| . | . | . | . | . | MEN1 Manifestations . | . | . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Family number . | Number of MEN1 patients (N) . | Number of generations (N) . | Gender (N [%] female) . | Age (median, range) . | Primary hyper-parathyroidism (N, %) . | Pancreatic- and duodenal NET (N, %) . | Anterior pituitary tumor (N, %) . | Adrenal tumor (N, %) . | Bp-NET (N, %) . | Th-NET (N, %) . | Gastric NET (N, %) . | Death (N) . | MEN1-related death (N) . |
| 1 | 29 | 3 | 11 (38) | 45 (19–84) | 27 (93) | 20 (69) | 15 (52) | 12 (41) | 11 (38) | 0 | 0 | 3 | 1a |
| 2 | 23 | 4 | 15 (65) | 50 (21–80) | 23 (100) | 17 (74) | 11 (48) | 10 (43) | 5 (22) | 1 (4) | 0 | 7 | 3b |
| 3 | 18 | 3 | 8 (44) | 51 (22–72) | 15 (83) | 9 (50) | 6 (33) | 6 (33) | 6 (33) | 1 (6) | 0 | 4 | 1c |
| 4 | 14 | 3 | 8 (57) | 52 (19–70) | 12 (86) | 12 (86) | 8 (57) | 6 (43) | 3 (21) | 0 | 1 (7) | 1 | 1d |
| 5 | 12 | 3 | 8 (67) | 51 (22–75) | 10 (83) | 8 (67) | 2 (17) | 2 (17) | 5 (42) | 0 | 0 | 2 | 1e |
| 6* | 12 | 3 | 7 (58) | 52 (28–84) | 3 (25) | 3 (25) | 0 | 1 (8) | 2 (17) | 0 | 0 | 0 | – |
| 7 | 11 | 2 | 9 (82) | 38 (20–63) | 10 (91) | 8 (73) | 4 (36) | 3 (27) | 2 (18) | 0 | 2 (18) | 0 | – |
| 8 | 11 | 3 | 5 (45) | 48 (22–77) | 9 (82) | 6 (55) | 3 (27) | 3 (27) | 4 (36) | 0 | 1 (9) | 2 | 1f |
| 9 | 11 | 4 | 5 (45) | 49 (23–76) | 11 (100) | 8 (73) | 4 (36) | 3 (27) | 3 (27) | 0 | 0 | 3 | 2g |
| 10i | 11 | 2 | 4 (36) | 48 (36–80) | 1 (9) | 1 (9) | 2 (18) | 2 (18) | 2 (18) | 0 | 0 | 1 | 0h |
| TOTAL | 152 | 80 (53%) | 49 (19 – 84) | 121 (80%) | 92 (61%) | 55 (36%) | 48 (32%) | 43 (28%) | 2 (1%) | 4 (3%) | 23 (15%) | 10 (7%) | |
| . | . | . | . | . | MEN1 Manifestations . | . | . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Family number . | Number of MEN1 patients (N) . | Number of generations (N) . | Gender (N [%] female) . | Age (median, range) . | Primary hyper-parathyroidism (N, %) . | Pancreatic- and duodenal NET (N, %) . | Anterior pituitary tumor (N, %) . | Adrenal tumor (N, %) . | Bp-NET (N, %) . | Th-NET (N, %) . | Gastric NET (N, %) . | Death (N) . | MEN1-related death (N) . |
| 1 | 29 | 3 | 11 (38) | 45 (19–84) | 27 (93) | 20 (69) | 15 (52) | 12 (41) | 11 (38) | 0 | 0 | 3 | 1a |
| 2 | 23 | 4 | 15 (65) | 50 (21–80) | 23 (100) | 17 (74) | 11 (48) | 10 (43) | 5 (22) | 1 (4) | 0 | 7 | 3b |
| 3 | 18 | 3 | 8 (44) | 51 (22–72) | 15 (83) | 9 (50) | 6 (33) | 6 (33) | 6 (33) | 1 (6) | 0 | 4 | 1c |
| 4 | 14 | 3 | 8 (57) | 52 (19–70) | 12 (86) | 12 (86) | 8 (57) | 6 (43) | 3 (21) | 0 | 1 (7) | 1 | 1d |
| 5 | 12 | 3 | 8 (67) | 51 (22–75) | 10 (83) | 8 (67) | 2 (17) | 2 (17) | 5 (42) | 0 | 0 | 2 | 1e |
| 6* | 12 | 3 | 7 (58) | 52 (28–84) | 3 (25) | 3 (25) | 0 | 1 (8) | 2 (17) | 0 | 0 | 0 | – |
| 7 | 11 | 2 | 9 (82) | 38 (20–63) | 10 (91) | 8 (73) | 4 (36) | 3 (27) | 2 (18) | 0 | 2 (18) | 0 | – |
| 8 | 11 | 3 | 5 (45) | 48 (22–77) | 9 (82) | 6 (55) | 3 (27) | 3 (27) | 4 (36) | 0 | 1 (9) | 2 | 1f |
| 9 | 11 | 4 | 5 (45) | 49 (23–76) | 11 (100) | 8 (73) | 4 (36) | 3 (27) | 3 (27) | 0 | 0 | 3 | 2g |
| 10i | 11 | 2 | 4 (36) | 48 (36–80) | 1 (9) | 1 (9) | 2 (18) | 2 (18) | 2 (18) | 0 | 0 | 1 | 0h |
| TOTAL | 152 | 80 (53%) | 49 (19 – 84) | 121 (80%) | 92 (61%) | 55 (36%) | 48 (32%) | 43 (28%) | 2 (1%) | 4 (3%) | 23 (15%) | 10 (7%) | |
Abbreviation: NET, neuroendocrine tumor.
