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High-grade (HG) urothelial carcinoma (UC) with variant histology has historically been managed conservatively. The presented case details a solitary lesion of muscle-invasive bladder cancer (MIBC) with sarcomatoid variant (SV) histology treated by partial cystectomy (PC) and adjuvant chemotherapy. A 71-year-old male with a 15-pack year smoking history presented after outside transurethral resection of bladder tumor (TURBT). Computerized tomography imaging was negative for pelvic lymphadenopathy, a 2 cm broad-based papillary tumor at the bladder dome was identified on office cystoscopy. Complete staging TURBT noted a final pathology of invasive HG UC with areas of spindle cell differentiation consistent with sarcomatous changes and no evidence of lymphovascular invasion. The patient was inclined toward bladder-preserving options. PC with a 2 cm margin and bilateral pelvic lymphadenectomy was performed. Final pathology revealed HG UC with sarcomatoid differentiation and invasion into the deep muscularis propria, consistent with pathologic T2bN0 disease, a negative margin, and no lymphovascular invasion. Subsequently, the patient pursued four doses of adjuvant doxorubicin though his treatment was complicated by hand-foot syndrome. At 21 months postoperatively, the patient developed a small (

Background: Sarcomatoid urothelial carcinoma (SUC) is a rare renal malignancy. Its biological malignancy is high, the prognosis is poor, diagnostic and treatment options are few, and there is no standard treatment plan. Case presentation: In this case, a 64-year-old woman was hospitalized with fever and lower back pain one week previously. The preliminary diagnosis was a right kidney stone with a urinary tract infection. After the anti-infection treatment, a percutaneous right nephrostomy was performed. The intraoperative biopsy (renal pelvis) finding was infiltrating urothelial carcinoma with a sarcomatoid variation. Subsequently, radical surgery was performed for cancer of the right renal pelvis. Implant metastasis of the abdominal wall and adjacent abdominal cavity occurred half a month after the surgery. The lesion was resected again, and two cycles of doxorubicin plus carboplatin chemotherapy were administered. However, the disease progressed more rapidly after the chemotherapy. With the written consent of the patient, the treatment was altered to targeted immune therapy with toripalimab plus anlotinib. A clinical cure was achieved after nine cycles of treatment with no obvious lesions on imaging. The maintenance therapy was administered consecutively for over a year, and the patient is at present still in good condition with a disease-free survival exceeding two years. Conclusion: This case proves that the combination of toripalimab and anlotinib is effective in the treatment of recurrent renal SUC. To the best of our knowledge, this is the first reported case of a patient with advanced recurrent urothelial carcinoma of the renal pelvis sarcomatoid cured with this therapy. [ABSTRACT FROM AUTHOR]

Abstract Background Sarcomatoid urothelial carcinoma (SUC) is a rare and highly malignant form of bladder cancer with a poor prognosis. Currently, there is limited information on the imaging features of bladder SUC and reliable indicators for distinguishing it from conventional urothelial carcinoma (CUC). The objective of our study was to identify the unique imaging characteristics of bladder SUC and determine factors that aid in its differential diagnosis. Materials and methods This retrospective study enrolled 22 participants with bladder SUC and 61 participants with CUC. The clinical, pathologic, and CT/MRI data from both groups were recorded, and a comparison was conducted using univariate analysis and multinomial logistic regression for distinguishing SUC from CUC. Results The majority of SUCs were located in the trigone of the bladder and exhibited large tumor size, irregular shape, low ADC values, Vesical Imaging-Reporting and Data System (VI-RADS) score ≥ 4, the presence of necrosis, and an invasive nature. Univariate analysis revealed significant differences in terms of tumor location, shape, the maximum long-axis diameter (LAD), the short-axis diameter (SAD), ADC-value, VI-RADS scores, necrosis, extravesical extension (EVE), pelvic peritoneal spread (PPS), and hydronephrosis/ureteral effusion (p

Sarcomatoid urothelial carcinoma (SUC), a rare tumor of the urinary tract epithelium, exhibits a high degree of malignancy and therefore a poor prognosis. Due to the absence of specific clinical presentations and imaging findings, SUC of the renal pelvis masquerades as a renal abscess is frequently under-recognized or misdiagnosed as benign inflammatory disease, resulting in delayed or erroneous treatment. Here, we report a patient with SUC of the renal pelvis who presented with a renal abscess. Repeated anti-inflammatory treatment was ineffective. Unexpectedly, cancerous cells were detected in subsequent exfoliative cytology of nephrostomy drainage fluid. In accordance with this, radical surgery and postoperative chemotherapy were conducted. Fortunately, neither recurrence nor metastasis occurred during a one-year follow-up. [ABSTRACT FROM AUTHOR]

