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透明细胞肾细胞癌伴合胞体样多核巨细胞肿瘤:14 例临床病理研究。

Clear Cell Renal Cell Carcinoma With Syncytial-Type Multinucleated Giant Tumor Cells: A Clinicopathologic Study of 14 Cases.

机构信息

Department of Pathology, Donostia University Hospital, San Sebastian, Spain.

Department of Pathology, Stanford University, Stanford, CA, USA.

出版信息

Int J Surg Pathol. 2024 Jun;32(4):731-737. doi: 10.1177/10668969231189798. Epub 2023 Jul 31.

DOI:10.1177/10668969231189798
PMID:37525565
Abstract

The presence of syncytial-type multinucleated giant tumor cells with emperipolesis in clear cell renal cell carcinoma (RCC) is uncommon, with only 31 cumulative published cases to date. After a rereview of 125 clear cell RCC of World Health Organization/International Society of Urological Pathology grade 3 or 4, 14 clear cell RCCs with admixed syncytial-type giant cells (to our knowledge, the largest series to date) were found with a mean patient age of 67 years and with no sex difference (M = 7, F = 7). Mean tumor size was 7.3 cm. The syncytial-type giant cells comprised between 2% and 20% of the tumor and were present mainly around areas of necrosis. Five tumors were staged as pT1 or pT2, 8 as pT3, and 1 as pT4. Other findings included sarcomatoid differentiation (3/14), rhabdoid differentiation (4/14), and emperipolesis (12/14). Positive immunostains included keratin AE1/AE3 (13/13), carbonic anhydrase 9 and CD10 (12/14 each), vimentin (8/14), EMA (5/12), and alpha-methyacyl-CoA racemase (3/12). Keratin 7, keratin 20, human melanoma black 45, KIT, TFE3, cathepsin K, CD68, CD61, and beta human chorionic gonadotropin were negative. Six of 13 patients had recurrence or metastases during a mean follow-up time of 56 months. Four of 13 patients died of disease, 2 of 13 patients were alive with the disease, and 7 of 13 patients had no evidence of disease. Although the incidence of finding syncytial-type multinucleated giant tumor cells in clear cell RCC is low (approximately 1.2%), given that a subset of the patients showed poor outcomes while lacking other poor histologic parameters (eg, sarcomatoid or rhabdoid differentiation), it may be prudent to recognize and report this feature when encountered.

摘要

在肾透明细胞癌 (RCC) 中,存在合胞体型多核巨细胞伴有吞噬现象并不常见,迄今为止,仅有 31 例累积病例报道。在重新复习了 125 例世界卫生组织/国际泌尿病理学会 3 级或 4 级的肾透明细胞癌后,我们发现 14 例肾透明细胞癌中存在混合合胞体型巨细胞(据我们所知,这是迄今为止最大的系列),患者平均年龄为 67 岁,男女比例无差异(男 7 例,女 7 例)。肿瘤平均大小为 7.3cm。合胞体型巨细胞占肿瘤的 2%至 20%,主要存在于坏死区周围。5 例肿瘤分期为 pT1 或 pT2,8 例为 pT3,1 例为 pT4。其他发现包括肉瘤样分化(3/14)、横纹肌样分化(4/14)和吞噬现象(12/14)。免疫组化阳性包括细胞角蛋白 AE1/AE3(13/13)、碳酸酐酶 9 和 CD10(各 12/14)、波形蛋白(8/14)、上皮膜抗原(5/12)和 α-甲酰基辅酶 A 消旋酶(3/12)。角蛋白 7、角蛋白 20、人黑色素瘤黑 45、KIT、TFE3、组织蛋白酶 K、CD68、CD61 和人绒毛膜促性腺激素β均为阴性。13 例患者中有 6 例在平均 56 个月的随访期间复发或转移。13 例患者中有 4 例因疾病死亡,2 例患者疾病仍在进展,7 例患者无疾病证据。尽管在肾透明细胞癌中发现合胞体型多核巨细胞的发生率较低(约 1.2%),但由于一部分患者预后不良,而缺乏其他不良组织学参数(如肉瘤样或横纹肌样分化),因此在遇到这种情况时,识别并报告这一特征可能是谨慎的做法。

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