge, no systematic review of the clinical effectiveness of PT in children has been reported in the scientific literature. A systematic search for clinical outcome studies on PT published between 2007 and 2015 was performed in Medline (through OVID), EMBASE, and the Cochrane Library. Twenty-three primary studies were identified, including approximately 650 patients overall. The median/mean follow-up times were limited (range, 19-91 months). None of the studies were randomized, 2 were comparative, and 20 were retrospective. Most suffered from serious methodologic limitations, yielding a very low level of clinical evidence for the outcomes in all indications. For example, for retinoblastoma, very low-level evidence was found that PT might decrease the incidence of second malignancies. For chondrosarcoma, chordoma, craniopharyngioma, ependymoma, esthesioneuroblastoma, Ewing sarcoma, central nervous system germinoma, glioma, medulloblastoma, osteosarcoma, and rhabdomyosarcoma, there was insufficient evidence to either support or refute PT in children. For pelvic sarcoma (ie, nonrhabdomyosarcoma and non-Ewing sarcoma), pineal parenchymal tumor, primitive neuroectodermal tumor, and “adult-type” soft tissue sarcoma, no studies were identified that fulfilled the inclusion criteria. Although there is no doubt that PT reduces the radiation dose to normal tissues and organs, to date the critical clinical data on the long-term effectiveness and harm associated with the use of PT in the 15 pediatric cancers under investigation are lacking. High-quality clinical research in this area is needed.

Treatment of pediatric patients and young adults with particle therapy at the Heidelberg Ion Therapy Center (HIT: establishment of workflow and initial clinical data

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Combs Stephanie E

2012-10-01

Full Text Available Abstract Background To report on establishment of workflow and clinical results of particle therapy at the Heidelberg Ion Therapy Center. Materials and methods We treated 36 pediatric patients (aged 21 or younger with particle therapy at HIT. Median age was 12 years (range 2-21 years, five patients (14% were younger than 5 years of age. Indications included pilocytic astrocytoma, parameningeal and orbital rhabdomyosarcoma, skull base and cervical chordoma, osteosarcoma and adenoid-cystic carcinoma (ACC, as well as one patient with an angiofibroma of the nasopharynx. For the treatment of small children, an anesthesia unit at HIT was established in cooperation with the Department of Anesthesiology. Results Treatment concepts depended on tumor type, staging, age of the patient, as well as availability of specific study protocols. In all patients, particle radiotherapy was well tolerated and no interruptions due to toxicity had to be undertaken. During follow-up, only mild toxicites were observed. Only one patient died of tumor progression: Carbon ion radiotherapy was performed as an individual treatment approach in a child with a skull base recurrence of the previously irradiated rhabdomyosarcoma. Besides this patient, tumor recurrence was observed in two additional patients. Conclusion Clinical protocols have been generated to evaluate the real potential of particle therapy, also with respect to carbon ions in distinct pediatric patient populations. The strong cooperation between the pediatric department and the department of radiation oncology enable an interdisciplinary treatment and stream-lined workflow and acceptance of the treatment for the patients and their parents.

Assessment of Early Toxicity and Response in Patients Treated With Proton and Carbon Ion Therapy at the Heidelberg Ion Therapy Center Using the Raster Scanning Technique

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Rieken, Stefan; Habermehl, Daniel; Nikoghosyan, Anna; Jensen, Alexandra [Department of Radiation Oncology, University Hospital of Heidelberg, Heidelberg (Germany); Haberer, Thomas [Heidelberg Ion Therapy Center, Heidelberg (Germany); Jaekel, Oliver [Heidelberg Ion Therapy Center, Heidelberg (Germany); Department of Medical Physics, German Cancer Research Center (DKFZ), Heidelberg (Germany); Muenter, Marc W.; Welzel, Thomas; Debus, Juergen [Department of Radiation Oncology, University Hospital of Heidelberg, Heidelberg (Germany); Combs, Stephanie E., E-mail: Stephanie.Combs@med.uni-hedielberg.de [Department of Radiation Oncology, University Hospital of Heidelberg, Heidelberg (Germany)

2011-12-01

Puropose: To asses early toxicity and response in 118 patients treated with scanned ion beams to validate the safety of intensity-controlled raster scanning at the Heidelberg Ion Therapy Center. Patients and Methods: Between November 2009 and June 2010, we treated 118 patients with proton and carbon ion radiotherapy (RT) using active beam delivery. The main indications included skull base chordomas and chondrosarcomas, salivary gland tumors, and gliomas. We evaluated early toxicity within 6 weeks after RT and the initial clinical and radiologic response for quality assurance in our new facility. Results: In all 118 patients, few side effects were observed, in particular, no high numbers of severe acute toxicity were found. In general, the patients treated with particle therapy alone showed only a few single side effects, mainly Radiation Therapy Oncology Group/Common Terminology Criteria grade 1. The most frequent side effects and cumulative incidence of single side effects were observed in the head-and-neck patients treated with particle therapy as a boost and photon intensity-modulated RT. The toxicities included common radiation-attributed reactions known from photon RT, including mucositis, dysphagia, and skin erythema. The most predominant imaging responses were observed in patients with high-grade gliomas and those with salivary gland tumors. For skull base tumors, imaging showed a stable tumor outline in most patients. Thirteen patients showed improvement of pre-existing clinical symptoms. Conclusions: Side effects related to particle treatment were rare, and the overall tolerability of the treatment was shown. The initial response was promising. The data have confirmed the safe delivery of carbon ions and protons at the newly opened Heidelberg facility.

Metrizamide cisternography and metrizamide CT cisternography in suprasellar masses

International Nuclear Information System (INIS)

Uchino, Akira

1985-01-01

Ninety patients with suprasellar masses were evaluated by metrizamide cisternography (MC) with or without metrizamide CT cisternography (MCTC). Included were 54 cases with pituitary tumors, 12 with meningiomas arising from the tuberculum sellae, 10 with craniopharyngiomas, and 14 with other rare suprasellar masses. The author established a ''Height x Length Index (HLI)'' as a parameter of size of suprasellar masses on MC, in the mid sagittal plane. Non-functioning pituitary adenomas were larger than hormoneproducing pituitary adenomas. Most meningiomas were of moderate size, and most craniopharyngiomas were larger than other suprasellar masses. Some of the large masses were poorly visualized on MC; however, nearly all masses were well visualized on MCTC. Pituitary tumors and craniopharyngiomas had smooth surfaces, but, the surfaces of meningiomas were rough. Eleven per cent of pituitary tumors, 25 % of meningiomas, and 50 % of craniopharyngiomas were lobulated and were relatively large. Most pituitary tumors were hemispherical or spherical; meningiomas were relatively flat; and craniopharyngiomas were relatively tall. The centers of the pituitary tumors were just above the diaphragma sellae. The centers of meningiomas were located anterior to and the centers of craniopharyngiomas were slightly posterior to the centers of pituitary tumors. The above characteristics facilitated the differential diagnosis of pituitary tumors, meningiomas, and craniopharyngiomas using MC or MCTC. However, a Rathke's cleft cyst and a granuloma mimicked small pituitary tumors. Another Rathke's cleft cyst and granuloma simulated craniopharyngiomas. Chordomas and a mucocele of the sphenoid sinus mimicked pituitary tumors having inferior extensions. It was concluded that MC with or without MCTC is useful not only for detecting, but also for differentiating suprasellar masses. When a mass is poorly visualized on MC, MCTC is indicated. (author)

