Advanced intraocular tumors and metastatic disease in retinoblastoma patients still occur frequently in developing countries. The aim of this retrospective study was to describe the clinical and epidemiological characteristics of patients with retinoblastoma and the effects of these features on disease prognosis in the authors' pediatric oncology unit as a developing country profile to define the problem. A retrospective chart review of 91 patients who presented to the unit between May 1996 and December 2003 was conducted in this study. Patients with unilateral disease presented at a median age of 24 months and those with bilateral disease at a median age of 9.5 months (p < .01). Most of the eyes with retinoblastoma (68.6%) had Reese-Ellsworth stage V disease. Metastatic disease was diagnosed in 19 (20.9%) patients. Cases with metastatic disease presented at a median age of 24 months and those without metastatic disease at a median age of 12.5 months (p < .05). In 31 patients (34.1%) there was a delay in diagnosis. The enucleation ratio in eyes with advanced intraocular stage was significantly higher than in eyes with early intraocular stage (57.9 vs. 3.8%) (p < .001). In patients with metastatic disease, tumor recurrence was more frequent than in the nonmetastatic patients (36.8 vs. 4.2%) (p < .01). Seven children (7.7%) died due to central nervous system (CNS) metastasis (p < .01). Advanced intraocular disease and distant metastases occur more frequently in Turkish children with retinoblastoma than in children in developed countries, causing a higher rate of enucleation and mortality. Late referral might account for the delayed diagnosis.

Obstructive jaundice is an unusual manifestation of non-Hodgkin lymphomas in children. Although surgical drainage is one of the initial treatment choices in some cases, usually lymphomatous masses rapidly response to chemotherapy and jaundice decreases due to regression of the mass, without any surgical procedure. The authors report the case of a 16-year-old girl who presented with biliary obstruction due to a neoplasm involving the duodenum. Histological examination of the specimen, which was taken from the mass by endoscopic biopsy, revealed Burkitt lymphoma infiltrating the duodenum. Chemotherapy including cyclophosphamide was started immediately. In a few days, jaundice decreased rapidly by the shrinkage of the mass. Neither surgery nor percutaneous drainage were needed. In conclusion, biliary tract obstruction due to non-Hodgkin lymphoma can be effectively treated with chemotherapy alone without any surgical procedure.

Iron-deficiency anemia may be a factor contributing to breath-holding spells. The serum transferrin receptor provides a useful measure of tissue iron deficiency. In this study of 50 breath-holders, while iron-deficiency anemia was detected in 28 (56%) of patients with routine tests, serum transferrin receptor levels were found increased in all patients. A positive correlation was detected between serum soluble transferrin receptor levels and frequency of attacks. It is suggested that the serum transferrin receptor level is useful as a single test for identification of iron deficiency in breath-holders. Moreover, if iron deficiency can be diagnosed earlier, then patients can be treated earlier.

Thymic carcinoma, which is a thymic epithelial neoplasm with obvious cytologic atypia, is a rare neoplasm. The authors report on a 10-year-old boy who presented with respiratory distress due to bulky anterior mediastinal mass. Histological and immunohistochemical studies confirmed a lymphoepithelioma-like pattern thymic carcinoma. In addition, evaluation of the specimen showed foci of caseation and multiple granulomas with extensive central necrosis within the thymic tissue. The child received chemotherapy, followed by surgery and radiotherapy. To rule out difficulties of tuberculosis he also received antituberculosis therapy. Two years after cessation of treatment, he is still in remission for thymic carcinoma.

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