Cranial Chordomas: Clinical Presentation and Results of Operative and Radiation Therapy in Twenty-six Patients

Neurosurgery ◽  
1985 ◽  
Vol 17 (5) ◽  
pp. 703-710 ◽  
Author(s):  
Corey Raffel ◽  
Donald C. Wright ◽  
Philip H. Gutin ◽  
Charles B. Wilson

Abstract The clinical presentation and the results of operative and radiation therapy in a series of 26 patients with cranial chordomas seen at the University of California, San Francisco, between 1940 and 1984 are reviewed. There were 14 men and 12 women, with a mean age of 39.6 years. Six patients had chondroid chordomas. The most common presenting symptoms were headache and diplopia, and the most common presenting sign was extraocular palsy. Fifty-three operations directed at removal of the tumor were performed. Twenty-three patients received various forms of radiation therapy postoperatively, including conventional external beam therapy, heavy charged particles, and interstitial implants. The average length of follow-up is 5.6 years. Eleven of 26 patients have died; the mean duration of survival in this group, excluding 1 perioperative death, was 4 years and 2 months. Although the average survival time of 6 years and 4 months was the same in patients with typical chordomas (excluding the perioperative death and 1 patient lost to follow-up) and in those with the chondroid variant, all of the latter are still alive, whereas more than half of the patients with typical chordoma have died.

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Qionghua Chen ◽  
Yuelin Shen ◽  
Hui Xu ◽  
Xiaolei Tang ◽  
Haiming Yang ◽  
...  

Abstract Background Since public awareness of cystic fibrosis (CF) has increased, more children have been diagnosed with CF in China. This study aimed to investigate medical and other challenges faced by pediatric CF patients in China. Method Treatments and treatment outcomes were retrospectively analyzed for 46 pediatric CF patients diagnosed from August 2009 to June 2019. Pre- and post-treatment results were compared using independent samples t-test. Results Of 46 pediatric CF study patients, four died and five were lost to follow-up. Thirty-seven patients were monitored for 0.03 to 9.21 years; patients exhibited fewer attacks of respiratory tract infections after diagnosis (4.49 ± 2.13 episodes/year before diagnosis vs 1.97 ± 1.87 times/year after 1-year treatment, p < 0.05), significantly reduced sputum production and experienced 1.62 ± 1.71 exacerbations/year. Patient mean body mass index was 16.87 ± 3.53 and pancreatic malfunction persisted in 15 patients. For 17 children, no significant differences in lung function were found at follow-up as compared to lung function at diagnosis (FEV1: 82.45% ± 16.56% vs 75.26% ± 22.34%, FVC: 87.18% ± 13.64% vs 86.99% ± 19.95%, FEF75%: 46.51% ± 28.78% vs 36.63% ± 24.30%, P = 0.27, 0.97, 0.20, respectively). Pseudomonas aeruginosa (17/27) and bronchiectasis (22/22) were found during follow-up evaluation. Twenty-four patients (64.8%) maintained good adherence to therapies. Overall, azithromycin and tobramycin treatments were administered for 0.5–62 months and 0.5–48 months, respectively, and triggered no obvious adverse reactions. Conclusion No obvious declines in clinical presentation or lung function were found in Chinese pediatric CF patients after receiving standard therapeutic and active treatments, although malnutrition and low compliance were persistent challenges.


2021 ◽  
pp. 1-7
Author(s):  
Kimberly L. Yan ◽  
Nerissa U. Ko ◽  
Steven W. Hetts ◽  
Shantel Weinsheimer ◽  
Adib A. Abla ◽  
...  

