Objective To improve the diagnostic level by analysing and the CT features of undifferentiated embryonal sarcoma of liver(UES) in 6 cases all proved by pathology. Methods We retrospectively reviewed the clinical,pathologic,and imaging features in 6 cases.6 cases underwent unenchanced CT scan,5 case enchanced CT Results CT scan revealed a large uniloculated cyst in 3 and multiloculated cyst within hyperdense septations of variable shapes and thickness in 3.The margins of the lesions were well defined. Some solid portions at the periphery or adjacent to the septa were found in all cases. Punctate calcification at the periphery of the lesion was found in 1 case. A thin rim was seen surrounding the cystic tumor in all cases corresponding to the pathologic psedcapsule. The solid portions and the psedcapsule of the tumor had increased attenuation values and the cystic areas showed low attenuation after injection of contrast material. US demonstrated a single echogenic mass within some anechoic spaces of variable size in 4 cases. Conclusion CT scan showed well defined uniloculated or multiloculated cyst within variable amount of solid portions and an enhancing rim and predominantly solid at US. Discrepancy of internal architecture between US and CT was one of the important characteristic of UES.

Objective:To establish a method which could quantify human IL-8 mRNA in peripheral blood monocytes.Methods:Two probe were designed aiming at amplicon of RT-PCR,of which one being capture probe adorned 5,end by ammonia and one being monitor probe adorned 3,end by bintony.So as,the capture probe could couple with the N-oxysuccinmide esters(NOS) group on the DNA-binding wells through covalent attachment.In this way,oligonucleotides were immobilized on the plate surface and arise straightly to capture target amplicon.Total RNA was extracted from peripheral blood mononuclear cell and IL-8 mRNA was amplified by using RT-PCR.Heating denatured products and denatured probe,were then added to wells and hybridization occurs between capture probe,target amplicon and detection probe.After the addition of Avidiu-peroxidase developing system,the optical density were read at 450 nm.Results:Sensitivity,detecting PCR product of 16 PCR cycling,of 5?10~3 PBMCs and of a dilute 1∶256 were its prior.Meanwhile,this method could get a specify result and a precise of 5.2%.Conclusion:Resulting in simplicity,sensitivity and specificity results,this method may be a good choice for quantify of PCR products.

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.

Objective: To summarize the clinical and pathological features of small vessel-childhood primary angiitis of the central nervous system (SV-cPACNS), discuss the immune therapy and increase the attention to brain biopsy in SV-cPACNS. Methods: The clinical data, pathology of brain biopsy, treatment and outcome of an SV-cPACNS patient hospitalized in Beijing Children's Hospital in February 2016 were analyzed retrospectively. The cases reported at Pubmed, CNKI and Wanfang databases from 2007 to 2017 were searched, the clinical and pathological features, immunotherapy and prognosis of the disease were summarized according to the literature review. Results: A 70 months old girl had 6 times relapses during 5 months' disease course. Symptoms included convulsions, limb paralysis, blurred vision and speech difficulty. Multiple cortical lesions were found successively in brain MRI but CT angiography was negative. The pathology of brain biopsy revealed thickening of small vessel walls together with lymphocytic infiltration. After the treatment with rituximab, remission was achieved and remained stable without recurrence in 1 year follow up. A total of 44 pathologically confirmed cases reported in nearly 10 years were retrieved. Male to female ratio was 1∶3.5. The average onset age was 9.8 years. Clinical manifestations included seizures (37/45, 82%), headache (35/45, 78%), cognitive decline (28/45, 62%), speech regression(20/45, 44%), paralysis (15/45, 33%), and so on; 70% (19/27) patients experienced relapses. Erythrocyte sedimentation rate and C-reactive protein slightly elevated, antinuclear antibody and other autoimmune antibodies were mostly negative. Mild lymphocytosis in cerebrospinal fluid was found in 67%(29/43) patients. 53%(23/43) patients had elevated CSF protein level, several had elevated IgG and positive oligoclonal band. Bilateral multifocal lesions were revealed in 80% (36/45) brain MRIs, meanwhile all angiographies were unremarkable. The pathology showed small angiitis and immunohistochemistry positive for CD3 and CD20. Twenty-seven patients had detailed therapeutic information; 25 of them received immunosuppressive agents, including cyclophosphamide, mycophenolate mofetil and rituximab. One patient died, 26 patients achieved remission with 54% (22/44) had neurological sequelae. Conclusions SV-cPACNS had varied clinical manifestations, there was no specificity in laboratory and imaging examination and angiography was often negative. The definite diagnosis relied on brain biopsy, which showed lymphocytic inflammation of small vessels. SV-cPACNS tended to relapse and induce neurologic deterioration. Treatment required long-term use of steroids and immunosuppressive agents. Rituximab could be an effective agent.

