Adrenal cortical neoplasms in pediatric population are very rare. The clinical manifestations and biological behavior can be different from their counterparts in adults. This review contains data of epidemiology, clinical features, histology, biological behavior of adrenal cortical neoplasms in adults and children with particular attention to the approaches to malignancy assessment in different age groups.

Pigmented epithelioid melanocytoma (PEM) is an uncommon, recently described melanocytic lesion. There is a high rate of regional metastases, but limited evidence of distant metastases, an indolent clinical course and favorable outcome. The article presents a clinical case of PEM with aggressive behavior and distant metastases in patient with Fanconi Anemia after hematopoietic stem cell transplantation. Parents gave their consent to use information about the child, including fotos, in the article.