During the past decate, Chinese pathologists have made remarkable achievements in the area of soft tissue tumors. They have not only done in-depth researches in selected entities like liposarcoma and round cell sarcomas, but have also issued expert consenses and guideline, as well as published professional books and translation books, with purpose to comprehensively improve the level of diagnosis nationwide.

During the past decate, Chinese pathologists have made remarkable achievements in the area of soft tissue tumors. They have not only done in-depth researches in selected entities like liposarcoma and round cell sarcomas, but have also issued expert consenses and guideline, as well as published professional books and translation books, with purpose to comprehensively improve the level of diagnosis nationwide.

Objective To investigate the characteristics of spatial orientation language comprehension and expression behavior ofchildren with autism. MethodsA three-ladder test was designed to test the comprehension and expression of "(go/come) to" sentences, including general subject-predicate sentences, conjunctive-predicate sentences with displacement purposes, and conjunctive-predicate sentences with displacement modes. March to June, 2022, the test was conducted on 17 children with autism from a special education school and 17 healthy children matched the scores of Peabody Picture Vocabulary Test-Chinese revised from a kindergarten. The typical errors were analyzed for children with autism. ResultsThe main effects of sentence ladders (F > 3.718, P < 0.05) and children groups (F > 8.782, P < 0.001) were significant on comprehension and expression of sentences, while the performance was poor for the complex sentences and for the children with autism. The common types of errors in expression were missing components, mixed sentences, autonomous sentences, inaccurate wording, inconsistent content, blending errors and non-response. The numbers and the distribution of error types were quite different from the children with autism to the healthy children. ConclusionChildren with autism are more difficult to understand and express "(go/come) to" sentences, mainly manifested in a higher error frequency and more kinds of error.

Due to long-term use of immunosuppressant, poor immune function and a higher risk of critical diseases after novel coronavirus pneumonia in kidney transplant recipients, it is of significance to deliver prophylactic vaccination for this high-risk population. Studies have shown that the immune reaction of kidney transplant recipients to novel coronavirus vaccine is significantly lower than that of healthy counterparts. Standard vaccination program in the United States, such as 2 doses of messenger RNA (mRNA) vaccine, fails to provide sufficient protection for kidney transplant recipients. Many studies have proven that increasing the frequency of vaccination for kidney transplant recipients may enhance the vaccine efficacy. Nevertheless, the role of adjusting immunosuppressive therapy in increasing vaccine efficacy remains to be elucidated. In this article, the importance, effectiveness and particularity of novel coronavirus vaccine for kidney transplant recipients and the effect of immunosuppressive therapy on the efficacy of novel coronavirus vaccine were reviewed, aiming to provide reference on the vaccination for kidney transplant recipients.

Objective:To determine the long-term efficacy and safety of intra-cervical lymphatic immunotherapy (ICLIT) for adult allergic rhinitis (AR) by comparing it with subcutaneous immunotherapy (SCIT).Methods:A total of 100 adult AR patients with dust mite allergy in Department of Otorhinolaryngology, First People′s Hospital of Foshan from Feb 2018 to Dec 2019 were randomly divided into two groups, 50 in SCIT group [including 42 males and 8 females, aging (32.55±9.72) years] and 50 in ICLIT group [including 45 males and 5 females, aging (31.33±9.84) years]. The changes in total symptom score (total system score, TSS), nasal symptom score (total nasal symptom score, TNSS), eye symptom score (total ocular scoring system, TOSS), drug score (total medication score, TMS), and quality of life score of the two groups of patients were evaluated before and after treatment, and the adverse reactions of all patients during the treatment period were recorded. The changes in the level of dust mite specific IgE (sIgE) in the serum were evaluated. GraphPad Prism 9.0 software was used for statistical analysis.Results:In the SCIT group, 38 patients completed treatment and follow-up, with a dropout rate of 24%. In the ICLIT group, 48 patients completed treatment and follow-up, with a dropout rate of only 4%. The scores of TSS, TNSS, TOSS, TMS, and quality of life in the ICLIT group before treatment were 32.1±3.0, 27.3±3.1, 4.8±2.8, 2.3±0.9, and 68.1±28.7, respectively; After 36 months of treatment, the scores were 21.8±11.4, 18.1±9.4, 3.7±2.9, 1.3±1.1, and 36.0±26.7, respectively, which were significantly lower than those before treatment (all P<0.001). After 36 months of treatment, the TSS of the ICLIT group improved by 10.3±11.2 compared to before, while the TSS of the SCIT group improved significantly by 21.9±11.0 compared to before, with statistically significant differences between the groups ( P<0.001). No serious systemic adverse reactions occurred in both groups of patients. Conclusions:ICLIT treatment for adult AR has long-term efficacy, high safety, and high compliance, but its long-term efficacy is not as good as SCIT. ICLIT can be considered as a new complementary option for AR immunotherapy.

