[Plasmacytoid urothelial carcinoma: a rare cause of localized lymphedema]. / Plasmazytoides Urothelkarzinom: eine seltene Ursache des lokalisierten Lymphödems.

Localized lymphedema of the genital region is a rare medical condition. It is named primary lymphedema if caused by a congenital malformation of the lymphatic system. Secondary lymphedemas might be induced by exogenous damage to lymphatic vessels as a result of surgical interventions, obesity, filariasis, radiotherapy or malignancy. We report a case of localized lymphedema of the genial region for which a previously unknown urothelial carcinoma turned out to be the underlying cause.

Plasmacytoid urothelial carcinoma of the urinary bladder-A clinicopathological and molecular analysis of 52 cases.

Plasmacytoid urothelial carcinoma (UC) is a rare histologic subtype of bladder cancer that is associated with an aggressive clinical behavior. We analyzed the clinicopathologic and molecular features of plasmacytoid UC in 52 patients from a single institute. The patients included 44 men and 8 women, with a mean age of 64 years (range, 41-91 years). All bladder cancers were high-grade UC, and plasmacytoid component accounted for a mean of 47% of bladder tumors (range, 5-100%). Distinct gene mutations were found in most plasmacytoid UCs (n = 49); the most common mutations were TP53 (n = 30), followed by TERT (n = 20), and CDH1 (n = 18). Copy number analysis was performed in 34 patients, and 13 of them showed copy number variations. Expression of HER2 was analyzed in 18 patients by immunohistochemistry, and 3 of them showed HER2 overexpression, which was confirmed by fluorescence in situ hybridization analysis. Thirty-two patients died of disease in a median of 15 months (range, 1-45 months). No individual gene mutations were significantly associated with clinical outcome, but mutations in the mammalian target of rapamycin (mTOR) pathway, including PICK3CA and PIK3R1 mutations, were associated with a significantly shorter survival duration (p < 0.05). Plasmacytoid UC is an aggressive histologic subtype that demonstrates frequent somatic gene mutations and CNVs, which may underlie its oncogenesis and progression. Gene mutations of the mTOR pathway are associated with poor outcome in a subset of patients with plasmacytoid UC.

Carcinomatous Meningitis and Hydrocephalus in Plasmacytoid Urothelial Carcinoma of the Urinary Bladder With Extremely Elevated CA19-9 Levels.

This case report describes a rare and aggressive presentation of plasmacytoid urothelial carcinoma (PUC) with carcinomatous meningitis, hydrocephalus, extensive organ involvement, and extremely elevated serum CA19-9 levels. Autopsy findings revealed that PUC of the urinary bladder origin caused carcinomatous meningitis and hydrocephalus, with exacerbation of hydrocephalus as the direct cause of death. Immunohistochemical studies confirmed the bladder origin of PUC, and PUC cells were positive for CA19-9, a tumor marker commonly associated with gastrointestinal malignancies, suggesting that the markedly high serum CA19-9 level was related to the tumor-producing mechanism.

Impact of surgical margin and extent of lymphadenectomy on oncologic outcomes in plasmacytoid urothelial carcinoma.

OBJECTIVE: Guideline recommendations disagree on template boundaries for pelvic lymph node dissection (PLND) in conventional urothelial carcinoma. Less is known about PLND in variant histology. We aimed to analyze the role of LND in plasmacytoid urothelial carcinoma (PUC). METHODS: A retrospective review of patients with cTanyNanyM0 PUC who underwent radical cystectomy (RC) with PLND was performed from 2012 to 2022. Lymph node count (LNC) was a surrogate for extent of lymph node dissection and dichotomized based on maximally selected rank statistics. Multivariable cox hazard regression analysis (MVA) for overall survival (OS) corrected for age, perioperative chemotherapy, soft tissue margin status, and stage ≥pT3 and/or pN+ was performed. Disease free survival (DFS) and OS were estimated using Kaplan-Meier (KM) analysis. RESULTS: Sixty-seven patients with median age of 71, who were 79.1% male were included. Neoadjuvant and adjuvant chemotherapy were administered in 61.2% and 19.4% of patients, respectively. At RC, 70.1% were ≥pT3. Median LNC was 22 (IQR 14-27) with 43.3% of patients being pN+. Calculated optimal-LNC cut point for DFS and OS was 19. Grouping by optimal (≥20) vs. suboptimal-LNC (<20), no significant clinicodemographic differences were found. Optimal-LNC provided improved DFS (P = 0.05) and OS (P = 0.02). Optimal-LNC (HR 0.47, 0.24-0.93 CI 95%, P = 0.03) and negative soft tissue margin (HR 0.38, 0.19-0.76 CI 95%, P = 0.01) was associated with improved OS on MVA. Receipt of perioperative chemotherapy did not improve OS (P = 0.46). CONCLUSION: In PUC, complete surgical extirpation achieving negative soft tissue margins and removing ≥20 lymph should be prioritized if operative intervention is pursued.