Reoptimization of Intensity Modulated Proton Therapy Plans Based on Linear Energy Transfer

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Unkelbach, Jan, E-mail: junkelbach@mgh.harvard.edu [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Botas, Pablo [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Faculty of Physics, Ruprecht-Karls-Universität Heidelberg, Heidelberg (Germany); Giantsoudi, Drosoula; Gorissen, Bram L.; Paganetti, Harald [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States)

2016-12-01

Purpose: We describe a treatment plan optimization method for intensity modulated proton therapy (IMPT) that avoids high values of linear energy transfer (LET) in critical structures located within or near the target volume while limiting degradation of the best possible physical dose distribution. Methods and Materials: To allow fast optimization based on dose and LET, a GPU-based Monte Carlo code was extended to provide dose-averaged LET in addition to dose for all pencil beams. After optimizing an initial IMPT plan based on physical dose, a prioritized optimization scheme is used to modify the LET distribution while constraining the physical dose objectives to values close to the initial plan. The LET optimization step is performed based on objective functions evaluated for the product of LET and physical dose (LET×D). To first approximation, LET×D represents a measure of the additional biological dose that is caused by high LET. Results: The method is effective for treatments where serial critical structures with maximum dose constraints are located within or near the target. We report on 5 patients with intracranial tumors (high-grade meningiomas, base-of-skull chordomas, ependymomas) in whom the target volume overlaps with the brainstem and optic structures. In all cases, high LET×D in critical structures could be avoided while minimally compromising physical dose planning objectives. Conclusion: LET-based reoptimization of IMPT plans represents a pragmatic approach to bridge the gap between purely physical dose-based and relative biological effectiveness (RBE)-based planning. The method makes IMPT treatments safer by mitigating a potentially increased risk of side effects resulting from elevated RBE of proton beams near the end of range.

Nivolumab and Ipilimumab in Treating Patients With Rare Tumors

Science.gov (United States)

2018-05-14

Acinar Cell Carcinoma; Adenoid Cystic Carcinoma; Adrenal Cortex Carcinoma; Adrenal Gland Pheochromocytoma; Anal Canal Neuroendocrine Carcinoma; Anal Canal Undifferentiated Carcinoma; Appendix Mucinous Adenocarcinoma; Bartholin Gland Transitional Cell Carcinoma; Bladder Adenocarcinoma; Cervical Adenocarcinoma; Cholangiocarcinoma; Chordoma; Colorectal Squamous Cell Carcinoma; Desmoid-Type Fibromatosis; Endometrial Transitional Cell Carcinoma; Endometrioid Adenocarcinoma; Esophageal Neuroendocrine Carcinoma; Esophageal Undifferentiated Carcinoma; Extrahepatic Bile Duct Carcinoma; Fallopian Tube Adenocarcinoma; Fallopian Tube Transitional Cell Carcinoma; Fibromyxoid Tumor; Gastric Neuroendocrine Carcinoma; Gastric Squamous Cell Carcinoma; Gastrointestinal Stromal Tumor; 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 Testicular Sex Cord-Stromal Tumor; Metaplastic Breast Carcinoma; Metastatic Malignant Neoplasm of Unknown Primary Origin; Minimally Invasive Lung Adenocarcinoma; 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; Ovarian Transitional 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

2-[F-18] fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) in uncommon malignancies

International Nuclear Information System (INIS)

Nair, N.; Basu, S.

2004-01-01

This is a retrospective study with an aim to evaluate the clinical role of FDG-PET imaging in changing the decision making process or management strategies in patients with various uncommon malignancies or in malignancies where there is paucity of literature regarding application of PET at present. The study population consisted of a wide variety of various uncommon malignancies who were mainly referred from the neighboring Tata Memorial Hospital with few cases from the outside centers. Patients were fasting at least for 6 hours. Sixty minutes after injection of 370 MBq FDG, patients were imaged on the dedicated BGO based GE Advance PET scanner (General Electric Medical systems, Milwaukee, WI). Images were reconstructed using the attenuation weighted Ordered Subsets Expectation Maximization (OSEM) algorithm. Axial, coronal, sagittal and 3D images were visually interpreted and foci of increased tracer uptake were considered as disease involvement. The findings were compared lesion by lesion with other imaging procedures regarding staging, treatment response evaluation and residual disease evaluation. The malignancies selected for retrospective analysis, along with the number of cases are presented. 2 cases of chordoma were evaluated of which one had primary in the cervicodorsal region and came for PET evaluation post surgery and RT. PET showed metastatic foci in left axillary node, body of sacrum and right lower neck region in addition to the persistence of disease in the primary. The other was a case of petroclival chordoma came for disease evaluation post surgery. The MRI was inconclusive regarding persistence of residual disease and postoperative changes. PET scan was normal in the case. In a solitary case of operated Dermato-fibrosarcoma, PET revealed a hypermetabolic focus at one end of scar, subsequently proven as scar recurrence of the primary. 3 cases of skin adenexal tumour were evaluated, all of whom were upstaged by PET and changed the subsequent

The results of gamma knife radiosurgery for malignant skull base tumors

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Tanaka, Takayuki; Kobayashi, Tatsuya; Kida, Yoshihisa; Oyama, Hirofumi; Niwa, Masahiro [Komaki City Hospital, Aichi (Japan)

1996-03-01

The results of gamma knife radiosurgery for malignant skull base tumors were analyzed using repeated magnetic resonance imagings and neurological examinations. Nineteen malignant skull base tumors were treated and followed up for 22.3 months (5-40 months) using MR imagings. The mean age was 54.4 years old (ranging from 16-85). Ten were male and 9 were female. Prior to the radiosurgery, removal of the tumors in 17 cases, conventional radiation therapy in 7, and chemotherapy in 4 etc. were performed. The pathological diagnoses were chordoma in 6 patients, metastatic tumors in 5, epipharyngeal carcinoma in 2, adenoid cystic carcinoma in 2, and others in 4. The locations of tumors were clivus in 8, parasellar region in 5, epipharynx in 2, paranasal sinus in 2, C-P angle in 1, and intraorbital region in 1 (14 intracranial and 5 extracranial). The mean diameter of the tumor was 33.5 mm. The mean maximum dose was 26.8 Gy and the mean marginal dose was 12.9 Gy during treatment. Repeated MR imagings revealed decrease of tumor size in 12 cases, showing no change in 1, and increase of tumor size in 5 (unknown in 1). Follow-up neurological examinations showed improvement in 3 patients, no change in 9, and deterioration in 7. There were 11 deaths during a mean follow-up period of 17.8 months (5-32 months) and another 8 cases are alive for a mean follow-up of 30.5 months (20-40 months) after the radiosurgery. Although the tumor size was large at the time of treatment, the results of gamma knife radiosurgery were promising. Considering the quality of life of patients with malignant skull base tumors, it is emphasized that gamma knife treatment is the method of choice compared with radical removal of the tumors. (author).

