PMC3236709_01
(A) One-month-old child with unilateral retinoblastoma and a left macular tumor. (B) Excellent regression of the tumor after three sessions of systemic chemotherapy, causing complete calcification of the macular lesion. (C) Tumor recurrence along temporal edge of the tumor, with associated vitreous seeding. (D) Excellent regression of the retinal tumor recurrence but no response by vitreous seeding after systemic chemotherapy.
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Fundus evaluation revealed a left macular tumor which was whitish in appearance with fine retinal vasculature ( Figure 1A). She received three sessions of systemic chemotherapy using a three drug regimen (carboplatin, etoposide, vincristine), with excellent clinical regression of the tumor (Figure 1B). There was also diffuse vitreous seeding arising from this area of tumor recurrence (Figure 1C). The patient was treated with two additional courses of systemic chemotherapy (topotecan and cyclophosphamide) with excellent regression of the retinal tumor recurrence, but there was no response by the vitreous seeding (Figure 1D).
(A) Diffuse periocular edema following intra-arterial infusion of melphalan, which resolved without sequelae. (B) Fundus photograph showing progression of vitreous seeding despite two sessions of intra-arterial chemotherapy.
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After the second session of IAC, the patient developed diffuse periocular edema (Figure 2A), which resolved within 1 week. Unfortunately, the vitreous seeding appeared to increase in severity (Figure 2B), and the left eye was enucleated to prevent tumor spread, approximately 2 1/2 years after initial diagnosis.
(A) Gross photograph of enucleated eye showing a calcific scar of the regressed macular retinoblastoma and extensive vitreous seeding. (B) Section through the macula with the optic nerve to the right of the photograph. Macular scar in area without calcification; the scar consists of glial cell proliferation and retinal pigment epithelium hyperplasia. In addition, a portion of the retina has shifted into the choroid through a break in Bruch's membrane (hematoxylin-eosin, original magnification 100x). (C) Temporal, equatorial retina with numerous tumor seeds (basophilic cells, see arrow) in the overlying vitreous (hematoxylin-eosin, original magnification 100x). (D) Vitreous seeds with markedly positive nuclear reaction to Ki67 (original magnification 200x).
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Gross examination of the sectioned enucleated eye showed extensive tumor seeding within the vitreous cavity (Figure 3A). Histopathologic examination showed complete regression and calcification of the macular tumor. (Figure 3B). Numerous tumor seeds were seen lying on the internal limiting membrane (Figure 3C), but the retina away from the macular scar was otherwise normal in appearance. The diffuse vitreous seeding demonstrated 80% positive staining with Ki67 (Figure 3D), an immunohistochemistry marker for cellular proliferation.
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(A) Fundus photograph of the right eye showing partially regressed superotemporal tumor and vitreous seeds overlying the macula. (B) Same view of right fundus showing no regression of the vitreous seeds despite three sessions of intra-arterial chemotherapy.
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Fundus examination revealed a partially regressed supero-temporal tumor, with multiple vitreous seeds overlying the macula (Figure 4A). Examination under anesthesia after the third session of IAC showed a slight increase in the vitreous seeding, and no response by the main tumor mass (Figure 4B).
(A) Gross photograph of enucleated eye showing main superotemporal tumor and vitreous seeds overlying the macula (arrow). (B) The main tumor consisting of well-differentiated cells with abundant cytoplasm (hematoxylin-eosin, original magnification 400x). (C) A vitreous seed containing differentiated cells, with negative staining with Ki67 (hematoxylin-eosin, original magnification 400x).
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Gross evaluation of the sectioned enucleated eye revealed a 5 x 5 mm mass superotemporally on the retina with calcifications (Figure 5A). The mass was differentiated, containing cells with small bland nuclei and relatively abundant eosinophilic cytoplasm (Figure 5B). The immunohistochemical stain for Ki-67, a cellular proliferation marker, revealed no positive reactions in either the main tumor or the vitreous seeds, consistent with their differentiated appearance (Figure 5C).