A review of 400 orbital space occupying lesions during a 30-year period

400 orbital space occupying lesions encountered during a 30 year period were reviewed to determine the distribution of various pathologic processes and the trends over lime. Secondary and metastatic tumors were by far the most common (131 cases, 32.8%) followed by vascular lesions (61 cases, 15.2%), inflammatory lesions (53 cases, 13.2%) and cystic lesions (39 cases, 9.8%). In children (147 cases), the four most common space occupying orbital lesions were secondary and metastatic tumors (32 cases, 21.8%), rhabdomyosarcoma (24 cases, 16.3%), vascular lesions (21 cases, 14.3%) and inflammatory lesions (21 cases, 14.3%). The high frequency of secondary tumors were due to late presentation of many lid, conjunctival and ocular tumor cases. The distribution of the lesions in the first (1966-1980) and second half (1981-1996) were compared. The percentage of secondary and metastatic tumors fell from 48.5% in the first half to 27.6% in the second half. This difference seems to result from earlier recognition and treatment of primary neoplasms

Excision and cryosurgery in the treatment of conjunctival malignant epithelial tumours

Purpose To evaluate the long-term results of combined treatment with excision and cryosurgery for malignant epithelial tumours of the conjunctiva

Clinical features of tuberous sclerosis cases

Tuberous sclerosis (TS) is an autosomal dominant, multisystemic and neurocutaneous disease with high spontaneous mutation rate, and it mostly involves the skin, brain, kidneys, heart and the eyes. This study included 35 patients diagnosed with tuberous sclerosis and aged 6 months to 17 years, with a mean age of 6.5+/-4.8 years. The most frequently observed manifestations were those of the skin (97.1%) and of the central nervous system (seizures 94.2%, mental retardation 51.4%), followed by renal (32.2%), cardiac (25.8%) and ocular (22.5%) manifestations. Among cutaneous manifestations, hypomelanotic macules (94.3%), facial angiofibromas (40%), shagreen spots (20%), fibrous plaques on the forehead (5.7%) and ungula fibromas (5.7%) were observed. Tonic seizures (37.1%) and infantile spasms (21.2%) accounted for majority of seizures. Neurophysiological development was normal in 25.6% of cases, retarded in 51.4% and borderline in 23%. Thirty-four patients had typical pathological findings on magnetic resonance imaging (MRI)

Familial retinoblastoma in developing countries.

Background. Although screening for familial retinoblastoma has been shown to be beneficial we Suspected that Such screening programs may be less than optimal in developing countries (DC). Methods. Retrospective cohort Study comparing patients with familial retinoblastoma from five centers in DC (Argentina, Brazil, Turkey, Jordan, and Venezuela) versus a reference center in the USA. Results. Ninety-two (32 from the USA and 60 from DO patients were included. Forty-one (44.6%) patients avoided enucleation, 42 (45.7%) had I eye removed, and 9 (9.8%) underwent bilateral enucleation. Eleven 0 1.9%) had major pathology risk factors at enucleation. There were no cases of metastatic disease at diagnosis. Detection via screening was significantly less common in DC than in the USA (23.3% vs. 71.8%, P<0.0001). Patients in DC were diagnosed at a significantly later age and with more advanced intraocular disease that led to increased risk of bilateral enucleation. Patients detected by screening in DC were significantly Younger at diagnosis, had less advanced intraocular disease, better ocular preservation rates and Survival results than those whose retinoblastoma was not detected via early screening. Five-year pEFS was 0.92 for the patients treated in the USA and 0.81 for the patients in DC (P=0.42). Seven events occurred (extraocular relapse four in patients from DC and second malignancies in three). Conclusions. Patients with familial retinoblastoma are less likely to be diagnosed by screening in DC and had higher morbidity and mortality caused by recurrent extraocular retinoblastoma. Pediatr Blood Cancer 2009;53:338-342. (C) 2009 Wiley-Liss, Inc