scholarly journals Evolution of treatment for children with esthesioneuroblastoma (1969–2019)

2021 ◽  
Vol 11 (3) ◽  
pp. 56-71
Author(s):  
A. D. Rodina ◽  
A. S. Krylov ◽  
T. V. Gorbunova ◽  
V. A. Korolyov ◽  
O. A. Merkulov ◽  
...  

Introduction. Esthesioneuroblastoma is a rare neuroectodermal malignant tumor. The incidence of this tumor in children under 15 years of age is 0.1 per 100,000 pediatric population. The information on incidence, disease course, and treatment of esthesioneuroblastoma in children vary significantly and depend on the follow-up period and statistical methods.Objective – to assess risk factors for esthesioneuroblastoma and evaluate treatment strategies used in children and their prospects.Materials and methods. This study included 29 patients aged 2 to 17 years diagnosed with esthesioneuroblastoma and treated at the Research Institute of Oncology and Hematology, N.N. Blokhin Russian Cancer Research Center, Ministry of Health of Russia between 1969 and 2019. The median age upon diagnosis was 10 years. Six patients (20 %) developed the disease early in their life (being under 3 years of age). Esthesioneuroblastoma was equally common in boys and girls. More than half of the patients (n = 15; 51 %) had stage IV disease. The primary tumor invaded the maxillary sinus (n = 25; 86 %), orbit (n = 10; 34 %), ethmoid sinus (n = 18; 62 %), and cranial cavity (n = 12; 41 %). The majority of children (n = 28; 96 %) received pharmacotherapy and radiotherapy (n = 27; 93 %); the total focal dose was 50 Gy in case of primary tumor. The affected cervical lymph nodes were irradiated in 10 (35 %) patients (total focal dose 40 Gy). Ten (35 %) patients have undergone surgery.Results. The follow-up period varied between 3 months and 50 years. Nine (34 %) patients are still alive. Fourteen patients (48 %) died due to tumor progression; 1 (3 %) patient died due to complications from special treatment. Five (17 %) patients were lost to follow-up at different stages.Conclusion. The following risk factors were associated with poor prognosis in children with esthesioneuroblastoma: disease onset in early childhood, no adequate diagnosis of distant metastases prior to specific treatment initiation, disseminated disease, and high Ki-67 index. In patients with advanced cancer, surgeries were effective only in combination with chemoradiotherapy. Long-term treatment outcomes in children with esthesioneuroblastoma are poor due to high risk of recurrence and tumor dissemination.  

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Yu Liu ◽  
Chunjie Liu ◽  
Dongmei Guo ◽  
Ning Wang ◽  
Ying Zhao ◽  
...  

Abstract Background The medical community has recognized overweight as an epidemic negatively affecting a large proportion of the pediatric population, but few studies have been performed to investigate the relationship between overweight and failure of conservative treatment for distal radius fractures (DRFs). This study was performed to investigate the effect of overweight on the outcome of conservative treatment for DRFs in children. Methods We performed a retrospective study of children with closed displaced distal metaphyseal radius fractures in our hospital from January 2015 to May 2020. Closed reduction was initially performed; if closed reduction failed, surgical treatment was performed. Patients were followed up regularly after treatment, and redisplacement was diagnosed on the basis of imaging findings. Potential risk factors for redisplacement were collected and analyzed. Results In total, 142 children were included in this study. The final reduction procedure failed in 21 patients, all of whom finally underwent surgical treatment. The incidences of failed final reduction and fair reduction were significantly higher in the overweight/obesity group than in the normal-weight group (P = 0.046 and P = 0.041, respectively). During follow-up, 32 (26.4%) patients developed redisplacement after closed reduction and cast immobilization. The three risk factors associated with the incidence of redisplacement were overweight/obesity [odds ratio (OR), 2.149; 95% confidence interval (CI), 1.320–3.498], an associated ulnar fracture (OR, 2.127; 95% CI, 1.169–3.870), and a three-point index of ≥ 0.40 (OR, 3.272; 95% CI, 1.975–5.421). Conclusions Overweight increases the risk of reduction failure and decreases the reduction effect. Overweight children were two times more likely to develop redisplacement than normal-weight children in the present study. Thus, overweight children may benefit from stricter clinical follow-up and perhaps a lower threshold for surgical intervention.