Primary sarcomatoid urothelial carcinoma of the ureter with heterologous elements is rare and carries a poor prognosis. Although there is some literature on primary bladder sarcomatoid urothelial carcinoma, ureteric involvement has been reported infrequently, and this case report describes this unusual histological finding with concurrent divergent squamous differentiation. Despite laparoscopic radical nephroureterectomy, our patient died within six months of diagnosis with local recurrence and metastatic spread. A more thorough understanding of this disease process and consideration of standardised guidelines for treatment are needed to improve patient outcomes.

Abstract Background Sarcomatoid urothelial carcinoma is a very dangerous malignant tumour derived from the epithelium. Primary sarcomatoid carcinoma of the ureter is extremely rare in clinical practice. The prognosis of this kind of disease is really poor, and there is still not a diagnosis standard in the world. Case presentation An 82-year-old female patient who had intermittent waist pain without any other symptoms, had diagnosed as urothelial cancer on computerised tomography urography. Considering the patient’s age and quality of life, we made a preserving kidneys resection of the local tumour. The tumour was composed of sarcomatous and carcinomatous elements, and immunohistochemical examination showed that tumour cells were positive for cytokeratin, epithelial membrane antigen, vimentin, and GATA3 markers. There were no complications after 1-hour surgery. After 3 months, there was no signs of recurrence and metastasis. Conclusion This case was a patient with sacomatoid urothelial carcinoma. Through a transurethral resection with laser, the patient recovered well, and there was no sign of any recurrence of the tumour after 3 months. With the development of technology and science, more and more cancerous patients’ living quality and survival rate were improved. Maybe it is essential for urologists and scientists to entirely understand the characteristics of the sarcomatoid urothelial carcinoma and make a better clinical guideline.

Aims: Pseudosarcomatous myofibroblastic proliferations (PSMPs) of the urinary bladder are diagnostically challenging. Diagnostic difficulties are mainly due to frequent cytokeratin expression, variable ALK expression and worrisome morphological features suggestive of malignancy. Conversely, sarcomatoid urothelial carcinoma (UC) may show bland inflammatory myofibroblastic tumour (IMT)‐like morphology. TERT promoter mutations are characteristic events in urothelial cancers, but have not been studied in PSMPs. Methods and results: We compared histomorphological and immunohistochemical features and TERT promoter status in 16 PSMPs and 18 sarcomatoid UC. In a subset of PSMPs, RNA sequencing was performed. At least focal IMT‐like morphology was seen in nine of 17 sarcomatoid UC. Atypical mitoses, differentiated urothelial component and heterologous elements were the most reliable distinguishing histomorphological features of sarcomatoid UC, if present. A panel of immunohistochemistry (IHC) including ALK (clone D5F3), p53 pattern, p63 and GATA3 reliably distinguished PSMP from sarcomatoid UC. GATA3 (P = 0.001) and p53 patterns (mutant versus wild‐type; P < 0.001) were differentially expressed between PSMPs and sarcomatoid UC. Diffuse pancytokeratin staining was significantly associated with PSMPs (10 of 13) compared to four of 14 sarcomatoid UCs (P = 0.012). TERT promoter mutations were found in 17 of 18 sarcomatoid UC versus none of 16 PSMPs (P < 0.001). RNA sequencing revealed ALK genetic rearrangements in one of two ALK‐positive and one of 10 ALK‐negative PSMPs, which revealed a novel FN1/RET gene fusion. Conclusion: Careful histomorphological analysis and differential IHC reliably distinguish the majority of PSMPs and sarcomatoid UC. In equivocal cases, TERT promoter mutation analysis and/or detection of ALK expression/rearrangements are valuable additional diagnostic adjuncts, strongly supporting sarcomatoid UC and PSMP, respectively. [ABSTRACT FROM AUTHOR]

