Department of Pathology and Laboratory Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA.
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH; Division of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Department of Pathology and Laboratory Medicine, University of Cincinnati College of Medicine, Cincinnati, OH.
Am J Surg Pathol. 2024 Mar 1;48(3):309-316. doi: 10.1097/PAS.0000000000002174. Epub 2023 Dec 29.
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.
小儿肾上腺皮质肿瘤(ACN)与成人相比极为罕见。单纯基于形态学区分良恶性具有挑战性。此前,小儿 ACN 提出了 2 种评分系统,包括 Wieneke 标准(WC)及其改良版(改良 WC [mWC])。在成人中,网状纤维算法(RA)已被证明价格低廉、可靠、具有预测性且可重现;然而,它仅在最近在少数病例中得到验证。本研究旨在评估 RA 与其他评分系统在 92 例小儿 ACN 系列中的应用。对所有病例进行单独评分,并记录有丝分裂率临界值。网状纤维改变分为定量和定性。59/92 例可获得转归数据。中位年龄为 5 岁(0.1 至 18 岁),男女比为 0.6。临床表现包括男性化(39%)、库欣综合征(21%)、其他症状(4%)和无症状(36%)。27%的病例网状纤维框架完整,73%的病例网状纤维改变,表现为定性(22%)、定量(73%)和两者兼有(5%)改变。在预后良好的患者中,59%的患者显示网状纤维完整或定性改变,而预后不良的患者中,90%的患者显示定量改变。所有评分系统 WC(P<0.0001)、mWC(P=0.0003)和成人/小儿 RA(P<0.0001)均具有预测价值。RA 与 WC 和 mWC 相当,更易于应用,是识别小儿 ACN 侵袭性行为的最敏感的组织病理学方法。将其纳入 WC 可能有助于不确定恶性潜能的 ACN,并值得进一步研究。