Brainstem tolerance to conformal radiotherapy of skull base tumors

International Nuclear Information System (INIS)

Debus, J.; Hug, E.B.; Liebsch, N.J.; O'Farrel, D.; Finkelstein, D.; Efird, J.; Munzenrider, J.E.

1997-01-01

Purpose: The aim of this study was to analyze the long-term incidence of brainstem toxicity in patients treated for skull base tumors with high dose conformal radiotherapy. Methods and Materials: Between 1974 and 1995, 367 patients with chordomas (n = 195) and chondrosarcomas (n = 172) of the base of skull have been treated with combined megavoltage photon and 160 MeV proton radiotherapy. Following 3D treatment planning with delineation of target volumes and critical nontarget structures dose distributions and dose-volume histograms were calculated. Radiotherapy was given an 1.8 Gy or CGE (=Cobalt Gray Equivalent) dose per fraction, with prescribed target doses ranging from 63 CGE to 79.2 CGE (mean = 67.8 CGE). Doses to the brainstem surface were limited to ≤64 CGE and to the brainstem center to ≤53 CGE. Results: Follow-up time ranged from 6 months to 21.4 years (mean = 42.5 months). Brainstem toxicity was observed in 17 of 367 patients attributable to treatment, resulting in death of three patients. Actuarial rates of 5 and 10-year high-grade toxicity-free survival were 94 and 88%, respectively. Increased risk of brainstem toxicity was significantly associated with maximum dose to brainstem, volume of brainstem receiving ≥50 CGE, ≥55 CGE, and ≥60 CGE, number of surgical procedures, and prevalence of diabetes or high blood pressure. Multivariate analysis identified three independent factors as important prognosticators: number of surgical procedures (p < 0.001), volume of the brainstem receiving 60 CGE (p < 0.001), and prevalence of diabetes (p < 0.01). Conclusions: Tolerance of brainstem to fractionated radiotherapy appears to be a steep function of tissue volume included in high dose regions rather than the maximum dose of brainstem alone. In addition, presence of predisposing factors as well as extent of surgical manipulation can significantly lower brainstem tolerance in the individual patient

Perioperative fractionated high-dose rate brachytherapy for malignant bone and soft tissue tumors

International Nuclear Information System (INIS)

Koizumi, Masahiko; Inoue, Takehiro; Yamazaki, Hideya; Teshima, Teruki; Tanaka, Eiichi; Yoshida, Ken; Imai, Atsushi; Shiomi, Hiroya; Kagawa, Kazufumi; Araki, Nobuto; Kuratsu, Shigeyuki; Uchida, Atsumasa; Inoue, Toshihiko

1999-01-01

Purpose: To investigate the viability of perioperative fractionated HDR brachytherapy for malignant bone and soft tissue tumors, analyzing the influence of surgical margin. Methods and Materials: From July 1992 through May 1996, 16 lesions of 14 patients with malignant bone and soft tissue tumors (3 liposarcomas, 3 MFHs, 2 malignant schwannomas, 2 chordomas, 1 osteosarcoma, 1 leiomyosarcoma, 1 epithelioid sarcoma, and 1 synovial sarcoma) were treated at the Osaka University Hospital. The patients' ages ranged from 14 to 72 years (median: 39 years). Treatment sites were the pelvis in 6 lesions, the upper limbs in 5, the neck in 4, and a lower limb in 1. The resection margins were classified as intracapsular in 5 lesions, marginal in 5, and wide in 6. Postoperative fractionated HDR brachytherapy was started on the 4th-13th day after surgery (median: 6th day). The total dose was 40-50 Gy/7-10 fr/ 4-7 day (bid) at 5 or 10 mm from the source. Follow-up periods were between 19 and 46 months (median: 30 months). Results: Local control rates were 75% at 1 year and 48% in 2 years, and ultimate local control was achieved in 8 (50%) of 16 lesions. Of the 8 uncontrolled lesions, 5 (63%) had intracapsular (macroscopically positive) resection margins, and all the 8 controlled lesions (100%) had marginal (microscopically positive) or wide (negative) margins. Of the total, 3 patients died of both tumor and metastasis, 3 of metastasis alone, 1 of tumor alone, and 7 showed no evidence of disease. Peripheral nerve palsy was seen in one case after this procedure, but no infection or delayed wound healing caused by tubing or irradiation has occurred. Conclusion: Perioperative fractionated HDR brachytherapy is safe, well tolerated, and applicable to marginal or wide surgical margin cases

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

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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

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

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

2014-02-15

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

Typical tumors of the petrous bone; Typische Tumoren des Felsenbeins

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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

NIRS methods of specifying carbon ion dose verification of RBE and tumour specific radiosensitivity

International Nuclear Information System (INIS)

Matsufuji, Naruhiro; Kanai, Tatsuaki; Kanematsu, Nobuyuki

2006-01-01

Clinical dose distribution of therapeutic carbon beams, currently used at National Institute of Radiological Sciences (NIRS) Heavy Ion Medical Accelerator in Chiba (HIMAC), is designed based on in-vitro Human Salivary Gland (HSG) cell survival response and clinical experiences of fast neutron radiotherapy. At first, the biological dose distribution is designed so as to cause a flat biological effect on HSG cells in spread-out Bragg peak (SOBP) region. Then, the entire biological dose distribution is evenly raised in order to attain relative biological effectiveness (RBE)=3.0 at a depth where dose-averaged linear energy transfer (LET) is 80 keV/μm. A retrospective analysis was made to examine appropriateness on the estimation of the biological effectiveness of carbon-ion radiotherapy using resultant data of clinical trials at HIMAC. Using this RBE system, over 2,700 patients have been treated by carbon beams. As a part of these patient data, local control rate of non-small lung cancer, were analysed to verify the clinical RBE of the carbon beam. The local control rate was compared with those for published by groups of Gunma University and Massachusetts General Hospital. Using a simplified tumour control probability (TCP) model, clinical RBE values were obtained for different level of the tumour control probability. For the 50% level of the clinical TCP, the RBE values nearly coincide with those of in-vitro human salivary gland cell survival at 10%. For the higher level of the clinical TCP, the RBE values approach closer to those adapted in clinical trials at HIMAC. The approach was also applied for those of chordoma, bone and soft tissue sarcoma and rectal cancer. Difference in radiosensitivity is observed for the tumours. (author)

