Nickel-Catalyzed Direct Sulfonylation of Styrenes and Unactivated Aliphatic Alkenes with Sulfonyl Chlorides.

A nickel-catalyzed direct sulfonylation of alkenes with sulfonyl chlorides has been developed using 1,10-phenanthroline-5,6-dione as the ligand. Unactivated alkenes and styrenes including 1,1-, 1,2-disubstituted alkenes can be subjected to the protocol, and a wide range of vinyl sulfones was obtained in high to excellent yields with good functional group compatibility. Notably, the process did not allow the desulfonylation of sulfonyl chloride or chlorosulfonylation of alkenes. Radical-trapping experiment supported that a sulfonyl free-radical was likely produced and triggered subsequent transformation in the process.

Genetic Landscape and Its Prognostic Impact in Children With Langerhans Cell Histiocytosis.

CONTEXT.­: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm that predominantly affects young children. OBJECTIVE.­: To investigate genetic alterations and their correlation with clinical characteristics and prognosis in pediatric LCH. DESIGN.­: We performed targeted sequencing to detect mutations in LCH lesions from pediatric patients. RESULTS.­: A total of 30 genomic alterations in 5 genes of the MAPK pathway were identified in 187 of 223 patients (83.9%). BRAF V600E (B-Raf proto-oncogene, serine/threonine kinase) was the most common mutation (51.6%), followed by MAP2K1 (mitogen-activated protein kinase kinase 1) alterations (17.0%) and other BRAF mutations (13.0%). ARAF (A-Raf proto-oncogene, serine/threonine kinase) and KRAS (KRAS proto-oncogene, GTPase) mutations were relatively rare (2.2% and 0.9%, respectively). Additionally, FNBP1 (formin-binding protein 1)::BRAF fusion and MAP3K10 (mitogen-activated protein kinase kinase 10) mutations A17T and R823C were identified in 1 case each, with possible constitutive activation of ERK1/2 phosphorylation. BRAF V600E was more frequent in patients with risk organ involvement, while MAP2K1 mutation was more prevalent in patients with single-system LCH (P = .001). BRAF V600E was associated with craniofacial bone, skin, liver, spleen, and ear involvement (all P < .05). Patients with other BRAF mutations had a higher proportion of spinal column involvement (P = .006). Univariate analysis showed a significant difference in progression-free survival among the 4 molecular subgroups for patients treated with first-line therapy (P = .02). According to multivariate analysis, risk organ involvement was the strongest independent adverse prognostic factor (hazard ratio, 8.854; P < .001); BRAF or MAP2K1 mutation was not an independent prognostic factor. CONCLUSIONS.­: Most pediatric patients with LCH carry somatic mutations involving the MAPK pathway, correlating with clinical characteristics and outcomes for first-line chemotherapy.

Characteristics of patients with recurrent retinoblastoma: a survival analysis.

BACKGROUND: Management guidelines and corresponding survival data for patients with recurrent retinoblastoma (RB) are lacking. This study aimed to summarize the clinical characteristics of patients with recurrent RB and analyze their survival outcomes. METHODS: We retrospectively analyzed 68 patients with recurrent RB who underwent treatment in our institution from January 2016 to December 2020. Patients were grouped according to location of recurrence: intraocular, orbital, and distant metastasis. RESULTS: The male:female ratio was 1.3:1 and the median age at recurrence was 37.5 months (range, 30.3-62.8). The number of patients in the intraocular recurrence, orbital recurrence, and metastasis groups was 13 (19.1%), 23 (33.8%), and 32 (47.1%), respectively. Thirty patients died, 36 were alive at last follow-up, and two were lost to follow-up. Eye enucleation was performed in 94.1% of patients. Five-year overall survival in patients with intraocular recurrence, orbital recurrence, and metastasis was 84.6%, 69.6%, and 31.3%, respectively (P = 0.001). Most deaths occurred within 2 years of recurrence. Presence of high-risk pathological factors, central nervous system invasion, and absence of combination therapy were independent predictors of worse 5-year overall survival. CONCLUSION: The rate of eye preservation in survivors of recurrent RB was very low. Although 5-year overall survival in patients who underwent treatment for intraocular and orbital recurrence was high, it was low in those with metastasis. RB patients may need lifelong follow-up for recurrence and secondary malignancy.

