Objective:To analyze the clinical characteristics of children with head and neck rhabdomyosarcoma (RMS) and to summarize the mid-long term efficacy of Beijing Children′s Hospital Rhabdomyosarcoma 2006 (BCH-RMS-2006) regimen and China Children′s Cancer Group Rhabdomyosarcoma 2016 (CCCG-RMS-2016) regimen.Methods:A retrospective cohort study. Clinical data of 137 children with newly diagnosed head and neck RMS at Beijing Children′s Hospital, Capital Medical University from March 2013 to December 2021 were collected. Clinical characteristic of patients at disease onset and the therapeutic effects of patients treated with the BCH-RMS-2006 and CCCG-RMS-2016 regimens were compared. The treatments and outcomes of patients with recurrence were also summarized. Survival analysis was performed by Kaplan-Meier method, and Log-Rank test was used for comparison of survival rates between groups.Results:Among 137 patients, there were 80 males (58.4%) and 57 females (41.6%), the age of disease onset was 59 (34, 97) months. The primary site in the orbital, non-orbital non-parameningeal, and parameningeal area were 10 (7.3%), 47 (34.3%), and 80 (58.4%), respectively. Of all patients, 32 cases (23.4%) were treated with the BCH-RMS-2006 regimen and 105 (76.6%) cases were treated with the CCCG-RMS-2016 regimen. The follow-up time for the whole patients was 46 (20, 72) months, and the 5-year progression free survival (PFS) and overall survival (OS) rates for the whole children were (60.4±4.4)% and (69.3±4.0)%, respectively. The 5-year OS rate was higher in the CCCG-RMS-2016 group than in BCH-RMS-2006 group ((73.0±4.5)% vs. (56.6±4.4)%, χ2=4.57, P=0.029). For the parameningeal group, the 5-year OS rate was higher in the CCCG-RMS-2016 group (61 cases) than in BCH-RMS-2006 group (19 cases) ((57.3±7.6)% vs. (32.7±11.8)%, χ2=4.64, P=0.031). For the group with meningeal invasion risk factors, the 5-year OS rate was higher in the CCCG-RMS-2016 group (54 cases) than in BCH-RMS-2006 group (15 cases) ((57.7±7.7)% vs. (30.0±12.3)%, χ2=4.76, P=0.029). Among the 10 cases of orbital RMS, there was no recurrence. In the non-orbital non-parameningeal RMS group (47 cases), there were 13 (27.6%) recurrences, after re-treatment, 7 cases survived. In the parameningeal RMS group (80 cases), there were 40 (50.0%) recurrences, with only 7 cases surviving after re-treatment. Conclusions:The overall prognosis for patients with orbital and non-orbital non-parameningeal RMS is good. However, children with parameningeal RMS have a high recurrence rate, and the effectiveness of re-treatment after recurrence is poor. Compared with the BCH-RMS-2006 regimen, the CCCG-RMS-2016 regimen can improve the treatment efficacy of RMS in the meningeal region.

Objective:To summarize the clinical characteristics, diagnosis and treatment experience of salivary pleomorphic adenoma in children. Methods:Thirty patients with salivary pleomorphic adenomas treated in Beijing Childrens Hospital from January 2008 to December 2022 were retrospectively reviewed, including 11 boys and 19 girls, with the age ranging from 0.3 to 14.4 years（median age 10.4 years）. Initial presentation, medical history, imaging workups, surgical approaches, complications, rates of recurrence were evaluated. Results:Major salivary gland lesions were most common（n=24, 80%）; 53.3%（16 of 30） arising in the submandibular glands and 26.7%（8 of 30） in the parotid. Minor salivary gland lesions（n=6, 20%） were removed from the palate, tongue, face, trachea, nasopharynx, and upper mediastinumand. Preoperative imaging was reviewed in all patients and consisted of 26 ultrasound exams, 2 computerized tomography（CT） exams, and 15 magnetic resonance imaging（MRI） exams. Fine needle aspiration biopsy was performed in 12 patients. Surgical excision was performed in all patients. Postoperative complications included transient facial paresis（n=3）, Pneumonia and pleural effusion（n=1）. Average length of follow-up was 36.7 months; confirmed recurrence occurred in one patients. Conclusion:The symptoms of salivary gland pleomorphic adenoma in children are different according to the location of the tumor. The treatment is complete surgical resection, and a small amount of normal tissue around the tumor should be removed to reduce recurrence.
Humans ; Male ; Female ; Child ; Adenoma, Pleomorphic/diagnosis* ; Adolescent ; Retrospective Studies ; Salivary Gland Neoplasms/diagnosis* ; Child, Preschool ; Infant ; Neoplasm Recurrence, Local

