A 37-year-old woman diagnosed with VHL presented with a ventricular peritoneal shunt.
He was operated on two occasions by renal carcinomas (right partial renal resection and a radiofrequency of a nodule in the left kidney), and in four occasions hemangioblastomas were excised.
During follow-up, two lesions in the head of the pancreas with a standardized maximum uptake value (SUV max.) 14.94 pancreatic cysts g/ml were detected by computerized positron emission tomography (PET-CT).
A pancreatic functional study was then performed, which showed elevated serum gastrin (2,319 pg/ml).
A transgastric needle aspiration biopsy (FNAB) of solid lesions showed a cytology compatible with neuroendocrine tumor of the pancreas.
With the diagnosis of 2 functioning gastrinomas smaller than 2 cm (T1N0M0 stage IA, with degree of differentiation G2, less than 2 mitosis per field and Ki-67 2-5% multidisciplinary committee) was indicated conservative surgery.
During the intervention, after a wide exposure of the pancreas, two solid lesions in the head and uncinate pancreatic process were located with ultrasound, which were enucleated with ultrasonic harmonic (CUSA) incidence.
The postoperative course was uneventful.
The pathological study showed two moderately differentiated neuroendocrine tumors (G2) (chromogranin, synaptophysin and CD 56 +), with a cellular proliferation index of 10% (Ki-67) mitotic fields.
Postoperative gastrin showed a significant decrease but did not reach normal range (1,383 pg/ml).
At 7 months of follow-up, a new elevation of this biomarker and a high determination of chromogranin A (181 ng/ml) were observed.
Although octreoscan did not detect abnormal accumulation of activity, magnetic resonance imaging (MRI) and PET-CT showed, in addition to tumor growth in the left kidney previously treated with radiofrequency ablation of the pancreas in a new lesion.
Associated findings, and in the context of a multicystic pancreas, a total pancreatoduodenectomy was performed in a multidisciplinary committee, without preservation of the upper pole of the left kidney.
A transmesocolic end-to-side gastrojejunostomy and a Roux-en-Y hepatic jejunostomy were performed for intestinal transit reconstruction.
Since no separation plane was identified between the portal vein and pancreas, a lateral partial resection of the portal vein was performed, which was repaired with continuous suture.
The extemporaneous biopsy ruled out tumoral necrosis of the vessel.
The histological report described a pancreatic neuroendocrine tumor of 9 mm in the process of moderate differentiation without lymph node absence / multiple cystic formations throughout the pancreas (serobic lymph node cystadenomas multicystic and multifocal adenomas 2.88 cm)
Postoperatively, the patient developed a urinary fistula requiring drainage by interventional radiology, resolved after 2 months of follow-up (Class III complication of the Clavien-D classification).
