Cho Min Hyun, Kim Sung Han, Park Weon Seo, Joung Jae Young, Seo Ho Kyung, Chung Jinsoo, Lee Kang Hyun
Department of Urology, Seoul National University Hospital, Seoul, South Korea.
Department of Urology, Center for Prostate Cancer, Research Institute and Hospital of National Cancer Center, 323 Ilsan-ro, Ilsandong-gu, Goyang-si, Gyeonggi-do, 410-769, South Korea.
World J Surg Oncol. 2016 Oct 20;14(1):270. doi: 10.1186/s12957-016-1021-3.
Sarcomatoid urothelial carcinoma (SUC) is a rare malignant neoplasm of the urinary bladder comprising 0.2-0.6 % of all histological bladder tumor subtypes. It presents as a high-stage malignancy and exhibits aggressive biological behavior, regardless of the treatment employed. It is defined as histologically indistinguishable from sarcoma and as a high-grade biphasic neoplasm with malignant epithelial and mesenchymal components. The mean age of patients presenting with SUC is 66 years, and the male-to-female ratio is 3:1. In addition, gross hematuria is usually present. The prognosis of SUC is poorer than that of typical urothelial carcinoma because of uncertainty concerning the optimal treatment regimen.
We report the case of a 77-year-old woman with SUC containing a chondrosarcoma component who, 12 years previously, had undergone a nephroureterectomy for pT3N0M0 ureter cancer of the contralateral upper urinary tract. From the 4th year of follow-up after nephroureterectomy, multiple recurrent bladder tumors staged as Ta transitional cell carcinoma developed, and six transurethral resections of the bladder (TURB) with multiple intravesical instillations were performed without any evidence of metastases and upper tract recurrences. In 2015, a right partial distal ureterectomy and an additional TURB were performed due to a papillary mass at the right contralateral ureterovesical junction of the bladder, which was confirmed as a high-grade pT1 transitional cell carcinoma. After a further 2 years of follow-up, total pelvic exenteration with an ileal conduit diversion was performed to remove the mass, which was a pT4N0M0 tumor composed of carcinomatous and sarcomatous elements compatible with a sarcomatoid carcinoma including grade 3 transitional cell carcinoma and chondrosarcoma. Immunohistochemical examination showed that tumor cells were positive for vimentin and p63 and negative for NSE and Cd56 markers. In the first postoperative month, a metastatic lung nodule was detected on chest CT. The patient was scheduled for adjuvant gemcitabine-cisplatin chemotherapy.
The present case was interesting because we cannot be sure if the SUC chondrosarcoma originated from the 12-year-ago proximal ureter tumor, the 2-year-ago contralateral distal ureter tumor, or a new primary bladder tumor. Genetic profiling might have been useful to determine the origin of the SUC chondrosarcoma.
肉瘤样尿路上皮癌（SUC）是一种罕见的膀胱恶性肿瘤，占所有组织学膀胱肿瘤亚型的0.2 - 0.6%。它表现为高分期恶性肿瘤，无论采用何种治疗方法，均具有侵袭性生物学行为。它在组织学上被定义为与肉瘤难以区分，是一种具有恶性上皮和间充质成分的高级别双相肿瘤。SUC患者的平均年龄为66岁，男女比例为3:1。此外，通常会出现肉眼血尿。由于最佳治疗方案尚不确定，SUC的预后比典型尿路上皮癌更差。
我们报告一例77岁患有包含软骨肉瘤成分的SUC的女性病例，该患者12年前因对侧上尿路pT3N0M0输尿管癌接受了肾输尿管切除术。在肾输尿管切除术后的第4年，出现了分期为Ta的移行细胞癌的多发性复发性膀胱肿瘤，并进行了6次经尿道膀胱肿瘤切除术（TURB）及多次膀胱内灌注，未发现转移和上尿路复发的迹象。2015年，由于膀胱右侧对侧输尿管膀胱连接处的乳头状肿块，进行了右输尿管远端部分切除术及再次TURB，该肿块被确认为高级别pT1移行细胞癌。经过进一步2年的随访，为切除肿块进行了全盆腔脏器切除术及回肠导管改道术，该肿块为pT4N0M0肿瘤，由与肉瘤样癌相符的癌性和肉瘤性成分组成，包括3级移行细胞癌和软骨肉瘤。免疫组化检查显示肿瘤细胞波形蛋白和p63呈阳性，神经元特异性烯醇化酶（NSE）和Cd56标记物呈阴性。术后第一个月，胸部CT检测到一个转移性肺结节。该患者计划接受吉西他滨 - 顺铂辅助化疗。
本病例很有趣，因为我们无法确定SUC软骨肉瘤是起源于12年前的近端输尿管肿瘤、2年前的对侧远端输尿管肿瘤还是新的原发性膀胱肿瘤。基因谱分析可能有助于确定SUC软骨肉瘤的起源。
