Epithelioid haemangioendothelioma.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 10569-10569
Author(s):  
Nadia Yousaf ◽  
Charlotte Benson ◽  
Omar Al-muderis ◽  
Cyril Fisher ◽  
Ian Robert Judson

10569 Background: Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour which can arise in any site. There is little published data about the clinical behaviour and management of these tumours. Methods: A retrospective review of the management of patients with histologically proven EHE presenting to the Royal Marsden Hospital from January 1999 to January 2012. Demographics, site, treatment and survival outcomes were collected. Results: 28 patients (21 females) were identified with a mean (SD) age of 44 (17). 20 patients presented with diffuse disease involving one or more organs at presentation. The predominant site of disease was identified as liver n=8, mediastinum n=6, lung n= 2, limb n=1, pancreas n=1, spleen n=1, back n=1, abdominal wall n=1, oesophagus n=1, neck n=1, pelvis n=1, not known n=2. Median overall survival (Interquartile range) [OS (IQR)] in months was the longest in 10 patients who had localised operable disease at presentation, 116 (71). Amongst those with inoperable disease (n=16), patients with liver disease had the longest OS (IQR), 18 (43) months and those with lung and mediastinal disease had the shortest OS (IQR), 10 (2.5) and 11 (17) months respectively. A variety of treatment modalities were used for patients with inoperable disease and these are summarised in the Table. Conclusions: The clinical behaviour of EHE can vary depending on the site of disease. Surgery, if feasible, has the best outcome. In those with inoperable disease a period of observation to assess the clinical behaviour of the tumour is recommended. Non-steroidal anti-inflammatory drugs are a reasonable, relatively non-toxic first line treatment option. The use of anti-angiogenic drugs merits further exploration. [Table: see text]

2002 ◽  
Vol 15 (3) ◽  
pp. 241-249
Author(s):  
Joseph Reilly ◽  
Edmund J. Bini

There have been a number of exciting advancements and changes in the gastroenterology field over the last few years. A new mesalamine formulation, balsalazide disodium, is now available for the treatment of ulcerative colitis (UC). Balsalazide may be utilized as first line therapy for patients with UC, or patients who are intolerant to other mesalamine preparations. In the area of irritable bowel syndrome, alosetron was removed from the market after reports that it was associated with severe constipation and death, although a causal relationship could not be established. Published data examines the risk of certain medications and the development of reflux disease and esophageal adenocarcinoma. Also, new proton pump inhibitors (PPIs) are available, esomeprazole, as well as alternate methods of administration for others. The first intravenous PPI, pantoprazole, is available in the United States, although currently there is a paucity of data regarding its efficacy. Peptic ulcer disease is also discussed, including Helicobacter pylori resistance, nonsteroidal anti-inflammatory drugs, and the treatment of bleeding ulcers. With pegylated interferon, progress in the treatment of hepatitis C offers promise for many patients. This review will highlight many recent changes in gastroenterology an offer a perspective on how disease management has changed.


F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 697
Author(s):  
Priyanka Agrawal ◽  
Pedro M. Machado

Axial spondyloarthritis (axSpA) is a chronic inflammatory rheumatic disease that predominantly affects the axial skeleton. The advent of biologic drugs has transformed the management of patients with axSpA. However, non-steroidal anti-inflammatory drugs remain the first-line drug treatment for axSpA. The optimal management of patients with axSpA requires a combination of pharmacological and non-pharmacological treatment modalities, namely exercise and physical therapy. This review looks at novel therapeutic options in patients with axSpA. It also summarises current evidence regarding radiographic progression and treat-to-target in axSpA.


Author(s):  
Bernard Bannwarth ◽  
Francis Berenbaum

Apart from non-steroidal anti-inflammatory drugs (NSAIDs), there are only two categories of systemic analgesics, namely paracetamol (acetaminophen) and opioids, that are currently available worldwide for clinical use. Paracetamol is poorly effective in relieving pain and improving function in patients with symptomatic osteoarthritis (OA). Furthermore, its safety profile is less favourable than classically thought. In fact, there is evidence paracetamol acts as a weak inhibitor of the cyclooxygenase enzymes. Given that paracetamol poses a lower risk of severe adverse events than NSAIDs while being better tolerated than opioids, it is usually considered as the first-line systemic analgesic for OA. Commonly prescribed opioids are primarily agonists of the mu receptors, thereby producing similar desirable (analgesia) and untoward effects. Meta-analyses of short-term clinical trials showed that, on average, the modest clinical benefits of opioids did not outweigh the side effects in patients with knee or hip OA. Accordingly, most current guidelines support the use of opioids for selected OA patients only (e.g. patients who have not had an adequate response to other treatment modalities and are not candidates for total joint arthroplasty). In view of the limited efficacy and/or potential harms of available analgesics, particular attention was paid to novel painkillers, especially nerve growth factor (NGF) antagonists. Although these agents provided clinically meaningful improvements in pain and physical function in patients with hip or knee OA, they lead to severe side effects, including rapidly destructive arthropathies and neuropathies. Thus, if approved for marketing, NGF antagonists would be reserved for selected and well-defined patients with OA.


