Chordomas of the skull base: follow-up review and prognostic factors

2001 ◽  
Vol 10 (3) ◽  
pp. 1-11 ◽  
Author(s):  
Benedicto O. Colli ◽  
Ossama Al-Mefty
Keyword(s):  
Prognostic Factors ◽  
Proton Beam ◽  
Age At Onset ◽  
Craniocervical Junction ◽  
Surgical Approaches ◽  
Subtotal Resection ◽  
Extensive Resection ◽  
Period Range ◽  
Proton Beam Radiotherapy

Object Chordomas are rare tumors that arise from the remnants of the notochord. Because of their deep location, local infiltrative nature, and involvement of surrounding bone, treatment of chordoma is a challenge. The authors analyze the data and prognostic factors obtained during the follow-up period (range 1–150 months, median 38 months) in 53 patients with craniocervical junction chordoma and 10 patients with chondrosarcoma. Methods Several surgical approaches were used, and some tumor excisions required staged procedures. Survival was calculated according the Kaplan–Meier method. Statistical analysis was performed using the Fisher exact and Kruskal–Wallis tests. Radical/subtotal resection was achieved in 77.8% of the patients. The mortality rate during the follow-up period was 14.3%. In patients harboring chondrosarcoma better 5-year recurrence-free survival (RFS) rates were demonstrated than in those with chordoma (100% and 50.7%, respectively). Histological patterns (typical or chondroid chordoma) and patient age at onset of symptoms had no effect on the RFS rates. Radical/subtotal resections were associated with better RFS rates than partial resection. Adjuvant proton-beam radiotherapy was shown to increase the RFS rates compared with conventional radiotherapy (90.9% and 19.4%, respectively at 4 years posttreatment). Karyotypically abnormal tumors were associated with worst RFS rates as compared with karyotypically normal lesions (44.5% and 90.3%, respectively at 3 years). Cases of cranial nerve palsy, followed by CSF leakages were the most frequent postoperative complication. Permanent postoperative neurological deficit was observed in 28.6% of the patients. Conclusions A better prognosis was observed in patients with chondrosarcoma compared with those harboring chordoma. Histological pattern and the patient's age at symptom onset were not factors that influenced prognosis in cases of chordoma. Extensive resection and possibly adjuvant proton-beam radiotherapy provided better prognosis for these patients.

Chordomas of the craniocervical junction: follow-up review and prognostic factors

2001 ◽  
Vol 95 (6) ◽  
pp. 933-943 ◽  
Author(s):  
Benedicto O. Colli ◽  
Ossama Al-Mefty
Keyword(s):  
Prognostic Factors ◽  
Proton Beam ◽  
Age At Onset ◽  
Proton Beam Therapy ◽  
Craniocervical Junction ◽  
Patient Age ◽  
Content Type ◽  
Patient Âgé ◽  
Fine Print

Object. Chordomas are rare tumors that arise from the remnants of the notochord. Because of their deep location, local infiltrative nature, and involvement of surrounding bone, treatment of chordomas is a challenge. In this study the authors analyze the data and prognostic factors obtained during the follow-up period (range 1–150 months, median 38 months) in 53 patients with craniocervical junction chordomas and 10 patients with chondrosarcomas. Methods. Several surgical approaches were used, and some tumor excisions required staged procedures. Survival was calculated according the Kaplan—Meier method. Statistical analysis was performed using Fisher exact, log rank and Kruskal—Wallis tests. Radical/subtotal resection was achieved in 77.8% of the patients. The mortality rate during the follow-up period was 14.3%. In patients harboring chondrosarcoma better 5-year recurrence-free survival (RFS) rates were demonstrated than in those with chordoma (100% and 50.7%, respectively). Histological patterns (typical or chondroid chordomas) and patient age at onset of symptoms had no effect on the RFS rates. Radical/subtotal resections were associated with better RFS rates than partial resection. Adjuvant proton-beam therapy was shown to increase the RFS rates compared with conventional radiotherapy (90.9% and 19.4%, respectively, at 4 years posttreatment). Karyotypically abnormal tumors were associated with the worst RFS rates compared with karyotypically normal lesions (44.5% and 90.3%, respectively, at 3 years). Cases of cranial nerve palsy followed by those with cerebrospinal fluid leakages were the most frequent postoperative complications. Permanent postoperative neurological deficit was observed in 28.6% of the patients. Conclusions. A better prognosis was observed in patients with chondrosarcoma compared with those harboring chordoma. Histological pattern and patient age at symptom onset were not factors that influenced prognosis in cases of chordoma. Extensive resection and possibly adjuvant proton-beam therapy provided better prognoses for these patients.