SU-F-T-214: Re-Thinking the Useful Clinical Beam Energy in Proton Therapy: An Opportunity for Cost Reduction

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Bentefour, El H [IBA, Advanced Technology Group, Louvain La Neuve (Belgium); Lu, H [Massachusetts General Hospital and Harvard Medical School, Boston, MA (United States)

2016-06-15

Purpose: We conducted a retrospective study of the useful clinical proton beam energy based on the beam range data of patients treated over the last 10 years at Massachusetts General Hospital Proton Therapy Center. Methods: Treatment field information were collected for all patients treated over the last 10 years (2005–2015) in the two gantry treatment rooms at MGH. The beam ranges for these fields were retrieved and categorized per treatment site. The 10 prostate patients that required the highest beam range (lateral fields) were selected. For these patients, anterior oblique beams (30–40 degrees) were simulated in a planning system to obtain the required beam ranges including the margins for potential range uncertainties. Results: There were a total of 4033 patients, treated with combined total of 23603 fields. All treatment indications were considered with the exception of ocular tumors generally treated in a fixed beam room. For all non-prostate treatments (21811 fields), only 5 fields for 4 patients (1-pancreas, 1-lumbar chordoma, 2-spine mets) required beam range greater than 25 cm. There were 446 prostate patients (1792 fields), with the required beam range from 22.3 to 29.0 cm; 386 of them had at least one of their lateral beam range greater than 25 cm. For the 10 prostate patients with highest lateral beam ranges (26 to 29 cm), their treatment with anterior oblique beams would drop the beam ranges below 25 cm (17.3 to 18.5 cm). Conclusion: if prostate patients are treated with anterior fields only, the useful maximum beam range is reduced to 25 cm. Thus a proton therapy system with maximum beam energy of 196 MeV is sufficient to treat all tumors sites with very rare exceptions (<0.1%). Designing such PT system would reduce the cost of proton therapy for hospitals and patients and increase the accessibility to the treatment.

Acinic cell carcinoma of parotid gland metastasis to left cavernous sinus: a case report and review of literature

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LIN Xiao-yan

2013-12-01

Full Text Available Objective To investigate the clinical manifestations and pathological features of parotid gland papillary acinic cell carcinoma metastasis to left cavernous sinus. Methods The clinical manifestations, pathological features and differential diagnosis were studied in one case of parotid papillary acinic cell carcinoma metastasis to left cavernous sinus. Related literatures were also reviewed. Results The patient was a 50-year-old female who presented paroxysmal dizziness for 5 months and blurred vision in her left eye for 10 months. The MRI examination showed left parasellar space-occupying mass in the cavernous sinus. In operation, the tumor was located in the superior wall of left cavernous sinus, soft and red-grey in color, with abundant blood supply. The histomorphological examination revealed the tumor cells were arranged in solid, acinar or papillary pattern. The tumor cells were large, with eosinophilic cytoplasm, round or oval nuclei and small nucleoli. Immunohistochemical staining found that the tumor cells expressed cytokeratin (CK, epithelial membrane antigen (EMA, vimentin (Vim and S-100 protein (S-100, and showed weak positive expression of glial fibrillary acidic protein (GFAP and focal positive expression of P53 protein. Ki-67 labeling index was about 5%-10% . The tumor cells were negative for neuroendocrine markers and pituitary hormone protein markers. This case was difficult to differentiate from other primary intracalvarium tumors, including papillary meningioma, papillary tumor of choroid plexus, papillary ependymoma, papillary glioneuronal tumors as well as chordoma. According to the medical history and the comparison of histomorphology and immunophenotyping between parotid gland tumor cells and left cavernous sinus tumor cells, the final diagnosis was metastatic papillary parotid acinar cell carcinoma of the left cavernous sinus. The patient was followed for 21 months and no recurrence was seen. Conclusion It is very rare and

SU-D-BRC-03: Development and Validation of an Online 2D Dose Verification System for Daily Patient Plan Delivery Accuracy Check

International Nuclear Information System (INIS)

Zhao, J; Hu, W; Xing, Y; Wu, X; Li, Y

2016-01-01

Purpose: All plan verification systems for particle therapy are designed to do plan verification before treatment. However, the actual dose distributions during patient treatment are not known. This study develops an online 2D dose verification tool to check the daily dose delivery accuracy. Methods: A Siemens particle treatment system with a modulated scanning spot beam is used in our center. In order to do online dose verification, we made a program to reconstruct the delivered 2D dose distributions based on the daily treatment log files and depth dose distributions. In the log files we can get the focus size, position and particle number for each spot. A gamma analysis is used to compare the reconstructed dose distributions with the dose distributions from the TPS to assess the daily dose delivery accuracy. To verify the dose reconstruction algorithm, we compared the reconstructed dose distributions to dose distributions measured using PTW 729XDR ion chamber matrix for 13 real patient plans. Then we analyzed 100 treatment beams (58 carbon and 42 proton) for prostate, lung, ACC, NPC and chordoma patients. Results: For algorithm verification, the gamma passing rate was 97.95% for the 3%/3mm and 92.36% for the 2%/2mm criteria. For patient treatment analysis,the results were 97.7%±1.1% and 91.7%±2.5% for carbon and 89.9%±4.8% and 79.7%±7.7% for proton using 3%/3mm and 2%/2mm criteria, respectively. The reason for the lower passing rate for the proton beam is that the focus size deviations were larger than for the carbon beam. The average focus size deviations were −14.27% and −6.73% for proton and −5.26% and −0.93% for carbon in the x and y direction respectively. Conclusion: The verification software meets our requirements to check for daily dose delivery discrepancies. Such tools can enhance the current treatment plan and delivery verification processes and improve safety of clinical treatments.

SU-F-T-124: Radiation Biological Equivalent Presentations OfLEM-1 and MKM Approaches in the Carbon-Ion Radiotherapy

International Nuclear Information System (INIS)

Hsi, W; Jiang, G; Sheng, Y

2016-01-01

Purpose: To study the correlations of the radiation biological equivalent doses (BED) along depth and lateral distance between LEM-1 and MKM approaches. Methods: In NIRS-MKM (Microdosimetric Kinetic Model) approach, the prescribed BED, referred as C-Eq, doses aims to present the relative biological effectiveness (RBE) for different energies of carbon-ions on a fixed 10% survival value of HCG cell with respect to convention X-ray. Instead of a fixed 10% survival, the BED doses of LEM-1 (Local Effect Model) approach, referred as X-Eq, aims to present the RBE over the whole survival curve of chordoma-like cell with alpha/beta ratio of 2.0. The relationship of physical doses as a function of C-Eq and X-Eq doses were investigated along depth and lateral distance for various sizes of cubic targets in water irradiated by carbon-ions. Results: At the center of each cubic target, the trends between physical and C-Eq or X-Eq doses can be described by a linear and 2nd order polynomial functions, respectively. Using fit functions can then calculate a scaling factor between C-Eq and X-Eq doses to have similar physical doses. With equalized C-Eq and X-Eq doses at the depth of target center, over- and under-estimated X-Eq to C-Eq are seen for depths before and after the target center, respectively. Near the distal edge along depth, sharp rising of RBE value is observed for X-Eq, but sharp dropping of RBE value is observed for C-Eq. For lateral locations near and just outside 50% dose level, sharp raising of RBE value is also seen for X-Eq, while only minor increasing with fast dropping for C-Eq. Conclusion: An analytical function to model the differences between the CEq and X-Eq doses along depth and lateral distance need to further investigated to explain varied clinic outcome of specific cancers using two different approaches to calculated BED doses.

