1.Evaluation of 18F-fluorodeoxyglucose positronemission tomography/computed tomography imaging in childhood neuroblastoma chinical staging
Ying CHEN ; Xiaoli MA ; Xisi WANG ; Tong YU ; Mei JIN ; Wen ZHAO ; Qian ZHAO ; Chao DUAN ; Xingjun LI ; Liwei LI ; Dawei ZHANG
Chinese Journal of Applied Clinical Pediatrics 2017;32(15):1149-1152
Objective To explore the significance of 18 F-fluorodeoxyglucose positronemission tomography/ computed tomography (18F-FDG PET/CT) scanning for childhood neuroblastoma clinical staging.Methods From December 2014 to December 2015,59 patients confirmed as NB in Beijing Children's Hospital,Capital Medical University,and finding or clinical features were selected by histopathology.Those patients underwent 18 F-FDG PET/CT,bone scan,cranial magnetic resonance image (MRI),bone marrow puncture (two sites) and biopsy,regional CT,ultrasound,serum tumor markers like lactic dehydrogenase and neuronspecific enolase.In comparison of 18F-FDG PET/CT image findings with other detective methods,the differences were analyzed between the primary sites and metastasis were analyzed.Results (1) General features:Out of 59 children with NB,31 were males,28 were females.Primary lesions were as follows:14 cases located in the postmediastinum,44 cases in the retroperitoneum (1 case in retroperitoneum + postmediastinum),1 case in left neck.International Neuroblastoma Staging System Ⅰ to Ⅳ:O,1,9 and 49 cases,respectively.(2)18F-FDG PET-CT manifestations:The maximum of standardized uptake value (SUVmax):before-treatment group was (2.34 ± 1.06) which was larger than the post-surgery group value of (1.08 ± 0.50),and the difference was significant(F =5.699,P =0.000);bone marrow metastasis ranged from 1.5 to 2.9,regional lymphatic metastasis ranged from 1.0 to 2.1.(3)18F-FDG PET-CT imaging compared with other detection:in bone metastases,the whole body bone scan finding were normal in 3 cases,while PET/CT showed disseminated bone and bone marrow involvement and bone scanning showed 2 cases had metastases,combined with 18 F-FDG PET/CT,which were considered as postoperative inflammatory reaction or residual tumor tissues invade adjacent intervertebral foramen.Bone cytology was positive in 30 cases,while PET/CT showed 34 patients with bone marrow metastasis,and 1 case was suspicious.In central metastasis,1 case of PET/CT showed epidural metastasis,while cranial MRI was negative;Cranial MRI showed 9 cases had skull metastasis,1 case had orbital metastasis,1 case had meningeal metastases;while 18F-FDG PET/CT showed no abnormality.All children had no parenchymal metastasis.Corresponding tumor markers as LDH was related to the maximum value of primary tumor focal SUVmax (rs =0.581,P < 0.01).Conclusions 18 F-FDG PET/CT can fully display the distribution of primary sites and metastases,and can be served as medical imaging evidence for both the diagnosis and staging of neuroblastoma.But as for cranial bone and central metastasis,it has high false negative rate.Clinical practice should be combined with cranial MRI to improve relevance ratio.
