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Objective:To investigate the clinical manifestations, pathological features, treatment and prognosis of primary bone lymphoma in children.Methods:The clinical data of children who were initially diagnosed as primary bone lymphoma and treated in Beijing Children's Hospital Affiliated to Capital Medical University from January 2016 to January 2020 were retrospectively analyzed, including gender, onset age, primary involvement site, clinical stage, pathological type, fracture, and clinical outcome. The related literature was reviewed.Results:All 11 children were initially diagnosed as primary bone lymphoma, with a median age of onset of 8.6 years old (2.7-12.3 years old), including 7 males and 4 females. There were 7 cases of diffuse large B-cell lymphoma (DLBCL), 3 cases of B lymphoblastic lymphoma (BLL), and 1 case of anaplastic large cell lymphoma (ALCL). The initial symptoms were bone pain in 8 cases, local swelling in 1 case, limp in 1 case, and fever in 1 case. One case was in stage Ⅰ, 7 cases were in stageⅡ, and 3 cases were in stage Ⅳ, and the most common sites of involvement were femur and tibia. All 11 cases were treated with chemotherapy according to different pathological types, with a median follow-up time of 45 months (7-80 months). Ten cases got complete remission, 1 case of BLL died of bone marrow recurrence after chemotherapy remission.Conclusions:The clinical manifestations of primary bone lymphoma in children are insidious, DLBCL is the most common pathological type, and the prognosis is good after standardized treatment.
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Objective:To summarize the clinical features of neuroblastoma (NB) with bone metastasis in infants and the prognostic factors.Methods:A retrospective analysis was performed on 32 patients aged ≤12 months who were enrolled in Beijing Children′s Hospital, Capital Medical University from January 2010 to December 2019 and had imaging findings suggesting signs of distant bone metastasis.The control group was included NB children, aged ≤12 months, who were admitted to Beijing Children′s Hospital, Capital Medical University during the same period, without signs of distant bone destruction.The clinical manifestations and auxiliary examinations of infants with bone metastasis were summarized, and the efficacy evaluation and survival analysis of infants with regular treatment and follow-up were conducted until December 31, 2020. Kaplan- Meier survival analysis was used for prognostic analysis, and Log Rank test was used for univariate prognostic analysis. Results:There were 32 NB infants with bone metastases, involving 12 males (37.5%) and 20 females (62.5%), accounting for 16.0% (32/200 cases) of infants diagnosed with NB du-ring the same period.The median age of onset was 9 (4.5-12.0) months.The main primary site included the retroperitoneal and adrenal region in 24 cases(75.0%) and mediastinum in 3 cases (9.4%). Among the 32 cases, 14 cases (43.8%) had simple bone metastasis, 19 cases (59.4%) had distant lymph nodes, 18 cases (56.3%) had bone marrow, and 3 cases (9.4%) had intracranial and meningeal metastasis.Bone metastasis mainly occurred in the skull, with 11 cases of single bone metastases and the remaining with 2 or more bone metastases.Compared with 168 NB infants without bone metastasis, the prognosis of those with bone metastasis was significantly worse [3-year overall survival(OS) rate 97.6% vs.82.7%, P=0.001]. Univariate analysis showed that the prognosis of NB children with bone marrow metastasis, meningeal and intracranial metastasis, MYCN gene amplification, and high-risk group was poor (all P<0.05). Two patients returned to the local hospital for treatment after diagnosis.A total of 30 children were recruited for efficacy evaluation and prognostic analysis.Twenty-nine children underwent surgery, of which 6 cases received surgery before chemotherapy and 23 cases received surgery after chemotherapy.One case received chemotherapy only.The mean course of chemotherapy was 6.2 (4-13) times.One case was treated with radiotherapy, 1 case was treated with Metaiodobenzylguanidine (MIBG) therapy, and 1 case was treated with stem cell transplantation.A total of 18 cases (62.1%) event-free survived, and 12 cases (40.0%) had a mean event at 7 (1.5-32.0) months.Among them, 7 cases survived and 5 cases died (16.7%). The expected 3-year event-free survival rate and OS rate were 57.1% and 82.7%, respectively. Conclusions:The most common sites of infant NB metastasis are bone and bone marrow, and the most common sites of bone metastasis are skull.Infants with bone metastasis had a worse prognosis than those without bone metastasis, and infants with bone and bone marrow metastasis had a worse prognosis than infants with single bone metastasis.