Objective To observe the effect of different concentrations of glutamine(Gln)on the radiosensitivity of colorectal cancer HT-29 cells and explore the possible mechanism.Methods According to different Gln concentrations,HT-29 cells at logarithmical growth were divided into control group(2 mmol/L,as the basal medium concentration group)and experimental groups Ⅰ,Ⅱ and Ⅲ(4,6 and 8 mmol/L).After a 2-hour pre-treatment,all groups were exposed to 8 Gy irradiation of a Co-60 radiation source.CCK-8 assay and clonal formation assay were used respectively to explore the effects of different Gln concentrations on cell viability and cell radiosensitivity after irradiation.The level of reactive oxygen species(ROS)in each group was measured in 24 h after irradiation,and the apoptotic rate was detected with flow cytometry in 48 h after irradiation.The protein expression levels of Nrf2,HO-1,and cleaved-Caspase3 were determined by Western blotting.Results In 24 h after Gln intervention,the cell viability of experimental groups Ⅱ and Ⅲof non-irradiated HT-29 cells was significantly higher than that of the control group and of experimental group Ⅰ(P＜0.05).In 24 h after radiation,the cell viability of each experimental group was significantly higher than that of the control group(P＜0.05).In 14 d after radiation,there were more clone formation in each experimental group than the control group(P＜0.05).The ROS level was significantly lower in each experimental group than the control group in 24 h after radiation(P＜0.05).After 48 h of radiation,the apoptotic rate was notably lower in each experimental group than the control group(P＜0.05).The expression level of Nrf2 in the experimental group Ⅰ was higher than that of the control group(P＜0.05),those of Nrf2 and HO-1 in the experimental groups Ⅱ and Ⅲ were higher than those of the control group and experimental group Ⅰ(P＜0.05).While the expression of cleaved-Caspase3 in the experimental groups Ⅱ and Ⅲ was lower than the control group and experimental group Ⅰ(P＜0.05),and it in the experimental group Ⅲ was lower than that of experimental group Ⅱ(P＜0.05).Conclusion Gln can significantly reduce the radiosensitivity of HT-29 cells,which is associated with its reducing oxidative stress damage and reducing cell apoptosis.Our results suggest that Gln might be detrimental to radiation therapy in patients with colorectal cancer.

Objective:To analyze the treatment outcomes and prognoses of children with head and neck non-parameningeal rhabdomyosarcoma (HNnPM RMS).Methods:A retrospective analysis was performed on the clinical data of children with HNnPM RMS admitted to Beijing Children′s Hospital from September 2012 to September 2022. The clinical features, comprehensive treatment modes and prognoses of the patients were analyzed. The overall survival rate (OS) and event free survival rate (EFS) were calculated using the Kaplan-Meier method, and univariate analysis was performed using the Log-rank test.Results:A total of 70 children were included in this study, 38 males and 32 females, with a median age of 47 months (2-210 months). Pathological subtypes including the embryonal in 27 cases, the alveolar in 36 cases and the spindle cell and sclerosing in 7 cases. Thirty children (83.3%) with alveolar type were positive for FOXO1 gene fusion. All 70 children underwent chemotherapy, including 38 with neoadjuvant chemotherapy and 32 with adjuvant chemotherapy. Sixty of 70 children underwent surgery, of whom, 10 underwent two or more surgeries. There were 63 children underwent radiotherapy, including 54 with intensity-modulated radiation therapy, 4 with particle implantation and 5 with proton therapy. The median follow-up was 45 (5-113) months, the 5-year OS was 73.2%, and the 5-year EFS was 57.7%. Univariate analysis showed lymph node metastasis ( χ2=5.022, P=0.025), distant metastasis ( χ2=8.258, P=0.004), and high Intergroup Rhabdomyosarcoma Study (IRS) group ( χ2=9.859, P=0.029) as risk factors for poor prognosis. Before June 2016, the 5-year OS based on BCH-RMS-2006 scheme was 63.6%, and after 2016, the 5-year OS based on CCCG-RMS-2016 scheme was 79.6%. Conclusion:Multidisciplinary combined standardized treatment can offer good treatment outcome and prognosis for children with HNnPM RMS. Local control is a key to the efficacy of comprehensive treatment.

