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Metastatic Extramammary Paget Disease of the Perianal Region.

A 72-year-old man with history of itching for 8 months presented with an ill-defined, ulcerated, eczematous, thickened cutaneous lesion in the left perianal region. F-FDG PET/CT was performed, and neoplastic skin thickening with FDG uptake was seen at the left gluteal cleft. Hypermetabolic inguinal, retroperitoneal, and mediastinal lymph nodes, liver, and bone metastases were seen. Histopathology and immunohistochemistry of the perianal growth and left inguinal lymph node were positive for cytokeratin7 and gross cystic disease fluid protein and negative for cytokeratin 20 and CDX2, confirming the diagnosis of primary extramammary Paget disease with metastases.