Presentation
Hypertension.
Patient Data
Moderate size predominantly isoechoic heterogenous circular mass anterior to the left kidney (please note that the patient was scanned from the back). In these images, the organ of origin is not clear.
Axial arterial phase CT images showing a heterogeneously enhancing left adrenal mass.
6.0 x 5.8 x 6.7 cm avidly but heterogeneously enhancing mass arising from the lateral limb of the left adrenal gland. The mass has internal necrotic and cystic areas. The solid areas show persistent enhancement on both arterial and portal venous phases. In the setting of hypertension, this mass is highly suspicious for pheochromocytoma.
Axial gallium 68 dotatate images and MIP scan show very prominent tracer activity with an SUV up to 67 associated with a large mass in the left upper quadrant with photopenic, necrotic center is most consistent with pheochromocytoma.
No other somatostatin avid lesions to suggest metastatic disease.
Case Discussion
A 15-year-old male patient with a history of hypertension and a 2-year history of intermittent headaches was initially evaluated with renal US which showed an isoechoic heterogenous mass in front of the left kidney. Subsequent contrast-enhanced CT of the abdomen showed the mass to be arising from the left adrenal gland with findings highly suggestive of pheochromocytoma as described above. The diagnosis was confirmed when plasma and urine catecholamines were checked and found to be high, as follows (normal range in parenthesis):
24-hour urine metanephrine: 840 micrograms (56-298)
metanephrine ratio to creatinine: 419 (0-320)
24-hour urine metanephrine, normetanephrine: 11968 micrograms (82-553)
metanephrine, Normetanephrine ration to creatinine: 5978 (0-450)
plasma normetanephrine: 35.90 nmol/L (0.00-0.89)
plasma metanephrine: 1.06 nmol/L (0.0-0-0.49)
Subsequent Ga-68 Dotatate study showed a very high uptake in the mass, consistent with pheochromocytoma. The study did not show any additional somatostatin avid lesions, excluding metastasis.
The patient subsequently underwent left adrenalectomy after appropriate alpha and beta blockade. The biopsy findings were consistent with pheochromocytoma. The pathology specimen showed a neuroendocrine tumor with moderate to abundant cytoplasm, moderate to focally marked atypia, and areas of cellular spindling with a mitotic count of 4/10 and having a PASS (Pheochromocytoma of the adrenal gland scaled score) of at least 6. A PASS score of >4 is suggestive of greater potential for biologically aggressive behavior compared to PASS less than 4.
Immunostains showed that the tumor cells were strongly positive for synaptophysin and chromogranin; and negative for S100, Mart-1, or inhibit, further supporting the above diagnosis.
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