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CASE REPORT Table of Contents  
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Plasmacytoid urothelial carcinoma with duodenal metastasis—A rare tumor with unusual site of metastasis with grim outcome

1 Department of Pathology (Histopathology Section), Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India
2 Department of Urology and Renal Transplantation, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India
3 Department of Radiodiagnosis, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India

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Date of Submission18-Aug-2021
Date of Decision20-Jan-2022
Date of Acceptance30-Jan-2022
Date of Web Publication27-Jan-2023


Plasmacytoid urothelial carcinoma (PUC) is a newly recognized rare variant of urothelial carcinoma, which is now being increasingly diagnosed prospectively as well as by retrospective analysis of cases with a poor prognosis. Morphologically, these tumors simulate plasma cell neoplasms and pose a diagnostic challenge. Identifying this variant is essential in two aspects: therapy and prognosis. Here, we present a case who underwent multiple transurethral resection of bladder tumor (TURBT) procedures, each with a diagnosis of urothelial carcinoma, the plasmacytoid type which was confirmed on radical cystectomy, and after 1 year, the patient presented with duodenal metastasis. We discuss the morphological aspects of differentiating this tumor from variants of urothelial carcinoma and other tumors with a plasmacytoid appearance. Despite the recognition and aggressive treatment, the patient expires within 2 years of the first diagnosis of bladder carcinoma.

Keywords: Duodenal metastasis, plasmacytoid, urothelial carcinoma

How to cite this URL:
Chenniappan A, Stephen NS, Srinivas BH, Sreenivasan SK, Govindarajalou R. Plasmacytoid urothelial carcinoma with duodenal metastasis—A rare tumor with unusual site of metastasis with grim outcome. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Nov 30]. Available from:

   Introduction Top

Urothelial carcinoma is the most common bladder tumor comprising 90% of all bladder malignancies.[1] The 2016 WHO classification included new variants, of which plasmacytoid urothelial carcinoma (PUC) and micropapillary carcinoma are considered as most aggressive variants.[2] PUC is a rare variant with morphology similar to plasma cells and should be distinguished from plasma cell neoplasm.

To date, a few case series are reported in the literature. We record our case of PUC with an aggressive nature, metastasizing to the duodenum, which is rarely reported in the literature.

   Case Summary Top

A 71-year-old man presented elsewhere with complaints of frequency of micturition and hematuria. The patient also had a loss of weight and appetite and was a known diabetic and hypertensive for 16 years. Transurethral resection of bladder tumor (TURBT) was performed which was reported as low-grade papillary urothelial carcinoma. After 6 months, TURBT was performed again due to recurrent complaints, which was reported as high-grade urothelial carcinoma with lamina propria invasion.

After 1 year, due to urinary retention, a biopsy was performed which revealed high-grade urothelial carcinoma with muscularis invasion. A repeat TURBT biopsy for persistent problems was submitted to our institution for review.

Microscopically, the tumor cells were seen infiltrating the deep muscle of the bladder in sheets. These neoplastic cells were poorly cohesive with a prominent plasmacytoid morphology, eccentrically placed nucleus, and single nucleolus. Signet ringlike malignant cells that were seen stained negative for mucicarmine. Lymphovascular emboli were noted.

Imaging revealed a bladder neck growth involving the prostate measuring 3.5 × 3 cm. Perivesical extension was not seen. External iliac and perirectal nodes were noted. The patient underwent radical cystectomy with an ileal conduit [Figure 1] and [Figure 2].
Figure 1: MRI T2-weighted coronal and sagittal images show growth in the region of bladder neck with contiguous involvement of prostate. Coronal reformatted contrast-enhanced CT scan image shows long segment of thickening involving the third and fourth parts of the duodenum

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Figure 2: Photomicrographs of (a) TURBT biopsy show tumor arranged in sheets and cords (H and E X40): (b) poorly cohesive tumor cells having prominent plasmacytoid morphology with eccentric nuclei and focal signet ring morphology (arrow head) (H and E X200): (c) mucicarmine stain shows the absence of mucin (Mucicarmine x200): immunohistochemistry (IHC) on tumor cells with (d and e) GATA3 and P63 show strong nuclear positivity (DABX400 and DABX200): (f) IHC with CD138 shows strong continuous membranous positivity (DAB X200)

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Radical cystectomy specimen revealed an ulcero-proliferative growth measuring 4 × 3 × 3 cm infiltrating the bladder's neck with involvement of underlying muscle.

