Ameloblastic fibrosarcoma of the maxilla arising in an old woman, a rare case report and literature review.

BACKGROUND: Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults and typically affecting the mandibular region. We report an exceptionally rare and highly atypical case of AFS in an elderly female patient originating from the maxillary bone. CASE PRESENTATION: A 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up. CONCLUSION: Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.

Multidisciplinary management of pediatric malignant rhabdoid tumor based on 5 years of experience at a tertiary care center.

OBJECTIVE: Malignant rhabdoid tumor (MRT) is a highly aggressive tumor that occurs mostly in young children with extremely poor prognosis. Standardized and effective treatment strategies for MRT have yet to be established because of its rarity. Here, we report our single-institutional experience involving MRT treatment. METHODS: Patients with newly diagnosed MRT between March 2016 and October 2021 were included. The clinical characteristic, treatment-related toxicities, clinical outcomes, and prognostic factor were retrospectively analyzed. RESULTS: A total of 18 patients with MRT were enrolled during the 5 years. The median age was 42.8 months (range 10 to 82 years). Among the 18 patients, 9 patients died after a median of follow-up 26 months (range 3 to 42 months). The 1-year event-free survival (EFS) and 3-year overall survival (OS) rates of the entire cohort were 63% (95% CI 46% to 74%) and 67% (95% CI 49% to 82%), respectively. Univariate analysis of patients who underwent gross or total resection followed by adjuvant chemotherapy and radiotherapy demonstrated an improvement in 1-year EFS. However, only gross resection and total resection predicted a better 3-year OS. CONCLUSIONS: Surgical excision is still the mainstream treatment for MRT. Postoperative adjuvant treatments including chemotherapy and radiotherapy contribute to improved disease control rate. Our single-institute experience may provide insights into the multimodal treatment of MRT.

Clinical characteristics, outcomes, and risk factors for mortality in patients with cancer and COVID-19 in Hubei, China: a multicentre, retrospective, cohort study.

BACKGROUND: Patients with cancer are a high-risk population in the COVID-19 pandemic. We aimed to describe clinical characteristics and outcomes of patients with cancer and COVID-19, and examined risk factors for mortality in this population. METHODS: We did a retrospective, multicentre, cohort study of 205 patients with laboratory-confirmed severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and with a pathological diagnosis of a malignant tumour in nine hospitals within Hubei, China, from Jan 13 to March 18, 2020. All patients were either discharged from hospitals or had died by April 20, 2020. Clinical characteristics, laboratory data, and cancer histories were compared between survivors and non-survivors by use of χ2 test. Risk factors for mortality were identified by univariable and multivariable logistic regression models. FINDINGS: Between Jan 13 and Mar 18, 2020, 205 patients with cancer and laboratory-confirmed SARS-CoV-2 infection were enrolled (median age 63 years [IQR 56-70; range 14-96]; 109 [53%] women). 183 (89%) had solid tumours and 22 (11%) had haematological malignancies. The median duration of follow-up was 68 days (IQR 59-78). The most common solid tumour types were breast (40 [20%] patients), colorectal (28 [14%]), and lung cancer (24 [12%]). 54 (30%) of 182 patients received antitumour therapies within 4 weeks before symptom onset. 30 (15%) of 205 patients were transferred to an intensive care unit and 40 (20%) died during hospital admission. Patients with haematological malignancies had poorer prognoses than did those with solid tumours: nine (41%) of 22 patients with haematological malignancies died versus 31 (17%) of 183 patients with solid tumours (hazard ratio for death 3·28 [95% CI 1·56-6·91]; log rank p=0·0009). Multivariable regression analysis showed that receiving chemotherapy within 4 weeks before symptom onset (odds ratio [OR] 3·51 [95% CI 1·16-10·59]; p=0·026) and male sex (OR 3·86 [95% CI 1·57-9·50]; p=0·0033) were risk factors for death during admission to hospital. INTERPRETATION: Patients with cancer and COVID-19 who were admitted to hospital had a high case-fatality rate. Unfavourable prognostic factors, including receiving chemotherapy within 4 weeks before symptom onset and male sex, might help clinicians to identify patients at high risk of fatal outcomes. FUNDING: National Natural Science Foundation of China.

Inhibitory effect of endostar combined with radiotherapy on gastric cancer animal models.

