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Sample records for chordoma clinical characteristics

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

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

    2014-01-01

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

  2. 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

  3. Chordoma

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

    1981-12-01

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

  4. Chordoma

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

    1981-12-01

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

  5. Chordoma

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    Saxton, J.P.

    1981-07-01

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

  6. Sellar chondroid chordoma

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    Xia LI

    2014-02-01

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

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

    International Nuclear Information System (INIS)

    parameters of the time-dose-response relationship for the analyzed group of patients. For example, the maximum likelihood estimates of surviving fraction at 2Gy (SF2) are 0.47 with 95% confidence limits of [0.45-0.49] for male and 0.53 [0.51-0.55] for female, with the coefficient of inter-patient variation in SF2 of 4.3%. The density of clonogens was estimated to be 108.2 clonogens per cubic centimeter. In effect, the slope of the dose-response curve, γ50, was estimated to be 2.7 [1.9-3.2] for both male and female, and the ED50 doses to be 67Gy and 73Gy respectively. Skull base chordomas of the female patients seemed to be not only more resistant to radiation but also recurring faster than that for male patients (the maximum likelihood estimates of the Weibull shape parameter β are 2.6 for female and 1.7 for male patients). Conclusions: This analysis revealed several clinically important characteristics of radioresponsiveness of skull base chordomas. The comprehensive patient data obtained using three-dimensional treatment planning system allowed us to demonstrate and quantify the existence of dose-response and dose-volume relationships. In consequence, we are able to estimate prospectively the individual's probability of staying recurrence-free and her/his overall survival characteristics as a function of the applied three-dimensional dose distribution and time after treatment. Based on the analysis our treatment protocols have been modified to account for differences in radiosensitivity between female and male patients

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

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

    2016-01-01

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

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

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

    2016-01-01

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

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

    International Nuclear Information System (INIS)

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

  11. 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.

  12. Characteristics and Patterns of Metastatic Disease from Chordoma

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

    2015-01-01

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

  13. Characteristics and Patterns of Metastatic Disease from Chordoma

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    Victoria A. Young

    2015-01-01

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

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

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

    2003-07-01

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

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

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

    2014-01-01

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

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

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    孙成良; 董军; 李栋

    2011-01-01

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

  17. Primary chordoma of the nose

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    牟忠林; 刘兆华

    2003-01-01

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

  18. Clival chordoma in an infant.

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    Goel A

    1996-04-01

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

  19. Vaccine Therapy for Unresectable Chordoma

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

  20. Recurrent cervical chordoma: A case illustration

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

    2009-01-01

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

  1. 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.

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

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    Yutaka Nibu

    2013-01-01

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

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

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

    2015-01-01

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

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

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

    2011-01-01

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

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

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    Jensen Alexandra D

    2010-11-01

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

  6. Apoplexy in an intradural clival chordoma causing intraventricular bleed

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

    2016-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    李晓祥; 姬振伟; 马云雷; 范清宇

    2011-01-01

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

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

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

    2015-01-01

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

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

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    G K Sawke

    2014-01-01

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

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

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    Yongkun Yang

    2015-12-01

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

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

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    Yan-da LIN

    2016-04-01

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

  12. Molecular profiling of chordoma

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

    2014-01-01

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

  13. 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

  14. Cervical spine chordoma

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    Díez-González L

    2012-03-01

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

  15. Giant clival chordoma causing pathological laughter

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

    2014-01-01

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

  16. Giant clival chordoma causing pathological laughter

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    Daniel Andrade Gripp

    2014-01-01

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

  17. 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.

  18. Lumbar spine chordoma

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    Hatem, M.A.

    2015-01-01

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

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

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

    2002-07-01

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

  20. 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

    2016-01-01

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

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

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

    2013-01-01

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

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

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

    1983-01-01

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

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

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

    2014-01-01

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

  4. Sacrococcygeal chordoma, a rare cause of coccygodynia

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    Gavriilidis, Paschalis; Kyriakou, Dimitrios

    2013-01-01

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

  5. Chordoma Of Lumbar Spine

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

    2010-10-01

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

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

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    Beate Rinner

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

  7. Chordoma Cutis – A Diagnosis not to be Missed

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    Mishra, Kiran

    2014-01-01

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

  8. 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.

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

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

    2015-01-01

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

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

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

    2014-01-01

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

  11. Craniospinal dissemination of clival chondroid chordoma

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    Shinde S

    2005-01-01

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

  12. Genomic and epigenetic instability in chordoma: current insights

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    Feng Y

    2014-05-01

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

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

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

    2015-01-01

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

  14. 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.)

  15. AMBIGUITY IN DIAGNOSING CHONDROID CHORDOMA-A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Swarnagowri

    2014-08-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Arghya BANDYOPADHYAY

    2011-05-01

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

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

    Directory of Open Access Journals (Sweden)

    Prabodh Karnik

    2014-06-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2000-12-01

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

  20. Thoracic chordoma: CT and MR findings

    Energy Technology Data Exchange (ETDEWEB)

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

    1993-05-15

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2008-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Linlin Zhang

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

  3. Intradural Clival Chordoma: A Case Report

    Science.gov (United States)

    2014-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

    覃相志; 雷进

    2015-01-01

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

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

    Science.gov (United States)

    Gibbins, Ruth; Evans, Guy; Grimer, Robert

    2007-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1994-09-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Alexa Burger

    2014-07-01

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

  9. A unique presentation of retroclival chordoma.

    Directory of Open Access Journals (Sweden)

    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.

  10. Unusual Presentation of Chordoma in Nose.

    Science.gov (United States)

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

    2016-09-01

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

  11. Hand metastasis from a sacral chordoma

    Science.gov (United States)

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

    2014-01-01

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

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

    Science.gov (United States)

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

    2016-06-01

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

  13. CHONDROID CHORDOMA OF NASAL SEPTUM : A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Gayattre

    2015-04-01

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

  14. Proton radiation therapy for clivus chordoma; Case report

    Energy Technology Data Exchange (ETDEWEB)

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

    1993-03-01

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

  15. Molecular Characterization of Putative Chordoma Cell Lines

    Directory of Open Access Journals (Sweden)

    Silke Brüderlein

    2010-01-01

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

  16. Radiologic findings in posterior mediastinal chordoma

    Energy Technology Data Exchange (ETDEWEB)

    Stratt, B.; Steiner, R.M.

    1980-07-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Dagmar Kolb

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

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

    International Nuclear Information System (INIS)

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

  20. Clinical characteristics of Caroli's syndrome

    Institute of Scientific and Technical Information of China (English)

    Ozlem Yonem; Yusuf Bayraktar

    2007-01-01

    Caroli's syndrome is characterized by multiple segmental cystic or saccular dilatations of intrahepatic bile ducts associated with congenital hepatic fibrosis. The clinical features of this syndrome reflect both the characteristics of congenital hepatic fibrosis such as portal hypertension and that of Caroli's disease named as recurrent cholangitis and cholelithiasis. The diagnosis depends on both histology and imaging methods which can show the communication between the sacculi and the bile ducts. Treatment consists of symptomatic treatment of cholangitis attacks by antibiotics, some endoscopic,radiological and surgical drainage procedures and surgery. Liver transplantation seems the ultimate treatment for this disease. Prognosis is fairly good unless recurrent cholangitis and renal failure develops.

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

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

    2014-01-01

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

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

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

    2016-01-01

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

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

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

    2001-09-01

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

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

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    Vasu Reddy Challa

    2014-05-01

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

  7. Multifocal thoracic chordoma mimicking a paraganglioma

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    Giovanni Conzo

    2013-01-01

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

  8. Multifocal thoracic chordoma mimicking a paraganglioma.

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

    2013-01-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

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    Edwin Choy

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

  11. 18F-FMISO PET/CT Visualization of Tumor Hypoxia in Patients with Chordoma of the Mobile and Sacrococcygeal Spine

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

    2014-01-01

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

  12. Novel targeted therapies in chordoma: an update

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

    2015-01-01

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

  13. Cervical Vertebral Body Chordoma in a Cat.

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

    2016-05-01

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

  14. 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

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

    Science.gov (United States)

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

    2015-01-01

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

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

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    Dae-Hee Lee

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

  17. Intensity-Modulated Radiotherapy of Sacral Chordoma

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

    2002-08-01

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

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

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

    2014-01-01

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

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

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    Anand Goomany

    2016-01-01

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

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

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

    2016-01-01

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

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

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    Ma Baoan

    2010-12-01

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

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

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    Stigen Øyvind

    2011-10-01

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

  3. Combined Therapy for Distant Metastasis of Sacral Chordoma

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

    2015-01-01

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

  4. Combined Therapy for Distant Metastasis of Sacral Chordoma

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    Birol Özkal

    2015-01-01

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

  5. 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

  6. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

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

    Science.gov (United States)

    Tranesh, Ghassan; Nassar, Aziza

    2016-01-01

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

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

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    Sira Carrasco García de León

    2016-08-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  11. 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.

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

    Directory of Open Access Journals (Sweden)

    Agrawal Amit

    2008-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Roberto Jose Diaz

    2012-09-01

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

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

    Science.gov (United States)

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

    2016-07-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Murat Akyol

    2014-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  18. Novel targeted therapies in chordoma: an update

    Directory of Open Access Journals (Sweden)

    Di Maio S

    2015-05-01

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

  19. Spill pleural: Clinical and cytological characteristics

    International Nuclear Information System (INIS)

    The spill pleural is a frequent entity in congestive heart failure. The congestive heart failure, is probable that the cause more common of pleural spill, an analysis of the spill causes is made, the pathology is revised from the clinical point of view, highlighting the most excellent characteristics in each entity and an cytological analysis is made

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

    Science.gov (United States)

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

    2015-01-01

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

  1. Intracranial Infections: Clinical and Imaging Characteristics

    International Nuclear Information System (INIS)

    The radiologist plays a crucial role in identifying and narrowing the differential diagnosis of intracranial infections. A thorough understanding of the intracranial compartment anatomy and characteristic imaging findings of specific pathogens, as well incorporation of the clinical information, is essential to establish correct diagnosis. Specific types of infections have certain propensities for different anatomical regions within the brain. In addition, the imaging findings must be placed in the context of the clinical setting, particularly in immunocompromised and human immunodeficiency virus (HIV)-positive patients. This paper describes and depicts infections within the different compartments of the brain. Pathology-proven infectious cases are presented in both immunocompetent and immunocompromised patients, with a discussion of the characteristic findings of each pathogen. Magnetic resonance spectroscopy (MRS) characteristics for several infections are also discussed

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1989-07-01

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

  3. Investigating Microenvironmental Regulation of Human Chordoma Cell Behaviour

    Science.gov (United States)

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

    2014-01-01

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

  4. Investigating microenvironmental regulation of human chordoma cell behaviour.

    Directory of Open Access Journals (Sweden)

    Priya Patel

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1991-07-01

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

  6. Clival chordoma; CT and MR findings

    Energy Technology Data Exchange (ETDEWEB)

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

    1993-07-15

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

  7. The clinical characteristics of the radiation pneumonia

    International Nuclear Information System (INIS)

    Objective: To analyse the clinical characteristics of the radiation pneumonia, sum the experience and the basis of the radiation pneumonia for its prevention and treatment. Method: Twenty three cases with radiation pneumonia from 1991 to 1998 were retrospectively analysed. Its clinical manifestation, chest X-ray, thoracic CT and blood routine were evaluated. Result: The acute manifestation was fever, cough, dyspnea, and the chronic manifestation was cough and insufficiency of pulmonary function. Conclusion: The prevention of radiation pneumonia is more important, high dose cortical steroids and antibiotics were prescribed during the acute stage and the chronic radiation pneumonia is irreversible

  8. Nocardiosis: Risk Factors, Clinical Characteristics and Outcome

    OpenAIRE

    Alavi Darazam, Ilad; Shamaei, Masoud; Mobarhan, Mandana; Ghasemi, Shahin; Tabarsi, Payam; Motavasseli, Masoud; Mansouri, Davood

    2013-01-01

    Nocardiosis has been reported increasingly in recent two decades, probably due to improvement in isolation of the organism and increased burden of immune compromised patients. Nocardia occasionally has been reported in healthy people. A case series of definitive Nocardiosis (2002 to 2010), clinical characteristics, underlying diseases, immune status and in-patient outcome were studied in a tertiary referral center. Twenty one patients with definite diagnosis of Nocardiosis were studied. 17 ca...

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

    Science.gov (United States)

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

    2014-01-01

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

  10. Two Cases of Dedifferentiated Chordoma in the Sacrum

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2016-08-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

  13. Chordoma of skull base presenting as nasopharyngeal mass

    Directory of Open Access Journals (Sweden)

    Sant Prakash Kataria

    2013-01-01

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

  14. CT and MRI appearances of a thoracic chordoma

    Energy Technology Data Exchange (ETDEWEB)

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

    1998-12-01

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

  15. Pituitary gigantism: Causes and clinical characteristics.

    Science.gov (United States)

    Rostomyan, Liliya; Daly, Adrian F; Beckers, Albert

    2015-12-01

    Acromegaly and pituitary gigantism are very rare conditions resulting from excessive secretion of growth hormone (GH), usually by a pituitary adenoma. Pituitary gigantism occurs when GH excess overlaps with the period of rapid linear growth during childhood and adolescence. Until recently, its etiology and clinical characteristics have been poorly understood. Genetic and genomic causes have been identified in recent years that explain about half of cases of pituitary gigantism. We describe these recent discoveries and focus on some important settings in which gigantism can occur, including familial isolated pituitary adenomas (FIPA) and the newly described X-linked acrogigantism (X-LAG) syndrome.

  16. Pituitary gigantism: Causes and clinical characteristics.

    Science.gov (United States)

    Rostomyan, Liliya; Daly, Adrian F; Beckers, Albert

    2015-12-01

    Acromegaly and pituitary gigantism are very rare conditions resulting from excessive secretion of growth hormone (GH), usually by a pituitary adenoma. Pituitary gigantism occurs when GH excess overlaps with the period of rapid linear growth during childhood and adolescence. Until recently, its etiology and clinical characteristics have been poorly understood. Genetic and genomic causes have been identified in recent years that explain about half of cases of pituitary gigantism. We describe these recent discoveries and focus on some important settings in which gigantism can occur, including familial isolated pituitary adenomas (FIPA) and the newly described X-linked acrogigantism (X-LAG) syndrome. PMID:26585365

  17. Clinic Characteristics of Varicella Zoster Myocarditis

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective To analyze the clinic characteristics of varicella zoster myocarditis and to determine the diagnostic value of serum cardiac troponin I (cTnI ) on the disease. Methods Information of 58 hospitalized patients with varicella zoster was collected, and the incidence of varicella zoster myocarditis and clinic characteristics (ECG, cTnI, age and sex distribution, etc. ) were analyzed respectively. Results It was found that 36.2% of the patients developed myocarditis. The old and female persons were much more susceptible to varicella zoster myocarditis. All patients had responsive ECG manifestations, and the abnormal ST-T changes were more common than other ECG abnormalities. CTnI remained higher than normal and had significant diagnostic value. Most of the patients had good prognosis, only a few patients lasted a long time and even progressed into cardiomyopathy. Nearly all the patients came to see dermatologists when they felt ill initially. That would lead to more misdiagnosis. Conclusion Infection of varicella zoster can complicate myocarditis, we must pay more attention to the patients who suffer from varicella zoster especially in the aged and female; cTnI is an important and effective index for diagnosis of varicella zoster myocarditis.

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

    Directory of Open Access Journals (Sweden)

    Uda Takehiro

    2006-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Alshammari Jaber

    2012-11-01

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

  20. 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.

  1. Primary sacrococcygeal chordoma with unusual skeletal muscle metastasis

    Science.gov (United States)

    Vu, Lisa; Haygood, Tamara Miner

    2015-01-01

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

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

    Science.gov (United States)

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

    2016-07-01

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

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

    Directory of Open Access Journals (Sweden)

    Kachhara R

    2002-04-01

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

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

    Science.gov (United States)

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

    2016-07-01

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

  5. Genomic Aberrations Frequently Alter Chromatin Regulatory Genes in Chordoma

    Science.gov (United States)

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

    2016-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

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

    Directory of Open Access Journals (Sweden)

    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.

  8. [18F]-Fluoromisonidazole Positron Emission Tomography/Computed Tomography Visualization of Tumor Hypoxia in Patients With Chordoma of the Mobile and Sacrococcygeal Spine

    International Nuclear Information System (INIS)

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

  9. [18F]-Fluoromisonidazole Positron Emission Tomography/Computed Tomography Visualization of Tumor Hypoxia in Patients With Chordoma of the Mobile and Sacrococcygeal Spine

    Energy Technology Data Exchange (ETDEWEB)

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

    2014-12-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    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)

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

    Directory of Open Access Journals (Sweden)

    Kenneth A Moore

    2015-01-01

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

  12. Clinical characteristics and pathogenesis of cerebellar glioblastoma.

    Science.gov (United States)

    Takahashi, Yoshinobu; Makino, Keishi; Nakamura, Hideo; Hide, Takuichiro; Yano, Shigetoshi; Kamada, Hajime; Kuratsu, Jun-Ichi

    2014-11-01

    Cerebellar glioblastomas (GBMs) are rare, with neither their pathogenesis nor prognosis being completely understood. The present study aimed to clarify the clinical characteristics of cerebellar GBMs by comparison with supratentorial GBMs, focusing particularly on the pathogenesis. The clinical factors between cerebellar (n=10) and supratentorial (n=216) GBMs were compared. Additionally, p53 and epidermal growth factor receptor (EGFR) levels were investigated in six patients by immunostaining as well as the isocitrate dehydrogenase 1 (IDH1) status of five patients by direct sequencing. Eight males and two females participated in the present study, the mean age at diagnosis was 56.6 years and the range 37-75 years. Four patients presented with hydrocephalus and one with brainstem involvement, and two patients were diagnosed with neurofibromatosis type 1. Two patients had previously received radiotherapy, eight patients received postoperative radiotherapy and seven chemotherapy. The mean Karnofsky performance status (KPS) score was lower in patients with cerebellar GBMs compared to those with supratentorial GBM; however, the survival times did not differ between the two groups. All of the cases of six cerebellar GBMs were p53‑positive and EGFR‑negative, as detected by immunostaining, consistent with secondary GBM. However, no IDH1 mutations were detected in any of the five cases of cerebellar GBMs analyzed, indicating that these tumors were not of the secondary type. The KPS score with cerebellar GBMs may be lower due to hydrocephalus, which was ameliorated by surgery but may have impacted the survival rate. It was confirmed that cerebellar GBMs were identical to supratentorial GBMs with respect to its clinical features, with the possible exception of the KPS score. The present study's genetic analyses indicated that cerebellar GBMs may develop via a pathway different from that of either primary or secondary GBM. PMID:25199771

  13. Clinical characteristics of patients with conjunctivochalasis

    Directory of Open Access Journals (Sweden)

    Balci O

    2014-08-01

    Full Text Available Ozlem Balci Ophthalmology Department, Kolan Hospitalium Group, Istanbul, Turkey Purpose: To evaluate the clinical characteristics of patients with conjunctivochalasis (CCh. Methods and materials: This retrospective study enrolled 30 subjects diagnosed with conjunctivochalasis. Complete ophthalmic examination, including visual acuity assessment, slit-lamp examination, applanation tonometry, dilated funduscopy, tear break-up time, Schirmer 1 test, and fluorescein staining were performed in all patients. Age, sex, laterality, ocular history, symptoms, and clinical findings were recorded. Results: The study included 50 eyes from 30 cases. Ages ranged from 45 to 80 years, with a mean age of 65±10 years. CChs grading were as follows: 30 (60% eyes with grade 1 CCh; 15 (30% eyes with grade 2 CCh; and five (10% eyes with grade 3 CCh. CCh was located in the inferior bulbar conjunctiva in 45 (90% eyes, and in the remaining five (10% CCh was located in the superior bulbar conjunctiva. Ten (33.3% patients had no symptoms. Dryness, eye pain, redness, blurry vision, tired eye feeling, and epiphora were the symptoms encountered in the remaining twenty (63.6% patients. Altered tear meniscus was noted in all cases. The mean tear break-up time was 7.6 seconds. The mean Schirmer 1 test score was 7 mm. Pinguecula was found in ten patients. Conclusion: Dryness, eye pain, redness, blurry vision, and epiphora were the main symptoms in patients with CCh. Dryness, eye pain, and blurry vision were worsened during downgaze and blinking. So CCh should be taken into consideration in the differential diagnosis of chronic ocular irritation and epiphora. Keywords: ocular irritation, epiphora, dryness, eye pain, blurry vision

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2013-03-15

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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  16. [Clinical characteristics of pancreatitis after cardiovascular surgery].

    Science.gov (United States)

    Ikegami, H; Sumiyoshi, T; Ishizuka, N; Ueda, M; Inaba, T; Hosoda, S; Aomi, S; Endo, M; Hashimoto, A; Koyanagi, H

    1995-10-01

    Increases in pancreatic enzyme levels after cardiovascular surgery were studied, and their clinical characteristics evaluated. The subjects were 128 patients who had undergone cardiovascular surgery (65 patients after valve replacement, 32 after coronary bypass surgery and 31 after aortic artificial graft replacement). The pancreatic enzyme (serum amylase and lypase) levels were monitored serially before and after operation, and amylase fractions were measured at their peaks. The relationships of the peak lypase level with underlying cardiac diseases, background factors, factors related to surgery, factors related to the extracorporeal circulation, presence or absence of symptoms, and treatments were examined. The amylase level exhibited biphasic changes consisting of a peak in which salivary glands amylase (S type) was dominant and a peak in which pancreatic amylase (P type) was dominant. The second peak coincided with the peak lypase and occurred mostly 3 to 10 days after operation. The peak lypase level exceeded the normal range in 78% of all the patients. It exceeded 564 U/l, 4 times the normal value in 28% of the patients, many of whom were symptomatic. So, we recommended that these cases should be treated as "postoperative pancreatitis". A high peak lypase level showed a significant correlation with the history of gallbladder and pancreatic diseases and diabetes mellitus among the background factors and emergency operation and the use of IABP among the surgery-related factors.(ABSTRACT TRUNCATED AT 250 WORDS)

  17. The "PIP problem": clinical and histologic characteristics.

    Science.gov (United States)

    Correia-Sá, Inês; Rodrigues-Pereira, Pedro; Marques, Marisa

    2013-10-01

    Implants from Poly Implant Prothése (PIP), the French manufacturer, showed increased risk of implant rupture and silicone leakage through the shell. Concerns also exist about the potential irritant behavior of silicone gel filler in these implants. This report presents the clinical, histologic, and microbiologic characteristics of a capsule and a siliconoma from a patient with a ruptured PIP implant. A 41-year-old woman submitted to breast augmentation in 2005 with PIP silicone gel implants presented with a recent history of progressive asymmetric breast enlargement and an enlarged lymph node on her right axilla. No capsular contracture was observed. A breast ultrasonography showed intra- and extracapsular ruptures of the right implant. The woman underwent explantation. Histologic analysis of the breast capsules showed a thin capsule with a chronic, mild inflammatory response. Microbiologic analysis showed no bacterial agent. The irritant behavior of the PIP silicone gel previously described was not able to produce capsular contracture or an exuberant inflammatory reaction. Studies to evaluate the potential risks of the silicone gel and to define the hazards for women implanted with those prostheses are urgently needed. PMID:23943050

  18. Chordomas: Is It Possible to Predict Recurrence?

    Directory of Open Access Journals (Sweden)

    Banu SARSIK

    2009-06-01

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

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

    Science.gov (United States)

    2014-01-01

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

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

    Science.gov (United States)

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

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    I-Mei Siu

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2012-06-01

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

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

    International Nuclear Information System (INIS)

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

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

    Institute of Scientific and Technical Information of China (English)

    许霖; 周毅; 杜希娜; 崔博; 吕秀花; 杨广夫

    2015-01-01

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

  6. Clinical Characteristics and Treatment of Malignant Granuloma

    Institute of Scientific and Technical Information of China (English)

    Chongjin Feng; Junbing Guo; Yong Chen

    2005-01-01

    OBJECTIVE To analyse the clinical characteristics of malignant granuloma(MG) and evaluate its treatment.METHODS From March 1985 to May 1998, 101 cases of malignant granuloma were treated with radiation (RT) alone or radiotherapy followed by chemotherapy or chemotherapy followed by radiotherapy. For chemotherapy, a CCNU, COMP (CTX, VCR, MTX, PDN) or CHOP (CTX,ADM, VCR, PDN) regimen was given. Radiation was directed through the anterior field of the nose mainly for nasal and paranasal sinus malignant granuloma, and through the faciocervical field for malignant granuloma of Walderyer's ring or for patients with cervical lymphadenectasis. Total dose was 45-65 Gy over 5-6 weeks.RESULTS The overall 3-year, 5-year and 10-year survival rates were 78.2%, 56.1% and 39.7% respectively. The 5-year survival rate was: RT group 60.3%, RT + CHOP or CHOP + RT group 64.7% (P >0.05), RT +CCNU group 40%, and RT + COMP group 33.3%. The 5-year survival rates of patients with one involved focus and more than 2 involved foci were 75.6%(34/45) and 39.3% (22/56) (P<0.001). The 5-year survival rates of patients with or without body symptoms were 39.6% (18/48) and 67.9%(36/53) (P<0.05). The 5-year survival rate of the 50-60 Gy group and the <50Gy group were 60.1% (40/66) and 20% (1/5)(P <0.05). The local and regional recurrence rate was 20.8 % (21/101 ).CONCLUSION Radiotherapy alone should be the treatment of choice for patients with one site involvement and without body symptoms. Radiation fields should be large enough to include the potentially involved sites. The recommended dose is 50-60 Gy over 5-6 weeks. It is suggested that patients with more than 2 foci involvement and those with body symptoms should receive the combination therapy.

  7. Characteristics and Roles of Literacy Clinic Directors

    Science.gov (United States)

    Ortlieb, Evan; Pearce, Daniel L.

    2013-01-01

    A literacy clinic is an ideal setting where research and exploration often lead to breakthroughs in reading remediation; that information can then be transferred to classroom instruction (Morris, 2003). Although it is clear that literacy clinics should be structured around what works for their student populations, there remains ambiguity…

  8. Athletic Training Students' Perception of Significant Clinical Instructor Demographic Characteristics

    Science.gov (United States)

    Mulholland, Carlen; Martin, Malissa

    2010-01-01

    Context: Characteristics of a model clinical instructor (CI) continue to be defined. However, certain characteristics are still unknown. Objective: To more fully define and describe quality clinical instruction by examining the impact of employment status, years of experience as a certified athletic trainer (AT), and employment setting on athletic…

  9. Malignant fibrous histiocytoma developing in irradiated sacral chordoma

    Energy Technology Data Exchange (ETDEWEB)

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

    1984-06-15

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

  10. Chondroid Chordoma and Nasal Adenocarcinoma: An Exceptional Association

    Directory of Open Access Journals (Sweden)

    Patrice Gallet

    2012-01-01

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

  11. Clinical characteristics of cutaneous lupus erythematosus

    OpenAIRE

    Szczęch, Justyna; Rutka, Maja; Samotij, Dominik; Zalewska, Agnieszka; Reich, Adam

    2016-01-01

    Introduction Lupus erythematosus (LE) shows a wide variety of clinical manifestations, skin involvement being one of the most important. Aim To analyze the clinical presentation of cutaneous variants of lupus erythematosus in terms of skin lesion spectrum and extracutaneous involvement. Material and methods A total of 64 patients with cutaneous LE (CLE) were included. The study was based on the “Core Set Questionnaire” developed by the European Society of Cutaneous Lupus Erythematosus (EUSCLE...

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

    Institute of Scientific and Technical Information of China (English)

    王长峰; 肖建如; 魏海峰; 杨兴海; 贾连顺

    2003-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Uesaka Mitsuru

    2011-09-01

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

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

    International Nuclear Information System (INIS)

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

  15. Somnambulism. Clinical characteristics and personality patterns.

    Science.gov (United States)

    Kales, A; Soldatos, C R; Caldwell, A B; Kales, J D; Humphrey, F J; Charney, D S; Schweitzer, P K

    1980-12-01

    Fifty adults with either a present or past complaint of somnambulism were evaluated to determine the development and clinical course of their disorder as well as their personality patterns. Generally, when sleepwalking was outgrown, its onset was before age 10 years and its termination before age 15 years. Current sleepwalkers, compared with past sleepwalkers, started sleepwalking at a later age, had a higher frequency of events, and had episodes earlier in the night. Their episodes were also characterized by more intense clinical manifestations. Furthermore, current sleepwalkers demonstrated high levels of psychopathology, whereas past sleepwalkers had essentially normal psychological patterns. Specifically, the current sleepwalkers showed active, outwardly directed behavioral patterns, suggestive of difficulties in handling aggression. The clinical application of these findings is discussed and practical recommendations are given for the evaluation and management of sleepwalking. PMID:7447621

  16. Clinical characteristics of idiopathic portal hypertension

    Institute of Scientific and Technical Information of China (English)

    Ozgur Harmanci; Yusuf Bayraktar

    2007-01-01

    Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".

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

    Science.gov (United States)

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

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Matteo M Trucco

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

  19. Clinical characteristics of anxiety disordered youth

    OpenAIRE

    KENDALL, Philip C.; Compton, Scott N.; Walkup, John T.; Birmaher, Boris; Albano, Anne Marie; Sherrill, Joel; Ginsburg, Golda; Rynn, Moira; McCracken, James; Gosch, Elizabeth; Keeton, Courtney; Bergman, Lindsey; Sakolsky, Dara; Suveg, Cindy; Iyengar, Satish

    2010-01-01

    Reports the characteristics of a large, representative sample of treatment seeking anxious youth (N =488). Participants, aged 7–17 years (mean 10.7 yrs), had a principal DSM-IV diagnosis of separation anxiety disorder (SAD), generalized anxiety disorder (GAD), or social phobia (SP). Although youth with a co-primary diagnosis for which a different disorder-specific treatment would be indicated (e.g., major depressive disorder, substance abuse) were not included, there were few other exclusion ...

  20. Sacrococcygeal chordoma presenting as a retro rectal tumour

    Science.gov (United States)

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

    2014-01-01

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

  1. Neonatal peripheral hypotonia: clinical and electromyographic characteristics.

    Science.gov (United States)

    Parano, E; Lovelace, R E

    1993-06-01

    Hypotonia is a common occurrence in pediatrics, especially in the neonatal period. The hypotonic neonate represents a diagnostic challenge for the general pediatrician because hypotonia may be caused by a lesion at any level in the neuraxis: (1) central nervous system (CNS), (2) peripheral nerves (PN), (3) neuromuscular junction, or (4) muscles. Distinguishing among these pathologies is a particularly arduous task. This review will discuss the clinical approach to neonatal hypotonia with emphasis on disorders of the peripheral nervous system and muscle, and the importance of the electrophysiological study as a diagnostic test.

  2. 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.

  3. Corticosteroid responsiveness and clinical characteristics in childhood difficult asthma

    OpenAIRE

    Bossley, C.J.; Saglani, S; Kavanagh, C.; Payne, D.N.R.; Wilson, N; Tsartsali, L.; Rosenthal, M; Balfour-Lynn, I M; Nicholson, A.G.; Bush, A

    2009-01-01

    This study describes the clinical characteristics and corticosteroid responsiveness of children with difficult asthma (DA). We hypothesised that complete corticosteroid responsiveness (defined as improved symptoms, normal spirometry, normal exhaled nitric oxide fraction (FeNO) and no bronchodilator responsiveness (BDR

  4. Acute demyelinating encephalomyelitis: Clinical characteristics and outcome

    Directory of Open Access Journals (Sweden)

    Ahmed Farag Elhassanien

    2013-01-01

    Full Text Available Background: ADEM, although relatively uncommon, is probably under-recognized. Objectives: To spotlight the clinical profile and therapeutic outcome of children with ADEM. Materials and Methods: This is a prospective study of patients with ADEM who were admitted to the Pediatric Departments in Aladan and Alfarawanya Hospitals in Kuwait, from January 2009 to January 2011. Clinical, microbiological and radiological data were analyzed. Results: Of 48 patients presented with acute neurological symptoms and signs, 21 patients fulfilled criteria for ADEM. 80.95% of cases were presenting in winter and spring, 57% of patients had a history of upper respiratory tract illness. The commonest presentations were motor deficits, convulsions and altered consciousness. CSF virology studies showed herpes simplex virus (HSV and Epstein-Barr virus (EBV (3 patients whereas nasal and nasopharyngeal swab showed evidence of influenza H1N1 virus (1 patient. Brain MRI was performed in all patients and revealed multiple hyperintense supratentorial brain lesions on T2/FLAIR images. 85.7% of patients had cortical and/or subcortical white matter lesions which were bilateral and asymmetric in location and size. Conclusion: ADEM although rare must be considered in children with acute onset of neurological signs and symptoms and must be distinguished from any acute neurological insult.

  5. Clinical, pathological and sonographic characteristics of unexpected gallbladder carcinoma

    OpenAIRE

    Jin-huan WANG; Liu, Bo-Ji; Xu, Hui-Xiong; Sun, Li-ping; Li, Dan-Dan; Guo, Le-Hang; Liu, Lin-Na; Xu, Xiao-Hong

    2015-01-01

    Objectives: To investigate the clinical, pathological, and sonographic characteristics of unexpected gallbladder carcinoma (UGC). Methods: Of 5424 patients who had undergone cholecystectomy from December 2006 to October 2013, 54 patients with primary gallbladder carcinomas confirmed by pathological diagnosis were identified. The patients were divided into two groups: diagnosed before operation (n=34) and UGC groups (n=20), of whom the clinical, pathological, and sonographic characteristics we...

