scholarly journals Case Report: Kaposiform Hemangioendothelioma With Spinal Involvement

2021 ◽  
Vol 9 ◽  
Author(s):  
Tong Qiu ◽  
Kaiying Yang ◽  
Shiyi Dai ◽  
Siyuan Chen ◽  
Yi Ji

Introduction: Kaposiform hemangioendothelioma (KHE) is a rare, locally invasive vascular tumor that mostly appears in infants and adolescents. KHE with spinal involvement is extremely rare. The aim of this study was to review the imaging features, clinical manifestations and treatment of KHE patients with spinal involvement.Materials and Methods: We reviewed patients with KHE who were admitted to Pediatric Surgery of West China Hospital of Sichuan University from April 2014 to August 2020, and the cases were evaluated.Results: Seven patients with spinal involvement were enrolled in the study, including four (57.1%) males and three (42.9%) females. The age at onset ranged from 1.0 day to 4.0 years, with an average of 1.6 years. Five (71.4%) had pain due to bone destruction, three patients (42.9%) had decreased range of motion (ROM), four (57.1%) patients had scoliosis, two (28.6%) patients developed claudication, and three patients (42.9%) presented with a soft tissue mass in the neck of the back. Five patients (71.4%) had the Kasabach-Merritt phenomenon (KMP), with a minimum platelet value of 8 × 109/L. All patients were treated with sirolimus, and showed regression of the lesion and/or normalization of the hematologic parameters.Conclusion: KHE with spinal involvement is difficult to diagnose due to its rarity and variable symptoms, which need to be recognized to start early treatment. The management of KHE with spinal involvement should be performed by a multidisciplinary team. Sirolimus can improve outcomes in patients with KHE with spinal involvement.

2022 ◽  
Author(s):  
Yanna Lei ◽  
Xiufeng Zheng ◽  
Qian Huang ◽  
Xiaoying Li ◽  
Meng Qiu ◽  
...  

Abstract Immune-related myocarditis is a severe and even life-threatening immune-related adverse event (irAE) which may also be under-estimated due to the challenge in diagnosis. There have been few reports about the intrinsic difference between patients with immune-associated myocarditis. A retrospective analysis was conducted between March 2019 to June 2020 in West China Hospital and 18 patients with immune-related myocarditis were studied. Cases were classified as mild (n=12) or severe myocarditis (n=6) according to the clinical manifestations and haemodynamic complications. Covariates extracted from medical records were compared in different groups, and factors associated with severe myocarditis were identified. In this retrospective analysis, the median age of the 18 patients was 60 years old. Most cases occur early and approximately after the first or second ICI infusion. The severity of myocarditis may be correlated with lactate dehydrogenase (LDH) (P=0.04) and troponin (P=0.0057). The relationship between troponin and myocarditis was further confirmed in another cohort which including 30 patients. In addition, patients are more likely to develop multi-irAEs, and myositis was the most common second irAE. Those who experience multi-irAEs had significantly higher LDH (P=0.02) as well as myoglobin levels (P=0.02) than did not experience. All patients were treated with steroids timely and the mortality rate was 5.6% in our study. In this study, we explore risk factors for severe myocarditis and we emphasized the importance of a multidisciplinary team in assisting diagnosis and treatment options. It is critical to initiate corticosteroid therapy, regardless of the severity of the myocarditis.


2020 ◽  
Vol 3 (1) ◽  
pp. 9-13 ◽  
Author(s):  
Kaiyue Diao ◽  
Peilun Han ◽  
Tong Pang ◽  
Yuan Li ◽  
Zhigang Yang

Abstract With the spread of novel coronavirus (2019-nCoV) pneumonia, chest high-resolution computed tomography (HRCT) has been one of the key diagnostic tools. To achieve early and accurate diagnostics, determining the radiological characteristics of the disease is of great importance. In this small scale research we retrospectively reviewed and selected six cases confirmed with 2019-nCoV infection in West China Hospital and investigated their initial and follow-up HRCT features, along with the clinical characteristics. The 2019-nCoV pneumonia basically showed a multifocal or unifocal involvement of ground-glass opacity (GGO), sometimes with consolidation and fibrosis. No pleural effusion or lymphadenopathy was identified in our presented cases. The follow-up CT generally demonstrated mild to moderate progression of the lesion, with only one case showing remission by the reducing extent and density of the airspace opacification.