scholarly journals Long-Term Visual Outcomes for Small Uveal Melanoma Staged T1 Treated by Proton Beam Radiotherapy

Cancers ◽  
2019 ◽  
Vol 11 (8) ◽  
pp. 1047 ◽  
Author(s):  
Adélaïde Toutée ◽  
Martina Angi ◽  
Sylvain Dureau ◽  
Christine Lévy-Gabriel ◽  
Livia Lumbroso-Le Rouic ◽  
...  
Keyword(s):  
Proton Beam ◽  
Optic Disc ◽  
Uveal Melanoma ◽  
Early Stage ◽  
Tumor Control ◽  
Proton Beam Radiotherapy ◽  
Visual Outcomes ◽  
The Mean

There is increasing evidence of the survival benefit of treating uveal melanoma in an early stage, however it is important to discuss with the patient the associated risk of visual loss. We investigated visual outcomes for uveal melanomas staged T1 (T1UM) treated by proton beam radiotherapy (PBR) as a function of their distance to fovea-optic disc. This retrospective study included a cohort of 424 patients with T1UM treated with PBR between 1991 and 2010 with at least a 5-year follow-up. Visual acuity (VA) was analyzed for patients with posterior edge of tumor located at ≥3 mm (GSup3) or <3 mm (GInf3) from fovea-optic disc. The mean follow-up duration was 122 months, no tumor recurrence was observed. The mean baseline and final VA were 20/25 and 20/32 for GSup3 (n = 75), and 20/40 and 20/80 for GInf3 (n = 317) respectively. The frequency of a 20/200 or greater visual conservation was 93.2%(CI95%:87.7–99.1) and 60.1%(CI95%:54.9–65.9) for GSup3 and GInf3 respectively. This difference between groups was statistically significant (p < 0.001). The risk factors for significant VA loss (less than 20/200) were GInf3 location (p < 0.001), tumor touching optic disc (p = 0.04), initial VA inferior to 20/40 (p < 0.001), documented growth (p = 0.002), and age greater than 60 years (p < 0.001). In summary, PBR for T1UM yields excellent tumor control and good long-term visual outcomes for tumors located ≥3 mm from fovea-optic disc.

scholarly journals Ultra-wide-field imaging of choroidal melanoma before and after proton beam radiation therapy

2019 ◽  
Vol 30 (6) ◽  
pp. 1397-1402
Author(s):  
Angeliki Psomiadi ◽  
Gertrud Haas ◽  
Michael Edlinger ◽  
Nikolaos E Bechrakis ◽  
Georgios Blatsios
Keyword(s):  
Proton Beam ◽  
Choroidal Melanoma ◽  
Subretinal Fluid ◽  
Scanning Laser ◽  
Wide Field ◽  
Scanning Laser Ophthalmoscopy ◽  
Proton Beam Radiotherapy ◽  
Imaging Characteristics ◽  
Before And After

Objective: To evaluate the imaging characteristics of choroidal melanoma before and after proton beam radiotherapy via Optos® ultra-wide-field scanning laser ophthalmoscopy. Methods: Retrospective, descriptive study of choroidal melanoma patients treated with proton beam radiotherapy. All patients underwent full clinical evaluation, including best-corrected visual acuity, ultrasound examination and ultra-wide-field scanning laser ophthalmoscopy imaging in the pseudo-colour (red and green channel) as well as auto-fluorescence mode. Tumours were classified and evaluated according to their location, size, presence of subretinal fluid, drusen, orange pigment and reflectance intensity in ultra-wide-field scanning laser ophthalmoscopy. Tumour sonographic (basal diameter, height) and ultra-wide-field scanning laser ophthalmoscopy imaging dimensions (maximal diameter) were documented. Results: A total of 39 eyes (38 patients) were followed for 24 months (range 6–48 months). Mean best-corrected visual acuity dropped from 20/40 to 20/63 after proton beam radiotherapy. There was no change in the imaging tumour characteristics during follow-up. Subretinal fluid changes were better detected in the autofluorescence compared to pseudo-colour mode. Mean tumour diameter did not significantly change in the ultra-wide-field scanning laser ophthalmoscopy although it did so in the ultrasound. No patient showed local tumour recurrence. Conclusion: The ultra-wide-field scanning laser ophthalmoscopy imaging characteristics of choroidal melanoma in the Optos® system do not significantly change after proton beam radiotherapy after a mean follow-up of 2 years.

