ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
The gene of bromodomain PHD finger transcription factor (BPTF) is expressed in all tissues and its protein regulate the transcription process.Overexpression and mutations of BPTF are found in a variety of tumors,which demonstrate poor prognosis.It is also reported that BPTF plays an important role in the development of childhood malignancy.The study aims to summarize the latest studies of tumors in children associated with BPTF.