scholarly journals EPEN-06. CHEMOTHERAPY OF RECURRENT EPENDYMOMA: LONG-TERM RESPONSE ONLY IN FEW CASES

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii309-iii309
Author(s):  
Jonas Adolph ◽  
Stephan Tippelt ◽  
Ruth Mikasch ◽  
Stefan Pfister ◽  
Kristian Pajtler ◽  
...  
Keyword(s):  
Neoadjuvant Treatment ◽  
Molecular Data ◽  
Incomplete Resection ◽  
Response Evaluation ◽  
Treatment Switching ◽  
Tumor Response Evaluation ◽  
First Relapse ◽  
Recurrent Ependymoma ◽  
First Recurrence

Abstract INTRODUCTION The efficacy of chemotherapy in recurrent ependymoma is unclear. We present results from the German HIT-REZ-studies. METHODS 137 patients were analyzed regarding the treatment with chemotherapy at first recurrence, the time from first relapse to progression (PFS) and to either time-point of death or last follow-up (OS). Tumor response evaluation was based on MRI and clinically; molecular data was available in 80. RESULTS In our cohort, 96 patients (20 supratentorial, 73 infratentorial, 3 spinal) received chemotherapy during first recurrence: 49 (51.0%) temozolomide (TMZ) monotherapy, 12 (12.5%) HIT-SKK regime, 9 (9.4%) carboplatin/etoposide (CE) and 26 (27.1%) other combinations. In 19.8% (26.5% in TMZ), chemotherapy was administered prior to surgery (neoadjuvant), which resulted in tumor progression in 78% (85% in TMZ). Gross-total resection was achieved in 86% without neoadjuvant chemotherapy and in 74% (69% in TMZ) with neoadjuvant treatment. Switching to trofosfamide/etoposide (TE) after surgery and unresponsiveness to TMZ showed further progression in all cases of tumor-residuum after surgery. Regarding 1-year-PFS, treatment with HIT-SKK (50.0%±14.4%) or CE (55.6%±16.6%) was advantageous over TMZ (30.2%±6.7%). However, 5-y-OS was lower in CE (19.0% ±16.8%) than in TMZ (39.8%±7.7%) and HIT-SKK (42.9%±8.7%). Long-term control was seen in individual cases of TMZ, HIT-SKK and CE, with TMZ providing longest response of 72 months. CONCLUSION Neoadjuvant TMZ has no significant advantage regarding PFS. However, in few cases chemotherapy prevented progression after incomplete resection. Difficulties in response evaluation and variability in therapies hinder conclusions. Supported by the German Children’s Cancer Foundation

Magnetic resonance-guided histopathology for improved accuracy of tumor response evaluation of neoadjuvant treatment in organ-infiltrating rectal cancer

2013 ◽  
Vol 107 (2) ◽  
pp. 178-183 ◽  
Author(s):  
Knut Håkon Hole ◽  
Stein Gunnar Larsen ◽  
Krystyna Kotanska Grøholt ◽  
Karl-Erik Giercksky ◽  
Anne Hansen Ree
Keyword(s):  
Rectal Cancer ◽  
Magnetic Resonance ◽  
Tumor Response ◽  
Neoadjuvant Treatment ◽  
Response Evaluation ◽  
Tumor Response Evaluation ◽  
Improved Accuracy

Intraoperative, postoperative and long-term oncosurgical safety of therapeutic mammaplasty

2013 ◽  
Vol 154 (33) ◽  
pp. 1291-1296 ◽  
Author(s):  
László Romics Jr. ◽  
Sophie Barrett ◽  
Sheila Stallard ◽  
Eva Weiler-Mithoff
Keyword(s):  
Recurrence Rate ◽  
Tumour Size ◽  
Breast Conservation ◽  
Malignant Lesion ◽  
Surgical Techniques ◽  
Incomplete Resection ◽  
Immediate Reconstruction ◽  
Oncological Safety

