The Rarity in the Rarity: Presentation of Three Cases of Cutaneous Carcinosarcoma with Clinical and Histopathological Insights

first_page settings Order Article Reprints Open AccessCase Report The Rarity in the Rarity: Presentation of Three Cases of Cutaneous Carcinosarcoma with Clinical and Histopathological Insights by Gerardo Cazzato 1,* [ORCID] , Anna Colagrande 1 [ORCID] , Valentina Caputo 2 [ORCID] , Giuseppe Ingravallo 1 [ORCID] , Eliano Cascardi 1 [ORCID] , Francesco Fortarezza 3 [ORCID] , Emanuela Bonoldi 2 and Franco Rongioletti 4 [ORCID] 1 Section of Molecular Pathology, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J), University of Bari “Aldo Moro”, 70124 Bari, Italy 2 U.O. Anatomia Patologica, ASST Grande Ospedale Metropolitano Niguarda, 20162 Milan, Italy 3 Surgical Pathology and Cytopathology Unit, University Hospital of Padova, 35100 Padova, Italy 4 Dermatology Clinic, Vita-Salute San Raffaele University, 20132 Milan, Italy * Author to whom correspondence should be addressed. Dermatopathology 2024, 11(3), 209-217; https://doi.org/10.3390/dermatopathology11030022 Submission received: 31 May 2024 / Revised: 10 July 2024 / Accepted: 12 July 2024 / Published: 15 July 2024 Download keyboard_arrow_down Browse Figures Review Reports Versions Notes Abstract A cutaneous carcinosarcoma (cCS) is a rare and aggressive skin cancer characterized by both carcinomatous (epithelial) and sarcomatous (mesenchymal) components, making it a biphasic tumor. Despite its occurrence in various organs, a cCS is exceptionally rare in the skin, predominantly affecting older males. The etiology of a cCS is unclear, but it may originate from a single progenitor cell capable of dual differentiation or from a collision of carcinoma and sarcoma cells. Clinically, a cCS presents as a rapidly growing, painful, ulcerated nodule or plaque on sun-exposed skin, with a high risk of local invasion and metastasis. Histopathologically, a cCS includes various epithelial components, such as squamous cell carcinoma and basal cell carcinoma, along with undifferentiated sarcomatous components resembling atypical fibroxanthoma. The tumor may also exhibit heterologous differentiation like angiosarcomatous or rhabdomyosarcomatous features. We present three cases of a cCS, highlighting their clinical and histological characteristics and comparing them with previously reported cases. Understanding a cCS is complicated by its rarity and diverse presentation, emphasizing the need for further research to elucidate its pathogenesis and optimal management.