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Extramammary Paget's disease with distant skin metastasis
1Department of Dermatology, National Sapporo Hospital, Japan.
The Journal of Dermatology
|June 1, 1996
Summary
This study details a rare case of invasive extramammary Paget's disease (EPD) in a Japanese man, presenting with extensive local spread and distant metastases, including a rare scalp metastasis.
Area of Science:
- Oncology
- Dermatology
- Pathology
Background:
- Extramammary Paget's disease (EPD) is a rare malignancy typically affecting the anogenital region.
- Invasive EPD can exhibit aggressive behavior and metastasis, though cutaneous metastasis is uncommon.
Observation:
- A 68-year-old Japanese man presented with invasive EPD originating in the inguinal area, spreading to the scrotum, penis, and pubic region, causing significant lymphedema.
- Histopathology confirmed intraepidermal and intradermal Paget cells, with dissemination to lymph nodes, lungs, bones, and the adrenal gland.
- A rare metastatic cutaneous nodule on the scalp was observed, with Paget cells confined to the dermis.
Findings:
- The patient exhibited extensive local EPD with regional and systemic lymph node involvement.
- Distant metastases included lungs, bones, and the left adrenal gland.
- A unique scalp metastasis, confined to the dermis, was documented, highlighting an unusual pattern of EPD spread.
Implications:
- This case underscores the potential for aggressive local and distant spread of invasive EPD.
- The rare occurrence of scalp metastasis provides insights into the diverse metastatic pathways of EPD.
- Further research into the mechanisms and management of rare metastatic patterns in EPD is warranted.