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To analyze the clinical characteristics, treatment and prognosis of mediastinal germ cell tumors (GCTs) with concurrent hematologic malignancy (HM). The clinical features, treatment and prognosis of 3 cases of HM associated with mediastinal GCTs treated in the Department of Medical Oncology, Beijing Children′s Hospital from November 2014 to September 2018 were retrospectively analyzed.Meanwhile, relevant cases were searched in the PubMed and Wanfang database from their establishment to December 2019.Three male cases of HM associated with mediastinal GCTs aged from 12 to 16 years.The pathogenesis of mediastinal masses suggested teratoma or yolk sac tumor.All of them were treated with surgery and chemotherapy.Acute myeloid leukemia (AML) or myelodysplastic syndromes (MDS) was diagnosed respectively at 5 months, 9 months and 31 months after initial GCTs in the 3 cases.Two patients died and 1 child survived at the last follow-up.A total of 135 cases of concurrent GCTs and HM (or leukemia) were reviewed in online databases, involving 127 cases (94.1%) with the mediastinal GCTs associated with HM and 8 cases(5.9%) with GCTs related HM from another original sites.One hundred and twenty-six cases (99.2%) were male and the median age of GCTs diagnosis was 22 (10-48) years.Fifty-three cases (41.7%) were teratoma and 94 cases (74.0%) were GCTs containing teratoma with or without yolk sac tumor.Among the types of HM, 72 cases (56.7%) were AML and 31 cases (24.4%) were AML-M7.The median interval between GCTs and HM was 3 (0-122) months.Forty-six cases (36.2%) presented 2 malignancies simultaneously.HM were diagnosed within 12 months of GCTs in 85 cases (66.9%). The survival data were known in 107 cases, involving 94 (87.9%) deaths and 13 (12.1%) survivors.The median survival time after diagnosis of HM was 2 (0-48) months.The tendency of HM must be highly concerned in adolescent male patients with primary mediastinal GCTs, especially those with yolk sac tumor or teratoma.Their prognoses are very poor.Allogeneic hematopoietic stem cell transplantation is an alternative treatment.
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Objective:To investigate the improved performance of hepatic elastography combined with the serum biomarkers for the diagnosis of biliary atresia.Methods:A total of 193 patients with suspected biliary atresia in Beijing Children′s Hospital from March 2019 to November 2020 were consecutively collected. All patients were randomly divided into the training cohort and validation cohort at a ratio of 7∶3. LASSO regression analysis was used for the selection of the model index based on the data set from the training cohort including the serum biomarkers, demographic features (age and sex) and hepatic elastic measurement, and a diagnostic model for biliary atresia was subsequently developed by weighting on the basis of the dominance ration. The performance of the model was respectively evaluated with respect to the discrimination and calibration in each cohort.Results:Alanine aminotransferase (ALT), glutamyl transferase (GGT) and hepatic elastic measurement were selected to build the model. The area under the ROC curve of the final diagnostic model was 0.943 with a sensitivity of 90.9% and a specificity of 85.7% in the training cohort, and 0.955 in the validation cohort. Hosmer-Lemeshow test ( P=0.292, P=0.951) and calibration curves further validated its satisfactory calibration in both cohorts. As demonstrated by Delong et al.test, employing the model in the training cohort achieved the best diagnostic performance compared with using single model index ( P<0.001, P=0.016, P<0.001). In the validation cohort, the decision curve analysis showed the model had a higher overall net benefit over using hepatic elastography alone in every predicted probability. Conclusions:The diagnostic model for biliary atresia, which incorporates ALT, GGT and hepatic elastic measurement, can improve the performance of hepatic elastography with a higher clinical value.