Background: Sporadic brain arteriovenous malformations (BAVM) are a major cause of hemorrhagic stroke in younger persons. Prior studies have reported contradictory results regarding the risk of hemorrhage during pregnancy, and there are no standard guidelines for the management of pregnant women who present with BAVM rupture. The purpose of this study is to describe maternal and fetal outcomes and treatment strategies in patients with BAVM hemorrhage during pregnancy. Methods: We performed a retrospective review of the University of California, San Francisco Brain AVM Project database for female patients who were pregnant at the time of BAVM hemorrhage between 2000 and 2017. Clinical and angiographic characteristics at presentation, BAVM treatment, and maternal outcomes using modified Rankin scale (mRS) score at presentation and 2-year follow-up were recorded. Fetal outcomes were abstracted from medical records and maternal reports. Results: Sixteen patients presented with BAVM hemorrhage during pregnancy, 81% (n = 13) of whom were in their second or third trimester. Three patients (19%) who were in their first trimester terminated or miscarried pregnancy prior to BAVM intervention. Of the remaining 13 patients, 77% (n = 10) received emergent BAVM treatment at time of hemorrhage prior to delivery, and 85% of patients achieved BAVM obliteration and good maternal outcomes (mRS 0–2) at 2-year follow-up. All patients had uncomplicated deliveries (69% cesarean and 23% vaginal) with no reports of postnatal cognitive or developmental delays in infants at 2-year follow-up. Conclusions: Our study shows good long-term maternal and fetal outcomes in ruptured BAVM patients presenting during pregnancy, the majority who received BAVM interventional treatment prior to delivery.


1979 ◽  
Vol 87 (3) ◽  
pp. 359-363 ◽  
Author(s):  
John V. O'Neill ◽  
Arthur H. Katz ◽  
Emanuel M. Skolnik

Radiation therapy has proved to be a valuable modality in the management of patients with nasopharyngeal tumors. Routine follow-up of patients in the tumor clinic of the University of Illinois Eye and Ear Infirmary appeared to indicate an increasing incidence of otologic pathology. For this reason, a retrospective study focusing on the otologic findings in patients whose radiation therapy for nasopharyngeal tumors included the external auditory canal, middle ear space, or eustachian tube was performed. The results of this study are discussed in relationship to total radiation dosage, time of survival after therapy, and pretreatment otologic status. The complications covered a spectrum from intermittent serous otitis media to osteoradionecrosis of the external auditory canal. Possible contributing factors are discussed, and preventive measures are suggested.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 11569-11569
Author(s):  
Edwards Kasonkanji ◽  
Yolanda Gondwe ◽  
Morgan Dewey ◽  
Joe Gumulira ◽  
Matthew Painschab ◽  
...  

11569 Background: Kaposi sarcoma (KS) is the leading cancer in Malawi (34% of cancers). Outside of clinical trials, prospective KS studies from sub-Saharan Africa (SSA) are few and limited by loss to follow up. We conducted a prospective KS cohort study of standard of care bleomycin/vincristine (BV) at Lighthouse HIV clinic, in Lilongwe, Malawi. Methods: We enrolled pathologically confirmed, newly diagnosed, HIV+ KS patients from Feb 2017 to Jun 2019. We collected clinical and treatment characteristics, toxicity, and outcomes of KS with follow-up censored Jun 2020. Patients were treated with bleomycin (25 mg/m2) and vincristine (0.4 mg/m2) every 14 days for a planned maximum of 16 cycles. STATA v13.0 was used to calculate descriptive statistics and Kaplan Meier survival analysis. Toxicity was graded using NCI CTCAE v5.0. Results: We enrolled 138 participants, median age 36 (IQR 32-44) and 110 (80%) male. By ACTG staging, 107 (78%) were T1 (tumour severity), 46 (33%) were S1 (illness severity) and 46 (33%) had Karnofsky performance status ≤70. Presenting symptoms included edema in 69 (53%), visceral disease in 9 (7%), and oral involvement in 43 (33%). Prior to KS diagnosis, 70 (51%) participants were aware of being HIV+ for median 17 months (IQR 6-60) and had been on ART for median 16 months (IQR 6-60). Median CD4 count was 197 (IQR 99-339), median HIV-viral load was 2.6 log copies/mL (IQR 1.6 – 4.8) and 57% were HIV-suppressed ( < 1000 HIV copies/ml). The median number of cycles was 16 (IQR 7-16). 62 (45%) participants missed at least one dose due to stock out. Amongst patients with missed doses, the median number was 3 (IQR 2-4) for bleomycin and 2 (IQR 1-3) for vincristine. 14 (10%) participants experienced at least one reduced dose due to toxicity. 5 (4%) participants suffered grade ≥3 anaemia, 13 (9%) grade ≥3 neutropenia, and one participant had grade 4 bleomycin-induced dermatitis. There was no reported grade ≥3 bleomycin lung toxicity or vincristine-induced neuropathy. Of 115 evaluable participants, responses at the end of therapy were: complete response in 52 (45%), partial response in 27 (23%) stable disease in 5 (4%), and progressive disease in 31 (28%). Median duration of follow-up was 20 months. At censoring, 69 (50%) were alive, 36 (26%) dead, and 33 (24%) lost to follow-up. Overall survival is shown Table as crude and worst-case scenario; worst-case assumes all participants lost to follow up died. Conclusions: Here, we present one of the most complete characterizations of KS presentation and treatment from SSA. As in other studies from the region, the majority of patients presented with advanced disease, chemotherapy stock-outs and loss to follow up were common, and mortality was high. Studies are planned to understand the virologic characteristics, improve therapies, and better implement existing therapies.[Table: see text]


