PATH-08. PROGNOSTIC IMPLICATIONS FROM LONG-TERM SURVIVORS (LTS) OF GLIOBLASTOMA

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi116-vi116
Author(s):  
Nicole Briceno ◽  
Zied Abdullaev ◽  
Elizabeth Vera ◽  
Anna Choi ◽  
Alexa Christ ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
Subtotal Resection ◽  
Specific Gene ◽  
Tumor Biopsy ◽  
Term Survival ◽  
Prognostic Features ◽  
Molecular Features ◽  
Mgmt Promoter ◽  
Long Term Survivors

Abstract Glioblastoma (GBM) is the most aggressive primary brain malignancy with < 45% living a year beyond diagnosis which drops to 7% at five years. However, there have been reports of long-term survivors (LTS) living three to ten years beyond diagnosis. Few studies have reported on molecular factors in tumors from LTS cohorts. We identified GBM (IDH1/2 wildtype) patients living at least 3 years post diagnosis (N=25), including 16 with pre-treatment tumor tissue, from our Natural History Study. Available pre- or post-treatment tumors were analyzed with targeted panel sequencing and methylation analysis for classification, MGMT promoter status and copy number changes. Classical clinical prognostic features such as limited resection or older age did not preclude long-term survival as patients with tumor biopsy (n=1) or subtotal resection (n= 5) and patients > 60 were included in the LTS cohort. Furthermore, tumors with molecular features typically associated with poor prognosis were also in this GBM LTS group. MGMT promoter was unmethylated in 17% of tumors; EGFRvIII mutation in 13%, EGFR amplification in 33%, CDKN2A homozygous loss in 30% and complete chromosome 7 gain with 10 loss in 55%. Additionally, the methylation classifier found a higher-than-expected incidence of mesenchymal tumors (29%) and RTK II (57%). Tumors had a higher percent of TP53 mutations (44%) but lower pTERT (76%) compared to TCGA. These data suggest an individual patient’s prognosis cannot easily be predetermined based on classical clinical and molecular data. This underscores the need for further analyses to discover additional factors leading to their unexpected, prolonged survival and elucidate the role of factors typically associated with poor prognosis. Future work will include RNA-sequencing and germline whole genome sequencing to determine tumor specific gene expression and identify any possible genomic alterations that confer improved survival.

scholarly journals Actual Long-Term Survival After Resection of Stage III Soft Tissue Sarcoma

2020 ◽  
Author(s):  
Do Weon Lee ◽  
Han-Soo Kim ◽  
Ilkyu Han
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Tumor Size ◽  
Poor Prognosis ◽  
Histological Grade ◽  
Stage Iii ◽  
Term Survival ◽  
Long Term Survival ◽  
Long Term Survivors

Abstract Background: Actuarial survival based on the Kaplan–Meier method can overestimate actual long-term survival, especially among those with factors of poor prognosis. Patients with American Joint Committee on Cancer stage III soft tissue sarcoma (STS) represent a subset with a high risk of STS-specific mortality. Therefore, we aimed to characterize the clinicopathological characteristics associated with actual long-term survival in patients with stage III STS.Methods: We retrospectively reviewed 116 patients who underwent surgical resection for stage III STS with curative intent between March 2000 and December 2013. Long-term survivors (n = 61), defined as those who survived beyond 5 years, were compared with short-term survivors (n = 36), who died of STS within 5 years.Results: Multivariate logistic regression analyses showed that a tumor size <10 cm [odds ratio (OR) 3.95, p = 0.047], histological grade of 2 (OR 8.12, p = 0.004), and American Society of Anesthesiologists (ASA) score of 1 (OR 11.25, p = 0.001) were independently associated with actual 5-year survival. However, 66% of the long-term survivors exhibited factors of poor prognosis: 36% had a tumor size >10 cm and 48% had a histological grade of 3. Leiomyosarcoma (3 of 10) was negatively associated with actual long-term survival.Conclusions: Actual 5-year survival after resection of stage III STS was associated with tumor size, histological grade, and ASA score. However, majority of the actual 5-year survivors exhibit factors of poor prognosis, suggesting that resection should be offered for a chance of long-term survival in these patients.

scholarly journals Actual long-term survival after resection of stage III soft tissue sarcoma

