scholarly journals NQPC-09 PROGNOSIS AND DELAYED COMPLICATIONS OF MEDULLOBLASTOMA IN KOBE CHILDREN’S HOSPITAL

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii31-ii31
Author(s):  
Atsufumi Kawamura ◽  
Junji Koyama ◽  
Nobuyuki Akutsu ◽  
Shunsuke Yamanishi

Abstract Medulloblastoma is the most common pediatric malignant tumor and about 84.2% of cases are under 15 years old. With a current combined-modality approach that includes surgical approach, chemotherapy and radiation therapy, 5 year-survival rates have become 70–85% and also the so-called long-term survivors have increased in our country. This report reviews the prognosis and delayed complications of medulloblastoma in our institute. Cases were 14 boys and 11 girls from January,2010 to May 2019. Mean age was 6.1 years old (4 months to 14 years old). 18 cases receiver gross total removal and 12 cases were high risk group. Exclude recent 4 cases, 15 cases have indicated complete reaction though 6 cases had relapsed or new tumor. The results are that 5 year-survival rates in our institute is 86.7%, but 4 cases had neuro-psychological complications. 5 cases suffered from endocrinal disorders which were not confirmed after the end of all treatments. 2 secondary cancer had appeared 5 and 8 years after the first treatments. The more the survivor in medulloblastoma cases increase, neurosurgeons have to consider long term follow up more than 5 years and pay more attention to support activity of daily life of these patients.

2020 ◽  
Vol 26 (1) ◽  
pp. 22-26 ◽  
Author(s):  
Tryggve Lundar ◽  
Bernt Johan Due-Tønnessen ◽  
Radek Frič ◽  
Petter Brandal ◽  
Paulina Due-Tønnessen

OBJECTIVEEpendymoma is the third most common posterior fossa tumor in children; however, there is a lack of long-term follow-up data on outcomes after surgical treatment of posterior fossa ependymoma (PFE) in pediatric patients. Therefore, the authors sought to investigate the long-term outcomes of children treated for PFE at their institution.METHODSThe authors performed a retrospective analysis of outcome data from children who underwent treatment for PFE and survived for at least 5 years.RESULTSThe authors identified 22 children (median age at the time of surgery 3 years, range 0–18 years) who underwent primary tumor resection of PFE during the period from 1945 to 2014 and who had at least 5 years of observed survival. None of these 22 patients were lost to follow-up, and they represent the long-term survivors (38%) from a total of 58 pediatric PFE patients treated. Nine (26%) of the 34 children treated during the pre-MRI era (1945–1986) were long-term survivors, while the observed 5-year survival rate in the children treated during the MRI era (1987–2014) was 13 (54%) of 24 patients. The majority of patients (n = 16) received adjuvant radiotherapy, and 4 of these received proton-beam irradiation. Six children had either no adjuvant treatment (n = 3) or only chemotherapy as adjuvant treatment (n = 3). Fourteen patients were alive at the time of this report. According to MRI findings, all of these patients were tumor free except 1 patient (age 78 years) with a known residual tumor after 65 years of event-free survival.Repeat resections for residual or recurrent tumor were performed in 9 patients, mostly for local residual disease with progressive clinical symptoms; 4 patients underwent only 1 repeated resection, whereas 5 patients each had 3 or more resections within 15 years after their initial surgery. At further follow-up, 5 of the patients who underwent a second surgery were found to be dead from the disease with or without undergoing additional resections, which were performed from 6 to 13 years after the second procedure. The other 4 patients, however, were tumor free on the latest follow-up MRI, performed from 6 to 27 years after the last resection. Hence, repeated surgery appears to increase the chance of tumor control in some patients, along with modern (proton-beam) radiotherapy. Six of 8 patients with more than 20 years of survival are in a good clinical condition, 5 of them in full-time work and 1 in part-time work.CONCLUSIONSPediatric PFE occurs mostly in young children, and there is marked risk for local recurrence among 5-year survivors even after gross-total resection and postoperative radiotherapy. Repeated resections are therefore an important part of treatment and may lead to persistent tumor control. Even though the majority of children with PFE die from their tumor disease, some patients survive for more than 50 years with excellent functional outcome and working capacity.


