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

Diagnostic Pathology

Walquiria Quida Salles Pereira Primo¹  Filomena M. Carvalho²  Dunya Bachour Basilio³  Guttenberg Rodrigues Pereira Primo⁴  Jesus Paula Carvalho⁵  Karime Kalil Machado⁶ 

[1] Department of Gynecology, Faculdade de Medicina, Universidade de Brasília;Department of Pathology, Faculdade de Medicina FMUSP, Universidade de São Paulo;Department of Pathology, Instituto Hospital de Base do Distrito Federal IHBDF, Secretaria de Estado da Saude;Gynecology Service, Hospital Regional da Asa Norte (HRAN), Secretaria de Estado da Saude;Instituto do Cancer do Estado de São Paulo ICESP, Faculdade de Medicina, Universidade de São Paulo;Medical Oncology Service, Hospital Sírio Libanês;

关键词: Extramammary Paget disease;    Urothelial carcinoma;    Vulva;    Lymphatic embolization;   

DOI  :  10.1186/s13000-019-0895-0

来源: DOAJ

【 摘 要 】

Abstract Background Vulvar 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 presentation We 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. Conclusions Eczematoid 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.

【 授权许可】

Unknown