PMC6555272_01
Pre-operative contrast enhanced computed tomography axial image showing a heterogeneous mass, with hypo-attenuating areas corresponding to necrosis or liquid intermixed with stippled calcifications (arrows). *indicates major vessels.
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F1
On the post-contrast study, the mass showed a moderate mixed contrast uptake pattern, with hypointense central areas, suggestive of necrosis or fluid intermixed with stippled calcifications (Figure 1). Cytology revealed non-differentiated neoplasia (Supplementary Figure 1). The tumor was attached to more than 50% of circumference of the vessel, but complete encasement was not observed (Figures 1, 3).
(A) Pre-operative contrast enhanced computed tomography axial image of the right adrenal gland (arrow) with the corresponding phrenicoabdominal vein. (B) Pre-operative contrast enhanced computed tomography axial image of the expected location of the left adrenal gland (arrow). The arrow points to the left phrenicoabdominal vein. The mass extends ventrally.
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F2
The right ureter, adrenal gland, and corresponding phrenicoabdominal vein were clearly identifiable (Figure 2A). The left phrenicoabdominal vein was identified whereas the left adrenal gland and left ureter, which was encased in the retroperitoneal mass, were not (Figure 2B). Tumor cells were positive for neurofilament protein (estimated positivity-10% of the tumor area), neuron specific enolase (NSE), and synaptophysin (SYN) (Supplementary Figures 2-4), and negative for glial fibrillary acidic protein (GFAP), pancytokeratin, lymphoid cell markers CD3 and CD20, and vimentin.
Pre-operative contrast enhanced computed tomography reconstructed oblique sagittal image along the cava vein. The arrow also points to the two compression points.
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F3
Compression led to a reduction of more than 50% of its diameter at the second point (Figure 3). The tumor was attached to more than 50% of circumference of the vessel, but complete encasement was not observed (Figures 1, 3). Tumor cells were positive for neurofilament protein (estimated positivity-10% of the tumor area), neuron specific enolase (NSE), and synaptophysin (SYN) (Supplementary Figures 2-4), and negative for glial fibrillary acidic protein (GFAP), pancytokeratin, lymphoid cell markers CD3 and CD20, and vimentin.
(A) Intraoperative image at time of excision of an adrenal neuroblastoma. (B) Abdominal neuroblastoma excised together with the kidney. Point of mass attachment to the kidney hilus and ureter (asterisks).
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F4
The mass was well-defined but lobulated and heterogeneous, grossly attached to the parietal peritoneum, left kidney, and ureter at the proximal third of the ureter medially (Figure 4A). Therefore, as planned, a nephrectomy was performed (Figure 4B). Tumor cells were positive for neurofilament protein (estimated positivity-10% of the tumor area), neuron specific enolase (NSE), and synaptophysin (SYN) (Supplementary Figures 2-4), and negative for glial fibrillary acidic protein (GFAP), pancytokeratin, lymphoid cell markers CD3 and CD20, and vimentin.
(A) Adrenal tumor. Low magnification image showing round to oval cells organized in ill-defined lobules, within a very poor stroma. (H&E, bar = 100 mum). (B) Adrenal tumor. High magnification image of the tumor cells. The cell limits are ill-defined, the cytoplasm is scant and pale, and the ovoid-shaped nucleus shows stippled chromatin distribution with no conspicuous nucleolus. (H&E, bar = 20 mum).
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F5
Tumor cells were round to oval, organized in lobules within a poor stroma (Figure 5). A very high division rate, estimated at 68%, was confirmed by Ki67 labeling (Supplementary Figure 5).