Objective:To investigate the clinicopathological features, diagnosis and differential diagnosis of adrenal cortical carcinoma (ACC) in children.Methods:Twenty-five children with ACC diagnosed in the Department of Pathology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China from March 2014 to August 2022 were retrospectively analyzed. The related literature was reviewed.Results:A total of 25 children with ACC were collected, including 11 males and 14 females, with a male to female ratio of 1.0∶1.3. The patient ages ranged from 8 months to 14 years (median, 4 years). Eighteen cases with clinical data had functional tumors (18/22, 81.8%) presenting with virilization or precocious puberty (15/18), symptoms related to hypercortisolism (8/18) or endocrine symptoms mixed with both (5/18), while 3 cases (3/22, 13.6%) had unknown clinical data. The clinical manifestations of four patients with nonfunctional tumors were an abdominal mass and/or abdominal pain, walking instability and others. Grossly, the average maximum diameter of the tumor was 9.4 cm. Most of the tumors were nodular and partially encapsuled. The cut surfaces were gray or gray brown, soft with hemorrhage. Histologically, the tumor cells were diffusely distributed, separated by a vascular-rich network. The tumor cells were large, with distinct nucleoli, abundant eosinophilic or clear cytoplasm, and round or oval nuclei. The mitotic index was high, and atypical mitoses were common. Necrosis, calcification, capsule invasion or/and venous invasion were present. In some cases, the tumor invaded the surrounding soft tissues or kidneys. Immunohistochemically, the tumor cells were diffusely positive for syn and SF1 and focally positive for α-inhibin, Melan A and Calretinin, but negative for CgA. Ki-67 proliferation index ranged from 2%-90%. TP53 gene status was examined in 7 cases, in which mutations were detected in 4 cases. Follow-up data was obtained in 21 patients, among whom 18 received chemotherapy and 3 received radiotherapy. Distant metastasis occurred in 13 patients. Median progression-free survival (PFS) was 11.2 months and median overall survival (OS) was 54.7 months. Patients aged less than 5 years had a better prognosis for OS ( P<0.05) than the older ones (≥5 years), but a similar PFS ( P>0.05). Male patients and Ki-67 proliferation index <15% had a better prognosis tendency for OS, but there was no statistically significant difference ( P>0.05). Conclusions:ACC in children is a rare, often functional tumor associated with Li-Fraumeni genetic syndrome and has a poor prognosis. Diagnosis and differential diagnosis require a combination of morphological, phenotypic and clinical analysis.

Purpose To investigate the clinicopathological features and molecular features of diffuse paediatric-type high-grade glioma,H3-wildtype and IDH-wildtype(pHGG H3/IDH WT)of central nervous system.Methods The clinical and pathological data of 6 cases of pHGG H3/IDH WT diagnosed by Department of Pathology,Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine were retrospectively ana-lyzed.The expression of GFAP,Olig2,Syn,NeuN,IDH1,H3K27M was detected by immunohistochemistry(automatic im-munohistochemical staining device).The EGFR and MYCN gene amplification was detected by FISH.IDH,H3F3A and TERT gene mutations were detected by Sanger sequencing.The literatures were reviewed.Results The 6 patient's age ranged from 5 to 11 years,with a median age of 7.5 years.Among them,there were 2 males and 4 females,with a male to female ratio of 1∶2.The clinical symptoms were limb weakness,hemi-plegia,vomiting,convulsions,blurred vision and so on.Tumors were located in supratentorial brain for 5 cases and one in brain stem and cerebellum.Histologically,3 cases showed the mor-phological features of high-grade glioma,2 of which with giant cells.Two cases showed embryonal tumor-like features,and one had both high-grade glioma and embryonal tumor-like morpho-logical features.Microvascular proliferation and/or necrosis were present in 5 cases.Myxoid/microcystic stroma was found in 1 case.By immunohistochemistrically,the tumor cells were par-tially or focally positive for GFAP(6/6)and Olig2(6/6),fo-cally positive for Syn(3/6)and NeuN(1/6),and negative for IDH1,H3K27M,H3G34V and H3G34R.ATRX,H3K27me3,INI1 and BRG1 were diffusely positive(6/6).The positive rate of p53 was 5％-95％,and Ki67 proliferation index was 40％-90％.Molecular analysis showed that all 6 cases were IDH1/2 and H3F3A wild-type.MYCN amplification was observed in 2 cases.Two cases of EGFR amplification with polyploidy;one case had both EGFR amplification and MYCN amplification.PDGFRA amplification was observed in one case.For treatment and follow-up,the patients received postoperative radiotherapy and/or temozolomide chemotherapy;three patients died at 1 to 5 months after operation.Two patients survived and were followed up for 4 and 7 months,respectively.One patient was lost to fol-low-up.Conclusions pHGG H3/IDH WT is a highly malignant tumor with glioblastoma-like or embryonal tumor-like features.According to the molecular characteristics,it can be divided into three molecular subtypes,RTK1,RTK2 and MYCN.pHGG MYCN has the worst prognosis.Attention should be paid to the differential diagnosis of other pediatric or adult high-grade glio-mas and embryonal tumors.