The future and progress of proton beam radiotherapy

International Nuclear Information System (INIS)

Tsujii, Hirohiko

1994-01-01

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

SU-D-BRC-03: Development and Validation of an Online 2D Dose Verification System for Daily Patient Plan Delivery Accuracy Check

Energy Technology Data Exchange (ETDEWEB)

Zhao, J; Hu, W [Fudan University Shanghai Cancer Center, Shanghai, Shanghai (China); Xing, Y [Fudan univercity shanghai proton and heavy ion center, Shanghai (China); Wu, X [Fudan university shanghai proton and heavy ion center, Shanghai, shagnhai (China); Li, Y [Department of Medical physics at Shanghai Proton and Heavy Ion Center, Shanghai, Shanghai (China)

2016-06-15

Purpose: All plan verification systems for particle therapy are designed to do plan verification before treatment. However, the actual dose distributions during patient treatment are not known. This study develops an online 2D dose verification tool to check the daily dose delivery accuracy. Methods: A Siemens particle treatment system with a modulated scanning spot beam is used in our center. In order to do online dose verification, we made a program to reconstruct the delivered 2D dose distributions based on the daily treatment log files and depth dose distributions. In the log files we can get the focus size, position and particle number for each spot. A gamma analysis is used to compare the reconstructed dose distributions with the dose distributions from the TPS to assess the daily dose delivery accuracy. To verify the dose reconstruction algorithm, we compared the reconstructed dose distributions to dose distributions measured using PTW 729XDR ion chamber matrix for 13 real patient plans. Then we analyzed 100 treatment beams (58 carbon and 42 proton) for prostate, lung, ACC, NPC and chordoma patients. Results: For algorithm verification, the gamma passing rate was 97.95% for the 3%/3mm and 92.36% for the 2%/2mm criteria. For patient treatment analysis,the results were 97.7%±1.1% and 91.7%±2.5% for carbon and 89.9%±4.8% and 79.7%±7.7% for proton using 3%/3mm and 2%/2mm criteria, respectively. The reason for the lower passing rate for the proton beam is that the focus size deviations were larger than for the carbon beam. The average focus size deviations were −14.27% and −6.73% for proton and −5.26% and −0.93% for carbon in the x and y direction respectively. Conclusion: The verification software meets our requirements to check for daily dose delivery discrepancies. Such tools can enhance the current treatment plan and delivery verification processes and improve safety of clinical treatments.

TH-CD-209-06: LET-Based Adjustment of IMPT Plans Using Prioritized Optimization

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Unkelbach, J; Giantsoudi, D; Paganetti, H [Massachusetts General Hospital, Boston, MA (United States); Botas, P [Massachusetts General Hospital, Boston, MA (United States); Heidelberg University, Heidelberg, DE (Germany); Qin, N; Jia, X [The University of Texas Southwestern Medical Ctr, Dallas, TX (United States)

2016-06-15

Purpose: In-vitro experiments suggest an increase in proton relative biological effectiveness (RBE) towards the end of range. However, proton treatment planning and dose reporting for clinical outcome assessment has been based on physical dose and constant RBE. Therefore, treatment planning for intensity-modulated proton therapy (IMPT) is unlikely to transition radically to pure RBE-based planning. We suggest a hybrid approach where treatment plans are initially created based on physical dose constraints and prescriptions, and are subsequently altered to avoid high linear energy transfer (LET) in critical structures while limiting the degradation of the physical dose distribution. Methods: To allow fast optimization based on dose and LET we extended a GPU-based Monte-Carlo code towards providing dose-averaged LET in addition to dose for all pencil beams. After optimizing an initial IMPT plan based on physical dose, a prioritized optimization scheme is used to modify the LET distribution while constraining the physical dose objectives to values close to the initial plan. The LET optimization step is performed based on objective functions evaluated for the product of physical dose and LET (LETxD). To first approximation, LETxD represents a measure of the additional biological dose that is caused by high LET. Regarding optimization techniques, LETxD has the advantage of being a linear function of the pencil beam intensities. Results: The method is applicable to treatments where serial critical structures with maximum dose constraint are located in or near the target. We studied intra-cranial tumors (high-grade meningiomas, base-of-skull chordomas) where the target (CTV) overlaps with the brainstem and optic structures. Often, high LETxD in critical structures can be avoided while minimally compromising physical dose planning objectives. Conclusion: LET-based re-optimization of IMPT plans represents a pragmatic approach to bridge the gap between purely physical dose

Including robustness in multi-criteria optimization for intensity-modulated proton therapy

Science.gov (United States)

Chen, Wei; Unkelbach, Jan; Trofimov, Alexei; Madden, Thomas; Kooy, Hanne; Bortfeld, Thomas; Craft, David

2012-02-01

We present a method to include robustness in a multi-criteria optimization (MCO) framework for intensity-modulated proton therapy (IMPT). The approach allows one to simultaneously explore the trade-off between different objectives as well as the trade-off between robustness and nominal plan quality. In MCO, a database of plans each emphasizing different treatment planning objectives, is pre-computed to approximate the Pareto surface. An IMPT treatment plan that strikes the best balance between the different objectives can be selected by navigating on the Pareto surface. In our approach, robustness is integrated into MCO by adding robustified objectives and constraints to the MCO problem. Uncertainties (or errors) of the robust problem are modeled by pre-calculated dose-influence matrices for a nominal scenario and a number of pre-defined error scenarios (shifted patient positions, proton beam undershoot and overshoot). Objectives and constraints can be defined for the nominal scenario, thus characterizing nominal plan quality. A robustified objective represents the worst objective function value that can be realized for any of the error scenarios and thus provides a measure of plan robustness. The optimization method is based on a linear projection solver and is capable of handling large problem sizes resulting from a fine dose grid resolution, many scenarios, and a large number of proton pencil beams. A base-of-skull case is used to demonstrate the robust optimization method. It is demonstrated that the robust optimization method reduces the sensitivity of the treatment plan to setup and range errors to a degree that is not achieved by a safety margin approach. A chordoma case is analyzed in more detail to demonstrate the involved trade-offs between target underdose and brainstem sparing as well as robustness and nominal plan quality. The latter illustrates the advantage of MCO in the context of robust planning. For all cases examined, the robust optimization for

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

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

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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

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

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

2014-04-01

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

Small bowel toxicity after high dose spot scanning-based proton beam therapy for paraspinal/retroperitoneal neoplasms

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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.)

