J Korean Soc Radiol.  2017 Jul;77(1):14-18. 10.3348/jksr.2017.77.1.14.

Extra-Adrenal Malignant Paragangliomas Presenting as Mesenteric and Pararectal Masses: A Case Report

• Lee S
• Lee J
• Kim BH
• Kim KA
• Park CM

• KMID: 2384730
• DOI: http://doi.org/10.3348/jksr.2017.77.1.14

Abstract

Extra-adrenal paraganglioma is a rare tumor arising from the neural crest cells. Most tumors that develop in the abdomen arise from paraganglia along the paravertebral and para-aortic areas, in particular the organ of Zuckerkandl, which is close to the origin of the inferior mesenteric artery. However, extra-adrenal paraganglioma also occurs in relatively rare places such as the urinary bladder, gallbladder, hepatoduodenal ligament, and gastrointestinal tract. Here, we report imaging findings of extra-adrenal paragangliomas presenting as mesenteric and pararectal masses with lymph node metastasis.

MeSH Terms

Abdomen
Gallbladder
Gastrointestinal Tract
Ligaments
Lymph Nodes
Magnetic Resonance Imaging
Mesenteric Artery, Inferior
Mesentery
Neoplasm Metastasis
Neural Crest
Para-Aortic Bodies
Paraganglioma*
Paraganglioma, Extra-Adrenal
Urinary Bladder

Figure

• 

  Fig. 1 Mesenteric and pararectal paragangliomas in a 70-year-old man. A. The portal venous phase coronal computed tomography (CT) scan shows a 15 cm-sized, heterogeneous, hypervascular soft tissue mass, with a lobulated contour (arrows) in the small bowel mesentery of the abdomen. B. The contrast enhanced portal venous phase axial CT image shows another well-defined, hypervascular mass, approximately 2.8 cm in size, adjacent to the right rectal wall (arrow). C. The T1-weighted axial magnetic resonance (MR) image without a contrast agent shows the heterogeneous signal intensity of the mesenteric mass (arrows), which is similar to that of the pararectal mass (not shown). D. The T2-weighted coronal MR image reveals a large, lobulated soft tissue mass (arrows) with heterogeneous high signal intensity, internal signal void structures, and engorged supplying vessels (blank arrows) in the small bowel mesentery. Heterogeneous high signal intensity with internal signal void was observed in the pararectal mass (not shown). E. The high signal intensity of the mesenteric mass (arrows) is shown on the diffusion-weighted MR image with a b-value of 800 s/mm2 (left). Some portions of the mesenteric mass show a low apparent diffusion coefficient value (arrows), indicating the presence of diffusion restriction (right). F. Microscopic features of the paraganglioma. Some tumor cells show a nested pattern, and others show a diffuse pattern. The tumor cells exhibit eosinophilic cytoplasm and ‘salt and pepper’ patterned chromatin with moderate nuclear pleomorphism (hematoxylin-eosin stain; original magnification, × 200), consistent with the findings of a paraganglioma.

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