Objective:To investigate the current status of research in gene therapy for retinitis pigmentosa (RP) from 2005 to 2024.Methods:The literature related to gene therapy for RP included in the Web of Science Core Collection dataset from January 1, 2005 to September 15, 2024 was retrieved and screened. The bibliometrix package of R software was used to analyze the annual trend of the number of publications, citation frequency, distribution of countries/regions of the literature, and distribution of journals containing the articles. CiteSpace software was used to perform keyword clustering analysis and the keywords bursts analysis.Results:A total of 209 articles were included. There was an overall fluctuating upward trend of annual publications from 2005 to 2024, with the highest number of publications in 2023 at 26 (12.4%, 26/209), and the lowest number of publications in 2006 at 2 (0.9%, 2/209). There was an overall increasing trend in the frequency of citations to relevant literature. Corresponding authors from the United States had the highest total number of publications with 98 (46.9%, 98/209). Among authors, Hauswirth from the University of Florida, USA, had the most with 25 (12.0%, 25/209). Among institutions, Columbia University, USA, had the most with 55 (26.3%, 55/209). Among journals, Mol Ther had the most with 25 (12.0%, 25/209), and it had the highest 2023 impact factor of 12.1. Keyword clustering analysis yielded eight valid clusters, namely #0 P23H, #1 AAV, #2 PDE6B,#3 CRB1, #4 RPGR, #5 antisense oligonucleotide, #6 NR2E3, and #7 NRL, which intersected with each other with good continuity. The keywords bursts analysis showed that the keyword with the longest emergence time was RNAi, followed by PDE and PDE6. USH2A, CRB1, CRISPR Cas9, base editing, and ORF15 were keywords that emerged in recent years and were continuously studied. Conclusions:RP gene therapy research literature has shown an increasing trend from 2005 to 2024, with the highest number of publications from research organizations and scholars in the United States. Currently, studies focus on RHO, PDE6B, CRB1, RPGR, NR2E3, and NRL gene. In recent years, there has been a gradual increase in studies on USH2A, CRB1 genes, and the RPGR ORF15 region. CRISPR Cas9 and base editing gene therapy strategies are being developed.

Objective:To investigate the current status of research in gene therapy for retinitis pigmentosa (RP) from 2005 to 2024.Methods:The literature related to gene therapy for RP included in the Web of Science Core Collection dataset from January 1, 2005 to September 15, 2024 was retrieved and screened. The bibliometrix package of R software was used to analyze the annual trend of the number of publications, citation frequency, distribution of countries/regions of the literature, and distribution of journals containing the articles. CiteSpace software was used to perform keyword clustering analysis and the keywords bursts analysis.Results:A total of 209 articles were included. There was an overall fluctuating upward trend of annual publications from 2005 to 2024, with the highest number of publications in 2023 at 26 (12.4%, 26/209), and the lowest number of publications in 2006 at 2 (0.9%, 2/209). There was an overall increasing trend in the frequency of citations to relevant literature. Corresponding authors from the United States had the highest total number of publications with 98 (46.9%, 98/209). Among authors, Hauswirth from the University of Florida, USA, had the most with 25 (12.0%, 25/209). Among institutions, Columbia University, USA, had the most with 55 (26.3%, 55/209). Among journals, Mol Ther had the most with 25 (12.0%, 25/209), and it had the highest 2023 impact factor of 12.1. Keyword clustering analysis yielded eight valid clusters, namely #0 P23H, #1 AAV, #2 PDE6B,#3 CRB1, #4 RPGR, #5 antisense oligonucleotide, #6 NR2E3, and #7 NRL, which intersected with each other with good continuity. The keywords bursts analysis showed that the keyword with the longest emergence time was RNAi, followed by PDE and PDE6. USH2A, CRB1, CRISPR Cas9, base editing, and ORF15 were keywords that emerged in recent years and were continuously studied. Conclusions:RP gene therapy research literature has shown an increasing trend from 2005 to 2024, with the highest number of publications from research organizations and scholars in the United States. Currently, studies focus on RHO, PDE6B, CRB1, RPGR, NR2E3, and NRL gene. In recent years, there has been a gradual increase in studies on USH2A, CRB1 genes, and the RPGR ORF15 region. CRISPR Cas9 and base editing gene therapy strategies are being developed.

