PMC8848499_01
(A) Axial post-contrast fat-suppressed T1W image; (B) coronal T2W fat-suppressed image that the mass has hypointense weighted heterogeneity in T1 image and hyperintense signaling features in T2 image.
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F1
The mass had about 28x21 cm in size with thick septations in a distinctly thick irregular wall (Fig. 1). In the postcontrast series, there was a contrast enhancement in septa and wall (Fig. 1).
Appearance of the neurofibromas, the cafe-au-lait spots, and the right lobe of the liver through a subcostal incision in the abdomen, right lobe of the liver pushed to the left side of the abdominal cavity by the mass and composite pheochromocytoma that was surgically removed.
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F2
Following subcostal incision, an open was made through an adrenal zone and a mass with 30x23 cm in size was removed (Fig. 2).
Microscopic view of the tumor with areas of pheochromocytoma on the left side and ganglioneuromas on the right (hematoxylin and eosin stain x40) and gastrointestinal stromal tumor composed of fibroid cells in bundle structures (hematoxylin and eosin stain x40).
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F3
S100 (+), neurofilament protein (+), neuron-specific enolase (+), inhibin (-), melanin A (-), CD10 (-), HMB45 (-), and hepar (-). Sustentacular cells were also detected to be S100 (+) (Fig. 3). The tumor was positive in CD117, DOG1, DKA (weakly positive), and negative in S100 and desmin (Fig. 3).