Purpose To investigate the clinicopathological features,molecular features,treatment and prognosis of infant-type hemispheric glioma(IHG)with ALK gene fusion of central nervous system.Methods Clinical data of 2 cases of IHG were collected,and the expression of GFAP,Olig2,Syn,NeuN,H3K27M,H3K27me3,H3G34V,H3G34R,ALK,and BRAF were detected using immunohistochemistry.FISH and NGS method was used to detect ALK fusion genes,and relevant liter-atures were reviewed.Results Case 1,a male,15 months old,showed a huge mass in the left frontal and temporal lobe on cranial MRI.Case 2(external hospital consultation),a male,18 months old,was found to have a space occupying lesion in the frontaltemporal lobe.Microscopic examination:in case 1,tumor cells showed diffuse patchy pattern,with small nodular shapes in some areas which separated by fibrous blood vessels.The cell density was high,and the nucleus was round or oval.The mitotic was easy to see,accompanied by bleeding and nec-rosis.In case 2,tumor cells were diffusely distributed,some of them formed pseudorosette around blood vessels and others ar-ranged in bundles.The cells were mild to moderate dysplasia with round or oval nuclei,light staining,small nucleoli,rare mitotic figures,and no microvascular proliferation or necrosis.Immunophenotypes:in case 1 tumor cells were focal positive for GFAP and NeuN,diffuse positive for H3K27me3,ALK(cyto-plasm and cell membrane),and ATRX,p53 positive(30％),negative for Olig2,Syn,H3K27M,H3G34V,H3G34R,and BRAF,Ki67 proliferation index of 20％.In case 2:tumor cells were diffusedly expressed GFAP,ATRX,H3K27me3,ALK(cytoplasm and cell membrane),and Olig2,Syn was weakly positive,NeuN and p53 were negative,and Ki67 proliferation index was 5％.FISH and NGS tests showed ALK gene fusion in both cases,with EML4::ALK fusion in case 1 and LRRFIP1::ALK fusion in case 2.Follow up for 7 months showed that the residual lesion in case 1 had shrunk and was generally in good condition;case 2 lost follow-up.Conclusion The histology of IHG often presents as high-grade gliomas or accompanied by an-aplastic features.The heterogeneity of ALK variants can also manifest as low-grade gliomas,which need to be differentiated from other high-grade gliomas,desmoplastic infantile gangliogli-oma/astrocytoma,ependymoma,etc.

Objective:To investigate the clinicopathological features of children with metachronous or synchronous primary tumors and to identify related genetic tumor syndromes.Methods:The clinicopathological data of 4 children with multiple primary tumors diagnosed in the Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China from 2011 to 2023 were collected. The histological, immunophenotypic and molecular characteristics were examined using H&E staining, immunohistochemical staining, PCR, Sanger sequencing and next-generation sequencing (NGS). The patients were followed up.Results:Case 1 was an 8-year-old boy with the adrenal cortical carcinoma, and 5 years later a poorly differentiated gastric adenocarcinoma was detected. Case 2 was a 2-year-old boy, presented with a left ventricular choroid plexus carcinoma, and a hepatoblastoma was detected 8 months later. Case 3 was a 9-month-old girl, diagnosed with renal rhabdoid tumor first and intracranial atypical teratoid/rhabdoid tumor (AT/RT) 3 months later. Case 4 was a 7-year-old boy and had a sigmoid colon adenocarcinoma 3 years after the diagnosis of a glioblastoma. The morphology and immunohistochemical features of the metachronous or synchronous primary tumors in the 4 cases were similar to the corresponding symptom-presenting/first-diagnosed tumors. No characteristic germ line mutations were detected in cases 1 and 2 by relevant molecular detection, and the rhabdoid tumor predisposition syndrome was confirmed in case 3 using NGS. Case 4 was clearly related to constitutional mismatch repair deficiency as shown by the molecular testing and clinical features.Conclusions:Childhood multiple primary tumors are a rare disease with histological morphology and immunophenotype similar to the symptom-presenting tumors. They are either sporadic or associated with a genetic (tumor) syndrome. The development of both tumors can occur simultaneously (synchronously) or at different times (metachronously). Early identification of the children associated with genetic tumor syndromes can facilitate routine tumor screening and early treatment.

