Fetal mesenchymal hamartoma of the liver: A case report and literature review.

This paper reports the diagnosis and treatment process of a case of fetal mesenchymal hamartoma of the liver (MHL), and reviews the previous literature reports. At 38+2 weeks of gestation, prenatal ultrasound found a well bound mixed solid and cystic mass, which was located at the lower edge of the right lobe of the liver and in front of the right kidney of the fetus, but the source and nature of the mass were not clear by ultrasound. Due to the approaching due date, the fetus showed no other abnormal symptoms, and no special treatment was given with the consent of the family members. A female fetus was delivered weighing 3,520 g at 39 weeks. An exploratory laparotomy was performed on the eighth day after delivery. During the operation, it was found that the tumor originated from the fifth, sixth and seventh hepatic segment and the corresponding hepatic segments were removed. Recovery was uneventful and the infant was discharged on the 6th day after surgery. Follow-up at 2 years showed a thriving young girl, and there was no tumor recurrence.

Tumor Microenvironment Profiling Identifies Prognostic Signatures and Suggests Immunotherapeutic Benefits in Neuroblastoma.

The tumor microenvironment (TME) influences disease initiation and progression. Cross-talks of cells within TME can affect the efficacy of immunotherapies. However, a precise, concise, and comprehensive TME landscape in neuroblastoma (NB) has not been established. Here, we profiled the TME landscape of 498 NB-related patients on a self-curated gene list and identified three prognostic TMEsubgroups. The differentially expressed genes in these three TMEsubgroups were used to construct a genetic signature of the TME landscape and characterize three GeneSubgroups. The subgroup with the worst overall survival prognosis, the TMEsubgroup/GeneSubgroup3, lacked immune cell infiltration and received the highest scores of MYCN- and ALK-related signatures and lowest scores of immune pathways. Additionally, we found that the GeneSubgroup3 might be benefited from anti-GD2 instead of anti-PD-1 therapy. We further created a 48-gene signature, the TMEscore, to infer prognosis and validated it in three independent NB cohorts and a pan-cancer cohort of 9,460 patients. We did RNA-seq on 16 samples and verified that TMEscore was higher in patients with stage 3/4 than stage 1/2 diseases. The TMEscore could also predict responses for several immunotherapies. After adding clinical features, we found that the nomogram-based score system, the TMEIndex, surpassed the current risk system at predicting survivals. Our analysis explained TME at the transcriptome level and paved the way for immunotherapies in NB.

Analysis of the Clinical Characteristics of Spontaneous Bile Duct Perforation in Children.

Objective: This study aimed to explore the etiology, clinical features, diagnosis, and treatment of spontaneous bile duct perforation (SBDP) in children. Methods: The clinical data of children with SBDP who were admitted to Wuhan Children's Hospital between January 2014 and January 2020 were retrospectively analyzed. Results: In all, 28 cases of children with SBDP (male, 28.6%; female, 71.4%; male-to-female ratio, 1:2.5; average age, 2.15 years) were analyzed. The most common symptoms were fever (85.7%), nausea and vomiting (78.6%), and abdominal distension (67.9%). Among the 28 patients, 26 (92.9%) had elevated hypersensitive C-reactive protein, 24 (85.7%) had an increased neutrophil percentage, and 22 (78.6%) had raised peripheral blood leukocyte counts. Moreover, 19 patients (67.9%) showed increased serum total bilirubin levels, and 5 (17.9%) showed an elevated conjugated bilirubin level. Abdominal CT examination revealed that the gallbladder wall of patients was thickened with edema, accompanied by gallbladder stenosis and gallbladder mucosa enhancement; furthermore, ascites was found in the abdominal cavity and lesser omental bursa. Twenty-two patients underwent abdominal paracentesis, and 20 (90.9%) of them were exposed to bile-based ascites. Among the 28 patients, four recovered with conservative treatment, whereas the others (85.7%) were surgically treated. Of the twenty-four patients undergoing surgery, the perforation site was found at the union of the hepatic and cystic ducts in 12 patients (50%), no perforation site was observed in 9 patients (37.5%), and a common hepatic duct was observed in 3 patients (12.5%). All 24 patients underwent stage I surgery, and temporary biliary drainage was performed because of severe abdominal inflammation. Cholangiography and enhanced CT revealed an abnormal location of the pancreatic duct joining the bile duct in 64.3% patients. Following surgery, 15 patients underwent hepaticojejunostomy. Subsequently, 3-month to 6-year follow-up (median, 30 months) indicated that the patients recovered well with no serious complications. Conclusion: SBDP in children may be associated with pancreaticobiliary malunion (PBM) and congenital weakness of the bile duct wall. However, the clinical manifestations of this condition lack specificity; this limitation can be assisted through diagnosis via abdominal CT and by performing abdominal paracentesis. Once SBDP diagnosis is confirmed, the patient should follow the principles of individualized treatment.