Objective:To investigate the current situation of the use of transjugular intrahepatic portosystemic shunt (TIPS) for portal hypertension, which should aid the development of TIPS in China.Methods:The China Portal Hypertension Alliance (CHESS) initiated this study that comprehensively investigated the basic situation of TIPS for portal hypertension in China through network research. The survey included the following: the number of surgical cases, main indications, the development of Early-TIPS, TIPS for portal vein cavernous transformation, collateral circulation embolization, intraoperative portal pressure gradient measurement, commonly used stent types, conventional anticoagulation and time, postoperative follow-up, obstacles, and the application of domestic instruments.Results:According to the survey, a total of 13 527 TIPS operations were carried out in 545 hospitals participating in the survey in 2021, and 94.1% of the hospital had the habit of routine follow-up after TIPS. Most hospitals believed that the main indications of TIPS were the control of acute bleeding (42.6%) and the prevention of rebleeding (40.7%). 48.1% of the teams carried out early or priority TIPS, 53.0% of the teams carried out TIPS for the cavernous transformation of the portal vein, and 81.0% chose routine embolization of collateral circulation during operation. Most of them used coils and biological glue as embolic materials, and 78.5% of the team routinely performed intraoperative portal pressure gradient measurements. In selecting TIPS stents, 57.1% of the hospitals woulel choose Viator-specific stents, 57.2% woulel choose conventional anticoagulation after TIPS, and the duration of anticoagulation was between 3-6 months (55.4%). The limitation of TIPS surgery was mainly due to cost (72.3%) and insufficient understanding of doctors in related departments (77.4%). Most teams accepted the domestic instruments used in TIPS (92.7%).Conclusions:This survey shows that TIPS treatment is an essential part of treating portal hypertension in China. The total number of TIPS cases is far from that of patients with portal hypertension. In the future, it is still necessary to popularize TIPS technology and further standardize surgical indications, routine operations, and instrument application.

Since the beginning of the 21st century, with the continuous development of anti-HER2-targeted drugs, more treatment options have been provided for patients with HER2-positive breast cancer and the survival prognosis has been significantly improved. At present, anti-HER2 targeted drugs mainly include monoclonal antibody drugs such as trastuzumab and pertuzumab, small molecule tyrosine kinase inhibitors such as lapatinib and neratinib, and antibody-drug conjugates such as TDM1 and T-DXd, which play an extremely important role in different disease processes. The treatment of HER2-positive breast cancer is based on targeted therapy with trastuzumab. Early-stage patients with high risk factors can be treated with intensive targeted therapy to further improve the prognosis, while advanced patients need a reasonable arrangement of targeted therapy to overcome drug resistance and prolong survival. This article will review the current status, the latest research progress and the future prospects of anti-HER2 targeted therapy in different stages of the disease.

Objective:To evaluate the psychological pain of patients with head and neck cancer aged ≥60 years old before and after intensity-modulated radiotherapy (IMRT).Methods:Distress Thermometer (DT)(Chinese version) was used to investigate the degree and problems of psychological pain before and after IMRT for 85 elderly patients with head and neck cancer. The results before and after IMRT were compared by paired t-test. Relevant factors were identified by Logistic regression analysis. Results:The median age in the cohort was 66 years old (60-85 years old). The incidence rates of psychological pain were 73% and 87% before and after IMRT ( P<0.001). The corresponding incidence rates of severe distress were 6% and 34%( P<0.001). The main distress problems before IMRT were memory loss/attention deficit, worry, oral pain, economic problems, stress, sleep problems, and dry nose. The significantly-increased distress problems after IMRT were oral pain, constipation, eating, nausea, and dry nose. Logistic regression analysis showed gender ( OR=5.520, 95% CI 1.437-21.212, P=0.013), pre-treatment PG-SGA score ( OR=1.220, 95% CI 1.048-1.421, P=0.010) and medical insurance ( OR=0.230, 95% CI 0.053-0.995, P=0.049) were the relevant factors of the severe psychological distress before IMRT. Occupation ( OR=2.286, 95% CI 1.291-4.050, P=0.005) and medical insurance ( OR=0.089, 95% CI 0.029-0.276, P<0.001) were the relevant factors of severe psychological distress after IMRT. Conclusion:The incidence rate of distress is high in elderly patients with head and neck cancer before IMRT, which can be aggravated after IMRT, primarily the treatment-related physical pain problems.

