Takemura Kosuke, Motoi Toru, Tonooka Akiko, Funata Nobuaki, Nakanishi Yasukazu, Kataoka Madoka, Ito Masaya, Sakamoto Kazumasa, Suzuki Hiroaki, Tobisu Ken-Ichi, Koga Fumitaka
1 Department of Urology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan.
2 Department of Pathology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan.
Int J Surg Pathol. 2019 Aug;27(5):547-552. doi: 10.1177/1066896919828111. Epub 2019 Feb 15.
Carcinosarcoma of the upper urinary tract is very rare. In this article, we report a case of upper urinary tract carcinosarcoma with rhabdomyoblastic differentiation showing distinct transition between the epithelial and mesenchymal components confirmed by morphology and immunohistochemistry. An 81-year-old female underwent radical nephroureterectomy under the diagnosis of left ureteral urothelial carcinoma (UC). Multiple invasive tumors showed combined histology with UC and rhabdomyosarcomatous elements (pT2-ureter and pT3-renal pelvis, pN0, u-lt0, ly0, v0, RM0). Each element demonstrated typical epithelial or mesenchymal staining patterns (positive for AE1/AE3 in the former and positive for vimentin and myogenin in the latter). Notably, immunohistochemical transition patterns of GATA-3, p63, SOX2, and myogenin between UC and rhabdomyosarcomatous elements were observed, implying possible involvement of neoplastic stem cells in the process of carcinosarcoma formation. The patient did not receive any adjuvant therapy and eventually succumbed to multiple visceral metastases (lungs and liver) at 11 months postoperatively. No autopsy was performed.
上尿路癌肉瘤非常罕见。在本文中，我们报告了一例具有横纹肌母细胞分化的上尿路癌肉瘤病例，其上皮和间充质成分之间呈现出明显的转变，这一点通过形态学和免疫组织化学得以证实。一名81岁女性在诊断为左输尿管尿路上皮癌（UC）后接受了根治性肾输尿管切除术。多个浸润性肿瘤呈现出UC与横纹肌肉瘤成分的联合组织学表现（pT2-输尿管和pT3-肾盂，pN0，u-lt0，ly0，v0，RM0）。每个成分都显示出典型的上皮或间充质染色模式（前者AE1/AE3阳性，后者波形蛋白和肌细胞生成素阳性）。值得注意的是，观察到UC与横纹肌肉瘤成分之间GATA-3、p63、SOX2和肌细胞生成素的免疫组织化学转变模式，这意味着肿瘤干细胞可能参与了癌肉瘤的形成过程。该患者未接受任何辅助治疗，最终在术后11个月死于多处内脏转移（肺和肝）。未进行尸检。