The cut section of the tumor was gray-white, solid, and homogenous. Microscopically, the tumor had plasmacytoid morphology with infiltration into the muscularis propria and prostate. The posterior wall of the bladder, bilateral seminal vesicles, internal and external iliac, obturator nodes, and distal and proximal urethral margins were free of tumor. The pathological stage was pT4NoMx. Immunohistochemistry (IHC) revealed positivity for cytokeratin 7 and 20, CD138, GATA3, and p63 [Figure 3].
Figure 3: (a) Gross specimen of the cystectomy shows ulcero-proliferative growth in the neck of the bladder: (b) shows deep muscle infiltration and angioinvasion by tumor cells (H and E X100): (c) shows normal duodenal mucosa along with sheets of similar metastatic tumor cells in lamina propria (H and E X100): (d) tumor cells show plasmacytoid and signet ring cell morphology (H and E X200): (e) tumor cells show strong IHC positivity with CD138 (DABX200): (f) GATA3 shows nuclear positivity (DAB X100)

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After 6 months, due to abdominal symptoms, upper gastrointestinal endoscopy was performed, and a duodenal growth was noted. Contrast-enhanced CT showed long segment thickening involving the third and fourth parts of the duodenum. Duodenal biopsy showed histomorphology and IHC as that of bladder tumor, being consistent with metastasis.

   Discussion Top

PUC is a rare variant of urothelial carcinoma, accounting for 1–3% of all urothelial carcinomas,[1] and is characterized by tumor cells having plasmacytoid and signet ring cell morphology. These tumors pose difficulties in diagnosis when associated with lytic lesions due to plasmacytoid morphology and cause confusion with multiple myeloma.[3]

To date, the largest case series of PUC consisted of 60 to 70 cases comprising patients with a mean age of 68 years with a male predominance.[4] The most common presentation is hematuria and frequency of micturition.

Most patients present with a higher stage at diagnosis. They carry a poor prognosis and have an increased risk of extravesical disease, involvement of perirectal, pararectal, and perivesical fascial planes. Rare cases showed scrotal invasion.[5] In our case, perivesical extension was not noted. Morphologically, the plasmacytoid appearance of the tumor has a variety of differential diagnoses, mainly plasma cell neoplasm. The morphological clue to differentiate PUC from plasmacytoma is that plasma cells have a perinuclear halo and exhibit binucleation and multinucleation.[6]

Extensive examination for foci of papillary/squamous architecture of tumor favors the former diagnosis. IHC plays a significant role in definitive diagnosis. Other differential diagnosis includes metastatic gastric adenocarcinoma, rhabdomyosarcomas, melanoma, and mammary carcinomas with plasmacytoid morphology.[7]

PUC has also been described as signet ring cell carcinoma by some authors. It has to be differentiated from true signet ring carcinomas. True signet ring carcinomas have a peripheral nucleus with intracytoplasmic mucin, whereas, in PUC, the nucleus is just eccentrically placed but not pushed to the periphery and absence of mucin.[6] The present case also showed signet ring cells with a lack of mucin as confirmed by mucicarmine.

Immunohistochemically, PUC is immunoreactive for cytokeratin 7 and 20, CAM 5.2, EMA, p63, and rarely CA 19-9 and beta-HCG.[3] Some studies suggest that loss of E-cadherin favors plasmacytoid differentiation. Others say that loss of E-cadherin is mere evidence of epithelial–mesenchymal transition associated with invasive nature, muscularis mucosal involvement, and tumor recurrence.[1],[3] GATA3 is immunoreactive in many variants of urothelial carcinoma, including PUC.[8]

Thus, GATA3 is an appropriate marker in the differential diagnosis and useful in recognizing the urothelial lineage of PUC in metastatic settings, like our case.[9] CD138 is a member of the transmembrane heparan sulfate proteoglycan family, which is expressed in normal and malignant plasma cells. It has been criticized by many authors that it is immunoreactive in a variety of non-hematopoietic neoplasms.[10] Many studies have documented the immunoreactivity of this marker in PUC.[5],[6]

It is weakly positive in normal urothelium and benign urothelial lesions and strongly reactive in many variants of urothelial carcinoma apart from PUC. It cannot be evidenced as a definitive marker for PUC.[11] Nevertheless, CD138 positivity is a sign of plasmacytoid differentiation.