scholarly journals Prognostic factors and clinical outcome in a rare form of spastic paraplegia (Spoan syndrome): a 10-year follow-up

2019 ◽  
Author(s):  
Cláudia R. C. Galvão ◽  
Priscilla M. A. Cavalcante ◽  
Ricardo Olinda ◽  
Zodja Graciani ◽  
Mayana Zatz ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Clinical Outcome ◽  
Statistical Significance ◽  
Hand Function ◽  
Age At Onset ◽  
Spastic Paraplegia ◽  
Prospective Longitudinal Study ◽  
The Mean ◽  
Prospective Longitudinal

Abstract Background: Spastic paraplegia, optic atrophy and neuropathy (Spoan syndrome) is an autosomal recessive disease with approximately 70 cases recorded in Brazil and Egypt. Methods: This is a prospective longitudinal study performed with 47 patients affected with Spoan syndrome of seven communities of Rio Grande do Norte (Brazil) to investigate prognostic factors and clinical outcome based on comparative data obtained from a 10-year follow-up. Results: The mean age of the participants was 47.21±12.42 years old, and the mean ages at loss of ambulation and hand function were 10.78±5.55 and 33.58±17.47 years old, respectively. Spearman’s correlation analysis between the score on the Modified Barthel Index and the investigated variables evidenced statistical significance for age (p<0.001) and right- and left-hand grip strength (p=0.042 and p=0.021, respectively). Statistical significance was not evidenced for the remainder of the variables, including age at onset of symptoms (p=0.634), age at loss of ambulation (p=0.664) and age at loss of hand function (p=0.118). Conclusions: Our analysis allows asserting that the participants exhibited slight dependence until age 35. The greatest losses occurred from ages 35 to 41, and starting at 50, practically all patients become completely dependent. In fact, age is the main prognostic factor of impaired motor function among Spoan syndrome patients.

scholarly journals ATRT-06. RESULTS OF MULTICENTER TRIAL CONCERNING THE TREATMENT OF CHILDREN WITH ATYPICAL TERATOID RHABDOID TUMORS OF THE CENTRAL NERVOUS SYSTEM UNDER 3 YEARS OLD

2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i2-i2
Author(s):  
Zheludkova Olga ◽  
Olkhova Liudmila ◽  
Zubarovskaya Ludmilla ◽  
Dinikina Julia ◽  
Smirnova Anna ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Tumor Location ◽  
Multicenter Trial ◽  
Regional Chemotherapy ◽  
Individual Treatment ◽  
Subtotal Resection ◽  
Tumor Biopsy ◽  
Regional Administration ◽  
Extent Of Surgery

Abstract Objective To evaluate the prognostic factors in children with AT/RT aged under 3 years. Patients and methods: The prognostic factors were analyzed in 106patients under 3 years who got treatment and follow-up from 2008 to 2020.There were 41children younger than 12 months and 65patients older than 12 months. The location of the tumor was infratentorial in 58 patients, supratentorial in 46, and spinal cord in 2. There were 54 boys and 52 girls. Among the patients,57 had stage M0,36 had stage М+ or a multifocal tumor, and 13 had stage Mx. All the patients had undergone surgical treatment: total tumor removal in 27, subtotal-33, partial-42, biopsy - 4;67patients had got chemoradiotherapy according to the ATRT-2006(IRS III) protocol; 15, MUV-ATRT protocol; 3, CWS protocol; 9, EU-RHAB protocol; 6, HIT-SKK protocol; and 6 according to individual treatment schemes.12 patients received HDC with AuHCR. Results 47 are alive,1 was lost to follow-up, and 58 died:52 of progressive disease,6 of chemotherapy complications. The five-year PFS was 0.27; the five-year OS was 0.40. The PFS was significantly better in patients older than 12months old compared to patients under 12months: 0.33 and 0.17, respectively; p=0.0047. The PFS after total resection was higher than after subtotal resection, partial resection, and tumor biopsy: 0.51, 0.29, 0.09, and 0%, respectively (p=0.025).The PFS after radiotherapy was markedly higher compared to patients without radiotherapy: 0.63 and 0.0, respectively (р&lt;0.001). The tumor location, stage, and gender did not affect the PFS. The survival rate was statistically significantly higher among the patients who had got treatment according to the ATRT-2006 protocol compared to MUV-ATRT,EU-RHAB, individual therapeutic regimens, CWS, and HIT-SKK: 0.33, 0.26, 0.11, 0.30, and 0.0, respectively; p=0.0020. The PFS was higher among the patients who had got intraventricular/intrathecal chemotherapy; p=0.0002. HDC with AuHCR did not statistically affect the PFS; p=0.0546.In multivariate analysis, the PFS was influenced by the age, tumor location, extent of surgery, radiotherapy, regional chemotherapy, HDC with AuHCR. Conclusions The survival of patients with CNS AT/RT aged under 3years significantly depended on the patients’ age, extent of surgery, chemotherapy protocol, radiotherapy, and regional administration of chemotherapeutic drugs.

