Paget's Disease of the Vulva: Pathology, Pattern of Involvement, and Prognosis
ABSTRACT The aim of this study was to determine prognostic factors and risk factors for recurrence in patients with Paget's disease of the vulva.
The medical records of 76 patients with a diagnosis of Paget's disease of the vulva were retrospectively reviewed. The diagnosis in each case was confirmed by reviewing the pathology. Patients were then divided into four groups by diagnosis: intraepithelial Paget's disease (IEP) (n = 46), invasive Paget's disease (IP) (n = 9), intraepithelial Paget's disease with underlying adenocarcinoma (IEPUA) (n = 13), and intraepithelial Paget's disease with a coexisting cancer (CCA) (n = 8). Comorbid conditions, location of disease, pathologic diagnosis, method of treatment, margin status, and current status of the patient were evaluated. Descriptive statistical data and univariate analysis were generated using the Statview statistical package.
A diagnosis of IEPUA, IP, or CCA predicted a poor survival (P = 0. 0017). Patients who had received chemotherapy or radiation as treatment had a poor survival (P < 0.0001 and 0.0002). Patients with clitoral Paget's disease had a higher incidence of death from disease (P = 0.026). When death from all causes was considered, patients treated with wide local excision (WLE) had a significantly longer survival than patients treated with other more radical treatments (P = 0.02). Risk factors for recurrence included treatment with WLE (P = 0.004).
Patients with IP, IEPUA, or CCA have a poorer prognosis than patients with IEP. Location of Paget's disease is important for prognosis; and patients with clitoral Paget's disease may require more aggressive treatment. WLE is associated with a higher risk of recurrence, but overall patients with WLE tend to survive longer than patients treated more radically.
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ABSTRACT: Artículo original Patología 2011;49(1):11-24 RESUMEN Las neoplasias malignas de anexos cutáneos son poco frecuentes; sin embargo, es importante conocerlas porque muchas son de evolución rápida y mortal, pero su diagnóstico puede representar para el patólogo general alto grado de dificultad debido a su gran similitud con su contraparte benigna y también porque el diagnóstico diferencial entre ellas mismas y con algunas neoplasias metastásicas (glándula salival y mama) puede ser problemático. No hay ningún marcador clínico que nos ayude al diagnóstico, aunque generalmente son mayores de 2.5 cm. En la histopatología lo más relevante es la silueta irregular y asimétrica; las células neoplásicas pueden exhibir cambios atípicos leves hasta polimorfismo acentuado; generalmente las mitosis atípicas son escasas (una por campo de 40 X); la necrosis comedónica, invasión neural, vascular y linfática son frecuentes. La inmunohistoquímica es de utilidad sólo en algunos casos. El tratamiento de elección es la resección quirúrgica amplia, con disección ganglionar regional electiva. Palabras clave: neoplasias malignas, anexos cutáneos. ABSTRACT Malignant cutaneous adnexal neoplasms are rare; however, it is important to know them because some are rapidly progressive and fatal, and their diagnosis is one of the most challenging tasks for the general pathologist due to its great similarity with its benign counterpart and also because the differential diagnosis between themselves and some metastatic tumors (salivary gland and breast) may be problematic. There is no clinical findings that help us to their diagnosis, although they generally are grater than 2.5 cm. Histopathologically, the irregular and asymmetric shape is the most relevant finding; the neoplastic cells may exhibit mild to atypical changes, accentuated polymorphism; generally atypical mitosis are rare (one per field of 40 X); necrosis, lymphatic, vascular and neural invasions are frequent. The IHQ is useful only in some cases. Treatment of choice is wide surgical resection with elective lymph node dissection.
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ABSTRACT: Extramammary Paget disease (EMPD) is often associated with underlying or distant synchronous malignancies. The prognosis for affected patients is generally favourable; however, the risk of secondary malignancies is unknown. The goal of the study was to analyse the incidence, prognosis and pattern of secondary malignancies for patients with invasive EMPD using data from the Surveillance, Epidemiology and End Results (SEER) Program. We searched the SEER Program database for patients diagnosed with invasive EMPD between 1973 and 2008. Demographic data, outcome and secondary malignancies more than 1 year after the initial diagnosis of invasive EMPD were included in the analysis. We calculated the standardized incidence ratio (SIR) and estimated the excess absolute risk (EAR) per 10 000 person-years (PY). There were 1439 patients who were diagnosed with invasive EMPD. Most patients (80·4%) had localized disease, while 17·1% had locoregional spread and 2·5% presented with distant disease. The SIR for secondary malignancies in patients with invasive EMPD was significantly elevated with an EAR of 97·4 additional malignancies per 10 000 PY. The excess risk was mostly due to a significantly increased incidence of colorectal and anal malignancies. The initial site of disease predicted the site of the secondary malignancies, with patients with colorectal, anal, vulvar and scrotal disease showing an increased risk of colorectal, anal, vulvar and scrotal malignancies, respectively. Our study identified a long-term increased risk of developing secondary malignancies in patients with invasive EMPD that are mainly related to the site of origin of this disease. Patients with invasive EMPD require prolonged follow-up and screening for these malignancies.British Journal of Dermatology 03/2014; 170(3):661-71. · 3.76 Impact Factor
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ABSTRACT: Extramammary Paget's disease (EPD) is a malignant disease observed, especially in areas with high density of apocrine glands. We report a case of a patient of 68 years old, attended at the Colposcopy Clinic of Oncology, National Institute of Perinatology Isidro Espinosa de los Reyes, diagnosed with Paget's disease of the vulva, vagina, perineum region and perianal region, and managed with laser vaporization sessions with satisfactory outcome. Since it is a rare disease, more common in females and in whites, with the highest incidence in the seventh decade of life, as is the case of our patient, propose in this paper to present a review of the clinical evaluation with emphasis on the differential diagnosis, use of colposcopy for diagnosis and dividing up conservative treatment with laser vaporization of the disease. The low incidence of Paget's disease of the vulva, vagina, perineum and perianal region, and the absence of reports on the possibility of nonsurgical treatment encouraged to inform the scientific community about the management with laser vaporization, with the intention of avoiding morbidity that can lead to the conventional surgical maneuvers.Perinatología y reproducción humana / INPer 03/2013; 27(1):44-50.