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Extraadrenal Paragangliomas of the Body: Imaging Features

Ki Yeol Lee1, Yu-Whan Oh1, Hyung Jun Noh1, Yu Jin Lee1, Hwan-Seok Yong1, Eun-Young Kang1, Kyeong Ah Kim1 and Nam Joon Lee1

1 All authors: Department of Radiology, Korea University Anam Hospital, College of Medicine Korea University; #126-1, 5-Ka, Anam-Dong, Sungbuk-Ku, Seoul 136-705, Korea.


Figure 1
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  		Fig. 1A —Drawings of paraganglion system. Drawings show anatomic distribution of healthy extraadrenal paraganglia connected with parasympathetic system (A) and sympathetic system (B). APP = aorticopulmonary paraganglia, CBP = carotid body paraganglion, JTP = jugulotympanic paraganglia, VP = vagal paraganglia.

 

Figure 2
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  		Fig. 1B —Drawings of paraganglion system. Drawings show anatomic distribution of healthy extraadrenal paraganglia connected with parasympathetic system (A) and sympathetic system (B). APP = aorticopulmonary paraganglia, CBP = carotid body paraganglion, JTP = jugulotympanic paraganglia, VP = vagal paraganglia.

 

Figure 3
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  		Fig. 2 —Gross pathologic specimen obtained at surgery in 23-year-old man with retroperitoneal paraganglioma. Photograph of resected specimen shows meaty to light tan appearance of 5.5-cm tumor with slightly bulging surface. Note multiple feeding vessels within tumor (arrowheads). Scale = 0.5 cm.

 

Figure 4
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  		Fig. 3 —Gross pathologic specimen obtained at surgery in 64-year-old woman with retroperitoneal paraganglioma. Photograph of cut section of specimen shows marked hemorrhage (arrowheads) within tumor. Scale = 0.5 cm.

 

Figure 5
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  		Fig. 4 —Microscopic appearance of carotid body tumor from 30-year-old woman. Photomicrograph of histologic specimen shows organoid arrangement of neoplastic chief cells (arrowhead) surrounded by highly vascularized fibrous septa (arrows). (H and E, x400)

 

Figure 6
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  		Fig. 5A —30-year-old woman with carotid body tumor. On unenhanced (A) and contrast-enhanced (B) CT scans, hyperenhancing mass measuring 1.2 cm in diameter (arrow) is seen in left carotid space, where tumor is typically located between left internal carotid artery and external carotid artery.

 

Figure 7
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  		Fig. 5B —30-year-old woman with carotid body tumor. On unenhanced (A) and contrast-enhanced (B) CT scans, hyperenhancing mass measuring 1.2 cm in diameter (arrow) is seen in left carotid space, where tumor is typically located between left internal carotid artery and external carotid artery.

 

Figure 8
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  		Fig. 6A —35-year-old man with carotid body tumor. Coronal T1-weighted image shows tumor (arrow) of isointensity at left common carotid bifurcation. Note multiple flow voids (arrowheads) within tumor.

 

Figure 9
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  		Fig. 6B —35-year-old man with carotid body tumor. Axial T2-weighted image shows heterogeneous high signal intensity of tumor (arrow) in left carotid space. Salt-and-pepper appearance is noted in tumor.

 

Figure 10
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  		Fig. 6C —35-year-old man with carotid body tumor. Gadolinium-enhanced T1-weighted image shows heterogeneously enhancing tumor mass (arrow) in left carotid space.

 

Figure 11
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  		Fig. 6D —35-year-old man with carotid body tumor. Lateral angiogram of left common carotid artery shows splaying of internal carotid artery and external carotid artery by hypervascular mass (arrow).

 

Figure 12
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  		Fig. 7A —31-year-old woman with glomus jugulare tumor. Contrast-enhanced axial CT scan shows left jugular foramen mass (arrow) with irregular margins and lytic changes of surrounding temporal bone.

 

Figure 13
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  		Fig. 7B —31-year-old woman with glomus jugulare tumor. Axial CT scans obtained 5 mm (B) and 10 mm (C) cranial to A show well-enhanced tumor mass (arrowhead) extending through jugular foramen into posterior cranial fossa.

 

Figure 14
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  		Fig. 7C —31-year-old woman with glomus jugulare tumor. Axial CT scans obtained 5 mm (B) and 10 mm (C) cranial to A show well-enhanced tumor mass (arrowhead) extending through jugular foramen into posterior cranial fossa.

 

Figure 15
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  		Fig. 8A —51-year-old woman with glomus tympanicum tumor. Axial (A) and coronal (B) thin-section CT scans of temporal bone (bone window) show 5-mm soft-tissue mass (arrow) filling hypotympanum of left middle ear cavity.

