scholarly journals Cutaneous Ewing Sarcoma and Ewing Sarcoma of the Bone: Distinct Diseases

2018 ◽  
Vol 11 (3) ◽  
pp. 729-734 ◽  
Author(s):  
Montreh Tavakkoli ◽  
Lisa Mueller

Ewing sarcoma is an aggressive mesenchymal malignancy. It is the second most common bone tumor among children and adolescents and less commonly presents as a soft tissue or primary skin lesion. Cutaneous Ewing sarcoma has only been reported in case reports and case series. In this article, we describe a 12-year-old Hispanic female cured of localized, cutaneous Ewing sarcoma (pT1aN0M0) at the 40-month follow-up following surgical resection and adjuvant chemotherapy according to the COG AEWS1031 protocol for Ewing sarcoma of the bone. To our knowledge, this is the first article to provide a potential biological explanation for the differences in the prognosis of Ewing sarcoma of the bone, soft tissue, and skin.

2019 ◽  
Vol 24 (5) ◽  
pp. 558-571 ◽  
Author(s):  
Kartik Bhatia ◽  
Hans Kortman ◽  
Christopher Blair ◽  
Geoffrey Parker ◽  
David Brunacci ◽  
...  

OBJECTIVEThe role of mechanical thrombectomy in pediatric acute ischemic stroke is uncertain, despite extensive evidence of benefit in adults. The existing literature consists of several recent small single-arm cohort studies, as well as multiple prior small case series and case reports. Published reports of pediatric cases have increased markedly since 2015, after the publication of the positive trials in adults. The recent AHA/ASA Scientific Statement on this issue was informed predominantly by pre-2015 case reports and identified several knowledge gaps, including how young a child may undergo thrombectomy. A repeat systematic review and meta-analysis is warranted to help guide therapeutic decisions and address gaps in knowledge.METHODSUsing PRISMA-IPD guidelines, the authors performed a systematic review of the literature from 1999 to April 2019 and individual patient data meta-analysis, with 2 independent reviewers. An additional series of 3 cases in adolescent males from one of the authors’ centers was also included. The primary outcomes were the rate of good long-term (mRS score 0–2 at final follow-up) and short-term (reduction in NIHSS score by ≥ 8 points or NIHSS score 0–1 at up to 24 hours post-thrombectomy) neurological outcomes following mechanical thrombectomy for acute ischemic stroke in patients < 18 years of age. The secondary outcome was the rate of successful angiographic recanalization (mTICI score 2b/3).RESULTSThe authors’ review yielded 113 cases of mechanical thrombectomy in 110 pediatric patients. Although complete follow-up data are not available for all patients, 87 of 96 (90.6%) had good long-term neurological outcomes (mRS score 0–2), 55 of 79 (69.6%) had good short-term neurological outcomes, and 86 of 98 (87.8%) had successful angiographic recanalization (mTICI score 2b/3). Death occurred in 2 patients and symptomatic intracranial hemorrhage in 1 patient. Sixteen published thrombectomy cases were identified in children < 5 years of age.CONCLUSIONSMechanical thrombectomy may be considered for acute ischemic stroke due to large vessel occlusion (ICA terminus, M1, basilar artery) in patients aged 1–18 years (Level C evidence; Class IIb recommendation). The existing evidence base is likely affected by selection and publication bias. A prospective multinational registry is recommended as the next investigative step.


Author(s):  
Sandeep Mohindra ◽  
Manjul Tripathi ◽  
Aman Batish ◽  
Ankur Kapoor ◽  
Ninad Ramesh Patil ◽  
...  

Abstract Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.


Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110108
Author(s):  
Ashley D Hickman ◽  
Evandro D Bezerra ◽  
Anja C Roden ◽  
Matthew T Houdek ◽  
Jonathan D Barlow ◽  
...  

Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm which typically originates from liver, lung, or bone. Due to the low incidence of disease, the most effective treatment is not easily studied and much of the information known about EHE has been learned through case reports and case series. In this case, we will present an uncommon form of primary soft tissue EHE with local recurrence, bone metastasis, and lymphangitic spread to the lungs leading to respiratory failure. Imaging of the chest was atypical for EHE with intraseptal thickening and hilar lymphadenopathy. Respiratory failure was progressive despite aggressive multimodal treatment. This case highlights an unusually aggressive recurrence and metastasis of primary soft tissue EHE with atypical pulmonary imaging findings.


