Retrospective VIPoma

Level: Level 2

Launch date: 1 March 2023

Principle investigators: Detlef BARTSCH

Project description:

Background: VIPoma is an extremely rare functional neuroendocrine pancreatic neoplasia, (incidence <1/1000.000/year), which typically causes watery diarrhea by a secretory excess of vasoactive intestinal polypeptide (1). The cornerstone of treatment is complete tumor resection, whenever possible. Since VIPomas have a high potential to metastasize, however, curative resection is not possible in a significant proportion of patients. There is a lack of data regarding treatment and outcome of these tumors, since only small case series of up to 15 patients have been published (2, 3).

Aim: Retrospective data analysis of patients with histologically proven VIPoma regarding demographics, present syndrome, stage at diagnosis, surgical and non-surgical treatments as well as prognosis.  

Inclusion criteria: Patients with histologically proven VIPoma who were treated from 1.07.2012 until 31.09.2023 at NEN referral centers. Patients‘ informed consent to be documented in the ENETS database. Written consent of participating centers to participate in this retrospective study. The local responsible investigator has to be determined beforehand.

Methods: Anonymized patients with VIPoma will be captured in the extended data set (level II) of the ENETS-DB by participating centers until 31.09.2023. No additional items will be programmed, but the items of the extended data set should be completed as far as ever possible. The data will be extracted, analysed and statistical analyses performed by a statistician of the Marburg team until 30.06.2024. Thereafter it is planned to prepare a publication and submit it under the ENETS umbrella after been consented by all participants.

Publication rules: Every participating institution will have 1 (co)-authorship, further participating persons of the same institution will be listed in the acknowledgement. If the number of co-authors has to be limited to a journals policy, the order will be determined according to the patient numbers recruited.

• The first author is the chairperson or PI of the study group of the study (designer/methodologist of the study).
• The second author is the best recruiter in terms of numbers and quality or the co-main author (if not the PI of the study).
• Subsequent author hierarchy is dependent on quality/quantity of recruitment/methodologist.
• The last author is the second chairperson/PI of the study (if among best recruiter).

D. Bartsch of the Marburg group, who coordinates the study, will be shared senior author.

References:

• Falconi M, et al. ENETS Consensus Guidelines Update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology. 2016;103:153-171.
• Angelousi A, et al. Diagnostic and management challenges in vasoactive intestinal peptide secreting tumors: a series of 15 patients. Pancreas 2019; 48: 934-942.
• Peng SY, et al. Diagnosis and treatment of VIPoma in China (case report and 31 cases literature review). Pancreas 004; 28: 93-97.