BLK positively regulates TLR/IL-1R signaling by catalyzing TOLLIP phosphorylation.

TLR/IL-1R signaling plays a critical role in sensing various harmful foreign pathogens and mounting efficient innate and adaptive immune responses, and it is tightly controlled by intracellular regulators at multiple levels. In particular, TOLLIP forms a constitutive complex with IRAK1 and sequesters it in the cytosol to maintain the kinase in an inactive conformation under unstimulated conditions. However, the underlying mechanisms by which IRAK1 dissociates from TOLLIP to activate TLR/IL-1R signaling remain obscure. Herein, we show that BLK positively regulates TLR/IL-1R-mediated inflammatory response. BLK-deficient mice produce less inflammatory cytokines and are more resistant to death upon IL-1ß challenge. Mechanistically, BLK is preassociated with IL1R1 and IL1RAcP in resting cells. IL-1ß stimulation induces heterodimerization of IL1R1 and IL1RAcP, which further triggers BLK autophosphorylation at Y309. Activated BLK directly phosphorylates TOLLIP at Y76/86/152 and further promotes TOLLIP dissociation from IRAK1, thereby facilitating TLR/IL-1R-mediated signal transduction. Overall, these findings highlight the importance of BLK as an active regulatory component in TLR/IL-1R signaling.

Prognostic factors for intermediate- or high-risk neuroblastomas in children in China.

BACKGROUND: Evidence regarding the characteristics and prognosis of neuroblastoma (NBL) in China is limited. We aimed to investigate the characteristics and prognosis of intermediate- or high-risk NBL in children in China. METHODS: We included 147 patients with intermediate- or high-risk NBL evaluated from January 2006 to March 2015. The patients were aged 1 month to 15.5 years, 66% of them were boys, and 117 (79.6%) were diagnosed with high-risk NBL. RESULTS: After a median follow-up of 32.5 months, 80 (45.6%) patients survived, with a median survival time of 48 months (95% confidence interval [CI]: 36.41-59.59). High-risk patients (hazard ratio [HR]: 12.467; 95% CI: 11.029-12.951), partial response (PR) (HR: 1.200; 95% CI: 1.475-2.509) or progression disease (PD) (HR: 1.924; 95% CI: 1.623-3.012) after induction chemotherapy, and intracranial metastasis (HR: 3.057; 95% CI: 0.941-4.892) were independent risk factors for survival (p < 0.05) and postrelapse survival (p < 0.05). NBL relapse, male sex, and PR or PD after induction chemotherapy were risk factors for event-free survival (p < 0.05). CONCLUSIONS: In addition to previously established independent risk factors, such as age, risk group, and relapse, efficacy of induction chemotherapy and intracranial metastasis play significant roles in the prognosis of NBL.

Tyrosine phosphorylation of IRF3 by BLK facilitates its sufficient activation and innate antiviral response.

Viral infection triggers the activation of transcription factor IRF3, and its activity is precisely regulated for robust antiviral immune response and effective pathogen clearance. However, how full activation of IRF3 is achieved has not been well defined. Herein, we identified BLK as a key kinase that positively modulates IRF3-dependent signaling cascades and executes a pre-eminent antiviral effect. BLK deficiency attenuates RNA or DNA virus-induced ISRE activation, interferon production and the cellular antiviral response in human and murine cells, whereas overexpression of BLK has the opposite effects. BLK-deficient mice exhibit lower serum cytokine levels and higher lethality after VSV infection. Moreover, BLK deficiency impairs the secretion of downstream antiviral cytokines and promotes Senecavirus A (SVA) proliferation, thereby supporting SVA-induced oncolysis in an in vivo xenograft tumor model. Mechanistically, viral infection triggers BLK autophosphorylation at tyrosine 309. Subsequently, activated BLK directly binds and phosphorylates IRF3 at tyrosine 107, which further promotes TBK1-induced IRF3 S386 and S396 phosphorylation, facilitating sufficient IRF3 activation and downstream antiviral response. Collectively, our findings suggest that targeting BLK enhances viral clearance via specifically regulating IRF3 phosphorylation by a previously undefined mechanism.