Helical tomotherapy. Experiences of the first 150 patients in Heidelberg

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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

Myxoid Chondrosarcoma of the Sinonasal Cavity in a Child: a Case Report

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Kim, Yeo Ju; Im, Soo Ah; Lim, Gye Yeon; Chun, Ho Jong; Park, Hyun Jin; Kim, Min Sik; Choi, Yeong Jin [The Catholic University of Korea, Seoul (Korea, Republic of)

2007-10-15

Chondrosarcomas are malignant tumors of cartilage that rarely involve the sinonasal region, and myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma that usually occurs in the soft tissue of extremities. Although several case reports and results of small series of chondrosarcomas in the sinonasal region in children are available, myxoid type chondrosarcoma is extremely rare. We recently experienced a case of low grade myxoid chondrosarcoma involving the sinonasal cavity in a 10-year-old boy, and here we report its radiologic-pathologic findings. In this case, chondroid calcification on CT and septal and marginal enhancement on MRI suggested a chondrosarcoma. Whole body PET-CT demonstrated no definite metastatic lesion and a low peak standardized uptake value primary tumor. However, no definite distinguishing imaging features were observed that distinguished low grade myxoid chondrosarcoma from conventional chondrosarcoma. hondrosarcomas are malignant mesenchymal tumors of cartilage, and usually involve the long bone and pelvis. Less than 10% of chondrosarcomas are found in the head and neck region. Chondrosarcomas can occur at any age, but the majority present between the 5th and 7th decades. Therefore, chondrosarcomas of the head and neck in children are rare; only about 13 cases of sinonasal chondrosarcoma in children have been reported. Myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma, and is characterized by abundant chondroid matrix and malignant chondroblastic cells arranged in cords resembling chordoma. Myxoid chondrosarcomas are typically located in the limbs in older patients, and only rarely originate in the head and neck in children. To the best of our knowledge, only one case report of myxoid type chondrosarcoma in the sinonasal region in a child is available. We report a case of myxoid chondrosarcoma involving the sinonasal cavity in a child and describe its computed tomography (CT), magnetic resonance imaging (MRI

Myxoid Chondrosarcoma of the Sinonasal Cavity in a Child: a Case Report

International Nuclear Information System (INIS)

Kim, Yeo Ju; Im, Soo Ah; Lim, Gye Yeon; Chun, Ho Jong; Park, Hyun Jin; Kim, Min Sik; Choi, Yeong Jin

2007-01-01

Chondrosarcomas are malignant tumors of cartilage that rarely involve the sinonasal region, and myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma that usually occurs in the soft tissue of extremities. Although several case reports and results of small series of chondrosarcomas in the sinonasal region in children are available, myxoid type chondrosarcoma is extremely rare. We recently experienced a case of low grade myxoid chondrosarcoma involving the sinonasal cavity in a 10-year-old boy, and here we report its radiologic-pathologic findings. In this case, chondroid calcification on CT and septal and marginal enhancement on MRI suggested a chondrosarcoma. Whole body PET-CT demonstrated no definite metastatic lesion and a low peak standardized uptake value primary tumor. However, no definite distinguishing imaging features were observed that distinguished low grade myxoid chondrosarcoma from conventional chondrosarcoma. hondrosarcomas are malignant mesenchymal tumors of cartilage, and usually involve the long bone and pelvis. Less than 10% of chondrosarcomas are found in the head and neck region. Chondrosarcomas can occur at any age, but the majority present between the 5th and 7th decades. Therefore, chondrosarcomas of the head and neck in children are rare; only about 13 cases of sinonasal chondrosarcoma in children have been reported. Myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma, and is characterized by abundant chondroid matrix and malignant chondroblastic cells arranged in cords resembling chordoma. Myxoid chondrosarcomas are typically located in the limbs in older patients, and only rarely originate in the head and neck in children. To the best of our knowledge, only one case report of myxoid type chondrosarcoma in the sinonasal region in a child is available. We report a case of myxoid chondrosarcoma involving the sinonasal cavity in a child and describe its computed tomography (CT), magnetic resonance imaging (MRI

Critical evaluation of contemporary management in a new Pelvic Exenteration Unit: The first 25 consecutive cases.

Science.gov (United States)

Chew, Min Hoe; Yeh, Yu-Ting; Toh, Ee-Lin; Sumarli, Stephen Aditya; Chew, Ghee Kheng; Lee, Lui Shiong; Tan, Mann Hong; Hennedige, Tiffany Priyanthi; Ng, Shin Yi; Lee, Say Kiat; Chong, Tze Tec; Abdullah, Hairil Rizal; Goh, Terence Lin Hon; Rasheed, Mohamed Zulfikar; Tan, Kok Chai; Tang, Choong Leong

2017-05-15

To critically appraise short-term outcomes in patients treated in a new Pelvic Exenteration (PE) Unit. This retrospective observational study was conducted by analysing prospectively collected data for the first 25 patients (16 males, 9 females) who underwent PE for advanced pelvic tumours in our PE Unit between January 2012 and October 2016. Data evaluated included age, co-morbidities, American Society of Anesthesiologists (ASA) score, Eastern Cooperative Oncology Group (ECOG) status, preoperative adjuvant treatment, intra-operative blood loss, procedural duration, perioperative adverse event, lengths of intensive care unit (ICU) stay and hospital stay, and oncological outcome. Quantitative data were summarized as percentage or median and range, and statistically assessed by the χ 2 test or Fisher's exact test, as applicable. All 25 patients received comprehensive preoperative assessment via our dedicated multidisciplinary team approach. Long-course neoadjuvant chemoradiotherapy was provided, if indicated. The median age of the patients was 61.9-year-old. The median ASA and ECOG scores were 2 and 0, respectively. The indications for PE were locally invasive rectal adenocarcinoma ( n = 13), advanced colonic adenocarcinoma ( n = 5), recurrent cervical carcinoma ( n = 3) and malignant sacral chordoma ( n = 3). The procedures comprised 10 total PEs, 4 anterior PEs, 7 posterior PEs and 4 isolated lateral PEs. The median follow-up period was 17.6 mo. The median operative time was 11.5 h. The median volume of blood loss was 3306 mL, and the median volume of red cell transfusion was 1475 mL. The median lengths of ICU stay and of hospital stay were 1 d and 21 d, respectively. There was no case of mortality related to surgery. There were a total of 20 surgical morbidities, which occurred in 12 patients. The majority of the complications were grade 2 Clavien-Dindo. Only 2 patients experienced grade 3 Clavien-Dindo complications, and both required procedural interventions

Preliminary experience with frameless stereotactic radiotherapy

International Nuclear Information System (INIS)

Buatti, John M.; Bova, Frank J.; Friedman, William A.; Meeks, Sanford L.; Ellis, Thomas L.; Marcus, Robert B.; Zuofeng, Li; Mendenhall, William M.

