Objective:To describe the clinicopathological features of pulmonary artery intimal sarcoma (PAIS), and to understand its molecular alterations.Methods:Sixty cases of pulmonary artery endarterectomy performed at the China-Japan Friendship Hospital, Beijing, China from January 2017 to January 2020 were reviewed. Clinical data of 5 patients with pulmonary artery intimal sarcoma were collected. Hematoxylin-eosin staining, immunohistochemistry staining and fluorescence in situ hybridization (FISH) were performed to evaluate the pathological features. RNA sequencing was conducted to assess the fusion gene changes in PAIS.Results:The detection rate of PAIS was 8.3% (5/60), with the median age of 49 years and a female predominance. Their clinical manifestations were non-specific. Histopathological examination showed that the tumors were composed of malignant spindle or epithelioid cells, with various degrees of atypia. Focal heterologous osteosarcomatous or leiomyosarcomatous differentiation was noted. The tumor cells could express PDGFRA, CDK4 and MDM2 with co-amplification of MDM2, CDK4 and EGFR genes. RNA sequencing detected multiple in-frame fusions in the tumors.Conclusions:PAIS is a rare, highly heterogeneous, and poorly-or un-differentiated sarcoma accompanied by complex changes of multiple genes.It has no known effective treatments, and thus has a poor prognosis.

Objective:To investigate the clinicopathological features and prognostic factors of primary mediastinal large B-cell lymphoma (PMBL).Methods:The clinical data of 60 patients with PMBL including 44 biopsy cases and 16 consultation cases from September 2000 to November 2019 in the Department of Pathology, China-Japan Friendship Hospital (14 cases) and Peking Union Medical College Hospital (46 cases) were enrolled. Pathologic features, immunophenotype, immunoglobulin (Ig) gene rearrangement and microRNA expression profile were retrospectively studied.Results:Of the 60 patients, 23 were males and 37 were females, age ranged from 15 to 64 years (median 28 years). Immunohistochemical staining showed that the tumor cells were positive for pan-B cell antigens, CD30 (77.4%, 24/31), CD23 (73.1%, 19/26), MUM1 (45.8%, 11/24), Ki-67 index ≥70 % (90.6%, 29/32). EBER in situ hybridization was analyzed in 21 PMBL, only one case (4.8%) was positive. Ig gene rearrangement was performed in 20 cases, and seven were positive (35.0%). MicroRNA gene expression profiles were analyzed in seven cases of PMBL and nine cases of diffuse large B-cell lymphoma, and there were 33 microRNAs with significant difference ( P<0.05). Univariate analysis indicated that the poor prognostic factors included serum lactate dehydrogenase (LDH) level,International Prognostic Index (IPI) score ≥3, stages Ⅲ-Ⅳ, chemotherapy not combined with rituximab and MUM1 positivity ( P<0.05). Multivariate analysis showed that the treatment combined with rituximab was independently related to prognosis ( P<0.05). Conclusions:PMBL is different from diffuse large B-cell lymphoma in clinicopathologic features, immunophenotypic presentation and molecular features. The prognostic factors, molecular genetics and immunological characteristics reveal that this study has enriched our understanding of the biology of PMBL, thus providing evidence and strategies for treatment.

Objective:To describe the clinicopathological features of the lung cancers in the lungs explanted from lung transplant recipients, and to understand the molecular alterations of these cancers.Methods:The patients who underwent lung transplantation in China-Japan Friendship Hospital from March 2017 to December 2018 were reviewed. Clinical data of the patients with lung cancer associated with end-stage interstitial lung diseases (ILD) were collected. Hematoxylin-eosin staining and immunohistochemistry were performed to evaluate the pathological feature. Real-time quantitative PCR was performed to analyze the hotspots and targeted regions of 9 cancer-associated genes.Results:Among the 154 identified patients, 10 met the inclusion criteria and were included. The detection rate of lung cancer in the lung transplantation patients was 6.5%(10/154). All of the included 10 patients were male, with an average age of 59 years. They all had a history of heavy smoking. Three cases had a lung cancer diagnosed before operation, while the other 7 cases were concealed in the specimen of end-stage ILD. All of lung cancers were non-small-cell carcinoma, including 8 cases of adenocarcinoma and 2 cases of squamous cell carcinoma. The proportion of mucinous adenocarcinoma components was 3/10. The mutations in KRAS gene exon 2 were detected in two patients with mucous adenocarcinoma, while no alterations in NRAS, EGFR, ALK, ROS1, BRAF, HER2, PI3KCA and RET were detected in the remaining patients.Conclusions:Lung cancers are difficult to detect in patients with end-stage ILD. They are mainly adenocarcinomas and associated with a higher frequency of mutations in KRAS gene. These cancers have limited treatment options and a poor prognosis.

