Objective:To investigate the clinicopathological features including immunophenotype, molecular characteristics, differential diagnosis and prognosis of SMARCB1-deficient renal medullary carcinoma (RMC) without sickle cell trait.Methods:The clinicopathological data of 12 cases of SMARCB1-deficient RMC without sickle cell trait were collected from 7 domestic institutions during the period of 2015 to 2024. Their clinical characteristics, morphological features and immunohistochemical properties were observed and analyzed. High-throughput DNA-targeted next-generation sequencing was performed, and follow-up data were gathered along with relevant literature review.Results:Among the 12 patients, 5 were female and 7 were male. The patients age ranged from 27 to 84 years with a median age of 58.5 (46.0, 71.0) years. None of them had sickle cell disease or other hemoglobinopathies. Eight cases occurred in the left kidney and 4 cases were located in the right kidney. The average maximum diameter of the tumor was 6.1 (4.0,7.5) cm, with a range of 2.0 to 14.9 cm (the median maximum diameter 5.5 cm). Histologically, the tumors showed poorly differentiated adenocarcinoma, arranged in solid and tubular patterns. Papillary structure was noted in 5 cases, cribriform structure in 3 cases, rhabdoid differentiation in 3 cases, and sarcomatoid differentiation in 2 cases. Inflammatory desmoplastic stromal reaction was observed in 8 cases, among which stromal myxoid degeneration was seen in 6 cases. Tumor necrosis was apparent in 6 cases. The tumor cells had abundant eosinophilic or clear cytoplasm and prominent nucleoli. The nuclear grading was grade 3 or 4 according to the International Society of Urological Pathology (ISUP). Immunohistochemical staining showed that the tumor cells of all 12 cases expressed PAX8 and loss of SMARCB1/INI1 protein expression, and 5 of 10 cases expressed OCT3/4. Seven samples had valid archived paraffin tissues for high-throughput DNA-targeted next-generation sequencing. The results showed that all 7 cases had pathogenic mutations in the SMARCB1 gene. The mutation sites included exon5 c.595A>T (p.K199*), exon2 c.200_207del (p.S67*), exon2 p.G69VfsTer16, exon7 c.986G>T (p.S329I), exon7 c.886A>T (p.K296*), exon6 c.635T>A (p.L212*), exon5 c.577del (p.M193Wfs16), and exon6 c.784del (p.V262Sfs5). Follow-up data were obtained for 6 of 12 patients. Among them, 1 patient had lung and bone metastases, 1 patient had liver and bone metastases and 1 patient had multiple bone metastases at the time of diagnosis; 1 patient had bone metastases 5 months after surgery. One patient died of postoperative complications 10 days after surgery, 4 patients died of tumors (the survival time ranged from 4 to 8 months), and 1 patient had no recurrence or metastasis during the 8-month follow-up after surgery.Conclusions:SMARCB1-deficient RMC without sickle cell trait is a highly aggressive and poorly differentiated renal cell carcinoma. It has similar histomorphology, immunophenotype, molecular characteristics and prognosis to RMC, which further supports that it is a sporadic subtype of RMC related to sickle cell trait.

Objective:To investigate the clinicopathological characteristics, immunophenotypes, diagnostic criteria and differential diagnosis of atrophic kidney-like lesion (AKLL).Methods:Three cases of AKLL were collected from April 2021 to October 2023 at the Xiangya Hospital of Central South University, Changsha, Zhejiang Provincial People′s Hospital, Hangzhou and Ningbo Clinical Pathology Diagnosis Center, Ningbo, China. The clinical, morphological, and immunohistochemical characteristics were analyzed. Relevant literature was also reviewed. A targeted DNA-based next-generation sequencing (a panel of 150 genes) was performed on one of the three cases.Results:There were 1 female and 2 males, aged 30, 57, and 17 years (mean 34.6 years), respectively. The lesions were all incidentally identified during physical or imaging examination. Radiologically, they were all presented as a unilateral renal parenchymal mass. Grossly, the maximum diameters of the lesions were 1.8, 4.0, and 6.5 cm (mean 4.1 cm), respectively. The tumor cut-surfaces were sponge-like, multilocular cystic, and solid, respectively. At low magnification, the lesions were well-circumscribed, while a thick fibromuscular capsule was noted in cases 1 and 3. Cases 1 and 2 were composed of thin-walled cysts or follicular like structures of varying sizes, with the cyst wall lined by flattened and atrophic, or hobnail cells. The luminal spaces contained dense eosinophilic secretion and associated calcifications, while some cysts contained discohesive cells floating in the eosinophilic material. The tissue between the cysts showed predominantly small atrophic tubular structures. Case 3 was almost entirely composed of atrophic and collapsed tubular structures with focal cyst formation, imparting a solid sheets growth pattern under low magnification. Immunohistochemical staining revealed that the cyst lining cells and the intracystic floating cells were WT1 positive, PAX8 negative and CK7 negative, while the atrophic renal tubules were WT negative, PAX8 positive and CK7 positive. Targeted next-generation sequencing in case 1 showed no significant genetic abnormalities. All 3 patients underwent partial nephrectomy. No evidence of recurrence or metastasis was found with a follow-up of 17 to 36 months.Conclusions:AKLL is a rare and novel benign renal disease. It is easily misdiagnosed as a renal neoplasm grossly and histologically. Careful morphological observation combined with characteristic immunophenotypes can aid in its diagnosis and differential diagnosis.