Clinical analysis of 2790 children with retinoblastoma: a single-center experience in China.

BACKGROUND: In this study, we aimed to analyze the clinical characteristics and prognosis of children with retinoblastoma (RB) in a single center in China with a large sample collection spanning 17 years. METHODS: The clinical data of 2790 children with RB treated in Beijing Tongren Hospital from 2005 to 2021 were collected, and a retrospective analysis was conducted. RESULTS: The median age of the participants was 28.3 months. There were 3624 affected eyes, 12.4% of which were in groups A-C, 67.1% in groups D-E and 16.2% were not specified. The primary symptom observed in most cases was a white pupil, accounting for 66.5%, followed by strabismus (12.8%). The median follow-up time was 59.7 months. The enucleation rate was 71.3% (703/986) in a single left eye and 72.5% (702/968) in a single right eye. The overall survival (OS) rate was 95.8% (2444/2552) because 237 patients dropped out, and 109 died. Kaplan‒Meier survival analysis showed that the median survival time (MST) was 125.92 months [95% confidence interval (CI) = 124.83-127.01]. Cox multivariate survival analysis showed that trilateral RB (P = 0.017), metastasis site (P = 0.001), and combined distant tissue metastasis (P = 0.001) were independent prognostic factors for RB. The OS of 44 cases of familial RB was 93.2% (41/44), with an MST of 80.62 months (95% CI = 67.70-93.54). CONCLUSIONS: The timing of eye protection treatment and enucleation should be comprehensively judged to avoid worsening prognosis due to operation time delay. More importantly, the promotion and popularization of diagnosis and treatment technologies are necessary to further improve RB prognosis.

Progestin-Primed Ovarian Stimulation Protocol for Patients With Endometrioma.

Objective: To evaluate the pregnancy outcomes of progestin-primed ovarian stimulation (PPOS) protocol for patients with endometrioma underwent in vitro fertilization/intra-cytoplasmic sperm injection embryo transfer (IVF/ICSI-ET). Design: Observational retrospective cohort study. Setting: University affiliated reproductive center. Study Participants: 605 infertile patients with endometrioma underwent IVF/ICSI-ET from January 2016 to March 2021 were included in this study. Methods: Multivariable logistic regression analyses were conducted to determine the independent effect of controlled ovarian stimulation (COS) protocols on reproductive outcomes of first embryo transfer (ET) cycles. The live birth was primary outcome, the implantation rate, biochemical pregnancy, clinical pregnancy and ongoing pregnancy were secondary outcomes. Results: Compared to PPOS protocol, the probability of implantation showed no significant difference with ultra-long gonadotrophin-releasing hormone agonist (GnRHa) protocol and gonadotrophin-releasing hormone antagonist (GnRHant) protocol (OR 1.7, 95% CI 0.9-3.1, OR 1.2, 95% CI 0.7-2.1, respectively). The PPOS protocol was correlated with a significantly lower biochemical pregnancy and clinical pregnancy than ultra-long GnRHa protocol in the multivariable logistic regression analysis (OR 2.3, 95% CI 1.1-4.9, OR 2.4, 95% CI 1.1-5.3, respectively). However, there was no significant difference in terms of biochemical pregnancy, clinical pregnancy and ongoing pregnancy between PPOS and GnRHant protocol (OR 1.4, 95% CI 0.7-2.7, OR 1.3, 95% CI 0.7-2.4, OR 1.1, 95% CI 0.6-2.3, respectively). In addition, compared to PPOS protocol, ultra-long GnRHa protocol and GnRHant protocol demonstrated no statistical difference in ongoing pregnancy (OR 2.0, 95% CI 0.9-4.5, OR 2.1, 95% CI 0.6-2.3, respectively). Notably, the ultra-long GnRHa protocol was associated with a significant higher probability of live birth than PPOS protocol both in crude analysis and multivariable logistic regression analysis (OR 2.6, 95% CI 1.3-5.1, OR 2.5, 95% CI 1.1-5.7, respectively). Nevertheless, no statistical difference was found in live birth between PPOS and GnRHant protocol either in crude analysis and multivariable logistic regression analysis (OR1.2, 95% CI 0.6-2.3, OR 1.2, 95% CI 0.6-2.5, respectively). Conclusions: Based on the reproductive outcomes of the first ET cycles in patients with endometrioma, PPOS protocol may associated with inferior reproductive outcomes in terms of biochemical pregnancy, clinical pregnancy and live birth than ultra-long GnRHa protocol. However, there was no significant difference in implantation rate, clinical pregnancy, ongoing pregnancy and live birth between PPOS and GnRHant protocol.

