Basal cell carcinosarcoma: a systematic review and reappraisal of its challenges and the role of Mohs surgery

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.