ABSTRACT
Genetic and oncological counselling is available for family members with TP53 mutations. Careful monitoring with a combination of imaging and thyroglobulin measurement is necessary, and lifelong oncological follow-up is necessary as late relapses are well recognised. Diagnosis, treatment and follow-up of children with adrenocortical carcinoma (ACT) are paradigm of multi-disciplinary approach in paediatric oncology, where endocrinologist, oncologist, surgeon, pathologist and clinical geneticist work together in management and long-term follow-up of the patients. In very young children a differential diagnosis of stage Ms neuroblastoma with diffuse metastatic spread to the liver can usually be made by identifying an adrenal primary tumour by ultrasound. ACT also occurs more often in patients with hemihypertrophy and Beckwith–Wiedemann syndrome. Adrenocortical carcinoma is more common in females and presents with signs of virilisation, Cushingoid features or persistent, refractory hypertension. The histology of rhabdomyosarcoma varies, with two main subtypes alveolar and embryonal found in children, while the third pleomorphic subtype is mainly diagnosed in adults.
