ACC in Kids & Teens
Adrenal Cortical Carcinoma in Kids & Teens
Infants, children and adolescents are not just small adults! The special needs of this group of patients are addressed by pediatric physicians. It is now proven that there are fundamental differences in the development and genetic characteristics between tumors in children and adults. Therefore, the findings regarding adrenal cancer cannot be uncritically transferred from adults to children.
Since abnormal growth of tissue (neoplasms) that arise from the adrenal cortex tends to be faster in younger patients, it is essential that pediatricians work closely with physicians who treat adult tumors to ensure comprehensive care. It is also critical to treat any abnormal tissue growth as early as possible. The medical term adrenocortical tumor (ACT) is used more broadly to refer to benign, malignant and borderline/ premalignant adrenal neoplasms. This term also encompasses the cancerous form known as adrenocortical carcinoma (ACC).
Specific Characteristics
Especially in childhood, ACC ACTs are often associated with so-called cancer predisposition syndromes, meaning a genetically inherited, familial occurrence of these tumors (e.g., Li-Fraumeni, Lynch or Beckwith-Wiedemann syndromes). Genetic testing is generally recommended for all children with ACC. If a genetic predisposition is identified, it is advisable for family members to undergo genetic testing for the associated syndrome. This may help reduce cancer risk through close monitoring.
In contrast to ACC tumors in adults, almost all tumors in children produce hormones (80% of cases are hormonally active). Primarily, the stress hormone cortisol and male hormone precursors are secreted. However, about 60 percent of those affected actually suffer from hormonal disorders and therefore seek medical help. The main conditions include Cushing’s syndrome (with symptoms such as weight gain, high blood pressure, skin changes, etc.), early puberty development, or abdominal pain.
Generally, the following factors are favorable for the prognosis in children with ACC:
- Age under four years
- Androgen hormone production
- Complete tumor removal (R0 resection)
- Low tumor stage
- Low Ki67 level
- Absence of distant metastasis
Nevertheless, each case must be considered and assessed individually. No two children are alike. The following steps outline a standard procedure from first checks to various therapy options.
Checks and diagnosis
Comprehensive hormonal tests, especially of the steroid hormones produced in the adrenal cortex, are important. A thorough endocrinological examination at the onset of the disease is important, as it will later be used to assess the response to therapy and detect early recurrence. Imaging techniques such as ultrasound, magnetic resonance imaging (MRI), or positron emission tomography (PET) determine the size and extent of the tumor and any metastases. Regular blood and urine tests are becoming standard.
Surgery
If the disease is limited to the adrenal glands, complete surgical removal of the tumor is the most important first step in therapy. This procedure should be performed in a specialized center with significant experience. It has been shown that the quality of the surgery is a crucial factor for the prognosis. In the ENS@T network, a European association specializing in the study of adrenal tumors: “European Network for the Study of Adrenal Tumors,” a reference surgeon is expected to perform a significant number of adrenal surgeries per year. Relevant experience with pediatric patients can only be developed in larger centers with close interdisciplinary collaboration between pediatric surgeons and experienced tumor surgeons. Please consult the Let’s Cure ACC list of medical experts (including pediatric specialists, accessible to you) or contact us directly for support.
Medical Therapy
After surgery, or for patients who cannot be operated on right away, a medical treatment usually follows, depending on the findings. As with adults, the first-choice medication for children is also Mitotane. For children with advanced-stage disease, additional therapy combinations with chemotherapy or possibly radiation must be carefully considered. In case of recurrence or disease progression despite standard therapies, new therapeutic approaches such as immunotherapy and CAR-T cell therapies can be discussed. A genetic analysis of the tumor (through molecular pathology) to potentially apply a targeted therapy might be another pathway. Medicine continues to make progress and offers new therapeutic possibilities.
Follow-up and Care
All patients with adrenal carcinoma require long-term follow-up care, which should be coordinated between pediatric ACC specialists and local physicians. In addition, connection to a specialized general ACC center, if one exists near your home, is beneficial to quickly implement any necessary therapy changes or new treatment approaches.
Contact and Second Opinion
Since pediatric ACC is very rare, experts worldwide collaborate closely together, both clinically and scientifically. For pediatric tumors, the international working group “KIDS” of ENS@T-PACT has been established. These experts are working closely together with the Brazilian/American working group IC-PACT. Only in this way can children worldwide benefit from medical advances, and the disease be made curable for everyone in the long term.
Both groups ENSAT-PACT and St. Jude Children’s Research Hospital, USA, offer second opinions for cases of ACT in children and adolescents up to 21 years of age. As part of this review, pathology, imaging, and surgical records are evaluated by specialists with expertise in pediatric ACT. Additionally,genetic and clinical-research studies of the tumor are provided free of charge to support precision diagnostics and individualized treatment planning.
For inquiries regarding second opinions and registration of IC-PACT/ENSAT-PACT registry, contact: joseph.brigance@stjude.org or ensatpact@ukw.de
Author:
Prof. Dr. Verena Wiegering,
Phone: +49 931 201-27728,
University Hospital of Würzburg, Germany;
with contributions by Dr. Raul C. Ribeiro, MD, St. Jude Children’s Research Hospital, USA,
Sources:
– ENSAT-PACT and IC-PACT
– Prof. Dr. Verena Wiegering,
Phone: +49 931 201-27728
University Hospital of Würzburg
St. Jude Children’s Research Hospital: https://together.stjude.org/en-us/conditions/cancers/adrenocortical-tumor.html