  6. Night terrors. Clinical characteristics and personality patterns.

    Science.gov (United States)

    Kales, J D; Kales, A; Soldatos, C R; Caldwell, A B; Charney, D S; Martin, E D

    1980-12-01

    The development and clinical course of night terrors and the personality patterns of patients with this disorder were evaluated in 40 adults who had a current complaint of night terrors. Compared with a group of adult sleepwalkers, the patients with night terrors had a later age of onset for their disorder, a higher frequency of events, and an earlier time of night for the occurrence of episodes. Both groups had high levels of psychopathology, with higher values for the night terror group. This sleepwalkers showed active, outwardly directed behavioral patterns, whereas the night terror patients showed an inhibition of outward expressions of aggression and a predominance of anxiety, depression, tendencies obsessive-compulsive/, and phobicness. Although night terrors and sleepwalking in childhood seem to be related primarily to genetic and developmental factors, their persistence and especially their onset in adulthood are found to be related more to psychological factors. PMID:7447622

  7. Clinical characteristics of eosinophilic asthma exacerbations

    DEFF Research Database (Denmark)

    Bjerregaard, Asger; Laing, Ingrid A; Backer, Vibeke;

    2016-01-01

    BACKGROUND AND OBJECTIVE: Airway eosinophilia is associated with an increased risk of asthma exacerbations; however, the impact on the severity of exacerbations is largely unknown. We describe the sputum inflammatory phenotype during asthma exacerbation and correlate it with severity and treatment...... response. METHODS: Patients presenting to hospital with an asthma exacerbation were recruited during a 12-month period and followed up after 4 weeks. Induced sputum was collected at both visits. Patients underwent spirometry, arterial blood gas analysis, fractional exhaled nitric oxide analysis, white...... with a sensitivity of 86% and a specificity of 70%. CONCLUSION: Our findings suggest that eosinophilic asthma exacerbations may be clinically more severe than NEEs, supporting the identification of these higher risk patients for specific interventions....

  8. [Clinical and echocardiographic characteristics of scimitar syndrome].

    Science.gov (United States)

    Espinola-Zavaleta, Nilda; Játiva-Chávez, Silvio; Muñoz-Castellanos, Luis; Zamora-González, Carlos

    2006-03-01

    Seven patients with scimitar syndrome underwent retrospective clinical and echocardiographic examination. The findings were: respiratory infection with dyspnea on moderate exercise in 90%, scimitar sign in four (57%), dextrocardia in five (71%), and interatrial septal defect in five (71%), one of whom had patent ductus arteriosus. Overall, two patients had patent ductus arteriosus: one also had aortic coarctation and the other, a bicuspid aortic valve. Dilation of the right cavities was found in five (71%) and blunt edge in five (71%). In two patients, anomalous drainage was into the right atrium; in another two, into the inferior vena cava; and in three, towards the junction of the right atrium and the inferior vena cava. In three patients, drainage was obstructed. Six patients with cardiac abnormalities proceeded to surgery. Scimitar syndrome is a rare entity. In the large majority of patients (86%), diagnosis and follow-up can be performed echocardiographically.

  9. Clinical characteristics of patients with persistent postural-perceptual dizziness

    OpenAIRE

    Roseli Saraiva Moreira Bittar; Eliane Maria Dias von Söhsten Lins

    2015-01-01

    INTRODUCTION: Persistent postural-perceptual dizziness is the dizziness that lasts for over three months with no clinical explanation for its persistence. The patient's motor response pattern presents changes and most patients manifest significant anxiety. OBJECTIVE: To evaluate the clinical characteristics of patients with persistent postural and perceptual dizziness. METHODS: statistical analysis of clinical aspects of patients with persistent postural-perceptual dizziness. RESULTS: 81 pati...

  10. Clinical characteristics and outcomes in biclonal gammopathies.

    Science.gov (United States)

    Mullikin, Trey C; Rajkumar, S Vincent; Dispenzieri, Angela; Buadi, Francis K; Lacy, Martha Q; Lin, Yi; Dingli, David; Go, Ronald S; Hayman, Suzanne R; Zeldenrust, Steven R; Russell, Stephen J; Lust, John A; Leung, Nelson; Kapoor, Prashant; Kyle, Robert A; Gertz, Morie A; Kumar, Shaji K

    2016-05-01

    A single monoclonal protein typically characterizes monoclonal gammopathies, but a small proportion may have more than one M protein identifiable. In the setting of symptomatic multiple myeloma (MM), the development of a new monoclonal protein following therapy is associated with better outcomes. As for the precursor conditions, monoclonal gammopathy undetermined significance (MGUS) and smoldering multiple myeloma (SMM), there is limited information on the impact of a second monoclonal protein on the disease course, including progression and response to treatment. The outcomes of patients with MGUS and SMM with more than one monoclonal protein, after identifying 539 patients with biclonal proteins on electrophoresis and/or immunofixation, were reported. About 22 of 393 patients with MGUS/biclonal gammopathy of undetermined significance (BGUS) progressed to SMM (6), MM (11), AL (3), or WM (2), and 5 of 16 patients with biclonal SMM progressed to MM. The rate of progression for BGUS was approximately 1% per year, which is similar to MGUS with one monoclonal protein. The median estimated time of progression of biclonal SMM was 2.6 years; similar to monoclonal SMM. For patients with biclonal MM, both M spikes responded to treatment and, upon relapse, the original dominant M protein remained dominant as the disease progressed. In conclusion, the presence of a second monoclonal protein does not appear to affect the progression of precursor states and suggests multiple monoclonal proteins do not clinically impact one another in the course of the disease. Am. J. Hematol. 91:473-475, 2016. © 2016 Wiley Periodicals, Inc. PMID:26840395

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2001-12-01

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

  12. Patient, provider, and clinic characteristics associated with public STD clinic patient satisfaction

    OpenAIRE

    Mehta, S; Zenilman, J; Erbelding, E

    2005-01-01

    Objectives: There is a lack of information describing levels of patient satisfaction among patients seeking sexually transmitted diseases (STD) care in a public clinic setting. We sought to identify patient, provider, and clinic characteristics associated with patient satisfaction within public STD clinics.

  13. Clinic Characteristics of Varicella Zoster Myocarditis

    Institute of Scientific and Technical Information of China (English)

    FANG; Wu-wang

    2001-01-01

    [1]Kokstein Z, Balatka J, Horacek J. ECG changes in children with acute myocarditis[J]. Sb Ved Pr Lek Fak Karlovy Univerzity Hradci Kralove Suppl, 1990, 33(4): 373-379.[2]Rich R, McErlean M. Complete heart block in a child with varicella[J]. Am J Emerg M, 1993, 11(6): 602-605.[3]Sobrino MJM, Lopez PF, Cisneros JM, et al. Thoracic pain as early clinical manifestation of myocarditis caused by varicella [J]. Med Clin Barc,1992, 99(6): 236-237.[4]Teravanichpong S, Chuangsuwanich T. Fatal varicella in a healthy girl[J]. J Med Assoc Thai, 1990, 73(11): 648-651.[5]Amral FT, Bestetti RB, Araujo RC, et al. Transient atroventricular conduction disordor: a potential cause of sudden death in myocarditis due to varicella[J]. Arq Bras Cardiol, 1989, 53(2): 129-131.[6]Lorber A, Zonis Z, Maisuls E, et al. The scale of myocardial involvement in varicella myocarditis[J]. Int J Cardiol,1988, 20(2): 257-262.[7]Ettedgui JA, Ladusans E, Bamford M. Complete heart block as a complication of varicella[J]. Int J Cardiol, 1987, 14(3): 362-365.[8]Waagner DC, Murphy TV. Varicella myocarditis[J]. Pediatr Infect Dis J, 1990, 9(5): 360-363.[9]Rivera CF, Omar M, Aliaga ML, et al. Varicella complicated by pericarditis and pneumonia [J]. An Med Interna, 1996, 13(3): 130-132.[10]Civico RF, Omar M, Martinez LA, et al. Varicella complicated by pericarditis and pneumonia [J]. An Med Interna, 1996, 13(3): 130-132.[11]Bachli E, Kagi MK, Krause M. Visceral and neurological complications in varicella infections of adults [J]. Schweiz Med Wochenschr, 1996, 126(11): 440-446.[12]Senellart F, Bozio A, Sassolas F, et al. Varicella myocarditis and junctional ectopic tachycardia [J]. Pediatrie Bucur, 1991, 46(3): 267-270.[13]Straus S, Ostrove J, Inchauspe G, et al. Varicella- zoster virus infections[J]. Ann Intern Med,1988, 108: 221-237.[14]Tsintsof A, Delprado WJ, Keogh AM. Varicella zoster myocarditis progressing to cardiomyopathy and cardiac

  14. Characteristics of student preparedness for clinical learning: clinical educator perspectives using the Delphi approach

    Directory of Open Access Journals (Sweden)

    Chipchase Lucinda S

    2012-11-01

    Full Text Available Abstract Background During clinical placements, clinical educators facilitate student learning. Previous research has defined the skills, attitudes and practices that pertain to an ideal clinical educator. However, less attention has been paid to the role of student readiness in terms of foundational knowledge and attitudes at the commencement of practice education. Therefore, the aim of this study was to ascertain clinical educators’ views on the characteristics that they perceive demonstrate that a student is well prepared for clinical learning. Methods A two round on-line Delphi study was conducted. The first questionnaire was emailed to a total of 636 expert clinical educators from the disciplines of occupational therapy, physiotherapy and speech pathology. Expert clinical educators were asked to describe the key characteristics that indicate a student is prepared for a clinical placement and ready to learn. Open-ended responses received from the first round were subject to a thematic analysis and resulted in six themes with 62 characteristics. In the second round, participants were asked to rate each characteristic on a 7 point Likert Scale. Results A total of 258 (40.56% responded to the first round of the Delphi survey while 161 clinical educators completed the second (62.40% retention rate. Consensus was reached on 57 characteristics (six themes using a cut off of greater than 70% positive respondents and an interquartile deviation IQD of equal or less than 1. Conclusions This study identified 57 characteristics (six themes perceived by clinical educators as indicators of a student who is prepared and ready for clinical learning. A list of characteristics relating to behaviours has been compiled and could be provided to students to aid their preparation for clinical learning and to universities to incorporate within curricula. In addition, the list provides a platform for discussions by professional bodies about the role of placement

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1999-07-01

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

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

    Directory of Open Access Journals (Sweden)

    Bhawna Bhutoria Jain

    2013-01-01

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

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

    Science.gov (United States)

    Patel, Shalin S; Schwab, Joseph H

    2016-09-01

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

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

    Science.gov (United States)

    Patel, Shalin S; Schwab, Joseph H

    2016-09-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

  2. Intra-His bundle block: clinical, electrocardiographic, and electrophysiologic characteristics

    OpenAIRE

    Andréa Eduardo M.; Atié Jacob; Maciel Washington A.; Oliveira Jr Nilson A. de; Camanho Luiz Eduardo; Belo Luís Gustavo; Carvalho Hecio Affonso de; Siqueira Leonardo; Saad Eduardo; Venancio Ana Claudia

    2002-01-01

    OBJECTIVE: To assess the clinical, electrocardiographic, and electrophysiologic characteristics of patients (pt) with intra-His bundle block undergoing an electrophysiologic study (EPS). METHODS: We analyzed the characteristics of 16 pt with second-degree atrioventricular block and symptoms of syncope or dyspnea, or both, undergoing conventional EPS. RESULTS: Intra-His bundle block was documented in 16 pt during an EPS. In 15 (94%) pt, the atrioventricular block was recorded in sinus rhythm; ...

  3. Clinical characteristics analysis of adult human adenovirus type 7 infection

    Institute of Scientific and Technical Information of China (English)

    张乃春

    2014-01-01

    Objective To investigate the clinical characteristics of patients infected with human adenovirus type 7 and to provide guidance for early diagnosis and timely control of the outbreak.Methods A total of 301 patients infected with the human adenoviruses who were quarantined in hospital from December 2012 to February 2013 were observed.Epidemiological questionnaires were used to collect data of clinical features of the disease including

  4. Patient characteristics affecting attendance at general outpatient clinics.

    OpenAIRE

    McClure, R J; Newell, S J; Edwards, S

    1996-01-01

    A study was carried out to identify the characteristics of children who do not attend appointments at general outpatient clinics. Over six months, 359 children who had an appointment at a general clinic were studied using a questionnaire given to parents (74% response rate) and by inspection of case notes. Based on their first appointment in the study period, children were divided into 'attenders' (n = 262) and 'non-attenders' (n = 97) for analysis. Non-attenders were significantly more likel...

  5. Clinical characteristics of Takotsubo cardiomyopathy in North America

    OpenAIRE

    Saeed Ahmed; Patompong Ungprasert; Supawat Ratanapo; Tanveer Hussain; Riesenfeld, Erik P.

    2013-01-01

    Background: Takotsubo cardiomyopathy (TC) or transient left ventricular apical ballooning syndrome is an acute cardiac syndrome characterized by transient wall motion abnormalities extending beyond a single epicardial vessel in the absence of significant obstructive coronary artery disease. Aim: This study was to describe the clinical characteristics of TC in North America. Materials and Methods: We identified 10 patients who met the Mayo Clinic criteria for TC using our Electronic Medical Re...

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

    Directory of Open Access Journals (Sweden)

    Patel

    2014-05-01

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

  7. Imaging features of posterior mediastinal chordoma in a child

    Energy Technology Data Exchange (ETDEWEB)

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

    2007-05-15

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

  8. Histochemical and immunohistochemical characterization of chordoma in ferrets

    Science.gov (United States)

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

    2015-01-01

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

  9. Computer Navigation-aided Resection of Sacral Chordomas

    Directory of Open Access Journals (Sweden)

    Yong-Kun Yang

    2016-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2016-10-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    Hudson, T.M.; Galceran, M.

    1983-05-01

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

  13. Sex differences in clinical characteristics and outcomes after myocardial infarction

    DEFF Research Database (Denmark)

    Lam, Carolyn S P; McEntegart, Margaret; Claggett, Brian;

    2015-01-01

    BACKGROUND: We examined the association of sex with clinical characteristics and outcomes in patients following myocardial infarction (MI) in the Valsartan in Acute Myocardial Infarction Trial (VALIANT). METHODS AND RESULTS: A total of 4570 women and 10 133 men with heart failure (HF), left ventr...

  14. Characteristics and Clinical Practices of Rural Marriage and Family Therapists

    Science.gov (United States)

    Morris, James

    2007-01-01

    This report presents a subset of data collected from the American Association for Marriage and Family Therapy (AAMFT) Practice Research Network project conducted in 2002. A sample of 47 clinical members of AAMFT who indicated they practiced in a rural community provided descriptive information on demographic characteristics, training, clinical…

  15. [Hereditary optic neuropathies: clinical and molecular genetic characteristics].

    Science.gov (United States)

    Khanakova, N A; Sheremet, N L; Loginova, A N; Chukhrova, A L; Poliakov, A V

    2013-01-01

    The article presents a review of literature on hereditary optic neuropathies: Leber mitochondrial hereditary optic neuropathy, autosomal dominant and autosomal recessive optic neuropathies, X-linked optic atrophy. Clinical and molecular genetic characteristics are covered. Isolated optic neuropathies, as well as hereditary optic disorders, being a part of a complex syndromic disease are described.

  16. Clinical and Biochemical Characteristics of Children with Juvenile Idiopathic Arthritis

    International Nuclear Information System (INIS)

    Objective: To determine the clinical and biochemical characteristics of children with Juvenile Idiopathic Arthritis (JIA) at a tertiary care centre in Karachi, Pakistan. Study Design: A descriptive study. Place and Duration of Study: Paediatric Rheumatology Clinic of The Aga Khan University Hospital (AKUH), Karachi, from January 2008 to December 2011. Methodology: Clinical and laboratory profile and outcome of children less than 15 years of age attending the Paediatric Rheumatology Clinic of the Aga Khan University, Karachi with the diagnosis of Juvenile Idiopathic Arthritis according to International League against Rheumatism were studied. These children were classified into different types of JIA; their clinical and laboratory characteristics, response to therapy and outcome was evaluated. Results: Sixty eight patients satisfying the criteria of International League against Rheumatism (ILAR) for Juvenile Idiopathic Arthritis were enrolled during the study period of four consecutive years, their age ranged from 9 months to 15 years. Mean age at onset was 6.45 +- 4.03 years while mean age at diagnosis was 7.60 +- 3.93 years. Polyarticular was the most predominant subtype with 37 (54%) patients, out of these, 9 (24%) were rheumatoid factor positive. An almost equal gender predisposition was observed. Fever and arthritis were the most common presenting symptoms, with only 2 patients presenting with uveitis. Conclusion: The clinico-biochemical characteristics of JIA at the study centre showed a pattern distinct with early onset of disease, high frequency of polyarticular type and a higher rheumatoid factor (QRA) and ANA positivity in girls. (author)

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2003-04-01

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

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

    Science.gov (United States)

    2014-01-01

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

  19. Genetic characteristics of Japanese clinical Listeria monocytogenes isolates.

    Directory of Open Access Journals (Sweden)

    Satoko Miya

    Full Text Available Listeria monocytogenes causes foodborne illnesses through consumption of ready-to-eat foods. Although 135-201annual listeriosis cases have been estimated in Japan, the details regarding the clinical isolates such as infection source, virulence level, and other genetic characteristics, are not known. In order to uncover the trends of listeriosis in Japan and use the knowledge for prevention measures to be taken, the genetic characteristics of the past human clinical isolates needs to be elucidated. For this purpose, multilocus tandem-repeat sequence analysis (MLTSA and multi-virulence-locus sequence typing (MVLST were used in this study. The clinical isolates showed a variety of genetically distant genotypes, indicating they were from sporadic cases. However, the MVLST profiles of 7 clinical isolates were identical to those of epidemic clone (EC I isolates, which have caused several serious outbreaks in other countries, suggesting the possibility that they have strong virulence potential and originated from a single outbreak. Moreover, 6 Japanese food isolates shared their genotypes with ECI isolates, indicating that there may be risks for listeriosis outbreak in Japan. This is the first investigational study on genetic characteristics of Japanese listeriosis isolates. The listeriosis cases happened in the past are presumably sporadic, but it is still possible that some isolates with strong virulence potential have caused listeriosis outbreaks, and future listeriosis risks also exist.

  20. Clinical and ultrasonographic characteristics of salivary mucoceles in 13 dogs.

    Science.gov (United States)

    Torad, Faisal A; Hassan, Elham A

    2013-01-01

    Salivary mucocele is one of the causes of submandibular swelling in dogs and is due to a collection of mucoid saliva that has leaked from a damaged salivary gland. The purpose of this case series report was to describe the clinical and ultrasonographic characteristics of confirmed salivary mucoceles in 13 dogs admitted to the Faculty of Veterinary Medicine at Cairo University. The final diagnosis of salivary mucocele was based on aspirate cytology for all dogs and additional surgical excision for seven dogs. For dogs admitted from 2 weeks to 1 month from the onset of clinical signs, the cervical mucocele appeared as a round echogenic structure with a large volume of central anechoic content. The wall was a clearly identified hyperechoic structure surrounding the gland. For dogs admitted between 1 to 2 months from the onset of clinical signs, the volume of anechoic material appeared less than that seen in the acute cases. The overall appearance of the salivary mucocele was heterogenous. For dogs admitted after 2 months from the onset of clinical signs, the salivary mucocele appeared grainy or mottled, with a heterogenous appearance and a further decrease in anechoic content. For one dog that presented after 3 months from the onset of clinical signs, the salivary mucocele was hard on palpation and appeared hyperechoic with distal acoustic shadowing. Findings from this study indicated that ultrasonographic characteristics of salivary mucoceles in dogs vary depending on the chronological stage of the disease. PMID:23442204

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

    Science.gov (United States)

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

    2015-01-01

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

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

    International Nuclear Information System (INIS)

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

  3. Clinical characteristics associated with illness perception in psoriasis.

    Science.gov (United States)

    Wahl, Astrid K; Robinson, Hilde S; Langeland, Eva; Larsen, Marie H; Krogstad, Anne-Lene; Moum, Torbjørn

    2014-05-01

    Knowledge of illness perception may aid the identification of groups of patients with a higher risk of coping poorly with the demands of their illness. This study aims to investigate associations between illness perception, clinical characteristics, patient knowledge, quality of life and subjective health in persons with psoriasis. The present study was based on cross-sectional data from patients awaiting climate therapy in Gran Canaria. We included 254 eligible patients (74%) who completed a questionnaire including the revised Illness Perception Questionnaire, the Psoriasis Knowledge Questionnaire, and the Dermatological Life Quality Index. Disease severity was measured using the Psoriasis Area and Severity Index. Several statistically significant associations between clinical characteristics, knowledge and various illness perception dimensions were found. Illness perception was also significantly related to disease-specific quality of life and subjective health. These findings contradict previous findings, which suggested that objective disease factors are not relevant to illness perception in psoriasis.

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

    International Nuclear Information System (INIS)

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

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

    International Nuclear Information System (INIS)

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

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

    Science.gov (United States)

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

    2012-11-01

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

  7. Clinical characteristics, prognosis and treatment for pelvic cryptorchid seminoma

    OpenAIRE

    Li; Coucke, Philippe; Qian,, S.J..; Huang, Yi-Rong; Gu, Da-Zhong; Mirimanoff, René-Olivier; Yu, Zi-Hao

    1997-01-01

    Purpose: To analyze the clinical characteristics, prognosis, and treatment outcome of pelvic cryptorchid seminoma (PCS), and to determine whether whole abdominal-pelvic irradiation for Stage I disease is necessary. Methods and Materials: From 1958 to 1991,60 patients with PCS were treated at the Cancer Hospital of Chinese Academy of Medical Sciences, Beijing. They presented with a lower abdominal mass and showed a predominance for the right side. A high proportion of patients with...

  8. Clinical characteristics of sarcoidosis patients diagnosed in a university hospital

    OpenAIRE

    Özlem Abakay; Abdurrahman Abakay; Abdullah Çetin Tanrıkulu; Fatih Meteroğlu; Cengizhan Sezgi; Hadice Selimoğlu Şen; Ayşe Dallı; Mehmet Kabak

    2012-01-01

    Objectives: The clinical characteristics and treatment resultsof patients diagnosed with sarcoidosis was investigatedin Dicle University Medical Faculty Hospital.Materials and methods: A total of 39 patients were includeddiagnosed with sarcoidosis between 01 January2008 and31 December 2011.Demographic data, laboratoryfindings, spirometric test data, diagnostic methodsand treatment regimens for the study were recorded form.According to pulmonary function test results of patients,61.5% were nor...

  9. Investigation of Tinnitus Patients in Italy: Clinical and Audiological Characteristics

    OpenAIRE

    Francesco Martines; Daniela Bentivegna; Fabiola Di Piazza; Enrico Martines; Vincenzo Sciacca; Gioacchino Martinciglio

    2010-01-01

    Objective. 312 tinnitus sufferers were studied in order to analyze: the clinical characteristics of tinnitus; the presence of tinnitus-age correlation and tinnitus-hearing loss correlation; the impact of tinnitus on subjects' life and where possible the etiological/predisposing factors of tinnitus. Results. There is a slight predominance of males. The highest percentage of tinnitus results in the decades 61–70. Of the tinnitus sufferers, 197 (63.14%) have a hearing deficit (light hearing loss...

  10. Clinical Characteristics and Awareness of Skin Cancer in Hispanic Patients

    OpenAIRE

    Javed, Saba; Javed, Syed A; Mays, Rana M; Tyring, Stephen K.

    2013-01-01

    Skin cancer in darker skin is associated with considerable morbidity and mortality. We sought to assess the clinical characteristics of cutaneous malignancy amongst Hispanic skin cancer patients and compare them to age-matched non-Hispanic Caucasians.  In this retrospective study, 150 Hispanic skin cancer patients were identified from electronic medical records and age-matched to 150 non-Hispanic Caucasian controls with skin cancer.  The incidence of actinic keratoses (AKs) in Hispanic skin c...

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

    Science.gov (United States)

    Safari, Mojdeh; Khoshnevisan, Alireza

    2014-01-01

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

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

    Science.gov (United States)

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

    2009-07-01

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

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

    Directory of Open Access Journals (Sweden)

    Zhen Wu

    2013-10-01

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

  14. Clinical and Epidemiologic Characteristics of Patients with Childhood Psoriazis Seen in Dermatology Clinic

    OpenAIRE

    Emine Elvan Taşğın; Göknur Kalkan; Hatice Meral Ekşioğlu; Güler Vahaboğlu

    2013-01-01

    Objective: To evaluate the epidemiologic characteristics of the pediatric patients with psoriazis applied to our outpatients' clinic and compare these data with the literature. Methods: The study population consisted of 37 patients younger than 16 years with the diagnosis of psoriazis in dermatology outpatient clinic between May 2009 and December 2010. The patients were evaluated with regard to age, gender, age of onset of the disease, duration of the disease, the presence of accompanying ...

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2000-06-01

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

  16. Computer Navigation-aided Resection of Sacral Chordomas

    Institute of Scientific and Technical Information of China (English)

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

    2016-01-01

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

  17. Chordoma. Report on treatment results in eighteen cases

    Energy Technology Data Exchange (ETDEWEB)

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

    1986-01-15

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

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

    International Nuclear Information System (INIS)

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1998-09-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2012-08-15

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

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

    Directory of Open Access Journals (Sweden)

    Naoshi Hagihara

    2012-01-01

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

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

    Science.gov (United States)

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

    2014-03-16

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1992-05-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2002-07-01

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

  10. Site Characteristics Influencing the Translation of Clinical Research Into Clinical Practice

    DEFF Research Database (Denmark)

    Smed, Marie; Getz, Kenneth A.

    2014-01-01

    , although both academic and independent sites generate the same level of knowledge, academic sites share more of this knowledge with sponsor companies. This study suggests new strategies that sponsors can leverage to drive greater transfer of clinical research knowledge into clinical practice and ultimately...... on to sponsor companies and may ultimately assist in positioning new products and driving commercialization success. This study evaluates site characteristics that influence the acquisition and sharing of knowledge gained through clinical trial experience. The impact of 2 central site characteristics...... on the process of translating drug experience is assessed: site location (North America/rest of the world) and site type or setting (academic/independent). The results show that investigative sites located outside North America generate and share more knowledge than those within North America. Furthermore...

  11. Characteristics of liver cancer stem cells and clinical correlations.

    Science.gov (United States)

    Cheng, Zhuo; Li, Xiaofeng; Ding, Jin

    2016-09-01

    Liver cancer is an aggressive malignant disease with a poor prognosis. Patients with liver cancer are usually diagnosed at an advanced stage and thus miss the opportunity for surgical resection. Chemotherapy and radiofrequency ablation, which target tumor bulk, have exhibited limited therapeutic efficacy to date. Liver cancer stem cells (CSCs) are a small subset of undifferentiated cells existed in liver cancer, which are considered to be responsible for liver cancer initiation, metastasis, relapse and chemoresistance. Elucidating liver CSC characteristics and disclosing their regulatory mechanism might not only deepen our understanding of the pathogenesis of liver cancer but also facilitate the development of diagnostic, prognostic and therapeutic approaches to improve the clinical management of liver cancer. In this review, we will summarize the recent advances in liver CSC research in terms of the origin, identification, regulation and clinical correlation.

  12. Characteristics of liver cancer stem cells and clinical correlations.

    Science.gov (United States)

    Cheng, Zhuo; Li, Xiaofeng; Ding, Jin

    2016-09-01

    Liver cancer is an aggressive malignant disease with a poor prognosis. Patients with liver cancer are usually diagnosed at an advanced stage and thus miss the opportunity for surgical resection. Chemotherapy and radiofrequency ablation, which target tumor bulk, have exhibited limited therapeutic efficacy to date. Liver cancer stem cells (CSCs) are a small subset of undifferentiated cells existed in liver cancer, which are considered to be responsible for liver cancer initiation, metastasis, relapse and chemoresistance. Elucidating liver CSC characteristics and disclosing their regulatory mechanism might not only deepen our understanding of the pathogenesis of liver cancer but also facilitate the development of diagnostic, prognostic and therapeutic approaches to improve the clinical management of liver cancer. In this review, we will summarize the recent advances in liver CSC research in terms of the origin, identification, regulation and clinical correlation. PMID:26272183

  13. Clinical characteristics of 41 patients with intractable asthma

    OpenAIRE

    Bai, Li; Zhang, Xu-Sheng; Wang, Bin; Xiao-ming CHENG; Zhang, Qiao; Hua-ping CHEN; Ma, Qian-li; Zou, Li-Guang; Wang, Chang-Zheng

    2011-01-01

    Objective To explore the clinical characteristics of intractable asthma,and to provide new knowledge for diagnosis and treatment of the disease.Methods Forty one patients with intractable asthma,admitted to the Institute of Respiratory Disease,Xinqiao Hospital of Third Military Medical University from Jan.2009 to Dec.2010,were included in present study.Spirometry tests were performed for all the 41 patients.Cell classification and counting were done in the induced sputum of 37 patients,and 34...

  14. Clinical and the demographic characteristics of patients with alopecia areata

    OpenAIRE

    Mustafa Arıca; Roza Zelal Abdioğlu; Ruken Azizoğlu Anlı; Sibel Yorgancılar

    2013-01-01

    Background and Design: In this study, our aim was to determine clinical and the demographical characteristics of the patients with alopecia areata in our region.Materials and Metods: In the study, 100 patients who received alopecia areata diagnosis with ages raging from 2 to 52 and who applied to the polyclinic of ………… Medical Faculty Dermatology Department between October and November 2009 were evaluated.Results: Of 100 patients included into the study 44 (44%) were female and 56 (56%) male....

  15. Clinical characteristics of patients with persistent postural-perceptual dizziness

    Directory of Open Access Journals (Sweden)

    Roseli Saraiva Moreira Bittar

    2015-06-01

    Full Text Available INTRODUCTION: Persistent postural-perceptual dizziness is the dizziness that lasts for over three months with no clinical explanation for its persistence. The patient's motor response pattern presents changes and most patients manifest significant anxiety. OBJECTIVE: To evaluate the clinical characteristics of patients with persistent postural and perceptual dizziness. METHODS: statistical analysis of clinical aspects of patients with persistent postural-perceptual dizziness. RESULTS: 81 patients, average age: 50.06 ± 12.16 years; female/male ratio: 5.7/1; main reasons for dizziness: visual stimuli (74%, body movements (52%, and sleep deprivation (38%. The most prevalent comorbidities were hypercholesterolemia (31%, migraine headaches (26%, carbohydrate metabolism disorders (22% and cervical syndrome (21%. DHI, State-Trait Anxiety Inventory - Trait, Beck Depression Inventory, and Hospital Anxiety and Depression Scale questionnaires were statistically different (p < 0.05 when compared to controls. 68% demonstrated clinical improvement after treatment with serotonin reuptake inhibitors. CONCLUSION: Persistent postural-perceptual dizziness affects more women than men, with a high associated prevalence of metabolic disorders and migraine. Questionnaires help to identify the predisposition to persistent postural-perceptual dizziness. The prognosis is good with adequate treatment.

  16. Characteristics of demand and psychological treatments in a university clinic

    Directory of Open Access Journals (Sweden)

    Francisco J. Labrador

    2016-04-01

    Full Text Available The objectives of this study are to describe the most common characteristics of patients receiving psychological treatment and the treatments administered. We analyzed a sample of 856 patients at the University Psychology Clinic of the Complutense University of Madrid. Five diagnostic categories accounted for 78.4% of demand: anxiety disorders (31.9%, no diagnosis (15.4%, other problems requiring clinical attention (14.2%, mood disorders (9.5% and adaptive disorders (7.4%. A total of 17.7% presented a comorbid diagnosis and 49.3% had received treatment previously. The mean of assessment and treatment sessions was 3.5 and 12.7, respectively. The most commonly applied techniques included psychoeducation (95.1%, cognitive restructuring (74.8%, relaxation (74.4%, and control of internal dialogue (68.1%.Of the patients that had finished contact with the clinic, 68.3% were a therapeutic success. We discuss the generalization of the results and the implications for the profession and clinical practice.

  17. Sociodemographic and clinical characteristics of patients with recurrent aphthous stomatitis

    Directory of Open Access Journals (Sweden)

    Anıl Gülsel Bahalı

    2014-12-01

    Full Text Available Background and Design: The purpose of this study was to obtain data that may provide an insight into the etiopathogenesis of recurrent aphtous stomatitis (RAS by the way of analysing the sociodemographic and clinical characteristics of patients who had been diagnosed with RAS. Materials and Metods: The patients, who were diagnosed with RAS in the dermatology outpatient clinic, between May 2007 and May 2010, were evaluated retrospectively. The data including sociodemografic and clinical characteristics, and treatment options were recorded. Results: A hundred patients (68 women, 32 men were included in this study. The average age was 40±13.6 years. RAS was more common in patients with middle-income and low education. The most common type of RAS was minor aphtous ulcers (88%. The lesions were most frequently seen on the lateral side of the tongue (34% and cheek (34%. Sixty percent of patients had a positive family history. Some factors such as biting (12%, tooth brushing (18%, dental disease presence (82%, food (39%, menstruation (10.3%, stress (76%, iron deficiency (16.7%, vitamin B12 deficiency (22.4%, low serum ferritin levels (18%, and seasonal variability (32% showed positive correlation with RAS. A negative correlation was found between RAS and smoking. Forty-nine percent of patients had used alternative therapies in addition to drug therapy. The most frequently used alternative method was consumption of sumac (26.5%. Conlucions: In contrast to the literature, our study found that RAS is started in the third decade of life and, approximately 50% of patients prefered alternative treatment methods, particularly sumac. Nowadays, discussions about the etiopathogenesis of RAS continue. In this study, we found that different sociodemographic and clinical factors may be associated with the etiopathogenesis of the disease. Our study will be followed by further studies using prospective design to identify the the etiopathogenesis of RAS.

