Background: Retinoblastoma (RB) is a relatively uncommon tumour in childhood. The incidence of retinoblastoma in Mexico is probably higher than the incidence reported worldwide, however there is not enough information about the characteristics of this illness in Mexico. This report aims to present the results of a multicentre clinical survey of RB in Mexico. Methods: A retrospective study was carried out on all RB cases treated in 16 institutions during the last six years. The variables analysed were age at diagnosis, sex, affected eyes, treatment modalities, and pathological staging. Overall survival was obtained. Results: The authors analysed 500 cases; age range was 0-182 months. There were 364 unilateral cases (72.8%). Enucleation was performed in 84.9% of the patients. The St Jude's staging was: 7.4% stage I, 52.8% stage II, 18.0% stage III, 11.4% stage IV, 7.2% not evaluated, and 3.2% missing data. Chemotherapy was used in 74.4% of the patients. Disease free survival was 89% at 73 months follow up. Conclusions: The paper presents a great number of cases and pioneers multicentre studies in paediatric ophthalmology and oncology in this country. Given the great number of patients in advanced stages and the variability on treatment schemes, it is evident that it is mandatory to work in a cooperative group and develop a national early detection programme as well as a treatment protocol which include all specialists involved in the care of patients with RB.
Alveolar soft part sarcoma (ASPS) is a rare, malignant tumor of uncertain histogenesis that has no benign counterpart. In some cases, a structural rearrangement of chromosome 17 involving band q25 has been reported. The neoplasm occurs most frequently in female adolescents and young adults, where it arises predominantly in the extremities. In contrast, the most common sites of occurrence in infants and children are the orbit (41%) and the tongue (25%). The primary therapeutic option is a complete surgical excision. Because of the indolent growth and lack of pain associated with the mass, 20% of patients have metastases at the time of initial diagnosis. Median survival time reported for all sites of the body is 79 months. When ASPS presents in the tongue region, however, the patients involved are usually children and have a better prognosis than patients affected in the extremities. The utility of adjuvant chemotherapy or radiation therapy in children is open to question. Because metastases may occur after several decades, children with ASPS should be followed throughout adolescence and well into adulthood. Only 10 cases of ASPS occurring in the tongues of children younger than 5 years of age were indexed by MEDLINE between 1952 and 2006. Here, we describe the 1st case consistent with typical ASPS of the tongue in 15 years at our hospital. The patient is a 2-year-old girl who has been disease-free for 32 months.
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