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PubMed1964

MELANOMA OR NO.

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Black People↗
PubMed2002

(Neonatal) retinoblastoma in the first month of life.

OBJECTIVES: To identify patients with retinoblastoma whose conditions were diagnosed at the age of 1 month or younger and to describe their clinical features (including ocular and patient survival) and the development of second nonocular tumors. MATERIALS AND METHODS: A retrospective study of 1831 patients. The cumulative incidence of second cancer development was analyzed using the Kaplan-Meier method. RESULTS: Forty-six patients were identified as having a diagnosis of retinoblastoma at the age of 1 month or younger (mean age, 18.5 days). Family history (31 patients [67%]) exceeded leukocoria (6 patients [13%]) as the most common reason for detection. Twenty-six (56%) of the 46 patients were seen with unilateral retinoblastoma, with 22 ultimately developing cancer in the fellow eye. At the initial diagnosis, 81 (85%) of the 95 tumors were detected in zones 1 and 2. Eighty-two (93%) of the 88 subsequent tumors were located in zones 2 and 3. In the 26 patients who had unilateral retinoblastoma, 16 of the initially affected eyes and 21 of the fellow eyes were salvaged. In the 19 (44%) of 20 patients who were seen initially with bilateral retinoblastomas, 31 (82%) of the 38 eyes were salvaged. The mean follow-up was 10.9 years. The incidence of second nonocular cancers reached 54% by 23.7 years for the patients who received radiation therapy, while the incidence was 0% for the patients who did not. Four (8.7%) of the 46 patients developed metastatic disease and died; 3 of these patients had documented metastases in the first month of life (one at birth). CONCLUSIONS: The most common manifesting sign of children diagnosed as having retinoblastoma in the first month of life is family history. Eyes with Reese-Ellsworth group I retinoblastomas were the most common. In patients with bilateral and unilateral retinoblastoma, new (subsequent) ocular tumors developed in a centrifugal pattern. Despite an early diagnosis, patients' eyes came to enucleation, and metastatic disease and death occurred from ocular metastases. In patients who received radiation therapy, the probability of developing second nonocular cancer is 54% by 23.7 years; no second cancers developed in patients who did not receive radiation therapy.

Cohort Studies↗
PubMed2003

Histopathologic findings in eyes with retinoblastoma treated only with chemoreduction.

OBJECTIVE: To evaluate the histopathologic findings in the eyes with retinoblastoma that had been treated only with chemoreduction. DESIGN: Clinicopathologic series. Study Material Ten eyes of 8 patients with retinoblastoma that were enucleated after therapy consisting only of systemic chemotherapy (chemoreduction). METHODS: All cases received a chemoreduction regimen including a combination of intravenous carboplatin, etoposide phosphate, and vincristine sulfate. Adjuvant treatment to the tumor was not provided in any case. The enucleated globes were studied by routine light microscopy. Main Outcome Measure Histopathologic features of retinoblastoma following chemoreduction. RESULTS: At presentation, there were 8 eyes in Reese-Ellsworth group V, 1 eye in group IV, and 1 eye in group III. After chemoreduction (mean, 4 cycles; range, 1-6 cycles), the main tumor regressed a mean 34% in thickness and 24% in basal diameter. The indication for enucleation was retinoblastoma recurrence as subretinal and/or vitreous seeds in 7 eyes and extensive vitreous hemorrhage in 3 with uncertainty about viable-appearing tumor. In no case was enucleation performed for recurrence of the main tumor. In all eyes, there was histopathologic evidence of tumor regression. In 8 of 10 eyes, histopathologic examination disclosed tumor regression without viable-appearing retinoblastoma in the main tumor. Of these 8 eyes, 2 showed a completely calcified glial scar and 6 showed an apical calcified glial scar and a basal residual well-differentiated component with retinomalike and/or retinocytomalike features. In the remaining 2 eyes, an area of posttherapeutic regression was present but contained foci of mitotically active, viable-appearing malignant retinoblastoma cells. The 6 eyes found to contain well-differentiated component with retinomalike and/or retinocytomalike features showed a mean decrease of 17% in largest basal dimension and 32% in thickness after a mean of 3 cycles of chemoreduction. In contrast, the 4 eyes that did not contain well-differentiated component with retinomalike and/or retinocytomalike features showed a mean decrease of 35% in largest basal dimension and 55% in thickness after a mean of 5 cycles of chemoreduction. Of those 7 eyes enucleated for recurrent subretinal and/or vitreous seeds, viable tumor seeds were confirmed histopathologically in all cases. There was no histopathologic evidence of chemotherapeutic toxicity to the eye. CONCLUSIONS: Histopathologic examination of 10 enucleated eyes following chemoreduction alone revealed that the main retinoblastoma regressed in all eyes. Additionally 6 eyes showed basal residual well-differentiated component with retinomalike and/or retinocytomalike features, and these eyes also displayed less shrinkage with chemoreduction. Despite the lack of viable-appearing retinoblastoma within the main tumor, enucleation was performed for viable subretinal and/or vitreous seeds in 7 cases and confirmed histopathologically.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

Outcome of patients with a history of bilateral retinoblastoma treated for a second malignancy: the Memorial Sloan-Kettering experience.

BACKGROUND: Patients with bilateral retinoblastoma are well recognized to have a high risk of developing a second malignancy, but there are little published data regarding the outcome of these patients following treatment. PATIENTS AND METHODS: We identified 15 patients with a history of bilateral retinoblastoma who received treatment at Memorial Sloan-Kettering Cancer Center for a newly diagnosed second malignancy. The median age of second tumor occurrence was 18 years (range 10-32 years). Three patients later had a third tumor (18 tumors total). Tumor sites included facial structures in 14 cases and extremities in 4. Histologies included osteosarcoma (5), leiomyosarcoma (5), high-grade spindle cell sarcoma (3), malignant fibrous histiocytoma (3), malignant mesenchymoma (1), and angiosarcoma (1). RESULTS: Nine patients are alive: 7 disease free at a median of 29 months (range 6-214 months) and 2 with residual disease 59 and 148 months post-diagnosis of the second malignancy. Six patients have died at a median of 31 months (range 16-98 months) after diagnosis of the second malignancy. CONCLUSIONS: Patients with a history of bilateral retinoblastoma who develop a second malignancy may enjoy extended periods of survival. Aggressive therapy appropriate to the tumor histology and site is indicated.

Adolescent↗