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

International Nuclear Information System (INIS)

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

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

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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 ...

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

International Nuclear Information System (INIS)

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.

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.

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

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.

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

Science.gov (United States)

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

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.)

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.)

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

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

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.)

Lumbar spine chordoma

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

2014-01-01

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

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

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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.

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

Science.gov (United States)

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.

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

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

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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

Augmented reality-assisted skull base surgery.

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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.

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.

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

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

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

Isolated Petroclival Craniopharyngioma with Aggressive Skull Base Destruction

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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

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.

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

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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.

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

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.

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

Proton radiation therapy for clivus chordoma

International Nuclear Information System (INIS)

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)

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

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

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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.

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

International Nuclear Information System (INIS)

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

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).

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.

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.

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

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

Directory of Open Access Journals (Sweden)

Ying Zhang

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

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

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

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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.

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.

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

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

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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.

Skull base tumours

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Borges, Alexandra [Instituto Portugues de Oncologia Francisco Gentil, Servico de Radiologia, Rua Professor Lima Basto, 1093 Lisboa Codex (Portugal)], E-mail: borgesalexandra@clix.pt

2008-06-15

With the advances of cross-sectional imaging radiologists gained an increasing responsibility in the management of patients with skull base pathology. As this anatomic area is hidden to clinical exam, surgeons and radiation oncologists have to rely on imaging studies to plan the most adequate treatment. To fulfil these endeavour radiologists need to be knowledgeable about skull base anatomy, about the main treatment options available, their indications and contra-indications and needs to be aware of the wide gamut of pathologies seen in this anatomic region. This article will provide a radiologists' friendly approach to the central skull base and will review the most common central skull base tumours and tumours intrinsic to the bony skull base.

Skull base tumours

International Nuclear Information System (INIS)

Borges, Alexandra

2008-01-01

With the advances of cross-sectional imaging radiologists gained an increasing responsibility in the management of patients with skull base pathology. As this anatomic area is hidden to clinical exam, surgeons and radiation oncologists have to rely on imaging studies to plan the most adequate treatment. To fulfil these endeavour radiologists need to be knowledgeable about skull base anatomy, about the main treatment options available, their indications and contra-indications and needs to be aware of the wide gamut of pathologies seen in this anatomic region. This article will provide a radiologists' friendly approach to the central skull base and will review the most common central skull base tumours and tumours intrinsic to the bony skull base

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).

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

Skull base tumours part I: Imaging technique, anatomy and anterior skull base tumours

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Borges, Alexandra [Instituto Portugues de Oncologia Francisco Gentil, Centro de Lisboa, Servico de Radiologia, Rua Professor Lima Basto, 1093 Lisboa Codex (Portugal)], E-mail: borgesalexandra@clix.pt

2008-06-15

Advances in cross-sectional imaging, surgical technique and adjuvant treatment have largely contributed to ameliorate the prognosis, lessen the morbidity and mortality of patients with skull base tumours and to the growing medical investment in the management of these patients. Because clinical assessment of the skull base is limited, cross-sectional imaging became indispensable in the diagnosis, treatment planning and follow-up of patients with suspected skull base pathology and the radiologist is increasingly responsible for the fate of these patients. This review will focus on the advances in imaging technique; contribution to patient's management and on the imaging features of the most common tumours affecting the anterior skull base. Emphasis is given to a systematic approach to skull base pathology based upon an anatomic division taking into account the major tissue constituents in each skull base compartment. The most relevant information that should be conveyed to surgeons and radiation oncologists involved in patient's management will be discussed.

Skull base tumours part I: Imaging technique, anatomy and anterior skull base tumours

International Nuclear Information System (INIS)

Borges, Alexandra

2008-01-01

Advances in cross-sectional imaging, surgical technique and adjuvant treatment have largely contributed to ameliorate the prognosis, lessen the morbidity and mortality of patients with skull base tumours and to the growing medical investment in the management of these patients. Because clinical assessment of the skull base is limited, cross-sectional imaging became indispensable in the diagnosis, treatment planning and follow-up of patients with suspected skull base pathology and the radiologist is increasingly responsible for the fate of these patients. This review will focus on the advances in imaging technique; contribution to patient's management and on the imaging features of the most common tumours affecting the anterior skull base. Emphasis is given to a systematic approach to skull base pathology based upon an anatomic division taking into account the major tissue constituents in each skull base compartment. The most relevant information that should be conveyed to surgeons and radiation oncologists involved in patient's management will be discussed

Cytodiagnosis of Sacral Chordoma

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Saumya Shukla

2014-12-01

Full Text Available We report the cytological findings of a sacro-coccygeal chordoma in a 53 year male diagnosed preoperatively by guided fine needle aspiration cytology. The smears shows characteristic Physalliphorous cells in a metachromatic background of myxoid material entrapping cords of cuboidal cells. Differential diagnosis in cytology include conventional and myxoid chondrosarcoma, myxoid liposarcoma, myxoid malignant fibrous histiocytoma, metastatic mucinous carcinoma and myxo-papillary ependymoma. The distinguishing features between these neoplasms are discussed. Preoperative diagnosis of chordoma permits optimum planned surgery. Keywords: chordoma; myxoid; sacral.

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

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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 | Fitchart | South African Medical Journal

African Journals Online (AJOL)

The history, origin, pathology, diagnosis, treatment and prognosis of chordoma involving the axial skeleton are reviewed, and the clinical histories of 3 cases of chordoma are given, noting some remarkable features, along with one case misdiagnosed as chordoma on the radiological appearance of the sacrum and treated ...

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

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

Hemorrhagic chondroid chordoma mimicking pituitary apoplexy

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

1998-11-01

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

Petrous apex chordoma - a case report

International Nuclear Information System (INIS)

Silveira, Claudio Regis S.; Barreto, Cristina Marques; Rossi, Luis Antonio; Michiloski, Custodio; Rotta, Jose Marcus; Almeida, Serguey Malaquias de

2001-01-01

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)

Imaging of skull base: Pictorial essay

International Nuclear Information System (INIS)

Raut, Abhijit A; Naphade, Prashant S; Chawla, Ashish

2012-01-01

The skull base anatomy is complex. Numerous vital neurovascular structures pass through multiple channels and foramina located in the base skull. With the advent of computerized tomography (CT) and magnetic resonance imaging (MRI), accurate preoperative lesion localization and evaluation of its relationship with adjacent neurovascular structures is possible. It is imperative that the radiologist and skull base surgeons are familiar with this complex anatomy for localizing the skull base lesion, reaching appropriate differential diagnosis, and deciding the optimal surgical approach. CT and MRI are complementary to each other and are often used together for the demonstration of the full disease extent. This article focuses on the radiological anatomy of the skull base and discusses few of the common pathologies affecting the skull base

Combined Therapy for Distant Metastasis of Sacral Chordoma

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Birol Özkal

2015-01-01

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

Clival chordomas

International Nuclear Information System (INIS)

Schamschula, R.G.; Soo, M.Y.S.

1993-01-01

Three cases of clival chordomas are reviewed and the findings are compared to those in the recent literature. Chordomas are rare neoplasms that arise from primitive notochord remnants. In all three cases, computed tomography was an excellent modality for demonstrating bone destruction, sequestra and calcification but inferior to magnetic resonance imaging (MRI) in demonstrating the soft tissue extent of the tumour. The multiplanar capability of MRI was found to be particularly useful for planning treatment. Vertebral angiography can demonstrate the tumours by vessel displacement, encasement and vascular staining. Two cases had angiography and demonstrated tumour blush. Pre-operative embolization was helpful in one case. 13 refs., 1 tab., 6 figs

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.

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

Intracranial metastasis from a sacrococcygeal chordoma. Case report.

LENUS (Irish Health Repository)

Kamel, Mahmoud Hamdy

2012-02-03

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

Radiation therapy for chordomas

International Nuclear Information System (INIS)

Ikeda, Hajime; Takahashi, Takeo; Nakamura, Yuji; Niibe, Hideo

1995-01-01

Chordomas are slow-growing primary malignant bone tumors which originate from remnants of the fetal notochordal system. They are difficult to control by surgery alone. Four patients with chordomas treated with radiation therapy were studied, and the effectiveness of radiotherapy was evaluated. These 4 (3.8%) patients were among 106 patients with primary malignant bone tumors referred to us from 1959 to 1987. Primary sites were the sacrococcygeal region in three patients and the clivus in one. The patients' ages ranged from 51 to 75 years. The male : female ratio was 1 : 1. Patients received 48 to 60 Gy of radiation to the primary sites. Because the radiosensitivity of the tumors was low, the responses were poor. The duration of survival was 6, 33, 68, and 125 months. The cause of death in each case was local recurrence of tumor. As a result, a dose greater than 60 Gy is thought to be necessary for curative radiotherapy. Proton beam therapy seems to be best choice for chordomas in the clivus, and mixed-beam (proton and megavolt age X-ray) therapy or multiportal irradiation, which gives an ideal spatial dose distribution, seems to be most suitable for sacrococcygeal chordomas. (author)

The contribution of high-resolution multiplanar reformats of the skull base to the detection of skull-base fractures

International Nuclear Information System (INIS)

Connor, S.E.J.; Flis, C.

2005-01-01

AIM: To investigate the contribution of routine review of submillimetric multiplanar reformats to the diagnosis of skull-base fractures. METHODS: A prospective analysis was performed of 407 cases referred over a 6-month period for CT of the skull following cranial trauma. The reformatted 5-mm axial sections and subsequently the high-resolution multiplanar reformats (HRMPRs) were viewed on an ADW 4.1 workstation using bone windows and algorithm. All skull-base fractures and related features, recorded by the consensus of two radiologists, were classified as anatomically significant or non-significant on the basis of eight criteria. The clinical features of skull-base injury and any subsequent treatment were noted in all cases of skull-base fracture. RESULTS: HRMPRs detected 80 separate skull-base fractures in 36/407 cases. Of these 80 fractures, 57 were visible on 5-mm axial sections. In 8 of the 36 cases, the significant anatomical features were only evident on review of the HRMPRs. In 6 of the 36 cases, none of the skull-base fractures was visible on 5-mm sections, but these individuals had only minor associated clinical features and no therapeutic requirements. Review of HRMPRs could have been confined to patients with skull-base fractures, abnormal intracranial and extracranial air collections or opacified mastoid air cells revealed by 5-mm axial sections. This policy would have led to the detection of 79/80 (99%) of skull-base fractures and all significant anatomical features. CONCLUSION: The 5-mm axial sections demonstrated 71% of skull-base fractures and 78% of skull-base fractures with significant anatomical features, using HRMPRs as a gold standard. There were no significant clinical sequelae at short-term follow-up of those fractures only evident on HRMPRs

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

CT atlas of the skull base

International Nuclear Information System (INIS)

Inoue, Hiroshi; Kawafuchi, Jun-ichi; Takahashi, Kazukuni

1980-01-01

Although CT is generally used for lesions of the face, the orbit, the nasal and paranasal cavity, and the skull base, a CT atlas of these regions has not been reported. Furthermore, the skull base, that lies nearly tangential to the conventional axial plane of CT, can not be precisely evaluated on ordinary horizontal pictures. For the purpose of a clear demonstration of the skull-base structures by CT, a model human skull was investigated. The results and its clinical value have previously been reported. For the CT atlas of the skull base, three model human skulls (embedded in agar gel containing iodine in a manner previously reported) were also examined by EMI-CT1010 with a 5 mm thickness. The magnification and wide-window techniques were also used for demonstration. Ordinary-0 sections (scanning plane at 0 0 to Reid's base line), ordinary-25 sections (+25 0 to RBL), reverse-20 sections (-20 0 to RBL), reverse-80 sections (-80 0 to RBL; coronal sections), and sagittal sections were selected in order to illustrate the anatomical details of the skull base. Pictures of the inner aspect and the outer aspect of the skull base were also provided. Clinically it is very important to recognize osseous change and the relationship between the lesion and the skull base in three dimensions. In evaluating lesions of the skull base and those of the tentorial notch a two-plane CT examination (ordinary-25 sections and reverse-20 sections) is usually used. This method is useful in determining the surgical approach, for instance, to decide between a transsphenoidal approach or intracranial approach for a sellar lesion, or between a subtemporal approach, posterior fossa approach, or combined approach for a lesion of the tentorial notch. It is also helpful to make a map of the lesin on a plain craniogram using this two-plane method in some cases for radiotherapy and stereotactic brain biopsy. (author)

A unique presentation of retroclival chordoma.

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Warakaulle D

2002-10-01

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

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

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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.

Imaging in tuberculosis of the skull and skull-base: case report

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Sencer, S.; Aydin, K.; Poyanli, A.; Minareci, O.; Sencer, A.; Hepguel, K.

2003-01-01

We report a 19-year-old girl, who presented with headache and tonic/clonic seizures. Imaging revealed a lytic parietal skull lesion with an adjacent epidural mass, masses in the right parietal lobe and a posterior skull-base mass. The diagnosis of tuberculosis was made after resection of the extradural mass and later verified with culture of Mycobacterium tuberculosis. The parenchymal and skull-base lesions resolved following antituberculous treatment. We present CT, scintigraphic, angiographic and MRI findings. (orig.)

Differential proteomic profiling of primary and recurrent chordomas.

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Chen, Su; Xu, Wei; Jiao, Jian; Jiang, Dongjie; Liu, Jian; Chen, Tenghui; Wan, Zongmiao; Xu, Leqin; Zhou, Zhenhua; Xiao, Jianru

2015-05-01

Chordomas are locally destructive tumors with high rates of recurrence and a poor prognosis. The mechanisms involved in chordoma recurrence remain largely unknown. In the present study, we examined the proteomic profile of a chordoma primary tumor (CSO) and a recurrent tumor (CSR) through mass spectrum in a chordoma patient who underwent surgery. Bioinformatic analysis of the profile showed that 359 proteins had a significant expression difference and 21 pathways had a striking alteration between the CSO and the CSR. The CSR showed a significant increase in carbohydrate metabolism. Immunohistochemistry (IHC) confirmed that the cancer stem cell marker activated leukocyte cell adhesion molecule (ALCAM or CD166) expression level was higher in the recurrent than that in the primary tumor. The present study analyzed the proteomic profile change between CSO and CSR and identified a new biomarker ALCAM in recurrent chordomas. This finding sheds light on unraveling the pathophysiology of chordoma recurrence and on exploring more effective prognostic biomarkers and targeted therapies against this devastating disease.

Thoracic chordoma: CT and MR findings

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Cha, Yoo Mi; Hwang, Hee Young; Kim, Sang Joon; Chung, Hyo Sun; Han, Heon

1993-01-01

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

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 .

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

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Baek, Seung Hwan; Yu, In Kyu; Kim, Seong Min; Park, Ki Seok; Son, Hyun Jin

2015-01-01

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

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

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

2015-07-15

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

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

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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

Metastatic Chordoma: A Diagnostic Challenge on Fine Needle Aspiration

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Ghassan Tranesh

2016-01-01

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

Clival chordoma manifesting as nasal bleeding. A case report

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

2005-05-01

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

Clival chordoma manifesting as nasal bleeding. A case report

International Nuclear Information System (INIS)

Kitai, Ryuhei; Yoshida, Kazuhiko; Kubota, Toshihiko; Sato, Kazufumi; Handa, Yuji; Kasahara, Kazuma; Nakajima, Hirofumi

2005-01-01

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

Update on the Cytogenetics and Molecular Genetics of Chordoma

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Larizza Lidia

2005-02-01

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

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.)

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.

'Do not touch' lesions of the skull base

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Dobre, Mircea C.; Fischbein, Nancy

2014-01-01

Imaging of the skull base presents many challenges due to its anatomical complexity, numerous normal variants and lack of familiarity to many radiologists. As the skull base is a region which is not amenable to physical examination and as lesions of the skull base are generally difficult to biopsy and even more difficult to operate on, the radiologist plays a major role in directing patient management via accurate image interpretation. Knowledge of the skull base should not be limited to neuroradiologists and head and neck radiologists, however, as the central skull base is routinely included in the field of view when imaging the brain, cervical spine, or head and neck with computed tomography or magnetic resonance imaging, and hence, its nuances should be familiar to general radiologists as well. We herein review the imaging findings of a subcategory of lesions of the central skull base, the 'do not touch' lesions.

Introduction: surgical management of skull base meningiomas.

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Zada, Gabriel; Başkaya, Mustafa K; Shah, Mitesh V

2017-10-01

Meningiomas represent the most common primary intracranial neoplasm treated by neurosurgeons. Although multimodal treatment of meningiomas includes surgery, radiation-based treatments, and occasionally medical therapy, surgery remains the mainstay of treatment for most symptomatic meningiomas. Because of the intricate relationship of the dura mater and arachnoid mater with the central nervous system and cranial nerves, meningiomas can arise anywhere along the skull base or convexities, and occasionally even within the ventricular system, thereby mandating a catalog of surgical approaches that neurosurgeons may employ to individualize treatment for patients. Skull base meningiomas represent some of the most challenging pathology encountered by neurosurgeons, on account of their depth, invasion, vascularity, texture/consistency, and their relationship to bony anatomy, cranial nerves, and blood vessels. Resection of complex skull base meningiomas often mandates adequate bony removal to achieve sufficient exposure of the tumor and surrounding region, in order to minimize brain retraction and optimally identify, protect, control, and manipulate sensitive neurovascular structures. A variety of traditional skull base approaches has evolved to address complex skull base tumors, of which meningiomas are considered the paragon in terms of both complexity and frequency. In this supplemental video issue of Neurosurgical Focus, contributing authors from around the world provide instructional narratives demonstrating resection of a variety of skull base meningiomas arising from traditionally challenging origins, including the clinoid processes, tuberculum sellae, dorsum sellae, petroclival region, falco-tentorial region, cerebellopontine angle, and foramen magnum. In addition, two cases of extended endoscopic endonasal approaches for tuberculum sellae and dorsum sellae meningiomas are presented, representing the latest evolution in accessing the skull base for selected tumors

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

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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

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.

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

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

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

International Nuclear Information System (INIS)

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)

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

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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

Skull base tumors: a kaleidoscope of challenge.

Science.gov (United States)

Khanna, J N; Natrajan, Srivalli; Galinde, Jyotsna

2014-08-01

Resection of skull base lesions has always been riddled with problems like inadequate access, proximity to major vessels, dural tears, cranial nerve damage, and infection. Understanding the modular concept of the facial skeleton has led to the development of transfacial swing osteotomies that facilitates resection in a difficult area with minimal morbidity and excellent cosmetic results. In spite of the current trend toward endonasal endoscopic management of skull base tumors, our series presents nine cases of diverse extensive skull base lesions, 33% of which were recurrent. These cases were approached through different transfacial swing osteotomies through the mandible, a midfacial swing, or a zygomaticotemporal osteotomy as dictated by the three-dimensional spatial location of the lesion, and its extent and proximity to vital structures. Access osteotomies ensured complete removal and good results through the most direct and safe route and good vascular control. This reiterated the fact that transfacial approaches still hold a special place in the management of extensive skull base 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.

A panoramic view of the skull base: systematic review of open and endoscopic endonasal approaches to four tumors.

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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.

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.)

Management of osteomyelitis of the skull base

International Nuclear Information System (INIS)

Benecke, J.E. Jr.

1989-01-01

Osteomyelitis of the skull base is the most severe form of malignant otitis externa. As a result of having treated 13 patients with skull base osteomyelitis over a 4-year period, we have developed a method of staging and monitoring this malady using gallium and technetium scanning techniques. Stage I is localized to soft tissues, stage II is limited osteomyelitis, and stage III represents extensive skull base osteomyelitis. All stages are treated with appropriate antipseudomonal antibiotics. The duration of therapy depends upon the clearing of inflammation as shown on the gallium scan. Each case must be looked at independently and not subjected to an arbitrary treatment protocol

Skull defect reconstruction based on a new hybrid level set.

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Zhang, Ziqun; Zhang, Ran; Song, Zhijian

2014-01-01

Skull defect reconstruction is an important aspect of surgical repair. Historically, a skull defect prosthesis was created by the mirroring technique, surface fitting, or formed templates. These methods are not based on the anatomy of the individual patient's skull, and therefore, the prosthesis cannot precisely correct the defect. This study presented a new hybrid level set model, taking into account both the global optimization region information and the local accuracy edge information, while avoiding re-initialization during the evolution of the level set function. Based on the new method, a skull defect was reconstructed, and the skull prosthesis was produced by rapid prototyping technology. This resulted in a skull defect prosthesis that well matched the skull defect with excellent individual adaptation.

Skull base, orbits, temporal bone, and cranial nerves: anatomy on MR imaging.

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Morani, Ajaykumar C; Ramani, Nisha S; Wesolowski, Jeffrey R

2011-08-01

Accurate delineation, diagnosis, and treatment planning of skull base lesions require knowledge of the complex anatomy of the skull base. Because the skull base cannot be directly evaluated, imaging is critical for the diagnosis and management of skull base diseases. Although computed tomography (CT) is excellent for outlining the bony detail, magnetic resonance (MR) imaging provides better soft tissue detail and is helpful for evaluating the adjacent meninges, brain parenchyma, and bone marrow of the skull base. Thus, CT and MR imaging are often used together for evaluating skull base lesions. This article focuses on the radiologic anatomy of the skull base pertinent to MR imaging evaluation. Copyright © 2011 Elsevier Inc. All rights reserved.

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.

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

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.

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.

Anisotropic composite human skull model and skull fracture validation against temporo-parietal skull fracture.

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Sahoo, Debasis; Deck, Caroline; Yoganandan, Narayan; Willinger, Rémy

2013-12-01

A composite material model for skull, taking into account damage is implemented in the Strasbourg University finite element head model (SUFEHM) in order to enhance the existing skull mechanical constitutive law. The skull behavior is validated in terms of fracture patterns and contact forces by reconstructing 15 experimental cases. The new SUFEHM skull model is capable of reproducing skull fracture precisely. The composite skull model is validated not only for maximum forces, but also for lateral impact against actual force time curves from PMHS for the first time. Skull strain energy is found to be a pertinent parameter to predict the skull fracture and based on statistical (binary logistical regression) analysis it is observed that 50% risk of skull fracture occurred at skull strain energy of 544.0mJ. © 2013 Elsevier Ltd. All rights reserved.

Open Approaches to the Anterior Skull Base in Children: Review of the Literature.

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Wasserzug, Oshri; DeRowe, Ari; Ringel, Barak; Fishman, Gadi; Fliss, Dan M

2018-02-01

Introduction  Skull base lesions in children and adolescents are rare, and comprise only 5.6% of all skull base surgery. Anterior skull base lesions dominate, averaging slightly more than 50% of the cases. Until recently, surgery of the anterior skull base was dominated by open procedures and endoscopic skull base surgery was reserved for benign pathologies. Endoscopic skull base surgery is gradually gaining popularity. In spite of that, open skull base surgery is still considered the "gold standard" for the treatment of anterior skull base lesions, and it is the preferred approach in selected cases. Objective  This article reviews current concepts and open approaches to the anterior skull base in children in the era of endoscopic surgery. Materials and Methods  Comprehensive literature review. Results  Extensive intracranial-intradural invasion, extensive orbital invasion, encasement of the optic nerve or the internal carotid artery, lateral supraorbital dural involvement and involvement of the anterior table of the frontal sinus or lateral portion of the frontal sinus precludes endoscopic surgery, and mandates open skull base surgery. The open approaches which are used most frequently for surgical resection of anterior skull base tumors are the transfacial/transmaxillary, subcranial, and subfrontal approaches. Reconstruction of anterior skull base defects is discussed in a separate article in this supplement. Discussion  Although endoscopic skull base surgery in children is gaining popularity in developed countries, in many cases open surgery is still required. In addition, in developing countries, which accounts for more than 80% of the world's population, limited access to expensive equipment precludes the use of endoscopic surgery. Several open surgical approaches are still employed to resect anterior skull base lesions in the pediatric population. With this large armamentarium of surgical approaches, tailoring the most suitable approach to a

Robotic Anterior and Midline Skull Base Surgery: Preclinical Investigations

International Nuclear Information System (INIS)

O'Malley, Bert W.; Weinstein, Gregory S.

2007-01-01

Purpose: To develop a minimally invasive surgical technique to access the midline and anterior skull base using the optical and technical advantages of robotic surgical instrumentation. Methods and Materials: Ten experimental procedures focusing on approaches to the nasopharynx, clivus, sphenoid, pituitary sella, and suprasellar regions were performed on one cadaver and one live mongrel dog. Both the cadaver and canine procedures were performed in an approved training facility using the da Vinci Surgical Robot. For the canine experiments, a transoral robotic surgery (TORS) approach was used, and for the cadaver a newly developed combined cervical-transoral robotic surgery (C-TORS) approach was investigated and compared with standard TORS. The ability to access and dissect tissues within the various areas of the midline and anterior skull base were evaluated, and techniques to enhance visualization and instrumentation were developed. Results: Standard TORS approaches did not provide adequate access to the midline and anterior skull base; however, the newly developed C-TORS approach was successful in providing the surgical access to these regions of the skull base. Conclusion: Robotic surgery is an exciting minimally invasive approach to the skull base that warrants continued preclinical investigation and development

Imaging of the skull base anatomy; Schnittbildanatomie der Schaedelbasis

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Wuest, Wolfgang; Uder, Michael; Lell, Michael [Erlangen-Nuernberg Univ., Universitaetsklinikum (Germany). Radiologisches Institut

2016-09-15

The skull base divides the extracranial from the intracranial compartment and contains a multiplicity of bony and soft tissue structures. For evaluating the skull base profound knowledge of the complex anatomy is mandatory. To limit the number of differential diagnosis it is important to be familiar with the contents of the different compartments. Due to the technical progress and the difficulty in assessing the skull base clinically imaging plays a significant role in diagnosis. For imaging both MRI and CT are used, which represent not competing but complementary methods.

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

Directory of Open Access Journals (Sweden)

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.

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

Modified three-dimensional skull base model with artificial dura mater, cranial nerves, and venous sinuses for training in skull base surgery: technical note.

Science.gov (United States)

Mori, Kentaro; Yamamoto, Takuji; Oyama, Kazutaka; Ueno, Hideaki; Nakao, Yasuaki; Honma, Keiichirou

2008-12-01

Experience with dissection of the cavernous sinus and the temporal bone is essential for training in skull base surgery, but the opportunities for cadaver dissection are very limited. A modification of a commercially available prototype three-dimensional (3D) skull base model, made by a selective laser sintering method and incorporating surface details and inner bony structures such as the inner ear structures and air cells, is proposed to include artificial dura mater, cranial nerves, venous sinuses, and the internal carotid artery for such surgical training. The transpetrosal approach and epidural cavernous sinus surgery (Dolenc's technique) were performed on this modified model using a high speed drill or ultrasonic bone curette under an operating microscope. The model could be dissected in almost the same way as a real cadaver. The modified 3D skull base model provides a good educational tool for training in skull base surgery.

[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

Radiologic assessment of maxillofacial, mandibular, and skull base trauma

International Nuclear Information System (INIS)

Schuknecht, Bernhard; Graetz, Klaus

2005-01-01

Cranio-maxillofacial injuries affect a significant proportion of trauma patients either in isolation or concurring with other serious injuries. Contrary to maxillofacial injuries that result from a direct impact, central skull base and lateral skull base (petrous bone) fractures usually are caused by a lateral or sagittal directed force to the skull and therefore are indirect fractures. The traditional strong role of conventional images in patients with isolated trauma to the viscerocranium is decreasing. Spiral multislice CT is progressively replacing the panoramic radiograph, Waters view, and axial films for maxillofacial trauma, and is increasingly being performed in addition to conventional films to detail and classify trauma to the mandible as well. Imaging thus contributes to accurately categorizing mandibular fractures based on location, into alveolar, mandibular proper, and condylar fractures - the last are subdivided into intracapsular and extracapsular fractures. In the midface, CT facilitates attribution of trauma to the categories central, lateral, or combined centrolateral fractures. The last frequently encompass orbital trauma as well. CT is the imaging technique of choice to display the multiplicity of fragments, the degree of dislocation and rotation, or skull base involvement. Transsphenoid skull base fractures are classified into transverse and oblique types; lateral base (temporal bone) trauma is subdivided into longitudinal and transverse fractures. Supplementary MR examinations are required when a cranial nerve palsy occurs in order to recognize neural compression. Early and late complications of trauma related to the orbit, anterior cranial fossa, or lateral skull base due to infection, brain concussion, or herniation require CT to visualize the osseous prerequisites of complications, and MR to define the adjacent brain and soft tissue involvement. (orig.)

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

Imaging of the central skull base.

Science.gov (United States)

Borges, Alexandra

2009-11-01

The central skull base (CSB) constitutes a frontier between the extracranial head and neck and the middle cranial fossa. The anatomy of this region is complex, containing most of the bony foramina and canals of the skull base traversed by several neurovascular structures that can act as routes of spread for pathologic processes. Lesions affecting the CSB can be intrinsic to its bony-cartilaginous components; can arise from above, within the intracranial compartment; or can arise from below, within the extracranial head and neck. Crosssectional imaging is indispensable in the diagnosis, treatment planning, and follow-up of patients with CSB lesions. This review focuses on a systematic approach to this region based on an anatomic division that takes into account the major tissue constituents of the CSB.

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

Science.gov (United States)

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)

Positional skull deformation in infants: heading towards evidence-based practice

NARCIS (Netherlands)

van Wijk, Renske

2014-01-01

The shape of a young infant’s skull can deform as a result of prolonged external forces. The prevalence of positional skull deformation increased dramatically during the last decades. The primary aim of this dissertation was to provide a stronger evidence base for the treatment of skull deformation.

Surgical outcomes after reoperation for recurrent skull base meningiomas.

Science.gov (United States)

Magill, Stephen T; Lee, David S; Yen, Adam J; Lucas, Calixto-Hope G; Raleigh, David R; Aghi, Manish K; Theodosopoulos, Philip V; McDermott, Michael W

2018-05-04

OBJECTIVE Skull base meningiomas are surgically challenging tumors due to the intricate skull base anatomy and the proximity of cranial nerves and critical cerebral vasculature. Many studies have reported outcomes after primary resection of skull base meningiomas; however, little is known about outcomes after reoperation for recurrent skull base meningiomas. Since reoperation is one treatment option for patients with recurrent meningioma, the authors sought to define the risk profile for reoperation of skull base meningiomas. METHODS A retrospective review of 2120 patients who underwent resection of meningiomas between 1985 and 2016 was conducted. Clinical information was extracted from the medical records, radiology data, and pathology data. All records of patients with recurrent skull base meningiomas were reviewed. Demographic data, presenting symptoms, surgical management, outcomes, and complications data were collected. Kaplan-Meier analysis was used to evaluate survival after reoperation. Logistic regression was used to evaluate for risk factors associated with complications. RESULTS Seventy-eight patients underwent 100 reoperations for recurrent skull base meningiomas. Seventeen patients had 2 reoperations, 3 had 3 reoperations, and 2 had 4 or more reoperations. The median age at diagnosis was 52 years, and 64% of patients were female. The median follow-up was 8.5 years. Presenting symptoms included cranial neuropathy, headache, seizure, proptosis, and weakness. The median time from initial resection to first reoperation was 4.4 years and 4.1 years from first to second reoperation. Seventy-two percent of tumors were WHO grade I, 22% were WHO grade II, and 6% were WHO grade III. The sphenoid wing was the most common location (31%), followed by cerebellopontine angle (14%), cavernous sinus (13%), olfactory groove (12%), tuberculum sellae (12%), and middle fossa floor (5%). Forty-four (54%) tumors were ≥ 3 cm in maximum diameter at the time of the first

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

International Nuclear Information System (INIS)

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

SPECT/CT in the Diagnosis of Skull Base Osteomyelitis

International Nuclear Information System (INIS)

Damle, Nishikant Avinash; Kumar, Rakesh; Kumar, Praveen; Jaganthan, Sriram; Patnecha, Manish; Bal, Chandrasekhar; Bandopadhyaya, Gurupad; Malhotra, Arun

2011-01-01

Skull base osteomyelitis is a potentially fatal disease. We demonstrate here the utility of SPECT/CT in diagnosing this entity, which was not obvious on a planar bone scan. A 99mT c MDP bone scan with SPECT/CT was carried out on a patient with clinically suspected skull base osteomyelitis. Findings were correlated with contrast enhanced CT (CECT) and MRI. Planar images were equivocal, but SPECT/CT showed intense uptake in the body of sphenoid and petrous temporal bone as well as the atlas corresponding to irregular bone destruction on CT and MRI. These findings indicate that SPECT/CT may have an additional role beyond planar imaging in the detection of skull base osteomyelitis.

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

International Nuclear Information System (INIS)

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

[The Base of the Skull. Rudolf Virchow between Pathology and Anthropology].

Science.gov (United States)

Seemann, Sophie

2016-01-01

Throughout his scientific career, the pathologist and anthropologist Rudolf Virchow (1821-1902) examined countless skulls, gradually changing his perspective on this object of research. Initially, he was mainly concerned with pathologically deformed skulls. From the 1850s onwards, he gradually developed a more anthropological approach, and anthropology increasingly came to dominate his scientific interest. This article shows how different influences became central for the establishment of his specific and dynamic model of the human skull development and its successful application in anthropology. Crucial for this process were Virchow's collaboration with his teacher Robert Froriep (1804-1861) in the department of pathology of the Charité, his research on cretinism and rickets, as well as his description of the base of the skull as the center of skull development. His research work was attended by and showed a reciprocal interaction with the buildup of large skull collections. This article uses Virchow's original publications on skull pathology as well as his still preserved skull specimens from the collection of the Berlin Museum of Medical History at the Charité for an integrated text and object based analysis.

Characteristics and Patterns of Metastatic Disease from Chordoma

Directory of Open Access Journals (Sweden)

Victoria A. Young

2015-01-01

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

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.

CHONDROID SKULL BASE TUMORS (A REVIEW OF LITERATURE

Directory of Open Access Journals (Sweden)

T. G. Gasparyan

2012-01-01

Full Text Available Chondroid skull base tumors are a rare and little studied pathology; many problems of their classification, diagnosis and treatment remain to be solved. This group of neoplasms is referred to as bone tumors arising from the cartilaginous tissue of the skull base bones, particularly from the bones formed during chondral osteogenesis. The paper details the clinical picture, X-ray and morphological diagnosis of chondroid tumors. Particular attention is given to surgery and radiotherapy for this category of tumors.

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

Directory of Open Access Journals (Sweden)

Guiramand Jérôme

2011-10-01

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

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

Science.gov (United States)

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

Imaging diagnosis of Granulocytic Sarcoma in the skull base

International Nuclear Information System (INIS)

Zheng Shaoyan; Xie Jiming; Yang Zhiyun; Zhou Zhou; Li Shurong

2010-01-01

Objective: To improve the understanding and imaging diagnosis of granulocytic sarcoma in the skull base. Methods: Three cases of granulocytic sarcomas in the skull base are reported. The clinical features and imaging findings were analyzed. Results: The three cases occurred in children with acute myeloid leukemia. Two patients presented with oculomotor paralysis before the diagnosis of leukemia, the third patient with history of leukemia presented with headache. Diffuse infiltration of basal skull bone marrow and extracranial soft tissue masses were shown on MRI. The signal intensities of the masses were similar to that of gray matter on T 1 WI and T 2 WI with marked contrast enhancement. The soft tissue masses were located in the para-sellar region and surrounded the lateral wall of the maxillary sinus in one case. The soft tissue mass of the second case infiltrated the orbital cavity, cavernous sinus and oculomotor nerve. Tumor infiltrating the meninges, cranial nerves and paranasal sinuses was seen in the third patient. Conclusion: Cranial nerve paralysis can be the presenting symptom of basal skull granulocytic sarcoma in children. Granulocytic sarcoma should be considered in the different diagnosis when diffuse abnormal signal intensities in the basal skull bone marrow with solitary or multiple soft tissue masses are shown on MRI. (authors)

Endoscopic Resection of Clival Chordoma. A Case Report

Directory of Open Access Journals (Sweden)

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.

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

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

Science.gov (United States)

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

2016-06-07

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

International Nuclear Information System (INIS)

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)

Skull base tumor model.