Clinical Analysis of 16 Distant Metastatic Retinoblastoma Cases with Event-Free Survival.

AIM: This study aimed to summarize the clinical characteristics, treatment, and outcomes of distant metastatic retinoblastoma with event-free survival. DESIGN: Retrospective interventional case series. METHODS: We screened patients with retinoblastoma who survived without events after the comprehensive treatment of distant metastases from June 2015 to February 2021 and collected information regarding their basic characteristics, diagnosis, and treatment. All patients received systemic intravenous chemotherapy. Other treatments included surgical treatment, radiotherapy, intrathecal chemotherapy, and autologous stem cell transplantation. RESULTS: Among 780 hospitalized patients with retinoblastoma in the pediatric ward, a total of 94 patients with retinoblastoma were diagnosed with distant metastases, and 16 patients with distant metastatic retinoblastoma who survived more than 6 months without events were screened, including eight male and eight female patients. The median age of onset was 29 (range, 11-120) months. Among the 16 patients, central nervous system metastasis (8/16), bone metastasis (8/16), bone marrow infiltration (4/16), lymph node metastasis (4/16), and parotid gland metastasis (3/16) were presented. All patients received treatment for more than 6 months, completed their regimen by February 2021, and survived without events. The median survival time after the onset of retinoblastoma was 50.5 (range, 23-102) months, the median survival time after metastasis was 43.5 (range, 16-71) months, and the median event-free survival was 29.0 (range, 6-59) months. CONCLUSION: Metastatic retinoblastoma may benefit from comprehensive treatments including systemic intravenous chemotherapy and hematopoietic stem cell transplantation. However, recurrence after treatment still needs attention, and patients in complete remission still need long-term follow-up.

Synchronous concomitant pancreatic acinar cell carcin and gastric adenocarcinoma: A case report and review of literature.

BACKGROUND: Multiple primary malignant tumors are two or more malignancies in an individual without any relationship between the neoplasms. In recent years, an increasing number of cases have been reported. However, concomitant primary gastric and pancreatic cancer reported a relatively small incidence, involving no pancreatic acinar cell carcinoma reports. Here, we present the first case of concomitant pancreatic acinar cell carcinoma and gastric adenocarcinoma. CASE SUMMARY: A 69-year-old male presented to our department with a history of vomiting, epigastric pain, and weight loss. Imaging revealed space-occupying lesions in the stomach and the tail of the pancreas, respectively. The patient underwent laparoscopic radical gastrectomy and pancreatectomy simultaneously. The pathologies of surgical specimens were completely different: The resected gastric specimen was moderate to poorly differentiated adenocarcinoma, whereas the pancreatic tumor was consistent with acinar cell carcinoma. The patient was treated with six cycles of oxaliplatin and S-1 chemotherapy. As of March 2021, the patient was healthy without any recurrence or metastasis. After thoroughly reviewing the literature on simultaneous pancreatic and gastric cancers at home and abroad, we discussed the clinical characteristics of these rare synchronous double cancers. Most of the cases had undergone surgery and adjuvant chemotherapy, and all of the cases were pathologically confirmed by the postoperative specimen. CONCLUSION: Synchronous pancreatic acinar cells and gastric adenocarcinoma can occur and should be considered when tumors are found in these organs.

