A Late-Detected Paraganglioma in a Young Patient with Resistant Hypertension and Severe Aortic Regurgitation-A Case Report and Review of the Literature

Abstract

Background: Paraganglioma is a rare neuroendocrine tumor derived from chromaffin cells. The overproduction of catecholamines accounts for the presenting symptoms and cardiovascular complications. The clinical presentation frequently overlaps with the associated cardiac diseases, delaying the diagnosis. Multimodality imaging and a multidisciplinary team are essential for the correct diagnosis and adequate clinical management.

Case summary: A 37-year-old woman with a personal medical history of long-standing arterial hypertension and radiofrequency ablation for atrioventricular nodal reentry tachycardia presented with progressive exertional dyspnea and elevated blood pressure values, despite a comprehensive pharmacological treatment with six antihypertensive drugs. The echocardiography showed a bicuspid aortic valve and severe aortic regurgitation. The computed tomography angiography revealed a retroperitoneal space-occupying solid lesion, with imaging characteristics suggestive of a paraganglioma. The multidisciplinary team concluded that tumor resection should be completed first, followed by an aortic valve replacement if necessary. The postoperative histopathology examination confirmed the diagnosis of paraganglioma. After the successful resection of the tumor, the patient was asymptomatic, and the intervention for aortic valve replacement was delayed.

Discussion: This was a rare case of a late-detected paraganglioma in a young patient with resistant hypertension overlapping the clinical presentation and management of severe aortic regurgitation. A multimodality imaging approach including transthoracic and transesophageal echocardiography, computed tomography, and magnetic resonance imaging had an emerging role in establishing the diagnosis and in guiding patient management and follow-up. The resection of paraganglioma was essential for the optimal timing of surgical correction for severe aortic regurgitation. We further reviewed various cardiovascular complications induced by pheochromocytomas and paragangliomas.

Keywords: aortic regurgitation; multimodality imaging; paraganglioma; secondary arterial hypertension.

Figure 1

Transthoracic and transesophageal echocardiography. (A,B) Mid-esophageal short-axis view- bicuspid aortic valve with the fusion of the right and noncoronary cusp and color Doppler of the aortic valve with severe regurgitation. (C) Mid-esophageal long-axis view- color Doppler showing severe eccentric aortic regurgitation jet, with the jet width/left ventricle outflow tract (LVOT) diameter >90%. (D) Parasternal short-axis view- severe concentric left ventricular hypertrophy. (E) Bull’s-eye plot of longitudinal strain showing the reduced global longitudinal strain. (F) Pulsed Doppler recording within the descending aorta demonstrates flow reversal throughout the diastole, with an end diastolic flow velocity of 27 cm/s.

Figure 2

Abdominal CT with contrast shows an inhomogeneous, well-delimited solid mass in the retroperitoneal cavity with areas of necrosis and with strong arterial enhancement (white arrows). (A) Axial section. (B) Sagittal oblique MPR reconstruction. (C) Horseshoe kidney, with symphysis at the level of the lower pole of the right kidney and left kidney.

Figure 3

Intraoperative images: (A) retroperitoneal paraganglioma; (B) left colic artery; (C) inferior mesenteric vein; (D) Treitz angle; (E) main arterial pedicle—branch of the inferior mesenteric artery.

Figure 4

Histopathological examination. (A) Hematoxylin–eosin staining, ×100: nests and cords of epithelioid cells separated by a thin fibrovascular stroma. (B) Synaptophysin immunohistochemical stain, ×100: tumor cells have a diffuse strong cytoplasmatic positivity.

Figure 5

Cardiac magnetic resonance imaging: (A) Cine Fiesta sequence in the transverse plane: bicuspid aortic valve type 0, lateral (arrows). (B) Cine Fiesta sequence 3: aortic insufficiency jet (arrow). (C,D) Sequence 4D flow: aorta in systole (C), blood flow coded in red (arrow), and in diastole (D), highlighting a jet of severe aortic valvular insufficiency with oblique orientation (arrow).