2.Serum urea nitrogen and creatinine changes and its cIinicaI significance in chiIdren with retinobIastoma before and after chemotherapy
Huimin HONG ; Mei JIN ; Junyang ZHAO ; Chengyue ZHANG ; Wen ZHAO ; Xisi WANG ; Qian ZHAO ; Chao DUAN ; Yan SU ; Xiaoli MA
Chinese Journal of Applied Clinical Pediatrics 2019;34(3):183-187
Objective To analezk thk altkrations in blood urka nitrogkn( BRN)and skrum crkatinink( Scr) in childrkn with rktinoblastoma( Ab)bkfork and aftkr chkmothkrape and thk clinical significanck of thk chkmothkrape kffkct,and to providk thk kvidknck for thk furthkr improvkmknt of thk safkte of trkatmknt. Methods L total of 280 chil-drkn with Ab wkrk knrollkd in thk stude,and kach of thkm was trkatkd with CEV( Carboplatin+Etoposidk+Vincris-tink)mkthods. Thkrk subjkcts includkd 153 malks and 127 fkmalks,with a mkan trkatmknt of 4. 5 ceclks(rangk 2 to 12 ceclks)and a mkan agk of 21. 5 months(rangk 1 to 84 months). Lmong thkm,149 casks wkrk diagnoskd clinicalle,131 casks wkrk diagnoskd pathologicalle. Eight casks wkrk in thk kxtraocular stagk,3 casks wkrk in glaucoma and 269 casks in intraocular pkriod(101 casks of singlk keks and 168 casks of doublk keks). BRN and Scr wkrk dktkctkd bkfork thk first coursk of chkmothkrape and aftkr thk last coursk of chkmothkrape. ResuIts BRN and Scr valuks wkrk analezkd bk-fork and aftkr chkmothkrape. BRN was 3. 05 mmol╱F bkfork chkmothkrape and 3. 46 mmol╱F aftkr chkmothkrape in thk group agkd from 4 months to lkss than 12 months(73 casks),thk valuks of BRN aftkr chkmothkrape was highkr than that bkfork chkmothkrape,and onle in this group thk changk was statisticalle diffkrknt(t﹦ -2. 829,P﹦0. 006),but all BRN valuks in this group wkrk not bkeond thk highkst rkfkrknck valuk(1. 70 mmol╱F-7. 10 mmol╱F). Bkfork initial chkmothkrape,149 patiknts( 53. 2﹪)had Scr bklow thk rkfkrknck rangk( malk:30 -104 μmol╱F,fkmalk:30 -84 μmol╱F),and 20 casks(7. 0﹪)had thk BRN bklow thk rkfkrknck valuk. In 2 casks,BRN(7. 25 mmol╱F and 7. 34 mmol╱F, rkspkctivkle)bkfork thk initial chkmothkrape was slightle highkr than thk normal valuk,but thk valuk was normal(5. 01 mmol╱F and 4. 98 mmol╱F,rkspkctivkle)aftkr thk last chkmothkrape. In onk cask,thk BRN(5. 62 mmol╱F)was normal bkfork thk initial chkmothkrape,but it was klkvatkd(7. 33 mmol╱F)aftkr thk last chkmothkrape. In anothkr onk cask,thk BRN was normal bkfork and aftkr chkmothkrape,but thk valuk aftkr chkmothkrape was 4. 69 timks highkr than that bk-fork chkmothkrape. ConcIusions Aknal function of Ab childrkn bkfork trkatmknt is normal. Skvkn pkrcknt of thksk patiknts havk BRN undkr thk BRN rkfkrknck rangk,and 53. 2﹪ of thksk patiknts havk Scr undkr thk Scr rkfkrknck rangk. It suggkstkd that thk rkfkrknck valuks of BRN and Scr nkkd to bk adjustkd. BRN of infant Ab mae incrkask signifi-cantle aftkr chkmothkrape,but it doks not mkkt thk currknt diagnostic critkria of mild nkphrotoxicite. Still,thk karle rknal damagk nkkds to bk notickd.
3.AnaIysis of cIinicaI characteristics and prognosis of chiIdren of Iess-than-2-year oId with rhabdomyosarcoma
Qian ZHAO ; Mei JIN ; Dawei ZHANG ; Wen ZHAO ; Xisi WANG ; Chao DUAN ; Xiaoli MA
Chinese Journal of Applied Clinical Pediatrics 2019;34(3):192-195
Objective To summarizk clinical charactkristics,thkrapkutic kffkct and prognosis of rhabdomeosar-coma(AMS)childrkn of lkss-than-2 -ekar old and its rklatkd factors,to improvk thk awarknkss of this agk group with AMS. Methods Thk clinical information and thkrapkutic kffkct of 20 AMS patiknts of lkss-than-2-ekar old wkrk diagnoskd and trkatkd at thk hkmatologe -oncologe ckntkr in Bkijing Childrkn's Hospital( BCH),from Januare 2012 to Lpril 2017,and thk clinical data wkrk analezkd rktrospkctivkle. Lccording to thk intkrnational critkria for thk trkatmknt of solid tumors in childrkn,thk patiknts wkrk dividkd into a complktk rkmission group and a progrkss╱rklapsk group,and thk risc factors affkcting thk prognosis wkrk analezkd. ResuIts Twknte casks of lkss-than-2 ekar old chil-drkn with AMS wkrk collkctkd,accounting for 12. 4﹪ of all AMS during thk samk pkriod in BCH hkmatologe-oncologe ckntkr. Thk mkdian follow-up timk was(16. 1 ± 1. 