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Objective:To analyze the ultrasonographic images and clinical characteristics of congenital mesoblastic nephroma (CMN), and to investigate the differential performances with Wilm′s tumor (WT).Methods:Twenty-one cases of CMN patients confirmed by pathology from December 2008 to December 2019 in Beijing Children′s Hospital, Capital Medical University were collected as the CMN group, and in the same criterion, 51 cases of WT patients were taken as WT group. Ultrasonographic images and clinical characteristics were collected retrospectively, and then the tumor size, site, echo and age were compared and analyzed between the two groups. ROC curve was used to evaluate the differential performance.Results:The difference analysis showed that except for echo ( P=0.694), there were statistically significant differences in tumor size, site and age between the two groups (all P<0.05). In prenatal, the incidence of CMN was significantly higher than WT (61.9% vs 3.9%, P<0.001), and the specificity was 96.1%. The median age (interquartile range) of CMN after birth was significantly earlier than WT( Z=-4.044, P<0.001). The area under the ROC was 0.949, the best cutoff was 112.5 days, with a sensitivity of 87.5% and a specificity of 93.9%. Conclusions:It is difficult to distinguish CMN and WT by echo, but the diagnosis performance can be improved through combining tumor size with site, especially age.
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Objective@#To investigate the clinicopathological manifestations, molecular genetic, diagnostic histology and differential diagnosis of alveolar soft part sarcoma (ASPS) in children.@*Methods@#A total of 13 cases of ASPS diagnosed at Beijing Children′s Hospital from August 2009 to November 2018 were collected. HE staining, histochemical staining for PAS and D-PAS, immunohistochemical (IHC) staining for TFE3, INI1 and CD68 and florescence in situ hybridization (FISH) for TFE3 gene translocation were performed.@*Results@#There were four males and nine females, age ranged from 1 year and 2 months to 13 years and 8 months (mean 7.8 years); and four patients were under 5 years old. Histologically, the tumors showed a distinctive and characteristic nested or organoid growth pattern (11 cases) or solid, diffuse growth (2 cases). The tumor cells possessed abundant eosinophilic, or glycogen-rich and clear to vacuolated cytoplasm. The chromatin was relatively dispersed, with prominent and pleomorphic nucleoli; mitotic figures were rare. Vascular invasion was frequently seen. IHC staining showed specific nuclear TFE3 staining. The tumor cells were also positive for INI1,CD68 and vimentin; but were negative for MyoD1, Myogenin, CK and S-100 protein. Seven cases showed PAS and D-PAS staining, with fuchsia acicular or rod-shaped crystals in tumor cytoplasm. Nine cases showed TFE3 break-apart signals by FISH.@*Conclusions@#ASPS is a rare soft tissue sarcoma in children. Compared with ASPA in adults, it has both similarities and unique clinicopathologic characteristics. The diagnosis needs to be confirmed by combining clinical, pathologic, IHC and genetic testing.
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Objective@#To investigate the differential performance of ultrasound between infantile fibrosarcoma(IFS) and soft tissue hemangioma.@*Methods@#The retrospective study was conducted from January 2012 to January 2019 in Beijing Children′s Hospital, Capital Medical University. A total of 16 patients with IFS were consecutively collected as IFS group, and 26 consecutive patients with soft tissue hemangioma (hemangioma group) were selected for comparison according to the tumor size of IFS. All the final diagnosis got confirmed by pathological analysis. The ultrasonographic features of IFS and hemangioma were compared and analyzed, and after weighting on the basis of the dominance ration (OR), the ultrasonic features with diagnostic value were used to establish the prediction equation. Then, the differential performance of every feature and the prediction equation was evaluated via the ROC analysis.@*Results@#There were differences in the lesion echo, margin and blood flow characteristics between IFS group and hemangioma group (P=0.013, 0.002, 0.005), the area under the ROC (AUC) of applying every ultrasonic feature with diagnostic value for differentiation were 0.695, 0.740, and 0.700, respectively. Benefiting from the employment of the prediction equation, the AUC was improved to 0.887 with a sensitivity of 81.3% and a specificity of 96.2%, which were better than the diagnosis by any single feature (P=0.017, 0.035, 0.003).@*Conclusions@#Ultrasound can be utilized to discriminate infantile fibrosarcoma from soft tissue hemangioma. The use of prediction equation can further improve the differential performance with higher clinical value.
