scholarly journals RARE-50. TREATMENT RESPONSE OF CNS HIGH-GRADE NEUROEPITHELIAL TUMORS WITH MN1 ALTERATION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii453-iii453
Author(s):  
Lorena V Baroni ◽  
Carlos Rugilo ◽  
Fabiana Lubieniecki ◽  
Claudia Sampor ◽  
Candela Freytes ◽  
...  

Abstract BACKGROUND CNS high-grade neuroepithelial tumor with MN1 alteration (CNS HGNET-MN1) are a rare entity recently described as a high-grade tumor containing a mixture of solid and pseudopapillary patterns with MN1 rearrangement. METHODS CNS HGNET-MN1 patients were identified using genome wide methylation arrays across 5 institutions (the Hospital JP Garrahan, Hospital for Sick Children, the University Hospital Motol, Royal Children`s Hospital and Christchurch Hospital) and was correlated with treatment and outcome. Central imaging review with radiological features analysis was performed. RESULTS We identified 9 patients harboring CNS HGNET-MN1 tumors through application of the Heidelberg brain tumor classifier. Seven tumors were T supratentorial and two in the spinal cord. Median age was 5 (range 3.6–14.6). All patients had surgery (6 GTR and 3 STR) as initial management followed by radiotherapy (focal 5/CSI 1) and systemic chemotherapy in 2 patients. Four of the 9 patients relapsed by 3 years post diagnosis, with 2 local and 2 metastatic failures despite complete surgical resections and radiotherapy. Three patients died due to tumor relapse after 24 months despite upfront radiotherapy. Seven of 9 patients had an initial diagnosis of ependymoma. CONCLUSION Treatment of CNS HGNET-MN1 remains a major challenge with multiple failures, despite aggressive surgical resections and upfront involved field radiotherapy. Further multicenter, international prospective studies are required to determine the optimal treatment strategy for this group of tumors.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2603-2603 ◽  
Author(s):  
Gandhi Damaj ◽  
Marc Bernard ◽  
Steven Legouill ◽  
Guillaume Cartron ◽  
Annie Le Mevel ◽  
...  

Abstract Diffuse large cell lymphoma is the most common non-Hodgkin Lymphoma (NHL) that ranks among the most curable diffuse intermediate grade lymphomas. Most investigators have recognized that practically all patients with this cell type who are able to attain and maintain a complete response for 24 consecutive months are cured because late relapses seldom occur after this period of follow-up. However, relapses do occur late after the remission and the characteristics of patients who experience these relapses rarely have been studied especially for localized disease. From 1984 to 1996, three hundred and five patients aged more than 18 years were enrolled in a prospective multicenter study for primary treatment of localized stage I/II nodal and extra nodal high-grade NHL. The initial therapy consisted in three cycles of high dose CHOP regimen followed by involved field radiotherapy (40Gy). Of the 283 (93%) patients who obtained complete remission (CR), 36 (12.7%) patients relapsed more than 2 years after CR and 28 (9.8%) of them experienced relapse more than three years after CR. Clinical characteristics of patients at diagnosis are summarized in table 1. For the 28 patients who relapsed more than 3 years after diagnosis (group A), the median age was 55 years. There were 17 men and 11 women with predominant histology of diffuse large B cell lymphoma (n=15) and mixed small and large B-cell (n=6). Stage I disease was predominant (n=16) with normal LDH level (n=14). Extranodal disease was present in 11 patients. Most of the patients had no or one IPI risk factor at diagnosis (n=18). Relapses occurred after median CR duration of 84 (36–156) months. Seven patients (7/28; 25%) relapsed as a disseminated lymphoma and 17 of 21 patients who relapsed as a localized disease, relapsed at sites other than those of diagnosis. Only 4 patients relapsed at the same initial site of lymphoma. Nodal relapses remained the most frequent type of relapse (n=18; 64%). However, it is important to note that 10 patients experienced extranodal relapses especially in the central nervous system, the Waldeyer’s ring and the gastrointestinal tract. Three patients had multiple extranodal localisations. All relapsed patients received chemotherapy alone (n=20) or followed by autologous stem cell transplantation (n=8). CR was obtained in 13 patients (13/23; 56%), and partial response in 1 patients. Chemotherapy failed in 5 patients and 4 patients deceased from toxicities. At the last follow-up, only 8 (8/24; 33%) patients are still alive and disease free (n=5) or in relapse (n=3). Sixteen patients deceased and lymphoma was the most frequent cause of death (13/24; 54%). In conclusion, late relapses in the setting of localized high grade NHL treated with short course of chemotherapy and involved field radiotherapy are frequent. Relapses occurred frequently at different sites from the initial presentation mostly nodal and extranodal localisations are frequent. The rate of cure for relapsed patients is low and death from lymphoma remains high. The role of novel therapeutic approaches with the addition of rituximab to chemotherapy with or without radiotherapy in preventing late relapses is awaited. More data will be presented at the meeting Table 1: clinical characteristics at diagnosis