8)months,5 patiknts got progrkss╱rklapsk at 9-12 months during trkatmknt and 2-3 months aftkr thkrape finishkd. Thk ratk of 2 -ekar-kvknt-frkk survival( EPS)ratk and ovkrall survival(OS)ratk wkrk 48﹪ and 61﹪,rkspkctivkle. Thk 2-ekar EPS ratk of kmbreonal AMS childrkn was significant-le highkr than that of alvkolar AMS childrkn,and thk diffkrknck was statisticalle significant(χ2 ﹦0. 854,P﹦0. 034). In thk progrkss╱rklapsk group and thk childrkn with complktk rkmission childrkn,factors lick boe,kmbreonal tepk,primare tumor location of not bad,and of mkdium risc might rkduck thk risc of progrkss╱rklapsk of AMS,whilk tumor diamktkr >5 cm and clinical stagk Ⅳ might incrkask thk risc of progrkss╱rklapsk of AMS,but thk diffkrknck was not statisticalle significant(P>0. 05). ConcIusions Thk prognosis of AMS childrkn lkss-than-2-ekar old was vkre poor,and thk ratks of 2 ekars EPS and OS ratks wkrk lowkr than ovkrall indkx of childrkn with AMS. Thksk patiknts nkkd to undkrgo a varikte of trkatmknt mkthods flkxible,including dklaekd radiothkrape and 125 I radioactivk skkds brachethkrape,to improvk thkrapkutic kfficace and long-tkrm outcomks.
4.Clinical study on 48 children with head and neck rhabdomyosarcoma treated by multi-disciplinary therapy
Chao DUAN ; Shengcai WANG ; Mei JIN ; Dawei ZHANG ; Wen ZHAO ; Xisi WANG ; Qian ZHAO ; Jun TAI ; Jie ZHANG ; Jianguo ZHANG ; Lejian HE ; Xin NI ; Xiaoli MA
Chinese Journal of Applied Clinical Pediatrics 2019;34(7):529-533
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.
5.Analysis of the initial symptoms and its diagnostic significance in children with neuroblastoma
Xindi WANG ; Chao DUAN ; Dawei ZHANG ; Mei JIN ; Wen ZHAO ; Xisi WANG ; Qian ZHAO ; Xingjun LI ; Cheng HUANG ; Sihui LI ; Ying CHEN ; Xiaolu NIE ; Xiaoxia PENG ; Xiaoli MA
Chinese Journal of Applied Clinical Pediatrics 2019;34(5):359-363
Objective To explore the significance of the initial clinical symptoms and clinical manifestations of neuroblastoma(NB)to achive early identification of NB. Methods A retrospective study was performed on patients diagnosed with NB,who attended the Hematology Oncology Center,Beijing Childrenˊs Hospital from March 31st ,2007 to March 31st,2015. The clinical characteristics were compared between the children 〈1_year_old and≥1_year_old. The result was analyzed to compare the difference in clinical symptoms and tumor biologic characteristics of patients with different initial clinical symptoms between 2 groups. Results A total of 330 patients were included in the study,43 of them were younger than 1 year old,and their most common symptoms were cough and fever( each 17 cases,and accounted for 39. 5%,respectively);while the most common symptoms in patients≥1_year_old(287 cases)was fe_ver(177 cases,61. 7%),followed by lymphadenopathy(107 cases,37. 3%),bone pain(97 cases,33. 8%)and anemia (48 cases,16. 7%). The frequency of symptoms differed significantly between 2 groups(all P〈0. 05),such as fever (39. 5% vs. 61. 7%,χ2 ﹦6. 68),anemia(4. 7% vs. 16. 7%,χ2 ﹦6. 00),bone pain(0 vs. 33. 8%,χ2 ﹦18. 99),abdo_minal pain(0 vs. 25. 3%,χ2 ﹦10. 19),diarrhea(16. 3% vs. 3. 0%,χ2 ﹦12. 73),lymphadenopathy(7. 0% vs. 37. 3%, χ2 ﹦14. 12)and anorexia(9. 3% vs. 33. 4%,χ2 ﹦9. 21). Datients had fever,anemia,lymphadenopathy,cutaneous hemorrhagic spot or periorbital ecchymosis,bone pain,abdominal pain,exophthalmos,and anorexia early in the initial course of the disease,whose serum lactate dehydrogenase values were significantly increased(P〈0. 05). Datients suf_fered from fever,anemia,lymphadenopathy,bone pain,limbs dysfunctions,abdominal pain at the beginning of the disease,whose urine vanillymandelic acid values were higher than normal(P〈0. 05). Conclusions The study of sympto_mology suggests the most common symptoms in patients with NB 〈1_year_old are cough and fever,while those ≥1_year_old are fever,lymphadenopathy,bone pain,and anemia. Por patients with symptoms mentioned,carrying out the necessary NB_diagnose_related laboratory and imaging studies was statistically relevant to patientsˊ ages(〈1_year_old and≥1_year_old),which may contribute to earlier identification and diagnosis of NB.
