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Adrenal cortical carcinoma: Paediatric aspects - literature review
Patrycja Dasiewicz1, Elżbieta Moszczyńska1, Wiesława Grajkowska2
1Department of Endocrinology and Diabetology, The Children's Memorial Health Institute, Warsaw, Poland.
Insights
Adrenocortical carcinoma (ACC) in children often presents with virilization before age 5. Genetic testing for TP53 mutations is crucial, and treatment requires individualized, expert multidisciplinary care.
Area of Science:
- Pediatric Oncology
- Endocrinology
- Genetics
Background:
- Adrenocortical carcinoma (ACC) is a rare pediatric malignancy requiring specialized, multidisciplinary management.
- Early diagnosis and tailored treatment are critical for improving outcomes in pediatric ACC.
Purpose of the Study:
- To review current knowledge on pediatric adrenocortical carcinoma.
- Focus on presenting symptoms, genetic factors, and diagnostic approaches.
Main Methods:
- Comprehensive literature search of PubMed.
- Inclusion of randomized clinical trials, reviews, systematic reviews, meta-analyses, and case reports.
Main Results:
- ACC predominantly affects children under 5 years old.
- Rapid virilization is the most common symptom (60-80%).
- TP53 gene mutations are frequently identified genetic predisposing factors.
Conclusions:
- Diagnosis and management should occur at experienced, high-volume centers.
- Individualized treatment strategies are essential.
- Genetic testing for TP53 mutations is recommended for pediatric ACC patients.
Introduction:
Adrenocortical carcinoma (ACC) is a rare malignancy in children. Because of this, each patient with suspected ACC requires individualised management, which should be determined at a meeting of a team of multidisciplinary experts in the field.
Aim Of The Study:
To summarise data on symptoms, genetic predisposition, and diagnostic procedures for ACC in children.
Material And Methods:
Papers were searched in the PubMed database to identify published randomised clinical trials, reviews, systematic reviews, meta-analyses, and case reports.
Results:
Most cases of ACC in children occur under the age of 5 years. The most common presenting symptom in 60-80% of paediatric patients is rapidly progressive virilisation. Diagnostics are based on laboratory and imaging evaluation. The mainstay of treatment is surgery, with laparotomy being the preferred method of surgery. Diagnosis is based on histological examination of surgically removed tissue. The Wieneke index is most commonly used in paediatric practice. However, some cases are still classified as "indeterminate histology". Predisposing genetic factors are found in most children with ACC, most commonly a mutation of the TP53 gene.
Conclusions:
Patients should be diagnosed in large clinical centres with experience in this field. The treatment strategy should be individualised. Genetic testing for TP53 gene mutations is indicated in patients with ACC.
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