Characterization of the Strasbourg Cohort of Patients With Adrenal Cortex Carcinoma

Condition(s) studied

Study summary

Adrenal corticosteroid carcinoma (ACC) is a rare tumor of the adrenal cortex, with an estimated prevalence of 0.5 to 2 cases per million inhabitants per year.

Two peaks in incidence have been described: during the first decade of life and between 40 and 50 years of age, with a slight female predominance (female-to-male ratio of 1.5). The majority of cases (\>90%) are sporadic, particularly in adults.

ACC may be discovered incidentally during an imaging examination performed for another reason (10 to 20% of cases) or in connection with a tumor syndrome (40-60% of cases) or hormonal hypersecretion (40 to 74% of cases). The diagnosis of CCS can be suggested by the combination of morphological characteristics seen on imaging and clinical and biological features (secretory syndrome), but only histopathology allows for a definitive diagnosis. Furthermore, histopathology enables the assessment of aggressiveness criteria (Weiss score, Ki67), which will influence further management and prognosis.

Given that CCS is a rare tumor, the investigators aim to study the clinical, biological, morphological, and histological characteristics and evaluate the prognosis of patients treated at our center in order to better understand the natural history of CCS and improve patient management.

Eligibility

Primary outcome measure(s)

• Overall, specific, and progression-free survival in patients treated for adrenal cortical carcinoma (ACC) — Up to 12 months
  Overall survival: Is how long patients live after being diagnosed or treated, regardless of the cause of death. Example: If 100 patients are treated for ACC and 60 are alive after 5 years, the 5-year overall survival is 60%. It counts everyone, whether they die from ACC or something else, like an accident or another illness.

Trial sites (1)

More University Hospital, Strasbourg, France trials in France