BACKGROUND: Inhibitory effect of endostar combined with radiotherapy on gastric cancer (GC) animal models and its effect on transforming growth factor-ß1 (TGF-ß1) and inter-leukin-10 (IL-10) were evaluated. METHODS: Forty mice of GC model xenograft tumors were prepared and randomly divided into blank control group, endostar group, radiotherapy group, and endostar combined with radiotherapy group (combination group). From the 14th day, a vernier caliper was used for measuring the long and short diameters of the xenograft tumors. The formula V = ab2/2 was used for calculating the tumor volume and to obtain its average value. Tumor growth curves were plotted to calculate the tumor inhibition rate. The growth of xenograft tumors and the behavioral changes of mice were observed. Enzyme-linked immunosorbent assay (ELISA) was used for detecting the expression levels of IL-10 and TGF-ß1. RESULTS: The tumor growth in the combination group was significantly inhibited, and the tumor volume was the smallest compared with the other groups (p < 0.05). Compared to the blank control group, the tumor inhibition rate was 11.8% in endostar group, 33.0% in radiotherapy group, and 52.1% in combination group (p < 0.01). Endostar combined with radiotherapy had an interaction in decreasing the expression levels of TGF-ß1 and IL-10 (F = 4.35 and 5.12, p < 0.05). Leucocyte count was significantly higher in control and combination groups than that in endostar and radiotherapy groups. The body weight of mice in endostar and radiotherapy groups decreased after treatment (p < 0.05). The body weight of mice after treatment in control and combination groups increased, with a statistically significant difference compared to that before treatment (p < 0.05). There was a statistically significant difference among all groups after treatment (F = 198.1, p < 0.01). CONCLUSIONS: Endostar combined with radiotherapy can inhibit tumor growth and downregulate the expression levels of TGF-ß1 and IL-10 through synergistic action.

Effect of genistein on apoptosis of lung adenocarcinoma A549 cells and expression of apoptosis factors.

PURPOSE: To investigate the effect of genistein (GEN) on the apoptosis of lung adenocarcinoma cells and the expression of Bcl-2 and Bax, so as to screen effective antitumor drugs for the clinical treatment of lung adenocarcinoma. METHODS: Lung adenocarcinoma A549 cells were cultured in vitro and treated with different concentrations of GEN. The untreated cells were set as control group. MTT assay and Annexin V/PI double staining were used to analyze cell proliferation and apoptosis after GEN treatment. RT-qPCR and Western blot were used to detect the expression of Bcl-2 and Bax at mRNA and protein levels, respectively. RESULTS: Cell p roliferation a ssay s howed t hat GEN c ould inhibit the proliferation of lung adenocarcinoma cell line A549 in vitro in a dose-dependent manner. Cell apoptosis assay showed that, compared with the control group, the apoptotic rate of A549 cells was significantly increased after GEN treatment. RT-qPCR and Western blot showed that the expression levels of anti-apoptotic factor Bcl-2 in A549 cells were significantly decreased at both mRNA and protein levels at 48 hrs after treatment with GEN, but the levels of pro-apoptotic Bax were significantly increased at both mRNA and protein levels. CONCLUSION: GEN can inhibit the proliferation of A549 lung adenocarcinoma cells in vitro and induce their apoptosis. The antitumor activity of GEN is achieved by downregulating Bcl-2 and upregulating Bax. Therefore, GEN can be applied to the clinical treatment of lung adenocarcinoma.

Effects of javanica oil emulsion injection combined with radiotherapy versus radiotherapy alone on the efficacy and safety in patients with esophageal cancer: a pooled analysis of 1269 cases.

