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Best Cases from the AFIP

Paraganglioma of the Organs of Zuckerkandl¹

¹ From the Departments of Radiology (D.P.S., J.B.K.), Pathology (I.E.S.), and Surgery (S.P.), Beth Israel Deaconess Medical Center and Harvard Medical School, 1 Deaconess Rd, West 203B, Boston MA 02215. Received January 28, 2003; revision requested February 26 and received April 2; accepted April 4. Address correspondence to J.B.K. (e-mail: jkruskal@bidmc.harvard.edu).

View larger version (125K): [in a new window]   Figure 1a.  (a) Unenhanced abdominal CT scan demonstrates a 6 x 6-cm soft-tissue mass adjacent to the aorta, just superior to the bifurcation. Note the small focus of peripheral calcification (arrow). (b) Contrast-enhanced arterial phase CT scan demonstrates prompt peripheral enhancement with central nonenhancement. Note the thin slip of enhancing tissue just anterior to the aortic wall (arrow); this tissue is not readily distinguishable from the aorta.

View larger version (131K): [in a new window]   Figure 1b.  (a) Unenhanced abdominal CT scan demonstrates a 6 x 6-cm soft-tissue mass adjacent to the aorta, just superior to the bifurcation. Note the small focus of peripheral calcification (arrow). (b) Contrast-enhanced arterial phase CT scan demonstrates prompt peripheral enhancement with central nonenhancement. Note the thin slip of enhancing tissue just anterior to the aortic wall (arrow); this tissue is not readily distinguishable from the aorta.

View larger version (128K): [in a new window]   Figure 2a.  (a) Axial unenhanced in-phase T1-weighted MR image shows a heterogeneous mass that is both slightly hyperintense and hypointense relative to the adjacent psoas muscle. (b) Axial out-of-phase T1-weighted MR image shows no appreciable lipid component. (c) Coronal T2-weighted MR image demonstrates the mass with a stellate hyperintense center. Note that the mass adheres closely to the aorta but does not directly invade the lumen. Note also that the viable tumor rim lacks the typical T2-weighted hyperintensity of most paragangliomas. (d) On a contrast-enhanced arterial phase T1-weighted MR image, the mass demonstrates prompt peripheral enhancement with central nonenhancement.

View larger version (137K): [in a new window]   Figure 2b.  (a) Axial unenhanced in-phase T1-weighted MR image shows a heterogeneous mass that is both slightly hyperintense and hypointense relative to the adjacent psoas muscle. (b) Axial out-of-phase T1-weighted MR image shows no appreciable lipid component. (c) Coronal T2-weighted MR image demonstrates the mass with a stellate hyperintense center. Note that the mass adheres closely to the aorta but does not directly invade the lumen. Note also that the viable tumor rim lacks the typical T2-weighted hyperintensity of most paragangliomas. (d) On a contrast-enhanced arterial phase T1-weighted MR image, the mass demonstrates prompt peripheral enhancement with central nonenhancement.

View larger version (138K): [in a new window]   Figure 2c.  (a) Axial unenhanced in-phase T1-weighted MR image shows a heterogeneous mass that is both slightly hyperintense and hypointense relative to the adjacent psoas muscle. (b) Axial out-of-phase T1-weighted MR image shows no appreciable lipid component. (c) Coronal T2-weighted MR image demonstrates the mass with a stellate hyperintense center. Note that the mass adheres closely to the aorta but does not directly invade the lumen. Note also that the viable tumor rim lacks the typical T2-weighted hyperintensity of most paragangliomas. (d) On a contrast-enhanced arterial phase T1-weighted MR image, the mass demonstrates prompt peripheral enhancement with central nonenhancement.

View larger version (138K): [in a new window]   Figure 2d.  (a) Axial unenhanced in-phase T1-weighted MR image shows a heterogeneous mass that is both slightly hyperintense and hypointense relative to the adjacent psoas muscle. (b) Axial out-of-phase T1-weighted MR image shows no appreciable lipid component. (c) Coronal T2-weighted MR image demonstrates the mass with a stellate hyperintense center. Note that the mass adheres closely to the aorta but does not directly invade the lumen. Note also that the viable tumor rim lacks the typical T2-weighted hyperintensity of most paragangliomas. (d) On a contrast-enhanced arterial phase T1-weighted MR image, the mass demonstrates prompt peripheral enhancement with central nonenhancement.

View larger version (137K): [in a new window]   Figure 3a.  (a) Intraoperative photograph demonstrates an extraadrenal paraganglioma (large arrow) that adheres to the aorta (small arrows). (b) Photograph of the gross specimen shows a 9.5 x 7 x 5-cm mass with focal hemorrhage (curved arrow) and an area of tumor necrosis with a pale yellow rim (straight arrow).

View larger version (164K): [in a new window]   Figure 3b.  (a) Intraoperative photograph demonstrates an extraadrenal paraganglioma (large arrow) that adheres to the aorta (small arrows). (b) Photograph of the gross specimen shows a 9.5 x 7 x 5-cm mass with focal hemorrhage (curved arrow) and an area of tumor necrosis with a pale yellow rim (straight arrow).

View larger version (209K): [in a new window]   Figure 4.  Photomicrograph (original magnification, x40; hematoxylin-eosin [H-E] stain) of the paraganglioma shows a population of cells with abundant cytoplasm and indistinct cell borders (arrow).

View larger version (166K): [in a new window]   Figure 5a.  (a) Photomicrograph (original magnification, x40; H-E stain) demonstrates hemosiderin deposition (arrow) within the neoplasm, a finding that indicates prior hemorrhage. (b) Photomicrograph (original magnification, x40; H-E stain) shows coagulative necrosis with pyknotic nuclear debris (arrow) and neutrophils (arrowhead).

View larger version (196K): [in a new window]   Figure 5b.  (a) Photomicrograph (original magnification, x40; H-E stain) demonstrates hemosiderin deposition (arrow) within the neoplasm, a finding that indicates prior hemorrhage. (b) Photomicrograph (original magnification, x40; H-E stain) shows coagulative necrosis with pyknotic nuclear debris (arrow) and neutrophils (arrowhead).

View larger version (208K): [in a new window]   Figure 6.  Photomicrograph (original magnification, x40; immunoperoxidase stain for synaptophysin, a neuroendocrine marker) shows diffuse positive staining of the cytoplasm. Note the negatively staining blood vessels (arrow).