Invasive vulvar extramammary Paget's disease in the United States. 2020

Toni P Kilts, and Beverly Long, and Amy E Glasgow, and Jamie N Bakkum-Gamez, and Elizabeth B Habermann, and William A Cliby
Division of Gynecologic Oncology, Mayo Clinic, Rochester, MN, USA.

To assess the incidence, treatment, and outcomes in patients with invasive vulvar extramammary Paget's disease (EMPD) in a national cohort of patients. Patients from the Surveillance, Epidemiology and End Results (SEER) database with diagnoses of vulvar EMPD from 1992 to 2016 were included. Demographic, treatment, and outcome data were analyzed. A total of 1268 cases of invasive EMPD were identified. Of those, 69.6% had localized disease, 12.0% regional disease, 1.3% distant disease, and 17.1% were unstaged. The annual incidence of invasive vulvar EMPD was 0.36 per 100,000 person years: rates have increased >2-fold since 1992 (1992: 0.19 per 100,000 person years to 0.50 per 100,000 person years in 2016). Most patients underwent primary surgery (n = 1034; 81.5%). Five-year cancer specific survival (CSS) was 95.5% and was associated with stage. Compared to patients with localized disease, patients with distant metastases had dramatically worse CSS (HR: 85.8 (31.8-248) p < 0.0001). Synchronous cancers (diagnosed within one calendar year of EMPD diagnosis year) were observed in 35 cases (2.8%), and 195 patients (15.4%) developed a secondary malignancy (diagnosed >one year from year of EMPD diagnosis year). The most common synchronous breast, gastrointestinal tract, melanoma and the most common secondary cancers were breast, gastrointestinal tract and genitourinary tract. The incidence of invasive vulvar EMPD has increased over time. CSS is excellent for localized disease, but those with metastatic disease are in need of novel therapies. Approximately 15% will develop a secondary malignancy, indicating that patients with invasive vulvar EMPD should undergo site specific preventative health screens during recurrence surveillance.

UI MeSH Term Description Entries
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010145 Paget Disease, Extramammary A rare cutaneous neoplasm that occurs in the elderly. It develops more frequently in women and predominantly involves apocrine gland-bearing areas, especially the vulva, scrotum, and perianal areas. The lesions develop as erythematous scaly patches that progress to crusted, pruritic, erythematous plaques. The clinical differential diagnosis includes squamous cell carcinoma in situ and superficial fungal infection. It is generally thought to be an adenocarcinoma of the epidermis, from which it extends into the contiguous epithelium of hair follicles and eccrine sweat ducts. (DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1478) Paget Disease Extramammary,Paget's Disease, Extramammary,Extra-Mammary Paget Disease,Extra-Mammary Paget's Disease,Extramammary Paget Disease,Extramammary Paget's Disease,Paget Disease, Extra-Mammary,Paget's Disease, Extra-Mammary,Extra Mammary Paget Disease,Extra Mammary Paget's Disease,Extra-Mammary Pagets Disease,Extramammary Pagets Disease,Extramammary, Paget Disease,Paget Disease, Extra Mammary,Paget's Disease, Extra Mammary,Pagets Disease, Extra-Mammary,Pagets Disease, Extramammary
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D000369 Aged, 80 and over Persons 80 years of age and older. Oldest Old
D014481 United States A country in NORTH AMERICA between CANADA and MEXICO.
D014844 Vulva The external genitalia of the female. It includes the CLITORIS, the labia, the vestibule, and its glands. Vulvas
D014846 Vulvar Neoplasms Tumors or cancer of the VULVA. Cancer of Vulva,Vulvar Cancer,Cancer of the Vulva,Neoplasms, Vulvar,Vulva Cancer,Vulva Neoplasms,Cancer, Vulva,Cancer, Vulvar,Cancers, Vulva,Cancers, Vulvar,Neoplasm, Vulva,Neoplasm, Vulvar,Neoplasms, Vulva,Vulva Cancers,Vulva Neoplasm,Vulvar Cancers,Vulvar Neoplasm

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