Objective:To evaluate the efficacy and safety of cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) in the treatment of pseudomyxoma peritonei (PMP).Methods:In this descriptive case series study, we retrospective analyzed the records of PMP patients treated with CRS and HIPEC between January 2013 and June 2023 at Affiliated Cancer Hospital and Institute of Guangzhou Medical University. The inclusion criteria were as follows: (1) Aged 18 to 75 years and nonpregnant women. (2) Histologically confirmed diagnosis of pseudomyxoma peritonei. (3) Karnofsky Performance Scale (KPS)>70. (4) The functions of major organs such as the heart, liver, lungs, and kidneys can tolerate major surgery for long periods of time. (5) No evidence of extra-abdominal metastasis. Patients with extensive intra-abdominal adhesions or severe infectious diseases were excluded. The main outcomes were overall survival (OS) and postoperative major complications. The postoperative major complications were graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events (version 5.0). We used the peritoneal cancer index (PCI) score to quantitatively assess the peritoneal metastases and the completeness of cytoreduction (CCR) score at the end of surgery (CCR-0 and CCR-1 considered to be complete CRS).Results:A total of the 186 PMP patients with a median age of 56 (interquartile range extremes (IQRE), 48-64) years were included, 65 (34.9%) males and 121 (65.1%) females. The median peritoneal cancer index (PCI) score was 28 (20-34). Appendiceal origin accounted for 91.4%. Histological types were low grade in 99 patients (53.2%), high grade in 57 patients (30.6%), and 55 patients (29.6%) received complete cytoreduction (CCR-0/1). The median operative duration was 300 (211-430) minutes for all patients. Treatment-related 30-day mortality was 2.7%; 90-day mortality 4.3%; reoperation 1.6%; and severe morbidity 43.0%. Within the entire series, anemia(27.4%), electrolyte disturbance(11.6%), and hypoalbuminemia(7.5%) were the most frequent major complications (grade 3-4). The incidences of gastrointestinal anastomotic leakage, abdominal bleeding, and abdominal infection were 2.2%, 2.2%, and 4.3%, respectively. After a median follow-up of 38.1 (95%CI:31.2-45.1) months, the 5-year OS was 50.3% (95%CI: 40.7%-59.9%) with a median survival time of 66.1 (95%CI: 43.1-89.1) months. The survival analysis showed that patients with pathological low grade, low PCI, and low CCR score had better survival with statistically significant differences (all P<0.05). Further stratified into complete and incomplete CRS subgroups, the 5-year OS of the CCR-0 and CCR-1 subgroups was 88.9% (95%CI: 68.3%-100.0%) and 77.6% (95%CI: 62.7%-92.5%), respectively; and 42.0% (95%CI: 29.5%-54.5%) in the CCR-2/3 subgroup. Conclusions:CRS and HIPEC may result in a long-term survival benefit for PMP patients with acceptable perioperative morbidity and mortality. This strategy, when complete CRS is possible, could significantly prolong survival for strictly selected patients at experienced centers.

