PMC9758552_01
Physical findings included (A) globular asymmetric abdomen with (B) wide violaceous striae and (C) ecchymoses.
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Her abdomen was asymmetric, with diffuse wide, purplish striae and a palpable mass at the left side (Figures 1A and 1B). Ecchymoses were noted on the extremities (Figure 1C).
Computerized tomography prior to treatment. (A) Plain scan of the whole abdomen showed a heterogeneously large left adrenal mass measuring 19 cm x 14.4 cm x 14 cm in its greatest and perpendicular dimensions (yellow arrow); (B) angiography of the aorta showed the extent of the tumor at different aortic segments: aortic root mid-ascending, proximal arch, mid-descending, distal descending, suprarenal abdominal and infrarenal abdominal. Enlargement of the inferior vena cava with intraluminal filling defects was seen along its entire course from T11 to the bifurcation of common iliac veins, as well as in the left renal, left common iliac, and left ovarian veins (red arrows). The stomach and spleen were also displaced.
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Abdominal computed tomography (CT) revealed a large, heterogeneously enhancing left adrenal mass measuring 19 cm x 14.4 cm x 14 cm, displacing the stomach and spleen (Figure 2A). The IVC was enlarged with an intraluminal filling defect along the entire course of the IVC from the level of T11 down to the bifurcation of common iliac veins, including the left renal vein, left common iliac, and left ovarian vein (Figure 2B).
Open biopsy showing a large and firm left adrenal mass with hypervascularity.
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A large and firm left adrenal mass with hypervascularity was noted on open biopsy (Figure 3).
Histopathologic examination showed diffuse sheets of cells containing eosinophilic cytoplasm, <25% clear cells, focal areas with highly pleomorphic tumor cells, mitosis in less than 5 per 50 hpf and no atypical mitotic figures. Hemorrhage and necrosis without lympho-vascular invasion were also observed. (H&E, 200x).
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Microscopically, the specimen showed diffuse sheets of cells having eosinophilic cytoplasm with clear cells comprising <25% of the field. (Figure 4). There were areas with hemorrhage and necrosis, with no lymphovascular invasion (Figure 4).
Immunohistochemical staining for was positive for (A) Melan A, (B) Inhibin, (C) Vimentin and (D) Synaptophysin and negative for (E) Chromogranin, (F) Ki-67 positivity was seen in 30% of neoplastic cells.
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Ki-67, an indicator of poor prognosis, was estimated at 30% (Figure 5).
Computerized tomography of the abdomen with intravenous contrast after treatment. (A) Sagittal view taken three months after treatment. There was no interval change in the left adrenal mass size (yellow arrow), with tumor thrombus involving the inferior vena cava (IVC) and left renal, left adrenal and bilateral common iliac veins. (B) Coronal view taken six months after treatment. The left adrenal mass was found be slightly increased in size, abutting the spleen and pancreas, with probable invasion of the adjacent left kidney. Similar findings of tumor thrombus involving the IVC and left renal, left adrenal and both common iliac veins were also seen (red arrows).
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On the third month of follow-up, a repeat abdominal CT scan with contrast showed stable disease (Figure 6A). Eight months post-chemotherapy, a surveillance abdominal CT scan showed progressive disease (Figure 6B).