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Objective@#To investigate the clinical, pathological features and differential diagnosis of testicular Leydig cell hyperplasia (LCH) .@*Methods@#Clinical data, histological features, immunohistochemical findings, ultrastructural characteristics and follow-up data were analyzed in three cases of LCH. The cases were collected from 2011 to 2014 at Beijing Children′s Hospital. A literature review was performed.@*Results@#Two males (1.8 years and 2.9 years of age) showed isosexual pseudoprecocity with elevated serum testosterone. Imaging study showed bilateral testicular enlargement with multiple small nodules in the parenchyma. Another 13 years-old patient showed male pseudohermaphroditism and cryptorchism. Gross examination showed the bilateral markedly enlarged testis without discrete lesion. Histologically, LCH was seen in both nodular and diffuse patterns without destruction of seminiferous tubules. Adjacent spermatogenesis was noted. Immunohistochemically, the Leydig cells were positive for inhibin, calretinin and Melan A and ultrastructural analysis showed enriched cytoplasmic endoplasmic reticulum. Two cases had followed up for 7 years. One patient was symptom-free and one was stable.@*Conclusion@#LCH is a rare benign condition, which is easily misinterpreted as testicular tumor or non-neoplastic diseases. Clinical presentation, imaging study and pathological evaluation are required for the diagnosis.
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Objective@#To investigate histopathological characteristics, and differential diagnoses of childhood synovial sarcoma.@*Methods@#HE staining, immunohistochemical staining and fusion gene detection by FISH were performed in 12 cases of synovial sarcoma in childhood at Beijing Children′s Hospital from 2016 to 2018.@*Results@#There were 6 cases of biphasic type, 1 case of monophasic epithelial type, 3 cases of monophasic spindle cell type and 2 cases of poorly differentiated synovial sarcomas. EMA, CKpan, bcl-2, CD99, TLE1 and CD34 immunostain positivities were observed in 10/12, 9/12, 12/12, 10/12, 10/12 and 0/12 cases respectively. Unique INI1 immunohistochemical staining was observed in 9/12 cases. SS18-SSX gene fusion was detected in 8 of 11 cases by FISH.@*Conclusions@#Synovial sarcoma is rare in children. Histological morphology combined with immunohistochemistry and FISH SS18-SSX fusion gene detection are important for the diagnosis and differential diagnosis of synovial sarcoma in children.
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Objective@#To summarize the clinical characteristics, treatment response and prognostic factors of rhabdomyosarcoma (RMS) in children.@*Methods@#The clinical characteristics such as age at diagnosis, primary tumor site, tumor size, pathological type, clinical stage, and risk grouping of 213 RMS patients (140 males and 73 females) treated in Hematology Oncology Center of Beijing Children′s Hospital, Capital Medical University, from May 2006 to June 2018 were analyzed retrospectively. The clinical characteristics, overall survival (OS), event free survival (EFS) and prognostic factors of children treated with the Beijing Children′s Hospital-Rhabdomyosarcoma (BCH-RMS) regimen were analyzed. Survival data were analyzed by Kaplan-Meier survival analysis, and single factor analysis was performed by Log-Rank test.@*Results@#The diagnostic age of 213 cases was 48.0 months (ranged 3.0-187.5 months), of which 136 cases (63.8%) were younger than 10 years old. The head and neck region was the most common primary site of tumor (30%, 64 cases), followed by the genitourinary tract (26.8%, 57 cases). Among pathological subtypes, embryonal RMS accounted for 71.4% (152 cases), while alveolar RMS and anaplastic RMS accounted for only 26.8% (57 cases) and 1.9% (4 cases), respectively. According to the Intergroup Rhabdomyosarcoma Study Group (IRS), IRS-Ⅲ and Ⅳ accounted for 85.0% (181 cases) of all RMS patients. In all patients, 9.4% (20 cases) patients were divided in to low-risk group, 52.1% (111 cases) patients in to intermediate -risk group, 25.8% (55 cases) patients in to high-risk group, and 12.7% (27 cases) patients in to the central nervous system invasion group, respectively. All patients with RMS received chemotherapy. The cycles of chemotherapy were 13.5 (ranged 5.0-18.0) for patients without event occurrence, while 14.2 (ranged 3.0-30.0) for patients with event occurrence. Among the 213 patients, 200 patients had surgical operation, of whom 103 patients underwent surgery before chemotherapy and 97 patients at the end of chemotherapy, 21 patients had secondary surgical resection. Radiotherapy was performed in 114 patients. The follow-up time was 23.0 months (ranged 0.5-151.0 months) . There were 98 patients with relapsed or progressed disease and 67 patients