Author(s):  
Chávez Hernández María Margarita ◽  
Jiménez Báez María Valeria ◽  
Armijo Medina María Fernanda ◽  
Domínguez Leyva Jorge Miguel ◽  
Góngora Valencia Karen Alejandra ◽  
...  

Prolactinomas are the most common type of functional pituitary tumor. The present manuscript is an update on the treatment modalities for prolactinomas. Effective hyperprolactinemia treatment is of great importance, due to its potential deleterious effects including infertility, gonadal dysfunction and osteoporosis. Dopamine agonist therapy is the first line of treatment for prolactinomas; recurrence of disease after cessation of the drug may occur in patients. Its safety profile remains high, allowing its use during pregnancy.


2021 ◽  
Vol 14 (1) ◽  
pp. e232385
Author(s):  
Jessica Farnan ◽  
Ellen Morrison ◽  
Derek Barrry Hennessey

Proximal-type epithelioid sarcoma is an ultra-rare, high-grade soft tissue malignancy usually presenting as a deep-seated painless mass in the proximal extremities. Most patients are diagnosed as young adults, between 20 and 40 years of age. Perineal and genital masses do occur but are extremely rare and represent a challenging tumour to diagnose and treat. Early radical excision is recommended due to its aggressive behaviour and poor prognosis. Median overall survival from initial diagnosis is 30 months. We present the case of a 22-year-old man with a left groin proximal-type epithelioid sarcoma who is sadly deceased 12 months after initial presentation despite early surgical excision, completion of both first-line and palliative chemotherapy, and palliative radiotherapy.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1613.1-1613
Author(s):  
K. Ben Abdelghani ◽  
Y. Gzam ◽  
A. Fazaa ◽  
S. Miladi ◽  
K. Ouenniche ◽  
...  

Background:For decades, NSAID have been used as the first-line drugs to treat axial spondyloarthritis (ax-SpA). However, the NSAID prescription strategy is not clearly detailed and it varies from one clinician to another.Objectives:The aim of this study was to assess the NSAID prescription modalities adopted in ax-SpA and the differences between these modalities.Methods:This is a descriptive study including 200 cases of ax-SpA fulfilling the ASAS 2009 criteria and diagnosed between January 2000 and October 2019. The demographic and clinical features of the ax-SpA were collected and the modalities of prescription of NSAID were retrospectively assessed.Results:Our population consists of 138 men and 62 women with a mean age of 43,3 ± 11,2 years. The HLA B-27 antigen was present in 50,8% of cases. The ax-SpA was a pure axial form in 67% of patients, associated with peripheral arthritis, enthesitis and dactylitis in 19%, 21,5% and 1,5% respectively.One hundred eighty patients (90%) had been treated with NSAIDs. The NSAIDs used were: the Diclofenac (57.5%), Indomethacin (37.5%), Piroxicam (36%), clecoxib (34%), Naproxen (29.5%) and ketoprofen (13%). Seventy-three patients (36.5%) had used at least 3 NSAIDs.Among the 180 patients treated with NSAID, 88 patients (48,8%) were treated with conventional synthetic DMARDs (csDMARDs) in association with NSAID: Salazopyrine (43,3%) and Methotrexate (13,3%). Seventy-one patients (39,4%) had necessitated the use of anti-TNF alpha.NSAIDs were used continuously in 115 patients (63.8%) and the maximum dose of NSAIDs was used in 78 patients (43.3%). By comparing patients who used maximum doses of NSAIDs and those who used NSAID continuously with other patients, we noticed that the use of biological treatments was more frequent in those groups (p = 0,01 and p=0,004 respectively).In addition, while comparing the group of patients co-treated with csDMARDs with other patients treated with NSAID on monotherapy, we noted that this group of patients had more arthritis (p<0,0001), enthesitis (p=0,02), psoriasis (p=0,04) and necessitated more biological treatments (p=0,01).Conclusion:Our results suggest that maximal doses and/or continuous prescription of NSAID were mainly used if there was no response to that treatment. The csDMARDs were more prescribed if there were peripheral manifestations or psoriatic arthritis and those forms were also more candidates to biological treatments.References:[1]Wang R, et al. Arthritis Rheumatol Hoboken NJ. 2019;Disclosure of Interests:None declared