VASA ◽  
2004 ◽  
Vol 33 (2) ◽  
pp. 78-81 ◽  
Author(s):  
Thalhammer ◽  
Aschwanden ◽  
Jeanneret ◽  
Labs ◽  
Jäger

Background: Haemostatic puncture closure devices for rapid and effective hemostasis after arterial catheterisation are a comfortable alternative to manual compression. Implanting a collagen plug against the vessel wall may become responsible for other kind of vascular injuries i.e. thrombotic or stenotic lesions and peripheral embolisation. The aim of this paper is to report our clinically relevant vascular complications after Angio-Seal® and to discuss the results in the light of the current literature. Patients and methods: We report the symptomatic vascular complications in 17 of 7376 patients undergoing diagnostic or therapeutic catheterisation between May 2000 and March 2003 at the University Hospital Basel. Results: Most patients presented with ischaemic symptoms, arterial stenoses or occlusions and thrombotic lesions (n = 14), whereas pseudoaneurysms were extremely rare (n = 3). Most patients with ischaemic lesions underwent vascular surgery and all patients with a pseudoaneurysm were successfully treated by ultrasound-guided compression. Conclusions: Severe vascular complications after Angio-Seal® are rare, consistent with the current literature. There may be a shift from pseudoaneurysms to ischaemic lesions.


1993 ◽  
Vol 32 (05) ◽  
pp. 365-372 ◽  
Author(s):  
T. Timmeis ◽  
J. H. van Bemmel ◽  
E. M. van Mulligen

AbstractResults are presented of the user evaluation of an integrated medical workstation for support of clinical research. Twenty-seven users were recruited from medical and scientific staff of the University Hospital Dijkzigt, the Faculty of Medicine of the Erasmus University Rotterdam, and from other Dutch medical institutions; and all were given a written, self-contained tutorial. Subsequently, an experiment was done in which six clinical data analysis problems had to be solved and an evaluation form was filled out. The aim of this user evaluation was to obtain insight in the benefits of integration for support of clinical data analysis for clinicians and biomedical researchers. The problems were divided into two sets, with gradually more complex problems. In the first set users were guided in a stepwise fashion to solve the problems. In the second set each stepwise problem had an open counterpart. During the evaluation, the workstation continuously recorded the user’s actions. From these results significant differences became apparent between clinicians and non-clinicians for the correctness (means 54% and 81%, respectively, p = 0.04), completeness (means 64% and 88%, respectively, p = 0.01), and number of problems solved (means 67% and 90%, respectively, p = 0.02). These differences were absent for the stepwise problems. Physicians tend to skip more problems than biomedical researchers. No statistically significant differences were found between users with and without clinical data analysis experience, for correctness (means 74% and 72%, respectively, p = 0.95), and completeness (means 82% and 79%, respectively, p = 0.40). It appeared that various clinical research problems can be solved easily with support of the workstation; the results of this experiment can be used as guidance for the development of the successor of this prototype workstation and serve as a reference for the assessment of next versions.


1995 ◽  
Vol 34 (01/02) ◽  
pp. 75-78 ◽  
Author(s):  
R. D. Appel ◽  
O. Golaz ◽  
Ch. Pasquali ◽  
J.-C. Sanchez ◽  
A. Bairoch ◽  
...  

Abstract:The sharing of knowledge worldwide using hypermedia facilities and fast communication protocols (i.e., Mosaic and World Wide Web) provides a growth capacity with tremendous versatility and efficacy. The example of ExPASy, a molecular biology server developed at the University Hospital of Geneva, is striking. ExPASy provides hypermedia facilities to browse through several up-to-date biological and medical databases around the world and to link information from protein maps to genome information and diseases. Its extensive access is open through World Wide Web. Its concept could be extended to patient data including texts, laboratory data, relevant literature findings, sounds, images and movies. A new hypermedia culture is spreading very rapidly where the international fast transmission of documents is the central element. It is part of the emerging new “information society”.


2019 ◽  
Author(s):  
M Stättermayer ◽  
F Riedl ◽  
S Bernhofer ◽  
A Stättermayer ◽  
A Mayer ◽  
...  

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