Korean J Gastroenterol.  2009 Dec;54(6):409-412. 10.4166/kjg.2009.54.6.409.

Paraganglioma of the Pancreas Metastasized to the Adrenal Gland: A Case Report

• Paik KY

• KMID: 1775943
• DOI: http://doi.org/10.4166/kjg.2009.54.6.409

Abstract

Paraganglioma is a rare neuroendocrine tumor arising from the neural crest, which includes tissues such as the adrenal medulla, carotid and aortic body, organs of Zuckerkandl, and other unnamed paraganglia. The head, neck, and retroperitoneum are the most common sites for paraganglioma. However, paraganglioma of the pancreas is extremely rare. We report our experience of this rare disease. A 70-year old female patient admitted for a pancreas tail mass detected by computed tomography (CT) scan checked for vague left flank pain. CT with contrast enhancement showed a 4.2-cm heterogeneously enhanced lesion in the tail of the pancreas. A well defined ovoid shape mass in left adrenal gland was suggested adenoma. Distal pancreatectomy with left adrenalectomy was performed. Any lymph node enlargement was not found. Pancreas mass did not invade adjacent organ. Microscopic examination with pancreas and adrenal gland revealed that the cells were arranged in a characteristic Zellballen pattern. Immunohistochemical staining revealed positivity for neuron-specific enolase, chromogranin A, synaptophysin, and S-100 protein. On the basis of these findings, we diagnosed the tumor as a paraganglioma of the pancreas and adrenal gland. We report the first case of pancreas paraganglioma in Korea.

Keyword

Paraganglioma; Pancreas; Adrenal gland

MeSH Terms

Adenoma/diagnosis/surgery
Adrenal Gland Neoplasms/*diagnosis/secondary/surgery
Aged
Chromogranin A/metabolism
Female
Humans
Pancreatic Neoplasms/*diagnosis/pathology/surgery
Paraganglioma/*diagnosis/secondary/surgery
Phosphopyruvate Hydratase/metabolism
S100 Proteins/metabolism
Synaptophysin/metabolism
Tomography, X-Ray Computed

Figure

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  Fig. 1. (A, B) Portal phase CT images showed a well-demarcated and heterogeneously enhancing mass (arrows) with a lobu-lating margin in the tail of the pancreas. (C) CT image cranial to A and B demonstrated a low attenuating small nodular lesion (arrow) in left adrenal gland.

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  Fig. 2. A photograph of gross specimen obtained after distal pancreatectomy with splenectomy depicted a well-defined mass in the tail of the pancreas.

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  Fig. 3. Microscopic findings. (A) A microscopic photograph (original magnification ×200, H&E staining) showed that the tumor cells were arranged in a characteristic Zellballen pattern with anastomosing sheets around a delicate vascular network. (B) Immunohistochemical staining revealed that cells were positive for neuron-specific enolase.

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