Objective:To identify the population who can obtain clinical benefit from concurrent chemoradiotherapy through the survival analysis during concurrent chemoradiotherapy in different subgroups.Methods:All data from a phase Ⅲ randomized controlled clinical trial were collected to compare the efficacy between preoperative concurrent chemoradiotherapy and preoperative radiotherapy from 2002 to 2012 in Cancer Hospital of the Chinese Academy of Medical Sciences. A total of 222 patients received radiation therapy with a median dose of 69.96 Gy (27.56-76.00 Gy). The cisplatin chemotherapy regimen was adopted and the median dose was 250 mg (100-570 mg). In total, 98 patients received intensity-modulated radiotherapy (IMRT). The survival analysis was conducted with Kaplan- Meier method and univariate analysis was performed with log-rank test. The multivariate prognostic analysis was conducted with Cox’s regression model. Results:The median follow-up time was 59 months (7-139 months). Among them, 104 patients were assigned in the chemoradiotherapy group and 118 patients in the radiotherapy alone group. The local and regional recurrence rates did not significantly differ between two groups (both P>0.05), while chemoradiotherapy tended to decrease the distant metastasis rate compared with the radiotherapy alone (14.4% vs. 24.6, P=0.058). Univariate analysis showed that concurrent chemoradiotherapy significantly increased the local recurrence-free survival in the early N stage subgroup ( P=0.009), and there was an increasing trend in patients aged≤55 years and female patients ( P=0.052, 0.066). The distant metastasis-free survival was significantly improved in T 4( P=0.048), N 3( P=0.005), non-IMRT treatment ( P=0.001) and hypopharyngeal carcinoma ( P=0.004) subgroups, there was an increasing trend in male ( P=0.064), high-and moderate-grade squamous cell carcinoma ( P=0.076) and non-surgical treatment subgroups ( P=0.063). Multivariate analyses showed that concurrent chemoradiotherapy significantly prolonged the progression-free survival and overall survival in patients aged≤55 years ( P=0.017 and 0.039), women ( P=0.041 and 0.039), high-and moderate-grade squamous cell carcinoma ( P=0.006 and 0.022), N 3 stage ( P=0.001 and 0.017), non-surgical treatment ( P=0.007 and 0.033) and non-IMRT treatment subgroups ( P=0.030 and 0.024), and it significantly increased the progression-free survival in patients with hypopharyngeal carcinoma ( P=0.022). Conclusion:Concurrent chemoradiotherapy can be actively delivered for young age, female, high-and moderate-grade squamous cell carcinoma, N 3 stage, non-surgical treatment and non-IMRT treatment patients.

Objective:To compare the effects of comprehensive treatment with different combinations of radiotherapy, chemotherapy and surgery on the survival of patients with locally advanced head and neck squamous cell carcinoma (LA-HNSCC).Methods:From September 2002 to May 2012, 222 patients were enrolled in a randomized controlled clinical trial to compare the clinical efficacy between preoperative radiotherapy and preoperative concurrent chemoradiotherapy. The chemotherapy was performed at the beginning of the radiotherapy, with cisplatin 30 mg/m 2 every week. Conventional radiotherapy or intensity-modulated radiotherapy (IMRT) was adopted. Clinical efficacy was evaluated during radiotherapy to 50 Gy in all patients. Whether surgery or original treatment regime was given was determined according to the clinical efficacy. The survival of different therapeutic methods was analyzed by Kaplan- Meier method. Results:The median follow-up time was 59 months (7-139 months). All patients were divided into four groups: radiotherapy group (R group, n=84), concurrent chemo-radiotherapy group (R+ C group, n=67), preoperative radiotherapy group (R+ S group, n=34) and preoperative concurrent chemoradiotherapy group (R+ C+ S group, n=37). The 5-year overall survival rates were 32%, 44%, 51%, and 52%, respectively (R+ C+ S group vs. R group, P=0.047). The 5-year progression-free survival rates were 34%, 48%, 49%, and 61%, respectively (R+ C Group vs. R group, P=0.081; R+ C+ S group vs. R group, P=0.035). The 5-yeal distant metastasis-free survival rates were 70%, 85%, 65%, and 73%, respectively (R+ C group vs. R+ S group, P=0.064; R+ C group vs. R+ S group, P=0.016). Conclusions:Compared with radiotherapy alone, comprehensive treatment with different combinations can improve the long-term survival of LA-HNSCC patients. Radiotherapy combined with chemotherapy has a tendency to improve the distant metastasis-free survival rate, The optimal comprehensive treatment modality that improves the overall survival of LA-HNSCC patients remains to be explored.

