Kantrow Sara M, Ivan Doina, Williams Michelle D, Prieto Victor G, Lazar Alexander J
University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
Am J Dermatopathol. 2007 Oct;29(5):462-6. doi: 10.1097/DAD.0b013e31814a50c6.
Nevus sebaceus of Jadassohn is a hamartoma of multiple skin structures. Many neoplasms have been reported to arise in association with nevus sebaceus, most commonly trichoblastoma/basal cell carcinoma and syringocystadenoma papilliferum. We report a case of a 66-year-old woman with an adenocarcinoma as well as multiple neoplastic proliferations arising in a long standing nevus sebaceus on the scalp, with subsequent occipital neck metastatic disease. On histologic evaluation, the epidermis showed changes reminiscent of tumor of the follicular infundibulum as well as basaloid proliferations resembling superficial trichoblastoma. A focus suggestive of syringofibroadenoma was also present. A small dermal collection of basaloid and more mature sebocytes was consistent with a sebaceoma/sebaceous epithelioma. Most of the lesion was composed of an adenocarcinoma with areas showing ductal differentiation with decapitation secretion, well-formed papillae and focal cribriform structures. Other portions demonstrated a high-grade neoplasm with prominent nuclear atypia and a solid pattern of growth resembling high-grade breast carcinoma. Anti-epithelial membrane antigen strongly labeled tumor cells and highlighted ductal structures. Less than 1% of cells expressed progesterone or estrogen receptors. Her2/neu reactivity was focally present, showing 1+ membranous reactivity in 10% of cells. Anti-p63 labeled basaloid cells surrounding the tumor lobules. A breast primary was ruled out by clinical and radiologic examination. This report illustrates an extraordinary case of adnexal neoplasia displaying various lines of differentiation arising in association with nevus sebaceus.
Jadassohn皮脂腺痣是一种多种皮肤结构的错构瘤。据报道，许多肿瘤与皮脂腺痣相关，最常见的是毛母细胞瘤/基底细胞癌和乳头状汗管囊腺瘤。我们报告一例66岁女性病例，其头皮上长期存在的皮脂腺痣发生腺癌以及多种肿瘤性增殖，并伴有枕颈部转移瘤。组织学评估显示，表皮呈现出类似毛囊漏斗部肿瘤的变化以及类似浅表毛母细胞瘤的基底样增殖。还存在一个提示汗腺纤维腺瘤的病灶。真皮内一小群基底样细胞和更成熟的皮脂腺细胞符合皮脂腺瘤/皮脂腺上皮瘤。病变大部分由腺癌组成，部分区域显示导管分化伴断头分泌、形成良好的乳头和局灶筛状结构。其他部分表现为高级别肿瘤，具有明显的核异型性和类似于高级别乳腺癌的实体生长模式。抗上皮膜抗原强烈标记肿瘤细胞并突出显示导管结构。不到1%的细胞表达孕激素或雌激素受体。Her2/neu反应呈局灶性，10%的细胞显示1+膜反应。抗p63标记肿瘤小叶周围的基底样细胞。临床和放射学检查排除了乳腺原发肿瘤。本报告阐述了一例与皮脂腺痣相关的显示多种分化谱系的附件肿瘤的特殊病例。