Science.gov (United States)

Gragnaniello, Cristian; Nader, Remi; van Doormaal, Tristan; Kamel, Mahmoud; Voormolen, Eduard H J; Lasio, Giovanni; Aboud, Emad; Regli, Luca; Tulleken, Cornelius A F; Al-Mefty, Ossama

2010-11-01

Resident duty-hours restrictions have now been instituted in many countries worldwide. Shortened training times and increased public scrutiny of surgical competency have led to a move away from the traditional apprenticeship model of training. The development of educational models for brain anatomy is a fascinating innovation allowing neurosurgeons to train without the need to practice on real patients and it may be a solution to achieve competency within a shortened training period. The authors describe the use of Stratathane resin ST-504 polymer (SRSP), which is inserted at different intracranial locations to closely mimic meningiomas and other pathological entities of the skull base, in a cadaveric model, for use in neurosurgical training. Silicone-injected and pressurized cadaveric heads were used for studying the SRSP model. The SRSP presents unique intrinsic metamorphic characteristics: liquid at first, it expands and foams when injected into the desired area of the brain, forming a solid tumorlike structure. The authors injected SRSP via different passages that did not influence routes used for the surgical approach for resection of the simulated lesion. For example, SRSP injection routes included endonasal transsphenoidal or transoral approaches if lesions were to be removed through standard skull base approach, or, alternatively, SRSP was injected via a cranial approach if the removal was planned to be via the transsphenoidal or transoral route. The model was set in place in 3 countries (US, Italy, and The Netherlands), and a pool of 13 physicians from 4 different institutions (all surgeons and surgeons in training) participated in evaluating it and provided feedback. All 13 evaluating physicians had overall positive impressions of the model. The overall score on 9 components evaluated--including comparison between the tumor model and real tumor cases, perioperative requirements, general impression, and applicability--was 88% (100% being the best possible

[Extensive tumor of the skull base: sphenoid sinus adenocarcinoma].

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Kallel, Souha; Sellami, Moncef

2017-01-01

We report a rare case of adenocarcinoma of the sphenoid sinus manifesting as extended skull base tumor. The patient included in the study was a 42-year old woman presenting with unilateral right symptomatology consisting of nasal obstruction, diplopia and hemifacial neuralgias. Clinical examination showed paralysis of the cranial nerve pairs V and VI. Brain scanner showed voluminous heterogeneous sphenoid and clival mass reaching the right cavernous sinus, with a peripheral tissue component at the level of the sphenoid sinus. Biopsy was performed under general anesthesia, through endonasal sphenoidotomy approach. Histological examination showed non-intestinal adenocarcinoma. The patient died due to impaired general condition occurred during examinations. Skull base adenocarcinomas mainly occur in the ethmoid bone. Sphenoid origin is exceptional. Radiological appearance is not specific and suggests malignancy. Diagnosis should be suspected in patients with aggressive tumor, even when it occurs in the midline skull base.

Juvenile nasopharyngeal angiofibroma with skull base invasion : intratumoral direct puncture embolization

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Shin, Hye Young; Kim, Sun Yong; Suh, Jung Ho; Park, Kee Hyun [Ajou Univ., Suwon (Korea, Republic of). Coll. of Medicine

1998-04-01

The purpose of this study is to demonstrate the utility and efficacy of percutaneous direct glue embolization for juvenile nasopharyngeal angiofibromas with skull base invasion. In nine cases of juvenile nasopharyngeal angiofibromas with invasion of the skull base, embolization under general anethesia was performed. Using an 18G spinal needle, direct puncture were made via the transnasal or mandibular sciatic notch. A glue-lipiodol mixture (1:1 -1:3) was injected slowly for 15 to 30 seconds under fluoroscopic control; the number of post-embolization angiography and the distribution of embolic materials was assessed on CT within 1-3 days. The mass was surgically removed 3 to 7 days after embolization. Direct glue embolization of juvenile angiofibroma with skull base invasion appears to be a simple and safe procedure. The technique could be used for other hypervascular lesions in the base of the skull or parapharyngeal space. (author). 19 refs., 1 tab., 2 figs.

Juvenile nasopharyngeal angiofibroma with skull base invasion : intratumoral direct puncture embolization

International Nuclear Information System (INIS)

Shin, Hye Young; Kim, Sun Yong; Suh, Jung Ho; Park, Kee Hyun

1998-01-01

The purpose of this study is to demonstrate the utility and efficacy of percutaneous direct glue embolization for juvenile nasopharyngeal angiofibromas with skull base invasion. In nine cases of juvenile nasopharyngeal angiofibromas with invasion of the skull base, embolization under general anethesia was performed. Using an 18G spinal needle, direct puncture were made via the transnasal or mandibular sciatic notch. A glue-lipiodol mixture (1:1 -1:3) was injected slowly for 15 to 30 seconds under fluoroscopic control; the number of post-embolization angiography and the distribution of embolic materials was assessed on CT within 1-3 days. The mass was surgically removed 3 to 7 days after embolization. Direct glue embolization of juvenile angiofibroma with skull base invasion appears to be a simple and safe procedure. The technique could be used for other hypervascular lesions in the base of the skull or parapharyngeal space. (author). 19 refs., 1 tab., 2 figs

Application of CUSA Excel ultrasonic aspiration system in resection of skull base meningiomas.

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Tang, Hailiang; Zhang, Haishi; Xie, Qing; Gong, Ye; Zheng, Mingzhe; Wang, Daijun; Zhu, Hongda; Chen, Xiancheng; Zhou, Liangfu

2014-12-01

Here, we introduced our short experience on the application of a new CUSA Excel ultrasonic aspiration system, which was provided by Integra Lifesciences corporation, in skull base meningiomas resection. Ten patients with anterior, middle skull base and sphenoid ridge meningioma were operated using the CUSA Excel ultrasonic aspiration system at the Neurosurgery Department of Shanghai Huashan Hospital from August 2014 to October 2014. There were six male and four female patients, aged from 38 to 61 years old (the mean age was 48.5 years old). Five cases with tumor located at anterior skull base, three cases with tumor on middle skull base, and two cases with tumor on sphenoid ridge. All the patents received total resection of meningiomas with the help of this new tool, and the critical brain vessels and nerves were preserved during operations. All the patients recovered well after operation. This new CUSA Excel ultrasonic aspiration system has the advantage of preserving vital brain arteries and cranial nerves during skull base meningioma resection, which is very important for skull base tumor operations. This key step would ensure a well prognosis for patients. We hope the neurosurgeons would benefit from this kind of technique.

Clinicopathological and Molecular Histochemical Review of Skull Base Metastasis from Differentiated Thyroid Carcinoma

International Nuclear Information System (INIS)

Matsuno, Akira; Murakami, Mineko; Hoya, Katsumi; Yamada, Shoko M.; Miyamoto, Shinya; Yamada, So; Son, Jae-Hyun; Nishido, Hajime; Ide, Fuyuaki; Nagashima, Hiroshi; Sugaya, Mutsumi; Hirohata, Toshio; Mizutani, Akiko; Okinaga, Hiroko; Ishii, Yudo; Tahara, Shigeyuki; Teramoto, Akira; Osamura, R. Yoshiyuki; Yamazaki, Kazuto; Ishida, Yasuo

2013-01-01

Skull base metastasis from differentiated thyroid carcinoma including follicular thyroid carcinoma (FTC) and papillary thyroid carcinoma (PTC) is a rare clinical entity. Eighteen FTC cases and 10 PTC cases showing skull base metastasis have been reported. The most common symptom of skull base metastasis from FTC and PTC is cranial nerve dysfunction. Bone destruction and local invasion to the surrounding soft tissues are common on radiological imaging. Skull base metastases can be the initial clinical presentation of FTC and PTC in the presence of silent primary sites. The possibility of skull base metastasis from FTC and PTC should be considered in patients with the clinical symptoms of cranial nerve dysfunction and radiological findings of bone destruction. A variety of genetic alterations in thyroid tumors have been identified to have a fundamental role in their tumorigenesis. Molecular histochemical studies are useful for elucidating the histopathological features of thyroid carcinoma. Recent molecular findings may provide novel molecular-based treatment strategies for thyroid carcinoma

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

Radiation-induced osteosarcoma of the jaw treated with skull base surgery

International Nuclear Information System (INIS)

Yamamoto, Misaki; Asato, Ryo; Torii, Hiroko; Kanda, Tomoko; Tamura, Yoshihiro; Hirano, Shigeru; Ito, Juichi; Tanaka, Shinzou

2009-01-01

Head and neck osteosarcomas are rare. A 33-year-old woman received radiation therapy for lymphoepithelioma of the epipharynx in her childhood. After twenty-two years, she presented with a swelling of the right cheek. We did a work up, and diagnosed her radiation-induced osteosarcoma of the jaw. We treated her with neoadjuvant chemotherapy, surgery including skull base resection, and adjuvant chemo-therapy. A small skin recurrence developed after one year, but it was resected under local anesthesia, and there have been no recurrences since. We think that skull base surgery with a combined approach is a useful method in therapy for osteosarcomas in the skull base region. (author)

Skull-base invasion of nasopharyngeal carcinoma: magnetic resonance imaging findings and therapeutic implications

International Nuclear Information System (INIS)

Nishioka, Takeshi; Shirato, Hiroki; Kagei, Kenji; Abe, Satoru; Hashimoto, Seiko; Ohmori, Keiichi; Yamazaki, Akira; Fukuda, Satoshi; Miyasaka, Kazuo

2000-01-01

Purpose: To evaluate the value of skull-base abnormality on MRI for predicting local recurrence in nasopharyngeal carcinoma. Materials and Methods: Between November 1988 and February 1997, 48 patients with NPC were examined with both MRI (1.5 T) and CT prior to radiation therapy. T classification (1987 UICC) based on physical examination and CT findings were T1 in 3 cases, T2 in 22, T3 in 9, and T4 in 14. On MRI, low-intensity tissue with Gd enhancement in the marrow of the skull was considered to be a suspicious finding of skull-base invasion. CT simulation was performed in all patients. The total dose to the primary tumor was 60-75 Gy (mean, 67 Gy). The mean follow-up period was 42 months. Results: All 14 T4 patients had abnormal tissue in the marrow of the skull base on MRI. Thirty-eight percent (13 of 34) of T1-3 patients were suspected to have skull-base invasion based on MRI (0% for T1, 27% [6 of 22] for T2, and 78% [7 of 9] for T3). The 5-year local control rate was significantly different between T1-3 and T4 tumors (97% vs. 69%, p < 0.025) but was not different by the presence of the MRI abnormality in the skull base. Conclusion: Skull-base invasion suspected solely by MRI does not relate to local recurrence provided that careful treatment planning is performed with the aid of MRI and CT simulator

The Making of a Skull Base Team and the Value of Multidisciplinary Approach in the Management of Sinonasal and Ventral Skull Base Malignancies.

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Snyderman, Carl H; Wang, Eric W; Fernandez-Miranda, Juan C; Gardner, Paul A

2017-04-01

The management of sinonasal and ventral skull base malignancies is best performed by a team. Although the composition of the team may vary, it is important to have multidisciplinary representation. There are multiple obstacles, both individual and institutional, that must be overcome to develop a highly functioning team. Adequate training is an important part of team-building and can be fostered with surgical telementoring. A quality improvement program should be incorporated into the activities of a skull base team. Copyright © 2016 Elsevier Inc. All rights reserved.

Difficulties of clinical radiodiagnosis of concomitant injuries of fornix and base of the skull

International Nuclear Information System (INIS)

Krutitskij, A.G.; Semisalov, S.Ya.

1987-01-01

Clinical radiological semiotics in 234 patients with injuries of fornix and base of the skull is studied. Among skull injuries the most critical are those of fornix and base of anterior parts of the skull. Severity of state doesn't exclude, but requires an obligatory X-ray examination, at least - review radiographs of the skull. When choosing the volume of surgical intervention the data on X-ray examination along with clinical pattern should be taken account of

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.

Endoscopic skull base training using 3D printed models with pre-existing pathology.

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Narayanan, Vairavan; Narayanan, Prepageran; Rajagopalan, Raman; Karuppiah, Ravindran; Rahman, Zainal Ariff Abdul; Wormald, Peter-John; Van Hasselt, Charles Andrew; Waran, Vicknes

2015-03-01

Endoscopic base of skull surgery has been growing in acceptance in the recent past due to improvements in visualisation and micro instrumentation as well as the surgical maturing of early endoscopic skull base practitioners. Unfortunately, these demanding procedures have a steep learning curve. A physical simulation that is able to reproduce the complex anatomy of the anterior skull base provides very useful means of learning the necessary skills in a safe and effective environment. This paper aims to assess the ease of learning endoscopic skull base exposure and drilling techniques using an anatomically accurate physical model with a pre-existing pathology (i.e., basilar invagination) created from actual patient data. Five models of a patient with platy-basia and basilar invagination were created from the original MRI and CT imaging data of a patient. The models were used as part of a training workshop for ENT surgeons with varying degrees of experience in endoscopic base of skull surgery, from trainees to experienced consultants. The surgeons were given a list of key steps to achieve in exposing and drilling the skull base using the simulation model. They were then asked to list the level of difficulty of learning these steps using the model. The participants found the models suitable for learning registration, navigation and skull base drilling techniques. All participants also found the deep structures to be accurately represented spatially as confirmed by the navigation system. These models allow structured simulation to be conducted in a workshop environment where surgeons and trainees can practice to perform complex procedures in a controlled fashion under the supervision of experts.

Fungal Infection of the Sinus and Anterior Skull Base

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Morteza Javadi

2008-11-01

Full Text Available   Abstract   Background: Invasive fungal infection is an opportunistic infection caused commonly   by mucoraccae and aspergillus. It mostly occurs in patients with underlying disease.   Since it has a high mortality and morbidity rate, considering a treatment strategy seems   necessary.   Objective: Since there has not been a clear protocol for treating these patients, we decided   to establish a protocol for fungal infection of sinus and anterior skull base management.   Methods: This retrospective and descriptive case study series included 30 patients.   After confirming the pathogen, the authors came to a proper protocol for treatment which   is mentioned later.   Results: The site involvement included nose and orbital cavity (53.3%, anterior skull   base and brain in conjunction with sinonasal (36.6% and simple nasal cavity involvement   (10%. 86.6% of the patients had underlying diseases. 56.6% of patients had diabetes   as a single underlying disease, while 13.3% had both diabetes and renal failure in   combination. Acute lymphocytic leukemia was present in 6.6%, renal failure in 3.3%, lupus   in 3.3% and chronic lymphocytic leukemia in 3.3% of patients. Mortality rate was   40%. We categorized the patients into 3 groups: only sinonasal, sinonasal and orbit, and   associated anterior skull base and brain involvement.   Conclusion: Early diagnosis is an important factor in improving survival. Anterior   skull base and brain involvement has a very poor prognosis.  

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 ...

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.

Reconstruction for Skull Base Defect Using Fat-Containing Perifascial Areolar Tissue.

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Choi, Woo Young; Sung, Ki Wook; Kim, Young Seok; Hong, Jong Won; Roh, Tai Suk; Lew, Dae Hyun; Chang, Jong Hee; Lee, Kyu Sung

2017-06-01

Skull base reconstruction is a challenging task. The method depends on the anatomical complexity and size of the defect. We obtained tissue by harvesting fat-containing perifascial areolar tissue (PAT) for reconstruction of limited skull base defects and volume augmentation. We demonstrated the effective option for reconstruction of limited skull base defects and volume augmentation. From October 2013 to November 2015, 5 patients underwent operations using fat-containing PAT to fill the defect in skull base and/or perform volume replacement in the forehead. Perifascial areolar tissue with 5- to 10-mm fat thickness was harvested from the inguinal region. The fat-containing PAT was grafted to the defect contacting the vascularized wound bed. Patients were followed up in terms of their clinical symptoms and postoperative magnetic resonance imaging findings. Four patients were treated using fat-containing PAT after tumor resection. One patient was treated for a posttraumatic forehead depression deformity. The fat-containing PAT included 5- to 9-mm fat thickness in all cases. The mean size of grafted PAT was 65.6 cm (28-140 cm). The mean follow-up period was 18.6 months (12-31 months). There was no notable complication. There was no donor site morbidity. We can harvest PAT with fat easily and obtain the sufficient volume to treat the defect. It also could be used with other reconstructive method, such as a free flap or a regional flap to fill the left dead space. Therefore, fat-containing PAT could be additional options to reconstruction of skull base defect.

Preformed titanium cranioplasty after resection of skull base meningiomas - a technical note.

Science.gov (United States)

Schebesch, Karl-Michael; Höhne, Julius; Gassner, Holger G; Brawanski, Alexander

2013-12-01

Meningiomas of the fronto-basal skull are difficult to manage as the treatment usually includes extensive resection of the lesion, consecutive reconstruction of the meninges and of the skull. Especially after removal of spheno-orbital and sphenoid-wing meningiomas, the cosmetic result is of utmost importance. In this technical note, we present our institutional approach in the treatment of skull base meningiomas, focussing on the reconstruction of the neurocranium with individually preformed titanium cranioplasty (CRANIOTOP(®), CL Instruments, Germany). Two female patients (40 years, 64 years) are presented. Both patients presented with skull base lesions suggestive of meningiomas. The preoperative thin-sliced CT scan was processed to generate a 3D-model of the skull. On it, the resection was mapped and following a simulated resection, the cranioplasty was manufactured. Intra-operatively, the titanium plate served as a template for the skull resection and was implanted after microsurgical tumour removal, consecutively. The cosmetic result was excellent. Immediate postoperative CT scan revealed accurate fitting and complete tumour removal. Control Magnetic Resonance Imaging (MRI) within 12 weeks was possible without any artifacts. The comprehensive approach described indicates only one surgical procedure for tumour removal and for reconstruction of the skull. The titanium plate served as an exact template for complete resection of the osseous parts of the tumour. Cosmetic outcome was excellent and control MRI was possible post operatively. CRANIOTOP(®) cranioplasty is a safe and practical tool for reconstruction of the skull after meningioma surgery. Copyright © 2013 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Skull Thickness Morphing for an Age and Sex Specific FE Model of the Skull.

Science.gov (United States)

Jones, Derek A; Urban, Jillian E; Lillie, Elizabeth M; Stitzel, Joel D

2015-01-01

Skull deformation is believed to be a contributing factor in traumatic brain injury (TBI). Furthermore, skull thickness is thought to be an important factor governing deformation of the skull and its susceptibility to fracture. Although many studies have been done to understand the mechanisms of brain injury and skull fracture, the majority of the cadaveric and finite element (FE) modeling efforts are comprised of older males and 50th percentile male skulls, respectively, which do not accurately represent the population as a whole. This study employed a set of skull table thickness regressions defined at homologous landmarks on the skull which were calculated from 123 pre-existing head CT scans (ages 20-100) using a cortical density-based algorithm. A method was developed to morph the Global Human Body Models Consortium (GHBMC) 50th percentile male skull model to age and gender specific geometries based on the full thickness regressions using a Thin Plate Spline algorithm. A quantitative measure of morphing error was devised and measured using the morphed and desired full thickness values at the homologous landmark locations. This methodology can be used to create gender and age-specific FE models of the skull and will ultimately be used to understand the relationship between cortical thickness, skull deformation, and head injury.

Skull base osteomyelitis: role of three phase and hybrid SPECT/CT bone scintigraphy

International Nuclear Information System (INIS)

Chakraborty, D.; Bhattacharaya, A.; Kamaleshwaran, K.K.; Mittal, B.R.; Aggarwal, K.; Singh, B.; Bhoil, A.

2010-01-01

Full text: Skull base osteomyelitis is the infection that has spread to the skull base, beyond the external auditory canal and seen in advanced stage of malignant otitis externa. Early diagnosis of this condition includes the use of bone scintigraphy since clinical assessment alone cannot differentiate the skull base osteomyelitis from the severe type of otitis externa in which there is no extension to the adjacent bone. Objective: To determine the role of three phase bone scintigraphy and delayed SPECT/CT in detection of skull base osteomyelitis in patients with malignant otitis externa. Material and Methods: Clinical records of 20 patients (14 Males and 6 Females; mean age 72 yrs) of otitis externa with suspected skull base involvement referred for bone scintigraphies were analyzed retrospectively. Three phase bone scintigraphy was acquired under dual detector gamma camera after intravenous injection of 20 mCi (740 MBq) 99m Tc-MDP followed by SPECT/CT of the skull. Scintigraphic findings were compared with clinical symptoms, signs and diagnostic CT scan findings. Results: All the patients except one were diabetic and having elevated ESR. 18 patients presented with bilateral symptoms and rest unilateral. Cranial nerves were involved in 8 patients (40%). Ear discharge culture sensitivity report was found in three patients; it was positive for Pseudomonas aeruginosa for two patients and in Diptheroids for one. In 9 patients (45%) increased flow of tracer and 10 patients (50%) increased blood pool phase in the temporal region was found. Delayed phase images showed increased uptake in skull bone in 19 patients (95%). Hybrid SPECT/CT of the skull localized areas of increased tracer uptake to the mastoid part in 15 patients (75%), petrous part in 11 patients (55%), sphenoid in 3 patients (15%) and zygomatic bone in one patient (5%) with CT showing destructive changes in 5 patients (25%) which were corroborated with diagnostic CT findings. SPECT/CT along with three phase

Radiological assessment of skull base changes in children with syndromic craniosynostosis: role of ''minor'' sutures

International Nuclear Information System (INIS)

Calandrelli, Rosalinda; D'Apolito, Gabriella; Gaudino, Simona; Stefanetti, Mariangela; Colosimo, Cesare; Massimi, Luca; Di Rocco, Concezio

2014-01-01

This study aims to identify the premature synostosis of ''major'' and ''minor'' sutures of the four ''sutural arches'' of the skull and to perform a morphometric analysis in children with syndromic craniosynostosis in order to evaluate changes in the skull base linked with premature suture synostosis. We reviewed multiplanar high-resolution CT images, implemented with 3D reconstructions, from 18 patients with complex syndromic craniosynostosis and compared them with 18 age-matched healthy subjects. We assessed the calvarial sutures and their extension to the skull base, and then we correlated specific types of synostosis with the size, shape and symmetry of the cranial fossae. We found a marked asymmetry of the skull base growth in all patients. The synostotic involvement around the coronal ring caused a reduction in the growth of the anterior and middle fossae. The size of the posterior cranial fossa was related not only to ''major'' but also to ''minor'' suture synostosis of the lambdoid and parieto-squamosal arches. Changes in the skull base and craniofacial axis symmetry are due to structural and functional relationships between ''major'' and ''minor'' skull sutures, suggesting a structural and functional relationship between the neurocranium and basicranium. The early recognition of prematurely closed skull base sutures may help clinicians and neurosurgeons to establish correct therapeutic approaches. (orig.)

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.)

CT and magnetic resonance imaging finding of lipomatous hemanioperisytoma of skull base: A case report

Energy Technology Data Exchange (ETDEWEB)

Park, Hee Girl; Yu, In Kyu; Kim, Han Kyu; Kim, Seung Min; Kang, Dong Wook [Eulji University Hospital, Daejeon (Korea, Republic of)

2013-07-15

Lipomatous hemangiopericytoma (LHPC) is recently recognized as a rare hemangiopericytoma variant. To our knowledge, imaging features of LHPC involving skull base have not yet been reported. We present the imaging features of LHPC of skull base in a 44-year-old female, along with a literature review CT and magnetic resonance imagings showed well-enhanced fatty issues containing temporal skull base masses, with pressure bony erosions.

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.)

Skull base tumors: a comprehensive review of transfacial swing osteotomy approaches.

Science.gov (United States)

Moreira-Gonzalez, Andrea; Pieper, Daniel R; Cambra, Jorge Balaguer; Simman, Richard; Jackson, Ian T

2005-03-01

Numerous techniques have been proposed for the resection of skull base tumors, each one unique with regard to the region exposed and degree of technical complexity. This study describes the use of transfacial swing osteotomies in accessing lesions located at various levels of the cranial base. Eight patients who underwent transfacial swings for exposure and resection of cranial base lesions between 1996 and 2002 were studied. The mandible was the choice when wide exposure of nasopharyngeal and midline skull base tumors was necessary, especially when they involved the infratemporal fossa. The midfacial swing osteotomy was an option when access to the entire clivus was necessary. An orbital swing approach was used to access large orbital tumors lying inferior to the optic nerve and posterior to the globe, a region that is often difficult to visualize. Gross total tumor excision was possible in all patients. Six patients achieved disease control and two had recurrences. The complications of cerebrospinal fluid leak, infection, hematoma, or cranial nerve damage did not occur. After surgery, some patients experienced temporary symptoms caused by local swelling. The aesthetic result was considered good. Transfacial swing osteotomies provide a wide exposure to tumors that occur in the central skull base area. Excellent knowledge of the detailed anatomy of this region is paramount to the success of this surgery. The team concept is essential; it is built around the craniofacial surgeon and an experienced skull base neurosurgeon.

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)

Teamwork in skull base surgery: An avenue for improvement in patient care.

Science.gov (United States)

McLaughlin, Nancy; Carrau, Ricardo L; Kelly, Daniel F; Prevedello, Daniel M; Kassam, Amin B

2013-01-01

During the past several decades, numerous centers have acquired significant expertise in the treatment of skull base pathologies. Favorable outcomes are not only due to meticulous surgical planning and execution, but they are also related to the collaborative efforts of multiple disciplines. We review the impact of teamwork on patient care, elaborate on the key processes for successful teamwork, and discuss its challenges. Pubmed and Medline databases were searched for publications from 1970 to 2012 using the following keywords: "teamwork", "multidisciplinary", "interdisciplinary", "surgery", "skull base", "neurosurgery", "tumor", and "outcome". Current literature testifies to the complexity of establishing and maintaining teamwork. To date, few reports on the impact of teamwork in the management of skull base pathologies have been published. This lack of literature is somewhat surprising given that most patients with skull base pathology receive care from multiple specialists. Common factors for success include a cohesive and well-integrated team structure with well-defined procedural organization. Although a multidisciplinary work force has clear advantages for improving today's quality of care and propelling research efforts for tomorrow's cure, teamwork is not intuitive and requires training, guidance, and executive support. Teamwork is recommended to improve quality over the full cycle of care and consequently patient outcomes. Increased recognition of the value of an integrated team approach for skull base pathologies will hopefully encourage centers, physicians, allied health caregivers, and scientists devoted to treating these patients and advancing the field of knowledge to invest the time, effort, and resources to optimize and organize their collective expertise.

Alignment of CT images of skull dysmorphology using anatomy-based perpendicular axes

International Nuclear Information System (INIS)

Yoo, Sun K; Kim, Yong O; Kim, Hee-Joung; Kim, Nam H; Jang, Young Beom; Kim, Kee-Deog; Lee, Hye-Yeon

2003-01-01

Rigid body registration of 3D CT scans, based on manual identification of homologous landmarks, is useful for the visual analysis of skull dysmorphology. In this paper, a robust and simple alignment method was proposed to allow for the comparison of skull morphologies, within and between individuals with craniofacial anomalies, based on 3D CT scans, and the minimum number of anatomical landmarks, under rigidity and uniqueness constraints. Three perpendicular axes, extracted from anatomical landmarks, define the absolute coordinate system, through a rigid body transformation, to align multiple CT images for different patients and acquisition times. The accuracy of the alignment method depends on the accuracy of the localized landmarks and target points. The numerical simulation generalizes the accuracy requirements of the alignment method. Experiments using a human dried skull specimen, and ten sets of skull CT images (the pre- and post-operative CT scans of four plagiocephaly, and one fibrous dysplasia patients), demonstrated the feasibility of the technique in clinical practice

Skull Base Osteomyelitis from Otitis Media Presenting as the Collet-Sicard Syndrome

Directory of Open Access Journals (Sweden)

Wong-Kein Low

2018-01-01

Full Text Available Skull base osteomyelitis can involve the jugular foramen and its associated cranial nerves resulting in specific clinical syndromes. The Collet-Sicard syndrome describes the clinical manifestations of palsies involving cranial nerves IX, X, XI, and XII. We present a rare atypical case of skull base osteomyelitis originating from infection of the middle ear and causing the Collet-Sicard syndrome. Caused by Pseudomonas aeruginosa and Klebsiella pneumoniae, this occurred in an elderly diabetic man subsequent to retention of a cotton swab in an ear with chronic suppurative otitis media. This case report illustrates the possibility of retained cotton swabs contributing to the development of otitis media, skull base osteomyelitis, and ultimately the Collet-Sicard syndrome in the ears of immune-compromised patients with chronically perforated eardrums.

Comparison of SPECT and CT in detecting skull base invasion in nasopharyngeal carcinoma

International Nuclear Information System (INIS)

Zhang Li; Wang Jinchuan; Pu Nuo; Song Wenzhong; Chen Mingxi

2002-01-01

Objective: To investigate the detecting ability of single photon emission computed tomography (SPECT) and CT in skull base invasion in nasopharyngeal carcinoma. Methods: Sixty-three patients with nasopharyngeal carcinoma were examined by whole body and skull base SPECT and CT of nasopharynx and skull base before radiotherapy. The results were double-blind compared and evaluated. Results: The overall positive rates of skull base invasion detected by SPECT and CT were 63.5% and 25.4%. In patients with headache, cranial nerve palsy and both, they were 87.9%, 93.3%, 92.3% and 42.4%, 46.7%, 46.2%. In patients with T 1 + T 2 and T 3 + T 4 lesions, they were 37.5%, 90.3% and 0.0%, 51.6%. In patients with N 0 + N 1 and N 2 + N 3 lesions, they were 63.9%, 63.0% and 19.4%, 33.3%. The positive rates of SPECT were higher than those of CT (McNemar Test, P < 0.05). The conformation rate between SPECT and CT was 61.9% and the dissimilitude rate was 38.1%. Binary Logistic regression analysis showed that headache and T stages were risk factors of positive SPECT rate (ORheadache = 3.864, ORTstage= 6.422) while Tstage and Nstage were the risk factors for positive CT rate (ORTstage = 48.932, ORNstage = 2.860). Conclusions: The detection sensitivity of SPECT in skull base invasion in nasopharyngeal carcinoma is superior to that of CT. But its specificity is inferior to that of CT. The detecting results in SPECT are better related to symptoms, signs and stage. Combining headache and cranial nerve palsy with T and N stage, the authors may much improve the results of SPECT and CT in the detection of skull base invasion in nasopharyngeal carcinoma. Further study is warranted

Skull base osteomyelitis: current microbiology and management.

Science.gov (United States)

Spielmann, P M; Yu, R; Neeff, M

2013-01-01

Skull base osteomyelitis typically presents in an immunocompromised patient with severe otalgia and otorrhoea. Pseudomonas aeruginosa is the commonest pathogenic micro-organism, and reports of resistance to fluoroquinolones are now emerging, complicating management. We reviewed our experience of this condition, and of the local pathogenic organisms. A retrospective review from 2004 to 2011 was performed. Patients were identified by their admission diagnostic code, and computerised records examined. Twenty patients were identified. A facial palsy was present in 12 patients (60 per cent). Blood cultures were uniformly negative, and culture of ear canal granulations was non-diagnostic in 71 per cent of cases. Pseudomonas aeruginosa was isolated in only 10 (50 per cent) cases; one strain was resistant to ciprofloxacin but all were sensitive to ceftazidime. Two cases of fungal skull base osteomyelitis were identified. The mortality rate was 15 per cent. The patients' treatment algorithm is presented. Our treatment algorithm reflects the need for multidisciplinary input, early microbial culture of specimens, appropriate imaging, and prolonged and systemic antimicrobial treatment. Resolution of infection must be confirmed by close follow up and imaging.

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

Directory of Open Access Journals (Sweden)

Amin El-Heliebi

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

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.)

Chondroblastoma with secondary aneurysmal bone cyst in the anterior skull base

Directory of Open Access Journals (Sweden)

Ming Jie Wang, MD, PhD

2016-06-01

Full Text Available Chondroblastoma with secondary aneurysmal bone cyst (ABC, especially in the anterior skull base, is an extremely rare condition. A 5-year-old boy presented with a large space-occupying lesion in the anterior skull base along with a left sided-epistaxis, proptosis and decreased vision. Radical excision of the lesion was performed by an endoscopic transnasal and transethmoidal approach. The patient recovered without any recurrence during a follow-up period of up to 28 months. Here, we review this rare case and discuss the clinical presentation and surgical treatment.

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

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.

Thin-section CT of the skull base

International Nuclear Information System (INIS)

Beyer-Enke, S.A.; Goerich, J.; Gamroth, A.; Tiedemann, K.

1987-01-01

High-resolution CT-images of the skull base are depicted and anatomical structures are described. A large variety of osseous and soft tissue structures can be differentiated in the temporal bone, nasopharynx and orbita. Knowledge of the anatomical structures is essential for the recognition of pathological changes and also plays an essential role for the diagnostically involved radiologist. (orig.) [de

[Anatomy of the skull base and the cranial nerves in slice imaging].

Science.gov (United States)

Bink, A; Berkefeld, J; Zanella, F

2009-07-01

Computed tomography (CT) and magnetic resonance imaging (MRI) are suitable methods for examination of the skull base. Whereas CT is used to evaluate mainly bone destruction e.g. for planning surgical therapy, MRI is used to show pathologies in the soft tissue and bone invasion. High resolution and thin slice thickness are indispensible for both modalities of skull base imaging. Detailed anatomical knowledge is necessary even for correct planning of the examination procedures. This knowledge is a requirement to be able to recognize and interpret pathologies. MRI is the method of choice for examining the cranial nerves. The total path of a cranial nerve can be visualized by choosing different sequences taking into account the tissue surrounding this cranial nerve. This article summarizes examination methods of the skull base in CT and MRI, gives a detailed description of the anatomy and illustrates it with image examples.

Anatomy of the skull base and the cranial nerves in slice imaging

International Nuclear Information System (INIS)

Bink, A.; Berkefeld, J.; Zanella, F.

2009-01-01

Computed tomography (CT) and magnetic resonance imaging (MRI) are suitable methods for examination of the skull base. Whereas CT is used to evaluate mainly bone destruction e.g. for planning surgical therapy, MRI is used to show pathologies in the soft tissue and bone invasion. High resolution and thin slice thickness are indispensible for both modalities of skull base imaging. Detailed anatomical knowledge is necessary even for correct planning of the examination procedures. This knowledge is a requirement to be able to recognize and interpret pathologies. MRI is the method of choice for examining the cranial nerves. The total path of a cranial nerve can be visualized by choosing different sequences taking into account the tissue surrounding this cranial nerve. This article summarizes examination methods of the skull base in CT and MRI, gives a detailed description of the anatomy and illustrates it with image examples. (orig.) [de

A Review of Stereotactic Radiosurgery Practice in the Management of Skull Base Meningiomas

OpenAIRE

Vera, Elena; Iorgulescu, J. Bryan; Raper, Daniel M.S.; Madhavan, Karthik; Lally, Brian E.; Morcos, Jacques; Elhammady, Samy; Sherman, Jonathan; Komotar, Ricardo J.

2014-01-01

Gross total resection of skull base meningiomas poses a surgical challenge due to their proximity to neurovascular structures. Once the gold standard therapy for skull base meningiomas, microsurgery has been gradually replaced by or used in combination with stereotactic radiosurgery (SRS). This review surveys the safety and efficacy of SRS in the treatment of cranial base meningiomas including 36 articles from 1991 to 2010. SRS produces excellent tumor control with low morbidity rates compare...

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.

Multi-atlas and label fusion approach for patient-specific MRI based skull estimation.

Science.gov (United States)

Torrado-Carvajal, Angel; Herraiz, Joaquin L; Hernandez-Tamames, Juan A; San Jose-Estepar, Raul; Eryaman, Yigitcan; Rozenholc, Yves; Adalsteinsson, Elfar; Wald, Lawrence L; Malpica, Norberto

2016-04-01

MRI-based skull segmentation is a useful procedure for many imaging applications. This study describes a methodology for automatic segmentation of the complete skull from a single T1-weighted volume. The skull is estimated using a multi-atlas segmentation approach. Using a whole head computed tomography (CT) scan database, the skull in a new MRI volume is detected by nonrigid image registration of the volume to every CT, and combination of the individual segmentations by label-fusion. We have compared Majority Voting, Simultaneous Truth and Performance Level Estimation (STAPLE), Shape Based Averaging (SBA), and the Selective and Iterative Method for Performance Level Estimation (SIMPLE) algorithms. The pipeline has been evaluated quantitatively using images from the Retrospective Image Registration Evaluation database (reaching an overlap of 72.46 ± 6.99%), a clinical CT-MR dataset (maximum overlap of 78.31 ± 6.97%), and a whole head CT-MRI pair (maximum overlap 78.68%). A qualitative evaluation has also been performed on MRI acquisition of volunteers. It is possible to automatically segment the complete skull from MRI data using a multi-atlas and label fusion approach. This will allow the creation of complete MRI-based tissue models that can be used in electromagnetic dosimetry applications and attenuation correction in PET/MR. © 2015 Wiley Periodicals, Inc.

