腎盂肉瘤樣泌尿上皮癌合併類噬骨細胞分化之罕見案例

江衍諭¹、吳俊賢^1、2、林嘉祥^1、2、陳思翰¹

¹義大醫療財團法人義大醫院 泌尿科；²義守大學 醫學系

A Case of Sarcomatoid urothelial carcinoma

with osteoclast-like giant cells differentiation

Yen-Yu Chiang ¹, Chun-Hsien Wu^1,2, Victor C. Lin^1,2, Szu-Han Chen ¹

Department of Urology¹, E-Da Hospital, Kaohsiung, Taiwan;

School of Medicine, College of Medicine², I-Shou University, Kaohsiung, Taiwan

Introduction:

The sarcomatoid variant (SV) of urothelial cancer is rare (0.3% of cases) and often occurs in men over 50, with symptoms similar to other renal tumors. Its origins are unclear but linked to tobacco, radiation, and cyclophosphamide exposure. Surgery is the primary treatment, while radiotherapy and chemotherapy have limited documented effectiveness.

An even rarer subtype, urothelial carcinoma with osteoclast-like giant cells, is highly aggressive, with most patients succumbing within 15 months due to metastasis. Giant cells complicate diagnosis, often requiring differentiation from other giant cell tumors. Surgery remains the standard approach, though optimal treatment is uncertain.

Cases with both sarcomatoid and osteoclast-like giant cell differentiation are extremely rare and carry a poor prognosis.

Case Description:

We present the case of a 68-year-old male with acute onset respiratory failure with hematuria. Computed tomography with contrast revealed multiple complicated cysts over left kidney with hydroureteronephrosis and kidney stones (Figure A-B). Open nephroureterectomy with bladder cuff excision (Figure C-D)was then conducted with final pathology report confirmed sarcomatoid urothelial carcinoma with osteoclast-like giant cell differentiation(Figure E-H). Given the poor prognosis and the patient’s low ECOG score, palliative hospice care was initiated, and the patient passed away one month after surgery

Discussion:

 Given the lack of consensus on treatment, radical surgery with systemic chemotherapy or radiation remains the recommended approach, with platinum-based chemotherapy as the first line for metastatic upper tract urothelial carcinomas. SVUC may benefit from immunotherapy, specifically pembrolizumab, in advanced cases, but for SVUC with osteoclast-like giant cells, an optimal treatment strategy beyond surgery is still undetermined due to limited data.

Conclusion:

  Sarcomatoid urothelial carcinoma with osteoclast giant cells is rare, challenging to diagnose, and often requires surgery, though prognosis remains poor. Accurate differentiation from other renal carcinomas is crucial for improving survival. Further research is needed to establish effective treatment strategies.