| Background and purposeThe tissue differentiation of rhabdomyosarcoma originates from primitive mesenchymal stem cells.Rhabdomyosarcoma is the most frequently occurring soft tissue sarcoma in children patients,which accounts for about 5%of all childhood malignant tumors.Orbital rhabdomyosarcoma is the most common primary orbital malignant tumor in children with a high degree of malignancy.At present,the comprehensive treatment of surgical resection,radiotherapy and chemotherapy has become the main treatment plan for orbital rhabdomyosarcoma.Radiotherapy has been used to treat orbital rhabdomyosarcoma for decades.It has undergone continuous improvement from the original traditional irradiation mode.External radiation technologies such as three-dimensional conformal radiation therapy,intensity-modulated radiation therapy,image-guided radiation therapy,and proton radiation therapy have been applied successively in the clinic.Although the 5-year survival rate of children with orbital rhabdomyosarcoma has been gradually improved,and the current external irradiation radiotherapy technology has also greatly reduced the radiotherapy complications brought to children patients with orbital rhabdomy osarcoma,it has caused serious orbital and facial malformations which affects the quality of life of these children patients.Radioactive 125I seeds implantation brachytherapy has been widely used in children’s head and neck rhabdomyosarcoma in recent years based on its unique radiotherapy mode.However,there were few reports on the treatment of orbital rhabdomyosarcoma,and there were very few records about complications of eye and the periorbital area.The purpose of this study is to observe the efficacy and complications of 125I seeds implantation brachytherapy in the comprehensive treatment of pediatric orbital rhabdomyosarcoma,and to provide references for the clinical application of 125I seeds implantation brachytherapy in the comprehensive treatment of pediatric orbital rhabdomyosarcoma.Methods1 Study subjectsIn this retrospective study,the clinical data of 8 children with orbital rhabdomyosarcoma treated at the First Affiliated Hospital of Zhengzhou University from January 2013 to January 2017 were collected.These children were not diagnosed in other hospitals.The inclusion criteria:①children less than 14 years old;②orbital was the primary focus,and the tumor did not invade the tissue around the orbit and the brain;③did not receive standard treatment(surgery,chemotherapy and radiotherapy).Exclusion criteria:①non-children over 14 years old;②orb it was not the primary focus,or the tumor has invaded the tissue around the orbit and the brain;③has received standard treatment(surgery,chemotherapy and radiotherapy).This study followed the requirements of the Declaration of the World Medical Association Helsinki and was approved by the Ethics Committee of the First Affiliated Hospital of Zhengzhou University.The implementation of the seeds implantation operation obtained the informed consent of the guardians of the children.2 Treatments2.1 Comprehensive treatmentsAll children underwent anterior segment,visual acuity,fundus examination,B-ultrasound,orbital CT and MRI to evaluate the conditions of the eyes with orbital rhabdomyosarcoma.According to the preoperative imaging examination,surgical resection first if the tumor could be completely removed;pathological examination and clear diagnosis first if the tumor could not be completely removed,neoadjuvant chemotherapy should be applied before surgical resection.Corresponding chemotherapy was performed according to the risk grouping criteria established by the United States Children’s Oncology Collaborative Group(COG).Total chemotherapy is no less than 8 cycles(treatment cycle+consolidation cycle).The criteria for termination of chemotherapy:if there were no obvious tumor traces showed on the imaging examinations to evaluate control situation of the tumor during chemotherapy,then chemotherapy for the next 4 cycles to consolidate.All children underwent 125I seeds implantation brachytherapy as planned 1 month after surgical resection.The arrangement of seeds was planned according to the preoperative orbital CT examination.Postoperative CT examination was performed to verify that the seeds implantation was as expected.If the seeds were not in place,or if a local(suspected)recurrence of the tumor was found during follow-up,additional seeds implantation was feasible.2.2 Follow up and observation of complicationsThe follow-up started as the pathological diagnosis was confirmed,and the curative effects and radiation complications were observed.Monthly reexamine within 6 months after surgical resection and/or 125I seeds implantation.Reexamine once every 3 months between 6 months and 3 years.Reexamine once every 6 months in the 4th year.Reexamine once a year for more than 5 years.Orbital CT or MRI was reexamined every 3 months within 1 year,and was reexamined every 6 months within 1 to 4 years,and also was reexamined once a year for more than 5 years.Complications of 125I seeds implantation brachytherapy include puncture complications,the short-term complications of radiotherapy(less than 6 months),and the long-term complications of radiotherapy(after 6 months).Results1 Study subjectsThere were 4 males and 4 females,with an average age of 7.5 years(3 months to 12 years).All children were first diagnosed by surgical resection and pathological examination.According to the histopathological classification standard developed by WHO:there were 6 cases with embryonal type and 2 cases with acinar type.1 case was in stage Ⅰ,3 cases were in stage Ⅱ,and 4 cases were in stage Ⅲ according to the Intergroup Rhabdomyosarcoma Study Group(IRSG)staging.Risk groups:6 cases in the low-risk group and 2 cases in the medium-risk group.2 TreatmentsSurgical resection:tumors of 4 cases were completely removed cleanly with the naked eye in the surgeries,and the others were not.Chemotherapy:7 cases achieved the chemotherapy as planned,however,1 case did not.6 cases in the low-risk group received alternate chemotherapy of VCP(vincristine+cyclophosphamide+cisplatin)and IEV(ifosfamide+Etoposide+vincristine);and 2 cases in the middle-risk group received alternate chemotherapy of AVCP(adriamycin+vincristine+cyclophosphamide+cisplatin)and IEV.Seeds implantation:4 cases underwent seeds implantation at 1 month after the surgical resection,and the others underwent seeds implantation when local signs of recurrence appeared.5 cases underwent seeds implantation only once,of which one died of brain metastasis although there was no local sign of recurrence appeared.2 cases underwent twice seeds implantation,and 1 case underwent seeds implantation for three times.The 4-year tumor-free survival rate of the 7 cases who completed the comprehensive treatment was 100%,and the median follow-up time was 48 months(37～66 months).1 case did not complete comprehensive treatment.Although the orbit was locally stable and controlled,the child eventually died of brain metastasis.3 ComplicationsPuncture complications:1 case of puncture tract bleeding.Early radiotherapy complications(≤6 months):corneal irritating symptoms in 5 cases,eyelashes missing in 5 cases,local skin pigmentation in 2 cases;late radiotherapy complications(>6 months):visual loss in 5 cases,ocular surface neovascularization in 5 cases,subcutaneous atrophy and sclerosis in 4 cases,corneal irritating symptoms and local skin pigmentation in 3 cases,eyelashes missing and neovascular glaucoma in 2 cases,corneal ulcer and perforation in 1 case.All children had no radioactive cataract,ischemic retinal lesions,atrophy of the eyeballs,and orbital and peripheral facial hypoplasia.Of the 7 surviving children,6 eyes remained intact and visual acuity was not affected in 3 cases.Conclusion125I seeds implantation radiotherapy has a good effect on children’s primary orbital rhabdomyosarcoma.It does not affect the development of the orbit and the surrounding facial bones.It is very suitable for children’s orbital rhabdomyosarcoma,but it is easy to cause neovascular lesions,especially for younger children and more implanted seeds are more likely to cause neovascular glaucoma,and further observation is needed. |
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