Primary Omental Lipoma in a Child: A Case Report and Literature Review.

BACKGROUND: Lipoma is a common benign tumor derived from adipose tissue, with an incidence of nearly 10%. It is the most common mesenchymal tumor throughout the body. However, the pathogenesis of lipoma is not clear yet, and the increased incidence is attributable to obesity, elevated serum cholesterol, diabetes, trauma, radiation, familial predisposition, and chromosome. Primary omental tumor is a rare lipoma occurring in the greater omentum, most of which is reported in the form of clinical case reports. Nevertheless, primary omental tumor is even rarer in children. To date, there have been few reports of clinical cases. CASE PRESENTATION: We report a rare case of primary omental lipoma in a 6-year-old boy. After an accidental fall, a CT scan found that he had a tumor in the left upper abdomen. He had no history of abdominal pain, abdominal mass, vomiting, etc. The boy was admitted to the hospital within 3 days, and was diagnosed with an intra-abdominal tumor. After admission, abdominal ultrasound and enhanced CT showed a 71 ×40 ×60 mm mass in the left middle abdomen, which was considered a lipoma. There was no abnormality in tumor markers. Through laparoscopic surgery, intraoperative exploration revealed that the tumor was located in the left mid-upper abdomen, and was yellow, solid, soft, and isolated. The intraoperative diagnosis was an omental lipoma. We used an ultrasonic knife to resect the omentum close to the base of the tumor. The tumor was completely resected, put in a retrieval bag and sealed. Finally, the left and right sides of the umbilical incision were extended to take out the tumor tissue. The child received liquid food 6 h after the operation and was discharged 3 days later. The postoperative pathological diagnosis was an omental lipoma. He was seen at follow-up 3 months after discharge and had no complaints, an abnominal ultrasound showed no tumor recurrence. CONCLUSION: Primary omental lipoma in children is a rare benign tumor of the omentum. Its etiology and pathology are not clear. US, CT, and MRI can facilitate clinical diagnosis and preoperative evaluation. Laparoscopic surgery is an effective treatment, and the prognosis of children is favorable. This case is beneficial to improve the clinical knowledge of pediatric surgeons about this rare disease.

Risk factors of recurrent acute pancreatitis after conservative treatment in pediatric population: a single-center study.

Background: Although complete resolution and recovery occurs in most children with an initial attack of acute pancreatitis (AP), a subset of children may progress to recurrent AP (RAP). RAP has serious effects to the individual and the socioeconomic burden. The aim of this project was to identify the independent risk factors for pediatric RAP so as to provide evidence for its prevention, early diagnosis and treatment. Methods: A retrospective cohort study of children discharged from Tianjin Children's Hospital from June 2017 to January 2020 was performed. Demographic and clinical variables, treatment strategies, clinical course and outcomes were collected. Independent risk factors of RAP were identified using the logistic regression model. Results: Of the total 96 enrolled children, 30 (31.3%) developed RAP during the follow-up period. The majority (27/30, 90%) of the children with AP developed RAP within 6 months of their first AP attack. The presence of systemic inflammatory response syndrome (SIRS) [odds ratio (OR)=6.652, 95% confidence interval (CI) 1.989 to 22.247], fasting time (OR=1.267, 95% CI 1.104 to 1.583), whether meet all three AP diagnostic criteria (OR=7.438, 95% CI 1.346 to 41.103) and abnormal amylase/lipase value on the seventh day of hospitalization (OR=3.601, 95% CI 0.972 to 13.342) were independent risk factors of RAP in children. Conclusions: Most children who developed RAP had progressed within 6 months after their first episode of AP. RAP was more common in children who met all three AP diagnostic criteria at initial attack and in children with SIRS, long fasting time and abnormal amylase/lipase value on the seventh day of hospitalization.

The Roles of Neuropilin 2/VEGF-C Axis in a Series of Recurrent Lymphangioma.

