Objective:To determine the effect of Hes1 on bone marrow CD34+cells in acute myeloid leukemia (AML). Meth-ods:Bone marrow mononuclear cells were isolated by using Ficoll. Then, the proportion and cell cycle of CD34+cells were analyzed by using fluorescence-activated cell sorting (FACS). CD34+cells were cultured in vitro for colony-forming cells (CFC). The expression of Hes1 in CD34+cells was evaluated by using real-time polymerase chain reaction. After upregulating the expression of Hes1 in CD34+cells, the cell cycle was analyzed through FACS, and the colony formation of CD34+Hes1+cells was analyzed by CFC. Results:The ra-tio of CD34+cells in the bone marrow was lower in the AML group than in the control group. In addition, more CD34+cells underwent quiescence in the AML group than in the control group. In vitro assay showed that the colony formation of CD34+cells was lower in the AML group than in the control group. The expression of Hes1 was higher in the CD34+cells from the AML patients than that in the CD34+ cells from normal donors. After Hes1 transduction, more CD34+ cells underwent quiescence and showed weak proliferation. Conclusion:The proportion of CD34+cells in the bone marrow was lower in AML patients than in normal donors. A large proportion of CD34+cells underwent quiescence, which was related to Hes1, in AML patients.

Objective To explore the clinical significance of PET-CT in evaluating distant metastasis and M staging of nasopharyngeal carcinoma(NPC). Methods 257 NPC patients with no prior treatment were investigated with PET-CT and conventional imaging (chest X-ray, abdominal ultrasound, and bone scan). The findings of PET-CT in diagnosing distant metastasis and M staging were compared with those of conventional imaging according to the results of biopsy and follow-up. Results PET-CT disclosed 34 of 39 patients with distant malignancy compared with 22 patients disclosed by conventional imaging. The false positive rate of PET-CT was 12.8 %. On region-based analyses, PET-CT was more effective than bone scan and chest X-ray for detecting mediastinum metastasis (x2=4.063, P =0.041) and bone metastasis (x2=5.939, P=0.015), respectively. Compared with conventional imaging, PET-CT had an impact on the M staging of 19 patients (7.4 %), of which 15 patients were truly staged and 4 patients incorrectly staged. Conclusion PET-CT is superior to MRI in evaluating distant metastasis and M staging of NPC.

Objective To analyze the clinicopathologic characteristics,treatment and prognosis of retinoblasto-ma without calcification.Methods The clinic and pathologic data of 20 cases of retinoblastoma without calcification were analyzed retrospectively.All the cases were selected from 710 cases of retinoblastoma archived in Beijing Tongren Hospital,Capital Medical University from April 2010 to April 2015.The inclusion criteria was no calcification on CT scan before treatment.The patients after enuclea-tion were diagnosed according to pathology;the patients without enu-cleation were diagnosed according to clinical manifestations,Retcam fundus,ophthalmic ultrasound and the reduction of tumor after chemotherapy.The clinical features,treatment and prognosis were summarized through statistical analysis of the 20 cases.The patients were followed -up through clinical reexamination and telephone calls.Results In this study,there were 14(70.0%)male and 6(30.0%)female patient′s;there were 19 cases(95.0%)less than 5 -year -old,14 cases (70.0%)less than 3 -year -old,1 case (5.0%)less than 1 -year -old.Eighteen cases (90.0%)were unilateral disease,and 2 cases (10.0%)were bilateral disease.According to international Intraocular Retinoblastoma Classification stage:4 cases (18.2%)in stage E ,17 cases (77.3%)in stage D ,1 case (4.5%)in stage C.Five cases (25.0%)were enucleated eyes,and pathology report was retinoblastoma.Nine cases (45.0%) were treated with pre -enucleation chemotherapy,and finally enucleated after poor tumor shrinkage or recurrence,and the pathology was retinoblastoma.Five cases (25.0%)without enucleation were stable.One case (5.0%)failed to follow -up owing to the patients irregular treatment.In 14 enucleation cases,histopathologic high risk factors could be found in 3 cases (21 .4%),and couldn′t be found in the other 11 cases (78.6%).In the cases that were not enuclea-ted,the tumor diminished in size or new calcification presented in the fundus examination,and in 12 cases,new calcifi-cation was pre-sented on follow -up CT scan.Follow -up failed in 1 case(5.0%),but follow -up was available for 19 cases,and among them 13 cases (68.4%)were disease -free survival and 6 cases (31 .6%)were alive with tumor. Conclusions Close attention should be paid to the untypical retinoblastoma with no calcification on CT scan through well -taken medical history and meticulous imaging examination,in order to give early diagnosis and therapy.

