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Metastasis of Retinoblastoma to bone.Clinical History: 3y/f, h/o of enucleation done for retinoblastoma of the left eye 18 mths back. recent h/o swelling over the scalp for a period of 6 mths. Findings: Fig 1 showing a strongly enhancing soft tissue mass lesion in the left orbit with intracranial extension and similar lesion in the right parieto-occipital region. Fig 3 showing bone window of the same patient with bone erosion due to the soft tissue mass of the underlying left zygomatic and right parieto-occipital bone. Fig 4 showing histopathological evaluation of the scalp swelling showing round cells in rossettes and string arrangement suggestive of metastatic lesion. Diagnosis: Metastasis of retinoblastoma to bone. Discussion: Retinoblastoma is the most common malignant intraocular neoplasm of childhood. Distant metastases to bone and bone marrow occur. Secondary nonocular malignant tumors should be considered in the differential diagnosis of patients with a history of retinoblastoma who have a painful extremity. Radiation treatment of the primary tumor increases the risk for nonocular secondary neoplasms. The structural alterations in the retinoblastoma gene in patients with osteosarcomas and the possible role of the retinoblastoma gene in the origin of osteosarcomas are also discussed. References / Suggested Reading:
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Dear Vipul and Dr.Tavri:
Thank you for submitting such an excellent teaching case.
Vikram S Dogra, MD
Professor of Radiology, Urology & Biomedical Engineering.
University of Rochester, NY,USA
Vikram Dogra, MD Professor of Radiology,Urology & BME University of Rochester, NY