Abstract
Introduction: Pancreatic neuroendocrine tumours (PNETs) occur as sporadic neoplasms or as a manifestation of multiple endocrine neoplasia type 1 (MEN-1) and other genetic syndromes. Systematic comparison of MEN 1-PNETs and sporadic PNETs may help to reveal new features.
Aim: Comparison of demographic, clinical and histological characteristics of MEN-1 and sporadic PNETs.
Patients and Methods: A total of 96 patients with PNETs were identified from the Oxford NET database. 37 patients (21 males and 16 females) had MEN-1, whilst the remaining 59 patients (34 males and 25 females) had non-MEN-1 (i.e. sporadic) PNETs. The median age at diagnosis of PNETs was 31.5 years (9.7-70) for the MEN-1 group and 57.6 years (28-82.2) for the sporadic group. In the MEN-1 group, 19/37 (51.4%) of patients had at least one functional PNET, of which 9 (47.3%) were insulinomas, 7 (36.8%) gastrinomas and 3 (15.8%) were glucagonomas. In the sporadic PNETs group, 25/59 (42.4%) of patients had a functioning tumour of which 17 (68%) were insulinomas, 1 (4%) hyperplasia of Langerhans cells, 2 (8%) gastrinomas, 3 (12%) glucagonomas and 2 (8%) were somatostatinomas.
Histological data were available for 24/37 (64.9%) of MEN-1 PNETs and for 51/59 (86.4%) of sporadic PNETs. Based on the ENETS grading system, 23 (95.8%) of MEN-1 PNETs were classified as G1 and 1 (4.2%) as G2, whilst in the sporadic PNETs, 26 (51%) were classified as G1, 18 (35.3%) as G2, 6 (11.7%) as G3 and 1 (1.9%) as G2-3.
Conclusion: MEN-1 associated PNETs appear to present at an earlier age and are usually G1 grade, whilst sporadic PNETs present later and are commonly G1-G2 grade. This may be because of increased surveillance of patients with cancer predisposition syndromes.
Aim: Comparison of demographic, clinical and histological characteristics of MEN-1 and sporadic PNETs.
Patients and Methods: A total of 96 patients with PNETs were identified from the Oxford NET database. 37 patients (21 males and 16 females) had MEN-1, whilst the remaining 59 patients (34 males and 25 females) had non-MEN-1 (i.e. sporadic) PNETs. The median age at diagnosis of PNETs was 31.5 years (9.7-70) for the MEN-1 group and 57.6 years (28-82.2) for the sporadic group. In the MEN-1 group, 19/37 (51.4%) of patients had at least one functional PNET, of which 9 (47.3%) were insulinomas, 7 (36.8%) gastrinomas and 3 (15.8%) were glucagonomas. In the sporadic PNETs group, 25/59 (42.4%) of patients had a functioning tumour of which 17 (68%) were insulinomas, 1 (4%) hyperplasia of Langerhans cells, 2 (8%) gastrinomas, 3 (12%) glucagonomas and 2 (8%) were somatostatinomas.
Histological data were available for 24/37 (64.9%) of MEN-1 PNETs and for 51/59 (86.4%) of sporadic PNETs. Based on the ENETS grading system, 23 (95.8%) of MEN-1 PNETs were classified as G1 and 1 (4.2%) as G2, whilst in the sporadic PNETs, 26 (51%) were classified as G1, 18 (35.3%) as G2, 6 (11.7%) as G3 and 1 (1.9%) as G2-3.
Conclusion: MEN-1 associated PNETs appear to present at an earlier age and are usually G1 grade, whilst sporadic PNETs present later and are commonly G1-G2 grade. This may be because of increased surveillance of patients with cancer predisposition syndromes.
| Original language | English |
|---|---|
| Publication status | Published - 7 Dec 2015 |
| Event | UKI NETS 13th National Conference - Royal College of Physicians, London, London, United Kingdom Duration: 7 Dec 2015 → 7 Jan 2016 |
Conference
| Conference | UKI NETS 13th National Conference |
|---|---|
| Country/Territory | United Kingdom |
| City | London |
| Period | 7/12/15 → 7/01/16 |