Objective:This study aims to explore the causal relationship between obstructive sleep apnea (OSA) and type 2 diabetes (T2D) using bidirectional Mendelian randomization (MR).Methods:The genetic data related to OSA were obtained from the FinnGen Biobank (Ncase=16, 761, Ncontrol=201, 194) in the Genome-wide association study (GWAS). Three single nucleotide polymorphism (SNP) were screened out as instrumental variable (IV) of OSA. The genetic data related to T2D were derived from a large Meta-analysis of GWAS (Ncase=62, 892, Ncontrol=596, 424), 114 SNP were selected as IV of T2D. Multiple MR methods were used for analysis and inverse variance weighted (IVW) was performed as main method. The sensitivity of MR analytic results was analyzed using MR-Egger and other methods, and the IV was evaluated using F-value statistics. Results:MR analysis showed that OSA was significantly associated with increased risk of T2D ( OR=2.016, 95% CI: 1.185-3.429, P<0.05). There was no significant relationship between T2D and OSA risk ( OR=1.030, 95% CI: 0.980-1.082, P=0.238). There was heterogeneity in both-way results (OSA?T2D, P=1.808×10 -11; T2D?OSA, P=1.729×10 -7), and no horizontal pleiotropy (OSA?T2D, P=0.477; T2D?OSA, P=0.349). IV of OSA and T2D-selected in the study were strong instrumental variables ( F statistics of OSA=20.543; F statistics of T2D=30.117). Conclusion:Our results supported that OSA was a risk factor for T2D, but T2D had no significant impact on the incidence of OSA. Blood glucose monitoring and diabetes screening in OSA patients might be beneficial to the early detection and intervention of T2D.

ObjectiveTo analyze the typical performance of initiating and responding behaviors of turn-taking in operational games for autistic children with low language function in special education schools and to provide a reference for intervention of turn-taking behaviors in operational games. MethodsFrom November, 2021 to January, 2022, a total of 23 autistic children with low language function (language ability ≤ three years old) in Shanghai Putuo District Qixing School were selected. Their linguistic ability was evaluated. A behavioral assessment approach was used to evaluate the behavior of initiating and responding behaviors of turn-taking in three operational games. The typical errors in initiating behaviors were summarized as difficult to initiate, untimely initiation, no response and abnormal initiation. The typical errors in responding behaviors of turn-taking in operational games were summarized as difficult to respond, untimely response, no response and abnormal response. ResultsThere was no significant differences in the performance of initiating behaviors among three types of operational games (χ² = 11.106, P = 0.196), and there were significant differences in the performance of responding behaviors among operational games (χ² = 26.256, P = 0.001). The initiating behaviors were postively correlated with word comprehension (r = 0.420, P < 0.05), word naming (r = 0.510, P < 0.05), and sentence imitation (r = 0.505, P < 0.05). The responding behaviors were postively correlated with word comprehension (r = 0.546, P < 0.01), word naming (r = 0.728, P < 0.01), sentence comprehension (r = 0.668, P < 0.01) and sentence imitation (r = 0.656, P < 0.01). ConclusionAutistic children with low language function showed different typical behaviors of initiating and responding behaviors of turn-taking in operational games. It is suggested that when designing training programs for turn-taking skills, targeted interventions should be made to address the typical types of errors in response and initiation turns, and individualized intervention programs should be designed to enhance children's communicative efficacy in play game and promote their language development and social participation.

Objective:To investigate the clinicopathological diagnosis and differential diagnosis of inflammatory myofibroblastic tumor (IMT).Methods:Thirty-two cases of IMT collected at the People′s Hospital of Jiangsu Province from May 2010 to May 2020 were evaluated for their clinical, histologic, immunohistochemical and genomic features, and relevant literature was reviewed.Results:There were 19 male and 13 female patients, with age ranging from 5 to 65 years (mean, 37 years). The tumors were located in the lung and mediastinum (10 cases), gastrointestinal tract and mesentery/omentum (12 cases), urinary bladder (5 cases), head and neck (3 cases), somatic soft tissue (1 case), and retroperitoneum (1 case). Four cases of epithelioid inflammatory myofibroblastic sarcoma (EIMS) were all located intra-abdominally. Histologically, the tumor cells were myofibroblasts and fibroblasts arranged in predominantly fusiform pattern, with variably edematous to myxoid background or sclerotic collagenized stroma, and variably mixed chronic or acute inflammatory cells infiltration. EIMS were composed mainly of epithelioid tumor cells, with myxoid stroma and numerous neutrophils. Immunohistochemically, the tumor cells expressed cytoplasmic ALK (25/32, 78%), whereas the four EIMS showed nuclear membrane ALK staining pattern. The tumor cells also expressed CKpan (8/19), SMA (24/32, 75%) and desmin (12/32, 38%); all four EIMS also showed strong positivity for desmin. Fluorescence in situ hybridization (FISH) for ALK gene rearrangement showed split apart signals in 12 of 15 cases, most commonly with atypical signals. Next-generation sequencing (NGS) was performed in three tumors and showed that one case of lower leg IMT harbored a novel CLIP2-ALK fusion, and two cases of EIMS harbored RANBP2-ALK fusion. Follow-up data were available in 29 patients. Twenty-two patients were alive with no evidence of tumor, four patients had tumor recurrences (three patients were treated with crizotinib and were alive with tumor), and three patients died of the disease (including two patients with EIMS).Conclusions:IMTs show a wide morphologic spectrum, and should be differentiated form a variety of benign or malignant tumors. Immunohistochemistry (ALKp80, ALKD5F3) and FISH (ALK break-apart probe) could assist the diagnosis of IMT, with NGS recommended for the atypical cases.