Introduction: (Pre)malignant lesion in the breast requiring mastectomy conventionally may be treated with breast conservation by using oncoplastic breast surgical techniques, which is called therapeutic mammaplasty. However, no reliable data has been published so far as regards the oncological safety of this method. Aim: The aim of the authors was to analyse the oncological safety of therapeutic mammaplasty in a series of patients. Method: 99 patients were treated with therapeutic mammaplasty and data were collected in a breast surgical database prospectively. Results were analysed with respect to intraoperative, postoperative and long-term oncological safety. Results: Incomplete resection rate was 14.1%, which correlated with tumour size (p = 0.023), and multifocality (p = 0.012). Time between surgery (therapeutic mammaplasty) and chemotherapy was similar to time between conventional breast surgeries (wide excision, mastectomy, mastectomy with immediate reconstruction) and chemotherapy (mean 29–31 days; p<0.05). Overall recurrence rate was 6.1%, locoregional recurrence rate was 2% during 27 month (1–88) mean follow-up. Conclusions: Since literature data are based on relatively short follow-up and low patient number, it is highly important that all data on therapeutic mammaplasty is collected in a prospectively maintained breast surgical database in order to determine true recurrence after long-follow-up. Orv. Hetil., 2013, 154, 1291–1296.

scholarly journals Phenylketonuria and juvenile idiopathic arthritis: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Ting Ting Zhu ◽  
Jin Wu ◽  
Li Yuan Wang ◽  
Xiao Mei Sun
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Case Report ◽  
Autoimmune Diseases ◽  
Hip Joint ◽  
Metabolic Disorder ◽  
Rare Case ◽  
Molecular Data ◽  
Methotrexate Therapy ◽  
Case Presentation

Abstract Background Phenylketonuria (PKU) is a genetic metabolic disorder in which patients have no ability to convert phenylalanine to tyrosine. Several autoimmune diseases have been reported to combine with PKU, co-existent of PKU and Juvenile Idiopathic Arthritis (JIA) has not been presented. Case presentation The girl was diagnosed with PKU at the age of 1 month confirmed by molecular data. At the age of 3.5 years, she presented with pain and swelling of her right ankle, right knee, and right hip joint. After a serial of examinations, she was diagnosed with JIA and treated with a nonsteroidal anti-inflammatory drug. Conclusions We report a rare case of a 4-year-old girl with PKU and JIA, which supports a possible interaction between PKU and JIA. Long-term metabolic disturbance may increase the susceptibility to JIA. Further chronic inflammation could alter the metabolism of tryptophan and tyrosine to increase blood Phe concentration. In addition, corticosteroid and methotrexate therapy for JIA may increase blood Phe concentration.

First repeated bone scan in the observation of patients with operable breast cancer.

1986 ◽  
Vol 4 (3) ◽  
pp. 389-394 ◽  
Author(s):  
A Pedrazzini ◽  
R Gelber ◽  
M Isley ◽  
M Castiglione ◽  
A Goldhirsch
Keyword(s):  
Breast Cancer ◽  
Alkaline Phosphatase ◽  
Bone Scan ◽  
Serum Alkaline Phosphatase ◽  
Clinical Situation ◽  
Operable Breast Cancer ◽  
First Relapse ◽  
Bone Scans ◽  
First Recurrence

Data on 1,601 patients with node-positive operable breast cancer who were randomized in four different prospective adjuvant therapy trials were analyzed to evaluate the role of routine bone scans and the alkaline phosphatase value at regular intervals in screening for bone involvement. Bone scan was a prerequisite for randomization and was repeated within the first 12 months in 90% (1,441) of the patients. Abnormal or doubtful scan findings had to be verified by x-ray examination. The repeated scan results were normal in 1,263 (87.8%) patients, doubtful but with no radiologic evidence of bone metastasis in 161 (11%), and abnormal (radiologically confirmed) in 17 (1.2%). After a median observation of 4 years bone metastases as the first relapse developed in 136 (8.5%) patients. This occurred in 87 of 1,263 (6.9%) of the patients with normal repeated scan results and in 18 of 161 (11.2%) of those with doubtful repeated scan findings. Based on the results of the first repeated scan, early detection of a first recurrence in bone might have been possible for 2.4% of the population. Serum alkaline phosphatase levels were also without clinical use. Bone scan in the observation of patients with operable breast cancer should be performed only as dictated by the clinical situation.