aMEN1-related: progressive pancreatic NET. Non-MEN1-related: irresectable myxofibrosarcoma (n = 1), end-stage heart failure (n = 1).
bMEN1-related: progressive dpNET (n = 1), progressive th-NET (n = 1), complications of MEN1-related operation (n = 1). Non-MEN1-related: pneumonia (n = 1), heart failure (n = 1), pulmonary embolism (n = 1), unknown (n = 1).
cMEN1-related: progressive th-NET (n = 1). Non-MEN1-related: progressive prostate cancer (n = 1), progressive colorectal cancer (adenocarcinoma) (n = 1), subarachnoid hemorrhage (n = 1).
dMEN1-related: progressive pancreatic NET.
eMEN1-related: progressive pancreatic NET (n = 1). Non-MEN1-related: progressive colorectal cancer (n = 1).
fMEN1-related: progressive nesidioblastoma (n = 1). Non-MEN1-related: dementia (n = 1).
gMEN1-related: progressive dp-NET (n = 2). Non-MEN1-related: unknown (n = 1).
hNon-MEN1-related: progressive colorectal cancer (n = 1).
iFamilies with low penetrance of disease
| . | . | . | . | . | MEN1 Manifestations . | . | . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Family number . | Number of MEN1 patients (N) . | Number of generations (N) . | Gender (N [%] female) . | Age (median, range) . | Primary hyper-parathyroidism (N, %) . | Pancreatic- and duodenal NET (N, %) . | Anterior pituitary tumor (N, %) . | Adrenal tumor (N, %) . | Bp-NET (N, %) . | Th-NET (N, %) . | Gastric NET (N, %) . | Death (N) . | MEN1-related death (N) . |
| 1 | 29 | 3 | 11 (38) | 45 (19–84) | 27 (93) | 20 (69) | 15 (52) | 12 (41) | 11 (38) | 0 | 0 | 3 | 1a |
| 2 | 23 | 4 | 15 (65) | 50 (21–80) | 23 (100) | 17 (74) | 11 (48) | 10 (43) | 5 (22) | 1 (4) | 0 | 7 | 3b |
| 3 | 18 | 3 | 8 (44) | 51 (22–72) | 15 (83) | 9 (50) | 6 (33) | 6 (33) | 6 (33) | 1 (6) | 0 | 4 | 1c |
| 4 | 14 | 3 | 8 (57) | 52 (19–70) | 12 (86) | 12 (86) | 8 (57) | 6 (43) | 3 (21) | 0 | 1 (7) | 1 | 1d |
| 5 | 12 | 3 | 8 (67) | 51 (22–75) | 10 (83) | 8 (67) | 2 (17) | 2 (17) | 5 (42) | 0 | 0 | 2 | 1e |
| 6* | 12 | 3 | 7 (58) | 52 (28–84) | 3 (25) | 3 (25) | 0 | 1 (8) | 2 (17) | 0 | 0 | 0 | – |
| 7 | 11 | 2 | 9 (82) | 38 (20–63) | 10 (91) | 8 (73) | 4 (36) | 3 (27) | 2 (18) | 0 | 2 (18) | 0 | – |
| 8 | 11 | 3 | 5 (45) | 48 (22–77) | 9 (82) | 6 (55) | 3 (27) | 3 (27) | 4 (36) | 0 | 1 (9) | 2 | 1f |
| 9 | 11 | 4 | 5 (45) | 49 (23–76) | 11 (100) | 8 (73) | 4 (36) | 3 (27) | 3 (27) | 0 | 0 | 3 | 2g |
| 10i | 11 | 2 | 4 (36) | 48 (36–80) | 1 (9) | 1 (9) | 2 (18) | 2 (18) | 2 (18) | 0 | 0 | 1 | 0h |
| TOTAL | 152 | 80 (53%) | 49 (19 – 84) | 121 (80%) | 92 (61%) | 55 (36%) | 48 (32%) | 43 (28%) | 2 (1%) | 4 (3%) | 23 (15%) | 10 (7%) | |