BMC Cancer ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Do Weon Lee ◽  
Han-Soo Kim ◽  
Ilkyu Han
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Tumor Size ◽  
Poor Prognosis ◽  
Histological Grade ◽  
Stage Iii ◽  
Term Survival ◽  
Long Term Survival ◽  
Long Term Survivors

Abstract Background Actuarial survival based on the Kaplan–Meier method can overestimate actual long-term survival, especially among those with factors of poor prognosis. Patients with American Joint Committee on Cancer stage III soft tissue sarcoma (STS) represent a subset with a high risk of STS-specific mortality. Therefore, we aimed to characterize the clinicopathological characteristics associated with actual long-term survival in patients with stage III STS. Methods We retrospectively reviewed 116 patients who underwent surgical resection for stage III STS with curative intent between March 2000 and December 2013. Long-term survivors (n = 61), defined as those who survived beyond 5 years, were compared with short-term survivors (n = 36), who died of STS within 5 years. Results Multivariate logistic regression analyses showed that a tumor size < 10 cm [odds ratio (OR) 3.95, p = 0.047], histological grade of 2 (OR 8.12, p = 0.004), and American Society of Anesthesiologists (ASA) score of 1 (OR 11.25, p = 0.001) were independently associated with actual 5-year survival. However, 66% of the long-term survivors exhibited factors of poor prognosis: 36% had a tumor size > 10 cm and 48% had a histological grade of 3. Leiomyosarcoma (3 of 10) was negatively associated with actual long-term survival. Conclusions Actual 5-year survival after resection of stage III STS was associated with tumor size, histological grade, and ASA score. However, majority of the actual 5-year survivors exhibit factors of poor prognosis, suggesting that aggressive treatment should be offered for a chance of long-term survival in these patients.

scholarly journals Atypical anaplastic astrocytoma with unique molecular features and diffuse leptomeningeal spread in a child with long-term survival

2019 ◽  
Vol 12 (2) ◽  
pp. e228153 ◽  
Author(s):  
Yasmin Aghajan ◽  
Denise M Malicki ◽  
Michael L Levy ◽  
John Ross Crawford
Keyword(s):  
Anaplastic Astrocytoma ◽  
Histological Grade ◽  
Extent Of Resection ◽  
Subtotal Resection ◽  
Term Survival ◽  
Craniospinal Radiation ◽  
Molecular Features ◽  
Central Nervous System Tumours ◽  
Gene Panel Sequencing

Paediatric high-grade gliomas, including glioblastoma and anaplastic astrocytoma, make up 8%–12% of paediatric central nervous system tumours 1 and have poor prognosis, with 2-year survival less than 30% 2 and overall survival less than 10%. The only known prognostic factors in this population include extent of resection and tumour histological grade. We present the case of a 9-year-old boy with disseminated anaplastic astrocytoma treated with subtotal resection, craniospinal radiation and temozolomide, with 8-year survival despite metastatic disease at presentation and subtotal resection. Next generation cancer gene panel sequencing revealed an usual pattern of 12 amplifications and four mutations not previously described.

Aggressive Treatment in Glioblastoma: What Determines the Survival of Patients?

2020 ◽  
Author(s):  
Lei Yu ◽  
Guozhong Zhang ◽  
Songtao Qi
Keyword(s):  
Overall Survival ◽  
Malignant Gliomas ◽  
Epidemiological Studies ◽  
Control Cohort ◽  
Poor Overall Survival ◽  
Term Survival ◽  
Significant Difference ◽  
Positive Rate ◽  
Long Term Survivors

Abstract Background and Study Aims The exact reason of long-term survival in glioblastoma (GBM) patients has remained uncertain. Molecular parameters in addition to histology to define malignant gliomas are hoped to facilitate clinical, experimental, and epidemiological studies. Material and Methods A population of GBM patients with similar clinical characteristics (especially similar resectability) was reviewed to compare the molecular variables between poor (overall survival [OS] < 18 months, control cohort) and long-term survivors (overall survival > 36 months, OS-36 cohort). Results Long-term GBM survivors were younger. In the OS-36 cohort, the positive rate of isocitrate dehydrogenase (IDH) mutation was very low (7.69%, 3/39) and there was no statistical difference in OS between IDH mutant and wild-type patients. The results of 1p/19q codeletions are similar. Besides, there were no significant difference in MGMT promoter methylation, telomerase reverse transcriptase (TERT) promoter mutation, and TP53 mutations between OS-36 cohort and control cohort. Conclusions No distinct markers consistently have been identified in long-term survivors of GBM patients, and great importance should be attached to further understand the biological characteristics of the invasive glioma cells because of the nature of diffuse tumor permeation.