6.Related factors of intestinal necrosis caused by midgut volvulus in neonates
Xisi GUAN ; Zhe WANG ; Qiuming HE ; Haiqing ZHENG ; Wei ZHONG ; Jiakang YU
Chinese Journal of Neonatology 2022;37(3):225-228
Objective:To study the characteristics and related factors of neonatal intestinal necrosis caused by midgut volvulus.Methods:We retrospectively analyzed the clinical data of neonates with midgut volvulus who were admitted to Guangzhou Women and Children's Medical Center, from January 2009 to December 2019 and confirmed by surgery. The cases with intestinal necrosis belong to the intestinal necrosis group, and those without intestinal necrosis, the non-intestinal necrosis group which was randomly sampled at a ratio of about 4∶1 to the number of cases in the intestinal necrosis group. The two groups were compared in terms of personal history, age of onset, initial symptoms, vital signs within 2 h after admission, time from symptom onset to operation, clinical outcome, laboratory indicators within 2 h after admission, etc. Multivariate Logistic regression analysis was used to screen the related factors of intestinal necrosis in midgut volvulus. The effective warning indexes are screened by receiver operating characteristic (ROC) curve.Results:(1) Among 231 cases of midgut volvulus, 21 cases (9.1%, 21/231) had intestinal necrosis at the time of operation, 87 cases were included in the non-intestinal necrosis group. (2) The levels of heart rate within 2 h after admission, mean arterial pressure, WBC, C reactive protein (CRP), blood glucose and potassium in intestinal necrosis group were significantly higher than those in non-intestinal necrosis group ( P<0.05). Admission days of age, hemoglobin, serum albumin, serum sodium, pH and BE levels were significantly lower than those in the group without intestinal necrosis ( P<0.05). (3) In the multivariate analysis, increased heart rate, mean arterial pressure, serum CRP, and decreased serum sodium, serum albumin, and pH levels were predictors related to intestinal necrosis in patients with midgut volvulus. (4) The area under the ROC curve (AUC) of CRP was 0.883, the cutoff value was 9.88 mg/L, the sensitivity was 76.2%, and the specificity was 94.3%. The ROC curve of serum albumin was 0.792, the cut-off value was 36.65 g/L, the sensitivity was 70.1%, and the specificity was 94.3%. Conclusions:Heart rate, mean arterial pressure, increased CRP, decreased serum sodium, serum albumin and pH are helpful to predict whether intestinal necrosis occurs in midgut volvulus, and CRP > 9.88 mg/L and serum albumin < 36.65 g/L are likely warning indicators.
7.Analysis of clinical features and prognostic factors of neuroblastoma with bone metastasis in infants
Sidou HE ; Shihan ZHANG ; Chiyi JIANG ; Zhixia YUE ; Libing FU ; Tong YU ; Xisi WANG ; Mei JIN ; Yan SU ; Xiaoli MA
Chinese Journal of Applied Clinical Pediatrics 2022;37(10):758-762
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.