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

    Science.gov (United States)

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

    2014-01-01

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

  19. Clinical, angiographic and procedural characteristics of longitudinal stent deformation.

    Science.gov (United States)

    Guler, A; Guler, Y; Acar, E; Aung, S M; Efe, S C; Kilicgedik, A; Karabay, C Y; Barutcu, S; Tigen, M K; Pala, S; İzgi, A; Esen, A M; Kirma, C

    2016-08-01

    Recently, longitudinal stent deformation (LSD) has been reported increasingly. Even though the reported cases included almost all stent designs, most cases were seen in the Element™ stent design (Boston Scientific, Natick, MA, USA). It is considered that stent design, lesion and procedural characteristics play a role in the etiology of LSD. Yet, the effect of LSD on long-term clinical outcomes has not been studied well. Element stents implanted between January 2013 and April 2015 in our hospital were examined retrospectively. Patients were grouped into two according to the presence of LSD, and their clinical, lesion and procedural characteristics were studied. Twenty-four LSD's were detected in 1812 Element stents deployed in 1314 patients (1.83 % of PCI cases and 1.32 % of all Element stents). LMCA lesions (16.7 % vs 1.6 %, p LSD than in cases without it. In addition, the number of stents, stent inflation pressure and the use of post-dilatation were significantly different between the two groups. Two patients had an adverse event during the follow-up period. LSD is a rarely encountered complication, and is more common in complex lesions such as ostial, bifurcation and LMCA lesions. The use of extra-support guiding catheter, extra-support guidewires and low stent inflation pressure increases the occurrence of LSD. Nevertheless, with increased awareness of LSD and proper treatment, unwanted long-term outcomes can be successfully prevented. PMID:27198891

  20. Clinical Characteristics of Cases with Spinal Muscular Atrophy

    Directory of Open Access Journals (Sweden)

    Mehmet Canpolat

    2016-04-01

    Full Text Available Introduction: The aim of this study is was to evaluate the clinical features of cases with diagnosis of spinal muscular atrophy (SMA. Materials and Methods: Thirty-eight pediatric patients were evaluated retrospectively. All patients were followed in the Pediatric Neurology Department of Erciyes University Faculty of Medicine. The diagnosis of patients had been confirmed by genetic analysis of homozygous deletions of survival motor neuron 1 gene. Detailed history, newborn symptoms, nutritional characteristics, initial complaints, physical examination, concomitant pathologies, genetic characteristics, and treatment modalities were investigated in all patients. Results: The study population consisted of 19 boys (50% and 19 girls (50%. The mean age of patients was 26.9±25.7 months (range: 3-96 months. The mean follow-up period was 12.2±13.3 months (range: 2-48 months. According to SMA classification, 22 patients (57.8% were type 1, 8 patients (21.1% were type 2, and 8 patients were (21.1% type 3. Neonatal respiratory distress, age at early diagnosis, nutritional problems, and recurrent lung diseases were detected as poor prognostic factors. Conclusions: SMA is a neuromuscular disease that requires multidisciplinary approach to medical care. There is a wide range of clinical severity. Identification of poor prognostic factors will help in terms of guiding close monitoring and timely treatments of children with SMA.

  1. Clinical Characteristics of Epidermoid Cysts of the External Auditory Canal

    Science.gov (United States)

    Kim, Go-Woon; Park, Jang-Hee; Kwon, Oh-Joon; Kim, Dong Hyun

    2016-01-01

    Background and Objectives The epidermoid cyst is a common benign disease of the skin caused by inflammation of hair cortex follicles and proliferation of epidermal cells within the dermis or superficial subcutaneous tissue. The purpose of this study was to investigate the characteristics of epidermoid cysts of the external auditory canal (EAC) by analyzing the clinical and radiologic features. Subjects and Methods The clinical records were retrospectively reviewed for patients diagnosed with epidermoid cyst of the EAC from March 2004 to December 2013. The epidermoid cysts were diagnosed clinically by endoscopy and microscopy examinations and by temporal bone CT images, and were confirmed by histopathologic examination. Characteristics of epidermoid cysts in bony EAC and cartilaginous EAC were compared. Results Eight patients had an epidermoid cyst in the bony EAC and nine patients had one in the cartilaginous EAC. Swelling and otalgia were common symptoms, but 47% of cysts were found incidentally. The mean age of patients was 49.6 years (age range, 26-67 years) in the bony EAC cases and 26.1 years (age range, 6-57 years) in the cartilaginous EAC cases. The mean size of the epidermoid cyst was 3.50 mm (size range, 2-7 mm) in the bony EAC cases and 9.55 mm (size range, 2-20 mm) in the cartilaginous EAC cases. Conclusions Comparison of epidermoid cysts of the bony EAC and the cartilaginous EAC revealed that epidermoid cysts of the bony EAC is usually found incidentally, arose in older patients and had smaller size. PMID:27144232

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1988-03-01

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

  3. Clinical and genetic characteristics of craniosynostosis in Hungary.

    Science.gov (United States)

    Bessenyei, Beáta; Nagy, Andrea; Szakszon, Katalin; Mokánszki, Attila; Balogh, Erzsébet; Ujfalusi, Anikó; Tihanyi, Mariann; Novák, László; Bognár, László; Oláh, Éva

    2015-12-01

    Craniosynostosis, the premature closure of cranial sutures, is a common craniofacial disorder with heterogeneous etiology and appearance. The purpose of this study was to investigate the clinical and molecular characteristics of craniosynostoses in Hungary, including the classification of patients and the genetic analysis of the syndromic forms. Between 2006 and 2012, 200 patients with craniosynostosis were studied. Classification was based on the suture(s) involved and the associated clinical features. In syndromic cases, genetic analyses, including mutational screening of the hotspot regions of the FGFR1, FGFR2, FGFR3, and TWIST1 genes, karyotyping and FISH study of TWIST1, were performed. The majority (88%) of all patients with craniosynostosis were nonsyndromic. The sagittal suture was most commonly involved, followed by the coronal, metopic, and lambdoid sutures. Male, twin gestation, and very low birth weight were risk factors for craniosynostosis. Syndromic craniosynostosis was detected in 24 patients. In 17 of these patients, Apert, Crouzon, Pfeiffer, Muenke, or Saethre-Chotzen syndromes were identified. In one patient, multiple-suture craniosynostosis was associated with achondroplasia. Clinical signs were not typical for any particular syndrome in six patients. Genetic abnormalities were detected in 18 syndromic patients and in 8 relatives. In addition to 10 different, known mutations in FGFR1,FGFR2 or FGFR3, one novel missense mutation, c.528C>G(p.Ser176Arg), was detected in the TWIST1 gene of a patient with Saethre-Chotzen syndrome. Our results indicate that detailed clinical assessment is of paramount importance in the classification of patients and allows indication of targeted molecular testing with the highest possible diagnostic yield. PMID:26289989

  4. Proton Therapy for Reirradiation of Progressive or Recurrent Chordoma

    Energy Technology Data Exchange (ETDEWEB)

    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

  5. Clinical manifestation, imageological and pathological characteristics of Wernicke encephalopathy

    Institute of Scientific and Technical Information of China (English)

    Shunchang Han; Chuanqiang Pu; Qiuping Gui; Xusheng Huang; Senyang Lang; Weiping Wu; Peifu Wang

    2006-01-01

    BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been reported suecessively. But its imageological detection needs to be further investigated.OBJECTIVE: To analyze the eharacteristics of clinical manifestations, skull MRI examination and pathological results in patients with WE.DESTGN: Retrospective analysis.SETTTNG: The General Hospital of Chinese PLA.PARTTCTPANTS: Ten patients of WE admitted to the Department of Neurology, General Hospital of Chinese PLA were recruited. Among them, five patients were diagnosed pathologically after death. Their pathological changes accorded with the pathological characteristics of WE. The other 5 patients were diagnosed clinically before death. Their pathological changes accorded with clinical and imageological manifestations and had definite reaction to the treatment of thiamine. Ten patients, 7 males and 3 females, were aged (47±13) years ranging from 33 to 73 years. Their disease courses averaged 6 weeks ranging from 3 to 10 weeks. They all were non-alcoholics. Four patients developed WE after acute pancreatitis, two patients after the recurrence of gastric cancer, two patients after cholecystectomy, one patient after hepatitis medicamentosa, one patient after Alzheimer disease. Informed consents were obtained from all the patients and their relatives.METHODS: After admission, clinical manifestations of patients were observed and recorded. Five patients underwent skull MRI examination and their detected results were recorded. Five dead patients underwent autopsy and brain pathological examinations. Neuropathological examination involved cerebrum, cerebellum and brain stem.MATN OUTCOME MEASURES: Clinical manifestations, MRI examination results, pathological analysis results and prognosis of all the patients.RESULTS: Ten patients with WE were involved in the final

  6. Clinical and Molecular Characteristics of Post-Colonoscopy Colorectal Cancer

    DEFF Research Database (Denmark)

    Stoffel, Elena M; Erichsen, Rune; Frøslev, Trine;

    2016-01-01

    BACKGROUND AND AIMS: Colonoscopy provides incomplete protection from colorectal cancer (CRC), but determinants of post-colonoscopy CRC are not well understood. We compared clinical features and molecular characteristics of CRCs diagnosed at different time intervals after a previous colonoscopy....... METHODS: We performed a population-based, cross-sectional study of incident CRC cases in Denmark (2007-2011), categorized as post-colonoscopy or detected during diagnostic colonoscopy (in patients with no prior colonoscopy). We compared prevalence of proximal location and DNA mismatch repair deficiency (d......MMR) in CRC tumors, relative to time since previous colonoscopy, using logistic regression and cubic splines to assess temporal variation. RESULTS: Of 10,365 incident CRCs, 725 occurred after colonoscopy examinations (7.0%). These were more often located in the proximal colon (odds ratio [OR], 2.34; 95% CI, 1...

  7. Isolation Frequency Characteristics of Candida Species from Clinical Specimens

    Science.gov (United States)

    Kim, Ga-Yeon; Jeon, Jae-Sik

    2016-01-01

    Candida spp. is an invasive infectious fungus, a major risk factor that can increase morbidity and mortality in hospitalized patients. In this study, 2,508 Candida spp. were isolated from various clinical specimens collected from university hospitals from July 2011 to October 2014. They were identified in order to determine isolation frequencies and characteristics by specimen, gender, age group, year, season, and month. The strain-specific isolation rate of Candida spp. is in the order of Candida albicans (1,218 strains, 48.56%), Candida glabrata (416 strains, 16.59%), Candida utilis (305 strains, 12.16%), Candida tropicalis (304 strains, 12.12%), and Candida parapsilosis (116 strains, 4.63%) and these five species accounted for more than 94% of the total strains. Of the specimens, Candida spp. were most frequently isolated from urine-catheter, followed by urine-voided, blood, sputum, other, open pus, vaginal discharge, Tip, ear discharge, bronchial aspiration and bile, in that order. Looking at the age distribution, the detection rate of patients in their 60s and older was significantly higher at 75.8% (1,900/2,508). The detection rate of patients in their 20s and younger was shown to be very low at 2.55% (64/2,508). By year, the detection rate of non-albicans Candida spp. showed a tendency to gradually increase each year compared with C. albicans. As isolation of Candida spp. from clinical samples at the specie level can vary depending on characteristics of the patient, sample, season, etc., continual studies are required. PMID:27433120

  8. Clinical characteristics of 41 patients with intractable asthma

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    Li BAI

    2011-09-01

    Full Text Available Objective To explore the clinical characteristics of intractable asthma,and to provide new knowledge for diagnosis and treatment of the disease.Methods Forty one patients with intractable asthma,admitted to the Institute of Respiratory Disease,Xinqiao Hospital of Third Military Medical University from Jan.2009 to Dec.2010,were included in present study.Spirometry tests were performed for all the 41 patients.Cell classification and counting were done in the induced sputum of 37 patients,and 34 patients underwent high-resolution chest computed tomography(HRCT.Results Incomplete reversibility of airflow obstruction(FEV1/FVC 0.03 of the total cells,13(35.1% and increased neutrophils( > 0.61 of total cells,6(16.2% showed increased both eosinophils and neutrophils,and only that of one patient showed normal percentage of the eosinophils and neutrophils.Chest HRCT of 34 patients showed thickening of bronchial wall in visible segment in 28 cases(82.3%,and in 22 cases(64.7% thickening of bronchial wall in secondary segments was accompanied with narrowed bronchus lumen,cylindrical bronchiectasis was predominant in 7 patients,and centrilobular emphysema was seen in 5 patients.Conclusion Airway remodeling,incomplete reversibility of airflow obstruction,airway inflammation appear to be the major clinical characteristics of intractable asthma.Combined use of chest HRCT,spirometry test,and cellular analysis of induced sputum may be helpful for identifying intractable asthma,and they provide the basis for individualized strategies to manage the disease.

  9. Isolation Frequency Characteristics of Candida Species from Clinical Specimens.

    Science.gov (United States)

    Kim, Ga-Yeon; Jeon, Jae-Sik; Kim, Jae Kyung

    2016-06-01

    Candida spp. is an invasive infectious fungus, a major risk factor that can increase morbidity and mortality in hospitalized patients. In this study, 2,508 Candida spp. were isolated from various clinical specimens collected from university hospitals from July 2011 to October 2014. They were identified in order to determine isolation frequencies and characteristics by specimen, gender, age group, year, season, and month. The strain-specific isolation rate of Candida spp. is in the order of Candida albicans (1,218 strains, 48.56%), Candida glabrata (416 strains, 16.59%), Candida utilis (305 strains, 12.16%), Candida tropicalis (304 strains, 12.12%), and Candida parapsilosis (116 strains, 4.63%) and these five species accounted for more than 94% of the total strains. Of the specimens, Candida spp. were most frequently isolated from urine-catheter, followed by urine-voided, blood, sputum, other, open pus, vaginal discharge, Tip, ear discharge, bronchial aspiration and bile, in that order. Looking at the age distribution, the detection rate of patients in their 60s and older was significantly higher at 75.8% (1,900/2,508). The detection rate of patients in their 20s and younger was shown to be very low at 2.55% (64/2,508). By year, the detection rate of non-albicans Candida spp. showed a tendency to gradually increase each year compared with C. albicans. As isolation of Candida spp. from clinical samples at the specie level can vary depending on characteristics of the patient, sample, season, etc., continual studies are required.

  10. Clinical characteristics of patients in the persistent vegetative state.

    Science.gov (United States)

    Tresch, D D; Sims, F H; Duthie, E H; Goldstein, M D; Lane, P S

    1991-05-01

    Little is known concerning the specific clinical characteristics of patients in persistent vegetative states (PVS). Fifty-one patients from four nursing homes, approximately 3% of the total patients, were identified as being in a PVS. The mean age of the patients was 64.8 +/- 3.2 years (range, 19 to 96 years) and the mean duration of the PVS was 3.3 +/- 5.0 years (range, 1 to 16.8 years), with 13 patients' PVS being longer than 5 years. Cerebrovascular accidents and dementia were the most common causes of the PVS, accounting for 32 of the cases (63%). In the younger patients cerebral trauma secondary to motor vehicle accidents was the most common cause. All 51 patients were fed via tube feeding and 35 patients had urinary catheters (75%). All patients were receiving daily medications, with greater than 50% taking daily vitamins. Over 30% were taking digitalis and/or diuretics and over 32% were taking H2 blockers. Transfer of patients to an acute care hospital was not uncommon, with 31 patients (61%) requiring 63 acute care hospitalizations during their stay in the nursing home. As expected, infections were the most common reason for acute care hospitalization, although 15 of the patients were hospitalized for surgical procedures. Another common problem encountered by the patients was pressure sores, with 78% of patients requiring specific therapy for at least one pressure sore. Surprisingly, only 27 (53%) of 51 patients had a specific resuscitation status designation in the medical chart, and neither presence of a chart designation nor specific resuscitation order was related to the patient's age or the cause or duration of PVS. From these data it would appear that clinical characteristics of patients in PVS are variable. Some patients are young, others are old. The cause varies from cerebrovascular accidents to cerebral trauma. Survival may be prolonged; complications are not uncommon, with some patients requiring acute care hospitalization. PMID:2025140

  11. Clinical characteristics of 4355 patients with anterior cruciate ligament injury

    Institute of Scientific and Technical Information of China (English)

    MEI Yu; AO Ying-fang; WANG Jian-quan; MA Yong; ZHANG Xin; WANG Jia-ning; ZHU Jing-xian

    2013-01-01

    Background Clinical features of anterior cruciate ligament (ACL) injury are important for its prevention,diagnosis and treatment.However,few studies have reported such data,especially in China.The purpose of this study was to describe the clinical characteristics of ACL injury on a large cohort.Methods Between 1993 and 2007,a total of 4355 ACL deficient inpatients (612 athletes and 3743 non-athletes) were registered.Data were collected using a special database system.And the distributions of characteristics in different groups were compared and analyzed statistically.Results All subjects were confirmed with ACL tear during surgery.Statistical analysis revealed that the percentage of females in Athlete Group was significantly higher than that in Non-athlete Group (56.05% vs.24.95%,P<0.001).This study also found that sports trauma was the main cause of ACL tears.Soccer,basketball,judo,wrestling and track and field were the five most responsible activities for athletes.The average injury time for athletes was significantly shorter than that for non-athletes (413.3 days vs.717.5 days,P<0.001).Three thousand nine hundred and eight cases were ordered ACL reconstruction (76.04% single-bundle,18.30% double-bundle).Three hundred and forty-five patients (7.92%)were combined with other ligaments injuries,2667 (61.24%) were found with various grades of cartilage lesions,and 3377 (77.54%) were found with meniscal injury.Conclusions Sports trauma was the main cause of ACL tears in China,and reconstruction had become the principal surgical choice.In order to restore knee joint stability and reduce the incidence of cartilage and meniscal injury,patienttailored ACL reconstruction should be suggested at the right moment.

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

    Science.gov (United States)

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

    2014-01-01

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

  13. Clinical and laboratory characteristics of children with Kawasaki disease

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    Fatih Akın

    2015-03-01

    Full Text Available Objective: In this study, we aimed to evaluate clinical and laboratory characteristics of patients with Kawasaki disease (KD in Konya region of Turkey. Methods: The hospital records of patients who were hospitalized with the diagnosis of KD in the Pediatrics Clinics of Konya Training and Research Hospital between May 2010 and June 2012 were reviewed retrospectively. Results: Seven cases were found to have the diagnosis of KD, two of whom were incomplete KD. Oropharynx changes were the most common (100% feature in our patients. Five (71% patients had bulbar conjunctivitis. Three (43% patients had erythema at the site of BCG inoculation. Adenopathy was present in all of our patients with the classical form. A desquamation was observed in one case at the seventh day of fever. No cardiac manifestation was seen. Elevated erythrocyte sedimentation rate and thrombocytosis were present in all patients. All of the patients were received intravenous immunoglobulin in the first ten days of the fever. Conclusion: KD should be considered as a possible diagnosis in any child presenting with prolonged fever. BCG reaction can be attributed as a diagnostic criterion for incomplete form of the disease especially in countries where BCG vaccination is routinely performed. Early treatment is essential to prevent cardiovascular complications.

  14. Clinical and epidemiological characteristics of infectious keratitis in Paraguay.

    Science.gov (United States)

    Nentwich, Martin M; Bordón, M; di Martino, D Sánchez; Campuzano, A Ruiz; Torres, W Martínez; Laspina, F; Lichi, S; Samudio, M; Farina, N; Sanabria, Rosa R; de Kaspar, Herminia Mino

    2015-06-01

    To describe the clinical and epidemiological characteristics of patients with severe infectious keratitis in Asunción, Paraguay between April 2009 and September 2011. All patients with the clinical diagnosis of severe keratitis (ulcer ≥2 mm in size and/or central location) were included. Empiric treatment consisted of topical antibiotics and antimycotics; in cases of advanced keratitis, fortified antibiotics were used. After microbiological analysis, treatment was changed if indicated. In total 48 patients (62.5 % males, 25 % farmers) were included in the analysis. A central ulcer was found in 81.3 % (n = 39). The median delay between onset of symptoms and time of first presentation at our institution was 7 days (range 1-30 days). Fungal keratitis was diagnosed in 64.5 % (n = 31) of patients, of which Fusarium sp. (n = 17) was the most common. Twenty-one patients (43.8 %) reported previous trauma to the eye. The globe could be preserved in all cases. While topical therapy only was sufficient in most patients, a conjunctival flap was necessary in six patients suffering from fungal keratitis. The high rate of fungal keratitis in this series is remarkable, and microbiological analysis provided valuable information for the appropriate treatment. In this setting, one has to be highly suspicious of fungal causes of infectious keratitis.

  15. Clinical and molecular characteristics of pyometra in female dogs.

    Science.gov (United States)

    Hagman, R

    2012-12-01

    Pyometra is a common disease of female dogs. In Sweden, where approximately 90% of the dog population is intact (not neutered), nearly 25% of all female dogs are diagnosed with the disease before 10 years of age. In certain high-risk breeds, this risk of developing pyometra exceeds 50%. Various clinical signs associated with the genital tract as well as with systemic disease are present in dogs with pyometra. A frequent and serious consequence of the uterine infection is endotoxaemia and progression into the systemic inflammatory response syndrome (SIRS), or sepsis, and the disease is then regarded as a medical emergency. Acute phase proteins and inflammatory markers associated with SIRS and with the outcome as measured by length of hospitalization have been identified in blood samples. Recently, the inflammatory response in infected uterine tissue during pyometra has been more closely explored. The expression of many genes associated with chemokines, cytokines, inflammatory cell extravasation, anti-bacterial action, the complement system and innate immune responses and also a large panel of proteases are upregulated in the uterine tissue in pyometra. Products of certain upregulated genes may be detected systemically and used for diagnostic or prognostic purposes provided that tests are developed in the future. More knowledge of the complex local and systemic inflammatory response in pyometra may allow identification of novel disease biomarkers or future targets for treatment. In this article, clinical as well as molecular characteristics of the disease are reviewed. PMID:23279529

  16. Clinical trial participant characteristics and saliva and DNA metrics

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    Richards Julie

    2009-10-01

    Full Text Available Abstract Background Clinical trial and epidemiological studies need high quality biospecimens from a representative sample of participants to investigate genetic influences on treatment response and disease. Obtaining blood biospecimens presents logistical and financial challenges. As a result, saliva biospecimen collection is becoming more frequent because of the ease of collection and lower cost. This article describes an assessment of saliva biospecimen samples collected through the mail, trial participant demographic and behavioral characteristics, and their association with saliva and DNA quantity and quality. Methods Saliva biospecimens were collected using the Oragene® DNA Self-Collection Kits from participants in a National Cancer Institute funded smoking cessation trial. Saliva biospecimens from 565 individuals were visually inspected for clarity prior to and after DNA extraction. DNA samples were then quantified by UV absorbance, PicoGreen®, and qPCR. Genotyping was performed on 11 SNPs using TaqMan® SNP assays and two VNTR assays. Univariate, correlation, and analysis of variance analyses were conducted to observe the relationship between saliva sample and participant characteristics. Results The biospecimen kit return rate was 58.5% among those invited to participate (n = 967 and 47.1% among all possible COMPASS participants (n = 1202. Significant gender differences were observed with males providing larger saliva volume (4.7 vs. 4.5 ml, p = 0.019, samples that were more likely to be judged as cloudy (39.5% vs. 24.9%, p 0.21, P Conclusion Findings from this study show that demographic and behavioral characteristics of smoking cessation trial participants have significant associations with saliva and DNA metrics, but not with the performance of TaqMan® SNP or VNTR genotyping assays. Trial registration COMPASS; registered as NCT00301145 at clinicaltrials.gov.

  17. Clinical characteristics and prognostic factors of severe acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Lei Kong; Nn Santiago; Tian-Quan Han; Sheng-Dao Zhang

    2004-01-01

    AIM: To investigate the clinical characteristics and prognostic factors of a consecutive series of patients with severe acute pancreatitis (SAP).METHODS: Clinical data of SAP patients admitted to our hospital from January 2003 to January 2004 were retrospectively reviewed. Collected data included the age, gender, etiology,length of hospitalization, APACHE Ⅱ score at admission,local and organ/systemic complications of the patients.RESULTS: Of the 268 acute pancreatitis patients, 94 developed SAP. The mean age of SAP patients was 52 years, the commonest etiology was cholelithiasis (45.7%), the mean length of hospitalization was 70 d, the mean score of APACHE Ⅱ was 7.7. Fifty-four percent of the patients developed necrosis, 25% abscess, 58% organ/systemic failure. A total of 23.4% (22/94) of the SAP patients died. Respiratory failure was the most common organ clysfunction (90.9%) in deceased SAP patients, followed by cardiovascular failure (86.4%),renal failure (50.0%). In the SAP patients, 90.9% (20/22)developed multiple organ/systemic failures. There were significant differences in age, length of hospitalization,APACHE Ⅱ score and incidences of respiratory failure, renal failure, cardiovascular failure and hematological failure between deceased SAP patients and survived SAP patients.By multivariate logistic regression analysis, independent prognostic factors for mortality were respiratory failure,cardiovascular failure and renal failure.CONCLUSION: SAP patients are characterized by advanced age, high APACHE Ⅱ score, organ failure and their death is mainly due to multiple organ/systemic failures. In patients with SAP, respiratory, cardiovascular and renal failures can predict the fatal outcome and more attention should be paid to their clinical evaluation.

  18. Sepsis in AIDS patients: clinical, etiological and inflammatory characteristics

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    João Manoel Silva

    2013-01-01

    Full Text Available Introduction: Intensive care mortality of HIV-positive patients has progressively decreased. However, critically ill HIV-positive patients with sepsis present a worse prognosis. To better understand this condition, we propose a study comparing clinical, etiological and inflammatory data, and the hospital course of HIV-positive and HIV-negative patients with severe sepsis or septic shock. Methods: A prospective observational study enrolling patients with severe sepsis or septic shock associated or not with HIV infection, and admitted to intensive care unit (ICU. Clinical, microbiological and inflammatory parameters were assessed, including C-reactive protein (CRP, procalcitonin (PCT, interleukin-6, interleukin-10 and TNF-α. Outcome measures were in-hospital and six-month mortality. Results: The study included 58 patients with severe sepsis/septic shock admitted to ICU, 36 HIV-positive and 22 HIV-negative. All HIV-positive patients met the criteria for AIDS (CDC/2008. The main foci of infection in HIV-positive patients were pulmonary and abdominal (p=0.001. Fungi and mycobacteria were identified in 44.4% and 16.7% of HIV-positive patients, respectively. In contrast, the main etiologies for sepsis in HIV-negative patients were Gram-negative bacilli (36.4% and Gram-positive cocci (36.4% (p=0.001. CRP and PCT admission concentrations were lower in HIV-positive patients (130 vs. 168 mg/dL p=0.005, and 1.19 vs. 4.06 ng/mL p=0.04, respectively, with a progressive decrease in surviving patients. Initial IL-10 concentrations were higher in HIV-positive patients (4.4 pg/mL vs. 1.0 pg/mL, p=0.005, with moderate accuracy for predicting death (area under receiver-operating characteristic curve =0.74. In-hospital and six-month mortality were higher in HIV-positive patients (55.6 vs. 27.3% p=0.03, and 58.3 vs. 27.3% p=0.02, respectively. Conclusions: The course of sepsis was more severe in HIV-positive patients, with distinct clinical, etiological and

  19. Clinical and psychosocial characteristics of children with nonepileptic seizures

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    Chinta Sri

    2008-01-01

    Full Text Available Objective: The aim of this study is to present a comprehensive profile of clinical and psychosocial characteristics of children with psychogenic nonepileptic seizures and to assess the short-term outcome of these patients. Materials and Methods: The subjects were consecutive cases of children with a diagnosis of nonepileptic seizures (N=17, mean age = 10.7 years, S.D. = 1.26 and two groups of control groups matched on age and sex: true seizure group and healthy controls. All the children were recruited from the out-patient services of the Department of Pediatrics of a tertiary care teaching hospital in North India. Detailed history taking and clinical examination was done in the case of every child. A standard 18 channel EEG was done in all the children and a video EEG was done in 12 cases of children with nonepileptic seizures. The Childhood Psychopathology Measurement Schedule (CPMS and Life Events Scale for Indian Children (LESIC were used to measure the children′s emotional and behavioral functioning at home, and the number of life events and the stress associated with these events in the preceding year and the year before that. Short-term outcome was examined three to six months after the diagnosis of nonepileptic seizures was made. Results: Unresponsiveness without marked motor manifestations was the most common "ictal" characteristic of the nonepileptic seizures. Pelvic thrusting, upper and lower limb movements, head movements, and vocalization were observed in less than one-third of the patients. Increased psychosocial stress and significantly higher number of life events in the preceding year were found to characterize children with nonepileptic seizures, as compared to the two control groups. The nonepileptic seizures and true seizures groups had a higher proportion of children with psychopathology scores in the clinically significant maladjustment range, as compared to those in the healthy control group. A majority of the patients

  20. Sacrococcygeal chordoma: Magnetic resonance imaging and computed tomography

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

    1985-07-01

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

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

    Science.gov (United States)

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

    2016-09-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2011-11-15

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

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

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    Weber Walter

    2005-05-01

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

  4. Carbon Ion Radiotherapy for Skull Base Chordoma

    OpenAIRE

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

    2009-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1998-02-01

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

  6. Holoprosencephaly: Epidemiologic and clinical characteristics of a California population

    Energy Technology Data Exchange (ETDEWEB)

    Croen, L.A.; Shaw, G.M. [California Birth Defects Monitoring Program, Emeryville, CA (United States); Lammer, E.J. [Children`s Hospital, Oakland, CA (United States)

    1996-08-23

    Holoprosencephaly is a brain defect resulting from incomplete cleavage of the embryonic forebrain. It involves forebrain and facial malformations that can range from mild to severe. The epidemiology of holoprosencephaly is largely unknown. Published prevalence estimates have been derived from clinic-based case series, and suggested risk factors for holoprosencephaly have been identified in case reports, without confirmation from systematically conducted population-based studies. Using data from a population-based birth defects registry in California, we described the epidemiologic and clinical characteristics of cytogenetically and phenotypically distinct types of holoprosencephaly. A total of 121 cases was identified among a cohort of 1,035,386 live births and fetal deaths. The prevalence of holoprosencephaly was 1.2 per 10,000 births (95% confidence interval 1.0-1.4 per 10,000). Of all cases, 41% (50/121) had a chromosomal abnormality, most commonly Trisomy 13. Among the 71 cytogenetically apparently normal cases, 18 had recognizable syndromes and the remaining 53 were of unknown cause. Among the cytogenetically abnormal cases, females had a greater risk than males (odds ratio = 2.3, 95% confidence interval [1.2, 4.4]). Among the cytogenetically normal cases, increased risks were observed among Hispanic whites (OR = 1.8 [0.9, 3.6]) and cases whose mother was born in Mexico (OR = 2.2 [1.0, 4.5]). Approximately 46% of all cases had alobar holoprosencephaly, the most severe form of the forebrain malformation. The facial phenotype did not strongly predict the severity of the brain defect; however, severity was inversely correlated with length of survival. This study is the first to present findings based on such a large population-based series of infants/fetuses affected by holoprosencephaly, and demonstrates the importance of investigating the component subgroups of this rare phenotype. 47 refs., 7 tabs.

  7. Clinical characteristics of intermediate uveitis in adult Turkish patients

    Institute of Scientific and Technical Information of China (English)

    Esra; Kardes; Betul; Ilkay; Sezgin; Akcay; Kansu; Bozkurt; Cihan; Unlu; Gurkan; Erdogan; Gulunay; Akcali

    2015-01-01

    ·AIM: To describe the clinical characteristics of Turkish patients with intermediate uveitis(IU) and to investigate the effect of clinical findings and complications on final visual acuity(VA).·METHODS: We retrospectively analyzed the medical records of patients with IU who had at least 6mo of follow-up and were older than 16 y.· RESULTS: A total of 78 eyes of 45 patients were included in the study and the mean follow-up period was19.4mo. The mean age at the time of presentation was42.9s. Systemic disease associations were found in17.7% of cases; sarcoidosis(8.8%) and multiple sclerosis(6.6%) were the most common diseases. Recurrence rate(odds ratio=45.53; 95%CI: 2.181-950.58), vitritis equals to or more than 3+ cells(odds ratio =57.456; 95%CI: 4.154-794.79) and presenting with VA less than 20/40(odds ratio =43.81; 95% CI: 2.184-878.71) were also found as high risk factors for poor final VA. At the last follow-up examination, 67.9% of eyes had VA of 20/40 or better.·CONCLUSION: IU is frequently seen at the beginning of the fourth decade of life. The disease is most commonly idiopathic in adult Turkish patients. Patients with severe vitritis at presentation and patients with frequent recurrences are at high risk for poor visual outcome.