Multiparametric magnetic resonance imaging of plasmacytoid urothelial carcinoma with histopathological correlation: A case report.

Plasmacytoid urothelial carcinomas of the bladder are rare, aggressive variants with a poor prognosis. Few reports have described the correlation of histopathological features with multiparametric magnetic resonance imaging findings in the local staging of plasmacytoid urothelial carcinoma. An 82-year-old woman with hematuria was referred to our hospital. Magnetic resonance imaging showed diffuse bladder wall thickening, with different signal intensities in the 2 layers-inner and outer. This case suggests that the presence of diffuse bladder wall thickening and varying signal intensities in the 2 layers could aid in the local staging of plasmacytoid urothelial carcinoma. A thickened bladder wall with restricted diffusion suggests tumor invasion, indicating that the tumor can invade the organ in contact with the thickened bladder wall.

Outcomes of poorly differentiated and plasmacytoid variant bladder urothelial carcinoma.

Objectives: The aim of this study is to assess the course and management of poorly differentiated bladder urothelial carcinoma (UC), including plasmacytoid UC (PUC), in our local area. Although bladder cancer is relatively common, PUC is a rare and aggressive subtype with a poor prognosis that is still poorly understood. Materials and Methods: A retrospective assessment of all poorly differentiated high-grade UC over the last 15 years (2005-2020) in the Hunter New England area was completed. In total, 37 patients were included, and PUC variant was compared with the remaining poorly differentiated UC. Results: Of the included cases, eight were PUC, nine squamous variant, two neuroendocrine, and one sarcomatoid. Overall, 23 cases proceeded to cystectomy, 15 had chemotherapy (six neoadjuvant), and 11 had radiation therapy. In the PUC subgroup, three had metastatic disease at diagnosis (37.5%). Of the three PUC patients who underwent cystectomy, all were upstaged. Two PUC cases had adjuvant chemotherapy, and one case had radiation. Within the follow-up period, the PUC group had a cause-specific mortality of 50% with a mean survival in these patients of 202 days, compared with 37.9% cause-specific mortality with survival of 671.55 days (p = 0.23) in all other undifferentiated UC cases; 5-year cause-specific mortality with Kaplan-Meier analysis was estimated at 26% compared with 59%, respectively (p = 0.058). Conclusion: Poorly differentiated UC is demonstrated to have a poor prognosis with a high mortality rate, particularly when PUC is present. Given the rarity of these variants, further studies are necessary to explore the impact of current treatment options.

Plasmacytoid urothelial carcinoma of the bladder: MRI features and their association with survival.

OBJECTIVES: Plasmacytoid urothelial carcinomas (PUC) of the bladder are rare variants known for diffuse and infiltrative spread, however their magnetic resonance imaging (MRI) features are not well established. We aimed to evaluate MRI features of PUC of the bladder and their association with survival. METHODS AND MATERIALS: This retrospective single-center study included 41 patients with pathologically-proven bladder PUC of the bladder that underwent pre-treatment MRI between January 2000 and March 2020. Two radiologists reviewed MRIs independently followed by consensus with a third radiologist. On MRI, tumor extent, size, Vesical Imaging-Reporting and Data System (VI-RADS) scores (≥4, muscle-invasive; 5, extravesical extension [EVE]), pelvic peritoneal spread (PPS), hydronephrosis, pelvic adenopathy and clinicopathological factors of age, gender, pathological stage, and treatment type were extracted. Kaplan-Meier curves and Cox proportional-hazards models were used to evaluate association with survival. RESULTS: Thirty-two men and 9 women (median age 70 years, IQR 64-76) were included. Most were muscle-invasive (n = 30 [73.2%]). On MRI, most tumors were diffuse (n = 28 [68.3%]), >5 cm (n = 30 [73.2%]), VI-RADS 4 to 5 (n = 36 [87.8%]) with features of EVE and (n = 31 [75.6%]) and PPS (n = 25 [61.0%]). Variables associated with survival were: Larger tumors (>5 cm; hazard ratio [HR] = 5.0; 95% confidence interval [CI] 1.6-15.5; P < 0.01), diffuse extent (HR = 4.0; 95% CI 1.4-11.2; P = 0.01), EVE (HR = 4.5; 95% CI 1.5-13.6; P < 0.01), PPS (HR = 3.0; 95% CI 1.2-7.4; P = 0.01), hydronephrosis (HR = 13.7; 95% CI 3.1-60.9; P < 0.01), pathologic stage (≥pT3 vs. pT1; HR = 5.6; 95% CI 1.3-22.0; P = 0.02), and margin positivity (HR = 4.4 [95% CI 1.2-16.4], P = 0.03). CONCLUSION: PUCs of the bladder are commonly large, diffuse VI-RADS score 4 to 5 tumors with MRI features of EVE and PPS. These features and pathological stage were associated with survival.

