Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Retinoblastoma, the most common primary ocular malignancy of childhood, is a tumor in which the pediatrician and pediatric oncologist can now play a much more significant role in therapy. Developments in molecular biology have now made carrier testing and prenatal diagnosis feasible. In the near future, these developments should greatly augment the pediatrician's and pediatric oncologist's ability to offer accurate and appropriate genetic counseling for affected families. A practical staging system for extraocular retinoblastoma together with stage-related effective chemotherapy and radiation therapy was presented in this chapter. These modalities now make possible long-term survival for the majority of the 1 out of 8 children with retinoblastoma who would otherwise die from metastatic disease. Finally, 40 per cent of all children with retinoblastoma (those with the germinal mutation) are at lifelong risk for second, nonocular malignancies. The recognition that more than half of these children will actually develop second tumors by the fourth decade of life makes vigilant follow-up care for these patients a necessity.
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PMID:Intraocular and extraocular retinoblastoma. 332 80

Extraocular retinoblastoma is associated with a very poor outcome. At Children's Hospital Los Angeles, 10 of 207 patients with retinoblastoma had extraocular disease. Four patients with no histopathologic risk factors developed extraocular disease. All patients with direct extension into the central nervous system or with distant metastatic disease died. One of three patients with trilateral retinoblastoma and one patient with regional recurrence are alive after autologous bone marrow transplant. Patients with extraocular retinoblastoma who achieve remission may benefit from consolidation of their therapy with autologous bone marrow transplant.
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PMID:Approaches to treatment for extraocular retinoblastoma: Children's Hospital Los Angeles experience. 1470 10

Since the development of chemotherapy regimens for patients with retinoblastoma started in the 1950s, various agents and regimens have been employed for various kinds of patients. Chemotherapy has been employed for: (1) patients with high-risk features for metastases, such as patients with optic nerve involvement, (2) patients with orbital involvement, and (3) patients with distant metastasis. Effective systemic chemotherapeutic agents include vincristine, doxorubicin, cyclophosphamide, etoposide, cisplatin, and carboplatin, and, as well, intrathecal agents, including methotrexate, cytarabine, and corticosteroids are available. With the addition of appropriate chemotherapies to the conventional treatment modalities such as enucleation and radiotherapy, patients with advanced retinoblastoma are expected to obtain a survival benefit. Moreover, a new modality combined with autologous stem cell support allowed us to use high-dose alkylating agents such as thiotepa, melphalan, and cyclophosphamide, which resulted in better prognosis for patients with metastatic retinoblastoma. Because of the small number of patients with retinoblastoma and the diversity of the disease characteristics in individual patients, there have been no clinical trials to determine whether to recommend a particular regimen, or to identify specific criteria in patients who would benefit from chemotherapy. Well-designed prospective controlled trials are warranted to establish a standard treatment strategy for patients with extraocular retinoblastoma.
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PMID:Results of treatment of retinoblastoma that has infiltrated the optic nerve, is recurrent, or has metastasized outside the eyeball. 1516 20

A 6-year-old girl had total hyphema and elevated left intraocular pressure following trivial trauma. B-scan with vector A-scan revealed vitreous opacities consistent with hemorrhage. The drained hyphema did not recur. A left vascular conjunctival mass and massive cervical lymphadenopathy occurred 7 months later. Biopsy revealed extraocular retinoblastoma and lymph node metastasis. Computed tomography showed an intraocular mass with intracranial extension. She died of metastatic disease despite intensive chemotherapy. Retinoblastoma should be suspected in a child with hyphema following trivial trauma.
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PMID:Systemic metastasis following hyphema drainage in an unsuspected retinoblastoma. 1741 Sep 64