PURPOSE: To assess the effectiveness and safety of javanica oil emulsion injection (JOEI) when combined with radiotherapy (RT) in patients with esophageal cancer. METHODS: Electronic databases including EMBASE, PUBMED, the COCHRANE Library, China Academic Journals Full-text Database, Chinese Biomedical Literature Database, and Chinese Scientific Journals Database were searched. Two reviewers performed the search, identified and extracted eligible studies. Items including response rate, survival and safety were extracted and analyzed using Review Manager 5.3. RESULTS: A total of 16 clinical studies with 1269 esophageal cancer patients were included. The results showed that adding JOEI to RT could improve the complete response (CR rate) (Odds Ratio/OR 1.63; 95% confidence interval (CI), 1.27 to 2.10; p=0.0001), partial response (PR) (OR 1.25; 95% CI, 0.97 to 1.60; p=0.09), Relative Risk/RR (OR 1.42; 95% CI, 1.19 to 1.70; p<0.0001), quality of life (OR 3.01; 95% CI, 1.72 to 5.25; p=0.0001), and reduce the incidence of adverse events including nausea and vomiting (OR 0.81; 95% CI, 0.45 to 1.44; p=0.46) and radiation esophagitis (OR 0.47; 95% CI, 0.33 to 0.68; p<0.0001). The 1-, 2-, and 3-year survival rates in the JOEI group were significantly higher than those in the RT alone group (p<0.001). CONCLUSIONS: JOEI in combination with RT could benefit esophageal cancer patients with improved rate of response and quality of life, prolonged survival, and reduced incidence of adverse events. However, these results should be viewed with caution due to the limited quality of the included studies.

Therapeutic effect of gefitinib in advanced non-small cell lung cancer and its effect on the EGFR level in peripheral blood.

PURPOSE: This study was designed to investigate the therapeutic effect of gefitinib in advanced non-small cell lung cancer (NSCLC) and its effect on the level of epidermal growth factor receptor (EGFR) in peripheral blood. METHODS: A total of 58 patients with NSCLC were treated with gefinitib (iressa) (250 mg per day). EGFR levels in the peripheral blood were measured with ELISA assay before and after treatment. Statistical analyses of patient quality of life, survival and other clinical data were conducted including logistic regression analysis, x2 test and t-test. Quality of life assessment was quantified based on the Chinese version of the QLQ-C30 and QLQ-LC13 questionnaires of the European Organization for Research and Treatment of Cancer. RESULTS: The overall response rate to iressa was 38% (22 patients), and the disease control rate (response+stable) was 74% (43 patients). The mean scores of assessment of physiological functions and comprehensive quality of life in QLQ-C30 questionnaire were significantly increased with an improvement rate of 91-100%. Similarly, the mean scores of assessment of disease symptoms in QLQ-LC12 questionnaire were significantly reduced with an overall improvement rate of 73-100). Adverse drug effects were mainly grade I and II skin rashes and diarrhea. The EGFR levels in peripheral blood were significantly decreased after treatment (p<0.05). CONCLUSION: Based on our results, gefitinib showed meaningful effects in treating advanced NSCLC, significantly improving clinical symptoms and ameliorating the patient quality of life.

Analysis of short and long term therapeutic effects of radiofrequency hyperthermia combined with conformal radiotherapy in hepatocellular carcinoma.

PURPOSE: To investigate the immediate and long term therapeutic effects of radiofrequency hyperthermia combined with conformal radiotherapy in patients with hepatocellular carcinoma (HCC). METHODS: Data was collected from 80 patients with HCC. All of the patients had confirmed primary HCC according to their clinical symptoms, imaging examinations, biochemical examinations and pathology. Patients were randomly divided into control group and experimental group, with 40 cases each. Patients in the control group were treated with conformal radiotherapy while patients in the experimental group were treated with conformal radiotherapy combined radiofrequency hyperthermia. Finally, the short and long term outcomes were compared between the groups. RESULTS: The levels of bilirubin, ALT and prothrombin time (PT) in both groups reduced after treatment and the reduction was more pronounced in the experimental group. In both groups of patients albumin was elevated and in the experimental group this elevation was significantly more pronounced (p<0.05). The total efficiency for patients in the experimental group was significantly higher than that in the control group (p<0.05). Follow-up results showed that 6-month and 1-year recurrence and mortality rates were significantly lower in the experimental group compared with the control group (p<0.05). CONCLUSION: Radiofrequency hyperthermia combined conformal radiotherapy is remarkably effective in treating patients with HCC, which could effectively reduce the damages of radiotherapy to the liver, enhance the patient' s tolerability and improve the patients' short and long term survival.

Diffuse infantile hepatic hemangioma successfully treated with propranolol orally: a case report and literature review.

