The rate of early misdiagnosis in patients with nodular fasciitis of the ear is high. To provide a basis for clinical diagnosis and treatment, we aimed to summarise the clinical manifestations, ...imaging results, pathological findings, treatment strategies, and postoperative follow-up results for three cases of paediatric nodular fasciitis (two girls, one boy) treated in the Department of Otorhinolaryngology, Head and Neck Surgery, at Beijing Children’s Hospital of Capital Medical University from 2018 to 2020. The average age at diagnosis was 24 months. Lesions occurred in the left ear in two cases and right ear in one case. All patients had a history of biopsy before surgery. Rapid growth was observed following biopsy in two patients, and anti-inflammatory treatment was ineffective in all three cases. Fluorescence in situ hybridisation analysis of ubiquitin-specific peptidase 6 (
USP6
) was performed in two of the three cases, with positive results. The lesions exhibited hypo-intensity or iso-intensity on T1-weighted magnetic resonance imaging (MRI) and heterogeneous hyper-intensity on T2-weighted MRI. “Fascial tail” signs were observed on imaging in all cases. Surgical resection was performed in all cases. Intact ear appearance was observed at follow-up, and there were no cases of recurrence.
Conclusion
: Combining clinical features with imaging findings may improve the accuracy of preoperative diagnosis in patients with nodular fasciitis. In addition to pathological findings, genetic testing for
USP6
may aid in diagnosis. The final diagnosis should be based on comprehensive assessment. Complete surgical resection can prevent recurrence.
What is Known:
• Paediatric NF around the ear is rare and is easily misdiagnosed as other inflammatory masses that have a higher incidence in children.
• Most previous reports of NF were case reports.
What is New:
• Combining clinical and imaging findings with genetic testing for USP6 rearrangement may improve the accuracy of preoperative diagnosis in patients with NF. Nonetheless, the final diagnosis should be based on comprehensive assessment.
• The present paper is significant in that it represents the only report of three cases of ear NF in children with a complete medical history and prognosis.
Author Affiliation: (1) Department of Infectious Diseases, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China (2) Department of Respiratory ...Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China (3) Department of Dermatology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China (4) Department of Pathology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China (5) Department of Pediatrics, Beijing Shijitan Hospital, Capital Medical University, Beijing, China (6) Beijing Key Laboratory for Genetics of Birth Defects, Beijing Pediatric Research Institute, Key Laboratory of Major Diseases in Children, Ministry of Education, Genetics and Birth Defects Control Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China (n) liwei@bch.com.cn (p) liugangbch@sina.com Article History: Registration Date: 11/27/2020 Received Date: 04/18/2020 Accepted Date: 11/27/2020 Online Date: 01/27/2021 Byline:
Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder in children, and liver involvement in LCH is rare. This retrospective study reported the clinical features and prognosis of ...patients with hepatic LCH. Liver involvement was defined by histopathological findings, liver dysfunction or abnormalities, or ultrasound imaging. A total of 130 patients (14.5%) with hepatic LCH out of 899 in the LCH population were enrolled. Patients with liver involvement had greater frequencies of skin, lung, hearing system, and haematologic system involvement, and hemophagocytic lymphohistiocytosis (P<0.001, 0.001, 0.002, 0.009, and <0.001, respectively). Overall survival and progression-free survival were lower in LCH patients with liver involvement than in those without liver involvement (P<0.001 and <0.001). In patients with liver involvement, the overall survival (OS) and progression-free survival (PFS) rates were lower in patients with cholangitis than in those without cholangitis (P<0.020 and 0.030). For the treatment response, the response rate of hepatic LCH patients to initial first-line therapy (n=89) was 22.5%. However, there was no significant difference in the response rate or recurrence rate between patients who shifted from first-line treatment to second-line treatment (n=29) or to targeted therapy (n=13) (P=0.453 and 1.000). The response rate of hepatic LCH patients who received initial second-line therapy (n=13) was 38.5%. Two of these patients subsequently experienced bone recurrence. The response rate of hepatic LCH patients who received initial targeted therapy (n=16) was 75.0%. Three patients subsequently experienced recurrence, including 2 in the bone and 1 in the liver and skin. A total of 39.3% of patients who received second-line treatment had severe myelosuppression (grade III-IV), and 50.8% had varying degrees of gastrointestinal events, whereas there was no severe toxicity in patients who received first-line treatment and targeted therapy. Four patients underwent liver transplantation because of liver cirrhosis. The patients' liver disease improved within a follow-up period of 18-79 months. This study demonstrated that LCH with liver involvement, especially cholangitis, indicates a poor prognosis. Targeted therapy provides a good treatment response and less toxicity. However, it may relapse after withdrawal. Liver transplantation is still a reliable salvage option for patients with end-stage liver disease.
