title: Late recurrence after surgical resection of a pancreatic tumor in von Hippel-Lindau disease.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Digestive and Liver Disease, Second School of Medicine, Sapienza University, Rome, Italy. vito.corleto@uniroma1.it
authors.forename: Vito Domenico
authors.initials: VD
authors.lastname: Corleto
authors.forename: Dario
authors.initials: D
authors.lastname: Cotesta
authors.forename: Luigi
authors.initials: L
authors.lastname: Petramala
authors.forename: Francesco
authors.initials: F
authors.lastname: Panzuto
authors.forename: Cristiano
authors.initials: C
authors.lastname: Pagnini
authors.forename: Luigi
authors.initials: L
authors.lastname: Masoni
authors.forename: Antonella
authors.initials: A
authors.lastname: Verrienti
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Delle Fave
authors.forename: Sebastiano
authors.initials: S
authors.lastname: Filetti
authors.forename: Claudio
authors.initials: C
authors.lastname: Letizia
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000473:pathology; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000150:complications / Q000473:pathology* / Q000601:surgery*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery*; D016577:Pancreaticoduodenectomy; D010673:Pheochromocytoma / Q000473:pathology; D012008:Recurrence; D013997:Time Factors; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000473:pathology