Background: Infantile hepatic hemangioma (IHH) is a common vascular, fast-growing hepatic tumor that is usually accompanied by multiple cutaneous hemangiomas. Diffuse IHH (DIHH) is a rare type of IHH that exhibits many tumors with nearly complete hepatic parenchymal replacement. At present, there is no specific standardized treatment plan for DIHH. Herein, we present the case of a 2-month-old girl with DIHH and without cutaneous hemangioma who achieved complete remission after undergoing propranolol monotherapy. Case presentation: The infant with low birth weight was presented to the pediatric department with a 2-month history of persistent vomiting and feeding difficulty. Ultrasonography and abdominal magnetic resonance imaging revealed hepatomegaly and diffused intrahepatic lesions. A computed tomography-guided percutaneous liver biopsy was performed, and the pathological examination suggested the diagnosis was DIHH. The patient exhibited remarkably response to an increasing dose of oral propranolol, from 0.5 mg/kg to 2 mg/kg every day. The intrahepatic lesions were almost completely regressed after one year of treatment and no distinct adverse reaction was observed. Conclusion: DIHH can induce life-threatening complications that require prompt interventions. Propranolol monotherapy can be an effective and safe first-line treatment strategy for DIHH.

GPNMB Expression Associates with Inferior Prognosis in Patients with Small Cell Lung Cancer.

Purpose: Small cell lung cancer (SCLC) is widely recognized for its propensity for early and frequent metastases, which contribute to its status as a refractory malignancy. While the high expression of GPNMB in SCLC is well-documented, the precise correlation between GPNMB expression and the prognosis of SCLC remains undetermined. Methods: HTG Edge-seq was used to screen the differential gene expression between primary SCLC lesions and paired metastatic lymph nodes (LN). The plasma concentration of GPNMB was measured using enzyme-linked immunosorbent assay (ELISA). The relationship between GPNMB concentration and clinical characteristics, as well as overall survival (OS) was assessed. One-to-one propensity score matching (PSM) was performed to reduce bias from confounding factors between groups. The invasive, migratory, proliferative, and apoptotic abilities of SCLC cells were evaluated using migration and matrigel invasion assays, CCK8 assay and flow cytometry respectively. Results: GPNMB exhibited a significant up-regulation in LN compared to primary SCLC lesions as determined by HTG Edge-seq. Furthermore, patients with extensive disease demonstrated a significantly elevated plasma GPNMB concentration compared to those with local disease (P = 0.043). Additionally, patients with a high baseline plasma GPNMB level exhibited a shorter OS (10.32 vs. 16.10 months, P = 0.0299). Following PSM analysis, the statistical significance of the difference between the two groups persisted (9.43 vs. 15.27 months, P = 0.0146). Notably, both univariate and multivariate analyses confirmed that higher expression of GPNMB served as an independent biomarker for OS before PSM (P = 0.033, HR = 2.304) and after PSM (P = 0.003, HR = 6.190). Additionally, our study revealed that the inhibition of GPNMB expression through the use of siRNA effectively diminished the metastatic and proliferative capabilities of SCLC. Furthermore, this inhibition resulted in an enhanced ability to induce apoptosis. Conclusions: In light of our findings, it can be inferred that the expression of GPNMB is linked to metastasis and an unfavorable prognosis, thus suggesting its potential as a novel therapeutic target in the treatment of SCLC.

Case Report: A case of synchronous right upper lobe adenocarcinoma and left lower lobe squamous cell carcinoma treated with immune checkpoint inhibitor plus chemotherapy.

Globally, lung cancer is the leading cause of cancer-related mortality. Multiple primary lung cancers (MPLC) account for a very small portion of all primary lung cancer cases. Importantly, a quick and precise differentiation between MPLC and intrapulmonary metastases is directly related to patient prognoses as treatment strategies vary according to pathological type. Synchronous MPLC are most commonly seen in the same lung. Here, we report a rare case of a patient with synchronous MPLC of both lungs. A 67-year-old man, with a 1-month cough and expectoration history, was admitted in our hospital. Computed tomography (CT) chest scan revealed a lower lobe nodule in the left lung and an upper lobe nodule in the right lung. He underwent successive fiberoptic bronchoscopy and CT-guided percutaneous pulmonary aspiration biopsy of both lungs. The pathological diagnosis was squamous cell carcinoma of the left lung and adenocarcinoma of the right lung.

Synchronous multiple primary cancers involving rectal cancer and pelvic classical hodgkin lymphoma: the first case report.