Objectives:
Bronchogenic cyst is a rare congenital disease which occurs especially in the neck region. This report presents 6 cases of bronchogenic cysts and discusses the diagnosis and surgical ...experience of this anomaly.
Methods:
A retrospective study of 6 pediatric patients with cervical bronchogenic cysts treated in our hospital during 2016 to 2019 was performed. We recorded and analyzed the clinical data of the patients, including age, symptoms, imaging findings, surgical procedure, and complications.
Results:
All patients underwent surgical excision. The chondroid tissues were found at the base of cysts which clung to the trachea in 5 patients and completely removed by surgery without recurrence. One patient showed recurrence due to residual cartilage after the first surgery, and the second surgery was required to resect the remaining cartilage. During the surgery, the recurrent laryngeal nerve (RLN) detector was used, which confirmed that all the RLNs clung to the side wall of cysts. All cases were cured without complications.
Conclusions:
Although rare, bronchogenic cysts should be considered in the differential diagnosis of peritracheal masses in children. Complete resection of the bronchogenic cysts, including the cartilages at the base, is vital in preventing recurrence. The RLN must be protected during the surgery.
The recent advancements in multimodal dialogue systems have been gaining importance in several domains such as retail, travel, fashion, among others. Several existing works have improved the ...understanding and generation of multimodal dialogues. However, there still exists considerable space to improve the quality of output textual responses due to insufficient information infusion between the visual and textual semantics. Moreover, the existing dialogue systems often generate defective knowledge-aware responses for tasks such as providing product attributes and celebrity endorsements. To address the aforementioned issues, we present a Transformer-based Multimodal Infusion Dialogue (TMID) system that extracts the visual and textual information from dialogues via a transformer-based multimodal context encoder and employs a cross-attention mechanism to achieve information infusion between images and texts for each utterance. Furthermore, TMID uses adaptive decoders to generate appropriate multimodal responses based on the user intentions it has determined using a state classifier and enriches the output responses by incorporating domain knowledge into the decoders. The results of extensive experiments on a multimodal dialogue dataset demonstrate that TMID has achieved a state-of-the-art performance by improving the BLUE-4 score by 13.03, NIST by 2.77, image selection Recall@1 by 1.84%.
Massive thymic hyperplasia (MTH) is a very rare entity, with fewer than 20 cases reported in the literature in infancy. Most patients have respiratory symptoms and the enlarged thymus gland occupies ...one side of the thoracic cavity. Posterolateral thoracotomy or median sternotomy is the main treatment for MTH in infants. We report a case of an infant with MTH in which the enlarged thymus occupied his bilateral thoracic cavity and he underwent video-assisted thoracoscopic surgery (VATS). In addition, we reviewed and summarized the relevant literature.
A 4-month-old boy was admitted to the hospital with no apparent cause of dyspnea for 18 days, with cough and sputum. On examination, the patient was found to have cyanotic lips, diminished breath sounds in both lungs, and a positive three concave sign. There was no fever or ptosis. Preoperative imaging showed large soft tissue shadows in the bilateral thoracic cavity, with basic symmetry between the right and left sides. Tumor markers were within the normal range. Ultrasound-guided fine needle biopsy showed normal thymic structures with no evidence of malignancy. As his symptoms worsened, he eventually underwent unilateral thoracic approach video-assisted thoracoscopic exploratory surgery, during which a large mass occupying the bilateral thoracic cavity was removed in a separate block and part of the thymus in the left lobe was preserved. Pathological examination confirmed true thymic hyperplasia (TTH). No relevant complications occurred at the 2-month postoperative follow-up.
In infants, MTH occupying the bilateral thoracic cavity can produce severe respiratory and circulatory symptoms due to occupying effects. Although a definitive preoperative diagnosis is sometimes difficult, after combining computed tomography (CT) and fine needle biopsy to exclude evidence of other malignancies, the enlarged thymus occupying the bilateral thoracic cavity can be resected
VATS. Whether the enlarged thymus occupies the bilateral thoracic cavity and the size of the thymus are not absolute contraindications to thoracoscopic surgery. The method is safe, feasible, and minimally invasive to the patient.
Indocyanine Green (ICG) fluorescence imaging has been widely used in the surgical treatment of adult renal cancers, but its application in pediatric renal cancers has rarely been reported. This study ...aims to summarize the experience of ICG fluorescence imaging in pediatric renal cancers and explores its safety and feasibility.