with death. The median time to progression was 10 months, of which 67 (68.4%) relapse occurred within 1 year and no recurrence occurred after follow-up for more than 5 years. The 3-year EFS and 5-year EFS were (52±4) % and (48±4) %, while the 3-year OS and 5-year OS were (65±4) % and (64±4) % by survival analysis. The 5-year OS of the low-risk, intermediate-risk, the high-risk were 100%, (74±5) %, (48±8) %, and the 2-year OS of the central nervous system invasion group was (36±11) % (χ2=33.52, P<0.01). The 5-year EFS of the low-risk, intermediate-risk, the high-risk were (93±6) %, (51±5) %, (36±7) % and the 2-year EFS of the central nervous system invasion group was (31±10) % (χ2=24.73, P<0.01) . Survival factor analysis suggested that the OS of children was correlated with age(χ2=4.16, P=0.038), tumor TNM stage (χ2=22.02, P=0.001), IRS group (χ2=4.49, P<0.01) and the risk group (χ2=33.52, P<0.01).@*Conclusions@#This study showed that the median age of newly diagnosed RMS patients was 4 years. The head and neck and the genitourinary tract were the most common primary origin of RMS. The OS was low in single-center RMS children. The median time to recurrence was 10 months, and recurrence was rare 3 years later.
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Objective To investigate the differential performance of ultrasound between infantile fibrosarcoma(IFS) and soft tissue hemangioma.Methods The retrospective study was conducted from January 2012 to January 2019 in Beijing Children's Hospital,Capital Medical University.A total of 16 patients with IFS were consecutively collected as IFS group,and 26 consecutive patients with soft tissue hemangioma (hemangioma group) were selected for comparison according to the tumor size of IFS.All the final diagnosis got confirmed by pathological analysis.The ultrasonographic features of IFS and hemangioma were compared and analyzed,and after weighting on the basis of the dominance ration (OR),the ultrasonic features with diagnostic value were used to establish the prediction equation.Then,the differential performance of every feature and the prediction equation was evaluated via the ROC analysis.Results There were differences in the lesion echo,margin and blood flow characteristics between IFS group and hemangioma group (P =0.013,0.002,0.005),the area under the ROC (AUC) of applying every ultrasonic feature with diagnostic value for differentiation were 0.695,0.740,and 0.700,respectively.Benefiting from the employment of the prediction equation,the AUC was improved to 0.887 with a sensitivity of 81.3 ％ and a specificity of 96.2 ％,which were better than the diagnosis by any single feature (P =0.017,0.035,0.003).Conclusions Ultrasound can be utilized to discriminate infantile fibrosarcoma from soft tissue hemangioma.The use of prediction equation can further improve the differential performance with higher clinical value.
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Objective@#To investigate the clinicopathological and ultrastructural characteristics of Langerhans cell histiocytosis (LCH) in children.@*Methods@#A total of 345 cases of LCH from the Department of Pathology, Beijing Children Hospital from January 2012 to March 2016 were investigated by hematoxylin-eosin stain, EnVision immunohistochemistry and transmission electron microscopy.@*Results@#The rate of primary clinical diagnosis of LCH in children was 46.0%(210/457). Among 345 patients of LCH, 213 were male and 132 were female, the male to female ratio was 1.6∶1.0, and the median age was 21 months (range from 2 days after birth to 13.3 years). There were total 597 lesions, including bony lesions (258, 43.2%), skin lesions (206, 34.5%) , followed by lymph node (16, 2.7%), lung (28, 4.7%), liver (25, 4.2%) and head-neck (50, 8.4%). Single organ system LCH (SS-LCH) was seen in 295 cases (85.5%) and 50 cases (14.5%) presented with multiple organ system involvement LCH (MS-LCH). There was no significant difference in age and gender between SS-LCH and MS-LCH groups. Regarding sites, more lesions were seen in bone and skin in SS-LCH group, in contrast lymph node, lung, liver and head-neck involvements were often seen in MS-LCH group. Immunohistochemically, the expression of CD1a and Langerin was seen in 99.7% (341/342) and 98.8% (338/342) of the cases respectively. The diagnostic rates by light and transmission electron microscopy were 98.8% (341/345) and 97.4% (112/115) respectively (P>0.05).@*Conclusions@#LCH of children occurs predominantly in SS-LCH pattern, frequently involving bone, skin, lymph node, lung and liver and other sites with unique histopathological, immunophenotypical and ultrastructural features. Accurate diagnosis relies on the morphology, immunophenotype and ultrastructural features. Further refinement of specimen processing may improve the accuracy of pathological diagnosis.
