ECE2018 Poster Presentations: Adrenal and Neuroendocrine Tumours Adrenal cortex (to include Cushing's) (70 abstracts)
1Fundacion Jimenez Diaz University Hospital, Madrid, Spain; 2La Paz University Hospital, Madrid, Spain.
Introduction: Adrenocortical carcinoma (ACC) is a rare and aggressive tumour. At diagnosis 21% has metastasis. Even after complete surgical removal, patients are at risk of recurrence as late as 10-12 years. The 5-year survival rate of Stage I to IV (ENSAT 2008) is 82%, 61%, 50% and 13% respectively.
Objetive: To describe the epidemiological and clinical characteristics, as well as the evolution, treatments and overall survival of patients diagnosed with ACC.
Methods: We identified 41 cases of ACC in the archive of the Pathological Anatomy department in La Paz University Hospital-Madrid (19692017). We reviewed the medical records of 24 cases that were followed in the our center (19842017).
Results: Of the 41 cases, 9 were children (21.5%), 23 women (56.10%). The median age of the children was 4 years, (P25P50: 26), the mean age of the adults was 49.9 years (S.D.: 14.08). Total mortality in children was 50% and in adults 59%. The main characteristics of the 24 cases followed in our hospital is shown in the Table. An Stage IV case with complete remission and survival of more than 10 years was identified. ENSAT stage at diagnosis was a statistically significant variable for overall survival; it was not significant for sex, age, secretory status neither mitotane therapy in our cohort.
Children | Adults | |
N | 4(16%) | 20 (83%) |
Female | 4 (100%) | 12 (60%) |
Age (years) | 5.5 (1.89.0) | 53 (3662) |
Smoking/Previous Smoking | 0/0 | 9 (47%)/4(21%) |
Left Location | 3 (75%) | 8 (40%) |
Secretory Tumor | 4(100%) | 10 (50%) |
Cortisol | 0 | 3 |
Androgens | 4 | 3 |
Cortisol and Androgens | 0 | 3 |
Aldosterone | 0 | 1 |
ENSAT Stage at Diagnosis | ||
I | 1 (25%) | 6 (32%) |
II | 3 (75%) | 10 (53%) |
III | 0 | 1 (5%) |
IV | 0 | 2 (10%) |
ENSAT Stage at follow-up | ||
I | 1 (25%) | 5 (25%) |
II | 1 (25) | 4 (20%) |
III | 0 | 2 (10%) |
IV | 2 (50%) | 9 (45%) |
Surgery Approach | ||
Laparoscopic | 0 | 2 (10%) |
Laparotomy | 3 (75%) | 16 (80%) |
No surgery | 1 (25%) | 2 (10%) |
Chemotherapy | 1 (25%) | 8(42%) |
Radiotherapy | 4% | 0 |
Mitotane treatment | 2 (50%) | 10 (50%) |
Recurrent Disease | 1(25%) | 7 (42%) |
Survival | ||
1-year | 2 (50%) | 17 (85%) |
5-year | 2 (50%) | 9 (45%) |
10-year | 2 (50%) | 7 (35%) |
Conclusions: Due to its infrequency, we believe that the epidemiological registry and the results of the different therapeutic approaches are convenient for a better treatment of this pathology.