The Philippine General Hospital experience with extramammary Paget's disease from 1997 to 2006

ABSTRACT

BACKGROUND: Extramammary Paget's disease (EMPD) is a rare intraepidermal adenocarcinoma seen in areas with high density of apocrine glands such as the vulva, perianal area, scrotal and penile regions. Clinically the lesions appear to be well-defined erythematous or white plaques accompanied by pruritus; often misdiagnosed as inflammatory or infectious conditions i.e. psoriasis, candidiasis or dermatitis. At present, there is no retrospective study on the prevalence of EMPD in the Philippines. OBJECTIVE: This study aimed to conduct a ten-year retrospective review of histologically diagnosed extramammary Paget's disease in the Philippine General Hospital from 1997 to 2006, and confirm the diagnoses by carcinoembryonic antigen immunostain (CEA). METHODS: Histopathology logbooks and organ files of the PGH Section of Dermatology and PGH Department of Pathology from January 1997 to December 2006 were searched. Records and specimen paraffin blocks of EMPD patients were retrieved for review and CEA immunostain, respectively. RESULTS: Nine EMPD patients were seen in a ten-year period, eight were positive for CEA, and one had no paraffin block available for review. The vulva was the most common site (89 percent) followed by the perianal area (11 percent), and all patients were females, aged 47 to 86 years old. The most common complaint was pruritus, often misdiagnosed as fungal infection, contact dermatitis, vulvar carcinoma or malignant melanoma; with one week to seven years before a final diagnosis of EMPD was made. Lesions were mostly erythematous, indurated plaques ranging from 0.48 cm to 299 cm with a mean of 120.67 cm . Malignancy was noted in one patient who had breast cancer prior to appearance of vulvar lesions. Surgical removal of the lesion was the main mode of-treatment. Vulvectomy, wide excision or local excision has been done with surgical flaps or grafts depending on the size of the lesion. CONCLUSION: From 1997 to 2006 the Philippine General Hospital had nine histopathologically diagnosed EMPD, eight of which in the vulvar area and eight had CEA positive staining. Recurrence of lesions and appearance of concomitant malignancy could not be assessed due to the poor follow up of patients and incomplete records.