8.Programmed management in prenatal diagnosed pyriform sinus fistula: analysis of eight cases
Xinhao ZENG ; Qiuming HE ; Wei ZHONG ; Zhe WANG ; Bin YAN ; Xisi GUAN ; Xiaoli XIE ; Jiakang YU
Chinese Journal of Perinatal Medicine 2021;24(10):734-740
Objective:To evaluate the clinical effect of applying the programmed management procedure in the prenatal diagnosis of pyriform sinus fistula(PSF).Methods:This study retrospectively enrolled eight fetuses with PSF who were managed according to the programmed management procedure for prenatal diagnosis of PSF, which was established in January 2016, in Guangzhou Women's and Children's Medical Center from January 2016 to October 2020. The procedure consisted of the detection of fetal neck cysts by prenatal ultrasound followed by further confirmation by MRI, evaluation of the degree of airway compression, indwelling gastric tube after birth, no oral feeding, complement of CT/MRI, and surgical treatment within a limited time after necessary preoperative examination. The prenatal diagnosis, postnatal treatment, and follow-up were summarized using descriptive analysis.Results:(1) Prenatal: The gestational age at the first detection of cervical cysts by prenatal ultrasound was (27.1±4.1) weeks and all the cysts were located on the left side. Prenatal MRI indicated that the largest cysts was (32.0±12.2) mm in diameter, and the tracheal transit index was (10.9±2.8) mm. (2) After birth: Among the eight children, five were males and three were females, with the gestational age of (38.0±0.9) weeks and birth weight of (3 020±459) g. One case was intubated during labor due to a intrauterine tracheal transposition index of 17.4 mm. All infants were not allowed for oral feeding. The median age at CT/MRI examination was 2.5 d (1-8 d), which revealed that the maximum diameter of the cysts was (40.6±6.9) mm and visible air bubbles in all cysts. The infection index before operation was not high and the age at operation was (8.6±2.3) d. All cysts were completely removed and the PSFs were ligated at a higher position, with the average operative duration of (95.0±19.6) min, and the postoperative duration of mechanical ventilation and hospitalization of 5 h (3-71 h) and (8.8±1.0) d, respectively. No complications such as hoarseness were reported. During the follow-up of 4 to 58 months through outpatient clinic and telephone, no recurrence were observed.Conclusions:The programmed management procedure can provide guidance for postnatal treatment of patients with a prenatal diagnosis of PSF, and help to achieve a successful treatment result.
9.Cause of death of children with intraocular retinoblastoma in the Beijing Children's Hospital 2009-2017
Shihan ZHANG ; Xiaoli MA ; Chengyue ZHANG ; Dayong BAI ; Yanhui CUI ; Yan SU ; Dawei ZHANG ; Xisi WANG ; Chao DUAN ; Junyang ZHAO ; Mei JIN
Chinese Journal of Ocular Fundus Diseases 2022;38(3):182-186
Objective:To observe and analyze the clinical characteristics of children who died of intraocular retinoblastoma (RB).Methods:A retrospective clinical study. Fourteen children (23 eyes) with intraocular RB who died after receiving treatment in Beijing Children's Hospital from 2009 to 2017 were included in the study. Among the children, there were 7 males (10 eyes) and 7 females (13 eyes); 5 had unilateral and 9 had bilateral tumor. Age were 17.2±15.5 months. All children underwent RetCam examination. RB was staged according to the international intraocular RB classify. Among the 23 eyes, 1 eye was in stage B, 2 eyes were in stage C, 12 eyes in stage D, and 8 eyes in stage E. Treatment methods included a systemic (vincristine, etoposide and carboplatin) chemotherapy (VEC chemotherapy), enucleation surgery, and vitrectomy. The basic conditions including age, time of diagnosis, pathological diagnosis, treatment and main causes of death were retrospectively analyzed.Results:Among the 14 cases, the first symptom was leukemia in 12 cases, red eye in 1 case, and squintin in 1 case. Systemic VEC chemotherapy was used for 1-6 courses of treatment; 5 cases were enucleated, 3 cases underwent histopathological examination; 3 cases were treated with vitrectomy. Among the 3 cases who underwent histopathological examination, the sclera and optic nerve, optic nerve and optic disc were invasted respectively. Seven patients died of tumor metastasis and/or intracranial lesions (50.0%, 7/14); the median survival time was 19 months. Four patients died of treatment (28.6%, 4/14), including 3 patients died of chemotherapy-related side effects, and 1 died of organ failure after enucleation surgery (7.1%); the median survival time was 3.5 months. Early abandonment of treatment died in 3 cases (21.4%, 3/14); the median survival time was 15 months.Conclusion:Intracranial metastasis is the main cause of death in children with intraocular RB.
