PMC3971937_01
Automated visual field testing shows bilateral cecocentral visual defects.
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Automated visual field testing (Humphrey 30-2 threshold, Swedish Interactive Threshold Algorithm-standard strategy; Carl Zeiss Meditec, Dublin CA, USA) demonstrated bilateral enlarged blind spots and cecocentral scotomas (Figure 1).
Fundus photos demonstrates bilateral optic disc edema and extensive circumferential exudation with macular involvement and macular star formation.. Note: Color fundus photography of the right and left eyes at presentation demonstrate bilateral optic disc edema and extensive exudation with macular involvement and macular star formation.
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The optic nerve heads had no spontaneous venous pulsations (Figure 2).
MRI FLAIR.. Notes: MRI FLAIR sequencing through the (A) cerebellum and (B) frontal lobes shows cortical and subcortical lesions. One lesion (C) in the left posterior frontal lobe enhanced with contrast and bright on FLAIR also showed hypointensity on T1 signaling and was (D) found to have calcification on the CT scan. DWI MRI (not shown) was negative for acute infarction.. Abbreviations: MRI, magnetic resonance imaging; FLAIR, fluid-attenuated inversion-recovery; DWI, diffusion weighted imaging.
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The brain magnetic resonance imaging (MRI) with and without contrast revealed multiple high T2 and fluid-attenuated inversion-recovery (FLAIR) signal lesions found diffusely in the cortical and subcortical white matter region of the frontal and parietal lobes (Figure 3A). One lesion in the left frontal posterior frontal lobe had higher T1 signaling, and a computed tomography (CT) scan confirmed calcifications of this single lesion (Figure 3C and D).
Contrast-enhanced abdominal CT-scan.. Note: Demonstrates a 3.5x3.0x3.0 cm enhancing lobulated-noncalcified mass (asterisk) in the right adrenal gland.. Abbreviation: CT, computed tomography.
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In an extensive workup, an abdominal CT scan showed a 3.5x3.0x3.0 cm enhancing noncalcified mass in the right adrenal gland (Figure 4).
Histopathology specimen of resected adrenal tumor.. Notes: (A) Tumor arising from the medulla (asterisk) compressing the adrenal cortex (H&E, x40). (B, C) High magnification (H&E, x200 and x400) shows whorl-like nests of tumor cells surrounded by a fibrovascular stroma (asterisks).. Abbreviations H&E, hematoxylin and eosin.
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The pathological specimen showed whorled nests of chromaffin tumor cells (Figure 5) embedded in a fibrovascular stroma.
Automated visual fields, fundus of both eyes, sequential axial FLAIR MRI.. Notes: Six months after resection of the adrenal tumor, automated visual fields and fundus of both eyes improved (A, B). The sequential axial FLAIR MRI shows diminished size and signal of cortical and subcortical lesions (C).. Abbreviations: FLAIR, fluid-attenuated inversion-recovery; MRI, magnetic resonance imaging.
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Fundoscopically, the optic disc edema and exudates improved, and the postoperative MRI showed a subsequent decrease in white matter lesions (Figure 6).
Fundus photograph, fluorescein angiogram.. Notes: Fundus photograph of the right eye shows a vascular dilation (A, asterisk). Fluorescein angiogram of the right eye shows two small hemangioblastomas (B, C). Note the feeder artery and draining vein (C).
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Ophthalmic evaluation at this time showed multiple peripheral vascular dilations and small retinal capillary hemangioblastomas (Figure 7).