Workup for metastatic retinoblastoma. A review of 261 patients.

ABSTRACT

PURPOSE: The means available to screen for retinoblastoma metastases, including bone marrow aspiration, lumbar puncture, and radionuclide scans, offer variable usefulness at different stages of the disease. In this study, the authors attempted to assess the value of these tests as part of the initial workup for metastases. METHODS: Medical files of 261 patients with retinoblastoma were reviewed, and the results of bone marrow, lumbar puncture, and bone and liver radionuclide scans were correlated with the laterality, clinical staging, and histopathologic findings. The presence or absence of tumor in the choroid and the optic nerve also were correlated with the results of diagnostic tests. RESULTS: Of 261 patients with retinoblastoma, 147 (56.3%) and 114 (43.7%) had unilateral and bilateral disease, respectively; 11.6% of unilateral cases and 14.9% of bilateral cases had distant metastasis (P = 0.2). Bone marrow aspirations were performed on 101 patients (38.7%), and 10 (9.9%), of these specimens showed the presence of retinoblastoma cells in the aspirate. Ninety-four (36%) of the patients were examined with spinal tap cytology, and the results of four (4.3%) of these were positive for tumor cells. Radionuclide bone and liver scans were performed on 49 (18.8%) and 48 (18.4%) patients, respectively; 5 (10.2%) had abnormal bone scans and 3 (6.2%) had abnormal liver scans. The average age in the metastatic group was 3.1 years, whereas the average age in the nonmetastatic group was 2.3 years. All of our abnormal test results were found in patients with stages III and IV disease, with the exception of one abnormal lumbar puncture in a patient with stage II disease. The correlation of diagnostic tests with the histopathologic findings showed that with no choroidal involvement, no positive diagnostic tests were encountered. Correlation between positive diagnostic test results and the level of optic nerve involvement failed to indicate any trends. CONCLUSION: Our study indicated a good interrelation between the positivity of diagnostic tests (bone marrow and bone scan) and higher stages of the disease (stages III and IV) and choroidal involvement in enucleated eyes, with statistically significant correlations; correlations with lumbar puncture and liver scan were not significant.