Awareness, attitude and barriers of colorectal cancer screening among high-risk populations in China: a cross-sectional study.

OBJECTIVE: To assess the awareness, attitude and barriers of colorectal cancer screening among high-risk populations in China. DESIGN: A cross-sectional study was employed. SETTING: This study was conducted in nine hospitals in Hunan province, China. PARTICIPANTS: Individuals with a high-risk for colorectal cancer were interviewed using a pretested structured questionnaire. PRIMARY AND SECONDARY OUTCOME MEASURES: Knowledge, attitude towards colorectal cancer screening, sociodemographic factors associated with screening knowledge and behaviour and barriers of colorectal cancer screening. RESULTS: This study included 684 participants. The mean knowledge score was 11.86/24 (SD 4.84). But over 70% of them held a positive attitude towards screening. Only 13.3% had undergone colorectal cancer screening. Independent factors related to knowledge were education level of college or above, working as a white collar, higher income, having health insurance, having seen a doctor in the past year and with a high perceived risk (p<0.05). Factors independently associated with screening behaviour included personal history of colorectal disease, having seen a doctor in the past year, previous discussion of colorectal cancer screening, high perceived risk and better knowledge (p<0.05). Main reasons for not undergoing screening were no symptoms or discomfort (71.1%), never having thought of the disease or screening (67.4%) and no doctor advised me (29.8%). CONCLUSION: In China, the majority of high-risk people had deficient knowledge and had never undergone colorectal cancer screening. But most of them held a positive attitude towards the benefits of colorectal cancer screening. This has promising implications to design targeted educational campaigns and establish screening programmes to improve colorectal cancer awareness and screening participation. Healthcare professionals should advise high-risk individuals to participate in screening and inform them about cancer risk.

A new risk-stratification system for hepatoblastoma in children under six years old and the significance for prognosis evaluation-a 14-year retrospective study from a single center.

BACKGROUND: This study explores and analyzes the clinical characteristics and prognostic factors of hepatoblastoma (HB) in children under 6 years old and establishes a new risk-stratification system for individualized therapy. METHODS: The clinical data of 382 pediatric patients under 6 years old (231 males and 151 females) who had been diagnosed with HB by pathology between May 2005 and May 2019 were collected. By analyzing the risk factors influencing the survival rate of patients with HB, a new risk-stratification system was established, and it was compared with previous risk-stratification systems by a receiver operating characteristic (ROC) curve. RESULTS: (1) According to a Kaplan-Meier survival analysis, the one-year, three-year, and five-year overall survival (OS) was 93.7, 84.0, and 73.9%, respectively, and the event-free survival (EFS) was 90.5, 79.2, and 67.5%, respectively. (2) The independent risk factors influencing prognosis in pediatric patients with HB were alpha-fetoprotein (AFP) < 100 ng/ml or > 1000 ng/ml (HR = 3.341, P = 0.005); platelet count > 400 × 109/L (pooled hazard ratio [HR] = 2.123, P = 0.026); PRETEXT stage IV (HR = 4.026, P = 0.001); vascular involvement (HR = 2.178, P = 0.019); distant metastasis (HR = 2.634, P = 0.010);and multifocality (HR = 2.215, P = 0.012). (3) A new risk-stratification system was established and divided into three groups: low risk, moderate risk, and high risk. There were statistical differences among the three groups (P = 0.002). Compared with the previous risk-staging systems, there was no significant difference in the survival rate. Although the effect in the guiding therapy was the same, the area under the curve for the ROC curve was 0.835 (95% CI: 0.784-0.885) for the new stratification system. CONCLUSION: This new risk-stratification system had a better predictive value for the prognosis of pediatric patients with HB than other stratification systems.

Clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma and intracranial segment of Retrobulbar optic nerve invasion.

BACKGROUND: To analyze the clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma (RB) and intracranial segment of retrobulbar optic nerve invasion. METHODS: A total of 14 children with unilateral RB and intracranial segment of retrobulbar optic nerve invasion were enrolled in this retrospective study from January 2009 to December 2018. Clinical characteristics, treatment and prognosis were collected and analyzed. Survival curves were calculated by Kaplan-Meier method. RESULTS: Of 14 cases, there were 7 male and 7 female, ranging in age from 22.85 to 121.97 months (median, 41.03 months). Seventy-one percent of patients came from first-tier cities in China and effected in the left eye. Magnetic resonance imaging (MRI) results indicated that all patients presented with thickened and enhanced optic nerve and intracranial segment of optic nerve invasion. Nine patients received comprehensive therapeutic regimen (chemotherapy, eye enucleation, radiotherapy and intrathecal therapy). The patients were followed up to December 2019, with a median follow-up of 20.6 months. The median disease specific survival was 48.99 ± 8.62 months, and the overall survival (OS) rate was 64.3%. Radiotherapy and comprehensive therapeutic regimen had significant impact on survival time (all p < 0.05). CONCLUSIONS: The overall prognosis of unilateral RB patients with intracranial segment of retrobulbar optic nerve invasion was poor. Chemotherapy and surgical treatment were necessary, but more attention should be paid to radiotherapy and intrathecal therapy for improving prognosis.

Prevalence, clinical features and prognosis of malignant solid tumors in infants: a 14-year study.

The onset of malignant solid tumors in infants is insidious and difficult to diagnose on time. The purpose of our study is to provide a theoretical basis for clinical diagnosis by retrospective analysis of the data in the past 14 years. Here, we retrospectively collected the clinical data of infants aged 0-12 months with malignant solid tumors in Beijing Tongren Hospital Affiliated to Capital Medical University from May 2005 to May 2019. The epidemiology, clinical characteristics, treatments and prognosis were statistically analyzed. A total of 496 infants (294 males and 202 females) with malignant solid tumors were analyzed. The main period of onset was 1-11 months. The most common tumor was retinoblastoma (RB, 51.8%), followed by hepatoblastoma (HB, 26.6%), neuroblastoma (NB, 10.5%), rhabdomyosarcoma (RMS, 3.4%), malignant renal tumors (3.2%), infantile fibrosarcoma (IFS, 1.6%), malignant teratoma (1.2%), Ewing's sarcoma (ES, 0.8%), medulloblastoma (MB, 0.4%) and inflammatory myofibroblastic tumor (IMT, 0.4%). The median follow-up time was 32 months (range 2-162 months). The 1-year, 3-year, and 5-year overall survival of all patients were 97.3%, 89.2%, and 81.1%, respectively, and event-free survival was 94.7%, 84.8%, and 75.8%, respectively. In conclusion, as a special group, malignant solid tumors in infants are complex, heterogeneous, and relatively rare. The prognosis of RB, HB, NB, RMS, malignant renal tumors, IFS, malignant teratoma, ES, MB, and IMT, were excellent duo to timely diagnosis and rational treatment.

High BMI and Insulin Resistance Are Risk Factors for Spontaneous Abortion in Patients With Polycystic Ovary Syndrome Undergoing Assisted Reproductive Treatment: A Systematic Review and Meta-Analysis.

