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Genital Paget's disease in a man
Walter Krause1, Andreas Krisp, Stefan Hörster
1Department of Andrology and Venerology, University Hospital, Philipp University, Deutschhausstr. 9, D-35033 Marburg, Germany. krause@med.uni-marburg.de
Abstract:
Pagetoid cells are large intraepidermal cells which spread intraepidermally. We report a 67-year old Caucasian male, who presented for the first time in 1993 with a long-standing pruritic lesion at the scrotum. He was treated for several years by antiinflammatory ointments. Only in July 2003 was a biopsy taken for the first time. The histopathological evaluation revealed the diagnosis of an extramammary Paget's disease (EMPD). Pagetoid cells are large intraepidermal cells with a large nucleus and ample cytoplasm. EMPD consists of primary malignant cells of epidermal origin, but in rare cases, pagetoid cells may also originate from carcinomas with epidermotropic growth. EMPD is a slowly progressing disease, but invading and metastasing tumors may also develop. Considering the good prognosis with long-term survival, nonsurgical modalities should be considered as primary treatment for noninvasive EMPD.
Insights
Extramammary Paget's disease (EMPD) is a rare intraepidermal malignancy. Early diagnosis and considering non-surgical options for noninvasive EMPD can lead to good long-term survival.
Area of Science:
- Dermatology
- Oncology
- Pathology
Background:
- Extramammary Paget's disease (EMPD) is a rare intraepidermal malignancy characterized by the proliferation of Pagetoid cells.
- Pagetoid cells are large intraepidermal cells with distinct morphological features.
Observation:
- A 67-year-old male presented with a long-standing pruritic scrotal lesion treated initially with anti-inflammatory ointments.
- A biopsy performed a decade after initial presentation confirmed the diagnosis of extramammary Paget's disease (EMPD).
Findings:
- Histopathological evaluation revealed EMPD, originating from primary malignant cells of epidermal origin.
- While EMPD is typically slow-progressing, invasive and metastatic potential exists.
Implications:
- Nonsurgical treatment modalities should be considered for noninvasive EMPD, given the generally good prognosis and potential for long-term survival.
- This case highlights the importance of timely biopsy for accurate diagnosis and appropriate management of suspected EMPD.
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