Multiple primary cancers (MPC) are characterized by the presence of synchronous and metachronous occurrence of two or more distinct histological tumor types. In this study, an exceptional clinical case was presented, demonstrating the coexistence of rectal adenocarcinoma and pelvic classical Hodgkin lymphoma (cHL). A 65-year-old male patient with a 2-year history of persistent mucous bloody stools was admitted to our hospital. Colonoscopy and subsequent biopsy confirmed the diagnosis of rectal adenocarcinoma. The patient underwent laparoscopic abdominoperineal resection of the rectum and regional lymph node dissection. Postoperative histopathological analysis not only substantiated the presence of rectal adenocarcinoma, but also unexpectedly identified pelvic lymph nodes harboring the features of cHL.

Case Report: Tumor-to-tumor metastasis with prostate cancer metastatic to lung cancer: the first reported case.

Tumor-to-tumor metastasis (TTM) occurs rarely in tumor progression, but this event has significant clinical implications. Although the impact of TTM on patient prognosis and survival has been increasingly recognized, understanding of TTM biology and treatment is limited. Prostate cancer is among the most common malignancies threatening male health. Prostate cancer can potentially metastasize to primary lung Cancer; however, this is an exceedingly rare event. We here report for the first time a case of TTM from a prostate cancer to a coexisting primary lung cancer.

BR vs. R­miniCHOP in unfit patients with B­cell non­Hodgkin lymphoma: A randomized, two­center, cohort study.

The aim of the present study was to compare the efficacy and safety between the bendamustine plus rituximab (BR) regimen and rituximab combined with low-dose doxorubicin, cyclophosphamide, vincristine and prednisone (R-miniCHOP) in the treatment of 'unfit' patients with diffuse large B-cell lymphoma (DLBCL) and follicular lymphoma grade 3B (FL3B). Patients, >70 years of age with DLBCL or FL3B, defined as unfit according to Comprehensive Geriatric Assessment, were included in the present study. All patients received 4-6 cycles of a BR or R-miniCHOP regimen at a three-week interval. The objective remission rate (ORR) and adverse reactions were evaluated between the two groups. A total of 35 patients, recruited between January 2020 and December 2021, were included in this prospective study. The median age was 74 years (range, 70-82 years). The ORR in the BR group was similar to that in the R-miniCHOP group (73.3 vs. 75.0%; P=0.606). However, the BR group exhibited a lower incidence of leukopenia than the R-miniCHOP group (20.0 vs. 60.0%; P=0.037). The univariate analysis revealed that the ORR was influenced by the serum ß2 microglobulin level. The BR regimen showed equivalent efficacy but more improved safety compared with R-miniCHOP in unfit patients with DLBCL and FL3B. The BR regimen may be considered as an alternative treatment in these subgroups of patients.

Clinical Management of Embryonal Tumor with Multilayered Rosettes: The CCMC Experience.

OBJECTIVE: Embryonal tumors with multilayered rosettes (ETMRs) are highly aggressive pediatric brain tumors with poor prognosis. No standard treatment strategy for them exists because of their rarity. This study aimed to share experiences on the clinical diagnosis and treatment of ETMRs at China Children's Medical Center (CCMC). METHODS: Patients who received a diagnosis of an ETMR between January 2017 and June 2020 were included. Clinical characteristics, such as age of onset, tumor size, stage, tumor site, treatment strategy, and clinical outcome, were retrospectively analyzed. RESULTS: There were four boys and one girl within 4 years who received a diagnosis during this 4-year timeframe, and were thus included. The average age of morbidity was 29 months (range 16-66 months). The common clinical presentation was headaches and nausea caused by intracranial hypertension. All four patients were chromosome 19 microRNA cluster (C19MC) amplification positive. Two patients achieved complete remission, and one patient attained partial remission after multimodal treatment. Of the two deaths, one died from the rapid progression of the disease and another from tumor-related complications. CONCLUSION: ETMRs are extremely rare brain tumors with a high, early mortality in children. Surgery is the mainstream treatment for ETMRs. Some patients may also benefit from postoperative adjuvant chemotherapy and radiotherapy.