  8. Clinical characteristics of sarcoidosis patients diagnosed in a university hospital

    Directory of Open Access Journals (Sweden)

    Özlem Abakay

    2012-09-01

    Full Text Available Objectives: The clinical characteristics and treatment resultsof patients diagnosed with sarcoidosis was investigatedin Dicle University Medical Faculty Hospital.Materials and methods: A total of 39 patients were includeddiagnosed with sarcoidosis between 01 January2008 and31 December 2011.Demographic data, laboratoryfindings, spirometric test data, diagnostic methodsand treatment regimens for the study were recorded form.According to pulmonary function test results of patients,61.5% were normal pattern, 30.8% were restrictive patternand 7.7% were obstructive pattern.Results: Of the 39 patients 15.4% male, 84.6% werefemale. The mean age was 39.5±13.1 years for males,females 44.8±14.0 years. All patients of was 17.9%stage 1, 66.7% stage 2, 10.3% stage 3 and 5.1% stage4. 61.5% were symptomatic, 38.5% were asymptomatic.Methods of diagnosis of the patients examined, 25.6% ofpatients bronchoscopic biopsy procedures, 74.4% of patientssurgical biopsy procedures. Of the 56.4% patientshad received corticosteroid treatment, 5.1% patients hadreceived corticosteroid + methotrexate treatment, 38.5%were followed up without the pharmacological treatment.Pharmacological treatment in the group complete at thetime of the study 20.5% concluded the treatment of theperson. Patients who treatment ending 8.4 months hadused pharmacological treatment.Conclusions: Interstitial lung diseases which are prevalentamong patients with sarcoidosis diagnosed by examiningthe clinical features, differential diagnosis and treatmentmay be possible detection of potential problems. JClin Exp Invest 2012; 3 (3: 363-367Key words: Sarcoidosis diagnosis, treatment

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

    Institute of Scientific and Technical Information of China (English)

    庞超楠; 刘晓光; 袁慧书

    2015-01-01

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

  10. Acute Viral Hepatitis A – Clinical, Laboratory and Epidemiological Characteristics

    Directory of Open Access Journals (Sweden)

    Melinda HORVAT

    2013-06-01

    Full Text Available Background and Aims: Infection with hepatitis A virus is still one of the most common causes of hepatitis worldwide. The clinical manifestation of acute hepatitis A (AHA in adults can vary greatly, ranging from asymptomatic infection to severe and fulminant hepatitis. The aim of this study was to describe the demographic, clinical characteristics, laboratory features and hospital outcome of adult patients with AHA over a consecutive period of 4 years within an area from Eastern European country. Methods: Two hundred and two adult patients diagnosed with AHA were retrospective, observational and analytic analized over a period of 4 years. Based on prothrombin time less than 50, the study group was stratified in medium (79.2% and severe forms (20.8%. We investigated the clinical, laboratory and epidemiological features. Statistical analysis were applied to compare the medium and severe forms of AHA. Results: Most patients (72.7% were younger than 40 years. The main symptoms included: dyspepsia (72.07%, jaundice (86.63%, asteno-adynamia (86.72%, and flu-like symptoms (53.46%. The hemorrhagic cutaneous-mucous manifestations (6.93% associated with the severe forms of AHA (OR =12.19, 95%CI -3.59 - 41.3, p =0.001. We found statistically significant differences for PT (p <0.001, INR (p <0.001, TQ (p <0.001, ALAT (p <0.001, ASAT (p <0.001, ALP (p <0.001 and platelets (p =0.009 between severe and medium AHA forms. We found that TQ, INR, ALAT and ASAT have the highest diagnostic values, statistically significant (p <0.05 for severe AHA forms with AUC (0.99, 0.99, 0.72, 0.70 at values of sensitivity (95%, 90.5%, 89%, 95% and specificity (98%, 99%, 88%,94%. Conclusions Medium severity AHA forms were found in most of the study group patients (79.2%. The severe AHA forms were associated with hemorrhagic cutaneous-mucous manifestations (OR =12.19, p =0.001. The univariate analysis proved a negatively statistically significant correlation between IP and ALAT

  11. Clinical Characteristics of Adults with Asperger's Syndrome Assessed with Self-Report Questionnaires

    Science.gov (United States)

    Kanai, Chieko; Iwanami, Akira; Ota, Haruhisa; Yamasue, Hidenori; Matsushima, Eisuke; Yokoi, Hideki; Shinohara, Kazuyuki; Kato, Nobumasa

    2011-01-01

    Diagnosis of Asperger's Syndrome (AS) in adults is difficult, and clinical sample-based studies that systematically illustrate the clinical characteristics of adult AS patients are needed so that appropriate treatment can be provided. Here we examined the clinical characteristics of AS in 112 adults (median age, 28.0 years [range, 18-52]; 71 men…

  12. [Active acromegaly and gigantism: some clinical characteristics of 50 patients].

    Science.gov (United States)

    Pumarino, H; Oviedo, S; Michelsen, H; Campino, C

    1991-08-01

    50 patients with autonomous growth hormone excess (48 with adult acromegaly and 2 with gigantism) were studied between 1966 to 1986 (2.38 pts/year). Characteristic clinical presentation, an increase in growth hormone (GH) uninhibited by glucose, and/or hyperphosphemia and hyperhydroxiprolinuria were present in all patients. No cases of hypercalcemia were recorded. Phosphemia was increased in 55.8%, alkaline phosphatases in 61.7%, calciuria in 26.9% and hydroxyprolinuria in 74.2% of the patients. Basal GH was over 5 ng/ml (89.9 DS +/- 170.9) in 42 pts, and in 37 was not suppressed after glucose administration, 38% had an increased (paradoxical response) and 62% a flat response (less than 50% change of basal values). TRH test was performed in 14 patients, 8 presented an increase in GH titer. Hyperprolactinemia was seen in 4 of 12 patients in whom this hormone was measured. The size of the sella turcica was increased in 93%, and although the larger sellar size correlated to higher levels of GH, correlation was not significant. 20% of the pts had rheumatological disease, 14% goiter, 12% cardiac disease, 26.5% had diastolic hypertension and 4% renal lithiasis (hypercalciuric pts). 38% had hyperglycemia with a diabetic glucose tolerance test and 18% had non-diabetic abnormal glucose tolerance test. PMID:1844771

  13. Clinical, demographic, and laboratory characteristics of children with nephrolithiasis.

    Science.gov (United States)

    Sas, David J; Becton, Lauren J; Tutman, Jeffrey; Lindsay, Laura A; Wahlquist, Amy H

    2016-06-01

    While the incidence of pediatric kidney stones appears to be increasing, little is known about the demographic, clinical, laboratory, imaging, and management variables in this patient population. We sought to describe various characteristics of our stone-forming pediatric population. To that end, we retrospectively reviewed the charts of pediatric patients with nephrolithiasis confirmed by imaging. Data were collected on multiple variables from each patient and analyzed for trends. For body mass index (BMI) controls, data from the general pediatrics population similar to our nephrolithiasis population were used. Data on 155 pediatric nephrolithiasis patients were analyzed. Of the 54 calculi available for analysis, 98 % were calcium based. Low urine volume, elevated supersaturation of calcium phosphate, elevated supersaturation of calcium oxalate, and hypercalciuria were the most commonly identified abnormalities on analysis of 24-h urine collections. Our stone-forming population did not have a higher BMI than our general pediatrics population, making it unlikely that obesity is a risk factor for nephrolithiasis in children. More girls presented with their first stone during adolescence, suggesting a role for reproductive hormones contributing to stone risk, while boys tended to present more commonly at a younger age, though this did not reach statistical significance. These intriguing findings warrant further investigation. PMID:26467033

  14. Epidemiological, clinical, and immunological characteristics of neuromyelitis optica: A review.

    Science.gov (United States)

    Pereira, Wildéa Lice de Carvalho Jennings; Reiche, Edna Maria Vissoci; Kallaur, Ana Paula; Kaimen-Maciel, Damacio Ramón

    2015-08-15

    The aim of this study was to review the epidemiological and clinical characteristics of neuromyelitis optica (NMO) and the immunopathological mechanisms involved in the neuronal damage. NMO is an inflammatory demyelinating autoimmune disease of the central nervous system that most commonly affects the optic nerves and spinal cord. NMO is thought to be more prevalent among non-Caucasians and where multiple sclerosis (MS) prevalence is low. NMO follows a relapsing course in more than 80-90% of cases, which is more commonly in women. It is a complex disease with an interaction between host genetic and environmental factors and the main immunological feature is the presence of anti-aquaporin 4 (AQP4) antibodies in a subset of patients. NMO is frequently associated with multiple other autoantibodies and there is a strong association between NMO with other systemic autoimmune diseases. AQP4-IgG can cause antibody-dependent cellular cytotoxicity (ADCC) when effector cells are present and complement-dependent cytotoxicity (CDC) when complement is present. Acute therapies, including corticosteroids and plasma exchange, are designed to minimize injury and accelerate recovery. Several aspects of NMO pathogenesis remain unclear. More advances in the understanding of NMO disease mechanisms are needed in order to identify more specific biomarkers to NMO diagnosis.

  15. Clinical Characteristics and Genetic Variability of Human Rhinovirus in Mexico

    Directory of Open Access Journals (Sweden)

    Hilda Montero

    2012-01-01

    Full Text Available Human rhinovirus (HRV is a leading cause of acute respiratory infection (ARI in young children and infants worldwide and has a high impact on morbidity and mortality in this population. Initially, HRV was classified into two species: HRV-A and HRV-B. Recently, a species called HRV-C and possibly another species, HRV-D, were identified. In Mexico, there is little information about the role of HRV as a cause of ARI, and the presence and importance of species such as HRV-C are not known. The aim of this study was to determine the clinical characteristics and genetic variability of HRV in Mexican children. Genetic characterization was carried out by phylogenetic analysis of the 5′-nontranslated region (5′-NTR of the HRV genome. The results show that the newly identified HRV-C is circulating in Mexican children more frequently than HRV-B but not as frequently as HRV-A, which was the most frequent species. Most of the cases of the three species of HRV were in children under 2 years of age, and all species were associated with very mild and moderate ARI.

  16. Clinical characteristics and risk factors of ocular candidiasis.

    Science.gov (United States)

    Nagao, Miki; Saito, Takashi; Doi, Shoichi; Hotta, Gou; Yamamoto, Masaki; Matsumura, Yasufumi; Matsushima, Aki; Ito, Yutaka; Takakura, Shunji; Ichiyama, Satoshi

    2012-06-01

    Ocular candidiasis is a major complication of Candida bloodstream infection (BSI). This study was performed to reveal the clinical characteristics of ocular candidiasis. Of the 220 patients with Candida BSI, 204 cases received ophthalmology consultations between January 2005 and December 2011 at 2 teaching hospitals. Fifty-four (26.5%) cases had findings consistent with the diagnosis of ocular candidiasis. Of these 54 cases, 43 (79.6%) were diagnosed within 7 days after a positive blood culture. Among ocular candidiasis cases, more cases were due to Candida albicans (P =0.034 odds ratio [OR]; 3.68 95% confidence interval [CI] 1.11-12.2) and had higher β-d-glucan values (P = 0.001 OR; 9.99 95% CI 2.60-21.3). We need to consider fundoscopic examination to be performed within the first 7 days of therapy, especially for those patients who have C. albicans BSIs and higher β-d-glucan values. Additionally, follow-up fundoscopic examination should be considered before stopping therapy for high-risk patients.

  17. Spinal epidural angiolipomas: Clinical characteristics, management and outcomes

    Science.gov (United States)

    Bouali, Sofiene; Maatar, Nidhal; Bouhoula, Asma; Abderrahmen, Khansa; Said, Imed Ben; Boubaker, Adnen; Kallel, Jalel; Jemel, Hafedh

    2016-01-01

    Purpose: The spinal epidural angiolipomas are rare expansive processes made of mature lipomatous and angiomatous elements. They often have a benign character. Their etiology, pathogenesis remains uncertain, and it is a cause of spinal cord compression. The magnetic resonance imaging is the most important neuroradiological examination. Histological examination is the only examination to confirm the diagnosis. Surgery is the treatment of choice. Methods: A retrospective study of all patients operated on for a spinal epidural angiolipoma at the Department of Neurosurgery at the National Institute of Neurology of Tunis between January 2000 and December 2014 (15 years) was performed. The aim of this study is to describe the clinical, radiological, histological characteristics and the treatment of this tumor. Results: A total of nine patients were operated from January 01, 2000 to November 30, 2014. The average age of our patients was 51 years with ages that ranged from 29 to 65 with a male predominance. The period between onset of symptoms and diagnosis ranged from 24 months with an average 12 months. Posterior localization of the tumor was seen in all patients. Surgical resection was performed for all cases. The postoperative course has been satisfactory, with a complete recovery of neurological functions in all patients. Conclusions: The spinal epidural angiolipomas is rare expansive process causing spinal cord compression. Treatment is exclusively surgical resection. The functional outcome of spinal epidural angiolipomas is particularly favorable with a complete neurological recovery is if the patient was quickly operated.

  18. Spinal epidural angiolipomas: Clinical characteristics, management and outcomes

    Science.gov (United States)

    Bouali, Sofiene; Maatar, Nidhal; Bouhoula, Asma; Abderrahmen, Khansa; Said, Imed Ben; Boubaker, Adnen; Kallel, Jalel; Jemel, Hafedh

    2016-01-01

    Purpose: The spinal epidural angiolipomas are rare expansive processes made of mature lipomatous and angiomatous elements. They often have a benign character. Their etiology, pathogenesis remains uncertain, and it is a cause of spinal cord compression. The magnetic resonance imaging is the most important neuroradiological examination. Histological examination is the only examination to confirm the diagnosis. Surgery is the treatment of choice. Methods: A retrospective study of all patients operated on for a spinal epidural angiolipoma at the Department of Neurosurgery at the National Institute of Neurology of Tunis between January 2000 and December 2014 (15 years) was performed. The aim of this study is to describe the clinical, radiological, histological characteristics and the treatment of this tumor. Results: A total of nine patients were operated from January 01, 2000 to November 30, 2014. The average age of our patients was 51 years with ages that ranged from 29 to 65 with a male predominance. The period between onset of symptoms and diagnosis ranged from 24 months with an average 12 months. Posterior localization of the tumor was seen in all patients. Surgical resection was performed for all cases. The postoperative course has been satisfactory, with a complete recovery of neurological functions in all patients. Conclusions: The spinal epidural angiolipomas is rare expansive process causing spinal cord compression. Treatment is exclusively surgical resection. The functional outcome of spinal epidural angiolipomas is particularly favorable with a complete neurological recovery is if the patient was quickly operated. PMID:27695535

  19. Dosimetric characteristics of a MOSFET dosimeter for clinical electron beams.

    Science.gov (United States)

    Manigandan, D; Bharanidharan, G; Aruna, P; Devan, K; Elangovan, D; Patil, Vikram; Tamilarasan, R; Vasanthan, S; Ganesan, S

    2009-09-01

    The fundamental dosimetric characteristics of commercially available metal oxide semiconductor field effect transistor (MOSFET) detectors were studied for clinical electron beam irradiations. MOSFET showed excellent linearity against doses measured using an ion chamber in the dose range of 20-630cGy. MOSFET reproducibility is better at high doses compared to low doses. The output factors measured with the MOSFET were within +/-3% when compared with those measured with a parallel plate chamber. From 4 to 12MeV, MOSFETs showed a large angular dependence in the tilt directions and less in the axial directions. MOSFETs do not show any dose-rate dependence between 100 and 600MU/min. However, MOSFETs have shown under-response when the dose per pulse of the beam is decreased. No measurable effect in MOSFET response was observed in the temperature range of 23-40 degrees C. The energy dependence of a MOSFET dosimeter was within +/-3.0% for 6-18MeV electron beams and 5.5% for 4MeV ones. This study shows that MOSFET detectors are suitable for dosimetry of electron beams in the energy range of 4-18MeV. PMID:19128995

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Aline Houessinon

    2015-01-01

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

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

    Science.gov (United States)

    Ieni, Antonio; Branca, Giovanni

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Valeria Barresi

    2014-01-01

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

  4. Clinical and Epidemiologic Characteristics of Patients with Childhood Psoriazis Seen in Dermatology Clinic

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    Emine Elvan Taşğın

    2013-06-01

    Full Text Available Objective: To evaluate the epidemiologic characteristics of the pediatric patients with psoriazis applied to our outpatients' clinic and compare these data with the literature. Methods: The study population consisted of 37 patients younger than 16 years with the diagnosis of psoriazis in dermatology outpatient clinic between May 2009 and December 2010. The patients were evaluated with regard to age, gender, age of onset of the disease, duration of the disease, the presence of accompanying diseases, family history, clinical features, and triggering factors. Results: Of 37 pediatric patients with psoriazis evaluated in this study, 21 were girls and 16 were boys. The ages of the patients ranged between 2 and 15 years. 32.4% of the patients had family history. Plaque (59.45% of the patients, guttate (29.7% of the patients and pustular (10.8% of the patientstypes of psoriazis were determined. The psoriazis lesions involved most frequently the hairy skin (73% and the disease generally originated from this region. Ungual involvement was determined in 27% of the patients. None of the patients had psoriatic arthritis. Conclusion: Our study might contribute to generate epidemiological data's about the pediatric patients with psoriazis in our population.

  5. Clinical characteristics and prognostic factors of primary gastric lymphoma

    Science.gov (United States)

    Wang, Yi-Gao; Zhao, Lin-Yong; Liu, Chuan-Qi; Pan, Si-Cheng; Chen, Xiao-Long; Liu, Kai; Zhang, Wei-Han; Yang, Kun; Chen, Xin-Zu; Zhang, Bo; Chen, Zhi-Xin; Chen, Jia-Ping; Zhou, Zong-Guang; Hu, Jian-Kun

    2016-01-01

    Abstract Primary gastric lymphoma (PGL) is the most common extranodal non-Hodgkin lymphoma. This retrospective study aimed to analyze the clinical characteristics, prognostic factors, and roles of different treatment modalities in patients with PGL. From January 2003 to November 2014, 165 patients who were diagnosed with PGL at West China Hospital were enrolled in this study. The clinical features, treatment, and follow-up information were analyzed. In this study, diffuse large B-cell lymphoma (DLBCL) (108, 65.5%) and mucosa-associated lymphoid tissue (MALT) lymphoma (52, 31.5%) were two predominant histological subtypes. One-year and 5-year overall survival (OS) rates of all patients were 95.2% and 79.5%, respectively; in whom 110 (66.7%) underwent surgery, 110 (66.7%) received chemotherapy, 12 (7.3%) received radiotherapy, and 10 (6.1%) received Helicobacter pylori eradication. And 75 patients (45.5%) were treated with at least 2 different types of therapies. Elevated lactic dehydrogenase (LDH) levels, poor performance status (PS), advanced stage, International Prognostic Index (IPI) score ≥3, conservative treatment, and high-grade histological subtype were associated with worse prognosis in univariate analysis. Cox regression analysis showed that LDH levels, PS, staging, and histological subtype were independent predictors of survival outcomes. In the DLBCL type, 5-year OS was significantly better in the surgically treated group (80.1%) than that of patients conservatively treated (49.8%) (P = 0.001). Surgical treatment had almost no impact on OS in the MALT type than conservative treatment (P = 0.597). The proportion of patients received conservative treatment increased from 4.5% in period 1 to 51.7% in period 4. High LDH levels, poor PS, advanced staging, and malignant pathological type at diagnosis are significantly associated with poor OS. Our data suggest that surgery is superior in prognosis over conservative treatment in the DLBCL type, but not

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

    Directory of Open Access Journals (Sweden)

    Tamara eAleksic

    2016-05-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2013-01-01

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

  9. Lobular breast cancer: Clinical, molecular and morphological characteristics.

    Science.gov (United States)

    Christgen, Matthias; Steinemann, Doris; Kühnle, Elna; Länger, Florian; Gluz, Oleg; Harbeck, Nadia; Kreipe, Hans

    2016-07-01

    Infiltrating lobular breast cancer (ILBC) is the most common special breast cancer subtype. This review provides a comprehensive description of ILBC characteristics, including epidemiology, clinical features, molecular genetics and histomorphology. Twenty detailed supplemental data tables guide through primary data of more than 200 original studies. Meta-analyses indicate that ILBC is at least twice as common in the Western world as it is in other geographic regions. ILBC is over-represented in so-called interval carcinomas and in primary metastatic breast cancer. ILBC is also associated higher age, higher pT stage and hormone receptor (ER/PR) positivity. Pathological complete response rates after neoadjuvant chemotherapy are low, ranging between 0% and 11%. Positive resection margins after breast-conserving surgery are comparatively frequent and 17% to 65% of patients undergo a second surgical intervention. Depending on the morphological stringency in the diagnosis of ILBC, lack of E-cadherin expression is observed in 55% to 100% of cases. CDH1/E-cadherin mutation detection rates vary between 12% and 83%. Various additional molecular factors, including PIK3CA, TP53, FOXA1, FGFR1, ZNF703 and BCAR4, have been implicated in ILBC or progression of lobular carcinoma in situ (LCIS) to invasive cancer and are discussed in detail. Eight instructive figure plates recapitulate the histomorphology of ILBC and its variants. Furthermore, we draw attention to rarely addressed histological details, such as two-sided nuclear compression and fat-avoiding growth at the invasion front. Last but not least, we discuss future translational research directions and emphasize the concept of synthetic lethality, which promises new options for targeted ILBC therapy.

  10. Severe acute pancreatitis in the elderly: Etiology and clinical characteristics

    Institute of Scientific and Technical Information of China (English)

    Ming-Jun Xin; Hong Chen; Bin Luo; Jia-Bang Sun

    2008-01-01

    AIM: To investigate the etiology and clinical characteristics of severe acute pancreatitis (SAP) in elderly patients (≥60 years of age).METHODS: We reviewed retrospectively all the SAP cases treated in Xuanwu Hospital in Beijing between 2000 and 2007.RESULTS: In 169 patients with SAP, 94 were elderly and 16 died.Biliary and idiopathic etiologies were the first two causes that accounted for over 90% of SAP in the elderly.Biliary, hyperlipemic and alcoholic etiologies were the first three causes in the young.The proportion of comorbidity of cholelithiasis, biliary infection, hypertension and coronary heart disease in the aged was significantly higher than that in their young partners.The scores of APACHE Ⅱ and Ranson were also significantly higher in the elderly except the CT score.Organ failures were more common in the elderly, but the local pancreatic complications were not different between the two groups.Mortality of the aged was correlated with the severity of SAP, multiple co-morbidity and incidence of multiple organ dysfunction syndrome (NODS).NODS was the main cause of death.CONCLUSION: The etiology of SAP in the elderly is quite different from that in the young.Biliary and unknown factors are main causes in the aged.The elderly are subject to major organ failures but there is no difference in the occurrence of local pancreatic complications between the elderly and the young.It is crucial to monitor and improve the functions of major organs so as to prevent MODS in the aged with SAP.

  11. Children and adolescents referred for treatment of anxiety disorders: differences in clinical characteristics

    OpenAIRE

    Waite, Polly; Creswell, Catharine

    2014-01-01

    Background Reports of the clinical characteristics of children and adolescents with anxiety disorders are typically based on community populations or from clinical samples with exclusion criterion applied. Little is known about the clinical characteristics of children and adolescents routinely referred for treatment for anxiety disorders. Furthermore, children and adolescents are typically treated as one homogeneous group although they may differ in ways that are clinically meaningful. Method...

  12. 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.

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

    Institute of Scientific and Technical Information of China (English)

    徐凯; 魏永鸽; 崔正军; 黄器伟

    2015-01-01

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

  14. Genital ulcers in women: clinical, microbiologic and histopathologic characteristics.

    Science.gov (United States)

    Gomes, Christiane Maria Moreira; Giraldo, Paulo César; Gomes, Francis de Assis Moraes; Amaral, Rose; Passos, Mauro Romero Leal; Gonçalves, Ana Katherine da Silveira

    2007-04-01

    Female genital ulcer is a disease that affects a large number of women, and its etiologic diagnosis can be difficult. The disease may increase the risk of acquiring HIV. Genital ulcer may be present in sexually transmitted diseases (STD)--syphilis, chancroid, genital herpes, donovanosis, lymphogranuloma venereum; and other non-STD disorders (NSTD)--Behçet's syndrome, pemphigus, Crohn's disease, erosive lichen planus and others. This study evaluated the clinical-histopathologic-microbiologic characteristics of female genital ulcers. A cross-sectional descriptive prospective study was conducted during a six-month period to investigate the first 53 women without a definitive diagnosis, seeking medical care for genital ulcers at a genital infections outpatient facility in a university hospital. A detailed and specific history was taken, followed by a dermatologic and gynecologic examination. In addition to collecting material from the lesions for microbiologic study, a biopsy of the ulcer was performed for histopathologic investigation. The average age of the patients was 32.7 years, 56.6% had junior high school education and higher education. The most frequent etiology was herpetic lesion, followed by auto-immune ulcers. At the time of their first consultation, around 60% of the women were using inadequate medication that was inconsistent with the final diagnosis. Histologic diagnosis was conclusive in only 26.4% of the patients (14/53). Cure was obtained in 99% of the cases after proper therapy. The female genital ulcers studied were equally distributed between sexually transmitted and non-sexually transmitted causes. Herpes was the most frequent type of genital ulcer, affecting women indiscriminately, mostly between the ages of 20 and 40 years. The etiologic diagnosis of herpetic ulcers is difficult to make even when various diagnostic methods are applied. It is imperative that NSTD should be included in the differential diagnoses of female genital ulcers. The

  15. Genital ulcers in women: clinical, microbiologic and histopathologic characteristics

    Directory of Open Access Journals (Sweden)

    Christiane Maria Moreira Gomes

    2007-04-01

    Full Text Available Female genital ulcer is a disease that affects a large number of women, and its etiologic diagnosis can be difficult. The disease may increase the risk of acquiring HIV. Genital ulcer may be present in sexually transmitted diseases (STD - syphilis, chancroid, genital herpes, donovanosis, lymphogranuloma venereum and other non-STD disorders (NSTD - Behçet's syndrome, pemphigus, Crohn's disease, erosive lichen planus and others. This study evaluated the clinical-histopathologic-microbiologic characteristics of female genital ulcers. A cross-sectional descriptive prospective study was conducted during a six-month period to investigate the first 53 women without a definitive diagnosis, seeking medical care for genital ulcers at a genital infections outpatient facility in a university hospital. A detailed and specific history was taken, followed by a dermatologic and gynecologic examination. In addition to collecting material from the lesions for microbiologic study, a biopsy of the ulcer was performed for histopathologic investigation. The average age of the patients was 32.7 years, 56.6% had junior high school education and higher education. The most frequent etiology was herpetic lesion, followed by auto-immune ulcers. At the time of their first consultation, around 60% of the women were using inadequate medication that was inconsistent with the final diagnosis. Histologic diagnosis was conclusive in only 26.4% of the patients (14/53. Cure was obtained in 99% of the cases after proper therapy. The female genital ulcers studied were equally distributed between sexually transmitted and non-sexually transmitted causes. Herpes was the most frequent type of genital ulcer, affecting women indiscriminately, mostly between the ages of 20 and 40 years. The etiologic diagnosis of herpetic ulcers is difficult to make even when various diagnostic methods are applied. It is imperative that NSTD should be included in the differential diagnoses of female

  16. Sacrococcygeal chordoma. A clinicoradiological study of 60 patients

    Energy Technology Data Exchange (ETDEWEB)

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

    1987-01-01

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

  17. Prognostic factors in chordoma: role of postoperative radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

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

    1995-12-31

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

  18. Clinical characteristics,prognosis and indicators of glucose metabolism of liver cirrhosis complicated with diabetes

    Institute of Scientific and Technical Information of China (English)

    吴娟

    2013-01-01

    Objective To investigate clinical characteristics and outcome of patients with liver cirrhosis complicated with diabetes,and to explore the differences of clinical characteristics and prognosis between hepatogenous diabetes (HD) and type 2 diabetes mellitus (T2DM) .Methods From November 2010 to April 2012,246 patients with liver cirrhosis were collected and divided into liver cir-

  19. Comorbid Depressive Disorders in Anxiety-Disordered Youth: Demographic, Clinical, and Family Characteristics

    Science.gov (United States)

    O'Neil, Kelly A.; Podell, Jennifer L.; Benjamin, Courtney L.; Kendall, Philip C.

    2010-01-01

    Research indicates that depression and anxiety are highly comorbid in youth. Little is known, however, about the clinical and family characteristics of youth with principal anxiety disorders and comorbid depressive diagnoses. The present study examined the demographic, clinical, and family characteristics of 200 anxiety-disordered children and…

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    James Barrese

    2014-12-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1989-01-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2013-12-01

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

  5. Periocular and orbital amyloidosis - Clinical characteristics, management, and outcome

    NARCIS (Netherlands)

    I. Leibovitch; D. Selva; R.A. Goldberg; T.J. Sullivan; P. Saeed; G. Davis; J.D. McCann; A. Mcnab; J. Rootman

    2006-01-01

    Objective: To present the clinical features and management outcome in a large series of patients with periocular and orbital amyloidosis. Design: Retrospective, noncomparative, interventional case series. Patients: All patients diagnosed with periocular and orbital amyloidosis in 6 oculoplastic and

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

    Directory of Open Access Journals (Sweden)

    Kumar Pavan

    2009-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1993-12-31

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

  8. Analysis of clinical characteristics of 950 cases of cervical cancer

    Directory of Open Access Journals (Sweden)

    Shu-li ZHU

    2015-04-01

    Full Text Available Objective To discuss the clinical features of the patients suffering from cervical cancer who visited Daping Hospital affiliated to Third Military Medical University in recent 10 years. Methods The clinical data of the patients who were pathologically diagnosed as invasive cervical cancer in Daping Hospital of TMMU from Jan. 2004 to Dec. 2013 were retrospectively analyzed. They were divided into different age groups and analyzed according to age, clinical features, pathological type, and surgical approach. Results Clinical data of 950 patients with invasive cervical cancer were reviewed in this study. The mean age of the patients was 46.9 years. The clinical features, pathological type, and surgical approaches were different in different age groups. Analysis of the age structure of the patients, the onset age of cervical cancer seemed to increase year by year. Conclusion The clinical features of cervical cancer are diversity in different age, and the strategy for controlling its development should be varied according to age. DOI: 10.11855/j.issn.0577-7402.2015.03.09

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Institute of Scientific and Technical Information of China (English)

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Alireza Khoshnevisan

    2012-02-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

  13. Genomic and clinical characteristics of microduplications in chromosome 17.

    Science.gov (United States)

    Shchelochkov, Oleg A; Cheung, S W; Lupski, J R

    2010-05-01

    Genomic disorders have been increasingly recognized as a significant source of clinically relevant phenotypes largely fostered by advances in technologies for genome-wide analyses. Molecular and clinical studies of copy number variants involving chromosome 17 began with locus-specific studies of Charcot-Marie-Tooth disease type 1A (CMT1A, OMIM #118220) and hereditary neuropathy with liability to pressure palsies (HNPP, OMIM #162500), which laid the foundation for the paradigm of duplication/deletion and gene-dosage for our understanding of genomic disorders. With the clinical introduction of high-resolution array comparative genomic hybridization (aCGH) the number of recognized genomic disorders including microduplications has been increasing rapidly. A relatively high proportion of disease-associated copy number variants map to chromosome 17. This may result from its unique structural features, such as relative abundance of segmental duplications and interspersed repetitive elements, high gene content, and the presence of dosage-sensitive genes. These genomic rearrangements are mediated by diverse mechanisms including Non-Allelic Homologous Recombination (NAHR), Non-Homologous End-Joining (NHEJ), and Fork Stalling and Template Switching (FoSTeS). We provide specific examples of chromosome 17 microduplications with the emphasis on their phenotype, specific clinical features aiding in their diagnosis, and counseling. PMID:20425816

  14. Comparing School and Clinical Psychology Internship Applicant Characteristics

    Science.gov (United States)

    Mahoney, Emery B.; Perfect, Michelle M.; Edwinson, Roxanne M.

    2015-01-01

    The ratio of internship applicants to internship positions listed in the online directory of the Association of Psychology Postdoctoral and Internship Centers (APPIC) is estimated at 1.23:1. In 2014a, approximately 14% of all students who participated in the match were not placed. Although the internship crisis impacts students in clinical,…

  15. Mesenchymal stem cells: biological characteristics and potential clinical applications

    DEFF Research Database (Denmark)

    Kassem, Moustapha

    2004-01-01

    are among the first stem cell types to be introduced in the clinic. Several studies have demonstrated the possible use of MSC in systemic transplantation for systemic diseases, local implantation for local tissue defects, as a vehicle for genes in gene therapy protocols or to generate transplantable tissues...

  16. Characteristics of patients with ADHD in psychiatric and pediatric ADHD clinics

    OpenAIRE

    Kolar, Dusan; Hechtman, Lily; Francoeur, Emmett; Paterson, Jodi

    2013-01-01

    Abstract. This study explores whether the child psychiatry ADHD clinic and the pediatric ADHD clinic serve different population of patients regarding the patient characteristics, severity of ADHD symptoms and comorbid disorders, thereby testing the efficacy of a triage system set up to direct patients referred for Attention Deficit Disorder symptoms to the appropriate clinics. Charts of 163 children and adolescents treated in two clinical settings were analyzed with regard to demographic char...

  17. Clinical characteristics of polycystic ovary syndrome in Indian women

    OpenAIRE

    Sunita J. Ramanand; Balasaheb B Ghongane; Jaiprakash B Ramanand; Milind H Patwardhan; Ghanghas, Ravi R.; Jain, Suyog S

    2013-01-01

    Background: Polycystic ovary syndrome (PCOS) is common diagnosis in women presenting with infertility. All the dimensions of PCOS have not been completely explored. Many studies have tried to characterize the exact presentation of the disease. In this study we studied clinical features of PCOS in Indian women to characterize different phenotypes of this syndrome. Prevalence of acanthosis nigricans (AN) as surrogate marker of insulin resistance, obesity, hirsutism and hypothyroidism in PCOS wo...

