Table 1 Prevalence of MEN1 tumours/lesions in our series with respect to published data*
From: Multiple endocrine neoplasia type 1: extensive analysis of a large database of Florentine patients
Prevalence in our patients n. (%) | Type and combination of tumours/lesions in our patients n. (%) | Prevalence in published literature (%) | |
|---|---|---|---|
PHPT | 139 (95.86%) | 139 PHPT: | 90% |
- 85 asymptomatic cases (61.15%) | |||
- 36 with nephrocalcinosis (25.90%) | |||
- 7 with osteoporosis (5.03%) | |||
- 7 with nephrocalcinosis and osteoporosis (5.03%) | |||
- 2 with osteopenia (1.44%) | |||
- 2 with nephrocalcinosis and osteopenia (1.44%) | |||
GEP-NETs | 86 (59.31%) | 30–70% | |
100 total tumours: | |||
- 41 gastrinomas (28.27% of MEN1 affected patients; 41% of GEP-NETs) | 40% | ||
- 16 insulinomas (11.03% of MEN1 affected patients; 16% of GEP-NETs) | 10% | ||
- 39 pNFTs (including 1 PPoma) (26.90% of MEN1 affected patients; 39% of GEP-NETs) | 20–55% | ||
- 2 VIPomas (1.38% of MEN1 affected patients; 2% of GEP-NETs) | < 1% | ||
- 1 glucagonoma (0.69% of MEN1 affected patients; 1% of GEP-NETs) | < 1% | ||
- 1 gastric NF-NET (0.69% of MEN1 affected patients; 1% of GEP-NETs) | 10% | ||
Combinations: | |||
- 28 gastrinoma alone (32.56% of patients affected by GEP-NETs) | |||
- 13 insulinoma alone (15.12% of patients affected by GEP-NETs) | |||
- 29 pNFTs alone (including 1 PPoma) (33.72% of patients affected by GEP-NETs) | |||
- 2 VIPoma alone (2.33% of patients affected by GEP-NETs) | |||
- 9 gastrinoma-pNFTs (10.47% of patients affected by GEP-NETs) | |||
- 3 gastrinoma-insulinoma (3.49% of patients affected by GEP-NETs) | |||
- 1 gastrinoma-glucagonoma (1.16% of patients affected by GEP-NETs) | |||
- 1 pNFT-gastric NF-NET (1.16% of patients affected by GEP-NETs) | |||
Pituitary tumours | 75 (51.72%) | 30–40% | |
76 total tumours: | |||
- 60 PRLomas (41.38% of MEN1 affected patients; 78.95% of pituitary tumours) | 20% | ||
- 12 NFA (8.28% of MEN1 affected patients; 15.79% of pituitary tumours) | < 5 | ||
- 3 ACTH-secreting tumours (corticotropinomas) (2.07% of MEN1 affected patients; 3.95% of pituitary tumours) | < 5% | ||
- 1 GH-secreting tumour (somatotropinoma) (0.69% of MEN1 affected patients; 1.32% of pituitary tumours) | 10% | ||
Combinations: | |||
- Only one combination of PRLoma-somatotropinoma (1.33% of patients affected by pituitary adenoma) | |||
Carcinoids | 17 (11.72%) | 17 bronchopulmonary (11.72% of MEN1 affected patients) | Bronchopulmonary NETs 2% Thymic NETs 2% |
Skin lesions | 44 (30.34%) | 53 total skin lesions: | 30% |
- 37 lipomas: 17 single lipomas and 20 multiple lipomatosis (25.52% of MEN1 affected patients; 69.81% of skin lesions) | 85% | ||
- 9 angiofibromas (6.21% of MEN1 affected patients; 16.98% of skin lesions) | n.a | ||
- 4 angiomas (2.76% of MEN1 affected patients; 7.55% of skin lesions) | n.a | ||
- 3 fibromas (2.07% of MEN1 affected patients; 5.66% of skin lesions) | |||
Adrenocortical tumours/lesions | 27 (18.62%) | 40% | |
27 total tumours/lesions of adrenal glands: | |||
- 9 hyperplasia (5 monolateral, 4 bilateral) (6.21% of MEN1 affected patients; 33.33% of adrenal gland tumours/lesions) | |||
- 18 adenomas (15 monolateral, 3 bilateral) (12.41% of MEN1 affected patients; 66.67% of adrenal gland tumours/lesions) | |||
Other lesions | 19 (13.10%) | n.a. | |
20 total other associated tumours: | |||
- 3 meningiomas (2.07% of MEN1 affected patients) | 8% | ||
- 4 breast cancers (4.21% of MEN1 affected women) | n.a | ||
- 12 uterine lesions (12.63% of MEN1 affected women) | n.a | ||
- 1 perineal aggressive angiomixoma (0.69% of MEN1 affected patients) | n.a. |