Pagetoid Lesions Associated with Carcinoma of the Bladder

Abstract

Three cases of pagetoid infiltrations by a transitional cell carcinoma are reported. The theories of histogenesis of extramammary Paget's disease are reviewed and discussed. Evidence from these cases suggests that the lesion develops because of the intraepithelial migration of cells.

The pagetoid variant of bladder urothelial carcinoma in situ

“…The age, sex, and race of patients with and without pagetoid changes are variable, but the progression and survival rates of these patients were similar to the reported series [20]. Most authors agreed that pagetoid changes should not be labeled as urothelial dysplasia, and cases misdiagnosed as dysplasia that remain untreated are at higher risk for recurrence and progression [3, 8,26] Pagetoid changes at extramammary sites (EMPD) are rare alterations most often recorded in the anogenital region [1,3,4,7,9,11,21,23,24,26,27]. Cells comprising EMPD may not be associated with carcinomas in adjacent sites [24].…”

“…In addition, its recognition in a small biopsy specimen can alert the pathologist to the presence of a conventional urothelial CIS elsewhere in the bladder, even in cases with extensive bladder denudation in which the lesion may be present in normal-looking urothelium [5]. Some cases of pagetoid CIS may extend to the urethra, ureter, and beyond to the external genitalia where they represent an important differential diagnostic problem, given that primary extramammary Paget disease (EMPD) of the external genitalia may also extend to the bladder [1,3,4,5,7,9,11,21,23,24,26,27]. An appropriate approach to differential diagnosis in this case, may need immunohistochemical analysis in addition to a complete patient's work-up.…”

Paget's Disease of the Gians penis: An Unusual Urological Malignancy

“…Originally local spread via an intra-epithelial route seemed unlikely as our patient had had a total urethrectomy as a primary procedure; however, the re sected urethral specimen had TCC within it and spread could have occurred, with the tumour cells being dormant for 3 years. This long latent period before any sign of the Paget's lesion appears is common to other cases [5,6], TCC with radiotherapy and then cysto-urethrectomy with removal of glans pagetoid lesion; he was found subsequently to have an underlying peri-urethral gland adenocarcinoma, to explain the Paget's lesion Thus, intra-epidermal spread appears a possible explana tion of pagetoid lesions, but we cannot rule out seeding of exfoliated cells. De novo tumour arising in a different epi thelium from the original tumour seems an unlikely prop osition.…”

“…We would suggest that tumour cell migration via the cor pus spongiosum route, occurring due to either direct spread or as a consequence of coughing/straining forcing blood back retrogradely, is a possible explanation for the origin of secondary Paget's disease. Only 6 patients have been reported in the literature with a true pagetoid lesion affecting the glans penis, 4 of which were associated with TCC of the bladder [4][5][6][7], 1 with primary prostatic carcinoma [8] and another with an adenocarcinoma of the peri-urethral gland [9], These lesions have all contained the diagnostic features of a pagetoid lesion, namely large pale cells with pale cyto plasm, large nuclei and a priminent nucleolus. Of the 6 reported cases, 3 people died from metastatic disease within 6 months of the development of the lesion on the glans penis and 2 within a year.…”

“…The anogenital region is typically involved, but the disease has been reported to occur in other areas including the axilla [1], eyelid [2] and external auditory canal [3], Paget's disease of the genital mucosa in association with carcinoma of the lower urinary tract is well documented. However, to our knowledge, only 4 cases of intra-epithelial spread onto the glans penis from transitional cell car cinoma (TCC) of the bladder have been reported in the literature [4][5][6][7], a further associated with primary carci noma of the prostate [8] and another with adenocarcino ma of a peri-urethral gland [9], We present a further case of Paget's disease of the glans penis with an underlying TCC of both bladder and urethra and discuss the clinical significance of this condition.…”