High-grade (HG) urothelial carcinoma (UC) with variant histology has historically been managed conservatively. The presented case details a solitary lesion of muscle-invasive bladder cancer (MIBC) with sarcomatoid variant (SV) histology treated by partial cystectomy (PC) and adjuvant chemotherapy. A 71-year-old male with a 15-pack year smoking history presented after outside transurethral resection of bladder tumor (TURBT). Computerized tomography imaging was negative for pelvic lymphadenopathy, a 2 cm broad-based papillary tumor at the bladder dome was identified on office cystoscopy. Complete staging TURBT noted a final pathology of invasive HG UC with areas of spindle cell differentiation consistent with sarcomatous changes and no evidence of lymphovascular invasion. The patient was inclined toward bladder-preserving options. PC with a 2 cm margin and bilateral pelvic lymphadenectomy was performed. Final pathology revealed HG UC with sarcomatoid differentiation and invasion into the deep muscularis propria, consistent with pathologic T2bN0 disease, a negative margin, and no lymphovascular invasion. Subsequently, the patient pursued four doses of adjuvant doxorubicin though his treatment was complicated by hand-foot syndrome. At 21 months postoperatively, the patient developed a small (<1 cm) papillary lesion near but uninvolved with the left ureteral orifice. Blue light cystoscopy and TURBT revealed noninvasive low-grade Ta UC. To date, the patient has no evidence of HG UC recurrence; 8 years after PC. Patient maintains good bladder function and voiding every 3-4 h with a bladder capacity of around 350 ml. Surgical extirpation with PC followed by adjuvant chemotherapy may represent a durable solution for muscle invasive (pT2) UC with SV histology if tumor size and location are amenable. Due to the sparse nature of sarcomatous features within UC, large multicenter studies are required to further understand the clinical significance and optimal management options for this variant histology., Competing Interests: The authors declare that there is no conflict of interest., (© The Author(s), 2024.)

Aim: To determine TERT promoter mutation status as well as the expression of PAX8, GATA3, p63, p40, p53 and uroplakin III in 17 patients with the upper urinary tract sarcomatoid urothelial carcinoma.Methods& Results:  TERT C228T mutations were found in six of 17 cases (35%). p53 was expressed in 77% of these tumors. PAX8, GATA3, p40 and uroplakin III are less frequently expressed. Lymph node metastases were present in ten cases (59%). Eight patients (47%), including all three patients with TERT mutation, died of cancer within 2 years after surgery.Conclusion: Sarcomatoid carcinoma of the upper urinary tract is an aggressive tumor and the presence of TERT mutation may portend poor prognosis. [ABSTRACT FROM AUTHOR]

Inflammatory myofibroblastic tumor (IMFT) of the urinary bladder is an unusual spindle cell lesion that exhibits cytologic atypia, infiltrative growth, and mitotic activity mimicking malignant tumors, such as leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid carcinoma. Recently, anaplastic lymphoma kinase (ALK) gene translocations or ALK protein expression in IMFT has been reported, especially in patients of children and young adults. This lesion has been described in numerous locations in addition to the urinary bladder. The detection of ALK protein and ALK gene rearrangements are useful in distinguishing IMFT from spindle cell malignancies in the urinary bladder.

We present an extremely rare case where the sarcomatoid urothelial carcinoma of the urinary bladder was present with chondrosarcomatous and squamous cell differentiation. A 74-year-old male smoker presented with intermittent hematuria with the passage of clots. On imaging, an irregular polypoidal lesion was diagnosed near the right vesicoureteric junction, and transurethral resection of the bladder tumor was performed. Histopathological examination showed sarcomatoid urothelial carcinoma with chondrosarcoma and squamous cell differentiation. He refused the surgical treatment of radical cystectomy and underwent Gemcitabine and Cisplatin chemotherapy. He died nine months after the diagnosis. Sarcomatoid urothelial carcinoma is a high-grade biphasic neoplasm with malignant epithelial and mesenchymal components. Its association with squamous cell carcinoma is infrequent. It is very aggressive, and there is no standard treatment for this disease. The radical surgical option appears to be the main form of treatment. It is scarce with a grave prognosis., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Kumar Pal et al.)