2020 ◽  
Author(s):  
Alessandro Giollo ◽  
Giovanni Cioffi ◽  
Federica Ognibeni ◽  
Giovanni Orsolini ◽  
Andrea Dalbeni ◽  
...  

Abstract Background. Major cardiovascular disease (CVD) benefits of disease-modifying anti-rheumatic drugs (DMARDs) therapy occur in early RA patients with treat-to-target strategy. However, it is unknown whether long-term DMARDs treatment in established RA could be useful to improve CVD risk profile.Methods. Ultrasound aortic stiffness index (AoSI) has to be considered a proxy outcome measure in established RA patients. We measured AoSI in a group of RA patients on long-term treatment with tumour necrosis factor inhibitors (TNFi) or conventional synthetic DMARDs (csDMARDs). Eligible participants were assessed at baseline and after 12 months; changes in serum lipids, glucose and arterial blood pressure were assessed. All patients were on stable medications during the entire follow-up. Results. We included 107 (64 TNFi and 43 csDMARDs) RA patients. Most patients (74%) were in remission or low disease activity and had some CVD risk factors (45.8% hypertension, 59.8% dyslipidemia, 45.3% smoking). The two groups did not differ significantly for baseline AoSI (5.95±3.73% vs 6.08±4.20%, p=0.867). Follow-up AoSI was significantly increased from baseline in the csDMARDs group (+1.00%; p<0.0001) but not in the TNFi group (+0.15%, p=0.477). Patients on TNFi had significantly lower follow-up AoSI from baseline than the csDMARD group (-1.02%, p<0.001; ANCOVA corrected for baseline AoSI, age and systolic blood pressure). Furthermore, follow-up AoSI was significantly lower in TNFi users with 1-2 or >2 CVD risk factors than in those without. Conclusion. Long-term treatment with TNFi was associated with reduced aortic stiffness in patients with established RA and several CVD risk factors.


Author(s):  
Fanny Delehaye ◽  
Sabine SARNACKI ◽  
Daniel Orbach ◽  
Alaa Cheikhelard ◽  
Jérémie Rouger ◽  
...  

Objective Ovarian mature teratoma (OMT) is a common ovarian tumor found in the pediatric population. In 10% to 20% of cases, OMT occurs as multiple synchronous or metachronous lesions on ipsi- or contralateral ovaries. Ovarian sparing surgery (OSS) is recommended to preserve fertility, but total oophorectomy (TO) is still performed. This study reviews the clinical data of patients with OMT, and analyzes risk factors for second events. Design A retrospective review of all girls under 18 years of age with OMTs was performed. Data on clinical features, imaging, laboratory studies, surgical reports, follow-up second events and their management were retrieved. Results Overall, 350 children were identified. Eighteen patients (5%) presented with a synchronous bilateral form at diagnosis. Surgery was performed by laparotomy (85%) and laparoscopy (15%). OSS and TO were performed in 59% and 41% of cases respectively. Perioperative tumor rupture occurred in 23 cases, independently of the surgical approach. Twenty-nine second events occurred (8.3%) in a median time of 30.5 months from diagnosis (ipsilateral: 8 cases including one malignant tumor, contralateral: 18 cases, both ovaries: 3 cases). A large palpable mass, bilateral forms at diagnosis and perioperative rupture had a statistical impact on the risk of second event, whereas type of surgery or approach did not. Conclusion This study is a plea in favor of OSS as the first choice of treatment of OMT when possible. Close follow-up during the first five years is mandatory considering the risk of 8.3% of second events especially in cases with risk factors.


Author(s):  
Sam Sedaghat ◽  
Alexey Surov ◽  
Sebastian Krohn ◽  
Maya Sedaghat ◽  
Benjamin Reichardt ◽  
...  