scholarly journals DIPG-72. LONG-TERM SURVIVAL OF A CLASSIC DIFFUSE INTRINSIC PONTINE GLIOMA TREATED WITH NIMOTUZUMAB

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii301-iii301
Author(s):  
Sidnei Epelman ◽  
Vijay Ramaswamy ◽  
Ethel Gorender ◽  
Luis Henrique Sakamoto
Keyword(s):  
Diffuse Intrinsic Pontine Glioma ◽  
Pontine Glioma ◽  
Tumor Biopsy ◽  
Term Survival ◽  
Long Term Survival ◽  
Good School ◽  
Cranial Mri ◽  
Cranial Neuropathies ◽  
Image Assessment

Abstract BACKGROUND Long-term survival in diffuse intrinsic pontine glioma is rare, and typically associated with atypical imaging and/or atypical clinical course. Although most patients harbor hotspot mutations in H3.1/3-K27M, a proportion of patients have alternate mutations, despite a typical clinicoradiological course. Herein we describe a long-term survivor with a classical presentation, treated with nimotuzumab, highlighting the challenges associated with such cases. CASE REPORT: A 5 year old male, diagnose in 2012 with a 10 day history multiple cranial neuropathies and a right hemiparesis. Cranial MRI revealed a poorly delimited diffuse pontine tumor and secondary hydrocephalus. Tumor biopsy was not performed due to the classic clinical presentation, and he received 54Gy/30 of radiation plus concomitant weekly nimotuzumab 150mg/m2. Initial tumor dimensions were 43x31x28mm. Nimotuzumab 150mg/m2 was continued every 2 weeks. Image assessment at week 12 of treatment revealed 16.9% volume increase, 4 weeks after radiotherapy completion. Nevertheless, subsequent neuroimaging at 24th, 36th, 60th, 96th and 108th weeks of nimotuzumab therapy showed a sustained and progressive tumor cytoreduction of 47.5%, 59%, 62.2%, 63.8% and 67%, respectively, when compared with post-radiotherapy dimensions. Currently, the patient is 13y old, good school performance, no neurologic disabilities. The last MRI at 394 weeks of nimotuzumab revealed dimensions of 21x19x14mm which corresponds to 70% of reduction compared with initial volume. CONCLUSIONS Our case of progressive cytoreduction over two years of a classic DIPG, diagnosed in the era prior to the discovery of the K27M mutation, highlights the challenges associated with long-term survival of this devastating entity.

Mortality After Cure of Testicular Seminoma

2004 ◽  
Vol 22 (4) ◽  
pp. 640-647 ◽  
Author(s):  
Gunar K. Zagars ◽  
Matthew T. Ballo ◽  
Andrew K. Lee ◽  
Sara S. Strom
Keyword(s):  
Observation Interval ◽  
Cancer Center ◽  
Standardized Mortality Ratio ◽  
Testicular Seminoma ◽  
Term Survival ◽  
Risk Of Death ◽  
Cancer Management ◽  
Long Term Survivors

Purpose To determine the incidence of potentially treatment-related mortality in long-term survivors of testicular seminoma treated by orchiectomy and radiation therapy (XRT). Patients and Methods From all 477 men with stage I or II testicular seminoma treated at The University of Texas M.D. Anderson Cancer Center (Houston, TX) with postorchiectomy megavoltage XRT between 1951 and 1999, 453 never sustained relapse of their disease. Long-term survival for these 453 men was evaluated with the person-years method to determine the standardized mortality ratio (SMR). SMRs were calculated for all causes of death, cardiac deaths, and cancer deaths using standard US data for males. Results After a median follow-up of 13.3 years, the 10-, 20-, 30-, and 40-year actuarial survival rates were 93%, 79%, 59%, and 26%, respectively. The all-cause SMR over the entire observation interval was 1.59 (99% CI, 1.21 to 2.04). The SMR was not excessive for the first 15 years of follow-up: SMR, 1.30 (95% CI, 0.93 to 1.77); but beyond 15 years the SMR was 1.85 (99% CI, 1.30 to 2.55). The overall cardiac-specific SMR was 1.61 (95% CI, 1.21 to 2.24). The cardiac SMR was significantly elevated only beyond 15 years (P < .01). The overall cancer-specific SMR was 1.91 (99% CI, 1.14 to 2.98). The cancer SMR was also significant only after 15 years of follow-up (P < .01). An increased mortality was evident in patients treated with and without mediastinal XRT. Conclusion Long-term survivors of seminoma treated with postorchiectomy XRT are at significant excess risk of death as a result of cardiac disease or second cancer. Management strategies that minimize these risks but maintain the excellent hitherto observed cure rates need to be actively pursued.