2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S524-S525
Author(s):  
David Zhang ◽  
Julia Rosebush ◽  
Palak Bhagat ◽  
Allison Nelson ◽  
Veena Ramaiah ◽  
...  

Abstract Background In July 2017, The University of Chicago Comer Children’s Hospital Emergency Department (ED) transitioned from a 5-day to a 28-day HIV nPEP (non-occupational post-exposure prophylaxis) dispensation model in an effort to increase adherence. Anecdotal reports of patients lost to follow-up after ED discharge called into question the utility and cost-effectiveness of this practice. We analyzed HIV nPEP follow-up rates in our clinic, explored reasons for nonadherence, and performed basic cost-savings analyses to inform potential changes to our dispensation model. Methods A retrospective review of both electronic health and pharmacy records was conducted for patients prescribed 28-days of HIV nPEP in the ED and scheduled for outpatient follow-up in Pediatric ID clinic from July 2017-June 2019. Clinic provider documentation of nPEP adherence and reasons for nonadherence were examined. Patients were given an initial dose of nPEP regimen in the ED and provided all subsequent doses to complete at home. Using average wholesale price (AWP), we calculated the total cost of each regimen and potential savings if a shorter duration of HIV nPEP supply was dispensed. Results 50 patients received a 28-day supply of HIV nPEP. Please refer to Table 1 regarding baseline patient characteristics. Of these, only 19 (38%) patients had documented outpatient follow-up after nPEP initiation. Median time to follow-up was 6 days (IQR: 3.0-9.0 days). Of the 19 patients with follow-up, 3 admitted to medication non-adherence. Although side effects were elicited in a total of 9 patients (18%), only 1 cited medication intolerance as the reason for discontinuing their nPEP. Given the relatively short time to follow-up, a potential savings of $1720-2211/patient could be achieved if a 10-14 day supply was dispensed. Conclusion Outpatient follow-up after 28-day HIV nPEP dispensation in our ED was &lt; 40%, calling into question the cost-effectiveness of this dispensation model. While our current practice alleviates nPEP interruption due to potential insurance issues and pick-up delays, follow-up and adherence are not assured. The significant cost-savings with a shorter supply at the outset may encourage more robust follow-up and adherence. Disclosures All Authors: No reported disclosures


2007 ◽  
Vol 106 (5) ◽  
pp. 839-845 ◽  
Author(s):  
Jason Sheehan ◽  
Chun Po Yen ◽  
Yasser Arkha ◽  
David Schlesinger ◽  
Ladislau Steiner

