Guelmim Faculty of Medicine and Pharmacy, Ibn Zohr Agadir University, Agadir, Morocco.
Al AMAL Pathological Anatomy Laboratory, Guelmim, Morocco.
J Med Case Rep. 2024 Nov 29;18(1):579. doi: 10.1186/s13256-024-04927-z.
Syringocystadenocarcinoma papilliferum is an extremely rare malignant adnexal tumor that typically arises from a papilliferous syringocystadenoma (World Health Organization classification of skin tumors, 2018.). This tumor predominantly occurs in the cephalic region.
We present the case of a 68-year-old Moroccan male with no significant medical history who presented with a slowly progressing skin mass in the perianal region. Despite the chronic nature of the swelling, the patient remained in overall good health. Physical examination revealed a firm 2.3-cm mass in the perianal area, with no other remarkable findings. The patient underwent surgical excision of the mass. Macroscopic examination showed a solid-cystic, rounded mass. Microscopic examination revealed a malignant adnexal tumor with apocrine differentiation and papillary architecture. Immunohistochemistry was positive for p63, cytokeratin 7 (CK7), and smooth muscle actin, with a Ki67 labeling index in 60% of tumor cells, and negative for p16. The diagnosis of syringocystadenocarcinoma papilliferum was confirmed. A further surgical resection was performed, and the patient's postoperative course was unremarkable.
Syringocystadenocarcinoma papilliferum is an extremely rare tumor, with its occurrence in the perianal region being particularly uncommon. This case contributes to the limited literature on this malignancy, highlighting its clinical and pathological features.
汗管囊腺癌是一种极其罕见的恶性附属器肿瘤，通常起源于乳头状汗管囊腺瘤（2018 年世界卫生组织皮肤肿瘤分类）。这种肿瘤主要发生在头部。
我们报告了一例 68 岁的摩洛哥男性病例，无明显病史，表现为肛周进行性皮肤肿块。尽管肿胀呈慢性，但患者总体健康状况良好。体格检查显示肛周有一个 2.3 厘米的坚实肿块，无其他明显发现。患者接受了肿块的手术切除。大体检查显示为实性-囊性、圆形肿块。显微镜检查显示为具有大汗腺分化和乳头状结构的恶性附属器肿瘤。免疫组织化学染色 p63、细胞角蛋白 7（CK7）和平滑肌肌动蛋白阳性，肿瘤细胞中有 60%的 Ki67 标记指数，p16 阴性。确诊为汗管囊腺癌。进一步进行了手术切除，患者的术后过程无异常。
汗管囊腺癌是一种非常罕见的肿瘤，发生在肛周的情况尤为罕见。本病例增加了对这种恶性肿瘤的有限文献的了解，突出了其临床和病理特征。