Management of Anterior Skull Base Defect Depending on Its Size and Location

Science.gov (United States)

Bernal-Sprekelsen, Manuel; Rioja, Elena; Enseñat, Joaquim; Enriquez, Karla; Viscovich, Liza; Agredo-Lemos, Freddy Enrique; Alobid, Isam

2014-01-01

Introduction. We present our experience in the reconstruction of these leaks depending on their size and location. Material and Methods. Fifty-four patients who underwent advanced skull base surgery (large defects, >20 mm) and 62 patients with CSF leaks of different origin (small, 2–10 mm, and midsize, 11–20 mm, defects) were included in the retrospective study. Large defects were reconstructed with a nasoseptal pedicled flap positioned on fat and fascia lata. In small and midsized leaks. Fascia lata in an underlay position was used for its reconstruction covered with mucoperiosteum of either the middle or the inferior turbinate. Results. The most frequent etiology for small and midsized defects was spontaneous (48.4%), followed by trauma (24.2%), iatrogenic (5%). The success rate after the first surgical reconstruction was 91% and 98% in large skull base defects and small/midsized, respectively. Rescue surgery achieved 100%. Conclusions. Endoscopic surgery for any type of skull base defect is the gold standard. The size of the defects does not seem to play a significant role in the success rate. Fascia lata and mucoperiosteum of the turbinate allow a two-layer reconstruction of small and midsized defects. For larger skull base defects, a combination of fat, fascia lata, and nasoseptal pedicled flaps provides a successful reconstruction. PMID:24895567

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

Energy Technology Data Exchange (ETDEWEB)

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

2003-06-01

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

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

International Nuclear Information System (INIS)

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)

Subcranial approach in the surgical treatment of anterior skull base trauma.

Science.gov (United States)

Schaller, B

2005-04-01

Fractures of the anterior skull base, because of the region's anatomical relationships, are readily complicated by neurological damage to the brain or cranial nerves. This review highlights the use of a subcranial approach in the operative treatment of injuries of the anterior skull base and compares it to the more traditional neurosurgical transcranial approach. The extended anterior subcranial approach takes advantage of the specific features of injuries in this region and allows direct access to the central anterior cranial base in order to repair fractures, close CSF fistulae and relieve of optic nerve compression. It avoids extensive frontal lobe manipulation. The success of the approach in achieving the aims of surgery with low morbidity is reviewed.

Natural history of chondroid skull base lesions - case report and review

International Nuclear Information System (INIS)

Schmidinger, A.; Rosahl, S.K.; Vorkapic, P.; Samii, M.

2002-01-01

Long-term follow-up reports on chondroid lesions of the skull base are rarely presented in the literature. There are virtually no data on natural growth rates of these tumors based on MRI obtained over a period of 10 years or longer. We followed a patient who has had such a lesion for more than 12 years. A non-progressive, slight abducens palsy has been the only associated symptom so far. Even though the patient was operated on for an additional intracranial arterio-venous malformation, clinical features and chromosomal testing excluded Maffucci's syndrome. The MRI follow-up in this case provides an extraordinary perspective on the natural history of chondroid skull base tumors. (orig.)

Fractionated external beam radiotherapy of skull base metastases with cranial nerve involvement

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Droege, L.H.; Hinsche, T.; Hess, C.F.; Wolff, H.A. [University Hospital of Goettingen, Department of Radiotherapy and Radiation Oncology, Goettingen (Germany); Canis, M. [University of Goettingen, Department of Otorhinolaryngology, Head and Neck Surgery, Goettingen (Germany); Alt-Epping, B. [University of Goettingen, Department of Palliative Medicine, Goettingen (Germany)

2014-02-15

Skull base metastases frequently appear in a late stage of various tumor entities and cause pain and neurological disorders which strongly impair patient quality of life. This study retrospectively analyzed fractionated external beam radiotherapy (EBRT) as a palliative treatment approach with special respect to neurological outcome, feasibility and acute toxicity. A total of 30 patients with skull base metastases and cranial nerve disorders underwent EBRT with a mean total dose of 31.6 Gy. Neurological status was assessed before radiotherapy, during radiotherapy and 2 weeks afterwards categorizing orbital, parasellar, middle fossa, jugular foramen and occipital condyle involvement and associated clinical syndromes. Neurological outcome was scored as persistence of symptoms, partial response, good response and complete remission. Treatment-related toxicity and overall survival were assessed. Before EBRT 37 skull base involvement syndromes were determined with 4 patients showing more than 1 syndrome. Of the patients 81.1 % responded to radiotherapy with 10.8 % in complete remission, 48.6 % with good response and 21.6 % with partial response. Grade 1 toxicity of the skin occurred in two patients and grade 1 hematological toxicity in 1 patient under concurrent chemoradiotherapy. Median overall survival was 3.9 months with a median follow-up of 45 months. The use of EBRT for skull base metastases with symptomatic involvement of cranial nerves is marked by good therapeutic success in terms of neurological outcome, high feasibility and low toxicity rates. These findings underline EBRT as the standard therapeutic approach in the palliative setting. (orig.)

Fractionated external beam radiotherapy of skull base metastases with cranial nerve involvement

International Nuclear Information System (INIS)

Droege, L.H.; Hinsche, T.; Hess, C.F.; Wolff, H.A.; Canis, M.; Alt-Epping, B.

2014-01-01

Skull base metastases frequently appear in a late stage of various tumor entities and cause pain and neurological disorders which strongly impair patient quality of life. This study retrospectively analyzed fractionated external beam radiotherapy (EBRT) as a palliative treatment approach with special respect to neurological outcome, feasibility and acute toxicity. A total of 30 patients with skull base metastases and cranial nerve disorders underwent EBRT with a mean total dose of 31.6 Gy. Neurological status was assessed before radiotherapy, during radiotherapy and 2 weeks afterwards categorizing orbital, parasellar, middle fossa, jugular foramen and occipital condyle involvement and associated clinical syndromes. Neurological outcome was scored as persistence of symptoms, partial response, good response and complete remission. Treatment-related toxicity and overall survival were assessed. Before EBRT 37 skull base involvement syndromes were determined with 4 patients showing more than 1 syndrome. Of the patients 81.1 % responded to radiotherapy with 10.8 % in complete remission, 48.6 % with good response and 21.6 % with partial response. Grade 1 toxicity of the skin occurred in two patients and grade 1 hematological toxicity in 1 patient under concurrent chemoradiotherapy. Median overall survival was 3.9 months with a median follow-up of 45 months. The use of EBRT for skull base metastases with symptomatic involvement of cranial nerves is marked by good therapeutic success in terms of neurological outcome, high feasibility and low toxicity rates. These findings underline EBRT as the standard therapeutic approach in the palliative setting. (orig.)

Endovascular treatment for arterial injuries of skull base

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Li Tianxiao; Bai Weixing; Zai Suiting; Wang Ziliang; Xue Jiangyu

2008-01-01

Objective: To explore the role of endovascular techniques in treatment for arterial injuries of skull base. Methods: A total of 53 consecutive cases suffered from skull base arterial injuries were enrolled in our hospital from Oct 2004 to May 2007, including 44 male and 9 female cases with average age of 23.3 years. Thirty-nine cases presented with pulsatile exophthalmos and intracranial vascular murmur, cerchnus and dysphagia in another 9, epistaxis in the remaining 5 cases. Diagnosis of 39 carotid cavernous fistulae (CCF)and 14 carotid pseudoaneurysm were performed by angiography (DSA). Alternative endovascular procedures were performed depending on lesions characteristics and follow-up was done by telephone and outpatient work up. Results: Procedures were performed involving 56 carotid arteries in all 53 cases including 34 CCF with embolization of detachable balloon(33 cases), 3 with balloon and coils, and 3 by stent-graft placement. 8 carotid pseudoaneurysms were cured by parent artery occlusion with balloon, 2 experienced endovascular isolation with balloon and coils, and 4 with stent-graft. Follow-up for mean 9.5 months (range from 2 to 25 months) revealed that the chief symptoms of 45 cases (85%) were relieved within 6 months after the procedure but ocular movement and visual disorder remained in 8 cases (15%)till 12 months. Six pseudoaneurysms and 3 residual leak were found in reexamination, of which 2 cases underwent intervention again 2 and 3 months later due to dural arterial-venous fistula in cavernous sinus, respectively. Conclusions: Endovascular treatment is safe and effective therapeutic option with minimal invasion for skull base arterial injuries. Detachable balloon embolization is the first choice for CCF and carotid pseudoaneurysm. Spring coil packing and stent-graft implantation should be in alternation as combination for special cases. (authors)

Osteochondroma of the skull base: MRI and histological correlation

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Sato, K.; Kodera, T.; Kitai, R.; Kubota, T.

1996-01-01

A skull base osteochondroma (benign exostosis) in a 38-year-old man is reported. MRI was not only very useful for determining the extent of the tumour, but also showed its far content and, on contrast-enhanced fat-suppressed images, its vascularity. (orig.)

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.

Postnatal development of the anterior skull base and nasal septum: CT study

International Nuclear Information System (INIS)

Kim, Kwan Soo; Kim, Hyung Jin; Lee, Kyung Hee; Roh, Hong Gee; Lim, Myung Kwan

2002-01-01

To know the normal CT appearance of the anterior skull base and nasal septum after birth. Coronal CT scans with a helical mode were performed from the nasal bone to the sphenoid sinus in 99 children whose ages ranged from 27 days to 14 years. We investigated the CT appearance of the developing anterior skull base and nasal septum with particular attention to the anteroposterior length of the anterior skull base and the ossification patterns of the cribriform plate, perpendicular plate, crista galli, and vomer. The anteroposterior length of the anterior skull base statistically significantly increased with age. The cribriform plate showed partial or complete ossification in at least one segment at more than 3 months of age and in all three segments at more than 6 months of age. Ossification of the cribriform plate occurred earlier in the middle segment than in the anterior and posterior segments. It began exclusively in the region of the lateral mass of the ethmoid and proceeded medially toward the crista galli. Partial ossification of the perpendicular plate was noted as early as 9 months of age, and complete ossification as early as 13 months of age. All children at 18 months and older showed at least partial ossification of the perpendicular plate. Partial ossification of the crista galli was noted as early as 27 days of age, and complete ossification as early as 3 months of age. CT showed complete ossification of the crista galli in all but two children at 6 months and older. The superior aspect of the vomer exhibited a V- or Y-shape on all CT scans in 66%(65/99) of children at any age. It appeared as an undivided single lump anteriorly and a V or Y posteriorly in 34%(34/99). Knowledge of the normal developing patterns of ossification of the anterior skull base and nasal septum could help prevent errors in interpreting CT scans in this region, especially in infants and young children

Surgical resection of a huge cemento-ossifying fibroma in skull base by intraoral approach.

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Cheng, Xiao-Bing; Li, Yun-Peng; Lei, De-Lin; Li, Xiao-Dong; Tian, Lei

2011-03-01

Cemento-ossifying fibroma, also known as ossifying fibroma, usually occurs in the mandible and less commonly in the maxilla. The huge example in the skull base is even rare. We present a case of a huge cemento-ossifying fibroma arising below the skull base of a 30-year-old woman patient. Radiologic investigations showed a giant, lobulated, heterogeneous calcified hard tissue mass, which is well circumscribed and is a mixture of radiolucent and radiopaque, situated at the rear of the right maxilla to the middle skull base. The tumor expands into the right maxillary sinus and the orbital cavity, fusing with the right maxilla at the maxillary tuberosity and blocking the bilateral choanas, which caused marked proptosis and blurred vision. The tumor was resected successfully by intraoral approach, and pathologic examination confirmed the lesion to be a cemento-ossifying fibroma. This case demonstrates that cemento-ossifying fibroma in the maxilla, not like in the mandible, may appear more aggressive because the extensive growth is unimpeded by anatomic obstacles and that the intraoral approach can be used to excise the tumor in the skull base.

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 endonasal double flap technique for reconstruction of large anterior skull base defects: technical note.

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Dolci, Ricardo Landini Lutaif; Todeschini, Alexandre Bossi; Santos, Américo Rubens Leite Dos; Lazarini, Paulo Roberto

2018-04-19

One of the main concerns in endoscopic endonasal approaches to the skull base has been the high incidence and morbidity associated with cerebrospinal fluid leaks. The introduction and routine use of vascularized flaps allowed a marked decrease in this complication followed by a great expansion in the indications and techniques used in endoscopic endonasal approaches, extending to defects from huge tumours and previously inaccessible areas of the skull base. Describe the technique of performing endoscopic double flap multi-layered reconstruction of the anterior skull base without craniotomy. Step by step description of the endoscopic double flap technique (nasoseptal and pericranial vascularized flaps and fascia lata free graft) as used and illustrated in two patients with an olfactory groove meningioma who underwent an endoscopic approach. Both patients achieved a gross total resection: subsequent reconstruction of the anterior skull base was performed with the nasoseptal and pericranial flaps onlay and a fascia lata free graft inlay. Both patients showed an excellent recovery, no signs of cerebrospinal fluid leak, meningitis, flap necrosis, chronic meningeal or sinonasal inflammation or cerebral herniation having developed. This endoscopic double flap technique we have described is a viable, versatile and safe option for anterior skull base reconstructions, decreasing the incidence of complications in endoscopic endonasal approaches. Copyright © 2018 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Eosinophilic granuloma of the skull base: patient with unique clinical moreover, radiographic presentation.

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Hosein Dalili

2015-01-01

Full Text Available This case report presents an eight-year-old girl having periauricular swelling and severe pain during mouth opening on the right-side temporomandibular joint (TMJ. CBCT showed extensive destruction of the base of the skull and the roof of the glenoid fossa on the right side. The findings based on CT and MRI images with and without contrast are discussed herein. This report highlights a skull base eosinophilic granuloma that mimics TMJ disorder and the importance of proper evaluation of CBCT images to make an early diagnosis.

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.

Comparison of SPECT/CT, MRI and CT in diagnosis of skull base bone invasion in nasopharyngeal carcinoma.

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Zhang, Shu-xu; Han, Peng-hui; Zhang, Guo-qian; Wang, Rui-hao; Ge, Yong-bin; Ren, Zhi-gang; Li, Jian-sheng; Fu, Wen-hai

2014-01-01

Early detection of skull base invasion in nasopharyngeal carcinoma (NPC) is crucial for correct staging, assessing treatment response and contouring the tumor target in radiotherapy planning, as well as improving the patient's prognosis. To compare the diagnostic efficacy of single photon emission computed tomography/computed tomography (SPECT/CT) imaging, magnetic resonance imaging (MRI) and computed tomography (CT) for the detection of skull base invasion in NPC. Sixty untreated patients with histologically proven NPC underwent SPECT/CT imaging, contrast-enhanced MRI and CT. Of the 60 patients, 30 had skull base invasion confirmed by the final results of contrast-enhanced MRI, CT and six-month follow-up imaging (MRI and CT). The diagnostic efficacy of the three imaging modalities in detecting skull base invasion was evaluated. The rates of positive findings of skull base invasion for SPECT/CT, MRI and CT were 53.3%, 48.3% and 33.3%, respectively. The sensitivity, specificity and accuracy were 93.3%, 86.7% and 90.0% for SPECT/CT fusion imaging, 96.7%, 100.0% and 98.3% for contrast-enhanced MRI, and 66.7%, 100.0% and 83.3% for contrast-enhanced CT. MRI showed the best performance for the diagnosis of skull base invasion in nasopharyngeal carcinoma, followed closely by SPECT/CT. SPECT/CT had poorer specificity than that of both MRI and CT, while CT had the lowest sensitivity.

Normal Development of Sutures and synchondroses in the central skull base : CT study

International Nuclear Information System (INIS)

Roh, Hong Gee; Kim, Hyung Jin; Kang, Jee Hee; Lee, Kyung Hee; Lim, Myung Kwan; Cho, Young Kuk; Ok, Cheol Su; Suh, Chang Hae

2000-01-01

To evaluate the developmental patterns of the sutures and synchondroses in the central skull base. We evaluated the CT scans of 109 children (age range 29 days to 15 years) with no skull base abnormality who had undergone axial CT of the skull base with 1-mm collimation. Using a five-tier scheme, we analyzed the developmental patterns of the 18 sutures and synchondroses related to the sphenoid and occipital bones. Fusion of the sutures and synchondroses related to the sphenoid bone progressed rapidly during the first two years. Thereafter, changes in the sphenoid bone were dominated by pneumatization of the sphenoid sinus. Fusion of the synchondroses within the sphenoid body, including intersphenoidal, intrapresphenoidal, intrapostsphenoidal synchondrosis occurred early and in most cases was graded ≥3D4. Fusion of the sphenosquamosal, sphenoethmoidal, and frontosphenoidal sutures was delayed, and residual sclerosis was a common finding. Except for Kerckring-supraoccipital synchondrosis, fusion of the six sutures and synchondroses related to the occipital bone occurred more gradually than that of those related to the sphenoid bone. Among these, fusion of the occipitomastoidal suture and petro-occipital synchondrosis was the last to occur. A knowledge of the developmental patterns of sutures and synchondroses can help differentiate normal conditions from those such as fracture, osseous dysplasia, or congenital malformation, which are abnormal. Our results provide certain basic information about skull base maturity in children. (author)

Skull base tumors

International Nuclear Information System (INIS)

Kikinis, R.; Matsumae, M.; Jolesz, F.A.; Black, P.M.; Cline, H.E.; Lorenson, W.E.

1991-01-01

This paper reports on an image processing procedure for the planning of surgery of skull base tumors that can extract bone, vessels, tumor, and brain parenchyma and that permits resolution of cranial nerves. Three-dimensional (3D) reconstructions were generated from double-echo long TR interleaved conventional spin-echo and fast-spin-echo MR imaging data. Sixteen cases have been analyzed preoperatively. Image processing consisted of a multistep procedure combining a supervised multivariate analysis with neighborhood operations such as connectivity and erosion/dilation. 3D renderings of anatomic structures of interest were then generated. Cases were evaluated preoperatively and manipulated interactively with the computer-generated images by a team consisting of neuroradiologists, neurosurgeons, and craniofacial surgeons. The preparation of 3D reconstructions required only a few hours and was performed mostly by a research assistant. The preoperative analysis of the 3D reconstructions was found to be a valuable tool, providing information complementing the surgeon's understanding of a case as derived from conventional imaging. The interactive manipulation of data proved to be a powerful way to evaluate alternative surgical approaches

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.

Fractionated external beam radiotherapy of skull base metastases with cranial nerve involvement.

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Dröge, L H; Hinsche, T; Canis, M; Alt-Epping, B; Hess, C F; Wolff, H A

2014-02-01

Skull base metastases frequently appear in a late stage of various tumor entities and cause pain and neurological disorders which strongly impair patient quality of life. This study retrospectively analyzed fractionated external beam radiotherapy (EBRT) as a palliative treatment approach with special respect to neurological outcome, feasibility and acute toxicity. A total of 30 patients with skull base metastases and cranial nerve disorders underwent EBRT with a mean total dose of 31.6 Gy. Neurological status was assessed before radiotherapy, during radiotherapy and 2 weeks afterwards categorizing orbital, parasellar, middle fossa, jugular foramen and occipital condyle involvement and associated clinical syndromes. Neurological outcome was scored as persistence of symptoms, partial response, good response and complete remission. Treatment-related toxicity and overall survival were assessed. Before EBRT 37 skull base involvement syndromes were determined with 4 patients showing more than 1 syndrome. Of the patients 81.1 % responded to radiotherapy with 10.8 % in complete remission, 48.6 % with good response and 21.6 % with partial response. Grade 1 toxicity of the skin occurred in two patients and grade 1 hematological toxicity in 1 patient under concurrent chemoradiotherapy. Median overall survival was 3.9 months with a median follow-up of 45 months. The use of EBRT for skull base metastases with symptomatic involvement of cranial nerves is marked by good therapeutic success in terms of neurological outcome, high feasibility and low toxicity rates. These findings underline EBRT as the standard therapeutic approach in the palliative setting.

Skull-base Osteomyelitis: a Dreaded Complication after Trivial Fall and Inadequate Management

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Kundan Mittal

2015-10-01

Full Text Available Introduction: Skull-based osteomyelitis is bony infection which generally originates from inadequately treated chronic infection, adjoining tissue infection or after trauma.Case: 11 month female child had a trivial fall while standing near a bucket. The child developed fracture of right clavicle and left orbital swelling which was inadequately treated. This resulted in in spread of infection to adjoining tissues, skull bones, sinuses and brain.Conclusion: Cranial base osteomyelitis is rare but dreaded condition which requires early diagnosis and prompt treatment to avoid mortality and morbidity in form of neurological deficits and permanent disability

Neurotization of oculomotor, trochlear and abducent nerves in skull base surgery

Institute of Scientific and Technical Information of China (English)

李世亭; 潘庆刚; 刘宁涛; 刘忠; 沈峰

2003-01-01

Objective To anatomically reconstruct the oculomotor nerve, trochlear nerve, and abducent nerve by skull base surgery. Methods Seventeen cranial nerves (three oculomotor nerves, eight trochlear nerves and six abducent nerves) were injured and anatomically reconstructed in thirteen skull base operations during a period from 1994 to 2000. Repair techniques included end-to-end neurosuture or fibrin glue adhesion, graft neurosuture or fibrin glue adhesion. The relationships between repair techniques and functional recovery and the related factors were analyzed.Results Functional recovery began from 3 to 8 months after surgery. During a follow-up period of 4 months to 6 years, complete recovery of function was observed in 6 trochlear nerves (75%) and 4 abducent nerves (67%), while partial functional recovery was observed in the other cranial nerves including 2 trochlear nerves, 2 abducent nerves, and 3 oculomotor nerves.Conclusions Complete or partial functional recovery could be expected after anatomical neurotization of an injured oculomotor, trochlear or abducent nerve. Our study demonstrated that, in terms of functional recovery, trochlear and abducent nerves are more responsive than oculomotor nerves, and that end-to-end reconstruction is more efficient than graft reconstruction. These results encourage us to perform reconstruction for a separated cranial nerve as often as possible during skull base surgery.

Intraoperative biopsy of the major cranial nerves in the surgical strategy for adenoid cystic carcinoma close to the skull base.

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Tarsitano, Achille; Pizzigallo, Angelo; Gessaroli, Manlio; Sturiale, Carmelo; Marchetti, Claudio

2012-02-01

Adenoid cystic carcinoma of the salivary glands has a propensity for perineural invasion, which could favor spread along the major cranial nerves, sometimes to the skull base and through the foramina to the brain parenchyma. This study evaluated the relationship between neural spread and relapse in the skull base. During surgery, we performed multiple biopsies with extemporaneous examination of the major nerves close to the tumor to guide the surgical resection. The percentage of actuarial local control at 5 years for patients with a positive named nerve and skull base infiltration was 12.5%, compared with 90.0% in patients who were named nerve-negative and without infiltration of the skull base (P = .001). Our study shows that local control of disease for patients who are named nerve-positive with skull base infiltration is significantly more complex compared with patients who are named nerve-negative without infiltration of the skull base. Copyright © 2012. Published by Mosby, Inc.

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

The pioneering contribution of italian surgeons to skull base surgery.

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Priola, Stefano M; Raffa, Giovanni; Abbritti, Rosaria V; Merlo, Lucia; Angileri, Filippo F; La Torre, Domenico; Conti, Alfredo; Germanò, Antonino; Tomasello, Francesco

2014-01-01

The origin of neurosurgery as a modern, successful, and separate branch of surgery could be dated back to the end of the 19th century. The most important development of surgery occurred in Europe, particularly in Italy, where there was a unique environment, allowing brilliant open-minded surgeons to perform, with success, neurosurgical operations. Neurosurgery began at the skull base. In everyday practice, we still pay tribute to early Italian neuroanatomists and pioneer neurosurgeons who represented a starting point in a new, obscure, and still challenging field of medicine and surgery during their times. In this paper, we report at a glance the contributions of Tito Vanzetti from Padua (1809-1888), for his operation on a destructive skull base cyst that had, indeed, an intracranial expansion; of Davide Giordano (1864-1954) from Venice, who described the first transnasal approach to the pituitary gland; and, most importantly, of Francesco Durante from Messina (1844-1934), who was the first surgeon in the history of neurosurgery to successfully remove a cranial base meningioma. They carried out the first detailed reported surgical excision of intracranial lesions at the skull base, diagnosed only through clinical signs; used many of the advances of the 19th century; and conceived and performed new operative strategies and approaches. Their operations were radical enough to allow the patient to survive the surgery and, in the case of Durante, for the first time, to obtain more than 12 years of good survival at a time when a tumor of this type would have been fatal. Copyright © 2014 Elsevier Inc. All rights reserved.

Efficacy of navigation in skull base surgery using composite computer graphics of magnetic resonance and computed tomography images

International Nuclear Information System (INIS)

Hayashi, Nakamasa; Kurimoto, Masanori; Hirashima, Yutaka; Ikeda, Hiroaki; Shibata, Takashi; Tomita, Takahiro; Endo, Shunro

2001-01-01

The efficacy of a neurosurgical navigation system using three-dimensional composite computer graphics (CGs) of magnetic resonance (MR) and computed tomography (CT) images was evaluated in skull base surgery. Three-point transformation was used for integration of MR and CT images. MR and CT image data were obtained with three skin markers placed on the patient's scalp. Volume-rendering manipulations of the data produced three-dimensional CGs of the scalp, brain, and lesions from the MR images, and the scalp and skull from the CT. Composite CGs of the scalp, skull, brain, and lesion were created by registering the three markers on the three-dimensional rendered scalp images obtained from MR imaging and CT in the system. This system was used for 14 patients with skull base lesions. Three-point transformation using three-dimensional CGs was easily performed for multimodal registration. Simulation of surgical procedures on composite CGs aided in comprehension of the skull base anatomy and selection of the optimal approaches. Intraoperative navigation aided in determination of actual spatial position in the skull base and the optimal trajectory to the tumor during surgical procedures. (author)

How to perform 3D reconstruction of skull base tumours.

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Bonne, N-X; Dubrulle, F; Risoud, M; Vincent, C

2017-04-01

The surgical management of skull base lesions is difficult due to the complex anatomy of the region and the intimate relations between the lesion and adjacent nerves and vessels. Minimally invasive approaches are increasingly used in skull base surgery to ensure an optimal functional prognosis. Three-dimensional (3D) computed tomography (CT) reconstruction facilitates surgical planning by visualizing the anatomical relations of the lesions in all planes (arteries, veins, nerves, inner ear) and simulation of the surgical approach in the operating position. Helical CT angiography is performed with optimal timing of the injection in terms of tumour and vessel contrast enhancement. 3D definition of each structure is based on colour coding by automatic thresholding (bone, vessels) or manual segmentation on each slice (tumour, nerves, inner ear). Imaging is generally presented in 3 dimensions (superior, coronal, sagittal) with simulation of the surgical procedure (5 to 6 reconstructions in the operating position at different depths). Copyright © 2016. Published by Elsevier Masson SAS.

Experimental demonstration of passive acoustic imaging in the human skull cavity using CT-based aberration corrections.

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Jones, Ryan M; O'Reilly, Meaghan A; Hynynen, Kullervo

2015-07-01

Experimentally verify a previously described technique for performing passive acoustic imaging through an intact human skull using noninvasive, computed tomography (CT)-based aberration corrections Jones et al. [Phys. Med. Biol. 58, 4981-5005 (2013)]. A sparse hemispherical receiver array (30 cm diameter) consisting of 128 piezoceramic discs (2.5 mm diameter, 612 kHz center frequency) was used to passively listen through ex vivo human skullcaps (n = 4) to acoustic emissions from a narrow-band fixed source (1 mm diameter, 516 kHz center frequency) and from ultrasound-stimulated (5 cycle bursts, 1 Hz pulse repetition frequency, estimated in situ peak negative pressure 0.11-0.33 MPa, 306 kHz driving frequency) Definity™ microbubbles flowing through a thin-walled tube phantom. Initial in vivo feasibility testing of the method was performed. The performance of the method was assessed through comparisons to images generated without skull corrections, with invasive source-based corrections, and with water-path control images. For source locations at least 25 mm from the inner skull surface, the modified reconstruction algorithm successfully restored a single focus within the skull cavity at a location within 1.25 mm from the true position of the narrow-band source. The results obtained from imaging single bubbles are in good agreement with numerical simulations of point source emitters and the authors' previous experimental measurements using source-based skull corrections O'Reilly et al. [IEEE Trans. Biomed. Eng. 61, 1285-1294 (2014)]. In a rat model, microbubble activity was mapped through an intact human skull at pressure levels below and above the threshold for focused ultrasound-induced blood-brain barrier opening. During bursts that led to coherent bubble activity, the location of maximum intensity in images generated with CT-based skull corrections was found to deviate by less than 1 mm, on average, from the position obtained using source-based corrections. Taken

Skull Radiography

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What you need to know about… Skull Radiography X-ray images of the skull are taken when it is necessary to see the cranium, facial bones or jaw bones. ... Among other things, x-ray exams of the skull can show fractures. Patient Preparation Before the examination, ...

MRI-detected skull-base invasion. Prognostic value and therapeutic implication in intensity-modulated radiotherapy treatment for nasopharyngeal carcinoma

International Nuclear Information System (INIS)

Cheng, Yi-Kan; Jiang, Ning; Yue, Dan; Tang, Ling-Long; Zhang, Fan; Lin, Li; Liu, Xu; Chen, Lei; Ma, Jun; Liu, Li-Zhi

2014-01-01

With advances in imaging and radiotherapy, the prognostic value of skull-base invasion in nasopharyngeal carcinoma (NPC) needs to be reassessed. We aimed to define a classification system and evaluate the prognostic value of the classification of magnetic resonance imaging (MRI)-detected skull-base invasion in NPC treated with intensity-modulated radiotherapy (IMRT). We retrospectively reviewed 749 patients who underwent MRI and were subsequently histologically diagnosed with nondisseminated NPC and treated with IMRT. MRI-detected skull-base invasion was not found to be an independent prognostic factor for overall survival (OS), distant metastasis-free survival (DMFS), local relapse-free survival (LRFS), or disease-free survival (DFS; p > 0.05 for all). Skull-base invasion was classified according to the incidence of each site (type I sites inside pharyngobasilar fascia and clivus vs. type II sites outside pharyngobasilar fascia). The 5-year OS, DMFS, LRFS, and DFS rates in the classification of skull-base invasion in NPC were 83 vs. 67 %, 85 vs.75 %, 95 vs. 88 %, and 76 vs. 62 %, respectively (p [de

Computed tomography in neoplastic diseases of the skull base and adjacent areas using EMI scanner CT-1000

International Nuclear Information System (INIS)

Ono, Yoshimi; Ohtake, Eiji; Asakura, Koichi; Tanohata, Kazunori; Ito, Otomasa

1980-01-01

CT-findings of 145 patients with and without neoplastic diseases of the skull base were evaluated using EMI CT-1000. Analysis of 64 patients without any lesion at the skull base showed more artifacts compared with that of higher slices, so that good or fair images were obtained only in 64% of this groups of patients. The most important factor in producing artifacts are considered to be caused by patient's movement. We also evaluated the tumor extension to the skull base in 81 patients. They were 24 brain tumors, 21 pituitary adenomas, and 36 nasopharyngeal and paranasal cancers. Four out of 24 brain tumors showed extracranial extension, 12 pituitary adenomas infiltrating outside of the sella turcica, and 10 cases of nasopharyngeal cancers showed intracranial extension. It was concluded that CT presented an excellent information in evaluating the degree of extension of neoplasms which invading to the skull base. (author)

Evaluation of Three Cases Using a Novel Titanium Mesh System-Skull-Fit with Orbital Wall (Skull-Fit WOW)-For Cranial Base Reconstructions.

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Hattori, Noriko; Nakajima, Hideo; Tamada, Ikkei; Sakamoto, Yoshiaki; Ohira, Takayuki; Yoshida, Kazunari; Kawase, Takeshi; Kishi, Kazuo

2011-09-01

Cranial base reconstructions associated with tumor resections around the orbital wall often require that both the upper and lateral orbital walls be reconstructed during a single procedure. Previously, we used titanium mesh plates that were preoperatively fabricated based on three-dimensional models. Although these plates are precise and do not increase the probability of infection, we still had to use autologous bones to reconstruct the orbital walls. Recently, we developed a new titanium mesh plate-called Skull-Fit(®)-with orbital wall (Skull-Fit WOW(®)), enabling us to reconstruct the cranial base and orbital walls without bone grafts. Here, we report on three reconstruction cases in which the novel titanium mesh-orbital wall system was used. In all three cases, the customized titanium mesh system performed satisfactorily with little, if any, complications.

Skull Practice.

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Slesnick, Irwin L.

1988-01-01

Disguises a lesson about skulls with some fun to cause less fear among students. Outlines strategies, questions, and answers for use. Includes a skull mask which can be photocopied and distributed to students as a learning tool and a fun Halloween treat. Also shown is a picture of skull parts. (RT)

Localized intraoperative virtual endoscopy (LIVE) for surgical guidance in 16 skull base patients.

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Haerle, Stephan K; Daly, Michael J; Chan, Harley; Vescan, Allan; Witterick, Ian; Gentili, Fred; Zadeh, Gelareh; Kucharczyk, Walter; Irish, Jonathan C

2015-01-01

Previous preclinical studies of localized intraoperative virtual endoscopy-image-guided surgery (LIVE-IGS) for skull base surgery suggest a potential clinical benefit. The first aim was to evaluate the registration accuracy of virtual endoscopy based on high-resolution magnetic resonance imaging under clinical conditions. The second aim was to implement and assess real-time proximity alerts for critical structures during skull base drilling. Patients consecutively referred for sinus and skull base surgery were enrolled in this prospective case series. Five patients were used to check registration accuracy and feasibility with the subsequent 11 patients being treated under LIVE-IGS conditions with presentation to the operating surgeon (phase 2). Sixteen skull base patients were endoscopically operated on by using image-based navigation while LIVE-IGS was tested in a clinical setting. Workload was quantitatively assessed using the validated National Aeronautics and Space Administration Task Load Index (NASA-TLX) questionnaire. Real-time localization of the surgical drill was accurate to ~1 to 2 mm in all cases. The use of 3-mm proximity alert zones around the carotid arteries and optic nerve found regular clinical use, as the median minimum distance between the tracked drill and these structures was 1 mm (0.2-3.1 mm) and 0.6 mm (0.2-2.5 mm), respectively. No statistical differences were found in the NASA-TLX indicators for this experienced surgical cohort. Real-time proximity alerts with virtual endoscopic guidance was sufficiently accurate under clinical conditions. Further clinical evaluation is required to evaluate the potential surgical benefits, particularly for less experienced surgeons or for teaching purposes. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014.

Automatic Sex Determination of Skulls Based on a Statistical Shape Model

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Li Luo

2013-01-01

Full Text Available Sex determination from skeletons is an important research subject in forensic medicine. Previous skeletal sex assessments are through subjective visual analysis by anthropologists or metric analysis of sexually dimorphic features. In this work, we present an automatic sex determination method for 3D digital skulls, in which a statistical shape model for skulls is constructed, which projects the high-dimensional skull data into a low-dimensional shape space, and Fisher discriminant analysis is used to classify skulls in the shape space. This method combines the advantages of metrical and morphological methods. It is easy to use without professional qualification and tedious manual measurement. With a group of Chinese skulls including 127 males and 81 females, we choose 92 males and 58 females to establish the discriminant model and validate the model with the other skulls. The correct rate is 95.7% and 91.4% for females and males, respectively. Leave-one-out test also shows that the method has a high accuracy.

[Management of occult malformations at the lateral skull base].