Objective:To develop the children′s postoperative health-related quality of life scale for thyroid cancer and to test its reliability and validity.Methods:The first draft of the scale was developed through literature search, focus group meetings, and a pre-survey, and 116 children (76 for testing and 40 for external validation) with thyroid cancer attending Beijing Children′s Hospital of Capital Medical University were selected to answer the scale, to screen and categorize the questions and to form the final scale with multiple dimensions.Results:The children′s postoperative health-related quality of life scale for thyroid cancer contained 5 dimensions and 29 questions. Exploratory factor analysis showed that the cumulative variance explained by the 5 factors was 64.343%. Confirmatory factor analysis showed correlations between the questions and dimensions of this scale, fair convergent validity for the scale, and good discriminant validity. The validity of the validity scale showed that there was a existing correlation between the questions and the validity scale of this scale. The item-dimension correlation coefficients showed that the questions in each dimension were well differentiated. The total Cronbach′s α coefficient of the scale was 0.930, the folded half reliability was 0.843, and retest reliabilities at 2 weeks, 1 month, and 3 months after the initial test were respectively 0.936, 0.922, and 0.910.Conclusion:The developed children′s postoperative health-related quality of life scale for thyroid cancer has good reliability and validity and can be used to assess the health-related quality of life of children after thyroid cancer surgery.

Objective:To evaluate the effectiveness and safety of ultrasound-guided percutaneous polidocanol sclerotherapy of the thyroglossal duct cysts in children.Methods:A retrospective analysis of 26 children who were treated with ultrasound-guided percutaneous puncture sclerosis for thyroglossal duct cysts in Beijing Children′s Hospital, Capital Medical University from October 2019 to April 2022, the size of the cyst before treatment was recorded, and the cyst volume in accordance with V(ml) =1/6 πabc cyst volume was calculated. The follow-up time was the 1st, 3rd, 6th, and 12th months after sclerotherapy. And the cyst volume and volume reduction ratio at different time points was calculated. According to changes in the cystic volume, the treatment was considered effective if the cyst volume reduction rate was≥50%. Cosmetic grading scores (CGS) were performed pre-treatment and at the last follow-up after sclerotherapy using the WHO grading system. Complications were recorded during the follow-up period.Results:The cyst volume before treatment was 2.67 (3.78)ml, and the cyst volume at the 1st, 3rd, 6th and 12th month after treatment were 0.66(1.83), 0.45(0.87), 0.40(0.70), 0.38 (0.63)ml, respectively, there were significant differences between pre-treatment and each time point after-treatment (all P<0.001); the volume reduction rate at the last follow-up was (81.48±14.57)%. The reduction rate of cyst volume was more than 50% at the last follow-up in 22 children, the treatment efficiency was 84.62% (22/26). The cosmetic grading scores at the last follow-up after sclerotherapy was 1.74(1.50), and it decreased significantly compared with the pre-treatment score 3.85(0)( P<0.001). There was no skin pigmentation, pain in the injection area, local urticaria and blistering after sclerotherapy, no adverse reactions such as cyst bleeding and drunkenness-like reaction, and no serious complications occurred. There were 2 cases of cysts that presented infection with fever, and no serious complications occurred. Conclusions:Ultrasound-guided percutaneous sclerotherapy is a safe and effective minimally invasive treatment for thyroglossal duct cysts in children.