Cyto-histo correlations of plasmacytoid and micropapillary variants of high-grade urothelial carcinoma: do they fit well in The Paris System for reporting urinary cytology?

INTRODUCTION: Plasmacytoid and micropapillary variants of high-grade urothelial carcinoma (HGUC) exhibit unique histologic morphology and very aggressive clinical behavior. However, the morphology of these 2 variants in urinary cytology is not well studied and evaluated using The Paris System for reporting urinary cytology. MATERIALS AND METHODS: A database search was performed in all patients with the diagnosis of plasmacytoid or micropapillary HGUC. A total of 5 patients with positive urinary cytology cases were identified. The cytomorphology of every urinary cytology case was correlated with the histologic features in the surgical specimens from the same patient. RESULTS: One urine and 4 bladder washings were evaluated. Cytologically, plasmacytoid HGUCs are characterized by single, large tumor cells with hyperchromasia, irregular nuclear membranes, and vacuolated cytoplasm. The nuclear-to-cytoplasmic (N:C) ratio was less than 0.5 in many of the malignant cells due to the abundant cytoplasm. The cytology features of micropapillary HGUC include the presence of micropapillae of tumor cells with no fibrovascular core. Individual high-grade urothelial cells were also identified in all 4 cases, but 1 (25%) of these had only rare cells meeting The Paris System criteria for HGUC due to abundant cytoplasm and lack of hyperchromasia in most malignant cells. CONCLUSIONS: Plasmacytoid and micropapillary variants of HGUC have unique cytomorphologic features in urinary cytology specimens, which are reflective of the corresponding histological findings. These 2 clinically aggressive variants of HGUC may not be as readily interpreted as malignant using The Paris System for reporting urinary cytology, creating potential diagnostic pitfalls.

Plasmacytoid urothelial carcinoma of renal pelvis with positive zinc finger E-box-binding homeobox 1: a case report.

BACKGROUND: The tumor transformation mechanism of a plasmacytoid urothelial carcinoma remains unexplained. We describe the case of a plasmacytoid urothelial carcinoma of the renal pelvis in which the expression of zinc finger E-box-binding homeobox 1 (ZEB1), a key nuclear transcription factor in an epithelial-mesenchymal transition, is involved in tumor transformation. CASE PRESENTATION: The patient had a left nephrectomy with the clinical diagnosis of left pelvic renal cancer. The resected specimen showed that the tumor surface comprised a noninvasive papillary urothelial carcinoma with the carcinoma in situ, and the invasive area comprised a plasmacytoid urothelial carcinoma characterized by the presence of single dyscohesive malignant cells that resembled plasma cells in a loose myxoid stroma. The noninvasive urothelial carcinoma was positive for cytokeratin and E-cadherin, and negative for vimentin and ZEB1. In contrast, the invasive plasmacytoid urothelial carcinoma was positive for cytokeratin and also vimentin and ZEB1, and negative for E-cadherin. Additionally, this component was immunoreactive for CD138 and CD38 that are immunohistochemical markers for plasma cells. CONCLUSION: We suggest that ZEB1 is involved in the plasmacytoid transformation by repressing the E-cadherin in a plasmacytoid urothelial carcinoma.

Plasmacytoid variant of urothelial carcinoma of the bladder manifesting as bilateral ureteral and small bowel obstruction.

Plasmacytoid urothelial carcinoma (PUC) is a rare variant of bladder cancer characterized by distinct histopathology and advanced stage at diagnosis. Multimodal treatment is usually indicated. We present a case of PUC causing bilateral ureteral obstruction with subsequent renal failure followed shortly by malignant small bowel obstruction, demonstrating the need for a high degree of clinical suspicion in diagnosis of this aggressive subtype. Moreover, the local invasiveness of the disease cannot be understated, given that it can rapidly spread with little radiologic evidence of progression until it is at an advanced stage.

