Bilateral pansinusitis was the most common cause. All patients received an initial trial of intravenous antibiotics. Based on the Fisher exact test, no statistically significant differences were detected for age, sex, presence of gaze restriction, and radiographic findings. Based on multiple logistic regression, degree of proptosis was the only significant multivariate predictor of surgery (P =.003). The estimated probability of surgery was 6% when there was no proptosis, and 92% for 2 mm of proptosis. The location of the SPA determined the route of surgical drainage. Eleven patients with a medially based SPA underwent drainage via the transnasal endoscopic approach, and 3 with a superior SPA underwent drainage externally. The external approach was associated with a longer hospital stay (median, 7 days) than either the endoscopic or the intravenous antibiotic approach (median, 5 days).
BACKGROUND
The leading cause of death for patients with hereditary retinoblastoma is second malignancy. Radiotherapy (RT), despite its high rate of efficacy, is often avoided due to fear of inducing a secondary tumor. Proton RT allows for significant sparing of non-target tissue. We compared the risk of second malignancy in retinoblastoma patients treated with photon and proton RT.
METHODS
We performed a retrospective review of patients with retinoblastoma treated with proton RT at the Massachusetts General Hospital or photon RT at Children’s Hospital Boston between 1986 and 2011.
RESULTS
Eighty-six patients were identified. Fifty-five patients received proton RT, and 31 patients received photon RT. Patients were followed for a median of 6.9 years (range, 1.0 to 24.4) in the proton cohort and 13.1 years (range, 1.4 to 23.9) in the photon cohort. The 10-year cumulative incidence of RT-induced or in-field second malignancies was significantly different between radiation modalities (Proton vs. Photon; 0% vs. 14%; p=0.015). The 10-year cumulative incidence of all second malignancies was also different, though with borderline significance (5% vs. 14%; p=0.120).
CONCLUSION
Retinoblastoma is highly responsive to radiation. The central objection to the use of radiation--the risk of second malignancy--is founded on studies of patients treated with antiquated, relatively non-conformal techniques. We present the first series of patients treated with the most conformal of currently available external beam therapy modalities. While longer follow up is necessary, our preliminary data suggest that proton RT significantly lowers the risk of RT-induced malignancy.
To test the hypothesis that the more severe the acute phase retinopathy of prematurity (ROP) was in the preterm weeks, the more severely compromised is rod photoreceptor function after the ROP has resolved.Methods: Electroretinographic (ERG) responses were recorded from 25 dark-adapted children (ages 2.5 months' postterm to 14 years) categorized by maximum, acute phase ROP (None to Very Severe). From the ERG a-wave "S," a sensitivity parameter for the rod photoreceptor response, and R mp3 , the saturated amplitude of the rod photoreceptor response were calculated using a model of the activation of rod phototransduction. The patients' results were compared with those of healthy controls (n=71).Results: Among those in the None, Mild, Moderate, and Severe categories, both S and R mp3 varied significantly with severity of acute phase ROP. In the Very Severe category, ERG responses were too attenuated to calculate S and R mp3 .
Conclusions:The rod photoreceptors must be involved in ROP. The more severe the acute phase ROP, the more severe is the compromise of the processes involved in the activation of phototransduction in the rods.
Mutations of the retinoblastoma gene, most of which cannot be detected by conventional Southern blotting, are known to cause both the nonhereditary and hereditary forms of retinoblastoma and have been implicated in the development of other cancers. Nonhereditary retinoblastoma is caused by a somatic mutation. Hereditary retinoblastoma is caused by a germ-cell mutation, most often a new one, and thus there is usually no family history of the disease. Unlike patients with the nonhereditary disease, those with the hereditary form are at risk for additional retinoblastomas, and their progeny are at risk for the tumors. We used a sensitive technique of primer-directed enzymatic amplification, followed by DNA sequence analysis, to identify mutations as small as a single nucleotide change in tumors from seven patients with simplex retinoblastoma (with no family history of the disease). In four patients the mutation involved only the tumor cells, and in three it involved normal somatic cells as well as tumor cells but was not found in either parent; thus, these mutations appeared to be new, germ-cell mutations. In addition, we found point mutations in cells from a bladder carcinoma, a small-cell carcinoma of the lung, and another retinoblastoma. We conclude that the technique that we have described can distinguish hereditary from nonhereditary retinoblastoma and that it is useful in risk estimation and genetic counseling.
We studied the frequency of chromosome 13 homozygosity in tumor tissue obtained directly from eyes harboring retinoblastomas. The data indicate that approximately half of all retinoblastomas are homozygous for large portions of 13q, that the homozygosity occurs in vivo and not as an event secondary to culture of the tumor cells, that chromosome 13 homozygosity is not correlated with the degree of histopathologic differentiation of the tumor, and that the homozygosity occurs in both sporadic and hereditary retinoblastomas. The development of chromosome 13 homozygosity may represent a fundamental event in the oncogenesis of a considerable number of retinoblastomas. This finding may have implications for the genetic counseling of patients with hereditary retinoblastoma. It may also be important in understanding the mechanism of oncogenesis of other tumors, especially hereditary tumors.
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