Objective To analyze the clinical features,treatment and prognosis of the pancreatoblastoma (PB).Methods A total of 9 patients including 4 boys and 5 girls diagnosed as pancreatoblastoma in Beijing Tongren Hospital Affiliated to Capital Medical University between July 2008 and February 2016 were enrolled.The clinical features,therapy and prognosis were analyzed.Results (1) Clinical manifestations:6 cases had abdominal mass,1 case had jaundice,2 cases had abdominal pain at the time of diagnosis;5 cases were found in caput pancreatis,2 cases were found in corpora pancreatis and 2 cases were found in cauda pancreatis;4 cases had local tumor,3 cases had local invasion,and 2 cases had liver metastasis at diagnosis.(2) Enzymology and tumor marks:elevated alpha-fetoprotein (AFP) was found in 5 cases (48.5-52 971.0 μg/L),elevated neuron-specific enolase(NSE) was found in 5 cases(22.4-53.4 pg/L),elevated carbohydrate antigen 125 (CA-125) was found in 5 cases [(40.3-122.8) × 103 U/ L],elevated carbohydrate antigen 19-9 (CA19-9) was found in 2 cases [(65.4-362.5) × 103 U/ L],elevated lactate dehydrogenase was found in 2 cases(380 ～ 838 U/L) and elevated hemodiastase was found in 2 cases (105.5-122.8 U/L).(3) Treatment and prognosis:in 9 patients,the tumors were resected at first in 6 patients,and 5 of them remitted until now,1 of them relapsed after the comprehensive treatment for 9 months.Two of the patients underwent operation after the chemotherapy,in which 1 case received complete resection but without chemotherapy after the surgery,whom relapsed 1 year later;and the other one was resected partly.But after stopping the comprehensive treatment for 6 months,the disease relapsed.One patient could not receive operate because of huge tumor size,and the chemotherapy failed to control tumor progression;the patient died without operation.Three relapsed children were still in chemotherapy.The median follow-up period was 21 months (8-98 months).Conclusions AFP may be used as monitoring indicator for PB.Complete tumor resection is an important factor affecting the outcome.Adjuvant chemotherapy may reduce recurrence and prolong survival.

Objective To summarize the clinical,pathological and radiological features of retinoblastoma (RB)with central nervous system (CNS)metastasis.Methods Twenty -three patients were confirmed to have RB with CNS metastasis in Beijing Tongren Hospital from December 2005 to December 201 3,and their clinical data were retrospectively analyzed.Results (1 )The incidence of RB with CNS metastasis was 1 .83% (23 /1 260 cases),and the incidence of CNS metastasis was 7.64%(1 1 /1 44 cases)if RB with optic nerve involvement which was confirmed through pathology.At first visit 1 0 cases had a confirmed diagnosis of RB with CNS metastasis,while the other 1 3 cases had a diagnosis of RB in extraocular stages.(2)Eleven patients with RB in extraocular stages had the pathological evi-dence of optic nerve involvement which was pathologically confirmed,6 patients had optic nerve involvement with cho-roid involvement,2 patients had optic nerve involvement with nerve sheath involvement.(3)All the patients whose diagnosis of CNS metastasis had the radiological evidence of CNS metastases,and 1 case had autopsy and pathological evidence for extensive brain metastases.The main radiological feature of CNS metastasis was meninges metastasis.Thir-teen cases had the radiological features of meninges metastasis and 5 cases accompanied with spinal cord meninges me-tastasis.Then,the following radiological feature was that 7 cases had the mass of suprasellar pool.(4)Twelve cases with CNS metastasis showed the cerebrospinal fluid (CSF)RB cells positive,and the positive rate was 52.1 7%(1 2 /23 ca-ses).Conclusions (1 )The occurrence of RB with CNS metastasis is very low,but the risk of CNS metastasis in-creased in the patients with the pathological evidence of the optic nerve involvement.(2)The main site of CNS metasta-ses is meninges,followed by mass formation in the suprasellar pool.The main route for CNS metastases was along the optic nerve and /or nerve sheath directly invading and /or disseminating via the CSF.(3)The positive rate of CSF cy-tology is low,and it can be improved through repeated examination.Enhanced magnetic resonance imaging should be the most effective radiology examination for diagnosis and monitoring CNS metastasis.

