title: [Clinical features, treatment and prognosis of retinoblastoma in distant metastasis stage].
abstract: Objective:To summarize the clinical features, treatment and prognosis of retinoblastoma (RB) patients in distant metastasis stage.Methods:Retrospective case series study. Clinical data of 24 cases (30 eyes) with diagnosis of distant metastasis stage RB were collected in pediatrics department of Beijing Tongren Hospital affiliated with Capital Medical University, from September 2005 to December 2013. In these cases, including 11 male cases and 13 female cases, the treatment age ranged from 7 months to 9 years with the median of 26 months. There were 18 unilateral cases (12 cases of right eye and 6 cases of left eye) and 6 bilateral cases. All cases were treated with surgery, chemotherapy and radiotherapy. The adverse reaction, blood routine and biochemical routine were monitored dynamically and regularly during treatment. The clinical manifestations and auxiliary examination were combined to evaluate the therapeutic effect. Determining the prognosis with death and survival, the survival curve and the median survival time were calculated by the product limit method.Results:The results of pathologic examination of 13 cases showed 11 cases of optic nerve involvement, 6 cases of optic nerve and choroid involvement, 1 case of post laminar involvement, 1 case of cornea and conjunctiva involvement. The most common metastatic site was central nerve system (CNS) (23 cases), followed by orbital involvement (10 cases). Other metastatic sites included bone (9 cases), bone marrow (1 case), pleura (1 case) and lymph node (1 case). 23 patients with CNS metastasis underwent systemic chemotherapy and intrathecal injection. The toxicity and side effects of radiotherapy and chemotherapy were evaluated and classified as grade I-II. Those adverse effects were tolerated by all cases. Followed up to December 31, 2015, all patients with CNS metastasis died and the median survival time was 6 months. Only 1 patient without CNS metastasis disease-free survived for 64 months.Conclusions:The most common metastasis site of RB was CNS. The overall prognosis of RB with distant metastasis was poor, in spite of given combined treatment mainly including surgery, chemotherapy and radiotherapy. But the prognosis of RB patients without CNS metastasis in distant metastasis stage was better than with CNS metastasis.(Chin J Ophthalmol, 2017, 53: 121-126).
authors.affiliation: Department of paediatrics, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
authors.forename: H M
authors.initials: HM
authors.lastname: Hu
authors.forename: W L
authors.initials: WL
authors.lastname: Zhang
authors.forename: Y Z
authors.initials: YZ
authors.lastname: Wang
authors.forename: J T
authors.initials: JT
authors.lastname: Shi
authors.forename: B
authors.initials: B
authors.lastname: Li
authors.forename: Y
authors.initials: Y
authors.lastname: Zhang
authors.forename: J
authors.initials: J
authors.lastname: Li
authors.forename: Y
authors.initials: Y
authors.lastname: Wen
authors.forename: D S
authors.initials: DS
authors.lastname: Huang
mesh_terms: D016543:Central Nervous System Neoplasms / Q000556:secondary; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009900:Optic Nerve / Q000473:pathology; D011379:Prognosis; D019572:Retinal Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000628:therapy*; D012175:Retinoblastoma / Q000401:mortality* / Q000556:secondary* / Q000628:therapy*; D012189:Retrospective Studies
keywords: Neoplasm metastaasis; Prognosis; Retinoblastoma