The Weiss Score and Beyond-Histopathology for Adrenocortical Carcinoma
The Weiss Score and Beyond-Histopathology for Adrenocortical Carcinoma
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DOI：
10.1007/s12672-011-0088-0
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发表时间：
2011-12-01
影响因子：
3
通讯作者：
Tissier, Frederique
中科院分区：
文献类型：
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作者：
Papotti, Mauro;Libe, Rossella;Tissier, Frederique
肾上腺皮质癌 (ACC) 的病理诊断仍然具有挑战性，因为其罕见且存在特殊变异（儿童、嗜酸细胞、粘液样和肉瘤样）。根据 Weiss 评分系统，它基于光学显微镜对九个形态参数中至少三个的识别，该系统于 27 年前推出，如今是最广泛使用的。然而，该系统的诊断性能非常高，但未达到100%的敏感性和特异性，其诊断适用性在非专业病理学家中可能较低，并且存在一组仅具有一两个标准的边缘病例的不确定行为。此外，它在 ACC 形态变异中几乎不可重现。事实上，特别是对于与传统 ACC 相比似乎具有更好预后的纯嗜酸细胞肿瘤，已经提出了一种改进的系统（Lin-Weiss-Bisceglia）。为了简化 ACC 诊断，两年前，基于观察到肿瘤网状蛋白框架（通过网状蛋白银组织化学染色突出显示）在恶性病例中持续被破坏，但仅在一小部分良性病例中被破坏，提出了“网状蛋白”诊断算法。按照该算法，在存在网状蛋白改变的情况下，通过识别至少以下参数之一来进一步定义恶性肿瘤：坏死、高有丝分裂率和静脉侵犯。作为形态学方法的补充，一些免疫组织化学标记物（例如类固醇生成因子 1）已被提议作为诊断和预后的辅助手段，但仍缺乏广泛的临床验证。
The pathological diagnosis of adrenocortical carcinoma (ACC) is still challenging for its rarity and the presence of special variants (pediatric, oncocytic, myxoid, and sarcomatoid). It is based on the recognition at light microscopy of at least three among nine morphological parameters, according to the Weiss scoring system, which has been introduced 27 years ago and nowadays is the most widely employed. Nevertheless, the diagnostic performance of this system is very high but does not reach a sensitivity and specificity of 100%, its diagnostic applicability is potentially low among non-expert pathologists, and a group of borderline cases with only one or two criteria exist of uncertain behavior. Moreover, it is scarcely reproducible in the ACC morphological variants. In fact, specifically for the pure oncocytic neoplasms that seem to have a better prognosis in comparison to the conventional ACCs, a modified system (the Lin-Weiss-Bisceglia) has been proposed. With the aim to simplify the ACC diagnosis, 2 years ago, the "reticulin" diagnostic algorithm has been proposed, based on the observation that the tumoral reticulin framework (highlighted by reticulin silver-based histochemical staining) is consistently disrupted in malignant cases but only in a small subset of benign cases. Following this algorithm, in the presence of reticulin alterations, malignancy is further defined through the identification of at least one of the following parameters: necrosis, high mitotic rate, and venous invasion. As a complement to the morphological approach, some immunohistochemical markers (such as steroidogenic factor 1) have been proposed as diagnostic and prognostic adjuncts but still lack wide clinical validation.