PUC has a predilection for intraperitoneal spread with an aggressive course. Our patient had duodenal metastasis and succumbed to death within 2 years of diagnosis. A literature search revealed two prior cases of PUC, which have metastasized one to the stomach and the other to the duodenum.[11],[12] To the best of our knowledge, this is the second case with isolated duodenal metastasis. The tumor's metastatic pattern has to be studied to identify the propensity for duodenal metastasis.

Usually, metastatic urothelial carcinomas are treated with cisplatin-based chemotherapy. The role of neoadjuvant chemotherapy is still under trial.[2] Despite radical surgeries and chemotherapy, PUC runs a worse course and proves fatal.

   Conclusion Top

PUC is a variant with a dismal prognosis. Early diagnosis facilitated by morphology and immunohistochemistry can help in aggressive treatment. This, to an extent, can prolong the life of these patients, however, with significant morbidity. Thus, recognition of this entity is prognostically significant.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Fox MD, Xiao L, Zhang M, Kamat AM, Siefker-Radtke A, Zhang L, et al. Plasmacytoid urothelial carcinoma of the urinary bladder. Am J Clin Pathol 2017;147:500-6.  Back to cited text no. 1
Keck B, Wach S, Stoechr R, Kunath F, Bertz S, Lehmann J, et al. Plasmacytoid variant of bladder cancer defines patients with a poor prognosis if treated with cystectomy and adjuvant cisplatin-based chemotherapy. BMC Cancer 2013;13:71.  Back to cited text no. 2
Wang Z, Lu T, Du L, Hu Z, Zhuang Q, Li Y, et al. Plasmacytoid urothelial carcinoma of the urinary bladder: A clinical-pathological study and literature review. Int J Clin Exp Pathol 2012;5:601-8.  Back to cited text no. 3
Haeshemi-Sadraei N, Perrino CM, Monn MF, Bandali E, Cheng L, Idrees M. Plasmacytoid urothelial carcinoma: A clinicopathological study. J Clin Oncol 2018;36:482.  Back to cited text no. 4
Wang YG, Perera M, Gleeson J. Plasmacytoid urothelial carcinoma of the bladder with extensive scrotal wall invasion. Urol Ann 2016;8:381-3.  Back to cited text no. 5
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Nigwekar P, Tamboli P, Amin MB, Osumkova AO, Ben-Dor D, Amin MB. Plasmacytoid urothelial carcinoma: Detailed analysis of morphology with clinicopathological correlation in 17 cases. Am J Surg Pathol 2009;33:417-2.  Back to cited text no. 6
Rahman K, Menon S, Patil A, Bakshi G, Desai S. A rare case of plasmacytoid urothelial carcinoma of the bladder: Diagnostic dilemmas and clinical implications. Indian J Urol 2011;27:144-66.  Back to cited text no. 7
[PUBMED]  [Full text]  
Brustmann H. Plasmacytoid urothelial carcinoma of the urinary bladder metastatic to the duodenum: A case report- Diagnostic relevance of GATA3 immunohistochemistry. Case Rep Pathol 2017;2017:1-4.  Back to cited text no. 8
Paner GP, Annaiah C, Gulmann C, Rao P, Ro JY, Hansel DE, et al. Immunohistochemical evaluation of novel and traditional markers associated with urothelial differentiation in a spectrum of variants of urothelial carcinoma of the urinary bladder. Hum Pathol 2014;45:1473-82.  Back to cited text no. 9
O'Connell FP, Pinkus JL, Pinkus GS. CD138 (syndecan-1), a plasma cell marker. Immunohistochemical profile in hematopoietic and nonhematopoietic neoplasms. Am J Clin Pathol 2004;121:254-63.  Back to cited text no. 10
Goto K. Cd138 expression is observed in the urothelial epithelium and in various urothelial cancers, and cannot be evidence for plasmacytoid urothelial carcinoma. Int J Surg Pathol 2016;24:614-9.  Back to cited text no. 11
Nabbout P, Furr J, Paari M, Slobodov G. Plasmacytoid urothelial carcinoma of the bladder metastatic to the stomach: A case report. Case Rep Urol 2012;2012:1-4.  Back to cited text no. 12

Correspondence Address:
Bheemanathi H Srinivas,
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry - 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_840_21


  [Figure 1], [Figure 2], [Figure 3]


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