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摘要: To aid in establishing a diagnosis of ACC, several multifactorial scoring schemes/algorithms have been described that evaluate several features that have been associated with poor outcome (Fig. 14). I ...

To aid in establishing a diagnosis of ACC, several multifactorial scoring schemes/algorithms have been described that evaluate several features that have been associated with poor outcome (Fig. 14). In 1984, Weiss proposed the first diagnostic scheme, colloquially known as the Weiss criteria. The original Weiss criteria focused on the assessment of 9 features: high nuclear grade (Fuhrman grading system), mitotic rate more than 5 mitoses per 50 high-power fields (Fig. 15), atypical mitotic figures, less than 25% clear cells, diffuse architecture (defined as patternless sheets exceeding 30% of the tumor; nested/ alveolar, columnar, trabecular, or cordlike areas are defined as nondiffuse growth), tumor necrosis (Fig. 16), venous invasion(Fig. 17), sinusoidal invasion, and capsular invasion.The original Weiss criteria have since been modified because of the lack of reproducibility and interpretive difficulties. The modified Weiss criteria (by Aubert and colleagues) were thought to be more reproducible and easier to apply because they called for the assessment of 5 instead of 9 criteria.


【参考文献1Weiss system revisited: a clinicopathologic and immunohistochemical

study of 49 adrenocortical tumors. Am J Surg Pathol2002;26:1612-9.】。

Fig. 14. Multifactorial diagnostic schemes to aid in making the diagnosis of adrenocortical carcinoma. Several multifactorial scoring schemes/algorithms have been described to evaluate several features that have been associated with poor outcome in adrenocortical carcinomas. The Weiss and modified Weiss scoring schemes have been used to distinguish conventional adrenocortical carcinomas identified in adults. Pediatric adrenocortical tumors are typically assessed using the Wieneke multifactorial system. Oncocytic adrenocortical tumors are assessed using the Lin-Weiss-Bisceglia scoring scheme. The reticulin algorithm can be applied to conventional, oncocytic, and myxoid adrenocortical tumors identified in adults. The Helsinki scoring scheme can distinguish malignancy in conventional adrenocortical tumors in adults. This approach also provides prognostic information. Although the data on myxoid adrenocortical tumors are limited, recent evidence suggests that the diagnostic performance of the Helsinki scoring system in oncocytic adrenocortical tumors was not as good as the Lin-Weiss-Bisceglia system. However, the Helsinki score was able to predict poor prognostic subgroups of oncocytic adrenocortical carcinomas.HPF, high-power field; UMP, uncertain malignant potential.

图14 辅助肾上腺皮质癌诊断的多因素诊断方案:已经描述了几种多因素评分方案/算法来评估与肾上腺皮质癌不良预后相关的几个特征。Weiss评分法和改良的Weiss评分法被用来鉴别成人经典型肾上腺皮质癌。儿童肾上腺皮质肿瘤通常使用Wieneke多因素系统进行评估。用Lin-Weiss-Bisceglia评分法评估嗜酸细胞肾上腺皮质肿瘤。reticulin法可应用于成人经典型、嗜酸细胞和黏液样肾上腺皮质肿瘤。Helsinki评分法可以区分成人经典型肾上腺皮质肿瘤的恶性程度。这种方法也提供了预测信息。虽然黏液样肾上腺皮质肿瘤的资料有限,但最近的证据表明Helsinki评分系统对嗜酸细胞肾上腺皮质肿瘤的诊断效果不如Lin-Weiss-Bisceglia系统。然而,Helsinki评分能够预测嗜酸细胞肾上腺皮质癌的预后不良亚组。HPF高倍视野;UMP恶性潜能未定。

Fig. 15. Increased mitotic activity.Anaccurate assessment of mitotic activity is important when evaluating cortical neoplasms. Increased mitotic activity is defined as a mitotic count exceeding 5 per 50HPF. Adrenocortical carcinomas are categorized as high grade or low grade based on mitotic count fromhot spots, depending on whether up to 20 (low grade) ormore than 20mitoses (high grade) are seen per 50 HPF. Shown is an adrenocortical carcinoma with increased mitotic activity. Mitotic figures are circled.

