Kim Jee Woo, Shin Jung Won, Huh Chang-Hun
Department of Dermatology, Seoul National University Bundang Hospital, Seongnam, Korea.
Ann Dermatol. 2023 May;35(Suppl 1):S4-S9. doi: 10.5021/ad.21.039.
Nevus sebaceus is a hamartomatous lesion characterized by epidermal, follicular, sebaceus, and apocrine gland abnormalities. Approximately 25% of affected individuals may develop benign or malignant secondary neoplasms within the preceding nevus sebaceus. Primary cutaneous apocrine carcinoma (PCAC) is a rare malignant skin tumor affecting elderly adults in their sixth decade of life. Histologically, PCAC appears as a dermal tumor displaying apocrine differentiation with decapitation secretion and malignant features. Secondary malignancy arising from nevus sebaceus is a rare complication, especially for apocrine carcinoma. To date, approximately 200 cases of PCAC have been reported in the literature, and only a few cases have developed PCAC on the scalp. Very few cases (approximately only 12) of PCACs developing in nevus sebaceus have been reported. Here, we report an extremely rare case of the coexistence of PCAC and syringocystadenoma papilliferum arising within nevus sebaceus of the scalp.
皮脂腺痣是一种错构瘤性病变，其特征为表皮、毛囊、皮脂腺和大汗腺异常。约25%的受累个体可能在先前的皮脂腺痣内发生良性或恶性继发性肿瘤。原发性皮肤大汗腺癌（PCAC）是一种罕见的恶性皮肤肿瘤，好发于60岁左右的老年人。组织学上，PCAC表现为具有断头分泌和恶性特征的大汗腺分化的真皮肿瘤。皮脂腺痣继发恶性肿瘤是一种罕见的并发症，尤其是大汗腺癌。迄今为止，文献报道了约200例PCAC，仅有少数病例发生于头皮。在皮脂腺痣中发生PCAC的病例报道极少（约仅12例）。在此，我们报告1例极其罕见的头皮皮脂腺痣内同时存在PCAC和乳头状汗管囊腺瘤的病例。
