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Pheochromocytoma: An Imaging Chameleon¹

¹ From the Department of Radiology, Division of Abdominal Imaging and Intervention, Massachusetts General Hospital, White 270, 55 Fruit St, Boston, MA 02114 (M.A.B., M.K.K., M.M.M., D.V.S., P.R.M., P.F.H., G.W.B.); and the Department of Medicine, Division of Endocrinology, St Elizabeth’s Medical Center, Boston, Mass (A.T.S.). Presented as an education exhibit at the 2003 RSNA scientific assembly. Received February 12, 2004; revision requested March 14 and received April 27; accepted May 5. All authors have no financial relationships to disclose. Address correspondence to M.A.B. (e-mail: mblake2@partners.org).

View larger version (157K): [in a new window]   Figure 1a.  Paraganglioma at the organ of Zuckerkandl in a 45-year-old woman. (a, b) Axial T2-weighted (a) and coronal gadolinium-enhanced T1-weighted (b) fat-saturated MR images show an avidly enhancing mass with high signal intensity at the aortic bifurcation (arrow in b). (c) Contrast material-enhanced CT scan demonstrates the mass with high attenuation. Paragangliomas can occur anywhere along the course of the sympathetic chain.

View larger version (183K): [in a new window]   Figure 1b.  Paraganglioma at the organ of Zuckerkandl in a 45-year-old woman. (a, b) Axial T2-weighted (a) and coronal gadolinium-enhanced T1-weighted (b) fat-saturated MR images show an avidly enhancing mass with high signal intensity at the aortic bifurcation (arrow in b). (c) Contrast material-enhanced CT scan demonstrates the mass with high attenuation. Paragangliomas can occur anywhere along the course of the sympathetic chain.

View larger version (154K): [in a new window]   Figure 1c.  Paraganglioma at the organ of Zuckerkandl in a 45-year-old woman. (a, b) Axial T2-weighted (a) and coronal gadolinium-enhanced T1-weighted (b) fat-saturated MR images show an avidly enhancing mass with high signal intensity at the aortic bifurcation (arrow in b). (c) Contrast material-enhanced CT scan demonstrates the mass with high attenuation. Paragangliomas can occur anywhere along the course of the sympathetic chain.

View larger version (164K): [in a new window]   Figure 2a.  Large pheochromocytoma in a 42-year-old woman. (a) Contrast-enhanced CT scan demonstrates a right adrenal mass due to pheochromocytoma (arrow). (b, c) Axial T2-weighted (b) and coronal gadolinium-enhanced T1-weighted (c) MR images demonstrate the large nonfunctioning pheochromocytoma (arrow in c). Clinically silent pheochromocytomas tend to be larger.

View larger version (158K): [in a new window]   Figure 2b.  Large pheochromocytoma in a 42-year-old woman. (a) Contrast-enhanced CT scan demonstrates a right adrenal mass due to pheochromocytoma (arrow). (b, c) Axial T2-weighted (b) and coronal gadolinium-enhanced T1-weighted (c) MR images demonstrate the large nonfunctioning pheochromocytoma (arrow in c). Clinically silent pheochromocytomas tend to be larger.

View larger version (148K): [in a new window]   Figure 2c.  Large pheochromocytoma in a 42-year-old woman. (a) Contrast-enhanced CT scan demonstrates a right adrenal mass due to pheochromocytoma (arrow). (b, c) Axial T2-weighted (b) and coronal gadolinium-enhanced T1-weighted (c) MR images demonstrate the large nonfunctioning pheochromocytoma (arrow in c). Clinically silent pheochromocytomas tend to be larger.

View larger version (147K): [in a new window]   Figure 3a.  Malignant pheochromocytoma in a 62-year-old man. (a) Contrast-enhanced CT scan shows a complex left adrenal mass (solid arrows) representing a malignant pheochromocytoma with hepatic metastases (open arrow) and portocaval adenopathy. (b) Pelvic CT scan shows sacral and left iliac bone metastases (arrow). Metastatic spread is the only reliable criterion for differentiating a benign from a malignant pheochromocytoma.

