Histological scores and tumor size on stage II in adrenocortical carcinomas

Abstract

Adrenocortical carcinomas (ACC) are aggressive tumors with a poor prognosis. Histological scores are advised for the diagnosis, however, there are borderline cases that may be misjudged as adrenocortical adenomas (ACA). The three main scores used are: Weiss Modified System (WMS), Reticulin Algorithm (RA), and Helsinki Score (HS). We intend to compare the accuracy of the three scores in ACC diagnosis and to identify predictive factors of overall survival (OS). Retrospective study (2004–2016) at Centro Hospitalar e Universitário de Coimbra of the adrenal tumors, classified as ACC or ACA, with a history of posterior tumor relapse/metastases, without lesions in the contralateral adrenal gland: 13F and 6M, with a median age of 51 ± 12.41 years. Nodules’ median size was 9.20 ± 6.2 cm. Patients had a median OS of 52 ± 18.6 months, with 57.9% and 46.3%, at 3 and 5 years. Seven patients had local recurrence and nine had metastases. Thirteen cases were in stage II. The WMS and the HS allowed a diagnosis of ACC in 15 cases and the RA defined ACC in 17 cases. All cases had, at least, focal disruption of the reticulin framework. More than 5 mitosis/50 HPF was associated with worse OS: 49.67 ± 21.43 versus 108.86 ± 14.02 months ( p = 0.026). In patients with stage II, tumor size ⩾10 cm was associated with worse OS: 19.25 ± 7.15 versus 96.11 ± 16.7 months ( p = 0.007), confirmed by multivariate analysis ( p = 0.031). The correct diagnosis of ACC is a pathologist responsibility. The RA seems the most accurate. Any loss of the reticulin framework should raise awareness for malignancy. In patients on stage II, a size ⩾10 cm is a predictor of worse prognosis.

Was It an Adrenocortical Adenoma or an Adrenocortical Carcinoma? Limitation of the Weiss Scoring System in Determining the Malignant Potential of Adrenocortical Tumor: Report on Two Cases

“…Being easier to use in clinical practice and more reproducible, the modified Weiss score has a reported specificity of 96.9% (vs. 90.2% in the original score) [ 7 ]. More recently, the reticulin algorithm and the Helsinki score ( Table 3 ) have been validated and are shown to be highly accurate in differentiating between benign and malignant adrenocortical tumors with 100% or nearly 100% sensitivity and specificity [ 3 , 4 , 14 ]. The rationale of the reticulin algorithm relies on the histochemical assessment of the reticulin network, which is commonly disrupted in ACC [ 12 ].…”

“…An accurate diagnosis of ACC is of paramount importance as it greatly impacts the management, prognosis, and surveillance strategy of a patient. Although ACC can be readily diagnosed in the presence of metastases, the differentiation between early stage ACC and ACA could be challenging histologically, especially in the setting of a well-differentiated low-grade ACC [3][4][5]. e Weiss scoring system, first proposed in 1984 and subsequently modified in 2002, is the most widely employed pathology classification system in adrenocortical tumors and is recommended as the gold standard for the diagnosis of ACC by international guidelines [2,6,7].…”