Dermatology ◽  
2021 ◽  
pp. 1-7
Author(s):  
Jurr Boer

<b><i>Background:</i></b> Patients with hidradenitis suppurativa (HS) are still often disappointed with the current treatments offered and there is a clear demand for more effective options. Since the late 1990s there has been a revival in the use of radiotherapy (RT) for different benign diseases, including HS. During the past 20 years one case series and some scattered case reports have described promising results of RT. <b><i>Objectives:</i></b> To evaluate the long-term efficacy of RT in early-stage HS. <b><i>Methods:</i></b> A postal survey-based long-term follow-up with simple factual questions of partly retrospective and partly contemporary characteristics was performed. Sixty-four patients (96 axillae), diagnosed with mild to moderate HS were irradiated with a orthovoltage unit with 100 kV, 3 mm Al or 200 kV, 0.5 Cu filtering, respectively. Four to six biweekly fractional doses ranging from 0.75 to 1 Gy up to a total dose of 6 Gy in one series, and in chronic cases followed by four daily fractions of 2 Gy up to a total dose of 14 Gy, were given. Late treatment toxicity and the rate of remission of the disease were evaluated. <b><i>Results:</i></b> The overall response rate of the survey was 64.1% with 40.6% (26/64) valid, complete questionnaires. In total, 40 axillae were irradiated in these 26 patients. After a median follow-up of 40 years (range 32–52) complete remission of the lesions occurred in 34 of the 40 sites (85%). None of the 26 patients with 40 irradiated sites reported adverse effects at the time of the survey. <b><i>Conclusions:</i></b> RT appears to be an effective treatment for early and mild HS in the majority of patients. In this case series, no side effects were reported after a median follow-up period of 40 years.


2021 ◽  
Author(s):  
Huy Gia Vuong ◽  
Truong P.x. Nguyen ◽  
Hanh T.t. Ngo ◽  
Lewis Hassell ◽  
Kennichi Kakudo

Malignant thyroid teratoma (MTT) is a very rare thyroid malignancy. These neoplasms have been reported only in case reports and small-sized case series so far. In this study, we searched for MTTs in the Surveillance, Epidemiology, and End Result (SEER) program during 1975-2016. Subsequently, we incorporated the SEER data with published MTT cases in the literature to analyze the characteristics and prognostic factors of MTTs. Integrated data were analyzed using Chi-square or Fisher’s exact test for categorical covariates, and t-test or Mann-Whitney test for continuous variables. We included 28 studies with 36 MTT cases and found additional 8 cases from the SEER program for final analyses. Our results showed that MTT is typically seen in adult females. These neoplasms were associated with an aggressive clinical course with high rates of extrathyroidal extension (80%) and nodal involvement (62%). During follow-up, the development of recurrence and metastases were common (42% and 46%, respectively), and one-third of patients died at the last follow-up. Large tumor size (p = 0.022) and the presence of metastases during follow-up (p = 0.008) were associated with a higher mortality rate. In conclusion, our study demonstrated the characteristic features of MTT patients and outlined some parameters associated with a negative outcome which could help clinicians better predict the clinical course of these neoplasms.


Author(s):  
Francesco Lancellotti ◽  
Luigi Solinas ◽  
Davide Telesco ◽  
Andrea Sagnotta ◽  
Augusto Belardi ◽  
...  

Abstract Gastrointestinal neuroendocrine tumor (NET) associated with a metachronous intestinal adenocarcinoma is rare. We report the case of a 71-year-old man with an ileal NET. Patient has previously undergone a left colectomy for sigmoid cancer. We report a complete review both of the metachronous and synchronous NET. A comprehensive systematic literature search in PubMed, EMBASE, and MEDLINE identified a total of 35 relevant studies. This study includes an analysis of review articles, case reports, case series, retrospective studies and population-based studies. In the English literature to date, there are 21 case reports (19 synchronous cases and 2 metachronous cases), 3 case series and 3 review articles, and less than 10 retrospective studies or population-based studies. A total of 31 patients in 24 articles were included in the study: 28 patients with a synchronous gastrointestinal NET and colorectal adenocarcinoma and 3 patients with metachronous gastrointestinal NET and colorectal adenocarcinoma. The incidence of synchronous cancer (particularly for colorectal and gastric cancer) with a gastrointestinal NET ranges from 10 to 50%, while for the metachronous ones it is still unclear. This is the third metachronous case report and the first descriptive case of gastrointestinal NET diagnosed 2 years after a colorectal adenocarcinoma. An endoscopic follow-up program for gastrointestinal NET patients and/or for first-degree relatives of NET patients appears recommendable.


2020 ◽  
Vol 14 (3) ◽  
pp. 260-263
Author(s):  
Guillermo Arrondo ◽  
Daniel Gómez ◽  
Germán Joannas ◽  
Xavier Martín-Oliva ◽  
Matías Iglesias ◽  
...  

