@entity1636 ( XXXX ): diagnosis, prognosis, and treatment.

@entity1 @entity632 @entity8449 @entity5 @entity900 @entity2879 @entity1636 @entity479
@entity1636 ( @entity1636 ) is a rare @entity5 with an incidence of 0.7-2.0 cases/million habitants/year. The diagnosis of @entity5 relies on careful investigations of clinical, biological, and imaging features before surgery and pathological examination after @entity5 removal. Most @entity1 present with @entity479 hormone excess or abdominal mass effects, but 15% of @entity1 with @entity1636 is initially diagnosed incidentally. After the diagnosis, in order to assess the @entity1636 prognosis and establish an adequate basis for treatment decisions different tools are proposed. The stage classification proposed by the European Network for the Study of @entity5 (ENSAT) is recommended. Pathology reports define the Weiss score, the resection status and the proliferative index, including the mitotic count and the Ki67 index. As far as the treatment is concerned, in case of @entity5 limited to the adrenal gland, the complete resection of the @entity5 is the first option. Most @entity1 benefit from adjuvant @entity8449 treatment. In metastatic disease, @entity8449 is the cornerstone of initial treatment, and cytotoxic drugs should be added in case of progression. Recently, the First International Randomized (FIRM-ACT) Trial in metastatic @entity1636 reported the association between @entity8449 and @entity2879 / @entity632 / @entity900 (EDP) as the new standard in first line treatment of @entity1636 . In last years, new targeted therapies, including the IGF-1 receptor inhibitors, have been investigated, but their efficacy remains limited. Thus, new treatment concepts are urgently needed. The ongoing "omic approaches" and next-generation sequencing will improve our understanding of the pathogenesis and hopefully will lead to better therapies.