Objective: To investigate the clinical pathologic characteristics of extranodal follicular dendritic cell sarcoma (FDCS). Methods: We collected 7 cases of extranodal FDCS, HE staining, immunohistochemical study were performed. The V600E mutation of BRAF in 7 cases were detected by real-time PCR and EBER in situ hybridization was performed on 4 cases. Results: Among the 7 cases of FDCS, 5 cases were male and 2 cases were female, the median age was 55 years old, including 4 cases of low-grade FDCS and 3 cases of high-grade FDCS. The tumor location of 2 cases was in mediastinum, the tumor locations of others were in nasopharynx, kidney, lung, rectum and liver, respectively. The results of immunohistochemistry showed that, the tumor cells were diffusely or focally positive for CD21, CD23, CD35, D2-40, EGFR and CXCL13, but negative for S-100, CD68, HMB45, SMA, Desmin, CD117, Dog-1, CD34, CD30, EMA and CK.Five cases were positive for PD-L1 and the its expression in high-grade FDCS were higher than that in low-grade FDCS.Two cases of low-grade FDCS were positive for BRAF V600E, but the BRAF V600E mutation weren′t detected in all of 7 cases. The result of EBER in-situ hybridization showed that only the nasopharynx FDCS was positive.The follow-up information of 5 patients were available (7~43 months), 4 patients died and 1 still alive with rectum metastasis. Conclusions FDCS is a rare malignant disease with relapse and metastatic tendency. The combined applications of the first-line antibodies including CD21, CD23, CD35 and second-line antibodies including D2-40, CXCL13, EGFR are helpful for its diagnosis and differential diagnosis. The high expression of PD-L1 implicates the potential benefit of FDCS patients acquired from immunotherapy.

Objective: To evaluate the risk factors of postoperative upgrade to pT₃a of cT₁ renal cell carcinoma, and to establish a nomogram prediction model to improve the ability of predicting locally advanced renal cell carcinoma and provide a reference for clinical surgical decision-making. Methods: Clinical data of 1 376 patients with cT₁ (diameter ≤ 7 cm) renal tumor hospitalized for surgery from January 2010 to December 2016 were retrospectively analyzed. There were 979 males and 397 females, with the mean age of (57.65±10.92) years. The mean body mass index (BMI) was (25.47±3.27) kg/m² and the average tumor size was (4.02±1.52) cm. There were 711 tumors on the left and 665 on the right. There were 363 cases with clinical symptoms, 567 patients with smoking history , 732 cases with history of chronic disease. There were 289 cases with tumor necrosis, 636 cases with tumor protrusion, 822 cases with irregular tumor , and 738 cases with renal sinus compression. Partial nephrectomy and radical nephrectomy were performed in 396 cases and 980 cases respectively. Mann-whitney U test and chi-square test were used for univariate analysis, logistic regression analysis was used for multivariate analysis to analyze the predictors of upgrading, R software was used to construct the nomogram predictive model, C-index was used to evaluate the model discrimination, and calibration curve method was used to evaluate the consistency of the model. Results: Postoperative pathology of total 1 376 cases showed that there were 1 195 cases of clear cell carcinoma of kidney, 48 cases of papillary cell carcinoma, 57 cases of chromophobe cell carcinoma, and 76 cases of other types. Among the 1 376 patients with cT₁ renal tumor, 75 patients were upgraded to pT_3a, accounting for 5.5% of all patients. Univariate analysis showed that the patients who upgraded to pT_3a were older [(63.08±10.17) years old and (57.34±10.88) years old], and the tumor length and diameter were larger [(5.24±1.35) cm and (3.95±1.51) cm]. Patients with clinical symptoms [46.7% (35/75) vs. 25.2%(328/1 301)], patients with CT indication of tumor necrosis [40.0%(30/25 975) vs. 19.9% (259/1 301)], patients with irregular tumor contour [73.3%(55/76 775) vs. 59.0%(767/1 301)], and patients with radical nephrectomy were higher [(92.1% (70/91 075) vs. 70%(910/1 301)]. All the differences were statistically significant (P<0.01). Multivariate analysis showed that the independent predictors of upgrading were age (OR=1.046, P<0.001), larger tumor (OR=1.504, P<0.001), clinically symptom (OR=2.153, P=0.004), irregular tumor profile (OR=2.466, P=0.002), and tumor necrosis on CT (OR=2.588, P<0.001). The C-index was 0.808, the calibration curve of forecasting curve with the standard curve fit was good, and the prediction of renal cancer are better in predict consistency. Conclusions Based on the five preoperative predictors, including age, tumor size, clinical presence or absence of symptoms, tumor profile, and whether or not the tumor necrosis indicated by CT, this study developed a nomogram of cT₁ renal cancer upgrade to pT_3a. This nomogram has a good statistical significance, and this model can provide prognosis consultation for patients and provide reference for doctors to make decisions before treatment.