2016 ◽  
Vol 2016 ◽  
pp. 1-6
Author(s):  
Xiangshui Sun ◽  
Yue Lou ◽  
Xiaodong Wang

Iliac bone destruction in children is uncommon and presents various imaging features. Correct diagnosis based on clinical and imaging features is difficult. This research aimed to retrospectively explore the clinical features, imaging, and histopathological diagnosis of children with iliac bone destruction. A total of 22 children with iliac bone destruction were enrolled in this retrospective analysis from two children’s hospitals during July 2007 to April 2015. Clinical features, imaging, and histopathological findings were analysed. The mode of iliac bone destruction, lesion structure, and the relationship between the range of soft tissue mass and cortical destruction were determined based on imaging data. The data were analysed using descriptive methods. Of the iliac bone destruction cases, eight cases were neuroblastoma iliac bone metastasis, seven cases were bone eosinophilic granuloma, two cases were Ewing’s sarcoma, two cases were osteomyelitis, one case was bone cyst, one case was bone fibrous dysplasia, and one case was non-Hodgkin’s lymphoma. Iliac bone destruction varies widely in children. Metastatic neuroblastoma and eosinophilic granuloma are the most commonly involved childhood tumours.


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Yu-Wei Chen ◽  
A. I. E. R. K. E. N. aikebaier ◽  
Yuan Zhao

Abstract Background Hydatid disease (HD) is a zoonotic parasitic disease caused by the larvae of Echinococcus, It is mainly prevalent in pastoral areas. Bone echinococcosis is rare, accounting for 0.5 to 4.0% of all echinococcosis. It is likely to miss the diagnosis and misdiagnose due to non-specific early symptoms and the clinical manifestations and imaging features. The clinical data of 15 patients with pelvic cystic echinococcosis were analyzed retrospectively, and the X-ray, CT, and MRI imaging features of the disease were discussed, which are reported below. Methods All 15 patients underwent CT scan evaluation. A total of 8 patients underwent coronal, sagittal, and three-dimensional reconstruction with 3-mm-slice thickness, and 4 patients underwent X-ray plain film examination. Five cases underwent MRI scan. Eight cases underwent MRI or CT enhanced scan. Results X-ray plain film is characterized by continuous cystic bone destruction, irregular low-density shadow when invading soft tissue, and sometimes calcification which can be seen on the wall or inside the cyst. The involved sacroiliac joint or hip joint may narrow or disappear. The involvement of pelvic cystic echinococcosis is relatively wide, and 80% of patients with pelvic cysts in this group had multiple lesions in the same period. Cystic expansive bone destruction was the most common. Pelvic CT revealed a lobulated hypodense lesion of varying size with internal septae, causing cortical thinning and destruction. Most of them had no periosteal reaction. The iliopsoas muscle is most easily invaded. Single cystic echinococcosis of pelvis showed intermediate or low signal intensity on T1-weighted images and hyperintensity on T2-weighted images in the involved bone and surrounding soft tissue on MRI, and the cyst wall showed linear low signal in T1WI, T2WI, and STIR sequences. The polycystic type is characterized by multiple cysts of varying signal intensity (daughter cysts) within a larger cyst is the typical MRI finding, forming “small vesicles” high signal daughter cysts. Osteosclerosis or calcification showed low signal in T1WI and T2WI. Conclusions The results of this study suggest that the lesions of pelvic cystic echinococcosis are mostly cystic expansive and osteolytic bone destruction, which is easy to invade the surrounding soft tissue, often accompanied with calcification; among them, multiple cystic lesions are characteristic.


2020 ◽  
Author(s):  
Xuewen Xiao ◽  
Dongni Fu ◽  
Li Feng

Abstract Background Hypertrophic pachymeningitis (HP) is a fibrotic disorder featuring a thickening of the dura matter. Most HP studies were from Caucasian population and only a few studies of HP are available in China. In this study, we investigated the causes, clinical and imaging features and therapeutic implications of HP in a southern Chinese population.Methods We retrospectively analyzed 48 patients with HP with different causes from 1 January 2006 to 31 December 2018. Clinical manifestation, laboratory findings, neuroimaging results, and clinical course were evaluated in all HP patients.Results The mean age at onset was 50±12 years. The most common diagnosis was idiopathic HP (67%), followed by ANCA-associated vasculitis (15%), tuberculous meningitis (8%), viral meningitis (6%), and bacterial meningitis (4%). The main clinical manifestations were headache and cranial nerve deficits. The most frequently changed laboratory finding was elevated ESR. Imaging was characterized by cerebral or spinal dura matter enhancement in MRI scan with contrast. Enhancements were mainly located in the posterior fossa for idiopathic HP; frontal, parietal and occipital lobes for ANCA-related HP; and the posterior fossa for tuberculous-associated HP. Diffuse enhancement was found in most cases, except for tuberculous-associated HP. Glucocorticoid or immunosuppressive treatment were applied in most cases.Conclusions HP was diagnosed based on clinical manifestations, imaging and laboratory results. Etiology varied among patients, with idiopathic HP being the most common. MRI with contrast showed enhancement of the dura matter, which differed according to different etiologies. Glucocorticoid or immunosuppressive agents were the primary drugs for treatment.