Objective:To evaluate the efficacy and safety of cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) in the treatment of pseudomyxoma peritonei (PMP).Methods:In this descriptive case series study, we retrospective analyzed the records of PMP patients treated with CRS and HIPEC between January 2013 and June 2023 at Affiliated Cancer Hospital and Institute of Guangzhou Medical University. The inclusion criteria were as follows: (1) Aged 18 to 75 years and nonpregnant women. (2) Histologically confirmed diagnosis of pseudomyxoma peritonei. (3) Karnofsky Performance Scale (KPS)>70. (4) The functions of major organs such as the heart, liver, lungs, and kidneys can tolerate major surgery for long periods of time. (5) No evidence of extra-abdominal metastasis. Patients with extensive intra-abdominal adhesions or severe infectious diseases were excluded. The main outcomes were overall survival (OS) and postoperative major complications. The postoperative major complications were graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events (version 5.0). We used the peritoneal cancer index (PCI) score to quantitatively assess the peritoneal metastases and the completeness of cytoreduction (CCR) score at the end of surgery (CCR-0 and CCR-1 considered to be complete CRS).Results:A total of the 186 PMP patients with a median age of 56 (interquartile range extremes (IQRE), 48-64) years were included, 65 (34.9%) males and 121 (65.1%) females. The median peritoneal cancer index (PCI) score was 28 (20-34). Appendiceal origin accounted for 91.4%. Histological types were low grade in 99 patients (53.2%), high grade in 57 patients (30.6%), and 55 patients (29.6%) received complete cytoreduction (CCR-0/1). The median operative duration was 300 (211-430) minutes for all patients. Treatment-related 30-day mortality was 2.7%; 90-day mortality 4.3%; reoperation 1.6%; and severe morbidity 43.0%. Within the entire series, anemia(27.4%), electrolyte disturbance(11.6%), and hypoalbuminemia(7.5%) were the most frequent major complications (grade 3-4). The incidences of gastrointestinal anastomotic leakage, abdominal bleeding, and abdominal infection were 2.2%, 2.2%, and 4.3%, respectively. After a median follow-up of 38.1 (95%CI:31.2-45.1) months, the 5-year OS was 50.3% (95%CI: 40.7%-59.9%) with a median survival time of 66.1 (95%CI: 43.1-89.1) months. The survival analysis showed that patients with pathological low grade, low PCI, and low CCR score had better survival with statistically significant differences (all P<0.05). Further stratified into complete and incomplete CRS subgroups, the 5-year OS of the CCR-0 and CCR-1 subgroups was 88.9% (95%CI: 68.3%-100.0%) and 77.6% (95%CI: 62.7%-92.5%), respectively; and 42.0% (95%CI: 29.5%-54.5%) in the CCR-2/3 subgroup. Conclusions:CRS and HIPEC may result in a long-term survival benefit for PMP patients with acceptable perioperative morbidity and mortality. This strategy, when complete CRS is possible, could significantly prolong survival for strictly selected patients at experienced centers.

Objective:To explore the diagnostic value of transrectal ultrasound(TRUS)/multiparametric magnetic resonance imaging(mpMRI) fusion targeted biopsy(FTB) for clinically significant prostate cancer(PCa) detection by using both biopsy histopathology and radical prostatectomy histopathology as reference standards.Methods:A total of 303 consecutive patients with suspicious lesions detected by mpMBI and underwent prostate biopsy at Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine between November 2017 to January 2020 were retrospectively analyzed. All the suspicious lesions were sampled by TRUS/mpMRI FTB in addition with standard 12-core systematic biopsy(SB). The clinically significant PCa detection rates by TRUS/mpMRI FTB and SB were compared by using both biopsy histopathology and radical prostatectomy histopathology as reference standards.Results:The diagnosis of PCa was histologically confirmed in 189 of 303 patients, including 178 patients with clinically significant PCa and 11 patients with clinically insignificant PCa. With biopsy histopathology as reference standard, the clinically significant PCa detection rate of TRUS/mpMRI FTB was statistically higher than SB (57.1% vs 45.9%, P<0.001). Among 189 patients with biopsy proven PCa, 80 patients underwent radical prostatectomy, and the radical prostatectomy histopathology confirmed 79 patients with clinically significant PCa.With radical prostatectomy as reference standard, the clinically significant PCa detection rate of TRUS/mpMRI FTB was statistically higher than SB (91.1% vs 74.7%, P<0.001). Conclusions:Compared with SB, MRI/US FTB can offer more accurate sampling of suspicious lesions on mpMRI, and consequently improve the clinically significant PCa detection rate.