图15 核分裂象增加:在评估皮质肿瘤时,核分裂象的重新评估是重要的。核分裂象增加是指核分裂象>5个/50HPF。肾上腺皮质癌根据热点区核分裂象计数分为高级别或低级别,这取决于每50HPF出现20个(低级别)或>20个(高级别)。图示肾上腺皮质癌伴有核分裂象增加。核分裂象被圈出。

Fig. 16. Tumor necrosis. The upper and lower parts of this composite photomicrograph show focal coagulative and geographic necrosis in adrenocortical carcinomas, respectively.

图16 肿瘤坏死:显微照片的上部和下部分别显示肾上腺皮质癌的局灶性凝固性坏死和地图样坏死。

Fig. 17. Vascular invasion (angioinvasion). Tumor cells invading through a vessel wall and/or intravascular tumor cells admixed with fibrinoid material qualify for vascular invasion.

图17 血管侵犯:肿瘤细胞侵入血管壁和/或血管内肿瘤细胞与纤维蛋白样物混合,符合血管侵犯的条件。

Because the Weiss criteria include some features of malignancy that are morphologically inherent to pure oncocytic ACTs (eg, diffuse growth pattern, scarcity of clear cells, and prominent nucleoli leading to high-grade nuclear scoring), the Lin-Weiss-Bisceglia criteria were described with an increased focus on invasiveness and mitotic count and less focus on some other morphologic features defined in the Weiss criteria. The Lin-Weiss-Bisceglia criteria use a major and minor criteria framework to classify oncocytic ACTs for which the presence of 1 major criteria (mitotic rate >5 mitoses per 50 high-power fields, atypical mitotic figures, venous invasion) indicates malignancy (Fig. 18) and the presence of 1 to 4 minor criteria (tumor size >10 cm and/or weight >200 g, necrosis, sinusoidal invasion, capsular invasion) indicates a tumor of uncertain malignant potential. Oncocytic ACAs should not show any of the major or minor criteria.

因为Weiss标准包括一些恶性肿瘤的特征,这些特征在形态学上是单纯的嗜酸细胞ACTs所固有的(例如,弥漫性生长模式,缺乏透明细胞,核仁明显导致评分高级别核),Lin-Weiss- Bisceglia标准增加对侵袭性和核分裂象计数的关注,减少对Weiss标准中的一些其他形态学特征关注。Lin-Weiss- Bisceglia标准使用一个主要和次要标准嗜酸细胞ACTs分类,存在1个主要标准(核分裂象>5个/50HPF,非典型核分裂象,静脉侵犯)表明恶性肿瘤(图18)存在1~4个次要标准(肿瘤>10cm和/或重量>200g、 坏死、窦侵犯、包膜侵犯)表明肿瘤恶性潜能未定。嗜酸细胞ACAs不应显示任何主要或次要标准。

Fig. 18. Atypical mitotic figure. Shown is a tripolar mitotic figure in an adrenocortical carcinoma.

图18 非典型核分裂象:肾上腺皮质癌中可见一个三极核分裂象。

The first algorithm to consider the use of a histochemical stain as a requirement in the morphologic evaluation of ACTs is the reticulin algorithm. This algorithm is gaining significant popularity among diagnosticians because of the objective nature of evaluating the tumor reticulin network combined with the limited number of features needed to define malignancy. ACAs typically show preserved reticulin framework (Fig. 19). When an altered reticulin network in an ACT is identified (Fig. 20) in combination with 1 or more parameters (vascular invasion, tumor necrosis, or mitotic activity >5 per 50 high-power fields), the diagnosis of ACC can be made. In addition to conventional forms of ACCs, recent series have also underscored the usefulness of the reticulin algorithm in oncocytic and myxoid ACTs. Because the distinction of myxoid ACCs can be challenging using the Weiss parameters, the reticulin algorithm may add value to the diagnostic work-up. Of note, a multicentric validation series of 245 ACTs also included 2 cases of pediatric ACT. Although the interpretation of the reticulin histochemistry is often an easy task for diagnosticians, they should be aware of the common pitfalls related to its interpretation (discussed later).


Fig. 19. Reticulin histochemistry in adrenocortical adenomas. A preserved reticulin framework is a feature of adrenocortical adenomas.


Fig. 20. Reticulin histochemistry in adrenocortical carcinomas. Loss of the reticulin framework is often seen in adrenocortical carcinomas. The upper photomicrograph represents an adrenal cortical carcinoma with altered reticulin framework (low magnification). The lower photomicrograph represents the high magnification of this tumor showing loss of reticulin framework.






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