Objective:To investigate the clinicopathological features including immunophenotype, molecular characteristics, differential diagnosis and prognosis of SMARCB1-deficient renal medullary carcinoma (RMC) without sickle cell trait.Methods:The clinicopathological data of 12 cases of SMARCB1-deficient RMC without sickle cell trait were collected from 7 domestic institutions during the period of 2015 to 2024. Their clinical characteristics, morphological features and immunohistochemical properties were observed and analyzed. High-throughput DNA-targeted next-generation sequencing was performed, and follow-up data were gathered along with relevant literature review.Results:Among the 12 patients, 5 were female and 7 were male. The patients age ranged from 27 to 84 years with a median age of 58.5 (46.0, 71.0) years. None of them had sickle cell disease or other hemoglobinopathies. Eight cases occurred in the left kidney and 4 cases were located in the right kidney. The average maximum diameter of the tumor was 6.1 (4.0,7.5) cm, with a range of 2.0 to 14.9 cm (the median maximum diameter 5.5 cm). Histologically, the tumors showed poorly differentiated adenocarcinoma, arranged in solid and tubular patterns. Papillary structure was noted in 5 cases, cribriform structure in 3 cases, rhabdoid differentiation in 3 cases, and sarcomatoid differentiation in 2 cases. Inflammatory desmoplastic stromal reaction was observed in 8 cases, among which stromal myxoid degeneration was seen in 6 cases. Tumor necrosis was apparent in 6 cases. The tumor cells had abundant eosinophilic or clear cytoplasm and prominent nucleoli. The nuclear grading was grade 3 or 4 according to the International Society of Urological Pathology (ISUP). Immunohistochemical staining showed that the tumor cells of all 12 cases expressed PAX8 and loss of SMARCB1/INI1 protein expression, and 5 of 10 cases expressed OCT3/4. Seven samples had valid archived paraffin tissues for high-throughput DNA-targeted next-generation sequencing. The results showed that all 7 cases had pathogenic mutations in the SMARCB1 gene. The mutation sites included exon5 c.595A>T (p.K199*), exon2 c.200_207del (p.S67*), exon2 p.G69VfsTer16, exon7 c.986G>T (p.S329I), exon7 c.886A>T (p.K296*), exon6 c.635T>A (p.L212*), exon5 c.577del (p.M193Wfs16), and exon6 c.784del (p.V262Sfs5). Follow-up data were obtained for 6 of 12 patients. Among them, 1 patient had lung and bone metastases, 1 patient had liver and bone metastases and 1 patient had multiple bone metastases at the time of diagnosis; 1 patient had bone metastases 5 months after surgery. One patient died of postoperative complications 10 days after surgery, 4 patients died of tumors (the survival time ranged from 4 to 8 months), and 1 patient had no recurrence or metastasis during the 8-month follow-up after surgery.Conclusions:SMARCB1-deficient RMC without sickle cell trait is a highly aggressive and poorly differentiated renal cell carcinoma. It has similar histomorphology, immunophenotype, molecular characteristics and prognosis to RMC, which further supports that it is a sporadic subtype of RMC related to sickle cell trait.

