Department of Dermatology, University of Maryland School of Medicine, Baltimore, MD, USA.
Department of Dermatology, Veterans Affairs Medical Center, and State University of New York, New York, NY, USA.
Arch Dermatol Res. 2023 Oct;315(8):2195-2205. doi: 10.1007/s00403-023-02551-3. Epub 2023 Feb 15.
Basal cell carcinosarcoma (BCCS) is a rare malignant biphasic tumor of the skin, composed of epithelial and mesenchymal components, and may be underdiagnosed. We sought to summarize the current understanding of BCCS including its reported history, clinical presentation, diagnosis, and treatment. We also reappraise and present our recommendations of histological interpretation for its diagnosis and treatment. A systematic review of PubMed and EMBASE, from inception of databases to December 1, 2022, identified all reported cases of basal cell carcinosarcoma. A total of 34 reports containing 54 patients with basal cell carcinosarcoma were included. The neoplasm was most commonly associated in areas of sun-exposed skin and primarily affected the elderly. Diagnosis was made on histology specimens using H&E. To address underdiagnosis, additional immunohistochemical markers have been proposed due to unreliable phenotypic appearance in this poorly differentiated neoplasm. Treatment consists of excision of the tumor, typically with Mohs surgery, and is curative in most cases. There are limited treatment options for metastatic disease. There were limitations to this study as various immunohistochemical stains used on suspected BCCS without providing an explanation as to why certain markers were included and others were excluded. Continued efforts in characterizing this complex neoplasm are critical in establishing reliable and accurate diagnostic tests and accompanying treatment options, especially in cases of metastatic disease.
基底细胞癌肉瘤（BCCS）是一种罕见的皮肤恶性双相肿瘤，由上皮和间充质成分组成，可能诊断不足。我们旨在总结对 BCCS 的现有认识，包括其报告的历史、临床表现、诊断和治疗。我们还重新评估并提出了我们对其诊断和治疗的组织学解释建议。对 PubMed 和 EMBASE 进行了系统评价，从数据库建立到 2022 年 12 月 1 日，确定了所有报告的基底细胞癌肉瘤病例。共纳入 34 份报告，包含 54 例基底细胞癌肉瘤患者。该肿瘤最常与暴露于阳光的皮肤区域相关，主要影响老年人。通过 H&E 在组织学标本上做出诊断。为了解决诊断不足的问题，由于这种分化不良的肿瘤在表型上不可靠，因此提出了额外的免疫组织化学标志物。治疗包括切除肿瘤，通常采用 Mohs 手术，大多数情况下可治愈。对于转移性疾病，治疗选择有限。本研究存在一定局限性，因为对疑似 BCCS 使用了各种免疫组织化学染色，但没有解释为什么包括某些标志物而排除了其他标志物。继续努力描述这种复杂的肿瘤对于建立可靠和准确的诊断测试以及相应的治疗选择至关重要，特别是在转移性疾病的情况下。