Background: The risk of spontaneous abortion in patients with polycystic ovary syndrome (PCOS) undergoing assisted reproductive treatment (ART) is higher than that in patients without PCOS, however, no definitive risk factors have been confirmed to associate with the high spontaneous abortion rate in PCOS patients undergoing ART. This study was performed to assess the impact of relevant risk factors on spontaneous abortion in patients with PCOS. Clinical questions were formulated and organized according to the PICOS principle. Methods: A systematic review and meta-analysis were conducted on all published studies on PCOS and spontaneous abortion in Embase, PubMed, Web of Science and Cochrane Library. Related risk factors included body mass index (BMI), age, insulin resistance (IR), hyperandrogenism, and chromosome aberrations. All patients were diagnosed as PCOS using the Rotterdam criteria. The primary endpoint was miscarriage and live birth rate. Fixed-effect models were used to analyze homogeneous data, and subgroup and sensitivity analyses were performed on heterogeneous data. The source of heterogeneity was evaluated, and the random effect model was used to summarize the heterogeneity. Results: Among 1836 retrieved articles, 22 were eligible and included in the analysis with 11182 patients. High BMI (OR = 1.48, 95% CI [1.32, 1.67], MD = 1.35, 95% CI [0.58,2.12]) and insulin resistance (MD = 0.32, 95% CI [0.15, 0.49]) were associated with an increased risk of spontaneous abortion in PCOS patients undergoing ART. Older age (OR = 0.29, 95% CI [0.29, 0.44], MD = 2.01, 95% CI [0.04, 4.18]), embryonic chromosomal aberrations (OR = 0.75, 95%CI [0.31,1.77]), and hyperandrogenism (MD = 0.10, 95% CI [- 0.02, 0.22]) were not associated with the high spontaneous abortion rate in patients with PCOS. A subgroup analysis of BMI showed that there was no statistically significant difference in the effect between overweight and obesity on spontaneous abortion in PCOS patients undergoing ART (OR = 1.34, 95% [0.97, 1.85]). Conclusion: High BMI and insulin resistance are two risk factors for an increased risk of spontaneous abortion in PCOS patients undergoing ART, and losing weight and mitigating insulin resistance may decrease the spontaneous abortion rate in these patients undergoing ART.

Natural killer/T-cell lymphoma with intracranial infiltration and Epstein-Barr virus infection: A case report.

BACKGROUND: Because of atypical clinical symptoms, lymphoma is easily confused with infectious diseases. Extranodal nasal-type natural killer/T-cell lymphoma (NKTL) is more common, and there are few cases of eyelid site onset and intracranial infiltration, which increases the difficulty of diagnosis. This disease usually has a very poor prognosis and there are few reports of recovery. CASE SUMMARY: A 3-year-old boy was admitted to our hospital due to an initial misdiagnosis of "eyelid cellulitis" and failed antibiotic treatment. He was characterized by fever, right eyeball bulging, convulsions, and abnormal liver function. His blood Epstein-Barr virus (EBV) DNA was positive (8.798 × 104 copies/mL), and remained positive for about half a year. The cranial imaging examination suggested a space-occupying lesion in the right eyelid, with the right temporal lobe and meninges involved. The boy underwent ocular mass resection. The pathological diagnosis was NKTL. He was diagnosed as having NKTL with intracranial infiltration, combined with chronic active EBV infection (CAEBV). Then he underwent systemic chemotherapy and intrathecal injection. The boy suffered from abnormal blood coagulation, oral mucositis, diarrhea, liver damage, and severe bone marrow suppression but survived. Finally, the tumor was completely relieved and his blood EBV-DNA level turned negative. The current follow-up has been more than 2 years and his condition is stable. CONCLUSION: This case suggests that chemotherapy combined with intrathecal injection may have a good effect on intracranial infiltrating lymphoma and CAEBV, which deserves further study and discussion.

Prognostic factors in children with head and neck rhabdomyosarcoma: A 12-year retrospective study.