  18. Characteristic clinical and pathologic features for preoperative diagnosed groove pancreatitis

    OpenAIRE

    Kim, Joo Dong; Han, Young Seok; Choi, Dong Lak

    2011-01-01

    Purpose Groove pancreatitis is a rare specific form of chronic pancreatitis that extends into the anatomical area between the pancreatic head, the duodenum, and the common bile duct, which are referred to as the groove areas. We present the diagnostic modalities, pathological features and clinical outcomes of a series of symptomatic patients with groove pancreatitis who underwent pancreaticoduodenectomy. Methods Six patients undergoing pancreaticoduodenectomy between May 2006 and May 2009 due...

  19. Near Fatal Asthma: Clinical and Airway Biopsy Characteristics

    OpenAIRE

    Barbers, Richard G.; Papanikolaou, Ilias C.; Michael N. Koss; Ashish Patel ,; Elton Katagihara; Maggie Arenas; Khalid Chan; Azen, Colleen G.; Sharma, Om P.

    2012-01-01

    Background. Inflammation and remodeling are integral parts of asthma pathophysiology. We sought to describe the clinical and pathologic features of near fatal asthma exacerbation (NFE). Methods. Bronchial biopsies were collected prospectively from NFE I subjects. Another NFE II group and a moderate severity exacerbation control group (ME II) were retrospectively identified—no biopsies obtained. Results. All NFE II ( = 9 ) subjects exhibited remodeling and significant inflammation (eosinophi...

  20. Clinical and morphological characteristics of chronic duodenitis in children

    OpenAIRE

    Tishchenko D.V.; Matveeva O.V.; Chernenkov Yu.V.; Maslyakova G.N.; Bucharskaya A.B.

    2012-01-01

     

    The research goal is to determine clinical, endoscopic and morphological signs of chronic duodenitis in children. Materials and methods: The diagnostic value of molecular markers has been revealed by immunohistochemical research of biopsy. It has been received from endoscopic examination of 32 children aged from 3 to 17 years old with chronic duodenitis. Morphometric investigation of markers expression has been ca...

  1. Paranasal sinuses malignancies: A 12-year review of clinical characteristics

    Science.gov (United States)

    Sarafraz, Alireza; Chamani, Mojtaba; Derakhshandeh, Hossein

    2016-01-01

    Background Inadequate epidemiologic investigations of the paranasal sinuses malignancies prompted this retrospective study with special emphasis on a major group of 111 tumors. Material and Methods Clinical records of 111 patients with histologically confirmed malignant tumors of the paranasal sinuses were investigated retrospectively from April 2000 to January 2012. Collection of data included demographic information, clinical manifestations, treatment plans, and histopathology of the tumor. Results There were 69 (62.16%) male and 42 (37.83%) female patients (male-to-female ratio of 1.6:1), with a median age of 49±12.2 years (range 21 to 88 years). A high level of occurrence was noticed in the fifth (26.3%) decade of life. The most frequent histological types were squamous cell carcinoma (43.5%) and adenoid cystic carcinoma (19%). Among clinical manifestations, nasal obstruction was the most frequent followed by diplopia, and facial swelling. Fifty three patients (47.74%) were treated with combined approach of surgery and radiation therapy. Conclusions Paranasal sinuses malignancies are rare conditions with nonspecific symptoms which make early diagnosis of the lesions more challenging. The optimal therapeutic protocol for patients suffering from these tumors is still a somewhat controversial entity and requires further studies. Key words:Paranasal sinuses, malignancy, surgery,radiotherapy. PMID:27475693

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

    Institute of Scientific and Technical Information of China (English)

    李帅; 杨操; 杨述华; 邵增务; 郑启新

    2011-01-01

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

  3. Clinical characteristics and treatment of neurofibroma of the choroid

    Institute of Scientific and Technical Information of China (English)

    WEI Wen-bin; JIE Ying; MO Jing; LI Bin

    2012-01-01

    Background Neurofibroma is a kind of benign neoplasm that derives from nervous tissues.Though this tumor is the most common types in the peripheral nervous system,it is rarely seen in the choroid and easy to be misdiagnosed of choroidal melanoma.The aim of this study was to review the clinical features of neurofibroma of the choroid in the Chinese race.Methods A retrospective case series design was used.Two male and one female patients diagnosed with choroidal neurofibroma in Beijing Tongren Eye Center were included in this study.The clinical histories were abstracted from the patients' medical records.Routine eye examinations including visual acuity,intraocular pressure,slit lamp and ophthalmoscope were performed.Auxiliary examinations included fluorescein fundus angiography (FFA),AB-ultrasound scan,color doppler imaging (CDI),and magnetic resonance imaging (MRI).Local resection of the tumors was performed and the specimens underwent pathological examinations.Results The tumors were of yellow-pink color with pigmentation on the surface.CDI showed arterial blood signals in the tumor and MRI showed high-intensity in the T1-weighted image and a slightly increased intensity in the T2-weighted image.FFA and indocyaninegreen angiography demonstrated the tumors were of hypofluorescence at early stage and hyperfluorescence with prominent leakage at late stage.The pathological examination confirmed the tumors were choroidal neurofibroma.After 5-10-year follow-up,there were no recurrences of the tumors and the retinas were well attached.Conclusions Choroidal neurofibroma is difficult to be diagnosed clinically and pathological confirmation is important.These tumors can be managed conservatively by local resection.

  4. Comparative clinical characteristics of the methods for digoxin microassay

    Energy Technology Data Exchange (ETDEWEB)

    Trusov, V.V.; Zelenin, A.A.; Dundua, M.G. (Izhevskij Meditsinskij Inst. (USSR))

    1984-11-01

    A comparative characteristic of the methods for radioimmunological and immunofermental control of supporting therapy is given and the mechanisms of digoxin tolerance in cases of treatment of 89 patients with circulatory insufficiency are investigated. Pharmacokinetic parameters were calculated on the basis of bilocular mathematical model following the intravenous injection of 0.5 mg of digoxin. The absence of substantial differences in the methods under consideration is shown but a certain preference is given to the method of immunofermental analysis as it is convenient to be used for the daily control of the glycoside level in case of supporting therapy and investigations of the preparation pharmacokinetics.

  5. Clinical Characteristics and Treatment of Cardiomyopathies in Children.

    Science.gov (United States)

    Price, Jack F; Jeewa, Aamir; Denfield, Susan W

    2016-01-01

    Cardiomyopathies are diseases of the heart muscle, a term introduced in 1957 to identify a group of myocardial diseases not attributable to coronary artery disease. The definition has since been modified to refer to structural and or functional abnormalities of the myocardium where other known causes of myocardial dysfunction, such as systemic hypertension, valvular disease and ischemic heart disease, have been excluded. In this review, we discuss the pathophysiology, clinical assessment and therapeutic strategies for hypertrophic, dilated and hypertrophic cardiomyopathies, with a particular focus on aspects unique to children.

  6. Psychological characteristics and nursing measures of patients in dental clinic

    OpenAIRE

    Zhu, Hong-ying

    2014-01-01

    Dental clinic include dental pulp disease, dental, orthodontic repair and others. This work is  precise and meticulous.And the course of treatment and follow-up time is longer, the number of patients is much more. Patients need spend much time to wait,and need pay for high cost of this. Patients would inevitably have  negative emotions, under the influence of many factors. The doctors and nurses could use mental nursing to establish a good relationship between doctors and patients, and create...

  7. Imaging and clinical characteristics of sporadic Creutzfeldt-Jakob disease

    Directory of Open Access Journals (Sweden)

    HAN Shun-chang

    2013-04-01

    Full Text Available Five patients with sporadic Creutzfeldt-Jakob disease (sCJD presented rapidly progressive dementia which were subacute onset from 1 to 4 months. Among these cases, periodic synchronous discharge (PSD of electroencephalography (EEG was seen in 2 patients. Besides, 4 patients obtained positive results in cerebrospinal fluid (CSF analysis for 14-3-3 protein. The cranial MRI examination showed symmetrical or asymmetrical colored-ribbon-shaped high signals in cerebral cortex or basal ganglia by diffusion weighted imaging (DWI, suggesting that DWI had high sensitivity and specificity for the diagnosis of sCJD as a preferred method in the clinical examination of sCJD.

  8. Ideological and political education for clinical graduates on the basis of medical characteristics

    Directory of Open Access Journals (Sweden)

    Yun-chuan JING

    2014-09-01

    Full Text Available Along with the reform of medical system, medical education in China is also undergoing great changes. Due to the special characteristics of medical education, it differs from other educational characteristics. It carries with the characteristics of clinical practice on the basis distributed learning, physical and mental development along with ages, enrollment expansion and medical requirement, and standardization training for resident doctors. So, ideological and political education of clinical graduates showed many new characteristics. First, medical ethics education is the basic step, combined with the related disciplines of medical humanity connotation. Second, flexible and diversified form of ideological and political education on the basis of medical work is necessary. Third, establish a system of ideological and political education for clinical graduates, to build up new education concept, and to develop ideological and political education activities for clinical graduates in depth.

  9. Clinical characteristics of patients with gastroesophageal reflux disease in several centers of Northwest China

    Institute of Scientific and Technical Information of China (English)

    高麦仓

    2013-01-01

    Objective To investigate the clinical characteristics of gastroesophageal reflux disease (GERD) in several endoscopy centers of Northwest China.Methods From September 2008 to September 2009,a questionnaire survey was carried out in the endoscopy centers of four hospitals

  10. Zoonotic bacterial meningitis in adults: clinical characteristics, etiology, treatment and outcome

    NARCIS (Netherlands)

    A. van Samkar

    2016-01-01

    In this thesis, we describe the clinical characteristics, etiology, treatment and outcome of zoonotic bacterial meningitis. Each chapter describes meningitis patients infected by a specific zoonotic pathogen, such as Streptococcus equi, Streptococcuis suis, Capnocytophaga canimorsus, Campylobacter f

  11. Clinical characteristics and genetic analysis of three pediatric patients with idiopathic restrictive cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    杨世伟

    2013-01-01

    Objective Restrictive cardiomyopathy(RCM) is rare in children,and little is known about the molecular basis of RCM.The aim of this study was to investigate the clinical and myopathological characteristics and to detect

  12. Clinical management of behavioral characteristics of Prader–Willi syndrome

    Directory of Open Access Journals (Sweden)

    Alan Y Ho

    2010-04-01

    Full Text Available Alan Y Ho, Anastasia DimitropoulosDepartment of Psychology, Case Western Reserve University, Cleveland, OH, USAAbstract: Prader–Willi syndrome (PWS is a complex neurodevelopmental disorder caused by an abnormality on the long arm of chromosome 15 (q11–q13 that results in a host of phenotypic characteristics, dominated primarily by hyperphagia and insatiable appetite. Characteristic behavioral disturbances in PWS include excessive interest in food, skin picking, difficulty with a change in routine, temper tantrums, obsessive and compulsive behaviors, and mood fluctuations. Individuals with PWS typically have intellectual disabilities (borderline to mild/moderate mental retardation and exhibit a higher overall behavior disturbance compared to individuals with similar intellectual disability. Due to its multisystem disorder, family members, caregivers, physicians, dieticians, and speech-language pathologists all play an important role in the management and treatment of symptoms in an individual with PWS. This article reviews current research on behavior and cognition in PWS and discusses management guidelines for this disorder.Keywords: Prader–Willi syndrome; neurodevelopment, hyperphagia, disability

  13. Clinical characteristics of polycystic ovary syndrome in Indian women

    Directory of Open Access Journals (Sweden)

    Sunita J Ramanand

    2013-01-01

    Full Text Available Background: Polycystic ovary syndrome (PCOS is common diagnosis in women presenting with infertility. All the dimensions of PCOS have not been completely explored. Many studies have tried to characterize the exact presentation of the disease. In this study we studied clinical features of PCOS in Indian women to characterize different phenotypes of this syndrome. Prevalence of acanthosis nigricans (AN as surrogate marker of insulin resistance, obesity, hirsutism and hypothyroidism in PCOS women have been simultaneously studied. Materials and Methods: Present work is a non comparative cross-sectional open label study carried out over a period of 18 months in an endocrinology hospital in western Maharashtra, India. Results and Conclusion: Authors conclude that PCOS occurs both in obese and non-obese women; AN and hirsutism occur in equal proportion of patients. AN is correlated with obesity. Hormonal dysfunctions in PCOS manifested together or independently. PCOS women can be sub grouped based on clinical features suggestive of endocrinological malfunctions and can be investigated accordingly for selection of appropriate treatment modalities.

  14. Clinical and laboratory characteristics of women with uterine leiomiyoma

    Directory of Open Access Journals (Sweden)

    Özgür ÖZKUL

    2009-06-01

    Full Text Available The aim of this study was to compare clinical and laboratory findings of women with or without uterine leiomyoma.Study group consisted of 82 women with uterine leiomyoma and the control group comprised 42 healthy women. Women’s age, gravity, parity, blood groups, pattern of menstrual cycles, complaints at presentation, fertility, ultrasonographical findings, surgical operations and thyroid function tests were evaluated.There were no significant differences in blood group distribution, gravity, parity and thyroid function test results between the patients and the control subjects (P>0.05. A significant difference was found in the complaints at presentation between two groups (P<0.001. Mentrual cycles irregularity was more frequently found in the patients compared with the controls (57.3% vs. 42.9%, respectively, P=0.009. Although no infertile woman was found in the control group, 8.5% of patients were found to have infertility. The sensitivity of ultrasonography was found to be 97.6%. Except for the existence of higher infertility rate and the menstrual cycles irregularities, no significant difference was found in the clinical and laboratory findings between women with or without uterine leiomyoma. Therefore, physical examination and imaging methods are remained as the most important diagnostic tools for uterine leiomyoma.

  15. [Clinical-electroneuromyographical characteristics of facial nerve paralysis in children].

    Science.gov (United States)

    Gribova, N P; Galitskaia, O S

    2009-01-01

    A clinical-electroneuromyographical study of 40 children (32 (80%) of them aged from 12 to 17 years, mean age 13,9+/-1,8 years, and 8 (20%) - from 1 to 8 years, mean age 4,4+/-2,1 years) were studied in the acute period of facial nerve paralysis (FNP). Six (15%) children had FNP in the anamnesis. Among precipitating factors were the cold exposure the day before disease onset (20 (50%) patients), symptoms of flu (13 (32,5%) patients) and psycho-emotional tension (3 (7,5%) patients). No precipitation was noted in 4 (10%) children. The degree of muscle paresis was 81,9+/-7% that corresponded to clinical stages III-IV according to K. Rosler. An electroneuromyographical analysis of motor ortho- and antidromic response to the facial nerve stimulation on the side of paresis and on the contralateral side in patients and controls revealed the presence of proximal axon- and myelinopathy of facial nerve with the involvement of its own motorneurons and brain stem interneurons. The maintenance of wink reflex and F-wave blocks in the period over 3 weeks are prognostically unfavorable factors for restoration of mimic muscle function in the early stage of disease.

  16. Clinical and diagnostic characteristics of patients with suspected polyneuropathy

    Directory of Open Access Journals (Sweden)

    Mikhailova Е.V.

    2013-09-01

    Full Text Available Objective: to study the causes and clinical manifestations of disease in children referred for hospitalization in children infectious diseases hospital in Saratov with a diagnosis of «acute flaccid paralysis». Material and methods: 157 children with the diagnosis on admission of the guide «acute flaccid paralysis». Conducted clinical examination and laboratory tests included a general analysis of cerebrospinal fluid, urine, virological examination of nasal swabs and faeces, with the definition of a serological ELISA method and RPHA immunoglobulins to influenza, rubella, and enterovirus, immunological study of blood, cerebrospinal fluid PCR, electromyography of the affected limbs. Results. 77 patients (49% with the disease associated with the violation of the musculoskeletal system were registered. In the other cases revealed polyneuropathy was not of poliovirus etiology. Etiological nature of the disease could be explained by 54% of patients. In 37 (46% patients the diagnosis was formulated in accordance with the severity of paralysis. One child was diagnosed with a vaccine-associated poliomyelitis. Conclusion. The diagnosis of «acute flaccid paralysis» used as administered requires a detailed interpretation in a hospital.

  17. Hemangioblastomas in the elderly: epidemiology and clinical characteristics.

    Science.gov (United States)

    Kassardjian, Charles D; Macdonald, R Loch; Munoz, David G

    2014-07-01

    Intracranial hemangioblastomas are benign vascular tumors. The peak age of incidence is between 20 to 50 years. Hemangioblastomas rarely occur in patients over the age of 65. To our knowledge there is no review of the prevalence and clinical features in an elderly population. We reviewed our 12 year experience with intracranial hemangioblastomas, and characterized the clinical features of hemangioblastomas in patients over the age of 65. We present a 72-year-old man with a cerebellar mass initially thought to be a metastasis as an illustrative case. We reviewed our pathology database and identified all patients with a histopathologically confirmed diagnosis of hemangioblastoma over the last 12 years in a large tertiary adult hospital; all patients were over the age of 18. Of all cases of hemangioblastoma in the last 12 years, six of 77 (7.7%) occurred in patients over the age of 65. All were cerebellar in location, and none were associated with von-Hippel Lindau disease. Hemangioblastomas are uncommon, but not rare, in patients over the age of 65, and should be included in the differential diagnosis of patients presenting with gait ataxia and a cerebellar lesion in this age group. PMID:24629394

  18. Clinical and Histopathological Characteristics between Familial and Sporadic Melanoma in Barcelona, Spain

    OpenAIRE

    Aguilera, Paula; Malvehy, Josep; Carrera, Cristina; Palou, Josep; Puig-Butillé, Joan Anton; Alòs, Llúcia; Badenas, Celia; Puig, Susana

    2014-01-01

    Background About 6 to 14% of melanoma cases occur in a familial setting. Germline mutations in CDKN2A are detected in 20 to 40% of melanoma families. Objective To characterise the clinical and histopathological characteristics of familial melanoma thus providing more information to clinicians and contribute to the understanding of the genetic-environment interplay in the pathogenesis of melanoma. Methods Clinical, histological and immunohistochemical characteristics of 62 familial melanomas w...

  19. Monosomal karyotype among adult acute myeloid leukemia: clinical characteristic and prognostic analysis

    Institute of Scientific and Technical Information of China (English)

    冯茹

    2014-01-01

    Objective To explore the clinical characteristics and prognostic value of monosomal karyotype(MK)patients in adult acute myeloid leukemia(AML).Methods We retrospectively studied 45 patients of MK+in newly-diagnosed adult AML in our center from Oct 2000 to Dec2012.Clinical characteristics,cytogenetic data and prognostic features were analyzed in the cohort of MK+patients.Results MK was found in 45 patients(19.0%)

  20. Near Fatal Asthma: Clinical and Airway Biopsy Characteristics

    Directory of Open Access Journals (Sweden)

    Richard G. Barbers

    2012-01-01

    Full Text Available Background. Inflammation and remodeling are integral parts of asthma pathophysiology. We sought to describe the clinical and pathologic features of near fatal asthma exacerbation (NFE. Methods. Bronchial biopsies were collected prospectively from NFE I subjects. Another NFE II group and a moderate severity exacerbation control group (ME II were retrospectively identified—no biopsies obtained. Results. All NFE II (=9 subjects exhibited remodeling and significant inflammation (eosinophilic, neutrophilic. NFE II group (=37 had a significant history of prior intubation and inhaled corticosteroids usage compared to ME II group (=41. They also exhibited leukocytosis, eosinophilia, and longer hospitalization days. Conclusions. Remodeling, eosinophilic, and neutrophilic inflammation were observed in NFE. NFE is associated with prior intubation and inhaled corticosteroids usage.

  1. Intussusception in adults: Clinical characteristics, diagnosis and operative strategies

    Institute of Scientific and Technical Information of China (English)

    Savas Yakan; Cemil Caliskan; Ozer Makay; Ali Galip Denecl; Mustafa Ali Korkut

    2009-01-01

    AIM: To evaluate 20 adults with intussusception and to clarify the cause, clinical features, diagnosis, and management of this uncommon entity.METHODS: A retrospective review of patients aged >18 years with a diagnosis of intestinal intussusception between 2000 and 2008. Patients with rectal prolapse,prolapse of or around an ostomy and gastroenterostomy intussusception were excluded.RESULTS: There were 20 cases of adult intussusception.Mean age was 47.7 years. Abdominal pain, nausea,and vomiting were the most common symptoms. The majority of intussusceptions were in the small intestine (85%). There were three (15%) cases of colonic intussusception. Enteric intussusception consisted of five jejunojejunal cases, nine ileoileal, and four cases of ileocecal invagination. Among enteric intussusceptions,14 were secondary to a benign process, and in one of these, the malignant cause was secondary to metastatic lung adenocarcinoma. All colonic lesions were malignant. All cases were treated surgically.

  2. Fatal tumors: prenatal ultrasonographic findings and clinical characteristics

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Jeong Yeon [Dept. of Radiology, Seoul National University College of Medicine, Seoul (Korea, Republic of); Lee, Young Ho [Dept. of Radiology, Cheil General Hospital and Women' s Healthcare Center, Kwandong University College of Medicine, Seoul (Korea, Republic of)

    2014-12-15

    The incidence of fetal tumors has been increased due to generalization of prenatal evaluation and improvement of imaging techniques. The early detection of a fetal tumor and understanding of its imaging features are very important for fetal, maternal, and neonatal care. Ultrasonography is usually used for the detection and differential diagnosis of fetal tumors, and magnetic resonance imaging is increasingly being used as a complementary study. Many fetal tumors have different clinical and imaging features compared with pediatric tumors. Although several fetal tumors may mimic other common anomalies, some specific imaging features may carry early accurate diagnosis of fetal tumors, which may alter the prenatal management of a pregnancy and the mode of delivery, and facilitate immediate postnatal treatment.

  3. A case of clinical Reye syndrome presenting characteristic CT changes

    International Nuclear Information System (INIS)

    A 9-month-old male infant was admitted to our hospital on the second day of cold like syndrome because of high fever, convulsion, coma, and decerebrate rigidity. Serum GOT, GPT, LDH, and CPK were markedly elevated. Serum ammonia was slightly increased, and hypoglycemia was present. The cerebrospinal fluid showed no pleocytosis, normal sugar content, but increased protein. Thus we made a diagnosis of clinical Reye syndrome according to the criteria by Yamashita, et al. A CT on the day of admission showed symmetrical low-density areas in the posterior fossa and the regions of thalamus. Ringed enhancements were seen around the areas of low density in the thalamus on the twenty-second hospital day. We consider that these lesions may represent the infarction due to obstruction of the thalamoperforant arteries caused by cerebral edema in the early stage of the disease. (author)

  4. Clinical characteristics of patients with benign nonlesional temporal lobe epilepsy

    Science.gov (United States)

    Kim, Jiyeon; Kim, Seong Hoon; Lim, Sung Chul; Kim, Woojun; Shon, Young-Min

    2016-01-01

    Purpose To evaluate the evolution of nonlesional temporal lobe epilepsy (TLE-NL) in patients treated exclusively with antiepileptic drugs and to elucidate clinical phenotypes related to the prognosis of these patients. Methods Clinical, radiological, and electroencephalographic (EEG) findings in 84 patients with TLE-NL were reviewed. A good response group (GRG) and a poor response group (PRG) were defined if the duration of their seizure-free period was >1 year, or <1 year, respectively. Results There were 46 (54.8%) patients in the GRG and 38 (45.2%) patients in the PRG. The number of antiepileptic drugs administered was significantly lower in the GRG than that in the PRG (1.3±0.8 vs 2.8±1.0, respectively; P<0.05). The GRG had a significantly older age of onset than the PRG and a lower occurrence of initial precipitating events, such as febrile seizures, central nervous system infection, and head trauma (P<0.05). The prevalence of EEG abnormality, presence of aura, generalized seizures, and automatism was less frequently observed in the GRG (P<0.05). Multivariate analysis showed that the presence of automatism and initial precipitating events were significantly associated with a poor prognosis (P<0.05). Conclusion In contrast to the commonly assumed intractability of TLE, we found that more than 54% of patients with TLE-NL achieved a long seizure-free period. Older age at onset of TLE-NL was associated with a better prognosis. However, the presence of automatism and initial precipitating events were related to a poor prognosis. Future prospective studies with a much larger population are warranted. PMID:27555776

  5. CLINICAL AND BIOCHEMICAL CHARACTERISTICS AT THE CIRRHOSIS OF VARIOUS GENESIS

    Directory of Open Access Journals (Sweden)

    A. R. Bilalova

    2016-01-01

    Full Text Available Purpose of the study. To carry out a comparative description of the clinical and biochemical parameters at patients with cirrhosis of an alcoholic genesis (CP-HGA and cirrhosis of mixed etiologies — CP-HGM (HСV+ alcohol.Materials and methods. The study involved 62 patients with cirrhosis of different etiologies, who carries out clinical, immunogenetic and biochemical studies.Results. Patients with the 3d genotype and low viral load were registered with cirrhosis of mixed etiologies (HСV+ alcohol. At the cirrhosis Class B for Child-Pugh basic data biochemical parameters were similar in patients with CP-HGA and CP-HGM, but ALT and AST activity, which are significantly higher than observed in patients with CP-HGM. At dismissal, ALT and GGT activities were detected significantly higher in patients with CP-HGM than the CP-HGA. At the cirrhosis Class C for Child-Pugh the baseline, reflecting cholestasis — is total bilirubin, GGT and alkaline phosphatase and were detected significantly higher in the CP-HGA, than with CP-HGM significantly reduced, and thore is no differences between the groups to be discharged from the hospital, in addition to the activity of GGT, which it remained significantly higher in the CP-HGA, than with CP-HGM. Cytolytic activity of enzymes (ALT, AST during the entire period of the disease was observed significantly higher normal values and did not depend on the CP etiology.Conclusion decision. The maximum rate of change of the basic biochemical parameters is observed in patients with cirrhosis of mixed etiologies Class B for Child-Pugh and at the cirrhosis of an alcoholic genesis — in patients with cirrhosis Class C.

  6. Clinical Characteristics and Prognosis of End-stage Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Yan Xiao

    2015-01-01

    Full Text Available Background: End-stage hypertrophic cardiomyopathy (HCM is complicated by substantial adverse events. However, few studies have focused on electrocardiographic features and their prognostic values in HCM. This study aimed to evaluate the clinical manifestations and prognostic value of electrocardiography in patients with end-stage HCM. Methods: End-stage HCM patients were enrolled from a total of 1844 consecutive HCM patients from April 2002 to November 2013 at Fuwai Hospital. Clinical data, including medical history, electrocardiography, and echocardiography, were analyzed. Cox hazards regression analysis was used to assess the risk factors for cardiovascular mortality. Results: End-stage HCM was identified in 99 (5.4% patients, averaged at 52 ± 16 years old at entry. Atrial fibrillation was observed in 53 patients and mural thrombus in 19 patients. During 3.9 ± 3.0 years of follow-up, embolic stroke, refractory heart failure, and death or transplantation were observed in 20, 39, and 51 patients, respectively. The incidence of annual mortality was 13.2%. Multivariate Cox hazards regression analysis identified New York Heart Association Class (NYHA III/IV at entry (hazard ratio [HR]: 1.99; 95% confidence interval [CI]: 1.05-3.80; P = 0.036, left bundle branch block (LBBB (HR: 2.80; 95% CI: 1.47-5.31; P = 0.002, and an abnormal Q wave (HR: 2.21; 95% CI: 1.16-4.23; P = 0.016 as independent predictors of cardiovascular death, in accordance with all-cause death and heart failure-related death. Conclusions: LBBB and an abnormal Q wave are risk factors of cardiovascular mortality in end-stage HCM and provide new evidence for early intervention. Susceptibility of end-stage HCM patients to mural thrombus and embolic events warrants further attention.

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

    Science.gov (United States)

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

    2014-01-01

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

  8. 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.

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

    Science.gov (United States)

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

    2015-01-01

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

  10. Clinical and social characteristics of women registered on a dispensary as drug addiction

    OpenAIRE

    М.SH. JAMANTAYEVA; A.S. SUBHANBERDINA; N.E. DUNENBAYEV; D.M.SERGAZIYEV

    2015-01-01

    The article reveals some clinical and social characteristics of women registered on a drug-abuse dispensary with mental and behavioral disorders due to use of drugs. The present study points to their influence on structural dynamic characteristics of social adaptation of patients. There are some comprehensive measures of medical and rehabilitative activities directed at outpatient drug treatment.

  11. Prevalence, clinical characteristics and management of atrial fibrillation in patients with Brugada syndrome

    NARCIS (Netherlands)

    Rodriguez-Manero, M.; Namdar, M.; Sarkozy, A.; Casado-Arroyo, R.; Ricciardi, D.; Asmundis, C. de; Chierchia, G.B.; Wauters, K.; Rao, J.Y.; Bayrak, F.; Malderen, S. Van; Brugada, P.

    2013-01-01

    Atrial fibrillation (AF) can be the first manifestation of latent Brugada syndrome (BS). The aim of our study was to assess the prevalence of AF as the first clinical diagnosis in patients with BS and their demographic and clinical characteristics and diagnosis management in a large cohort of patien

  12. Clinical Application and Characteristics of KONG's Scalp Acupuncture and Daoyin Technique

    Institute of Scientific and Technical Information of China (English)

    JIANG Ling-zhen; XIAO Yuan-chun

    2004-01-01

    @@ Scalp Acupuncture and Daoyin Technique, which are summarized by Dr. KONG in the light of his long-term acupuncture practice, has been considered as one of the clinical diagnostic and treatment programs in Chinese medicine by the State Administration of Traditional Chinese Medicine. In this paper, the characteristics and clinical application of Scalp Acupuncture and Daoyin Technique are presented as follows.

  13. The clinical characteristics of 625 patients with malignant small bowel tumors

    Institute of Scientific and Technical Information of China (English)

    俞利结

    2012-01-01

    Objective To investigate the clinical characteristics of MSBT(malignant small bowel tumors) .Methods Six hundred and twenty-five cases of MSBT were recruited in this study and their clinical records and information including age,gender,blood types,family history,personal

  14. Characteristics of Placebo Responders in Pediatric Clinical Trials of Attention-Deficit/Hyperactivity Disorder

    Science.gov (United States)

    Newcorn, Jeffrey H.; Sutton, Virginia K.; Zhang, Shuyu; Wilens, Timothy; Kratochvil, Christopher; Emslie, Graham J.; D'Souza, Deborah N.; Schuh, Leslie M.; Allen, Albert J.

    2009-01-01

    Objective: Understanding placebo response is a prerequisite to improving clinical trial methodology. Data from placebo-controlled trials of atomoxetine in the treatment of children and adolescents with attention-deficit/hyperactivity disorder (ADHD) were analyzed to identify demographic and clinical characteristics that might predict placebo…

  15. Clinical characteristics of patients with benign nonlesional temporal lobe epilepsy

    Directory of Open Access Journals (Sweden)

    Kim J

    2016-07-01

    Full Text Available Jiyeon Kim,1 Seong Hoon Kim,2 Sung Chul Lim,2 Woojun Kim,2 Young-Min Shon3 1Department of Neurology, Korea University Ansan Hospital, College of Medicine, Korea University, Ansan, 2Department of Neurology, Catholic Neuroscience Institute, College of Medicine, The Catholic University of Korea, Seocho-gu, 3Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Purpose: To evaluate the evolution of nonlesional temporal lobe epilepsy (TLE-NL in patients treated exclusively with antiepileptic drugs and to elucidate clinical phenotypes related to the prognosis of these patients.Methods: Clinical, radiological, and electroencephalographic (EEG findings in 84 patients with TLE-NL were reviewed. A good response group (GRG and a poor response group (PRG were defined if the duration of their seizure-free period was >1 year, or <1 year, respectively.Results: There were 46 (54.8% patients in the GRG and 38 (45.2% patients in the PRG. The number of antiepileptic drugs administered was significantly lower in the GRG than that in the PRG (1.3±0.8 vs 2.8±1.0, respectively; P<0.05. The GRG had a significantly older age of onset than the PRG and a lower occurrence of initial precipitating events, such as febrile seizures, central nervous system infection, and head trauma (P<0.05. The prevalence of EEG abnormality, presence of aura, generalized seizures, and automatism was less frequently observed in the GRG (P<0.05. Multivariate analysis showed that the presence of automatism and initial precipitating events were significantly associated with a poor prognosis (P<0.05.Conclusion: In contrast to the commonly assumed intractability of TLE, we found that more than 54% of patients with TLE-NL achieved a long seizure-free period. Older age at onset of TLE-NL was associated with a better prognosis. However, the presence of automatism and initial precipitating events were related to a poor prognosis. Future prospective

  16. Comparison of clinical and laboratory characteristics of viral hepatitis A and E in Montenegro

    OpenAIRE

    Terzić Dragica; Mijović Gordana; Dupanović Brankica; Drašković Nenad; Švirtlih Neda

    2010-01-01

    Introduction Hepatitis E has many similarities in with hepatitis A concerning clinical picture, route of transmission and nonexistence of chronicity. Comparison of clinical and laboratory parameters of patients with hepatitis A and E to estimate characteristics of these diseases. Material and methods Total of 54 patients divided into two groups was investigated: 27 had hepatitis A, others had hepatitis E. Detailed history past, clinical examination, liver function tests and ultrasonography of...