2007 ◽  
Vol 122 (3) ◽  
pp. 221-229
Author(s):  
V C Cousins

AbstractThe management of lesions of the lateral skull base is a highly sophisticated branch of surgery generally performed by otolaryngology–head and neck surgeons as part of a multi-disciplinary team. Assessment of patients with diseases affecting the lateral skull base can be complex, as can the application of the various treatment modalities and the management of the expected and unexpected side effects of that treatment.A wide range of pathological conditions occur in the lateral skull base. Many operations and procedures have been described for dealing with them. There is not necessarily one correct solution to the management of any particular problem in the skull base, with multiple factors to be considered in planning and intervention.As surgeons, we need to know how our own results and outcomes compare with pooled, published data concerning the implications and complications occurring as a result of intervention, in order to better advise our patients on their management.


2002 ◽  
Vol 88 (4) ◽  
pp. 273-276 ◽  
Author(s):  
Mario Airoldi ◽  
Fulvia Pedani ◽  
Sara Marchionatti ◽  
Anna Maria Gabriele ◽  
Giovanni Succo ◽  
...  

Background Recurrent undifferentiated nasopharyngeal carcinoma is a chemosensitive disease. Few third-line treatments have been reported. Methods Twelve patients (9 males, 3 females; median age 50 years, range, 20-62) with recurrent undifferentiated nasopharyngeal carcinoma were treated with carboplatin AUC 5.5 + paclitaxel (175 mg/m2, 3-hr infusion) on day 1 every 3 weeks. All patients had been previously treated for recurrent disease with a first-line cisplatin-based chemotherapy and a second-line therapy with low-dose continous infusion 5-fluorouracil. Results Overall, 54 courses were given (median, 5; range, 2-6). Three patients (25%) obtained a partial response lasting 6, 10 and 26+ months, 1 (8.3%) a minimal response lasting 6 months, and 3 (25%) no change with a median duration of 5 months. The median survival time was 14 months for patients who had a partial or minimal response or no change, and 5 months for nonresponders. Median overall survival was 9.5 months (3-30+). The treatment was well tolerated, and toxicity was manageable. Conclusions The combination has a good pallitive role as third-line chemotherapy in recurrent undifferentiated nasopharyngeal cancer.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 4139-4139
Author(s):  
Chris Poki Leung ◽  
Minal A. Barve ◽  
Ming-Shiang Wu ◽  
Kathleen F. Pirollo ◽  
James F. Strauss ◽  
...  

4139 Background: Nearly all stage IV pancreatic adenocarcinoma (PAC) patients progress after first-line treatment, and second-line options are limited. SGT-53 is an investigational product for tumor-targeted TP53 gene therapy that has completed phase Ia/Ib trials [Senser et al (2013), Mol Ther 21:1096; Pirollo et al (2016) Mol Ther 24:1697]. Methods: Here we provide an interim analysis of a Phase II trial (SGT53-02-1; NCT02340117) combining SGT-53 with gemcitabine/nab-paclitaxel (GEM/ABX). Eligible were first-line patients or those who had progressed after FOLFIRINOX (FFX) and/or gemcitabine-based therapy (second-line). In a 7-week treatment cycle, SGT-53 (3.6 mg DNA) was given once or twice weekly with GEM/ABX (1000 mg/m2/wk and 125 mg/m2/wk, respectively, for 3 of 4 weeks). Progression-free survival (PFS) and objective response rate (ORR) are primary endpoints.Overall survival (OS) and PFS are estimated by Kaplan-Meier analysis. Results: Of all evaluable patients (n=20), best response in 7 patients was determined to be partial response (PR) and 13 had stable disease (SD); none had progressive disease. In the second-line patients (n=11) there were 5 PR and 6 SD after 9 had failed FFX treatment, 3 had failed gemcitabine-based treatment and 1 had failed both. For patients with elevated CA19-9, SGT-53 + GEM/ABX resulted in marked reductions in the tumor marker. Published data for patients with PAC after therapy failure [Mita et al (2019) J Clin Med 8: 761; Portal et al (2015) Br J Cancer 113:989; Wang-Gillam et al (2016) Lancet 387:545] are shown for comparison. Notably, mPFS in our second-line patients was 7.4 months versus 3.1 months for the approved second-line therapy [Wang-Gillam et al (2016)]. This improvement in PFS exceeds the benchmark proposed to predict a clinically meaningful Phase III trial [Rahib et al (2016) Lancet Oncol 2:1209]. Conclusions: Our data suggest a clinically meaningful benefit of adding SGT-53 to GEM/ABX particularly for second-line PAC patients, most of whom had failed prior FFX treatment. Clinical trial information: NCT02340117. [Table: see text]


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