1997-01-01

Purpose/Objective: To report our initial clinical experience using a novel high-precision frameless stereotactic radiotherapy system in 50 patients who have received 1271 treatments. Materials and Methods: Fifty patients ranging in age from 2 to 72 yr were treated with fractionated stereotactic radiotherapy. Thirty-two were treated with stereotactic radiotherapy alone, and 18 had stereotactic radiotherapy interdigitated as a boost in addition to standard irradiation. Pathologies treated included meningioma (13), low grade astrocytoma (10), germinoma (9), craniopharyngioma (4), schwannoma (2), and pituitary adenoma (2). Two additional patients had miscellaneous benign neoplasms and 8 patients had the technique used as a dose escalation strategy for malignant lesions including chordoma, primitive neuroectodermal tumor, sarcoma, and anaplastic oligoastrocytoma. Treatment reproducibility was initially gauged by comparing the bite plate position using infrared light emitting diodes (irleds) with the stereotactic radiosurgery reference system. This test of accuracy consisted of 10 bite plate repositionings for each patient and 100 readings of each of the 6 irleds on the bite plate at each new position. Each of the 1271 patient treatments was monitored for continuous digital position, and a reading was made before treating each arc of radiation. We chose 0.3 mm translation and 0.3 degrees rotation as the maximum tolerated misalignment before treating each arc. Results: With a mean follow-up of 9 mo, no patient had a marginal or distal failure. One patient with a malignant glioma had central disease progression. Acute side effects were minimal. In 3 of 9 low grade astrocytomas, a marked increase in imaging enhancement and edema occurred in the first year after treatment that resolved with steroids. The initial test of accuracy revealed bite plate reproducibility as follows. Translational errors (mm): Anterior-posterior, 0.06 ± 0.06; lateral, 0.03 ± 0.05; axial, 0.07 ± 0

Impact of tissue specific parameters on the predition of the biological effectiveness for treatment planning in ion beam therapy

International Nuclear Information System (INIS)

Gruen, Rebecca Antonia

2014-01-01

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

[Maxillary swing approach in the management of tumors in the central and lateral cranial base].

Science.gov (United States)

Liao, Hua; Hua, Qing-quan; Wu, Zhan-yuan

2006-04-01

A retrospective review of seventeen patients who were operated through the maxillary swing approach was carried out to assess the efficacy of this approach in the management of tumors of the central and lateral cranial base. From May 2000 to January 2005, 17 patients with primary or recurrent neoplasms involving the central cranial or lateral base underwent surgical resection via maxillary swing approach. Ten patients were male, and other seven patients were female, and age range was 7 to 58 years, with a mean age of 42. 6 years. Eight patients had tumors originally involving lateral cranial base, and nine patients had tumors originated from central cranial base. The pathology spectrum was very wide. Among them, five suffered from chordoma, two had rhabdomyosarcoma, two had squamous cell carcinoma, one had malignant fibrous histiocytoma, one had malignant melanoma, one had esthesioneuroblastoma, one had invaded hypophysoma, two had schwannoma, one had pleomorphic adenoma, and one had angiofibroma. Three patients had received previous surgery, two patients had previous radiation therapy and nine patients received postoperative radiotherapy. Sixteen of all seventeen patients had oncologically complete resection, one had near-total resection. This group patients was followed up from 10 to 60 months, with a median follow-up time of 28 months. Two patients died 14 and 26 months after surgery respectively, as a result of local recurrence and metastasis. One patient defaulted follow-up at 12 months after operation, and the other 14 patients were alive at the time of analysis. Of the 12 malignant tumors, the 1-and 2-year survival rate were 91.67% and 72.92%, respectively. The facial wounds of all patients healed primarily, and there were no necrosis of the maxilla, damage of the temporal branch of the facial nerve, lower-lid ectropion, and facial deformity. Epiphora and facial hypoesthesia were detected in all patients. Four patients (23.5%) developed palatal fistula, ten

Tumors of the connective and supporting tissues

International Nuclear Information System (INIS)

Suit, Herman

1995-01-01

morbidity/functional decrement consequent upon the surgery judged reasonable; proton beam radiation therapy has been accepted as being superior to conventional external beam radiation therapy for chondrosarcoma and chordoma of the skull base; and attempts to utilize brachytherapy for sarcomas of the spine/sacrum appear to offer promise. Projected advances in the coming two decades include: designation of sarcoma type on genetic characterization; molecular genetics will provide prognostic information as to probability of distant metastasis, response to chemotherapeutic agents and radiation; important further reductions in the radiation treatment volume due to the many technical developments entering, or soon to enter, the clinic; non-invasive assessment of the response to chemotherapy; much increased appreciation of the late sequella of treatment, both radiation and chemotherapy

Optic neuropathy following combined proton and photon radiotherapy for base of skull tumors

International Nuclear Information System (INIS)

Kim, June; Munzenrider, John; Maas, Alicea; Finkelstein, Dianne; Liebsch, Norbert; Hug, Eugen; Suit, Herman; Smith, Al; Goitein, Michael

1997-01-01

Purpose/Objective: To evaluate the risk of radiation injury to the optic pathway following high dose radiation therapy (RT) for base of skull tumors with regard to the following variables: diabetes, hypertension, number of surgical procedures, use of patch, patch distance, radiation dose, and volume of optic structures receiving 50, 55, or 60 Cobalt Gray Equivalent (CGE). Materials and Methods: A total of 359 patients with base of skull chordoma or low grade chondrosarcoma received high dose radiation therapy. Patients were treated with external beam radiotherapy utilizing protons alone or combined protons and photons. Protons of 160 MeV were delivered at the Harvard Cyclotron Laboratory using a modulated Bragg peak. The tumor dose ranged from 61 to 76 CGE. CGE was used because modulated protons have an RBE of 1.1 compared to 60 Co. Among 359 patients, 85 patients were excluded from evaluation based on age, tumor location, and pre-RT treatment criteria. All 274 evaluable patients had a minimum follow up of 12 months. Medical records were reviewed to determine the actual cause of vision changes. A total of 12 patients with grade II, III, and IV radiation-induced optic neuropathy were identified. Twenty-four patients without complications who closely matched the aforementioned 12 cases with optic neuropathy were selected from the 274 patients as a control group. Dose volume histograms of 12 cases and 24 controls were reviewed to determine minimum, median, and maximum dose to the optic apparatus as well as dose volume at 50, 55, and 60 CGE. Other information regarding remaining potential risk factors, such as diabetes, hypertension, number of surgical procedures, use of patch, and patch distance, was also obtained. Results: A total of 12 patients (4.4%) developed radiation-induced optic neuropathy: 1 grade II, 9 grade III, and 2 grade IV. Specific sites of involvement were left optic nerve in 9, right optic nerve in 5, and chiasm in 4 cases. The duration to the onset

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.

