Trilateral Retinoblastoma Studies

Trilateral retinoblastoma is a cancer that affects children in infancy. It begins as bilateral retinoblastoma, the development of malignant tumors on the retinas of the eyes, and develops with the manifestation of a third tumor, an intracranial brain tumor near either the pituitary or pineal glands. Studies of the disease have shown that children die from a spread of the bilateral retinoblastoma before the intracranial tumor develops or is diagnosed, and once an intracranial tumor is found, life expectancy can be one to eight months.

Assessment of Tumor Progression

• Published in the American Journal of Roentgenology, doctors from Duke University Medical Center's departments of radiology, pediatrics and pathology completed a study in 2003 assessing tumor progression in children with trilateral retinoblastoma. It was found that children were normally diagnosed at four months of age with bilateral retinoblastoma, while the third tumor inside the cranium did not manifest until about 21 months of age. Further, the discouraging fact was published that fatal metastasis, or spread of the cancer to the spine and membrane surrounding the brain, occurred before the third intracranial tumor even developed or could be seen on radiology scans. This study suggests that effective treatment of trilateral retinoblastoma requires close evaluation for metastasis.

Differences in Intracranial Tumor Locations

• Published in the medical journal Cancer in 1999, radiotherapists from the Cardinal Bernardin Cancer Center and Ronald McDonald Children's Hospital in Maywood, Illinois, studied if the effects of treatment varied according to the location of the intracranial tumor. Researchers reviewed 94 cases of trilateral retinoblastoma. In all cases, intracranial tumors developed in either the suprasellar region, which involves the area of the pituitary gland, and the area involving the brain's pineal gland. Although suprasellar tumors presented up to 23 months earlier than pineal tumors, the average survival rate after diagnosis was the same, eight months with treatment and one month without treatment. Children who were not showing symptoms of disease at the time of intracranial tumor diagnosis did survive longer suggesting that frequent screening for intracranial tumors may be a vital strategy in treating patients with bilateral retinoblastoma.

Detailed Information about Trilateral Retinoblastoma

• Published in the Journal of Clinical Oncology in 1999, researchers at the Helsinki University Central Hospital in Helsinki, Finland, reviewed 106 case studies of trilateral retinoblastoma to obtain identifying information about the disease. Already associated with genetic heredity, researchers believed that control of the disease could only be accomplished by gathering more facts. They found that trilateral retinoblastoma was not confined to any gender. Diagnosis was usually made at five months of age, and the disease skipped a generation, presenting in mainly second and third generations. Third intracranial tumors that classify the disease as trilateral usually occurred at 21 months of age. Earlier detection meant longer survival, but all children with an intracranial tumor larger than 15 mm died.