CASE REPORT

JOP. J Pancreas (Online) 2009 Sep 4; 10(5):562-565.

Late Recurrence after Surgical Resection of a Pancreatic Tumor in von Hippel-Lindau Disease

Vito Domenico Corleto1,4, Dario Cotesta3, Luigi Petramala3, Francesco Panzuto1, Cristiano Pagnini1,4, Luigi Masoni2, Antonella Verrienti3, Gianfranco Delle Fave1, Sebastiano Filetti3, Claudio Letizia3

Departments of 1Digestive and Liver Disease and 2Surgery, Second School of Medicine, “Sapienza” University; 3Department of Clinical Sciences, First School of Medicine, “Sapienza” University; 4Research Center “S. Pietro”, “S. Pietro” Hospital. Rome, Italy

ABSTRACT

Context Patients with von Hippel-Lindau syndrome, a dominantly inherited familial cancer syndrome, develop a variety of tumors in different organ systems which make the clinical management of these patients complex.

Case report The long clinical history of a 45-year-old woman started at 22 years of age when she had surgery for a right adrenal pheochromocytoma. Two years later, a pancreaticoduodenectomy was performed to remove a pancreatic mass which turned out to be a pancreatic neuroendocrine tumor. After a long period of relative wellness, 21 years after the surgical resection of her primary pancreatic neuroendocrine tumor, abdominal lymph node metastases of pancreatic neuroendocrine origin occurred. In fact, three abdominal nodules were removed by laparoscopic surgery, and the histological examination showed well-differentiated neuroendocrine tumors with similar immunohistochemical characteristics and Ki67 below 1%. Considering the patient’s clinical history, an inherited cause was postulated and multiple endocrine neoplasia type 1 was first investigated, but the result was negative. Then, a missense mutation in exon 3 of the VHL gene (ACT>ATT; Thr157Ile) was found.

Conclusion Although no local and/or distant tumor recurrences are usually reported in radically operated on von Hippel-Lindau pancreatic neuroendocrine tumor patients after a median time of five years of follow-up, the present patient had a recurrence after a very long period of time, suggesting that a pancreatic neuroendocrine tumor associated with von Hippel-Lindau syndrome may behave more aggressively than that has previously been described, thus requiring a life-long follow-up.

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Keywords Neuroendocrine Tumors; Pheochromocytoma; von Hippel-Lindau Disease

Correspondence Vito D Corleto: vito.corleto@uniroma1.it

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