Progressive plasmacytoid variant bladder cancer with retroperitoneal dissemination: An autopsy case report.

INTRODUCTION: Plasmacytoid urothelial carcinoma is a rare and aggressive variant of bladder cancer. CASE PRESENTATION: A 75-year-old woman presented with plasmacytoid urothelial carcinoma with retroperitoneal dissemination was treated with chemotherapy. After an unsuccessful first-line chemotherapy with gemcitabine and cisplatin, we assessed circulating tumor cells; one such cell was found to be positive for programmed death-ligand 1. The patient received second-line chemotherapy with pembrolizumab. However, the tumor extended to the retroperitoneal organs, and the patient eventually died. Autopsy revealed a widespread diffuse scirrhous infiltration of the carcinoma into the retroperitoneum. However, distant metastasis was not observed. CONCLUSION: The evaluation of circulating tumor cells and autopsy revealed a disease state of progressive plasmacytoid urothelial carcinoma treated with pembrolizumab.

What Is the Significance of Variant Histology in Urothelial Carcinoma?

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.

First-line pembrolizumab therapy in a cisplatin-ineligible patient with plasmacytoid urothelial carcinoma: A case report.

BACKGROUND: Plasmacytoid urothelial carcinoma (PUC) is a rare but aggressive variant of transitional cell carcinoma. In patients with unresectable disease, cisplatin-based combination chemotherapy is the most commonly used treatment. However, many patients are cisplatin-ineligible due to poor performance status or other comorbidities. We report a case of a cisplatin-ineligible patient with metastatic PUC who was treated with pembrolizumab. CASE REPORT: A 71-year-old man with 30 pack-year smoking history and schizoaffective disorder was found to have multiple right-sided lung nodules after presenting with atypical chest pain. Staging CT showed bilateral adrenal masses and a large soft tissue mass in the right iliac fossa. Tissue pathology and immunohistochemical staining was consistent with PUC. As the patient was cisplatin-ineligible due to poor performance status and multiple medical comorbidities, the decision was made to treat with pembrolizumab. Repeat CT chest and abdomen showed partial response at three months and stable disease at six months. DISCUSSION: The KEYNOTE-052 study found that first-line pembrolizumab in cisplatin-ineligible patients with urothelial cancer resulted in complete or partial response in 24% of patients with few adverse effects. However, it is unclear if patients with plasmacytoid variant were included. To our knowledge, this is the first case report of a patient with metastatic PUC not only treated with pembrolizumab but shown to have clinical response. CONCLUSION: Given our patient's clinical response, pembrolizumab is a promising first-line agent for treating cisplatin-ineligible patients with metastatic PUC. Further evaluation is warranted to confirm the benefit of treating this patient population with pembrolizumab.

Cytological features of micropapillary and plasmacytoid variants of urothelial carcinoma.

BACKGROUND: Micropapillary and plasmacytoid variants of urothelial carcinoma (UC) exhibit very aggressive clinical behavior. To date, only a small number of cytology cases have been reported in either of these variants. Herein, we report 15 cases of UC with combined micropapillary and plasmacytoid features based on urine cytology. METHODS: We performed a retrospective analysis of all patients with carcinoma of bladder with predominant plasmacytoid and micropapillary histology who had been seen from 2005 to 2017. A total of 15 cases (six cases of plasmacytoid variant and nine cases of micropapillary variant of bladder cancer) with urine specimen were evaluated. The cytomorphological features were compared between two histological variants. RESULTS: Fifteen urine cytology cases with the diagnosis of high-grade UC were investigated. The ratio man to women was 5:1 with a median age of 79 years (range: 72-90 years). Single-cell pattern, flat sheets, three-dimensional clusters, micropapillae, nuclear grade, cytoplasmic vacuoles, and necrosis, were evaluated in urine samples of micropapillary variant. The cytological features of plasmacytoid are characterized by large, discohesive, isolated tumor cells that have abundant, thick cytoplasm, and eccentrically located, hyperchromatic nuclei with coarse chromatin and inconspicuous nucleoli. CONCLUSION: It is important to recognize the cytological characteristics of these uncommon but aggressive entities to determine a precise diagnosis. Attention to morphological features, together with clinical history and appropriate immunohistochemical studies may be useful to urologist in pre-operative planning and may lead to a more aggressive surgical approach.

