Retinoblastoma in an adult

Abstract

BackgroundRetinoblastoma is the most common pediatric ocular tumour. It may rarely present in adults. The present case adds to the number of 26 cases already published in literature since 1919 till 2013. Our aim is to highlight the rare occurrence of retinoblastoma in adults along with its features which differentiate it from paediatric retinoblastoma.Case presentationWe describe a case of adult onset retinoblastoma (group E, according to the international classification of retinoblastoma) occurring in a 25 year old male. He presented with decreasing visual acuity in the right eye of 4 months duration. He had neo-vascular glaucoma and pseudohypopyon. B scan ultrsonography of his right eye showed intraocular growth without any calcification. The CT scan of the orbits and brain showed intraocular growth in the right eye with no calcification. Enucleation of the right eye was carried out. Retinoblastoma was confirmed on histopathology of the enuleated globe.ConclusionsThe present case adds to the number of adult Rb patients reported in literature. Early detection to salvage the life can be made possible if the clinician keeps a high index of suspicion when observing retinal mass of adult onset. Proper counselling of the patient in order to seek his full involvement in management may help in improving the prognosis of the disease.

Adult onset retinoblastoma

“…[ 4 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 ] From 2005 onward, there has been a sudden proliferation of cases where 23 more cases have been published including 16 cases reported in the last 3 years. [ 5 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 ]…”

“…Rest of the three cases had distant metastasis and unfortunately expired at 12, 16, and 60 months. [ 28 33 ]…”

“…reported possible upstaging of tumor due to vitreous tap. [ 33 ] Vitrectomy or vitreous tap in unsuspected RB needs enucleation with chemotherapy or radiotherapy or both without delay to prevent systemic tumor dissemination. [ 55 ] Cases with diagnostic dilemma might benefit from FNAB followed by IHC staining of the specimens.…”

“…Among the 27 subsequent cases reported, primary enucleation was done in 14 cases of which 1 case underwent preoperative radiation, 2 required additional postenucleation chemotherapy,[ 28 31 ] and 1 both chemo and radiotherapy. [ 33 ] Two cases underwent pars plana vitrectomy for retinal detachment with unsuspected RB ending up finally in enucleation. [ 26 29 ] Seven eyes had to be enucleated after the failure of other treatment modalities.…”

The bone marrow metastasis niche in retinoblastoma

“…In general, Rb is a rare cancer with an incidence rate of 1:15,000 to 1:18,000 [2]. Rb rarely occurs in adults, i.e., as yet only 26 adult cases of Rb have been reported worldwide [3]. Rb can occur in two forms: hereditary, with bilateral tumors and, sometimes, multiple tumors in one eye, and non-hereditary, with a single unilateral tumor in one eye.…”