Author(s):  
Liana Tripto-Shkolnik ◽  
Yair Liel ◽  
Naama Yekutiel ◽  
Inbal Goldshtein

AbstractDenosumab discontinuation is associated with rapid reversal of bone turnover suppression and with a considerable increase in fracture risk, including a risk for multiple vertebral fractures (MVF). Long-term follow-up of patients who sustained MVF after denosumab discontinuation has not been reported. This case-series was aimed to provide a long-term follow-up on the management and outcome of denosumab discontinuers who initially presented with multiple vertebral fractures. Denosumab discontinuers were identified from a computerized database of a large healthcare provider. Baseline and follow-up clinical, laboratory, and imaging data were obtained from the computerized database and electronic medical records. The post-denosumab discontinuers MVF patients consisted of 12 women aged 71±12. Osteoporotic fractures were prevalent before denosumab discontinuation in 6 of the patients. The majority received bisphosphonates before denosumab. MVF occurred 134±76 days after denosumab discontinuation. The patients were followed for a median of 36.5 (IQR 28.2, 42.5) months after MVF. Two patients passed-away. Two patients suffered recurrent vertebral fractures. Following MVF, patients were treated inconsistently with denosumab, teriparatide, oral, and intravenous bisphosphonates, in various sequences. Two patients underwent vertebroplasty/kyphoplasty. This long-term follow-up of real-world patients with MVF following denosumab discontinuation reveals that management is inconsistent, and recurrent fractures are not uncommon. It calls for clear management guidelines for patients with MVF after denosumab discontinuation and for special attention to this high-risk group.


2021 ◽  
Vol 11 ◽  
Author(s):  
Young Dong Yu ◽  
Young Hwii Ko ◽  
Jong Wook Kim ◽  
Seung Il Jung ◽  
Seok Ho Kang ◽  
...  

AimThis study evaluated the prognosis and survival predictors for bladder urachal carcinoma (UC), based on large scale multicenter cohort with long term follow-up database.MethodsA total 203 patients with bladder UC treated at 19 hospitals were enrolled. Clinical parameters on carcinoma presentation, diagnosis, and therapeutic methods were reviewed for the primary cancer and for all subsequent recurrences. The stage of UC was stratified by Mayo and Sheldon pathological staging system. Oncological outcomes and the possible clinicopathological parameters associated with survival outcomes were investigated.ResultsThe mean age of the patients was 54.2 years. Among the total of 203 patients, stages I, II, III, and IV (Mayo stage) were 48 (23.8%), 108 (53.5%), 23 (11.4%), and 23 (11.4%), respectively. Gross hematuria and bladder irritation symptoms were the two most common initial symptoms. The mean follow-up period was 65 months, and 5-year overall survival rates (OS), cancer-specific survival rates (CSS), and recurrence-free survival rates (RFS) were 88.3, 83.1, and 63.9%, respectively. For the patients with Mayo stage ≥III, OS, CSS, and RFS were significantly decreased to 38.0, 35.2, and 28.4%, respectively. The higher pathological stage (Mayo stage ≥III, Sheldon stage ≥IIIc), positive surgical margin (PSM), and positive lymphovascular invasion (PLM) were independent predictors of shorter OS, CSS, and RFS.ConclusionThe pathological stage, PSM, and PLM were significantly associated with the survival of UC patients, emphasizing an importance of the complete surgical resection of tumor lesion.


2021 ◽  
Vol 11 (18) ◽  
pp. 8601
Author(s):  
Marco Tallarico ◽  
Gabriele Cervino ◽  
Marco Montanari ◽  
Roberto Scrascia ◽  
Emiliano Ferrari ◽  
...  

The purpose of this multicenter randomized controlled trial was to compare the clinical, radiographic, and patient-centered outcomes of early loaded mandibular overdentures deliberately placed on two or three implants. The outcomes were: implant and prosthesis success and survival rates; biological and technical complications; marginal bone loss; patient satisfaction; and periodontal parameters. The results showed no differences between the groups in any of the outcomes analyzed. With the limitations of the present study, and looking at long-term follow-up, the gold standard of prosthetic rehabilitations with attachments, in agreement with the scientific community, should prefer two non-splinted implants.


2015 ◽  
Vol 2 (4) ◽  
pp. 199-204 ◽  
Author(s):  
Paul D. Brown ◽  
S. Keith Anderson ◽  
Xiomara W. Carrero ◽  
Brian P. O'Neill ◽  
Caterina Giannini ◽  
...  

