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Presented by Dr. Jonathan Epstein and prepared by Dr. Robby Jones.
A 65 year old woman underwent a transurethral resection for a bladder mass.
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Clinical History: A 65 year old woman underwent a transurethral resection for a bladder mass. The tumor was positive for Gross Cystic Duct Fluid Protein (GCDFP), progesterone receptor (PR), GATA3, uroplakin II with negative staining for estrogen receptor (ER). Some of the cells with signet-ring cell vacuoles were positive for mucicarmine.
Choose the correct diagnosis:
Correct
D. Infiltrating plasmacytoid urothelial carcinoma
Histological Description: The tumor consists of infiltrating cells with variable cytology. Many of the cells have eccentric cytoplasm which is amphophilic, resembling plasma cells. However, the nuclei have a single small central nucleolus as opposed to the more stippled chromatin in plasma cells. Some cells have clear cytoplasmic vacuoles with a signet-ring cell appearance. Other cells have central uniform nuclei with a scant ring of amphophilic cytoplasm similar to the cells in lobular carcinoma of the breast. No in situ urothelial carcinoma component is noted.
Discussion: This tumor is typical of plasmacytoid urothelial carcinoma. Other cases, in addition to cells with plasmacytoid morphology, may have signet-ring cells, cells resembling lobular carcinoma of the breast, and cells with a rhabdoid appearance. The unifying feature in plasmacytoid urothelial carcinoma is the dyscohesive nature of the cells which is also manifested by loss of e-cadherin immunohistochemically similar to what is seen with lobular carcinoma of the breast and signet-ring cell adenocarcinoma from the gastrointestinal tract. In a woman, plasmacytoid urothelial carcinoma must be differentiated from metastatic lobular carcinoma of the breast and metastatic signet ring cell adenocarcinoma from the gastrointestinal tract, if there is no associated more definitive urothelial component (ie CIS or papillary urothelial carcinoma). We have recently shown the plasmacytoid urothelial carcinoma can express GCDFP and PR, mimicking lobular carcinoma. GATA3 is not helpful in this differential as it is positive in both breast and urothelial carcinoma. The two useful immunohistochemical stains for this differential diagnosis are ER, which is negative in plasmacytoid urothelial carcinoma and almost uniformly positive in lobular carcinoma, as well as uroplakin II, which is positive in 50% of plasmacytoid urothelial carcinomas and negative in lobular carcinoma. GATA3 positivity, present in almost all plasmacytoid urothelial carcinomas, is negative in signet-ring cell adenocarcinoma from the stomach. Plasmacytoid urothelial carcinoma is a very aggressive variant of urothelial carcinoma with a predilection for spreading within the peritoneal cavity.
Incorrect
D. Infiltrating plasmacytoid urothelial carcinoma
Histological Description: The tumor consists of infiltrating cells with variable cytology. Many of the cells have eccentric cytoplasm which is amphophilic, resembling plasma cells. However, the nuclei have a single small central nucleolus as opposed to the more stippled chromatin in plasma cells. Some cells have clear cytoplasmic vacuoles with a signet-ring cell appearance. Other cells have central uniform nuclei with a scant ring of amphophilic cytoplasm similar to the cells in lobular carcinoma of the breast. No in situ urothelial carcinoma component is noted.
Discussion: This tumor is typical of plasmacytoid urothelial carcinoma. Other cases, in addition to cells with plasmacytoid morphology, may have signet-ring cells, cells resembling lobular carcinoma of the breast, and cells with a rhabdoid appearance. The unifying feature in plasmacytoid urothelial carcinoma is the dyscohesive nature of the cells which is also manifested by loss of e-cadherin immunohistochemically similar to what is seen with lobular carcinoma of the breast and signet-ring cell adenocarcinoma from the gastrointestinal tract. In a woman, plasmacytoid urothelial carcinoma must be differentiated from metastatic lobular carcinoma of the breast and metastatic signet ring cell adenocarcinoma from the gastrointestinal tract, if there is no associated more definitive urothelial component (ie CIS or papillary urothelial carcinoma). We have recently shown the plasmacytoid urothelial carcinoma can express GCDFP and PR, mimicking lobular carcinoma. GATA3 is not helpful in this differential as it is positive in both breast and urothelial carcinoma. The two useful immunohistochemical stains for this differential diagnosis are ER, which is negative in plasmacytoid urothelial carcinoma and almost uniformly positive in lobular carcinoma, as well as uroplakin II, which is positive in 50% of plasmacytoid urothelial carcinomas and negative in lobular carcinoma. GATA3 positivity, present in almost all plasmacytoid urothelial carcinomas, is negative in signet-ring cell adenocarcinoma from the stomach. Plasmacytoid urothelial carcinoma is a very aggressive variant of urothelial carcinoma with a predilection for spreading within the peritoneal cavity.
