Left subdiaphragmatic paraganglioma supplied by contralateral right renal artery

Abstract

Introduction: Paragangliomas are neuroendocrine tumors of the autonomic nervous system that arise from neural crest cells. Paragangliomas are typically extra-adrenal, non-functional and can be discovered incidentally, posing diagnostic and therapeutic challenges.

Presentation of case: We present the unusual case of a young man who presented with an acute abdomen secondary to small bowel obstruction, and was found to have an incidental left-sided subdiaphragmatic paraganglioma supplied by a branch of the contralateral right renal artery.

Discussion: Emergent laparotomy revealed an internal hernia as the source of the small bowel obstruction. Initial attempts to remove an incidental left subdiaphragmatic mass were aborted because of bleeding. Subsequently, after preoperative superselective embolization of the feeding vessel arising from the right renal artery, the mass was successfully resected. Pathology was consistent with a paraganglioma.

Conclusion: This report highlights the rarity of the location and blood supply of an incidental left subdiaphragmatic paraganglioma found in the setting of a small bowel obstruction.

Fig. 1

Thoraco-abdominal computed tomography. Panels A and B = Axial and lateral computed tomography scan image with intravenous contrast showing the tumor (T) adjacent to the liver (L) and abdominal wall. Panel C = Three dimensional reconstructed image showing tumor (T) in contiguity to the upper third segment of the left hepatic lobe. Panel D = Coronal computed tomography scan image showing the tumor (T) adjacent to left ventricle (LV).

Fig. 2

Abdominal arteriography. Aorta (Ao), right renal artery (RRA), feeding artery off the right renal artery bifurcation (Arrows), left renal artery (LRA), common hepatic artery (CHA).

Fig. 3

Intraoperative view. Tumor (T) is seen between diaphragma (DH) and liver (L). Abdominal wall (AW).

Fig. 4

Macroscopic pathology. Tumor described as irregular shape, soft, brown and measured at 5.42 cm × 4.2 cm × 3.2 cm.

Fig. 5

Microscopic pathology. Panel A = High powered field showing compact nest of polygonal cells, with highly vascular stroma. Cells with ovoid nuclei, granular chromatin, small eosinophilic nucleoli with mild reactive atypia, and a broad cytoplasm. Panel B = Low powered field showing extra-adrenal paraganglioma surrounded by capsule of dense fibroconnective tissue in continuity with striated muscle fibers.