Objective:To investigate the clinicopathological characteristics, immunophenotypes, diagnostic criteria and differential diagnosis of atrophic kidney-like lesion (AKLL).Methods:Three cases of AKLL were collected from April 2021 to October 2023 at the Xiangya Hospital of Central South University, Changsha, Zhejiang Provincial People′s Hospital, Hangzhou and Ningbo Clinical Pathology Diagnosis Center, Ningbo, China. The clinical, morphological, and immunohistochemical characteristics were analyzed. Relevant literature was also reviewed. A targeted DNA-based next-generation sequencing (a panel of 150 genes) was performed on one of the three cases.Results:There were 1 female and 2 males, aged 30, 57, and 17 years (mean 34.6 years), respectively. The lesions were all incidentally identified during physical or imaging examination. Radiologically, they were all presented as a unilateral renal parenchymal mass. Grossly, the maximum diameters of the lesions were 1.8, 4.0, and 6.5 cm (mean 4.1 cm), respectively. The tumor cut-surfaces were sponge-like, multilocular cystic, and solid, respectively. At low magnification, the lesions were well-circumscribed, while a thick fibromuscular capsule was noted in cases 1 and 3. Cases 1 and 2 were composed of thin-walled cysts or follicular like structures of varying sizes, with the cyst wall lined by flattened and atrophic, or hobnail cells. The luminal spaces contained dense eosinophilic secretion and associated calcifications, while some cysts contained discohesive cells floating in the eosinophilic material. The tissue between the cysts showed predominantly small atrophic tubular structures. Case 3 was almost entirely composed of atrophic and collapsed tubular structures with focal cyst formation, imparting a solid sheets growth pattern under low magnification. Immunohistochemical staining revealed that the cyst lining cells and the intracystic floating cells were WT1 positive, PAX8 negative and CK7 negative, while the atrophic renal tubules were WT negative, PAX8 positive and CK7 positive. Targeted next-generation sequencing in case 1 showed no significant genetic abnormalities. All 3 patients underwent partial nephrectomy. No evidence of recurrence or metastasis was found with a follow-up of 17 to 36 months.Conclusions:AKLL is a rare and novel benign renal disease. It is easily misdiagnosed as a renal neoplasm grossly and histologically. Careful morphological observation combined with characteristic immunophenotypes can aid in its diagnosis and differential diagnosis.

Objective To construct a key nursing technology system for the treatment of patients exposed to nuclear radiation in hospitals,and provide technical guidance and support for emergency nursing rescue in hospitals of nuclear radiation accidents.Methods A research group was composed of a team with rich experience in nuclear radiation accidents.Based on 4 scenarios of nuclear radiation accidents(including external irradiation,internal irradiation,external contamination,internal contamination),the literature search was conducted to form the first draft of the system.Delphi method was used to complete 2 rounds of expert letter consultation,and the final draft of the key nursing technology system for hospital treatment of patients with nuclear radiation exposure was constructed according to the revised opinions of experts.Results A total of 16 experts completed 2 rounds of correspondence.The effective recovery rates were 100％and 80％;the recommendation rates were 65％and 50％;the authority coefficients(Cr)were 0.778 and 0.797;the coefficient of variation(CV)of the 2 rounds of expert letter consultation was ≤0.25.Finally,a key nursing technology system for in-hospital treatment of patients with nuclear radiation exposure was formed,including 5 first-level indicators,26 second-level indicators and 74 third-level indicators.Conclusion The constructed key nursing technology system for hospital treatment of patients with nuclear radiation exposure is highly practical and scientific,and it is conducive to the formation of standardized nuclear radiation exposure treatment procedures,and provides a theoretical basis for the training and evaluation of nursing staff related to nuclear radiation exposure.

Background Bisphenol compounds are non-persistent environmental endocrine disruptors and frequently detected in drinking water systems, indicating potential human health risks through drinking water. Objective To establish and optimize a simultaneous determination method for 16 BPs in drinking water by online solid-phase extraction-ultra high-performance liquid chromatography-triple quadrupole mass spectrometry, in order to efficiently monitoring BPs in drinking water. Methods Candidate online solid-phase extraction conditions, chromatographic columns, mobile phase systems, mass spectrometry parameters, and other conditions were compared by chromatographic peaks of BPs, and processing conditions such as water sample preservation and pretreatment were optimized. The pH level of drinking water samples was adjusted and solid particles were removed. After extraction and purification by an online solid-phase extraction system, samples were detected by ultra-high performance liquid chromatography tandem mass spectrometry and quantified by isotope internal standard method. The proposed method was verified by pure water and terminal tap water, and evaluated by spiked recovery rate and relative standard deviation. Eighty-eight tap water samples from different regions of local pipeline network were collected for method application. Results For the 16 BPs, the calibration curves showed good linearity between 1.0 and 75 ng·L⁻¹ and the correlation coefficients were greater than 0.995. The detection limit of the method was less than 0.30 ng·L⁻¹, and the quantification limit of the method was less than 1.0 ng·L⁻¹. When the spiked concentrations for the 16 BPs were 5.0, 15, and 40 ng·L⁻¹, the average spiked recovery rates of the test substances were between (100 ± 10)%, and the relative standard deviations were all below 10%. In the method application to the local terminal water samples, the positive rates of bisphenol S (BPS), bisphenol A (BPA), and bisphenol AF (BPAF) were as high as 93.2%, 77.3%, and 29.5%, respectively. The concentrations of BPS were from not detected (N.D.) to 37.8 ng·L⁻¹, and the concentrations of BPA were from N.D. to 52.0 ng·L⁻¹. Conclusion The method using an online solid-phase extraction system is established, featuring simple pre-treatment, small sample volume, high degree of automation, low detection limit, and good accuracy and precision. This method can be applied to the quantitative monitoring of 16 BPs at ng·L⁻¹ level in drinking water.