Plasmacytoid Urothelial Carcinoma of the Bladder: Case Report and Review of the Literature / Carcinoma urotelial de vejiga, variante plasmocitoide: Reporte de un caso y revisión de la literatura

Introduction Plasmacytoid urothelial carcinoma (PUC) of the bladder is a rare histological variant, accounting for 1 to 3% of the invasive urothelial carcinomas, and it is typically aggressive. So far, it has not been well characterized, and the literature is based on reports and case series. Case Report A 70-year-old male patient presenting with 4 months of constitutional and urinary symptoms, with an ultrasound finding of bilateral hydronephrosis and diffuse thickening of the bladder walls. In the cystoscopy, trigone of infiltrated appearance, a biopsy wass performed, whose immunohistochemistry revealed a PUC. The abdominopelvic image showed an infiltrative lesion that compromised the muscle of the bladder and extended to the perivesical fat, without adequate plane of cleavage with the prostate and a single hypogastric adenopathy suspected of malignancy. It was classified as cT3b vs cT4aN1M0 (chest computed tomography [CT] negative for malignancy), and the patient was submitted to a radical cystoprostatectomy, extended pelvic lymphadenectomy and non-continent urinary diversion with ileal conduit. The pathology revealed a diffuse PUC with prostatic stromal involvement and 22 of 39 lymph nodes positive for malignancy. Finally, the patient presented a series of postoperative complications and died. Conclusion Plasmacytoid urothelial carcinoma of the bladder is a rare entity, characterized by high aggressiveness, an advanced stage at the time of diagnosis, and a poor prognosis. Currently, an aggressive approach is recommended due to its high invasive potential.

Introducción El carcinoma urotelial plasmocitoide (CUP) de la vejiga es una variante histológica poco frecuente; representa el 1 al 3% de los carcinomas uroteliales invasivos y es típicamente agresiva. Hasta el momento no ha sido bien caracterizada, y la literatura se basa en reportes y series de casos. Reporte de Caso Paciente masculino de 70 años presentando por 4 meses síntomas constitucionales y urinarios, con hallazgo ecográfico de hidronefrosis bilateral y engrosamiento difuso de las paredes vesicales. En la cistoscopia, trígono de apariencia infiltrada, se realizó biopsia cuya inmunohistoquímica reveló un CUP. En imagen abdominopélvica, se evidenció lesión infiltrativa que comprometía la muscular de la vejiga y se extendía a la grasa perivesical, sin adecuado plano de clivaje con la próstata y un único ganglio hipogástrico sospechoso de malignidad. Se clasificó como cT3b vs cT4aN1M0 (TAC tórax negativo para malignidad) y fue llevado a cistoprostatectomía radical, linfadenectomía pélvica extendida y derivación urinaria no continente con conducto ileal. La patología reveló un carcinoma urotelial variante difusa plasmocitoide con compromiso de estroma prostático y 22 de 39 ganglios positivos para malignidad. Finalmente, el paciente presentó una serie de complicaciones posoperatorias y falleció. Conclusión El carcinoma urotelial de vejiga variante plasmocitoide es una entidad poco frecuente, caracterizada por alta agresividad, un estadio avanzado al momento del diagnóstico, y un pobre pronóstico. En la actualidad, se recomienda un enfoque agresivo dado su alto potencial invasivo.

Organ-confined plasmacytoid urothelial carcinoma: implication of the lack of expression of the receptor tyrosine kinase MET.

Plasmacytoid urothelial carcinoma (PUC), a morphological variant of urothelial carcinoma (UC), is composed of cancer cells that resemble plasma cells, monocytes, or both, and is clinicopathologically distinguished by its aggressive, non-organ-confined features. Here, we present a case of a patient with PUC with early invasion at diagnostic transurethral resection. Histopathologically, no residual cancer or lymph node metastasis was observed by total cystectomy. The patient remains disease-free after 18 months, without undergoing adjuvant chemotherapy. Interestingly, the immunoreactivity of MET, a receptor tyrosine kinase expressed in many invasive UCs, was minimal in the cancer cells. In contrast, archival pathological, non-organ-confined PUC cells exhibited strong MET immunoreactivity. The present case may imply a role for the MET protein in the aggressive behavior of PUCs. We propose the putative usefulness of MET inhibitors for the treatment of aggressive PUCs.