Purpose To analyze the appearance of primary and recurrent aggressive fibromatosis (AF) on MRI with a focus on configuration and to determine potential risk factors for recurrences detected on MRI follow-up scans. Methods From 79 consecutive patients with histologically proven diagnosis of AF, 39 patients underwent a minimum of four 1.5 T MRI follow-up scans after resection of primary AF between 2008 and 2018. The primary and recurrent tumors were radiographically examined for configuration, limitation and extent on MRI. Epidemiological data and loco-regional subcutaneous edema, muscle edema and post-operative seroma were included. Results The mean age of the patients was 39 ± 2.6 years. Primary and recurrent AF most often occurred in the thigh. The main appearance of primary AF was significantly most often fascicular (p = 0.001–0.01) with heterogeneous and marked contrast enhancement. 21 % (n = 8) of the patients developed recurrences of AF. A fascicular configuration with homogeneous/heterogeneous contrast enhancement was the main appearance of recurrent AF, but recurrent AF appeared nodular, polycyclic, ovoid or streaky/flat as well. Recurrent AF significantly most often occurred within the first 9 months after primary tumor resection (p = 0.009), especially in patients up to 25 years of age (RR = 6.1; 95 % CI: 1.8–20.9; p = 0.004). The cases of recurrent AF were altogether significantly smaller than the primary tumors (p = 0.001). Post-treatment subcutaneous and muscle edema were present in 77 % and 56 %, respectively. Patients with muscle edema after primary tumor resection had a significantly higher risk for AF recurrences (relative risk ratio (RR) = 1.8; 95 % CI: 1.16–2.8; p = 0.0096). There was no significant difference detected in patients with complete or incomplete resection of the primary tumor. Conclusion Primary and recurrent aggressive fibromatosis has a mostly fascicular configuration, but may appear ovoid, nodular, streaky/flat or polycyclic as well. High risks for tumor recurrences are detected for patients up to 25 years of age, patients within the first 9 post-operative months and patients with muscle edema after primary tumor resection. Key points:  Citation Format


2020 ◽  
Vol 28 (4) ◽  
pp. 192-195
Author(s):  
Duffield Ashmead ◽  
Haruko Okada ◽  
Jonathan Macknin ◽  
Steven Vander Naalt ◽  
Ilene Staff ◽  
...  

Trigger finger (TF) and carpal tunnel syndrome (CTS) are common conditions often occurring together with an unclear relationship. While some studies conclude that TFs occur as a result of carpal tunnel release (CTR), others have not established a causal relationship. Our purpose was to evaluate the prevalence and timing of TF development in the same hand after open CTR in our population. This was a retrospective review of 497 patients undergoing open CTR by a single surgeon. Two hundred twenty-nine charts were analysed for age, gender, handedness, BMI, workers’ compensation status, and background disease. We analysed the specific digit involved and timing to development of triggering after CTR. Thirty-one patients developed triggering after CTR (13.5%). Mean age was 52.5 (14.0) years. Follow-up ranged from 1 to 53 months with a median follow-up of 6 months (interquartile range = 2-13). The thumb was the most common to trigger (42.22%), followed by the ring 24.44%, middle 22.22%, little 8.89%, and index fingers 2.22%. Trigger thumb occurred at 3.5 months (3.6) post-operatively, while other digits triggered at 7.5 months (4-10.25) after surgery ( P = .022). No risk factors were associated with TF development. Our results suggest that a trigger thumb develops more frequently and earlier than other trigger digits after an open CTR. Further study is needed to clarify the mechanisms involved and may enable specific treatment such as local anti-inflammatory medication following CTR. We suggest educating prospective carpal tunnel surgery patients to high risk of triggering following CTR.


2012 ◽  
Vol 30 (28) ◽  
pp. 3478-3485 ◽  
Author(s):  
Voralak Vichapat ◽  
Hans Garmo ◽  
Marit Holmqvist ◽  
Göran Liljegren ◽  
Fredrik Wärnberg ◽  
...  