Long-Term Survival in Cerebral Glioblastoma. Case Report and Critical Review of the Literature

1998 ◽  
Vol 84 (1) ◽  
pp. 69-74 ◽  
Author(s):  
Fabrizio Puzzilli ◽  
Andrea Ruggeri ◽  
Luciano Mastronardi ◽  
Domenica Di Stefano ◽  
Pierpaolo Lunardi
Keyword(s):  
Glioblastoma Multiforme ◽  
Multimodal Therapy ◽  
Term Survival ◽  
Disease Free Interval ◽  
Average Survival ◽  
Younger Age ◽  
Aggressive Tumor ◽  
Long Term Survivors ◽  
Disease Free

Glioblastoma multiforme is the most malignant tumor of the glial series. The average survival of patients with this tumor ranges from 6 to 12 months. The case of a patient who survived for more than 11 years after diagnosis of a temporal-occipital glioblastoma which was treated with surgery, radiotherapy and chemotherapy is described. The authors deduce that among patients with glioblastoma multiforme (GM), those with a long disease-free interval after initial diagnosis who undergo multimodal therapy, including aggressive tumor removal, are the most likely long-term survivors (LS). Other factors which appeared to be related to longer survival were younger age and high Karnofsky scores.

Multiple Isolated Extramedullary Relapse and Long-term Survival of One AML Patient

2020 ◽  
Author(s):  
Jiman Li ◽  
Yang Liu ◽  
Yunzhu Li ◽  
liu weiping
Keyword(s):  
Complete Remission ◽  
Poor Prognosis ◽  
Unusual Case ◽  
Granulocytic Sarcoma ◽  
Accurate Diagnosis ◽  
Physical Damage ◽  
Term Survival ◽  
Case Presentation ◽  
Extramedullary Relapse

Abstract Background: Granulocytic sarcomas (GS) are very rare. If it occurs after complete remission of acute myeloblastic leukemia（AML）, it indicates a recurrence of AML and a poor prognosis. In such cases, relapse of leukemia occurs within a mean of 10 months following granulocytic sarcoma.Case presentation: Here we present an unusual case of 78-year-old male who presented with AML-M1 38 years ago. After complete remission from AML-M1 6 years later, he developed unusual multiple isolated extramedullary relapses. And the extramedullary relapse occurred 7 times and involved 8 anatomic sites during 15 years. Despite repeated relapses, treatment and physical damage, the patient managed to survive into 2016.However, we did not detect any signs of leukemia after 1992 and his bone marrow and peripheral blood remained normal Until his death. Immunohistochemical results of our case are all the same, suggesting that they were all derived from the recurrence of the same tumor.Conclusions: Extramedullary relapses may occur in AML patients after complete relieve and without the blood count and BM involvement. Accurate diagnosis of GS is important so the patient could to be treated timely. It is a challenge for the pathologist to make the diagnosis, and without immunohistochemistry (IHC), it may be misdiagnosed as another tumor.

Chemotherapy for poor prognosis non seminomatous germ cell tumors (NSGCT): Should doses be reduced at first cycle to prevent acute respiratory distress syndrome (ARDS) in patients with multiple lung metastases?

