Invasive vulvar extramammary Paget's disease in the United States.
Gynecol Oncol
; 157(3): 649-655, 2020 06.
Article
en En
| MEDLINE
| ID: mdl-32268952
ABSTRACT
OBJECTIVE:
To assess the incidence, treatment, and outcomes in patients with invasive vulvar extramammary Paget's disease (EMPD) in a national cohort of patients.METHODS:
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.RESULTS:
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.CONCLUSIONS:
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.
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Vulva
/
Neoplasias de la Vulva
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Enfermedad de Paget Extramamaria
Tipo de estudio:
Prognostic_studies
Límite:
Adult
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Aged
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Aged80
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Female
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Humans
/
Middle aged
País/Región como asunto:
America do norte
Idioma:
En
Revista:
Gynecol Oncol
Año:
2020
Tipo del documento:
Article
País de afiliación:
Estados Unidos