Article

Paget's disease of the vulva: pathology, pattern of involvement, and prognosis.

Department of Gynecologic Oncology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA.
Gynecologic Oncology (impact factor: 3.89). 04/2000; 77(1):183-9. DOI:10.1006/gyno.2000.5741 pp.183-9
Source: PubMed

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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    Article: Extramammary Paget's Disease: 20 Years of Experience in Chinese Population.
    Jimmy Yu Wai Chan, George Kam Hop Li, Joseph Hon Ping Chung, Velda Ling Yu Chow
    [show abstract] [hide abstract]
    ABSTRACT: Background. To examine the results of treatment of Extramammary Paget's disease (EMPD) in ethnic Chinese. Method. Between 1990 and 2010, patients treated for EMPD were reviewed. Data were analyzed retrospectively. Results. Forty-eight patients were treated by surgical resection. Local recurrence rate was 14.6%. The postresection defects were repaired by primary closure (8.3%), partial thickness skin graft (72.9%), or local/regional flaps (18.8%). Dermal invasion was found in 9 patients (18.8%). Seven patients (14.6%) developed regional lymph node metastasis (concurrent with surgery, n = 1; subsequent to surgery, n = 6), and 3 patients (6.3%) had systemic metastasis after surgery. The presence of dermal invasion was associated with significantly higher incidence of regional lymph nodes and systemic metastasis. The incidence of associated internal malignancy was 8.3%. Conclusion. The mainstay of treatment for EMPD is surgery. Pathological dermal invasion increases the chance of regional lymph node as well as systemic metastasis. The association with internal malignancy warrants preoperative endoscopic examination in all patients.
    International journal of surgical oncology. 01/2012; 2012:416418.
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Keywords

76 patients
 
clitoral Paget's disease
 
Comorbid conditions
 
current status
 
Descriptive statistical data
 
higher risk
 
intraepithelial Paget's disease
 
invasive Paget's disease
 
margin status
 
medical records
 
Paget's disease
 
pathologic diagnosis
 
Patients
 
poor survival
 
poorer prognosis
 
radical treatments
 
radically
 
Statview statistical package
 
univariate analysis
 
wide local excision
 

L P Parker