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.

Over the past decade, the field of bone tumor pathology in China has made remarkable progress. These achievements are reflected not only in the innovation and standardization of diagnostic techniques, which have significantly improved diagnostic accuracy, but also in the in-depth exploration of tumor pathogenesis and the continuous refinement of treatment protocols. More than one hundred research papers on bone tumor pathology published in the Chinese Journal of Pathology stand as a testament to the relentless efforts and practical contributions of Chinese pathologists in this field. On the occasion of the Chinese Journal of Pathology′s 70th anniversary, we summarize the progress in bone tumor pathology while also looking forward to the future, aiming to promote greater advancements in this field.

A 66-year-old male patient with esophageal cancer progressed after concurrent radiotherapy and chemoradiotherapy, and received immunotherapy combined with chemotherapy (intravenous infusion of sintilimab 200 mg on day 1, tegafur gimeracil oteracil potassium 50 mg orally twice daily from day 1 to 14), with 21 days as a cycle. After 13 days of treatments, diarrhea appeared, and gradually worsened with abdominal pain and a small amount of mucous bloody stools. Colonoscopy showed diffuse swelling and punctiform hemorrhagic erosion of the mucosa of the colon and rectum. Histopathological examination of the ascending colon and sigmoid colon showed regular arrangement of glands, infiltration of lymphocytes, neutrophils and eosinophils in the interstitium, and formation of crypt abscesses, which were considered to be immune-associated colitis caused by sintilimab. After discontinuing the use of sintilimab and administering low-dose methylprednisolone (24 mg orally twice daily) for 3 days, the patient′s symptoms were completely relieved. The dose of methylprednisolone was reduced gradually and discontinued within 4 weeks. The patient did not rechallenge immune checkpoint inhibitors and continued maintenance therapy with tegafur gimeracil oteracil potassium, and no diarrhea recurred

A 66-year-old male patient with esophageal cancer progressed after concurrent radiotherapy and chemoradiotherapy, and received immunotherapy combined with chemotherapy (intravenous infusion of sintilimab 200 mg on day 1, tegafur gimeracil oteracil potassium 50 mg orally twice daily from day 1 to 14), with 21 days as a cycle. After 13 days of treatments, diarrhea appeared, and gradually worsened with abdominal pain and a small amount of mucous bloody stools. Colonoscopy showed diffuse swelling and punctiform hemorrhagic erosion of the mucosa of the colon and rectum. Histopathological examination of the ascending colon and sigmoid colon showed regular arrangement of glands, infiltration of lymphocytes, neutrophils and eosinophils in the interstitium, and formation of crypt abscesses, which were considered to be immune-associated colitis caused by sintilimab. After discontinuing the use of sintilimab and administering low-dose methylprednisolone (24 mg orally twice daily) for 3 days, the patient′s symptoms were completely relieved. The dose of methylprednisolone was reduced gradually and discontinued within 4 weeks. The patient did not rechallenge immune checkpoint inhibitors and continued maintenance therapy with tegafur gimeracil oteracil potassium, and no diarrhea recurred

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.

Over the past decade, the field of bone tumor pathology in China has made remarkable progress. These achievements are reflected not only in the innovation and standardization of diagnostic techniques, which have significantly improved diagnostic accuracy, but also in the in-depth exploration of tumor pathogenesis and the continuous refinement of treatment protocols. More than one hundred research papers on bone tumor pathology published in the Chinese Journal of Pathology stand as a testament to the relentless efforts and practical contributions of Chinese pathologists in this field. On the occasion of the Chinese Journal of Pathology′s 70th anniversary, we summarize the progress in bone tumor pathology while also looking forward to the future, aiming to promote greater advancements in this field.

Objective:To investigate the radiologic, pathologic, and molecular features of simple bone cysts (SBC), and their differential diagnoses.Methods:Fourteen cases of SBC were collected at the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from 2017 to 2022, and fluorescence in situ hybridization (FISH) was performed for retrospective analysis.Results:There were 14 patients, including 7 females and 7 males, with age range of 7 to 45 (median 29) years. The most common complaint was pain, including 4 cases with pathological fracture and 5 with history of previous trauma. The tumor size ranged from 3.4 to 13.5 (median 5.6) cm. The lesion involved the femur ( n=4), humerus ( n=5) and iliac bone ( n=5). Radiologic diagnoses included SBC, aneurysmal bone cyst, and giant cell tumor of the bone or its combination with aneurysmal bone cyst-like region and fibrous dysplasia. Histologically, the cyst walls of the lesions were composed of fibrous tissue, fibrin-like collagen deposits, bone-like matrix and occasional woven bone. The lesional cells were spindled to ovoid, with scattered osteoclast-like giant cells, foamy histiocytes, hemosiderin deposits and cholesterol clefts. In 6 cases there were nodular fasciitis-like areas. Immunohistochemically, the spindled to ovoid cells were positive for SMA, EMA and SATB2 in varying degrees. FISH detection was performed in all 14 cases and EWSR1/FUS rearrangement were found in 9 cases. One case of FUS::NFATC2 fusion was detected by next-generation sequencing. Nine cases of SBC with the rearrangement were more cellular, and there were more mitotic figures in the recurrent FUS::NFATC2 fusion tumor. Clinical follow-up was obtained in all 14 cases with the time ranging from 5 to 105 (mean 46) months. Amongst them, the tumor with FUS::NFATC2 rearrangement had local recurrence twice after the first local excision, but had no more recurrence or metastasis 34 months after the subsequent segmental resection. The other 13 cases had no recurrence. Conclusions:EWSR1 or FUS rearrangement is most commonly identified in SBC, suggesting that SBC might be a neoplastic disease. In cases where the radiologic appearance and histomorphology are difficult to differentiate from aneurysmal bone cyst, FISH detection can aid in the definitive diagnosis.

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.

Objective:To investigate the correlation between protein C -1641A/-1654C haplotype and coagulation disorder in Chinese Han septic patients.Methods:The genotypes of protein C gene -1641A>G (rs1799809) and -1654C>T (RS1799808) in septic patients were detected by direct sequencing, and their haplotypes were analyzed and divided into two groups according to the haplotype, -1641A/-1654C (AC) carriers and non-AC haplotype carriers. At the same time, unpaired t test or Mann-Whitney U test was used to compare the differences in coagulation/fibrinolytic parameters, including partial activated thrombin time, prothrombin time, internationally standardized ratio of prothrombin time, thrombin time, fibrinogen and D-dimer levels, as well as APC levels between the two groups. Results:A total of 174 septic patients were included in this study, including 60 AC haplotype carriers and 114 non-AC haplotype carriers. Compared with non-AC haplotype carriers, AC haplotype carriers had significantly lower platelet counts, significantly longer partial activated thrombin time, and significantly decreased activated protein C levels. Other coagulation/fibrinolytic parameters including prothrombin time, internationally standardized ratio of prothrombin time, thrombin time, fibrinogen and D-dimer were not significantly different between the two groups.Conclusions:In this study, the protein C-1641A/-1654C haplotype was found to lead to decreased circulating activated protein C levels decreased platelet counts, and prolonged partial activated thrombin time in septic patients. These results suggest that the protein C-1641A/-1654C haplotype may directly affect the APC level and consequently influence the coagulation disorder of sepsis.

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.