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Bryson, E; Draf, W; Hofmann, E; Bockmühl, U

2005-12-01

Occult malformations of the lateral skull base are rare anomalies, but can cause severe complications such as recurrent meningitis. Therefore, they need to be precisely delineated and sufficient surgical closure is mandatory. Between 1986 and 2004 twenty patients (10 children and 10 adults) with occult malformations at the lateral skull base were treated surgically at the ENT-Department of the Hospital Fulda gAG. Of these 3 Mondini-malformations, 11 defects of the tegmen tympani or the mastoidal roof, 2 dural lesions to the posterior fossa and 4 malformations within the pyramidal apex have been found. Four patients have had multiple anomalies. Routing symptom was in all cases at least one previous meningitis. Radiological diagnostics included high-resolution computed tomography (CT) and magnetic resonance imaging (MRI) as well as CT- or MR-cisternography. Depending on type and localisation of the defect the following surgical algorithm was carried out: The trans-mastoidal approach was used in all cases of Mondini-malformation (including obliteration of the ear), in case of lesions to the posterior fossa as well as partly in anomalies at the tegmen tympani and mastoidal roof, respectively. Defects of the pyramidal apex should be explored via the trans-mastoidal way if the lesion is located caudally to the inner auditory canal (IAC), whereas the trans-temporal approach should be used if the lesion is situated ventral to the IAC and dorso-medially to the internal carotid artery (ICA). The trans-temporal approach was also performed in large defects of the tegmen tympani and mastoidal roof as well as in recurrences. In all cases of recurrent meningitis caused by agents of the upper airway tract the basic principle should be to search for occult skull base malformations radiologically as well as by sodium fluorescein endoscopy as long as the anomaly is detected.

Panorama of Reconstruction of Skull Base Defects: From Traditional Open to Endonasal Endoscopic Approaches, from Free Grafts to Microvascular Flaps

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Reyes, Camilo; Mason, Eric; Solares, C. Arturo

2014-01-01

Introduction A substantial body of literature has been devoted to the distinct characteristics and surgical options to repair the skull base. However, the skull base is an anatomically challenging location that requires a three-dimensional reconstruction approach. Furthermore, advances in endoscopic skull base surgery encompass a wide range of surgical pathology, from benign tumors to sinonasal cancer. This has resulted in the creation of wide defects that yield a new challenge in skull base reconstruction. Progress in technology and imaging has made this approach an internationally accepted method to repair these defects. Objectives Discuss historical developments and flaps available for skull base reconstruction. Data Synthesis Free grafts in skull base reconstruction are a viable option in small defects and low-flow leaks. Vascularized flaps pose a distinct advantage in large defects and high-flow leaks. When open techniques are used, free flap reconstruction techniques are often necessary to repair large entry wound defects. Conclusions Reconstruction of skull base defects requires a thorough knowledge of surgical anatomy, disease, and patient risk factors associated with high-flow cerebrospinal fluid leaks. Various reconstruction techniques are available, from free tissue grafting to vascularized flaps. Possible complications that can befall after these procedures need to be considered. Although endonasal techniques are being used with increasing frequency, open techniques are still necessary in selected cases. PMID:25992142

Postoperative otorhinolaryngologic complications in transnasal endoscopic surgery to access the skull base

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Ricardo Landini Lutaif Dolci

Full Text Available Abstract Introduction: The large increase in the number of transnasal endoscopic skull base surgeries is a consequence of greater knowledge of the anatomic region, the development of specific materials and instruments, and especially the use of the nasoseptal flap as a barrier between the sinus tract (contaminated cavity and the subarachnoid space (sterile area, reducing the high risk of contamination. Objective: To assess the otorhinolaryngologic complications in patients undergoing endoscopic surgery of the skull base, in which a nasoseptal flap was used. Methods: This was a retrospective study that included patients who underwent endoscopic skull base surgery with creation of a nasoseptal flap, assessing for the presence of the following post-surgical complications: cerebrospinal fluid leak, meningitis, mucocele formation, nasal synechia, septal perforation (prior to posterior septectomy, internal nasal valve failure, epistaxis, and olfactory alterations. Results: The study assessed 41 patients undergoing surgery. Of these, 35 had pituitary adenomas (macro- or micro-adenomas; sellar and suprasellar extension, three had meningiomas (two tuberculum sellae and one olfactory groove, two had craniopharyngiomas, and one had an intracranial abscess. The complications were cerebrospinal fluid leak (three patients; 7.3%, meningitis (three patients; 7.3%, nasal fossa synechia (eight patients; 19.5%, internal nasal valve failure (six patients; 14.6%, and complaints of worsening of the sense of smell (16 patients; 39%. The olfactory test showed anosmia or hyposmia in ten patients (24.3%. No patient had mucocele, epistaxis, or septal perforation. Conclusion: The use of the nasoseptal flap has revolutionized endoscopic skull base surgery, making the procedures more effective and with lower morbidity compared to the traditional route. However, although mainly transient nasal morbidities were observed, in some cases, permanent hyposmia and anosmia resulted

Intraoperative Neurophysiological Monitoring for Endoscopic Endonasal Approaches to the Skull Base: A Technical Guide

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Harminder Singh

2016-01-01

Full Text Available Intraoperative neurophysiological monitoring during endoscopic, endonasal approaches to the skull base is both feasible and safe. Numerous reports have recently emerged from the literature evaluating the efficacy of different neuromonitoring tests during endonasal procedures, making them relatively well-studied. The authors report on a comprehensive, multimodality approach to monitoring the functional integrity of at risk nervous system structures, including the cerebral cortex, brainstem, cranial nerves, corticospinal tract, corticobulbar tract, and the thalamocortical somatosensory system during endonasal surgery of the skull base. The modalities employed include electroencephalography, somatosensory evoked potentials, free-running and electrically triggered electromyography, transcranial electric motor evoked potentials, and auditory evoked potentials. Methodological considerations as well as benefits and limitations are discussed. The authors argue that, while individual modalities have their limitations, multimodality neuromonitoring provides a real-time, comprehensive assessment of nervous system function and allows for safer, more aggressive management of skull base tumors via the endonasal route.

A fiducial skull marker for precise MRI-based stereotaxic surgery in large animal models.

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Glud, Andreas Nørgaard; Bech, Johannes; Tvilling, Laura; Zaer, Hamed; Orlowski, Dariusz; Fitting, Lise Moberg; Ziedler, Dora; Geneser, Michael; Sangill, Ryan; Alstrup, Aage Kristian Olsen; Bjarkam, Carsten Reidies; Sørensen, Jens Christian Hedemann

2017-06-15

Stereotaxic neurosurgery in large animals is used widely in different sophisticated models, where precision is becoming more crucial as desired anatomical target regions are becoming smaller. Individually calculated coordinates are necessary in large animal models with cortical and subcortical anatomical differences. We present a convenient method to make an MRI-visible skull fiducial for 3D MRI-based stereotaxic procedures in larger experimental animals. Plastic screws were filled with either copper-sulfate solution or MRI-visible paste from a commercially available cranial head marker. The screw fiducials were inserted in the animal skulls and T1 weighted MRI was performed allowing identification of the inserted skull marker. Both types of fiducial markers were clearly visible on the MRÍs. This allows high precision in the stereotaxic space. The use of skull bone based fiducial markers gives high precision for both targeting and evaluation of stereotaxic systems. There are no metal artifacts and the fiducial is easily removed after surgery. The fiducial marker can be used as a very precise reference point, either for direct targeting or in evaluation of other stereotaxic systems. Copyright © 2017 Elsevier B.V. All rights reserved.

Radiological assessment of skull base changes in children with syndromic craniosynostosis: role of ''minor'' sutures

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Calandrelli, Rosalinda; D' Apolito, Gabriella; Gaudino, Simona; Stefanetti, Mariangela; Colosimo, Cesare [Universita Cattolica Sacro Cuore, Institute of Radiology, Rome (Italy); Massimi, Luca; Di Rocco, Concezio [Universita Cattolica Sacro Cuore, Institute of Neurosurgery, Rome (Italy)

2014-10-15

This study aims to identify the premature synostosis of ''major'' and ''minor'' sutures of the four ''sutural arches'' of the skull and to perform a morphometric analysis in children with syndromic craniosynostosis in order to evaluate changes in the skull base linked with premature suture synostosis. We reviewed multiplanar high-resolution CT images, implemented with 3D reconstructions, from 18 patients with complex syndromic craniosynostosis and compared them with 18 age-matched healthy subjects. We assessed the calvarial sutures and their extension to the skull base, and then we correlated specific types of synostosis with the size, shape and symmetry of the cranial fossae. We found a marked asymmetry of the skull base growth in all patients. The synostotic involvement around the coronal ring caused a reduction in the growth of the anterior and middle fossae. The size of the posterior cranial fossa was related not only to ''major'' but also to ''minor'' suture synostosis of the lambdoid and parieto-squamosal arches. Changes in the skull base and craniofacial axis symmetry are due to structural and functional relationships between ''major'' and ''minor'' skull sutures, suggesting a structural and functional relationship between the neurocranium and basicranium. The early recognition of prematurely closed skull base sutures may help clinicians and neurosurgeons to establish correct therapeutic approaches. (orig.)

[Applicability of the da Vinci robotic system in the skull base surgical approach. Preclinical investigation].

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Fernandez-Nogueras Jimenez, Francisco J; Segura Fernandez-Nogueras, Miguel; Jouma Katati, Majed; Arraez Sanchez, Miguel Ángel; Roda Murillo, Olga; Sánchez Montesinos, Indalecio

2015-01-01

The role of robotic surgery is well established in various specialties such as urology and general surgery, but not in others such as neurosurgery and otolaryngology. In the case of surgery of the skull base, it has just emerged from an experimental phase. To investigate possible applications of the da Vinci surgical robot in transoral skull base surgery, comparing it with the authors' experience using conventional endoscopic transnasal surgery in the same region. A transoral transpalatal approach to the nasopharynx and medial skull base was performed on 4 cryopreserved cadaver heads. We used the da Vinci robot, a 30° standard endoscope 12mm thick, dual camera and dual illumination, Maryland forceps on the left terminal and curved scissors on the right, both 8mm thick. Bone drilling was performed manually. For the anatomical study of this region, we used 0.5cm axial slices from a plastinated cadaver head. Various skull base structures at different depths were reached with relative ease with the robot terminals Transoral robotic surgery with the da Vinci system provides potential advantages over conventional endoscopic transnasal surgery in the surgical approach to this region. Copyright © 2014 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

Sinonasal outcomes following endoscopic anterior skull base surgery with nasoseptal flap reconstruction: a prospective study.

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Hanson, M; Patel, P M; Betz, C; Olson, S; Panizza, B; Wallwork, B

2015-07-01

To assess nasal morbidity resulting from nasoseptal flap use in the repair of skull base defects in endoscopic anterior skull base surgery. Thirty-six patients awaiting endoscopic anterior skull base surgery were prospectively recruited. A nasoseptal flap was used for reconstruction in all cases. Patients were assessed pre-operatively and 90 days post-operatively via the Sino-Nasal Outcome Test 20 questionnaire and visual analogue scales for nasal obstruction, pain, secretions and smell; endoscopic examination findings and mucociliary clearance times were also recorded. Sino-Nasal Outcome Test 20 questionnaire data and visual analogue scale scores for pain, smell and secretions showed no significant differences between pre- and post-operative outcomes, with visual analogue scale scores for nasal obstruction actually showing a significant improvement (p = 0.0007). A significant deterioration for both flap and non-flap sides was demonstrated post-operatively on endoscopic examination (p = 0.002 and p = 0.02 respectively). Whilst elevation of a nasoseptal flap in endoscopic surgery of the anterior skull base engendered significant clinical deterioration on examination post-operatively, quality of life outcomes showed that no such deterioration was subjectively experienced by the patient. In fact, there was significant nasal airway improvement following nasoseptal flap reconstruction.

A checklist for endonasal transsphenoidal anterior skull base surgery.

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Laws, Edward R; Wong, Judith M; Smith, Timothy R; de Los Reyes, Kenneth; Aglio, Linda S; Thorne, Alison J; Cote, David J; Esposito, Felice; Cappabianca, Paolo; Gawande, Atul

2016-06-01

OBJECT Approximately 250 million surgical procedures are performed annually worldwide, and data suggest that major complications occur in 3%-17% of them. Many of these complications can be classified as avoidable, and previous studies have demonstrated that preoperative checklists improve operating room teamwork and decrease complication rates. Although the authors' institution has instituted a general preoperative "time-out" designed to streamline communication, flatten vertical authority gradients, and decrease procedural errors, there is no specific checklist for transnasal transsphenoidal anterior skull base surgery, with or without endoscopy. Such minimally invasive cranial surgery uses a completely different conceptual approach, set-up, instrumentation, and operative procedure. Therefore, it can be associated with different types of complications as compared with open cranial surgery. The authors hypothesized that a detailed, procedure-specific, preoperative checklist would be useful to reduce errors, improve outcomes, decrease delays, and maximize both teambuilding and operational efficiency. Thus, the object of this study was to develop such a checklist for endonasal transsphenoidal anterior skull base surgery. METHODS An expert panel was convened that consisted of all members of the typical surgical team for transsphenoidal endoscopic cases: neurosurgeons, anesthesiologists, circulating nurses, scrub technicians, surgical operations managers, and technical assistants. Beginning with a general checklist, procedure-specific items were added and categorized into 4 pauses: Anesthesia Pause, Surgical Pause, Equipment Pause, and Closure Pause. RESULTS The final endonasal transsphenoidal anterior skull base surgery checklist is composed of the following 4 pauses. The Anesthesia Pause consists of patient identification, diagnosis, pertinent laboratory studies, medications, surgical preparation, patient positioning, intravenous/arterial access, fluid management

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.)

Lateral skull base approaches in the management of benign parapharyngeal space tumors.

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Prasad, Sampath Chandra; Piccirillo, Enrico; Chovanec, Martin; La Melia, Claudio; De Donato, Giuseppe; Sanna, Mario

2015-06-01

To evaluate the role of lateral skull base approaches in the management of benign parapharyngeal space tumors and to propose an algorithm for their surgical approach. Retrospective study of patients with benign parapharyngeal space tumors. The clinical features, radiology and preoperative management of skull base neurovasculature, the surgical approaches and overall results were recorded. 46 patients presented with 48 tumors. 12 were prestyloid and 36 poststyloid. 19 (39.6%) tumors were paragangliomas, 15 (31.25%) were schwannomas and 11 (23%) were pleomorphic adenomas. Preoperative embolization was performed in 19, stenting of the internal carotid artery in 4 and permanent balloon occlusion in 2 patients. 19 tumors were approached by the transcervical, 13 by transcervical-transparotid, 5 by transcervical-transmastoid, 6, 1 and 2 tumors by the infratemporal fossa approach types A, B and D, respectively. Total radical tumor removal was achieved in 46 (96%) of the cases. Lateral skull base approaches have an advantage over other approaches in the management of benign tumors of the parapharyngeal space due to the fact that they provide excellent exposure with less morbidity. The use of microscope combined with bipolar cautery reduces morbidity. Stenting of internal carotid artery gives a chance for complete tumor removal with arterial preservation. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Management of Benign Skull Base Meningiomas: A Review

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Mendenhall, William M.; Friedman, William A.; Amdur, Robert J.; Foote, Kelly D.

2004-01-01

The optimal management of benign meningiomas of the skull base is reviewed. Elderly patients with small, asymptomatic tumors can be observed and treatment can be initiated if and when progression occurs. Patients with tumors that appear to be amenable to complete resection with an acceptable rate of morbidity are optimally treated with surgery. Decompression of more extensive tumors through conservative subtotal resection and preservation of the involved cranial nerves may result in improved ...

Skull base development and craniosynostosis

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Blaser, Susan I. [The Hospital for Sick Children and University of Toronto, Department of Diagnostic Imaging, Division of Neuroradiology, Toronto (Canada); University of Toronto, Department of Otolaryngology - Head and Neck Surgery, Toronto (Canada); Padfield, Nancy [The Hospital for Sick Children and University of Toronto, Department of Diagnostic Imaging, Division of Neuroradiology, Toronto (Canada); Chitayat, David [The Hospital for Sick Children and University of Toronto, Division of Clinical and Metabolic Genetics, Toronto (Canada); Mount Sinai Hospital and University of Toronto, Prenatal Diagnosis and Medical Genetics Program, Toronto (Canada); Forrest, Christopher R. [The Hospital for Sick Children and University of Toronto, Centre for Craniofacial Care and Research, Division of Plastic and Reconstructive Surgery, Toronto (Canada)

2015-09-15

Abnormal skull shape resulting in craniofacial deformity is a relatively common clinical finding, with deformity either positional (positional plagiocephaly) or related to premature ossification and fusion of the skull sutures (craniosynostosis). Growth restriction occurring at a stenosed suture is associated with exaggerated growth at the open sutures, resulting in fairly predictable craniofacial phenotypes in single-suture non-syndromic pathologies. Multi-suture syndromic subtypes are not so easy to understand without imaging. Imaging is performed to define the site and extent of craniosynostosis, to determine the presence or absence of underlying brain anomalies, and to evaluate both pre- and postoperative complications of craniosynostosis. Evidence for intracranial hypertension may be seen both pre- and postoperatively, associated with jugular foraminal stenosis, sinovenous occlusion, hydrocephalus and Chiari 1 malformations. Following clinical assessment, imaging evaluation may include radiographs, high-frequency US of the involved sutures, low-dose (20-30 mAs) CT with three-dimensional reformatted images, MRI and nuclear medicine brain imaging. Anomalous or vigorous collateral venous drainage may be mapped preoperatively with CT or MR venography or catheter angiography. (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

Multimodal navigated skull base tumor resection using image-based vascular and cranial nerve segmentation: A prospective pilot study.

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Dolati, Parviz; Gokoglu, Abdulkerim; Eichberg, Daniel; Zamani, Amir; Golby, Alexandra; Al-Mefty, Ossama

2015-01-01

Skull base tumors frequently encase or invade adjacent normal neurovascular structures. For this reason, optimal tumor resection with incomplete knowledge of patient anatomy remains a challenge. To determine the accuracy and utility of image-based preoperative segmentation in skull base tumor resections, we performed a prospective study. Ten patients with skull base tumors underwent preoperative 3T magnetic resonance imaging, which included thin section three-dimensional (3D) space T2, 3D time of flight, and magnetization-prepared rapid acquisition gradient echo sequences. Imaging sequences were loaded in the neuronavigation system for segmentation and preoperative planning. Five different neurovascular landmarks were identified in each case and measured for accuracy using the neuronavigation system. Each segmented neurovascular element was validated by manual placement of the navigation probe, and errors of localization were measured. Strong correspondence between image-based segmentation and microscopic view was found at the surface of the tumor and tumor-normal brain interfaces in all cases. The accuracy of the measurements was 0.45 ± 0.21 mm (mean ± standard deviation). This information reassured the surgeon and prevented vascular injury intraoperatively. Preoperative segmentation of the related cranial nerves was possible in 80% of cases and helped the surgeon localize involved cranial nerves in all cases. Image-based preoperative vascular and neural element segmentation with 3D reconstruction is highly informative preoperatively and could increase the vigilance of neurosurgeons for preventing neurovascular injury during skull base surgeries. Additionally, the accuracy found in this study is superior to previously reported measurements. This novel preliminary study is encouraging for future validation with larger numbers of patients.

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

Directory of Open Access Journals (Sweden)

Constantine L. Karras

2016-01-01

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

The anterior interhemispheric approach: a safe and effective approach to anterior skull base lesions.

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Mielke, Dorothee; Mayfrank, Lothar; Psychogios, Marios Nikos; Rohde, Veit

2014-04-01

Many approaches to the anterior skull base have been reported. Frequently used are the pterional, the unilateral or bilateral frontobasal, the supraorbital and the frontolateral approach. Recently, endoscopic transnasal approaches have become more popular. The benefits of each approach has to be weighted against its complications and limitations. The aim of this study was to investigate if the anterior interhemispheric approach (AIA) could be a safe and effective alternative approach to tumorous and non-tumorous lesions of the anterior skull base. We screened the operative records of all patients with an anterior skull base lesion undergoing transcranial surgery. We have used the AIA in 61 patients. These were exclusively patients with either olfactory groove meningioma (OGM) (n = 43), ethmoidal dural arteriovenous fistula (dAVF) ( n = 6) or frontobasal fractures of the anterior midline with cerebrospinal fluid (CSF) leakage ( n = 12). Patient records were evaluated concerning accessibility of the lesion, realization of surgical aims (complete tumor removal, dAVF obliteration, closure of the dural tear), and approach related complications. The use of the AIA exclusively in OGMs, ethmoidal dAVFs and midline frontobasal fractures indicated that we considered lateralized frontobasal lesions not suitable to be treated successfully. If restricted to these three pathologies, the AIA is highly effective and safe. The surgical aim (complete tumor removal, complete dAVF occlusion, no rhinorrhea) was achieved in all patients. The complication rate was 11.5 % (wound infection (n = 2; 3.2 %), contusion of the genu of the corpus callosum, subdural hygroma, epileptic seizure, anosmia and asymptomatic bleed into the tumor cavity (n = 1 each). Only the contusion of the corpus callosum was directly related to the approach (1.6 %). Olfaction, if present before surgery, was preserved in all patients, except one (1.6 %). The AIA is an effective and a safe approach

Evaluation of Three Cases Using a Novel Titanium Mesh System—Skull-Fit® with Orbital Wall (Skull-Fit WOW®)—For Cranial Base Reconstructions

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Hattori, Noriko; Nakajima, Hideo; Tamada, Ikkei; Sakamoto, Yoshiaki; Ohira, Takayuki; Yoshida, Kazunari; Kawase, Takeshi; Kishi, Kazuo

2011-01-01

Cranial base reconstructions associated with tumor resections around the orbital wall often require that both the upper and lateral orbital walls be reconstructed during a single procedure. Previously, we used titanium mesh plates that were preoperatively fabricated based on three-dimensional models. Although these plates are precise and do not increase the probability of infection, we still had to use autologous bones to reconstruct the orbital walls. Recently, we developed a new titanium mesh plate—called Skull-Fit®—with orbital wall (Skull-Fit WOW®), enabling us to reconstruct the cranial base and orbital walls without bone grafts. Here, we report on three reconstruction cases in which the novel titanium mesh-orbital wall system was used. In all three cases, the customized titanium mesh system performed satisfactorily with little, if any, complications. PMID:22451827

Hepatocellular carcinoma metastasizing to the skull base involving multiple cranial nerves.

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Kim, Soo Ryang; Kanda, Fumio; Kobessho, Hiroshi; Sugimoto, Koji; Matsuoka, Toshiyuki; Kudo, Masatoshi; Hayashi, Yoshitake

2006-11-07

We describe a rare case of HCV-related recurrent multiple hepatocellular carcinoma (HCC) metastasizing to the skull base involving multiple cranial nerves in a 50-year-old woman. The patient presented with symptoms of ptosis, fixation of the right eyeball, and left abducens palsy, indicating disturbances of the right oculomotor and trochlear nerves and bilateral abducens nerves. Brain contrast-enhanced computed tomography (CT) revealed an ill-defined mass with abnormal enhancement around the sella turcica. Brain magnetic resonance imaging (MRI) disclosed that the mass involved the clivus, cavernous sinus, and petrous apex. On contrast-enhanced MRI with gadolinium-chelated contrast medium, the mass showed inhomogeneous intermediate enhancement. The diagnosis of metastatic HCC to the skull base was made on the basis of neurological findings and imaging studies including CT and MRI, without histological examinations. Further studies may provide insights into various methods for diagnosing HCC metastasizing to the craniospinal area.

Hepatocellular carcinoma metastasizing to the skull base involving multiple cranial nerves

Institute of Scientific and Technical Information of China (English)

Soo Ryang Kim; Fumio Kanda; Hiroshi Kobessho; Koji Sugimoto; Toshiyuki Matsuoka; Masatoshi Kudo; Yoshitake Hayashi

2006-01-01

We describe a rare case of HCV-related recurrent multiple hepatocellular carcinoma (HCC) metastasizing to the skull base involving multiple cranial nerves in a 50-yearold woman. The patient presented with symptoms of ptosis, fixation of the right eyeball, and left abducens palsy, indicating disturbances of the right oculomotor and trochlear nerves and bilateral abducens nerves. Brain contrast-enhanced computed tomography (CT) revealed an ill-defined mass with abnormal enhancement around the sella turcica. Brain magnetic resonance imaging (MRI)disclosed that the mass involved the clivus, cavernous sinus, and petrous apex. On contrast-enhanced MRI with gadolinium-chelated contrast medium, the mass showed inhomogeneous intermediate enhancement.The diagnosis of metastatic HCC to the skull base was made on the basis of neurological findings and imaging studies including CT and MRI, without histological examinations. Further studies may provide insights into various methods for diagnosing HCC metastasizing to the craniospinal area.

Prospective analysis of neuropsychological deficits following resection of benign skull base meningiomas.

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Zweckberger, Klaus; Hallek, Eveline; Vogt, Lidia; Giese, Henrik; Schick, Uta; Unterberg, Andreas W

2017-12-01

OBJECTIVE Resection of skull base tumors is challenging. The introduction of alternative treatment options, such as radiotherapy, has sparked discussion regarding outcome in terms of quality of life and neuropsychological deficits. So far, however, no prospective data are available on this topic. METHODS A total of 58 patients with skull base meningiomas who underwent surgery for the first time were enrolled in this prospective single-center trial. The average age of the patients was 56.4 ± 12.5 years. Seventy-nine percent of the tumors were located within the anterior skull base. Neurological examinations and neuropsychological testing were performed at 3 time points: 1 day prior to surgery (T1), 3-5 months after surgery (T2), and 9-12 months after surgery (T3). The average follow-up duration was 13.8 months. Neuropsychological assessment consisted of quality of life, depression and anxiety, verbal learning and memory, cognitive speed, attention and concentration, figural memory, and visual-motor speed. RESULTS Following surgery, 23% of patients showed transient neurological deficits and 12% showed permanent new neurological deficits with varying grades of manifestation. Postoperative quality of life, however, remained stable and was slightly improved at follow-up examinations at T3 (60.6 ± 21.5 vs 63.6 ± 24.1 points), and there was no observed effect on anxiety and depression. Long-term verbal memory, working memory, and executive functioning were slightly affected within the first months following surgery and appeared to be the most vulnerable to impairment by the tumor or the resection but were stable or improved in the majority of patients at long-term follow-up examinations after 1 year. CONCLUSIONS This report describes the first prospective study of neuropsychological outcomes following resection of skull base meningiomas and, as such, contributes to a better understanding of postoperative impairment in these patients. Despite deterioration in a minority

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.)

Skull (image)

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The skull is anterior to the spinal column and is the bony structure that encases the brain. Its purpose ... the facial muscles. The two regions of the skull are the cranial and facial region. The cranial ...

Preoperative Visualization of Cranial Nerves in Skull Base Tumor Surgery Using Diffusion Tensor Imaging Technology.

Science.gov (United States)

Ma, Jun; Su, Shaobo; Yue, Shuyuan; Zhao, Yan; Li, Yonggang; Chen, Xiaochen; Ma, Hui

2016-01-01

To visualize cranial nerves (CNs) using diffusion tensor imaging (DTI) with special parameters. This study also involved the evaluation of preoperative estimates and intraoperative confirmation of the relationship between nerves and tumor by verifying the accuracy of visualization. 3T magnetic resonance imaging scans including 3D-FSPGR, FIESTA, and DTI were used to collect information from 18 patients with skull base tumor. DTI data were integrated into the 3D slicer for fiber tracking and overlapped anatomic images to determine course of nerves. 3D reconstruction of tumors was achieved to perform neighboring, encasing, and invading relationship between lesion and nerves. Optic pathway including the optic chiasm could be traced in cases of tuberculum sellae meningioma and hypophysoma (pituitary tumor). The oculomotor nerve, from the interpeduncular fossa out of the brain stem to supraorbital fissure, was clearly visible in parasellar meningioma cases. Meanwhile, cisternal parts of trigeminal nerve and abducens nerve, facial nerve were also imaged well in vestibular schwannomas and petroclival meningioma cases. The 3D-spatial relationship between CNs and skull base tumor estimated preoperatively by tumor modeling and tractography corresponded to the results determined during surgery. Supported by DTI and 3D slicer, preoperative 3D reconstruction of most CNs related to skull base tumor is feasible in pathological circumstances. We consider DTI Technology to be a useful tool for predicting the course and location of most CNs, and syntopy between them and skull base tumor.

Intraoperative Magnetic Resonance Imaging in Skull Base Surgery: A Review of 71 Consecutive Cases.

Science.gov (United States)

Ashour, Ramsey; Reintjes, Stephen; Park, Michael S; Sivakanthan, Sananthan; van Loveren, Harry; Agazzi, Siviero

2016-09-01

Although intraoperative magnetic resonance imaging (iMRI) increasingly is used during glioma resection, its role in skull base surgery has not been well documented. In this study, we evaluate our experience with iMRI for skull base surgery. Medical records were reviewed retrospectively on all neurosurgical cases performed at our institution in the IMRIS iMRI suite between April 2014 and July 2015. During the study period, the iMRI suite was used for 71 skull base tumors. iMRI was performed in 23 of 71 cases. Additional tumor resection was pursued after scanning in 7 of 23 patients. There was a significant difference in procedure length between the scanned versus nonscanned groups, and this was likely attributable to a greater proportion of petroclival meningiomas in the scanned group. Further analyses revealed significant increases in procedure length for the following scanned subgroups: anterolateral approach, anterolateral and petroclival lesion locations, and meningiomas. The rate of non-neurologic complications was significantly greater in the scanned group, particularly for patients with tumors >3 cm. Despite the unique challenges associated with skull base tumor surgery, iMRI can be safely obtained while adding a modest although not prohibitive amount of time to the procedure. The immediate evidence of residual tumor with a patient still in position to have additional resection may influence the surgeon to alter the surgical plan and attempt further resection in a critical area. Copyright © 2016 Elsevier Inc. All rights reserved.

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.

Transcranial passive acoustic mapping with hemispherical sparse arrays using CT-based skull-specific aberration corrections: a simulation study

International Nuclear Information System (INIS)

Jones, Ryan M; O’Reilly, Meaghan A; Hynynen, Kullervo

2013-01-01

The feasibility of transcranial passive acoustic mapping with hemispherical sparse arrays (30 cm diameter, 16 to 1372 elements, 2.48 mm receiver diameter) using CT-based aberration corrections was investigated via numerical simulations. A multi-layered ray acoustic transcranial ultrasound propagation model based on CT-derived skull morphology was developed. By incorporating skull-specific aberration corrections into a conventional passive beamforming algorithm (Norton and Won 2000 IEEE Trans. Geosci. Remote Sens. 38 1337–43), simulated acoustic source fields representing the emissions from acoustically-stimulated microbubbles were spatially mapped through three digitized human skulls, with the transskull reconstructions closely matching the water-path control images. Image quality was quantified based on main lobe beamwidths, peak sidelobe ratio, and image signal-to-noise ratio. The effects on the resulting image quality of the source’s emission frequency and location within the skull cavity, the array sparsity and element configuration, the receiver element sensitivity, and the specific skull morphology were all investigated. The system’s resolution capabilities were also estimated for various degrees of array sparsity. Passive imaging of acoustic sources through an intact skull was shown possible with sparse hemispherical imaging arrays. This technique may be useful for the monitoring and control of transcranial focused ultrasound (FUS) treatments, particularly non-thermal, cavitation-mediated applications such as FUS-induced blood–brain barrier disruption or sonothrombolysis, for which no real-time monitoring techniques currently exist. (paper)

Transcranial passive acoustic mapping with hemispherical sparse arrays using CT-based skull-specific aberration corrections: a simulation study

Science.gov (United States)

Jones, Ryan M.; O’Reilly, Meaghan A.; Hynynen, Kullervo

2013-01-01

The feasibility of transcranial passive acoustic mapping with hemispherical sparse arrays (30 cm diameter, 16 to 1372 elements, 2.48 mm receiver diameter) using CT-based aberration corrections was investigated via numerical simulations. A multi-layered ray acoustic transcranial ultrasound propagation model based on CT-derived skull morphology was developed. By incorporating skull-specific aberration corrections into a conventional passive beamforming algorithm (Norton and Won 2000 IEEE Trans. Geosci. Remote Sens. 38 1337–43), simulated acoustic source fields representing the emissions from acoustically-stimulated microbubbles were spatially mapped through three digitized human skulls, with the transskull reconstructions closely matching the water-path control images. Image quality was quantified based on main lobe beamwidths, peak sidelobe ratio, and image signal-to-noise ratio. The effects on the resulting image quality of the source’s emission frequency and location within the skull cavity, the array sparsity and element configuration, the receiver element sensitivity, and the specific skull morphology were all investigated. The system’s resolution capabilities were also estimated for various degrees of array sparsity. Passive imaging of acoustic sources through an intact skull was shown possible with sparse hemispherical imaging arrays. This technique may be useful for the monitoring and control of transcranial focused ultrasound (FUS) treatments, particularly non-thermal, cavitation-mediated applications such as FUS-induced blood-brain barrier disruption or sonothrombolysis, for which no real-time monitoring technique currently exists. PMID:23807573

Health-related quality of life in patients with skull base tumours.

LENUS (Irish Health Repository)

Kelleher, M O

2012-02-03

The objective of the investigation was to report on the health-related quality of life (HRQoL) of patients diagnosed with skull base tumours using the Short Form Health Survey questionnaire (SF-36). Those patients suffering with vestibular schwannoma were examined to determine the effect facial nerve function had on their quality of life. It took place at the tertiary referral centre at the Department of Clinical Neurosciences, Western General Hospital, Edinburgh. A prospective study of 70 consecutive patients was taken, who harboured the following tumours: 54 vestibular schwannomas, 13 meningiomas, two haemangioblastomas and one hypoglossal schwannoma. Patients were interviewed using the short form 36 (SF-36) questionnaire. Facial nerve function was assessed in those patients who had vestibular schwannomas. The entire cohort of live skull base patients were assessed after a median follow-up time of 38.4 months. Patients with vestibular schwannoma treated conservatively with interval MRI had a quality of life similar to t he normal population. Those who underwent surgery had a significant difference in two of the SF-36 domains. No statistically significant correlation was found at final assessment between the degree of facial nerve functioning and any of the domains of SF-36. Patients with non-vestibular tumours had an impaired HRQoL in seven of the eight domains. Patients with skull base tumours have a significant impairment of their HRQoL. A conservative policy of follow up with interval MRI for patients with small vestibular schwannomas may therefore be more appropriate to preserve their HRQoL. Facial nerve outcome has little influence on quality of life in vestibular schwannoma patients.

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

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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.

Osteoradionecrosis of the skull base after radiotherapy for nasopharynx cancer

International Nuclear Information System (INIS)

Mnejja, W.; Siala, W.; Daoud, J.; Boudawara, T.; Ghorbel, A.; Frikha, M.

2009-01-01

The purpose of this work was to study the incidence and the risk factors of osteoradionecrosis occurrence at the skull base after radiotherapy for nasopharynx cancer. It is often asymptomatic. Its incidence is not low. The systematic realisation of radiological examinations during the surveillance allows to detect the asymptomatic forms. No factor of risk was identified in the study. (N.C.)

Juvenile psammomatoid ossifying fibroma in paranasal sinus and skull base.

Science.gov (United States)

Wang, Mingjie; Zhou, Bing; Cui, Shunjiu; Li, Yunchuan

2017-07-01

The endoscopic transnasal approach with IGS is a safe and effective technique, allowing completely resection of JPOF, with minimal morbidity and recurrence. JPOF is a benign but locally aggressive fibro-osseous lesion. This study presents a series of JPOF cases, involving anterior skull base and orbit, treated by endoscopic transnasal approach with image guidance system (IGS) to resect the mass completely. This study retrospectively reviewed the clinical presentations, surgical procedures, and complications of 11 patients with JPOF who were treated by endoscopic approach from May 2009 to April 2014. All patients were followed by endoscopic and CT scan evaluations during follow-up. All of the 11 cases were boys, with a mean age of 11.8 years (range = 6-17 years). The size of mass in the paranasal sinus ranged from 2.5-4.6 cm in greatest dimension (mean = 3.7 cm), and the medial orbital wall and cranial base were involved in all patients. All 11 patients received successful operation and were relieved from symptoms without mortality and major complications. During follow-up (range from 17-67 months; mean follow-up = 25.8 months), only one patient was recurrent in local position. The skull base partial resected during surgery was found to rebuild after 1 year.

Earliest directly-dated human skull-cups.