Objective To explore the short-term efficacy in children with head and neck rhabdomyosarcoma (HN-RMS) treated by multidisciplinary therapy,and to analyze the prognostic factors,so as to guide the diagnosis and treatment.Methods Patients with HN-RMS admitted at Hematology Oncology Center of Beijing Children's Hospital (BCH),Capital Medical University between December 2012 and May 2017,were included in this case-observation study.The clinical characteristics were analyzed and the treatment effect and prognostic factors were summarized.Results A total of 48 cases were collected,including 36 boys and 12 girls,with a median age of 4.6 years.Primarysite parameningeal RMS(PM-RMS) (34 cases,70.8％),orbital (2 cases,4.2％) and non-orbital,non-parameningeal region(12 cases,25.0％) were found.Twenty cases belonged to alveolar type(41.7％),and 28 cases were of embryonaltype(58.3％).The diameter of the tumor was ＞5 cm(n =25,52.1％),and ≤5 cm(n =23,47.9％).IRS staging:there were 29 cases(60.4％) of stage Ⅱ-Ⅲ,19 cases (39.6％) of stage Ⅳ;29 cases (60.4％) of low-medium risk,and 19 cases (39.6％) of high risk.Twenty-three patients (47.9％) received surgery,and 25 cases (52.1％) received biopsy only.All patients (48 cases) received systemic chemotherapy.Twenty patients (41.6％) received external radiation,15 cases (31.3％) received 125I particle implantation,6 cases (12.5％) received proton therapy,but 3 cases (6.2％)did not receive radiation.The follow-up time lasted 13-57 months[(24.1 ± 12.3) months].The 2-year overall survival(OS) rate was (66.4 ± 7.2)％,and 2-year event free survival (EFS) rate was (59.9 ± 7.5) ％.Patients with tumor diameter ≤ 5 cm had higher OS and EFS than patients with tumor diameter ＞5 cm [2-year OS (87.4±6.8)％ vs.(42.9 ±6.8)％,2-year EFS (78.8 ±8.6％) vs.(38.5 ±10.8)％],and the differences were statistically significant (all P =0.006).Patients with orbital and non-orbital,non-parameningeal RMS had higher OS and EFS than PM-RMS [2-year OS 100％ vs.(87.5％ ± 11.7) ％ vs.(57.0 ± 8.8) ％;2-year EFS 100％ vs.(88.9 ± 10.5)％ vs.(51.1 ± 8.9)％],and the differences were statistically significant (P =0.008,P =0.030).Patients who received surgery had higher OS and EFS than those who did not received surgery [2-year OS (80.7±8.8)％ vs.(53.3 ± 10.4)％;2-year EFS (71.1 ±10.1)％ vs.(49.5±10.4)％],and the differences were statistically significant (P =0.008,P =0.026).COX regression analysis showed tumor diameter ＞ 5 cm was an adverse prognostic factor (OR =4.124,95％ CI:1.213-14.025,P =0.023).Conclusions PM-RMS accounted for a high proportion in RMS patients.The primary site and the size of the tumor are the main prognostic factors.Intensive therapy is expected to improve the prognosis of HN-RMS with meningeal invasion.

OBJECTIVETo study the clinicopathological and immunohistochemical features, histogenesis and prognosis of pleuropulmonary blastoma (PPB) in children.

METHODSPPB 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.

RESULTSAmong 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.