Plasmacytoid Urothelial Carcinoma of the Urinary Bladder: A Clinicopathologic and Immunohistochemical Analysis of 49 Cases.

OBJECTIVES: Plasmacytoid urothelial carcinoma (PUC) of the bladder is a rare histologic variant. We retrospectively analyzed a large series of bladder PUC from a single institution. METHODS: The patients consisted of 44 men and five women with a mean age of 62 years (range, 45-86 years). RESULTS: PUC was pure in 23 cases and mixed with other histologic types in 26 cases. All PUCs diffusely invaded the bladder wall. Most PUCs lacked immunoreactivity for the retinoblastoma (RB) gene protein (12/32) and E-cadherin (8/30). Of the 44 patients with follow-up information, 25 died of PUC at a mean of 23 months, whereas 19 patients were alive at a mean of 22 months. CONCLUSIONS: Our findings support that bladder PUC is a highly aggressive disease. The lack of E-cadherin expression in PUCs may underlie the distinct discohesive histologic appearance, and abnormal function of the RB gene may be implicated in the development of PUC.

Plasmacytoid urothelial carcinoma of the urinary bladder: a clinical pathological study and literature review.

PURPOSE: Plasmacytoid carcinoma of the urinary bladder or plasmacytoid urothelial carcinoma (PUC) is a rare and only recently described histological variant of transitional cell carcinoma (TCC). We herein report the clinical and histopathological features for a new case of PUC. By combining with those reported cases, we intend to define the characteristics of PUC and to provide a therapeutic and prognostic guidance for this disease. MATERIALS AND METHODS: The index case at our institution was a patient with complaint of lower abdominal pain but without any urological symptoms. The patient underwent radical cystectomy, and the representative sections of tumor were submitted for immunohistochemical analysis. The data for this patient were collected from clinical charts, histological review and follow-up studies. We also performed an extensive literature review of PUC including clinical presentation, pathological features, therapy and prognosis. RESULTS: Clinically, patients with PUC are associated with nonspecific abdominal pain but absent of hematuria. Cystoscopy analysis revealed that PUC is manifested by the coarse and indurated mucosal fold. Macroscopic studies demonstrated an ulcerated firm mass which was present in the left lateral wall of the bladder. Histologically, PUC appeared to be dyscohesive, plasmacytoid cells with eccentric nuclei and abundant eosinophilic cytoplasm with characteristics of plasmacytoid morphology. The tumor cells are negative for E-cadherin, but positive for CD138 expression. This particular patient died 3 months after the radical cystectomy and one course of adjuvant chemotherapy. Literature review revealed that most PUC cases showed similar clinical and pathological features along with poor prognosis. CONCLUSIONS: PUC is a rare tumor associated with poor prognosis due to its advanced clinical stage upon its diagnosis. The delayed diagnosis is mainly due to the late occurrence of hematuria and absence of papulary mucosal surface at cystoscopy. Diagnosis can be achieved based on its typical histological features, clinical history and immunohistochemical results. Other than radical cystectomy, postoperative adjuvant treatment could be a good approach to prolong the survival time of PUC patients.

Cytologic Findings of a Plasmacytoid Variant of Urothelial Carcinoma of the Urinary Bladder in Voided Urine / 대한세포병리학회지

The plasmacytoid variant is an extremely rare form of urothelial carcinoma in which the malignant cells resemble those of plasmacytoma. We report the cytologic features of 3 cases of this disorder. All 3 patients were male and presented with painless macroscopic hematuria. The voided urine cytology revealed a few scattered clusters of tumor cells in a bloody background. Each tumor cell had an abundant amount of cytoplasm that was clear or densely stained and characterized by eccentrically located nuclei. A histological examination of tissue obtained from a radical cystectomy confirmed the cytologic diagnosis in each 3 case, revealing a diffusely infiltrating tumor composed of round, noncohesive tumor cells demonstrating a high nuclear grade. These cells had infiltrated the tunica propria in 2 cases, but were limited to the submucosa in 1 case. The tumor cells were plasmacytoid in appearance, each demonstrating an eccentric nucleus and dense cytoplasm, as seen in the cytologic findings. All of the tumors were immunoreactive for pancytokeratin, CK7, CK20; negative for epithelial membrane antigen (EMA), leukocyte common antigen (LCA), kappa, lambda, and CD79a. Thus, it is important to consider the plasmacytoid variant of urothelial carcinoma in addition to plasmacytoma or lymphoma as a diagnosis when encountering plasmacytoid tumor cells in a voided urine sample.