Objective: Impingement syndromes are recognized as an important cause of chronic ankle pain, which results from the entrapment of an inflamed soft-tissue component between the osteophytes. The predominant site of occurrence is the anterolateral aspect of the ankle for soft-tissue impingement, and anteromedial aspect for bony impingement. Symptoms related to the physical impact of bone or soft-tissue pain often result in limited ankle range of motion. Methods: We conducted a retrospective study of 34 patients (34 ankles) with anteromedial bony impingement. All patients underwent arthroscopy, with a mean follow-up of 34 months. Results: All osteophytes were removed, and the ankle range of motion improved. The AOFAS score improved from 73 preoperatively to 95 postoperatively. Conclusion: The arthroscopic removal of the anteromedial osteophytes of the ankle had excellent functional results. It is an effective procedure that allows rapid patient recovery. Level of Evidence IV; Therapeutic Studies; Case Series.


2021 ◽  
Vol 15 (5) ◽  
pp. 1529-1532
Author(s):  
M. S. Abdulqader ◽  
L. J. Khorsheed ◽  
Hwaizi .

Background and objectives: Closed reduction and short leg spica casting are the preferred treatment options for children with developmental dysplasia of the hip. This study aimed to show the efficacy behind a standardized closed reduction for managing patients with developmental dysplasia of the hip with concomitant soft tissue releases when indicated and using a short leg plaster of Paris cast to maintain reduction and reporting mid-term results. Methods: A case series of 95 hips in 84 children aged 6-18 months who had closed reduction, with five years follow up or until next operation, involved in this study. The protocol defines acceptable concentric reduction criteria and the indications for an associated soft tissue release. All the patients were immobilized in a short leg cast for three months. Multiple follow-up radiographs were taken to assess Tönnis grade, Severin grade, acetabular index, and osteonecrosis signs. Results: A total of 48 hips were Tönnis grade 3/4 hips. At one year, 15 reductions couldn’t be maintained, and these patients needed open reduction. Of these 15 failed reductions, 7 patients were Severin 1; others were Severin 2. Of the 80 successful closed reductions, 70 hips were Severin 1. Surgical management for residual dysplasia was offered for 8 hips. Osteonecrosis was seen in 23 hips but was transient in 20. Bilateral hip dislocations and most Tönnis 4 hips were more likely to fail. Two children had severe osteonecrosis. Conclusions: Closed reduction, with subsequent adductor and psoas releases, when indicated and using a short leg plaster of Paris cast for three months, brings about good mid-term results in children with developmental dysplasia of the hip aged 6-18 months. Keywords: Developmental dysplasia of the hip, closed hip reduction, open psoas release, short leg cast.


2020 ◽  
Vol 33 (4) ◽  
pp. 297-305
Author(s):  
Mostafa Mahmoud ◽  
Ahmed Farag ◽  
Mostafa Farid ◽  
Ahmed Elserwi ◽  
Amr Abdelsamad ◽  
...  

Introduction The treatment of aneurysms in the internal carotid bifurcation region (ICABR), including aneurysms of the true internal carotid artery (ICA) terminus, those inclined on the proximal A1 or M1 segments or at the most distal pre-bifurcation (ICA) segment, is often challenging in microsurgical clipping and endovascular surgery. Few reports had discussed flow diversion as a therapeutic option for this group. Methods This was a retrospective study analysing flow diversion in treating ICABR aneurysms. Seven patients harbouring eight aneurysms in the ICABR were treated with flow diversion. Five aneurysms were inclined on the proximal A1 segment, and three were located at the most distal pre-bifurcation segment. Patients’ demographics, presentation, procedure technical description, angiographic and clinical follow-up were recorded. PubMed and Ovid MEDLINE were also reviewed for articles published in English, including case series or case reports, for ICABR aneurysms treated with flow diverters. Results All patients except one underwent angiographic follow-up. The Karman–Byrne occlusion scale was used to determine the occlusion rate. All six patients with documented angiographic follow-up had a class IV occlusion score. No permanent or transient neurological or non-neurological complications were encountered in this study. Conclusion Treating ICABR aneurysms using flow diversion is feasible, with a promising angiographic occlusion rate. Further studies are needed to analyse long-term clinical and angiographic results.


2015 ◽  
Vol 41 (3) ◽  
pp. 314-320 ◽  
Author(s):  
Jorge Saade ◽  
Bruno Salles Sotto-Maior ◽  
Carlos Eduardo Francischone ◽  
Marcelo Bassani ◽  
André Navas Alves de Castro ◽  
...  

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