Treatment of retinoblastoma. Results obtained from a prospective study of 51 patients

Montebello

et al. 1991

Cancer

In this report, three new cases of trilateral retinoblastoma are presented. The clinical presentation, treatment, and outcome of the patients are described and compared with those of 32 cases that have been previously reported in the literature. A positive family history was obtained in 68% of the patients. The mean age at diagnosis of bilateral retinoblastoma was 7.2 months. The mean age at diagnosis of trilateral disease was 39.7 months, resulting in a mean latent interval of 32.6 months. The mean time from diagnosis of trilateral retinoblastoma to death was 6.6 months, and all patients died with spinal metastases. The patients who received no therapy survived an average of 1.3 months after the diagnosis of trilateral disease. The patients who received any form of definitive therapy survived 9.7 months. Five patients who had complete or dramatic response to therapy by computed tomography scans had local intracranial tumor present at autopsy. Therefore, more aggressive local therapy may be warranted.

Section: Discussionmentioning

confidence: 99%

Clinical presentation, treatment, and outcome of trilateral retinoblastoma

Montebello

et al. 1991

Cancer

“…The radiation therapy technique used has been described elsewhere. 6 The following doses were administered: 4500 cGy to the retina, 4000 cGy to the orbit, and 2800 cGy to the brain. The following scoring system was used to evaluate the effect on vision: Group 1, blindness; Group 2, severe amblyopia; Group 3, moderate amblyopia; and Group 4, normal vision.6 Chemotherapy Wac; administered according to stage as shown in Table 4.…”

Section: IVmentioning

confidence: 99%

A prospective study on the treatment of retinoblastoma in 72 patients

Zelter¹,

Damel²,

González³

et al. 1991

Cancer

Self Cite

Seventy‐two patients with retinoblastoma (RTB) (unilateral [47 patients] and bilateral [25 patients]) were studied for a period of 6 years. Treatment consisted of enucleation of those eyes that had severe involvement and was followed by radiation therapy and/or chemotherapy (systemic or intrathecal) according to clinical pathologic staging. All patients who underwent nonsurgical treatment received systemic chemotherapy. Intrathecal chemotherapy was administered when there was involvement of the central nervous system. Nonsurgical treatment consisted of radiation therapy and/or light coagulation, followed by enucleation if there was no response. The overall survival rates were 79% for unilateral disease (median follow‐up time, 63 months) and 72% for bilateral disease (median follow‐up time, 81 months). Vision was regained in 14 of 21 conserved eyes.

International Journal of Clinical Oncology

“…[9][10][11] Through the accumulation of data in several case series including patients with metastatic disease, the efficacy of vincristine, cyclophosphamide, and doxorubicin was established by the 1980s. 12,13 After the 1980s, combinations of etoposide and cisplatin or carboplatin were developed through trials that mainly recruited patients with retinoblastoma with orbital extension. 14,15 These chemotherapy regimens are currently being employed for patients with early-stage retinoblastoma in attempts to preserve the eye.…”

Section: Overview Of the Development Of Chemotherapy Regimens For Patmentioning

confidence: 99%

“…Zelter et al 13 used enucleation and chemotherapy that included cyclophosphamide, vincristine, and doxorubicin for tumors involving the optic nerve head, and additional cranial radiation with intrathecal chemotherapy for tumors extending beyond the cut end. Although the number of patients was limited, the overall survival rates were 100% (6 of 6) for patients with optic nerve extension without cut-end disease, 75% (3 of 4) for patients with unilateral disease and cut-end disease, and 50% (1 of 2) for patients with bilateral disease and cut-end disease.…”

Section: Adjuvant Chemotherapy For Patients With Optic Nerve Involvementmentioning

confidence: 99%

Results of treatment of retinoblastoma that has infiltrated the optic nerve, is recurrent, or has metastasized outside the eyeball

Makimoto¹

2004

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.