OBJECTIVES:

The primary objective of this study is to evaluate the effect of estrogen on the development of the PNET in MEN1 patients.

The secondary objective is to evaluate the overall survival and disease specific survival in patients who have confirmed MEN1 with or without PNET and a pancreatic neuroendocrine tumor in relation to their hormone status. The secondary objective is to evaluate clinicopathologic features in relation to hormone status.

Retrospective review of a prospectively maintained MEN1 database. The patient cohort consists of all patients with confirmed MEN1 with or without PNET who are included in the database. The database will be reviewed to obtain patient and disease characteristics, laboratory values, imaging results, hormone parameters (menopausal status, bioidentical hormone use, oral contraception use, gestational status and diagnosis of a prolactinoma) and vital status.

For the prospective portion of this study, investigators want to see the impact of estrogen on the PNET forming and progression, and would like to use two equations to assess the estrogen exposure, so investigators should collect the information of menopause, menarche, breast feed, HRT, OCP use and pregnancy. To this end, investigators will contact female patients in the database who are still living, and obtain verbal informed consent to send an email questionnaire regarding their pregnancy and hormone use history.

Inclusion Criteria:

1. The patient cohort for this study consists of all patients within the prospectively maintained MEN1 database within the Department of Surgical Oncology at the University of Texas MD Anderson Cancer Center.
2. Patients with a diagnosis of MEN1 with or without PNET based on mutational analysis or defined clinical criteria were considered.
3. All patients for whom hormone status variables and survival data are available will be included.

Exclusion Criteria:

1. Due to the nature of the questions included in the prospective estrogen questionnaire, only female patients will be verbally consented to receive the questionnaire and obtain prospective data.

Prospectively maintained MEN1 database within the Department of Surgical Oncology, Division of Surgical Endocrinology at the University of Texas MD Anderson Cancer Center.