scholarly journals Review of skull base chordomas: prognostic factors and long-term results of proton-beam radiotherapy

2001 ◽  
Vol 10 (3) ◽  
pp. 1-5 ◽  
Author(s):  
Eugen B. Hug
Keyword(s):  
Prognostic Factors ◽  
Skull Base ◽  
Proton Beam ◽  
The United States ◽  
Long Term Results ◽  
Severe Toxicity ◽  
Long Term Outcome ◽  
Proton Beam Radiotherapy ◽  
Skull Base Chordomas

Fractionated proton beam radiotherapy has been used for skull base tumors in the United States since the mid-70s, and more than 300 patients in whom diagnosis of chordoma of the skull base has been made have been treated. The ability to achieve high degrees of radiation dose conformity by using protons has resulted in higher radiation doses than can be delivered with conventional radiotherapy in the base of skull. High target volume doses have led to improved tumor control and patient survival. Side effects such as severe toxicity are acceptable considering the alternatives of uncontrolled tumor growth. The authors of various analyses have identified prognostic factors that can be used to predict a patient's chance of treatment success. On the horizon are important technical developments that will further increase dose conformity and increase target doses. In this paper the author reviews long-term outcome data and prognostic predictors for survival of patients with skull base chordomas based on the largest worldwide patient series.

scholarly journals Partial acute transverse myelitis is a predictor of multiple sclerosis in children

2014 ◽  
Vol 20 (11) ◽  
pp. 1485-1493 ◽  
Author(s):  
P Meyer ◽  
N Leboucq ◽  
N Molinari ◽  
A Roubertie ◽  
M Carneiro ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Prognostic Factors ◽  
Severe Disease ◽  
Brain Mri ◽  
Age At Onset ◽  
Transverse Myelitis ◽  
First Episode ◽  
Acute Transverse Myelitis ◽  
Subsequent Risk

Background: Acute transverse myelitis (ATM) in children is a rare and often severe disease for which there are few known prognostic factors, particularly the subsequent risk of multiple sclerosis (MS) diagnosis. Objectives: To determine the clinical course and prognostic factors after a first episode of ATM in children. Methods: Thirty children below 16 years of age diagnosed with a first neurological episode of ATM were included retrospectively. Clinical evaluation, treatment, laboratory, and MRI data were collected. Results: Median age at onset was 11 years (range 3–15 years). Follow-up data were available for a median of 4 years (range 0.5–16.7 years). Five patients subsequently had a diagnosis of MS (17%), which was associated with acute partial transverse myelitis (odds ratio 5; 95% confidence interval 2.3–11), with a 60% probability of having a relapse at five years ( p < 0.01). The 2011 Verhey criteria correctly identified MS in children with the highest specificity (96%) and sensitivity (80%). Conclusion: Acute partial transverse myelitis and brain MRI abnormalities at initial presentation are significantly predictive of a subsequent diagnosis of MS in children with ATM. These findings suggest that closer brain MRI monitoring after acute partial transverse myelitis might make the earlier introduction of disease-modifying therapies possible.

scholarly journals Extended Volumetric Follow-up of Juvenile Pilocytic Astrocytomas Treated with Proton Beam Therapy