INTRODUCTION: Vascular endothelial growth factor (VEGF) and its receptor act as a major contributor to lymphangioma, but their role on nonrecurrent and recurrent lymphangiomas remain unclear. We aim to investigate those factors in the generation of recurrent lymphangioma. MATERIALS AND METHODS: Patients diagnosed with lymphangioma from January 2005 to December 2012 in our hospital were collected and divided into nonrecurrent and recurrent lymphangiomas. The clinical characteristics including age, sex, symptoms, location, and size of lymphangioma were collected. Surgical resection samples were collected for histology, protein and mRNA detection of VEGF-C, VEGF receptor-3 (VEGFR-3), and neuropilin 2 (Nrp2). Follow-ups including lymphangioma recurrent and the local symptoms such as ulcer were reviewed. RESULTS: A total of 80 patients aged from 5 months to 12 years were enrolled in this study, 51 patients had no recurrence and other 29 patients suffered from recurrent lymphangioma. There was no significant difference in demographic data and clinical characters between the two groups (p > 0.05). Immunohistochemistry staining showed that VEGFR-3 remained unchanged between nonrecurrent and recurrent lymphangiomas (p > 0.05), and VEGF-C and Nrp2 were significantly increased in recurrent lymphangioma compared with nonrecurrent lymphangioma (p < 0.05). The same expression trend was proved as detected by protein and mRNA levels. CONCLUSION: The VEGF-C/Nrp2 axis was significantly increased in the recurrent lymphangioma, indicating that VEGF-C/Nrp2 targeted therapy may serve as a potential therapeutic strategy for recurrent lymphangioma.

Serum substance P and vasoactive intestinal peptide levels in infants with acute intussusception.

OBJECTIVE: We aimed to investigate the role of the intestinal neurotransmitters vasoactive intestinal peptide (VIP) and substance P (SP) at different time points in infants with acute intussusception. METHODS: Thirty patients who were diagnosed with acute intussusception were enrolled in the study and classified as the experimental group. Another 30 patients with an indirect inguinal hernia who had no intestinal injury were included as the control group. Serum SP and VIP levels at different time points, including pre- and postoperation, were detected by enzyme-linked immunosorbent assay and compared between the two groups. RESULTS: Serum SP levels in patients with acute intussusception were significantly higher than those in controls. However, with recovery of acute intussusception, SP levels gradually decreased after treatment. Serum VIP levels in patients with acute intussusception were significantly lower than those in controls. However, with recovery of acute intussusception, VIP levels gradually increased after treatment. CONCLUSIONS: SP and VIP levels may have a potential relationship with the pathogenetic process of intussusception.

Gastroduodenal perforation in the pediatric population: a retrospective analysis of 20 cases.

OBJECTIVE: The aim of this study was to investigate the pathogenesis, symptoms and individualized surgical management in pediatrics with gastroduodenal perforation (GDP). METHODS: Patients diagnosed with GDP from January 2013 to December 2016 in our hospital were collected and divided into gastric perforation (GP) group and duodenal perforation (DP) group. Demographics, clinical events, etiological factors, symptoms, the time from symptom onset to operation, intraoperative findings and surgical procedures were analyzed. Follow-ups including ulcer, perforations occurrence, and digestive symptoms were carried out by out-patient review or telephones. RESULTS: A total of 20 patients aged from 3 months to 14 years were enrolled in this study. The average age, main clinical presentations, size of perforations and operating time between two groups had no difference. The male to female ratio in DP group was higher than GP (P < 0.05). The high risk factor for DP was the use of dexamethasone, and for GP was HP infection. The most common site of perforation in DP group was duodenal bulb, and in GP group was pylorus area. Simple suture is the main management for both DP and GP, but distal gastrectomy combined with gastrojejunal Roux-en-Y anastomosis may be an alternative procedure for large perforation with diameter > 2 cm. The length of hospital days in GP group is shorter than DP group (P < 0.05). For follow-up, no patients had digestive symptoms. CONCLUSIONS: The general condition had no difference between GP and DP patients. But the risk factors and surgical repair differ depending on the patient's fundamental illness and the complexity of the perforation.

Clinical Practice Experiences in Diagnosis and Treatment of Traumatic Brain Injury in Children: A Survey among Clinicians at 9 Large Hospitals in China.