scholarly journals CEA, EpCAM, αvβ6 and uPAR Expression in Rectal Cancer Patients with a Pathological Complete Response after Neoadjuvant Therapy

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 516
Author(s):  
Daan Linders ◽  
Marion Deken ◽  
Maxime van der Valk ◽  
Willemieke Tummers ◽  
Shadhvi Bhairosingh ◽  
...  
Keyword(s):  
Rectal Cancer ◽  
Neoadjuvant Therapy ◽  
Cancer Patients ◽  
Pathological Complete Response ◽  
Neoadjuvant Treatment ◽  
Complete Response ◽  
Response Evaluation ◽  
Tumor Bed ◽  
Upar Expression ◽  
Diagnostic Biopsy

Rectal cancer patients with a complete response after neoadjuvant therapy can be monitored with a watch-and-wait strategy. However, regrowth rates indicate that identification of patients with a pathological complete response (pCR) remains challenging. Targeted near-infrared fluorescence endoscopy is a potential tool to improve response evaluation. Promising tumor targets include carcinoembryonic antigen (CEA), epithelial cell adhesion molecule (EpCAM), integrin αvβ6, and urokinase-type plasminogen activator receptor (uPAR). To investigate the applicability of these targets, we analyzed protein expression by immunohistochemistry and quantified these by a total immunostaining score (TIS) in tissue of rectal cancer patients with a pCR. CEA, EpCAM, αvβ6, and uPAR expression in the diagnostic biopsy was high (TIS > 6) in, respectively, 100%, 100%, 33%, and 46% of cases. CEA and EpCAM expressions were significantly higher in the diagnostic biopsy compared with the corresponding tumor bed (p < 0.01). CEA, EpCAM, αvβ6, and uPAR expressions were low (TIS < 6) in the tumor bed in, respectively, 93%, 95%, 85%, and 62.5% of cases. Immunohistochemical evaluation shows that CEA and EpCAM could be suitable targets for response evaluation after neoadjuvant treatment, since expression of these targets in the primary tumor bed is low compared with the diagnostic biopsy and adjacent pre-existent rectal mucosa in more than 90% of patients with a pCR.

scholarly journals Resection of cerebral gangliogliomas causing drug-resistant epilepsy: short- and long-term outcomes using intraoperative MRI and neuronavigation

2015 ◽  
Vol 38 (1) ◽  
pp. E5 ◽  
Author(s):  
Björn Sommer ◽  
Cornelia Wimmer ◽  
Roland Coras ◽  
Ingmar Blumcke ◽  
Bogdan Lorber ◽  
...  
Keyword(s):  
Diffusion Tensor ◽  
Intraoperative Imaging ◽  
Intractable Epilepsy ◽  
Complete Resection ◽  
Blood Oxygenation ◽  
Seizure Outcome ◽  
Incomplete Resection ◽  
Brain Areas ◽  
Total Resection

