Adrenal cancer: relevance of different grading systems and subtypes

Abstract

Purpose The subclassification of adrenal cancers according to the WHO classification in ordinary, myxoid, oncocytic, and sarcomatoid as well as pediatric types is well established, but the criteria for each subtype are not sufficiently determined and the relative frequency of the different types of adrenal cancers has not been studied in large cohorts. Therefore, our large collection of surgically removed adrenal cancers should be reviewed o establish the criteria for the subtypes and to find out the frequency of the various types. Methods In our series of 521 adrenal cancers the scoring systems of Weiss et al., Hough et al., van Slooten et al. and the new Helsinki score system were used for the ordinary type of cancer (97% of our series) and the myxoid type (0.8%). For oncocytic carcinomas (2%), the scoring system of Bisceglia et al. was applied. Results Discrepancies between benign and malignant diagnoses from the first thee classical scoring systems are not rare (22% in our series) and could be resolved by the Helsinki score especially by Ki-67 index (more than 8% unequivocally malignant). Since all our cancer cases are positive in the Helsinki score, this system can replace the three elder systems. For identification of sarcomatoid cancer as rarest type in our series (0.2%), the scoring systems are not practical but additional immunostainings used for soft tissue tumors and in special cases molecular pathology are necessary to differentiate these cancers from adrenal sarcomas. According to the relative frequencies of the different subtypes of adrenal cancers the main type is the far most frequent (97%) followed by the oncocytic type (2%), the myxoid type (0.8%) and the very rare sarcomatoid type (0.2%). Conclusions The Helsinki score is the best for differentiating adrenal carcinomas of the main, the oncocytic, and the myxoid type in routine work. Additional scoring systems for these carcinomas are generally not any longer necessary. Signs of proliferation (mitoses and Ki-67 index) and necroses are the most important criteria for diagnosis of malignancy.

The new histological system for the diagnosis of adrenocortical cancer

“…Among all the proposed assessment systems, according to the latest data, the Helsinki score is the most reliable ( 19 ). However, it should be borne in mind that the assessment of mitoses in the cells of myxoid and oncocytic variants of ACС may be difficult, which may also lead to underestimation of the malignant potential of the tumor.…”

A novel cuproptosis‐related prognostic gene signature in adrenocortical carcinoma

“…The World Health Organization (WHO) has recognized four histological variants of ACC, namely, oncocytic, myxoid, sarcomatoid and pediatric. 35,36 Different subtypes may from an article published in Science. 10 However, these 10 genes may not comprehensively illuminate the mechanism of cuproptosis, suggesting that there could be additional CRGs awaiting discovery.…”

“…This anomaly may have been caused by an insufficient number of patients with ACC. The World Health Organization (WHO) has recognized four histological variants of ACC, namely, oncocytic, myxoid, sarcomatoid and pediatric 35,36 . Different subtypes may have different prognoses.…”

“…The World Health Organization (WHO) has recognized four histological variants of ACC, namely, oncocytic, myxoid, sarcomatoid and pediatric. 35 , 36 Different subtypes may have different prognoses. It is crucial to further increase the biological research of ACC with a larger sample size in the future.…”

The Enhanced Expression of ZWILCH Predicts Poor Survival of Adrenocortical Carcinoma Patients

“…According to the WHO classification of adrenocortical tumors from 2017, the different subtypes of ACC include the main type, myxoid type, oncocytic type, sarcomatoid type, and pediatric type. [ 3 ]. Possible predictors for survival time among ACC patients are: Disease stage, defined by the European Network for the Study of Adrenal Tumours (ENSAT), radical surgery, age, cell proliferation potential, antigen Ki67, and Helsinki Score.…”