Institute of Pathology of the University of Hamburg, UKE, Martinistraße 52, 20246, Hamburg, Germany.
Clinic of Internal Medicine, Endocrinological Department of the University of Würzburg, 97080, Würzburg, Germany.
Clin Transl Oncol. 2021 Jul;23(7):1350-1357. doi: 10.1007/s12094-020-02524-2. Epub 2021 Apr 5.
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.
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.
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%).
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.
根据世界卫生组织（WHO）分类，将肾上腺癌细分为普通型、黏液样型、嗜酸细胞瘤型、肉瘤样型和儿童型已经得到广泛认可，但每种亚型的标准尚未充分确定，不同类型的肾上腺癌的相对频率也尚未在大样本中研究。因此，我们对大量手术切除的肾上腺癌进行了回顾性研究，以确定各亚型的标准，并确定各种类型的频率。
在我们的 521 例肾上腺癌系列中，使用 Weiss 等人、Hough 等人、van Slooten 等人以及新的赫尔辛基评分系统对普通型（我们系列的 97%）和黏液样型（0.8%）进行评分。对于嗜酸细胞瘤型（2%），应用 Bisceglia 等人的评分系统。
前三种经典评分系统的良性和恶性诊断之间存在差异并不罕见（我们系列中的 22%），并且可以通过赫尔辛基评分系统特别是通过 Ki-67 指数（超过 8%的病例为明确的恶性）得到解决。由于我们所有的癌症病例在赫尔辛基评分系统中均为阳性，因此该系统可以替代前三种系统。对于肉瘤样癌作为我们系列中最罕见的类型（0.2%），评分系统并不实用，但需要对软组织肿瘤进行额外的免疫组化染色，并在特殊情况下进行分子病理学检查，以将这些癌症与肾上腺肉瘤区分开来。根据不同亚型肾上腺癌的相对频率，主要类型是最常见的（97%），其次是嗜酸细胞瘤型（2%）、黏液样型（0.8%）和非常罕见的肉瘤样型（0.2%）。
在常规工作中，赫尔辛基评分是区分肾上腺癌主要类型、嗜酸细胞瘤型和黏液样型的最佳方法。对于这些癌症，一般不需要额外的评分系统。增殖（有丝分裂和 Ki-67 指数）和坏死是诊断恶性肿瘤的最重要标准。