Directory of Open Access Journals (Sweden)

Silvia M Bello

Full Text Available BACKGROUND: The use of human braincases as drinking cups and containers has extensive historic and ethnographic documentation, but archaeological examples are extremely rare. In the Upper Palaeolithic of western Europe, cut-marked and broken human bones are widespread in the Magdalenian (∼15 to 12,000 years BP and skull-cup preparation is an element of this tradition. PRINCIPAL FINDINGS: Here we describe the post-mortem processing of human heads at the Upper Palaeolithic site of Gough's Cave (Somerset, England and identify a range of modifications associated with the production of skull-cups. New analyses of human remains from Gough's Cave demonstrate the skilled post-mortem manipulation of human bodies. Results of the research suggest the processing of cadavers for the consumption of body tissues (bone marrow, accompanied by meticulous shaping of cranial vaults. The distribution of cut-marks and percussion features indicates that the skulls were scrupulously 'cleaned' of any soft tissues, and subsequently modified by controlled removal of the facial region and breakage of the cranial base along a sub-horizontal plane. The vaults were also 'retouched', possibly to make the broken edges more regular. This manipulation suggests the shaping of skulls to produce skull-cups. CONCLUSIONS: Three skull-cups have been identified amongst the human bones from Gough's Cave. New ultrafiltered radiocarbon determinations provide direct dates of about 14,700 cal BP, making these the oldest directly dated skull-cups and the only examples known from the British Isles.

Infant skull fracture (image)

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Skull fractures may occur with head injuries. Although the skull is both tough and resilient and provides excellent ... or blow can result in fracture of the skull and may be accompanied by injury to the ...

Quality criteria in diagnostic radiology of the skull

International Nuclear Information System (INIS)

Friedmann, G.

1985-01-01

Diagnostic survey radiology of the skull relies on pictures to be taken if indicated and to meet all conceivable requirements. Those radiograph directions and projections were selected out of the profusion of known and described ones which allow both as small a number of pictures and as comprehensive a demonstration of all skull sections and1structures as possible. With this in mind, quality criteria for plain radiographs of the skull taken laterally and sagittably, for partial radiographs of the visceral cranium including orbit and of the base of the skull including petrons bone are described. (orig./MG) [de

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

Energy Technology Data Exchange (ETDEWEB)

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

1998-10-01

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

Modeling skull's acoustic attenuation and dispersion on photoacoustic signal

Science.gov (United States)

Mohammadi, L.; Behnam, H.; Nasiriavanaki, M. R.

2017-03-01

Despite the great promising results of a recent new transcranial photoacoustic brain imaging technology, it has been shown that the presence of the skull severely affects the performance of this imaging modality. In this paper, we investigate the effect of skull on generated photoacoustic signals with a mathematical model. The developed model takes into account the frequency dependence attenuation and acoustic dispersion effects occur with the wave reflection and refraction at the skull surface. Numerical simulations based on the developed model are performed for calculating the propagation of photoacoustic waves through the skull. From the simulation results, it was found that the skull-induced distortion becomes very important and the reconstructed image would be strongly distorted without correcting these effects. In this regard, it is anticipated that an accurate quantification and modeling of the skull transmission effects would ultimately allow for skull aberration correction in transcranial photoacoustic brain imaging.

Clinical role of the skull base with increased uptake of 99mTc-MDP on SPECT/CT fused imaging with patients of nasopharyngeal

International Nuclear Information System (INIS)

Yao Hongxia; Zhang Jinshan; Liu Sheng

2009-01-01

Objective: To investigate the clinical role of the fused skull single photon emission computed tomographic and computed tomographic images (SPECT/CT) when there was 'hot' in the skull base of patients with NPC. Methods: 99 mTc-MDP SPECT/ CT and MRI were performed in a week in 44 patients (30 with first-visited cases and 14 with return-visited, 38 cases of poorly differentiated squamous cell carcinoma and 6 cases of undifferentiated cancer, 14 with headache). Region of interests (ROI) were drawn on the area of the suspected skull base and the upper cervical vertebral body on the same slice. A lesion-to-spine (L/S) ratio was interpreted on SPECT/CT as normal, benign, or malignant. L/S>1 indicated malignant skull base bone involvement (SBBI). Ten patients were studied as controls. Results: (1)Of the 44 study patients, 24 had SBBI (55%) based on SPECT/CT detecting skull base bone lesions with L/S =1.83±0.69. Twenty patients had normal or benign bone lesions on SPECT/CT with L/S =0.68±0.13. There was statistic significance compared SBBI with no SBBI subgroups (P 0.05). The numbers of SBBI had no relationship with positive rate of SPECT/CT (P >0.05). (3)There was no obviously increased uptake in the skull base in the 10 control patients (L/S<1). Conclusion: The skull SPECT/CT was recommended as one of clinical diagnosis tool for SBBI from NPC. Patients with headache should be highly suspected whether tumor cells involved the skull base bone or not. Further accumulation of other clinical factors would clarify the values of SPECT/CT. (authors)

Fibromyxoma of the Lateral Skull Base in a Child: Case Report

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Klimo, Paul; Jha, Tushar; Choudhri, Asim F.; Joyner, Royce; Michael, L. Madison

2013-01-01

Purpose Fibromyxomas and myxomas are benign tumors of mesenchymal origin usually found outside the nervous system, most commonly in the atrium of the heart. They can also arise in the mandible or maxilla, but it is exceedingly rare to find them within the skull base. The history, histologic features, and the literature, with emphasis on other pediatric cases, are reviewed for this uncommon skull base neoplasm. Methods We describe the case of a 13-year-old girl who presented with a 1-year history of facial weakness, numbness, and hearing loss. A large locally destructive tumor centered in the petrous bone was found on magnetic resonance imaging. Results A mastoidectomy combined with a middle fossa craniotomy was performed for gross total resection. The child is disease free 12 months after surgery. Conclusion Diagnosis could not be made solely on radiographic studies because of the lack of pathognomonic imaging features. Radical resection provided the patient the best chance of cure. Long-term surveillance is necessary to monitor for tumor recurrence. PMID:24303345

Analysis of skull asymmetry in different historical periods using radiological examinations

International Nuclear Information System (INIS)

Gawlikowska, A.; Czerwinski, F.; Dzieciolowska, E.; Miklaszewska, D.; Adamiec, E.; Szczurowski, J.

2007-01-01

Asymmetry is a very common phenomenon in nature. Occurrence of asymmetry and knowledge of correct structure, especially a range of variability which is not a pathology but only an individual variation, are the basis for interpretation of results of radiological examination of the skulls both in research work and in diagnostic examinations, which are widely performed in modern medicine. There are many methods of estimation of the asymmetry. The aim of this study was to estimate the symmetry of skulls from selected historic populations. The studied material consisted of two skull populations - contemporary consisting of 82 skulls and medieval - 77 skulls from Grodek. X-rays in P-A and skull-base projections were performed. The images were scanned and calibrated by means of MicroStation 95 Academic Edition software. Using tools for measurement of vector elements, distances between selected bilateral points of the skull were taken. All data were analyzed statistically. Asymmetry was observed in the skulls of both populations. Some diameters were higher on the left side, some on the right side. High levels of asymmetry index in the superior facial part and in the posterior part of the skull base were observed. The levels of the asymmetry indexes in both groups were similar. Radiological pictures in two projections should be taken for correct analysis of the skull asymmetry. The examination of the asymmetry of the landmarks should be based on the analysis of diameters from two different points of reference. The human skull does not demonstrate a clear domination of one side. The largest variations were observed in the shape and localization of the foramina of the skull . It is associated with the differences of the position of the neurovascular elements which pass through these foramina. (author)

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.)

Normal age-related conversion of bone marrow in the skull base. Assessment with MR imaging

International Nuclear Information System (INIS)

Kato, Koki; Tomura, Noriaki; Takahashi, Satoshi; Izumi, Junichi; Kurosawa, Ryo; Sashi, Ryuji; Watarai, Jiro

2000-01-01

The purpose of this study was to assess the normal age-related sequence of conversion from hematopoietic to fatty marrow in the skull base by means of MR imaging. We retrospectively reviewed T1-weighted MR images of the skull base for the distribution of hematopoietic and fatty marrow. The subjects consisted of 169 MR examinations that were performed with the spin-echo technique. The age of the subjects ranged from 0 months to 20 years old. Patients with known marrow abnormalities were excluded from this study. Marrow conversion was assessed in the presphenoid, postsphenoid, basiocciput, petrous apex, clivus, zygomatic bone, and condyle of the mandible. The signal intensity was visually graded, and the signal intensity ratio was determined on the basis of the intensities of the subcutaneous fat and air. The signal intensity of all observed regions was as low as that of muscles until 3 months of age. Conversion of hematopoietic to fatty marrow first occurred in the zygomatic bone until 6 months of age. The presphenoid increased in signal intensity from 5 months to 2 years of age, and the sphenoid sinus began to be pneumatic at this age. Marrow conversion of the postsphenoid and basiocciput was later than that of the presphenoid. Most of the bone marrow of the skull base appeared as fatty conversion until 3 years of age, although some mandibular condyles appeared hematopoietic at 3 years of age. The normal age-related conversion from hematopoietic to fatty marrow in the skull base followed a well-defined sequence. Knowledge of the normal bone marrow conversion by MR imaging is essential for the recognition of pathologic marrow processes. (author)

Proton and carbon ion radiotherapy for primary brain tumors and tumors of the skull base

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Combs, Stephanie E.; Kessel, Kerstin; Habermehl, Daniel; Debus, Jurgen [Univ. Hospital of Heidelberg, Dept. of Radiation Oncology, Heidelberg (Germany)], e-mail: Stephanie.Combs@med.uni-heidelberg.de; Haberer, Thomas [Heidelberger Ionenstrahl Therapiezentrum (HIT), Heidelberg (Germany); Jaekel, Oliver [Univ. Hospital of Heidelberg, Dept. of Radiation Oncology, Heidelberg (Germany); Heidelberger Ionenstrahl Therapiezentrum (HIT), Heidelberg (Germany)

2013-10-15

To analyze clinical concepts, toxicity and treatment outcome in patients with brain and skull base tumors treated with photons and particle therapy. Material and methods: In total 260 patients with brain tumors and tumors of the skull base were treated at the Heidelberg Ion Therapy Center (HIT). Patients enrolled in and randomized within prospective clinical trials as well as bony or soft tissue tumors are not included in this analysis. Treatment was delivered as protons, carbon ions, or combinations of photons and a carbon ion boost. All patients are included in a tight follow-up program. The median follow-up time is 12 months (range 2-39 months). Results: Main histologies included meningioma (n = 107) for skull base lesions, pituitary adenomas (n = 14), low-grade gliomas (n = 51) as well as high-grade gliomas (n = 55) for brain tumors. In all patients treatment could be completed without any unexpected severe toxicities. No side effects > CTC Grade III were observed. To date, no severe late toxicities were observed, however, for endpoints such as secondary malignancies or neuro cognitive side effects follow-up time still remains too short. Local recurrences were mainly seen in the group of high-grade gliomas or atypical meningiomas; for benign skull base meningiomas, to date, no recurrences were observed during follow-up. Conclusion: The specific benefit of particle therapy will potentially reduce the risk of secondary malignancies as well as improve neuro cognitive outcome and quality of life (QOL); thus, longer follow-up will be necessary to confirm these endpoints. Indication-specific trials on meningiomas and gliomas are underway to elucidate the role of protons and carbon ions in these indications.

Proton and carbon ion radiotherapy for primary brain tumors and tumors of the skull base

International Nuclear Information System (INIS)

Combs, Stephanie E.; Kessel, Kerstin; Habermehl, Daniel; Debus, Jurgen; Haberer, Thomas; Jaekel, Oliver

2013-01-01

To analyze clinical concepts, toxicity and treatment outcome in patients with brain and skull base tumors treated with photons and particle therapy. Material and methods: In total 260 patients with brain tumors and tumors of the skull base were treated at the Heidelberg Ion Therapy Center (HIT). Patients enrolled in and randomized within prospective clinical trials as well as bony or soft tissue tumors are not included in this analysis. Treatment was delivered as protons, carbon ions, or combinations of photons and a carbon ion boost. All patients are included in a tight follow-up program. The median follow-up time is 12 months (range 2-39 months). Results: Main histologies included meningioma (n = 107) for skull base lesions, pituitary adenomas (n = 14), low-grade gliomas (n = 51) as well as high-grade gliomas (n = 55) for brain tumors. In all patients treatment could be completed without any unexpected severe toxicities. No side effects > CTC Grade III were observed. To date, no severe late toxicities were observed, however, for endpoints such as secondary malignancies or neuro cognitive side effects follow-up time still remains too short. Local recurrences were mainly seen in the group of high-grade gliomas or atypical meningiomas; for benign skull base meningiomas, to date, no recurrences were observed during follow-up. Conclusion: The specific benefit of particle therapy will potentially reduce the risk of secondary malignancies as well as improve neuro cognitive outcome and quality of life (QOL); thus, longer follow-up will be necessary to confirm these endpoints. Indication-specific trials on meningiomas and gliomas are underway to elucidate the role of protons and carbon ions in these indications

Case of pycnodysostosis. Observation of skull by CT scan

Energy Technology Data Exchange (ETDEWEB)

Anegawa, Shigetaka; Bekki, Yoshiaki; Furukawa, Yasuhiro; Yokota, Seishi; Torigoe, Ryuichiro

1987-07-01

A 13-year-old boy was presented to the Department of Neurosurgery, Saiseikai Fukuoka General Hospital for further examinations concerning abnormal findings in the skull radiogram taken when he struck his head. His physical features showed some characteristics the same as those of pycnodysostosis as follows - proportionate dwarfism, prominent forehead, short spoon-shaped fingers, bilateral exophthalmos. A skull radiogram revealed widely open cranial sutures with no healing of the fracture and craniotomy which was performed for an acute epidural hematoma 6 years ago. Furthermore, the mandible was hypoplastic with a virtural loss of mandibular angle. CT of the soft tissues showed somewhat dilated cortical sulci and ventricles without any structural abnormalities in the brain. CT of bone algorythum revealed specific characteristics of this disease. The paranasal sinuses were quite hypoplastic. Especially in the maxillary sinuses, frontal sinussus and mastoid air cells, none of developments of sinuses were noted, even though the middle and internal ear seemed to be normal. Moreover, the ethomoid and sphenoid sinuses were noted, although their developments were poor. The appearance of skull base was normal, including the inlets and outlets of cranial nerves or vessels and synchondroses. However, the density of the skull base, especially in the diploe, was higher than normal in Hansfield number. Furthermore, detailed measurements of skull base demonstrated that the skull base itself was also dwarfish. In our study, the development of sinuses in bones with intramembranous ossification are worse than that with endochondral ossification. Furthermore, sutures or synchondroses in the skull base were well-developed than those of the convex. So, it is considered that pycnodysostosis must be the neighboring entity of diseases such as achondroplastic dwarfism or cleidocranial dysplasia. (J.P.N.).

Quality Assurance of Multiport Image-Guided Minimally Invasive Surgery at the Lateral Skull Base

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Maria Nau-Hermes

2014-01-01

Full Text Available For multiport image-guided minimally invasive surgery at the lateral skull base a quality management is necessary to avoid the damage of closely spaced critical neurovascular structures. So far there is no standardized method applicable independently from the surgery. Therefore, we adapt a quality management method, the quality gates (QG, which is well established in, for example, the automotive industry and apply it to multiport image-guided minimally invasive surgery. QG divide a process into different sections. Passing between sections can only be achieved if previously defined requirements are fulfilled which secures the process chain. An interdisciplinary team of otosurgeons, computer scientists, and engineers has worked together to define the quality gates and the corresponding criteria that need to be fulfilled before passing each quality gate. In order to evaluate the defined QG and their criteria, the new surgery method was applied with a first prototype at a human skull cadaver model. We show that the QG method can ensure a safe multiport minimally invasive surgical process at the lateral skull base. Therewith, we present an approach towards the standardization of quality assurance of surgical processes.

Quality assurance of multiport image-guided minimally invasive surgery at the lateral skull base.

Science.gov (United States)

Nau-Hermes, Maria; Schmitt, Robert; Becker, Meike; El-Hakimi, Wissam; Hansen, Stefan; Klenzner, Thomas; Schipper, Jörg

2014-01-01

For multiport image-guided minimally invasive surgery at the lateral skull base a quality management is necessary to avoid the damage of closely spaced critical neurovascular structures. So far there is no standardized method applicable independently from the surgery. Therefore, we adapt a quality management method, the quality gates (QG), which is well established in, for example, the automotive industry and apply it to multiport image-guided minimally invasive surgery. QG divide a process into different sections. Passing between sections can only be achieved if previously defined requirements are fulfilled which secures the process chain. An interdisciplinary team of otosurgeons, computer scientists, and engineers has worked together to define the quality gates and the corresponding criteria that need to be fulfilled before passing each quality gate. In order to evaluate the defined QG and their criteria, the new surgery method was applied with a first prototype at a human skull cadaver model. We show that the QG method can ensure a safe multiport minimally invasive surgical process at the lateral skull base. Therewith, we present an approach towards the standardization of quality assurance of surgical processes.

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...

Early harvesting of the vascularized pedicled nasoseptal flap during endoscopic skull base surgery.

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Eloy, Jean Anderson; Patel, Amit A; Shukla, Pratik A; Choudhry, Osamah J; Liu, James K

2013-01-01

The vascularized pedicled nasoseptal flap (PNSF) represents a successful option for reconstruction of large skull base defects after expanded endoscopic endonasal approaches (EEA). This vascularized flap can be harvested early or late in the operation depending on the anticipation of high-flow CSF leaks. Each harvesting technique (early vs. late) is associated with different advantages and disadvantages. In this study, we evaluate our experience with early harvesting of the PNSF for repair of large skull base defects after EEA. A retrospective review was performed at a tertiary care medical center on patients who underwent early PNSF harvesting during reconstruction of intraoperative high-flow CSF leaks after EEA between December 2008 and March 2012. Demographic data, repair materials, surgical approach, and incidence of PNSF usage were collected. Eighty-seven patients meeting the inclusion criteria were identified. In 86 procedures (98.9%), the PNSF harvested at the beginning of the operation was used. In 1 case (1.1%), the PNSF was not used because a high-flow intraoperative CSF leak was not encountered. This patient had recurrence of intradural disease 8months later, and the previously elevated PNSF was subsequent used after tumor resection. Based on our data, a high-flow CSF leak and need for a PNSF can be accurately anticipated in patients undergoing EEA for skull base lesions. Because of the advantages of early harvesting of the PNSF and the high preoperative predictive value of CSF leak anticipations, this technique represents a feasible harvesting practice for EEA surgeries. Copyright © 2013 Elsevier Inc. All rights reserved.

Stereotactic radiotherapy using Novalis for skull base metastases developing with cranial nerve symptoms.

Science.gov (United States)

Mori, Yoshimasa; Hashizume, Chisa; Kobayashi, Tatsuya; Shibamoto, Yuta; Kosaki, Katsura; Nagai, Aiko

2010-06-01

Skull base metastases are challenging situations because they often involve critical structures such as cranial nerves. We evaluated the role of stereotactic radiotherapy (SRT) which can give high doses to the tumors sparing normal structures. We treated 11 cases of skull base metastases from other visceral carcinomas. They had neurological symptoms due to cranial nerve involvement including optic nerve (3 patients), oculomotor (3), trigeminal (6), abducens (1), facial (4), acoustic (1), and lower cranial nerves (1). The interval between the onset of cranial nerve symptoms and Novalis SRT was 1 week to 7 months. Eleven tumors of 8-112 ml in volume were treated by Novalis SRT with 30-50 Gy in 10-14 fractions. The tumors were covered by 90-95% isodose. Imaging and clinical follow-up has been obtained in all 11 patients for 5-36 months after SRT. Seven patients among 11 died from primary carcinoma or other visceral metastases 9-36 months after Novalis SRT. All 11 metastatic tumors were locally controlled until the end of the follow-up time or patient death, though retreatment for re-growth was done in 1 patient. In 10 of 11 patients, cranial nerve deficits were improved completely or partially. In some patients, the cranial nerve symptoms were relieved even during the period of fractionated SRT. Novalis SRT is thought to be safe and effective treatment for skull base metastases with involvement of cranial nerves and it may improve cranial nerve symptoms quickly.

Computerized tomographic diagnosis of basal skull fracture

International Nuclear Information System (INIS)

Tanaka, Tokutaro; Shimoyama, Ichiro; Endoh, Mitsutoshi; Ninchoji, Toshiaki; Uemura, Kenichi.

1984-01-01

The diagnosis of basal skull fractures used to be difficult, particularly on the basis of routine skull roentgenography alone. We have now examined the diagnostic value of conventional computerized tomography in basal skull fractures. We studied 82 cases clinically diagnosed as basal skull fractures. We examined them based on at least one of the following computerized tomographic criteria for basal skull fractures: 1) fracture line(s), 2) intracranial air, 3) fluid in the paranasal sinuses, and 4) fluid in the middle ear, including the mastoid air cells. The signs of the fracture line and of the intracranial air are definite indications of basal skull fracture, but the signs of fluid in the paranasal sinuses and/or in the middle ear are not definite. When combined, however, with such other clinical signs as black eye, Battle's sign, CSF leakage, CSF findings, and profuse nasal or ear bleeding, the diagnosis is more reliable. Seventy cases (85.4%) in this series had basal skull fractures according to our computerized tomographic criteria. Among them , 26 cases (31.7%) were diagnosed with fracture lines, 17 cases (20.7%) with intracranial air, 16 cases (19.5%) with fluid in the paranasal sinuses, 10 cases (12.2%) with fluid in the middle ear, and one case (1.2%) with fluid in both. Twelve cases (14.6%) of the 82 cases clinically diagnosed as basal skull fractures could not have been diagnosed on our computerized tomographic criteria alone. We diagnosed them because of CSF leakage, CSF findings, surgical findings, etc. (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.

Endoscopic Endonasal Anterior Skull Base Surgery: A Systematic Review of Complications During the Past 65 Years.

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Borg, Anouk; Kirkman, Matthew A; Choi, David

2016-11-01

Endoscopic skull base surgery is becoming more popular as an approach to the anterior skull base for tumors and cerebrospinal fluid (CSF) fistulae. It offers the advantages of better cosmesis and improved quality of life after surgery. We reviewed the complication rates reported in the literature. A literature search was performed in the electronic database Ovid MEDLINE (1950 to August 25, 2015) with the search item "([Anterior] AND Skull base surgery) AND endoscopic." We identified 82 relevant studies that included 7460 cases. An average overall complication rate of 17.1% (range 0%-68.0%) and a mortality rate of 0.4% (0%-10.0%) were demonstrated in a total of 82 studies that included 7460 cases. The average CSF leak rate for all studies was 8.9% (0%-40.0%) with meningiomas and clival lesions having the greatest CSF leak rates. The most frequent benign pathology encountered was pituitary adenomas (n = 3720, 49.8% of all cases) and the most frequent malignant tumor was esthesioneuroblastoma (n = 120, 1.6% of all cases). Studies that included only CSF fistula repairs had a lower average total complication rate (12.9%) but a greater rate of meningitis compared with studies that reported mixed pathology (2.4% vs. 1.3%). A trend towards a lower total complication rate with increasing study size was observed. The endoscopic approach is an increasingly accepted technique for anterior skull base tumor surgery and is associated with acceptable complication rates. Increasing experience with this technique can decrease rates of complications. Copyright © 2016 Elsevier Inc. All rights reserved.

Endoscopic Resection of Skull Base Teratoma in Klippel-Feil Syndrome through Use of Combined Ultrasonic and Bipolar Diathermy Platforms

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Justin A. Edward

2017-01-01

Full Text Available Klippel-Feil syndrome (KFS is associated with numerous craniofacial abnormalities but rarely with skull base tumor formation. We report an unusual and dramatic case of a symptomatic, mature skull base teratoma in an adult patient with KFS, with extension through the basisphenoid to obstruct the nasopharynx. This benign lesion was associated with midline palatal and cerebral defects, most notably pituitary and vertebrobasilar arteriolar duplications. A multidisciplinary workup and a complete endoscopic, transnasal surgical approach between otolaryngology and neurosurgery were undertaken. Out of concern for vascular control of the fibrofatty dense tumor stalk at the skull base and need for complete teratoma resection, we successfully employed a tissue resection tool with combined ultrasonic and bipolar diathermy to the tumor pedicle at the sphenoid/clivus junction. No CSF leak or major hemorrhage was noted using this endonasal approach, and no concerning postoperative sequelae were encountered. The patient continues to do well now 3 years after tumor extirpation, with resolution of all preoperative symptoms and absence of teratoma recurrence. KFS, teratoma biology, endocrine gland duplication, and the complex considerations required for successfully addressing this type of advanced skull base pathology are all reviewed herein.

Sagittal synostosis: I. Preoperative morphology of the skull

DEFF Research Database (Denmark)

Guimaraes-Ferreira, J.; Gewalli, F.; David, L.

2006-01-01

The aim of this study was to characterise the preoperative morphology of the skull in sagittal synostosis in an objective and quantified way. The shapes of the skulls of 105 patients with isolated premature synostosis of the sagittal suture ( SS group) were studied and compared with those......, skull base, and orbit ( 42 in the lateral and 46 in the frontal projections), the production of plots of mean shape for each group, and the intergroup comparison of a series of 81 variables ( linear distance between selected landmarks, and angles defined by groups of three landmarks). Data from...... skull width. Comparison of the mean values of an SS subgroup to age-matched normative data showed a longer (p differ significantly...

Visual outcome after fractionated stereotactic radiation therapy of benign anterior skull base tumors

DEFF Research Database (Denmark)

Astradsson, Arnar; Wiencke, Anne Katrine; Munck af Rosenschold, Per

2014-01-01

To determine visual outcome including the occurrence of radiation induced optic neuropathy (RION) as well as tumor control after fractionated stereotactic radiation therapy (FSRT) of benign anterior skull base meningiomas or pituitary adenomas. Thirty-nine patients treated with FSRT for anterior...

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)

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

Energy Technology Data Exchange (ETDEWEB)

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

2013-03-15

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

Skull x-ray

Science.gov (United States)

X-ray - head; X-ray - skull; Skull radiography; Head x-ray ... There is low radiation exposure. X-rays are monitored and regulated to provide the minimum amount of radiation exposure needed to produce the image. Most ...

Skull anatomy (image)

Science.gov (United States)

The skull is anterior to the spinal column and is the bony structure that encases the brain. Its purpose ... the facial muscles. The two regions of the skull are the cranial and facial region. The cranial ...

Skull Base Langerhans Cell Histiocytosis with Diabetes Insipidus and Panhypopituitarism- A Rare Clinical Entity

Directory of Open Access Journals (Sweden)

Anirban Ghosh

2017-12-01

Case Report A 16 year old male presented with diminished vision, bilateral ptosis, left sided lateral rectus palsy, hypoesthesia of trigeminal nerve with nasal obstruction for last 5 months. There was polypoidal, bleeding mass in both nasal cavities. Contrast enhanced CT Scan showed a large homogenous mass arising from sphenoid extending into cavernous sinus and the suprasellar region. Endoscopic nasal biopsy revealed abundant Langerhans cell histiocytes, macrophages, neutrophils. Chemotherapy and radiotherapy were administered. But within 2 months the patient presented with Cushingoid features and further diminution of vision. Detailed work-up revealed Hypogonadotrophic hypogonadism and diabetes insipidus. Debulking of the tumour was done and left optic nerve decompression was done. PET scan was performed and showed large, well defined mass with increased FDG uptake in the skull base with suprasellar extension, reaching upto petrous temporal bone and causing bony erosion of ethmoid and sphenoid sinuses. Patient was then advised adjuvant chemotherapy.   Discussion Langerhans cell histiocytosis is a rare group of disorders characterised by abnormal clonal proliferation and accumulation of abnormal dendritic cells. Involvement of base of skull is even rarer. Though diabetes insipidus has been reported in Langerhans cell histiocytosis involving pituitary, panhypopituitarism is rare. These combinations of extensive Langerhans cell histiocytosis of base skull with clinical features of Diabetes insipidus and panhypopituitarism makes this case a rare clinical entity.

Radio-anatomical analysis of the pericranial flap "money box approach" for ventral skull base reconstruction.

Science.gov (United States)

Santamaría, Alfonso; Langdon, Cristóbal; López-Chacon, Mauricio; Cordero, Arturo; Enseñat, Joaquim; Carrau, Ricardo; Bernal-Sprekelsen, Manuel; Alobid, Isam

2017-11-01

To evaluate the versatility of the pericranial flap (PCF) to reconstruct the ventral skull base, using the frontal sinus as a gate for its passage into the sinonasal corridor "money box approach." Anatomic-radiological study and case series. Various approaches and their respective defects (cribriform, transtuberculum, clival, and craniovertebral junction) were completed in 10 injected specimens. The PCF was introduced into the nose through the uppermost portion of the frontal sinus (money box approach). Computed tomography (CT) scans (n = 50) were used to measure the dimensions of the PCF and the skull base defects. The vertical projection of the external ear canal was used as the reference point to standardize the incisions for the PCF. The surface area and maximum length of the PCF were 121.5 ± 19.4 cm 2 and 18.3 ± 1.3 cm, respectively. Using CT scans, we determined that to reconstruct defects secondary to transcribriform, transtuberculum, clival, and craniovertebral approaches, the PCF distal incision must be placed respectively at -3.7 ± 2.0 cm (angle -17.4 ± 8.5°), -0.2 ± 2.0 cm (angle -1.0 ± 9.3°), +5.5 ± 2.3 cm (angle +24.4 ± 9.7°), +8.4 ± 2.4 cm (angle +36.6 ± 11.5°), as related to the reference point. Skull base defects in our clinical cohort (n = 6) were completely reconstructed uneventfully with the PCF. The PCF renders enough surface area to reconstruct all possible defects in the ventral and median skull base. Using the uppermost frontal sinus as a gateway into the nose (money box approach) is feasible and simple. NA. Laryngoscope, 127:2482-2489, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

Central skull base osteomyelitis as a complication of necrotizing otitis externa: Imaging findings, complications, and challenges of diagnosis

International Nuclear Information System (INIS)

Adams, A.; Offiah, C.

2012-01-01

Central skull base osteomyelitis is a rare, life-threatening complication of necrotizing or “malignant” otitis externa (NOE), which results in destruction of the skull base. The imaging appearances can be misinterpreted as malignancy but consideration of this diagnosis, both radiologically and clinically, is imperative to avoid the need for biopsy. The aim of this review is to highlight the pertinent imaging findings on computed tomography and magnetic resonance imaging as well as the potential complications of this condition.

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.

Skull's acoustic attenuation and dispersion modeling on photoacoustic signal

Science.gov (United States)

Mohammadi, Leila; Behnam, Hamid; Tavakkoli, Jahan; Nasiriavanaki, Mohammadreza

2018-02-01

Despite the promising results of the recent novel transcranial photoacoustic (PA) brain imaging technology, it has been demonstrated that the presence of the skull severely affects the performance of this imaging modality. We theoretically investigate the effects of acoustic heterogeneity induced by skull on the PA signals generated from single particles, with firstly developing a mathematical model for this phenomenon and then explore experimental validation of the results. The model takes into account the frequency dependent attenuation and dispersion effects occur with wave reflection, refraction and mode conversion at the skull surfaces. Numerical simulations based on the developed model are performed for calculating the propagation of photoacoustic waves through the skull. The results show a strong agreement between simulation and ex-vivo study. The findings are as follow: The thickness of the skull is the most PA signal deteriorating factor that affects both its amplitude (attenuation) and phase (distortion). Also we demonstrated that, when the depth of target region is low and it is comparable to the skull thickness, however, the skull-induced distortion becomes increasingly severe and the reconstructed image would be strongly distorted without correcting these effects. It is anticipated that an accurate quantification and modeling of the skull transmission effects would ultimately allow for aberration correction in transcranial PA brain imaging.

First Application of 7T Magnetic Resonance Imaging in Endoscopic Endonasal Surgery of Skull Base Tumors

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Barrett, Thomas F; Dyvorne, Hadrien A; Padormo, Francesco; Pawha, Puneet S; Delman, Bradley N; Shrivastava, Raj K; Balchandani, Priti

2018-01-01

Background Successful endoscopic endonasal surgery for the resection of skull base tumors is reliant on preoperative imaging to delineate pathology from the surrounding anatomy. The increased signal-to-noise ratio afforded by 7T MRI can be used to increase spatial and contrast resolution, which may lend itself to improved imaging of skull base. In this study, we apply a 7T imaging protocol to patients with skull base tumors and compare the images to clinical standard of care. Methods Images were acquired at 7T on 11 patients with skull base lesions. Two neuroradiologists evaluated clinical 1.5T, 3T, and 7T scans for detection of intracavernous cranial nerves and ICA branches. Detection rates were compared. Images were utilized for surgical planning and uploaded to a neuronavigation platform and used to guide surgery. Results Image analysis yielded improved detection rates of cranial nerves and ICA branches at 7T. 7T images were successfully incorporated into preoperative planning and intraoperative neuronavigation. Conclusion Our study represents the first application of 7T MRI to the full neurosurgical workflow for endoscopic endonasal surgery. We detected higher rates of cranial nerves and ICA branches at 7T MRI compared to 3T and 1.5 T, and found that integration of 7T into surgical planning and guidance was feasible. These results suggest a potential for 7T MRI to reduce surgical complications. Future studies comparing standardized 7T, 3T, and 1.5 T MRI protocols in a larger number of patients are warranted to determine the relative benefit of 7T MRI for endonasal endoscopic surgical efficacy. PMID:28359922

First Application of 7-T Magnetic Resonance Imaging in Endoscopic Endonasal Surgery of Skull Base Tumors.

Science.gov (United States)

Barrett, Thomas F; Dyvorne, Hadrien A; Padormo, Francesco; Pawha, Puneet S; Delman, Bradley N; Shrivastava, Raj K; Balchandani, Priti

2017-07-01

Successful endoscopic endonasal surgery for the resection of skull base tumors is reliant on preoperative imaging to delineate pathology from the surrounding anatomy. The increased signal-to-noise ratio afforded by 7-T MRI can be used to increase spatial and contrast resolution, which may lend itself to improved imaging of the skull base. In this study, we apply a 7-T imaging protocol to patients with skull base tumors and compare the images with clinical standard of care. Images were acquired at 7 T on 11 patients with skull base lesions. Two neuroradiologists evaluated clinical 1.5-, 3-, and 7-T scans for detection of intracavernous cranial nerves and internal carotid artery (ICA) branches. Detection rates were compared. Images were used for surgical planning and uploaded to a neuronavigation platform and used to guide surgery. Image analysis yielded improved detection rates of cranial nerves and ICA branches at 7 T. The 7-T images were successfully incorporated into preoperative planning and intraoperative neuronavigation. Our study represents the first application of 7-T MRI to the full neurosurgical workflow for endoscopic endonasal surgery. We detected higher rates of cranial nerves and ICA branches at 7-T MRI compared with 3- and 1.5-T MRI, and found that integration of 7 T into surgical planning and guidance was feasible. These results suggest a potential for 7-T MRI to reduce surgical complications. Future studies comparing standardized 7-, 3-, and 1.5-T MRI protocols in a larger number of patients are warranted to determine the relative benefit of 7-T MRI for endonasal endoscopic surgical efficacy. Copyright © 2017 Elsevier Inc. All rights reserved.

Virtual surgical planning in endoscopic skull base surgery.

Science.gov (United States)

Haerle, Stephan K; Daly, Michael J; Chan, Harley H L; Vescan, Allan; Kucharczyk, Walter; Irish, Jonathan C

2013-12-01

Skull base surgery (SBS) involves operative tasks in close proximity to critical structures in a complex three-dimensional (3D) anatomy. The aim was to investigate the value of virtual planning (VP) based on preoperative magnetic resonance imaging (MRI) for surgical planning in SBS and to compare the effects of virtual planning with 3D contours between the expert and the surgeon in training. Retrospective analysis. Twelve patients with manually segmented anatomical structures based on preoperative MRI were evaluated by eight surgeons in a randomized order using a validated National Aeronautics and Space Administration Task Load Index (NASA-TLX) questionnaire. Multivariate analysis revealed significant reduction of workload when using VP (PNASA-TLX differences (P.05). Preoperative anatomical segmentation with virtual surgical planning using contours in endoscopic SBS significantly reduces the workload for the expert and the surgeon in training. Copyright © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

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)

Usefulness of Choline-PET for the detection of residual hemangiopericytoma in the skull base: comparison with FDG-PET

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Ito Shin

2012-02-01

Full Text Available Abstract Background Choline is a new PET tracer that is useful for the detection of malignant tumor. Choline is a precursor of the biosynthesis of phosphatidylcholine, a major phospholipid in the cell membrane of eukaryotic cells. Malignant tumors have an elevated level of phosphatidylcholine in cell membrane. Thus, choline is a marker of tumor malignancy. Method The patient was a 51-year-old man with repeated recurrent hemangiopericytoma in the skull base. We performed Choline-PET in this patient after various treatments and compared findings with those of FDG-PET. Results Choline accumulated in this tumor, but FDG did not accumulate. We diagnosed this tumor as residual hemangiopericytoma and performed the resection of the residual tumor. FDG-PET is not appropriate for skull base tumor detection because uptake in the brain is very strong. Conclusion We emphasize the usefulness of Choline-PET for the detection of residual hemangiopericytoma in the skull base after various treatments, compared with FDG-PET.