Objective:To investigate the clinicopathological and molecular features, diagnosis and differential diagnosis of TFE3-rearranged epithelioid hemangioendothelioma (EHE). Methods Two cases of TFE3-rearranged EHE arising from soft tissues, diagnosed by the Pathology Department of the First Affiliated Hospital of Nanjing Medical University from 2013 to 2020 were observed. EnVision method was used for immunophenotyping, fluorescence in situ hybridization (FISH) was used to test TFE3 gene rearrangements and WWTR1-CAMTA1 fusion gene,and next-generation sequencing (NGS) was used to delineate the fusion transcripts.Results:Details of these two cases were as follows: case 1, male, 51 years old, with tumor in the right temporal region; case 2, female, 42 years old, with tumor in the right neck. The tumors showed progressive painless enlargement. Grossly, the tumor of case 1 was multinodular with unclear boundary and grayish red cut surface, while the tumor of case 2, originating from a vein, appeared as a firm, tan mass within vessel wall. Microscopically, both tumors showed moderate cellularity and were consisted of plump, epithelioid, or histiocytoid cells with eosinophilic cytoplasm and mild-to-moderate nuclear pleomorphism. Most of the tumor cells were arranged in solid or alveolar growth patterns, while some tumor cells showed intraluminal papillary growth pattern in case 1 and anastomosing vascular channels and extramedullary hematopoiesis in case 2. Immunohistochemically, the tumor cells showed diffuse positivity for CD31, CD34, ERG, and TFE3. FISH revealed TFE3 break-apart signals in two cases, but WWTR1-CAMTA1 gene fusion was not detected. NGS identified YAP1 (exon1)-TFE3 (exon6) fusion gene in case 2. Clinical follow-up information was available in both cases for a follow-up period of 15 and 59 months respectively. Patient 1 had a relapse 22 months after surgery, and was currently alive with the tumor. Patient 2 remained disease-free.Conclusions:TFE3-rearranged EHE is a rare molecular subtype of EHE, with accompanying characteristic morphologic features. However the morphologic spectrum remains under-recognized, and more experience is needed. Immunohistochemical and molecular examinations are helpful for the diagnosis and differential diagnosis of the disease.

Objective:To investigate the clinicopathological characteristics, genetic features, diagnosis and differential diagnosis of pulmonary artery intimal sarcoma (PAIS).Methods:Three cases of PAIS were collected from Jiangsu Province People′s Hospital (from February 2016 to November 2019). The clinical data, imaging examination, morphology, immunostaining, and molecular changes were retrospectively analyzed.Results:There were 1 male and 2 females (age: 32, 50, 60 years), who had symptoms of cough, asthma or chest tightness. Imaging findings indicated low density filling defects which were suspected as thrombus, embolism or myxoma. Grossly, the main tumor was located in the elastic arteries and their lobar branches, also extended into the atrium and ventricle, with lung parenchymal infiltration focally. Microscopically, tumor cells were predominantly composed of abundant spindle cells with obvious atypia and myxoid background, resembling fibroblastic or myofibroblastic differentiation. Active mitotic figures and necrosis could be seen in some areas. Immunohistochemical staining of vimentin was strongly positive, while pan-cytokeratin, S-100, desmin, Fli-1, CD31, SMA and ERG etc were variably positive only in focal areas. FISH detection showed amplification of MDM2 gene in three cases and EGFR gene in two cases. Metastatic lesions were found in one case by 18, 32 and 42 months after surgery respectively. There was no recurrence or metastasis in the other two cases.Conclusions:PAIS is one of exceptionally poor differentiated mesenchymal tumor that arises from the arterial intima of elastic pulmonary arteries. There was no definite differention in morphology. Gene detection shows amplification of MDM2 and EGFR gene. This tumor often has poor prognosis with aggressive behavior. Complete resection is the only effective therapeutic option. There is disagreement as to whether chemotherapy and radiotherapy can improve survival.