Poorly differentiated cutaneous apocrine carcinomas: histopathological clues and immunohistochemical analysis for the diagnosis of this unusual neoplasm

Primary cutaneous apocrine carcinoma (PCAC) is a rare cutaneous malignancy that is derived from apocrine glands. Histologically, these tumours can appear well-differentiated where diagnosis should be relatively straightforward. However, occasionally these tumours can exhibit high-grade features, and in such instances the diagnosis can be challenging. A retrospective analysis of 12 cases of poorly differentiated PCAC, obtained from large academic institutions, was performed, and summarised below. Immunohistochemical studies were performed in all cases with antibodies against CK7, p63, CAM 5.2, GCDFP-15, GATA3, CEA, PR, ER, HER2, calponin, SMA, androgen receptor and EMA. All 12 cases were poorly differentiated; however, there were some histopathological clues to the diagnosis of apocrine carcinoma; namely, the presence of focal glandular formation, acrosyringial involvement and the presence of single ‘pagetoid’ cells within epidermis. All tumours were consistently positive for CK7, GATA3 and GCDFP-15 and negative for p63. The tumours had variable expression of CAM5.2, CEA, ER, PR, HER2, androgen receptor and EMA. In three cases, there was a preservation of the myoepithelial cell layer (with calponin and SMA), which also confirmed the primary cutaneous origin. PCAC is a difficult neoplasm to diagnose, as it can appear identical to metastatic carcinomas. We describe 12 cases of poorly differentiated PCAC, highlighting their salient clinical, histopathological and immunohistochemical features, and discuss the potential diagnostic pitfalls in distinguishing this entity from other malignant neoplasms. Our results indicate that a combination of thorough histological inspection coupled with an adequate battery of immunohistochemical stains is necessary to support the diagnosis of PCAC.