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Trilateral Retinoblastoma: Clinical and Radiologic Progression

¹ Department of Radiology, Duke University Medical Center, Box 3808, Durham, NC 27710.
² Department of Pediatrics, Duke University Medical Center, Durham, NC 27710.
³ Department of Pathology, Duke University Medical Center, Durham, NC 27710.

View larger version (11K): [in a new window]   Fig. 1. —Graph shows clinical milestones in eight patients with trilateral retinoblastoma. Line break in patient 7 indicates shortening of line. = ocular bilateral retinoblastoma, = intracranial retinoblastoma, = confirmed intracranial leptomeningeal disease, = confirmed spinal leptomeningeal disease, = death, = alive.

View larger version (148K): [in a new window]   Fig. 2A. —Progression of trilateral retinoblastoma in girl (patient 6) who was diagnosed with bilateral ocular retinoblastoma when 1 month old. Intracranial tumor was discovered when patient was 8 months old and remained stable for 26 months. Patient died at age of 3 years. Unenhanced axial CT scan obtained when girl was 8 months old shows calcified pineal mass that did not enhance after administration of contrast material. Hyperdense region in left frontal lobe presumably reflects radiation changes secondary to radiation therapy for retinoblastoma in left eye. Finding remained stable on multiple subsequent imaging studies.

View larger version (166K): [in a new window]   Fig. 2B. —Progression of trilateral retinoblastoma in girl (patient 6) who was diagnosed with bilateral ocular retinoblastoma when 1 month old. Intracranial tumor was discovered when patient was 8 months old and remained stable for 26 months. Patient died at age of 3 years. Contrast-enhanced coronal T1-weighted image of brain obtained when patient was 34 months old shows multiple enhancing leptomeningeal nodules consistent with metastatic pineoblastoma, as was confirmed by finding of multiple small malignant tumor cells in cerebrospinal fluid.

View larger version (77K): [in a new window]   Fig. 2C. —Progression of trilateral retinoblastoma in girl (patient 6) who was diagnosed with bilateral ocular retinoblastoma when 1 month old. Intracranial tumor was discovered when patient was 8 months old and remained stable for 26 months. Patient died at age of 3 years. Contrast-enhanced sagittal T1-weighted image of spine obtained at same time as B shows multiple leptomeningeal nodules consistent with metastatic tumors.

View larger version (126K): [in a new window]   Fig. 3A. —13-year-old boy (patient 7) who was 2 months old when bilateral retinoblastoma and pineal neoplasm were diagnosed, He was treated with radiation therapy and chemotherapy. Size of intracranial mass markedly decreased after many months of treatment, No evidence of central nervous system metastases was found. However, 10 years after apparently successful therapy, metastasis in right ulna was discovered. Contrast-enhanced axial CT scan obtained through the orbits when patient was 2 months old shows bilateral calcified ocular masses, consistent with retinoblastoma.

View larger version (129K): [in a new window]   Fig. 3B. —13-year-old boy (patient 7) who was 2 months old when bilateral retinoblastoma and pineal neoplasm were diagnosed, He was treated with radiation therapy and chemotherapy. Size of intracranial mass markedly decreased after many months of treatment, No evidence of central nervous system metastases was found. However, 10 years after apparently successful therapy, metastasis in right ulna was discovered. Contrast-enhanced axial CT of brain obtained 4 weeks after A shows nonenhancing, partially calcified mass in pineal region, consistent with pineoblastoma.

View larger version (178K): [in a new window]   Fig. 3C. —13-year-old boy (patient 7) who was 2 months old when bilateral retinoblastoma and pineal neoplasm were diagnosed, He was treated with radiation therapy and chemotherapy. Size of intracranial mass markedly decreased after many months of treatment, No evidence of central nervous system metastases was found. However, 10 years after apparently successful therapy, metastasis in right ulna was discovered. Contrast-enhanced axial MR image of brain obtained 8 weeks after B (6 weeks after beginning chemotherapy) shows that size of nonenhancing pineal tumor has somewhat decreased compared with its size in B. After radiation therapy and chemotherapy, size of mass further decreased over next few months. No metastases of central nervous system have developed during follow-up period of more than 13 years.

View larger version (158K): [in a new window]   Fig. 3D. —13-year-old boy (patient 7) who was 2 months old when bilateral retinoblastoma and pineal neoplasm were diagnosed, He was treated with radiation therapy and chemotherapy. Size of intracranial mass markedly decreased after many months of treatment, No evidence of central nervous system metastases was found. However, 10 years after apparently successful therapy, metastasis in right ulna was discovered. Contrast-enhanced axial T1-weighted image obtained 10 years after C shows enhancing soft-tissue mass (arrows) surrounding right ulna that represents periosteal reaction and tumoral infiltration. Biopsy of mass revealed small cell malignant neoplasm that is histopathologically consistent with metastatic retinoblastoma or new undifferentiated malignant tumor.

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