title: Bladder chondrosarcoma plus urothelial carcinoma in recurred transitional cell carcinoma of the upper urinary tract: a case report and literature review.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Urology, Seoul National University Hospital, Seoul, South Korea.
authors.forename: Min Hyun
authors.initials: MH
authors.lastname: Cho
authors.affiliation: 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.
authors.forename: Sung Han
authors.initials: SH
authors.lastname: Kim
authors.affiliation: Department of Pathology, Center for Prostate Cancer, National Cancer Center, Goyang, South Korea.
authors.forename: Weon Seo
authors.initials: WS
authors.lastname: Park
authors.affiliation: 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.
authors.forename: Jae Young
authors.initials: JY
authors.lastname: Joung
authors.affiliation: 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.
authors.forename: Ho Kyung
authors.initials: HK
authors.lastname: Seo
authors.affiliation: 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.
authors.forename: Jinsoo
authors.initials: J
authors.lastname: Chung
authors.affiliation: 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. uroonco@ncc.re.kr.
authors.forename: Kang Hyun
authors.initials: KH
authors.lastname: Lee
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000378:metabolism; D002295:Carcinoma, Transitional Cell / Q000150:complications / Q000473:pathology / Q000601:surgery*; D017024:Chemotherapy, Adjuvant / Q000009:adverse effects; D002813:Chondrosarcoma / Q000150:complications / Q000473:pathology / Q000601:surgery*; D002945:Cisplatin / Q000009:adverse effects / Q000627:therapeutic use; D003841:Deoxycytidine / Q000009:adverse effects / Q000031:analogs & derivatives / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology / Q000601:surgery; D007680:Kidney Neoplasms / Q000473:pathology / Q000601:surgery*; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000556:secondary; D009364:Neoplasm Recurrence, Local / Q000601:surgery*; D009367:Neoplasm Staging; D011379:Prognosis; D014057:Tomography, X-Ray Computed; D014516:Ureteral Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D001749:Urinary Bladder Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D013520:Urologic Surgical Procedures / Q000379:methods; D000093542:Gemcitabine
chemical_list: D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D003841:Deoxycytidine; D002945:Cisplatin; D000093542:Gemcitabine
keywords: Bladder; Chondrosarcoma; Recurrence; Ureter; Urothelial carcinoma