Abstract Background Pilocytic astrocytoma is a rare tumor in adults. This report is of a prospective clinical trial with long-term follow-up. Methods Between 1986 and 1994, 20 eligible adults with supratentorial pilocytic astrocytomas were enrolled in a prospective intergroup trial of radiotherapy (RT) after biopsy (3 patients) or observation after gross (11 patients) or subtotal (6 patients) resection. Results At the time of analysis (median follow-up, 20.8 years), 2 patients (10%) have died and 18 patients (90%) are alive. Neurologic and cognitive function were stable or improved over time for the majority of patients. No toxic effects of treatment or malignant transformations have been recorded at last follow-up. For the entire cohort the 20-year time to progression and overall survival rates are 95% and 90% respectively. The cause of death (2.2 and 16.1 years after enrollment) in both patients was unrelated to tumor although both were biopsy-only patients. One subtotally resected tumor progressed 1 month after enrollment requiring P32 injection into an enlarging cyst. Because of further progression this patient required RT 18 months later. This patient is alive without evidence of progression 18 years after RT. Conclusion The long-term follow-up results of this prospective trial confirm that adults with pilocytic astrocytomas have a favorable prognosis with regard to survival and neurologic function. Close observation is recommended for adults with pilocytic astrocytomas, reserving RT for salvage, as the majority remain stable after gross or subtotal resection and no adjuvant therapy.


Sarcoma ◽  
2020 ◽  
Vol 2020 ◽  
pp. 1-9 ◽  
Author(s):  
Alessandra Longhi ◽  
Marilena Cesari ◽  
Massimo Serra ◽  
Erminia Mariani

Background. In relapsed osteosarcoma, the 5-yr postrelapse disease-free survival (PRDFS) rate after the second relapse is <20%. In June 2007, a randomized study was started comparing oral etoposide vs Viscum album fermentatum Pini (an extract derived from the parasitic plant Viscum album L., European mistletoe) as maintenance therapy in patients with metastatic osteosarcoma in complete surgical remission after the second relapse. The primary endpoint was the PRDFS rate at 12 months (compared to the historical control rate). This is a long-term updated result. Patients and Methods. 10 patients received oral etoposide 50 mg/m2 daily for 21 days every 28 days for 6 months, and 9 patients received Viscum album fermentatum Pini 3 times/wk subcutaneously for 1 year. The study closed early in July 2011 due to insufficient recruitment. Lymphocyte subpopulations were analyzed at T0, T3, T6, T9, and T12 months. Results. On 30 June 2019, at a median follow-up ITT of 83 months (range 3–144 ms), a median PRDFS of 106 ms (2–144) was observed in the Viscum arm with 5/9 patients who never relapse vs a PRDFS of 7 months (3–134) in the etoposide arm (all patients in the Etoposide arm relapsed) (hazard ratio HR = 0.287, 95% CI: 0.076–0.884, p=0.03). Model forecast 10-yr overall survival rates as 64% in the Viscum arm and 33% in the etoposide arm. Lymphocyte subpopulation counts (CD3, CD4, and CD56) showed an increase in the Viscum arm while a decrease was observed in the etoposide arm during treatment. Conclusions. After 12 years from the start of the trial, the patients in the Viscum arm continue to show a considerably longer PRDFS compared to oral etoposide, and a trend for an advantage in OS is evident even if the number of treated patients is too small to draw conclusions. Viscum as maintenance treatment after complete surgical remission in relapsed osteosarcoma should be further investigated and compared with other drugs.


