WORK-UP FOR METASTATIC RETINOBLASTOMA - A REVIEW OF 261 PATIENTS

Purpose: The means available to screen for retinoblastoma metastases, including bone marrow aspiration, lumbar puncture, and radionuclide sc ans, offer variable usefulness at different stages of the disease. In this study, the authors attempted to assess the value of these tests a s part of the initial workup for metastases. Methods: Medical files of 261 patients with retinoblastoma were reviewed, and the results of bo ne 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 opt ic nerve also were correlated with the results of diagnostic tests. Re sults: Of 261 patients with retinoblastoma, 147 (56.3%) and 114 (43.7% ) had unilateral and bilateral disease, respectively; 11.6% of unilate ral 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 ce lls in the aspirate. Ninety-four (36%) of the patients were examined w ith spinal tap cytology, and the results of four (4.3%) of these were positive for tumor cells, Radionuclide bone and liver scans were perfo rmed on 49 (18.8%) and 48 (18.4%) patients, respectively; 5 (10.2%) ha d abnormal bone scans and 3 (6.2%) had abnormal liver scans. The avera ge age in the metastatic group was 3.1 years, whereas the average age in the nonmetastatic group was 2.3 years. AII of our abnormal test res ults were found in patients with stages III and IV disease, with the e xception of one abnormal lumbar puncture in a patient with stage II di sease. The correlation of diagnostic tests with the histopathologic fi ndings showed that with no choroidal involvement, no positive diagnost ic tests were encountered. Correlation between positive diagnostic tes t results and the level of optic nerve involvement failed to indicate any trends. Conclusion: Our study indicated a good interrelation betwe en the positivity of diagnostic tests (bone marrow and bone scan) and higher stages of the disease (stages III and IV) and choroidal involve ment in enucleated eyes, with statistically significant correlations; correlations with lumbar puncture and liver scan were not significant.