Purpose The number of breast cancer survivors at risk of developing contralateral breast cancer (CBC) is increasing. However, ambiguity remains regarding risk factors and prognosis for women with CBC. Patients and Methods In a cohort of 42,670 women with breast cancer in the Uppsala/Örebro and Stockholm regions in Sweden in 1992 to 2008, we assessed risk factors for and prognosis of metachronous CBC by using survival analysis. Breast cancer–specific survival for women with CBC was evaluated and compared with results for women with unilateral breast cancer (UBC) by using time-dependent Cox-regression modeling. Results An increased risk for CBC was observed among women who had primary breast cancer with ≥ 10 involved lymph nodes compared with node-negative women (adjusted hazard ratio [HR], 1.8; 95% CI, 1.2 to 2.7). The prognosis was poorer in women with CBC than with UBC. The hazard of dying from breast cancer was especially high for women with a short interval time to CBC (adjusted HR, 2.3; 95% CI, 1.8 to 2.8 for CBC diagnosed ≤ 5 years v UBC) and gradually decreased with longer follow-up time but remained higher than the hazard originating from the primary tumor for ≥ 10 years. Conclusion Women with advanced-stage primary breast cancer had an increased risk of developing CBC. CBC is associated with an increased risk of dying from breast cancer throughout a long period of follow-up after the primary tumor. Our findings suggest that the event of CBC marks a new clinical situation in terms of investigations for metastases, treatment considerations, and follow-up strategy.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Inés Pérez-Flores ◽  
Beatríz Macías-Murelaga ◽  
Jesús Barrio-Barrio ◽  
Inés Pérez Flores ◽  
Marta Valcárcel Vizcaíno ◽  
...  

AbstractTo evaluate the efficacy and safety of atropine 0.01% eye drops for myopia control in a multicentric pediatric Spanish cohort. An interventional, prospective, multicenter study was designed. Children aged between 6 and 14 years, with myopia between − 2.00 D to − 6.00 D, astigmatism < 1.50 D and documented previous annual progression greater than − 0.5 D (cycloplegic spherical equivalent, SE) were included. Once nightly atropine 0.01% eye drops in each eye were prescribed to all participants for 12 months. Age, gender, ethnicity and iris color were registered. All patients underwent the same follow-up protocol in every center: baseline visit, telephone consultation 2 weeks later and office controls at 4, 8 and 12 months. At each visit, best-corrected visual acuity, and cycloplegic autorefraction were assessed. Axial length (AL), anterior chamber depth and pupil diameter were measured on an IOL Master (Carl Zeiss Meditec, Inc, Dublin, CA). Adverse effects were registered in a specific questionnaire. Mean changes in cycloplegic SE and AL in the 12 months follow-up were analyzed. SE progression during treatment was compared with the SE progression in the year before enrollment for each patient. Correlation between SE and AL, and annual progression distribution were evaluated. Progression risk factors were analyzed by multivariate logistic regression analyses. Of the 105 recruited children, 92 completed the treatment. Mean SE and AL changes were − 0.44 ± 0.41 D and 0.27 ± 0.20 mm respectively. Mean SE progression was lower than the year before treatment (− 0.44 ± 0.41 D versus − 1.01 ± 0.38 D; p < 0.0001). An inverse correlation between SE progression and AL progression (r: − 0.42; p < 0.0001) was found. Fifty-seven patients (62%) had a SE progression less than − 0.50 D. No risk factors associated with progression could be identified in multivariate analyses. Mean pupil diameter increment at 12-months visit was 0.74 ± 1.76 mm. The adverse effects were mild and infrequent, and decreased over the time. Atropine 0.01% is effective and safe for myopia progression control in a multicentric Spanish children cohort. We believe this efficacy might be extensible to the myopic pediatric population from Western countries with similar social and demographic features. More studies about myopia progression risk factors among atropine treated patients are needed.