Object Trigeminal schwannomas are rare intracranial tumors. In the past, resection and radiation therapy were the mainstays of their treatment. More recently, neurosurgeons have begun to use radiosurgery in the treatment of trigeminal schwannomas because of its successful use in the treatment of vestibular schwannomas. In this article the authors evaluate the radiological and clinical outcomes in a series of patients in whom Gamma Knife surgery (GKS) was used to treat trigeminal schwannomas. Methods Twenty-six patients with trigeminal schwannomas underwent GKS at the University of Virginia Lars Lek-sell Gamma Knife Center between 1989 and 2005. Five of these patients had neurofibromatosis and one patient was lost to follow up. The median tumor volume was 3.96 cm3, and the mean follow-up period was 48.5 months. The median prescription radiation dose was 15 Gy, and the median prescription isodose configuration was 50%. There was clinical improvement in 18 patients (72%), a stable lesion in four patients (16%), and worsening of the disease in three patients (12%). On imaging, the schwannomas shrank in 12 patients (48%), remained stable in 10 patients (40%), and increased in size in three patients (12%). These results were comparable for primary and adjuvant GKSs. No tumor growth following GKS was observed in the patients with neurofibromatosis. Conclusions Gamma Knife surgery affords a favorable risk-to-benefit profile for patients harboring trigeminal schwannomas. Larger studies with open-ended follow-up review will be necessary to determine the long-term results and complications of GKS in the treatment of trigeminal schwannomas.


2007 ◽  
Vol 121 (8) ◽  
pp. 725-735 ◽  
Author(s):  
L R Lustig ◽  
J Sciubba ◽  
M J Holliday

AbstractObjective:To evaluate the clinical presentation and outcomes of treatment for patients with chondrosarcomas involving the skull base and temporal bone.Study design:Retrospective review.Setting:Tertiary medical centre.Patients:Cases of histologically confirmed chondrosarcoma involving the skull base and temporal bones.Intervention:Surgery.Main outcome measures:Demographic features of presenting patients; presenting symptoms and signs; surgical approach employed; use of post-operative radiation therapy; histological grade of tumour; and interval of post-operative follow up.Results: Twelve patients were identified with chondrosarcomas involving the skull base, with post-operative follow up ranging from three to 33 years. The average age at presentation was 42 years. The most common presenting symptoms were diplopia, decreased visual acuity and headaches. Five of the 12 patients required multiple surgical procedures.Conclusions:Patients with chondrosarcoma involving the skull base and temporal bone may present in a variety of ways. Surgical resection, even subtotal, in combination with radiation therapy, can often provide good tumour control over many years for these rare tumours.


2011 ◽  
Vol 29 (7_suppl) ◽  
pp. 215-215
Author(s):  
M. Mehra ◽  
Y. Wu ◽  
R. Dhawan

215 Background: Docetaxel is standard of care among late-stage prostate cancer patients. We analyzed patterns of health care resource utilization (RU) among patients before and after exposure to docetaxel using a large commercial claims database. Methods: A random sample of patients (N = 336) with a diagnosis of prostate cancer (ICD 9 code: 185.X) and a claim for docetaxel (2003–2009) was identified from the PharMetrics database, a nationally representative, non-payer-owned integrated commercial U.S. claims database. All patients had ≥ 12 months of enrollment prior to initiation of docetaxel. Patients were followed from their first docetaxel claim until lost to follow-up or June 30, 2009 (censored). RU was defined as all-cause hospitalization, ER, physician, and ambulatory visits. Incidence rates were derived. Results: Mean age of patients was 67.9 years (SD 10.6); mean number of docetaxel prescriptions was 9.9 (SD 10.3). Mean time to study end/lost to follow-up was 15.41 (SD 12.49) months from the index date. The table shows health care RU for the 12 months before, and over the follow-up period after docetaxel initiation. Hospitalizations, ER, physician, and ambulatory visits were significantly higher in the follow-up period. The average length of hospital stay was significantly longer after docetaxel treatment (8.2 vs 5.5 days). Prior to docetaxel, two-thirds of the patients were on hormonal therapy; 51% on analgesics, and 31% on bisphosphonates. After docetaxel, the proportions were 62%, 58%, and 54%, respectively. Conclusions: The significantly higher RU with disease progression in prostate cancer patients suggests a need for new treatment options that can effectively manage and improve patient outcomes. [Table: see text] [Table: see text]