Objective To investigate the clinical pathologic characteristics of extranodal follicular dendritic cell sarcoma ( FDCS ). Methods We collected 7 cases of extranodal FDCS, HE staining, immunohistochemical study were performed. The V600E mutation of BRAF in 7 cases were detected by real?time PCR and EBER in situ hybridization was performed on 4 cases. Results Among the 7 cases of FDCS, 5 cases were male and 2 cases were female, the median age was 55 years old, including 4 cases of low?grade FDCS and 3 cases of high?grade FDCS. The tumor location of 2 cases was in mediastinum, the tumor locations of others were in nasopharynx, kidney, lung, rectum and liver, respectively. The results of immunohistochemistry showed that, the tumor cells were diffusely or focally positive for CD21, CD23, CD35, D2?40, EGFR and CXCL13, but negative for S?100, CD68, HMB45, SMA, Desmin, CD117, Dog?1, CD34, CD30, EMA and CK.Five cases were positive for PD?L1 and the its expression in high?grade FDCS were higher than that in low?grade FDCS.Two cases of low?grade FDCS were positive for BRAF V600E, but the BRAF V600E mutation weren′t detected in all of 7 cases. The result of EBER in?situ hybridization showed that only the nasopharynx FDCS was positive.The follow?up information of 5 patients were available (7～43 months), 4 patients died and 1 still alive with rectum metastasis.Conclusions FDCS is a rare malignant disease with relapse and metastatic tendency. The combined applications of the first?line antibodies including CD21, CD23, CD35 and second?line antibodies including D2?40, CXCL13, EGFR are helpful for its diagnosis and differential diagnosis. The high expression of PD?L1 implicates the potential benefit of FDCS patients acquired from immunotherapy.

Objective To investigate the clinical pathologic characteristics of extranodal follicular dendritic cell sarcoma ( FDCS ). Methods We collected 7 cases of extranodal FDCS, HE staining, immunohistochemical study were performed. The V600E mutation of BRAF in 7 cases were detected by real?time PCR and EBER in situ hybridization was performed on 4 cases. Results Among the 7 cases of FDCS, 5 cases were male and 2 cases were female, the median age was 55 years old, including 4 cases of low?grade FDCS and 3 cases of high?grade FDCS. The tumor location of 2 cases was in mediastinum, the tumor locations of others were in nasopharynx, kidney, lung, rectum and liver, respectively. The results of immunohistochemistry showed that, the tumor cells were diffusely or focally positive for CD21, CD23, CD35, D2?40, EGFR and CXCL13, but negative for S?100, CD68, HMB45, SMA, Desmin, CD117, Dog?1, CD34, CD30, EMA and CK.Five cases were positive for PD?L1 and the its expression in high?grade FDCS were higher than that in low?grade FDCS.Two cases of low?grade FDCS were positive for BRAF V600E, but the BRAF V600E mutation weren′t detected in all of 7 cases. The result of EBER in?situ hybridization showed that only the nasopharynx FDCS was positive.The follow?up information of 5 patients were available (7～43 months), 4 patients died and 1 still alive with rectum metastasis.Conclusions FDCS is a rare malignant disease with relapse and metastatic tendency. The combined applications of the first?line antibodies including CD21, CD23, CD35 and second?line antibodies including D2?40, CXCL13, EGFR are helpful for its diagnosis and differential diagnosis. The high expression of PD?L1 implicates the potential benefit of FDCS patients acquired from immunotherapy.

