In Germany, only about eight children develop adrenocortical carcinoma (adrenocortical carcinoma, ACC) every year. The tumors differ biologically and clinically significantly from those in adults, almost always produce hormones themselves and are often associated with hereditary cancer predisposition syndromes. Many of the tumors grow aggressively and spread early, so complete surgical removal is crucial for prognosis.

The 5-year survival rate in advanced cases is less than 40 percent. The classic first-line therapy consists of platinum-containing chemotherapy combined with the active ingredient mitotane, which acts as a selective adrenal cell toxin. This treatment leads to temporary adrenal insufficiency, so the children have to take additional hormones.

The disease requires highly specialized care in pediatric oncology and pediatric pathology. In contrast to adults, tumors in children are almost always hormonally active and have different molecular genetic characteristics. Genetic predispositions often play a role. Therapies that work in adults cannot therefore simply be transferred.

Research on pediatric adrenal carcinoma is still limited. International cooperation, for example in the European Network for the Study of Adrenal Tumors (ENSAT) with the ENSAT Kids sub-project, is intended to help collect more data and samples. The current focus is on preclinical research with cell models to better understand resistance mechanisms and test new therapeutic approaches.

Prof. Dr. Verena Wiegering, a specialist in pediatric and adolescent medicine with a focus on pediatric hematology and oncology at the University Medical Center Frankfurt, emphasizes the need for specialized pediatric pathology. Tumors in childhood are often related to genetic syndromes and differ in origin from those in adults. Precise diagnostics by experienced pediatric pathologists are therefore crucial.

In total, around 2,300 children and adolescents are newly diagnosed with cancer in Germany every year. Adrenocortical carcinoma accounts for only a very small proportion of these, but poses a particular challenge due to its aggressiveness and complexity.

Editor: X-Press Journalistenbüro GbR

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