Yolk sac differentiation occurs in somatic neoplasms of the gastrointestinal and gynecologic tracts; it has rarely been reported in urothelial carcinoma. Here, we report three cases of yolk sac differentiation in sarcomatoid urothelial carcinoma. The epithelioid component of the sarcomatoid urothelial carcinoma showed divergent differentiation, including squamous, conventional glandular, small cell carcinoma, and yolk sac components. The sarcomatoid component showed malignant spindle cells admixed with focal chondroid and rhabdoid elements. In all three cases, the yolk sac areas were admixed with the sarcomatoid component and showed a glandular pattern, with vacuolated, eosinophilic cytoplasm. These areas were positive for SALL4, variably positive for glypican 3 and AFP, and negative for the conventional urothelial markers GATA3, p63, and 34βE12. Yolk sac differentiation is an extremely rare occurrence in sarcomatoid urothelial carcinoma. • Yolk sac tumor differentiation rarely reported in urothelial carcinomas • Urothelial carcinomas with yolk sac tumor features are distinct from AFP producing carcinomas. • The sarcomatoid component displayed an intermediate immunoprofile. • Yolk sac tumor differentiation likely arises from aberrant differentiation of the somatic neoplasm. [ABSTRACT FROM AUTHOR]

Immunohistochemistry for PAX8 and GATA3 are sensitive markers for renal cell carcinoma and urothelial carcinoma, respectively. However, there are limited data on these markers in sarcomatoid renal cell carcinoma (SARCRCC) and sarcomatoid urothelial carcinoma (SARCUC). Tissue micro-arrays (TMAs) were constructed from 45 cases of SARCRCC and 45 cases of SARCUC of the lower urinary tract, with an additional 11 SARCUCs of the upper tract. PAX8 and GATA3 were also evaluated in TMAs from 161 sarcomas from other sites, 14 atypical epithelioid angiomyolipomas (AMLs) of the kidney, 23 bladder inflammatory myofibroblastic tumors (IMTs), and 2 bladder and 4 renal leiomyosarcomas. In the SARCRCC, PAX8 and GATA3 were positive in the sarcomatoid areas in 31 (69%) and 0 (0%) of cases, respectively. In the bladder SARCUC, GATA3 and PAX8 were positive in 14 (31%) and 2 (4%) of cases, respectively. Of the 11 SARCUCs of the upper urinary tract, 2 (18%) cases were PAX8 positive and 2 (18%) separate cases were GATA3 positive. Only 1 tumor present on the sarcoma TMAs, a Ewing sarcoma/primitive neuroectodermal tumor, was PAX8 positive, and all sarcomas were GATA3 negative. Of the AMLs, IMTs, and leiomyosarcoma, only 1 case of IMT showed moderate GATA3 positivity, and all were negative for PAX8. PAX8 can be used to distinguish SARCCRCC from atypical epithelioid AMLs and primary renal or retroperitoneal sarcomas. However, in a kidney/renal pelvic tumor, PAX8 shows overlap in staining between SARCUC and SARCRCC. GATA3 lacks sensitivity but is more specific for SARCUC. [ABSTRACT FROM AUTHOR]

Inflammatory myofibroblastic tumor (IMFT) of the urinary bladder is an unusual spindle cell lesion that exhibits cytologic atypia, infiltrative growth, and mitotic activity mimicking malignant tumors, such as leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid carcinoma. Recently, anaplastic lymphoma kinase (ALK) gene translocations or ALK protein expression in IMFT has been reported, especially in patients of children and young adults. This lesion has been described in numerous locations in addition to the urinary bladder. The detection of ALK protein and ALK gene rearrangements are useful in distinguishing IMFT from spindle cell malignancies in the urinary bladder. [ABSTRACT FROM AUTHOR]