Objective To compare the value of low-dose spiral CT and conventional-dose CT for diagnosis of benign and malignant pulmonary nodules, so as to provide a reference for early screening of lung cancer. Methods A total of 288 patients diagnosed with pulmonary nodules in Ningguo Municipal People’s Hospital during the period from January 2019 to August 2023 were enrolled, and all patients underwent low-dose chest spiral CT and conventional-dose CT scans. The pathological biopsy of pulmonary nodules served as a gold standard. The sensitivity, specificity, and accuracy of low-dose spiral CT and conventional-dose CT for diagnosis of benign and malignant pulmonary nodules and the detection rates of vascular aggregation sign, pleural indentation sign, lobulation sign, and spiculation sign were compared. Results The 288 patients with pulmonary nodules included 186 men and 102 women, with a mean age of 61.12 ± 8.34 years. Among these patients, 218 had benign pulmonary nodules and 70 had malignant pulmonary nodules. There were no significant differences between patients with benign and malignant pulmonary nodules in terms of sex, mean age, and the size, location, and characteristics of pulmonary nodules (all P values > 0.05). However, significant differences were observed in terms of changes in pulmonary nodule size, history of smoking, history of pulmonary infections, and family history of cancers (all P values < 0.05). With pathological biopsy of pulmonary nodules as a gold standard, the sensitivity, specificity, and accuracy of conventional-dose CT and low-dose spiral CT for diagnosis of benign and malignant pulmonary nodules were 94.29% and 92.86%, 93.57% and 92.20%, and 93.75% and 92.36%, respectively, with no significant differences (χ² = 1.975, 1.012, and 1.911, all P values > 0.05). There was no significant difference in the area under the receiver operating characteristic curve between conventional-dose CT and low-dose spiral CT (0.937 vs. 0.921; t = 1.021, P > 0.05). The detection rates of vascular aggregation sign, pleural indentation sign, lobulation sign, and spiculation sign were 20.49% and 19.79%, 23.26% and 22.57%, 37.50% and 35.76%, and 29.17% and 27.43%, respectively, for conventional-dose CT and low-dose spiral CT, with no significant differences (χ² = 0.171, 0.212, 1.012, and 1.110, all P values > 0.05). Conclusion The diagnostic sensitivity, specificity, and accuracy of benign and malignant pulmonary nodules and the detection rate of pulmonary nodule signs are comparable between conventional-dose CT and low-dose spiral CT. However, low-dose spiral CT has lower radiation dose and higher safety, which deserves extensive applications in early screening for lung cancer.