2004 ◽  
Vol 62 (3a) ◽  
pp. 613-617
Author(s):  
Rubens José Gagliardi ◽  
Ibsen Thadeu Damiani ◽  
Rogério Menoncello ◽  
Manoel Carlos Sampaio de Almeida Ribeiro

OBJECTIVE: To evaluate the spontaneous evolution of extracranial carotid atherosclerosis in asymptomatic patients who did not present the main risk factors associated to the disease. METHOD:A prospective study including patients of both genders, age ranging from 40 to 70 years, not presenting any signs and symptoms of cerebrovascular disease and without the main atherosclerosis risk factors were included. Patients who were using or had used medication during the follow-up period that could potentially influence in the spontaneous course of atherosclerosis were excluded. The evaluation of the plaque and degree of stenosis were acquired using mode B, 7.5 MHz Doppler ultrasonography (USG). The follow-up was carried out for 36 months, with clinical, neurological, and USG exams repeated in a period of 6 to 8 months. Ninety-six individuals (48 women) completed the study with the presence of plaque, and 52 (26 women) with a degree of stenosis. RESULTS: As to the degree of stenosis, 25% of the patients had worsening, 69% remained stable and 6% improved. When only the presence or absence of plaque was considered, 20% showed worsening (plaque developed during follow-up), 7% improved (disappearance of plaque), and 73% remained stable. No differences were found between the male and female patients. CONCLUSION: These results confirm the dynamic characteristics of plaque. In asymptomatic individuals without specific treatment, spontaneous improvement may occur, however, rarely. These findings may contribute as an assessment criterion when a decision is to be made in high-risk patients.


2019 ◽  
Vol 2019 ◽  
pp. 1-9 ◽  
Author(s):  
Erminia Ridolo ◽  
Irene Martignago ◽  
Irene Pellicelli ◽  
Cristoforo Incorvaia

Background. Up to one-third of the patients suffering from eosinophilic esophagitis (EoE) present a refractory form, as defined by nonresponsiveness in clinical, endoscopic, or histological assessment after first-line therapy. Several studies recently investigated which factors can influence the development of this disease, but very few analyzed the factors underlying refractory EoE. Methods. Medical charts of patients affected by EoE were retrospectively evaluated. Phenotyping of patients was conducted according to demographic, clinical, histological, and treatment variables. Then, patients were divided into responder and nonresponder to therapy and distinguished among children and adults. Results. Forty-five children and 35 adult EoE patients were included. In the pediatric population, female sex (p<0.05) and a higher score of visual analogue scale (VAS) at the follow-up visit (p=0.02) were significantly associated to the risk of refractory EoE. Among adults, statistical significance was reached for years of follow-up (p=0.001), diagnostic delay (p=0.03), use of antibiotics during infancy (p=0.01), and food allergy (p=0.04). Conclusions. Our study highlighted female sex and a higher VAS score at the time of follow-up visits as risk factors for refractory EoE in children, while the risk factors in adults were identified as fewer years of follow-up, greater diagnostic delay, use of antibiotics during infancy, and food allergy.


2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Yu-Hsiang Ling ◽  
Yen-Feng Wang ◽  
Jiing-Feng Lirng ◽  
Jong-Ling Fuh ◽  
Shuu-Jiun Wang ◽  
...  

Abstract Background Chronic headache may persist after the remission of reversible cerebral vasoconstriction syndrome (RCVS) in some patients. We aimed to investigate the prevalence, characteristics, risk factors, and the impact of post-RCVS headache. Methods We prospectively recruited patients with RCVS and collected their baseline demographics, including psychological distress measured by Hospital Anxiety and Depression scale. We evaluated whether the patients developed post-RCVS headache 3 months after RCVS onset. The manifestations of post-RCVS headache and headache-related disability measured by Migraine Disability Assessment (MIDAS) scores were recorded. Results From 2017 to 2019, 134 patients with RCVS were recruited, of whom, 123 finished follow-up interviews (response rate 91.8%). Sixty (48.8%) patients had post-RCVS headache. Migrainous features were common in post-RCVS headache. Post-RCVS headache caused moderate-to-severe headache-related disability (MIDAS score > 10) in seven (11.7%) patients. Higher anxiety level (odds ratio 1.21, p = 0.009) and a history of migraine (odds ratio 2.59, p = 0.049) are associated with post-RCVS headache. Survival analysis estimated that 50% post-RCVS headache would recover in 389 days (95% confidence interval: 198.5–579) after disease onset. Conclusions Post-RCVS headache is common, affecting half of patients and being disabling in one-tenth. Higher anxiety level and migraine history are risk factors. Half of the patients with post-RCVS headache would recover in about a year.


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