Brainstem tolerance to conformal radiotherapy of skull base tumors

International Nuclear Information System (INIS)

Debus, J.; Hug, E.B.; Munzenrider, J.E.; Liebsch, N.J.; O'Farrell, D.; Efird, J.; Daly, W.; Suit, H.D.

1996-01-01

Purpose/Objective: Brainstem tolerance to inhomogenous radiation doses applied by modern conformal radiotherapy has not yet been examined. The aim of this study was to analyse the incidence of brainstem toxicity in patients treated for skull base tumors with high dose conformal radiotherapy. Materials and Methods: Between 1974 and 1995, 367 patients with chordomas (n=195) and chondrosarcomas (n=172) of the base of skull have been treated with combined megavoltage photon and 160 MeV proton radiotherapy. All patients had previously undergone biopsy, subtotal or total tumor removal. 104 patients had two or more surgical procedures before radiotherapy. Following 3D treatment planning with delineation of target volumes and critical non-target structures, dose distributions and dose volume histograms were calculated [at the time of treatment delivery]. Radiotherapy was given once a day, 1.8 Gy or CGE (Cobalt Gy Equivalent: Proton Gy X 1.1) per fraction, 5 fractions per week, with prescribed target doses ranging from 63 CGE to 79.2 CGE (mean = 67.8 CGE). Doses to the brainstem surface were limited to ≤64 CGE and to the brainstem center to ≤53 CGE. Dose distributions were developed to limit dose to brainstem surface and center; current plans limit dose to surface and center to ≤64 CGE and ≤53 CGE, respectively. Brainstem toxicity was scored according to the RTOG grading system. Results: Follow-up ranged from 6 months to 21.4 years (mean = 42.5 months). Brainstem symptoms, attributable to the treatment, developed in 17 of 282 patients with local tumor control (6.0%), resulting in death of three patients. The mean time to onset of symptoms was 17 months (range: 4.5 to 177 months). These symptoms appeared in 89.5% within 3 years. Grading of the brainstem toxicity is listed in table 1. Actuarial rates of 5 and 10 year toxicity free survival were 87% and 82% respectively. Increased risk of brainstem toxicity was significantly associated with maximum brainstem dose

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

CLINICAL ANALYSIS OF SERUM INTERLEUKIN-16 AND VASCULAR ENDOTHELIAL GROWTH FACTOR LEVELS DEPENDING ON MORPHOLOGICAL CHARACTERISTICS OF THE TUMORS AND LONG-TERM TREATMENT OUTCOMES IN PATIENTS WITH BONE NEOPLASMS

Directory of Open Access Journals (Sweden)

I. V. Babkina

2016-01-01

Full Text Available Background: The progress in cancer treatment, including bone malignancies, is associated with advances in molecular biology. Based on the results of a  number of studies, treatment of bone sarcomas have been expanded with targeted therapy that uses drugs with targeted actions, including anti-angiogenic and bevacizumab, in particular. It inhibits the binding of a key activator of neoangiogenesis, vascular endothelial growth factor (VEGF, with its receptors type 1 and 2 (Flt-1 and KDR on the surface of endothelial cells, which results in a  decrease in vascularization and in inhibition of tumor growth. Beyond VEGF, other activators of neoangiogenesis have been identified, such as interleukin 16 (IL-16. Aim: To compare baseline serum IL-16 and VEGF in patients with malignant, borderline and benign bone tumors. Materials and methods: Serum IL-16 and VEGF levels was compared in 138 patients with primary bone tumors: benign (n=10; borderline (giant cell bone, n=22; malignant (n=106, aged 14 to 50 years, by immunoenzyme assay (Biosource, USA for IL-16 and R&D, USA for VEGF before any specific treatment. Bone malignancies were identified as osteosarcoma (n=45, among them 35  typical, 6 parosteal, and 4 periosteal, chondrosarcoma (n=24, Ewing sarcoma (n=27, and undifferentiated pleomorphic sarcoma (n=7 and chordoma (n=3. Results: The rate of IL-16 identification in the serum of bone tumors patients was 93%, with no significant differences depending on the histological structure of the tumor. No association between the size of primary tumors and IL-16 serum levels was found. Overall 3 and 5-year survival of patients with malignant bone tumors with IL-16 serum levels>33 pg/mL was significantly lower than in those IL-16 levels of≤33 pg/mL. Overall 5-year survival in osteosarcoma patients with higher IL-16 serum levels 1.6-fold lower, in Ewing sarcoma patients, 1.7-fold lower, and in chondrosarcoma patients, 1.8-fold lower than that the patients with

Rubitecan: 9-NC, 9-Nitro-20(S)-camptothecin, 9-nitro-camptothecin, 9-nitrocamptothecin, RFS 2000, RFS2000.

Science.gov (United States)

2004-01-01

submitted to the FDA. The results of the study showed that rubitecan could not help all chemotherapy-resistant patients, but could increase survival in those that do respond. The other phase III pivotal trial was conducted in patients with pancreatic cancer who had failed treatment with gemcitabine. This trial completed enrollment in October 2001, and had enrolled approximately 448 patients. SuperGen is conducting phase II trials of rubitecan in patients with solid tumours in the UK, Italy, France, Germany, the Netherlands and Denmark. Each trial will enroll 100-150 patients with various tumour types, including colorectal, lung, breast, gastric, prostate, cervical and head and neck cancers. Phase I/II trials are underway to investigate rubitecan as a radiosensitiser in patients with lung cancer, and phase II trials in patients with breast cancer are also being conducted. A phase II study in ovarian cancer patients is also being conducted. Results from an ongoing phase II study in cancer patients have shown that rubitecan was effective against chordomas, a rare type of bone cancer. Phase II studies are also underway in haematological malignancies including myelodysplastic syndrome (preleukaemia) and chronic myelomonocytic leukaemia. In February 2000, SuperGen announced that its IND submission for rubitecan had been approved by the Therapeutics Products Programme of Canada. The company stated that it intended to begin clinical trials in Canada in the near future. In February 2004, SuperGen announced an offering of shares of its common stock to finance the commercialisation of rubitecan capsules. In July 2003, SuperGen was granted a US patent covering combination therapies with chemotherapeutic anthracycline agents and structural modifications that may one day lead to next-generation rubitecan compounds. In December 2002, SuperGen was granted US patent No. 6,482,830, covering its polymorphic formulations of rubitecan. The patent also covers a class of polymorphs that are