Department of Clinical and Biological Sciences, University of Turin at San Luigi Hospital, regione Gonzole 10, 10043, Orbassano, Turin, Italy.
Horm Cancer. 2011 Dec;2(6):333-40. doi: 10.1007/s12672-011-0088-0.
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.
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型（儿童型、嗜酸细胞瘤型、黏液样型和肉瘤样型）。根据 Weiss 评分系统，该诊断基于在光镜下识别至少九个形态学参数中的三个，该系统于 27 年前引入，目前应用最广泛。然而，该系统的诊断性能虽然非常高，但无法达到 100%的敏感性和特异性，在非专家病理学家中其诊断适用性可能较低，并且存在一组仅有一个或两个标准的边界病例，其行为不确定。此外，该系统在 ACC 的形态变异型中几乎无法重现。事实上，特别是对于单纯嗜酸细胞瘤型肿瘤，与传统的 ACC 相比，其预后似乎更好，因此提出了一种改良的系统（Lin-Weiss-Bisceglia 系统）。为了简化 ACC 的诊断，两年前，提出了“网状纤维”诊断算法，该算法基于观察到肿瘤网状纤维框架（通过网状纤维银染色组织化学染色突出显示）在恶性病例中始终被破坏，但仅在一小部分良性病例中被破坏。根据该算法，在存在网状纤维改变的情况下，通过确定以下至少一个参数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志物（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。
