Endocr Relat Cancer. 2014 May 6;21(3):405-14. doi: 10.1530/ERC-13-0494. Print 2014 Jun.
Phaeochromocytomas (PHEO) and paragangliomas are rare catecholamine-producing tumours. Although 10-30% of these tumours metastasise, histopathological criteria to discriminate malignant from benign tumours have not been established; therefore, reliable histopathological markers predicting metastasis are urgently required. A total of 163 tumours, including 40 metastatic tumours, collected by the Phaeochromocytoma Study Group in Japan (PHEO-J) were analysed using a system called grading system for adrenal phaeochromocytoma and paraganglioma (GAPP). The tumours were scored based on GAPP criteria as follows: histological pattern, cellularity, comedo-type necrosis, capsular/vascular invasion, Ki67 labelling index and catecholamine type. All tumours were scored from 0 to 10 points and were graded as one of the three types: well-differentiated (WD, 0-2 points), moderately differentiated (MD, 3-6 points) and poorly differentiated (PD, 7-10 points). GAPP scores of the non-metastatic and metastatic groups were 2.08±0.17 and 5.33±0.43 (mean±s.e.m., P<0.001) respectively. There was a significant negative correlation between the GAPP score and the interval until metastasis (r=-0.438, P<0.01). The mean number of years until metastasis after the initial operation was 5.5±2.6 years. The study included 111 WD, 35 MD and 17 PD types. The five-year survival of these groups was 100, 66.8 and 22.4% respectively. In addition, negative immunoreactivity for succinate dehydrogenase gene subunit B (SDHB) was observed in 13 (8%) MD or PD tumours and ten of the 13 (77%) had metastases. Our data indicate that a combination of GAPP classification and SDHB immunohistochemistry might be useful for the prediction of metastasis in these tumours.
嗜铬细胞瘤（PHEO）和副神经节瘤是罕见的儿茶酚胺产生肿瘤。尽管这些肿瘤中有 10-30%发生转移，但尚未建立用于区分良恶性肿瘤的组织病理学标准；因此，迫切需要可靠的组织病理学标志物来预测转移。使用一种名为肾上腺嗜铬细胞瘤和副神经节瘤分级系统（GAPP）的系统分析了日本嗜铬细胞瘤研究组（PHEO-J）收集的 163 个肿瘤，包括 40 个转移性肿瘤。根据 GAPP 标准对肿瘤进行评分，如下所示：组织学模式、细胞密度、粉刺样坏死、包膜/血管侵犯、Ki67 标记指数和儿茶酚胺类型。所有肿瘤的评分范围为 0 至 10 分，并分为以下三种类型之一：分化良好（WD，0-2 分）、中度分化（MD，3-6 分）和低分化（PD，7-10 分）。非转移性和转移性组的 GAPP 评分分别为 2.08±0.17 和 5.33±0.43（平均值±s.e.m.，P<0.001）。GAPP 评分与转移间隔时间呈显著负相关（r=-0.438，P<0.01）。初始手术后转移的平均时间为 5.5±2.6 年。该研究包括 111 例 WD、35 例 MD 和 17 例 PD 型肿瘤。这些组的五年生存率分别为 100%、66.8%和 22.4%。此外，13 例（8%）MD 或 PD 肿瘤的琥珀酸脱氢酶基因亚单位 B（SDHB）免疫反应阴性，其中 10 例（77%）发生转移。我们的数据表明，GAPP 分类和 SDHB 免疫组织化学的组合可能有助于预测这些肿瘤的转移。