  17. Clinical characteristics and prognosis of patients with retinoblastoma: 8-year follow-up

    OpenAIRE

    Luo, Xin; YE, HUIJING; Ding, Yun-gang; Du, Yi; Yang, Huasheng

    2015-01-01

    Background/aim: The aim of this study was to investigate the potential correlation between clinical characteristics and prognosis of patients with retinoblastoma (Rb). Materials and methods: This retrospective study included 314 Rb patients. Clinical data including laterality of eyes, sex, age, presenting signs, lag time, and survival were recorded and analyzed. Results: Leukocoria is the most common clinical presentation of Rb. Patients with isolated leukocoria had shorter lag time and e...

  18. Protein carriers of conjugate vaccines: characteristics, development, and clinical trials.

    Science.gov (United States)

    Pichichero, Michael E

    2013-12-01

    The immunogenicity of polysaccharides as human vaccines was enhanced by coupling to protein carriers. Conjugation transformed the T cell-independent polysaccharide vaccines of the past to T cell-dependent antigenic vaccines that were much more immunogenic and launched a renaissance in vaccinology. This review discusses the conjugate vaccines for prevention of infections caused by Hemophilus influenzae type b, Streptococcus pneumoniae, and Neisseria meningitidis. Specifically, the characteristics of the proteins used in the construction of the vaccines including CRM, tetanus toxoid, diphtheria toxoid, Neisseria meningitidis outer membrane complex, and Hemophilus influenzae protein D are discussed. The studies that established differences among and key features of conjugate vaccines including immunologic memory induction, reduction of nasopharyngeal colonization and herd immunity, and antibody avidity and avidity maturation are presented. Studies of dose, schedule, response to boosters, of single protein carriers with single and multiple polysaccharides, of multiple protein carriers with multiple polysaccharides and conjugate vaccines administered concurrently with other vaccines are discussed along with undesirable consequences of conjugate vaccines. The clear benefits of conjugate vaccines in improving the protective responses of the immature immune systems of young infants and the senescent immune systems of the elderly have been made clear and opened the way to development of additional vaccines using this technology for future vaccine products. PMID:23955057

  19. Histamine H1 antagonists and clinical characteristics of febrile seizures

    Directory of Open Access Journals (Sweden)

    Zolaly MA

    2012-03-01

    Full Text Available Mohammed A ZolalyDepartment of Pediatrics, College of Medicine, Taibah University, Al-Madinah Al-Munawarah, Kingdom of Saudi ArabiaBackground: The purpose of this study was to determine whether seizure susceptibility due to antihistamines is provoked in patients with febrile seizures.Methods: The current descriptive study was carried out from April 2009 to February 2011 in 250 infants and children who visited the Madinah Maternity and Children's Hospital as a result of febrile convulsions. They were divided into two groups according to administration of antihistamines at the onset of fever.Results: Detailed clinical manifestations were compared between patients with and without administration of antihistamines. The time from fever detection to seizure onset was significantly shorter in the antihistamine group than that in the nonantihistamine group, and the duration of seizures was significantly longer in the antihistamine group than in the nonantihistamine group. No significant difference was found in time from fever detection to seizure onset or seizure duration between patients who received a first-generation antihistamine and those who received a second-generation antihistamine.Conclusion: Due to their central nervous system effects, H1 antagonists should not be administered to patients with febrile seizures and epilepsy. Caution should be exercised regarding the use of histamine H1 antagonists in young infants, because these drugs could potentially disturb the anticonvulsive central histaminergic system.Keywords: antihistamine, nonantihistamine, histamine H1 antagonist, febrile seizures

  20. Clinical characteristics and current therapies for inherited retinal degenerations.

    Science.gov (United States)

    Sahel, José-Alain; Marazova, Katia; Audo, Isabelle

    2014-10-16

    Inherited retinal degenerations (IRDs) encompass a large group of clinically and genetically heterogeneous diseases that affect approximately 1 in 3000 people (>2 million people worldwide) (Bessant DA, Ali RR, Bhattacharya SS. 2001. Molecular genetics and prospects for therapy of the inherited retinal dystrophies. Curr Opin Genet Dev 11: 307-316.). IRDs may be inherited as Mendelian traits or through mitochondrial DNA, and may affect the entire retina (e.g., rod-cone dystrophy, also known as retinitis pigmentosa, cone dystrophy, cone-rod dystrophy, choroideremia, Usher syndrome, and Bardet-Bidel syndrome) or be restricted to the macula (e.g., Stargardt disease, Best disease, and Sorsby fundus dystrophy), ultimately leading to blindness. IRDs are a major cause of severe vision loss, with profound impact on patients and society. Although IRDs remain untreatable today, significant progress toward therapeutic strategies for IRDs has marked the past two decades. This progress has been based on better understanding of the pathophysiological pathways of these diseases and on technological advances.

  1. PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS

    Directory of Open Access Journals (Sweden)

    K. Yu. Mukhin

    2016-01-01

    Full Text Available Pseudo-Lennox syndrome (PLS, or atypical benign partial epilepsy of childhood, is a disease from a group of age-related epileptic encephalopathies with a phenomenon of continuous spike-wave activity during slow sleep, which manifests itself as frequent polymorphic focal motor and pseudogeneralized seizures, cognitive impairments, as well as regional and diffuse epileptiform activity on electroencephalogram (EEG by the morphology identical to that of benign epileptiform patterns of childhood. The disease was first described by J. Aicardi and J. J. Chevrie in 1982, based on a study of 7 cases. Its diagnostic complexity is the polymorphism of both epileptic seizures and EEG data, as well as low awareness of the syndrome among physicians and its absence in the international classification of epilepsies. The typical triad of seizures, which occurs in nearly 100 % of patients, encompasses focal motor paroxysms (identical to those as observed in Rolandic epilepsy, atypical absences, and atonic seizures. Seizures in PLS in its active period (generally up to 7–8 years are highly resistant to antiepileptic drugs. Only a few agents have been proven to be effective in PLS; these include valproates, succinimides, benzodiazepines, topiramate, and sulthiame. The frequency of seizures are noted to increase in patients with PLS treated with drugs, such as vigabatrin, gabapentin, lamotrigine, phenobarbital, or phenytoin. The author considers in detail the history of studies of the disease, clinical manifestations, diagnostic criteria, therapeutic approaches, and prognosis.

  2. Clinical disease characteristics according to karyotype in Turner syndrome

    Directory of Open Access Journals (Sweden)

    Chae Young Yeo

    2010-02-01

    Full Text Available Purpose : Turner syndrome (TS is a disorder in which various anomalies can be accompanied, especially cardiovascular, renal, thyroid and auditory problems. The aim of this study is to identify the incidence of these disorders in patients with TS according to karyotype. Methods : We reviewed medical records of 90 patients with TS diagnosed by chromosomal analysis in 4 hospitals from Jan 1998 to Dec 2007. We evaluated these cases by prepared protocol of 4 medical problems. Results : The distribution of karyotype was 45,X (47.8%, mosaic pattern (34.4% and structural aberration group (17.8 %. Renal anomalies, cardiovascular anomalies, thyroid disorders and auditory problems are accompanied in 4.4%, 10.0 %, 11.1% and 5.6%, respectively. 45,X group had renal anomalies (7.0%, cardiovascular anomalies (18.6%, thyroid disorders (9.3% and auditory problems (11.6%. Mosaic group had renal anomalies (3.2%, thyroid disorders (12.9%, no cardiovascular anomalies and auditory problems. Structural aberration group had cardiovascular anomalies (6.3%, thyroid disorders (12.5% and no other 2 problems. Patients with 45,X group had a significant higher incidence of cardiovascular anomalies (P=0.025. Conclusion : Our results indicate that there are differences clinically according to karyotype of TS, especially in incidence of cardiovascular anomalies.

  3. Clinical and morphological characteristics of chronic duodenitis in children

    Directory of Open Access Journals (Sweden)

    Tishchenko D.V.

    2012-09-01

    Full Text Available

     

    The research goal is to determine clinical, endoscopic and morphological signs of chronic duodenitis in children. Materials and methods: The diagnostic value of molecular markers has been revealed by immunohistochemical research of biopsy. It has been received from endoscopic examination of 32 children aged from 3 to 17 years old with chronic duodenitis. Morphometric investigation of markers expression has been carried out by means of analyzing system of digital images of Mikrovizor medical uVizo-103. Results: The index of proliferation has been higher in cells of cover epithelium than in glands. The inductor expression of apoptosis Bax has been poorly expressed in both groups of patients. It has been proved that proliferative processes are predominant in this pathology. Conclusion: It has been found out that proliferation of significant activity in the cover epithelium accompanies the development of preatrophic processes in children with chronic duodenitis. The greater degree of regeneration has been marked in crypts.

  4. Clinical characteristics and current therapies for inherited retinal degenerations.

    Science.gov (United States)

    Sahel, José-Alain; Marazova, Katia; Audo, Isabelle

    2015-02-01

    Inherited retinal degenerations (IRDs) encompass a large group of clinically and genetically heterogeneous diseases that affect approximately 1 in 3000 people (>2 million people worldwide) (Bessant DA, Ali RR, Bhattacharya SS. 2001. Molecular genetics and prospects for therapy of the inherited retinal dystrophies. Curr Opin Genet Dev 11: 307-316.). IRDs may be inherited as Mendelian traits or through mitochondrial DNA, and may affect the entire retina (e.g., rod-cone dystrophy, also known as retinitis pigmentosa, cone dystrophy, cone-rod dystrophy, choroideremia, Usher syndrome, and Bardet-Bidel syndrome) or be restricted to the macula (e.g., Stargardt disease, Best disease, and Sorsby fundus dystrophy), ultimately leading to blindness. IRDs are a major cause of severe vision loss, with profound impact on patients and society. Although IRDs remain untreatable today, significant progress toward therapeutic strategies for IRDs has marked the past two decades. This progress has been based on better understanding of the pathophysiological pathways of these diseases and on technological advances. PMID:25324231

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

    Directory of Open Access Journals (Sweden)

    Tiago Leal Ghezzi

    2009-06-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1994-12-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Science.gov (United States)

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

    2016-09-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2013-10-15

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

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

    Science.gov (United States)

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

    2016-09-01

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

  11. The Clinical Characteristics and Imaging Findings of Morning Glory Syndrome

    Institute of Scientific and Technical Information of China (English)

    Jun HU

    2008-01-01

    To investigate the features of CT, ultrasonography and fundus fluorescein angiography(FFA) of morning glory syndrome, the data on CT, A/B-scan ultrasonography and fundus fluorescein angiography (FFA) were retrospectively analyzed in 8 cases of morning glory syndrome (MGS).Among those cases, 6 were examined with CT, 4 with FFA and 8 with A/B-scan ultrasonography.Results showed that the characteristics of CT, A/B-scan ultrasonography and FFA in MGS included:(1) The attachment spot of optic nerve became thin and vitreous body protruded to the posterior wall of eyeball with a spherical shape on CT image; (2) in the early period of FFA, hypofluorescence appeared on the optic, the abnormal arteriae and veins around the optic papilla were displayed clearly and in the late period, optic disc was stained with fluorescein; (3) on B-scan ultrasonogram, the vitreous cavity extended to the posterior pole and optic papilla, and projected to the basal part of muscle cones and thus the posterior part of vitreous cavity looked like an upside-down bottleneck. Sometimes the echogenic band of retinal detachment could also be seen. On A-scan ultrasonogram, both vitreous cavity and bottleneck showed no ultrasonic echoes and presented a base line without any evident wave crest. It is concluded that CT, A/B-scan ultrasonography and FFA could show the imageological features of MGS from different aspects, which helps clinicians to differentiate it from other diseases such as optic disc coloboma. CT and A/B-scan ultrasonography, in particular, are considered to be reliable imageological methods for the accurate diagnosis of MGS and are superior to the traditional techniques.

  12. Maternal characteristics and clinical diagnoses influence obstetrical outcomes in Indonesia.

    Science.gov (United States)

    Adisasmita, Asri; Smith, Carl V; El-Mohandes, Ayman A E; Deviany, Poppy Elvira; Ryon, Judith J; Kiely, Michele; Rogers-Bloch, Quail; Gipson, Reginald F

    2015-07-01

    This Indonesian study evaluates associations between near-miss status/death with maternal demographic, health care characteristics, and obstetrical complications, comparing results using retrospective and prospective data. The main outcome measures were obstetric conditions and socio-economic factors to predict near-miss/death. We abstracted all obstetric admissions (1,358 retrospective and 1,240 prospective) from two district hospitals in East Java, Indonesia between 4/1/2009 and 5/15/2010. Prospective data added socio-economic status, access to care and referral patterns. Reduced logistic models were constructed, and multivariate analyses used to assess association of risk variables to outcome. Using multivariate analysis, variables associated with risk of near-miss/death include postpartum hemorrhage (retrospective AOR 5.41, 95 % CI 2.64-11.08; prospective AOR 10.45, 95 % CI 5.59-19.52) and severe preeclampsia/eclampsia (retrospective AOR 1.94, 95 % CI 1.05-3.57; prospective AOR 3.26, 95 % CI 1.79-5.94). Associations with near-miss/death were seen for antepartum hemorrhage in retrospective data (AOR 9.34, 95 % CI 4.34-20.13), and prospectively for poverty (AOR 2.17, 95 % CI 1.33-3.54) and delivering outside the hospital (AOR 2.04, 95 % CI 1.08-3.82). Postpartum hemorrhage and severe preeclampsia/eclampsia are leading causes of near-miss/death in Indonesia. Poverty and delivery outside the hospital are significant risk factors. Prompt recognition of complications, timely referrals, standardized care protocols, prompt hospital triage, and structured provider education may reduce obstetric mortality and morbidity. Retrospective data were reliable, but prospective data provided valuable information about barriers to care and referral patterns. PMID:25656716

  13. Clinical characteristics of adult asthma associated with small airway dysfunction.

    Science.gov (United States)

    Kjellberg, S; Houltz, B K; Zetterström, O; Robinson, P D; Gustafsson, Per M

    2016-08-01

    Suboptimal asthma control is common despite modern asthma therapy. The degree of peripheral airway involvement remains unclear and poor medication delivery to these regions might be a contributing reason for this failure in obtaining adequate symptom control. A cohort of 196 adults (median (range) age 44 (18-61) years, 109 females, 54 ex-smokers, six current smokers) with physician-diagnosed asthma were recruited from primary care. Subjects were characterized clinically by interviews, questionnaires, skin prick tests (SPT) and blood eosinophil counts. Lung function was assessed by spirometry, impulse oscillometry (IOS) and nitrogen multiple breath washout (N2 MBW). IOS assessed peripheral airway resistance (FDR, frequency dependence of resistance). N2 MBW assessed global ventilation inhomogeneity (LCI, lung clearance index), specific indices of peripheral airway function (Scond × VT and Sacin × VT; VT, tidal volume), and inter-regional inhomogeneity (specific ventilation ratio). Never-smoking healthy cohorts of 158 and 400 adult subjects provided local reference values for IOS and N2 MBW variables, respectively. Peripheral airway dysfunction was detected in 31% (FDR or specific ventilation ratio) to 47% (Scond x VT) of subjects. Risk factors for peripheral airway dysfunction were identified. Among subjects with low FEV1 and either positive smoking history and/or blood eosinophilia (>4.0%), 63% had abnormality across all peripheral airway outcomes, whilst only one subject was completely normal. Abnormal peripheral airway function was present in a large proportion of adult asthmatics at baseline. Reduced FEV1, a positive smoking history, and/or blood eosinophilia identified "a small airway asthma subtype" that might benefit from peripheral airway targeted therapy. PMID:27492518

  14. 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.)

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

    Science.gov (United States)

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

    2014-01-01

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

  16. 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.

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Alexander G Weil

    2015-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

  1. 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.

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

    Directory of Open Access Journals (Sweden)

    Cláudio Régis S. Silveira

    2001-02-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2014-01-01

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

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

    Science.gov (United States)

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

    2016-06-01

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

  6. Clinical Characteristics Associated With Aspiration or Penetration in Children With Swallowing Problem

    OpenAIRE

    Bae, Soon Ook; Lee, Gang Pyo; Seo, Han Gil; Oh, Byung-Mo; Han, Tai Ryoon

    2014-01-01

    Objective To evaluate demographic characteristics of children with suspected dysphagia who underwent videofluoroscopic swallowing study (VFSS) and to identify factors related to penetration or aspiration. Methods Medical records of 352 children (197 boys, 155 girls) with suspected dysphagia who were referred for VFSS were reviewed retrospectively. Clinical characteristics and VFSS findings were analyzed using univariate and multivariate analyses. Results Almost half of the subjects (n=175, 49...

  7. Early Period Results and Clinical Characteristics of Upper Gastrointestinal Endoscopy in Sivrihisar State Hospital

    OpenAIRE

    Ozgur Turk

    2014-01-01

    Aim: Our aim was to identify the characteristics of the patient that performed upper gastrointestinal endoscopy in a new established endoscopy unit of a state hospital. We want to present the spectrum of gastrointestinal diseases in our hospitals region. Material and Method: We analyzed patients upper endoscopy results according to age, sex, complaints, clinical characteristics, type of anesthesia, and the necessity of biopsy. We reviewed 256 patients data between 2013 December-2014 July. All...

  8. Clinical and immunogenetic characteristics of psoriatic arthritis: a single-center experience from South India

    OpenAIRE

    CB Mithun; Paul T Antony; Christina M Mariaselvam; Vir S Negi

    2013-01-01

    AimThe aim of this study was to determine the clinical characteristics and prevalence of HLA B27 in patients with psoriatic arthritis presenting to a tertiary care centre in South India. BackgroundAlthough the prevalence of psoriasis is high in India, there is paucity of data, especially on Ps A. Materials and methodsThis retrospective study included 141 patients satisfying the ClASsification criteria for Ps A (CASPAR). Demographic, clinical, and laboratory data of the patients were collected...

  9. Degeneration of Leiomyoma in Patients Referred for Uterine Fibroid Embolization: Incidence, Imaging Features and Clinical Characteristics

    OpenAIRE

    Han, Seung Chul; Kim, Man-Deuk; Jung, Dae Chul; Lee, Myungsu; Lee, Mu Sook; Park, Sung Il; Won, Jong Yun; Lee, Do Yun; Lee, Kwang Hun

    2012-01-01

    Purpose Imaging features and clinical characteristics of degenerated leiomyoma in patients referred for uterine fibroid embolization (UFE) were analyzed to assess the incidence of degenerated leiomyoma. Materials and Methods Patients referred for UFE between 2008 and 2009 were retrospectively analyzed (n=276). Patients ranged in age from 27 to 51 years (mean 38.0 years). All patients underwent screening MRI with contrast enhancement. Medical histories and clinical symptoms were evaluated. Res...

  10. The Relation between Diverse Phenotypes of PCOS with Clinical Manifestations, Anthropometric Indices and Metabolic Characteristics

    OpenAIRE

    Seyedeh Hajar Shahrami; Zahra Abbasi Ranjbar; Forozan Milani; Ehsan Kezem-Nejad; Afagh Hassanzadeh Rad; Seyedeh Fatemeh Dalil Heirat

    2016-01-01

    Critical issue regarding to variation of findings based on different phenotypes led investigators to define whether they are distinct features or overlapping ones. Therefore, we aimed to investigate the association between diverse phenotypes of PCOS (Poly Cystic Ovary Syndrome) with clinical manifestations, anthropometric indices, and metabolic characteristics. This was a descriptive cross-sectional study conducted in 15-39 years old women with PCOS referred to infertility clinics in the nort...

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2007-01-15

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

  13. Clinical microbiology in the intensive care unit: Strategic and operational characteristics

    Directory of Open Access Journals (Sweden)

    Bhattacharya S

    2010-01-01

    Full Text Available Infection is a major cause of morbidity and mortality among patients admitted in intensive care units (ICUs. The application of the principles and the practice of Clinical Microbiology for ICU patients can significantly improve clinical outcome. The present article is aimed at summarising the strategic and operational characteristics of this unique field where medical microbiology attempts to venture into the domain of direct clinical care of critically ill patients. The close and strategic partnership between clinical microbiologists and intensive care specialists, which is essential for this model of patient care have been emphasized. The article includes discussions on a variety of common clinical-microbiological problems faced in the ICUs such as ventilator-associated pneumonia, blood stream infections, skin and soft tissue infection, UTI, infection control, besides antibiotic management.

  14. The incidence and clinical characteristics of gastroenteropancreatic neuroendocrine neoplasm in Chengdu City in 2010

    Institute of Scientific and Technical Information of China (English)

    郭林杰

    2013-01-01

    Objective To investigate the incidence of gastroenteropancreatic neuroendocrine neoplasm(GEP-NEN) in Chengdu City in 2010 and summarize clinical characteristics of GEP-NEN. Methods The incidence of GEP-NEN was estimated with the data in 2010 from the

  15. Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

    OpenAIRE

    Narges Karimi; Athena Sharifi; Ashraf Zarvani; Hamed Cheraghmakani

    2015-01-01

    Context: Chronic inflammatory demyelinating polyradiculopathy (CIDP) is an acquired and autoimmune neuropathy, characterized by a chronic, rapidly progressive, symmetric weakness. In children, abnormal gait is as a first symptom of muscle weakness. Evidence Acquisition: The diagnosis of CIDP is on the basis of clinical characteristics, electrodiagnostic that shows the severity of the disease, lumbar puncture and spine magnetic res...

  16. Psychiatric Diagnoses and Clinical Characteristics of Asian American Youth in Children's Services

    Science.gov (United States)

    Nguyen, Ly; Arganza, Girlyn F.; Huang, Larke N.; Liao, Qinghong; Nguyen, Hoang T.; Santiago, Rolando

    2004-01-01

    This study examined the psychiatric diagnoses and clinical characteristics of the 981 Asian American children enrolled in the first phase of the Comprehensive Community Mental Health Services for Children and Their Families Program. Asian Americans were less likely than non-Asian Americans to receive diagnoses of depression and ADHD and more…

  17. Clinical patterns and characteristics of uveitis in a tertiary center for uveitis in China

    NARCIS (Netherlands)

    Yang, P.; Zhang, Z.; Zhou, H.; Li, B.; Huang, X.; Gao, Y.; Zhu, L.; Ren, Y.; Klooster, J.; Kijlstra, A.

    2005-01-01

    Purpose: To address the clinical pattern and characteristics of uveitis in a tertiary center for uveitis in China and compare the similarity and difference in the distribution of uveitis entities between China and other countries. Methods: A retrospective study was performed on the patients with uve

  18. Enterococcus hirae-associated endocarditis outbreaks in broiler flocks : clinical and pathological characteristics and molecular epidemiology

    NARCIS (Netherlands)

    Velkers, F C; van de Graaf-Bloois, L; Wagenaar, J A; Westendorp, S T; van Bergen, M A P; Dwars, R M; Landman, W J M; Wagenaar, Jaap

    2011-01-01

    BACKGROUND: Enterococcus hirae-associated endocarditis, characterized by a peak in mortality during the second week of the grow-out, and occasionally lameness, was diagnosed at Dutch broiler farms. OBJECTIVES: Field cases were studied to increase knowledge on clinical and pathological characteristic

  19. Enterococcus hirae-associated endocarditis outbreaks in broiler flocks: clinical and pathological characteristics and molecular epidemiology.

    NARCIS (Netherlands)

    Velkers, F.C.; Graaf-Blois, Van de L.; Wagenaar, J.A.; Westendorp, S.T.; Bergen, Van M.A.P.; Dwars, R.M.; Landman, W.J.M.

    2011-01-01

    Background: Enterococcus hirae-associated endocarditis, characterized by a peak in mortality during the second week of the grow-out, and occasionally lameness, was diagnosed at Dutch broiler farms. Objectives: Field cases were studied to increase knowledge on clinical and pathological characteristic

  20. The clinical characteristics,treatment and outcome of macrolide-resistant Mycoplasma pneumoniae pneumonia in children

    Institute of Scientific and Technical Information of China (English)

    鲍芳

    2013-01-01

    Objective To investigate the drug resistance of My-coplasma pneumoniae among children with community-acquired pneumonia (CAP) ,and to explore the clinical and radiological characteristics of and the role of azithromycin in the treatment of of macrolide-resistant (MR) Mycoplasma pneumoniae pneumonia.Methods Cases of CAP in children (n=179) were prospectively enrolled in

  1. Clinical characteristic of pulse wave velocity and arterial compliance in elderly patients with diabetes mellitus

    Institute of Scientific and Technical Information of China (English)

    张红

    2013-01-01

    Objective To explore the clinical characteristics of pulse wave velocity,arterial compliance and cardiovascular risk factors in elderly patients with type 2 diabetes mellitus.Methods A total of 363 patients were selected and divided into 4 groups:diabetic group,diabetic

  2. Cerebellar Ataxia with Bilateral Vestibulopathy: Description of a Syndrome and Its Characteristic Clinical Sign

    Science.gov (United States)

    Migliaccio, Americo A.; Halmagyi, G. Michael; McGarvie, Leigh A.; Cremer, Phillip D.

    2004-01-01

    We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are…

  3. Clinical characteristics and heterogeneity in patients with ketosis-prone diabetes

    Institute of Scientific and Technical Information of China (English)

    谭惠文

    2014-01-01

    Objective To investigate the clinical characteristics,peripheral insulin sensitivity,andβ-cell function in patients with ketosis-prone diabetes(KPD).Methods Thirty-one patients with newly diagnosed ketosis-prone diabetes were admitted to West China Hospital from January2004 to December 2009.They were divided into 2 groups according to

  4. The Relationship of Anxiety to Personality and Clinical Characteristics of a Prison Population.

    Science.gov (United States)

    Richmond, Bert O.; And Others

    1985-01-01

    Administered the Clinical Analysis Questionnaire and the What I Think and Feel test to 159 male prisoners. Participants reported low levels of anxiety and an absence of debilitating personality characteristics, suggesting the absence of a criminal personality that is a separate and distinct type of personality. (JAC)

  5. Association between neurovascular contact and clinical characteristics in classical trigeminal neuralgia

    DEFF Research Database (Denmark)

    Maarbjerg, Stine; Wolfram, Frauke; Gozalov, Aydin;

    2015-01-01

    and severe NVC. METHODS: Clinical characteristics were prospectively collected from consecutive TN patients using semi-structured interviews in a cross-sectional study design. We evaluated 3.0 Tesla MRI blinded to the symptomatic side. RESULTS: We included 135 TN patients. Severe NVC was more prevalent...

  6. Clinical and Biochemical Characteristics of Growth Hormone-Secreting Pituitary Tumors

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    To investigate the difference of biochemical characteristics on gsp-positive and gsp-negative growth hormone (GH)-secreting pituitary tumors, 18 GH-secreting pituitary tumors were examined for their clinical characteristics and gsp oncogenes. All patients received the pituitary function combinative stimulating test. It was found that there were no difference in the sex, age, tumor size, course of disease and plasma basal GH levels with gsp- positive and gsp-negative patients. The plasma levels of PRL were increased in most patients (11/18), and the plasma levels of TSH in gsp-positive patients were higher than those in gsp-negative patients (P<0.05). There was no significant difference in the responses to pituitary combinative stimulating test in gsp-positive and gsp-negative patients. It was concluded that there was little difference in the clinical biochemical characteristics of gsp-positive with gsp-negative GH-secreting pituitary tumors.

  7. Clinical trial enrollment, patient characteristics, and survival differences in prospectively registered metastatic colorectal cancer patients

    DEFF Research Database (Denmark)

    Sorbye, Halfdan; Pfeiffer, Per; Cavalli-Björkman, Nina;

    2009-01-01

    oncological consideration at 3 hospitals in Scandinavia covering defined populations were registered consecutively during 2003 to 2006. Clinical trial enrollment, patient characteristics, and treatment were recorded prospectively, and the follow-up was complete. RESULTS: Palliative chemotherapy was initiated...... was then only 2.1 months. The median survival for all 760 nonresectable mCRC patients was 10.7 months. CONCLUSIONS: mCRC patients enrolled into clinical trials differ in characteristics from patients receiving chemotherapy outside protocol and have better survival, even when given the same treatment. Although......BACKGROUND: Trial accrual patterns were examined to determine whether metastatic colorectal cancer (mCRC) patients enrolled in trials are representative of a general cancer population concerning patient characteristics and survival. METHODS: A total of 760 mCRC patients referred for their first...

  8. Chronic lymphocytic leukemia and myeloproliferative neoplasms concurrently diagnosed: clinical and biological characteristics.

    Science.gov (United States)

    Todisco, Gabriele; Manshouri, Taghi; Verstovsek, Srdan; Masarova, Lucia; Pierce, Sherry A; Keating, Michael J; Estrov, Zeev

    2016-05-01

    Chronic lymphocytic leukemia (CLL) and myeloproliferative neoplasms (MPN) may occur concomitantly. However, little is known about the pathobiological characteristics and interaction between the neoplastic clones in these rare cases of coinciding malignancies. We retrospectively examined the clinical and biological characteristics of 13 patients with concomitant CLL and MPN--eight primary myelofibrosis (PMF), three essential thrombocytosis (ET), and two polycythemia vera (PV)--who presented to our institution between 1998 and 2014, and tested all patients for MPN-specific aberrations, such as JAK2, MPL and CALR mutations. Along with epidemiological and molecular characterization of this rare condition, we found that JAK2 mutation can be detected 9 years prior to PMF diagnosis, suggesting that PMF clinical phenotype may require several years to develop and CLL/MPN clinical co-occurrence might be sustained by common molecular events. Some features of these patients suggest that pathobiologies of these diseases might be intertwined. PMID:26402369

  9. Restless legs syndrome in Parkinson's disease: clinical characteristics and biochemical correlations

    Directory of Open Access Journals (Sweden)

    Tiago Machado Guerreiro

    2010-12-01

    Full Text Available Restless legs syndrome (RLS is a neurological disorder that responds to dopaminergic drugs, indicating a common pathophysiology with Parkinson's disease (PD. The prevalence of RLS was estimated in a group of PD patients and its clinical and biochemical characteristics were analysed. Forty-eight patients with PD were evaluated into two groups, with and without RLS. Clinical characteristics assessed in both groups were age, gender, duration of PD, Hoehn and Yahr, and Schwab and England scales. Laboratory variables such as hemoglobin, s-iron, s-ferritin and creatinine were obtained. The prevalence of RLS was 18.75%. No significant differences regarding clinical variables and biochemical parameters were observed. The high prevalence of RLS found in PD patients suggests the concept of a common etiological link and it seems that secondary causes did not play a central role in the pathophysiology of RLS in this group of parkinsonian patients.

  10. Can Psychological, Social and Demographical Factors Predict Clinical Characteristics Symptomatology of Bipolar Affective Disorder and Schizophrenia?

    Science.gov (United States)

    Maciukiewicz, Malgorzata; Pawlak, Joanna; Kapelski, Pawel; Łabędzka, Magdalena; Skibinska, Maria; Zaremba, Dorota; Leszczynska-Rodziewicz, Anna; Dmitrzak-Weglarz, Monika; Hauser, Joanna

    2016-09-01

    Schizophrenia (SCH) is a complex, psychiatric disorder affecting 1 % of population. Its clinical phenotype is heterogeneous with delusions, hallucinations, depression, disorganized behaviour and negative symptoms. Bipolar affective disorder (BD) refers to periodic changes in mood and activity from depression to mania. It affects 0.5-1.5 % of population. Two types of disorder (type I and type II) are distinguished by severity of mania episodes. In our analysis, we aimed to check if clinical and demographical characteristics of the sample are predictors of symptom dimensions occurrence in BD and SCH cases. We included total sample of 443 bipolar and 439 schizophrenia patients. Diagnosis was based on DSM-IV criteria using Structured Clinical Interview for DSM-IV. We applied regression models to analyse associations between clinical and demographical traits from OPCRIT and symptom dimensions. We used previously computed dimensions of schizophrenia and bipolar affective disorder as quantitative traits for regression models. Male gender seemed protective factor for depression dimension in schizophrenia and bipolar disorder sample. Presence of definite psychosocial stressor prior disease seemed risk factor for depressive and suicidal domain in BD and SCH. OPCRIT items describing premorbid functioning seemed related with depression, positive and disorganised dimensions in schizophrenia and psychotic in BD. We proved clinical and demographical characteristics of the sample are predictors of symptom dimensions of schizophrenia and bipolar disorder. We also saw relation between clinical dimensions and course of disorder and impairment during disorder. PMID:26646576

  11. Septic pulmonary embolism caused by a Klebsiella pneumoniae liver abscess: clinical characteristics, imaging findings, and clinical courses

    Directory of Open Access Journals (Sweden)

    Deng-Wei Chou

    2015-06-01

    Full Text Available OBJECTIVES: Septic pulmonary embolism caused by a Klebsiella (K. pneumoniae liver abscess is rare but can cause considerable morbidity and mortality. However, clinical information regarding this condition is limited. This study was conducted to elucidate the full disease spectrum to improve its diagnosis and treatment. METHOD: We reviewed the clinical characteristics, imaging findings, and clinical courses of 14 patients diagnosed with septic pulmonary embolism caused by a K. pneumoniae liver abscess over a period of 9 years. RESULTS: The two most prevalent symptoms were fever and shortness of breath. Computed tomography findings included a feeding vessel sign (79%, nodules with or without cavities (79%, pleural effusions (71%, peripheral wedge-shaped opacities (64%, patchy ground-glass opacities (50%, air bronchograms within a nodule (36%, consolidations (21%, halo signs (14%, and lung abscesses (14%. Nine (64% of the patients developed severe complications and required intensive care. According to follow-up chest radiography, the infiltrates and consolidations were resolved within two weeks, and the nodular opacities were resolved within one month. Two (14% patients died of septic shock; one patient had metastatic meningitis, and the other had metastatic pericarditis. CONCLUSION: The clinical presentations ranged from insidious illness with fever and respiratory symptoms to respiratory failure and septic shock. A broad spectrum of imaging findings, ranging from nodules to multiple consolidations, was detected. Septic pulmonary embolism caused by a K. pneumoniae liver abscess combined with the metastatic infection of other vital organs confers a poor prognosis.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2003-04-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1986-03-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

    Baltsavias, G; Valavanis, A

    2010-06-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1993-02-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2002-07-01

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

  1. 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.