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Objective@#To summarize the clinical data including manifestations, diagnosis, treatment and prognosis of eosinophilic gastroenteritis (EGE) in children.@*Methods@#A retrospective analysis was performed in 71 patients with pathologically proven EGE at Beijing Children's Hospital Affiliated to Capital Medical University from January 2008 to January 2017. Their clinical manifestations, laboratory and imaging examinations, endoscopic findings, histopathological examinations, and treatment were collected and analyzed.@*Results@#Among 71 EGE cases, 47 (66%) cases were male and 24 (34%) cases were female, and the median age was 9.2 (0.2-16.5) years old. The main clinical manifestations included abdominal pain (76%, 54/71), vomiting (68%, 48/71), anorexia (54%, 38/71), weight loss (38%, 27/71), and diarrhea (37%, 26/71). There were 27 cases (38%) with a history of allergic diseases or family history. The median absolute value of eosinophil in peripheral blood of the 71 patients was 0.4 (0-36.8)×109/L, and 27 cases (38%) showed an increase in eosinophil counts. Serum IgE was measured in 52 patients (104.3 (3.4- 3 000.0)×103 U/L), and 30 patients (58%) showed an increase in serum IgE. A large number of eosinophils ((41.0±8.5)/HP) were found in 3 patients' ascites. The endoscopic examination of upper gastrointestinal tract revealed hyperemic edema in 62 cases (87%), plaque in 44 cases (62%), erosion in 17 cases (24%) and ulceration in 16 cases (23%). Histopathologically, in 8 cases (11%) the disease involved both stomach and duodeneum, in 21 cases (30%) involved stomach only, and in 37 cases (52%) involved duodeneum only. In addition, in 6 cases (8%) the disease involved esophagus and in 10 cases (14%) involved colorectum. Microscopically, eosinophil counts averaged 67/HP, 33/HP, 40/HP and 38/HP in esophageal, gastric, duodenal and colorectal mucosa respectively. A total of 34 cases were treated with glucocorticoid, and all these patients had alleviation of symptoms, which occurred within 14.9 days on average, but EGE recurred in 11 cases (32%).@*Conclusions@#The clinical symptoms and endoscopic findings of EGE are diverse and nonspecific. Histopathological examination of gastrointestinal mucosa is particularly important for the diagnosis. Glucocorticoid treatment is effective, but the patients with EGE are prone to relapse.
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Objective To evaluate CYP2C19 genotypes distribution in children with Helicobacter pylori (Hp) infection and to know its correlation with gender,age,severity and frequency of clinical symptoms,pathological classification of gastric antral mucosa.Methods Antral mucosas of 214 Hp infection patients who were hospitalized at Beijing Children's Hospital of Capital Medical University from July 2013 to December 2015 were collected.Genotypes were determined by PCR-sequence specific primer method,which were classified as homozygous extensive metabolizer (HomEM),heterozygous extensive metabolizer (HetEM) and poor metabolizer (PM).The differences in CYP2C19 genotype distribution in gender,age,severity and frequency of clinical symptoms,pathological classification of gastric antral mucosa were analyzed.Results (1) Among 214 Hp infected children,the percentage of HomEM was 48.1％ (103/214 cases),HetEM was 46.3％ (99/214 cases),and PM was 5.6％ (12/214 cases).PM in the CYP2C19 infection patients was lower than that in the normal Han nationality in China,and the difference was statistically significant (P ＜ 0.05).(2)Among the 214 Hp infected patients,there were 124 males and 90 females,and their median age was 9 years and 9 months(ranged from 2 years and 8 months to 17 years and 11 months).There was no statistical difference in gender and age of CYP2C19 genotypes(all P ＞ 0.05).(3) There was no statistical difference in severity and frequency of clinical symptoms of CYP2C19 genotypes(all P ＞0.05).