1991 ◽  
Vol 75 (4) ◽  
pp. 575-582 ◽  
Author(s):  
Mark G. Belza ◽  
Sarah S. Donaldson ◽  
Gary K. Steinberg ◽  
Richard S. Cox ◽  
Philip H. Cogen

✓ Seventy-seven patients presenting with medulloblastoma between 1958 and 1986 were treated at Stanford University Medical Center and studied retrospectively. Multimodality therapy utilized surgical extirpation followed by megavoltage irradiation. In 15 cases chemotherapy was used as adjunctive treatment. The 10- and 15-year actuarial survival rates were both 41% with an 18-year maximum follow-up period (median 4.75 years). There were no treatment failures after 8 years of tumor-free survival. Gross total removal of tumor was achieved in 22 patients (32%); the surgical mortality rate was 3.9%. No significant difference was noted in the incidence of metastatic disease between shunted and nonshunted patients. The classical form of medulloblastoma was present in 67% of cases while the desmoplastic subtype was found in 16%. Survival rates were best for patients presenting after 1970, for those with desmoplastic tumors, and for patients receiving high-dose irradiation (≥ 5000 cGy) to the posterior fossa. Although early data on freedom from relapse suggested a possible beneficial effect from chemotherapy, long-term follow-up results showed no advantage from this modality of treatment. The patterns of relapse and survival were examined; 64% of relapses occurred within the central nervous system, and Collins' rule was applicable in 83% of cases beyond the period of risk. Although patients treated for recurrent disease could be palliated, none were long-term survivors. The study data indicate that freedom from relapse beyond 8 years from diagnosis can be considered as a cure in this disease. Long-term follow-up monitoring is essential to determine efficacy of treatment and to assess survival patterns accurately.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 846-846
Author(s):  
Vikram Mathews ◽  
Biju George ◽  
Kavitha M Lakshmi ◽  
Auro Viswabandya ◽  
Ezhilarasi Chendamarai ◽  
...  

Abstract Abstract 846 We had previously reported a well tolerated regimen using single agent arsenic trioxide (ATO) (Blood 2006:107; 2627) leading to durable remissions in patients with newly diagnosed acute promyelocytic leukemia (APL). Briefly, the regimen consisted of ATO (10mg/day for adults and 0.15mg/kg/day for pediatric patients) for up to 60 days in induction; this was followed by a 28 day consolidation after a 4 week break. Four weeks after completion of consolidation, patients received ATO for 10 days/month for 6 months. A concern with the previous report was the relatively short duration of follow up. Here we report the long term follow-up data of the same cohort. As previously reported, 72 newly diagnosed cases of APL were enrolled. 62 patients (86.1%) achieved hematological remission. The remaining died prior to achieving remission. There were no primary induction failures. Twenty two (30.6%) of these patients were considered good risk group (WBC count at diagnosis <5×109/L and a platelet count >20×109/L), the rest were considered high risk. Since publication of the last report an additional 7 patients have relapsed to give a total of 13 relapses, 2 were in the good risk group and the remaining 11 in the high risk group. The relapses in the good risk group were salvaged with an autologous SCT and have durable continued second remissions. The median time to relapse was 1.5 years. Five (38.52%) of these relapses occurred beyond 2 years and included both relapses in the good risk group. At a median follow-up of 58 months the 5-year Kaplan-Meier overall survival (OS), event free survival (EFS) and disease free survival (DFS) of the entire cohort was 74.22±5.26%, 68.93±5.52% and 80.00±5.17% respectively. The 5-year OS and EFS of the good risk and high risk group was 100±00% vs. 63.30±6.9% and 90.00±6.71% vs. 59.66±6.99% respectively. Beyond induction, all deaths followed relapse of disease. There were no second malignancies reported. Besides the previously reported toxicities, which were mild and transient in most cases, there were no new toxicities that were reported on continued follow up of these cases. Since completion of therapy, in spite of counseling and advising against pregnancy, 3 males and 4 females in the reproductive age group have had 8 normal children. No abortions, still births or fetal defects were reported among patients in the reproductive age group in this cohort. Hair and nail samples from 5 cases that had completed maintenance therapy more than 24 months earlier have been collected for analysis, the results of which are awaited. At our center the cost of administering this regimen is a quarter of that of a conventional ATRA plus anthracycline based regimen. Additionally, after the initial induction therapy the rest of the treatment did not require hospital admission nor did it result in any Grade III/IV hematological toxicity. Single agent ATO based regimen as reported previously is well tolerated, results in durable remissions and does not have any significant late side effects. In the good risk group it is associated with excellent clinical outcomes while in the high risk group additional interventions are probably required to reduce the risk of late relapses. In a resource constrained environment it is probably the best option. Disclosures: No relevant conflicts of interest to declare.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 9005-9005 ◽  
Author(s):  
A. C. van Akkooi ◽  
P. Rutkowski ◽  
I. M. van der Ploeg ◽  
C. A. Voit ◽  
H. J. Hoekstra ◽  
...  