OBJECTIVETo observe the treatment and prognosis of optic nerve invasion in retinoblastoma (RB).

METHODThe children who had been diagnosed with unilateral RB and had received enucleation from January 2006 to December 2013 in our hospital were recruited. Tumor extension into the optic nerve were disclosed. Optic nerve involvement was classified into four grades according to the degree of invasion. Grade I is superficial invasion of the optic nerve head only, grade II is involvement up to and including the lamina cribrosa, grade III is involvement beyond the lamina cribrosa, and grade IV is involvement up to and including the surgical margin. Grade I and II are called invasion of the optic nerve before the sieve plate. Grade III and IV are called invasion of the optic nerve after the sieve plate. Other high-risk factors included extensive invasions of the choroid, sclera, anterior chamber, iris, and ciliary body. They were divided into two groups according to whether the merger of other high histopathologic risk factors. Treatment was delivered accordingly, and the prognosis of different degrees of optic nerve invasion was observed. The subjects were followed up for 6 months to 7 years (average: 43 months).

RESULTThere were 465 subjects in this study, including 279 boys and 186 girls. The right eye was affected in 260 patients and the left eye in 205 patients. The average time from onset of symptoms to visit was 2.7 months (range 1 day-24 months). Twenty-five patients died, resulting in an overall survival rate of 94.6%. The mortality rate of patients with optic nerve involvement with grade I was 0.4%, grade II was 1.0%, grade III was 8.7% was and grade IV was 60.9%. Of the 338 with invasion of the optic nerve before the sieve plate, two died of recurrence, with a survival rates of 99.4% (336/338). Of the 127 patients who had invasion of the optic nerve after the sieve plate, twenty-three died of recurrence, with a survival rate of 81.9% (104/127) , the difference was statistically significant (χ² = 52.299, P = 0.000). A total of 379 patients did not have any other merged pathology high-risk factors, 8 died, the mortality rate was 2.1%. Of the 86 patients who had complicated with other high-risk factors, 17 died, the mortality rate was 19.8%, the difference was statistically significant (χ² = 42.955, P = 0.000). Of the 338 patients, 304 had invasion of the optic nerve before the sieve plate had not merged other pathology high-risk factors, none died, of the 34 patients who had complicated with other pathology high-risk factors, 2 had died, the mortality rate was 5.9%, the difference was statistically significant (P = 0.010). Of the 127 patients with invasion of the optic nerve after the sieve plate, 76 had not complicated with other pathological high-risk factors, 9 of whom had died, the mortality rate was 11.8%, 51 had complicated with other pathological high-risk factors, 14 of whom had died, the mortality rate was 27.5%, outcomes did significantly differ between the two subgroups (χ² = 5.014, P = 0.025). Cox multivariate analysis showed that invasion of the retrolaminar optic nerve, surgical margin of the optic nerve and sclera were influential factors of colorectal cancer.

CONCLUSIONPatients with optic nerve invasion have an excellent outcome with current therapy. But for those whose resection margin was invaded, which has a high incidence of recurrence, chemotherapy is recommended for patients with postlaminar optic nerve involvement.

Child ; Female ; Humans ; Male ; Multivariate Analysis ; Neoplasm Invasiveness ; Neoplasm Recurrence, Local ; Optic Nerve Neoplasms ; pathology ; surgery ; Prognosis ; Retinoblastoma ; pathology ; surgery ; Retrospective Studies ; Survival Rate

