Late recurrence after surgical resection of a pancreatic tumor in von Hippel-Lindau disease.
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.
Corleto
Vito Domenico
VD
Department of Digestive and Liver Disease, Second School of Medicine, Sapienza University, Rome, Italy. vito.corleto@uniroma1.it
Cotesta
Dario
D
Petramala
Luigi
L
Panzuto
Francesco
F
Pagnini
Cristiano
C
Masoni
Luigi
L
Verrienti
Antonella
A
Delle Fave
Gianfranco
G
Filetti
Sebastiano
S
Letizia
Claudio
C
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