OBJECT Cerebral gangliogliomas (GGs) are highly associated with intractable epilepsy. Incomplete resection due to proximity to eloquent brain regions or misinterpretation of the resection amount is a strong negative predictor for local tumor recurrence and persisting seizures. A potential method for dealing with this obstacle could be the application of intraoperative high-field MRI (iopMRI) combined with neuronavigation. METHODS Sixty-nine patients (31 female, 38 male; median age 28.5 ± 15.4 years) suffering from cerebral GGs were included in this retrospective study. Five patients received surgery twice in the observation period. In 48 of the 69 patients, 1.5-T iopMRI combined with neuronavigational guidance was used. Lesions close to eloquent brain areas were resected with the implementation of preoperative diffusion tensor imaging tractography and blood oxygenation level–dependent functional MRI (15 patients). RESULTS Overall, complete resection was accomplished in 60 of 69 surgical procedures (87%). Two patients underwent biopsy only, and in 7 patients, subtotal resection was accomplished because of proximity to critical brain areas. Excluding the 2 biopsies, complete resection using neuronavigation/iopMRI was documented in 33 of 46 cases (72%) by intraoperative imaging. Remnant tumor mass was identified intraoperatively in 13 of 46 patients (28%). After intraoperative second-look surgery, the authors improved the total resection rate by 9 patients (up to 91% [42 of 46]). Of 21 patients undergoing conventional surgery, 14 (67%) had complete resection without the use of iopMRI. Regarding epilepsy outcome, 42 of 60 patients with seizures (70%) became completely seizure free (Engel Class IA) after a median follow-up time of 55.5 ± 36.2 months. Neurological deficits were found temporarily in 1 (1.4%) patient and permanently in 4 (5.8%) patients. CONCLUSIONS Using iopMRI combined with neuronavigation in cerebral GG surgery, the authors raised the rate of complete resection in this series by 19%. Given the fact that total resection is a strong predictor of long-term seizure control, this technique may contribute to improved seizure outcome and reduced neurological morbidity.

Early predictors of disability of paediatric-onset AQP4-IgG-seropositive neuromyelitis optica spectrum disorders

2021 ◽  
pp. jnnp-2021-327206
Author(s):  
Valentina Camera ◽  
Silvia Messina ◽  
Kariem Tarek Elhadd ◽  
Julia Sanpera-Iglesias ◽  
Romina Mariano ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Neuromyelitis Optica ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Onset Age ◽  
Visual Disability ◽  
Therapeutic Decisions ◽  
First Relapse ◽  
Edss Score ◽  
Spectrum Disorders

ObjectiveTo describe onset clinical features predicting time to first relapse and time to long-term visual, motor and cognitive disabilities in paediatric-onset aquaporin-4 antibody (AQP4-IgG) neuromyelitis optica spectrum disorders (NMOSDs).MethodsIn this retrospective UK multicentre cohort study, we recorded clinical data of paediatric-onset AQP4-IgG NMOSD. Univariate and exploratory multivariable Cox proportional hazard models were used to identify long-term predictors of permanent visual disability, Expanded Disability Status Scale (EDSS) score of 4 and cognitive impairment.ResultsWe included 49 paediatric-onset AQP4-IgG patients (38.8% white, 34.7% black, 20.4% Asians and 6.1% mixed), mean onset age of 12±4.1 years, and 87.7% were female. Multifocal onset presentation occurred in 26.5% of patients, and optic nerve (47%), area postrema/brainstem (48.9%) and encephalon (28.6%) were the most involved areas. Overall, 52.3% of children had their first relapse within 1 year from disease onset. Children with onset age <12 years were more likely to have an earlier first relapse (p=0.030), despite showing no difference in time to immunosuppression compared with those aged 12–18 years at onset. At the cohort median disease duration of 79 months, 34.3% had developed permanent visual disability, 20.7% EDSS score 4 and 25.8% cognitive impairment. Visual disability was associated with white race (p=0.032) and optic neuritis presentations (p=0.002). Cognitive impairment was predicted by cerebral syndrome presentations (p=0.048), particularly if resistant to steroids (p=0.034).ConclusionsAge at onset, race, onset symptoms and resistance to acute therapy at onset attack predict first relapse and long-term disabilities. The recognition of these predictors may help to power future paediatric clinical trials and to direct early therapeutic decisions in AQP4-IgG NMOSD.

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