Objective To kxplork thk clinical charactkristics,trkatmknt and prognosis of rhabdomeosarcoma (AMS)with intracranial kxtknsion in childrkn. Methods Thk clinical data of 12 casks of childrkn(5 malks and 7 fk-malks)with AMS and intracranial involvkmknt admittkd into thk Dkpartmknt of Pkdiatrics,Bkijing Tongrkn Hospital Lffiliatkd to Capital Mkdical Rnivkrsite from Dkckmbkr 2012 to Dkckmbkr 2017 wkrk analezkd rktrospkctivkle. Thkrk wkrk 5 malks and 7 fkmalks in 12 casks. Thk onskt agk rangkd from 1. 2 to 10. 2 ekars old,with a mkdian agk of 3. 4 ekars old. Thk clinical fkaturks,trkatmknt mkthods and prognosis wkrk summarizkd. ResuIts Thk primare sitks of 9 casks wkrk in thk hkad and nkcc(75﹪),2 casks wkrk in thk chkst and bacc(17﹪),and 1 cask was in thk pkrinkum (8﹪). Nink patiknts had obvious ckntral nkrvous sestkm involvkmknt semptoms,and cranial imaging findings wkrk studikd in all of thk 12 patiknts. Thk clinical pathological stagk and risc classification wkrk:6 casks of stagkⅣ(1 cask of ckntral aggrkssion);6 casks of stagk Ⅲ(4 casks of ckntral aggrkssion);10 casks wkrk in thk high-risc group(4 casks of stagk Ⅲ with ckntral violations),and 2 casks(stagkⅢ)in thk intkrmkdiatk risc group. Lll 12 casks rkckivkd sestkmic chkmothkrape,8 casks rkckivkd local or total cranial╱spink radiothkrape,9 casks undkrwknt primare rkskction, and 4 casks undkrwknt craniotome to rkmovk intracranial lksions. Rp to Januare 2018,4 casks of 12 childrkn survivkd (including 2 diskask-frkk patiknts)and 8 childrkn dikd. Thk ovkrall survival ratk was 33﹪(4╱12 casks)and thk diskask-frkk survival ratk was 17﹪(2╱12 casks). ConcIusions Thk prognosis of AMS with intracranial infiltration is vkre poor,and thk trkatmknt kffkct is not good. Complktk rkskction of karle lksions is thk cke to curk AMS. Aadiothkra-pe,individualizkd chkmothkrape and surgical rkmoval of intracranial lksions mae havk thk valuk in controlling diskask and prolonging survival timk.

Objective:To improve the clinicians′ understanding of central nervous system (CNS) metastasis in children with solid tumors.Methods:The clinical data of 33 cases of CNS metastasis among 611 children with malignant solid tumors in the chest and abdomen from September 2005 to December 2016 in Beijing Tongren Hospital, Capital Medical University were retrospectively analyzed. The clinical characteristics, treatment and prognosis of metastasis were statistically analyzed according to different pathological types of malignant solid tumors in children with CNS metastasis.Results:Among the 611 children with malignant solid tumors in the chest and abdomen, 15 patients were neuroblastoma with CNS metastasis (5.9%, 15/264), 13 patients were hepatoblastoma with CNS metastasis (4.8%, 13/274), two cases were Wilms tumor with CNS metastasis (3.2%, 2/63), and three cases were clear cell sarcoma of the kidney with CNS metastasis (3/10). The onset age of the malignant solid tumors was 3-189 months, and the median age of onset was 26.5 months. The time from the diagnosis of primary malignant solid tumor to diagnosis of CNS metastasis was 0-100 months, and the median time of metastasis was 18 months. The neuroimaging features of CNS metastasis were as follows:30 cases with brain metastasis, three cases with meningeal metastasis, and five cases with spinal cord involvement. Cerebrospinal fluid cytology was positive in two cases (2/3). Of the 33 patients with CNS metastasis, five underwent intracranial tumor resection combined with radiotherapy and chemotherapy, two underwent surgery alone, three underwent radiotherapy alone, and 23 underwent chemotherapy alone. After a median follow-up of 36 months, the survival time of 33 malignant solid tumors with CNS metastasis was (16.96±3.19) months, and the overall survival rate of three years was only 14.7%. The survival time of the patients with intracranial tumor surgery combined with radiotherapy and chemotherapy, surgery alone, radiotherapy alone, and chemotherapy alone was (40.50±4.32), (3.00±0), (35.50±5.50) and (10.35±2.84) months, respectively; and the three-year overall survival rates were 50.0%, 0, 50.0% and 4.3%, respectively (χ 2=15.19, P=0.002). The prognosis of the patients with intracranial tumor resection combined with radiotherapy and chemotherapy was the best. Conclusions:The highest incidence of CNS metastasis in children with malignant solid tumors in the chest and abdomen was found in the clear cell sarcoma of the kidney. The prognosis of CNS metastasis was found to be poor. But the comprehensive therapy of intracranial tumor resection combined with radiotherapy and chemotherapy can prolong survival and improve prognosis.