Construction of a three-dimensional interactive model of the skull base and cranial nerves.

Science.gov (United States)

Kakizawa, Yukinari; Hongo, Kazuhiro; Rhoton, Albert L

2007-05-01

The goal was to develop an interactive three-dimensional (3-D) computerized anatomic model of the skull base for teaching microneurosurgical anatomy and for operative planning. The 3-D model was constructed using commercially available software (Maya 6.0 Unlimited; Alias Systems Corp., Delaware, MD), a personal computer, four cranial specimens, and six dry bones. Photographs from at least two angles of the superior and lateral views were imported to the 3-D software. Many photographs were needed to produce the model in anatomically complex areas. Careful dissection was needed to expose important structures in the two views. Landmarks, including foramen, bone, and dura mater, were used as reference points. The 3-D model of the skull base and related structures was constructed using more than 300,000 remodeled polygons. The model can be viewed from any angle. It can be rotated 360 degrees in any plane using any structure as the focal point of rotation. The model can be reduced or enlarged using the zoom function. Variable transparencies could be assigned to any structures so that the structures at any level can be seen. Anatomic labels can be attached to the structures in the 3-D model for educational purposes. This computer-generated 3-D model can be observed and studied repeatedly without the time limitations and stresses imposed by surgery. This model may offer the potential to create interactive surgical exercises useful in evaluating multiple surgical routes to specific target areas in the skull base.

[Congenital skull base defect causing recurrent bacterial meningitis].

Science.gov (United States)

Berliner, Elihay; Bar Meir, Maskit; Megged, Orli

2012-08-01

Bacterial meningitis is a life threatening disease. Most patients will experience only one episode throughout life. Children who experience bacterial meningitis more than once, require further immunologic or anatomic evaluation. We report a 9 year old child with five episodes of bacterial meningitis due to a congenital defect of the skull base. A two and a half year old boy first presented to our medical center with pneumococcal meningitis. He was treated with antibiotics and fully recovered. Two months later he presented again with a similar clinical picture. Streptococcus pneumoniae grew in cerebrospinal fluid (CSF) culture. CT scan and later MRI of the brain revealed a defect in the anterior middle fossa floor, with protrusion of brain tissue into the sphenoidal sinus. Corrective surgery was recommended but the parents refused. Three months later, a third episode of pneumococcal meningitis occurred. The child again recovered with antibiotics and this time corrective surgery was performed. Five years later, the boy presented once again with clinical signs and symptoms consistent with bacterial meningitis. CSF culture was positive, but the final identification of the bacteria was conducted by broad spectrum 16S ribosomal RNA PCR (16S rRNA PCR) which revealed a sequence of Neisseria lactamica. CT and MRI showed recurrence of the skull base defect with encephalocele in the sphenoid sinus. The parents again refused neurosurgical intervention. A year later the patient presented with bacterial meningitis. CSF culture obtained after initiation of antibiotics was negative, but actinobacillus was identified in the CSF by 16S rRNA PCR. The patient is scheduled for neurosurgical intervention. In patients with recurrent bacterial meningitis caused by organisms colonizing the oropharynx or nasopharynx, an anatomical defect should be carefully sought and surgically repaired.

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.)

Diseases of the skull

International Nuclear Information System (INIS)

Koval', G.Yu.

1984-01-01

Different forms of skull diseases viz. inflammatory diseases, skull tumors, primary and secondary bone tumors, are considered. Roentgenograms in some above-mentioned diseases are presented and analysed

Functional relationship between skull form and feeding mechanics in Sphenodon, and implications for diapsid skull development.

Directory of Open Access Journals (Sweden)

Neil Curtis

Full Text Available The vertebrate skull evolved to protect the brain and sense organs, but with the appearance of jaws and associated forces there was a remarkable structural diversification. This suggests that the evolution of skull form may be linked to these forces, but an important area of debate is whether bone in the skull is minimised with respect to these forces, or whether skulls are mechanically "over-designed" and constrained by phylogeny and development. Mechanical analysis of diapsid reptile skulls could shed light on this longstanding debate. Compared to those of mammals, the skulls of many extant and extinct diapsids comprise an open framework of fenestrae (window-like openings separated by bony struts (e.g., lizards, tuatara, dinosaurs and crocodiles, a cranial form thought to be strongly linked to feeding forces. We investigated this link by utilising the powerful engineering approach of multibody dynamics analysis to predict the physiological forces acting on the skull of the diapsid reptile Sphenodon. We then ran a series of structural finite element analyses to assess the correlation between bone strain and skull form. With comprehensive loading we found that the distribution of peak von Mises strains was particularly uniform throughout the skull, although specific regions were dominated by tensile strains while others were dominated by compressive strains. Our analyses suggest that the frame-like skulls of diapsid reptiles are probably optimally formed (mechanically ideal: sufficient strength with the minimal amount of bone with respect to functional forces; they are efficient in terms of having minimal bone volume, minimal weight, and also minimal energy demands in maintenance.

Functional Relationship between Skull Form and Feeding Mechanics in Sphenodon, and Implications for Diapsid Skull Development

Science.gov (United States)

Curtis, Neil; Jones, Marc E. H.; Shi, Junfen; O'Higgins, Paul; Evans, Susan E.; Fagan, Michael J.

2011-01-01

The vertebrate skull evolved to protect the brain and sense organs, but with the appearance of jaws and associated forces there was a remarkable structural diversification. This suggests that the evolution of skull form may be linked to these forces, but an important area of debate is whether bone in the skull is minimised with respect to these forces, or whether skulls are mechanically “over-designed” and constrained by phylogeny and development. Mechanical analysis of diapsid reptile skulls could shed light on this longstanding debate. Compared to those of mammals, the skulls of many extant and extinct diapsids comprise an open framework of fenestrae (window-like openings) separated by bony struts (e.g., lizards, tuatara, dinosaurs and crocodiles), a cranial form thought to be strongly linked to feeding forces. We investigated this link by utilising the powerful engineering approach of multibody dynamics analysis to predict the physiological forces acting on the skull of the diapsid reptile Sphenodon. We then ran a series of structural finite element analyses to assess the correlation between bone strain and skull form. With comprehensive loading we found that the distribution of peak von Mises strains was particularly uniform throughout the skull, although specific regions were dominated by tensile strains while others were dominated by compressive strains. Our analyses suggest that the frame-like skulls of diapsid reptiles are probably optimally formed (mechanically ideal: sufficient strength with the minimal amount of bone) with respect to functional forces; they are efficient in terms of having minimal bone volume, minimal weight, and also minimal energy demands in maintenance. PMID:22216358

Observation of skull-guided acoustic waves in a water-immersed murine skull using optoacoustic excitation

Science.gov (United States)

Estrada, Héctor; Rebling, Johannes; Razansky, Daniel

2017-02-01

The skull bone, a curved solid multilayered plate protecting the brain, constitutes a big challenge for the use of ultrasound-mediated techniques in neuroscience. Ultrasound waves incident from water or soft biological tissue are mostly reflected when impinging on the skull. To this end, skull properties have been characterized for both high-intensity focused ultrasound (HIFU) operating in the narrowband far-field regime and optoacoustic imaging applications. Yet, no study has been conducted to characterize the near-field of water immersed skulls. We used the thermoelastic effect with a 532 nm pulsed laser to trigger a wide range of broad-band ultrasound modes in a mouse skull. In order to capture the waves propagating in the near-field, a thin hydrophone was scanned in close proximity to the skull's surface. While Leaky pseudo-Lamb waves and grazing-angle bulk water waves are clearly visible in the spatio-temporal data, we were only able to identify skull-guided acoustic waves after dispersion analysis in the wavenumber-frequency space. The experimental data was found to be in a reasonable agreement with a flat multilayered plate model.

21 CFR 882.4750 - Skull punch.

Science.gov (United States)

2010-04-01

... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Skull punch. 882.4750 Section 882.4750 Food and... NEUROLOGICAL DEVICES Neurological Surgical Devices § 882.4750 Skull punch. (a) Identification. A skull punch is a device used to punch holes through a patient's skull to allow fixation of cranioplasty plates or...

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...

The skull in renal osteodystrophy

International Nuclear Information System (INIS)

Orzincolo, C.; Tamarozzi, R.; Bedani, P.L.

1987-01-01

Skull X-ray of 60 patients with chronic renal failure were examined. Alterations included diminished or increased bone density, radiolucent areas, pepper pot skull and the disappearance of vascular grooves and sutures. It is suggested that the radiological aspect of the skull is of very little diagnostic use in the assessment of uremic osteopathy since specific alterations are rare and tardive and show no correlation with clinical and laboratory findings. Skull X-ray can be usefull in assessing the effects of treatment (vitamin D derivaties, parathyroidectomy) and for the identification of focal lesions (brown tumors)

An Innovate Robotic Endoscope Guidance System for Transnasal Sinus and Skull Base Surgery: Proof of Concept.

Science.gov (United States)

Friedrich, D T; Sommer, F; Scheithauer, M O; Greve, J; Hoffmann, T K; Schuler, P J

2017-12-01

Objective  Advanced transnasal sinus and skull base surgery remains a challenging discipline for head and neck surgeons. Restricted access and space for instrumentation can impede advanced interventions. Thus, we present the combination of an innovative robotic endoscope guidance system and a specific endoscope with adjustable viewing angle to facilitate transnasal surgery in a human cadaver model. Materials and Methods  The applicability of the robotic endoscope guidance system with custom foot pedal controller was tested for advanced transnasal surgery on a fresh frozen human cadaver head. Visualization was enabled using a commercially available endoscope with adjustable viewing angle (15-90 degrees). Results  Visualization and instrumentation of all paranasal sinuses, including the anterior and middle skull base, were feasible with the presented setup. Controlling the robotic endoscope guidance system was effectively precise, and the adjustable endoscope lens extended the view in the surgical field without the common change of fixed viewing angle endoscopes. Conclusion  The combination of a robotic endoscope guidance system and an advanced endoscope with adjustable viewing angle enables bimanual surgery in transnasal interventions of the paranasal sinuses and the anterior skull base in a human cadaver model. The adjustable lens allows for the abandonment of fixed-angle endoscopes, saving time and resources, without reducing the quality of imaging.

State-of-the-art treatment alternatives for base of skull meningiomas: complementing and controversial indications for neurosurgery, stereotactic and robotic based radiosurgery or modern fractionated radiation techniques

International Nuclear Information System (INIS)

Combs, Stephanie E; Ganswindt, Ute; Foote, Robert L; Kondziolka, Douglas; Tonn, Jörg-Christian

2012-01-01

For skull base meningiomas, several treatment paradigms are available: Observation with serial imaging, surgical resection, stereotactic radiosurgery, radiation therapy or some combination of both. The choice depends on several factors. In this review we evaluate different treatment options, the outcome of modern irradiation techniques as well as the clinical results available, and establish recommendations for the treatment of patients with skull-base meningiomas

Base of the skull morphology and Class III malocclusion in patients with unilateral cleft lip and palate

Directory of Open Access Journals (Sweden)

Mariana Maciel Tinano

2015-02-01

Full Text Available OBJECTIVE: The aim of the present study was to determine the morphological differences in the base of the skull of individuals with cleft lip and palate and Class III malocclusion in comparison to control groups with Class I and Class III malocclusion. METHODS: A total of 89 individuals (males and females aged between 5 and 27 years old (Class I, n = 32; Class III, n = 29; and Class III individuals with unilateral cleft lip and palate, n = 28 attending PUC-MG Dental Center and Cleft Lip/Palate Care Center of Baleia Hospital and PUC-MG (CENTRARE were selected. Linear and angular measurements of the base of the skull, maxilla and mandible were performed and assessed by a single calibrated examiner by means of cephalometric radiographs. Statistical analysis involved ANCOVA and Bonferroni correction. RESULTS: No significant differences with regard to the base of the skull were found between the control group (Class I and individuals with cleft lip and palate (P > 0.017. The cleft lip/palate group differed from the Class III group only with regard to CI.Sp.Ba (P = 0.015. Individuals with cleft lip and palate had a significantly shorter maxillary length (Co-A in comparison to the control group (P < 0.001. No significant differences were found in the mandible (Co-Gn of the control group and individuals with cleft lip and palate (P = 1.000. CONCLUSION: The present findings suggest that there are no significant differences in the base of the skull of individuals Class I or Class III and individuals with cleft lip and palate and Class III malocclusion.

Augmented real-time navigation with critical structure proximity alerts for endoscopic skull base surgery.

Science.gov (United States)

Dixon, Benjamin J; Daly, Michael J; Chan, Harley; Vescan, Allan; Witterick, Ian J; Irish, Jonathan C

2014-04-01

Image-guided surgery (IGS) systems are frequently utilized during cranial base surgery to aid in orientation and facilitate targeted surgery. We wished to assess the performance of our recently developed localized intraoperative virtual endoscopy (LIVE)-IGS prototype in a preclinical setting prior to deployment in the operating room. This system combines real-time ablative instrument tracking, critical structure proximity alerts, three-dimensional virtual endoscopic views, and intraoperative cone-beam computed tomographic image updates. Randomized-controlled trial plus qualitative analysis. Skull base procedures were performed on 14 cadaver specimens by seven fellowship-trained skull base surgeons. Each subject performed two endoscopic transclival approaches; one with LIVE-IGS and one using a conventional IGS system in random order. National Aeronautics and Space Administration Task Load Index (NASA-TLX) scores were documented for each dissection, and a semistructured interview was recorded for qualitative assessment. The NASA-TLX scores for mental demand, effort, and frustration were significantly reduced with the LIVE-IGS system in comparison to conventional navigation (P < .05). The system interface was judged to be intuitive and most useful when there was a combination of high spatial demand, reduced or absent surface landmarks, and proximity to critical structures. The development of auditory icons for proximity alerts during the trial better informed the surgeon while limiting distraction. The LIVE-IGS system provided accurate, intuitive, and dynamic feedback to the operating surgeon. Further refinements to proximity alerts and visualization settings will enhance orientation while limiting distraction. The system is currently being deployed in a prospective clinical trial in skull base surgery. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

Optimization of Stereotactic Radiotherapy Treatment Delivery Technique for Base-Of-Skull Meningiomas

International Nuclear Information System (INIS)

Clark, Brenda G.; Candish, Charles; Vollans, Emily; Gete, Ermias; Lee, Richard; Martin, Monty; Ma, Roy; McKenzie, Michael

2008-01-01

This study compares static conformal field (CF), intensity modulated radiotherapy (IMRT), and dynamic arcs (DA) for the stereotactic radiotherapy of base-of-skull meningiomas. Twenty-one cases of base-of-skull meningioma (median planning target volume [PTV] = 21.3 cm 3 ) previously treated with stereotactic radiotherapy were replanned with each technique. The plans were compared for Radiation Therapy Oncology Group conformity index (CI) and homogeneity index (HI), and doses to normal structures at 6 dose values from 50.4 Gy to 5.6 Gy. The mean CI was 1.75 (CF), 1.75 (DA), and 1.66 (IMRT) (p 3 , the CI (IMRT) was always superior to CI (DA) and CI (CF). At PTV sizes below 25 cm 3 , there was no significant difference in CI between each technique. There was no significant difference in HI between plans. The total volume of normal tissue receiving 50.4, 44.8, and 5.6 Gy was significantly lower when comparing IMRT to CF and DA plans (p 3 , due to improved conformity and normal tissue sparing, in particular for the brain stem and ipsilateral temporal lobe

MRI-detected skull-base invasion. Prognostic value and therapeutic implication in intensity-modulated radiotherapy treatment for nasopharyngeal carcinoma

Energy Technology Data Exchange (ETDEWEB)

Cheng, Yi-Kan; Jiang, Ning; Yue, Dan; Tang, Ling-Long; Zhang, Fan; Lin, Li; Liu, Xu; Chen, Lei; Ma, Jun [Sun Yat-sen University Cancer Center, Department of Radiation Oncology, Guangzhou (China); Liu, Li-Zhi [Sun Yat-sen University Cancer Center, Department of Radiology, Guangzhou (China)

2014-10-15

With advances in imaging and radiotherapy, the prognostic value of skull-base invasion in nasopharyngeal carcinoma (NPC) needs to be reassessed. We aimed to define a classification system and evaluate the prognostic value of the classification of magnetic resonance imaging (MRI)-detected skull-base invasion in NPC treated with intensity-modulated radiotherapy (IMRT). We retrospectively reviewed 749 patients who underwent MRI and were subsequently histologically diagnosed with nondisseminated NPC and treated with IMRT. MRI-detected skull-base invasion was not found to be an independent prognostic factor for overall survival (OS), distant metastasis-free survival (DMFS), local relapse-free survival (LRFS), or disease-free survival (DFS; p > 0.05 for all). Skull-base invasion was classified according to the incidence of each site (type I sites inside pharyngobasilar fascia and clivus vs. type II sites outside pharyngobasilar fascia). The 5-year OS, DMFS, LRFS, and DFS rates in the classification of skull-base invasion in NPC were 83 vs. 67 %, 85 vs.75 %, 95 vs. 88 %, and 76 vs. 62 %, respectively (p < 0.05 for all). Multivariate analysis indicated the classification of skull-base invasion was an independent prognostic factor. MRI-detected skull-base invasion is not an independent prognostic factor in patients with NPC treated with IMRT. However, classification according to the site of invasion has prognostic value. Therefore, patients with various subclassifications of stage T3 disease may receive treatment with different intensities; however, further studies are warranted to prove this. (orig.) [German] Aufgrund der Fortschritte der bildgebenden Verfahren und der Strahlentherapie muss der prognostische Wert der Invasion des nasopharyngealen Karzinoms (NPC) in die Schaedelbasis erneut bewertet werden. Unser Ziel ist die Definition eines Klassifikationssystems und die Untersuchung des prognostischen Werts der Klassifikation der MRT-ermittelten Invasion des mit

Cranial nerve palsies in metastatic prostate cancer--results of base of skull radiotherapy

International Nuclear Information System (INIS)

O'Sullivan, Joe M.; Norman, Andrew R.; McNair, Helen; Dearnaley, David P.

2004-01-01

We studied the rate of response to palliative external beam radiation therapy (20 Gy/5 or 30 Gy/10 fractions) to the base of skull in 32 prostate cancer patients with cranial nerve dysfunction. Sixteen patients (50%; 95% CI, 34-66%) had a useful response to therapy. The median survival post-therapy was 3 months

Segmentation of human skull in MRI using statistical shape information from CT data.

Science.gov (United States)

Wang, Defeng; Shi, Lin; Chu, Winnie C W; Cheng, Jack C Y; Heng, Pheng Ann

2009-09-01

To automatically segment the skull from the MRI data using a model-based three-dimensional segmentation scheme. This study exploited the statistical anatomy extracted from the CT data of a group of subjects by means of constructing an active shape model of the skull surfaces. To construct a reliable shape model, a novel approach was proposed to optimize the automatic landmarking on the coupled surfaces (i.e., the skull vault) by minimizing the description length that incorporated local thickness information. This model was then used to locate the skull shape in MRI of a different group of patients. Compared with performing landmarking separately on the coupled surfaces, the proposed landmarking method constructed models that had better generalization ability and specificity. The segmentation accuracies were measured by the Dice coefficient and the set difference, and compared with the method based on mathematical morphology operations. The proposed approach using the active shape model based on the statistical skull anatomy presented in the head CT data contributes to more reliable segmentation of the skull from MRI data.

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

Osteomyelitis of the base of the skull

International Nuclear Information System (INIS)

Chandler, J.R.; Grobman, L.; Quencer, R.; Serafini, A.

1986-01-01

Infection in the marrow of the temporal, occipital, and sphenoid bones is an uncommon, but increasing occurrence. It is usually secondary to infections beginning in the external auditory canal and is caused almost uniformly by the gram negative Pseudomonas aeruginosa bacteria. Technetium and gallium scintigraphy help in the early detection of such infections while CT scans demonstrate dissolution of bone in well-developed cases. Headache is the predominant symptom. Dysphagia, hoarseness, and aspiration herald the inevitable march of cranial nerves. We have diagnosed and treated 17 cases of osteomyelitis of the skull base. Although the total mortality rate is 53%, it is now a curable disease. Six of our last 8 patients remain alive, although 1 is still under treatment. Treatment is medical and requires the long-term concomitant intravenous administration of an aminoglycoside and a broad spectrum semisynthetic penicillin effective against the causative organism

Nasal and skull base anatomy of endoscopic endonasal transsphenoidal surgery with multi-detector computed tomography

International Nuclear Information System (INIS)

Hasegawa, Yuzo; Saeki, Naokatsu; Murai, Hisayuki; Horiguchi, Kentaro; Hanazawa, Toyoyuki; Okamoto, Miyoshi; Yanagawa, Noriyuki

2008-01-01

The endoscope is a new and highly useful instrument for transphenoidal surgery (TSS), and is generally used because of its minimally invasiveness. In addition, endoscopic transsphenoidal surgey (eTSS) has a potential for more radical tumor removal at the pituitary and the parasellar regions by wider visualization and more powerful illumination. To operate these regions safely, we need to know nasal and skull base anatomy under the endoscope which looks different from images under a microscope. In this paper, we demonstrated nasal and skull base anatomy with multi-detector computed tomography, which was performed in 23 recent patients with pituitary and parasellar legions. In the nasal legion, deviation of nasal septum and deviation of sphenoid ostium are important for endonasal approach of eTSS, and often determine the difficulty of surgery in the nasal cavity. Our study showed that deviation of nasal septum was seen in 26% of patients. Deviation of sphenoid ostium was 5.5±1.5 mm from the midline. The anatomy of sphenoid sinus plays a key role in our determination of the safety of a bony opening of the sella. In addition to sellar, presellar, and concha types, carotid prominence and optic prominence are important to determine the midline orientation. Development of carotid prominence was significantly related to the extent of lateral pneumatization of sphenoid sinus (P=0.0016). Reconstructed 3D-image of sphenoid sinus was very useful in visual understanding skull base anatomy. (author)

Endoscopic Endonasal Approach in Skull Base Chondrosarcoma Associated with Maffucci Syndrome: Case Series and Literature Review.

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Beer-Furlan, André; Balsalobre, Leonardo; Vellutini, Eduardo A S; Stamm, Aldo C

2016-01-01

Maffucci syndrome is a nonhereditary disorder in which patients develop multiple enchondromas and cutaneous, visceral, or soft tissue hemangiomas. The potential malignant progression of enchondroma into a secondary chondrosarcoma is a well-known fact. Nevertheless, chondrosarcoma located at the skull base in patients with Maffuci syndrome is a very rare condition, with only 18 cases reported in the literature. We report 2 other cases successfully treated through an expanded endoscopic endonasal approach and discuss the condition based on the literature review. Skull base chondrosarcoma associated with Maffucci syndrome is a rare condition. The disease cannot be cured, therefore surgical treatment should be performed in symptomatic patients aiming for maximal tumor resection with function preservation. The endoscopic endonasal approach is a safe and reliable alternative for the management of these tumors. Copyright © 2016 Elsevier Inc. All rights reserved.

Stereotactic Ablative Radiosurgery for Locally-Advanced or Recurrent Skull Base Malignancies with Prior External Beam Radiation Therapy

Directory of Open Access Journals (Sweden)

Karen Mann Xu

2015-03-01

Full Text Available Purpose: Stereotactic ablative radiotherapy (SABR is an attractive modality to treat malignancies invading the skull base as it can deliver a highly conformal dose with minimal toxicity. However, variation exists in the prescribed dose and fractionation. The purpose of our study is to examine the local control, survival and toxicities in SABR for the treatment of malignant skull base tumors. Methods and Materials: A total of 31 patients and 40 locally-advanced or recurrent head and neck malignancies involving the skull base treated with a common SABR regimen which delivers a radiation dose of 44 Gy in 5 fractions from January 1st, 2004 to December 31st, 2013 were retrospectively reviewed. The local control rate (LC, progression-free survival rate (PFS, overall survival rate (OS and toxicities were reported.Results: The median follow-up time of all patients was 11.4 months (range: 0.6-67.2 months. The median tumor volume was 27 cm3 (range: 2.4-205 cm3. All patients received prior EBRT with a median radiation dose of 64 Gy (range: 24-75.6 Gy delivered in 12 to 42 fractions. 20 patients had surgeries prior to SABR. 19 patients received chemotherapy. Specifically, 8 patients received concurrent cetuximab (ErbituxTM with SABR. The median time-to-progression (TTP was 3.3 months (range: 0-16.9 months. For the 29 patients (93.5% who died, the median time from the end of first SABR to death was 10.3 months (range: 0.5-41.4 months. The estimated 1-year overall survival (OS rate was 35%. The estimated 2-year OS rate was 12%. Treatment was well-tolerated without grade 4 or 5 treatment-related toxicities.Conclusions: SABR has been shown to achieve low toxicities in locally-advanced or recurrent, previously irradiated head and neck malignancies invading the skull base.

Prediction and near-field observation of skull-guided acoustic waves.

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Estrada, Héctor; Rebling, Johannes; Razansky, Daniel

2017-06-21

Ultrasound waves propagating in water or soft biological tissue are strongly reflected when encountering the skull, which limits the use of ultrasound-based techniques in transcranial imaging and therapeutic applications. Current knowledge on the acoustic properties of the cranial bone is restricted to far-field observations, leaving its near-field unexplored. We report on the existence of skull-guided acoustic waves, which was herein confirmed by near-field measurements of optoacoustically-induced responses in ex-vivo murine skulls immersed in water. Dispersion of the guided waves was found to reasonably agree with the prediction of a multilayered flat plate model. We observed a skull-guided wave propagation over a lateral distance of at least 3 mm, with a half-decay length in the direction perpendicular to the skull ranging from 35 to 300 μm at 6 and 0.5 MHz, respectively. Propagation losses are mostly attributed to the heterogenous acoustic properties of the skull. It is generally anticipated that our findings may facilitate and broaden the application of ultrasound-mediated techniques in brain diagnostics and therapy.

Prediction and near-field observation of skull-guided acoustic waves

Science.gov (United States)

Estrada, Héctor; Rebling, Johannes; Razansky, Daniel

2017-06-01

Ultrasound waves propagating in water or soft biological tissue are strongly reflected when encountering the skull, which limits the use of ultrasound-based techniques in transcranial imaging and therapeutic applications. Current knowledge on the acoustic properties of the cranial bone is restricted to far-field observations, leaving its near-field unexplored. We report on the existence of skull-guided acoustic waves, which was herein confirmed by near-field measurements of optoacoustically-induced responses in ex-vivo murine skulls immersed in water. Dispersion of the guided waves was found to reasonably agree with the prediction of a multilayered flat plate model. We observed a skull-guided wave propagation over a lateral distance of at least 3 mm, with a half-decay length in the direction perpendicular to the skull ranging from 35 to 300 μm at 6 and 0.5 MHz, respectively. Propagation losses are mostly attributed to the heterogenous acoustic properties of the skull. It is generally anticipated that our findings may facilitate and broaden the application of ultrasound-mediated techniques in brain diagnostics and therapy.

A small skull from Flores dated to the 20th century

DEFF Research Database (Denmark)

Villa, Chiara; Persson, Liselott; Alexandersen, Verner

2012-01-01

A human skull with mandible from the Ngada District on the island of Flores, Indonesia, is described in order to contribute to the knowledge of variation in cranial architecture, which is important in interpretations of evolutionary cerebralisation. The skull was excavated in 1924 and sent...... to the National Museum in Copenhagen. The "Copenhagen Flores" (CF) male skull is radiocarbon-dated and of modern age. The cranium is small, but larger than e.g. Liang Bua skull (LB1) in every measurement. The (CT-scan based) cranial capacity of 1258 ml is normal for modern humans, but somewhat lower than values...

Skull-base foramina of the middle cranial fossa : assessment of normal variation with high-resolution CT

International Nuclear Information System (INIS)

Kim, Hyae Young; Chung, Eun Chul; Suh, Jeong Soo; Choi, Hye Young; Ko, Eun Joo; Lee, Myung Sook

1997-01-01

To recognize foraminal variants of the foraminae of the skull base in the middle cranial fossa, and to thus understand and distinguish normal and potentially abnormal structures. We analysed 163 patients without intracranial disease who had undergone CT scanning. These comprised 82 men and 81 women with a mean age of 39 years (range, 4-73 years). HRCT was performed, using a GE 9800 scanner. All CT scans were obtained 6-7 slices at the base of the skull, with 1.5mm collimation at 1.5mm intervals parallel to the infraorbital line. We analysed the foraminae by closesly correlating imaging findings and established anatomic knowledge. In 45 cases (27.6%) the foramen ovale was 5-10mm in diameter and asymmetrical. Deficiency of the medial bony wall including persistent foramen lacerum medius was seen in five cases (3.1%). Confluence of the foramen ovale and the foramen spinosum was seen in 13 cases (8%) and confluence of the foramen ovale and the foramen of Vesalius in 23 (14.1%). Posterolateral groove for the accessory meningeal artery was observed in 36 cases (22%). The foramen spinosum was asymmetrical in 42 cases (25.8%). A small or absent foramen spinosum with a larger ipsilateral foramen ovale was observed in 11 cases (6.7%). Medial bony defect was seen in 16 cases (9.8%). The foramen spinosum was absent in four cases (2.5%). In 74 cases (45.4%), the foramen of Vesalius was absent; it was present unilaterally and bilaterally in 55 (33.7%) and 34 cases (20.9%), respectively. Five cases showed duplicated foramina. Canaliculus innominatus was seen in 14 cases (8.9%) and was present bilaterally in three (1.8%). HRCT clearly delineates bony structure and is well able to display the rich spectrum of anatomic variation found in the base of the skull. The recognition of these normal variants will result in a better understanding of skull base neurovascular anatomy and diminish speculation as to their true nature during the interpretation of CT images

Motor evoked potential monitoring of the vagus nerve with transcranial electrical stimulation during skull base surgeries.

Science.gov (United States)

Ito, Eiji; Ichikawa, Masahiro; Itakura, Takeshi; Ando, Hitoshi; Matsumoto, Yuka; Oda, Keiko; Sato, Taku; Watanabe, Tadashi; Sakuma, Jun; Saito, Kiyoshi

2013-01-01

Dysphasia is one of the most serious complications of skull base surgeries and results from damage to the brainstem and/or cranial nerves involved in swallowing. Here, the authors propose a method to monitor the function of the vagus nerve using endotracheal tube surface electrodes and transcranial electrical stimulation during skull base surgeries. Fifteen patients with skull base or brainstem tumors were enrolled. The authors used surface electrodes of an endotracheal tube to record compound electromyographic responses from the vocalis muscle. Motor neurons were stimulated using corkscrew electrodes placed subdermally on the scalp at C3 and C4. During surgery, the operator received a warning when the amplitude of the vagal motor evoked potential (MEP) decreased to less than 50% of the control level. After surgery, swallowing function was assessed clinically using grading criteria. In 5 patients, vagal MEP amplitude permanently deteriorated to less than 50% of the control level on the right side when meningiomas were dissected from the pons or basilar artery, or when a schwannoma was dissected from the vagal rootlets. These 5 patients had postoperative dysphagia. At 4 weeks after surgery, 2 patients still had dysphagia. In 2 patients, vagal MEPs of one side transiently disappeared when the tumors were dissected from the brainstem or the vagal rootlets. After surgery, both patients had dysphagia, which recovered in 4 weeks. In 7 patients, MEP amplitude was consistent, maintaining more than 50% of the control level throughout the operative procedures. After surgery all 7 patients were neurologically intact with normal swallowing function. Vagal MEP monitoring with transcranial electrical stimulation and endotracheal tube electrode recording was a safe and effective method to provide continuous real-time information on the integrity of both the supranuclear and infranuclear vagal pathway. This method is useful to prevent intraoperative injury of the brainstem

Skull Base Meningiomas and Cranial Nerves Contrast Using Sodium Fluorescein: A New Application of an Old Tool.

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da Silva, Carlos Eduardo; da Silva, Vinicius Duval; da Silva, Jefferson Luis Braga

2014-08-01

Objective The identification of cranial nerves is one of the most challenging goals in the dissection of skull base meningiomas. The authors present an application of sodium fluorescein (SF) in skull base meningiomas with the purpose of improving the identification of cranial nerves. Design A prospective study within-subjects design. Setting Hospital Ernesto Dornelles, Porto Alegre, Brazil. Participants Patients with skull base meningiomas. Main Outcomes Measures Cranial nerve identification. Results The group of nine meningiomas was composed of one cavernous sinus, three petroclival, one tuberculum sellae, two sphenoid wing, one olfactory groove, and one temporal floor meningioma. The SF enhancement in all tumors was strong, and the contrast with cranial nerves clearly evident. There were one definite olfactory nerve deficit, one transient abducens deficit, and one definite hemiparesis. All lesions were resected (Simpson grades 1 and 2). The analysis of the difference of the delta SF wavelength between the meningiomas and cranial nerve contrast was performed by the Wilcoxon signed rank test and showed p = 0.011. Conclusions The contrast between the enhanced meningiomas and cranial nerves was evident and assisted in the visualization and microsurgical dissection of these structures. The anatomical preservation of these structures was improved using the contrast.

Inflammatory Myofibroblastic Tumour of the Skull Base

Directory of Open Access Journals (Sweden)

Jean-Philippe Maire

2013-01-01

Full Text Available Inflammatory myofibroblastic tumors (IMTs are rare benign clinical and pathological entities. IMTs have been described in the lungs, abdomen, retroperitoneum, and extremities but rarely in the head and neck region. A 38-year-old man presented with headache, right exophthalmia, and right 6th nerve palsy. A CT scan revealed enlargement of the right cavernous sinus and osteolytic lesions of the right sphenoid and clivus. MR imaging showed a large tumor of the skull base which was invading the sella turcica, right cavernous sinus, and sphenoidal sinus. A biopsy was performed and revealed an IMT. Corticosteroids were given for 3 months but were inefficient. In the framework of our pluridisciplinary consultation, fractionated conformal radiotherapy (FRT was indicated at a low dose; 20 Gy in 10 fractions of 2 Gy over 12 days were delivered. Clinical response was complete 3 months after FRT. Radiological response was subtotal 6 months after FRT. Two years later, the patient is well.

21 CFR 882.4030 - Skull plate anvil.

Science.gov (United States)

2010-04-01

... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Skull plate anvil. 882.4030 Section 882.4030 Food... DEVICES NEUROLOGICAL DEVICES Neurological Surgical Devices § 882.4030 Skull plate anvil. (a) Identification. A skull plate anvil is a device used to form alterable skull plates in the proper shape to fit...

Magnetoencephalography signals are influenced by skull defects.

Science.gov (United States)

Lau, S; Flemming, L; Haueisen, J

2014-08-01

Magnetoencephalography (MEG) signals had previously been hypothesized to have negligible sensitivity to skull defects. The objective is to experimentally investigate the influence of conducting skull defects on MEG and EEG signals. A miniaturized electric dipole was implanted in vivo into rabbit brains. Simultaneous recording using 64-channel EEG and 16-channel MEG was conducted, first above the intact skull and then above a skull defect. Skull defects were filled with agar gels, which had been formulated to have tissue-like homogeneous conductivities. The dipole was moved beneath the skull defects, and measurements were taken at regularly spaced points. The EEG signal amplitude increased 2-10 times, whereas the MEG signal amplitude reduced by as much as 20%. The EEG signal amplitude deviated more when the source was under the edge of the defect, whereas the MEG signal amplitude deviated more when the source was central under the defect. The change in MEG field-map topography (relative difference measure, RDM(∗)=0.15) was geometrically related to the skull defect edge. MEG and EEG signals can be substantially affected by skull defects. MEG source modeling requires realistic volume conductor head models that incorporate skull defects. Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

[The transperygoid approach to the removal of a recurrent juvenile angiofibroma at the base of the skull without preoperative embolization].