  2. Etiology of aseptic meningitis and clinical characteristics in immune-competent adults.

    Science.gov (United States)

    Han, Su-Hyun; Choi, Hye-Yeon; Kim, Jeong-Min; Park, Kwang-Ryul; Youn, Young Chul; Shin, Hae-Won

    2016-01-01

    Viral meningitis is the most common cause of aseptic meningitis. Use of the polymerase chain reaction (PCR) has increased the ability to determine the etiology of viral meningitis. This study used PCR analysis to evaluate the etiology of aseptic meningitis in 177 previously healthy adults over a 5-year period, as well as analyzing the clinical characteristics, cerebrospinal fluid (CSF) findings, and prognosis according to each etiology. The most frequent cause of aseptic meningitis was enterovirus (EV), followed by varicella zoster virus (VZV). Patients with EV meningitis were significantly younger than those with VZV meningitis. The percentage of lymphocytes in white blood cell counts and protein concentrations in the CSF differed significantly among patients with EV, VZV and meningitis of undetermined etiology. Younger age and lower percentage of lymphocyte and protein level in CSF analysis may be suggestive of EV meningitis. Further prospective studies are warranted to identify the correlations between the clinical characteristics and the etiologies of meningitis.

  3. [Comments relating to the psychosexual characteristics of the male transsexual. A clinical study].

    Science.gov (United States)

    Fulcheri, M; Bertone, E; Barzega, G

    1995-03-01

    In this paper the authors analyse transexualism from a psychopathological, nosographic and clinical point of view. The re-examine the theories on the subjects elaborated by a number of authors and describe the clinical characteristics of the syndrome, paying special attention to problems relating to differential diagnosis. Moreover, they present the results ofa study carried out in a personal series of cases. The sample studied included 11 male transsexual subjects. The latter were evaluated through psychiatric interview and a questionnaire aimed at obtaining information on the following topics: the subjects' social and affective relations; their childhood experience; their perception and acceptance of themselves; the meaning, characteristics, localisation and experience of sexual pleasure. The results are discussed and compared with those of national and international literature on this subjects. PMID:7643729

  4. Self-perceived health and clinical characteristics in young adult students from the brazilian northeast

    Directory of Open Access Journals (Sweden)

    Thereza Maria Magalhães Moreira

    2014-10-01

    Full Text Available Objective To analyze the association between socioeconomic situation, clinical characteristics referred and the family history of cardiovascular disease, with the Self-perceived health of young adults education and their implications for clinical characteristics observed. Method Analytical study conducted with 501 young adults who are students in countryside city in the Brazilian Northeast. We used binary logistic regression. Results The final model explained 83.3% of the self-perceived positive health, confirming the association of Self-perceived health with male, residence in the community, have excellent/very good lifestyle and does not have or do not know that there are cases of stroke in the family. Conclusion Health perception was often optimistic, being important to identify devices to be worked closer to their perception of their actual health condition, increasing the effectiveness of health promotion activities undertaken by professionals.

  5. The influence of job characteristics on job outcomes of pharmacists in hospital, clinic, and community pharmacies.

    Science.gov (United States)

    Lin, Blossom Yen-Ju; Yeh, Ying-Chen; Lin, Wen-Hung

    2007-06-01

    This study examines the relationship between job characteristics and job outcomes of pharmacists in hospital, clinic, and community pharmacies in Taiwan. The structured questionnaires covered the items of job characteristics, job outcomes, and demographics of pharmacists, and were distributed between Feb 2004 and April 2004. Two hundred and ninety-eight pharmacists responded. Data were analyzed descriptively, and univariate analyses, factor analysis, and multiple regression analyses were used. It found the more enriched the job, the greater the job satisfaction and less intention to leave. And community pharmacists reported greater job enrichment and job satisfaction and less intention to leave than did hospital and clinic pharmacists. It suggests pharmacy managers could recognize the needs of pharmacists to redesign and enrich their work arrangements. PMID:17622026

  6. Clinical and Demographical Characteristics of Patients with Medication Overuse Headache in Argentina and Chile

    DEFF Research Database (Denmark)

    Shand, Beatriz; Goicochea, Maria Teresa; Valenzuela, Raul;

    2015-01-01

    BACKGROUND: Data on the characteristics of Medication Overuse Headache (MOH) in Latin American (LA) are scarce. Here we report the demographic and clinical features of the MOH patients from Argentina and Chile enrolled in the multinational COMOESTAS project in the period 2008-2010. METHODS: The LA...... population was formed by 240 MOH subjects, 110 from Chile and 130 from Argentina, consecutively attending the local headache centres. In each centre, specifically trained neurologist interviewed and confirmed the diagnosis according to the ICHD-II criteria. A detailed history was collected on an electronic......, where it affects mostly women, in the most active decades of life. Some differences emerge as regards the demographic and clinical characteristics of MOH in this population as compared to Europe or Northern America. What seems more worrying about MOH in Argentina and Chile is that most patients overuse...

  7. The Structure of Pathological Gambling among Korean Gamblers: A Cluster and Factor Analysis of Clinical and Demographic Characteristics

    Science.gov (United States)

    Lee, Tae Kyung; LaBrie, Richard A.; Grant, Jon E.; Kim, Suck Won; Shaffer, Howard J.

    2008-01-01

    This paper reports the underlying structure of the demographic and clinical characteristics of level 3 (i.e., pathological) Korean casino gamblers. The participants reported their gambling behavior and clinical characteristics known to be associated with gambling problems (e.g., alcohol use problems, eating disorders, depression, anxiety, and…

  8. The clinical characteristics and prognostic analysis of gastric mucosa-associated lymphoid tissue lymphoma of 103 cases

    Institute of Scientific and Technical Information of China (English)

    李晓武

    2012-01-01

    Objective To analyze the clinical characteristics and prognosis of the patients with gastric mucosa-associated lymphoid tissue(MALT) lymphoma. Methods The clinical characteristics and prognostic factors of 103 gastric MALT lymphoma patients admitted to our hospital from April 2001 to August 2011 were

  9. Clinical Characteristics and Outcomes for Solitary Fibrous Tumor (SFT): A Single Center Experience

    OpenAIRE

    DeVito, Nicholas; Henderson, Evita; Han, Gang; Reed, Damon; Marilyn M Bui; Lavey, Robert; Robinson, Lary; Zager, Jonathan S.; Gonzalez, Ricardo J; Sondak, Vernon K.; Letson, G. Douglas; Conley, Anthony

    2015-01-01

    Solitary fibrous tumor (SFT) is a mesenchymal neoplasm of fibrous origin. The 2013 WHO classification of soft tissue tumors defines malignant forms as hypercellular, mitotically active (>4 mitosis/10 high-power fields), with cytological atypia, tumor necrosis, and/or infiltrative margins. With an IRB-approved protocol, we investigated patient records and clinicopathologic data from our Sarcoma Database to describe the clinical characteristics of both benign and malignant SFT. All pathology sp...

  10. Clinical Characteristics of Children and Adolescents with a Primary Tic Disorder

    OpenAIRE

    Specht, Matt W.; Woods, Douglas W; Piacentini, John; Scahill, Lawrence; Wilhelm, Sabine; Peterson, Alan L.; Chang, Susanna; Kepley, Hayden; Deckersbach, Thilo; Flessner, Christopher; Brian A. Buzzella; McGuire, Joseph F.; Levi-Pearl, Sue; Walkup, John T.

    2011-01-01

    The clinical characteristics and rates of co-occurring psychiatric conditions in youth seeking treatment for a chronic tic disorder (CTD) were examined. Children and adolescents (N = 126) with a primary CTD diagnosis were recruited for a randomized controlled treatment trial. An expert clinician established diagnostic status via semi-structured interview. Participants were male (78.6%), Caucasians (84.9%), mean age 11.7 years (SD = 2.3) with moderate-to-severe tics who met criteria for Touret...

  11. Leber's hereditary optic neuropathy with late disease onset: clinical and molecular characteristics of 20 patients

    OpenAIRE

    Dimitriadis, Konstantin; Leonhardt, Miriam; Yu-Wai-Man, Patrick; Kirkman, Matthew Anthony; Korsten, Alex; De Coo, Irenaeus F; Chinnery, Patrick Francis; Klopstock, Thomas

    2014-01-01

    Background: Leber's hereditary optic neuropathy (LHON) is a mitochondrial disease that typically causes bilateral blindness in young men. Here we describe the clinical and molecular characteristics of 20 patients with disease onset after the age of 50 years (late onset-LHON). Methods: From a cohort of 251 affected and 277 unaffected LHON carriers, we identified 20 patients with onset of visual loss after the age of 50 years. Using structured questionnaires, data including basic demographic de...

  12. Clinical and microbiologic characteristics of tcdA-negative variant clostridium difficile infections

    Directory of Open Access Journals (Sweden)

    Kim Jieun

    2012-05-01

    Full Text Available Abstract Background The tcdA-negative variant (A-B+ of Clostridium difficile is prevalent in East Asian countries. However, the risk factors and clinical characteristics of A-B+C. difficile infections (CDI are not clearly documented. The objective of this study was to investigate these characteristics. Methods From September 2008 through January 2010, the clinical characteristics, medication history and treatment outcomes of CDI patients were recorded prospectively. Toxin characterization and antibiotic susceptibility tests were performed on stool isolates of C. difficile. Results During the study period, we identified 22 cases of CDI caused by tcdA-negative tcdB-positive (A-B+ strains and 105 cases caused by tcdA-positive tcdB-positive (A+B+ strains. There was no significant difference in disease severity or clinical characteristics between the two groups. Previous use of clindamycin and young age were identified as significant risk factors for the acquisition of A-B+ CDI (OR = 4.738, 95% CI 1.48–15.157, p = 0.009 and OR = 0.966, 95% CI 0.935–0.998, p = 0.038, respectively in logistic regression. Rates of resistance to clindamycin were 100% and 69.6% in the A-B+ and A+B+ isolates, respectively (p = 0.006, and the ermB gene was identified in 17 of 21 A-B+ isolates (81%. Resistance to moxifloxacin was also more frequent in the A-B+ than in the A+B+ isolates (95.2% vs. 63.7%, p = 0.004. Conclusions The clinical course of A-B+ CDI is not different from that of A+B+ CDI. Clindamycin use is a significant risk factor for the acquisition of tcdA-negative variant strains.

  13. Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis

    DEFF Research Database (Denmark)

    Denton, Christopher P; Krieg, Thomas; Guillevin, Loic;

    2012-01-01

    OBJECTIVES: The Digital Ulcers Outcome (DUO) Registry was designed to describe the clinical and antibody characteristics, disease course and outcomes of patients with digital ulcers associated with systemic sclerosis (SSc). METHODS: The DUO Registry is a European, prospective, multicentre......, observational, registry of SSc patients with ongoing digital ulcer disease, irrespective of treatment regimen. Data collected included demographics, SSc duration, SSc subset, internal organ manifestations, autoantibodies, previous and ongoing interventions and complications related to digital ulcers. RESULTS...

  14. Clinical Characteristics and Features of Frequent Idiopathic Ventricular Premature Complexes in the Korean Population

    OpenAIRE

    Hwang, Jin Kyung; Park, Seung-Jung; On, Young Keun; Kim, June Soo; Park, Kyoung-Min

    2015-01-01

    Background and Objectives Frequent ventricular premature complexes (VPCs) increase the risk of cardiomyopathy (CMP). However, most data regarding VPCs have been obtained from Western population and in-hospital patient-based studies. The objective of this study was to define the clinical characteristics and features of idiopathic VPCs in the Korean population. Subjects and Methods We investigated subjects undergoing transthoracic echocardiography and documented VPC burdens >1% by Holter monito...

  15. Prevalence and Clinical Characteristics Associated with Pulmonary Hypertension in African-Americans

    OpenAIRE

    Choudhary, Gaurav; Jankowich, Matthew; Wu, Wen-Chih

    2013-01-01

    Background Pulmonary hypertension (PH) is associated with increased mortality and morbidity. It is frequently associated with cardiopulmonary diseases that are prevalent in African Americans (AAs). However, the prevalence or determinants of PH in the AA population is not known. Methods We conducted a cross-sectional study to estimate the prevalence of PH (defined as trans-tricuspid gradient ≥ 35 mm Hg) and associated clinical characteristics in AAs using the Jackson Heart Study cohort (n=3,28...

  16. Clinical characteristics of vitamin D deficiency rickets in infants and preschool children

    OpenAIRE

    Kyoung Huh; Mi Kyeong Woo; Jung Rim Yoon; Gyu Hong Shin; Myoung Jae Chey; Mi Jung Park

    2010-01-01

    Purpose : Vitamin D deficiency rickets is a significant public health problem that results from insufficient exposure to sunlight and inadequate vitamin D supplementation. The purpose of this study is to identify the clinical characteristics of vitamin D deficiency rickets in infants. Methods : Data of 35 infants diagnosed as vitamin D deficiency rickets at Sanggye-Paik Hospital, Seoul, Korea, from March 2007 to May 2009 were reviewed. Children with plasma 25-hydroxyvitamin D levels&&#...

  17. PSA testing without clinical indication for prostate cancer in relation to socio-demographic and clinical characteristics in the Danish Diet, Cancer and Health Study

    DEFF Research Database (Denmark)

    Karlsen, Randi V; Larsen, Signe B; Christensen, Jane;

    2013-01-01

    associations between socio-demographic or clinical characteristics and PSA testing without clinical indication. Material and methods. In the Danish Diet, Cancer and Health Cohort, we identified 1051 men with PC diagnosed in 1993-2008. Diagnostic and clinical characteristics were obtained from medical records......, and socio-demographic information was retrieved from administrative registers. We used general logistic regression analysis to estimate odds ratios (ORs) and 95% confidence intervals (CIs) for associations between socio-demographic or clinical characteristics and PSA testing without clinical indication. Cox......Background. Social differences in prostate cancer (PC) incidence and mortality might be related to testing for prostate-specific antigen (PSA). Although routine PSA screening is not recommended in Denmark, testing without clinical indication increased during the past decade. We evaluated...

  18. Intraductal Oncocytic Papillary Neoplasm Having Clinical Characteristics of Mucinous Cystic Neoplasm and a Benign Histology

    Directory of Open Access Journals (Sweden)

    Takatomi Oku

    2007-03-01

    Full Text Available Context An intraductal oncocytic papillary neoplasm is a rare pancreatic tumor which was first described by Adsay et al. in 1996. It has been defined as a new subgroup of IPMN. Case report We report the case of a 76-year-old woman who presented with nausea. Imaging studies revealed a cystic mass in the body of the pancreas. She underwent a successful distal pancreatectomy and splenectomy, and has subsequently remained well. Microscopically, the cyst was lined by columnar epithelium similar to pancreatic duct epithelium, and the nodular projection consisted of arborizing papillary structures, lined by plump cells with abundant eosinophilic cytoplasm. These eosinophilic cells were immunohistochemically positively stained with anti-mitochondrial antibody. The cellular atypism was mild and the proliferating index was low, compatible with adenoma of an intraductal oncocytic papillary neoplasm. Although no ovarian type stroma was identified, in our case, no communication to main pancreatic duct (located in the pancreatic body and rapid growth by intracystic hemorrhage were clinical characteristics of a mucinous cystic neoplasm, but not IPMN. Conclusion With only 17 cases reported to date, the clinical and pathological details of an intraductal oncocytic papillary neoplasm are still unclear. We herein add one case with different characteristics from those of the past reports. To our knowledge, this is the first case report of an intraductal oncocytic papillary neoplasm with the clinical characteristics of a mucinous cystic neoplasm.

  19. Reporting of research quality characteristics of studies published in 6 major clinical dental specialty journals.

    Science.gov (United States)

    Pandis, Nikolaos; Polychronopoulou, Argy; Madianos, Phoebus; Makou, Margarita; Eliades, Theodore

    2011-06-01

    The objective of this article was to record reporting characteristics related to study quality of research published in major specialty dental journals with the highest impact factor (Journal of Endodontics, Journal of Oral and Maxillofacial Surgery, American Journal of Orthodontics and Dentofacial Orthopedics; Pediatric Dentistry, Journal of Clinical Periodontology, and International Journal of Prosthetic Dentistry). The included articles were classified into the following 3 broad subject categories: (1) cross-sectional (snap-shot), (2) observational, and (3) interventional. Multinomial logistic regression was conducted for effect estimation using the journal as the response and randomization, sample calculation, confounding discussed, multivariate analysis, effect measurement, and confidence intervals as the explanatory variables. The results showed that cross-sectional studies were the dominant design (55%), whereas observational investigations accounted for 13%, and interventions/clinical trials for 32%. Reporting on quality characteristics was low for all variables: random allocation (15%), sample size calculation (7%), confounding issues/possible confounders (38%), effect measurements (16%), and multivariate analysis (21%). Eighty-four percent of the published articles reported a statistically significant main finding and only 13% presented confidence intervals. The Journal of Clinical Periodontology showed the highest probability of including quality characteristics in reporting results among all dental journals. PMID:21605830

  20. The clinical characteristics of twenty-eight idiopathic pulmonary fibrosis cases

    International Nuclear Information System (INIS)

    Objective: To summarize the clinical characteristics of idiopathic pulmonary fibrosis (IPF). Methods: The clinical characteristics of twenty-eight cases diagnosed as IPF between 1991 and 2006 were studied retrospectively. Results: Most IPF patients had an insidious onset of progressive dyspnea and non-productive cough. Inspiratory crackles and finger clubbing were also noted in most patients. The most impressive appearance of their radiography was peripheral reticular and nodular opacities, distributed largely at the lung bases. Pulmonary function test showed restrictive impairment and impaired oxygen diffusion. The arterial blood gas analysis revealed type I respiratory failure. One IPF case was complicated with lung cancer. The symptoms of fifteen cases(71.4%) were relieved under the therapy with glucocorticoid. Seven patients died as yet in our group and the middle duration was 24 months. Conclusions: The diagnosis of IPF relies mostly on the clinical characteristics, radiography, pulmonary function test, blood gas analysis and exclusion of other ILD. Atypical cases need lung biopsy to do further the diagnosis. Therapy with glucocorticoid may be effective in some cases. Prognosis in IPF cases complicated with lung cancer is poor. (authors)

  1. 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.

  2. Trigeminal neuralgia--a prospective systematic study of clinical characteristics in 158 patients

    DEFF Research Database (Denmark)

    Maarbjerg, Stine; Gozalov, Aydin; Olesen, Jes;

    2014-01-01

     = .043. It affected solely the second and/or third trigeminal branch in 109 (69%) while the first branch alone was affected in only 7 (4%). Notably, 78 (49%) had concomitant persistent pain in addition to paroxysmal stabbing pain. Autonomic symptoms were present in 48 (31%). Patients who had...... not undergone surgery for TN had sensory abnormalities in 35 (29%). CONCLUSIONS: This, the first study in a series of papers focusing on the clinical, radiological, and etiological aspects of TN, revealed that the symptomatology of TN includes a high percentage of concomitant persistent pain, autonomic symptoms......, and sensory abnormalities. These findings offer new insights to the prevailing clinical impression of the clinical characteristics in TN....

  3. Idiopathic Inflammatory Myopathies; Association with Overlap Myositis and Syndromes: Classification, Clinical Characteristics, and Associated Autoantibodies

    Directory of Open Access Journals (Sweden)

    Pari Basharat

    2016-07-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are traditionally identified as a group of disorders that target skeletal muscle due to autoimmune dysfunction. The IIM can be divided into subtypes based on certain clinical characteristics, and several classification schemes have been proposed. The predominant diagnostic criteria for IIM is the Bohan and Peter criteria, which subdivides IIM into primary polymyositis (PM, primary dermatomyositis (DM, myositis with another connective tissue disease, and myositis associated with cancer. However, this measure has been criticised for several reasons including lack of specific criteria to help distinguish between muscle biopsy findings of PM, DM, and immune-mediated necrotising myopathy, as well as the lack of identification of cases of overlap myositis (OM. Because of this issue, other classification criteria for IIM have been proposed, which include utilising myositis-associated antibodies and myositis-specific antibodies, as well as overlap features such as Raynaud’s phenomenon, polyarthritis, oesophageal abnormalities, interstitial lung disease, small bowel abnormalities such as hypomotility and malabsorption, and renal crises, amongst others. Indeed, the identification of autoantibodies associated with certain clinical phenotypes of myositis, in particular connective tissue disease-myositis overlap, has further helped divide IIM into distinct clinical subsets, which include OM and overlap syndromes (OS. This paper reviews the concepts of OM and OS as they pertain to IIM, including definitions in the literature, clinical characteristics, and overlap autoantibodies.

  4. Behçet’s disease: The clinical and demographic characteristics of 182 patients

    Directory of Open Access Journals (Sweden)

    Ercan Karabacak

    2014-09-01

    Full Text Available Background and Design: Behçet’s disease (BD is a multisystem inflammatory disorder that is an important cause of morbidity worldwide. In this study, we aimed to investigate the clinical and demographic characteristics of Turkish patients diagnosed with BD. Materials and Methods: We retrospectively evaluated the hospital records of patients with BD who attended the Dermatoveneorology Department at the GATA Haydarpaşa Teaching and Research Hospital between 2001 and 2012. One hundred eighty-two male patients who met the diagnostic criteria of the International Study Group for BD were included in the study. The clinical and demographic characteristics, including symptoms and age at onset, systemic symptoms, duration of the disease and initial signs were recorded. Results: The ages of the 182 male patients included in the study varied from 20 to 53. The mean age at disease onset was 20.59±4.55 years. The leading clinical features were aphthous ulcers (100% followed by genital ulcers (86.8% and papulopustular lesions (80.3%. The pathergy test was positive in 57.3% of the patients. Ocular involvement was present in 527%, vascular involvement in 22.5%, and neurological manifestations was found in 7.1% of the patients. Joint involvement was reported in 18.1% of the subjects. Only 0.5% had genitourinary involvement. There was no relationship of BD with the age at onset and disease duration. Conclusion: We assume that the present study will contribute to the data on demographic and clinical characteristics of especially male BD patients in Turkey

  5. Clinical characteristics associated with Borrelia burgdorferi sensu lato skin culture results in patients with erythema migrans.

    Directory of Open Access Journals (Sweden)

    Franc Strle

    Full Text Available Clinical characteristics associated with isolation of Borrelia burgdorferi sensu lato from skin have not been fully evaluated. To gain insight into predictors for a positive EM skin culture, we compared basic demographic, epidemiologic, and clinical data in 608 culture-proven and 501 culture-negative adult patients with solitary EM. A positive Borrelia spp. skin culture was associated with older age, a time interval of >2 days between tick bite and onset of the skin lesion, EM ≥ 5 cm in diameter, and location of the lesion on the extremities, whereas several other characteristics used as clinical case definition criteria for the diagnosis of EM (such as tick bite at the site of later EM, information on expansion of the skin lesion, central clearing were not. A patient with a 15-cm EM lesion had almost 3-fold greater odds for a positive skin culture than patients with a 5-cm lesion. Patients with a free time interval between the tick bite and onset of EM had the same probability of a positive skin culture as those who did not recall a tick bite (OR=1.02; however, the two groups had >3-fold greater odds for EM positivity than patients who reported a tick bite with no interval between the bite and onset of the lesion. In conclusion, several yet not all clinical characteristics used in EM case definitions were associated with positive Borrelia spp. skin culture. The findings are limited to European patients with solitary EM caused predominantly by B. afzelii but may not be valid for other situations.

  6. [Characteristics of children and adolescents with gender dysphoria referred to the Hamburg Gender Identity Clinic].

    Science.gov (United States)

    Becker, Inga; Gjergji-Lama, Voltisa; Romer, Georg; Möller, Birgit

    2014-01-01

    Given the increasing demand for counselling in gender dysphoria in childhood in Germany, there is a definite need for empirical data on characteristics and developmental trajectories of this clinical group. This study aimed to provide a first overview by assessing demographic characteristics and developmental trajectories of a group of gender variant boys and girls referred to the specialised Gender Identity Clinic in Hamburg. Data were extracted from medical charts, transcribed and analysed using qualitative content analysis methods. Categories were set up by inductive-deductive reasoning based on the patients' parents' and clinicians' information in the files. Between 2006 and 2010, 45 gender variant children and adolescents were seen by clinicians; 88.9% (n = 40) of these were diagnosed with gender identity disorder (ICD-10). Within this group, the referral rates for girls were higher than for boys (1:1.5). Gender dysphoric girls were on average older than the boys and a higher percentage of girls was referred to the clinic at the beginning of adolescence (> 12 years of age). At the same time, more girls reported an early onset age. More girls made statements about their (same-sex) sexual orientation during adolescence and wishes for gender confirming medical interventions. More girls than boys revealed self-mutilation in the past or present as well as suicidal thoughts and/or attempts. Results indicate that the presentation of clinically referred gender dysphoric girls differs from the characteristics boys present in Germany; especially with respect to the most salient age differences. Therefore, these two groups require different awareness and individual treatment approaches. PMID:25296510

  7. [Clinical characteristics of histoplamosis in 8 patients: case report and literature review].

    Science.gov (United States)

    Zhou, Li; Fan, Songqing; Liang, Qingchun; Peng, Yating; Zong, Dandan; Ouyang, Ruoyun

    2016-06-28

    To explore the clinical characteristics, imaging manifestation, diagnosis and treatment for histoplasmosis and to improve therapeutic level, we retrospectively analyzed the clinical data of 8 patients with biopsy-confirmed histoplasmosis from 2004 to 2014 in the Second Xiangya Hospital of Central South University and reviewed relevant literatures. The main clinical symptoms of histoplasmosis included fever, cough, expectoration, chest pain, blood-stained sputum, lymphadenectasis, etc. The major lung imaging features were mass, node or pneumonia-like performance. No case was diagnosed as histoplasimosis firstly. Four patients whose imaging manifestations were focal pulmonary lesion received lobectomy of lung lesions or wedge resection. Clinical and imaging manifestations in 3 patients, who treated with amphotericin B or its liposomal, itraconazole or fluconazole, were improved. The clinical symptoms and imaging findings of histoplasmosis are nonspecific. It is easy for the physicians to misdiagnose histoplasmosis as bacterial infection, lung cancer, tuberculosis lymphoma, etc. Therefore, it is significant and necessary to carry out multiple biopsies combined with multiple etiological examinations for patients with difficult diagnosis.

  8. Chronic pain disorders in HIV primary care: clinical characteristics and association with healthcare utilization.

    Science.gov (United States)

    Jiao, Jocelyn M; So, Eric; Jebakumar, Jebakaran; George, Mary Catherine; Simpson, David M; Robinson-Papp, Jessica

    2016-04-01

    Chronic pain is common in HIV, but incompletely characterized, including its underlying etiologies, its effect on healthcare utilization, and the characteristics of affected patients in the HIV primary care setting. These data are needed to design and justify appropriate clinic-based pain management services. Using a clinical data warehouse, we analyzed one year of data from 638 patients receiving standard-of-care antiretroviral therapy in a large primary care HIV clinic, located in the Harlem neighborhood of New York City. We found that 40% of patients carried one or more chronic pain diagnoses. The most common diagnoses were degenerative musculoskeletal disorders (eg, degenerative spinal disease and osteoarthritis), followed by neuropathic pain and headache disorders. Many patients (16%) had multiple chronic pain diagnoses. Women, older patients, and patients with greater burdens of medical illness, and psychiatric and substance use comorbidities were disproportionately represented among those with chronic pain diagnoses. Controlling for overall health status, HIV patients with chronic pain had greater healthcare utilization including emergency department visits and radiology procedures. In summary, our study demonstrates the high prevalence of chronic pain disorders in the primary care HIV clinic. Colocated interventions for chronic pain in this setting should not only focus on musculoskeletal pain but also account for complex multifaceted pain syndromes, and address the unique biopsychosocial features of this population. Furthermore, because chronic pain is prevalent in HIV and associated with increased healthcare utilization, developing clinic-based pain management programs could be cost-effective.

  9. Clinical Characteristics, Radiological Features and Gene Mutation in 10 Chinese Families with Spinocerebellar Ataxias

    Institute of Scientific and Technical Information of China (English)

    Jian-Wen Chen; Li Zhao; Feng Zhang; Lan Li; Yu-Hang Gu; Jing-Yuan Zhou; Hui Zhang

    2015-01-01

    Background:Spinocerebellar ataxias (SCAs) are a group ofneurodegenerative disorders that primarily cause the degeneration in the cerebellum,spinal cord,and brainstem.We study the clinical characteristics,radiological features and gene mutation in Chinese families with SCAs.Methods:In this study,we investigated 10 SCAs Chinese families with SCA1,SCA3/Machado-Joseph disease (MJD),SCA7,SCAB.There were 27 people who were genetically diagnosed as SCA,of which 21 people showed clinical symptoms,and 6 people had no clinical phenotype that we called them presymptomatic patients.In addition,3 people with cerebellar ataxia and cataracts were diagnosed according to the Harding diagnostic criteria but failed to be recognized as SCAs on genetic testing.Clinical characteristic analyses of each type of SCAs and radiological examinations were performed.Results:We found that SCA3/MJD was the most common subtype in Han population in China,and the ratio of the pontine tegmentum and the posterior fossa area was negatively correlated with the number of cytosine-adenine-guanine (CAG) repeats;the disease duration was positively correlated with the International Cooperative Ataxia Rating Scale score;and the CAG repeats number of abnormal alleles was negatively correlated with the age of onset.Conclusions:Collectively our study is a systematic research on SCAs in China,which may help for the clinical diagnosis and prenatal screening of this disease,and it may also aid toward better understanding of this disease.

  10. Clinical characteristics, diagnosis and management of respiratory distress syndrome in full-term neonates

    Institute of Scientific and Technical Information of China (English)

    FENG Zhi-chun; SHI Yun; DONG Jian-ying; ZHENG Tian; LI Jing-ya; LU Li-li; LIU Jing-jing; LIANG Jing; ZHANG Hao

    2010-01-01

    Background Respiratory distress syndrome (RDS) is one of the most common causes of neonatal respiratory failure and neonatal death, however, its clinical characteristics are very different from premature RDS, and these characteristics have not been well documented as yet. This study was to investigate the pathogenesis, clinical characteristics and management strategies of RDS in full-term neonates, with the aim of developing a working protocol for improving the outcome in full-term neonates with RDS.Methods A total of 125 full-term infants with RDS were enrolled in this study. Their clinical and laboratory data were collected for analyzing the characteristics of full-term neonatal RDS.Results (1) The 125 cases included 94 male and 31 female infants, vaginal delivery occurred in 80 cases and cesarean section in 45 cases. (2) The onset time of RDS was (3.11±3.59) hours after birth. (3)The possible reasons included severe perinatal infections in 63 patients, elective cesarean section in 34 cases, severe birth asphyxia in 12 patients,meconium aspiration syndrome in 9 patients, pulmonary hemorrhage in 4 patients and matemal diabetes in 3 patients. (4)Complications included multiple organ system failure (MOSF) in 49 patients, persistent pulmonary hypertension of newborn (PPHN) in 25 patients, acute renal failure in 18 patients, severe hyperkalemia in 25 patients, severe metabolic acidosis in 6 cases, severe myocardial injury in 9 cases, pulmonary hemorrhage in 3 cases, disseminated intravascular coagulation in 14 patients and shock in 12 patients.(5) Four patients died, the mortality was therefore 3.2% with the main cause of septicemia complicating of MOSF, but their prognosis was improved while comprehensive treatment measures including early mechanical ventilation and broad spectrum antibiotics were taken into account.Conclusions RDS is not an uncommon disease in full-term infants and is associated with a higher mortality, its clinical characteristics are very

  11. Sociodemographic and Clinical Characteristics of Patients attending Psychotherapy in a Tertiary Care Hospital in Oman

    Directory of Open Access Journals (Sweden)

    Zena Al-Sharbati

    2012-02-01

    Full Text Available Objectives: There is significant evidence that psychotherapy is a pivotal treatment for persons diagnosed with Axis I clinical psychiatric conditions; however, a psychotherapy service has only recently been established in the Omani health care system. This study aimed to investigate the sociodemographic and clinical characteristics of attendees at a psychotherapy clinic at a tertiary care hospital. Methods: An analysis was carried out of 133 new referrals to the Psychotherapy Service at Sultan Qaboos University Hospital, a tertiary care hospital. Results: The majority of referrals were females (59%, aged 18–34 years, employed (38%, had ≤12 years of formal education (51%, and were single (54%. A total of 43% were treated for anxiety disorders (including obsessive compulsive disorder, while 22% were treated for depression. A total of 65% were prescribed psychotropic medications. The utilisation of the Psychotherapy Service and its user characteristics are discussed within the context of a culturally diverse Omani community which has unique personal belief systems such as in supernatural powers (Jinn, contemptuous envy (Hassad, evil eye (Ain and sorcery (Sihr which are often used to explain the aetiology of mental illness and influence personal decisions on utilising medical and psychological treatments. Conclusion: Despite the low number of referrals to the Psychotherapy Service, there is reason to believe that psychotherapy would be an essential tool to come to grips with the increasing number of mental disorders in Oman.

