No multiple endocrine neoplasia type 1 (MEN1) Patient died from pancreaticoduodenal neuroendocrine tumor – Results from long-term follow-up of the Uppsala model of early diagnosis and treatment

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Introduction: Progression of pancreaticoduodenal neuroendocrine tumor (P-NET) disease is the main death cause among MEN1 carriers. Although surgery is the only cure, indications vary substantially between centers. The Uppsala model of MEN1 management encompasses early diagnosis and treatment, aiming at lifelong malignancy prevention with preserved endocrine function. During three decades, all MEN1 patients harbouring P-NETs of at least 1 cm have been considered for surgery.

Aim(s): To evaluate malignancy-free and overall survival after P-NET surgery in MEN1 patients treated according to our model.

Materials and methods: Of all 160 MEN1 patients treated at Uppsala University Hospital 119 (74%) were found to have P-NETs. The mean follow-up after P-NET diagnosis was 13 ± 9 years. Patients with hormonal syndromes or tumors of ≥1cm in diameter were considered for pancreatic surgery. Fifty-nine patients were diagnosed early and treated according to the Uppsala model (group A), whereas 60 patients had already been diagnosed with P-NETs exceeding 1 cm upon referral to our clinic (group B).

Conference:

Presenting Author:

Authors: Bajic D, Kjaer J, Welin S, Norlén O, Öberg K,

Keywords: multiple endocrine neoplasia type 1, pancreatic neuroendocrine tumor, indication for surgery, malignancy prevention,