Nonfunctional paraganglioma of the head of the pancreas: A rare case report.
BACKGROUND
Paragangliomas are rare neoplasms that originate from the neural crest. They are malignant in approximately 10% of cases, with a 50% survival rate at 5 years from diagnosis. In most cases, manifestations of malignancy (such as metastasis) are lacking, and paragangliomas are considered benign lesions. Pancreatic paragangliomas are extremely rare, with only 31 cases described in the scientific literature to date.


METHODS
Here we describe a case of a 55-year-old Caucasian male patient referred to our institution in September 2013 for lumbar pain lasting five months. The ultrasound and the CT scan revealed a 2.5cm solid nodule located in the uncinate process of the pancreas. On the basis of this evidence, the preoperative diagnosis was a pancreatic neuroendocrine tumor (NET), which was further confirmed by a subsequent In-Pentetreotide Scan examination. A pylorus-preserving duodenocephalopancreasectomy was performed. Pancreatic paraganglioma was the final pathological diagnosis. Rare localizations of paraganglioma are often discovered casually, during imaging examinations for other clinical reasons, as happened in the case of our patient. It appears evident that the preoperative diagnosis of pancreatic paragangliomas is extremely challenging. Surgery represents the cornerstone of the clinical management of these neoplasms, primarily for the need of a definitive diagnosis, which is difficult to assess preoperatively in most cases.


CONCLUSIONS
Our strategy is the same as that adopted for the management of pancreatic NETs; the dimensional limit for a conservative resection is 2cm, while major resections (Whipple's approach or distal pancreatectomy) should be employed in larger tumors, which are generally associated with a worse prognosis.
Unit of General Surgery 2, Department of Clinical and Experimental Medicine, University of Sassari, Viale San Pietro 43, 07100 Sassari, Italy. Electronic address: ginesugc@uniss.it.
G C
GC
Ginesu
Unit of General Surgery 2, Department of Clinical and Experimental Medicine, University of Sassari, Viale San Pietro 43, 07100 Sassari, Italy. Electronic address: michelebarmina@gmail.com.
M
M
Barmina
Unit of Surgical Pathology, Department of Surgical, Microsurgical and Medical Sciences, University of Sassari, V.le San Pietro 43, 07100, Sassari, Italy. Electronic address: panospaliogiannis@gmail.com.
P
P
Paliogiannis
Unit of Pathology, Department of Surgical, Microsurgical and Medical Sciences, University of Sassari, V.le San Pietro 43, 07100, Sassari, Italy. Electronic address: matilde.trombetta@gmail.com.
M
M
Trombetta
Unit of General Surgery 2, Department of Clinical and Experimental Medicine, University of Sassari, Viale San Pietro 43, 07100 Sassari, Italy. Electronic address: mlcossu@uniss.it.
M L
ML
Cossu
Unit of General Surgery 2, Department of Clinical and Experimental Medicine, University of Sassari, Viale San Pietro 43, 07100 Sassari, Italy. Electronic address: cffeo@uniss.it.
C F
CF
Feo
Unit of General Surgery 2, Department of Clinical and Experimental Medicine, University of Sassari, Viale San Pietro 43, 07100 Sassari, Italy. Electronic address: fra.addis49@gmail.com.
F
F
Addis
Unit of General Surgery 2, Department of Clinical and Experimental Medicine, University of Sassari, Viale San Pietro 43, 07100 Sassari, Italy. Electronic address: alb.porcu@gmail.com.
A
A
Porcu
Cancer; Case report; NET; Pancreas; Pancreatectomy; Paraganglioma
J Clin Endocrinol Metab. 2001 Nov;86(11):5210-6
11701678
J Diet Suppl. 2013 Dec;10(4):381-90
24237192
Endocr Pathol. 2005 Spring;16(1):23-32
16000843
J Gastrointest Surg. 2016 Oct;20(10):1781-3
27184673
Hum Pathol. 1989 Jan;20(1):29-39
2912871