Track

Case Reports

Abstract

Primary cutaneous apocrine carcinoma (PCAC) is an exceptionally rare cutaneous malignancy originating from apocrine glands, occurring most commonly in the axilla and anogenital regions. Despite its infrequency, PCAC presents a diagnostic challenge owing to its nonspecific histopathological and immunohistochemical profile. Intriguingly, a number of cases of PCAC have been reported as extramammary Paget’s disease (EMPD), wherein single epithelial cells spread in a pagetoid pattern throughout all layers of the epidermis, adding to its diagnostic complexity. Its ability to mimic metastatic carcinomas from various organs necessitates thorough clinical and pathological correlation for an accurate diagnosis. While local recurrence and regional metastasis are not uncommon in PCAC, instances of distant metastases are rare, with only a handful of cases reported, including involvement of the liver, bone, and lung. This case report aims to illuminate the potentially aggressive behavior of this perplexing malignancy. To the best of our knowledge, this is the first reported case of PCAC with pagetoid features originating from the groin and metastasizing to the esophagus, highlighting the need for increased awareness in managing this unpredictable condition.