PDF(Pubmed)
Abstract：
Pediatric adrenocortical neoplasms (ACNs) are extremely rare tumors in contrast to their adult counterparts. Distinguishing benign from malignant is challenging based on pure morphologic grounds. Previously, 2 scoring systems were proposed in pediatric ACN, including the Wieneke criteria (WC) and its modified version (modified WC [mWC]). In adults, the reticulin algorithm (RA) has proven inexpensive, reliable, predictive, and reproducible; however, it has been validated only recently in children in a limited number of cases. This study aims to assess the RA utility compared with other scoring systems in a series of 92 pediatric ACNs. All cases were individually scored, and mitotic rate cutoffs were recorded. Reticulin alterations were classified as quantitative and qualitative. Outcome data were available in 59/92. The median age was 5 years (0.1 to 18 y) with an M:F of 0.6. Clinical presentation included virilization (39%), Cushing syndrome (21%), other symptoms (4%), and asymptomatic (36%). The reticulin framework was intact in 27% and altered in 73% of cases, showing qualitative (22%), quantitative (73%), and both (5%) alterations. In patients with favorable outcomes, 59% showed either intact reticulin or qualitative alteration compared with the unfavorable outcome group, where 90% showed quantitative alterations. All scoring systems WC ( P < 0.0001), mWC ( P = 0.0003), and the adult/pediatric RA ( P < 0.0001) had predictive value. The RA is comparable to WC and mWC, easier to apply, and is the most sensitive histopathological approach to identifying aggressive behavior in pediatric ACN. Its integration into the WC might be helpful in ACN of uncertain malignant potential and deserves further investigation.
摘要：
与成人相比，小儿肾上腺皮质肿瘤（ACN）是极为罕见的肿瘤。基于纯粹的形态学基础，区分良性和恶性具有挑战性。以前,在儿科ACN中提出了2种评分系统，包括Wieneke标准(WC)及其修改版本(修改WC[mWC])。在成年人中,网状蛋白算法(RA)被证明是廉价的，可靠,预测性，和可重现性；然而，直到最近，它才在有限数量的儿童中得到验证。本研究旨在评估RA效用与其他评分系统在一系列92个儿科ACN中的比较。所有病例都单独评分，并记录有丝分裂率截止值。网状蛋白改变分为定量和定性。结果数据在59/92。中位年龄为5岁（0.1至18岁），M：F为0.6。临床表现包括男性化（39%），库欣综合征（21%），其他症状（4%），无症状（36%）。27％的网状结构完整，73％的病例改变，显示定性（22%），定量（73%），和两个（5％）的改变。在结果良好的患者中，与不良结局组相比，59%表现出完整的网织蛋白或定性改变。其中90%表现出数量改变。所有评分系统WC（P<0.0001），mWC(P=0.0003),成人/儿童RA（P<0.0001）具有预测价值。RA与WC和mWC相当，更容易应用，并且是识别小儿ACN中攻击行为的最敏感的组织病理学方法。将其整合到WC中可能有助于具有不确定的恶性潜力的ACN，值得进一步研究。