INTRODUCTION: To identify possible prognostic factors in children with head and neck rhabdomyosarcoma (RMS). METHODS: A total of 98 patients with head and neck RMS were enrolled in this retrospective study from February 2005 to September 2017. Prognostic factors were evaluated by univariate and multivariate analysis using Cox's proportional hazards model. Survival curves were calculated by Kaplan-Meier method. RESULTS: At the study closing date, there were 60 patients alive, 37 patients died, one patient was lost to follow-up, and 47 patients relapsed. The median disease-specific survival was 60.00 ± 25.36 months, and the overall survival (OS) rate was 61.9%. Complete remission was associated with a longer disease-specific survival (86.6%) compared with partial remission (6.7%). In addition, patients with age >3 years had better OS rate (69.0%) compared with age ≤3 years (42.3%). Univariate and multivariate analysis showed that chemotherapy efficacy and age were prognostic factors of disease-specific survival. CONCLUSIONS: Improvement in outcome was obtained with comprehensive treatment for head and neck RMS. Both chemotherapy efficacy and age of patients were prognostic factors for children with head and neck RMS, which provide some valuable information for further treatment.

Comparative analysis on the effect of Z-plasty versus conventional simple excision for the treatment of sacrococcygeal pilonidal sinus: A retrospective randomised clinical study.

Sacrococcygeal pilonidal sinus is one of common diseases in general department. However, it is characterised, for surgeons, by high post-surgical recurrence and high incidence of post-surgical wound complications. Due to that fact, this retrospective randomised clinical study was designed to evaluate the surgical procedure effect of Z-plasty (ZP), compared with convention simple excision (SE). A total of 67 patients from May 2015 to May 2019 in our department were studied into two groups randomly, the group of ZP and the group of SE. The patients' characteristics, surgical data, hospital length of stay (LOS), and post-surgery complications were recorded. Statistical approaches were proceed with P-value analysis. The results are as follows. No significant differences were found between these two groups of the ages, gender distribution, Body Mass Index (BMI), smoking history, diabetes mellitus, and blood hypertension. The estimated blood loss, specimen volume, distance to anus, and drain output on the first day of post-surgery between the two groups were not statistically significant, either. However, surgical time in the ZP group was longer than that in the SE group (P < .0001). LOS in the ZP group was obviously shorter than that in the SE group (P = .0051). Furthermore, the patients of the ZP group were tending to suffer from fewer post-surgical complications than the ones of the SE group. In a conclusion, we hold the point view that the surgical procedure of ZP can lead a better outcome than SE because it demonstrated shortened LOS and fewer post-surgical complications.

Treatment outcomes for hepatoblastoma children with pulmonary metastasis and extrapulmonary involvement: experience of 36 cases at a single institution.

BACKGROUND: Hepatoblastoma (HB) was the most common primary liver malignant tumor in children. In this study, we aimed to analyze the clinical characteristics and outcome in HB children with pulmonary metastasis and extrapulmonary involvement. METHODS: This retrospective study enrolled 36 HB children with pulmonary metastasis and extrapulmonary involvement from January 2010 to December 2017. Clinical characteristics, treatment and outcomes were collected and analyzed. Survival curves were calculated by Kaplan-Meier method. RESULTS: Thirty-six patients (10 females and 26 males) were recruited, with a mean onset age of 2.13 years (range, 0.33-7.83). Four (11.11%) patients presented with single metastatic pulmonary nodules, 32 (88.89%) patients presented with multiple metastatic nodules in both lungs. There were 10 (27.78%) patients with extrahepatic abdomen involvement, 13 (36.11%) patients with brain metastasis, and 16 (44.44%) patients with vascular metastasis. All patients underwent liver tumorectomy and chemotherapy. The median chemotherapy cycle was 17 (range, 3-39). In addition, 19 (54.29%) patients underwent lung metastasectomy. The patients were followed up to December 2018, with a median follow-up of 32.5 months. At the study closing date, 9 patients were alive, 24 patients had died, and 3 patients were censored. Alpha fetoprotein (AFP) level, PRETEXT stage and distant metastases had significant impact on survival time (all P<0.05). CONCLUSIONS: The common sites of extrapulmonary metastasis of HB were blood vessels, brain and extrahepatic abdominal organs. The overall prognosis of HB patients with lung metastasis and extrapulmonary involvement was poor, especially those with PRETEXT stage IV, high AFP level or distant metastases.