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Presented by Jonathan Epstein, M.D. and prepared by Julie M. Wu, M.D.
Case 5: Fifteen months ago, a 5 year old boy presented with hematuria and ultrasonography showing a papillary mass in the bladder.
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1. Question
Week 349: Case 5
Fifteen months ago, a 5 year old boy presented with hematuria and ultrasonography showing a papillary mass in the bladder. The patient underwent resection where the diagnosis at an outside institution was “papilloma”. Hematuria recurred and now there was a more solid irregular mass within the bladder measuring 18 x 6 mm. On review of the original lesion signed out as papilloma it was my opinion that it was a low grade noninvasive papillary urothelial carcinomaimages/jmw051908/5.1.jpg
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images/jmw051908/5.5.jpgCorrect
Answer: High grade papillary urothelial carcinoma
Histology: The lesion consists of well formed papillary fronds seen at low magnification. Some of the fronds have dilated edematous cores. At higher magnification some of the papillae are lined by cells with minimal cytologic atypia. Others show loss of polarity, moderate to marked pleomorphism, and discohesion. Numerous mitotic figures are not identified.
Discussion: Despite edema within some of the papillary fronds, this lesion has too much cytologic atypia and none of the other inflammatory elements of polypoid cystitis such that this is a papillary urothelial neoplasm. Although the fronds appear discrete suggestive of a low grade neoplasm at scanning magnification, at higher magnification the lesion has the cytological features of a high grade papillary urothelial carcinoma. This includes loss of polarity where cells are irregularly distributed within the urothelium. In addition cells are loosely cohesive with some cells showing marked pleomorphism. If these same cells were in a flat urothelial lining of an adult, one would diagnose the degree of atypia as being consistent with CIS. This degree of atypia is compatible with a high grade papillary urothelial carcinoma. Papillary urothelial neoplasms are rare in children of this age. In a study we performed of urothelial neoplasms in patients younger than 20 the youngest patient was 4 years old (Am J Surg Pathol 174: 1976-80, 2005). The majority of papillary urothelial neoplasms in young patients tend to be low grade including urothelial papillomas, papillary urothelial neoplasms of low malignant potential and low grade carcinomas. In our series of 23 papillary tumors occurring in young patients there were only 3 high grade papillary urothelial carcinomas. In our series there were no associated risk factors either for the patient or from the patients’ family that would explain why these young individuals developed urothelial tumors.
Incorrect
Answer: High grade papillary urothelial carcinoma
Histology: The lesion consists of well formed papillary fronds seen at low magnification. Some of the fronds have dilated edematous cores. At higher magnification some of the papillae are lined by cells with minimal cytologic atypia. Others show loss of polarity, moderate to marked pleomorphism, and discohesion. Numerous mitotic figures are not identified.
Discussion: Despite edema within some of the papillary fronds, this lesion has too much cytologic atypia and none of the other inflammatory elements of polypoid cystitis such that this is a papillary urothelial neoplasm. Although the fronds appear discrete suggestive of a low grade neoplasm at scanning magnification, at higher magnification the lesion has the cytological features of a high grade papillary urothelial carcinoma. This includes loss of polarity where cells are irregularly distributed within the urothelium. In addition cells are loosely cohesive with some cells showing marked pleomorphism. If these same cells were in a flat urothelial lining of an adult, one would diagnose the degree of atypia as being consistent with CIS. This degree of atypia is compatible with a high grade papillary urothelial carcinoma. Papillary urothelial neoplasms are rare in children of this age. In a study we performed of urothelial neoplasms in patients younger than 20 the youngest patient was 4 years old (Am J Surg Pathol 174: 1976-80, 2005). The majority of papillary urothelial neoplasms in young patients tend to be low grade including urothelial papillomas, papillary urothelial neoplasms of low malignant potential and low grade carcinomas. In our series of 23 papillary tumors occurring in young patients there were only 3 high grade papillary urothelial carcinomas. In our series there were no associated risk factors either for the patient or from the patients’ family that would explain why these young individuals developed urothelial tumors.