Moch H
Institut für Klinische Pathologie, UniversitätsSpital Zürich, Schmelzbergstrasse 12, 8091, Zürich, Schweiz,
Pathologe. 2015 May;36(3):278-82. doi: 10.1007/s00292-015-0018-y.
The 2004 World Health Organization (WHO) classification of renal cancer includes renal carcinoid and neuroendocrine cancer of the kidneys in the group of primary renal neuroendocrine tumors. The histological features of primary renal carcinoids are similar to those of neuroendocrine tumors found in other anatomical locations. Primary carcinoid tumors of the kidneys are frequently misdiagnosed as other kidney cancers, such as papillary renal cell carcinoma, mesonephric tumors, Wilms tumor (WT) and undifferentiated carcinoma. Immunohistochemical staining results are consistent with the diagnosis of a neuroendocrine tumor with immunoreactivity for synaptophysin, chromogranin, CD56, and neuron-specific enolase (NSE). Positive expression of CD99 can also be seen. There is mainly absence of WT1, cytokeratin 7, cytokeratin 20, thyroid transcription factor (TTF1) and LCA, ruling out most other differential diagnoses. Renal carcinoid tumors are regarded as low-grade neuroendocrine tumors; however, many studies have demonstrated metastatic disease in patients with renal carcinoid tumors. The prognostic value of histological parameters is uncertain. Some studies have correlated poor patient prognosis with increased mitotic activity, presence of necrosis and cytological atypia. Cases with higher mitotic rates of > 2 mitoses/10 high power fields (HPF) developed metastases more frequently; therefore, the WHO classification of neuroendocrine tumors used in other organs is recommended for primary renal carcinoid tumors.
2004年世界卫生组织(WHO)的肾癌分类将肾类癌和肾神经内分泌癌归入原发性肾神经内分泌肿瘤组。原发性肾类癌的组织学特征与在其他解剖部位发现的神经内分泌肿瘤相似。肾原发性类癌常被误诊为其他肾癌,如乳头状肾细胞癌、中肾肿瘤、肾母细胞瘤(WT)和未分化癌。免疫组化染色结果与神经内分泌肿瘤的诊断一致,对突触素、嗜铬粒蛋白、CD56和神经元特异性烯醇化酶(NSE)呈免疫反应性。也可见CD99的阳性表达。主要缺乏WT1、细胞角蛋白7、细胞角蛋白20、甲状腺转录因子(TTF-1)和白细胞共同抗原(LCA),排除了大多数其他鉴别诊断。肾类癌被视为低级别神经内分泌肿瘤;然而,许多研究已证实肾类癌患者存在转移情况。组织学参数的预后价值尚不确定。一些研究将患者预后不良与有丝分裂活性增加、坏死的存在以及细胞异型性相关联。有丝分裂率>2个有丝分裂/10个高倍视野(HPF)的病例更常发生转移;因此,建议对原发性肾类癌采用用于其他器官的WHO神经内分泌肿瘤分类。