Objective:To introduce the Burnt Hand Outcome Tool (BHOT) into China to conduct sinicization, cross-cultural debugging, and test the reliability and validity, thus provide a reference for the evaluation of treatment effect and making of medical decisions of hand burn patients.Methods:The cross-sectional research was conducted. After obtaining the authorization of the original author, the Brislin translation mode was used to literally translate, synthesize, back-translate, and be checked by experts to form the Chinese version of BHOT (C-BHOT) 2. Ten experts in the field of burn rescue and treatment were invited by purpose sampling method to conduct cross-cultural debugging to form C-BHOT 3. A total of 21 hand burn patients who were treated in General Hospital of Ningxia Medical University from January to March 2020 and conformed to the inclusion criteria were selected by accidental sampling method for pre-testing. Then the final version of C-BHOT 4 was formed. Hospitalized patients who met the selection criteria were recruited using accidental sampling, multi-site and cross-regional survey method from May 2020 to March 2021 in General Hospital of Ningxia Medical University, the First Affiliated Hospital of Air Force Medical University, the First Affiliated Hospital of Army Medical University (the Third Military Medical University), and the First Affiliated Hospital of Nanchang University. The survey was performed with the general information questionnaire and C-BHOT 4. Critical ratio method and correlation coefficient method were used for item analysis, Cronbach's α coefficient test tool was used to test internal consistency, test-retest reliability was used to judge the stability of the tool, content validity index analysis tool was used to analyze the content validity, and exploratory factor analysis was used to test the validity of the structure. Data were statistically analyzed with independent sample t test or Pearson correlation analysis. Results:After the sinicization, cross-cultural debugging, and pre-testing, C-BHOT 4 was formed with a total of 20 items. All items were adjusted to positive scoring, and 7 modifications were made on the basis of the original tool. A total of 353 questionnaires were issued in the formal survey, and 344 valid questionnaires were returned, with an effective response rate of 97.45%. In the 344 patients, there were more male patients (297 patients) than female patients (47 patients), with ages of 23 to 57 years. The critical ratios of each item in the item analysis ranged from 5.48 to 12.59 ( P<0.05). There were statistically significant differences in the scores between patients in high-score group and low-score group ( t=-10.72--2.84, P<0.05 or P<0.01). There was significant positive relationship among the scores of each two items and between scores of each item and the overall tool score ( r=0.68-0.71, 0.47-0.63, P<0.05 or P<0.01). In reliability test, the Cronbach's α coefficient of the whole tool was 0.837, and the test-retest reliability was 0.702-0.793. In validity test, the content validity index of whole tool was 0.923. The exploratory factor analysis extracted 3 common factors, and the cumulative variance contribution rate was 76.162%. The comparative fit index in the confirmatory factor analysis was 0.924. Conclusions:After the BHOT tool being sinicized, cross-culturally debugged, pre-tested, and tested for reliability and validity, it was verified that C-BHOT 4 has good reliability and validity in assessing the outcome of Chinese hand burn patients, and therefore can be used as an effective tool for the evaluation.

Objective:To assess the utility of contrast-enhanced ultrasound (CEUS) targeted biopsy (TB) for clinically significant prostate cancer (PCa) detection.Methods:A total of 983 consecutive patients scheduled for prostate biopsy from October 2015 to March 2019 in Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine were enrolled in this retrospective study. All patients had suspicious lesions on CEUS, defined as increased focal contrast enhancement, rapid contrast enhancement and low enhancement lesions with ill-defined borders. Suspicious lesions on CEUS were sampled in addition with standard 12-core systematic biopsy(SB). Clinically significant PCa was defined using Epstein criteria. The clinically significant PCa detection rate by CEUS-TB and combined biopsy was evaluated in comparison with SB.Results:In 502 of the 983 patients, the diagnosis of PCa was histologically confirmed, including 445 patients with clinically significant PCa and 57 patients with clinically insignificant PCa. The clinically significant PCa by CEUS-TB and combined biopsy were 41.9% (412/983) and 45.3% (445/983) respectively, which was significantly higher than SB (36.8%, 362/983)(all P<0.001). CEUS-TB resulted in additional 83 cases of clinically significant PCa, including 61 patients missed by SB and 22 patients under-graded by SB. Conclusions:CEUS is helpful in the detection of PCa lesions. Combined CEUS-TB and SB can improve the clinically significant PCa detection rate.

Objective To retrospectively investigate the value of contrast enhanced ultrasound ( CEUS) in breast cancer biopsy . Methods A total of 49 consecutive patients with biopsy confirmed breast cancer were retrospectively analyzed . All patients underwent CEUS and biopsies were thus performed targeting both the high perfusion and low/non‐perfusion regions on CEUS . T he diagnostic performance and core cancer involvement of the biopsy cores taken from the high perfusion regions were compared with those from the low/non‐perfusion . Results A total of 53 breast cancer lesions were biopsy confirmed in 49 patients .CEUS revealed homogeneous enhancement in 8 lesions ( 15 .1% ) ,and heterogeneous enhancement in 45 lesions ( 84 .9% ) . T he diagnostic accuracy rate for biopsy cores taken from the high perfusion regions was significantly higher than that from the low/non‐perfusion regions ( 98 .5% vs 72 .9% , P ＜0 .01) . T he core cancer involvement was also higher in high perfusion lesions ( 55% vs 30% , P ＜0 .01) . Conclusions CEUS can differentiate the active area and necrotic fibrosis area of breast tumors by displaying the microvessels ,thus contributing to the selection of biopsy sites .