(4) In HomEM group,according to pathological classification of gastric mucosa,there were 14 cases of mild injury,36 cases of moderate injury and 53 cases of severe injury,respectively.In HetEM group,there were 17 cases of mild injury,29 cases of moderate injury and 53 cases of severe injury,respectively.In PM group,there were 2 cases of mild injury,3 cases of moderate injury and 7 cases of severe injury,respectively.There was no statistical difference in the pathological degree of inflammation changes in gastric antral mucosa of CYP2C19 genotypes(all P ＞0.05).(5)Thirty-five cases didn't receive treatment,78 cases received Hp eradication one time and failed,101 cases received no less than 2 times of unsuccessful Hp eradication.The number of Hp unsuccessful eradications were positively correlated with the degree of pathological inflammation changes (r =0.219,P ＜ 0.01).There was obvious difference between the number of Hp unsuccessful eradication and the pathological degree of inflammation changes in gastric antral mucosa (x2 =12.414,P ＜ 0.05).Conclusions There was no statistical difference in CYP2C19 genotypes distribution as for different gender,age,severity and frequency of clinical symptoms,pathological classification of gastric antral mucosa and CYP2C19 genotypes.The number of Hp unsuccessful eradication was positively correlated with the degree of pathological inflammation changes.
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Objective To study features of clinical manifestation,pathology and treatment effects of idiopathic membranous nephropathy(IMN).Methods A retrospective study was performed in patients with membranous nephropathy through renal biopsy diagnosis in Beijing Children's Hospital Affiliated to Capital Medical University from November 2009 to February 2013.Patients were diagnosed according to renal biopsy apart from hepatitis B virus-associated glomerulonephritis,Henoch-Schonlein purpura nephritis,lupus nephritis,tumor and drug associated nephritis.The study summed the clinical and pathological features of IMN and evaluated the effect of treatment according the follow up.Results (1) There were 13 patients,with the ratio of male and female being 1.0 ∶ 1.6,among them 1 patient was younger than 6 years old,3 cases were between 6 and 12 years old,and 9 cases were elder than 12 years old.Nine patients were nephrotic syndrome,and 3 patients had renal insufficiency.(2)Ten patients had infection,among them 5 cases were upper respiratory infection with agent unfound,4 patients were mcoplasma infection,1 patient was influenza B virus infection,1 patient had positive result of Hepatitis B surface antibody(HBsAb) and Hepatitis B core antibody (HBcAb),and 2 patients had positive result of purified protein derivative (PPD).(3)Pathological feature of IMN was atypical membranous nephropathy.Atypical membranous nephropathy was characterized by an irregular,thickened glomerular basement membrane(GBM),mesangial cells,and a mesangial matrix with mild to moderate hyperplasia.Immune complexes were stained in the subepithelial region,GBM,and mesangial region under light microscopy.Electron microscopy showed various sizes,density,and shapes of electron-dense deposits in multiple regions.The cases of immunofluorescence were 13 for IgG,10 for C3,9 for IgM,9 for C1q,5 for IgA,2 for C4,and 0 for fibrinogen.Seven patients were strongly positive(3 + and above) for IgG and one patient was strongly positive for C3.(4)Seven patients had complete remission,three patients had partial remission,and three patients had non-remission.Conclusions (1) In this study,children with IMN were mainly girls,and the age distribution was beyond 12 years.Nephrotic syndrome was the most common clinical manifestation.(2)Atypical membranous nephropathy was the most common pathological feature in children with IMN,which should be distinguished from hepatitis B virus-associated glomerulonephritis by testing HBV antigen in renal biopsy.(3) Prednisone was effective for IMN children,but it was suggested to give enough and long course of application.Effect of prednisone combined with cyclophosphamide was still undefined.