Objective To establish a novel clinical application process of the optical surface monitoring system(OSMS)guided volumetric modulated arc therapy(VMAT)for total body irradiation(TBI),and to assess the accuracy and effectiveness of OSMS in inter-fractional auxiliary positioning before radiotherapy and real-time monitoring of intra-fractional motion during radiotherapy.Methods A retrospective analysis was conducted on 15 leukemia patients who underwent OSMS-guided VMAT-TBI before hematopoietic stem cell transplantation.CT simulation positioning was performed,and the whole-body image data which were collected in head-first supine position(HFS)and feet-first supine position(FFS)were transmitted to the treatment planning system for image registration,multicenter VMAT planning and dose verification.The prescription dose was 800 cGy in 4 fractions twice daily.OSMS was used to assist positioning before delivery,and CBCT was used for position verification.During treatment,OSMS was used for monitoring.The intra-fractional error monitored by OSMS in real time was obtained by analyzing the offline log files.Results The mean dose and coverage of the target area in HFS plan were(905.4±19.0)cGy and 93.0%±2.8%.The mean doses to lung and kidney were(603.7±55.7)cGy and(600.4±49.6)cGy,respectively,and the maximum dose to the lens was(393.9±58.9)cGy.The mean dose and coverage of the target area in FFS plan were(888.5±58.9)cGy and 94.0%±3.2%;and the maximum dose at the junction was(1148.9±72.9)cGy.Fractional treatment delivery time was(75.1±15.1)min.OSMS-assisted positioning was carried out before delivery,and the total deviations of CBCT three-dimensional vector in translational and rotation directions were(2.71±1.96)mm and 0.91°±0.90°,respectively.The three-dimensional vector deviation of the intra-fractional motion amplitude in translational direction monitored by OSMS during the treatment was(1.95±1.88)mm,of which the deviation within 1 mm accounted for 57.5%,79.7%and 62.1%in longitudinal,lateral and vertical directions,respectively.The three-dimensional vector deviation in rotation direction was 0.76°±0.72°,of which the deviation within 1°accounted for 93.1%,85.7%and 94.3%in rotation,pitch and roll directions,respectively.Conclusion VMAT simplifies TBI process,while improving target coverage and organs-at-risk sparing.The use of OSMS can reduce positioning errors,especially rotation errors.In order to ensure the accurate implementation of TBI and the safety of patients,it is necessary to use OSMS for auxiliary positioning and intra-fractional position monitoring.

Objective:To understand the views and suggestions of medical staff on the key nursing techniques and norms of in-hospital treatment of patients with nuclear exposure, so as to provide reference for the continuous optimization of follow-up processes.Methods:From September to October 2021,purpose sampling was adopted to select doctors and nurses who engaged in medical emergency rescue of nuclear exposure-related work in two divisions of the Nuclear Accident Medical Emergency Center of the National Health Commission as the research subjects, using a combination of online and offline methods to carry out semi-structured interviews with 6 research subjects,using content analysis methods in descriptive research for data analysis.Results:A total of four themes were refined including the need for the establishment of nursing technology and process specification for nuclear accident emergency rescue specialty; the need for specialized training of nursing technology in nuclear accident emergency rescue; the need for the construction of specialized nursing team for nuclear accident emergency rescue; the need to strengthen the closeness of multi-team cooperation.Conclusions:The current in-hospital care technology and processes for nuclear exposed patients need to be further refined and standardized, and in the future, we need to establish a perfect in-hospital care technology and processes for nuclear exposed patients, and according to the corresponding technology and processes, strengthen nursing staff professional training and simulation training in nuclear accident emergency rescue, and establish a nuclear emergency rescue professional nursing team to promote the development of nuclear accident emergency rescue nursing specialists.

Objective:To study the impact of the Varian real-time position management (RPM) respiratory gating system on radiotherapy planning dosimetry.Methods:The radiotherapy plans of 40 cases with thoracic or abdominal tumors were retrospectively selected in this study. The motion phantom for quality control was adopted to generate respiratory gating signals, and the 30%-60% stable phase at the end of expiratory was selected as the respiratory gating window. The dose verification for the abovementioned radiotherapy plans was performed using the Portal Dosimetry (PD) system under RPM respiratory gating mode with the Edge accelerator. Afterwards, dose analysis was performed with different γ passing rate criteria and the distribution characteristics of γ values were analyzed. Finally, the verification results between the non-gating mode and the gating mode were compared.Results:Under the respiratory gating mode, the passing rates of all intensity-modulated radiation therapy/volumetric-modulated arc therapy (IMRT/VMAT) plans with or without flattening filters were over 95.5% by γ criteria of (3%, 3 mm) or (3%, 2 mm) and were over 90% by stricter γ criteria of (2%, 2 mm). All plans met the clinical requirements recommended by the American Association of Physicists in Medicine (AAPM). The passing rates of dose verification under non-gating mode were slightly better than those under respiratory gating mode, and the differences between the two modes were statistically significant (3%/3 mm, Z =-1.45; 3%/2 mm, Z =-2.86; 2%/2 mm, Z =-3.70; 1%/1 mm, Z =-4.52; P<0.05). There was no significant difference in the minimum and maximum values of γ and the share of γ > 1.5 of plan verification result under the two modes. However, the average value and standard deviation of the γ were generally smaller under the non-gating mode. Conclusions:The impact of the introduction of RPM respiratory gating technology on dose is clinically acceptable, and the execution of these plans in this gating mode is safe and reliable.