Objective:To summarize the prognostic characteristics of hepatoblastoma in children undergoing pulmonary metastasectomy.Methods:The clinical data of 48 hepatoblastoma patients with pulmonary metastases who underwent pulmonary metastases resection in our hospital from January 1 2009 to December 31, 2017 were collected, and prognosis of these patients was analyzed.Results:Forty eight cases were diagnosed as hepatoblastoma with pulmonary metastasis, including 30 cases with pulmonary metastasis alone and 18 cases accompany with other high risk factors affecting prognosis. All the 48 patients underwent pulmonary metastasectomy, in which 43 patients were given pulmonary metastases resection completely and 5 patients were given partial resection of metastases. Twenty-five cases recurred after the first pulmonary metastasis resection(25/48, 52.1%), and 2 cases progressed. With the median follow-up time of 45.5 months, survival time of 48 patients with pulmonary metastasectomy was(83.59±7.01)months, and the 3-year overall survival rate was 58%. Of the groups those were patients with pulmonary metastasis alone and patients with other high risk factors affecting prognosis, the survival time and the 3-year overall survival rate showed a significant statistical differences[(95.89±8.19)months vs.(58.95±9.40)months; 79.4% vs.50%; χ2=4.849, P= 0.028)]. The survival time and 3-year survival rate of the two groups between with recurrence or progression and the group without recurrence or progression after pulmonary metastases resection were showed a significant difference[(56.84±7.22)months vs.(112.48±7.08)months; 55.6% vs.90.5%; χ2=11.03, P= 0.001)]. Results of COX regression analysis showed that the main risk factor affecting prognosis was pulmonary metastasis accompany with other high-risk factors for hepatoblastoma patients. The death risk of patients with pulmonary metastasis accompany with other high-risk factors was 3.424 times than that of patients without other high-risk factors( RR=3.424, 95% CI: 1.264~9.275). Conclusion:The overall prognosis of patients with pulmonary metastases is not good, and the proportion of postoperative recurrence is very high. Pulmonary metastasis accompanied with other high risk factors is the main risk factor affecting the prognosis of patients with pulmonary metastasectomy.

Objective:To study the relationship between high-risk factors with the diagnosis, treatment and prognosis of children with high-risk head and neck rhabdomyosarcoma (RMS).Methods:Children with high-risk RMS primarily found in head and neck were selected as research objects according to the criteria of RMS risk degree formulated by Children Oncology Group (COG) and Children′s Rhabdomyosarcoma Cooperative Group, Children′s Hematological Oncology Group, China Anti-Cancer Association (CCCG-RMS), and combined treatment including puncture pathology/surgery, chemotherapy and radiotherapy were performed.The relationship with primary site, age, pathology type and metastasis and prognosis were analyzed.Results:Twenty-nine patients were collected as research object, 17 cases (58.6%) of male, and 12 cases (41.4%) of female.The primary site: orbit was 7 patients (24.1%), and para-meningeal was 22 patients (75.9%). Clinical stage: 2 cases were stage Ⅲ (6.9%), and 27 cases were stage Ⅳ (93.1%). Followed up to December 31, 2018, 14 patients were dead, 15 patients were survival, and 10 patients were event free survival.The overall survival rate was 48.3%(15/29 cases), and the event free survival rate was 34.5%(10/29 cases). According to Kaplan- Merier survival curve analysis, the average overall survival time was (76.0±12.0) months, 95% confidence interval (95% CI): (53.5-93.2)months.The event free survival time was (62.5±10.6) months, 95% CI: (47.0-83.9)months. Conclusions:The prognosis of high-risk head and neck RMS is poor.The center system metastasis is cause of death.Thus, the main task is how to close follow-up and prevention of central nervous system invasion for improving prognosis.