Science.gov (United States)

Grachev, N S; Vorozhtsov, I N

The authors report a clinical case of successful elimination of a recurrent juvenile angiofibroma at the base of the skull (JAFBS) with the application of the optical navigation system and a cold plasma scalpel in the absence of preoperative embolization. It has been demonstrated using the proposed transperygoid approach to the extirpation of the tumour that a recurrent juvenile angiofibroma at the base of the skull can be efficiently removed by means of a modern minimally invasive and at the same time radical surgical method.

Role of skull radiography in the initial evaluation of minor head injury: a retrospective study

International Nuclear Information System (INIS)

Murshid, W.R.

1994-01-01

The use of skull radiography in the initial evaluation of minor head injured patients is controversial. In an attempt to evaluate its benefits, a retrospective study of 566 cases subjected to skull radiography following close minor head trauma (Glasgow Coma Scale 13-15), is presented. A skull fracture (linear vault, depressed or base of skull) was present in 64 (11%) cases. Only three (5%) who were found to have a skull fracture on skull radiography developed an intracranial injury which required surgery. Intracranial injuries developed in 19 (3%) cases and were followed by surgery in six (32%). All, except for one case, had a decreased level of consciousness and a Glasgow Coma Scale less than 15, few had focal neurological deficits. Management had not been altered by the results of skull radiography in any of the cases. We concluded that skull radiographs are unnecessary for the decision process in closed minor head injury because management decisions are based primarily on a careful neurological examination. When intracranial injuries are a concern, a CT scan should be obtained. (author)

Thermal model to investigate the temperature in bone grinding for skull base neurosurgery.

Science.gov (United States)

Zhang, Lihui; Tai, Bruce L; Wang, Guangjun; Zhang, Kuibang; Sullivan, Stephen; Shih, Albert J

2013-10-01

This study develops a thermal model utilizing the inverse heat transfer method (IHTM) to investigate the bone grinding temperature created by a spherical diamond tool used for skull base neurosurgery. Bone grinding is a critical procedure in the expanded endonasal approach to remove the cranial bone and access to the skull base tumor via nasal corridor. The heat is generated during grinding and could damage the nerve or coagulate the blood in the carotid artery adjacent to the bone. The finite element analysis is adopted to investigate the grinding-induced bone temperature rise. The heat source distribution is defined by the thermal model, and the temperature distribution is solved using the IHTM with experimental inputs. Grinding experiments were conducted on a bovine cortical bone with embedded thermocouples. Results show significant temperature rise in bone grinding. Using 50°C as the threshold, the thermal injury can propagate about 3mm in the traverse direction, and 3mm below the ground surface under the dry grinding condition. The presented methodology demonstrated the capability of being a thermal analysis tool for bone grinding study. Copyright © 2013 IPEM. Published by Elsevier Ltd. All rights reserved.

[A case of pycnodysostosis--observation of the skull by CT scan].

Science.gov (United States)

Anegawa, S; Bekki, Y; Furukawa, Y; Yokota, S; Torigoe, R

1987-07-01

A 13-year-old boy was presented to the Department of Neurosurgery, Saiseikai Fukuoka General Hospital for further examinations concerning abnormal findings in the skull radiogram taken when he struck his head. His physical features showed some characteristics the same as those of pycnodysostosis as follows--proportionate dwarfism, prominent forehead, short spoon-shaped fingers, bilateral exophthalmos. A skull radiogram revealed widely open cranial sutures with no healing of the fracture and craniotomy which was performed for an acute epidural hematoma 6 years ago. Furthermore, the mandible was hypoplastic with a virtual loss of mandibular angle. CT of the soft tissues showed somewhat dilated cortical sulci and ventricles without any structural abnormalities in the brain. CT of bone algorithm revealed specific characteristics of this disease. The paranasal sinuses were quite hypoplastic. Especially in the maxillary sinuses, frontal sinuses and mastoid air cells, none of developments of sinuses were noted, even though the middle and internal ear seemed to be normal. Moreover, the ethmoid and sphenoid sinuses were noted, although their developments were poor. The appearance of skull base was normal, including the inlets and outlets of cranial nerves or vessels and synchondroses. However, the density of the skull base, especially in the diploe, was higher than normal in Hansfield number. Furthermore, detailed measurements of skull base demonstrated that the skull base itself was also dwarfism. Pycnodysostosis is a generalized skeletal disease whose cardinal features are moderate generalized osteosclerosis and dwarfism. However, the detailed observation on the cranium by CT has not been reported. In our study, the development of sinuses in bones with intramembranous ossification are worse than that with endochondral ossification.(ABSTRACT TRUNCATED AT 250 WORDS)

CT findings in patient with skull fractures

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Jo, Han Gi; Suh, Won Hyuck [Korea University College of Medicine, Seoul (Korea, Republic of)

1988-12-15

CT scan has been inevitable method for patient with head trauma. CT scans of 94 cases, which were confirmed skull fracture by plain film, were reviewed for better and useful dealing of CT. The results were as follows: 1. Car accident was the most frequent cause of head injury. 2. No evidence of intracranial abnormality in CT scan of skull fractures on plane film was 45.7%, and alert mentality was 46.8% of skull fracture on skull fracture on simple film. 3. Detection rate on CT scan to skull fractures was 27.7%, but detection rate to depression fractures of skull fracture was 70.2%. 4. Mortality rate of patients with skull fracture was 10.6%. 5. Associated CT findings were pneumocephalus on CT scan 3.2%, contusion of edema 4.2%, epidural hematoma 16.0%, subdural hematoma 17.0%, subdural hygroma 2.1%, intracerebral hemorrhage 4.9%, and subarachnoid hemorrhage 2.0%.

3D cinematic rendering of the calvarium, maxillofacial structures, and skull base: preliminary observations.

Science.gov (United States)

Rowe, Steven P; Zinreich, S James; Fishman, Elliot K

2018-06-01

Three-dimensional (3D) visualizations of volumetric data from CT have gained widespread clinical acceptance and are an important method for evaluating complex anatomy and pathology. Recently, cinematic rendering (CR), a new 3D visualization methodology, has become available. CR utilizes a lighting model that allows for the production of photorealistic images from isotropic voxel data. Given how new this technique is, studies to evaluate its clinical utility and any potential advantages or disadvantages relative to other 3D methods such as volume rendering have yet to be published. In this pictorial review, we provide examples of normal calvarial, maxillofacial, and skull base anatomy and pathological conditions that highlight the potential for CR images to aid in patient evaluation and treatment planning. The highly detailed images and nuanced shadowing that are intrinsic to CR are well suited to the display of the complex anatomy in this region of the body. We look forward to studies with CR that will ascertain the ultimate value of this methodology to evaluate calvarium, maxillofacial, and skull base morphology as well as other complex anatomic structures.

Role of targeted magnetic resonance imaging sequences in the surgical management of anterior skull base pathology.

Science.gov (United States)

Chawla, S; Bowman, J; Gandhi, M; Panizza, B

2017-01-01

The skull base is a highly complex anatomical region that provides passage for important nerves and vessels as they course into and out of the cranial cavity. Key to the management of pathology in this region is a thorough understanding of the anatomy, with its variations, and the relationship of various neurovascular structures to the pathology in question. Targeted high-resolution magnetic resonance imaging on high field strength magnets can enable the skull base surgeon to understand this intricate relationship and deal with the pathology from a position of relative advantage. With the help of case studies, this paper illustrates the application of specialised magnetic resonance techniques to study pathology of the orbital apex in particular. The fine anatomical detail provided gives surgeons the ability to design an endonasal endoscopic procedure appropriate to the anatomy of the pathology.

Epilepsy Surgery for Skull-Base Temporal Lobe Encephaloceles: Should We Spare the Hippocampus from Resection?

Science.gov (United States)

Bannout, Firas; Harder, Sheri; Lee, Michael; Zouros, Alexander; Raghavan, Ravi; Fogel, Travis; De Los Reyes, Kenneth; Losey, Travis

2018-01-01

The neurosurgical treatment of skull base temporal encephalocele for patients with epilepsy is variable. We describe two adult cases of temporal lobe epilepsy (TLE) with spheno-temporal encephalocele, currently seizure-free for more than two years after anterior temporal lobectomy (ATL) and lesionectomy sparing the hippocampus without long-term intracranial electroencephalogram (EEG) monitoring. Encephaloceles were detected by magnetic resonance imaging (MRI) and confirmed by maxillofacial head computed tomography (CT) scans. Seizures were captured by scalp video-EEG recording. One case underwent intraoperative electrocorticography (ECoG) with pathology demonstrating neuronal heterotopia. We propose that in some patients with skull base temporal encephaloceles, minimal surgical resection of herniated and adjacent temporal cortex (lesionectomy) is sufficient to render seizure freedom. In future cases, where an associated malformation of cortical development is suspected, newer techniques such as minimally invasive EEG monitoring with stereotactic-depth EEG electrodes should be considered to tailor the surrounding margins of the resected epileptogenic zone. PMID:29534521

Epilepsy Surgery for Skull-Base Temporal Lobe Encephaloceles: Should We Spare the Hippocampus from Resection?

Directory of Open Access Journals (Sweden)

Firas Bannout

2018-03-01

Full Text Available The neurosurgical treatment of skull base temporal encephalocele for patients with epilepsy is variable. We describe two adult cases of temporal lobe epilepsy (TLE with spheno-temporal encephalocele, currently seizure-free for more than two years after anterior temporal lobectomy (ATL and lesionectomy sparing the hippocampus without long-term intracranial electroencephalogram (EEG monitoring. Encephaloceles were detected by magnetic resonance imaging (MRI and confirmed by maxillofacial head computed tomography (CT scans. Seizures were captured by scalp video-EEG recording. One case underwent intraoperative electrocorticography (ECoG with pathology demonstrating neuronal heterotopia. We propose that in some patients with skull base temporal encephaloceles, minimal surgical resection of herniated and adjacent temporal cortex (lesionectomy is sufficient to render seizure freedom. In future cases, where an associated malformation of cortical development is suspected, newer techniques such as minimally invasive EEG monitoring with stereotactic-depth EEG electrodes should be considered to tailor the surrounding margins of the resected epileptogenic zone.

If the skull fits: magnetic resonance imaging and microcomputed tomography for combined analysis of brain and skull phenotypes in the mouse

Science.gov (United States)

Blank, Marissa C.; Roman, Brian B.; Henkelman, R. Mark; Millen, Kathleen J.

2012-01-01

The mammalian brain and skull develop concurrently in a coordinated manner, consistently producing a brain and skull that fit tightly together. It is common that abnormalities in one are associated with related abnormalities in the other. However, this is not always the case. A complete characterization of the relationship between brain and skull phenotypes is necessary to understand the mechanisms that cause them to be coordinated or divergent and to provide perspective on the potential diagnostic or prognostic significance of brain and skull phenotypes. We demonstrate the combined use of magnetic resonance imaging and microcomputed tomography for analysis of brain and skull phenotypes in the mouse. Co-registration of brain and skull images allows comparison of the relationship between phenotypes in the brain and those in the skull. We observe a close fit between the brain and skull of two genetic mouse models that both show abnormal brain and skull phenotypes. Application of these three-dimensional image analyses in a broader range of mouse mutants will provide a map of the relationships between brain and skull phenotypes generally and allow characterization of patterns of similarities and differences. PMID:22947655

Transsphenoidal Approach in Endoscopic Endonasal Surgery for Skull Base Lesions: What Radiologists and Surgeons Need to Know.

Science.gov (United States)

García-Garrigós, Elena; Arenas-Jiménez, Juan José; Monjas-Cánovas, Irene; Abarca-Olivas, Javier; Cortés-Vela, Jesús Julián; De La Hoz-Rosa, Javier; Guirau-Rubio, Maria Dolores

2015-01-01

In the last 2 decades, endoscopic endonasal transsphenoidal surgery has become the most popular choice of neurosurgeons and otolaryngologists to treat lesions of the skull base, with minimal invasiveness, lower incidence of complications, and lower morbidity and mortality rates compared with traditional approaches. The transsphenoidal route is the surgical approach of choice for most sellar tumors because of the relationship of the sphenoid bone to the nasal cavity below and the pituitary gland above. More recently, extended approaches have expanded the indications for transsphenoidal surgery by using different corridors leading to specific target areas, from the crista galli to the spinomedullary junction. Computer-assisted surgery is an evolving technology that allows real-time anatomic navigation during endoscopic surgery by linking preoperative triplanar radiologic images and intraoperative endoscopic views, thus helping the surgeon avoid damage to vital structures. Preoperative computed tomography is the preferred modality to show bone landmarks and vascular structures. Radiologists play an important role in surgical planning by reporting extension of sphenoid pneumatization, recesses and septations of the sinus, and other relevant anatomic variants. Radiologists should understand the relationships of the sphenoid bone and skull base structures, anatomic variants, and image-guided neuronavigation techniques to prevent surgical complications and allow effective treatment of skull base lesions with the endoscopic endonasal transsphenoidal approach. ©RSNA, 2015.

Isolated hypoglossal nerve palsy due to skull base metastasis from breast cancer

International Nuclear Information System (INIS)

Pavithran, K.; Doval, D.C.; Hukku, S.; Jena, A.

2001-01-01

We describe a 44-year-old woman who presented with an isolated unilateral hypoglossal nerve paralysis caused by a skull base metastasis from breast cancer. The patient had a modified radical mastectomy followed by local radiotherapy and adjuvant chemotherapy. Fourteen months later she presented with difficulty in speaking. Physical examination revealed an isolated left hypoglossal nerve paralysis. The MRI scan showed a mass lesion involving the left occipital condyle extending into hypoglossal canal. Copyright (2001) Blackwell Science Pty Ltd

New insights into the skull of Istiodactylus latidens (Ornithocheiroidea, Pterodactyloidea.

Directory of Open Access Journals (Sweden)

Mark P Witton

Full Text Available The skull of the Cretaceous pterosaur Istiodactylus latidens, a historically important species best known for its broad muzzle of interlocking, lancet-shaped teeth, is almost completely known from the broken remains of several individuals, but the length of its jaws remains elusive. Estimates of I. latidens jaw length have been exclusively based on the incomplete skull of NHMUK R3877 and, perhaps erroneously, reconstructed by assuming continuation of its broken skull pieces as preserved in situ. Here, an overlooked jaw fragment of NHMUK R3877 is redescribed and used to revise the skull reconstruction of I. latidens. The new reconstruction suggests a much shorter skull than previously supposed, along with a relatively tall orbital region and proportionally slender maxilla, a feature documented in the early 20(th century but ignored by all skull reconstructions of this species. These features indicate that the skull of I. latidens is particularly distinctive amongst istiodactylids and suggests greater disparity between I. latidens and I. sinensis than previously appreciated. A cladistic analysis of istiodactylid pterosaurs incorporating new predicted I. latidens skull metrics suggests Istiodactylidae is constrained to five species (Liaoxipterus brachyognathus, Lonchengpterus zhoai, Nurhachius ignaciobritoi, Istiodactylus latidens and Istiodactylus sinensis defined by their distinctive dentition, but excludes the putative istiodactylids Haopterus gracilis and Hongshanopterus lacustris. Istiodactylus latidens, I. sinensis and Li. brachyognathus form an unresolved clade of derived istiodactylids, and the similarity of comparable remains of I. sinensis and Li. brachyognathus suggest further work into their taxonomy and classification is required. The new skull model of I. latidens agrees with the scavenging habits proposed for these pterosaurs, with much of their cranial anatomy converging on that of habitually scavenging birds.

New Insights into the Skull of Istiodactylus latidens (Ornithocheiroidea, Pterodactyloidea)

Science.gov (United States)

Witton, Mark P.

2012-01-01

The skull of the Cretaceous pterosaur Istiodactylus latidens, a historically important species best known for its broad muzzle of interlocking, lancet-shaped teeth, is almost completely known from the broken remains of several individuals, but the length of its jaws remains elusive. Estimates of I. latidens jaw length have been exclusively based on the incomplete skull of NHMUK R3877 and, perhaps erroneously, reconstructed by assuming continuation of its broken skull pieces as preserved in situ. Here, an overlooked jaw fragment of NHMUK R3877 is redescribed and used to revise the skull reconstruction of I. latidens. The new reconstruction suggests a much shorter skull than previously supposed, along with a relatively tall orbital region and proportionally slender maxilla, a feature documented in the early 20th century but ignored by all skull reconstructions of this species. These features indicate that the skull of I. latidens is particularly distinctive amongst istiodactylids and suggests greater disparity between I. latidens and I. sinensis than previously appreciated. A cladistic analysis of istiodactylid pterosaurs incorporating new predicted I. latidens skull metrics suggests Istiodactylidae is constrained to five species (Liaoxipterus brachyognathus, Lonchengpterus zhoai, Nurhachius ignaciobritoi, Istiodactylus latidens and Istiodactylus sinensis) defined by their distinctive dentition, but excludes the putative istiodactylids Haopterus gracilis and Hongshanopterus lacustris. Istiodactylus latidens, I. sinensis and Li. brachyognathus form an unresolved clade of derived istiodactylids, and the similarity of comparable remains of I. sinensis and Li. brachyognathus suggest further work into their taxonomy and classification is required. The new skull model of I. latidens agrees with the scavenging habits proposed for these pterosaurs, with much of their cranial anatomy converging on that of habitually scavenging birds. PMID:22470442

Cloverleaf skull with generalised bone dysplasia

International Nuclear Information System (INIS)

Kozlowski, K.; Warren, P.S.; Fisher, C.C.; Royal Hospital for Women, Camperdown

1985-01-01

A case of cloverleaf skull with generalised bone dysplasia is reported. The authors believe that bone dysplasia associated with cloverleaf is neither identical with thanatophoric dysplasia nor achondroplasia. Until identity of thanatophoric dysplasia and cloverleaf skull with generalised bone dysplasia is proved the diseases should be looked upon as separate entities and the wording ''thanatophoric dysplasia with cloverleaf skull'' should be abolished. (orig.)

Important prognostic factors in patients with skull base erosion from nasopharyngeal carcinoma after radiotherapy

International Nuclear Information System (INIS)

Lu, T.-X.; Mai, W.-Y.; Teh, Bin S.; Hu, Y.-H.; Lu, Hsin H.; Chiu, J. Kam; Carpenter, L. Steven; Woo, Shiao Y.; Butler, E. Brian

2001-01-01

Purpose: To evaluate the long-term outcome and prognostic factors in patients with skull base erosion from nasopharyngeal carcinoma after initial radiotherapy (RT). Methods and Materials: From January 1985 to December 1986, 100 patients (71 males, 29 females) with a diagnosis of nasopharyngeal carcinoma were found on computed tomography (CT) to have skull base erosion. The mean age was 41 years (range 16-66). Ninety-six patients had World Health Organization type III undifferentiated carcinoma, and 4 had type I. The metastatic workup, including chest radiography, liver ultrasound scanning, and liver function test was negative. All patients underwent external beam RT (EBRT) alone to 66-80 Gy during 6-8 weeks. A daily fraction size of 2 Gy was delivered using 60 Co or a linear accelerator. No patient received chemotherapy. All patients were followed at regular intervals after irradiation. The median follow-up was 22.3 months (range 2-174). Survival of the cohort was computed by the Kaplan-Meier method. The potential prognostic factors of survival were examined. Multivariate analyses were performed using the Cox regression model. Results: The 1, 2, 5, and 10-year overall survival rate for the cohort was 79%, 41%, 27%, and 13%, respectively. However, the subgroup of patients with both anterior cranial nerve (I-VIII) and posterior cranial nerve (IX-XII) involvement had a 5-year survival of only 7.7%. A difference in the time course of local recurrence and distant metastasis was observed. Both local recurrence and distant metastasis often occurred within the first 2 years after RT. However, local relapse continued to occur after 5 years. In contrast, no additional distant metastases were found after 5 years. The causes of death included local recurrence (n=59), distant metastasis (n=21), both local recurrence and distant metastasis (n = 1), and unrelated causes (n=5). After multivariate analysis, complete recovery of cranial nerve involvement, cranial nerve palsy, and

Properties and architecture of the sperm whale skull amphitheatre.

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Alam, Parvez; Amini, Shahrouz; Tadayon, Maryam; Miserez, Ali; Chinsamy, Anusuya

2016-02-01

The sperm whale skull amphitheatre cradles an enormous two-tonne spermaceti organ. The amphitheatre separates this organ from the cranium and the cervical vertebrae that lie in close proximity to the base of the skull. Here, we elucidate that this skull amphitheatre is an elastic, flexible, triple-layered structure with mechanical properties that are conjointly guided by bone histology and the characteristics of pore space. We contend that the amphitheatre will flex elastically to equilibrate forces transmitted via the spermaceti organ that arise through diving. We find that collisions from sperm whale aggression do not cause the amphitheatre to bend, but rather localise stress to the base of the amphitheatre on its anterior face. We consider, therefore, that the uniquely thin and extended construction of the amphitheatre, has relevance as an energy absorptive structure in diving. Copyright © 2015 Elsevier GmbH. All rights reserved.

Clinical efficacy and safety of surface imaging guided radiosurgery (SIG-RS) in the treatment of benign skull base tumors.

Science.gov (United States)

Lau, Steven K M; Patel, Kunal; Kim, Teddy; Knipprath, Erik; Kim, Gwe-Ya; Cerviño, Laura I; Lawson, Joshua D; Murphy, Kevin T; Sanghvi, Parag; Carter, Bob S; Chen, Clark C

2017-04-01

Frameless, surface imaging guided radiosurgery (SIG-RS) is a novel platform for stereotactic radiosurgery (SRS) wherein patient positioning is monitored in real-time through infra-red camera tracking of facial topography. Here we describe our initial clinical experience with SIG-RS for the treatment of benign neoplasms of the skull base. We identified 48 patients with benign skull base tumors consecutively treated with SIG-RS at a single institution between 2009 and 2011. Patients were diagnosed with meningioma (n = 22), vestibular schwannoma (n = 20), or nonfunctional pituitary adenoma (n = 6). Local control and treatment-related toxicity were retrospectively assessed. Median follow-up was 65 months (range 61-72 months). Prescription doses were 12-13 Gy in a single fraction (n = 18), 8 Gy × 3 fractions (n = 6), and 5 Gy × 5 fractions (n = 24). Actuarial tumor control rate at 5 years was 98%. No grade ≥3 treatment-related toxicity was observed. Grade ≤2 toxicity was associated with symptomatic lesions (p = 0.049) and single fraction treatment (p = 0.005). SIG-RS for benign skull base tumors produces clinical outcomes comparable to conventional frame-based SRS techniques while enhancing patient comfort.

Cloverleaf skull with generalised bone dysplasia

Energy Technology Data Exchange (ETDEWEB)

Kozlowski, K.; Warren, P.S.; Fisher, C.C.

1985-09-01

A case of cloverleaf skull with generalised bone dysplasia is reported. The authors believe that bone dysplasia associated with cloverleaf is neither identical with thanatophoric dysplasia nor achondroplasia. Until identity of thanatophoric dysplasia and cloverleaf skull with generalised bone dysplasia is proved the diseases should be looked upon as separate entities and the wording ''thanatophoric dysplasia with cloverleaf skull'' should be abolished.

Avian skull morphological evolution: exploring exo- and endocranial covariation with two-block partial least squares.

Science.gov (United States)

Marugán-Lobón, Jesús; Buscalioni, Angela D

2006-01-01

While rostral variation has been the subject of detailed avian evolutionary research, avian skull organization, characterized by a flexed or extended appearance of the skull, has eventually become neglected by mainstream evolutionary inquiries. This study aims to recapture its significance, evaluating possible functional, phylogenetic and developmental factors that may be underlying it. In order to estimate which, and how, elements of the skull intervene in patterning the skull we tested the statistical interplay between a series of old mid-sagittal angular measurements (mostly endocranial) in combination with newly obtained skull metrics based on landmark superimposition methods (exclusively exocranial shape), by means of the statistic-morphometric technique of two-block partial least squares. As classic literature anticipated, we found that the external appearance of the skull corresponds to the way in which the plane of the caudal cranial base is oriented, in connection with the orientations of the plane of the foramen magnum and of the lateral semicircular canal. The pattern of covariation found between metrics conveys flexed or extended appearances of the skull implicitly within a single and statistically significant dimension of covariation. Marked shape changes with which angles covary concentrate at the supraoccipital bone, the cranial base and the antorbital window, whereas the plane measuring the orientation of the anterior portion of the rostrum does not intervene. Statistical covariance between elements of the caudal cranial base and the occiput inplies that morphological integration underlies avian skull macroevolutionary organization as a by-product of the regional concordance of such correlated elements within the early embryonic chordal domain of mesodermic origin.

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)

Deformed Skull Morphology Is Caused by the Combined Effects of the Maldevelopment of Calvarias, Cranial Base and Brain in FGFR2-P253R Mice Mimicking Human Apert Syndrome.

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Luo, Fengtao; Xie, Yangli; Xu, Wei; Huang, Junlan; Zhou, Siru; Wang, Zuqiang; Luo, Xiaoqing; Liu, Mi; Chen, Lin; Du, Xiaolan

2017-01-01

Apert syndrome (AS) is a common genetic syndrome in humans characterized with craniosynostosis. Apert patients and mouse models showed abnormalities in sutures, cranial base and brain, that may all be involved in the pathogenesis of skull malformation of Apert syndrome. To distinguish the differential roles of these components of head in the pathogenesis of the abnormal skull morphology of AS, we generated mouse strains specifically expressing mutant FGFR2 in chondrocytes, osteoblasts, and progenitor cells of central nervous system (CNS) by crossing Fgfr2 +/P253R-Neo mice with Col2a1-Cre, Osteocalcin-Cre (OC-Cre), and Nestin-Cre mice, respectively. We then quantitatively analyzed the skull and brain morphology of these mutant mice by micro-CT and micro-MRI using Euclidean distance matrix analysis (EDMA). Skulls of Col2a1-Fgfr2 +/P253R mice showed Apert syndrome-like dysmorphology, such as shortened skull dimensions along the rostrocaudal axis, shortened nasal bone, and evidently advanced ossification of cranial base synchondroses. The OC-Fgfr2 +/P253R mice showed malformation in face at 8-week stage. Nestin-Fgfr2 +/P253R mice exhibited increased dorsoventral height and rostrocaudal length on the caudal skull and brain at 8 weeks. Our study indicates that the abnormal skull morphology of AS is caused by the combined effects of the maldevelopment in calvarias, cranial base, and brain tissue. These findings further deepen our knowledge about the pathogenesis of the abnormal skull morphology of AS, and provide new clues for the further analyses of skull phenotypes and clinical management of AS.

The conductivity of neonatal piglet skulls

International Nuclear Information System (INIS)

Pant, Shilpa; Te, Tang; Tucker, Aaron; Sadleir, Rosalind J

2011-01-01

We report the first measured values of conductivities for neonatal mammalian skull samples. We measured the average radial (normal to the skull surface) conductivity of fresh neonatal piglet skull samples at 1 kHz and found it to be around 30 mS m −1 at ambient room temperatures of about 23 °C. Measurements were made on samples of either frontal or parietal cranial bone, using a saline-filled cell technique. The conductivity value we observed was approximately twice the values reported for adult skulls (Oostendorp et al 2000 IEEE Trans. Biomed. Eng. 47 1487–92) using a similar technique, but at a frequency of around 5 Hz. Further, we found that the conductivity of skull fragments increased linearly with thickness. We found evidence that this was related to differences in composition between the frontal and parietal bone samples tested, which we believe is because frontal bones contained a larger fraction of higher conductivity cancellous bone material

A Novel Augmented Reality Navigation System for Endoscopic Sinus and Skull Base Surgery: A Feasibility Study

Science.gov (United States)

Li, Liang; Yang, Jian; Chu, Yakui; Wu, Wenbo; Xue, Jin; Liang, Ping; Chen, Lei

2016-01-01

Objective To verify the reliability and clinical feasibility of a self-developed navigation system based on an augmented reality technique for endoscopic sinus and skull base surgery. Materials and Methods In this study we performed a head phantom and cadaver experiment to determine the display effect and accuracy of our navigational system. We compared cadaver head-based simulated operations, the target registration error, operation time, and National Aeronautics and Space Administration Task Load Index scores of our navigation system to conventional navigation systems. Results The navigation system developed in this study has a novel display mode capable of fusing endoscopic images to three-dimensional (3-D) virtual images. In the cadaver head experiment, the target registration error was 1.28 ± 0.45 mm, which met the accepted standards of a navigation system used for nasal endoscopic surgery. Compared with conventional navigation systems, the new system was more effective in terms of operation time and the mental workload of surgeons, which is especially important for less experienced surgeons. Conclusion The self-developed augmented reality navigation system for endoscopic sinus and skull base surgery appears to have advantages that outweigh those of conventional navigation systems. We conclude that this navigational system will provide rhinologists with more intuitive and more detailed imaging information, thus reducing the judgment time and mental workload of surgeons when performing complex sinus and skull base surgeries. Ultimately, this new navigational system has potential to increase the quality of surgeries. In addition, the augmented reality navigational system could be of interest to junior doctors being trained in endoscopic techniques because it could speed up their learning. However, it should be noted that the navigation system serves as an adjunct to a surgeon’s skills and knowledge, not as a substitute. PMID:26757365

A Novel Augmented Reality Navigation System for Endoscopic Sinus and Skull Base Surgery: A Feasibility Study.

Directory of Open Access Journals (Sweden)

Liang Li

Full Text Available To verify the reliability and clinical feasibility of a self-developed navigation system based on an augmented reality technique for endoscopic sinus and skull base surgery.In this study we performed a head phantom and cadaver experiment to determine the display effect and accuracy of our navigational system. We compared cadaver head-based simulated operations, the target registration error, operation time, and National Aeronautics and Space Administration Task Load Index scores of our navigation system to conventional navigation systems.The navigation system developed in this study has a novel display mode capable of fusing endoscopic images to three-dimensional (3-D virtual images. In the cadaver head experiment, the target registration error was 1.28 ± 0.45 mm, which met the accepted standards of a navigation system used for nasal endoscopic surgery. Compared with conventional navigation systems, the new system was more effective in terms of operation time and the mental workload of surgeons, which is especially important for less experienced surgeons.The self-developed augmented reality navigation system for endoscopic sinus and skull base surgery appears to have advantages that outweigh those of conventional navigation systems. We conclude that this navigational system will provide rhinologists with more intuitive and more detailed imaging information, thus reducing the judgment time and mental workload of surgeons when performing complex sinus and skull base surgeries. Ultimately, this new navigational system has potential to increase the quality of surgeries. In addition, the augmented reality navigational system could be of interest to junior doctors being trained in endoscopic techniques because it could speed up their learning. However, it should be noted that the navigation system serves as an adjunct to a surgeon's skills and knowledge, not as a substitute.

Benchmarking Distance Control and Virtual Drilling for Lateral Skull Base Surgery.

Science.gov (United States)

Voormolen, Eduard H J; Diederen, Sander; van Stralen, Marijn; Woerdeman, Peter A; Noordmans, Herke Jan; Viergever, Max A; Regli, Luca; Robe, Pierre A; Berkelbach van der Sprenkel, Jan Willem

2018-01-01

Novel audiovisual feedback methods were developed to improve image guidance during skull base surgery by providing audiovisual warnings when the drill tip enters a protective perimeter set at a distance around anatomic structures ("distance control") and visualizing bone drilling ("virtual drilling"). To benchmark the drill damage risk reduction provided by distance control, to quantify the accuracy of virtual drilling, and to investigate whether the proposed feedback methods are clinically feasible. In a simulated surgical scenario using human cadavers, 12 unexperienced users (medical students) drilled 12 mastoidectomies. Users were divided into a control group using standard image guidance and 3 groups using distance control with protective perimeters of 1, 2, or 3 mm. Damage to critical structures (sigmoid sinus, semicircular canals, facial nerve) was assessed. Neurosurgeons performed another 6 mastoidectomy/trans-labyrinthine and retro-labyrinthine approaches. Virtual errors as compared with real postoperative drill cavities were calculated. In a clinical setting, 3 patients received lateral skull base surgery with the proposed feedback methods. Users drilling with distance control protective perimeters of 3 mm did not damage structures, whereas the groups using smaller protective perimeters and the control group injured structures. Virtual drilling maximum cavity underestimations and overestimations were 2.8 ± 0.1 and 3.3 ± 0.4 mm, respectively. Feedback methods functioned properly in the clinical setting. Distance control reduced the risks of drill damage proportional to the protective perimeter distance. Errors in virtual drilling reflect spatial errors of the image guidance system. These feedback methods are clinically feasible. Copyright © 2017 Elsevier Inc. All rights reserved.

Skull development in the muscular dystrophic mouse

DEFF Research Database (Denmark)

Vilmann, H; Kirkeby, S; Moss, M L

1989-01-01

Roentgencephalometric tracings of skulls of 7-week-old normal and muscular dystrophic mice were compared. A marked size reduction of the dystrophic skulls relative to the normal ones was observed. However, the visceral parts of the dystrophic skull were more reduced in size than the neural parts....

Clinical anatomy and imaging of the cranial nerves and skull base.

Science.gov (United States)

Jha, Ruchira M; Klein, Joshua P

2012-09-01

Evaluation of patients with cranial neuropathies requires an understanding of brainstem anatomy and nerve pathways. Advances in neuroimaging, particularly high spatial resolution magnetic resonance imaging (MRI), have enabled visualization of these tiny structures and their related pathology. This review provides an approach toward using imaging in the evaluation of cranial nerve (CN) and skull base anatomy and pathology. Because brainstem nuclei are inextricably linked to the information contained within CNs, they are briefly mentioned whenever relevant; however, a comprehensive discussion of brainstem syndromes is beyond the scope of this review. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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

Osteointegration of a bisphenol-a-glycidyl-dimethacrylate composite and its use in anterior skull base defects: an experimental study in an experimental design model of cerebrospinal fluid leak.

Science.gov (United States)

Sanus, Galip Zihni; Kucukyuruk, Baris; Biceroglu, Huseyin; Isler, Cihan; Tanriverdi, Taner; Bas, Ahmet; Albayram, Sait; Kurkcu, Mehmet; Oz, Buge

2014-07-01

Promising clinical results were reported in watertight closure of anterior skull base defects (ASBDs) with bisphenol-a-glycidyl-dimethacrylate (bis-GMA)-based materials to prevent the cerebrospinal fluid leaks. However, interrelation of these materials with surrounding bones in histologic level, referred to as the osteointegration, has not been reported in the anterior skull base. In addition, an illustrative case with an ASBD that was repaired using a bis-GMA composite has been presented. Twenty New Zealand rabbits were divided into 4 groups: control and sham groups consisted of 2 and 6 rabbits, respectively. The "skull base defect" group (n = 6) underwent a unifrontal craniectomy and an iatrogenic ASBD followed by creating a dural defect to obtain a cerebrospinal fluid leak. Similar bony and dural defects were acquired in the "repair with bis-GMA based allograft" group (n = 6), but the bony defect was closed with bis-GMA-based allograft. All animals in the "skull base defect" group died in 3 weeks after surgery. There were no animal losses in the "repair with bis-GMA based allograft" group at the sixth month. Histologic evaluation revealed complete osteointegration of bis-GMA composite with surrounding bones. bis-GMA based allograft achieved a watertight repair of the ASBD. Histologic findings of this study showed that bis-GMA composite is a reliable material to be used in the closure of anterior skull base bony defects.

Analysis of six Vietnamese trophy skulls.

Science.gov (United States)

Sledzik, P S; Ousley, S

1991-03-01

This report presents morphologic, metric, and contextual information on six documented trophy skull specimens confiscated from U.S. servicemen during the Vietnam War. Additional information on the history and occurrence of trophy skull collecting is provided. This sample, consisting mostly of young Vietnamese males, exhibits graffiti, painting, and other evidence of postmortem decorative modification. Identification of trophy skulls is important to medicolegal and anthropological researchers in distinguishing trophy remains from archaeological and forensic specimens.

