快速轉移侵犯之腎盂泌尿上皮類肉瘤癌: 病例報告及文獻回顧

蔡易辰¹、謝昆霖¹、李健逢²、康乃文³、馮盈勳³、黃冠華¹

¹奇美醫療財團法人奇美醫學中心 外科部 泌尿科

²奇美醫療財團法人奇美醫學中心 病理部

 Extremely Rare but Rapidly Aggressive Sarcomatoid Urothelial Carcinoma: A Case report and Literature Review

I-Chen Tsai¹, Kun-Lin Hsieh¹, Chien-Feng Li², Nai-Wen Kang³, Yin-Hsun Feng³, Steven K. Huang¹

¹Divisions of Urology, Department of Surgery, Chi Mei Medical Center, Tainan, Taiwan;

²Department of Pathology, Chi Mei Medical Center, Tainan, Taiwan;

³Divisions of Oncology, Department of Internal Medicine, Chi Mei Medical Center, Tainan, Taiwan

Purpose: Sarcomatoid carcinoma is an uncommon form of carcinoma that combines epithelial and mesenchymal differentiation. Sarcomatoid urothelial carcinoma (UC) is a rare malignant neoplasm. Among previous literatures, this tumor accounts for 0.6% of all bladder tumors. Besides, it is rarer found in renal pelvix, where only fewer than 30 cases reported nowadays. There was limited data and consensus of the treatment after surgical resection. The poor outcome and rapidly progressed metastasis after diagnosis were worth discussion. We presented a 48-year-old female with sarcomatoid UC of the renal pelvis and rapid progression to wide-spread metastasis within few weeks following radical nephroureterectomy and para-aortic lymph node dissection.

Case presentation: The patient is a 44-year-old Taiwanese female who presented with fever with dysuria initially. Her underlying disease included hypertension and type 2 diabetes mellitus. She was admitted for infection control. Due to left renal staghorn stone and ureter stone with obstructive uropathy was also found on non-contrast CT, the ureteroscopy was performed and the ureter stone was removed. Under the ureterscopy, there was a bulging and tumor-like mass at renal pelvis, so endoscopic biopsy was done but pathology later demonstrated necrotic tissue without definitive evidence of malignant cells. Intraoperative cytology was negative for abnormal urothelial cells. She was discharged under stable condition.

       Due to persistent fever a week after the first operation, the contrast CT was carried. Occult tumor growth or pyelitis at lower pole of left renal pelvis with renal hilar reactive or metastatic lymphadenopathy was demonstrated. Sono-guided biopsy showed anaplastic carcinoma or sarcoma. Two weeks later after infection controlled, the surgery as left nephroureterectomy and para-aortic lymph node dissection was performed smoothly. However, left flank pain attacked two weeks later after the surgery, and we arranged following abdominal CT. Surprisingly, there were metastatic tumors in liver and lungs. Further chest CT also revealed multiple metastatic tumors within whole bilateral lung.  She then was transferred to Oncology for systemic therapy. However, she became rapidly symptomatic with dyspnea, fatigue, weakness, and weight loss associated with acute kidney injury and metabolic acidosis. After well discussion with her family, hospice care was given and she subsequently expired approximately 3 weeks later.

Conclusions: We here presented a highly aggressive sarcomatoid UC with very poor prognosis, and rapid progression to metastatic disease and death within a few months of presentation. Due to its rarity, only some case-presented studies had been reported, which most of them all emphasized about its very poor prognosis. Multidisciplinary treatment consisting of surgery, local radiotherapy, and chemotherapy of gemcitabine and cisplatin were introduced in previous literature. However, there are still no standard and good treatments about this kind of rare malignancy. Further work is needed to define the optimal multimodal management of this entity in an effort to optimize outcomes for patients with this rare and aggressive malignancy.