PMC8010837_01
Abdominal computed tomography scan and Positron emission tomography imaging. Contrast-enhanced abdominal CT scan showed inhomogeneous right adrenal mass measuring 8.1 x 7.7 x 7.9 cm (A). A 18F-fluorodeoxyglucose PET-CT showed an area of high uptake in the right adrenal gland, with a prevailing peripheral signal and central hypoactivity, and in the lumbar region suspected of adenopathic localization (B, C). A 68GaDOTA-octreotate (DOTATATE) PET confirmed previous localizations and also detected a high uptake are at the base of the left lung (D).
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Contrast enhanced abdominal CT scan confirmed the presence of inhomogeneous right adrenal mass measuring 8.1 x 7.7 x 7.9 cm ( Figure 1A ). A 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) coupled with CT showed an area of high uptake (maximum standardized uptake value, SUV max 8.6) in the right adrenal gland, with a prevailing peripheral signal and central hypoactivity, and another area of high uptake (SUV max 9.6) in the lumbar region suspicious of lymph node localization ( Figures 1B, C ). In addition, 68GaDOTA-octreotate (DOTATATE) PET confirmed the peripheral high uptake (SUV max 6) in the right adrenal gland and the high uptake area (SUV max 3.7) in the lumbar region; a high uptake (SUV max 4.5) was also detected at the base of the left lung ( Figure 1D ).
Sections from right adrenal gland Morphologic analysis with hematoxylin-eosin staining in (A-D) at different magnification. Panels show a pheochromocytoma, with a typical zellballen pattern of growth (A, 20X), with perivascular cell cuffing around a blood vessel called pseudo-rosette pattern (B, 20X), with neoplastic cells inside vascular spaces (C, 20X), and at greater magnification (D, 40X), an atypical mitotic figure. Positive immunohistochemical staining of Chromogranin A in Panel (E), VIP (F) and anti-Somatostatin Receptor 2A (SSTR2A, H). Double immunostaining showing the co-expression of Chromogranin A (brown) and VIP (red) (G).
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The histological features were consistent with a malignant PHEO, with a PASS score (Pheochromocytoma of the Adrenal Gland Scaled Score) of 20 ( Figures 2A-D ), indicating a high risk of aggressive cellular behavior (PASS>=4). Immunostaining revealed strong positivity for neuroendocrine marker chromogranin A and VIP ( Figures 2E, F ); a large number of cells co-expressed chromogranin A and VIP ( Figure 2G ). Weak was the positivity for SSTR2A ( Figure 2H ).
Metastatic diffusion at chest and hepatic computed tomography scan. Contrast-enhanced abdominal and chest computed tomography scan showed the appearance of multiple metastatic pulmonary and hepatic nodules (A-D).
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Mitotic figures were above 3/10 high power fields, with some atypical mitoses. At 2 months, multiple metastatic pulmonary and hepatic nodules were identified on CT scan ( Figures 3A-D ).