View larger version (151K): [in a new window]   Figure 3b.  Malignant pheochromocytoma in a 62-year-old man. (a) Contrast-enhanced CT scan shows a complex left adrenal mass (solid arrows) representing a malignant pheochromocytoma with hepatic metastases (open arrow) and portocaval adenopathy. (b) Pelvic CT scan shows sacral and left iliac bone metastases (arrow). Metastatic spread is the only reliable criterion for differentiating a benign from a malignant pheochromocytoma.

View larger version (36K): [in a new window]   Figure 4.  Diagram illustrates a proposed algorithm for the assessment of an adrenal mass and recognition of a pheochromocytoma (Pheo). Hx = history, MIBG = metaiodobenzylguanidine, PET = positron emission tomography.

View larger version (44K): [in a new window]   Figure 5.  Chart illustrates the key imaging features of pheochromocytoma. FDG = 2-[fluorine 18]fluoro-2-deoxy-D-glucose, MIBG = metaiodobenzylguanidine.

View larger version (153K): [in a new window]   Figure 6.  Hemorrhagic pheochromocytoma in a 42-year-old man. CT scan shows extensive hemorrhage (highest attenuation value = 100 HU) within and surrounding a left adrenal pheochromocytoma (arrow). Hyperattenuation within a pheochromocytoma may occur as a result of acute hemorrhage.

View larger version (151K): [in a new window]   Figure 7a.  Spectrum of complex pheochromocytomas. (a) Unenhanced CT scan in a 72-year-old woman demonstrates a heterogeneously attenuating mass in the right adrenal gland (arrow). (b) Contrast-enhanced CT scan in an 80-year-old man shows a mass in the right adrenal gland (arrow) with peripheral enhancement and a central area of low attenuation due to necrosis. (c) Unenhanced CT scan in a 41-year-old man shows a mass with cystic and solid components in the left adrenal gland. (d) Contrast-enhanced CT scan in a 59-year-old woman demonstrates a cystic adrenal lesion with a calcified rim (arrow). Pheochromocytomas display a wide variety of appearances at CT.

View larger version (193K): [in a new window]   Figure 7b.  Spectrum of complex pheochromocytomas. (a) Unenhanced CT scan in a 72-year-old woman demonstrates a heterogeneously attenuating mass in the right adrenal gland (arrow). (b) Contrast-enhanced CT scan in an 80-year-old man shows a mass in the right adrenal gland (arrow) with peripheral enhancement and a central area of low attenuation due to necrosis. (c) Unenhanced CT scan in a 41-year-old man shows a mass with cystic and solid components in the left adrenal gland. (d) Contrast-enhanced CT scan in a 59-year-old woman demonstrates a cystic adrenal lesion with a calcified rim (arrow). Pheochromocytomas display a wide variety of appearances at CT.

View larger version (169K): [in a new window]   Figure 7c.  Spectrum of complex pheochromocytomas. (a) Unenhanced CT scan in a 72-year-old woman demonstrates a heterogeneously attenuating mass in the right adrenal gland (arrow). (b) Contrast-enhanced CT scan in an 80-year-old man shows a mass in the right adrenal gland (arrow) with peripheral enhancement and a central area of low attenuation due to necrosis. (c) Unenhanced CT scan in a 41-year-old man shows a mass with cystic and solid components in the left adrenal gland. (d) Contrast-enhanced CT scan in a 59-year-old woman demonstrates a cystic adrenal lesion with a calcified rim (arrow). Pheochromocytomas display a wide variety of appearances at CT.

View larger version (177K): [in a new window]   Figure 7d.  Spectrum of complex pheochromocytomas. (a) Unenhanced CT scan in a 72-year-old woman demonstrates a heterogeneously attenuating mass in the right adrenal gland (arrow). (b) Contrast-enhanced CT scan in an 80-year-old man shows a mass in the right adrenal gland (arrow) with peripheral enhancement and a central area of low attenuation due to necrosis. (c) Unenhanced CT scan in a 41-year-old man shows a mass with cystic and solid components in the left adrenal gland. (d) Contrast-enhanced CT scan in a 59-year-old woman demonstrates a cystic adrenal lesion with a calcified rim (arrow). Pheochromocytomas display a wide variety of appearances at CT.