| . | . | . | . | . | MEN1 Manifestations . | . | . | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Family number . | Number of MEN1 patients (N) . | Number of generations (N) . | Gender (N [%] female) . | Age (median, range) . | Primary hyper-parathyroidism (N, %) . | Pancreatic- and duodenal NET (N, %) . | Anterior pituitary tumor (N, %) . | Adrenal tumor (N, %) . | Bp-NET (N, %) . | Th-NET (N, %) . | Gastric NET (N, %) . | Death (N) . | MEN1-related death (N) . |
| 1 | 29 | 3 | 11 (38) | 45 (19–84) | 27 (93) | 20 (69) | 15 (52) | 12 (41) | 11 (38) | 0 | 0 | 3 | 1a |
| 2 | 23 | 4 | 15 (65) | 50 (21–80) | 23 (100) | 17 (74) | 11 (48) | 10 (43) | 5 (22) | 1 (4) | 0 | 7 | 3b |
| 3 | 18 | 3 | 8 (44) | 51 (22–72) | 15 (83) | 9 (50) | 6 (33) | 6 (33) | 6 (33) | 1 (6) | 0 | 4 | 1c |
| 4 | 14 | 3 | 8 (57) | 52 (19–70) | 12 (86) | 12 (86) | 8 (57) | 6 (43) | 3 (21) | 0 | 1 (7) | 1 | 1d |
| 5 | 12 | 3 | 8 (67) | 51 (22–75) | 10 (83) | 8 (67) | 2 (17) | 2 (17) | 5 (42) | 0 | 0 | 2 | 1e |
| 6* | 12 | 3 | 7 (58) | 52 (28–84) | 3 (25) | 3 (25) | 0 | 1 (8) | 2 (17) | 0 | 0 | 0 | – |
| 7 | 11 | 2 | 9 (82) | 38 (20–63) | 10 (91) | 8 (73) | 4 (36) | 3 (27) | 2 (18) | 0 | 2 (18) | 0 | – |
| 8 | 11 | 3 | 5 (45) | 48 (22–77) | 9 (82) | 6 (55) | 3 (27) | 3 (27) | 4 (36) | 0 | 1 (9) | 2 | 1f |
| 9 | 11 | 4 | 5 (45) | 49 (23–76) | 11 (100) | 8 (73) | 4 (36) | 3 (27) | 3 (27) | 0 | 0 | 3 | 2g |
| 10i | 11 | 2 | 4 (36) | 48 (36–80) | 1 (9) | 1 (9) | 2 (18) | 2 (18) | 2 (18) | 0 | 0 | 1 | 0h |
| TOTAL | 152 | 80 (53%) | 49 (19 – 84) | 121 (80%) | 92 (61%) | 55 (36%) | 48 (32%) | 43 (28%) | 2 (1%) | 4 (3%) | 23 (15%) | 10 (7%) | |
Abbreviation: NET, neuroendocrine tumor.
aMEN1-related: progressive pancreatic NET. Non-MEN1-related: irresectable myxofibrosarcoma (n = 1), end-stage heart failure (n = 1).
bMEN1-related: progressive dpNET (n = 1), progressive th-NET (n = 1), complications of MEN1-related operation (n = 1). Non-MEN1-related: pneumonia (n = 1), heart failure (n = 1), pulmonary embolism (n = 1), unknown (n = 1).
cMEN1-related: progressive th-NET (n = 1). Non-MEN1-related: progressive prostate cancer (n = 1), progressive colorectal cancer (adenocarcinoma) (n = 1), subarachnoid hemorrhage (n = 1).
dMEN1-related: progressive pancreatic NET.
eMEN1-related: progressive pancreatic NET (n = 1). Non-MEN1-related: progressive colorectal cancer (n = 1).
fMEN1-related: progressive nesidioblastoma (n = 1). Non-MEN1-related: dementia (n = 1).
gMEN1-related: progressive dp-NET (n = 2). Non-MEN1-related: unknown (n = 1).
hNon-MEN1-related: progressive colorectal cancer (n = 1).
iFamilies with low penetrance of disease
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