Mucinous Tubular and Spindle Cell Carcinoma of the Kidney With Sarcomatoid Change
Mucinous Tubular and Spindle Cell Carcinoma of the Kidney With Sarcomatoid Change
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DOI：
10.1097/pas.0b013e3181829ed5
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发表时间：
2009-01-01
影响因子：
5.6
通讯作者：
Reuter, Victor E.
中科院分区：
文献类型：
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作者：
Dhillon, Jasreman;Amin, Malud B.;Reuter, Victor E.
肉瘤样改变在肾细胞癌 (RCC) 的各种亚型中已得到充分记录，并且已知其存在预示着肾细胞癌的预后较差。粘液性管状和梭形细胞癌是RCC亚型，其被定义为多形性组织学，其中梭形上皮细胞是固有的癌成分。许多这些假定的低级别肿瘤以前曾被误诊为未分类或肉瘤样乳头状肾细胞癌。我们提供了 2 例迄今为止未描述的粘液性管状细胞癌和梭形细胞癌中的肉瘤样改变，患者分别为一名 71 岁女性和一名 80 岁男性，两人均接受了根治性肾切除术。除了典型的粘液性管状细胞癌和梭形细胞癌形态外，两例病例还具有肉瘤样成分，其特征主要是高级别梭形细胞、实性多形性上皮样细胞和恶性纤维组织细胞瘤样席纹状模式。肉瘤样改变占肿瘤的 60% 和 20%。分别。与梭形肉瘤样细胞不同，粘液性管状癌和梭形细胞癌的固有梭形细胞成分具有明显的低级细胞学特征，并且偶尔与管状结构和可变的粘液性基质混合。肉瘤细胞与显着坏死、明显的核多形性、高达 5/10 高倍视野的有丝分裂、较高的增殖分数 (MIB1) 以及 α-甲基酰基辅酶 A 消旋酶或细胞角蛋白 7 表达的丧失相关。 1 例肿瘤的细胞遗传学分析显示 14 号和 15 号染色体缺失，2、5、7、9、10、12、177、19、20、22 和 X 号染色体增加。广泛转移至淋巴结。一名患者在肾切除术后 9 个月死亡，导致骨骼和肺部受损。区分肉瘤样成分与天然肿瘤成分的梭形细胞的有用特征包括存在高级细胞学、扩张性生长且失去与管状乳头成分典型的难以察觉的混合、广泛坏死、高有丝分裂活性、高增殖分数以及与经典区域形成对比的α-甲基酰基-CoA消旋酶的表达丧失。将肉瘤组织学与粘液性管状癌和梭形细胞癌的固有梭形细胞成分区分开来很重要，因为它具有不利的预后意义。
Sarcomatoid change has been well documented in the various subtypes of renal cell carcinoma (RCC) and its presence is known to portend a worse prognosis in RCC. Mucinous tubular and spindle cell carcinoma is a RCC Subtype, which is defined as polymorphous histology wherein the spindled epithelial cell is an inherent carcinomatous component. Many of these putatively low-grade tumors have been previously misdiagnosed as unclassified or sarcomatoid papillary RCC. We present 2 examples of hitherto undescribed sarcomatoid change in mucinous tubular and spindle cell carcinoma in a 71-year-old woman and an 80-year-old man who both underwent a radical nephrectomy procedure. In addition to the classic mucinous tubular and spindle cell carcinoma morphology, both cases had a sarcomatoid component characterized by predominantly high-grade spindle cells, solid pleomorphic epithelioid cells, and malignant fibrous histiocytoma-like storiform patterns. Sarcomatoid change comprised 60% and 20% of the tumors. respectively. Unlike the spindle sarcomatoid cells, the inherent spindle cell elements of mucinous tubular and spindle cell carcinoma had distinctively low-grade cytology and occasionally blended with tubular structures and variable mucinous stroma. The sarcomatoid cells were associated with significant necrosis, marked nuclear pleomorphism, mitoses of up to 5/10 high power field, higher proliferation fraction (MIB1), and loss of alpha-methylacyl-CoA racemase or cytokeratin 7 expression. Cytogenetic analysis in 1 tumor showed loss of chromosomes 14 and 15 and gains of chromosomes 2. 5. 7, 9 10, 12, 177 19, 20, 22, and X. Widespread metastasis to lymph nodes. bones and lungs occurred in one patient who Succumbed 9 months after nephrectomy. Helpful features in distinguishing spindle cells of sarcomatoid component versus that of the native tumor include the presence of high-grade cytology, expansile growth with loss of typical imperceptible blending with the tubulo-papillary component, extensive necrosis, high mitotic activity, high proliferation fraction, and loss of expression of alpha-methylacyl-CoA racemase that contrasted the classic areas. Distinction of the sarcomatoid histology from inherent spindle cell component of mucinous tubular and spindle cell carcinoma is important because of its unfavorable prognostic implication.