Table 4 Main characteristics and treatments of GEP-NETs in our series of MEN1 patients
From: Multiple endocrine neoplasia type 1: extensive analysis of a large database of Florentine patients
GEP-NETs | Patients n. (%) | Clinical presentation | Surgery | Pharmacological treatment | |||||
|---|---|---|---|---|---|---|---|---|---|
FTs n. (%) | NFTs (including PPoma) n. (%) | Combination of FT and NFT n. (%) | No surgery n. (%) | WP n. (%) | Enucleation or PPR n. (%) | (SSAs) n. (%) | Radionuclide therapy with (177)Lu-DOTATATE n. (%) | ||
Total patients with GEP-NETs | 86 (59.31%) | 47 (32.41%) - 28 gastrinomas alone (19.31%) - 13 insulinomas alone (8.97%) - 2 VIPomas alone (1.38%) - 3 gastrinoma-insulinoma (2.07%) - 1 gastrinoma-glucagonoma (0.69%) | 30 (20.69%) - 28 pNFTs (19.31%) - 1 PPoma (0.69%) - 1 pNFT-gastric NF NET (0.69%) | 9 (6.21%) - 9 gastrinoma-PNFTs (6.21%) | 41 (47.67%) | 17 (19.77%) | 28 (32.56%) | 18 (20.93%) | 2 (2.36%) |
A GEP-NET as first clinical manifestation | 20 (13.79%) | 18 (12.41%) - 9 gastrinomas (6.21%) - 8 insulinomas (5.52%) - 1 VIPoma (0.69%) | 2 (1.38%) - 1 PPoma (0.69%) - 1 gastric NF NET (0.69%) | 0 | 5 (5.81%) | 6 (6.98%) | 9 (10.47%) | 4 (4.65%) | 1 (1.16%) |
Index cases (probands) | 65 (44.83%) | 39 (26.90%) - 22 gastrinomas alone (15.17%) - 11 insulinomas alone (7.59%) - 2 VIPomas alone (1.38%) - 3 gastrinoma-insulinoma (2.07%) - 1 gastrinoma-glucagonoma (0.69%) | 17 (11.72%) - 16 pNFTs (11.03%) - 1 pNFT-gastric NF NET (0.69%) | 9 (6.21%) - 9 gastrinoma-PNFTs (6.21%) | 25 (29.07%) | 17 (19.77%) | 23 (26.74%) | 12 (13.95%) | 2 (2.36%) |
Relatives | 21 (14.48%) | 8 (5.52%) - 6 gastrinomas alone (4.14%) - 2 insulinomas alone (1.38%) | 13 (8.97%) - 12 pNFTs (8.28%) - 1 PPoma (0.69%) | 0 | 16 (18.60%) | 0 | 5 (5.81%) | 6 (6.98%) | 0 |