Clinical,pathological and radiological features of retinoblastoma with central nervous system metastasis / 中华实用儿科临床杂志

ABSTRACT

Objective To summarize the clinical,pathological and radiological features of retinoblastoma (RB)with central nervous system (CNS)metastasis.Methods Twenty -three patients were confirmed to have RB with CNS metastasis in Beijing Tongren Hospital from December 2005 to December 201 3,and their clinical data were retrospectively analyzed.Results (1 )The incidence of RB with CNS metastasis was 1 .83% (23 /1 260 cases),and the incidence of CNS metastasis was 7.64%(1 1 /1 44 cases)if RB with optic nerve involvement which was confirmed through pathology.At first visit 1 0 cases had a confirmed diagnosis of RB with CNS metastasis,while the other 1 3 cases had a diagnosis of RB in extraocular stages.(2)Eleven patients with RB in extraocular stages had the pathological evi-dence of optic nerve involvement which was pathologically confirmed,6 patients had optic nerve involvement with cho-roid involvement,2 patients had optic nerve involvement with nerve sheath involvement.(3)All the patients whose diagnosis of CNS metastasis had the radiological evidence of CNS metastases,and 1 case had autopsy and pathological evidence for extensive brain metastases.The main radiological feature of CNS metastasis was meninges metastasis.Thir-teen cases had the radiological features of meninges metastasis and 5 cases accompanied with spinal cord meninges me-tastasis.Then,the following radiological feature was that 7 cases had the mass of suprasellar pool.(4)Twelve cases with CNS metastasis showed the cerebrospinal fluid (CSF)RB cells positive,and the positive rate was 52.1 7%(1 2 /23 ca-ses).Conclusions (1 )The occurrence of RB with CNS metastasis is very low,but the risk of CNS metastasis in-creased in the patients with the pathological evidence of the optic nerve involvement.(2)The main site of CNS metasta-ses is meninges,followed by mass formation in the suprasellar pool.The main route for CNS metastases was along the optic nerve and /or nerve sheath directly invading and /or disseminating via the CSF.(3)The positive rate of CSF cy-tology is low,and it can be improved through repeated examination.Enhanced magnetic resonance imaging should be the most effective radiology examination for diagnosis and monitoring CNS metastasis.