CONCLUSIONSPPB 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.

Child, Preschool ; Cysts ; pathology ; Desmin ; analysis ; Female ; Humans ; Infant ; Lung Neoplasms ; chemistry ; pathology ; Male ; Microscopy, Electron ; Myogenin ; analysis ; Prognosis ; Pulmonary Blastoma ; chemistry ; pathology ; Sarcoma ; pathology ; Vimentin ; analysis

Objective:To analyze the clinical characteristics and prognosis of infants with rhabdomyosarcoma (RMS), thus enhancing the understanding of this disease.Methods:Clinical data of all RMS patients younger than 12 months treated in the Hematology Oncology Center, Beijing Children′s Hospital, Capital Medical University from May 2006 to June 2019 were retrospectively analyzed, including the age, gender, histological type, tumor primary site, tumor size, and the prognosis.Patients were followed up until December 31, 2019.The 3-year event free survival (EFS) rate of children was performed by plotting the Kaplan-Meier survival curves.Results:A total of 15 RMS children younger than 12 months were enrolled, accounting for 4.9% of all RMS cases in the same period, including 6 males and 9 females.The median age at diagnosis was 7.0 months (3.0-11.5 months). Classified by the primary site, 40.0% (6 cases) located in the head and neck, followed by 26.7% (4 cases) located in the limbs, 26.7% (4 cases) located in other parts, and 6.7% (1 case) located in the urogenital system.Embryonal RMS, alveolar RMS and spindle cell RMS accounted for 46.6% (7 cases), 26.7% (4 cases), and 26.7% (4 cases), respectively.Ten cases (66.7%) were stage Intergroup Rhabdomyosarcoma Study (IRS)-Ⅲ and 1 case (6.7%) was in stage Ⅳ.There were 10 cases (66.6%) in the middle-risk group, 4 cases (26.7%) in the low-risk group, and 1 case (6.7%) in the high-risk group.Two cases had a larger than 5 cm primary tumor; lymph node involvement was confirmed in 3 cases, and pulmonary metastasis occurred in 1 case at the time of diagnosis.All children were treated with chemotherapy, and 13 cases received postoperative chemotherapy and 1 case received preoperative chemotherapy.One case were not operated.Only 3 children underwent radiotherapy, including 1 case underwent particle implantation and 2 cases received external radiotherapy.Among the 15 children with RMS, 5 cases had relapse and disease progression with the 3-year EFS rate of (59.1±14.5)%, and 2 died with the 3-year overall survival rate of (80.8±12.6)%.Conclusions:The median age of diagnosis of RMS in single-center infants is 7 months.Head and neck are the most common primary sites of RMS.Nearly 50% of the children have the primary site of RMS with poor prognosis.More than a quarter of the pathological subtypes are the spindle cell type.Local treatment significantly influences the local progression or recurrence of RMS.