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 5087-5087 ◽  
Author(s):  
K. Fizazi ◽  
C. Massard ◽  
S. Postel-Vinay ◽  
B. Escudier ◽  
S. Culine ◽  
...  
Keyword(s):  
High Risk ◽  
Poor Prognosis ◽  
Lung Metastases ◽  
Germ Cell Tumors ◽  
Early Death ◽  
Visceral Metastases ◽  
Early Tumor ◽  
Long Term Survivors ◽  
Very High

5087 Background: Patients with multiple lung metastases from NSGCT and dyspnea at presentation are at high risk of ARDS and death in the first days after chemotherapy induction. This entity has been designated the “choriocarcinoma syndrome” or “very high risk NSGCT”. It is linked to acute intra-alveolar hemorrhage related to early tumor necrosis, which in turn, may be complicated by pulmonary infection promoted by neutropenia. To try to avoid this complication, The policy to manage these patients was changed at Institut Gustave Roussy in 1997. Methods: Data from all patients with lung metastases from NSGCT and dyspnea or a pO2 < 80 and treated between1980–2006 in our institution were collected. Patients were treated in a specialized intensive care unit. From 1980–1997, the first cycle of chemotherapy consisted in a full dose regimen. After 1997, it consisted in a 3-day reduced induction regimen of EP (cisplatin 20 mg/m2/day and etoposide 100 mg/m2/day), with bleomycin and two additional days of EP being postponed to day 15, and the regular BEP regimen being started at day 21. Results: 25 patients with a poor risk NSGCT according to the IGCCCG classification had extensive lung metastases plus either dyspnea at presentation (n=6) or a pO2<80 (n=2), or both criteria (n=17). Median age was 30 years (range 18–49). Median hCG was 200,000 UI (range 11–8,920,000) and 18/25 (72%) patients also had non-pulmonary visceral metastases. During the 1980–1997 period, 13/15 patients (87%) developed ARDS, of whom 10 died, and only 4/15 (27%) patients were long-term survivors. In contrast, during the 1997-2006 period, only 3/10 patients (30%) developed ARDS, of whom 2 died, and 4/10 (40%) survived. Conclusions: Reducing doses of chemotherapy during the first cycle of chemotherapy for poor-prognosis NSGCT with extensive and symptomatic lung metastases seems to prevent the risk of early death due to ARDS and should therefore be recommended. No significant financial relationships to disclose.

Predictors of long-term survival in patients with metastatic renal cell carcinoma in the cytokine era: An analysis of 473 patients treated between 1995 and 2005.

2011 ◽  
Vol 29 (7_suppl) ◽  
pp. 335-335
Author(s):  
N. Shinohara ◽  
S. Maruyama ◽  
T. Abe ◽  
A. Sazawa ◽  
K. Nonomura ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Logistic Regression ◽  
Regression Analysis ◽  
Renal Cell ◽  
Multiple Logistic Regression Analysis ◽  
Multiple Logistic Regression ◽  
Term Survival ◽  
Long Term Survivors ◽  
Metastatic Rcc

335 Background: Based on the clinical data in Japanese renal cell carcinoma (RCC) patients who underwent cytokine therapy (Naito et al, Eur Urol 2010), the overall survival (OS) of these patients has been considered to be better than that of Caucasian patients. We identified long-term survivors among a cohort of 473 metastatic RCC patients, and explored clinical predictors of OS in these patients. Methods: Between 1995 and 2005, 473 patients with metastatic RCC were the subjects of this retrospective analysis. Three hundred sixty-one (76%) patients received IFN-α and no patient did molecular-targeted drugs. The patients with survival times of greater than 5 years after the development of metastases were identified as long-term survivors. Multiple logistic regression analysis was performed to evaluate the impact of clinical variables potentially influencing OS. Results: Median OS for all patients was 22.3 months (95%CI 18.7-27.6), the estimated 5-year survival rate was 27.4% (95% CI 22.9-31.9). Sixty-eight patients (14.4%) among 473 patients were identified as long-term survivors. Twenty-six (38%) had metastases at diagnosis of kidney cancer, 3 (4%) and 11 (16%) had liver mets and bone mets, respectively. According to MSKCC risk classification, 19 (28%), 28 (41%), and 6 (9%) were classified as favorable, intermediate, and poor risk, respectively. All patients had undergone prior nephrectomy, and 40 (59%) underwent metastatectomy. Multiple logistic regression analysis from all patients identified metastatectomy (HR 5.0; 95%CI 2.7-9.5) and MSKCC risk group (HR 3.7; 95%CI 1.5-9.1) as adverse prognostic factors for long-term OS. Conclusions: Selected patients with metastatic RCC who can undergo metastatectomy have a good opportunity for long-term survival, especially those with favorable or intermediate risk classified by MSKCC risk classification. No significant financial relationships to disclose.

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