PATIENT GUIDE

ACC in Childhood

ACC in Childhood or pediatric ACC

Children are not just small adults. Specialized experts for tumors in children are needed, who work closely with physicians treating adults. The medical term for adrenal carcinoma (ACC) in childhood is pediatric ACC.

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.      

Specific Characteristics

Especially in childhood, ACC can be 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

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.  

Within these international networks, the ENSAT-PACT group offers you or your medical team free and non-binding advice or a second opinion. You will find the contact details below and the experts are committed to respond to your inquiries as promptly as possible via phone or email. Please contact: ensatpact@ukw.de

Source: ENSAT-PACT