9005 Background: Many studies have identified Sentinel Node (SN) tumor burden as a prognostic factor for additional non- SN (NSN) positivity and / or disease-free (DFS) and melanoma specific survival (MSS). It remains unclear if pts with minimal SN tumor burden can safely be managed without Completion Lymph Node Dissection (CLND). Pts with minimal SN tumor burden might be at risk for late recurrences (> 5 years). Methods: Slides of 595 SN positive patients were reviewed for this pan-European study collaboration in 5 major centers. Slides were reviewed for the microanatomic location and SN tumor burden according to the Rotterdam Criteria (< 0.1mm, 0.1 - 1.0mm and > 1.0 mm) for the maximum diameter of the largest metastasis. MSS, DFS and distant metastasis-free survival (DMFS) rates were calculated, as was NSN positivity. Results: In 595 SN positive pts, the mean and median Breslow thickness was 4.73 and 3.5 mm. Ulceration was present in 51% of melanomas. 67 pts had metastases < 0.1 mm (11%), 226 pts (38%) had 0.1 - 1.0 mm metastases and 302 pts had metastases > 1.0 mm (51%). Mean and median follow-up was 48 and 40 months for all patients (range 1 - 172). Patients with metastases < 0.1 mm had mean and median follow-up of 61 and 57 months, 46% (31pts) had follow up > 5 years and 25% (17 pts) had follow-up longer than 80 months (range 3 - 132). 5-year MSS rates were 94% for metastases < 0.1 mm, 70% for 0.1 - 1.0 mm and 57% for > 1.0 mm (p<0.001). 5-year DMFS rate was 91% for metastases < 0.1 mm. NSN positivity occurred in 5% of < 0.1mm, 17% of 0.1 - 1.0 and 29% of metastases > 1.0 mm (p<0.001). Conclusions: This large multicenter experience (n=595) has demonstrated that long-term follow-up of melanoma patients with minimal SN tumor burden (< 0.1 mm) indicates very low relapse rates and excellent MSS, seemingly identical to SN negative patients. With prolonged follow-up, an increase in the occurrence of relapses of any kind between 5 and 10 years follow up has not been identified, and excellent 10-year survival rates are expected. No significant financial relationships to disclose.


2006 ◽  
Vol 5 (4) ◽  
pp. 313-319 ◽  
Author(s):  
Hideki Sudo ◽  
Manabu Ito ◽  
Kuniyoshi Abumi ◽  
Yoshihisa Kotani ◽  
Tatsuto Takeuchi ◽  
...  

Object As increasing numbers of patients receive long-term hemodialysis, the number of reports regarding hemodialysis-related cervical spine disorders has also increased. However, there have been few reports summarizing the surgical results in patients with these disorders. The objective of this study was to evaluate the long-term follow up and clinical results after surgical treatment of cervical disorders in patients undergoing hemodialysis. Methods Seventeen patients in whom surgery was performed for cervical spine disorders while they received long-term hemodialysis therapy were enrolled in this study. Of these, 15 underwent follow-up review for more than 3 years after surgery, and these represent the study population. The remaining two patients died of postoperative sepsis. The average follow-up period was 120 months. Five patients without spinal instability underwent spinal cord decompression in which bilateral open-door laminoplasty was performed. Ten patients with destructive spondyloarthropathy (DSA) underwent reconstructive surgery involving pedicle screw (PS) fixation. In eight patients in whom posterior instrumentation was placed, anterior strut bone grafting was performed with autologous iliac bone to treat anterior-column destruction. Marked neurological recovery was obtained in all patients after the initial surgery. In the mobile segments adjacent to the site of previous spinal fusion, the authors observed progressive destructive changes with significant instability in four patients (40%) who underwent circumferential spinal fusion. No patients required a second surgery after laminoplasty for spinal canal stenosis without DSA changes. Conclusions Cervical PS-assisted reconstruction provided an excellent fusion rate and good spinal alignment. During the long-term follow-up period, however, some cases required extension of the spinal fusion due to the destructive changes in the adjacent vertebral levels. Guidelines or recommendations to overcome these problems should be produced to further increase the survival rates of patients undergoing hemodialysis.


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