Numerical evaluation of the skull for human neuromodulation with transcranial focused ultrasound

Science.gov (United States)

Mueller, Jerel K.; Ai, Leo; Bansal, Priya; Legon, Wynn

2017-12-01

Objective. Transcranial focused ultrasound is an emerging field for human non-invasive neuromodulation, but its dosing in humans is difficult to know due to the skull. The objective of the present study was to establish modeling methods based on medical images to assess skull differences between individuals on the wave propagation of ultrasound. Approach. Computational models of transcranial focused ultrasound were constructed using CT and MR scans to solve for intracranial pressure. We explored the effect of including the skull base in models, different transducer placements on the head, and differences between 250 kHz or 500 kHz acoustic frequency for both female and male models. We further tested these features using linear, nonlinear, and elastic simulations. To better understand inter-subject skull thickness and composition effects we evaluated the intracranial pressure maps between twelve individuals at two different skull sites. Main results. Nonlinear acoustic simulations resulted in virtually identical intracranial pressure maps with linear acoustic simulations. Elastic simulations showed a difference in max pressures and full width half maximum volumes of 15% at most. Ultrasound at an acoustic frequency of 250 kHz resulted in the creation of more prominent intracranial standing waves compared to 500 kHz. Finally, across twelve model human skulls, a significant linear relationship to characterize intracranial pressure maps was not found. Significance. Despite its appeal, an inherent problem with the use of a noninvasive transcranial ultrasound method is the difficulty of knowing intracranial effects because of the skull. Here we develop detailed computational models derived from medical images of individuals to simulate the propagation of neuromodulatory ultrasound across the skull and solve for intracranial pressure maps. These methods allow for a much better understanding of the intracranial effects of ultrasound for an individual in order to

Can skull form predict the shape of the temporomandibular joint? A study using geometric morphometrics on the skulls of wolves and domestic dogs.

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Curth, Stefan; Fischer, Martin S; Kupczik, Kornelius

2017-11-01

The temporomandibular joint (TMJ) conducts and restrains masticatory movements between the mammalian cranium and the mandible. Through this functional integration, TMJ morphology in wild mammals is strongly correlated with diet, resulting in a wide range of TMJ variations. However, in artificially selected and closely related domestic dogs, dietary specialisations between breeds can be ruled out as a diversifying factor although they display an enormous variation in TMJ morphology. This raises the question of the origin of this variation. Here we hypothesise that, even in the face of reduced functional demands, TMJ shape in dogs can be predicted by skull form; i.e. that the TMJ is still highly integrated in the dog skull. If true, TMJ variation in the dog would be a plain by-product of the enormous cranial variation in dogs and its genetic causes. We addressed this hypothesis using geometric morphometry on a data set of 214 dog and 60 wolf skulls. We digitized 53 three-dimensional landmarks of the skull and the TMJ on CT-based segmentations and compared (1) the variation between domestic dog and wolf TMJs (via principal component analysis) and (2) the pattern of covariation of skull size, flexion and rostrum length with TMJ shape (via regression of centroid size on shape and partial least squares analyses). We show that the TMJ in domestic dogs is significantly more diverse than in wolves: its shape covaries significantly with skull size, flexion and rostrum proportions in patterns which resemble those observed in primates. Similar patterns in canids, which are carnivorous, and primates, which are mostly frugivorous imply the existence of basic TMJ integration patterns which are independent of dietary adaptations. However, only limited amounts of TMJ variation in dogs can be explained by simple covariation with overall skull geometry. This implies that the final TMJ shape is gained partially independently of the rest of the skull. Copyright © 2017 Elsevier Gmb

A dual resolution measurement based Monte Carlo simulation technique for detailed dose analysis of small volume organs in the skull base region

International Nuclear Information System (INIS)

Yeh, Chi-Yuan; Tung, Chuan-Jung; Chao, Tsi-Chain; Lin, Mu-Han; Lee, Chung-Chi

2014-01-01

The purpose of this study was to examine dose distribution of a skull base tumor and surrounding critical structures in response to high dose intensity-modulated radiosurgery (IMRS) with Monte Carlo (MC) simulation using a dual resolution sandwich phantom. The measurement-based Monte Carlo (MBMC) method (Lin et al., 2009) was adopted for the study. The major components of the MBMC technique involve (1) the BEAMnrc code for beam transport through the treatment head of a Varian 21EX linear accelerator, (2) the DOSXYZnrc code for patient dose simulation and (3) an EPID-measured efficiency map which describes non-uniform fluence distribution of the IMRS treatment beam. For the simulated case, five isocentric 6 MV photon beams were designed to deliver a total dose of 1200 cGy in two fractions to the skull base tumor. A sandwich phantom for the MBMC simulation was created based on the patient's CT scan of a skull base tumor [gross tumor volume (GTV)=8.4 cm 3 ] near the right 8th cranial nerve. The phantom, consisted of a 1.2-cm thick skull base region, had a voxel resolution of 0.05×0.05×0.1 cm 3 and was sandwiched in between 0.05×0.05×0.3 cm 3 slices of a head phantom. A coarser 0.2×0.2×0.3 cm 3 single resolution (SR) phantom was also created for comparison with the sandwich phantom. A particle history of 3×10 8 for each beam was used for simulations of both the SR and the sandwich phantoms to achieve a statistical uncertainty of <2%. Our study showed that the planning target volume (PTV) receiving at least 95% of the prescribed dose (VPTV95) was 96.9%, 96.7% and 99.9% for the TPS, SR, and sandwich phantom, respectively. The maximum and mean doses to large organs such as the PTV, brain stem, and parotid gland for the TPS, SR and sandwich MC simulations did not show any significant difference; however, significant dose differences were observed for very small structures like the right 8th cranial nerve, right cochlea, right malleus and right semicircular

Broadband acoustic properties of a murine skull.

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Estrada, Héctor; Rebling, Johannes; Turner, Jake; Razansky, Daniel

2016-03-07

It has been well recognized that the presence of a skull imposes harsh restrictions on the use of ultrasound and optoacoustic techniques in the study, treatment and modulation of the brain function. We propose a rigorous modeling and experimental methodology for estimating the insertion loss and the elastic constants of the skull over a wide range of frequencies and incidence angles. A point-source-like excitation of ultrawideband acoustic radiation was induced via the absorption of nanosecond duration laser pulses by a 20 μm diameter microsphere. The acoustic waves transmitted through the skull are recorded by a broadband, spherically focused ultrasound transducer. A coregistered pulse-echo ultrasound scan is subsequently performed to provide accurate skull geometry to be fed into an acoustic transmission model represented in an angular spectrum domain. The modeling predictions were validated by measurements taken from a glass cover-slip and ex vivo adult mouse skulls. The flexible semi-analytical formulation of the model allows for seamless extension to other transducer geometries and diverse experimental scenarios involving broadband acoustic transmission through locally flat solid structures. It is anticipated that accurate quantification and modeling of the skull transmission effects would ultimately allow for skull aberration correction in a broad variety of applications employing transcranial detection or transmission of high frequency ultrasound.

Three-dimensional bone-free computed tomographic angiography of aneurysms near the skull base using a new bone-removal application

International Nuclear Information System (INIS)

Tomura, Noriaki; Otani, Takahiro; Sakuma, Ikuo; Takahashi, Satoshi; Nishii, Toshiaki; Watarai, Jiro

2009-01-01

Bone elimination is needed for computed tomography angiography (CTA) because bone structures obscure aneurysms located at the skull base. The purpose of our study was to evaluate the efficacy of three-dimensional (3D)-CTA using an application for bone elimination. A total of 27 patients with 32 angiographically confirmed aneurysms near the skull base were investigated. The 3D maximum intensity projection (MIP) images were initially obtained using the application. Further postprocessing was performed to obtain the MIP and volume-rendered (VR) images. The quality of the initial MIP images by the application was analyzed. Visualization of aneurysms after further processing was also reviewed. The initial MIP images by the application showed almost bone-free images in 23 of the 27 patients. In 8 patients, the image of the internal carotid artery (ICA) was segmentally removed in the initial MIP images by the application. Further postprocessing was able to recover all loss of the ICA image in these eight patients. For visualizing aneurysms and their necks, VR images with the application were significantly superior to VR images without the application. The application for bone elimination allows fast, selective elimination of bony structures and can improve the interpretation of aneurysms near the skull base. (author)

Congenital muscle dystrophy and diet consistency affect mouse skull shape differently.

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Spassov, Alexander; Toro-Ibacache, Viviana; Krautwald, Mirjam; Brinkmeier, Heinrich; Kupczik, Kornelius

2017-11-01

The bones of the mammalian skull respond plastically to changes in masticatory function. However, the extent to which muscle function affects the growth and development of the skull, whose regions have different maturity patterns, remains unclear. Using muscle dissection and 3D landmark-based geometric morphometrics we investigated the effect of changes in muscle function established either before or after weaning, on skull shape and muscle mass in adult mice. We compared temporalis and masseter mass and skull shape in mice with a congenital muscle dystrophy (mdx) and wild type (wt) mice fed on either a hard or a soft diet. We found that dystrophy and diet have distinct effects on the morphology of the skull and the masticatory muscles. Mdx mice show a flattened neurocranium with a more dorsally displaced foramen magnum and an anteriorly placed mandibular condyle compared with wt mice. Compared with hard diet mice, soft diet mice had lower masseter mass and a face with more gracile features as well as labially inclined incisors, suggesting reduced bite strength. Thus, while the early-maturing neurocranium and the posterior portion of the mandible are affected by the congenital dystrophy, the late-maturing face including the anterior part of the mandible responds to dietary differences irrespective of the mdx mutation. Our study confirms a hierarchical, tripartite organisation of the skull (comprising neurocranium, face and mandible) with a modular division based on development and function. Moreover, we provide further experimental evidence that masticatory loading is one of the main environmental stimuli that generate craniofacial variation. © 2017 Anatomical Society.

Anatomical features of skull base and oral cavity: a pilot study to determine the accessibility of the sella by transoral robotic-assisted surgery.

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Amelot, Aymeric; Trunet, Stephanie; Degos, Vincent; André, Olivier; Dionnet, Aurore; Cornu, Philippe; Hans, Stéphane; Chauvet, Dorian

2015-10-01

The role of transoral robotic surgery (TORS) in the skull base emerges and represents the natural progression toward miniinvasive resections in confined spaces. The accessibility of the sella via TORS has been recently described on fresh human cadavers. An anatomic study is mandatory to know if this approach would be feasible in the majority of patients regardless of their oral morphological features. From 30 skull base CT scans from patients who were asked to open their mouth as wide as they can, we measured specific dimensions of the oral cavity and the skull base, such as length of the palate, mouth opening and distance from the sella to the palate. All data were acquired on a sagittal midline plane and on a 25° rotation plane, which simulated the axis of the robotic instruments. Looking at the projection of the dental palatine line on the sella, we studied possible predictive factors of sellar accessibility and tried to bring objective data for surgical feasibility. We also proposed an angle α to study the working angle at the skull base. We observed that the maximal mouth opening was a good predictive factor of sellar accessibility by TORS (p < 0.05). The mouth aperture threshold value for a good sensitivity, over 80 %, was comparable to the mean value of mouth opening in our series, 38.9 and 39.4 mm respectively. Moreover, we showed a statistically significant increase of the working angle α at the skull base comparing the lateral access to the midline one (p < 0.05). This seemed to quantitatively demonstrate that the robotic arms placed at the labial commissure of the mouth can reach the sella. From these anatomical features and previous cadaveric dissections, we assume that TORS may be feasible on a majority of patients to remove pituitary adenomas.

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

Energy Technology Data Exchange (ETDEWEB)

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

Skull penetrating wound

International Nuclear Information System (INIS)

Gonzalez Orlandi, Yvei; Junco Martin, Reinel; Rojas Manresa, Jorge; Duboy Limonta, Victor; Matos Herrera, Omar; Saez Corvo, Yunet

2011-01-01

The cranioencephalic trauma is common in the emergence centers to care for patients with multiple traumata and it becames in a health problem in many countries. Skull penetrating trauma is located in a special place due to its low frequency. In present paper a case of male patient aged 52 severely skull-injured with penetrating wound caused by a cold steel that remained introduced into the left frontotemporal region. After an imaging study the emergence surgical treatment was applied and patient evolves adequately after 25 days of hospitalization. Nowadays, she is under rehabilitation treatment due to a residual right hemiparesis.(author)

Chronic imaging through "transparent skull" in mice.

Directory of Open Access Journals (Sweden)

Anna Steinzeig

Full Text Available Growing interest in long-term visualization of cortical structure and function requires methods that allow observation of an intact cortex in longitudinal imaging studies. Here we describe a detailed protocol for the "transparent skull" (TS preparation based on skull clearing with cyanoacrylate, which is applicable for long-term imaging through the intact skull in mice. We characterized the properties of the TS in imaging of intrinsic optical signals and compared them with the more conventional cranial window preparation. Our results show that TS is less invasive, maintains stabile transparency for at least two months, and compares favorably to data obtained from the conventional cranial window. We applied this method to experiments showing that a four-week treatment with the antidepressant fluoxetine combined with one week of monocular deprivation induced a shift in ocular dominance in the mouse visual cortex, confirming that fluoxetine treatment restores critical-period-like plasticity. Our results demonstrate that the TS preparation could become a useful method for long-term visualization of the living mouse brain.

Sexual dimorphism of pyrenean chamois (Rupicapra p. pyrenaica based on skull morphometry

Directory of Open Access Journals (Sweden)

García-González, R.

2002-12-01

Full Text Available Sexual dimorphism in skull characteristics of Pyrenean chamois is studied in a sample of 85 adults (36 males and 49 females by means of 26 quantitative variables. Skull variables were analised by multiple regression and principal component techniques. The Pyrenean chamois shows one of the smallest sexual skull dimorphisms of the Rupicapra subspecies. Only length, thickness, and related variables of horns present significant differences between sexes. Nevertheless, horn height was statistically identical in both sexes. Ecological implications of skull variability and skull variables relationships are discussed. Several discriminant functions were developed by means of discriminant analysis. Those that better identified sexes include horn core diameters. We also developed other functions based on upper skull variables that could be used to identify incomplete specimens or archaeological remains.

[fr]
Nous avons étudié les dimorphismes sexuels de l'isard en travaillant sur 26 mesures cranéométriques issues d'un échantillon de 85 crânes adultes (soit 36 mâles et 49 femelles. Les analyses ont porté sur la comparaison des moyennes, la régression multiple et l'analyse en composante principale. Il s'avère que l'isard pyrénéen présente un des plus bas dimorphisme craneal du genre Rupicapra. Seuls la longueur et l'épaisseur des cornes et leurs variables associées ont montré des différences significatives entre les sexes. Les implications écologiques de la variabilité entre les crânes et des relations entre les variables cranéométriques sont discutées. En utilisant l'analyse discriminante, nous arrivons à développer quelques fonctions nous permettant d'identifier le sexe des exemplaires complets ou incomplets. Les fonctions se basant sur l'épaisseur des cornes ont permis une meilleure classification.
[es]
Se ha estudiado el dimorfismo sexual del sarrio a partir de 26 medidas craneométricas tomadas en una

A clinical pilot study of a modular video-CT augmentation system for image-guided skull base surgery

Science.gov (United States)

Liu, Wen P.; Mirota, Daniel J.; Uneri, Ali; Otake, Yoshito; Hager, Gregory; Reh, Douglas D.; Ishii, Masaru; Gallia, Gary L.; Siewerdsen, Jeffrey H.

2012-02-01

Augmentation of endoscopic video with preoperative or intraoperative image data [e.g., planning data and/or anatomical segmentations defined in computed tomography (CT) and magnetic resonance (MR)], can improve navigation, spatial orientation, confidence, and tissue resection in skull base surgery, especially with respect to critical neurovascular structures that may be difficult to visualize in the video scene. This paper presents the engineering and evaluation of a video augmentation system for endoscopic skull base surgery translated to use in a clinical study. Extension of previous research yielded a practical system with a modular design that can be applied to other endoscopic surgeries, including orthopedic, abdominal, and thoracic procedures. A clinical pilot study is underway to assess feasibility and benefit to surgical performance by overlaying CT or MR planning data in realtime, high-definition endoscopic video. Preoperative planning included segmentation of the carotid arteries, optic nerves, and surgical target volume (e.g., tumor). An automated camera calibration process was developed that demonstrates mean re-projection accuracy (0.7+/-0.3) pixels and mean target registration error of (2.3+/-1.5) mm. An IRB-approved clinical study involving fifteen patients undergoing skull base tumor surgery is underway in which each surgery includes the experimental video-CT system deployed in parallel to the standard-of-care (unaugmented) video display. Questionnaires distributed to one neurosurgeon and two otolaryngologists are used to assess primary outcome measures regarding the benefit to surgical confidence in localizing critical structures and targets by means of video overlay during surgical approach, resection, and reconstruction.

REVIEW OF NASA FIBROMYXOMA: Aggressive Behaviour? (Case Report

Directory of Open Access Journals (Sweden)

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.

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

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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.

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

Energy Technology Data Exchange (ETDEWEB)

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

21 CFR 882.4460 - Neurosurgical head holder (skull clamp).

Science.gov (United States)

2010-04-01

... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Neurosurgical head holder (skull clamp). 882.4460... holder (skull clamp). (a) Identification. A neurosurgical head holder (skull clamp) is a device used to clamp the patient's skull to hold head and neck in a particular position during surgical procedures. (b...

A Three-Dimensional Statistical Average Skull: Application of Biometric Morphing in Generating Missing Anatomy.

Science.gov (United States)

Teshima, Tara Lynn; Patel, Vaibhav; Mainprize, James G; Edwards, Glenn; Antonyshyn, Oleh M

2015-07-01

The utilization of three-dimensional modeling technology in craniomaxillofacial surgery has grown exponentially during the last decade. Future development, however, is hindered by the lack of a normative three-dimensional anatomic dataset and a statistical mean three-dimensional virtual model. The purpose of this study is to develop and validate a protocol to generate a statistical three-dimensional virtual model based on a normative dataset of adult skulls. Two hundred adult skull CT images were reviewed. The average three-dimensional skull was computed by processing each CT image in the series using thin-plate spline geometric morphometric protocol. Our statistical average three-dimensional skull was validated by reconstructing patient-specific topography in cranial defects. The experiment was repeated 4 times. In each case, computer-generated cranioplasties were compared directly to the original intact skull. The errors describing the difference between the prediction and the original were calculated. A normative database of 33 adult human skulls was collected. Using 21 anthropometric landmark points, a protocol for three-dimensional skull landmarking and data reduction was developed and a statistical average three-dimensional skull was generated. Our results show the root mean square error (RMSE) for restoration of a known defect using the native best match skull, our statistical average skull, and worst match skull was 0.58, 0.74, and 4.4  mm, respectively. The ability to statistically average craniofacial surface topography will be a valuable instrument for deriving missing anatomy in complex craniofacial defects and deficiencies as well as in evaluating morphologic results of surgery.

Growth of the skull in young children in Baotou, China.

Science.gov (United States)

Hou, Hai-dong; Liu, Ming; Gong, Ke-rui; Shao, Guo; Zhang, Chun-Yang

2014-09-01

There are some controversies about the optimal time to perform skull repair in very young Chinese children because of the rapid skull growth in this stage of life. The purpose of this current study is to describe the characteristics of skull growth and to discuss the optimal time for skull repair in young Chinese children with skull defects. A total of 112 children born in the First Affiliated Hospital of Baotou Medical College were measured for six consecutive years starting in 2006. Cranial length (CL, linear distance between the eyebrows to the pillow tuberosity), cranial width (CW, double-sided linear distance of connection of external auditory canal), ear over the top line (EOTL), the eyebrows-the posterior tuberosity line (EPTL), and head circumference (HC) were measured to describe the skull growth. The most rapid period of skull growth occurs during the first year of life. The second and third most rapid periods are the second and third years, respectively. Then, the skull growth slowed and the values of the skull growth index of 6-year-old children were close to those of adults. Children 0-1 years old should not receive skull repair due to their rapid skull growth. The indexes of children 3 years old or older were close to those of the adult; therefore, 3 years old or older may receive skull repair.

The Development of Skull Prosthesis Through Active Contour Model.

Science.gov (United States)

Chen, Yi-Wen; Shih, Cheng-Ting; Cheng, Chen-Yang; Lin, Yu-Cheng

2017-09-09

Skull defects result in brain infection and inadequate brain protection and pose a general danger to patient health. To avoid these situations and prevent re-injury, a prosthesis must be constructed and grafted onto the deficient region. With the development of rapid customization through additive manufacturing and 3D printing technology, skull prostheses can be fabricated accurately and efficiently prior to cranioplasty. However, an unfitted skull prosthesis made with a metal implant can cause repeated infection, potentially necessitating secondary surgery. This paper presents a method of creating suitably geometric graphics of skull defects to be applied in skull repair through active contour models. These models can be adjusted in each computed tomography slice according to the graphic features, and the curves representing the skull defect can be modeled. The generated graphics can adequately mimic the natural curvature of the complete skull. This method will enable clinical surgeons to rapidly implant customized prostheses, which is of particular importance in emergency surgery. The findings of this research can help surgeons provide patients with skull defects with treatment of the highest quality.

Automated human skull landmarking with 2D Gabor wavelets

Science.gov (United States)

de Jong, Markus A.; Gül, Atilla; de Gijt, Jan Pieter; Koudstaal, Maarten J.; Kayser, Manfred; Wolvius, Eppo B.; Böhringer, Stefan

2018-05-01

Landmarking of CT scans is an important step in the alignment of skulls that is key in surgery planning, pre-/post-surgery comparisons, and morphometric studies. We present a novel method for automatically locating anatomical landmarks on the surface of cone beam CT-based image models of human skulls using 2D Gabor wavelets and ensemble learning. The algorithm is validated via human inter- and intra-rater comparisons on a set of 39 scans and a skull superimposition experiment with an established surgery planning software (Maxilim). Automatic landmarking results in an accuracy of 1–2 mm for a subset of landmarks around the nose area as compared to a gold standard derived from human raters. These landmarks are located in eye sockets and lower jaw, which is competitive with or surpasses inter-rater variability. The well-performing landmark subsets allow for the automation of skull superimposition in clinical applications. Our approach delivers accurate results, has modest training requirements (training set size of 30–40 items) and is generic, so that landmark sets can be easily expanded or modified to accommodate shifting landmark interests, which are important requirements for the landmarking of larger cohorts.

A QI Initiative to Reduce Hospitalization for Children With Isolated Skull Fractures.

Science.gov (United States)

Lyons, Todd W; Stack, Anne M; Monuteaux, Michael C; Parver, Stephanie L; Gordon, Catherine R; Gordon, Caroline D; Proctor, Mark R; Nigrovic, Lise E

2016-06-01

Although children with isolated skull fractures rarely require acute interventions, most are hospitalized. Our aim was to safely decrease the hospitalization rate for children with isolated skull fractures. We designed and executed this multifaceted quality improvement (QI) initiative between January 2008 and July 2015 to reduce hospitalization rates for children ≤21 years old with isolated skull fractures at a single tertiary care pediatric institution. We defined an isolated skull fracture as a skull fracture without intracranial injury. The QI intervention consisted of 2 steps: (1) development and implementation of an evidence-based guideline, and (2) dissemination of a provider survey designed to reinforce guideline awareness and adherence. Our primary outcome was hospitalization rate and our balancing measure was hospital readmission within 72 hours. We used standard statistical process control methodology to assess change over time. To assess for secular trends, we examined admission rates for children with an isolated skull fracture in the Pediatric Health Information System administrative database. We identified 321 children with an isolated skull fracture with a median age of 11 months (interquartile range 5-16 months). The baseline admission rate was 71% (179/249, 95% confidence interval, 66%-77%) and decreased to 46% (34/72, 95% confidence interval, 35%-60%) after implementation of our QI initiative. No child was readmitted after discharge. The admission rate in our secular trend control group remained unchanged at 78%. We safely reduced the hospitalization rate for children with isolated skull fractures without an increase in the readmissions. Copyright © 2016 by the American Academy of Pediatrics.

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

Institute of Scientific and Technical Information of China (English)

2011-01-01

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

Independent instances of "souvenir" Asian skulls from the Tampa Bay area.

Science.gov (United States)

Wienker, C W; Wood, J E; Diggs, C A

1990-05-01

In the summer of 1984, police in Pinellas County, Florida, confiscated six identically colored imported Asian skulls (in a shipping case) from a private citizen. In May 1988, in nearby Hillsborough County, police confiscated a very similar skull from another private citizen, who allegedly had found it in an abandoned house. Aside from slight color differences between the six found in Pinellas County and the one found in Hillsborough County, the skulls are virtually identical in their osteological characteristics and condition and in the vital statistics derived from each. Each skull is as clean and dry as those typically sold by commercial scientific supply outlets in the United States. Each is edentulous (primarily premortem), between approximately 20 and 60 years of age at death, and morphologically Asian. Five of the seven are morphologically male, one is morphologically female, and one is a mosaic with respect to gender-related features. Police, medical examiners, coroners, and forensic anthropologists should be aware of such "souvenir" specimens, in the event that they encounter similar skulls. Discriminant function analyses for race and sex yield considerably conflicting results, which underscores the need for using extreme caution when interpreting forensic science estimates based on such techniques.

21 CFR 882.5960 - Skull tongs for traction.

Science.gov (United States)

2010-04-01

... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Skull tongs for traction. 882.5960 Section 882...) MEDICAL DEVICES NEUROLOGICAL DEVICES Neurological Therapeutic Devices § 882.5960 Skull tongs for traction. (a) Identification. Skull tongs for traction is an instrument used to immobilize a patient with a...

Skull thickness in patients with clefts

DEFF Research Database (Denmark)

Arntsen, T; Kjaer, I; Sonnesen, L

2010-01-01

The purpose was to analyze skull thickness in incomplete cleft lip (CL), cleft palate (CP), and combined cleft lip and palate (UCLP).......The purpose was to analyze skull thickness in incomplete cleft lip (CL), cleft palate (CP), and combined cleft lip and palate (UCLP)....

Fractionated stereotactic radiosurgery for patients with skull base metastases from systemic cancer involving the anterior visual pathway

International Nuclear Information System (INIS)

Minniti, Giuseppe; Osti, Mattia Falchetto; Maurizi Enrici, Riccardo; Esposito, Vincenzo; Clarke, Enrico; Scaringi, Claudia; Bozzao, Alessandro; Falco, Teresa; De Sanctis, Vitaliana; Enrici, Maurizio Maurizi; Valeriani, Maurizio

2014-01-01

To analyze the tumor control, survival outcomes, and toxicity after stereotactic radiosurgery (SRS) for skull base metastases from systemic cancer involving the anterior visual pathway. We have analyzed 34 patients (23 females and 11 males, median age 59 years) who underwent multi-fraction SRS for a skull base metastasis compressing or in close proximity of optic nerves and chiasm. All metastases were treated with frameless LINAC-based multi-fraction SRS in 5 daily fractions of 5 Gy each. Local control, distant failure, and overall survival were estimated using the Kaplan-Meier method calculated from the time of SRS. Prognostic variables were assessed using log-rank and Cox regression analyses. At a median follow-up of 13 months (range, 2–36.5 months), twenty-five patients had died and 9 were alive. The 1-year and 2-year local control rates were 89% and 72%, and respective actuarial survival rates were 63% and 30%. Four patients recurred with a median time to progression of 12 months (range, 6–27 months), and 17 patients had new brain metastases at distant brain sites. The 1-year and 2-year distant failure rates were 50% and 77%, respectively. On multivariate analysis, a Karnofsky performance status (KPS) >70 and the absence of extracranial metastases were prognostic factors associated with lower distant failure rates and longer survival. After multi-fraction SRS, 15 (51%) out of 29 patients had a clinical improvement of their preexisting cranial deficits. No patients developed radiation-induced optic neuropathy during the follow-up. Multi-fraction SRS (5 x 5 Gy) is a safe treatment option associated with good local control and improved cranial nerve symptoms for patients with a skull base metastasis involving the anterior visual pathway

Implant-retained skull prosthesis to cover a large defect of the hairy skull resulting from treatment of a basal cell carcinoma: A clinical report.

Science.gov (United States)

Hoekstra, Jitske; Vissink, Arjan; Raghoebar, Gerry M; Visser, Anita

2017-05-01

Skin carcinoma, particularly basal cell carcinoma, and its treatment can result in large defects of the hairy skull. A 53-year-old man is described who was surgically treated for a large basal cell carcinoma invading the skin and underlying tissue at the top of the hairy skull. Treatment consisted of resecting the tumor and external part of the skull bone. To protect the brain and to cover the defect of the hairy skull, an acrylic resin skull prosthesis with hair was designed to mask the defect. The skull prosthesis was retained on 8 extraoral implants placed at the margins of the defect in the skull bone. The patient was satisfied with the treatment outcome. Copyright © 2016 Editorial Council for the Journal of Prosthetic Dentistry. Published by Elsevier Inc. All rights reserved.

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

CT findings of skull tumors forming subcutaneous masses

International Nuclear Information System (INIS)

Niida, Hirohito; Takeda, Norio; Onda, Kiyoshi; Tanaka, Ryuichi

1991-01-01

Some characteristics of CT findings in 27 patients with skull tumors forming subcutaneous tumors were studied. There were sixteen metastatic skull tumors, six primary skull tumors, and five meningiomas. A CT scan was found to be helpful in the diagnosis of the lesions. Especially, bone-window CT images proved very sensitive in the detection of destructive and permeative lesions of the skull. In 19 of the 27 cases, some lytic lesions were observed. In all cases with skull metastasis from carcinomas, a complete osteolytic change of the skull was observed. Furthermore, all of the metastatic tumors from thyroid carcinoma showed well circumscribed and homogeneously enhanced lesions, in contrast with the other metastatic carcinomas, which usually showed heterogeneously enhanced lesions with irregular margins. Osteoblastic changes were characteristically observed in all cases of meningiomas, osteosarcoma, and chondrosarcoma. Meningiomas were located mainly in the intracranial region and extended extracranially. In one case of malignant lymphoma, one of a neuroblastoma, and one of leukemia, there was little or no gross cortical bone change, despite a large mass. (author)

Application of Thinned-Skull Cranial Window to Mouse Cerebral Blood Flow Imaging Using Optical Microangiography

Science.gov (United States)

Wang, Ruikang K.

2014-01-01

In vivo imaging of mouse brain vasculature typically requires applying skull window opening techniques: open-skull cranial window or thinned-skull cranial window. We report non-invasive 3D in vivo cerebral blood flow imaging of C57/BL mouse by the use of ultra-high sensitive optical microangiography (UHS-OMAG) and Doppler optical microangiography (DOMAG) techniques to evaluate two cranial window types based on their procedures and ability to visualize surface pial vessel dynamics. Application of the thinned-skull technique is found to be effective in achieving high quality images for pial vessels for short-term imaging, and has advantages over the open-skull technique in available imaging area, surgical efficiency, and cerebral environment preservation. In summary, thinned-skull cranial window serves as a promising tool in studying hemodynamics in pial microvasculature using OMAG or other OCT blood flow imaging modalities. PMID:25426632

[Laser-based quality assurance for robot-assisted milling at the base of the skull].

Science.gov (United States)

Maassen, M M; Malthan, D; Stallkamp, J; Schäfer, A; Dammann, F; Schwaderer, E; Zenner, H P

2006-02-01

Implanting active hearing devices in the lateral base of the skull requires high-precision, secure fixation of the electromagnetic transducer and long-life anchorage using osteosynthetic fixation plates referred to as mountain brackets. Nonlinear distortion in the acoustic signal path and consecutive implant loosening can only be avoided by exact osseous milling to create the necessary cavity bed while avoiding excessive milling. Robot technology is ideal for high-precision milling. However, safety measures are necessary in order to prevent errors from occurring during the reduction process. Ideally, a robot should be guided by a navigation system. However, robotic systems so far available do not yet have an integrated global navigation system. We used an animal model under laboratory conditions to examine the extent to which the semiautomatic ROBIN assistant system developed could be expected to increase osseous milling accuracy before implanting active electronic hearing devices into the recipient tissue in the cranium. An existing prototype system for robot-assisted skull base surgery was equipped with laser sensors for geometric measurement of the operation site. The three-dimensional measurement data was compared with CT simulation data before, during, and after the robot-assisted operation. The experiments were conducted on test objects as well as on animal models. Under ideal conditions, the operation site could be measured at a spatial resolution of better than 0.02 mm in each dimension. However, reflections and impurities in the operation site from bleeding and rinsing fluids did have a considerable effect on data collection, necessitating specialised registering procedures. Using an error-tolerant procedure specifically developed, the effective registering error could be kept under 0.3 mm. After milling, the resulting shape matched the intended form at an accuracy level of 0.8 mm. The results show that robot systems can reach the accuracy required for

Skulls and Human Evolution: The Use of Casts of Anthropoid Skulls in Teaching Concepts of Human Evolution.

Science.gov (United States)

Gipps, John

1991-01-01

Proposes the use of a series of 11 casts of fossil skulls as a method of teaching about the theory of human evolution. Students explore the questions of which skulls are "human" and which came first in Homo Sapien development, large brain or upright stance. (MDH)

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

Craniometric Indices of Nigeria Skulls

OpenAIRE

Orish CN; Ibeachu PC

2016-01-01

Introduction: Craniometric indices show the percentage relationship between different dimensions. It is an important parameter for classification of race and sex of individuals of unknown identity. This study was undertaken to determine the craniometric indices of gnathic, palatal, orbital, cranial and nasal indices of Nigerian skulls. Materials and Methods: One hundred adult dry skulls, (78 males, and 22 females) free from damage and deformities from eleven Departments of Anatomy in Nige...

Relevance of Whitnall's tubercle and auditory meatus in diagnosing exclusions during skull-photo superimposition.

Science.gov (United States)

Jayaprakash, Paul T; Hashim, Natassha; Yusop, Ridzuan Abd Aziz Mohd

2015-08-01

Video vision mixer based skull-photo superimposition is a popular method for identifying skulls retrieved from unidentified human remains. A report on the reliability of the superimposition method suggested increased failure rates of 17.3 to 32% to exclude and 15 to 20% to include skulls while using related and unrelated face photographs. Such raise in failures prompted an analysis of the methods employed for the research. The protocols adopted for assessing the reliability are seen to vary from those suggested by the practitioners in the field. The former include overlaying the skull- and face-images on the basis of morphology by relying on anthropometric landmarks on the front plane of the face-images and evaluating the goodness of match depending on mix-mode images; the latter consist of orienting the skull considering landmarks on both the eye and ear planes of the face- and skull-images and evaluating the match utilizing images seen in wipe-mode in addition to those in mix-mode. Superimposition of a skull with face-images of five living individuals in two sets of experiments, one following the procedure described for the research on reliability and the other applying the methods suggested by the practitioners has shown that overlaying the images on the basis of morphology depending on the landmarks on the front plane alone and assessing the match in mix-mode fails to exclude the skull. However, orienting the skull relying on the relationship between the anatomical landmarks on the skull- and face-images such as Whitnall's tubercle and exocanthus in the front (eye) plane and the porion and tragus in the rear (ear) plane as well as assessing the match using wipe-mode images enables excluding that skull while superimposing with the same set of face-images. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

The 360 photography: a new anatomical insight of the sphenoid bone. Interest for anatomy teaching and skull base surgery.

Science.gov (United States)

Jacquesson, Timothée; Mertens, Patrick; Berhouma, Moncef; Jouanneau, Emmanuel; Simon, Emile

2017-01-01

Skull base architecture is tough to understand because of its 3D complex shape and its numerous foramen, reliefs or joints. It is especially true for the sphenoid bone whom central location hinged with most of skull base components is unique. Recently, technological progress has led to develop new pedagogical tools. This way, we bought a new real-time three-dimensional insight of the sphenoid bone that could be useful for the teacher, the student and the surgeon. High-definition photography was taken all around an isolated dry skull base bone prepared with Beauchêne's technique. Pictures were then computed to provide an overview with rotation and magnification on demand. From anterior, posterior, lateral or oblique views and from in out looks, anatomical landmarks and subtleties were described step by step. Thus, the sella turcica, the optic canal, the superior orbital fissure, the sphenoid sinus, the vidian canal, pterygoid plates and all foramen were clearly placed relative to the others at each face of the sphenoid bone. In addition to be the first report of the 360 Photography tool, perspectives are promising as the development of a real-time interactive tridimensional space featuring the sphenoid bone. It allows to turn around the sphenoid bone and to better understand its own special shape, numerous foramen, neurovascular contents and anatomical relationships. This new technological tool may further apply for surgical planning and mostly for strengthening a basic anatomical knowledge firstly introduced.

MR Imaging with Gadolinium-DTPA in skull-base tumors

International Nuclear Information System (INIS)

Bartolozzi, C.; Olmastroni, M.; Dal Pozzo, G.; Petacchi, D.

1988-01-01