10.Clinical analysis and prognosis of children with neuroblastoma with pancreatic infiltration
Xi CHAI ; Cheng HUANG ; Sihui LI ; Ying CHEN ; Xindi WANG ; Wen ZHAO ; Xisi WANG ; Qian ZHAO ; Zhixia YUE ; Xiaoman WANG ; Hong QIN ; Xiaoli MA
Chinese Journal of Applied Clinical Pediatrics 2018;33(7):523-527
Objective To summarize the clinical data and characteristics of neuroblastoma (NB) with pancreatic infiltration and to assess the clinical features and the prognosis of NB.Methods According to NB protocol at Beijing Children's Hospital,Capital Medical University(BCH-NB-2007),based on Hong Kong NB protocol,the patients were divided into 3 groups of low-risk (LR) group,medium risk (MR) group and high-risk (HR) group.All children were followed up till March 31,2017.Diagnosis of pancreatic infiltration of NB was made by abdominal enhancement of CT,enhanced magnetic resonance imaging (MRI) or 18-fluorodeoxyglucose-positron emission tomography-computed tomography(18F-FDG-PET/CT),any of which could suggest NB pancreatic infiltration or postoperative pathology prompted NB to infiltrate the pancreas.Retrospective summary and analysis of indicators were performed,which included the initial diagnosis of primary tumor and metastatic tumor site,tumor markers,clinical stage,risk group,imaging features and treatment.Results (1) Totally 50 eligible patients were included:27 females,23 males,median age of 33 months (7-129 months),10 cases ≤ 18 months,40 cases > 18 months;3 cases were of International Neuroblastoma Staging System(INSS)-Ⅲ,47 cases of INSS-Ⅳ;2 caes of LR,3 cases of MR,45 cases of HR;28 cases had a fever,27 cases with abdominal mass,14 cases with abdominal pain,9 cases with limb pain,5 cases with vomiting,4 cases with diarrhea,and 1 case with jaundice.Forty-nine cases of primary tumor were located in the retroperitoneal adrenal gland,and 1 case in the pelvic cavity.Thirty-two cases had tumor diameter≥ 10 cm.(2)Tumor markers and imaging features:the median serum lactate dehydrogenase (LDH) value in 50 cases was 669 U/L (263-6 762 U/L),of them 19 cases > 1 000 U/L.A total of 80% cases had neuron specific enolase (NSE) > 0.15 ng/L.Nine cases had elevated amylase (AMY),and 7 cases had elevated lippase (LPS),and all the levels were elevated in 5 cases.A total of 41 cases had pancreas infiltration by abdominal ultrasound,44 cases had pancreas infiltration by abdominal enhancement computed tomography (CT),100% (14/14 cases)of pancreas infiltration was confirmed by abdominal reconstruction enhancement nuclear imaging MRI,and NB pancreas infiltration was proved in 41.3% (19/46 cases) by 18F-FDG-PET/CT.Comparison of the above 4 imaging studies:one imaging examination index was positive in 7 cases,accounting for 14.0%,2 positive in 26 cases,accounting for 52.0%,3 positive in 15 cases,accounting for 30.0%,and 4 positive in 2 cases,accounting for 4.0%.(3) Treatment outcomes:totally 50 cases received treatment,including 2 cases of LR,all cases were of INSS-Ⅲ,and 1 case with complete remission (CR).Three cases of MR belonging to INSS-Ⅳ had complete resection of the tumor,1 case had recurrence and died,and the other two were stable.Forty-five cases with HR,median follow-up lasting for 15 (4-53) months,16 cases had occurrence (35.6%),3 cases were relapsed after stopping treatment for 2,3,18 months,respectively;tumor progressed in 12 patients during treatment,and 1 case got severe intracranial infection and gave up treatment before death.Kaplan-Meier analysis showed the expected 3-year event free survival(EFS) rate was 22.1%,and 3-year overall survival(OS) rate was 38.5%.Conclusions Preliminary results show that 90% with pancreatic infiltration of NB belong to Ⅳ HR group of children,and almost primary tumor is almost located in the retroperitoneal ragion.NB with pancreatic infiltration clinical manifestations is hidden and nonspecific.More than half of the children have no obvious abdominal pain or vomiting,and so imaging examination is needed to determine the situation of pancreatic metastasis further.Abdominal reconstruction enhancement MRI has a high sensitivity and specificity for pancreatic metastatic lesions,which can be used as the basis for early diagnosis.The overall prognosis is poor.The expected 3-year EFS rate can be 22.1%,3-year OS rate was 38.5%.