By Darci Picoult
It began with a bump. The size of a pinhead. Innocuous. An innocuous little pinhead of a bump on my vulva. Given that my gynecologist said the bump was probably nothing, I laughed it off. Which, in turn, made my bump mad. Very mad. It wanted my attention. And so it grew. I smeared it in medicine. It grew more. More medicine. More growth. Hanukkah came. Then Christmas. A war raged between us. I went to battle in the middle of the night with salt baths and creams. Prayed for its departure...
Added text to state that there may be an association between type of RB1 mutation and incidence of subsequent neoplasms (SNs), with complete loss of RB1 activity having a higher incidence of SNs (cited Dommering et al. as reference 26). Added additional text to state that of 245 patients, all of whom received etoposide, only one patient had acute promyelocytic leukemia after 79 months (cited Turaka et al. as reference 30).
Added text to state that orbital growth is somewhat diminished after enucleation; however, the impact of enucleation on orbital volume may be less after placement of an orbital implant (cited Chojniak et al as reference 42).
Added text to state that cavitary retinoblastoma, a rare variant of retinoblastoma, has ophthalmoscopically visible lucent cavities within the tumor; cavitary spaces appear hollow on ultrasonography and hypofluorescent on angiography; and that histopathologically, the cavitary spaces have been shown to represent areas of photoreceptor differentiation (cited Palamar et al. as reference 2). Also added text to state that these tumors have been associated with minimal visible tumor response to chemotherapy, which is thought to be a sign of tumor differentiation (cited Mashayekhi et el. as reference 3).
Treatment Option Overview
Added text to state that in patients with cavitary retinoblastoma, minimal visual response is seen after intravenous and/or intra-arterial chemotherapy; despite the blunted clinical response, cavitary retinoblastoma has a favorable long-term outcome with stable tumor regression and globe salvage. Aggressive or prolonged chemotherapy or adjunctive therapies are generally not necessary (cited Rojanaporn et al. as reference 12).
Intraocular Retinoblastoma Treatment
Added Aerts et al. as reference 23.
Revised text to state that other agents such as topotecan and carboplatin are also being tested, given as single agents or in combination (cited Marr et al. as references 32).
Added Abramson et al. as reference 36, Ghassemi et al. as reference 42 and level of evidence 3iiDiii, and Palioura et al. as reference 43 and level of evidence 3iiDiv.