Primary Cutaneous Carcinosarcoma: A Literature Review and a Report of Two Cases

Carcinosarcomas are rare malignant tumors with both epithelial and mesenchymal components, classified into visceral and cutaneous subtypes. Primary cutaneous carcinosarcoma (PCS) is exceptionally rare, and robust evidence to guide its management is lacking. This study aims to consolidate current knowledge on PCS through two new case reports and a comprehensive literature review, offering insights into its pathogenesis, clinical features, and management strategies. Two cases of PCS managed by a multidisciplinary team were reported, detailing clinical, histopathological, and immunohistochemical findings. A systematic literature review of PCS cases published between 2001 and 2021 was conducted, adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, and collating data on clinical presentation, histology, treatment, and outcomes. Literature analysis identified 74 patients across 42 case reports. Histopathology reveals a frequent epithelial component of squamous cell carcinoma (43.2%) or basal cell carcinoma (27.0%). Immunohistochemistry (IHC) aids diagnosis, with vimentin marking mesenchymal components. Surgical excision is the primary treatment; recurrence occurs in 16.2% of cases. Prognosis varies, with better outcomes linked to epithelial subtypes. PCS remains a diagnostic and therapeutic challenge due to its rarity and lack of standardized guidelines. This study highlights the importance of histopathology, IHC, and multidisciplinary management.