医之本 首页 医资讯 查看内容


2020-1-7 15:58| 免费平特一肖资料大全者: admin| 查看: 77| 评论: 0

摘要: Ki67 labeling index has been shown to be prognostically significant in ACCs (discussed later). The Helsinki scoring system, proposed in 2015 and subsequently validated in a later series, has incorpora ...

Ki67 labeling index has been shown to be prognostically significant in ACCs (discussed later). The Helsinki scoring system, proposed in 2015 and subsequently validated in a later series, has incorporated the role of the Ki67 labeling index into its criteria for malignancy along with mitotic rate (>5 per 50 high-power fields) and presence/absence of necrosis. The Helsinki system uses a weighted-point system: 5 points are awarded if necrosis is present and 3 points are awarded if more than 5 mitoses are seen in 50 high-power fields. The value of the Ki67 index (percentage positive tumor nuclei from hot spots) is used as the third scoring component. A score greater than 8.5 indicates a malignant lesion. Of note, the Ki67 index should be evaluated in the area of highest proliferative activity; no visual assessment is allowed, and, ideally, the evaluation should be done by an automated image analysis software nuclear algorithm. The Italian validation series included oncocytic (mixed and pure tumors) and myxoid ACCs in addition to conventional ACCs. In this validation cohort, oncocytic ACCs (diagnosed based on the Lin-Weiss-

Bisceglia criteria) had a Helsinki score ranging from 3 to 79. Although there were only a few myxoid tumors from which to draw a reliable conclusion, the Helsinki score of 19 or greater captured a significant proportion of aggressive forms of oncocytic ACCs. In a recent multicenter French cohort, the diagnostic performance of the Helsinki scoring scheme in oncocytic ACTs was not as good as other schemes, but the Helsinki score was more useful in the prediction of poor prognostic subgroups of oncocytic ACCs. These findings have pointed out the diagnostic limitations of this scoring system; however, further studies are still needed to expand the diagnostic limitations of this scheme.


Although rare, pediatric ACTs often represent a diagnostic conundrum because it has been shown that some histologic features typically associated with malignancy in adult adrenocortical neoplasms do not necessarily correlate with poor outcome in pediatric patients. Although this may partially be related to differences in application of some cardinal features of malignancy among diagnosticians, the Wieneke system was devised to better delineate benign from malignant ACTs in the pediatric setting. The Wieneke system incorporates some traditional prognostic variables (atypical mitoses, vascular invasion, capsular invasion, tumor necrosis, mitotic rate >15 per 20 highpower fields) into its scoring algorithm, in addition to gland size and weight as well as tumor involvement of local structures, including vena cava and other extra-adrenal structures. When 4 parameters are met, a diagnosis of pediatric ACC can be made. An assignment of uncertain malignant potential can be made with a score of 3, whereas a score of 1 or 2 is thought to suggest a benign course. This approach to pediatric ACTs has also been evaluated in subsequent series.


It has been the observation of our team members that areas suggestive of adenoma-tocarcinoma tumor progression exist in some ACTs (O.M., author observation). Moreover, ACA-like low-proliferative regions can be seen admixed with nodular areas with high-grade proliferation. These lesions can be particularly challenging for diagnosticians, especially when limited representative sampling was performed from the tumor. Even so, there are very rare ACTs that are in a diagnostic gray zone and, in those cases, some diagnosticians use the term atypical or borderline adrenocortical neoplasm, whereas others apply the term ACT of uncertain malignant potential (UMP) for similar presentations. When the rare diagnosis of an ACT-UMP is being considered, additional sections should be submitted of the entire periphery of the tumor along with the central portion, and a diligent search including serial/ deeper sections should be conducted to evaluate for features of malignancy. The diagnostic workup of an ACT is no longer restricted to conventional histomorphology. There are several immunohistochemical biomarkers that can be useful in establishing a diagnosis of malignancy. Similarly, the diagnosis and prognostication of ACC is now possible when applying various molecular biology techniques in the evaluation of an ACT (discussed later).


Once a diagnosis of malignancy has been established, ACCs are further categorized as high grade or low grade based on mitotic counts from hot spots, depending on whether up to 20 (low grade) or more than 20 mitoses (high grade) are seen per 50 high-power fields. This approach stems from the original article by Weiss and colleagues, but this has been adopted in the past decade in several practices given its prognostic significance. Volante and colleagues also showed that ACCs can be further prognosticated combining tumor stage and mitotic activity (≤9 per 50 high-power fields vs >9 per 50 high-power fields). Mete and colleagues also showed that the cutoff of 10 mitoses per 50 high-power fields had a better performance in the correlation of disease-free survival in ACCs.

一旦诊断恶性肿瘤,根据热点区核分裂象计数,ACCs被分为高级别或低级别,这取决于每50HPF是否出现20个(低级别)或超过20个(高级别)的核分裂象。这种方法源于Weiss和他的同事们最初的文章,但是考虑到它的预测意义,在过去十年中在一些实践中采用了这种方法。Volante和他的同事们还发现,结合肿瘤分期和核分裂象(≤9/50HPF vs >9/50HPF),可以进一步预测ACCs生物学行为。Mete及其同事还发现,在ACCs无病生存的相关性方面,核分裂象10个/50HPF是作为具有更好预后的一个阈值。

In practice, most ACCs are easily separated from ACAs, especially when they are widely invasive and highly proliferative. Overall, it has been reported that vascular invasion (see Fig. 17), defined as tumor cells invading through a vessel wall and/or intravascular tumor cells admixed with thrombus, is the strongest diagnostic parameter and predictive of poor outcome, and thus it is reasonable to suggest that every case should be thoroughly scrutinized to rule this finding in or out, even in cases that are obviously malignant.






座右铭:《为病寻理》就要爱病理 !