An adrenal cortical carcinoma (ACC) is a form of cancer originating in the adrenal gland. Most ACCs are diagnosed in adults between 30 and 50 years old or in children before the age of 6. An interesting feature of pediatric ACC is that the course of the disease often turns out to be benign (70% favorable outcomes in children compared to 35% in adults).

The authors of a new study published in the journal of Pediatric and Developmental Pathology investigated the link (or in this case, disconnection) between usually malignant pathological features of ACC and survival in early childhood.

Authors Louis Dehner and Ashley Hill found that both benign and malignant tumors had many of the same pathologically diagnostic features, and therefore these morphologies were not good indicators of the path of the disease in children.

The highest correlation was observed with tumor weight, followed by spillage and spread. The authors propose a new classification for disease stages: (1) low risk = tumors <200 g and smaller than 5 cm; (2) intermediate risk = tumors from 200 to 400 g and/or that have microscopic spillage to surrounding tissue; and (3) high risk = tumors >400 g and/or invasion of other organs or metastasis. These criteria provide clear, easy, and more effective diagnoses for childhood ACC stages.

The different progression of the tumor in children versus adults may result from the fact that cells affected by ACC in children are closer to fetal cells than adult cells, meaning it may be a “manifestation of biological regression rather than progression to malignancy.”

Pediatric and Developmental Pathology is the premiere journal dealing with the pathology of disease from conception through adolescence. It covers the spectrum of disorders developing in-utero (including embryology, placentology and teratology), gestational and perinatal diseases, and all diseases of childhood. It is published by the Society for Pediatric Pathology, a worldwide professional organization whose goal is to foster research, education, and practice as they pertain to pediatric pathology, and to provide a forum for discourse among its members. For more information about the society, visit http://www.spponline.org/.

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