Objective:To analyze the clinical characteristics and prognosis of infants with rhabdomyosarcoma (RMS), thus enhancing the understanding of this disease.Methods:Clinical data of all RMS patients younger than 12 months treated in the Hematology Oncology Center, Beijing Children′s Hospital, Capital Medical University from May 2006 to June 2019 were retrospectively analyzed, including the age, gender, histological type, tumor primary site, tumor size, and the prognosis.Patients were followed up until December 31, 2019.The 3-year event free survival (EFS) rate of children was performed by plotting the Kaplan-Meier survival curves.Results:A total of 15 RMS children younger than 12 months were enrolled, accounting for 4.9% of all RMS cases in the same period, including 6 males and 9 females.The median age at diagnosis was 7.0 months (3.0-11.5 months). Classified by the primary site, 40.0% (6 cases) located in the head and neck, followed by 26.7% (4 cases) located in the limbs, 26.7% (4 cases) located in other parts, and 6.7% (1 case) located in the urogenital system.Embryonal RMS, alveolar RMS and spindle cell RMS accounted for 46.6% (7 cases), 26.7% (4 cases), and 26.7% (4 cases), respectively.Ten cases (66.7%) were stage Intergroup Rhabdomyosarcoma Study (IRS)-Ⅲ and 1 case (6.7%) was in stage Ⅳ.There were 10 cases (66.6%) in the middle-risk group, 4 cases (26.7%) in the low-risk group, and 1 case (6.7%) in the high-risk group.Two cases had a larger than 5 cm primary tumor; lymph node involvement was confirmed in 3 cases, and pulmonary metastasis occurred in 1 case at the time of diagnosis.All children were treated with chemotherapy, and 13 cases received postoperative chemotherapy and 1 case received preoperative chemotherapy.One case were not operated.Only 3 children underwent radiotherapy, including 1 case underwent particle implantation and 2 cases received external radiotherapy.Among the 15 children with RMS, 5 cases had relapse and disease progression with the 3-year EFS rate of (59.1±14.5)%, and 2 died with the 3-year overall survival rate of (80.8±12.6)%.Conclusions:The median age of diagnosis of RMS in single-center infants is 7 months.Head and neck are the most common primary sites of RMS.Nearly 50% of the children have the primary site of RMS with poor prognosis.More than a quarter of the pathological subtypes are the spindle cell type.Local treatment significantly influences the local progression or recurrence of RMS.

Objective:To determine the characteristics of cervical neuroblastoma and the effect of resection extent on survival and outcomes.Methods:We performed a retrospective review of 32 children with cervical neuroblastoma treated at Beijing Children′s Hospital between April 2013 and August 2020. Data were collected from the medical record. The individualized therapy was designed based on staging and risk group. Based on the extent of resection, patients were divided into incomplete and complete resection groups. Event free and overall survival rates were compared between two groups using the Kaplan-Meier method.Results:The ages of patients ranged from 1 month to 81 months, with a median age of 11 months, including 7 males and 15 females. Twenty-nine patients (90.6%) presented with cervical painless mass. The average diameter of the primary tumors was (5.12±1.43) cm. Tumors were located in the parapharyngeal space in 25 cases (78.1%) and in the root of the neck in 7 cases (21.9%). None had MYCN amplification. According to International Neuroblastoma Staging System (INSS), 15 patients (46.9%) were identified as stage 1, 11 patients (34.3%) as stage 2B, 3 patients (9.4%) as stage 3 and 3 patients (9.4%) as stage 4. There were 12 patients (37.5%) at low risk, 17 patients (53.1%) at intermediate risk and 3 patients at high risk according to Children′s Oncology Group (COG) risk classification system. All patients underwent tumor resection. Postoperatively Horner′s syndrome occurred in 13 patients (40.6%), pneumonia in 9 patients (28.1%), pharyngeal dysfunction in 8 patients (25.0%) and transient hoarseness in 4 patients (12.5%). At a median follow-up of 36.5 months, the overall survival rate was 96.4%, with no significant difference between incomplete and complete resection groups (100.0% vs. 96.3%, χ 2=0.19, P=0.667); the event free survival rate was 78.1%, with a significant difference between the two groups (40.0% vs. 85.2%, χ2=6.71, P=0.010). Conclusions:Primary cervical neuroblastoma has a young onset age, mostly in low and medium risk groups, and represents favorable lesions with good outcomes after multidisciplinary therapy. Less aggressive surgery with preservation of important structures is recommended. Complete resection should not be attempted if it would compromise vital structures.

