Adrenal carcinoma is a rare disease with an annual worldwide incidence of 20,000 to 30,000 cases/million children under 15 years of age. This disease in the pediatric population is associated with virilizing features and hypercortisolism in most cases. Malignancy is defined when local invasion or metastasis is found, and is associated with a poor prognosis.
The rarity of this disease makes it a real challenge.This case is a male patient, 6 years 10 months old, who had no significant past history.
He had been suffering for 5 months of evolution, had incidental fever, multiple antibiotic and antipyretic treatments, without improvement. Physical examination revealed pale skin, malnutrition, mobile adenomegaly at submandibular level, hepatomegaly of 4 cm, non-painful abdomen. No clinical data of virilization. Rest of exploration without pathological data.
Image studies were performed as abdominal ultrasound, which reported a tumor dependent on the right adrenal gland, in addition to liver parenchyma with multiple calcifications. Confirming the diagnosis with abdominal tomography in which heterogeneous hepatic lesion with lobulated edges is reported, which displaces intra-abdominal structures, not being able to distinguish structures such as kidney or adrenal gland. As well as a PET CT with hyperfunctioning lesions in liver, lung and kidney.
The rarity of this disease, the differential diagnosis with other oncologic pathologies corresponding to the age group, and the frequent delay in diagnosis, contribute to the lack of understanding of this neoplasm and advanced stages at diagnosis. The prognosis of pediatric adrenal carcinoma is poor, with a 5-year event-free survival of 37%, associated with the lack of response to the few existing treatments, especially in non-functional carcinomas.
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2022: 6.012
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