Primary cutaneous apocrine carcinoma of scrotum with pagetoid phenomenon

Primary cutaneous apocrine carcinoma (PCAC) of the scrotum is an unwonted neoplasm that predominantly occurs in areas with high-density apocrine glands. However, the incidence of scrotal PCAC is uncommon. Further, the pagetoid phenomenon is a rare feature of PCAC involving malignant cells infiltrating the epidermis. We present a rare case of scrotal PCAC in an elderly patient who presented with a slow-growing scrotal lesion. A physical examination identified an ulcerated globular mass at the right scrotum with ipsilateral inguinal lymphadenopathy, and a biopsy confirmed the diagnosis of apocrine carcinoma. In addition, imaging studies revealed features of lymph nodes and bone metastasis. Subsequently, a wide local excision was performed. The clinical features of PCAC are not distinctive; thus, histopathology and immunohistochemistry are paramount to confirm the diagnosis. The ideal treatment to achieve clear margins is tumor excision, while other treatment modalities such as chemotherapy, targeted antibody therapy, and radiotherapy are not established.