Objective:To analyze the incidence and high-risk factors of lymph node metastasis in the retropharyngeal region of hypopharyngeal squamous cell carcinoma based on magnetic resonance imaging (MRI), aiming to guide the delineation of clinical target area.Methods:Clinical data of patients who were pathologically diagnosed with hypopharyngeal carcinoma from January 2012 to September 2018 in our center were retrospectively analyzed. All patients received head and neck MRI before treatment. The diagnosis of lymph node metastasis and the delineation of primary gross target volume (GTV p) and lymph nodes target volume (GTV nd) were determined by all the radiation oncologists in head and neck group through twice weekly general round discussion. The cut-off points of GTV p and GTV nd were defined by establishing the receiver operating characteristic curve. All patients were divided into the high GTV p, low GTV p and high GTV nd and low GTV nd groups. Chi-square test was used for univariate analysis and logistic regression was utilized for multivariate analysis to analyze the high-risk factors of patients with retropharyngeal lymph node metastasis. Results:A total of 326 patients were included in this study, 295 of whom were diagnosed with cervical lymph node metastasis, accounting for 90.5%. The most common involved area was Level Ⅱ a, followed by Level Ⅲ, Level Ⅱ b, Level IV, Level Ⅶ a (retropharyngeal), Level V a, and Level V b. The incidence of retropharyngeal lymph node metastasis was 21.5%, and the incidence was 53.1% in patients with primary tumor located in the posterior pharyngeal wall. Univariate and multivariate analyses showed that patients with tumor originated from the posterior pharyngeal wall ( P=0.002), bilateral cervical lymph node metastasis ( P=0.020), larger GTV p (greater than 47 cm 3, P=0.003), and larger GTV nd (greater than 22 cm 3, P=0.023) were significantly associated with the occurrence of retropharyngeal lymph node metastasis. Conclusions:The incidence of retropharyngeal lymph node metastasis is high in hypopharyngeal carcinoma, especially in patients with primary tumors in the posterior pharyngeal wall, bilateral cervical lymph node metastasis and larger primary burden. Therefore, for patients with these risk factors, it is highly recommended that the clinical target area should be delineated to include the retropharyngeal lymph node drainage area.

Objective:To investigate the patterns of regional lymphatic spread and the value of elective neck treatment (ENT) in oral mucosal melanoma (OMM).Methods:In this retrospective analysis, 61 OMM patients with no distant metastasis treated in Cancer Hospital of Chinese Academy of Medical Sciences between 1984 and 2016 were recruitred. The regional lymph node distribution of cN+ disease, the value of ENT in cN 0 disease, the failure patterns and prognostic factors were retrospectively analyzed. Results:Overall, 55.7% of the patients were clinical/pathological cN+ . The most frequently involved locations were the level Ⅰ b (76%), followed by level Ⅱ and level Ⅲ. For cN 0 patients, the 5-year regional failure-free survival rate was 91.7% in patients who received at least ipsilateral level Ⅰ b-Ⅲ ENT and 52.4% in patients who did not receive at least ipsilateral level Ⅰ b-Ⅲ ENT ( P=0.036). The regional failure rate was 6% for patients treated with at least ipsilateral leve Ⅰ b-Ⅲ ENT, while in their counterparts who did not receive at least ipsilateral level Ⅰ b-Ⅲ ENT was 46%( P=0.035). For the regional failure pattern, the most frequently failure sites were level Ⅰ b (93%), level Ⅱ(50%) and level Ⅲ(36%). Conclusions:The cervical lymph node metastasis rate is relatively high in OMM patients. The pathway of regional LN spread follows a regular pattern. The most frequently involved regions for clinical/pathological cN+ and regional failure are both level Ⅰ b-Ⅲ. Elective treatment including at least ipsilateral level Ⅰ b-Ⅲ ENT should be recommended for OMM patients with cN 0.

Objective: To analyze the clinical features, diagnosis and prognosis of patients with primary lymphoepithelial carcinoma of the parotid gland. Methods: Clinical data of 13 patients diagnosed with lymphoepithelial carcinoma of the parotid gland in our hospital from 2009 to 2017 were retrospectively analyzed. The median follow-up time was 38.5 months. All patients received radiotherapy after operation. Results: Of 13 patients, 9 cases were male and 4 female. The median age was 33 years. At the initial diagnosis, 9 cases had primary lesions limited to the parotid gland, and 4 cases of lymph node metastases located in Ⅰ_b and Ⅱ regions of the neck. According to UICC2010 staging, 1 case was classified as stage Ⅰ, 1 as stage Ⅱ, 6 as stage Ⅲ and 5 as stage Ⅳ, respectively. Eleven surgically pathological specimens were tested with EBER in-situ, and 10 cases were positive for EBER. No patient died in the whole group. The 3-year overall survival rate was 100%. The 3-year progression-free survival rate was 76%. The 3-year local control rate was 92%. The 3-year metastasis-free survival rate was 84%. Conclusions The incidence of lymphoepithelial carcinoma of the parotid gland is relatively low. The pathological features are associated with EB virus. It is prone to present with cervical lymph node metastasis. The possibility of lymph node metastasis of nasopharyngeal carcinoma to the parotid gland should be excluded before treatment. At present, surgery combined with postoperative radiotherapy is the main treatment. The overall survival is favorable. Local recurrence and distant metastasis are the main causes of treatment failure.