2016 ◽  
Vol 3 (2) ◽  
pp. 291-299 ◽  
Author(s):  
Edward M. Mannina ◽  
Greg K. Bartlett ◽  
Kevin P. McMullen
Keyword(s):  
Proton Beam ◽  
Response Rate ◽  
Lymphoblastic Leukemia ◽  
Proton Beam Therapy ◽  
Subtotal Resection ◽  
Histologic Diagnosis ◽  
Free Survival ◽  
Entire Cohort ◽  
Heavily Pretreated

Abstract Purpose: To describe volume changes following proton beam therapy (PBT) for juvenile pilocytic astrocytoma (JPA), we analyzed post-PBT magnetic resonance imaging (MRI) to clarify survivorship, response rate, and the concept of pseudoprogression. Materials and Methods: Pediatric patients with a histologic diagnosis of JPA after a biopsy or subtotal resection and at least 4 post-PBT MRIs were retrospectively reviewed. After PBT, tumors were contoured on follow-up T1-contrasted MRIs, and 3-dimensional volumes were plotted against time, with thresholds for progressive disease and partial response. Patterns of response, pseudoprogression, and progression were uncovered. Post-PBT clinical course was described by the need for further intervention and survivorship. Results: Fifteen patients with a median of 10 follow-up MRIs made up this report: 60% were heavily pretreated with multiple lines of chemotherapy, and 67% had undergone subtotal resection. With a median follow-up of 55.3 months after a median of 5400 centigray equivalents PBT, estimates of 5-year overall survival and intervention-free survival were 93% and 72%, respectively. The crude response rate of 73% included pseudoprogressing patients, who comprised 20% of the entire cohort; the phenomenon peaked between 3 and 8 months and resolved by 18 months. One nonresponder expired from progression. Post-PBT intervention was required in 53% of patients, with 1 patient resuming chemotherapy. There were no further resections or radiotherapy. One patient developed acute lymphoblastic leukemia, and another developed biopsy-proven radionecrosis. Conclusion: The PBT for inoperable/progressive JPA provided 72% 5-year intervention-free survival in heavily pretreated patients. Although most patients responded, 20% demonstrated pseudoprogression. The need for post-PBT surveillance for progression and treatment-induced sequelae should not be underestimated in this extended survivorship cohort.

scholarly journals Clinical phenotypes and prognostic factors of adult-onset Still’s disease: data from a large inpatient cohort

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Rui Li ◽  
Xiaolei Liu ◽  
Guangliang Chen ◽  
Guo Tang ◽  
Xiaoxiang Chen ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Infection Rate ◽  
Age At Onset ◽  
Clinical Manifestations ◽  
Adult Onset Still’S Disease ◽  
Still’S Disease ◽  
Clinical Phenotypes ◽  
Cluster 2 ◽  
Adult Onset Still's Disease

Abstract Objectives To define different clinical phenotypes and assess prognostic factors of adult-onset Still’s disease (AOSD). Methods Overall, 492 patients with AOSD seen between 2004 and 2018 at a single centre were retrospectively studied. Results Of these patients, 78% were female, and the median age at onset was 34 (25–49) years [median (25th–75th percentile)]. The median follow-up time was 7 (3–10) years [median (25th–75th percentile)]. Clinical manifestations at admission were used to subdivide patients with AOSD as follows: systemic inflammation (cluster 1), pure (cluster 2), and intermediate (cluster 3). Each subtype had distinct clinical manifestations and prognoses: cluster 1 (34.6%)—multiple organ manifestations, highest infection rate and mortality, and more than half of the patients with at least one relapse during follow-up; cluster 2 (21.3%)—exclusively female, rash and joint involvement, no internal organ involvement, no mortality, and most of the patients with a monocyclic course; and cluster 3 (44.1%)—less infection rate, no serious complications, and lower mortality rate. The 5- and 10-year survival rates after diagnosis were 92.4% and 86.9%, respectively. Independent risk factors for mortality were age at onset ≥50 (hazard ratio (HR): 6.78, 95% CI: 2.10–21.89), hepatomegaly (HR: 5.05, 95% CI: 1.44–17.70), infection (HR: 15.56, 95% CI: 5.88–41.20), and MAS (HR: 26.82, 95% CI: 7.52–95.60). Conclusion Three subtypes of AOSD were identified with distinct clinical manifestations and prognoses. Age at onset ≥50, hepatomegaly, infection, and MAS were prognostic factors for AOSD mortality.

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