Context: Urothelial carcinoma can exhibit a wide range of variant morphologies. Many variants present diagnostic challenges and carry clinical implications that inform prognosis and treatment decisions., Objective: To provide an overview of the diagnostic, therapeutic, and prognostic significance of histological variants of urothelial carcinoma., Evidence Acquisition: A PubMed/MEDLINE-based literature search was conducted using the key terms "urothelial carcinoma", "variant histology", "nested", "micropapillary", "microcystic", "sarcomatoid", "squamous differentiation", "glandular differentiation", "clear cell", "plasmacytoid", "lymphoepithelioma-like carcinoma", "squamous cell carcinoma", "small cell carcinoma", "adenocarcinoma", "radiotherapy", "neoadjuvant chemotherapy", and "adjuvant chemotherapy"., Evidence Synthesis: The incidence of variant histology is increasing due to improved recognition. Nonetheless, diagnosis can pose challenges due to sampling limitations and interobserver variability. Although associated with advanced disease at presentation, survival outcomes for most variants do not differ significantly compared with pure urothelial carcinoma of the same stage. Controversy exists regarding optimal management due to the low quality of available evidence. For most cases, radical cystectomy with pelvic lymph node dissection (with neoadjuvant chemotherapy when appropriate) represents the standard of care. Small cell carcinoma and lymphoepithelioma-like carcinoma appear to be particularly chemosensitive., Conclusions: Accurate identification of variant histological subtypes is an important part of risk stratification, as these variants exhibit aggressive biological behaviour. Variant histology tumours are associated with advanced disease at presentation, which must be considered when counselling patients regarding survival outcomes. Optimal management remains to be defined but in most cases; neoadjuvant chemotherapy and radical cystectomy with pelvic lymph node dissection remains the mainstay of treatment., Patient Summary: It is important to recognise histological variants of urothelial carcinoma as they indicate aggressive disease. When compared with patients with pure urothelial carcinoma of the same disease stage, survival does not appear to be significantly worse. In most cases, patients with invasive variant histology should be treated with neoadjuvant chemotherapy and radical cystectomy. Take Home Messages Accurate identification of variant histology is important as it exhibits aggressive biological behaviour and affects treatment. Although associated with advanced disease at presentation, with appropriate treatment, survival outcomes are not significantly different compared with pure urothelial carcinoma of the same stage., (Copyright © 2019 European Association of Urology. Published by Elsevier B.V. All rights reserved.)

Objective: Sarcomatoid urothelial bladder carcinoma comprises 3% of the tumours of the bladder and is considered one of the most aggressive tumours of the urinary tract. Our aim is to analyse the characteristics of sarcomatoid urothelial bladder carcinoma in adults, its treatments and survival., Method: A retrospective study performed between 2000 and 2017 of all the patients with a sarcomatoid urothelial bladder carcinoma in a single centre. We studied the anatomopathological characteristics, symptoms at time of diagnosis, treatment given and survival according to the treatment given., Results: Sixteen patients were diagnosed with sarcomatoid carcinoma, 11 with no heterologous component, one with rhabdomyosarcomatous components, 2 with chondrosarcomatous components and 2 with osteosarcomatous components. The mean age was 74 years (±20) and 88% were smokers. The primary symptom was haematuria, and the least well-tolerated was dysuria together with hypogastric pain. Ninety-four percent of the patients had muscle layer infiltration and 18% had metastases at the time of diagnosis. Thirty-seven percent of the patients were treated by radical cystectomy, thirteen percent by radical cystectomy plus adjuvant chemotherapy, and 50% were treated by palliative transurethral resection to control their symptoms. A survival curve was made with the different treatments given, which showed a mean global survival of 7 months and no statistically significant differences in terms of survival according to the treatment given., Conclusions: Sarcomatoid urothelial carcinoma is an aggressive disease, of rapid and torpid onset which occurs in patients of advanced age and smokers. There are no established treatment guidelines, and it appears that no treatment influences increased survival. Cystectomy should be evaluated as a treatment alternative for patients whose symptoms are difficult to control. The various heterologous components do not appear to influence the progression of the disease or patient survival., (Copyright © 2018 AEU. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background: Sarcomatoid urothelial carcinoma is a very dangerous malignant tumour derived from the epithelium. Primary sarcomatoid carcinoma of the ureter is extremely rare in clinical practice. The prognosis of this kind of disease is really poor, and there is still not a diagnosis standard in the world. Case presentation: An 82-year-old female patient who had intermittent waist pain without any other symptoms, had diagnosed as urothelial cancer on computerised tomography urography. Considering the patient's age and quality of life, we made a preserving kidneys resection of the local tumour. The tumour was composed of sarcomatous and carcinomatous elements, and immunohistochemical examination showed that tumour cells were positive for cytokeratin, epithelial membrane antigen, vimentin, and GATA3 markers. There were no complications after 1-hour surgery. After 3 months, there was no signs of recurrence and metastasis. Conclusion: This case was a patient with sacomatoid urothelial carcinoma. Through a transurethral resection with laser, the patient recovered well, and there was no sign of any recurrence of the tumour after 3 months. With the development of technology and science, more and more cancerous patients' living quality and survival rate were improved. Maybe it is essential for urologists and scientists to entirely understand the characteristics of the sarcomatoid urothelial carcinoma and make a better clinical guideline. [ABSTRACT FROM AUTHOR]