Proper diagnosis and treatment of traumatic brain injury (TBI) in children is becoming an increasingly problematic issue in China. This study investigated Chinese clinicians to provide information about their knowledge and experiences in diagnosis and treatment of pediatric TBI. We conducted a questionnaire survey among clinicians in the emergency departments and neurosurgery departments at 9 major hospitals in China. The questionnaire included demographic information, and knowledge and experiences regarding the diagnosis and treatment of pediatric TBI. A total of 235 clinicians completed questionnaires. 43.8% of the surveyed clinicians reported children with only scalp hematoma without any other signs and symptoms of concussion as TBI cases. Most clinicians (85.1%) reported no existing uniform diagnostic criteria for children with TBI in China. The majority of clinicians (91.9%) reported that CT scans were performed in all patients with suspected head injury as a routine procedure in their hospitals. Only 20.9% of clinicians believed that radiation from CT scanning may increase cancer risk in children. About 33.6% of the clinicians reported that they ordered CT scans to investigate suspected head injury due to the poor doctor-patient relationship in China, and to protect themselves against any medical lawsuits in the future. About 80% of the clinicians reported that there are no existing pediatric TBI treatment guidelines in China. Instead a senior doctor's advice is the most reported guidelines regarding treating pediatric TBI (66.0%). All of the surveyed clinicians reported that the lack of diagnosis and/or treatment standard is the biggest problem in effectively diagnosing and treating pediatric TBI in China. Developing guidelines for the diagnosis and treatment of children with TBI is a high priority in China. The extremely high usage of CT for pediatric TBI in China suggests that it is important to establish evidence-based clinical decision rules to help Chinese clinicians make diagnostic and therapeutic decisions during their practice in order to identify children unlikely to have a clinically-important TBI who can be safely discharged without a CT scan.

Laparoscopic vs. laparoscopically assisted management of Meckel's diverticulum in children.

To investigate the management of Meckel's diverticulum in children and the feasibility of using laparoscopic and laparoscopically assisted Meckel's diverticulum resection and intestinal anastomosis according to the different subtypes classified laparoscopically. 55 symptomatic Meckel's diverticulum cases were classified into two categories, the simple and the complex types depending on Meckel's diverticulum appearance upon laparoscopic exploration. Forty-one cases of simple Meckel's diverticulum were treated with simple diverticulectomy during laparoscopy, and 14 cases of complicated Meckel's diverticulum were treated with laparoscopically assisted Meckel's diverticulum resection and intestinal anastomosis. The operation time for the laparoscopically assisted was significant longer than laparoscopic-only surgeries [45~123 min (54.57 ± 20.17min) vs 29~78min (38.85 ± 9.75 min)], P = 0.013. Among the 55 cases, Just one child with simple type MD during laparoscopic exploration, and presented a diverticulum with a base that was considered to be in the mesangial margin. The remaining 54 patients were cured, and follow-up for 4~36 months revealed that they did not present abdominal pain, and no hematochezia occurred as a complication. Surgery selection either laparoscopy only or transumbilical laparoscopically assisted intestinal resection and intestinal anastomosis by laparoscopic exploration for Meckel's diverticulum treatment, based on the type of Meckel's diverticulum in children, is safe, feasible, and effective.

Inactivation of Smad4 is a prognostic factor in intrahepatic cholangiocarcinoma.

BACKGROUND: Smad4 is found mutated in many cancers. It acts as a tumor suppressor in the regulation of TGF-ß signaling pathway. The objective of this work was to study the expression of Smad4 in intrahepatic cholangiocarcinoma (ICC) and its relationship with the biological behavior and prognosis of the disease. METHODS: Forty-nine paraffin-embedded ICC specimens and nine normal liver tissues were analyzed by immunohistochemical methods using Smad4 monoclonal antibodies. The expression of Smad4 was compared with the clinical pathological characteristics of the patients. RESULTS: The expression of Smad4 was 100% positive in normal liver tissues, which was higher than that in the ICC (44.9%). Negative labeling of the Smad4 protein was found in 26.1% (6/23) of well-differentiated ICCs and 61.5% (16/26) of poorly to moderately differentiated ICCs, and 34.3% (12/35) and 71.4% (10/14) showed negative Smad4 labeling (P = 0.018) of ICC at pathological Tumor Node Metastasis (pTNM) stage I-II and pTNM stage III-IV separately. Furthermore, 72% (8/11) of lymph node metastatic ICCs and 73.3% (11/15) of intrahepatic metastatic ICCs showed negative labeling of the Smad4 protein. The loss of Smad4 expression in those metastatic ICCs was significantly more severe compared with non-metastatic ICCs (P = 0.000). CONCLUSIONS: The expression of Smad4 was associated with the histological grade, clinical stage, and metastasis of ICC (P < 0.05). The detection of Smad4 may be helpful in determining the degree of malignancy and prognosis of ICC.