  12. Clinical and pathological characteristics of septum pellucidum tumor and choice of surgical approaches for its resection

    Institute of Scientific and Technical Information of China (English)

    WANG Lei; ZHANG Mao-zhi; ZHANG Wei; ZHAO Shang-feng; ZHAO Ji-zong; JIA Jin-xiu

    2005-01-01

    Background Tumor involving the septum pellucidum is uncommon. Surgery as the main therapeutic procedure for this lesion is a challenge to neurosurgeons. We analyzed the clinical characteristics and pathological features of septum pellucidum tumor in 41 patients and compared the curative effects of frontal transcortical, trans-sulcal and interhemispheric transcallosal approaches. Methods Clinical characteristics and the pathological features of septum pellucidum tumor were investigated retrospectively in 41 patients. The differences in postoperative residual rates, extents of tumors and resection of normal brain tissues after use of the three approaches in these patients were analyzed statistically. Results Septum pellucidum tumor is more likely to attack young or middle-aged persons. The tumor mainly presents itself as a central neurocytoma or cerebral low-grade glioma in pathology and manifests as intracranial hypertension clinically. No difference was found in the extent of tumor resection but significant difference in the extent of normal brain tissue resection and in postoperative disability rate among the three approaches. The transcortical approach brought about the most serious injury to brain tissue and the highest disability rate, Whereas the frontal transcallosal approach the lightest injury and the lowest disability rate. The injury to brain tissue and the disability rate brought about by the front trans-sulcus approach were between the above two approaches. Conclusions Operation is still regarded the major treatment for septum pellucidum tumor. Transcallosal and trans-sulcus approaches are fit with the concept of minimally invasive surgery, and transcallosal approach is the first choice for septum pellucidum tumor.

  13. Clinical characteristics as a function of referral status among substance users in residential treatment.

    Science.gov (United States)

    Banducci, Anne N; Dahne, Jennifer; Magidson, Jessica F; Chen, Kevin; Daughters, Stacey B; Lejuez, C W

    2013-04-01

    In the United States, substance users who voluntarily (VO) elect to receive treatment and substance users who are court-mandated (CM) to receive treatment typically obtain care within the same facilities. Little is known about the clinical characteristics that differentiate these individuals. The current study provides rates of specific DSM-IV Axis I and Axis II psychiatric and substance use disorders, comorbidities, childhood trauma, motivation, and other clinical and demographic characteristics as a function of referral status, among individuals in residential substance use treatment (463 participants, M age=43.3; 69.7% male; 88.4% African American). Participants were interviewed and diagnosed using the Structure Clinical Interview for DSM-IV and the Diagnostic Interview for Personality Disorders. Within our sample, VO individuals, as compared to CM individuals had significantly higher rates of psychiatric disorders (68.7% versus 55.2%, respectively), including mood disorders, major depressive disorder, generalized anxiety disorder, and borderline personality disorder. Additionally, they were significantly more likely to have alcohol dependence (43.0% versus 20.8%) and cocaine dependence (66.5% versus 48.9%). Elevated rates of comorbidities and childhood abuse were also observed among VO individuals, while motivation did not differ as a function of referral status. Overall, VO individuals appeared to have more severe problems than their CM counterparts which may suggest that they require more intensive or different types of treatment. PMID:23380487

  14. Patients with Helicobacter pylori positive and negative duodenal ulcers have distinct clinical characteristics

    Institute of Scientific and Technical Information of China (English)

    Kent-Man Chu; Ka-Fai Kwok; Simon Law; Kam-Ho Wong

    2005-01-01

    AIM: To assess the clinical characteristics of Helicobacterpylori(H pylori) negative duodenal ulcer.METHODS: Patients with an endoscopic diagnosis of duodenal ulcer between 1996 and 2002 were included in the present study. Patients were considered to be negative for Hpylori, if both histological examination and rapid urease test of biopsy specimens were negative. A comparison was made between patients with H pyloripositive and negative duodenal ulcers.RESULTS: A total of 1 343 patients were studied. Their mean age was 54.7±0.5 years. There was a male preponderance (M:F = 2.5:1). Three hundred and ninetyeight patients (29.6%) did not have H pylori infection. The annual proportion of patients with H pylori negative duodenal ulcers increased progressively from 1996 to2002. On multivariate analysis, patients with H pylorinegative duodenal ulcer were more likely to be older, have concomitant medical problem, pre-existing malignancy, recent surgery, underlying sepsis, or taken non-steroidal anti-inflammatory drugs. In terms of clinical presentations, patients with H pylori negative duodenal ulcer were more likely to present with bleeding, multiple ulcers and larger ulcers.CONCLUSION: The proportion of patients with H pylori negative duodenal ulcers is on the rise because of a continued drop in incidence of H pylori positive duodenalulcers in recent years. Such patients have distinct clinical characteristics and it is important to ascertain the H pylori status before starting eradication therapy.

  15. Microdosimetric Characteristics of the Clinical Proton Beams at the JINR Phasotron, Dubna

    CERN Document Server

    Vlcek, B; Spurny, F

    2002-01-01

    The contribution of the high LET particles to dosimetric and microdosimetric characteristics of 150 and 205 MeV clinical proton beams was experimentally studied using track etched detectors. Secondary heavy charged particles produced from nuclear interactions and degraded protons at the Bragg peak region are particles with high LET. The method of the LET spectra measurement with track etched detectors allows one to determine the contribution of high LET particles to dosimetric characteristics of clinical proton beams: absorbed dose, equivalent dose and the value of the Relative Biological Effectiveness (RBE). Track detectors were irradiated in the various depth of clinical proton beams with the primary energies of 150 and 205 MeV. The LET spectra between 10 and 700 keV/m were measured by means of CR-39 track etched detectors and the automatic optical image analyzer LUCIA-II. The relative contribution of the high LET particles to absorbed dose increases from several per cent at the beam entrance to several ten...

  16. Acute HIV infection (AHI in a specialized clinical setting: case-finding, description of virological, epidemiological and clinical characteristics

    Directory of Open Access Journals (Sweden)

    Adriana Ammassari

    2014-11-01

    Full Text Available Introduction: Diagnosis of HIV infection during early stages is mandatory to catch up with the challenge of limiting HIV viral replication and reservoirs formation, as well as decreasing HIV transmissions by immediate cART initiation. Objectives: Aims were to describe (a virological characteristics of AHI identified, (b epidemiological and clinical factors associated with being diagnosed with AHI. Methods: Cross-sectional, retrospective study. All individuals diagnosed with AHI according to Fiebig's staging between Jan 2013 and Mar 2014 at the INMI “L. Spallanzani” were included. Serum samples reactive to a fourth generation HIV-1/2 assay (Architect HIV Ag/Ab Combo, Abbott were retested with another fourth generation assay (VIDAS DUO HIV Ultra, Biomérieux and underwent confirmation with HIV-1 WB (New Lav I Bio-Rad and/or with Geenius confirmatory assay (Bio-Rad. WHO criteria (two env products reactivity were used to establish positivity of confirmatory assays. In case of clinically suspected AHI, HIV-1 RNA (Real time, Abbott and p24 assay (VIDAS HIV P24 Bio-Rad were also performed. Avidity test was carried out, on confirmed positive samples lacking p31 reactivity, to discriminate between recent (true Fiebig V phase and late infections; to avoid possible misclassifications, clinical data were also used. Demographic, epidemiological, clinical and laboratory data are routinely, and anonymously recorded in the SENDIH and SIREA studies. Results: During the study period, we observed 483 newly HIV diagnosed individuals, of whom 40 were identified as AHI (8.3%. Fiebig classification showed: 7 stage II/III, 13 stage IV, 20 stage V. Demographic, epidemiological, and clinical characteristics of patients are shown in the Table. Overall, the study population had a median S/Co ratio at fourth generation EIA (Architect of 49.50 (IQR, 23.54–98.05: values were significantly lower in Fiebig II-IV than in Fiebig V (38.68 [IQR, 20.08–54.84] vs 75.72 [IQR

  17. Geographic differences in clinical characteristics and management of COPD: the EPOCA study

    Science.gov (United States)

    Miravitlles, Marc; Murio, Cristina; Tirado-Conde, Gema; Levy, Gur; Muellerova, Hana; Soriano, Joan B; Ramirez-Venegas, Alejandra; Ko, Fanny WS; Canelos-Estrella, Byron; Giugno, Eduardo; Bergna, Miguel; Chérrez, Ivan; Anzueto, Antonio

    2008-01-01

    Aims Data on differences in clinical characteristics and management of COPD in different countries and settings are limited. We aimed to characterize the profile of patients with COPD in a number of countries and their treatment in order to evaluate adherence to recommendations of international guidelines. Method This was an observational, international, cross-sectional study on patients with physician-diagnosed COPD. Demographic and clinical characteristics, risk factors, and treatment were collected by their physician via an internet web-based questionnaire developed for the study. Results A total of 77 investigators from 17 countries provided data on 833 patients. The countries with the highest number of patients included were: Argentina (128), Ecuador (134), Spain (162), and Hong Kong (153). Overall, 79.3% were men and 81% former smokers, with a mean FEV1 = 42.7%, ranging from 34.3% in Hong Kong to 58.8% in Ecuador. Patients reported a mean of 1.6 exacerbations the previous year, with this frequency being significantly and negatively correlated with FEV1(%) (r = −0.256; p < 0.0001). Treatment with short-acting bronchodilators and theophyllines was more frequent in Ecuador and Hong Kong compared with Spain and Argentina, and in patients belonging to lower socioeconomic levels (p < 0.0001 for all comparisons). Inadequacy of treatment with inhaled corticosteroids and theophyllines was high, with significant differences among countries. Conclusions Differences in the clinical characteristics and management of COPD were significant across countries. Adherence to international guidelines appears to be low. Efforts should be made to disseminate and adapt guidelines to the socioeconomic reality of different settings. PMID:19281096

  18. Clinical and genetic characteristics of Chinese hereditary nonpolyposis colorectal cancer families

    Institute of Scientific and Technical Information of China (English)

    Xu-Lin Wang; Ying Yuan; Su-Zhan Zhang; Shan-Rong Cai; Yan-Qin Huang; Qiang Jiang; Shu Zheng

    2006-01-01

    AIM: To analyze the clinical characteristics of Chinese hereditary nonpolyposis colorectal cancer (HNPCC) families and to screen the germline mutations of human mismatch repair genes hMLH1 and hMSH2 in the probands.METHODS: Thirty-one independent Chinese HNPCC families were collected in Zhejiang Province. All of them met Chinese HNPCC criteria. Clinical data about patient gender, site of colorectal cancer, age of onset, history of multiple colorectal cancer, associated extracolonic cancer were recorded. PCR and denaturing high performance liquid chromatography (DHPLC) were employed to screen the mutations. Sequencing analysis was used to find out the exact mutation site and characteristics of the samples showing abnormal DHPLC profiles.RESULTS: One hundred and thirty-six malignant neoplasms were found in 107 patients including 14 multiple cancers. One hundred and six of the 136 neoplasms (77.9%) were diagnosed as colorectal cancer, with an average age of onset at 48.57 ± 29.00 years. Gastric cancer was the most common extracolonic cancer (10.3%) in these families. Twenty-three different sequence variations in hMLH1 and hMSH2 genes were detected in these 17 families. Fifteen sequence variations were located in the exons, including 5 SNPs, 3 silent mutations, 3 missense mutations, 2 nonsense mutations and 2 frameshift mutations. The latter seven mutations seemed to be pathogenic.CONCLUSION: Germline mutations of hMLH1 and hMSH2 genes are identified in about one-third HNPCC kindreds fulfilling Chinese HNPCC criteria. Chinese HNPCC families have some particular clinical characteristics, such as a left-sided predominance, less synchronous or metachronous colorectal cancer, and frequent occurrence of gastric cancer.

  19. Clinical, epidemiological and social characteristics of long-lived persons of the Volga region industrial centre

    Directory of Open Access Journals (Sweden)

    Malinova L.I.

    2011-09-01

    Full Text Available Aim: In spite of recent achievements in public health, average lifetime in the Volga region as in whole Russia is rather short. Issues of longevity in the region produce scientific interest by clinical, epidemiological and social characteristics of long-lived persons. Material and methods: In total 167 long-lived persons were examined. Medical, social, psychic, emotional and epidemiological characteristics were assessed. Results: The typical long-lived person of the Volga region was brought out. The high quality of life was revealed. The negative influence of smoking and alcoholism on the longevity was shown. Most of the patients needed communication. Lack of complaints was revealed, patients mentioned only the general weakness. Conclusion: The study has explained new social and demographic features of the Volga region long-livers

  20. Observational study identifies non-attendance characteristics in two hospital outpatient clinics

    DEFF Research Database (Denmark)

    Blæhr, Emely; Søgaard, Rikke; Kristensen, Thomas;

    2016-01-01

    INTRODUCTION: Non-attended hospital appointments are receiving increasing attention in times when rapid access and efficient service delivery at public hospitals are on the agenda. The aim of this study was to investigate the extent of non-attendance in a Danish outpatient setting and its...... association with user-level and provider-level characteristics. METHODS: The study was based on appointments scheduled from June 2013 to March 2015 at an orthopaedic and a radiologic outpatient clinic. Data on outcomes of cancellation on the part of the user or the provider, and non-attendance without giving...... unattended without notice. The latter was significantly associated with male gender, younger age and longer time since referral. Other characteristics were identified as significant, but differed between departments. CONCLUSION: There seems to be a potential for a targeted effort aiming to reduce non-attendance...

  1. Incidence and clinical characteristics of fungal keratitis in a Danish population from 2000 to 2013

    DEFF Research Database (Denmark)

    Nielsen, Stine E; Nielsen, Esben; Julian, Hanne Olsen;

    2015-01-01

    PURPOSE: Fungal keratitis is a severe sight-threatening condition. The aim of this study was to investigate the incidence and clinical characteristics of fungal keratitis patients living in a temperate climate. METHODS: By reviewing medical records from 2000 to July 2013, patients with fungal...... keratitis were identified. Risk factors, clinical signs and outcome were registered. RESULTS: Twenty-five patients were identified: 52% with Candida, 20% with Fusarium, 16% with Aspergillus and 12% with mixed filamentous fungi. A minimum incidence of fungal keratitis of 0.6 cases per million per year...... was estimated. Prior topical steroid treatment was commonly found in our cases (44%). Trauma including contact lens wear was associated with infection with filamentous fungi, whereas in patients with Candida infection, ocular surface disease was a prominent feature. Median time from onset of symptoms...

  2. Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

    Directory of Open Access Journals (Sweden)

    Narges Karimi

    2015-07-01

    Full Text Available Context: Chronic inflammatory demyelinating polyradiculopathy (CIDP is an acquired and autoimmune neuropathy, characterized by a chronic, rapidly progressive, symmetric weakness. In children, abnormal gait is as a first symptom of muscle weakness. Evidence Acquisition: The diagnosis of CIDP is on the basis of clinical characteristics, electrodiagnostic that shows the severity of the disease, lumbar puncture and spine magnetic resonance imaging (MRI. Results: The first-line treatments in childhood CIDP are intravenous immunoglobulin (IVIG, corticosteroids, and plasmapheresis. Response to first-line therapies is usually satisfactory; nevertheless, recommendations regarding the choice of second-line therapy can only be prepared on the basis of the existing practice described in some of the case reports. Conclusions: This review demonstrated the clinical presentation, diagnosis, and treatment of childhood CIDP.

  3. Clinical Characteristics and Predictors of Outcome of Schizophrenia-Spectrum Psychosis in Children and Adolescents

    DEFF Research Database (Denmark)

    Stentebjerg-Olesen, Marie; Pagsberg, Anne K; Fink-Jensen, Anders;

    2016-01-01

    OBJECTIVE: Treatment of early-onset schizophrenia spectrum psychosis (EOS) is hampered by limited data on clinical presentation and illness course. We aimed to systematically review the clinical characteristics, diagnostic trajectories, and predictors of illness severity and outcomes of EOS....... METHODS: We conducted a systematic PubMed, PsycINFO, and Embase literature review including studies published from January 1, 1990 to August 8, 2014 of EOS patients with 1) ≥50% nonaffective psychosis cases; 2) mean age of subjects mental health services; 4...... independent samples (n = 1506, age = 15.6 years, age at illness onset = 14.5 years, males = 62.3%, schizophrenia-spectrum disorders = 89.0%), the most frequent psychotic symptoms were auditory hallucinations (81.9%), delusions (77.5%; mainly persecutory [48.5%], referential [35.1%], and grandiose [25...

  4. The clinical characteristics and therapy of syndrome of craniocerebral- cervical vertebral injury

    Institute of Scientific and Technical Information of China (English)

    LIU Sheng; LIU Yuan-xin; WANG Cheng

    2005-01-01

    Objective: To explore the clinical characteristics and new treatment for syndrome of craniocerebral-cervical vertebral injury. Methods: The clinical data of 52 patients with head injury accompanied by neck injury were analyzed retrospectively. Results: Craniocerebral injury could result in damage to cervical vertebrae, muscles, vessels and nerves, and even cause vertebral artery injury, which may lead to insufficient blood-supply of vertebral-basal artery. All patients were treated with cervical vertebral traction and the results were good. Conclusions: Acute craniocerebral injury with symptom of insufficient blood-supply of vertebral-basal artery, evident neurosis and atlas-axis half-dislocation in X-ray should be treated by cervical vertebral traction, which will yield better outcome.

  5. Clinical Characteristics and Prognostic Analysis of 107 Patients with Bronchioloalveolar Carcinoma

    Institute of Scientific and Technical Information of China (English)

    Dongsheng Yue; Changli Wang; Zhenfa Zhang; Zhongli Zhan

    2006-01-01

    OBJECTIVE To study the clinical characteristics and prognostic factors for bronchioloalveolar carcinoma.METHODS Clinical data from 107 inpatient cases at The Cancer Hospital of Tianjin Medical University, from 1990-2000, were retrospectively reviewed.RESULTS The overall 1, 3 and 5-year survival rates were 88.7, 64.8 and 48.6% respectively. The main prognostic factors were tumor diameter (P=0.022), bronchial stump (P=0.016), TNM stage (P=0.000), T stage (P=0.002), N stage (P=0.000) and postoperation radiotherapy (P=0.001). Coxregression analysis suggested that the TNM stage (P=0.000) and tumor diameter (P=0.015) are independent factors affecting the prognosis.CONCLUSION The overall survival rate of BAC patients was superior to those with other non-small cell lung cancer (NSCLC). The TNM stage and tumor diameter were independent factors affecting the prognosis for BAC.

  6. Clinical characteristics of the asthma-COPD overlap syndrome--a systematic review

    DEFF Research Database (Denmark)

    Nielsen, Mia; Bårnes, Camilla Boslev; Ulrik, Charlotte Suppli

    2015-01-01

    is described clinically as persistent airflow limitation combined with features of both asthma and COPD. The aim of this paper is, therefore, to review the currently available literature focusing on symptoms and clinical characteristics of patients regarded as having ACOS. METHODS: Based on the preferred...... reporting items for systematic reviews and meta-analyses (PRISMA) guidelines, a systematic literature review was performed. RESULTS: A total of 11 studies met the inclusion criteria for the present review. All studies dealing with dyspnea (self-reported or assessed by the Medical Research Council dyspnea......% predicted and FEV1/FVC ratio in spite of lower mean life-time tobacco exposure. Furthermore, studies have revealed that ACOS patients seem to have not only more frequent but also more severe exacerbations. Comorbidity, not least diabetes, has also been reported in a few studies, with a higher prevalence...

  7. Clinical and epidemiological characteristics of individuals with very high rapid plasma reagin.

    Science.gov (United States)

    Castro, Jose G; Alcaide, Maria L; Rosa-Cunha, Isabella

    2014-07-01

    The objective of the study was to identify clinical and epidemiological characteristics of patients with infectious syphilis who presented with a high rapid plasma reagin (RPR) titre (≥1: 512) during the year of 2009 at the Miami Dade County Health Department (MDCHD) STD clinic. Potential cases were identified by a search in the electronic database. Among 519 individuals identified with reactive RPR, 190 individuals met criteria for infectious syphilis and 32 of them had at least one RPR titre of ≥1: 512. We found that the majority of individuals with high RPR were men who have sex with men (82%), from ethnic minorities (91%), and HIV infected (75%) but only 3 of them were on antiretroviral therapy. Overall, 50% of these patients with very high RPR titres were symptomatic, and the most common symptom was skin rash (93%). PMID:24352133

  8. Clinical and Demographic Characteristics of 626 Patients with Moderate and Severe Psoriasis

    Directory of Open Access Journals (Sweden)

    Emine Rifaioglu

    2014-02-01

    Full Text Available Aim: In our study, we aim to investigate the demographic, clinic features and the treatments of patients with moderate to severe psoriasis who were followed in outpatient clinic of psoriasis of Department of Dermatology in Istanbul Medical School of Istanbul University. Material and Method: One thousand eight hundred and thirty-one patients, who were followed between 2002-2008 in outpatient clinic of psoriasis, were screened and 626 (34% of them, who were moderate and severe, were evaluated retrospectively. Results: Three hundred five of the patients who participated in our study were female and 321 were male. Mean age of onset was 28.1. Most frequently clinical type was plaque psoriasis. Most frequently nail involvement was pitting in patients. Nail involvement was statistically common in arthropathic patients. Family history was statistically common in psoriasis patients with early onset. Retinoid was the most common used systemic drug. Narrow band UVB was the most common used phototherapy. Discussion: Moderate to severe psoriasis rate was 34%. Female to male ratio was close to one another. Peak age of onset of psoriasis was between 16-25 years old. The most common concomitant systemic disease was diabetes mellitus. The findings were consistent with the literature. Consequently psoriasis is a chronic, recurrent, inflammatory dermatologic disease which affect 2% of the word and which negatively affect the quality of life in patients with comorbidities. Especially in severe forms of psoriasis can cause loss of labor in patients. A good knowledge of demographic and clinical characteristics of psoriasis is important to shedding light on the further clinical studies.

  9. Clinical characteristics of airway foreign bodies in which bronchoscopic removal was difficult

    International Nuclear Information System (INIS)

    Airway foreign bodies may cause suffocation and pneumonia. Therefore, accurate diagnosis and expeditious removal are needed. However, we sometimes experience difficulty in removing foreign bodies from the airway by bronchoscopy. We examined the clinical characteristics of 15 cases of foreign body managed in our institute over the past 19 years, and evaluated characteristic difficulties during bronchoscopic removal. The mean age of cases was 58 years. Four patients had dementia as their underlying disease. Episodes of aspiration occurred in 10 patients. Dental foreign bodies were seen in 9 patients (60%). Chest X-ray films detected foreign bodies in 11 patients (73%), and chest computed tomography identified them in all patients. Alligator forceps and basket forceps were mainly used for removal. Fogarty forceps were also used in combination with the above in 2 patients. The foreign body was removed during the first procedure in 12 patients; however, further procedures were needed (20%) in the remaining 3 patients: using different bronchoscopic techniques, tracheotomy and surgical resection, respectively. The characteristics of these 3 cases were as follows; granulation forming around a sharp foreign body obstructing the bronchus for a prolonged period, inability of the patient to lie still due to dementia, and tracheal stenosis after tracheotomy. Characteristic difficulties during bronchoscopic removal were granulation around the foreign body, inability of the patient to lie still, and tracheal stenosis. When difficulties in bronchoscopic removal are expected, it is necessary to prepare a variety of devices in advance. (author)

  10. STUDY ON THE CLINICAL CHARACTERISTICS OF ADULT PATIENTS WITH KETOSISONSET DIABETES

    Institute of Scientific and Technical Information of China (English)

    叶新华; 成金罗

    2013-01-01

    Objective To explore the classification and analyze the clinical characteristics of adult diabetic patients with ketosis as initial manifestation.Methods 142 cases with initial onset of ketosis were retrospectively analyzed and the levels of blood lipid,random blood glucose,glycosylated hemoglobin,islet function and islet autoantibody[insulin autoantibody (IAA) ,glutamic acid decarboxylase antibody (GADA) ]were measured.The blood pressure and family history of diabetes were also documented.Based on the presence of islet autoantibodies (A+or

  11. Mooren's ulcer in China: a study of clinical characteristics and treatment

    OpenAIRE

    Chen, J; Xie, H; Wang, Z.(Institute of High Energy Physics, Beijing, China); Yang, B.; Liu, Z.; Chen, L.; Gong, X.; Lin, Y.

    2000-01-01

    AIMS—To investigate the clinical characteristics and compare the effects of several methods of treatment of Mooren's corneal ulcer.
METHODS—550 consecutive cases of Mooren's corneal ulcer were analysed in patients, including age, sex, laterality of eye, ulcer location, perforative rate, cure rate of surgeries, recurrent rate, the effects of conjunctiva excision, lamellar keratoplasty (LKP), and LKP plus 1% cyclosporin A eye drops.
RESULTS—The average age of onset was 48.4 years of age. The ra...

  12. Extranodal marginal zone lymphoma in the ocular region: clinical, immunophenotypical, and cytogenetical characteristics

    DEFF Research Database (Denmark)

    Sjö, Lene D; Heegaard, Steffen; Prause, Jan U;

    2008-01-01

    PURPOSE: To evaluate clinical, immunophenotypical, and cytogenetical characteristics of 116 patients with a diagnosis of extranodal marginal zone lymphoma (EMZL) presenting primarily in the ocular region. METHODS: Specimens from all patients with a diagnosis of ophthalmic lymphoma in Denmark during......-translocation was the only factor associated with PFS, whereas a favorable International Prognostic Index (IPI) score was the most reliable predictor of OS. CONCLUSIONS: EMZL presenting in the ocular region usually runs an indolent course, but relapses are frequently seen. The IPI-score was the most reliable...

  13. Clinical characteristics of late-onset severe pneumonia after allogeneic hematopoietic stem cell transplantation

    Institute of Scientific and Technical Information of China (English)

    刘代红

    2013-01-01

    Objective To analyze the clinical characteristics of the late-onset severe pneumonia after allogeneic hematopoietic stem cell transplantation (allo-HSCT) .Methods A retrospective study was conducted in patients diagnosed as late-onset severe pneumonia after allo-HSCT from March,2009 to January,2013 in People’s Hospital of Peking University.Results Of 1538 patients receiving allo-HSCT,20 developed late-onset severe pneumonia with an incidence rate of 1.3%.Among the 20 patients,17 (85%) had human leukocyte antigen (HLA) identical donors.The other 3 (15%) patients had received haploidentical transplantation.Severe pneumonia occurred at a

  14. Attaching and effacing Escherichia coli isolates from Danish children: clinical significance and microbiological characteristics

    DEFF Research Database (Denmark)

    Jensen, C; Ethelberg, S; Olesen, B;

    2007-01-01

    This study describes the prevalence, clinical manifestations and microbiological characteristics of attaching and effacing Escherichia coli isolates, i.e., enteropathogenic E. coli (EPEC) belonging to the classical EPEC serotypes, non-EPEC attaching and effacing E. coli (A/EEC) and verocytotoxin......-producing E. coli (VTEC), isolated in a case-control study of Danish children aged ... encoding the bundle-forming pilus (bfpA) and enteroaggregative heat-stable enterotoxin (astA) were rare among all isolates, and seemed to be of limited pathogenic importance in this population. Virulence characterisation of A/EEC isolates did not reveal any significant differences between cases...

  15. Clinical characteristics of Raoultella ornithinolytica bacteremia: a case series and literature review.

    Science.gov (United States)

    Haruki, Yuto; Hagiya, Hideharu; Sakuma, Akiko; Murase, Tomoko; Sugiyama, Tetsuhiro; Kondo, Sachiyo

    2014-09-01

    Raoultella ornithinolytica is a rare pathogen in human infection and bacteremic cases had been scarcely reported. For further comprehension of the rare infection, we summarized clinical characteristics of 6 cases that were detected at our medical facility and 5 cases from previous literature. The most common infectious focus was biliary infection and elderly patients with a history of any biliary intervention or malignancy were considered to be at a great risk for the infection. The prognosis of the patients was quite satisfactory. Bacterial identification in this report was performed on the basis of biochemical tests alone, and further investigations by molecular analysis are required to confirm our findings.

  16. Poststroke Hip Fracture: Prevalence, Clinical Characteristics, Mineral-Bone Metabolism, Outcomes, and Gaps in Prevention

    OpenAIRE

    Alexander Fisher; Wichat Srikusalanukul; Michael Davis; Paul Smith

    2013-01-01

    Objective. To assess the prevalence, clinical and laboratory characteristics, and short-term outcomes of poststroke hip fracture (HF). Methods. A cross-sectional study of 761 consecutive patients aged ≥60 years (82.3 ± 8.8 years; 75% females) with osteoporotic HF. Results. The prevalence of poststroke HF was 13.1% occurring on average 2.4 years after the stroke. The poststroke group compared to the rest of the cohort had a higher proportion of women, subjects with dementia, history of TIA, hy...

  17. Clinical characteristics and prevalence of vitamin D insufficiency in children less than two years of age

    OpenAIRE

    Yoon, Ji Hyun; Park, Cheong Soo; Seo, Ji Young; Choi, Yun Sun; AHN, YOUNG MIN

    2011-01-01

    Purpose To evaluate the clinical characteristics of vitamin D deficiency and its association with iron deficiency anemia (IDA). Methods A total of 171 children aged less than two years underwent 25-hydroxyvitamin D3 tests between January 2007 and July 2009. The study was classified into two groups: normal and vitamin D insufficiency, by their vitamin 25-hydroxyvitamin D3 levels. Results In total, 120 children were in the normal group (mean age, body weight and heights 12.5±7.0, 9.3±0.9 kg and...

  18. Prevalence, clinical characteristics and management of atrial fibrillation in patients with Brugada syndrome.

    Science.gov (United States)

    Rodríguez-Mañero, Moisés; Namdar, Mehdi; Sarkozy, Andrea; Casado-Arroyo, Rubén; Ricciardi, Danilo; de Asmundis, Carlo; Chierchia, Gian-Battista; Wauters, Kristel; Rao, Jayakeerthi Y; Bayrak, Fatih; Van Malderen, Sophie; Brugada, Pedro

    2013-02-01

    Atrial fibrillation (AF) can be the first manifestation of latent Brugada syndrome (BS). The aim of our study was to assess the prevalence of AF as the first clinical diagnosis in patients with BS and their demographic and clinical characteristics and diagnosis management in a large cohort of patients. The patient group consisted of 611 patients with BS. The data from those with a diagnosis of AF previous to the identification of BS were analyzed (n = 35). Eleven cases were unmasked after the initiation of a class I antiarrhythmic drug and one during the establishment of general anesthesia. In the remaining population, BS was diagnosed using an ajmaline test performed mainly because of younger age in patients with lone AF (n = 13), previous syncope or sudden cardiac death (n = 3), or a clinical history of sudden cardiac death in the family (n = 5). The mean patient age was 49 ± 15 years, 21 were male patients, 14 had a family history of sudden death, 15 had had previous syncope, and 4 had survived cardiac arrest. Concomitant electrical disorder was found in 13 patients. Remarkably, 21 patients had normal findings on the baseline electrocardiogram. In conclusion, AF could be one of the first clinical manifestations of latent BS in a considerable number of patients. This identification is crucial because the treatment of these patients is subject to relevant changes. The ajmaline test plays an essential role, mainly in young patients with a family history of sudden death, despite having normal findings on a baseline electrocardiogram. PMID:23206922

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

    Institute of Scientific and Technical Information of China (English)

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

    2014-01-01

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

  20. Clinical and molecular characteristics of East Asian patients with von Hippel-Lindau syndrome

    Institute of Scientific and Technical Information of China (English)

    Meihua Wong; YingHsia Chu; Hwei Ling Tan; Hideharu Bessho; Joanne Ngeow; Tiffany Tang; MinHan Tan

    2016-01-01

    Background: Von Hippel–Lindau (VHL) syndrome is a dominantly inherited multisystem cancer syndrome caused by a heterozygous mutation in the VHL tumor suppressor gene. Previous studies suggested that similar populations of Caucasian and Japanese patients have similar genotype or phenotype characteristics. In this comprehensive study of East Asian patients, we investigated the genetic and clinical characteristics of patients with VHL syndrome. Methods: To create a registry of clinical characteristics and mutations reported in East Asian patients with VHL syndrome, we conducted a comprehensive review of English language and non‑English language articles identi‑fied through a literature search. Publications in Japanese or Chinese language were read by native speakers of the language, who then performed the data extraction. Results: Of 237 East Asian patients with VHL syndrome, 154 unique kindreds were identified for analysis. Analyzed by kindred, missense mutations were the most common (40.9%, 63/154), followed by large/complete deletions (32.5%, 50/154) and nonsense mutations (11.7%, 18/154). Compared with a previously reported study of both East Asian and non‑East Asian patients, we found several key differences. First, missense and frameshift mutations in the VHL gene occurred less commonly in our population of East Asian patients (40.9% vs. 52.0%; P = 0.012 and 8.4% vs.13.0%; P < 0.001, respectively). Second, large/complete deletions were more common in our population of East Asian patients (32.5% vs. 10.5%; P < 0.001). Third, phenotypically, we observed that, in our population of East Asian patients with VHL syndrome, the incidence of retinal capillary hemangioblastoma was lower, whereas the incidence of renal cell carcinoma was higher. Conclusions: Evidence suggests that the genotypic and phenotypic characteristics of East Asian patients with VHL syndrome differ from other populations. This should be considered when making screening recommendations for