Objective To investigate the clinical value of liquid-based cytology and cell paraffin blocks combined with immu-nocytochemical examination in cytopathologic diagnosis of hydrothorax and ascites.Methods One hundred and fourteen cases of hydrothorax and ascites sample were collected.The detection positive rates were compared between the liquid-based cytology and cell paraffin blocks combined with immunocyochemical examination.The suspected positive and positive cases were performed the immunocytochemical examination for further judging benign and malignancy and histological source.Results 114 cases of hydro-thorax and ascites samples,36 cases(31.58%)of positive were diagnosed by liquid-based cytologyamong and 55 cases(48.24%)of positive were diagnosed by cell paraffin blocks combined with immunocytochemical examination,the difference was statistically sig-nificant(P<0.01).There were 55 cases of malignant hydrothorax and ascites,45 cases were hydrothorax and 10 cases were ascites, in hydrothorax,there were 32 cases of lung adenocarcinoma,3 cases of small cell lung cancer,4 cases of breast cancer,1 case of en-dometrial carcinoma,1 case of lymphatic hematopoietic system tumor and 4 cases of unknown origin;in ascites,there were 2 cases of ovarian cancer,1 case of digestive system tumor,1 case of endometrial cancer,1 case of lymphatic hematopoietic system tumor and 5 cases of unknown origin.Conclusion The cell paraffin blocks combined with immunocytochemistry can significantly improve the positive detection rate of hydrothorax and ascites,and helps to judge the source of tissue.

Objective: To investigate clinic-pathological characteristics, diagnosis, treatment and prognosis of intravascular large B cell lymphoma (IVLBCL) in China. Methods: Clinical and pathological records were analyzed from 12 IVLBCL patients diagnosed between Jan 2010 to Jun 2016. Kaplan-Meier method was used to estimate overall survival (OS), and univariate analysis was performed to identify prognostic factors. Results: A series of 12 patients with IVLBCL (median age, 53.8 years; range, 32-76 years; 6 males and 6 females) was reviewed. Fever was the most common symptom (10/12), respiratory symptoms (cough, pleural effusion, dyspnea, 50%) and hemophagocytic lymphohistiocytosis (50%) were frequently observed, and only 12 patients had neurological symptom. All patients had elevated lactic dehydrogenase and serum ferritin. International Prognostic Index score was high in 75% of total patients. All patients had extra-nodal involved, pulmonary (6/12) and bone marrow (4/12) were frequently involved. Large lymphoid cells within vessel lumina or sinuses were observed in all patients. These cells were large, with scant cytoplasm, vesicular nuclei, and one or more nucleoli, and the structures of vessels and sinus were reserved. CD20 and CD79a were positive in all cases. 11patients received rituximab combined CHOP regimen chemotherapies, overall response rate (ORR) was 90.1%, and complete response rate was 66.7%. Median survival time and median progression time were not reached after a median follow-up of 20 months. Univariate analysis revealed that no clinical characters were associated with OS. Conclusion As a rare variant of DLBCL, IVLBCL presented with pulmonary involved frequently, and trans-bronchial lung biopsy had good positive rates. Rituximab contained chemotherapy was the backbone for IVLBCL.

Objective To investigate the efficacy of preemptive analgesiawith ultrasound-guided modified dorsal penile nerve block combined ketorolac tromethamine for circumcision in pediatric patients.Methods Forty-five pediatric patients with ASA grade Ⅰ undergoing elective circumcision were randomly divided into 3 groups (n=15 each).The same intravenous compound anesthesia was used in the three groups.The pediatric patients underwent ultrasound-guided modified dorsal penile nerve block with a mixture of 0.1 ml/kg of 0.2% ropivacaine and 0.8% lidocaine before operation in group A and group B.The pediatric patients were given ketorolac tromethamine 1 mg/kg intramuscular injection before operation in group A and group C.The total consumption of propfol and sufentanil, occurrence of intraoperative body movement and respiratory depression, emergence time, time from waking up to going out of PACU and adverse reactions such as postoperative agitation, nausea, vomiting and pruritus were aslo recorded.The requirement for postoperative paracetamol suppositories was recorded.Results Compared with group C, the total consumption of propfol and sufentanil were significantly decreased, incidence of body movement andrespiratory depression were significantly decreased, the emergence time and time from waking up to going out of PACU was significantly shortened, requirement for paracetamol suppositories were significantly decreased in group A and group B(P<0.05).Compared with group B, incidence of requirement for paracetamol suppositories was significantly decreased in group A (P<0.05).No pediatric patients developed postoperative nausea, vomiting, pruritus and incidence of emergence agitation had no statistical difference in the three groups.Conclusion Preemptive analgesia with ultrasound-guided modified dorsal penile nerve block combined ketorolac tromethamine is safe and effective when used for circumcision in pediatric patients, and it has good efficacy of postoperative analgesia.