Somatic PRKAR1A mutation in sporadic atrial myxoma with cerebral parenchymal metastases: a case report

Figures

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  Fig. 1. a–n Timeline of patient: representative images. Representative images from April 2017 (7 months after resection of atrial myxoma) with fluid-attenuated inversion recovery (a, b) and gradient recalled echo sequences (c, d) showing small hemorrhagic lesions throughout the bilateral frontal, parietal, and occipital lobes. Follow-up imaging from October 2017 shows interval increase in size of numerous lesions on fluid-attenuated inversion recovery (e, f) and susceptibility weighted imaging (g, h). i (right internal carotid artery) and j (left internal carotid artery) are representative images from cerebral angiogram done in October 2017, demonstrating multiple mycotic aneurysms. Fluid-attenuated inversion recovery (k, l) and susceptibility weighted imaging (m, n) sequences from repeat magnetic resonance imaging of the brain in March 2018 (post-radiation)

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  Fig. 2. a–g Left atrial myxoma. a In situ photograph of the myxoma at the time of surgery consisting of a complex papillary structure comprising grape-like clusters organized into an arborizing network. b Photograph of the excised atrial myxoma consisting of a tree-like structure with several arborizing branches. c Six micron section of the myxoma whole mount illustrating the arborizing network of grape-like clusters converging on a fibrous stalk. d, e Low (d) and high (e) power images illustrating lepidic cells lining the surfaces (d) and cells in a myxoid matrix forming abortive clusters of vessel-like structures (e). f, g Intermediate (f) and high (g) power images of occasional clusters of rounded cells at and near the surfaces displaying a lack of cohesion, forming small groups of cells separating from the surfaces of the myxoma

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  Fig. 3. a–c Micrographs of the image-directed brain biopsy. Micrographs of low (a), intermediate (b), and high (c) power illustrating images of the brain biopsy stained with anti-calretinin antibody. a Low power overview of an arterial vessel illustrating replacement of the normal lining endothelium with metastatic myxoma cells stained for calretinin and direct extension of the tumor cells through the vessel wall and into surrounding brain parenchyma. The box denotes the microscopic field presented in panel b. b Intermediate power image illustrating the replacement of normal endothelium with anti-calretinin-staining atrial myxoma cells and invasion of the vessel wall and brain parenchyma by the myxoma cells. c High power image illustrating individual calretinin-positive atrial myxoma cells which have replaced the normal vessel endothelium and the subendothelium (yellow arrowheads) with myxoid matrix and invasion by individual tumor cells through the media of the vessel wall and into adjacent brain parenchyma (yellow arrows). The dashed lines denote the internal and external elastic lamina marking the vascular smooth muscle and extracellular matrix the comprises the media of the vessel