Cases Of Vulvar Paget's Disease Research Articles

Vulvar Paget disease secondary to high-grade urothelial carcinoma with underlying massive vascular embolization and cervical involvement: case report of unusual presentation

BackgroundVulvar extramammary Paget disease is a rare chronic condition, that presents with non-specific symptoms such as pruritus and eczematous lesions. Because most of these lesions are noninvasive, the distinction between primary and secondary Paget disease is crucial to management.Case presentationWe report an unusual case of vulvar Paget disease associated with massive dermal vascular embolization, cervicovaginal involvement and metastasis to inguinal and retroperitoneal lymph nodes. The intraepithelial vulvar lesion had a classical appearance and was accompanied by extensive component of dermal lymphovascular tumor emboli, similar to those observed in inflammatory breast carcinoma. Immunohistochemical analysis revealed that the lesion was secondary to high-grade urothelial cell carcinoma. The patient had a history of superficial low-grade papillary urothelial carcinoma of the bladder, which had appeared 2 years before the onset of vulvar symptoms.ConclusionsEczematoid vulvar lesions merit careful clinical examination and biopsy, including vulva mapping and immunohistochemistry. The information obtained may help to define and classify a particular presentation of Paget disease. Noninvasive primary lesions do not require the same aggressive approaches required for the treatment of invasive and secondary disease.

Primary vulvar Paget disease – the importance of clinical suspicion

Extramammary Paget disease of the vulva is a rare condition that accounts for only 1-2% of vulvar malignancies and represents a frequent cause of misdiagnosis. It is most commonly seen in postmenopausal women. Clinically it is similar to Paget disease of the breast, appearing as red, well-demarcated eczematoid lesions, with slightly raised edges. A high degree of clinical suspicion is very important when evaluating these lesions in order to avoid misdiagnosis and delay of effective treatment. We present a case of vulvar Paget disease treated with vulvectomy.

Immunohistochemistry of p63 in primary and secondary vulvar Paget's disease

Vulvar Paget's disease (VPD) is classified into primary and secondary types. Differentiation of these subsets in biopsy specimen is important for appropriate therapy. Expression profile of cytokeratin (CK) 7 and CK20, gross cystic disease fluid protein-15 and uroplakin III has been reported as a differentiation marker of primary and secondary VPD. To examine the role of p63 immunostaining in differential diagnosis between primary VPD and VPD secondary to urothelial carcinoma (VPD-UC), expression of p63 was examined in nine cases of VPD. Paget cells in seven cases of VPD without UC did not express p63. In two cases of VPD associated with UC, Paget cells and UC cells had identical CK expression profile. UC cells were positive for p63 in both cases. In one case, Paget cells were positive for p63 and examination of the resected specimen showed that VPD was secondary to UC. In another case, Paget cells were negative for p63 and this was diagnosed as primary VPD independent of UC. This indicates that p63 is absent in Paget cells in primary VPD and is therefore useful in differentiating primary VPD from VPD-UC.