View larger version (128K): [in a new window]   Figure 8a.  Pheochromocytoma containing fat in a 49-year-old woman with refractory hypertension. (a) Unenhanced CT scan shows a low-attenuation (8 HU) adrenal mass (arrow). (b) Photomicrograph (original magnification, x300; hematoxylin-eosin stain) shows that the cells contain bubbly fat. Pheochromocytomas can infrequently mimic an adenoma at unenhanced CT.

View larger version (164K): [in a new window]   Figure 8b.  Pheochromocytoma containing fat in a 49-year-old woman with refractory hypertension. (a) Unenhanced CT scan shows a low-attenuation (8 HU) adrenal mass (arrow). (b) Photomicrograph (original magnification, x300; hematoxylin-eosin stain) shows that the cells contain bubbly fat. Pheochromocytomas can infrequently mimic an adenoma at unenhanced CT.

View larger version (179K): [in a new window]   Figure 9.  Paraganglioma containing fat in a 39-year-old woman. Contrast-enhanced CT scan shows a left paraaortic paraganglioma (solid arrow) that contains macroscopic fat-attenuation components (open arrow). The mass also contains highly vascular components posteriorly. Extraadrenal paragangliomas share many of the imaging features of adrenal pheochromocytomas.

View larger version (156K): [in a new window]   Figure 10a.  Pheochromocytoma in a 42-year-old man. Axial fat-saturated T1-weighted (a) and T2-weighted (b) MR images show a left adrenal mass (arrow in a) representing a pheochromocytoma. The mass has heterogeneous high signal intensity on the T2-weighted image. Approximately 65% of pheochromocytomas have high signal intensity at T2-weighted MR imaging.

View larger version (140K): [in a new window]   Figure 10b.  Pheochromocytoma in a 42-year-old man. Axial fat-saturated T1-weighted (a) and T2-weighted (b) MR images show a left adrenal mass (arrow in a) representing a pheochromocytoma. The mass has heterogeneous high signal intensity on the T2-weighted image. Approximately 65% of pheochromocytomas have high signal intensity at T2-weighted MR imaging.

View larger version (120K): [in a new window]   Figure 11.  Pheochromocytoma in a 37-year-old woman. T2-weighted MR image shows a low-signal-intensity mass (arrow). Although most pheochromocytomas have high signal intensity on T2-weighted MR images, low-signal-intensity pheochromocytomas are sufficiently common to make this finding unreliable for differentiating pheochromocytoma from other adrenal or retroperitoneal masses.

View larger version (126K): [in a new window]   Figure 12a.  Large pheochromocytoma with central necrosis in a 66-year-old man. Unenhanced T1-weighted (a), T2-weighted (b), and coronal gadolinium-enhanced T1-weighted (c) MR images show a large right adrenal mass with peripheral marked enhancement and central necrosis (arrows in a and c). Smaller pheochromocytomas tend to be solid.

View larger version (141K): [in a new window]   Figure 12b.  Large pheochromocytoma with central necrosis in a 66-year-old man. Unenhanced T1-weighted (a), T2-weighted (b), and coronal gadolinium-enhanced T1-weighted (c) MR images show a large right adrenal mass with peripheral marked enhancement and central necrosis (arrows in a and c). Smaller pheochromocytomas tend to be solid.

View larger version (125K): [in a new window]   Figure 12c.  Large pheochromocytoma with central necrosis in a 66-year-old man. Unenhanced T1-weighted (a), T2-weighted (b), and coronal gadolinium-enhanced T1-weighted (c) MR images show a large right adrenal mass with peripheral marked enhancement and central necrosis (arrows in a and c). Smaller pheochromocytomas tend to be solid.

View larger version (149K): [in a new window]   Figure 13a.  Paraganglioma in a 54-year-old man. (a) CT scan shows a mass with a focus of calcification near the splenic hilum (arrow) representing a paraganglioma. (b) MIBG scintigram shows corresponding avid uptake (arrow). MIBG scintigraphy is often useful for confirming that a mass is a pheochromocytoma. MIBG has almost 100% specificity but limited sensitivity.