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<p><b>OBJECTIVE</b>To study the clinicopathological and immunohistochemical features, histogenesis and prognosis of pleuropulmonary blastoma (PPB) in children.</p><p><b>METHODS</b>PPB specimens from 16 pediatric cases with an age ranging from 1 year and 7 months to 5 years and 3 months (mean age of 3 years) were retrieved and analyzed by routine histological, immunohistochemical and electron methods.</p><p><b>RESULTS</b>Among 16 patients, there were 2 type I, 7 type II and 7 type III PPB cases. Type I PPB as multilocular cystic structure, consisted of thin fibrous wall lining the respiratory epithelium, subepithelial primitive blastema or immature mesenchymal cells, with or without rhabdomyoblastic differentiation or cartilage; Type II PPB as cystic-solid tumor, comparing with type I, consisted of intracystic components with appearance of anaplastic tumor cells. Type III PPB consisted of completely solid mass, the same as the solid region of type II, had mixed pattern including blastema, undifferentiated spindle-cell proliferations and sarcomas. In addition, anaplastic tumor cells and intra-and extra- cytoplasmic eosinophilic globules were also commonly present. Epithelial components in PPB were benign. Immunohistochemical study showed primitive mesenchymal differentiation of tumors. All cases were positive for vimentin, desmin, myogenin and SMA in tumors with skeletal muscle differentiation, S-100 was positive in tumors with cartilage differentiation. All tumors were negative for synaptophysin, CD99, and CD117. Benign epithelial components were positive for AE1/AE3 and EMA. In 12 cases, electron microscopy revealed few organelles in the primitive mesenchymal cells and rich heterochromatin in mesenchymal cells, the latter also demonstrating cytoplasmic myofilament dysplasia. Nine cases had clinical follow-up ranging from 5 to 48 months, of which 4 patients died.</p><p><b>CONCLUSIONS</b>PPB is a rare lung neoplasm of children under the age of 6 years, with distinct pathological morphology. PPB may arise from lung or pleura mesenchymal cells and has a poor clinical outcome.</p>
Subject(s)
Child, Preschool , Female , Humans , Infant , Male , Cysts , Pathology , Desmin , Lung Neoplasms , Chemistry , Pathology , Microscopy, Electron , Myogenin , Prognosis , Pulmonary Blastoma , Chemistry , Pathology , Sarcoma , Pathology , VimentinABSTRACT
Objective To discuss the unique biological,histological and clinical features of pediatric renal cell carcinoma (RCC).Methods A retrospective review and biological analysis of all RCC cases presenting to our hospital from January 1973 to March 2012 was undertaken.Results Twenty-nine RCC pediatric patients (16 boys,13 girls) with mean age of 9.6 (range 2.5-16.0) years were identified.The presentations included hematuria in 17 (58.6％) cases with 3 who developed hematuria after trauma,abdominal mass with hematuria in 3 (10.3％),abdominal mass in 3,abdominal pain in 2,abdominal pain with hematuria in 1,and incidentally finding in 3.The diameter of tumor was from 2.5 cm to 25.0 cm,mean 6.8 cm.According to TNM stage grouping system,16 cases were stage Ⅰ,10 stage Ⅲ,and 3 stage Ⅳ.Xp1 1.2 translocation RCC was identified in 21 patients,clear cell RCC 6,papillary RCC 2.Of the 29 cases,3 patients with the tumor less than 7-cm had nephron-sparing surgery.A 15-cm tumor was incompletely removed in 1 patient and another patient with a 25 cm× 18 cm×15 cm tumor had gross residual.Nephrectomy was performed for the affected kidney in the remaining 24 patients.Twenty-one patients (Xp1 1.2 translocation RCC 13 patients,clear cell RCC 6,papillary RCC only 2) were followed up from 1.5 to 34.0 years,18 were living well (T1N0M0 in 11 cases,T1N1M0 in 2,T2N1M0 in 3,T3N1M0 in 1 and T4N1M1 in 1) and 3 died of recurrence.Conclusions Although RCC is rare in children,pediatric RCC behaves in a distinct fashion compared with adult forms of RCC.Hematuria is the main symptom in pediatric RCC.Xp11.2 translocation RCC is the predominant form,associated with an advanced stage at diagnosis.Nephrectomy is the common treatment for RCC and nephron sparing surgery could be a reasonable option for patients with tumor smaller than 7 cm.For localized RCC (T1-2 N0-1 M0),simple kidney removal surgery is sufficient for treatment without lymph node dissection and postoperative adjuvant treatment.