2010 ◽  
Author(s):  
Ernest Volinn ◽  
Bangxiang Yang ◽  
Jing He ◽  
Xiaoming Sheng ◽  
Jian Ying ◽  
...  

2020 ◽  
Author(s):  
Weihong Kuang ◽  
Guojun Zeng ◽  
Yunbo Nie ◽  
Yan Cai ◽  
Jin Li ◽  
...  

UNSTRUCTURED The COVID-19 pandemic spurred unprecedented progress on a paradigm shift to telemedicine to limit exposure to the virus. Telemedicine has many benefits. In the West China Hospital of Sichuan University, we use it to do COVID-19 related tele-educations to health professionals and general population, tele-diagnosis, online treatment and internet-based drug prescription and delivery. However, at our practices, we are noticing that many older adults could not make appointment with doctors due to their difficulty using the internet-based platform. We worried that older adults who need healthcare the most are not well prepared for this rapid change. We need to pay attention to avoid causing treatment disparities for vulnerable older adults 60 years of age and over. Researchers and policy makers should work together to study effective strategies and make proper policies to mitigate barriers older adults face when using telemedicine.


Lupus ◽  
2021 ◽  
pp. 096120332110142
Author(s):  
Tamer A Gheita ◽  
Rasha Abdel Noor ◽  
Esam Abualfadl ◽  
Osama S Abousehly ◽  
Iman I El-Gazzar ◽  
...  

Objective The aim of this study was to present the epidemiology, clinical manifestations and treatment pattern of systemic lupus erythematosus (SLE) in Egyptian patients over the country and compare the findings to large cohorts worldwide. Objectives were extended to focus on the age at onset and gender driven influence on the disease characteristics. Patients and method This population-based, multicenter, cross-sectional study included 3661 adult SLE patients from Egyptian rheumatology departments across the nation. Demographic, clinical, and therapeutic data were assessed for all patients. Results The study included 3661 patients; 3296 females and 365 males (9.03:1) and the median age was 30 years (17–79 years), disease duration 4 years (0–75 years) while the median age at disease onset was 25 years (4–75 years). The overall estimated prevalence of adult SLE in Egypt was 6.1/100,000 population (1.2/100,000 males and 11.3/100,000 females).There were 316 (8.6%) juvenile-onset (Jo-SLE) and 3345 adult-onset (Ao-SLE). Age at onset was highest in South and lowest in Cairo (p < 0.0001). Conclusion SLE in Egypt had a wide variety of clinical and immunological manifestations, with some similarities with that in other nations and differences within the same country. The clinical characteristics, autoantibodies and comorbidities are comparable between Ao-SLE and Jo-SLE. The frequency of various clinical and immunological manifestations varied between gender. Additional studies are needed to determine the underlying factors contributing to gender and age of onset differences.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zhehao Lyu ◽  
Lili Liu ◽  
Huimin Li ◽  
Haibo Wang ◽  
Qi Liu ◽  
...  

Abstract Background Collecting (Bellini) duct carcinoma (CDC) is a highly malignant and rare kidney tumor. We report our 12-year experience with CDC and the results of a retrospective analysis of patients and tumor characteristics, clinical manifestations, and imaging features by computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET)/CT. Methods Retrospective examination of tumors between January 2007 and December 2019 identified 13 cases of CDC from three medical centers in northern China. All 13 patients underwent CT scan, among which eight underwent dynamic enhanced CT scan, two underwent PET/CT scan, and one underwent magnetic resonance cholangiopancreatography (MRCP) examination. The lesions were divided into nephritis type and mass type according to the morphology of the tumors. Results The study group included ten men and three women with an average age of 64.23 ± 10.74 years. The clinical manifestations were gross hematuria, flank pain, and waist discomfort. The mean tumor size was 8.48 ± 2.48 cm. Of the 13 cases, six (46.2%) were cortical-medullary involved type and seven (53.8%) were cortex–medullary–pelvis involved type. Eleven (84.6%) cases were nephritis type and two (15.4%) were mass type. The lesions appeared solid or complex solid and cystic on CT and MRI. The parenchymal area of the tumors showed isodensity or slightly higher density on unenhanced CT scan in the 13 cases. PET/CT in two cases showed increased radioactivity intake. Evidence of intra-abdominal metastatic disease was present on CT in nine (69.2%) cases. Conclusions The imaging characteristics of CDC differ from those of other renal cell carcinomas. In renal tumors located in the junction zone of the renal cortex and medulla that show unclear borders, slight enhancement, and metastases in the early stage, a diagnosis of CDC needs to be considered. PET/CT provides crucial information for the diagnosis of CDC, as well as for designing treatment strategies including surgery.


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