View larger version (73K): [in a new window]   Figure 13b.  Paraganglioma in a 54-year-old man. (a) CT scan shows a mass with a focus of calcification near the splenic hilum (arrow) representing a paraganglioma. (b) MIBG scintigram shows corresponding avid uptake (arrow). MIBG scintigraphy is often useful for confirming that a mass is a pheochromocytoma. MIBG has almost 100% specificity but limited sensitivity.

View larger version (163K): [in a new window]   Figure 14a.  MEN in a 44-year-old woman. (a) T2-weighted MR image shows a right adrenal pheochromocytoma with high signal intensity (arrow). (b) T1-weighted MR image shows a pancreatic gastrinoma (arrow). (c) Axial scintigram obtained with octreotide shows increased uptake in both the right adrenal gland and pancreas that corresponds to sites of pheochromocytoma (solid arrow) and gastrinoma (open arrow), respectively. Pheochromocytomas are seen in a number of syndromes, including MEN II and III.

View larger version (135K): [in a new window]   Figure 14b.  MEN in a 44-year-old woman. (a) T2-weighted MR image shows a right adrenal pheochromocytoma with high signal intensity (arrow). (b) T1-weighted MR image shows a pancreatic gastrinoma (arrow). (c) Axial scintigram obtained with octreotide shows increased uptake in both the right adrenal gland and pancreas that corresponds to sites of pheochromocytoma (solid arrow) and gastrinoma (open arrow), respectively. Pheochromocytomas are seen in a number of syndromes, including MEN II and III.

View larger version (75K): [in a new window]   Figure 14c.  MEN in a 44-year-old woman. (a) T2-weighted MR image shows a right adrenal pheochromocytoma with high signal intensity (arrow). (b) T1-weighted MR image shows a pancreatic gastrinoma (arrow). (c) Axial scintigram obtained with octreotide shows increased uptake in both the right adrenal gland and pancreas that corresponds to sites of pheochromocytoma (solid arrow) and gastrinoma (open arrow), respectively. Pheochromocytomas are seen in a number of syndromes, including MEN II and III.

View larger version (101K): [in a new window]   Figure 15a.  Pheochromocytoma in a 50-year-old man. Coronal (a) and axial (b) FDG PET scans show increased uptake (arrow) due to a right adrenal pheochromocytoma. Pheochromocytomas and metastases usually show increased uptake at FDG PET. (c) Corresponding CT scan shows the right adrenal pheochromocytoma (arrow).

View larger version (91K): [in a new window]   Figure 15b.  Pheochromocytoma in a 50-year-old man. Coronal (a) and axial (b) FDG PET scans show increased uptake (arrow) due to a right adrenal pheochromocytoma. Pheochromocytomas and metastases usually show increased uptake at FDG PET. (c) Corresponding CT scan shows the right adrenal pheochromocytoma (arrow).

View larger version (175K): [in a new window]   Figure 15c.  Pheochromocytoma in a 50-year-old man. Coronal (a) and axial (b) FDG PET scans show increased uptake (arrow) due to a right adrenal pheochromocytoma. Pheochromocytomas and metastases usually show increased uptake at FDG PET. (c) Corresponding CT scan shows the right adrenal pheochromocytoma (arrow).

View larger version (179K): [in a new window]   Figure 16a.  Malignant paraaortic paraganglioma in a 32-year-old woman. Consecutive axial images from a contrast-enhanced CT study show a large left paraaortic paraganglioma (arrows) with central necrosis and invasion of the contiguous aortic wall. Ten percent of pheochromocytomas are malignant.

View larger version (191K): [in a new window]   Figure 16b.  Malignant paraaortic paraganglioma in a 32-year-old woman. Consecutive axial images from a contrast-enhanced CT study show a large left paraaortic paraganglioma (arrows) with central necrosis and invasion of the contiguous aortic wall. Ten percent of pheochromocytomas are malignant.