Treatment of Pediatric Inflammatory Myofibroblastic Tumor: The Experience from China Children's Medical Center.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor with intermediate malignancy that tends to affect children primarily. To date, no standardized therapies exist for the treatment of IMT. This study aimed to share experience from China Children's Medical Center for the explorative treatment of IMT. METHODS: Patients with newly diagnosed IMT between January 2013 and December 2018 were included. Patients were grouped according to surgical margins and Intergroup Rhabdomyosarcoma Study Group (IRSG) staging. The clinical characteristic, therapeutic schedules, treatment response and clinical outcome were described. RESULTS: Six patients were enrolled in this study, including two boys and four girls, with a median age of 57 months (range 10-148 months). Among them, five patients were anaplastic lymphoma kinase positive. Four patients achieved complete remission and two patients attained partial remission after treatment with this protocol. All patients were alive after a median follow-up of 4 years (range 3-7 years). The most common treatment-related adverse reaction was myelosuppression. CONCLUSION: In this study, we demonstrated that IMT has a good prognosis and the treatment selected according to risk stratification was effective and feasible.

Efficacy and Safety for the Use of Half-Dosed Pegylated Granulocyte Colony-Stimulating Factors in Preventing Febrile Neutropenia During Chemotherapy in Patients With Malignant Tumors: A Multicenter, Open-Labeled, Single-Arm Phase 2 Trial.

Background: Prophylactic granulocyte-colony stimulating factor (G-CSF) has been shown to effectively prevent febrile neutropenia (FN) and grade 3/4 neutropenia during myelosuppressive treatment. The present study reports the clinical efficacy and safety of the prophylactic use of G-CSF with a half dose for cancer patients with an intermediate risk of FN combined with ≥1 patient-specific risk during multiple chemotherapy. Methods: This multicenter, one-arm, and open-label clinical study involved 151 patients [median age, 54 years old (range, 46.0-62.5); 38.4% female] with malignant tumors, including >20 different cancers. These patients underwent a total of 604 cycles of chemotherapy and received a half dose of PEG-rhG-CSF administration prior to each cycle. Results: The incidence rate of FN was 3.3% for this cohort during chemotherapy. Chemotherapy delay occurred in 6 (4.0%) patients for 12 (2.0%) cycles. Early termination of cancer treatment occurred in 14 (9.3%) patients. In this cohort, 23 (15.2%) patients required antibiotic use during courses of chemotherapy. A total of 28 (18.5%) patients experienced clear adverse effects during cancer treatment. Conclusion: The prophylactic PEG-rhG-CSF with a half dose can both efficaciously and safely prevent neutropenia for patients of diverse cancers with an intermediate risk of FN combined with ≥1 patient-specific risk during chemotherapy.

Upfront Treatment of Pediatric High-Risk Neuroblastoma With Chemotherapy, Surgery, and Radiotherapy Combination: The CCCG-NB-2014 Protocol.

PURPOSE: The Chinese Children's Cancer Group developed the CCCG-NB-2014 study to formulate optimal treatment strategies for high-risk (HR) neuroblastoma (NB). The safety and efficacy of this protocol were evaluated. METHOD: Patients with newly diagnosed neuroblastoma and defined as HR according to the Children's Oncology Group study were included. They were treated with a combination of chemotherapy, surgery, and radiotherapy. The treatment-related toxicities, response rate, 3-year progression-free survival (PFS), and overall survival (OS) were analyzed. RESULTS: Of 159 patients enrolled between 2014 and 2018, 80 were eligible, including 19 girls and 61 boys, with a median age of 3.9 years (range 0.9-11). After a median follow-up of 24 months (range 3-40), the median OS was 31.8 months, and 3-year OS was 83.8%. In multivariate analyses, the OS was affected by N-MYC amplification (hazard ratio 0.212, 95% confidence interval (CI) 0.049-0.910; p = 0.037) and giant tumor mass (hazard ratio 0.197, 95% CI 0.071-0.552; p = 0.002). The median 3-year PFS was 25.8 months, and 3-year PFS was 57.5%. The univariate analysis showed that only the giant tumor mass was associated with the outcome. Of the 13 deaths, 11 died from the rapid progression of the disease and two from treatment-related toxicities. The most common adverse reaction was chemotherapy-induced hematological toxicity. CONCLUSION: The PFS and OS reported in our study were similar to Western countries. The CCCG-NB-2014 protocol proved to be an efficient regimen with tolerable side-effect for the treatment of pediatric HR-NB.