 

Figure 16
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  		Fig. 8B —51-year-old woman with glomus tympanicum tumor. Axial (A) and coronal (B) thin-section CT scans of temporal bone (bone window) show 5-mm soft-tissue mass (arrow) filling hypotympanum of left middle ear cavity.

 

Figure 17
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  		Fig. 9A —37-year-old woman with vagal paraganglioma. Axial T1-weighted MR image shows lobulated mass (arrows) with isointensity in right parapharyngeal space.

 

Figure 18
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  		Fig. 9B —37-year-old woman with vagal paraganglioma. Tumor (arrows) shows inhomogeneous high signal intensity on T2-weighted MR image.

 

Figure 19
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  		Fig. 9C —37-year-old woman with vagal paraganglioma. T2-weighted 2D fast low-angle shot (FLASH) image shows marked hyperintensity of tumor (arrow).

 

Figure 20
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  		Fig. 9D —37-year-old woman with vagal paraganglioma. Gadolinium-enhanced coronal T1-weighted MR image shows enhancing tumor extending from skull base down to level of common carotid bifurcation. Note multiple flow voids (arrowheads) within tumor.

 

Figure 21
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  		Fig. 10A —51-year-old man with aortic body tumor. Chest radiograph shows large mass (arrow) projecting from left side of mediastinum.

 

Figure 22
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  		Fig. 10B —51-year-old man with aortic body tumor. Contrast-enhanced CT scan shows large tumor (arrow) with mild homogeneous enhancement in anterior mediastinum.

 

Figure 23
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  		Fig. 11A —29-year-old man with posterior mediastinal paraganglioma. Chest radiograph shows large lobulated mass in left paraspinal area (arrow).

 

Figure 24
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  		Fig. 11B —29-year-old man with posterior mediastinal paraganglioma. Contrast-enhanced CT scan shows hyperenhancing mass with cystic change (arrowhead) in left posterior mediastinum.

 

Figure 25
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  		Fig. 11C —29-year-old man with posterior mediastinal paraganglioma. CT scan obtained 3 cm below B shows linear and punctate foci of calcification (arrow) within tumor mass.

 

Figure 26
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  		Fig. 12A —23-year-old man with retroperitoneal paraganglioma arising from organs of Zuckerkandl. Contrast-enhanced CT scan shows inhomogeneously enhancing soft-tissue mass (arrow) located in right paraaortic area. Note focal area of low attenuation (arrowhead) within tumor.

 

Figure 27
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  		Fig. 12B —23-year-old man with retroperitoneal paraganglioma arising from organs of Zuckerkandl. Metaiodobenzylguanidine (MIBG) scintigram shows focal area of intense radiotracer uptake (arrow) in mid abdomen.

 

Figure 28
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  		Fig. 13A —64-year-old woman with retroperitoneal paraganglioma. Unenhanced (A) and enhanced (B) CT scans show 4.5-cm mass (arrow) with heterogeneous enhancement in left paraaortic area. Note large cystic component with fluid-fluid level (arrowhead) within tumor mass. High attenuation of dependent fluid in cystic portion is suggestive of intratumoral hemorrhage.

 

Figure 29
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  		Fig. 13B —64-year-old woman with retroperitoneal paraganglioma. Unenhanced (A) and enhanced (B) CT scans show 4.5-cm mass (arrow) with heterogeneous enhancement in left paraaortic area. Note large cystic component with fluid-fluid level (arrowhead) within tumor mass. High attenuation of dependent fluid in cystic portion is suggestive of intratumoral hemorrhage.

 

Figure 30
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  		Fig. 14A —67-year-old man with duodenal gangliocytic paraganglioma. Unenhanced (A) and contrast-enhanced (B) CT scans show intraluminal soft-tissue mass (arrow) with hyperenhancement in second part of duodenum.

 

Figure 31
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  		Fig. 14B —67-year-old man with duodenal gangliocytic paraganglioma. Unenhanced (A) and contrast-enhanced (B) CT scans show intraluminal soft-tissue mass (arrow) with hyperenhancement in second part of duodenum.

 

Figure 32
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  		Fig. 14C —67-year-old man with duodenal gangliocytic paraganglioma. T2-weighted MR image shows duodenal mass (arrow) of high signal intensity relative to that of muscle.

 

Figure 33
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  		Fig. 14D —67-year-old man with duodenal gangliocytic paraganglioma. Endoscopic image of duodenum shows polypoid submucosal mass (arrow) with smooth margin in periampullary area.

 

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