A long follow-up of a patient with bilateral retinoblastoma who underwent globe-preserving therapy and had metastasis after 72mo

I am Dr. Yi Zhang, from the Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University,Beijing, China. I would like to describe the metastasis in the left parotid and cervical lymph nodes of a patient with bilateral retinoblastoma 72mo after the initial successful globe-preserving therapies, which included chemotherapy, laser photocoagulation, and cryotherapy.

Natural killer/T-cell lymphoma with intracranial infiltration and Epstein-Barr virus infection: A case report

BACKGROUND Because of atypical clinical symptoms,lymphoma is easily confused with infectious diseases.Extranodal nasal-type natural killer/T-cell lymphoma(NKTL)is more common,and there are few cases of eyelid site onset and intracranial infiltration,which increases the difficulty of diagnosis.This disease usually has a very poor prognosis and there are few reports of recovery.CASE SUMMARY A 3-year-old boy was admitted to our hospital due to an initial misdiagnosis of"eyelid cellulitis"and failed antibiotic treatment.He was characterized by fever,right eyeball bulging,convulsions,and abnormal liver function.His blood Epstein-Barr virus(EBV)DNA was positive(8.798×10^4 copies/mL),and remained positive for about half a year.The cranial imaging examination suggested a space-occupying lesion in the right eyelid,with the right temporal lobe and meninges involved.The boy underwent ocular mass resection.The pathological diagnosis was NKTL.He was diagnosed as having NKTL with intracranial infiltration,combined with chronic active EBV infection(CAEBV).Then he underwent systemic chemotherapy and intrathecal injection.The boy suffered from abnormal blood coagulation,oral mucositis,diarrhea,liver damage,and severe bone marrow suppression but survived.Finally,the tumor was completely relieved and his blood EBV-DNA level turned negative.The current follow-up has been more than 2 years and his condition is stable.CONCLUSION This case suggests that chemotherapy combined with intrathecal injection may have a good effect on intracranial infiltrating lymphoma and CAEBV,which deserves further study and discussion.

Clinical analysis of 2790 children with retinoblastoma: a single-center experience in China

Background In this study,we aimed to analyze the clinical characteristics and prognosis of children with retinoblastoma(RB)in a single center in China with a large sample collection spanning 17 years.Methods The clinical data of 2790 children with RB treated in Beijing Tongren Hospital from 2005 to 2021 were collected,and a retrospective analysis was conducted.Results The median age of the participants was 28.3 months.There were 3624 affected eyes,12.4%of which were in groups A–C,67.1%in groups D–E and 16.2%were not specified.The primary symptom observed in most cases was a white pupil,accounting for 66.5%,followed by strabismus(12.8%).The median follow-up time was 59.7 months.The enucleation rate was 71.3%(703/986)in a single left eye and 72.5%(702/968)in a single right eye.The overall survival(OS)rate was 95.8%(2444/2552)because 237 patients dropped out,and 109 died.Kaplan‒Meier survival analysis showed that the median survival time(MST)was 125.92 months[95%confidence interval(CI)=124.83–127.01].Cox multivariate survival analysis showed that trilateral RB(P=0.017),metastasis site(P=0.001),and combined distant tissue metastasis(P=0.001)were independent prognostic factors for RB.The OS of 44 cases of familial RB was 93.2%(41/44),with an MST of 80.62 months(95%CI=67.70–93.54).Conclusions The timing of eye protection treatment and enucleation should be comprehensively judged to avoid worsening prognosis due to operation time delay.More importantly,the promotion and popularization of diagnosis and treatment technologies are necessary to further improve RB prognosis.