The clinical features, surgical information, ICG administration regimen, near infrared radiography data
and ex vivo and pathological results of children with renal cancers using ICG navigation were analyzed and summarized.
There were 7 cases of renal cancer, including 4 cases of Wilms tumor (WT), 1 case of malignant rhabdoid tumor of the kidney (MRTK) and 2 cases of renal cell carcinoma (RCC). By intraoperative intravenous injection of ICG from 2.5 to 5 mg (0.05-0.67 mg/kg), the tumors were visualized in 6 cases
or ex vivo, and the tumor visualization failed in 1 case due to renal artery embolization before operation. By injecting 5 mg ICG into the normal renal tissue during the operation, 3 patients achieved fluorescent localization of sentinel lymph nodes. No ICG-related adverse reactions were found in any of the patients during or after operation.
ICG fluorescence imaging is safe and feasible for renal cancers in children. Intraoperative administration can achieve tumor and sentinel lymph node visualization which will facilitate the development of nephron sparing surgery (NSS). However, the technique is affected by ICG dose, anatomical conditions around the tumor, and renal blood flow. A proper dose of ICG and the complete removal of perirenal fat are helpful for the fluorescence imaging of the tumor. It has potential in the operation of renal cancer in children.
ABSTRACT
Introduction
Cardiac neoplasms are particularly rare in children, and the majority of these tumors are benign. Approximately 10% of cardiac neoplasms are malignant, including soft tissue ...sarcomas and lymphomas. Cardiac tumors could also be metastases. Primitive EWSR1‐negative round or spindle cell undifferentiated sarcoma harboring CIC gene translocation is a highly aggressive malignancy mainly occurring in soft tissues. However, it has not yet been described in the heart.
Case presentation
We report a sarcoma that arose from the right ventricle in a 1‐year‐old girl. Histologically, it was composed of closely arranged small round or oval undifferentiated cells with fibrovascular separation, hyaline degeneration, and geographical necrosis. Immunohistochemically, the neoplastic cells exhibited focal membrane positivity for CD99 and diffuse positivity for WT1 and ETV4. Fluorescent in situ hybridization analysis showed EWSR1‐negative but CIC‐positive split signals. The breakpoint was also confirmed by whole genome sequencing.
Conclusion
Based on morphological, immunohistochemical and molecular findings, this cardiac mass was diagnosed as CIC‐rearranged sarcoma.
Importance
Neuroblastoma is the most common extracranial malignant solid tumor in children. Multidisciplinary care is critical to improving the survival of pediatric patients with neuroblastoma.
...Objective
To systematically summarize the clinical characteristics of children with neuroblastoma and evaluate their prognosis with multidisciplinary care provided in a single center.
Methods
This retrospective study analyzed the clinical data of 1041 patients with neuroblastoma who were diagnosed, treated, and followed‐up in the Hematology‐Oncology Center of Beijing Children’s Hospital from 2007 to 2019.
Results
The median age at diagnosis was 34 months; 80.8% of the patients were younger than 5 years of age. Notably, 243 patients (23.3%) were classified as low‐risk, 249 patients (23.9%) were classified as intermediate‐risk, and 549 (52.7%) were classified as high‐risk. Furthermore, 956 patients underwent surgical resections; 986 (94.7%) patients received chemotherapy; and 176 patients with high‐risk neuroblastoma received hematopoietic stem cell transplantation. The 5‐year event‐free survival (EFS) rate was 91.3% and 5‐year overall survival (OS) rate was 97.5% in low‐risk group; in the intermediate‐risk group, these rates were 85.1% and 96.7%, respectively, while they were 37.7% and 48.9% in the high‐risk group (P < 0.001 for both). The 5‐year EFS and OS rates were significantly higher in patients diagnosed between 2015 and 2019 than in patients diagnosed between 2007 and 2014 (P < 0.001). In total, 278 patients (26.7%) exhibited tumor relapse or progression; the median interval until relapse or progression was 14 months. Of the 233 patients who died, 83% died of relapse or progression of neuroblastoma and 4.3% died of therapy‐related complications.
Interpretation
The 5‐year OS rate was low in high‐risk patients, compared with low‐and intermediate‐risk patients. Multidisciplinary care is critical for improvement of survival in pediatric patients with neuroblastoma. Additional treatment strategies should be sought to improve the prognosis of patients with high‐risk neuroblastoma.
A multidisciplinary modality for the diagnosis and treatment of neuroblastoma is the key to improve the survival of children with neuroblastoma.
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