Objective:To improve the awareness of neuroblastoma (NB) with cervical lymph node metastasis in children patients by summarizing the clinical features of such patients and analyzing their survival situation.Methods:The research analyzed the medical records of 225 patients with cervical lymph node metastasis of NB from April 1, 2007 to May 31, 2017 in Beijing Children′s Hospital, Capital Medical University.The treatment were divided into 2 phases according to treatment time (one from April 1, 2007 to December 31, 2011 and the other from January 1, 2012 to May 31, 2017). The survival situation and treatment effect in each phase were analyzed.The following up time ended at Jan 1, 2018.Results:(1)Clinical features: total 225 cases admitted in the research with about 37.3% of all the NB patients.One hundred girls(44.4%) and 125 boys(55.6%). The age from 2 months to 147 months, the me-dian age of patients was 37 months.The age under 18 months were 27 cases(12.1%). The majority primary site of tumor was located in retroperitoneal(174 cases, 77.3%) and mediastinum(48 cases, 21.3%). There were 33 cases which metastasic site confined to cervical lymph node(4N stage, 14.7%); 139 cases (62.1%) with bone marrow metastasis, 159 cases (71.0%) with bone metastasis.The number of patients in high-risk group was 208 cases(91.2%), while the low-risk and the medium-risk group was 17 cases(7.5%). (2)Treatment effect and survival analysis: a total of 200 cases received systemic treatment and followed-up in our hospital.The follow up time from 2 months to 123 months, the median follow up time was 23 months.The 3 year overall survival (OS) rate of all the patients was 56.4%, the 3 year event free survival (EFS) rate was 52.0%.While the 5-year OS was 46.5%, and the 5-year EFS was 39.7%.According to the first phase of treatment(April 1, 2007 to December 31, 2011), the 3-year EFS was 49.6%, and the 3-year OS was 54.4%, the 5-year EFS was 39.5%, and the 5-year OS was 44.7%.According to the second phase of treatment(January 1, 2012 to May 31, 2017), the 3-year EFS was 58.1%, and the 3-year OS was 57.3%.The 5-year EFS was 48.7%, and the 5-year OS was 46.8%.About the 4N patients, the 3-year EFS was 71.2%, and the 3-year OS was 82.2%.The 5-year EFS was 61.5%, and the 5-year OS was 76.7%.Conclusions:Cervical lymph node is one of the most common distant metastasis of NB.More than half of the patient with bone or bone marrow metastasis.The prognosis of patients′ metastatic disease limited to cervical lymph nodes(4N stage), but still worse than the foreigner haver reported.The probable reason is the proportion of high-risk patients in the center is higher than the foreign research.

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.

Objective:To summarize the clinical features, therapeutic effects and prognostic factors of children with extracranial malignant germ cell tumor (MGCT), and further to improve the recognition of this disease.Methods:The clinical information and therapeutic effects of children patients diagnosed with MGCT and treated in Hematology-Oncology Center in Beijing Children′s Hospital, Capital Medical University from January 2008 to December 2018 were analyzed retrospectively, and the risk factors affecting the prognosis were discussed.Results:A total of 35 patients were collected in this study, including 19 girls and 16 boys, with a median age of 71 months.Forty-two point four percent (14/33 cases) of them were <3 year-old, and 39.3% (13/33 cases) of them were >6 year-old.The primary tumors were located at the gonad in 9 cases (6 cases in the ovary and 3 cases in the testis), and extragonadal sites in 26 cases (8 cases in the pelvic cavity, 8 cases in the mediastinum, and 10 cases in the sacrococcygeal region). Five cases were in stage Ⅱ, 16 cases in stage Ⅲ, and 14 cases in stage Ⅳ.Eleven cases were at intermediate risk, and 24 cases were at high risk.All children were followed up for (25.43±4.82) months, and given standard treatment of surgery plus chemotherapy.The 2-year overall survival (OS) rate and event-free survival (EFS) rate were 87.4% and 67.1%, respectively.The 2-year OS rate and EFS rate in patients at stage Ⅱ and Ⅲ (93.8% and 80.4%, respectively) were significantly higher than those at stage Ⅳ (66.7% and 45.1%, respectively) ( χ2=0.649, 0.739; P=0.021, 0.037). The 2-year EFS rate in patients with and without distant metastasis at the early stage of the disease was 43.8% and 86.9%, respectively, and the differences were significant ( χ2=0.694, P=0.028). The complete remission (CR) rates of intermediate-risk and high-risk children with tumor completely resected at the early stage of the disease were 100.0% (8/8 cases) and 71.4% (5/7 cases), respectively.The CR rate of patients who received 2-4 cycles of chemotherapy and had complete tumor resection after diagnosis by puncture was 72.7% (8/11 cases), and 40.0% (2/5 cases) of the children with incomplete resection got tumor progression.For eight patients with events, their alpha-fetoprotein (AFP) levels decreased slowly at the early stage of the disease and increased significantly as the disease progressed. Conclusions:The onset-age of MGCT is mainly <3 year-old and >6 year-old.The stan-dard treatment that combines surgery and chemotherapy can achieve a good overall prognosis.Incomplete surgical resection and a slow decline of AFP during treatment contribute to adverse prognosis.The outcomes of patients with tumor progression can be improved by increasing chemotherapy courses, secondary surgery and autologous stem cell transplantation.