Objective:To evaluate the efficacy and influencing factors of surgery combined with neoadjuvant chemoradiotherapy in the treatment of children with non-orbital head and neck rhabdomyosarcoma (HNRMS).Methods:Information from 45 children diagnosed as non-orbital HNRMS and subjected to surgery combined with neoadjuvant chemoradiotherapy in Beijing Children′s Hospital affiliated to Capital Medical University from August 2017 to July 2021 was analyzed. The patients included 25 males and 20 females, aged from 1 to 17 years old. The primary tumor site, pathological subtype, clinical stage, risk group, therapeutic regimen, resection range and outcome of all cases were also collected. The survival curves were made using the Kaplan-Meier method and the potential prognostic factors were investigated by Cox regression analysis.Results:Fifteen (33.3%) of 45 children achieved negative surgical margin under complete tumor resection. The postoperative pathological results showed that there were 20 cases of embryonic subtype, 19 cases of alveolar subtype and 6 cases of spindle sclerosis subtype. The postoperative follow-up time ranged from 4 to 71 months, with a median of 26 months. During the follow-up period, 13 children died, among whom brain metastasis was the most common cause of death, accounting for 7/13. The 3-year overall survival rate was 67.6%. Multivariate analysis showed that non-embryonic subtype ( HR=6.26, 95% CI: 1.52-25.87, P=0.011) and failure to reach R0 resection ( HR=9.37, 95% CI: 1.18-74.34, P=0.034) were independent risk factors affecting overall survival rate. Conclusion:Surgery combined with neoadjuvant chemoradiotherapy can offer a good efficacy for children with non-orbital HNRMS. Non-embryonic subtype and resection without negative operative microscopic margins are independent risk factors for poor prognosis, and brain metastasis is the main cause of death in these children.

Objective:To evaluate the cancer detection rate in patients with multiparametric magnetic resonance imaging (mpMRI) PI-RADS 1-2 prior to initial biopsy, and analyze the risk factors of prostate cancer.Methods:A total of 196 patients undergoing initial prostate biopsy between July 2011 and June 2018 were retrospectively analyzed. According to ESUR PI-RADS system, the patients’ PI-RADS score was 1 and 2, with the mean age of 66.6±9.0 years, and the median PSA 7.44 ng/ml. Twenty-eight patients were enrolled with PSA<4 ng/ml but with abnormal directeral rectun examination. The rest 168 patients were enrolled with elevated PSA. According to the Epstein prostate risk classification criteria, clinically insignificant prostate cancer was defined as: PSA density ≤0.15 ng/ml 2, Gleason score≤6, less than 3 positive needles, <50% puncture length. If any of the above is not met, the diagnosis should be clinically significant prostate cancer(CsPCa). T test or Mann-Whitney U test were used for comparison between groups. Risk factors for diagnosis of prostate cancer and CsPCa were analyzed by chi square test(or Fisher’s exact probability method) and multivariate logistic regression analysis. Results:There were 42(21.4%) patients diagnosed with prostate cancer, 30(15.3%)patients were CsPCa. The negative predictive value of mpMRI was 78.6%(154/196)for prostate cancer overall, and 84.7%(166/196)for CsPCa. Patients with higher age and PSA density were associated with higher possibility of prostate cancer. Higher age, PSA level, PSA density, and lower PSA ratio were associated with higher possibility of CsPCa. Multivariate logistic regression analysis showed that PSA density>0.15 ng/ml 2( OR=2.94, 95% CI 1.45-5.95) was independent risk factor of prostate cancer.Ages over 70 years( OR=2.49, 95% CI 1.22-5.07), PSA ratio<0.2( OR=3.70, 95% CI 1.25-11.23), PSA density>0.15 ng/ml 2( OR=5.77, 95% CI 1.96-16.96) were independent risk factors of CsPCa ( P<0.05). Conclusions:The detection rate of prostate cancer was 21.4% in patients with elevated PSA or abnormal digital prostate examination but with PI-RADS score of 1-2. Higher age and PSA density were associated with higher risk of prostate cancer. The detection rate of CsPCa was 15.3%. Ages over 70 years, PSA ratio<0.2, PSA density>0.15 ng/ml 2 were independent risk factors of CsPCa.