2019 ◽  
Vol 50 (03) ◽  
pp. 188-192
Author(s):  
Saša Ilovar ◽  
Mirjana Benedik ◽  
Tina Vesnaver ◽  
Damjan Osredkar

Background Brain aneurysms are rare in the pediatric population. The diagnosis of a brain aneurysm in a child may be difficult because of its infrequency and often subtle or nonspecific clinical presentation. Endovascular therapy and microsurgical treatment are increasingly used approaches in treating children, possibly contributing to favorable outcomes if patients are treated in a timely manner. Objective We were interested in the clinical presentation, symptoms, diagnostics, treatment, and follow-up of pediatric patients with brain aneurysms in Slovenia. Methods This was a retrospective review of medical documentation of children with intracranial aneurysms treated at the University Children's Hospital in Ljubljana, Slovenia, from January 1998 to December 2017. Results We identified a cohort of eight children (median age: 14.9 years; range: 2.8–17.7). The estimated incidence of pediatric brain aneurysms in Slovenia is 0.12/100,000 children per year. We observed a male predominance (1.7:1). Half of the patients presented with acute onset of neurologic symptoms and three with subarachnoid hemorrhage. One of the patients had a related stroke. The presenting symptoms were tonic–clonic seizures, hemiparesis, paresthesias, speech disturbance, and cranial nerve palsy. The other half of aneurysms were identified incidentally. Five patients had anterior circulation aneurysms; the most prevalent location was the internal carotid artery. One patient was treated with surgical procedures, four patients were treated with endovascular procedures, and three patients were treated conservatively. Outcome was excellent in all patients. Conclusion Endovascular interventions and microsurgical procedures appear to be safe and effective in the treatment of brain aneurysms in the pediatric population. Asymptomatic patients with brain aneurysms need close follow-up.


2001 ◽  
Vol 19 (20) ◽  
pp. 4029-4036 ◽  
Author(s):  
William M. Mendenhall ◽  
Robert J. Amdur ◽  
Christopher G. Morris ◽  
Russell W. Hinerman

PURPOSE: The end results after radiation therapy for T1-T2N0 glottic carcinoma vary considerably. We analyze patient-related and treatment-related parameters that may influence the likelihood of cure. PATIENTS AND METHODS: Five hundred nineteen patients were treated with radiation therapy and had follow-up for ≥ 2 years. Three patients who were disease-free were lost to follow-up at 7 months, 21 months, and 10.5 years. No other patients were lost to follow-up. RESULTS: Local control rates at 5 years after radiation therapy were as follows: T1A, 94%; T1B, 93%; T2A, 80%; and T2B, 72%. Multivariate analysis of local control revealed that the following parameters significantly influenced this end point: overall treatment time (P < .0001), T stage (P = .0003), and histologic differentiation (P = .013). Patients with poorly differentiated cancers fared less well than those with better differentiated lesions. Rates of local control with laryngeal preservation at 5 years were as follows: T1A and T1B, 95%; T2A, 82%; and T2B, 76%. Cause-specific survival rates at 5 years were as follows: T1A and T1B, 98%; T2A, 95%; and T2B, 90%. One patient with a T1N0 cancer and three patients with T2N0 lesions experienced severe late radiation complications. CONCLUSION: Radiation therapy cures a high percentage of patients with T1-T2N0 glottic carcinomas and has a low rate of severe complications. The major treatment-related parameter that influences the likelihood of cure is overall treatment time.


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