Report of seven children with undifferentiated embryonal sarcoma of the liver

Undifferentiated embryonal sarcoma of the liver (UESL) very rarely presents clinically.Approximately 1% to 4% of all solid tumors in children are malignant liver tumors,and UESL accounts for 9% to 15% of the liver tumor.The onset of UESL usually occurs at 6 to 10 years of age and there is no obvious difference based on sex.[1] The first symptoms of UESL are abdominal distention and abdominal pain without jaundice,while a physical examination commonly reveals no positive vital signs except the palpable liver tumor.[1] The serum alphafetoprotein (AFP) test is not sensitive for UESL.[2] Preoperative diagnosis of UESL is very difficult.

Detection of Subclinical Anthracyclines＇ Cardiotoxicity in Children with Solid Tumor

Background： Cardiotoxicity is one of the most serious chronic complications ofanthracyclines therapy. Assessment of the left ventricular ejection fraction （LVEF） fails to detect subtle cardiac dysfunction of left ventricular （LV）. This study aimed to detect and evaluate new parameters of subclinical anthracyclines＇ cardiotoxicity in children with solid tumor. Methods： A detailed echocardiographic examination was performed in 36 children with hepatoblastoma or rhabdomyosarcoma after receiving anthracyclines＇ chemotherapy and 36 healthy controls from January 2015 to December 2016. The LVEF, ratio of early diastolic peak velocity of transmitral flow （E） and septal diastolic e＇ mitral annular peak velocity （e＇）, tricuspid annular plane systolic excursion （TAPSE）, and LV global longitudinal strain （GLS） were evaluated using M-mode, tissue Doppler imaging （TDI）, and two-dimensional speckle tracking echocardiography （2D-STE）, respectively. Echocardiographic parameters were compared between patient group and healthy controls. All patients were divided into two subgroups based on their anthracyclines＇ cumulative dosage （〈300 mg/m^2 subgroup and ≥300 mg/m^2 subgroup）. Results： All patients had no presentation of heart failure and LVEF within normal range （65.7 ± 5.1%）. Compared with healthy controls, the mean E/e＇ increased significantly （7.9 ±0.7 vs. 10.2 ± 3.5, t = 3.72, P 〈 0.01 ）, mean TAPSE decreased significantly （ 17.2 ± 1.3 mm vs. 14.2 ± 3.0 mm, t = -4.03, P 〈 0.01）, and mean LV GLS decreased significantly （-22.2% ± 1.9% vs. -17.9% ± 2.9%, t = -5.58, P 〈 0.01） in patient group. Compared with subgroup with anthracyclines＇ cumulative dosage 〈 300 mg/m^2, mean LV GLS decreased significantly （- 18.7 ＋ 2.7% vs. - 16.5 ~ 2. 1%, t = 2.15, P = 0.04）, the mean E/e＇ increased significantly （9.1 ±1.5 vs. 11.5 ± 4.9, t = -2.17, P = 0.04）, and mean TAPSE decreased significantly （14.2±2.1 mm vs. 12.5±2.2 mm, t = -2.82, P = 0.02） in subgroup with anthracyclines＇ cumulative dosage 〉300 mg/m^2. Conclusions： LV GLS is helpful in the early detection of subclinical LV dysfunction using 2D-STE. E/e＇ and TAPSE are other sensitive parameters in detecting subclinical cardiac dysfunction of both ventricles by TD1. These parameters show significant change with different anthracyclines＇ cumulative dosage, so cumulative dosage should be controlled in clinical treatment.

Perfect prognosis of a boy with alveolar rhabdomyosarcoma of the nasal wing treated with brachytherapy and chemotherapy

To the Editor:Alveolar rhabdomyosarcoma(ARMS)of nasalwingwith distant metastasis has a very poor prognosis.Because it is difficult to achieve complete surgical excision of the primary site of the nasal wing,distant metastasis is not easy to control.Here,we present an unusual case of a 29-month-old boy with ARMS in the left nasal wing accompanied by bone metastasis who was treated with systemic chemotherapy as well as 125I interstitial brachytherapy,and this special multidisciplinary management resulted in a perfect prognosis and mild long-term side effects.