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Von Hippel-Lindau Disease
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Amit Tirosh
Oct 04, 2018
Hi Xavier,
This is indeed an intriguing situation.
The patient has three lesions in the “intermediate risk zone” in terms of lesion diameter (>1.2-1.5 cm and <3 cm), based on the NIH data (JAMA Onc 2018). Also, neither of the lesions reached the 2.8 cm cutoff defined by Krauss et al (ERC 2018) in the analysis of the European-American-Asian-VHL-PanNET-Registry data. So there is no answer here.
The presence of missense mutation does suggest a possible risk of metastatic disease, but with relatively low positive predictive value (~10%), so it should not move us to action either.
The most important point here, in my view, is the distance between the pancreatic head lesion and the pancreatic duct. It seems (in the third figure) to be just next to it. I would perform an MRCP now, and if it is still possible to enucleate the tumors, especially the pancreatic head PNET named “lesion 1”, I would do it now, as there is a chance that we won’t be able to do so in 6-12 months, and then we are confronting Whipple procedure.
If there is no other possibility to resect these lesions other than Whipple, I would follow the patient in 6-12 months.
Let me know what you think.
Amit
Phil Hart
Nov 19, 2018
Hi Xavier, we would offer a pancreatic resection (ie, Whipple) for this patient at our center. As Dr. Tirosh points, all lesions are <3 cm in size so the current risk for metastasis is low. Rather the resection is considered somewhat 'prophylactic' for prevention of LN mets. Considering the appearance of the lesions and multifocality, resection would be favored over enucleation.
At the same time, as Dr. Tirosh pointed out, the immediate risk for LN involvement or progression is relatively low. Considering your patient's recent diagnosis, it would be reasonable to provide active surveillance for a short period of time (?6-12 months) to allow them to psychologically adapt to the diagnosis and improve their fitness prior to surgery. Best regards, Phil
Comment deleted by Moderator.
Apr 18, 2024
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Xavier
M
Keutgen
MD
Amit Tirosh
Oct 04, 2018
Phil Hart
Nov 19, 2018
Apr 18, 2024
Pending Moderator approval.