Kazakov Dmitry V, Calonje Eduardo, Zelger Bernhard, Luzar Bostjan, Belousova Irena E, Mukensnabl Petr, Michal Michal
Sikl's Department of Pathology, Charles University, Medical Faculty Hospital, Pilsen, Czech Republic.
Am J Dermatopathol. 2007 Jun;29(3):242-8. doi: 10.1097/DAD.0b013e3180339528.
The authors report a small series of sebaceous carcinoma developing in nevus sebaceus of Jadassohn (also known as organoid nevus) and analyze similar cases reported in the literature. All of our patients were females (age range 57-71 years; median 60 years) who had the organoid nevus on the scalp, face, or nuchal area, the rest of which was recognized clinically and/or histologically. All sebaceous carcinomas manifested unquestionable architectural (asymmetry, invasive growth) and cytological features of a carcinoma (cellular and nuclear pleomorphism, plentiful atypical mitoses, necroses en masse), demonstrated unambiguous sebaceous differentiation in the form of mature sebocytes, and lacked other differentiations. The sebaceous carcinoma was always accompanied by other benign or malignant adnexal lesions such as sebaceoma, syringocystadenoma papilliferum, syringocystadenocarcinoma papilliferum, trichoblastoma, tricholemmoma, desmoplastic tricholemmoma, or syringoma. In three cases, prominent mucinous metaplasia of sweat ducts and glands was seen. In two of these cases, sweat ducts exhibited hyperplastic changes. The analysis of the previously published material and our cases indicates that sebaceous carcinoma arising in organoid nevus has a female predilection and tends to occur in elderly patients. It may involve any site where nevus sebaceus typically occurs. Clinically, the tumor presents as a solitary nodule, ulcerated tumor, or mass, often with a recent history of rapid growth. It may arise alone, but it occurs more frequently as part of multiple benign and malignant adnexal tumors. The lesion does not seem to be associated with Muir-Torre syndrome. The rest of organoid nevus is usually recognized both clinically and microscopically, although large tumors may overgrow and mask the nevus. The tumor seems to be a low-grade carcinoma in terms of clinical behavior.
作者报告了一小系列发生于 Jadassohn 皮脂腺痣（也称为器官样痣）的皮脂腺癌，并分析了文献中报道的类似病例。我们所有的患者均为女性（年龄范围 57 - 71 岁；中位年龄 60 岁），其器官样痣位于头皮、面部或颈部区域，其余部分通过临床和/或组织学得以确认。所有皮脂腺癌均表现出明确无误的癌的结构特征（不对称性、浸润性生长）和细胞学特征（细胞及核的多形性、大量非典型有丝分裂、大片坏死），以成熟皮脂腺细胞的形式表现出明确的皮脂腺分化，且缺乏其他分化。皮脂腺癌总是伴有其他良性或恶性附属器病变，如皮脂腺瘤、乳头状汗腺囊腺瘤、乳头状汗腺囊腺癌、毛母细胞瘤、外毛根鞘瘤、促结缔组织增生性外毛根鞘瘤或汗管瘤。在 3 例中，可见汗腺导管和腺体显著的黏液化生。其中 2 例汗腺导管呈现增生性改变。对既往发表资料及我们病例的分析表明，发生于器官样痣的皮脂腺癌女性多见，且倾向于发生在老年患者。它可累及皮脂腺痣通常出现的任何部位。临床上，肿瘤表现为孤立性结节、溃疡性肿瘤或肿块，通常近期有快速生长史。它可单独出现，但更常见于多种良性和恶性附属器肿瘤之中。该病变似乎与 Muir - Torre 综合征无关。尽管大的肿瘤可能过度生长并掩盖痣，但器官样痣的其余部分通常在临床和显微镜下均可识别。就临床行为而言，该肿瘤似乎是一种低级别癌。
