The management of unilateral retinoblastoma without primary enucleation.

Sixty-six patients were treated "conservatively" for unilateral retinoblastoma. Forty-eight of 57 (84%) were treated primarily with unilateral radiation, one patient was treated with a cobalt plaque, and eight patients were treated with either cryopexy or xenon arc photocoagulation. With a median follow-up of 73 months, there have been no deaths. Five of 39 eyes that were in groups I to III have been salvaged. Virtually all eyes in groups IV and V (12 of 14) came to enucleation. The age at diagnosis for patients with a positive family history was early (2.5 months), and the patients had a greater number of individual tumors in one eye (2.4), compared with those without a positive family history (27 months, 1.2 tumors per eye). When unilateral retinoblastoma is detected at an early age, the most common sign is strabismus, not leukokoria.

Retreatment of retinoblastoma with external beam irradiation.

A retrospective review of cases on file at the Ophthalmic Oncology Center of New York Hospital-Cornell Medical Center, New York, was undertaken to examine the effectiveness of a second course of radiotherapy on retinoblastoma. One hundred four patients were found to have been treated with at least two courses of external beam irradiation to one eye. All but one of the cases were bilateral, the other eye with more advanced disease having been previously enucleated. At the time the decision was made to repeat radiotherapy, 15 eyes could be classified group I, II, or III. Twelve (80%) of these eyes survived. Of the 89 eyes that showed more advanced growth (group IV or V), only two (2.2%) survived. In all, 14 of the 104 eyes wer saved--nine with useful (macula) vision, five without. Second nonocular tumors developed in 18 patients (22.8% of those who survived retinoblastoma itself). There appears to be no increased risk of second tumors from the second course of irradiation.

Treatment of bilateral groups I through III retinoblastoma with bilateral radiation.

Thirty-seven patients with bilateral retinoblastoma, groups I, II, and III, were treated with simultaneous bilateral radiation. The median age at diagnosis was 4 months. Twenty-two of these patients were examined because of known history of retinoblastoma. Eight of these 22 patients had no evidence of retinoblastoma on at least one examination before the detection of retinoblastoma, and two had bilateral disease present at birth. Of 74 eyes treated, 39 required additional treatment and 11 ultimately were enucleated. Three patients died of metastatic disease. Seven patients subsequently had second nonocular tumors develop that were both in and out of the field of radiation.

Simultaneous bilateral radiation for advanced bilateral retinoblastoma.

A retrospective review of 1,424 cases of retinoblastoma disclosed 34 patients with advanced bilateral retinoblastoma whose sole initial treatment was simultaneous bilateral radiation. All patients had a combination of bilateral group IV or V retinoblastoma. Mean age at diagnosis was 13.1 months. Five-year survival of patients was 88% (28/32). In two (7%) of 28 surviving patients, a second nonocular tumor developed: one inside and one outside the field of radiation. Of the 64 eyes treated, 44 ultimately were enucleated. Of ten eyes requiring a second course of radiation, nine were enucleated. In only one eye was enucleation due to radiation complications. Eleven patients required additional treatment with light coagulation, cryopexy, or cobalt plaques.

The treatment of orbital rhabdomyosarcoma with irradiation and chemotherapy.

Fifty-eight patients with orbital rhabdomyosarcoma were treated with irradiation alone (25) or irradiation and chemotherapy (33) with follow-ups of 6 months to 14 years (mean 5.2 yr). At present, 74% are alive and 26% have died. Local control of the tumor was accomplished in 91% of cases. When local sinuses were invaded the survival rate was 55%. Chemotherapy appears to be of greatest value when disease is limited to the orbit. Irradiation or irradiation and chemotherapy should now be the treatment of choice for orbital rhabdomyosarcoma.

Primary malignant melanoma of the esophagus: report of two cases.

Two cases of primary esophageal malignant melanoma diagnosed at the Columbia-Presbyterian Medical Center are reported. One patient who received 3800 rads and had subsequent surgery lived for 18 months. The other had surgery and subsequent chemotherapy and is still alive three years later. Forty other cases reported in the literature are briefly analyzed.