Retinoblastoma

Retinoblastoma is a rare eye tumor of childhood that arises in the retina. It is the most common intraocular malignancy of infancy and childhood; with an incidence of 1/15,000–20,000 live births. The two most frequent symptoms revealing retinoblastoma are leukocoria and strabismus. Iris rubeosis, hypopyon, hyphema, buphthalmia, orbital cellulites and exophthalmia may also be observed. Sixty per cent of retinoblastomas are unilateral and most of these forms are not hereditary (median age at diagnosis two years). Retinoblastoma is bilateral in 40% of cases (median age at diagnosis one year). All bilateral and multifocal unilateral forms are hereditary. Hereditary retinoblastoma constitutes a cancer predisposition syndrome: a subject constitutionally carrying an RB1 gene mutation has a greater than 90% risk of developing retinoblastoma but is also at increased risk of developing other types of cancers. Diagnosis is made by fundoscopy. Ultrasound, magnetic resonance imaging (MRI) and computed tomography (CT) scans may contribute to diagnosis. Management of patients with retinoblastoma must take into account the various aspects of the disease: the visual risk, the possibly hereditary nature of the disease, the life-threatening risk. Enucleation is still often necessary in unilateral disease; the decision for adjuvant treatment is taken according to the histological risk factors. Conservative treatment for at least one eye is possible in most of the bilateral cases. It includes laser alone or combined with chemotherapy, cryotherapy and brachytherapy. The indication for external beam radiotherapy should be restricted to large ocular tumors and diffuse vitreous seeding because of the risk of late effects, including secondary sarcoma. Vital prognosis, related to retinoblastoma alone, is now excellent in patients with unilateral or bilateral forms of retinoblastoma. Long term follow-up and early counseling regarding the risk of second primary tumors and transmission should be offered to retinoblastoma patients

A temporal study of mycosporine-like amino acids in surface water phytoplankton from the English Channel and correlation with solar irradiation

A seasonal survey was undertaken, over a year, of phytoplankton from surface water in the western English Channel (Station L4) measuring mycosporine-like amino acids (MAAs), photosynthetic pigments and microscopic counts. Ground level solar radiation (ultraviolet-B, ultraviolet-A and photosynthetically active radiation; UV-B, UV-A and PAR) was measured at a nearby site. From this we estimated in situ solar irradiance received by phytoplankton using measurements of the mixed layer depth and calculations of the 50% light level cut-off. The MAAs occurred year round, with concentrations increasing rapidly during spring and summer (maximum 8·5 μg l−1) to levels exceeding those of chlorophyll-a (chl-a maximum 3·6 μg l−1). On two occasions, increases in specific MAAs coincide with algal blooms. In spring, increases in mycosporine-glycine (λmax 310 nm in the UV-B) coincide with a bloom of the prymnesiophyte Phaeocystis pouchetii and in July and August increases in an unidentified MAA (λmax 328 nm) match a bloom of the diatom, Guinardia striata (=Rhizosolenia stolterfothii). Concentration of MAAs, but not chlorophyll, correlate with in situ irradiance. The ratio of MAA to chl-a increases linearily with in situ irradiance received by phytoplankton reaching 13·9 nmol MAA (nmol chl-a)−1 at 101 W m−2. Evidence of photoinduction is observed during the P. pouchetii bloom with a four fold increase in the concentration of mycosporine-glycine (maximum 2 pg cell−1) as UV-B:PAR ratio increases from 0·0011 to 0·0014. Dinoflagellates, although contributing to &lt;10% of biomass, are found through the correlation of MAAs to the biomarker peridinin, to contribute to baseline levels of MAAs throughout the year. Our MAA:chl-a values for the English Channel are similar to those measured in coastal areas of southern USA. Similarities with studies on Antarctic phytoplankton are also found with the dominance of porphra-334 and the presence of mycosporine-glycine in P. pouchetti.</jats:p